Hypospadias Surgery An Illustrated Textbook (Second Edition) Ahmed T. Hadidi 2022 WOPS

Hypospadias
Surgery
An Illustrated Textbook
Ahmed T. Hadidi
Editor
Second Edition
123
Hypospadias Surgery
Ahmed T. Hadidi
Editor
Hypospadias Surgery
An Illustrated Textbook
Second Edition
Editor
Ahmed T. Hadidi
Hypospadias Center and Pediatric Surgery Department
Sana Klinikum Offenbach, Academic Teaching Hospital
of the Johann Wolfgang Goethe University
Offenbach, Frankfurt
Germany
ISBN 978-3-030-94247-2 ISBN 978-3-030-94248-9 (eBook)

https://doi.org/10.1007/978-3-030-94248-9
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To
my Sons, Taher and Helmy,
may God bless them and make them content with their lives
my Wife, Petra,
for her everlasting love, continuous support, and
encouragement
my Father, Taher,
for leading his children into intellectual pursuits
my Mother, Mawaheb,
for devoting her life to her children
my Uncle, Helmy,
for setting an outstanding example to follow
my Professors in Cairo University: Adel Loutfi and Nabhan

Kaddah,
for showing me the excitement and joy of Pediatric Surgery
my Mentors: Dan Young in Glasgow and John Duckett in

Philadelphia,
for paving the way for international recognition
all my children and their parents all over the world.

for teaching me and trusting me to try to help them to lead a
normal life
Ahmed T. Hadidi
Foreword to the Second Edition
Two great pioneers of pediatric urology, Sir David Innes Williams (1919–
2013) and Edward Durham Smith, each wrote a foreword to the first edition
of this book in 2004. Both felt that hypospadias was a complex condition,
poorly understood and difficult to treat. There was, therefore, a great need to
have a book, compiled by the experts of the time, covering all of the known
materials. Durham Smith wrote:
I know of no surgery that can quite humble a surgeon as much as hypospadias.
When I re-read these forewords, I felt that both could be transposed to the
present edition, and they would be as valid now as they were 18 years ago.
Why, then, is it necessary to have another edition?
The answer is very easy. Correction of hypospadias remains challenging,
but many of the unknowns highlighted in the first edition have been solved,
particularly things that should not be done based on new knowledge of anat-
omy and physiology. Surgical equipment and techniques have changed. The
importance of the psychological aspects is better understood.
Perhaps most importantly, parental and patient expectations have changed.
Criteria of success are better defined; assessment of success now involves
objectivity and measurement. The statement in many surgical papers, not
only those on hypospadias, that “the results and complications were satisfac-
tory” is not acceptable, especially without stating to whom they were satis-
factory. Most often it was to the satisfaction of the surgeon, rather than to the
patient.
Many of the “known unknowns” (Donald Rumsfeld during Department of
Defense News Briefing, archive.defense.gov. February 12, 2002) from 2004
have now been resolved. Unfortunately, new unknowns have replaced them.
The literature continues to expand. In PubMed, the research term
“Hypospadias” gives 3920 papers since 2004 and even with a filter for 2020
alone, produces 347 entries—nearly one every day.
Outcome analyses in many surgical specialties, including urology, have
shown that success is directly related to the number of cases performed by the
surgeon. This has led many units to insist on sub-specialization. Indeed, in
some countries, certain conditions may only be managed in units with a
defined minimum case load. When the present author entered medical school
in 1965, the most admired surgeons were those who “specialized in the skin
and its contents.” Today, this is not grounds for admiration. With the best
interests of the patients in mind, surgeons must have the humility to stick to
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viii Foreword to the Second Edition
the operations on which they are properly trained and have a case load to
maintain their skills.
This brings us to the justification for a second edition. Much has been
discovered since 2004. Much has changed. Some problems have been solved.
However, very much more needs to be done. Even the most dedicated reader
of current literature could not keep up with the new papers that appear daily.
The first edition is out of date. The present one provides all of the present
knowledge and is an essential guide for the present and the future.
Baron Christopher Woodhouse

Emeritus Professor of Adolescent
Urology University College London
London, UK
April, 2022
Foreword to the Second Edition
However beautiful the strategy, you should occasionally look at the results.
Winston Churchill
This message cautions that one should not be so infatuated with the beauty
of one’s design such that one fails to evaluate the outcomes. As has often been
stated, in a surgical discipline with well over 200 described techniques, none
can be universally applied or universally successful. In fact, the only uni-
formly successful hypospadias repair was that described by the Greek physi-
cians Heliodorus and Antyllus, which was amputation of the penis distal to
the hypospadiac meatus.
The “bible” of genital surgery during my residency was written by a plas-
tic surgeon, Charles Horton. It was entitled Plastic and Reconstructive
Surgery of the Genital Area (1973). Dr. Horton was a gifted surgeon, how-
ever, he realized that surgery of the genitalia in children and particularly
hypospadias, required expertise and techniques from across disciplines. He
partnered with a urology colleague, Charles Devine, to bring together the two
specialties in developing philosophies and techniques for correction of geni-
tal surgical anomalies. During my fellowship in Philadelphia, John Duckett
guest edited an issue of the Urology Clinics of North of North America (1981)
on hypospadias, in which he invited international experts to describe their
favorite hypospadias repair. Here, he coined the term “hypospadiology,” sig-
nifying a distinct subject of study. This certainly holds true, as evidenced by
the considerable amount of literature dedicated to this anomaly. As John
pointed out in 1981, truly original contributions to hypospadias repair are
rare; however, our literature is replete with modifications and alteration of
techniques, in quest of the perfect result.
To me, a successful hypospadiologist is one with a detailed understanding
of the anatomy, meticulous surgical technique, and an appreciation for art-
istry. One must be flexible in the operating room and never be bound to a
preoperatively determined technique, as during the course of a repair ana-
tomical minefields are common. It is not sufficient to be facile with a couple
of repairs, but instead be familiar with many different techniques as well as
their various modifications, alterations, and nuances. A robust armamentar-
ium is necessary to approach the multiple variances and complications of
hypospadias. I often tell trainees and medical students that
hypospadias is often what causes a pediatric urologist to become a pediatric urolo-
gist, and what causes an adult urologist to stay in adult urology.
ix
x Foreword to the Second Edition
Mastery of hypospadiology is a lifelong pursuit and is best done with

patience, persistence, and humility. Any surgeon who embarks on a career in
hypospadias surgery must accept the consequences of failure. At times fail-
ures present early in the postoperative course; however, others may not pres-
ent until puberty or into adulthood. In this work, particular attention is given
to a humbling list of postoperative complications, each presented in detail.
Professor Hadidi, a true pioneer in the science of hypospadias, has brought
together a group of international contributors to this compendium, amassing
a large breath of experience and opinions, techniques, and tricks. Our knowl-
edge of hypospadias embryology and pathogenesis, as well as treatment and
outcomes of hypospadias surgery, has been growing at a rapid rate. Compiling
the latest information together in this book will provide a valued resource for
our specialty and a lasting reference for the novice as well as the seasoned
hypospadiologist.
Anthony A. Caldamone
Editor-in-Chief, The Journal of Pediatric Urology
Professor of Urology and Pediatrics
Hasbro Children’s Hospital Providence
RI, USA
April, 2022
Preface to the Second Edition
A specialist is the one who knows more and more about less and less.
Charles Mayo, 1865–1939
Everybody is against specialization except the patient.

Francis More, 1913–2001
Hypospadias is a grievous deformity which must ever move us to the highest surgi-
cal endeavour. The refashioning of the urethra offers a problem as formidable as any
in the wide field of our art. The fruits of success are beyond rubies—the gratitude of
a boy has to be experienced to be believed.
T. Twistington Higgins, London, 1941
The purpose of this well-illustrated textbook is to provide a comprehen-

sive reference that contains the current knowledge on hypospadias from
embryology, anatomy, morphology, and pathogenesis to all available surgical
techniques and their results, long-term follow-up, and complications. The
aim is to help interested surgeons and trainees to have a strong basic science
background, understand the principles of available surgical techniques,
develop vision, talents, and philosophies, rather than just parrot enumerate
techniques. We aim to provide an up-to-date global view of the management
of children with hypospadias, focusing primarily on the treatment of the con-
dition and its associated problems. This book will be primarily of interest to
pediatric and adult urologists, pediatric and general surgeons, and plastic
surgeons.
The book contains four parts, part I deals with basic science, including the
history, embryology, anatomy, morphology, epidemiology, genetics, and
other important scientific matters that are usually overlooked by surgeons.
Part II describes in detail all operations for correction of hypospadias includ-
ing those that are more old-fashioned and are not used today. The idea is to
provide a reference for future generations. Part III deals with possible com-
plications and how to handle them. No surgeon should touch a patient with
hypospadias until he knows how to manage possible complications. Part IV
focuses on long-term follow-up, how to write a paper on hypospadias, future
research, training, and the establishment of hypospadias centers in different
parts of the world.
Change is the only permanent thing in life. When the decision was made
to write a second edition of the hypospadias surgery book, it was not antici-
pated that about 95% of the contents of the second edition would be new
information. Although we already had a vast experience in hypospadias sur-
xi
xii Preface to the Second Edition
gery with satisfactory results, we could not imagine that our knowledge,
understanding, and surgical techniques would change so much.
This reminds me of the story of the president of the patent office in the
USA at the end of the nineteenth century. He proposed permanently closing
the patent office in the USA as whatever could be discovered or invented had
already been done, and he could not imagine there would be anything new in
the twentieth century to discover or invent. We all know that the number of
inventions and discoveries in the twentieth century were more than what were
invented or discovered during the previous 19 centuries put together.
The original plan was to have the second edition out by 2008. However, when
we reviewed some of the chapters, particularly the basic science chapters, it
became clear that some of the standard information in the literature did not fit
with what we see in clinical practice. This prompted extensive basic research
and extensive literature research in the fields of history, embryology, anatomy,
and pathology. We had to wait until the results had final approval by peer review-
ers and were published before we could proceed with the second edition.
During the last decade, not only has there been new insight and better under-
standing of basic science, but long-term follow-up has prompted modifications
of current techniques to improve the final functional and cosmetic results.
The book is a perfect example of international cooperation. Thirty nine
experts from 19 countries residing in six continents have contributed to this
work. Each author is an authority in the field of hypospadias. A team of world
class contributors presented their expert opinions. The authors are members
of major urological and pediatric surgical associations in North and South
America, Europe, Africa, Asia, and Australia. I would like to express my
sincere thanks and deep gratitude to all contributors for the time and hard
work spent in preparing and writing their chapters and for participating in the
development of this book.
There are still a lot of “unknown unknowns,” the current theories of ure-
thral development and pathogenesis of hypospadias do not explain the clini-
cally different presentations such as chordee, torsion, different grooving of
the glans, or differing degrees of development of the urethral plate. There still
remains a “virgin” field for future research and studies. It is up to the new
generation to conduct joint research with different international centers and
diverse scientific disciplines to unravel the existing mysteries.
How many stages do we need to correct complex proximal forms of hypo-
spadias with severe chordee? The pendulum has swung from three stages in
the nineteenth century to one stage in the1970s and 1980s, to two stages, even
“one and half stages” in the twenty-first century. It has become clear that it is
not important how many stages the surgeon uses as long as he reaches his
goal of offering the child a good functioning and esthetically pleasing penis.
We have passed through an era in the 1990s and the early twenty-first century
where the focus was more on cosmesis and having a slit-like meatus while
ignoring function, resulting in a higher incidence of complications with some
of the newer techniques.
Old is Gold: we have also witnessed the revival of old techniques with
modifications to meet the expectations of the children and their parents. The
Thiersch-Duplay technique has been revised with a protective intermediate
Preface to the Second Edition xiii
layer to reduce fistulae incidence and preoperative hormone therapy to enlarge

the glans. The Mathieu technique has also been revised with the Slit-like
adjusted Mathieu technique (SLAM) to have a slit-like meatus at the tip of the
glans and a three-layer closure to minimize the incidence of complications.
Although hypospadias is the commonest anomaly affecting boys after
undescended testis, it is still a rather taboo subject. The public know very
little about it and most families with children with hypospadias go to a sur-
geon for correction and put it behind their backs. In reviewing the medical
literature when trying to write a detailed and accurate description about the
penis, one finds a lot written about the female genital system, some about the
testis but very little about the penis.
There have also been other major changes from a personal perspective.
God blessed me with a loving caring wife, Petra and two sons Taher and
Helmy. On the other hand, my mentors and Professor Dan Young of Glasgow,
Professor Nabhan Kaddah of Cairo, my co-editor Mr. Amir Azmy, Professor
Sava Perovic, Professor Howard Snyder, and my illustration artist Mr. Reinold
Henkel have left us, to a better world.
Several new chapters took years to complete (e.g., the Embryology and
Chordee Chapters).
The policy of the editor, as it was in the first edition, is that each author is
completely responsible for his chapter. In some chapters, the editor has added
comment to help the reader to “see the other face of the coin.”
Please allow me to share with you the Three Stages of Learning:
Stage I: READ what others say

Stage II: REPEAT what others say
Stage III: QUESTION what others say!
Following this rule, I have realized that several things we learn in

Embryology and Anatomy cannot be true. This has initiated new scientific
research in many fields to clarify the facts and hence the chapters of
Embryology, Anatomy, and even the chapter on the History of Hypospadias
are completely new. I would urge the reader of the book to apply this rule of
learning whenever reading any scientific publication (including this book of
course), and always question what is written until he is satisfied that it is sci-
entifically sound and true.
Another important point is that the surgeon should always ask himself or
herself: How can I improve my results? How can I increase the satisfaction of
my patients? How can I reduce the discomfort of my patients after hypospa-
dias surgery? In other words, there should always be new modifications of
hypospadias techniques and surgery. The moment we stop improving our
approach is the moment we should stop operating.
In 1951, D. M. Davis wrote:
I would like to say that I believe the time has arrived to state that the surgical repair
of hypospadias is no longer dubious, unreliable, or even extremely difficult. If tried
and proven methods are scrupulously followed, a good result should be obtained in
xiv Preface to the Second Edition
every case. Anything less than this suggests that the surgeon is not temperamentally
fitted for this kind of surgery.
I have had the privilege of operating on more than 10,000 hypospadias

patients over the past 35 years (60–70 hypospadias patients every month dur-
ing the past 10 years). This experience has made me an advocate of what the
Australian surgeon Durham Smith wrote half a century later in 2004:
I know of no surgery that can quite humble a surgeon as much as hypospadias—it

carries a vicious bite with its list of complications, most galling when they can occur
years later.
Reconstruction of a new urethra is like building a house: the surgeon

must use the appropriate raw material otherwise the house will collapse. Each
hypospadias is unique with a wide range of hypoplasia, dysgenesis of the
corpus spongiosum, and other ventral structures of the penis. The hypospadi-
ologist needs to tailor his/her technique according to the degree of hypoplasia
and the availability of healthy, well-vascularized tissue to reconstruct the new
urethra.
I am truly grateful to my wife, Petra Hadidi, for her continuous support,
who in addition to her commitment to her family and children, took over the
impossible task of editing, organizing, and bringing this book to its final form.
My thanks are also due to Mrs. Kate Moneer Hanna for reviewing and
editing several chapters in the book. I would also like to thank Mr. Ayhan
Yazgan, the art designer who kindly agreed to finish the remaining figures in
the same style after the unfortunate death of my good friend, Mr. Reinhold
Henkel. Mr. Henkel was the art designer who did all the drawings of the first
edition and most of the drawings in the second edition. He was an amazing
man, very dedicated to his work, who spent a lot of time perfecting each and
every drawing in the book.
This book is the result of the hard work of many contributors with about
35 years of experience in the field of hypospadias. It is hoped that it will be
helpful for the present generation and for many generations to come.
The froth will fade away but what is useful for mankind, will remain on Earth.
The Holy Koran, Thunder Chapter, Item 17
Offenbach, Frankfurt, Germany Ahmed T. Hadidi

Preface to the First Edition, 2004
The purpose of this comprehensive, well-illustrated textbook is to help inter-

ested surgeons to develop vision, philosophy, and talent, rather than just enu-
merate techniques. In this way, we aim to provide an up-to-date global view
of the management of the child with hypospadias, focusing primarily on the
care of the condition and its associated problems. The book will be of specific
interest to pediatric and adult urologists, pediatric and general surgeons, and
plastic surgeons.
The book is an example of international cooperation. Forty-six experts
from 12 countries on five continents have contributed to it.
Each of the chapters in the book is written by an authority in the field of
hypospadiology. A team of world class contributors present their expert opin-
ions. The authors are members of major urological and pediatric surgical
associations in the USA, the UK, Australia, Japan, and Europe.
Some of the chapters are written by non-surgeons and present new and
important knowledge concerning epidemiology, genetics, and child
psychology.
The book is well illustrated with ample color diagrams and photographs of
various operations together with many technical tips. There is a wide diver-
sity of opinion, and a large number of operations are described. Information
is occasionally intentionally repeated when the circumstances demand.
The book consists of three sections.
Section 1 contains chapters dealing with the historical perspective: the
evolution of hypospadias surgery, the basic principles of repair, the detailed
embryology and anatomical studies, the epidemiology and genetics of hypo-
spadias. Further contributions deal with general principles, including exami-
nation, imaging, intersexuality, preoperative preparation and hormonal
stimulation, assessment of the results of hypospadias surgery, principles of
plastic surgery, and general principles of hypospadias surgery. Sutures, stent-
ing materials, and dressings are described, and a comprehensive chapter cov-
ers pain control.
Section 2 includes chapters describing the operative techniques for correc-
tion of the proximal and distal types of hypospadias. There is emphasis on the
use of single-stage repair in severe types of hypospadias. However, the need
for two-stage repair is discussed in detail. Current controversial issues such
as the relative merits of single- versus two-stage repair, flaps versus grafts,
dressings, and urinary diversion are debated in depth.
xv
xvi Preface to the First Edition, 2004
Section 3 deals with the complications of hypospadias surgery and their

management, complex redo repairs, long-term results in adolescence, and
psychological adjustments.
Lastly, the editors present their personal overview of the current surgical
techniques and potential developments in hypospadias surgery.
In addition, the text features a glossary for any readers who may not be
familiar with the meanings of certain terms, as well as an appendix clarifying
commonly used abbreviations.
The editors wish to thank all the contributing authors for the time and the
hard work expended in producing their chapters and participating in the
development of this textbook.
Our deep appreciation and special thanks go to Reinhold Henkel, art
designer working with Springer-Verlag, for his major contribution in design-
ing all the illustrations within the book. We are also indebted to Gabriele
Schroeder, Executive Medical Editor, Stephanie Benko, David Roseveare,
and Ingrid Haas of Springer-Verlag for their constructive criticism, help, and
advice.
Cairo, Egypt Ahmed T. Hadidi

Yorkhill, Glasgow, UK Amir Fawzy Azmy
Foreword to the First Edition, 2004
The challenge of hypospadias correction has always tested the ingenuity of

surgeons and, although many of the older methods have been discredited and
discarded, the diversity of procedures in current practice suggests that the
perfect operation has yet to be devised. Evidently, there have been important
advances during the past 30 years, and the results now obtained would be the
envy of older surgeons. It is instructive to note that particular attention is now
given to securing a meatus at the tip of the glans (even in those minor degrees
of hypospadias which would formerly have been left untreated), reflecting the
pursuit of physical perfection which now fuels the popularity of cosmetic
surgery.
There was undoubtedly a need for a comprehensive text dealing with all
aspects of the abnormal development and its consequences as well as giving
guidance on methods of surgical correction. The editorial duo of Hadidi and
Azmy are to be congratulated on assembling a distinguished and expert team
to fulfill this requirement. This volume will be indispensable reading for all
those called upon to treat hypospadias, but it is gratifying to see that although
the techniques of plastic surgery have contributed to the newer methods, it is
the pediatric urologists who are now principally concerned. Hopefully, they
will ensure that hypospadias is fully corrected early in life and that adult
urologists will no longer be asked to rescue sadly mismanaged young men
with serious sexual and psychological handicaps.
Sir David Innes Williams

Founder of Pediatric Urology
Great Ormond Street Hospitals
London, UK
January, 2004
xvii
Foreword to the First Edition, 2004
An illness or abnormality that attracts a continuing bibliography of many

hundreds of journal articles implies that there are unresolved problems. Such
is the situation in hypospadias. And yet, at first glance, this is very surprising.
Here is a deformity in an organ so overtly recognizable for clinical and epide-
miological study, so easily accessible for tissue histology and histochemistry,
and so amenable, one could assume, to the simplest of plastic surgical repair,
given that there is total surgical access and exposure, surrounded by ample
available tissues, and with a rich blood supply for healing; it should be a
“gift” for even moderate surgical prowess! Yet nothing could be further from
the truth.
In almost every facet, we lack complete understanding. We have gaps in
genetic information; the embryology is unsettled as to the nature of the tissue
organizers, the relative contributions of the ectodermal folds and mesothe-
lium, or whether the deformity is not “developmental” at all, but rather, as
Douglas Stephens asserts, the result of distortion and displacement of early
fetal cells by external pressure; why the great variation in clinical types, for
example, variations in orifice size and site and degree of chordee, or a large
penile shaft orifice but no chordee, or a coronal orifice with complete pre-
puce, or a normally sited terminal orifice but marked chordee? We have
incomplete understanding of the pathology of chordee, from the simplistic
and probably mythical “white band” of Denis Browne to the varying contri-
butions of skin, fascial layers, and corpora, and what determines their contri-
butions. The confusion is even more marked in the surgical management.
With a lesion in which over 200 different repairs have been described, a cynic
may be excused in claiming that none can be very satisfactory! We have pro-
ceeded through Browne repairs and scrotal flaps, to Duplay tubes, free skin
grafts, island flaps and onlays, bladder and buccal mucosal repairs, a host of
single-stage innovations, to different concepts of chordee correction, and
with all manner of bladder drainage systems. Although the penile repairs can
be grouped into five or six major principles, depending on the tissues used,
each has been subject to countless variations as one surgeon after another
adds yet another modification to an already thrice-modified variation of a
procedure adapted from a principle derived from the original! Further, some
repairs are successful only in the hands of the original proponent, and the
subtleties escape the novice, who, in frustration, flips to yet another tech-
nique. Hypospadias is bedeviled by surgeons who swap to the latest gimmick
and never acquire the expertise of a practiced technique, which is essential for
xix
xx Foreword to the First Edition, 2004
good results. We still debate the place of one- or two-stage repairs. Some
repairs frankly offend major surgical principles in their complexity, the fash-
ioning of flaps, disrespect for blood supply, and in inadequate layers and ten-
sion. What are the indications to use a specific repair in a given clinical
situation? Do we need ten different procedures for ten different situations,
with the attendant risk of insufficient volume of experience to acquire the
necessary expertise in any of them, or can we reduce these to a manageable
and well-practiced two or three adequate techniques with appropriate adapta-
tions? In what circumstances is urinary diversion required and by means of
which technique?
The problems do not stop with the completion of apparently successful
surgery. I know of no surgery that can quite humble a surgeon as much as
hypospadias—it carries a vicious bite with its list of complications, most
galling when they can occur years later, such as a very late fistula or the
manifestation of recurrent chordee. Add to this list, problems of ejaculation
or sexual function and the psychological effects before and after surgery or in
later life, we have a lesion to test the ingenuity, inventiveness, and resilience
of surgeons as much as any in surgery.
These are just some of the unresolved problems in the surgery of hypospa-
dias. Given this maelstrom, there could be no better time for the compilation
of a volume bringing together the very best authorities in a critical analysis of
all aspects of the malformation. Under the stimulating editorship of Ahmed
Hadidi and Amir Azmy, themselves leaders in this field, the contributions of
an international faculty of world leaders have been assembled. As a perusal of
the table of contents will show, the book covers all the major operative repairs,
described in many cases by their originators, but the book is much more than
a collection of techniques. It rightly also covers the general aspects of history,
epidemiology, genetics, embryology, and pathology; surgical principles,
ancillary aids, and anesthesia; the long-term evaluation of results, including
early and late complications; and, importantly, an overview of management
to bring perspective to this complex lesion.
In a subject with reports spread so widely throughout a vast list of publica-
tions, the book is a major contribution to the effort to reduce to manageable
proportions the current accumulated knowledge of hypospadias, with each
area being assessed and analyzed by experts. We surely need such a volume.
I have every expectation that the breadth of cover and the quality of the con-
tributors are such that our puzzling unknowns will be addressed, and sur-
geons will have at hand an authoritative guide to practical management.
E. Durham Smith
Royal Children’s Hospital
Melbourne, VIC, Australia
January, 2004
Art Designers Acknowledgement
We would like to acknowledge the late art designer Herr Reinhold Henkel for
providing the following figures for the second edition:
Figs. 1.6, 1.7, 1.9, 1.10, 1.15, 1.17, 2.5e, 2.6e, 2.7d, 2.8g, 2.10f, 2.10g, 3.2,
3.3, 3.24, 3.25, 3.27, 3.29, 3.30a, 3.32, 3.34, 3.35, 3.40, 3.43, 3.44, 4.1,
4.2, 4.3, 4.5, 4.6, 4.7, 4.8, 4.11, 4.12, 5.3, 6.4, 6.7, 6.14a, 6.20a, 7.1, 7.2,
7.7, 7.9, 7.11, 7.17, 7.22, 7.26a, 7.28, 8.10, 8.11, 8.20, 8.25, 8.26, 8.33,
8.35, 8.39, 8.44, 8.46, 8.48, 8.50, 8.52, 9.2, 15.1, 19.3, 21.1, 22.1, 24.1,
25.1, 25.2, 25.3, 25.4, 30.1, 32.1, 33.1, 34.3, 34.4, 38.2, 38.3, 38.4, 39.3,
42.2, 42.3, 42.6, 42.7, 42.8, 42.10, 44.1, 44.2, 44.7, 44.9, 44.17, 44.18,
44.21, 44.24, 44.25, 44.26, 44.27, 45.1, 45.2, 45.6, 46.1, 46.3, 46.5, 47.1a,
49.1, 58.8, 58.10, 59.4, 62.3, 63.5
We would like also to acknowledge the art designer Herr Ayhan Yazgan for
providing the following figures for the second edition:
Figs. 9.4, 9.5, 9.6, 27.2, 47.1
xxi
Contents
Part I General and Basic Science
1 History of Hypospadias �� 3

Ahmed T. Hadidi
2 Men Behind Principles and Principles Behind Techniques�� 25
Ahmed T. Hadidi
3 Normal Development of the Penis and Urethra�� 65
Ahmed T. Hadidi
4 Surgical Anatomy of the Penis and Urethra�� 105
Ahmed T. Hadidi
5 Altered Development of Prepuce in Hypospadias
and Its Clinical Relevance �� 127
Ibrahim Ulman
6 Morphology of Hypospadias �� 137
Ahmed T. Hadidi
7 Pathogenesis of Hypospadias:
The Disorganization Hypothesis�� 163
Ahmed T. Hadidi
8 The Urethral Plate and Chordee�� 185
Ahmed T. Hadidi
9 Classification and Assessment of Hypospadias �� 237
Ahmed T. Hadidi
10 Epidemiology of Hypospadias�� 249
Loes F. M. van der Zanden, Iris A. L. M. van Rooij,
and Nel Roeleveld
11 Genetic Aspects of Hypospadias �� 271
Loes F. M. van der Zanden
12 Hormones and Growth of the Genital Tubercle�� 285
Mohamed Fawzy and Ahmed T. Hadidi
xxiii
xxiv Contents
13 The Role of Preoperative Androgen Stimulation in the

Management of Hypospadias�� 299
Fardod O’Kelly and Luis H. Braga
14 Timing of Surgery�� 305
Ahmed T. Hadidi
15 General Principles�� 309
Ahmed T. Hadidi
16 Plastic Surgery Principles �� 323
Ahmed T. Hadidi
17 Principles of Hypospadias Surgery�� 335
Ahmed T. Hadidi
18 Analgesia and Pain Control�� 347
Günther Federolf
Part II Operative Techniques
19 Grade I: Glanular Hypospadias;

Double Y Glanulomeatoplasty (DYG) Technique �� 361
Ahmed T. Hadidi
20 MAGPI and Modified MAGPI�� 371
Heather Di Carlo and John P. Gearhart
21 The Meatal-Based Flap Principle �� 377
Ahmed T. Hadidi
22 The Slit-Like Adjusted Mathieu (SLAM) Technique�� 391
Ahmed T. Hadidi
23 Megameatus Intact Prepuce (MIP) Deformity�� 401
Ahmed T. Hadidi
24 Thiersch-Duplay Principle�� 411
Moneer K. Hanna and Mark R. Zaontz
25 The “Inverted-Y Thiersch” (IT) Technique�� 425
Ahmed T. Hadidi
26 Incision of the Urethral Plate�� 433
Mark Rich
27 Dorsal Inlay TIP (DTIP)�� 451
Tariq O. Abbas and Joao Luiz Pippi Salle
28 Urethral Advancement for Treatment of Distal Hypospadias �� 461
Emir Q. Haxhija and Patrick McKenna
29 The Glanular Urethral Disassembly (GUD) Technique:
An Alternative to Distal Hypospadias�� 471
Antonio Macedo Jr. and Marcela Leal da Cruz
Contents xxv
30 Proximal Hypospadias With Small Flat Glans:

The Lateral-Based Onlay (LABO) Flap Technique�� 479
Ahmed T. Hadidi
31 The Onlay Island Hypospadias Repair�� 489
Howard M. Snyder III
32 Perineal Hypospadias: The Bilateral-Based (BILAB)
Skin Flap Technique�� 497
Ahmed T. Hadidi
33 Chordee Excision and Distal Urethroplasty (CEDU)
for Perineal Hypospadias�� 507
Ahmed T. Hadidi
34 Preputial Island Flaps�� 523
Minu Bajpai and Apoorv Singh
35 The Modified Asopa (Hodgson XX): The Procedure
to Repair Hypospadias with Chordee�� 533
Jeffrey Wacksman
36 Koyanagi Technique and Its Modifications in the Management
of Proximal Hypospadias�� 537
Adham Elsaied
37 The Yoke Hypospadias Repair�� 551
Brent W. Snow
38 Dorsal Longitudinal Penile Skin Island Flap in One-Stage
Repair of Hypospadias with Penoscrotal Transposition�� 557
Rados Djinovic and Sava V. Perovic
39 Grafts for One-Stage Repair�� 565
Ahmed T. Hadidi
40 Two-Stage Graft Urethroplasty; Free Full-Thickness
Wolfe Graft �� 579
Ahmed T. Hadidi
41 The Cecil-Culp Technique�� 593
Dana A. Weiss and Douglas A. Canning
42 The Foreskin and Circumcision�� 603
Ahmed T. Hadidi
43 Protective Intermediate Layer�� 619
Ahmed T. Hadidi
44 Procedures to Improve the Appearance of the Meatus
and Glans�� 631
Ahmed T. Hadidi
45 Buried Penis (BP) �� 665
Ahmed T. Hadidi
xxvi Contents
46 Penile Torsion (PT)�� 677

Ahmed T. Hadidi
47 Congenital Urethral Duplication�� 685
Grahame H. H. Smith
48 Enlarged Prostatic Utricle Associated to Hypospadias�� 697
Mario Lima and Michela Maffi
49 Penoscrotal Transposition �� 705
Ahmed T. Hadidi
50 Uncommon Conditions and Complications �� 715
Ahmed T. Hadidi
51 Hypospadias Surgery in Adults�� 729
Ahmed T. Hadidi
52 Flaps Versus Grafts�� 739
Michael Sennert and Ahmed T. Hadidi
53 Single-Stage Versus Two-Stage Repair�� 747
Ahmed T. Hadidi
54 Stenting Versus No Stenting�� 757
Ahmed T. Hadidi
55 Dressing Versus No Dressing�� 761
Ahmed T. Hadidi
56 Editorial Overview of the Current Management
of Hypospadias �� 765
Ahmed T. Hadidi
Part III Complications
57 Early Complications�� 771

Ahmed T. Hadidi
58 Meatal Stenosis and Urethral Strictures�� 777
Ahmed T. Hadidi
59 Functional Urethral Obstruction (FUO)�� 791
Ahmed T. Hadidi
60 Hypospadias Fistula�� 799
Ahmed T. Hadidi
61 Genital Lichen Sclerosus
(Balanitis Xerotica Obliterans): BXO�� 813
Christiane Schulze and Alexander Springer
62 Urethral Diverticula and Acquired Megalourethra�� 827
Ahmed T. Hadidi
Contents xxvii
63 Management of Failed Hypospadias Surgery �� 835

Ahmed T. Hadidi
64 Complex and Redo Hypospadias Repairs: Management
of 402 Patients�� 855
Moneer K. Hanna
Part IV Long-Term Results
65 Long-Term Consequences of Hypospadias Repair �� 879

Christopher Woodhouse
66 Long-Term Follow-Up in Hypospadias Repair: What Is
It and Are We There Yet?�� 891
Christopher J. Long
67 Hypospadias: Psychosocial and Sexual Development
and Consequences�� 897
Anne-Françoise Spinoit and Yakov Reisman
68 Tissue Engineering and Future Frontiers�� 907
Magdalena Fossum
69 Systematic Steps on How to Write a Scientific Paper
on Hypospadias�� 919
Luis H. Braga
70 Hypospadias Centers, Training,
and Hypospadias Diploma�� 929
Johannes Wirmer and Ahmed T. Hadidi
Index�� 933
Contributors
Tariq O. Abbas Division of Urology, Department of Pediatric Surgery,

Sidra Medicine, Sidra Medical and Research Centre, Doha, Qatar
Minu Bajpai Department of Paediatric Surgery, All India Institute of
Medical Sciences, New Delhi, India
Luis H. Braga Division of Urology, Department of Surgery, McMaster
Children’s Hospital, McMaster University, Hamilton, Ontario, Canada
Anthony A. Caldamone, MD, MMS, FACS, FAAP Editor-in-Chief, The
Journal of Pediatric Urology, Professor of Urology and Pediatrics, Hasbro
Children’s Hospital, The Warren Alpert School of Medicine, Brown
University, RI, USA
Douglas A. Canning Division of Urology, Perelman School of Medicine,
Children’s Hospital of Philadelphia, University of Pennsylvania, Philadelphia,
PA, USA
Heather Di Carlo Pediatric Urology, Brady Urological Institute, The Johns
Hopkins Hospital, Baltimore, MD, USA
Marcela Leal da Cruz Urology Department, AACD Hospital, Sao Paolo
Federal University, São Paulo, Brazil
Rados Djinovic Sava Perovic Foundation, Certified Center of Reconstructive
Urology, Belgrade, Serbia
Adham Elsaied Pediatric Surgery Department, Mansoura University,
Mansoura, Egypt
Mohamed Fawzy Hypospadias Center and Pediatric Surgery Department,
Sana Klinikum Offenbach, Academic Teaching Hospital of the Johann
Wolfgang Goethe University, Offenbach, Frankfurt, Germany
Günther Federolf Pediatric Anaesthesia Unit, Sana Klinikum Offenbach
GmbH, Offenbach am Main, Germany
Magdalena Fossum Department of Pediatric Surgery, Copenhagen
University Hospital, Rigshospitalet, Copenhagen, Denmark
Laboratory of Tissue Engineering, Rigshospitalet, Faculty of Health and
Medical Sciences, University of Copenhagen, Copenhagen, Denmark
Department of Women’s and Children’s Health, Laboratory of Tissue
Engineering, Karolinska Institutet, Stockholm, Sweden
xxix
xxx Contributors
John P. Gearhart Pediatric Urology, Brady Urological Institute, The Johns

Hopkins Hospital, Baltimore, MD, USA
Ahmed T. Hadidi Hypospadias Center and Pediatric Surgery Department,
Moneer K. Hanna Institute of Pediatric Urology, New York Weill Cornell
College, New York Hospital-Cornell University, New York, NY, USA
Emir Q. Haxhija Department of Pediatric and Adolescent Surgery, Medical
University Graz, Graz, Austria
Mario Lima Pediatric Surgery Department, IRCCS Sant’Orsola Polyclinic,
Alma Mater Studiorum - University of Bologna, Bologna, Italy
Christopher J. Long Perelman School of Medicine, University of
Pennsylvania, Children’s Hospital of Pennsylvania, Philadelphia, PA, USA
Antonio Macedo Jr Urology Department, AACD Hospital, Sao Paolo
Federal University, São Paulo, Brazil
Michela Maffi Pediatric Surgery Department, IRCCS Sant’Orsola
Polyclinic, Alma Mater Studiorum - University of Bologna, Bologna, Italy
Patrick McKenna, MD, FAAP, FACS Department of Mercyhealth
Hypospadias, Center Javon Bea Womens and Children’s Hospital, President
Societies for Pediatric Urology, Rockford, IL, USA
Fardod O’Kelly Departments of Urology and Paediatric Surgery, Beacon
Hospital and UCD School of Medicine and Medical Sciences, Dublin, Ireland
Sava V. Perovic Sava Perovic Foundation, Certified Center of Reconstructive
Urology, Belgrade, Serbia
Yakov Reisman, MD, PhD, FECS Sexologist, Andrologist, European
Society for Sexual Medicine (ESSM), Senior lecturer of Clinical Sexology at
the RINO, Amsterdam, Netherlands
Mark Rich Arnold Palmer Hospital for Children, Orlando, FL, USA
Nel Roeleveld Department for Health Evidence, Radboud Institute for
Health Sciences, Radboud University Medical Center, Nijmegen, The
Netherlands
Iris A. L. M. van Rooij Department for Health Evidence, Radboud Institute
for Health Sciences, Radboud University Medical Center, Nijmegen, The
Netherlands
Joao Luiz Pippi Salle Division of Urology, Department of Pediatric Surgery,
Sidra Medicine, Sidra Medical and Research Centre, Doha, Qatar
Christiane Schulze Department of Pediatric Surgery, Medical University of
Vienna, Vienna, Austria
Contributors xxxi
Michael Sennert Hypospadias Center and Pediatric Surgery Department,

Apoorv Singh Department of Paediatric Surgery, All India Institute of
Medical Sciences, New Delhi, India
Grahame H. H. Smith Department of Urology, The Children’s Hospital at
Westmead, Westmead, NSW, Australia
Children’s Hospital at Westmead Clinical School, The University of Sydney,
Sydney, NSW, Australia
Brent W. Snow Department of Pediatric Urology, Primary Children’s
Hospital, Salt Lake City, UT, USA
Howard M. Snyder III Department of Pediatric Urology, Children’s
Hospital of Philadelphia, Philadelphia, PA, USA
Anne-Françoise Spinoit Department of Urology: Pediatric and
Reconstructive Urology, Ghent University Hospital, Ghent, Belgium
Alexander Springer Department of Pediatric Surgery, Medical University
of Vienna, Vienna, Austria
Ibrahim Ulman Urology Division, Department of Pediatric Surgery, Ege
University Faculty of Medicine, Izmir, Turkey
Jeffrey Wacksman Emeritus of Pediatric Urology, University of Cincinnati
College of Medicine, Children’s Hospital Medical Center, Cincinnati, OH,
USA
Dana A. Weiss Division of Urology, Perelman School of Medicine,
Children’s Hospital of Philadelphia, University of Pennsylvania, Philadelphia,
PA, USA
Johannes Wirmer Hypospadias Center and Pediatric Surgery Department,
Christopher Woodhouse University College London, London, UK
Loes F. M. van der Zanden Department for Health Evidence, Radboud
Institute for Health Sciences, Radboud University Medical Center, Nijmegen,
The Netherlands
Department for Health Evidence, Radboud University Medical Center,
Nijmegen, The Netherlands
Mark R. Zaontz Department of Pediatric Urologist, Children’s Hospital of
Philadelphia, Philadelphia, PA, USA
Department of Clinical Urology in Surgery, Perelman School of Medicine,
University of Pennsylvania, Philadelphia, PA, USA
Part I
General and Basic Science
History of Hypospadias
1
Ahmed T. Hadidi
Abbreviations To advance, one must acknowledge the work of

the pioneers whose contribution has paved the
CEDU Chordee excision and distal way to the current state of the art.
urethroplasty Published work on the history of hypospadias
DYG Double Y glanulomeatoplasty has suffered from the reiteration of quotes from
LABO Lateral-based onlay flap earlier papers rather than the actual examination
MAGPI Meatal advancement and glanulo- of original sources.
plasty incorporated This chapter contains the authors impressions
UGPI Urethral advancement and glanulo- based on professional translation of the original
plasty procedure sources, direct observations, and the article pub-
lished in the Journal of Pediatric Surgery in 2017
[3]. In this chapter, we have tried to follow the
objective scientific approach of:
1.1 Introduction Read what others have reported,
quote what others have reported and then
Hypospadias is the most common congenital question what others have reported
malformation affecting the penis with incidence
approaching 1% of newborn boys [1]. Twistington A great deal of time and effort was spent to access
Higgins in 1941 [2] wrote: most of the original manuscripts in their original
Hypospadias is a grievous deformity which must languages. This chapter will deal with the follow-
ever move us to the highest surgical endeavour. ing topics: evolution of the hypospadias repair
The refashioning of the urethra offers a problem as techniques, hypospadias grades, chordee, stages
formidable as any in the wide field of our art. The of repair, protective intermediate layer, and suture
fruits of success are beyond rubies. The gratitude
of a boy has to be experienced to be believed. materials.
1.2 Evolution of Hypospadias

Repair Techniques
A. T. Hadidi (*)
Hypospadias Center and Pediatric Surgery
Department, Sana Klinikum Offenbach, Academic The evolution of the techniques of hypospadias
Teaching Hospital of the Johann Wolfgang Goethe repair could be grouped into three distinct peri-
University, Offenbach, Frankfurt, Germany ods, the early period during the first centuries
© The Author(s), under exclusive license to Springer Nature Switzerland AG 2022 3

A. T. Hadidi (ed.), Hypospadias Surgery, https://doi.org/10.1007/978-3-030-94248-9_1
4 A. T. Hadidi
after Christ, the middle or Renaissance period

during the middle ages, and the modern period
runs from the nineteenth century until today.
These periods seem to be closely linked to
advances in instrument manufacture, suture
material, and introduction of anesthesia. If we
think objectively, these three basic elements
(anesthesia, fine instruments, and fine absorbable
suture materials) are essential to the success of
any hypospadias operation. As these basic ele-
ments were only available starting from the mid-
dle of the nineteenth century on, prior hypospadias
repairs were entirely based on stretching and glue
material (early period) or tunneling (the middle Fig. 1.1 The first documentation of circumcision in the
ages). tomb of Ankh-mahor at Saqqara (near Cairo), Egypt,
some 4500 years ago (private photo ©Ahmed T. Hadidi
2022. All Rights Reserved)
1.2.1 he Early Period: The Period

T
of Stretching and Glue the first to describe urethral opening
malformation):
This could alternatively be called the “Period of Among certain individuals, the glans, because of
Stretching.” As instruments were very primitive an inborn defect, is not pierced according to nature,
and there was no anesthesia or the appropriate but the hole is found below the brake (frenum as is
suture materials, it is understandable that penile called in Greek), at the termination of the glans.
For this reason, the patient can neither urinate in
skin stretching over the urethra to the tip of the front, unless the penis is raised sharply towards the
penis with a pin or gummy materials extracted pubes, nor procreate children because the sperm
from plants was the surgical approach. cannot be directed in a straight line into the uterus,
The first documentation of a surgical proce- but forms on the side of the vagina.... Sometimes
the hole is situated far from the frenum; in the mid-
dure on the penis was circumcision and was dle of the urethra, these cases are incurable. The
shown in the famous relief in the tomb of Ankh- simplest, best, and least dangerous operative pro-
mahor at Saqqara (near Cairo), Egypt, dating cedure is what is called operation by resection, the
from the sixth dynasty, c. 2345 BC (Fig. 1.1). patient is placed on his back and the glans is fully
raised with the left hand. This part is then cut with
This relief shows two young men or adolescents the edge of the scalpel at the level of the crown.
being circumcised. Alexandria was a famous cen- However, the incision should not be slightly
ter for science in the early centuries after Christ. oblique but should resemble a delicate carving,
The two Alexandrian surgeons Helidorus and leaving a projection representing the form of a
glans. If little blood flows, it should be stopped
Antyllus, who lived in the first and second centu- with a bandage and vinegar; if this does not suffice
ries AD, are credited to be the first to describe, medications or cautery should be used. The resec-
classify, and define the pathophysiology and tion must be done in the glans rather than in the
treatment of hypospadias. penis, for because of its compact structure, the
glans is less likely to haemorrhage. (Bussemaker
Oribasius, doctor of the roman emperor Julian and Daremberg, 1851–1876) [4].
the Apostate (fourth century), left a medical
encyclopedia of 70 volumes (Iatrikai Synagogi, Galen (129–199 AD) (Fig. 1.2a), who spent a
Collecta Medica). Only a third of his writings are year as a scholar in Alexandria before he
preserved and in book 44 of Collecta Medica, he became a physician to the Roman gladiator, is
wrote (drawn from Heliodorus and Antyllus from credited as the first to use the term hypospadias
Alexandria, Egypt (first century AD) who were in his book Methodus Medendi to describe the
1 History of Hypospadias 5
Fig. 1.2 (a) Galen and his statue in Bergama, Turkey. (b) repository. Retrieved 18:19, September 15, 2021 from
Opera Omnia by Galen (copyrights: (a) File:Claude https://commons.wikimedia.org/w/index.
Galien CIPC0108.jpg. (2020, September 7). Wikimedia p h p ? t i t l e = F i l e : S t a t u e _ o f _ G a l e n _ o f _ P e rga m o n .
Commons. Retrieved 18:11, September 15, 2021 from jpg&oldid=573520010; (b) reprinted from Journal of
https://commons.wikimedia.org/w/index. Pediatric Surgery, Vol 52, Issue 2, by Hadidi, Ahmed T.:
php?title=File:Claude_Galien_CIPC0108. “History of Hypospadias: Lost in translation”, pp211–
jpg&oldid=451119223; File:Statue of Galen of Pergamon. 217, ©2017, with permission from Elsevier, [3])
jpg. (2021, July 6). Wikimedia Commons, the free media
condition hypospadias where the urethral open- I began to wonder about the accuracy of trans-
ing is on the under-surface of the penis. Some lation and transfer of knowledge over the centu-
papers quoted Galen to recommend “partial ries as glanular or even coronal hypospadias does
penectomy to the level of the urethral meatus not interfere with urination or procreation. In
with a conical incision to preserve a glanular addition, as the glans is the most sensitive part of
shape” [4, 5]. the penis, there seems to be little logic in making
6 A. T. Hadidi
it worse than it is without surgery. I was able to He was a strong advocate of cautery to treat
access and translate the original ancient Greek diseases.
manuscript of Galen (Omnia Opera, volume 10, The author could access a copy of the original
page 1001–1003) (Fig. 1.2b). In actuality, he did book Al-Tasreef in its original Arabic language
not recommend partial penectomy or amputation [7] and translate what he actually wrote about
but refreshing of skin edges and skin stretching to hypospadias in Chap. 55:
the tip of the penis. This technique he used Some boys are born and the glans is without a hole
successfully to treat patients (Gladiators) with or with a narrow meatus or the meatus may be dis-
“amputation” of the nose and ear. placed. When the glans is without a hole, you
should penetrate the glans with a trocar made of
Some other times removing excess skin with the lead and tie it for 3–4 days. It should be taken off
scalpel from the base of the glans and pushing when the boy needs to urinate and then put it back.
them downward and then fixing them as already It is possible that the passage of urine may help
said with something soft. This treatment resembles keep the tract open. Those who have a narrow
the one we use for amputations and deficiencies of meatus, should be dilated with lead trocar as we
the nose and lips and ears [5]. mentioned for many days.
In other words, Galen did not recommend partial Some of those boys with a displaced meatus can
not pass urine in a forward stream and cannot pro-
penectomy or amputation but refreshing of skin create as the semen can not reach the uterus. For
edges and skin stretching to the tip of the penis. those children, the child should lie on his back,
Oribasius, Celsius, Fallopio, and Paul of hold the penis with your left hand and incise the
Aegineta (625–690 AD) followed the same head of the glans with a knife to have the opening
in the middle and shave the glans as if you were
approach of Heliodorus, Antyllus, and Galen for cutting a quill or as if you want to carve a piece of
hypospadias [6]. wood in manner of reestablishing as natural shape
of the glans and in which the meatus falls into the
median position where it should be. You should be
careful and control bleeding using cautery if
1.2.2 he Renaissance or Middle
T needed.
Ages Period: The Period
of Tunneling In other words, Albucasis described two tech-
niques for hypospadias repair: (a) tunneling for
The fall of the Roman Empire heralded the arrival those with imperforate glans and (b) carving for
of the middle ages, in which the majority of med- those with displaced meatus. Albucasis is cred-
ical texts originated from Islamic physicians. ited to be the first to describe tunneling for the
Abu Al Qasim Al Zahrawi (known as Albucasis) correction of hypospadias. However, one can
of Cordoba, Andalusia, Spain (936–1013) realize that the carving technique is similar to
(Fig. 1.3) wrote a medical encyclopedia called that described by Heliodorus and Antyllus in the
Al-Tasreef (influenced by the work of Hippocrates, first two centuries AD.
Galen, and Paul of Aegina). In 1150AD, Gerard Serefeddin Sabuncuoglu of Anatolia (Turkey)
of Cremona translated “Al-Tasreef” into Latin was influenced by Albucasis and described a
and it remained the most important reference hypospadias repair similar to the canalization
book on surgery until the end of the eighteenth technique described by Albucasis but carefully
century [7]. Albucasis described (with illustra- included the necessity of placement of a hollow
tions) the tonsil guillotine, the true scissors, the “tin” catheter into the urethra to maintain patency
syringe, the vaginal speculum, the lithotrite, the [8]. His major contribution was probably to use a
Chamberlen obstetric forceps, and the Kocher hollow sound with a patent canal. This was very
method to correct dislocated shoulder and used practical for children, enabling them to urinate
animal gut as a suture material. He even sug- through it instead of removing it for every
gested the use of animal teeth to replace lost urination.
teeth. His surgical instruments are still preserved Guy de Chauliac (1363) [10] kept an instru-
in the Calla Hurra Museum in Cordoba (Fig. 1.3). ment into the canal until epithelialization. Amatus
Fig. 1.3 (a) Albucasis teaching his students on a patient (reprinted from Journal of Pediatric Surgery, Vol 52, Issue
(reproduced with permission from the Wellcome Library, 2, by Hadidi, Ahmed T.: “History of Hypospadias: Lost in
London) (b) Cover and a page from Albucasis original translation”, pp211–217, ©2017, with permission from
book “Al TASREEF“ (c and d) Albucasis original instru- Elsevier, [3])
ments in the Calla Hurra Museum, Cordoba, Spain
8 A. T. Hadidi
c d
Fig. 1.3 (continued)
Lusitanus (1511–1568) [11] reported a success- mortality (the patient and his assistant died
ful repair of a 2-year-old child with penoscrotal because of infection and a spectator because of
hypospadias using a silver cannula. Parey (1510– fright)! He was the first to use anesthesia in
1590) [12] and Dionis (1658–1718) [13] all fol- Europe to perform amputation [15].
lowed the same principle of introducing a trocar Liston had also operated on hypospadias, and
to create a canal and leaving an instrument inside in 1838, he reported the successful use of pre-
until epithelialization is complete. Further refine- puce to line the tunnel and avoid its closure. The
ments were made by Dupuytren, Sir Astley author could access his original book Practical
Cooper (1815), and Dieffenbach (1829) (Fig. 1.4) Surgery in 1838 where he wrote [14]:
who described the lock-stitch technique This hypospadias, as it is denominated, can occa-
“Schnurnaht Technik” [9]. sionally be remedied. Attempts have been made by
Sir Robert Liston (1794–1847) was a pio- perforating the glans in the course of the natural
neer Scottish surgeon who graduated from canal, and by the insertion of a tube in the perfora-
tion, to carry forward the current and elongate the
Edinburgh University and was trained by his urethra permanently; this method does not answer;
father before he moved down to London there is a want of substance in the part, and slough-
(Fig. 1.5a). In the days before anesthetics, suring is generally the consequence of the attempt. I
gery meant that patients had to be held down have sometimes succeeded, and in cases where
other means have been tried unsuccessfully, in pro-
and suffered terrible pain. Speed and skill by tecting the exposed and irritable lining membrane
the surgeon were therefore paramount, and of the passage, and continuing the canal open to
Liston had those skills in abundance. He was the apex of the organ, by turning back a portion of
famous to perform amputation in 2 and half the prepuce and uniting it without any twist, the
lining membrane presenting outwards; patients
minutes. It is even claimed that he performed have been thus relieved from the frequent calls to
one amputation in 25 seconds. He is also known make water, the nocturnal emissions, and other
for the only operation in history with 300% unpleasant consequences.
Fig. 1.4 Dieffenbach applied Albucasis principle and pierced the glans and left a catheter in place until the channel
became epithelialized. The operation was not successful [9]
He even described in his book the correction of technique of two longitudinal skin incisions for
epispadias in a 23-year-old man before the the correction of epispadias in 1869 [17].
description of Thiersch in 1869. He was probably the first to identify the
importance of avoiding two suture lines on top of
each other and designed the incisions in such a
1.2.3 he Modern Period:
T way that the suture line of the urethroplasty
The Period of Urethroplasty would be on one side of the midline and the skin
suture line on the other side of the midline
This was sparked by the introduction of ether (Fig. 1.9). This technique is still used success-
anesthesia in 1846 by Morton [16]. This enabled fully for epispadias repair in many centers around
surgeons to perform sophisticated urethral sur- the world today. His student, Theophile Anger
gery. Although already published by Liston in from Paris, applied his principle to repair peno-
1838, the modern era in hypospadias surgery is scrotal hypospadias successfully and reported it
commonly reported to start in 1869 when Karl in the 16th meeting of the French surgical society
Thiersch from Leipzig, Germany, described his in 1874 [18].
10 A. T. Hadidi
Fig. 1.5 (a and b) Robert Liston (Portrait by Samuel 2, by Hadidi, Ahmed T.: “History of Hypospadias: Lost in
Stump), Robert Liston Book Practical Surgery [14] translation”, pp211–217, ©2017, with permission from
(reprinted from Journal of Pediatric Surgery, Vol 52, Issue Elsevier, [3])
1.3 Grades of Hypospadias and graduated from Jena in 1878 before he moved
to New York in 1882 and established the
Probably, Kaufmann was the first that we could New York post-graduate medical school. He pre-
identify who classified hypospadias into three sented and published the principle of urethral
grades: grade I or glanular hypospadias where the mobilization and meatal advancement in 1897 in
urethral opening lies within the glans region; German language in “Deutsche Medicinische
grade II or penile hypospadias where the opening Gesellschaft” in New York without illustrations
lies within the body of the penis to the penoscro- [21] and 1 year later, 1898, in English with illus-
tal junction; and grade III or perineal, where the trations in the New York medical journal [22] and
opening lies proximal to the penoscrotal junction reported his experience in two patients, one with
and the scrotum is bifid [19]. This became popu- glanular hypospadias and it was successful and
lar in the twentieth century after the publication the second with penile hypospadias and he
of Smith in 1938 [20]. reported that it was less successful probably, he
Currently, primary hypospadias is classified wrote, because the second child had penile hypo-
into four grades [3] (Fig. 1.6). spadias. The principle is based on the fact that the
urethra is stretchable as evidenced by the remark-
able increase of the length of the urethra and
1.3.1 rade I or Glanular
G penis from the flaccid to the erected state
Hypospadias (Q54.0) (Fig. 1.7).
Viktor Ritter von Hacker, an Austrian surgeon
This is when the urethral opening lies within the who was trained by Billroth, published the same
region of the glans, distal to the coronal sulcus. principle, in 1898 in Germany [23] on one child
Commonly, there is a bridge just distal to the ure- who was 10 years old. The Hacker technique of
thral opening identifying the junction between advancement differed from that of Beck in that
the embryological glanular urethra and the penile he tunneled the urethra through the glans
urethra. (Fig. 1.8).
Carl Beck and Hacker are the pioneers for the This principle has been modified by many oth-
modern correction of glanular hypospadias. Carl ers including Horton and Devine (triangularized
Beck (1856–1911), born in Neckargemünd, glans) in 1961 [24], Mustardé in 1965 (V-flap)
Germany, studied medicine in Heidelberg, Berlin, [25], Duckett in 1981 as the meatal advancement
and glanuloplasty incorporated (MAGPI) [26],
Koff [27], Arap (M configuration) [28], Harrison
and Grobbelaar (urethral advancement and glan-
uloplasty procedure or UGPI) (1997) [29], and
lately Hadidi in 2010 (Double Y Glanulomeatoplasty
or the DYG technique) [30].
1.3.2 rade II or Distal Hypospadias

G
(Q54.1)
The urethral meatus in distal hypospadias lies in

Fig. 1.6 Classification of primary hypospadias into four the distal half of the penis. There are two main
grades: glanular (grade I), distal (grade II), proximal approaches for urethroplasty in distal
(grade III), and perineal (grade IV). Complicated, cicatri- hypospadias:
cial hypospadias with inadequate skin and scarred tissue
(inappropriately called crippled hypospadias) is classified
Anger [18] and Duplay [31] described the
as grade V hypospadias [3] (©Ahmed T. Hadidi 2022. All Tubularization of the Urethral Plate and ventral
Rights Reserved) penile skin to reconstruct fully epithelialized
12 A. T. Hadidi
Fig. 1.7 Artist

replication of Carl Beck
(1898) technique of
urethral mobilization for
glanular hypospadias.
Notice that he did not
try to wrap the glans
around the mobilized
meatus to avoid meatal
stenosis [22] (©Ahmed
T. Hadidi 2022. All
Rights Reserved)
Fig. 1.8 Viktor Ritter von Hacker (1898) operation for glanular hypospadias. He used the same advancement principle
but instead, he tunneled the urethra through the glans [23]
a b
Fig. 1.9 (a) Artist replication of the original drawing are not symmetrical so that the urethral incision lies to the
from Thiersch for epispadias correction [17]. (b) Artist right of the midline and the skin closure lies to the left of
replication of the Anger modification of the Thiersch prin- the midline (©Ahmed T. Hadidi 2022. All Rights
ciple to correct hypospadias [18]. Notice that the incisions Reserved)
A few years earlier, my aunt had called me to say

urethra in 1874 (Fig. 1.9). Duplay, 6 years later that my uncle was looking bad. When I arrived
in 1880 [32], reported his second operation before the ambulance came, there was my uncle
where he wrapped the urethral plate around a lying, simply dead. My aunt wanted my uncle to
catheter but did not suture the edges together wear his favorite hunter’s jacket. But by that time,
he had become pretty fat. So, on her advice, I
and covered it with skin. He probably thought of incised my uncle’s jacket on its back and we nicely
this approach to avoid burying the available, buttoned it in the front. That was it [38].
poor-quality catgut sutures inside the tissues.
This principle of forming an incomplete new Although incision of the urethral plate has good
urethra was further popularized in the 1940s by cosmetic results, it is associated with high inci-
Browne [33]. Incision of the urethral plate was dence of stenosis, stricture, and functional ure-
first described by Reddy in 1975 [34], thral obstruction (FUO) [39].
Orkiszewski in 1987 [35], Rich in 1989 [36], To reduce urethral strictures and functional
and Snodgrass in 1994 [37]. Orkiszewski from urethral obstruction associated with incision of
Poland called it the “Dead Man Jacket the urethral plate, Hayes [40], Gundeti [41], and
Technique.” He explained to me: Ferro [42] described covering of the raw surface
14 A. T. Hadidi
resulting from incision of the urethral plate with hypospadias reporting his experience with 60
grafts from the prepuce or buccal mucosa (dorsal cases of hypospadias over 25 years’ period. He in
inlay graft or DIG). fact set the golden standard guidelines of hypo-
The use of meatal-based flap is the other pop- spadias surgery more than a century ago:
ular approach for distal hypospadias and with- There is no place for the “occasional” surgeon who
stood the test of time for over 80 years. This was performs few cases of hypospadias in his life time,
described by Wood in 1875 [43], Ombrédanne in they should be referred to a specialist who per-
1911 [44], and Bevan in 1917 [45] and popular- forms hypospadias operations frequently and regu-
larly (like a gynecologist operating 40-50 fibroids
ized by Paul Mathieu in 1932 [46] (Fig. 1.10). per year). Theoretical knowledge can never teach a
Other modifications of the same principle to pro- surgeon how to operate on hypospadias. The pri-
duce a slit-like meatus were made by Mustardé mary results after operation are often good enough
[25], Barcat [47], Boddy and Samuel [48], and but after a year or two, the most promising primary
results often dwindle to insignificant advantage or
Hadidi [49]. even drawbacks. One has to study each case thor-
oughly and there is no ironclad rules. Urine diver-
sion is important by means of catheter or
1.3.3 rade III or Proximal
G urethrotomy. Delicate and exact suturing and the
use of proper suture material is essential. The after-
Hypospadias Without Severe treatment must be under the personal supervision
Deep Chordee (Q54.2) of the experienced surgeon. The operation is often
performed in an excellent way by the surgeon but
Where the urethral opening lies proximal to the the after-care is left in incompetent hands. Every
failure makes a secondary operation more difficult.
middle of the penis and there is no deep We found that in children and young people, it is
chordee. difficult to retain dressings in the genital region
Tubularization of the urethral plate as men- and if general cleanliness is observed, the patients
tioned above is common. Other popular tech- fare better without any dressings whatsoever. Cure
in hypospadias surgery should satisfy two condi-
niques include the preputial island onlay flap tions; 1) freely movable urethra permitting erec-
described by Elder et al. in 1987 [50] and the tion without angular deflection, 2) absolute
lateral-based onlay flap (LABO) by Hadidi in absence of stricture with free flow or urine after a
2012 [51]. lapse of years. It is not absolutely necessary to
obtain a cosmetic result with smooth surfaces and
a urethra in the center of the glans! This would be
a cosmetic but not a clinical success.
1.3.4 rade IV or Perineal
G
Hypospadias with Severe The use of prepuce to reconstruct a long urethral
Deep Chordee (Q54.3) tube was first described by Weller van Hook from
Chicago (1896) [53]. He suggested using lateral
Serineal hypospadias is actually a syndrome of a oblique vascularized flaps from the lateral part of
proximally located meatus, ventral penile hypo- the preputium and penile skin. This was a proxi-
plasia resulting in severe chordee (penile curva- mally based pedicled tube derived from the pre-
ture) that necessitates division of the urethral putium. These techniques were performed in two
plate, small sized glans penis, and short ano- stages. This was further modified by Mayo (1901)
scrotal distance or ASD. The scrotum is usually (Fig. 1.11) [54]; Broadbent, Woolf, and Toksu
bifid, and it may be associated with uni- or bilat- (1961) [55]; DesPrez, Persky, and Kiehn (1961)
eral undescended or retractile testis. [56]; Hodgson (1970) [57]; Toksu (1970) [58];
There is a “second” Carl Beck (1864–1952) Asopa et al. (1971) [59]; Hinderer (1978) [60];
pioneer [52] who was born in Bohemia (Czech Duckett (1980) [61]; Joseph (1995) [62]; and
Republic now) and studied in the German Hadidi (2009) [63].
University in Prague before moving to the states Koyanagi et al. described the use of lateral
where he became a professor of surgery in penile skin and foreskin from both sides for ure-
Illinois. He published an extensive article on thral reconstruction [64]. This was further modi-
Fig. 1.10 Artist

replication of the
a b
original Paul Mathieu
technique (1932) for
distal hypospadias [46].
(a) The skin incision
that continues deep into
the glans. (b) The use of
straight needle with knot
inside the urethra and
leaving the threads long
to come out of the
meatus. (c) The flap was
taken together with its
fascia to maintain the
vascularity of the flap.
(d) At the end of the
procedere, the end of the
threads are left to
protrude through the
rounded, terminal
meatus. Note that he did
not leave a catheter
through the urethra c d
(©Ahmed T. Hadidi
2022. All Rights
Reserved)
16 A. T. Hadidi
Fig. 1.11 Original Mayo technique for distal hypospadias [54]
fied by Snow and Cartwright [65], Emir et al. described the use of bladder mucosa in 1947
[66], Hayashi et al. [67], and Hadidi [68, 69]. [73], which was popularized by Hendren and
Reda [74] and Ransley [75], but it became less
popular because of mucosal prolapse through the
1.3.5 rade V or Cicatricial or
G meatus and a high incidence of strictures and
Crippled Hypospadias stenosis.
Devine and Horton reported the use of free
One of the most complex challenges facing the preputial graft in 1961 as a single stage repair
hypospadias surgeon is the cicatricial compli- with a tubularized grafting technique [24]. Meatal
cated hypospadias. These patients have typically stenosis, stricture, and scarring were the common
undergone multiple failed hypospadias repairs complications observed [76]. Cloutier in 1962
and have deficiency of healthy skin to use for recommended the use of preputial skin graft as a
neo-urethral reconstruction. Several authors use first stage and reconstruct the new urethra
the term “hypospadias cripple” [70, 71]. However, 6 months later as the second stage [77]. The
parents find this term offensive and insulting and Cloutier technique became popular in the 1990s
it is better to use the term cicatricial or grade V after Bracka presented a personal series of 600
hypospadias. By definition, those patients require cases using preputial skin graft or buccal mucosa
the use of extragenital mucosa or skin, usually for correction of all forms of hypospadias [78].
grafts due to deficiency of healthy local skin and Humby (1909–1970) is one of the pioneers in
severe scarring. plastic surgery who invented the Humby knife for
Nove-Josserand in 1897 was the first to use a skin grafts [2]. He first reported the use of buccal
free graft from the thigh that was placed around a mucosa for hypospadias repair in 1941. It became
trocar and inserted through the glans to create a popular after the publications of Duckett [79] and
neo-urethra (Fig. 1.12) [72]. Memmelaar first Dessanti [80]. Now, preputial skin, non-hair-
After Galen and a brief reference by Oribasus

(325–403) [4] that chordee interferes with inter-
course, there seem to be no reference to chordee
for 1200 years. The French anatomist and sur-
geon, Ambroise Paré (1510–1590), described
the accompanying chordee as to prevent “the
seed to be cast directly and plentifully into the
womb” [12]. He induced an artificial erection of
the penis 400 years before the description by
Gittes [81].
In 1547, the Duke of Orleans became King
Henry II of France. At age 14 years, he was mar-
ried to Catherine de Medici, also 14, to
strengthen tie with Italy; she was the daughter
of Lorenzo II (her uncle was Pope Clement VII).
The king had a mistress, Diane de Poitiers, but
despite this alliance and 10 years of trying with
Catherine, there were no children. He had a
severe chordee deformity. He was said to be
very robust and loved violent exercise, a degree
of athletic prowess he probably needed to per-
form at all with chordee! However, his surgeon
Jean Fernel corrected his chordee, after which
Fig. 1.12 Nove-Josserand was the first to use skin graft he fathered ten children by Catherine, surely
from the thigh (1897) [72] one of the most successful outcomes of hypo-
spadias surgery [82].
The term “chordee” was actually first intro-
bearing skin, or buccal mucosa grafts are used as duced into the medical literature in 1708 from
a two-stage repair to increase graft take and the French and was defined as “painful inflam-
decrease complications, such as urethral stricture matory downward curving of the penis” [84]
and meatal stenosis. (Fig. 1.13). This affection of the penis was
most common in the second and third weeks of
gonorrhea and occurred chiefly at night
1.4 Chordee (Penile Curvature) (Fig. 1.14) [83].
Forster in 1860 wrote:
The major significance of penile curvature asso- I saw a child 8 months old in 1857. The penis was
ciated with hypospadias was fully appreciated by (without erection) in a state resembling chordee,
Galen in the second century AD Galen (130– bent like a siphon. The only explanation that could
190 AD). He emphasized the major significance be obtained from the mother was that whilst preg-
nant, her husband had gonorrhea, and suffered
of chordee [5]: much from chordee [85].
Thus men afflicted with hypospadias find it impos-
sible to beget children, the meatus being turned A number of terms have been in common use to
away from the extremity of the penis by the frenu- describe the curvature element of hypospadias.
lum, not because they lack fertile sperm, but Mettauer [86] and Mayo [54] used the terms
because the curvature of the penis prevents its nor-
mal overflow from being conveyed forwards. This incurvation, ventral deformity, and ventral curva-
theory is confirmed by the ability to beget children ture. Nesbit in his first article on hypospadias in
if the frenulum is divided. 1941 used the term “ventral curvature” [87].
18 A. T. Hadidi
Fig. 1.14 The origin of the word “chordee” dates back to

1708. [ad. F. cordée in chaudepisse cordée.] Path. A pain-
ful inflammatory downward curving of the penis [84]
(reprinted from Journal of Pediatric Surgery, Vol 52, Issue
2, by Hadidi, Ahmed T.: “History of Hypospadias: Lost in
translation”, pp211–217, ©2017, with permission from
Elsevier, [3])
The first surgeon that I could identify who

used the term chordee in direct relation to hypo-
spadias was Clinton Smith [20]. He wrote:
Recalling the embryological aspects momentarily,
all hypospadias are associated with some degree of
“congenital chordee. This varies from slight down-
ward bending of the glans to complete anchorage
of the glans in the perineum in exaggerated
instances of the third degree type.
b He used the term “congenital chordee” to differ-

entiate it from the then-classic chordee associ-
ated with gonorrhea.
In 1932, Arthur Cecil [88] reported the experi-
ence of Dr. J Pettit who “dissected the genital
organs of a 10 years old child in 1870 whom he
had examined during life- and who had penoscro-
tal hypospadias. Dr. Pettit inflated the corpora cav-
ernosa and then separated the urethra from the
corpora cavernosa. He found that the penis still
kept its deformity and he attributed this to a fibrous
band beneath the penis but even division of this
Fig. 1.13 (a and b) Definition of chordee: new pro- band did not straighten the penis. In making a
nouncing dictionary of medicine (1892) [83] (reprinted study of the cells of the corpora cavernosa, Pettit
from Journal of Pediatric Surgery, Vol 52, Issue 2, by
Hadidi, Ahmed T.: “History of Hypospadias: Lost in
found that there was atrophy of these cells on the
translation”, pp211–217, ©2017, with permission from concave portion. He did not know whether they
Elsevier, [3]) had always been in this condition or whether this
atrophy was secondary to the hindering bands cia are abnormal. The third type was described
beneath the penis….”. It should be noted that the as a skin chordee and is due to abnormality of
autopsy report of Pettit was made on a child 10 the superficial dartos fascia. The fourth type was
years of age. described by Kramer [90] and may be the result
As early as 1931, Arthur Cecil wrote: of disproportional growth of the dorsal portion
Clinical experience would seem that if the skin, of the corporal bodies causing downward
subcutaneous tissue and fibrous bands extending deflection.
up between the corpora cavernosa are divided suf-
ficiently early in life that the corpora cavernosa
will fully develop… I can recall but one or two
authors who have suggested that this operation be 1.5 Stages of Repair
done within the first 2 years of life…The operation
for the correction of the deformity should be done The repairs of the late nineteenth century were
as soon as the child has been weaned and its nutri- three-stage repair. Duplay [31] recommended
tion has been fully established. Some authors have
suggested that in some instances it is wise to cut three steps or stages of repair: (1) excision of
into corpora cavernosa. It seems to me that this is a chordee, (2) neo-urethra tube reconstruction, and
bad procedure on the account of hemorrhage and (3) anastomosis of the neo-urethra to the proxi-
probably uncalled for if the operation of correcting mal native urethra (Fig. 1.15).
the deformity is done sufficiently early in life
(Cecil, 1932, page 514) [88]. The repairs of the early to mid-twentieth cen-
tury were usually performed in two stages.
Devine and Horton [89] described three patho- Edmunds advocated a two-stage repair with the
logic types of chordee without hypospadias. In chordee release and transfer of preputial skin to
the first type, the spongiosum is absent in the the ventrum that would later be tubularized [91].
distal penis and the urethra is often paper thin. By separating the chordee repair and the urethro-
In the second type, the urethra is completely plasty, the operation was simplified and could be
developed but Buck’s fascia and the dartos fas- performed reproducibly by many surgeons.
Fig. 1.15 Artist replication of the Duplay technique of incomplete urethral tube tubularization and the use of glass rods
(©Ahmed T. Hadidi 2022. All Rights Reserved)
20 A. T. Hadidi
Unfortunately these early repairs often resulted 1.6 Suture Materials

in a hair-bearing urethra and a ventrally displaced
meatus. Advances in suture material played an important
Beginning in the late 1950s and 1960s, there role in improving the results of hypospadias sur-
was renewed interest in one-stage hypospadias gery and reducing complications. Suture materi-
repairs. Before this period, one-stage operations als used included animal intestine, silver wire,
were performed using split-thickness free grafts horse hair, stainless steel, and synthetic suture
from the thigh or arm that were fraught with com- materials (Fig. 1.17).
plications, including marked contracture. Devine Albucasis used suture material made of ani-
and colleagues published data using free full- mal intestine [19]. Duplay preferred to use silver
thickness skin graft tubes as one-stage repair [24]. wire [32].
Devine’s group excised the urethral plate and cre- In 1917, Beck wrote:
ated the graft from preputial skin. The proximal Intra-urethral sutures of catgut, no matter how fine
anastomosis was oblique and the glans was incised is bad. The catgut swells and loosens and favors
to create the distal anastomosis. Tunneling through leakage. The borders and stitch holes are infiltrated
the glans was abandoned because of the high rate with urine and becomes easily infected, thus the
suture is spoiled. Fine horse-hair is the ideal mate-
of stricture. The ventral defect was covered using a rial and it need not be removed from inside of the
penile or scrotal skin flap. In addition, urine was urethra; it drops out when it is not needed, often
diverted by placement of a perineal urethrostomy months after operation [52].
catheter. The use of preputial skin graft was a sig-
nificant advantage to the previously used graft Mathieu used silk sutures that were coming out
sites in that the skin is thin, pliable, and hairless. through the urethra [46] (Fig. 1.17).
Meatal stenosis, stricture, and scarring were the Nesbit in his article in 1941 wrote:
common complications observed. Cloutier in 1962 we prefer the Duplay urethroplasty using stainless
recommended the use of preputial skin graft as a steel subcuticular sutures closure as suggested to
first stage and reconstruct the new urethra 6 months us by Lloyd Reynolds. … These stainless steel
later as the second stage [77]. The Cloutier tech- sutures are accompanied by a minimum of reaction
and infection and are easily removed on the four-
nique became popular in the 1990s after Bracka teenth postoperative day [87].
presented a personal series of 600 cases using pre-
putial skin graft or buccal mucosa for correction of Denis Browne used glass beads and metal pearls
all forms of hypospadias [78] (Fig. 1.16). to secure his sutures [33].
In the early twenty-first century, the neo- Most surgeons nowadays tend to use synthetic
urethra in hypospadias grades I, II, and III is usu- absorbable sutures like polyglactin (Vicryl, Ethicon)
ally reconstructed in a single stage. Perineal or polydioxanone (PDS©, Ethicon), polyglyconate
hypospadias (grade IV) and cicatricial compli- (Maxon©, Davis & Geck), or poliglecaprone 25
cated hypospadias (grade V) are preferably (Monocryl©, Ethicon) as fine as 6/0 and 7/0.
reconstructed in a two-stage repair with excision It might seem that with such volume and
of the deep chordee or cicatricial scar, and laying response, a standard approach and techniques
of healthy epithelium or graft in the first opera- would have evolved in the twenty-first century.
tion and urethroplasty is delayed 3–6 months Some surgeons may agree with what Davis
later. wrote in 1950 [92]:
Recently, Hadidi reported his experience with I would like to say that I believe the time has
the CEDU (Chordee Excision & Distal arrived to state that the surgical repair of hypospa-
Urethroplasty) technique where the deep chordee dias is no longer dubious, unreliable or even
is excised and distal urethroplasty are performed extremely difficult. If tried and proven methods are
scrupulously followed, a good result should be
is the first stage, leaving the original urethral obtained in every case. Anything less than this sug-
opening as a perineal urethrostomy to be closed gests that the surgeon is not temperamentally fitted
3–4 months later [69]. for this kind of surgery.
Fig. 1.16 Cloutier original description of two-stage Surgery, Vol 30, Issue 3, pp368–373, ©1962, from Wolters
repair for proximal hypospadias (from Cloutier: “A Kluwer Health Inc. with permission [77])
method for hypospadias repair”, Plastic and Reconstructive
However, many experienced hypospadias sur- Reconstruction of a new urethra is like building
geons would rather agree with the Australian sur- a house; the surgeon must use the appropriate
geon Smith who wrote half a century later in raw material otherwise the house will collapse.
2004 [93]: Each hypospadias patient is unique with a wide
I know of no surgery that can quite humble a sur- range of hypoplasia and dysgenesis of the cor-
geon as much as hypospadias—it carries a vicious pus spongiosum and other ventral structures of
bite with its list of complications, most galling the penis. The hypospadias surgeon needs to tai-
when they can occur years later.
lor the technique according to the degree of
22 A. T. Hadidi
a b c
Fig. 1.17 Suture materials used to correct hypospadias. outside the new meatus [46]. (c) Denis Browne used glass
(a) Artist replication: Duplay preferred to use silver wire beads and metal pearls [33] (a and b: ©Ahmed T. Hadidi
and a glass rod [32]. (b) Artist replication: Mathieu used 2022. All Rights Reserved) (c) photo courtesy of late Prof.
silk sutures that were tied inside the urethra and protruded Lotfy)
hypoplasia and the availability of healthy, well- 7. Spink MS, Lewis GL. Albucasis: on surgery and
vascularized tissues to reconstruct the new instruments. A definitive edition of the Arabic text
with English translation and commentary. Wellcome
urethra. Institute of the History of Medicine. London, UK;
1973. p. 170–827.
Acknowledgments The author is indebted to the late 8. Kendirci M, Kadioǧlu A, Boylu U, Miroǧlu
Professor Dan Young, Glasgow, Scotland, for providing C. Urogenital surgery of the 15th century in
the original manuscripts regarding Chordee. I would also Anatolia. J Urol. 2005; https://doi.org/10.1097/01.
like to thank Dr. Stella Platara, lecturer of literature and ju.0000158162.32214.de.
ancient Greek, Athens, Greece, for translating the original 9. Dieffenbach J. Guérison des fentes congénitales de
manuscript of Galen from ancient Greek to English. la verge, de l’hypospadias. Gaz Hebd Med. 1837:
5–156.
10. Nicaise E. La grande chirurgie de Guy de Chauliac.
Paris, London, UK; 1890.
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jpedsurg.2016.11.004. men! The bravado and bravery of Robert Liston.
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Tome 1/texte grec en grande partie inédit collationné 16. Keys TE. An epitome of the history of surgical anaes-
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français. John Churchill, Paris; 1858, p 615–34. 17. Thiersch K. Ueber die Enstehungsweise und opera-
5. Galen K. Opera omnia. In: Kuehn KG, editor. Leipzig: tive Behandlung der Epispadie. Arch der Heilkd.
C. Cnobloch; 1826. p. 1001–3. 1869:10–20.
6. Aegineta P. The seven books of Paulas Aegineta. 18. Anger T. Hypospadias. Paris Bull Soc Chir. 1874.
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Men Behind Principles
and Principles Behind Techniques 2
Ahmed T. Hadidi
Abbreviations In an address to the Royal College of Surgeons,

Winston Churchill remarked [1]:
BILAB Bilateral-based flap The longer you look back, the further you can look
CEDU Chordee excision and distal forward
urethroplasty
DUG Distal urethro-glanuloplasty As Durham Smith mentioned in his forward to
DYG Double Y glanulomeatoplasty the first edition of this book [2]:
LABO Lateral-based onlay flap Although the penile repairs can be grouped into
MAGPI Meatal advancement and glanulo- five or six major principles, depending on the tis-
plasty incorporated sues used, each has been subject to countless varia-
MAVIS Mathieu and V incision sutured tions as one surgeon after another adds yet another
modification to an already thrice-modified varia-
MIP Megameatus intact prepuce tion of a procedure adapted from a principle
OIF Onlay island flap derived from the original!
SLAM Slit-like adjusted Mathieu
TALE Tunica albuginea longitudinal excision From Alexandria, Egypt came the first hypospa-
TIP Tubularized incised plate dias pioneers, Heliodorus and Antyllus. Living in
TPIF Tubularized preputial island flap the first century, they were the first to describe
UGPI Urethral advancement and glanulo- and define the pathophysiology and treatment of
plasty procedure hypospadias [3].
The aim of this chapter is to categorize the
enormous variety of techniques in hypospadias
2.1 hort List of Men Behind
S into eight basic principles and to give credit to the
Principles great pioneers who first described these concepts
(Table 2.1).
The philosopher Santayana (1863–1952) said: Thus, it is easier for the reader interested in
Those who cannot remember the past are con- hypospadias to grasp the basic concepts of hypo-
demned to repeat it. spadias repair. The following account is by no
means exhaustive, nor does it include all the tech-
A. T. Hadidi (*) niques described for hypospadias repair.
Hypospadias Center and Pediatric Surgery Department, In dealing with a boy with hypospadias, the
Sana Klinikum Offenbach, Academic Teaching surgeon has to correct the following major
Hospital of the Johann Wolfgang Goethe University,
abnormalities:
Offenbach, Frankfurt, Germany

26 A. T. Hadidi
Table 2.1 Short list of men behind principles

Contributor Date Contribution
Heliodorus and Antyllus First First description of pathophysiology and repair of hypospadias
century AD
Galen [4] 130– First to use the term “hypospadias” and to emphasize significance of
199 AD penile curvature
Paré [5] 1510–1590 Extensive discussion of hypospadias and penile curvature and
treatment
Dieffenbach [6] 1837 Attempted urethroplasty by piercing the glans to the normal urethra
Mettauer [7] 1842 “Arrest of development” theory for hypospadias etiology; “skin
tethering” theory for curvature etiology
Physick [8] 1844 First described chordee correction by dorsal plication
Pancoast [8] 1844 First described chordee correction by transverse incision and
longitudinal closure
Bouisson [9] 1861 First to use scrotal skin for urethral reconstruction
Thiersch [10] 1869 U-shaped urethroplasty for epispadias; buttonholing of the prepuce
Wood [11] 1875 Described meatal-based flap with buttonhole of prepuce
Duplay [12] 1880 Acknowledged Thiersch; described incomplete urethroplasty
Van Hook [13] 1896 First description of “preputial vascular island flap” and lateral oblique
flap
Beck [14, 15] 1898, 1917 First to describe urethral mobilization; used rotation flap from
scrotum for coverage; used a bipedicled preputial flap to resurface
ventral penile skin
Nové-Josserand [16] 1897 First description of tubularized free skin graft urethroplasty
Edmunds [17] 1913 Three-stage ventral repositioned prepuce via buttonholed pedicle
followed by tubularized urethroplasty
Ombrédanne [18] 1932 Described meatal-based flap and a purse-string suture for
urethroplasty and button hole of prepuce for skin coverage
Mathieu [19] 1932 U-shaped flap from proximal skin and two suture lines
Davis [20] 1940 Utilized a tube of skin from the dorsal penile skin for urethral
reconstruction
Humby [21] 1941 Tubularized free skin graft urethroplasty; donor sites: inner upper
arm, thigh, “mucous membrane from lower lip”
Cecil [22] 1946 Popularized staged scrotal skin incorporation into urethroplasty
Memmelaar [23] 1947 First description of bladder mucosa use for one-stage urethroplasty
Browne [24] 1949 “Buried incomplete urethral plate” urethroplasty
Byars [25] 1955 Popularized midline incision and transposition of the prepuce with
second-stage tubularized urethroplasty
DesPrez et al. [26] 1961 Described “island pedicle flap” that circumscribes the meatus and
extends along the inner aspect of the prepuce
Devine and Horton [27] 1961 Popularized one-stage free skin graft urethroplasty and V incision of
the glans
Broadbent et al. [28] 1961 One-stage urethroplasty with dorsolateral penile skin
Cloutier [29] 1962 Described the two-stage hypospadias repair using preputial graft
Mustarde [30] 1965 Recommended V incision of the glans and large proximal meatal-
based flap
Barcat [31] 1969 Described balanic groove technique
Smith [32] 1973 Described de-epithelialization of skin as a second protective layer
Gittes and Maclaughlin [33] 1974 First described artificial erection test
De Sy and Oosterlinck [34] 1980 Introduced silicon foam dressing
Duckett [35] 1981b Described MAGPI procedure, transverse preputial island flap
Monfort and Lucas [36] 1982 Recommended testosterone stimulation preoperatively
Koyanagi et al. [37] 1983 Described parameatal foreskin flap
Koff and Eakins [38] 1984 Described chordee correction by corporal rotation
2 Men Behind Principles and Principles Behind Techniques 27
Table 2.1 (continued)

Contributor Date Contribution
Snow [39, 40] 1986, 1994 Described use of tunica vaginalis wrap, yoke repair
Elder et al. [41] 1987 Described onlay island flap
Retik et al. [42] 1988 Preputial vascular fascia as a second protective layer
Rich et al. [43] 1989 Described hinging of the urethral plate combined with Mathieu repair
Snodgrass [44] 1994 Described tubularized incised plate urethroplasty
Bracka [45, 46] 1995a, b Popularized two-stage repair with free skin graft
Hadidi [47–56] 1996, 2003, Developed “double Y glanulomeatoplasty” for glanular hypospadias,
2004, 2009, Y-V glanuloplasty and SLAM for distal hypospadias, LABO for
2010, 2012, proximal hypospadias without severe chordee, BILAB and CEDU for
2014, 2018 perineal hypospadias and proximal hypospadias with severe chordee,
described the TALE, founded the Hypospadias International Society,
developed the Hypospadias International Classification (MCGU) and
questioned the role of dressing and stenting
Decter [57] 1999 Described chordee correction by split and roll technique
Boddy and Samuel [58] 2000 Described excision of a triangle from apex of Mathieu flap for distal
types
Pippi Salle [59] 2016 Described multiple superficial corporotomies for severe chordee
1. Abnormal ventral curvature or chordee, by

orthoplasty
2. Abnormal proximal meatal insertion, by
urethroplasty
3. Abnormal-looking glans penis, by glanulo-
plasty and meatoplasty
4. Abnormal-looking prepuce, either by circum-
cision or prepuce reconstruction
2.2 Abnormal Ventral Curvature

of the Penis (Chordee)
and Orthoplasty
The history of chordee is mentioned in detail in the

History Chapter (see Chap. 1), and the different Fig. 2.1 Artificial erection test described by Gittes and
MacLaughlin (1974) [33]
approaches are mentioned in detail in the Chordee
Chapter (see Chap. 8). Here, we will try to describe
the principles behind the different approaches. There are three types of chordee associated
Gittes and McLaughlin, writing in 1974, with hypospadias. The first is the chordee that is
described intraoperative saline inflation of the occasionally present in patients with distal hypo-
corpora cavernosa. This guided and ensured suc- spadias (skin chordee). This superficial chordee
cessful orthoplasty. This artificial erection test is subcutaneous, is proximal to the meatus, and is
has been refined with saline and transglanular usually corrected by mobilization of the skin
needle placement [33] (Fig. 2.1). proximal to the meatus [60, 61] (Fig. 2.2).
28 A. T. Hadidi
Fig. 2.2 The superficial chordee associated with distal hypospadias (uncommon, usually proximal to the meatus, and
subcutaneous)
The second type of chordee is commonly a

associated with proximal hypospadias or deep
chordee. It is usually deep, fibrous, and located
distal to the meatus. It is due to inadequate or
arrested distal migration of the hypoplastic cor-
pus spongiosum. There are three basic techniques
to correct this type of deep, hypoplastic chordee
(Fig. 2.3).
(a) The abnormal ventral curvature can be
corrected by dorsal plication, first described by
Physick [8] and popularized as the Nesbit pro-
cedure [62], but this has the serious disadvan-
tage of shortening the penis. (b) More
appropriately, the chordee can be corrected by
excision of the ventral subcutaneous fibrous
bands, usually proximal to the meatus in distal
hypospadias (skin chordee). In proximal forms
with severe chordee, curvature can be corrected
by excision of the hypoplastic urethral plate
and the longitudinal layer of tunica albuginea
(tunica albuginea longitudinal excision or
TALE) (see Chap. 8). (c) A third method is by
incising the corpora ventrally at the point of
maximum curvature and putting grafts or flaps.
Another way of correcting chordee is by corpo-
ral rotation, first described by Koff and Eakins
[38]. Decter [57] added midline ventral split-
ting and called it the “split and roll” technique. Fig. 2.3 The deep chordee associated with proximal
Various skin and fascial grafts and flaps have hypospadias (common, distal to the meatus, and fibrous).
been used to cover the resultant defect in multi- Three different basic principles to correct deep chordee:
(a) dorsal plication, (b) Heineke-Mikulicz technique, (c)
stage repair. split and roll technique

30 A. T. Hadidi
2.3 bnormal Proximal Meatal

A 1898 [64] (quoted in Horton 1973 [65]) for
Insertion and Urethroplasty balanic hypospadias (Fig. 2.5).
The idea is to make use of the elasticity of the
To correct hypospadias and achieve a terminal urethra. It is easier to understand the principle
meatus, one may use one of the following basic when one realizes that the adult flaccid penis is
principles or tissues: (1) distal mobilization of the about 8 cm long. During erection, the penile
urethra; (2) skin distal to the meatus; (3) skin prox- length (including the urethra) reaches 15–20 cm
imal to the meatus; (4) preputial skin; (5) com- long. This means that the normal urethra is flexi-
bined prepuce and skin proximal to the meatus; (6) ble and can be stretched. The procedure has the
scrotal skin; (7) dorsal penile skin; and (8) differ- advantage that it is theoretically “risk-free” as the
ent grafts; a protective intermediate layer (Fig. 2.4). urethra remains completely intact. It has the
drawbacks that it can only be applied to very dis-
tal forms of hypospadias (grade I or glanular). If
2.3.1 Urethral Mobilization the stretched urethra is shorter than the stretched
penis, one may risk bringing the glans to the ure-
Urethral mobilization and meatal advancement thra rather than the urethra to the tip of the glans,
was first described by Beck [63] and Hacker in as the penis is not a rigid structure. Some sur-
Fig. 2.4 Different tissues used for correction of hypospadias

Fig. 2.5 Techniques of urethral mobilization: (a) Urethral ventral edge. (d) UGPI modification of MAGPI by Harrison
mobilization first described by Beck [63] and Hacker [64], and Grobbelaar [67], with a V-shaped incision around the
cited in Horton 1973). (b) MAGPI described by Duckett original meatus and with deep glanular wings before ure-
[35] (midline vertical incision closed transversely and thral advancement and upward rotation of the glanular
mobilization). (c) The M configuration by Arap et al. [66], wings. (e) DYG (double Y glanuloplasty) after Hadidi [51]
a modification of MAGPI by placing two sutures on the ((e) ©Ahmed T. Hadidi 2022. All Rights Reserved)
32 A. T. Hadidi
c d
geons reported good results with urethral mobili- wrapped around the urethra. Hadidi in his double
zation [68–71]. This technique is still popular in Y glanuloplasty (DYG) applied the Y-V princi-
some parts of Europe and America [72, 73]. ple to advance the mobile glanular meatus to the
Duckett [35] described the “meatal advance- tip of the glans and avoid the globular-looking
ment and glanuloplasty incorporated” (MAGPI) glans associated with MAGPI technique [51].
procedure, which combines the use of the
Heineke-Mikulicz technique with urethral mobi-
lization in glanular hypospadias characterized 2.3.2 se of Ventral Skin Distal
U
by mobile urethra. Arap and his colleagues, in to the Meatus
1984 [66], modified the MAGPI procedure by
placing two sutures on the ventral skin edge and Reconstruction of a completely epithelialized
forming an “M” configuration. Harrison and neourethra may make use of the ventral skin dis-
Grobbelaar [67] described the urethral advance- tal to the meatus as in the Thiersch technique
ment and glanuloplasty procedure (UGPI), [10], pyramid repair as described by Duckett and
which modifies MAGPI by having a V-shaped Keating [43] for “megameatus intact prepuce” or
incision around the original meatus before mobi- MIP, the glans approximation procedure (GAP)
lization and having deep glanular wings. The by Zaontz [74], distal urethroplasty and glanulo-
meatus is advanced to the tip of the glans, and plasty (DUG) by Stock and Hanna [75] (Fig. 2.6),
two deep glanular wings are rotated upward and and lateral-based onlay (LABO) by Hadidi [52],
Fig. 2.6 Use of ventral skin distal to the meatus to recon- [76]. (c) GAP repair by Zaontz (1989) for glanular hypo-
struct a completely epithelialized neourethra. (a) U-shaped spadias with cleft glans [74]. (d) DUG repair by Stock and
incision as first described by Thiersch (1869) [10]. Notice Hanna (1997) combining a U-shaped incision with a verti-
the U incision is not central in order to avoid suture lines cal midline incision closed transversely [75]. (e) Lateral-
on top of each other. (b) Pyramid repair by Duckett and based onlay flap (LABO) after Hadidi [52] ((e) ©Ahmed
Keating (1989) for megameatus intact prepuce (MIP) T. Hadidi 2022. All Rights Reserved)
34 A. T. Hadidi
where he used the penile skin lateral to the ure- prepuce” (MIP), present in about 6% of cases of
thral plate to reconstruct the new urethra to the tip distal hypospadias, and called it pyramid repair
of the glans. [43]. Stock and Hanna in 1997 [75] combined the
Thiersch, 1869, used two parallel vertical inci- Thiersch-Duplay principle with the longitudinal
sions to wrap the ventral skin distal to the meatus midline incision of MAGPI, closed transversely
around a catheter. He originally described this for (Heineke-Mikulicz).
repair of epispadias [10]. Then Theophile Anger Another principle recommended the use of
(1874, 1875) [77] applied Thiersch’s concepts for the ventral skin distal to the meatus to form an
epispadias to hypospadias. In the same edition of incompletely epithelialized neourethra.
the Bulletin of the Surgical Society of Paris Techniques adopting this principle include
(1874) Duplay of Paris described the three-stage those of Duplay [78], Browne [24], Reddy [79],
procedure for release of chordee and creation of Orkiszewski [80], Rich et al. [43], and
the ventral tube, which was later joined to the Snodgrass [44].
functional meatus [78]. The same principle was Duplay was the first to state that it did not mat-
adopted by Zaontz (1989) for patients with cleft ter whether the central tube was incompletely
glans and glanular hypospadias [74]. Also, formed [12]. He believed that epithelialization
Duckett and Keating used the same principle to would occur to form a channel if the incomplete
correct the defect known as “megameatus intact tube was buried under the lateral flaps. The same
principle was adopted by Browne in his tech- fied the Mathieu technique by mobilizing the ure-
nique described in 1949 [24]. In both techniques, thral plate and making a midline incision to push
the defect was ventral. In 1989, Rich et al. the neourethra deeper between the corpora [85].
described an incision of the urethral plate to The goal was always advancement of the neoure-
obtain a cosmetically acceptable vertical slit thra to the glans tip. Stenosis and fistula were fre-
meatus for Mathieu repair [43] . This dorsal mid- quently the price. Fevre (1961) used a longer
line incision was subsequently adopted for the meatal-based flap and folded it between the glan-
entire length of the urethral plate as a comple- ular wings [83]. Mustardé included a V incision
ment to the Thiersch-Duplay urethroplasty for at the glans to achieve a wider meatus [30].
distal hypospadias by Snodgrass in 1994 [44]. Hadidi included a Y incision at the tip of the
The tubularized incised plate (TIP) urethroplasty glans closed as a V [47]. He further modified the
differs in that the defect is dorsal and the suture technique in 2012 to achieve a terminal wide slit-
line is protected by a preputial subcutaneous fas- like meatus in his technique slit-like adjusted
cial flap (Fig. 2.7). Mathieu or SLAM technique [53] (Fig. 2.8).
Historically, incision of the urethral plate was
first described by Reddy [79], Orkiszewski who
called it “the Deadman jacket” [80], and Rich 2.4.1 Use of Preputial Skin
et al. [43], before Snodgrass popularized the
principle in 1994 [44]. The preputial skin plays a very important role in
the management of hypospadias (Fig. 2.9). It
may be used in different ways:
2.4 se of Ventral Skin Proximal
U
to the Meatus (Meatal-Based 1. The preputial skin may be mobilized ventrally
Flaps) to cover skin and fascial defects following
excision of chordee in two-stage repair.
A well-established group of techniques used the Thiersch did the first buttonhole flap in the
ventral skin proximal to the meatus as a meatal- prepuce to allow resurfacing of the penis with
based flap. This flap is used to form the ventral the prepuce [10].
part of the neourethra as in the technique first 2. The preputial skin may be divided in the mid-
described by Wood [11], Ombrédanne [18, 81], line to form two flaps to cover the skin defi-
and Bevan [82] and popularized by Mathieu in ciency of the penis after chordee resection and
1932 [19]. Fevre [83], Mustardé [30], Barcat [84, after urethroplasty. This was described by
85], and Hadidi (Y-V [47] and SLAM [53]) Edmunds [17] and popularized by Byars [25].
described techniques using the same principle. 3. The preputial skin may be used as a free skin
Wood [11] described a flap based distally on graft to cover ventral defect after excision of
the meatus to be turned over to form the ventral ventral chordee as the first stage of two-stage
surface of the neourethra. repair. This was first described by Cloutier in
Ombrédanne used a perimeatal flap but fash- 1962 [29] and popularized by Bracka [45, 46].
ioned the neourethra using a purse-string suture 4. The preputial skin may be used as a free skin
[81]. The repair was too baggy. Mathieu used a graft to form the neourethra in single-stage
perimeatal flap and constructed the neourethra urethroplasty as first described by Devine and
using two lateral suture lines [19]. Mustardé used Horton in 1961 [27].
the same principle but differed in that he used the 5. The preputial skin may be used as a pedicled
perimeatal flap to form the whole neourethra, not flap for reconstruction of the neourethral
just the ventral surface [30]. This bore the advan- tube. This may be vertical as described by
tage of having a single suture line deep to the ure- Van Hook [13], Toksu [86], and Hodgson
thra but has the disadvantage of losing the blood [87] or horizontal and double-faced as
supply of the healthy urethral plate. Barcat modi- described by Asopa et al. [88]. Duckett [89]
36 A. T. Hadidi
Fig. 2.7 Use of ventral skin distal to the meatus to recon- technique (1949) [24]; (c) hinging of the urethral plate
struct a partially epithelialized neourethra: (a) Duplay (Rich et al. 1989) [43]; (d) TIP urethroplasty (1994) [44]
incomplete urethroplasty (1880) [12]; (b) Denis-Browne
adopted this technique using the inner face of 8. The preputial skin may be used in continua-
the prepuce in a horizontal manner now tion with a parameatal skin flap as in Koyanagi
known as the tubularized preputial island flap et al. [37], yoke repair (Snow) [91], and along
technique (TPIF). Standoli described the with its fascia in the lateral-based flap tech-
same technique but used the outer face of the nique (Hadidi) [50], bilateral-based flap
prepuce [90]. (BILAB, Hadidi) [92], lateral-based onlay
6. The preputial skin may be used as a pedicled (LABO, Hadidi) [52], and chordee excision
flap to form the ventral wall of the neourethra and distal urethroplasty (CEDU, Hadidi) [55].
(not the whole neourethra), as described by
Elder et al. in the technique known as onlay
island flap (OIF) in 1987 [41]. 2.4.2 ombined Use of Prepuce
C
7. The preputial vascular fascia without skin and Skin Proximal
may be used as a protective second layer to to the Meatus
protect urethroplasty in the same way as after
Mathieu and Snodgrass procedures, as first In proximal hypospadias, one needs to recon-
described by Retik et al. in 1988 [42]. struct a long neourethra. Another principle
38 A. T. Hadidi
Fig. 2.8 Use of ventral skin proximal to the meatus to nique and a deep midline incision. (f) Hadidi (1996): Y-V
reconstruct a fully epithelialized neourethra. (a) Wood glanuloplasty, modified Mathieu—a Y incision in the
(1875): meatal-based flap with buttonhole of prepuce. (b) glans, the center at the tip of glans, closed as a V, and dog-
Ombrédanne (1911): a large round flap and a purse-string ears opened. A small V is excised from the distal end of
suture. (c) Mathieu (1932): a U-shaped incision and two the flap. (g) The slit-like adjusted Mathieu (SLAM) after
suture lines. (d) Mustardé (1965): a rectangular flap and Hadidi [53] ((g) ©Ahmed T. Hadidi 2022. All Rights
one suture line. (e) Barcat (1969): balanic groove tech- Reserved)

40 A. T. Hadidi


42 A. T. Hadidi
Fig. 2.9 Use of preputial skin for reconstruction of neo- thra as described by Devine and Horton [27]. (e) Preputial
urethra. (a) Buttonholing of the prepuce as described by island flap as described by Hook [13], Toksu [86],
Thiersch [10]. (b) Midline incision of the prepuce as Hodgson [87], Asopa [88], and Duckett et al. [89]. (f)
described by Edmunds [17] and Byars [25]. (c) Preputial Onlay island flap as described by Elder et al. [41]. (g)
skin as a skin graft to cover the ventral defect of the penis Preputial vascular fascia as a second protective layer as
as described by Nové-Josserand [16] and Bracka [45, 46]. described by Retik et al. [42]
(d) Preputial skin as a free skin graft to form the neoure-

44 A. T. Hadidi

employs the combined use of parameatal skin the technique by using two lateral flaps (from
and the prepuce. This was first suggested by van both sides). Snow [91] described the yoke tech-
Hook [13]. Many authors, including Broadbent nique, which differed from Koyanagi in button-
et al. [28], DesPrez et al. [26], Hinderer [93], holing the prepuce. Hadidi described the
Koyanagi et al. [37], Snow [91], and Hadidi [50, lateral-based flap technique using the same prin-
52, 54, 55], have described techniques using the ciple and combined it with Y-V glanuloplasty
same principle. [47]. The technique entails adequate mobiliza-
Van Hook [13] suggested the use of a “lateral tion of the preputial vascular fascia with the flap.
oblique flap” from the side of the penis. Broadbent The lateral-based flap enjoys a double blood sup-
et al. [28], DesPrez et al. [26], and Hinderer [93] ply from the meatal base and the preputial ves-
adopted the same principle and described a flap sels. The Y helps to bring the meatus to the tip,
extending obliquely from the parameatal skin and the V excised from the tube helps to achieve
into the prepuce. Koyanagi et al. [37] modified a terminal slit-like meatus (Fig. 2.10).
46 A. T. Hadidi
a
b
Fig. 2.10 Combined use of prepuce and the skin proxi- based flap combined with Y-V glanuloplasty described by
mal to the meatus. (a) Lateral oblique flap from the side of Hadidi [47]. (f) Bilateral-based flap (BILAB) after Hadidi
the penis suggested by Hook [13]. (b) One-stage repair for [54]. (g) Chordee excision and distal urethroplasty
proximal hypospadias described by Broadbent et al. [28]. (CEDU) after Hadidi [55] ((f, g) ©Ahmed T. Hadidi 2022.
(c) Parameatal foreskin flap described by Koyanagi et al. All Rights Reserved)
[37]. (d) Yoke repair described by Snow [91]. (e) Lateral-

48 A. T. Hadidi

2.4.3 Use of the Scrotum 1. The scrotal skin may be used to form a com-
pletely epithelialized neourethra. Bouisson
The scrotum may be used in four different ways was apparently the first to report the use of
in hypospadias reconstruction (Fig. 2.11): scrotal tissue to reconstruct the ventral wall
Fig. 2.11 Use of

scrotum in hypospadias a
repair. (a) Bouisson was
the first to use scrotal skin
for urethral reconstruction
[9]. (b) Rosenberger used
scrotal tissue for
urethroplasty and buried
the penis in the scrotum
[97]. (c) Rochet used a
large scrotal flap for total
urethroplasty [94]. (d)
Lowsley and Begg
constructed a long
urethral tube from
scrotum [95]. (e) Beck
suggested the Duplay
type of urethroplasty and
used a rotation flap from
the scrotum for coverage
[63]. (f) Cecil used a
modification of the b
Rosenberger operation
following reconstruction
of the urethra from
ventral penile skin [22]
50 A. T. Hadidi


e
f
52 A. T. Hadidi
of the neourethra [9]. Rochet used a large this tube was passed through a channel in the
scrotal flap for urethroplasty [94]. This flap glans and penis by angulating the penis acutely
was buried in a tunnel on the ventral surface upward and backward. In the second stage, the
of the penis. Lowsley and Begg constructed proximal pedicle was cut and the penis returned to
the neourethra completely from the scrotum its normal position. The penile gymnastics
[95]. This method fell into disuse because of required for the Davis procedure apparently
the problem of hair growth into the seemed too demanding for most surgeons.
neourethra. Hodgson [87] and Perovic [102] used longitudi-
2. The scrotal skin may be used to cover the nal dorsal skin flaps to reconstruct the new
neourethra. This was described by Beck in urethra.
1897 [96].
3. The scrotal skin may be used to reconstruct
the neourethra and at the same time the scro- 2.4.5 Use of Grafts
tum is used to protect the neourethra until
healing is complete. Rosenberger [97], Nové-Josserand in 1897 started another school
Landerer [98], and Bidder [99], used scrotal of urethroplasty which utilized the free inlay
skin for urethroplasty. They described for the graft [16]. He used a thin split-thickness free
first time burying of the penis in the scrotum graft from the inner thigh and applied the raw
to obtain skin coverage. This was modified by surface outward around a metal probe. Sir
Bucknall [100]. Archibald McIndoe used a partial-thickness
4. The scrotum may be used as a bed for the neo- skin graft from the inner upper arm [103].
urethra, a technique that was popularized by Young and Benjamin also used a split-thickness
Cecil [22] and Culp in 1966 [101] (Cecil-Culp skin graft from the medial aspect of the upper
technique). arm [104]. From 1909 to 1927, a whole series
of homograft including vein, urethra, and
appendix were attempted but never with any
2.4.4 Use of Dorsal Penile Skin consistent success. Cloutier used the full thick-
ness prepuce as the graft material after excising
Davis, in 1940, tubed the dorsal penile skin with the chordee using a T-shaped incision into the
the base proximal in the direction of the circula- glans [29]. He then stitched the graft to the
tion [20] (Fig. 2.12). The detached distal end of edges of the resulting defect (including the
Fig. 2.12 Davis operation (1940) using a dorsal tube pedicle flap to construct the neourethra [20]
glans). In addition, he quilted the graft to the of buccal mucosa for hypospadias repair,
center to ensure better take of the graft. Nicolle Duckett in 1986 [106] promoted the technique
[105] and Bracka [45] popularized the use of and is credited for the current enthusiasm and
full-
thickness skin graft from the prepuce. widespread acceptance of its use in complex
Memmelaar, in 1947, was the first to advocate hypospadias repairs (Fig. 2.13). Fine et al.
the use of bladder mucosa [23]. Although described tunneling of buccal mucosal tube
Humby [21] first proposed and reported the use graft for proximal hypospadias [107].
Fig. 2.13 Use of grafts

for urethral a
reconstruction. (a)
Nové-Josserand used a
split-thickness skin graft
on a metal probe [16],
(b) Devine and Horton
used preputial full-
thickness skin graft in a
single-stage repair [27].
(c) Bracka used
full-thickness skin graft
in a two-stage repair [45,
46]. (d) Memmelaar
used bladder mucosa for
urethral reconstruction
[23]. (e) Humby (1941)
first described the use of
buccal mucosa for
urethral reconstruction b
[21]
54 A. T. Hadidi
c d
2.5 Use of a Protective the use of an intermediate or interposition layer

Intermediate Layer between the neourethra and the cutaneous suture
[32]. Types of interposition waterproofing layer
An important principle in hypospadias repair is include Smith’s de-epithelialized skin [32],
to avoid having the skin suture line on top of the Snow’s tunica vaginalis wrap from the testicular
urethroplasty suture line. Thiersch as early as coverings [39], Retik et al.’s dorsal subcutaneous
1868 designed his asymmetric U-shaped incision flap from the foreskin [42], Motiwala’s dartos
to avoid having the two suture lines on top of flap from the scrotum [108], and Yamataka et al.’s
each other [10]. Smith was the first to describe external spermatic fascia flap [109] (Fig. 2.14).
Fig. 2.14 Methods for

protective intermediate a
layer. (a) Smith
de-epithelialization [32].
(b) Snow described the
use of a tunica vaginalis
wrap [39]. (c) Retik
et al. was the first to use
a dorsal subcutaneous
flap from the prepuce
[42]. (d) Motiwala
described the use of a
dartos flap from the
scrotum [108]. (e)
Yamataka et al. reported
the use of an external
spermatic fascia flap b c
[109]
e
d
56 A. T. Hadidi
2.6 Abnormal-Looking Glans rated” (MAGPI) procedure [35]. Arap et al.

Penis and Glanuloplasty modified the MAGPI technique by using
and Meatoplasty two sutures instead of one [66]. Decter, in
1991, described an “M inverted V” tech-
Thus, various tubes and patches were available to nique [117].
reconstruct the neourethra. The next step was to (f) Rich et al. described incising the urethral
bring the neourethra to the tip of the glans. The plate in the midline (hinging) [43]. This
glans had always posed a challenge and had helped to achieve a slit-like vertical
largely not been found amenable to tunneling. meatus.
Canalization, tunneling, and coring are essen- (g) Snodgrass extended the concept of urethral
tially the same, with progressively larger chan- plate hinging by incising the whole urethral
nels. These are a testimony to glans stenosis. plate in the midline from the hypospadiac
There are several techniques employed to meatus distally [44]. This helps in tubulariza-
achieve an apical meatus (Fig. 2.15): tion of the plate.
(h) Hadidi, in 1996, described the Y-V glanulo-
(a) Russell (1900) described the glans channel plasty [47]. The center of the Y is at the tip of
technique to deliver the urethra to the apex of the glans. Each limb is 0.5 cm long and the
the glans [110]. Bevan (1917) [82], Davis deep incision is closed as a V. The dog-ears
(1940) [20], Ricketson (1958) [111], Duckett created are widely opened to increase the cir-
(1980b) [112], and Hendren (1981) [113] cumference. A V is excised from the distal
used the same principle but different flaps or end of the neourethra to achieve a slit-like
grafts. meatus. The Y-V glanuloplasty can be com-
(b) Wing rotation is used in most recent bined with most techniques of hypospadias
techniques. repair, e.g., Mathieu, Onlay, Duplay, trans-
(c) Devine and Horton [27] and Mustardé [30] verse preputial island flap urethroplasty, and
popularized the glans channel procedure and lateral-based flap.
included a dorsal V-flap with the glans (i) Boddy and Samuel described the “Mathieu
channel. and V incision sutured” (MAVIS) technique,
(d) The glans split has been used in various tech- which results in a vertical slit meatus [58]. In
niques to move the meatus to the apex [15, this technique a V incision is made and
21, 85, 114–116]. excised at the apex of the parameatal-based
(e) Duckett, in 1981, described the “meatal flap. Then each side of the V is sutured to the
advancement and glanuloplasty incorpo- glanular wings.
Fig. 2.15 Techniques of glanuloplasty. (a) Glans tunnel- plasty (Hadidi 1996) [47]. (g) Hinging of the urethral plate
ing, canalization, or coring. (b) Wing rotation. (c) Glans V [43]. (h) The tubularized incised plate (Snodgrass 1994)
in the posterior wall by Devine and Horton [27] and follows the same principle [44]. (i) MAVIS, a modification
Mustardé [30]. (d) Glans splitting or kippering has been of the Mathieu technique, excises a triangle from the apex
used for 1000 years. (e) MAGPI [35]. (f) Y-V glanulo- of the parameatal flap to create a slit-like meatus [58]
58 A. T. Hadidi
c d


60 A. T. Hadidi
13. Van HW. A new operation for hypo-

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struction. Br J Urol. 1993;72:260.
Normal Development of the Penis
and Urethra 3
Ahmed T. Hadidi
Abbreviations Some authors suggested that the basic devel-

opmental processes are analogous in mice and
AM Anal membrane humans [17]. Hsu et al. showed that the rat penis
AT Anal tubercle and dog penis contain a short bone called os penis
CR Crown-rump which has a high concentration of androgen
CRL Crown-rump length receptors (Fig. 3.1) [18]. Hadidi et al. postulated
GA Gestational age that the developmental process in humans differs
GL Glans from that in many other animal species [15]. This
GM Genital membrane was further confirmed by Liu et al. [19].
GT Genital tubercle Confusion also arises from the considerable
H&E Hematoxylin and eosin differences in length or age of the embryos and
ICPs Intracavernosal pillars fetuses at the same developmental stage. Several
LS Longitudinal section references, although depending on “Streeter
LSF Labioscrotal folds Horizon Series,” Carnegie Institute of Washington
UF Urogenital fold [20], yet have different relations between the
crown-rump length (CRL) of the embryo and its
corresponding gestational age. For example, the
10th gestational week corresponds to 60 mm
3.1 Introduction CRL according to England (1990) and to
27–35 mm CRL according to Jirasek [21]. In
The embryology of the penis and urogenital addition, after week 12, flexion makes CRL mea-
region has been controversial and confusing. This surement unreliable and biparietal diameter is
is related to the fact that in literature, observa- measured till week 24. In this chapter we will use
tions have been extrapolated to the human from the timetable of O’Rahilly [22] for embryos with
many different species. There are reports in the postovulatory age modified to gestational age,
literature describing mice [1–7], pigs [8], dogs and of Moore and Persaud [23] for fetuses over
[9], and humans [10–16]. 10 weeks’ gestational age (over 32 mm CRL).
A third factor of confusion is terminology.
Simple terms like ventral and dorsal have differ-
A. T. Hadidi (*) ent meanings for embryologists and clinicians as
Hypospadias Center and Pediatric Surgery the penis changes position from projecting cau-
Department, Sana Klinikum Offenbach, Academic dally early in development to project cranially
Teaching Hospital of the Johann Wolfgang Goethe
University, Offenbach, Frankfurt, Germany when a patient with a fully developed penis lies

66 A. T. Hadidi
a d
b e
c f
Fig. 3.1 Comparison of penile structure in various spe- junction between the glans penis and the corpus caverno-
cies; cross sections of the corpora cavernosal and longitu- sum is similar to a knee joint and provides a flipping
dinal aspect of the glans penis are from (a) to (c) and (d) action during mating. The short os penis (right panel, 1×)
to (f), respectively. (a) In rats, the corpus cavernosum, can be better demonstrated after clearing and alizarin red
devoid of the medial septum and intracavernosal pillars S staining because only bony tissue is observed. (e) In a
(ICPs), is positioned between the deep dorsal vein (arrow) dog, the os penis (double-headed arrow) is enveloped with
and the urethra (arrowhead) (hematoxylin-eosin reduced a unique glans penis of two compartments (arrowhead and
from 7×). (b) In dogs, a complete septum (arrow) and arrow). (f) In a human being, an aggregation of the outer
abundant ICPs are obvious (1×). (c) In human beings, an longitudinal layer of the tunica albuginea acts as a buttress
ICP is not uncommonly encountered. A septum (arrow) is for the glans penis (1×). (From Hsu et al.: “Distal ligament
significant but is incomplete and dorsally fenestrated. (d) in human glans: A comparative study of penile architec-
In a rat, a short os penis is positioned between the glans ture”, Journal of Andrology, Vol 26, Issue 5, pp 624–628,
penis and the corpus cavernosum (left panel, 3×). The ©2005 with permission [18])
on his back. In this chapter, we follow the clinical penile urethra refers to the urethra within the
terms; that is, ventral means the undersurface of shaft and root of the penis and the term glanular
the erect penis (towards the foot), dorsal means urethra refers to the part of the urethra contained
the upper surface of the erect penis (towards the within the glans penis.
head), anterior means towards the front of the The description in this chapter is an up-to-
body, posterior means towards the back (verte- date description of the embryological develop-
bral column), distal means towards the tip of the ment of the penis and urethra based on the
glans penis, and proximal means towards the root histochemical study of 15 human embryos and
of the penis and scrotum. The term proximal fetuses [10, 13, 15, 21, 24–26] and the more
3 Normal Development of the Penis and Urethra 67
recent publications in this field [16]. This was 3.2 The Cloaca
supported by the clinical and operative findings
of more than 8000 patients with hypospadias, At about 6.5 weeks’ gestation (3–5 mm total
intrauterine photos, and other histological stud- length), the distal part of the hind gut widens into a
ies available in literature. The chapter starts by chamber. This chamber receives the allantois ante-
describing the developmental process, supported riorly and the gut posteriorly. It continues distally
by intrauterine photos and histological sections. as a narrow blind-ended tube called the tail gut
Under Sect. 3.3, review of literature and current (Fig. 3.2a). The fusion of the mesonephric ducts
theories of urethral development are discussed. with the lateral wall of this chamber marks the tran-
The histological and clinical findings have sition of this chamber into the cloaca. This endo-
resulted in the formation of the “migration dermal high columnar epithelium-lined chamber
hypothesis” to explain the development of penis will form the most caudal part of the definitive gut
and urethra. tube in the developing embryo (Fig. 3.2b).
a b c
d e
Fig. 3.2 Development of the cloaca and cloacal mem- rior anal compartments. Notice how the cloacal mem-
brane. (a) The distal end of the hind gut continues distally brane changes from being vertical into transverse. (e) The
as the tail gut. (b) It is called the cloaca after receiving the cloacal membrane disintegrates first posteriorly providing
mesonephric duct. (c) The tail gut disappears completely. one cloacal orifice for both compartments. (©Ahmed
(d) The cloaca divides into anterior urogenital and poste- T. Hadidi 2022. All Rights Reserved)
68 A. T. Hadidi
During the 6.5–8 weeks’ period (5–15 mm form a solid plate of cells called the urogenital
CRL, 40–49 days’ gestation), the cloaca divides plate.
internally into urogenital and anal compart- The posterior (dorsal) part of the cloacal
ments. Externally, the cloaca eminence develops membrane becomes gradually thinner and disin-
and forms the genital tubercle and urogenital tegrates to open into the amniotic cavity. It thus
folds. provides initially a single opening, the cloacal
The simple chamber-like cloaca becomes a orifice, for both urogenital and anal compart-
U-shaped structure due to an indentation, includ- ments (Fig. 3.2c). This means that, for a short
ing the coelom, that grows cranio-caudally and while, the orifices of the urogenital sinus and anal
divides the cloaca into anterior urogenital and compartment open separately connected by a
posterior anal compartments (Fig. 3.2d). The short cloacal groove which before long is incor-
communication between both compartments porated into the surface of the perineum
becomes gradually narrower (Fig. 3.2e). (Fig. 3.2d, e). Rapid proliferation of the urogeni-
Meanwhile, the tail gut disintegrates and disap- tal compartment follows which results in both
pears completely (Fig. 3.2c, d). urogenital and anal orifices being visible on the
surface (Fig. 3.3a).
3.2.1 Development and Fate

of the Cloacal Membrane 3.2.2 Development
of the Urogenital Fold
The cloacal membrane is the membrane that
forms the floor and limits the cloacal cavity ven- Early during the formation of the cloaca mesen-
trally in the midline. Here, the high endoderm- chyme migrates from the primitive streak and
derived columnar (pseudostratified) epithelium caudal eminence and proliferates around the clo-
of the cloaca (from inside) comes in contact with acal membrane to form a slightly elevated cloa-
the ectoderm-derived cuboidal epithelium of the cal eminence. The anterior part of the eminence
epidermis (from outside) (Fig. 3.2a). proliferates cranially to form the genital tubercle
The distal part of the cloacal membrane forms (Fig. 3.4). The lateral part forms the labia-like
a small plate which divides the ventral part of the urogenital folds anteriorly on either side of the
developing glans. This glans plate is already vis- cloacal membrane and the posterior part forms
ible in embryos as young as 6 weeks (8 mm) and anal folds posteriorly (Fig. 3.5).
will differentiate in solid squamous epithelium In the meantime, another pair of elevations,
[10]. the labioscrotal swellings, becomes visible on
Due to rapid proliferation of the infraumbili- each side of (lateral to) the urogenital folds.
cal mesenchyme in the midline, the cloacal cav- These swellings later form the scrotal swellings
ity is displaced caudally. The orientation of the in the male and the labia majora in the female
cloacal membrane gradually changes from being (Fig. 3.6).
vertical facing anteriorly to become horizontal
(Fig. 3.2a–c).
The cloacal membrane is now a longitudinal 3.2.3 Development
slit-like membrane that extends from the genital of the Urogenital Plate
tubercle ventrally (anteriorly) to the small post-
anal swellings posteriorly (Figs. 3.4, 3.5, and Between 6.5 and 8 weeks’ gestation (5–15 mm
3.6). It is opposed side-to-side in the sagittal CRL, 40–49 days’ gestation), the cloacal mem-
plane by the urogenital folds. brane transforms into a thick cloacal plate ven-
The anterior (ventral) part of the cloacal trally and disintegrates dorsally, where it opens
membrane becomes thickened (four layers) to the urogenital and anal orifices and a short cloa-
a b
c d
Fig. 3.3 Development of the penile urethra. (a) The cloa- orifice gradually migrates forward to reach the coronal
cal membrane continues to disintegrate to form the cloa- sulcus. (d) The glanular urethra has a different origin from
cal groove. (b) Gradually, both urogenital and anal orifices the glans plate. (©Ahmed T. Hadidi 2022. All Rights
reach the surface. (c) The posterior edge of the urogenital Reserved)
cal groove in between (Fig. 3.2c). Thus it pro- From the beginning, the solid urethral plate
vides initially a single opening, the cloacal (the remaining part of the cloacal membrane) has
orifice, for both urogenital and anal compart- two components: the glans plate (squamous,
ments (Fig. 3.2c). This means that, for a short ectodermal) and the penile plate (columnar,
while, the orifices of the urogenital sinus and anal endodermal). The point of junction will eventu-
compartment, separated by a bar, open separately ally be the fossa navicularis after complete devel-
into a common short cloacal groove (Fig. 3.2d, opment. As shown in Figs. 3.9 and 3.11, there is
e). Rapid proliferation of the urogenital compart- vacuolation of the urethral plate both in the glans
ment follows, which results in both urogenital region and in the penile region. Vacuolation of
and anal orifices reaching the surface. The c loacal the glans plate results in the formation of the
groove will be incorporated into the surface of temporary glans canal and the “epithelial tag.”
the perineum where the perineal raphe will form Vacuolation of the penile plate results in groov-
(Fig. 3.3). ing of the urethral plate without a canal (contrary
70 A. T. Hadidi
a b
Fig. 3.4 The genital tubercle appears at 6 weeks. The same embryo. (From: An Atlas of Human Prenatal
cloaca membrane is delineated by urogenital folds (UF). Developmental Mechanics, by Jan E. Jirasek, © 2004.
(a) Anterior is the genital tubercle (GT) and lateral cau- Reproduced by permission of Taylor & Francis Group
dally is the anal tubercle (AT). (b) The anterior view of the [21])
The urogenital plate becomes more superficial

due to rapid growth of the anterior (ventral part)
of the cloacal eminence. Later, vacuolation
results in the formation of the urethral groove and
widens the already existing urogenital orifice
(Fig. 3.7).
Until the end of the 9th week, it is impossible
to differentiate between the two sexes.
3.2.4 Development of the Male

Penile (Spongy) Urethra
3.2.4.1 Penile Urethra

In 8-week-gestation embryos (15 mm), the uro-
Fig. 3.5 The cloaca membrane has two components: gen-
genital compartment opens in the perineum close
ital membrane (GM) component and anal membrane com- to the anal orifice and becomes the urogenital
ponent (AM). (From: An Atlas of Human Prenatal sinus. Vacuolation of the central part of the uro-
Developmental Mechanics, by Jan E. Jirasek, © 2004. genital plate results in grooving and widening of
Reproduced by permission of Taylor & Francis Group
[21])
the urological orifice to extend from the perineum,
near the anal orifice till the base of the glans
(Figs. 3.7, 3.8, 3.9, 3.10, 3.11, and 3.12).
to what happens in some animal species). Aborted Early in development, the urethral orifice is a
vacuolation may result in accessory urethra. It longitudinal slit or oval opening (delta) that
seems reasonable to assume that the vacuolated extends from the perineum, near the anal orifice
cells of the urethral plate in the glans region form posteriorly to the base of the glans anteriorly, and
the so-called epithelial tag. is bounded laterally by the urethral labia (or uro-
a b
Fig. 3.6 (a) The early indifferent external genitalia are (G = GL), CCB the corpora cavernosa bodies, UF the ure-
formed by the phallus which is composed of the glans thral folds. The urethral groove lies between the urethral
(GL), the corpora cavernosa bodies, and the urogenital folds. (From: An Atlas of Human Prenatal Developmental
groove (arrow) with two urogenital folds. Laterally are the Mechanics, by Jan E. Jirasek, © 2004. Reproduced by
labioscrotal folds (LSF). (b) Electron microscope photo; permission of Taylor & Francis Group [21])
genital folds). Rapid growth of the urogenital

sinus and surrounding erectile and fascial pri-
mordial results in a lengthening of the penile ure-
thra and the formation of the penile shaft
(Figs. 3.13 and 3.14).
Due to differential rapid proliferation of the
ventral aspect of the genital tubercle, more than
the dorsal (cranial) aspect, there is gradual ante-
rior shift of the posterior edge of the urethral
opening. Consequently, the urethral opening
becomes gradually smaller, until the posterior
edge reaches the base of the glans (Fig. 3.3).
3.2.4.2 Glanular urethra

Examination of the 15 human specimens [15]
showed that during the 6th and 7th weeks of ges-
tation, the genital tubercle is conical, and a glans
penis could not be identified. The earliest indica-
tion of a coronal sulcus and glans penis differen-
Fig. 3.7 The early indifferent external genitalia and the tiation could be seen during the 8th week of
urogenital groove. (©Ahmed T. Hadidi 2022. All Rights gestation (embryos 6–10). An epithelial tag was
Reserved)
evident in the older embryos included in the
72 A. T. Hadidi
a b
Fig. 3.8 A 6-week-old embryo. (a) The genital tubercle homogenous, and not differentiated to form corpus caver-
is conical in shape with a longitudinal scar dorsally and a nosum. The mesenchyme on either side of the urethral
deep groove ventrally that does not reach the tip ×10. (b) plate is proliferating to raise low folds, covered by surface
A glans cannot be identified ×10. (c) The urethral plate is epithelium (magnified ×40). (©Ahmed T. Hadidi 2022.
a bilamellar structure. The whole mesenchyme is diffuse, All Rights Reserved)
study (13th and 14th weeks of gestation) mal). The point of junction will eventually be the
(Figs. 3.11 and 3.12). fossa navicularis after complete development.
A solid urethral plate reached the tip of the Central vacuolation resulted in a well-defined
genital tubercle during the 6th week of gestation blind central opening in the 13th week of gesta-
(20 mm long). The solid urethral plate (the tion (Fig. 3.15). This central glanular canal was
remaining part of the cloacal membrane) has two not continuous with the urethral groove of the
components: the glans plate (squamous, ectoder- penile shaft. During the 14th week of gestation,
mal) and the penile plate (columnar, endoder- the floor of the glanular canal started to degener-
a b c
d e f
Fig. 3.9 (a) Embryo 7, week 8: Serial histological sec- the epithelial edges resulting in the formation of an epi-
tions through proximal penis in the region of the urethral thelial plate of a double layer of epithelium is demon-
orifice during penile urethra formation demonstrate the strated. (d–f) 3: Apoptosis within this epithelial plate
three phases of fusion between epithelial structures (from results in disruption of the two epithelial layers including
distal to proximal, stained with hematoxylin and eosin their basement membrane after which the process of
×10); (b) 1: Proliferation of the epithelium and condensa- fusion is completed and the mesenchyme becomes in con-
tion of the underlying mesenchyme. (c) 2: Adherence of tinuity. (©Ahmed T. Hadidi 2022. All Rights Reserved)
74 A. T. Hadidi
a b c
Fig. 3.10 An 8-week-old embryo. Histological sections lial layers including their basement membrane after which
stained with the monoclonal antibody MIB-1 that reacts the process of fusion is completed and the mesenchyme
with the Ki-67 nuclear antigen, to label proliferating cells becomes in continuity ×40. (c) Same section b magnified
at the critical fusion point demonstrating two phases of ×50: The area of apoptosis was magnified to show the dis-
fusion between epithelial structures (from distal to proxi- ruption of the basement membrane before the proliferat-
mal): (a) 1: Adherence of the epithelial edges resulting in ing mesenchymal cells (brown) on either side become in
the formation of an epithelial plate of a double layer of continuity. (©Ahmed T. Hadidi 2022. All Rights
epithelium is demonstrated ×20 (b) 2: Apoptosis within Reserved)
this epithelial plate results in disruption of the two epithe-
ate and disappear gradually forming a deep glan- Careful re-examination of the histological
ular groove that became in continuity with the section in Baskin et al. [25] of a normal fetal
urethral groove of the shaft of the penis. glans penis at 25 weeks of gestation (a) and a
Histological sections of the 15 specimens full-term fetus (b) showed that the roof and lat-
showed the first three stages of glanular urethra eral walls are vertically oriented and lined by
development (Figs. 3.16 and 3.17): stratified squamous epithelium. The floor is hori-
1. A solid glanular epithelial plate (Fig. 3.16a). zontally oriented and is lined with pseudostrati-
2. A blind central glanular canal (Fig. 3.16b): fied columnar epithelium (Fig. 3.19).
The floor of the blind central canal starts to Van der Werff et al. [1] showed a very impor-
degenerate and disappear (Fig. 3.16c). tant frontal section through the phallus of a
3. A deep glanular urethral groove (Fig. 3.16d). 76 mm human embryo. The penile urethra was
Careful re-examination of the human histo- not completely developed and the emerging pre-
logical sections included in Kurzrock et al. [27] puce covered only the proximal glans (Fig. 3.20).
clearly documented the four stages of glanular Clinically, one sees often a bridge between the
urethra development: a solid glanular urethral proximal penile urethra and the blind glanular
plate in 9-week-old embryo, a glanular canal in canal (Fig. 3.21).
12-week-old embryo, a deep glanular groove in The ventral (caudal) rapid proliferation also
14-week-old embryo, and a completed glanular involves the median raphe which originates from
floor in 19-week-old embryo (Fig. 3.18). stroma at the perineum and migrates together
In Fig. 3.18a, cytokeratin K14 stained positively with the posterior urethral edge until it reaches
the epithelium of the roof and the lateral walls of the base of the glans. Further growth and migra-
the glanular urethra (vertically oriented, stratified tion of the penile septum and median raphe result
squamous epithelium) but NOT the floor of the in the formation of the frenulum and ventral
glanular urethra (horizontally oriented, pseu- median part of the prepuce to form the floor of
dostratified columnar epithelium). Positive staining the glanular urethra and the frenulum (Figs. 3.12,
for K14 did not occur in other areas of the urethra. 3.13, 3.22, and 3.23).
b c d
e f g
Fig. 3.11 A 14-week-old fetus. (a) The genital tubercle there is gradual vacuolation and increased grooving as one
is cylindrical with mild chordee and a coronary sulcus has progresses proximally (b, c). There is proliferation of the
appeared. The terminal tag and well-developed genital overlying epithelium (d, e) which adheres to each other
swellings are identified. The urethral groove extends from (f). Proximally, there is apoptosis of the epithelial seam
the base to the coronary sulcus. Histological examination and the mesenchyme becomes in continuity (g) (magni-
shows migration of the mesenchyme and undivided cor- fied). (©Ahmed T. Hadidi 2022. All Rights Reserved)
pus cavernosum ×20. The urethral plate is partly solid and
3.3 Discussion Fleishmann [32] in animals and Politzer [33]

in humans contradicted this concept. They
3.3.1 he Fate of the Cloacal
T believed that the urorectal septum does not fuse
Membrane with the cloacal membrane. On the contrary, they
assumed that the cloacal membrane first breaks
Keibel (1912) suggested that the urorectal sep- down in its dorsal region, and that this opening is
tum fuses with the cloacal membrane leading to then subdivided into an anal and urogenital ori-
its subdivision into the ventral “urogenital mem- fice by the urorectal septum which forms the
brane” and the dorsal “anal membrane” [28]. The body of the perineum.
urogenital membrane is thought to disintegrate, Penington and Hutson showed that the changes
providing the “urogenital sinus” with a first open- in the cloacal plate compared with growth and
ing. This view was shared by many investigators apoptosis in adjacent structures vary in timing
in the past [29, 30] and is still done by some across species [5].
authors [23, 31].
76 A. T. Hadidi
a b
Fig. 3.12 Masculinization of the external genitalia and plate, the epithelial tag has disappeared, and the urethral
lengthening of the anogenital distance. (a) The distal por- opening has migrated distally. (From: An Atlas of Human
tion of the urogenital groove is still open and epithelial tag Prenatal Developmental Mechanics, by Jan E. Jirasek, ©
can be seen for a short time at the tip of the glans. (b) A 2004. Reproduced by permission of Taylor & Francis
groove has appeared on the undersurface of the glans Group [21])
Tourneux [7], van der Putte [34, 35], and

Penington and Hutson [5] suggested that the cloa-
cal membrane is rather a plate than a membrane in
sheep, pigs, and humans. In later development, the
dorsal part of the cloacal plate loses height as the
tip of the urorectal septum approximates the
perineum. Finally, the dorsal cloacal plate reverts
into a two-layered membrane which ruptures soon
after, to expose the rectal and urogenital openings.
Hadidi et al. argued that the developmental
process in humans differs from that in different
animal species [15]. This was further confirmed
by Liu et al. [19].
Fig. 3.13 A patient with perineal hypospadias. The ure-

thral orifice is trumpet shaped and extends from the 3.3.2 The Penile (Spongy) Urethra
perineum to the base of the glans. The scrotum is bifid but
there is no penoscrotal transposition. (©Ahmed T. Hadidi
2022. All Rights Reserved) Two theories collided regarding the formation of
the human spongy penile urethra. The classic
a b
Fig. 3.14 Normal penis of a fetus of 12 weeks. A frontal plate (5); scrotum (6). The relationship is demonstrated in
section (a) shows the wide orifice (1) bordered by the ure- more detail in (b) revealing the irregular stroma of the
thral labia (2) extending from a broad raphe (3). Note the labium (2) meeting the parallel oriented stroma of the pre-
close relationship between the urethral labia and a very puce (4). (From van der Putte, reprinted from J Plast
early prepuce (4). In hypospadias this relationship persists Reconstr Aesthetic Surg, Vol. 60, Issue 1, © 2006 with
in the form of a branching raphe and “dog ears.” Glans permission from Elsevier [24])
theory proposed that the spongy urethra was Fusion of epithelial structures may occur
formed by fusion of the urethral folds like closing through the following three phases:
a zip in the midline [21]. This theory was greatly 1. Hyperplasia: There is condensation of mesen-
enhanced by the investigations of Glenister [10]. chyme and proliferation of the overlying epi-
Fleishmann (1907) [32] and others challenged thelial edges which expand and move towards
the fusion theory as they could not find evidence each other.
for fusion in their studies on human embryos. 2. Adhesion: The two epithelial structures show
Their ideas were recently supported by van der signs of adhesion of their epithelial linings
Putte in man [24] and Kluth [4, 36] in rats as forming a double layer of epithelium (seam).
these investigators also failed to find any sign of 3. Apoptosis: There is disruption of the two epi-
midline fusion. thelial layers including their basement mem-
Fusion is defined as a process during which branes and the mesenchyme becomes in
two separate elements come together and form a continuity. Examples of tubular structures
single new structure. This occurs frequently in formed through this process include the neu-
mesenchyme-derived structures, e.g., muscle ral tube and the primary nasal cavity [41].
and fat. However, fusion as such does not nor- The mesoderm of the genital tubercle is
mally occur when the opposing elements are unique in that it is sandwiched between endo-
covered by epithelium. Apposed epithelia may derm and ectoderm. Some authors compared the
stick together but do not fuse under normal emergence of the genital tubercle to early out-
circumstances. growth of the limb buds. Others proposed that
Apoptosis (programmed cell death) is a phe- early development of the external genitalia is
nomenon that has been described as early as the more similar to the hind gut in which signaling
nineteenth century as reviewed by Clarke and occurs between endoderm and the adjacent
Clarke [37] and is considered to be important for mesoderm.
normal embryonic development [38]. Many Baskin conducted a study on mice and hypoth-
developmental processes have been postulated to esized that the normal urethra in mice is similar
be partly regulated by the time- and area- to human and forms by fusion of an epithelial
dependent death of cells [1, 18, 39, 40]. seam [39]. The same team concluded later in
78 A. T. Hadidi
a b
c d e f
Fig. 3.15 Embryo 11, 13 weeks, horizon >23. (a, b) The penis forming the proximal urethra. Dorsal to the grooved
glans penis has appeared with early coronal sulcus. There urethral plate the mesenchyme has started to differentiate
is a blind central opening due to vacuolation of the central into (still undivided) corpus cavernosum. Also, through
glans plate but the glans plate is not turned into a groove the sections the mesenchyme around the grooved urethral
yet 10×. (c) Cut section through the distal penis showing a plate has started to proliferate and differentiate into cor-
wide-grooved urethral plate ×20. (d, e) Sections through pus spongiosum which is more pronounced proximally
mid penis showing grooved urethral plate ×20. (f) The ×25. (©Ahmed T. Hadidi 2022. All Rights Reserved)
edges of the urethral plate have fused at the very proximal
2018 that the development of the human urethra Glenister suggested that the urethral plate
differs than that in mice. Hadidi showed clearly develops as a lamellar outgrowth from the
the three steps (proliferation, adherence, and epithelium lining the anterior part of the endoder-
apoptosis) necessary for fusion between epithe- mal cloaca immediately adjacent to the cloacal
lial surfaces [42]. membrane. In the histological sections included
Keibel and Mall stated that the urethral open- in his study, one can see solid urethral plate in 13
ing moves gradually along the anal surface of the and 18 mm embryos and early signs of vacuola-
genital tubercle to the tip of the genital tubercle tion in 20 mm embryo [10]. Williams showed
[28]. The important role of migration of meso- solid urethral plate in histological sections of 10
derm in the formation of urethra was proposed by and 14 mm embryos [45]. Barnstein and
van der Putte [24] and Altemus and Hutchins [43] Mossman in their study on red squirrel suggested
on human embryos. Seifert et al.’s study on mice that the entire penile urethra is formed by canali-
suggested that the urorectal septum forms the zation of the solid urethral plate, which becomes
perineum and its mesoderm migrates distally into completely separated from the surface of the gen-
the penis causing masculinization of the urethral ital tubercle before it develops a lumen [6].
plate [44]. Hadidi et al. showed gradual vacuolation and
a b
c d
Fig. 3.16 Fetus 13, 14 weeks, horizon >23 histological level of distal glans, (c) gradual degeneration and disap-
sections with hematoxylin and eosin stains (H&E) through pearance of the floor of the glanular canal at the level of
the glans penis showing the first three stages of glanular the mid glans, and (d) complete disappearance of the floor
urethra development as one proceeds from distal to proxi- of the glanular canal at the level of the proximal glans
mal; (a) a solid epithelial plate at the tip of the glans, (b) a turning the glanular canal into a deep glanular groove
vacuolated epithelial plate forming a glanular canal at the ×20. (©Ahmed T. Hadidi 2022. All Rights Reserved)
80 A. T. Hadidi
a b c
Fig. 3.17 Fetus 13, 14 weeks, horizon >23. distal mid glans showing narrow central glanular canal
Immunohistochemical staining with vimentin. (a) ×25. (c) Section through proximal mid glans showing a
Histological section through the tip of the glans stained wide central glanular canal and a large cell (macrophage
with cytokeratin (specific for epithelium) shows a solid or scavenger cell in the center) ×45. (©Ahmed T. Hadidi
urethral plate at the distal glans ×25. (b) Section through 2022. All Rights Reserved)
Fig. 3.18 Section at

proximal glans in a male a
embryo at 19 weeks of
gestation. (a) Staining
with cytokeratin K14.
(b) Staining with
cytokeratin K8. u.p.
urethral plate, g.u.
glanular urethra, fr.
frenulum, gl. glans;
incomplete arrows point
to preputial lamella in
the original figure.
(Reprinted from
Differentiation, Vol 64,
Issue 2, by Kurzrock b
et al.: “Ontogeny of the
male urethra: Theory of
endodermal
differentiation”,
pp 115–122, ©1999,
with permission from
Elsevier [27])
a b
Fig. 3.19 Transverse sections of normal fetal glans penis There is continuity between the mesenchyme of the cor-
at 25 weeks of gestation (a) and a full-term fetus. (b) The pus spongiosum and the prepuce through the frenulum,
roof and lateral walls are vertically oriented and lined by reduced from ×25. (From Baskin et al. “Anatomical stud-
stratified squamous epithelium. The floor is horizontally ies of hypospadias”, J Urol, Vol 160, Issue 3 Part 2,
oriented and is lined with pseudostratified columnar epi- pp 1108–1115, ©1998, with permission from Wolters
thelium. The corpus spongiosum is fully developed and Kluwer [25])
well demarcated from the surrounding glanular tissue.
grooving of the urethral plate [15]. Abnormal Stage 3: Urethral tube: Differential growth and
vacuolation of the solid urethral plate may migration of the ventral penile mesenchyme
explain the accessory urethral tracts that are bring the edges of the urethral groove together
occasionally associated with hypospadias. This causing proliferation, adherence, and apopto-
was confirmed by Liu et al. in 2018 [16, 19]. sis of the urethral groove edges. This results in
Some textbooks report that the formation of fusion of the edges of the urethral groove to
the spongy urethra is completed by the 12th week form the floor of the penile urethra (Figs. 3.24
in contrast to Hadidi et al.’s study and other stud- and 3.25).
ies in literature. The difference is probably related
to the method of measurement of gestational age.
The Hadidi et al. study showed that the penile 3.3.3 The Glanular Urethra
(spongy) urethra develops through three stages
[15]: The development of glanular urethra has been a
Stage 1: Solid urethral plate: The urethral plate controversial subject in embryology for years.
begins as a solid outgrowth from the anterior The lining of the glanular urethra has been cited
wall of the urogenital sinus. as being ectodermal in origin, endodermal in ori-
Stage 2: Deep urethral groove: There is gradual gin, or mixed in origin.
vacuolation and degeneration of the caudal Wood-Jones in 1904 [46] and Hart in 1908
cells resulting in the formation of the urethral [47] believed that the glanular urethra develops
groove. The vacuolated cells degenerate ven- due to canalization of an ectodermal plug of cells
trally without the formation of a canal. that grow from the tip of the glans to join up with
82 A. T. Hadidi
the endodermal urethra. Keibel et al., on the other

hand, stated in 1912 that the glanular urethra is
due to canalization of a solid “urethral plate” that
extends up to the tip of the genital tubercle [28].
Hunter from Belfast (1935) is the pioneer who
accurately described the actual development of
the glanular urethra, frenulum, and prepuce [48].
He conducted an excellent study on human
embryos at 40 mm CR (11 weeks’ GA), 70 mm
CR (13 weeks’ GA), 100 mm CR (15 weeks’
GA), and 170 mm CR (about 20–22 weeks’ GA)
with sagittal (longitudinal)—along the long axis
of the penis—and coronal sections (right angle to
the long axis of the penis). His study documented
clearly the deep glanular groove in 100 mm Cr
human embryo (stage III according to Hadidi)
and the complete glanular canal (stage IV accord-
ing to Hadidi) in 170 mm CR (20–22 weeks’ GA)
human embryo where the floor of the glanular
canal is in continuity with the prepuce (Fig. 3.28).
According to his histological sections, the
penis at 40 mm CR represents a “naked” rounded
extremity with the terminal portion separated
from the shaft by a shallow groove (coronal sul-
cus). At 70 mm CR, there is more proliferation of
the surface epithelial tissue than the mesenchy-
Fig. 3.20 Frontal section through the phallus of a 76 mm mal spongiosal tissue that is disposed in an
human embryo. The penile urethra (U) is partly solid dis-
tally and partly grooved urethra (more proximally). The oblique direction radiating on either side of the
prepuce (P) covers the proximal glans only. There is a urethral opening like a “hood” on the dorsum but
blind central glanular canal that is not connected with the missing ventrally. The epithelial tissue on the
rest of the urethra. The epithelial plug (arrow) lies on top dorsum is thrown into folds suggesting that it
of the blind central glanular canal. (Reprinted from
“Teratology”, Vol 61, Issue 3, by Van der Werff et al.: proliferates much more than the mesenchymal
“Normal development of the male anterior urethra”, spongiosal tissue. At 100 mm CR serial coronal
pp 172–183, ©2000, with permission from Wiley and sections showed a well-marked groove on the
Sons [1])
Fig. 3.21 A child with coronal hypospadias. Note the grooving of the solid glans plate. (©Ahmed T. Hadidi
bridge between the proximal penile urethra and the glans. 2022. All Rights Reserved)
There is an incomplete cleft glans due to incomplete
a b c d
Fig. 3.22 (a–d) Transverse section of the glans at differ- showing stratified squamous epithelium at the roof of the
ent stages of development. (a) Transverse section of the glanular urethra and (d) pseudostratified epithelium at the
glans at 8 weeks showing solid glans plate. (b) Transverse floor of the glanular urethra. (©Ahmed T. Hadidi 2022.
section of the glans showing incomplete grooving of the All Rights Reserved)
glans. (c) Transverse section of the glans in a newborn
a b
Fig. 3.23 (a, b) The glanular urethra. (a) Transverse sec- sists of the penile septum (large arrowhead), the frenulum
tions of a 14-week-old (82 mm) fetus. (b) Transverse sec- narrowing by the expanding medial parts of the glanulo-
tion in a 19.5-week-old (155 mm) fetus. The glanular preputial lamella (large arrows), and the prepuce (small
urethra is lined by stratified squamous epithelium of the asterisks) with the raphe (small arrow). (Courtesy of van
glans plate (large asterisk) dorsally and pseudostratified der Putte)
columnar epithelium (small arrowhead). The floor con-
undersurface of the glans. Sections at 170 mm could not find any signs of cell invagination from
CR (20–22 weeks’ GA) showed the line of fusion the surface as suggested by several other
of the ventral margins of the prepuce and a conti- researchers.
nuity between these skin folds and the sub-glans He observed that the glanular urethra has a
groove; this continuity forms the frenulum. He vertical orientation of the longitudinal axis as
84 A. T. Hadidi
a b
c d
Fig. 3.24 Development of the penile urethra. (a) The urogenital orifice gradually migrates forward to reach the
cloacal membrane continues to disintegrate to form the coronal sulcus. (d) The glanular urethra has a different
cloacal groove. (b) Gradually, both urogenital and anal origin from the glans plate. (©Ahmed T. Hadidi 2022. All
orifices reach the surface. (c) The posterior edge of the Rights Reserved)
opposed to the transverse orientation of the longi- He demonstrated that the prepuce starts to
tudinal axis in the penile urethra. The fossa form as a thin layer of desquamating epithelial
navicularis is the region in the midline where cells are disposed in an oblique manner laterally
these two different forms of urethra unite. and dorsally as a kind of a “hood on the dorsum”
Hadidi et al. [15], Baskin [39], and many oth- and fades away ventrally. The invaginating cells
ers have clearly demonstrated the four stages of forming the prepuce
development. form a kind of plug closing off the fossa navicu-
Hunter in 1935 was in fact the first to report laris and the external opening of the urethra …. By
the role of prepuce in the formation of the floor of further growth of the dorsal prepuce, its margins
the glanular urethra (Fig. 3.26) [48]. He clearly flow over the sides of the glans and are fused
together by 2 sub-glans folds. The line of fusion
reported forms the frenulum.
the continuity of the glans tissue with the mesoder-
mal tissue of the prepuce and the V-shaped line of He also showed microphotographs of the cell
fusion between the two sides of the prepuce epithe- nests that result in the cleft between the prepuce
lium on its ventral aspect. and the glans.
Fig. 3.25 Stages of spongy (penile) urethra develop- cells resulting in the formation of the urethral groove.
ment. The development of spongy urethra is influenced by Stage 3: Urethral tube: Differential growth and migra-
the distal migration of the ventral penile mesenchyme and tion of the ventral penile mesenchyme bring the edges of
passes through three stages: Stage 1: Solid urethral the urethral groove together causing proliferation, adher-
plate: The urethral plate begins as a solid outgrowth from ence, and apoptosis of the urethral groove edges. This
the anterior wall of the urogenital sinus. Stage 2: Deep results in fusion of the edges of the urethral groove to
urethral groove: There is gradual grooving of the urethral form the floor of the penile urethra. (©Ahmed T. Hadidi
plate due to vacuolation and degeneration of the caudal 2022. All Rights Reserved)
86 A. T. Hadidi
a b
Fig. 3.26 Development of the glans urethra and prepuce. enclosing within it a plug of desquamating epithelium.
(a) Cut section of the glans region of penis from human Note the continuity of the glans tissue with the mesoder-
fetus 100 mm CR length (15 weeks). Note the two sub- mal tissue of the prepuce at the frenulum, and the V-shaped
glans folds closing off a portion of the desquamating epi- line of fusion between the two sides of the prepuce epithe-
thelium within the developing glans portion of the urethra. lium on its ventral aspect. (Reprinted from Journal of
Notice that there is a deep glanular groove, contrary to Anatomy, Vol 70 (Pt1), by Hunter: “Notes on the
what Baskin et al. suggested. (b) Cut section of glans Development of the Prepuce”, pp 68–75, ©1935, with
region of penis from human fetus 170 mm CR length (20– permission from Wiley and Sons [48])
22 weeks). The glans urethra is here seen closed off
Williams published in 1952 an elegant study Glenister in 1954, following his study of 37
of 41 human embryos serially sectioned [45]. He human embryos, suggested that the glanular ure-
also referred to the close relation between the thra has a dual origin with the proximal part
development of the prepuce and the urethra formed by fusion of the urethral folds enclosing
(though not clearly). He stated that: surface epithelium and the distal portion by a
the first sign of the prepuce (at about 60 mm stage) lamellar ingrowth of the surface epithelium [10].
is a solid lamella lying perpendicular to the surface It is interesting that Glenister concept has been
of the phallus at the level of the coronary sulcus. frequently interpreted as that the surface epithe-
The lamella is clearly arising from the tissues of lium forming the distal glanular urethra origi-
the shaft. The lamella is always incomplete ven-
trally, and when the lateral wings fuse, they leave nates from the tip of the glans (i.e., from distal to
the solid frenulum beneath them. Coincident with proximal ingrowth or invagination). However,
the fusion of the urethral folds, the wings of the careful examination of the histological slides
prepuce fuse in the ventral midline and they seem enclosed in his publication suggests that if there
to be dependant one upon the other.
is any ingrowth it is from lateral to medial. included in their study clearly documented the
Furthermore, the urethral plate shown in the his- four stages of glanular urethra development: a
tological sections enclosed in his study reached solid glanular urethral plate in 9-week embryo, a
the tip of the glans. glanular canal in 12-week embryo, a deep glanu-
Altemus and Hutchins in their study on 38 lar groove in 14-week embryo, and a completed
human fetuses showed a solid glans plate in the glanular floor in 19-week embryo (Fig. 3.18).
glans region at 40 mm CRL stage with no signs Van der Werff et al. suggested that the urethral
of development of the prepuce. There was grad- plate is present even before rupture of the cloacal
ual canalization of the solid glanular urethral membrane [1]. They proposed that the urethral
plate at 72 mm CRL stage while the penile plate in the glans region is of ectodermal origin
urethral opening was still on the ventral surface which becomes later on in continuity with the
of coronal sulcus [43]. endodermal urethral plate of the rest of the penis
Kurzrock et al. in their study on human at the level of fossa navicularis (Fig. 3.20).
embryos and mice suggested that mesenchymal Complete development of the glanular urethra
signaling may induce squamous differentiation is influenced by distal migration of ventral mes-
of urethral endothelium [27]. Careful re- enchyme and normal development of the prepuce
examination of the human histological sections and passes through four stages (Fig. 3.27):
Fig. 3.27 Stages of development of the glanular urethra. (©Ahmed T. Hadidi 2022. All Rights Reserved)
88 A. T. Hadidi
Stage 1: Solid epithelial plate: There is a solid human embryos (Figs. 3.15, 3.16, 3.17, 3.19,
epithelial plate that reaches the tip of the glans 3.20, and 3.22).
penis. Hadidi has clearly documented and confirmed
Stage 2: Blind central glanular canal: the findings of Hunter that the glanular urethra
Degeneration of the central cells of the epithe- passes through the stages of solid urethral plate,
lial plate results in the formation of blind cen- central canal, and deep glanular groove, as is
tral glanular canal. seen in Figs. 3.15, 3.16, 3.21, and 3.28.
Stage 3: Deep glanular groove: Degeneration of Liu et al.’s suggestion does not explain the
the floor of the central glanular canal results in development of the frenulum and the continuity
the formation of a deep glanular groove. of glanular urethra and prepuce at the region of
Stage 4: Complete glanular urethra: The floor of the frenulum [16, 19]. If Liu et al.’s suggestions
the glanular urethra develops from the frenu- were correct, we would only see patients either
lum and the glanulo-preputial lamella that with flat glans (solid glanular plate) or with a
forms the prepuce. glanular urethral canal not connected to the
The Hadidi et al. study confirmed the findings penile urethra. This is not the case and most
of Hunter [48] and showed that the development patients with hypospadias have some degree of
of the glanular urethra differs from that of the glanular grooving.
penile urethra [15]. There was no evidence of
ectodermal ingrowth. The glanular urethra seems
to develop through four stages (Fig. 3.27): 3.3.4 The Median Perineal Raphe
• A solid epithelial plate at the tip of the glans The median perineal raphe is generally consid-
penis during the 5–6 weeks of gestation. ered to mark the line of fusion of the urethral
• A well-defined blind central canal due to vac- folds [10, 50, 51], or a combination of urethral
uolation at the center of the solid plate. The folds and scrotal swellings [52, 53] or between
vacuolated cells seem to shed distally through the urethral folds for the penile raphe and
the tip of the glans forming the epithelial tag. between the scrotal swellings for the scrotal
• A deep glanular groove due to degeneration of raphe [23, 54, 55].
the floor of the glanular canal. However, Fleishmann [32] and Politzer [33]
• The floor of the glanular urethra is formed rejected the idea of fusion as a developmental
through distal migration of the glanulo- process with Politzer suggesting that the raphe is
preputial lamella which forms the prepuce and formed by embryonic tissue filling the temporary
fuses with the edges of the glanular groove to cloacal groove that had remained from the
form the floor of the glanular urethra. This regressing middle part of the cloaca between the
mesenchymal migration is associated with the urogenital and anal compartments.
development of the frenulum and frenular Clinically, the presence of raphe just anterior
artery. to the anus, an area where no assumed fusion
occurred, and the presence of ano-cutaneous fis-
Liu et al. in 2018 [16, 19] and Baskin et al. tula in some patients with imperforate anus, con-
[26, 49] suggested (rather inaccurately) that the tradicts the formation of raphe as a result of
glanular urethra passes through the two stages of fusion.
a solid plate that canalizes into “a stand-alone Histological examination of raphe in fetuses
urethral canal” without further development into and embryos showed a parallel longitudinal
a groove that will become a tube. This is not sup- arrangement of fibers and blood vessels and
ported by most studies in literature including his- structural unity of the perineal raphe and scrotal
tological sections by the same authors. The third septum. These histological findings contradict
stage of deep glanular groove is documented in fusion as a cause of formation of raphe. In addi-
Hunter and Baskin histological sections of the tion, the stratified squamous epithelium of the
a b
Fig. 3.28 The second and third stages of glanular ure- deep glanular groove. The epithelial tag has disappeared.
thral development. (a) The glanular urethra is in the form (From: An Atlas of Human Prenatal Developmental
of a central canal with epithelial tag at the tip. (b) The Mechanics, by Jan E. Jirasek, © 2004. Reproduced by
third stage of glanular urethral development in the form of permission of Taylor & Francis Group [21])
raphe is characteristically devoid of skin append- the ventral penile stroma anterior to the anus
ages in contrast to the hairy skin, rich in seba- which will result in the following:
ceous glands present on either side of midline
(Fig. 3.23) [13]. • Forward migration of the posterior urethral
edge to reach the base of the glans.
• The differentiation of the ventral penile stroma
3.4 The Migration Hypothesis into different fascial layers including the for-
mation of the penile septum and median raphe
The cloaca divides into urogenital and anal and the ventral prepuce. The median raphe
compartments. The cloacal membrane gradu- and septum begin at the perineum and prog-
ally disintegrates to form the cloacal groove ress forward along with the posterior urethral
with the result that the urethral opening edge. At the level of the glans, further devel-
becomes visible on the surface at the perineum opment and proliferation of the prepuce push
in front of the anus. The early genital tubercle the posterior edge forward over the already
is directed caudally and makes a 10° angle grooved glans to form the floor of the glanular
with the longitudinal body axis (Figs. 3.29 and urethra. They form the median ventral part of
3.30). the prepuce as well as the frenulum.
Further development of the penis and urethra • The differential growth on the ventral part
is highly dependent on the differential growth of more than the dorsal part of the penis changes
90 A. T. Hadidi
Fig. 3.29 Stages of urethral development. Early in devel- fascia causes forward migration of the posterior urethral
opment, the urethral orifice extends from the perineum to edge to reach the base of the glans. (©Ahmed T. Hadidi
the base of the glans. Differential growth of the ventral 2022. All Rights Reserved)
Fig. 3.30 (a) The migration hypothesis showing the cru- in the development of the floor of the glanular urethra.
cial role of ventral penile mesenchyme in the development Superficial stroma forms dartos fascia which continues to
of the penis, urethra, and penile fascia. Differential growth grow into the prepuce. For illustration purposes, only the
and distal migration of the ventral fascia bring the edges ventral dartos fascia is shown in the figure. (b) Sagittal
of the urethral groove together which fuse to form the section in 11 weeks’ gestation embryo. (©Ahmed
penile urethra. The corpus spongiosum grows around the T. Hadidi 2022. All Rights Reserved)
developing urethra. The prepuce plays an important role
the angulation of the penis to form a 90° angle long axis of the penis from 10° with the longitu-
instead of the 10° angle that was present early dinal axis of the body to 90°. Inadequate develop-
in the development. The scrotal folds together ment of the fascia and corpus spongiosum may
with the migrating testes move caudally to fill result in hypospadias and its associated
in the resultant gap while maintaining a anomalies.
constant relation to the symphysis pubis by a The glanular urethra has a different mecha-
process called merge. nism of development than that of the proximal
penile urethra. The roof of the glanular urethra
develops by vacuolation, canalization, and then
grooving of the solid ectodermal glans plate. The
3.5 Conclusion floor of the glanular urethra is formed by the for-
ward development and migration of the ventral
The posterior edge of the urethral orifice moves prepuce.
forward from the perineum, near the anus poste-
riorly, along the shaft of the penis to reach the tip
of the glans. Controversy remained whether this 3.6 Derivatives of the Genital
shift is due to the fusion of urethral folds from Tubercle
proximal to distal like a zip, or due to the rapid
proliferation and growth of mesenchyme on the As the infraumbilical mesenchyme migrates
ventral aspect of the developing penis. In addi- towards the cloacal membrane, it fuses anteriorly
tion to opposing histological evidence in human and proliferates to form the cloacal eminence
embryos, the fusion theory does not explain the with its prominent genital tubercle. Differences
presence of ano-cutaneous tracts in patients with in density and structure appear in this mesen-
imperforate anus, the presence of complete pre- chyme to form a dense fascial stroma and a fibro-
puce in some patients with hypospadias, and the vascular zone. The fibrovascular zone gives rise
wide spectrum of malformations associated with to a series of erectile structures: glans, corpora
hypospadias such as rotation and chordee. cavernosa, and bulbi of the corpus spongiosum
The migration hypothesis focuses on the (Fig. 3.31).
important role of the differential growth and pro-
liferation of the ventral penile stroma in the nor-
mal development of the penis and urethra 3.6.1 The Glans
(Figs. 3.29 and 3.30). This results in the forward
migration of the posterior edge of urethra to reach The glans forms the main component of the prim-
the tip of the penis and changes the angle of the itive genital tubercle. It is a cap-like structure
Fig. 3.31 The

derivatives of the
primitive genital
tubercle. (©Ahmed
T. Hadidi 2022. All
Rights Reserved)
92 A. T. Hadidi
formed of dense small cells. The glans is thick in tissue gradually differentiates into a loose con-
the center and becomes gradually thinner towards nective tissue rich in prominent arteries. These
the periphery. It envelops the distal parts of the prominent arteries are later surrounded by
corpora cavernosa. The undersurface of the glans venous plexuses that form large cavities or
contains the solid glans plate in the midline sinuses.
(Fig. 3.16). The glans is gradually separated from The distal part of the bulb grows along with
the rest of the shaft by the rapidly growing the proliferating spongy urethra between the lam-
glanulo-preputial lamella forming eventually the ina propria and the deep dorsal stroma distal to
prepuce. The glans itself does not form a sheath. the bulbospongiosus muscle. The longitudinal
It is linked to the corpus spongiosum and corpora venous plexuses of the lamina propria become
cavernosa by the sheaths of these three corpora gradually integrated into the distal corpus spon-
and by numerous vascular anastomosis. giosum and may even replace the bulbar compo-
nent of the corpus spongiosum near the urethral
orifice distally (Fig. 3.33).
3.6.2 The Corpora Cavernosa During development, the corpus spongiosum
receives an extravascular supply from plexuses
The two corpora cavernosa develop from the between the urethra and corpora cavernosa. Part
fibrovascular zone proximal to the glans. of these plexuses persists in the male as branches
Proximally, they form the deviating crura of the of the central arteries of the corpora cavernosa.
penis. Distally, they continue to grow, separated These branches perforate the tunica albuginea
only by a sheath. They are formed of dense small- and supply most of the distal part of the corpus
cells (like the glans) that differentiate later into a spongiosum.
central fibrovascular core and a peripheral tunica
albuginea (Fig. 3.32).
The corpora cavernosa gradually become 3.7 Development of the Fascial
large trunks with a thick double-layered tunica Stroma
albuginea on the outside and dense vascular net-
work centrally. This vascular network is predom- As the infraumbilical mesenchyme migrates
inantly venous which forms large communicating towards the cloacal membrane, it fuses anteriorly
channels and trabeculae. The arterial element of and proliferates to form the cloacal eminence
this network is supplied by the dorsal arteries of with its prominent genital tubercle. Differences
the penis. in density and structure appear in this mesen-
chyme to form a dense fascial stroma and a fibro-
vascular zone. The fibrovascular zone gives rise
3.6.3 The Corpus Spongiosum to the glans, corpora cavernosa, and bulbi of the
corpus spongiosum.
The corpus spongiosum develops from the deep- The fascial stroma develops into the fascia of
est part of the fibrovascular zone in the form of the penis and scrotum. The fascial stroma around
two oval fibrovascular structures (bulbi spongi- the urogenital sinus posteriorly differentiates into
osi) on both sides of the primitive spongy urethra deep and superficial components (Fig. 3.34).
and underneath the crura of the corpora caver- The deep dorsal urogenital stroma forms the
nosa. They are bordered by the lamina propria smooth muscular perineal body and the deep
medially and the bulbospongiosus muscle later- perineal septum. The deep perineal septum
ally (Fig. 3.32). divides the bulb of the corpus spongiosum and
The two bulbi approach each other in the separates the two halves of the scrotum. The deep
midline posterior to the urethra being separated septum continues forward to form the fascial
only by a thin deep perineal septum to form the sheath of the distal half of the corpus spongiosum
bulb of the penis (Fig. 3.32). The dense bulbar and the deep (Buck’s) fascia of the penis which
Fig. 3.32 Stages of development of the corpus spongiosum. The deep stroma forms the tunica albuginea and
sum and penile fascia. Two oval corpus spongiosum bulbi Buck’s (deep) fascia of the penis which covers the corpora
develop from the fibrovascular zone. They unite together cavernosa and corpus spongiosum. (©Ahmed T. Hadidi
to form the bulb of corpus spongiosum and grow forward 2022. All Rights Reserved)
around the developing urethra to form the corpus spongio-
94 A. T. Hadidi
a b c
Fig. 3.33 (a–c) Corpus spongiosum. Sagittal sections of lamina propria of the urethra. The two parts show distinct
a 20-week-old (160 mm) fetus. (a) Proximal, (b) mid- differences in the nature and pattern of blood vessels and
penile, (c) distal penile. The corpus spongiosum develops in the structure of the stroma between the proximal (a),
as a combination of a proximal part derived from the halfway demonstrating both components (b), and distal
paired bulbi spongiosi and a distal part derived from the (c) parts. (Courtesy of van der Putte)
surrounds the urethra, corpus spongiosum, and deep to superficial) lamina propria of the urethra,
corpora cavernosa (Fig. 3.35). deep septum, interwoven connective tissue
From the superficial dorsal urogenital stroma between the two halves of the bulbospongiosus
develop the superficial perineal septum, median muscle, superficial septum, and perineal raphe
perineal raphe, and dartos fascia. The superficial (Fig. 3.37).
perineal septum is connected to the superficial Early in development, the cloacal groove may
(dartos) fascia that fans out into the scrotal halves be seen, lined by pseudostratified columnar epi-
as the scrotal fascia. The scrotal septum and dar- thelium. Thereafter the raphe is covered by strati-
tos fascia continue into the shaft of the penis and fied squamous epithelium that characteristically
into the prepuce as the penile septum and penile covers the urogenital folds. This epithelium does
dartos fascia (Fig. 3.36). not form epidermal appendages later and as a
result the median perineal raphe is permanently
devoid of hair follicles and glands. The parallel
3.8 Development of the Median longitudinal fibers of the raphe gradually bend
Perineal Raphe and Septum inwards and continue into the superficial part of
the perineal septum.
The median perineal raphe and septum are devel-
oped from the superficial and deep dorsal uro-
genital stroma as mentioned above. 3.9 Development of the Scrotum
The median perineal raphe originates from a
dense fibrovascular tissue related to the vanishing During the cloacal period, the infraumbilical
cloacal groove just posterior to the urogenital mesenchymal cells migrate and proliferate
orifice. It is characterized by its parallel and lon- around the cloacal membrane to form the genital
gitudinal orientation. The perineal septum is a tubercle anteriorly. They form the urogenital
thin triangular vertical stroma between the peri- folds (or labia) on either side of the cloacal mem-
neal raphe and the urethra. It consists of (from brane. Later, another pair of elevations, the labio-
Fig. 3.34 Development

of the penile fascia from
the ventral stroma. From
the deep stroma
develops the tunica
albuginea and Buck’s
(deep) fascia of the
penis. From the
superficial stroma
develops the median
raphe and penile septum
and dartos fascia.
(©Ahmed T. Hadidi
2022. All Rights
Reserved)
96 A. T. Hadidi
Fig. 3.35 Differential growth theory showing the crucial spongiosum grows around the developing urethra. The
role of ventral stroma in the development of the penis, superficial stroma forms dartos fascia which continues to
urethra, and penile fascia. Differential growth of the ven- grow into the prepuce. For illustration purposes, only the
tral fascia causes forward migration of the posterior ure- ventral dartos fascia is shown in the figure. (©Ahmed
thral edge to reach the tip of the glans. The corpus T. Hadidi 2022. All Rights Reserved)
scrotal swellings, becomes visible on each side of to fill the space available as the penis moves for-
(lateral to) the urogenital folds (Fig. 3.38). ward. The median scrotal septum is not formed
Early in development, the genital tubercle by fusion of the scrotal swellings but by the
(the future penis) projects caudally making median stromal structures that originate in front
about 10° angle with the longitudinal axis of the of the anus [13]. This theory is supported by the
body and the labioscrotal folds lie lateral to the presence of ano-cutaneous fistula in the median
genital tubercle and urogenital folds (or labia) raphe between the scrotal hemispheres in some
(Figs. 3.40 and 3.41). The urethral orifice is an patients with imperforate anus (Fig. 3.39). This
oval, long slit-like structure with the posterior can only be explained by forward migration of
boundary close to anal orifice at the perineum the midline structures in front of the anus.
and the anterior boundary at the base of the In other words, with later development, three
glans. The urethral orifice is bounded laterally mechanisms occur simultaneously (Figs. 3.40
by the urogenital folds. Deep stromal tissue and and 3.41): (1) The corpus spongiosum, perineal
dartos fascia occupy most of the scrotal swell- septum, and raphe and other ventral penile struc-
ings (Fig. 3.38). tures proliferate much more than the dorsal
The scrotum is believed to develop by fusion structures of the penis, i.e., the corpora caver-
of the scrotal swellings at the base of the penis nosa. This increases the space between the anus
after they have moved downward and dorsal and the posterior boundary of the urethral orifice
ward (posteriorly) from their position on either which migrates gradually along the shaft of the
side of the genital tubercle. The scrotal septum is penis to reach the base of the glans. (2) The penis
thought to be formed where they meet [1, 50, 53, rotates cranially to project anteriorly so that its
54, 56, 57]. axis makes 90° angle with the longitudinal axis
However, examination of the relation of the of the body. (3) The two scrotal swellings move
scrotal swellings to a fixed point such as the sym- closer to the midline and occupy part of this
physis pubis, throughout development, demon- space.
strates that they retain their position to the It is worth noticing that all through the devel-
symphysis pubis while the shaft of the penis opmental process, the scrotal swellings maintain
shifts ventrally (anteriorly) and at the same time a fixed distance from the symphysis pubis. This
rotates from projecting caudally to project anteri- strongly suggests that it is the penis that rotates
orly. This process is explained by the rapid prolif- forward (anteriorly) and moves forward rather
eration of the stromal structures posterior to the than the scrotal swellings moving backwards
developing urethra. (posteriorly).
The single scrotum is thus formed as the The dartos fascia differentiates into bundles of
median stromal structure proliferates bringing smooth muscle cells. It is organized in a fan-
the two scrotal swellings together in the midline shaped manner from posterior to anterior. It also
a b
c d
Fig. 3.36 Penile fascia (a) and (b) transverse sections of (dartos) fascia (medium-sized arrow), and superficial con-
11-week-old (51 mm) fetus. (c) Transverse section of a nective tissue layer (small arrow) in frontal sections of an
penis of 14-week-old (82 mm) fetus. (d) Longitudinal 11-week-old (51 mm) fetus (a and b). These fascias
section of a penis of 14-week-old (82 mm) fetus. The spread from posterior to the urethral orifice and maintain
penile fascia can be recognized by pattern and topography their structure while forming the shaft of the penis by
from the beginning of penis development as illustrated by expanding as shown in transverse (c) and longitudinal sec-
the longitudinal orientation of the fibers of the raphe tions (d) of a penis of 14-week-old (82 mm) fetus.
(asterisk) and the parallel orientation of the primordial (Courtesy of van der Putte)
deep (Buck’s) fascia of the penis (large arrow), superficial
98 A. T. Hadidi
a b
Fig. 3.37 (a) Longitudinal section in a 15-week-old fetus Putte, reprinted from J Plast Reconstr Aesthetic Surg, Vol.
with proximal hypospadias. Notice in (b) the longitudinal 60, Issue 1, ©2006 with permission from Elsevier [24])
fibers and vessels of the median raphe. (From van der
a b
Fig. 3.38 (a) The early indifferent external genitalia are (G = GL), CCB the corpora cavernosa bodies, UF the ure-
formed by the phallus which is composed of the glans thral folds. The urethral groove lies between the urethral
(GL), the corpora cavernosa bodies, and the urogenital folds. (From: An Atlas of Human Prenatal Developmental
groove (arrow) with two urogenital folds. Laterally are the Mechanics, by Jan E. Jirasek, © 2004. Reproduced by
labioscrotal folds (LSF). (b) Electron microscope photo; permission of Taylor & Francis Group [21])
continues forward along the shaft of the penis puce does not grow forward as a free unattached
and into the prepuce (Figs. 3.33 and 3.34). sheath, but that the fold rolls forward to become
inverted “so that what was superficial is now
deep, facing the glans surface.” He then states
3.10 Development of the Prepuce that the glans and the fold become blended and
and Frenulum continuous as a tissue, the future preputial space
being filled with epidermal cells, the central of
Retterer and Neuville described a “glanulo- which correspond to the superficial epidermal
preputial epithelial invagination of cells” which layers. These central cells, Berry Hart states,
breaks down to form a slit-like space between the “desquamate,” and by this desquamation produce
preputial skin and the glans penis [58]. Hart the preputial space.
described the process differently [47]. According Hunter showed clearly in an excellent study
to him, the prepuce grows forward to cover a pre- on embryos between 11 weeks’ and 22 weeks’
viously naked glans penis. He states that the pre- GA the stages of prepuce and frenulum develop-
Fig. 3.39 Newborn baby with imperforate anus and ano-

cutaneous fistulous tract. The ano-fistulous tract can only Fig. 3.41 A child with penoscrotal hypospadias. Notice
occur if the median raphe develops in the perineum near that the penis has maintained 10° angle that the primitive
the anus and migrates distally towards the tip of the penis. genital tubercle had early in fetal life. (©Ahmed T. Hadidi
(Courtesy of Prof. Dan Young) 2022. All Rights Reserved)
10º
Fig. 3.40 The primitive genital tubercle makes 10° angle caudally to meet the median raphe in the midline while
with the body axis. Differential growth of the ventral fas- maintaining a fixed relation to the symphysis pubis.
cia changes the angle to 90° and the scrotal folds migrate (©Ahmed T. Hadidi 2022. All Rights Reserved)
100 A. T. Hadidi
ment [48]. Until 11 weeks’ GA, there is equal lying nerves to the glans and corpora cavernosa
development of the ectodermal and mesenchy- (Figs. 3.43, 3.44, and 3.45).
mal tissues and the glans is naked. Then, there is The formation of the median ventral part of
more rapid proliferation of the epithelium than the prepuce is due to rapid forward (distal) prolif-
the mesenchyme, in particular on the dorsum eration of the median ventral raphe and synchro-
forming a “hood,” and the proliferation slows nously the medial ventral part of the growing
towards the edges of the urethral opening. By fur- prepuce more than the dorsal part of the prepuce.
ther growth of the prepuce, its margins are drawn The formation of the frenulum is due to an expan-
together on its ventral surface by two sub-glans sion of the corona of the glans and glanulo-
folds, so that the glans is completely surrounded preputial lamella proximal to the urethral orifice.
by the prepuce. The line of fusion between the As a result, the median ventral raphe and septum
sub-glans folds and the margin of the prepuce form the floor of the glanular urethra and remain
forms the floor of the glanular urethra and the as the frenulum inside the medial ventral part of
frenulum (Fig. 3.42). the prepuce which covers the ventral surface of
The prepuce develops dorsally from two com- the glans (Figs. 3.42, 3.43, 3.44, and 3.45). This
ponents at the junction between the glans and the process ends beyond the tip of the penis.
shaft penis (the corona): (a) a major component Cornification of the epithelium of the lamella
from the shaft (dartos fascia and superficial during the last trimester of gestation forms the
stroma) and (b) a minor component from the preputial sac.
glans stroma. Between the glans stroma of the The migration hypothesis and the role of distal
glans itself and the preputial glans component mesenchymal migration in the development of
grows the solid glanulo-preputial lamella o utward the penis and urethra are supported by the fact
in a concerted growth. That growth is outward that no “skipped” segment of urethral fusion has
because the position of the deepest part of the been reported in literature as compared with
lamella remains the same in relation to the under- fusion of the spinal cord.
a b c d
Fig. 3.42 (a) LS. Penis of human fetus 40 mm CR length. human fetus 170 mm CR length. This section shows the
Note the ridge of ectoderm behind the corona and the prepuce covering the whole of the glans, with the layer of
glans, and the thin layer of desquamating cells on the sur- desquamating cells connecting the two. Note the “plug” of
face. (b) LS. Penis of human fetus 70 mm CR length. Note desquamating cells closing the opening of the urethra.
the dorsal ectodermal tissues thrown into loose folds, and (Reprinted from Journal of Anatomy, Vol 70 (Pt1), by
the distribution of the desquamating cells. (c) LS. Penis of Hunter: “Notes on the Development of the Prepuce”,
human fetus 100 mm CR length. The prepuce is here seen pp 68–75, ©1935, with permission from Wiley and Sons
“overflowing” the glans and enclosing a layer of desqua- [48])
mating cells between it and the glans. (d) LS. Penis of
Fig. 3.43 Development of the prepuce from the glanulo- the prepuce grows as an outgrowth over the glans surface
preputial lamella. Notice the constant distance between the and not as an ingrowth under the surface of the glans.
nerves (yellow) and the glans surface which confirms that (©Ahmed T. Hadidi 2022. All Rights Reserved)
3.11 Derivatives form the uterus and upper vagina. In the male,
of the Mesonephric they are rudimentary and are present as blind
and Paramesonephric Ducts prostatic utricle. The mesonephric (Wolffian)
ducts give rise to the vas deferens and seminal
The two paramesonephric (Mullerian) ducts give vesicle.
rise to the fallopian tubes before they unite to
102 A. T. Hadidi
Fig. 3.44 Development of the lateral part of the prepuce from the preputial lamella. The median part and frenulum
develop from the median raphe and penile septum. (©Ahmed T. Hadidi 2022. All Rights Reserved)
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Surgical Anatomy of the Penis
and Urethra 4
Ahmed T. Hadidi
Abbreviations
AGD Anogenital distance

ASD Anoscrotal distance
4.1 Introduction
There is little information written about the anat-

omy of the penis when compared to the huge
knowledge available about the anatomy of the
female genital system. The information written in
this chapter is based on free readings in several
text books and references listed at the end of the
chapter as suggested readings in addition to per- Fig. 4.1 Structure of the penis: The glans penis is classi-
cally described as the distal end of the corpus spongio-
sonal clinical observations in clinical practice. sum. However, in many cases of hypospadias, the glans
The penis can be divided into three parts: the penis is well developed (normal) while the corpus spon-
root in the perineum, the body, and the glans giosum is hypoplastic and often atretic (© Ahmed
(Fig. 4.1). The root is located within the superfi- T. Hadidi 2022. All Rights Reserved)
cial perineal pouch and provides fixation and sta-
bility. The body is formed by the three spongy
erectile entities: two corpora cavernosa and one 4.2 The Root of the Penis
corpus spongiosum. The glans is described in
most textbooks (though inaccurate) as the distal The root of the penis consists of three erectile
end of the corpus spongiosum [1, 2]. masses in the urogenital triangle, namely, the two
crura and the bulb. The two crura are the poste-
A. T. Hadidi (*) rior regions of the corpora cavernosa and are
Hypospadias Center and Pediatric Surgery firmly attached to the pubic arch. The bulb is the
Department, Sana Klinikum Offenbach, Academic posterior end of the corpus spongiosum and is
firmly attached to the perineal membrane.
University, Offenbach, Frankfurt, Germany
106 A. T. Hadidi
Each penile crus starts behind as a blunt, elon- 4.3 The Body of the Penis
gated, round process, attaching firmly to the
everted edge of the ischiopubic ramus and cov- The body of the penis is composed of two corpora
ered by ischiocavernosus muscle. Anteriorly, it cavernosa and corpus spongiosum containing the
converges toward its fellow. Near the inferior penile urethra. It can be divided into a distal portion
border of the symphysis pubis, the two crura con- that includes the glans, coronal sulcus, and fore-
verge and continue as the corpora cavernosa of skin and a proximal portion that includes the shaft.
the body of the penis. Surgically, the penis is covered by three dense
The bulb of the penis lies between the crura layers: the skin, Buck’s fascia, and the tunica
and is firmly connected to the inferior aspect albuginea. Between the skin and Buck’s fascia
of the perineal membrane, from which it lie the loose superficial fascia (dartos), the super-
receives a fibrous covering. Oval-shaped in ficial dorsal arteries, and veins. Between Buck’s
section, the bulb narrows anteriorly into the fascia and tunica albuginea lie the deep dorsal
corpus spongiosum. Its flattened deep surface arteries, deep dorsal veins, and dorsal nerves. The
is pierced above its center by the urethra, superficial dorsal arteries arise from the external
through which it traverses to reach the corpus pudendal artery and supply the skin of the penis
spongiosum. and prepuce (Fig. 4.2). The deep dorsal arteries
Fig. 4.2 Fascial coverings, vessels, and nerves in cut sec- Between the skin and Buck’s fascia lie the loose superfi-
tion of penis: The body of the penis is composed of three cial fascia (dartos) and superficial vessels. Between
cylinders: two corpora cavernosa and one corpus caverno- Buck’s fascia and tunica albuginea lie the deep vessels
sum. These three cylinders are covered with three dense and dorsal nerves (© Ahmed T. Hadidi 2022. All Rights
layers: the skin, Buck’s fascia, and the tunica albuginea. Reserved)
4 Surgical Anatomy of the Penis and Urethra 107
arise from the bulbourethral artery, a branch of Beneath the dartos fascia lies a very thin
the internal pudendal artery (Fig. 4.5). connective tissue layer called the tela subfas-
cialis. It is more prominent at the base of the
penis, where it covers the extracorporeal seg-
4.3.1 Coverings of the Penis ments of the cavernous arteries, veins, and
nerves.
(a) The skin over the penis is composed of a strat- (c) The Buck’s fascia (deep fascia of the penis,
ified squamous keratinized epidermis and der- Buck 1848) is a yellowish, dense elastic
mis. The stratified squamous keratinized layer that encloses the two corpora caver-
epidermis adapts readily to prolonged contact nosa and — in a separate compartment —
with urine, making it suitable material for ure- the corpus spongiosum. Buck’s fascia is
thral replacement. The dermis contains strands composed of obliquely running fibers and is
of smooth muscle of the dartos tunic, nerves, firmly attached to the underlying tunica
sebaceous glands, and peripheral blood ves- albuginea. Superficial to Buck’s fascia run
sels. The penile skin has peculiar characteris- the superficial dorsal arteries and veins and
tics, having fewer sweat glands and hair deep to it run the deep arteries, veins and
follicles compared to the skin of the scrotum nerves.
and also over the symphysis pubis. The darker At the distal end, Buck’s fascia is firmly
color of penile skin is due to melanin secreted attached to the base of the glans at the coro-
by a relatively large number of melanocytes. nal sulcus, where it fuses with the ends of
The connective tissue under the dermis is corpora. At the proximal end, it is firmly
thin, loose, and devoid of fat. The penile skin attached to the pubic rami and also the ischial
is particularly mobile and expansile to spines and tuberosities.
accommodate erection. Such flexibility
makes it susceptible to edema, much like the
This is of surgical importance as bleeding
eyelids. The lack of fat explains why obese
deep to Buck’s fascia is limited proximally
people have a normal sized penis.
by its firm attachment to the pubic and
(b) The dartos fascia (superficial fascia of the
ischial bones. Urinary extravasation, in
penis) consists of bundles of smooth muscle
contrast, travels through the dartos fascia
fibers embedded in a loose areolar layer. The
around the penis superiorly into the lower
smooth muscle fibers extend into the skin and
abdomen under Scarpa’s fascia and inferi-
Buck’s fascia. The dartos fascia is the continu-
orly into the scrotum.
ation of Colles’ fascia (membranous layer of
the superficial fascia of groin and perineum).
The penile dartos, similar to the scrotal coun-
terpart, produces a retraction of the genital (d) The tunica albuginea is the deepest covering
structures when the external temperature falls. of the penis. It forms a thick white sheath and
There is continuation of the dartos fascial is covered by Buck’s fascia. Two layers can
space between the penis, scrotum, and the be identified as follows (Fig. 4.3): (1) an
groin. This explains why urine leakage after outer longitudinal coat (tunica albuginea
urethral injury or urethroplasty may cause externa) and (2) an inner circular coat.
edema and swelling of the groin and scrotum.
The blood supply of the penile skin comes
from the superficial dorsal arteries in the dar- This is of surgical importance in the correc-
tos fascia, thus allowing complete degloving tion of chordee as it makes it possible to
of the penis without damage to the vascular- incise, dissect, and excise the hypoplastic
ity of the skin. It is of surgical interest that outer longitudinal layer and leaving the
skin incisions on the ventral aspect heal with inner circular layer intact to preserve the
less visible scars than incisions on the dorsal corpora cavernosa.
aspect of the penis.
108 A. T. Hadidi
The tunica albuginea becomes thicker ven- provide free vascular communication
trally as it forms a groove for the corpus between the corpora.
spongiosum. The tunica condenses in the
middle between the two corpora cavernosa to
form an intercavernous septum. The distal 4.3.2 The Corpora Cavernosa
part of this septum is called the pectiniform
septum and is perforated by openings that The two corpora cavernosa are closely opposed
for three-quarters of their length by the intercav-
ernous septum. Proximally, they diverge from
each other and form the blunt-ended crus.
Distally, their rounded ends come together where
they meet the upper two-thirds of the glans.
Each corpus cavernosum consists of a spongy
vascular core and also an inner and outer layer of
the tunica albuginea. Continuous with the inner
surface are numerous flat columns called sinusoi-
dal trabeculae.
The sinusoidal trabeculae are composed of
collagenous, elastic, nervous, and smooth muscle
fibers surrounding the cavernous sinuses (spaces
or veins). The cavernous sinuses of the corpora
cavernosa are lined with endothelium and receive
blood from the dorsal and deep arteries of the
penis. The deep arteries branch inside the corpora
cavernosa and form the helicine arteries, which
empty directly into the cavernous sinuses
(Fig. 4.4).
During erection, when the tunica albuginea
Fig. 4.3 Structural layers of tunica albuginea and cor- becomes distended with blood, its composition
pora. In hypospadias, it is possible to dissect the hypo- (two layers of fibers running at right angles to
plastic outer longitudinal layer during the correction of each other, as in an automobile tire) limits expan-
chordee (© Ahmed T. Hadidi 2022. All Rights Reserved) sion and provides the necessary rigidity. Because
Fig. 4.4 Transverse

section of the penis (low
magnification) (©
Ahmed T. Hadidi 2022.
All Rights Reserved)
the tunica is consequently under tension, it is the corpus cavernosa. Numerous blood vessels
subject to flexion injury — either a fracture or a supply the corpus spongiosum.
lesser injury that, if repeated, may result in a The cavernous sinuses in the corpus spongio-
scarring deformity in susceptible men (known as sum receive their blood supply from the bulbo-
Peyronie’s disease). urethral artery, a branch of the internal pudendal
artery. It must be noted that the dorsal arteries
and nerves curve ventrally as they approach the
4.3.3 The Corpus Spongiosum coronal sulcus before entering the glans (Fig. 4.5).
Surgical separation of the glans from the ends of
The corpus spongiosum, arising at the perineal the corpora will tether the blood supply of the
membrane, has its own relatively thin sheath and glans from the dorsal arteries. This explains why
also an extension of Buck’s fascia that surrounds the vascular pedicle has to be dissected intact
it and separates it from the corpora cavernosa. together with the glans.
The proximal end is known as the bulb of the
penis and its distal end merges with the glans.
The corpus spongiosum consists of cavernous 4.3.4 The Bulbospongiosus Muscle
sinuses lined by endothelial cells and surrounded
by connective tissue trabeculae that contain both The bulbospongiosus muscle lies in the midline,
smooth muscle and collagen fibers, like that of anterior to the perineal body, and consists of two
a b
Fig. 4.5 (a and b) Arterial supply to the glans penis. The the bulbourethral artery to the corpus spongiosum and
penile arteries originate from the internal pudendal artery. urethra) before continuing as the deep dorsal artery to
Each penile artery gives two branches at the root of the supply the glans of the penis ((a) © Ahmed T. Hadidi
penis (the cavernous artery to the corpus cavernosum and 2022. All Rights Reserved).
110 A. T. Hadidi
symmetrical parts united by a fibrous median nar epithelium) and the ectodermal glanular ure-
raphe. Its fibers diverge from the median raphe thra (lined with stratified squamous epithelium)
like the two halves of a feather. The posterior (see Chap. 3: Embryology).
fibers unite with the perineal membrane, whereas Throughout the urethra are numerous
the middle fibers encircle the bulb and corpus branched mucous urethral glands (Littre’s gland)
spongiosum. The anterior fibers spread out later- that extend into the corpus spongiosum. A series
ally to cover and also terminate in the corpora of strikingly large glands, opening at the distal
cavernosa in a tendinous expansion which covers dorsal wall, are called the urethral lacunae of
the dorsal vessels of the penis. Morgagni. Their large secretory ducts run
The bulbospongiosus muscle helps to expel obliquely forward to open into the urethral lumen
urine from the urethra after the bladder has been near the meatus. The secretions of the Littre’s
emptied. It may assist in the final stage of erec- glands lubricate the urethra prior to ejaculation.
tion as the middle fibers compress the erectile
tissue and the anterior fibers compress the deep
dorsal vein. It contracts six or seven times during
ejaculation, assisting the expulsion of semen
(Gray’s Anatomy 2005). In hypospadias, all the
ventral structures of the penis are hypoplastic and
the bulbospongiosus muscle may be deficient.
This explains why patients with hypospadias
may have urine dripping after micturition due to
the lack of bulbospongiosus contractions to
empty the urethra.
4.3.5 The Penile (Spongy) Urethra
The penile urethra spans the entire length of the

penis and is surrounded by the corpus spongio-
sum. Its intra-bulbar part is dilated and also dis-
tally at the navicular fossa (the widening of the
penile urethra as it enters the glans penis).
The lumen of the penile urethra is slit-like
horizontally until micturition, where it dilates.
The external meatus is slit-like vertically. This
change in orientation from horizontal to vertical
assists in achieving a focused, consolidated urine
stream.
The prostatic urethra is lined with transitional
epithelium, continuous with that of the bladder.
At the level of the verumontanum, just distal to
the openings of the ejaculatory ducts, the penile
urethra is lined with pseudostratified columnar
epithelium. As the urethra approaches the fossa
navicularis, the epithelium becomes stratified
squamous (Fig. 4.6). The fossa navicularis seems
to be the meeting point between the endodermal Fig. 4.6 Horizontal section of the urethra (© Ahmed
penile urethra (lined with pseudostratified colum- T. Hadidi 2022. All Rights Reserved)
4.4 The Glans fibers, but neither osteocytes nor chondrocytes

are found.
The glans is the part of the penis distal to the
coronal sulcus and is covered by a pink smooth
mucous membrane. It has the shape of an obtuse 4.4.1 Dimensions of the Glans
cone, similar to that of the cap of a mushroom. It
is molded over and attached to the blunt extrem- Sennert et al. in a recent study showed that the
ity of the corpora cavernosa and extends proxi- dorsal vertical length of the glans (DVL) is usu-
mally over the dorsal aspect more that ventrally ally equal to the glans width (GW) with a mean
(an oblique mushroom cap). The corpora caver- of 14 mm (range 10–18 mm) in infants
nosa encased by the tunica albuginea extend deep 6–18 months old [4]. The mean ventral vertical
into the glans in approximately 77% of cases length (VVL) measured from the tip of the exter-
(Figs. 4.7 and 4.8). nal meatus to the proximal end of the ventral
Hsu et al. described the presence of a distal glans opposition is 7 mm (range 5–9 mm). In
ligament in the human glans penis [3]. They com- other words, the ventral vertical length (VVL) is
pared this ligament to the os penis that is present half the dorsal vertical length (DVL) which is
in dogs and rats. The distal ligament is an aggre- equal to the glans width (GW) in most infants.
gation of the outer longitudinal layer of the tunica Hutton and Babu in 2007 examined the nor-
albuginea and is composed of type I collagen mal anatomy of the external urethral meatus in 75
Fig. 4.7 Longitudinal

section of the distal
penis (© Ahmed
T. Hadidi 2022. All
Rights Reserved)
112 A. T. Hadidi
Fig. 4.8 (a) Normal urethral meatus. (b) Glans configuration in hypospadias (© Ahmed T. Hadidi 2022. All Rights
Reserved)
“normal” boys (mean age 6.9 years) and reported

that the mean VVL is 10.1 mm in 7-year-old boys
(5.4 mm plus 4.7 mm) [5]. Bush et al. measured
the GW in 240 control infants (1.5 months old)
and found the mean GW to be 14.3 mm [6]. It
must be remembered that all these measurements
are highly variable among normal boys and are
operator dependent.
4.4.2 Glans-Penis Ratio
An interesting observation is that the dorsal verti-

cal length (DVL) of the glans is about half the
flaccid penile length (PL) in infants (1.5 cm:3 cm
or 1:2) and one third of the flaccid penile length
in adults (3 cm:9 cm or 1:3) (Hadidi personal
communication). Again, there are large individ-
ual variations among normal individuals.
The corona is the base of the conically
shaped glans and occupies 80% of the circum-
ferential head of the glans. Ventrally, the glanu-
lar wings do not fuse together in the midline as
they are separated by the urethral meatus and
the frenulum (Fig. 4.9). The diameter of the
Fig. 4.9 Transverse section of normal fetal glans penis at
corona is wider than the shaft, and the remain- 25 weeks of gestation. Note that the glanular wings do not
der of the glans, resulting in the coronal sulcus. fuse ventrally but are separated by the frenulum. Also note
From a surgical standpoint, this means that the that the corpus spongiosum is a separate structure that
glanular wings can be dissected from the cor- fuses with the glans (from Baskin et al. “Anatomical stud-
ies of hypospadias”, J Urol, Vol 160, Issue 3 Part 2,
pora cavernosa in a relatively bloodless plane. pp1108–1115, © 1998, with permission from Wolters
The coronal sulcus has been reported to have Kluwer [7])
Tyson’s glands, which are described as modified
sebaceous glands responsible for smegma pro-
duction. Smegma represents epithelial debris 4.4.3 Fossa Navicularis
and secretions collected in this space. However,
several studies failed to demonstrate these As the urethra enters the region of the glans, it
glands. Apparently, the original description by dilates to form the navicular fossa, and the lining
Tyson was based on primate studies that could of the urethra transforms from pseudostratified
not be confirmed in humans. Sebaceous glands columnar epithelium into stratified squamous
that are not related to hair follicles were found epithelium, confirming the theory of different
at the mucocutaneous junction of the foreskin embryological origins. The function of the fossa
and adjacent mucosa but not in the coronal sul- navicularis is probably as a control chamber that
cus [8]. converts the urinary stream in the distal urethra
Considering the glans as the distal tissues to a into a narrow stream with higher pressure. The
vertical line passing through the coronal sulcus result is increased velocity, as the stream passes
(Fig. 4.7), the corpora cavernosa encased by the through the narrow meatus. This provides a jet
tunica albuginea extend out into the glans in for directing the stream in order to prevent
about 77% of cases. self-contamination.
114 A. T. Hadidi
Holstein et al. in 1991 examined the histology ing erectile tissue. The transition between both
of the normal human urethra in 13 adult males. structures is sometimes difficult to determine at
They observed sharp demarcation at the level of medium or high magnifications. However, at low
fossa navicularis. They described five zones from power or even after careful gross inspection with
proximal to distal; from the urinary bladder to a magnifying lens, this transition is evident and
just proximal to fossa navicularis, the spongy follows a line corresponding to the geographic
penile urethra is lined with stratified columnar limit of the extension of venous sinuses of the
epithelium. At the fossa navicularis, there is corpus spongiosum. A predominance of free
sharp demarcation into non-keratinized stratified nerve endings over the genital corpuscles has
squamous epithelium which change at the ure- been described in the glans. A few neurosecre-
thral meatus at the top of the glans into keratin- tory dermal Merkel cells have been identified at
ized squamous epithelium. the end of the free nerves.
In the glans, the erectile tissue has the charac-
ter of a dense, venous plexus. Compared to the
4.4.4 The External Urethral Meatus corpora cavernosa, the interstitial fibrous connec-
tive tissue is more abundant and contains more
It is the narrowest part of the urethra and opens at elastic fibers and less smooth muscle bundles.
the lower half of the tip of the glans as a vertical
slit-like meatus (Fig. 4.8). Hutton and Babu in
2007 examined the normal anatomy of the exter- 4.5 The Prepuce
nal urethral meatus in 75 boys (mean age
6.9 years) and concluded that all 75 boys had a The prepuce (or foreskin) has been present in pri-
vertical slit-like meatus that commenced at the mates and most mammals for over 65 million
tip of the penis and ran ventrally [5]. Interestingly, years. It is prolongation of the shaft’s skin,
they reported that the mean (SD) vertical meatal reflecting beyond itself and passing into a muco-
length (AB) was 5.4 mm and the mean length of sal inner surface. This mucous membrane covers
ventral glans closure (BC) was 4.7 mm. In other the coronal sulcus as well as the surface of the
words, the external meatus is longer than the ven- glans. Grossly, the adult foreskin shows a cutane-
tral glans opposition and should normally accom- ous surface that is dark and wrinkled and a muco-
modate a F10 French. sal surface that ranges in color from pink to tan.
It must be noted that the authors described, Circumcision is documented on the Tomb of
rather inaccurately, their findings in the drawings Ankh-Ma-Hor 2340 BC, and circumcision of
and photos attached to the paper as “ventral glans infants (as described in Genesis 17) has been
closure” (BC), when they are actually describing practiced for approximately 4000 years.
the attachment of the frenulum. The glans does Circumcision on the eighth day represents a mark
not fuse together ventrally as evidenced in histo- of the convent between God and the Jewish peo-
logical sections (Fig. 4.9). ple. Even prior to monotheistic religions, circum-
The glans is covered with partially keratinized cision was routinely practiced on young children
stratified squamous epithelium. Some textbooks and adolescents.
state that the circumcised glans is much thicker Histologically, the foreskin is formed by five
and more keratinized than an uncircumcised layers (Fig. 4.10):
man, but no well-controlled studies support this The epidermis: a keratinized stratified squa-
statement. mous epithelium that contains melanocytes,
The lamina propria separates the erectile tis- Langerhans cells, and Merkel cells. The epider-
sue of the glans from the glans epithelium. In the mis is thinner than the mucosal epithelium and
glans, the penile fascial layers and the fibrous usually contains a pigmented basal layer. It
sheath of the corpus spongiosum are absent, so exhibits relatively few hairs, sebaceous, and
the lamina propria adheres firmly to the underly- sweat glands.
face of the glans, is accentuated near the frenu-

lum. Two hollow fossae flank the frenulum.
Yang and Bradley demonstrated that the fren-
ulum receives double innervations both from the
dorsal nerve of the penis and from the perineal
nerve [9]. This is in contrast to the rest of the
glans which is innervated through the dorsal
nerves of the penis only.
Fig. 4.10 Histological section of the prepuce (© Ahmed

T. Hadidi 2022. All Rights Reserved) 4.7 The Median Raphe
Normally, the penoscrotal raphe begins at the

The dermis: consists of finely fibrillar connec- central part of the perineum and passes forward
tive tissue with blood vessels and nerve bundles. in the midline to reach the frenulum in the glans.
Meissner corpuscles, Vater-Pacini corpuscles, Ben-Ari et al. in their prospective study on
and sweat glands can be observed. 274 male newborns found deviation of the
The dartos consists of smooth muscle fibers median raphe without deviation of the meatus in
invested with elastic fibers and is the middle layer 10% of the “normal” newborn male babies [10].
of the foreskin. It is an extension of the fascia in There were three patients with hypospadias (1%
the penile shaft which is continuous with the of male newborns). They found that deviation of
scrotal dartos muscle. At the level of the edge of the median raphe was associated with deviation
the foreskin, the fibers are transversely arranged of the meatus in 2.2%. The deviation was always
to form a sphincter to close this edge over the anticlockwise. It is possible that those patients
anterior end of the glans. had a mild degree of hypospadias (maldevelop-
The lamina propria is composed of a vascular ment of the ventral surface of the penis).
connective tissue that is looser than the lamina
propria of the glans.
The mucosal squamous epithelium is usually 4.8 Nerve Supply of the Penis
thicker with more irregular and broad rete ridges
than in the epidermis. Adnexal structures are 4.8.1 Somatic Innervation
absent.
The somatic nerve supply comes from the spinal
nerves S2, S3, and S4 by way of the pudendal
4.6 The Frenulum nerve. The pudendal nerve passes under the
sacrospinous ligament close to the ischial spine
It is a pyramidal median fold that passes from the (a landmark for perineal nerve block), over the
deep surface of the prepuce to the meatal groove. sacrotuberous ligament and then through the
Current embryological theory shows that the pre- pudendal canal (Alcock’s canal). There it gives
puce starts laterally and dorsally and then grows off the perineal nerve, with branches to the pos-
ventrally over the open glanular groove to form terior scrotum, and rectal nerve to the inferior
the floor of the glanular urethra and the frenulum. rectal area and the ventral aspect of the penis and
This is supported by the fact that the frenulum frenulum (this explains why some patients com-
has its own separate blood supply and comes plain of pain in the rectum after urethral
from the frenular branches of the dorsal artery surgery).
that curve around each side of the distal shaft to The pudendal nerve continues as the dorsal
enter the frenulum and the glans ventrally. nerve of the penis. The dorsal nerve runs on the
Cutaneous sensitivity, which is high over the sur- deep layer of the urogenital diaphragm, where it
116 A. T. Hadidi
gives off a branch to the crus. It then passes –– The other branch, the greater cavernous
through the deep transverse perineal muscle to lie nerve, supplies the erectile tissue of the cor-
on the dorsum of the penis between Buck’s fascia pora cavernosa before terminating as a deli-
and tunica albuginea. Along its course on the cate network of fibers in the hilum of the
dorsum of the penis, it is accompanied by the penis.
dorsal artery of the penis and terminates in mul-
tiple branches in the glans.
The main cutaneous nerve supply to the penis 4.8.3 Neurovascular Bundle
and scrotum comes through the dorsal and poste-
rior branches of the pudendal nerve. The anterior The cavernous nerve runs with the dorsal nerve
portion of the scrotum and proximal part of the of the penis and the dorsal arteries constitute
penis are supplied by the ilioinguinal nerve after the so-called the neurovascular bundle. They
it leaves the superficial inguinal ring. run superficial to the circumflex arteries and
In other words, the dorsal nerve of the penis veins, the lateral veins, and the retrocoronal
innervates the glans, the dorsal, and lateral aspects plexus.
of the penis. The frenulum, on the other hand, Some studies suggest that the neurovascular
receives dual innervations both from the dorsal bundle in the human penis extends from the 11
nerve of the penis and the perineal nerve which and 1 o’clock positions to the urethral spongio-
also innervates the under surface of the penis. sum junction. Based on this assumption, sur-
This has surgical implications that dorsal nerve geons who advocate tunica albuginea plication
block only is not adequate for penile surgery. (TAP) as a technique to correct chordee associ-
ated with hypospadias believe that plication in
the 12 o’clock position is safe and in a nerve-free
4.8.2 Autonomic Innervation zone [11] (Fig. 4.11).
The sympathetic nerves arise from the lumbar

nerves L1 and L2 and the parasympathetic nerves 4.9 Suspensory Ligaments
from the sacral nerves S2, S3, and S4. The pelvic
plexus adheres to the base of the bladder, forming The root of the penis is suspended by two liga-
the vesical and prostatic plexuses. Nerves from ments (Fig. 4.12):
the prostatic plexus supply the prostate, ejacula-
tory ducts, seminal vesicles, and the urethra. • The fundiform ligament: Is more superficial
The cavernous nerve arises as many fine fibers and is actually a diffuse midline thickening of
from the pelvic plexus. These are mixed nerves the superficial fascia (Scarpa’s). The ligament
with the sympathetic fibers producing vasodilata- originates at the linea alba and, after splitting
tion and the para-sympathetic fibers producing to pass around the base of the penis, joins
vasoconstriction. Colles fascia. It is not necessary to preserve or
The cavernous nerve leaves the pelvis between restore it at surgery.
the transverse perineal muscles and the membra- • The suspensory ligament proper: This is an
nous urethra before passing beneath the arch of important structure for the maintenance of the
the pubis to supply each corpus cavernosum. penile position during coitus. Surgical sever-
Two branches may be found: ance may cause the penis to assume a lower,
less effective position. It is advisable to
–– One branch is the lesser cavernous nerve that approximate the ligament with sutures if the
supplies the erectile tissue of the corpus spon- ligament is surgically divided during opera-
giosum as well as the penile urethra. tions on the urethra.
Fig. 4.11 Relations of nerves and vessels on the dorsum of the penis (© Ahmed T. Hadidi 2022. All Rights Reserved)
interobserver variations [12]. Some studies

showed direct relation between a stretched penis
and an erected penis [13, 14]. Measurements of
penile length for age have been reported as
3.9 ± 0.8 cm at 0 to 5 months, 4.3 ± 0.8 cm at 6 to
12 months, and 4.7 ± 0.8 cm at 1 to 2 years.
4.10.1 N
ormal Size of the External
Genitalia in Adults
Spyropoulos et al. in 2002 studied the normal size

of the penis, glans, and testes in 52 physically nor-
mal men [15]. Their ages ranged from 19 to 38 years
(mean 25). Interestingly, both testes were equal in
size in only 5.8% of the study with the right bigger
in 65% (not significant). The mean total penile
length (flaccid, slightly stretched) was 12.18 +/−
1.7 cm. There was a significant relation between the
Fig. 4.12 Ligaments supporting the penis (© Ahmed
index finger length and the length of the penis.
T. Hadidi 2022. All Rights Reserved)
4.10.2 A
noscrotal Distance (ASD)
4.10 Normal Size of the Penis (Figs. 4.13 and 4.14)
The size of penis varies among normal men just Anogenital distance (AGD) is an androgen-
like the weight and height. Several factors play a responsive anatomic measurement that may have
role including race, nutrition, as well as environ- significant utility in clinical and epidemiological
ment. The penis may be measured stretched, flac- research studies. Studies showed that the human
cid, or slightly stretched. There is also perineum is twice as long in males as in females
118 A. T. Hadidi
a b c
d e
Fig. 4.13 Anoscrotal distance (ASD). (a) Glanular hypo- that 3 cm is about 2 finger breadth. (d) Proximal hypospa-
spadias with 5 cm ASD. (b) Distal hypospadias with 3 cm dias with 2 cm ASD. (e) Perineal hypospadias with 1.5 cm
ASD. (c) Mid-penile hypospadias with 3 cm ASD. Notice ASD (© Ahmed T. Hadidi 2022. All Rights Reserved)
but males have more variance [17]. Measuring not every type of the nine phthalates tested being
the anogenital distance in neonatal humans has correlated with shorter AGD [8].
been suggested as a noninvasive method to deter- Salazar-Martinez et al. measured the anogeni-
mine male feminization and thereby predict neo- tal distance in 87 healthy, full-term newborns (42
natal and adult reproductive disorders. females, 45 males) from Mexico [19]. He con-
A study by Swan et al. determined that the cluded that the AGD measure was about twofold
AGD is linked to fertility in males, not penis size greater in males (mean, 22 mm) than in females
[18]. Males with an abnormally short AGD (mean, 11 mm).
(lower than the median around 52 mm (2 in)) Sathyanarayana et al. measured AGD in 169
have seven times the chance of being sub-fertile (82 male, 87 female) infants in Washington and
as those with a longer AGD. It is linked to both found out that the mean male and female AGD
semen volume and sperm count. A lower than measurements were 52.0 mm (SD ± 5.5) and
median AGD also increases the likelihood of 37.2 mm (SD ± 3.7). In 2015, they published
undescended testes and lowered sperm counts another study on 768 newborns and found that
and testicular tumors in adulthood. Babies with the mean AGD was 24 mm in males and 16 mm
high total exposure to phthalates were ninety in females and found a relation between the AGD
times more likely to have a short AGD, despite and penile width [20](Fig. 4.16).
Thankamony et al. measured AGD in 463 pattern. AGD was positively correlated with
male and 426 female full-term infants in the penile length at birth (r = 0.18, p = 0.003), and
UK. The mean AGD (± SD) at birth was the increase in AGD from birth to 3 months was
19.8 ± 6.1 mm in males and 9.1 ± 2.8 mm in correlated with penile growth (Fig. 4.15).
females (p < 0.0001) [22]. AGD increased up to Wirmer et al. in 2021 examined the anoscrotal
12 months in both sexes and in a sex-dimorphic distance in 817 boys. Their study showed that a
short ASD is a helpful marker of the severity of
chordee in hypospadias. The average ASD of
patients with proximal and perineal hypospadias
(2.26 cm) was significantly shorter than the aver-
age ASD of patients with glanular and distal
hypospadias (3.09 cm) (p < 0.05). Patients with
glanular hypospadias and short ASD had 38%
chance of having severe chordee [23].
4.10.3 Penile Length
Penile length significantly increases with gesta-

tional age (6 mm at 16 weeks to 26.4 mm at
38 weeks) [25]) and during the first 3 months of
life. The latter growth is a result of an increase in
Fig. 4.14 The anogenital distance (AGD) and the ano- Leydig cell production of testosterone caused by
scrotal distance (ASD) (reprinted from Journal of Pediatric
Urology, Vol 13, Issue 1, by Cox K. et al.: “Shorter ano-
the loss of the inhibitory effect on maternal estro-
genital and anoscrotal distances correlate with the severity gens on the fetal pituitary at birth, thereby caus-
of hypospadias: A prospective study”, pp57.e1–57.e5, © ing a surge in gonadotropins (mini-puberty).
2017, with permission from Elsevier [16]) During the remainder of childhood, penile length
a b
Fig. 4.15 Anogenital distance/anoscrotal distance/ano- junction of the perineum with the rugated scrotal skin
fourchette distance diagrams. (a) Center of the anus to the (ASD) measurements made in male subjects (reprinted
anterior clitoral surface (AGD) and the center of the anus from Int. Journal of Andrology, Vol 33, Issue 2, by
to the posterior fourchette (AFD) measurements made in Sathyanarayana et al.: “Measurement and correlates of
female subjects. (b) Center on the anus to the anterior anogenital distance in healthy, newborn infants “, pp317–
base of the penis (AGD) and the center of the anus to the 323, © 2010, with permission from Wiley and Sons, [24]).
120 A. T. Hadidi
increases very slowly till adolescence when there hair, penis and testes. These changes are helpful
is a sudden increase until the final size is reached. to degree of development and maturity in patients.
The stages range from preadolescent penis and
testes with no pubic hair (stage 1) to adult-size
penis and scrotum with adult hair distribution
The normal penile size in a full-term male (stage 5).
neonate is 3.5 ± 30.7 cm stretched length
and 1.1 ± 0.2 cm in diameter compared
with 13.3 ± 1.6 cm at adulthood.The 4.11 lood Supply of the Skin
B
stretched penile lengths (measures from of the Penis and Prepuce
meatus to peno-pubic junction) from birth
to adulthood are listed in Table 4.1. Sharony Surgically, the penis is covered by three dense
et al. demonstrated a positive correlation layers: the skin, Buck’s fascia, and the tunica
between prenatal penile length measure- albuginea. Between the skin and Buck’s fascia
ments and postnatal measurements [28]. lie the loose superficial fascia (dartos), the super-
ficial dorsal arteries, and veins (Fig. 4.2). The
superficial dorsal arteries arise from the external
pudendal artery and supply the skin of the penis
4.10.4 T
anner Classification of Sexual and prepuce (Fig. 4.5).
Maturity Stages in Boys The blood supply of the penile skin comes
(Fig. 4.16) from the superficial dorsal arteries in the dartos
fascia, thus allowing complete degloving of the
Tanner stages are a set of maturation changes that penis without damage to the vascularity of the
occur during puberty to three components: pubic skin.
Table 4.1 Stretched penile length (cm) in normal male

subjects (SD standard deviation) (Data from Feldman and
It is of surgical interest that skin incisions
Smith, J Pediatr. 86:895, 1975 [13], Schonfeld and Beebe, on the ventral aspect heal with less visible
J Urol. 30:554, 1975 [26], Tuladhar et al., J Paediatr Child scars than incisions on the dorsal aspect of
Health. 34:471, 1998 [27]) the penis.
Stretched penile length (cm) in normal male subjects
Age Mean ± SD Mean–2.5 SD
Neonate, 30-week 2.5 ± 0.4 1.5
gestation 4.11.1 P
reputial Arterial Supply
Neonate, 34-week 3.0 ± 0.4 2.0 and Venous Drainage
gestation
0–5 month 3.9 ± 0.8 1.9
The blood supply of the penile skin is symmetri-
6–12 mo 4.3 ± 0.8 2.3
cal [29]. The superior and inferior external
1–2 year 4.7 ± 0.8 2.6
2–3 year 5.1 ± 0.9 2.9
pudendal arteries arise from the femoral artery.
3–4 year 5.5 ± 0.9 3.3 They are attached to the Scarpa fascia, which
4–5 year 5.7 ± 0.9 3.5 extends to the base of the penis. At this point,
5–6 year 6.0 ± 0.9 3.8 they divide into four branches as superficial
6–7 year 6.1 ± 0.9 3.9 penile arteries. Two enter the superficial penile
7–8 year 6.2 ± 1.0 3.7 fascia (Colles fascia) dorsolaterally and two enter
8–9 year 6.3 ± 1.0 3.8 it ventrolaterally (Fig. 4.17). Numerous collater-
9–10 year 6.3 ± 1.0 3.8 als between these four arteries create a fine sub-
10–11 year 6.4 ± 1.1 3.7
cutaneous arterial plexus up to the preputial ring
Adult 13.3 ± 1.6 9.3
(Fig. 4.18) [30]. Behind the sulcus in the distal
Preadolescent: testes, scrotum, and penis are

Stage 1 about the same size in proportion as in early
I
<2,5
G1 childhood, Testicular volume < 4 ml or 3
long axis < 2.5 cm
Enlargement of scrotum and of testes. The

skin of the scrotum thickens without
2,5-3,2
Stage 2
G2
enlargement of penis, testicular volume 4 ml- II 4
8 ml (or 2.5 to 3.3 cm long), 1st pubertal sign
in males
Penile enlargement starts. Further growth of

Stage 3
III
3,6
testes and scrotum, testicular volume 9 ml- 10
G3
12 ml (or 3.4 to 4.0 cm long)
Increased size of penis, with growth of glans.
4,1-4,5
There is further enlargement of testes and
Stage 4
G4 scrotum, testicular volume 15-20 ml (or 4.1 to IV 16
4.5 cm long)
Stage 5 Genitalia are adult in size and shape,

testicular volume > 20 ml (or > 4.5 cm long)
>4,5
G5
V 25
Fig. 4.16 Tanner stages in boys showing changes in Retrieved 16:17, September 24, 2021 from https://com-
external genitalia, pubic hair, and testicular volume (data mons.wikimedia.org/w/index.php?title=File:Tanner_
from Tanner [21], File:Tanner scale-male.svg. (2021, June scale-male.svg&oldid=567207648
4). Wikimedia Commons, the free media repository.
part of the penile shaft, small vessels penetrate 4.11.2 Surgical Considerations
the Buck’s fascia, making an anastomosis with
the dorsal penile artery. Beyond the preputial ring According to the anatomical studies of Hinman
on the inner surface, the terminal branches [31], the prepuce develops over a mesodermal
become minute. Variations of the superficial core that is later split and not by distal shaft
penile arteries are possible with dominance of skin sliding to cover the glans. Although its
one side pair. arterial blood supply would be expected to run
The blood supply to the frenulum is also sym- distally in the connective core terminating at
metrical and arises from the dorsal penile artery, the preputial ring, this is not the case. The arte-
which branches at the level of the sulcus with rial supply of the prepuce origins from the dor-
small arteries that curve around each side of the sal aspect of the penis, and the minute arteries
distal shaft to enter the glans and the frenulum supplying the outer prepuce layer fold by 180°
ventrally (Fig. 4.18). to terminate at the corona. The reason is that
The venous drainage of the penile skin and the prepuce must form a one-layer skin sheet
prepuce is less well organized (Fig. 4.19). during erection and the terminal vessels
Multiple minute veins in the prepuce form a become straightened when erection and prepu-
plexus without particular orientation joining the tial retraction begin. When erection terminates
superficial dorsal penile vein, which drains into the prepuce vessels loop back upon themselves,
the external pudendal vein, which in turn empties terminating at the corona and not at the prepu-
into the saphenous or femoral vein. tial ring (Fig. 4.20).
122 A. T. Hadidi
a b
Fig. 4.17 End arteries to the prepuce. (a) Normal blood supply, equal distribution from both sides, (b and c) dominant
right-sided distribution
Fig. 4.18 Arterial blood supply of the frenulum
Fig. 4.19 Peripheral venous drainage of the penis

Fig. 4.20 One-layer skin sheet of the prepuce during erection
Taking into consideration the abovementioned of flap to be performed. In the majority of cases,
anatomy, an incision along the coronal sulcus the desired success should be guaranteed.
would not damage the arterial supply of the pre- However, in addition, some rules have to be
puce in any way as the vessels terminate at this kept in mind when operating:
site (Fig. 4.21). The inner layer of the prepuce
can be dissected without jeopardizing the vascu- • Keep intact at least one of the two branches of
lar pedicle (Fig. 4.21b). After separation of the the right or left external pudendal artery.
outer layer of the prepuce and careful dissection • Perform only longitudinal incisions in the
of the vascular pedicle, the flap may be rolled superficial penile fascia in order to preserve
into a tube for construction of the neourethra the axial arrangement of the superficial plexus.
(Fig. 4.21c). • Consider the prepuce as one unit with a com-
However, the clinical experience of a number mon blood supply to the inner and outer pre-
of authors that have widely published their opera- puce layers.
tive results as well as the follow-up of a great • The inner epithelial layer, corresponding to
number of patients has not encountered the limi- the more distal part of the unfolded prepuce,
tations of the abovementioned embryo- must be left generously attached to the super-
anatomical premises. ficial fascia supplying it with blood.
• The outer epithelial layer, corresponding to
the proximal part of the unfolded prepuce, is
4.12 Conclusions to be used to cover the neourethra. The preser-
vation of its arterial blood supply is essential.
The surgeon performing hypospadias repair using • The use of magnification for identifying the
a pedicle flap has to be aware of the normal vascu- terminal, minute arterial vessels is in my opin-
larization of the prepuce. Although it is very diffi- ion a prerequisite for every hypospadias repair.
cult to identify individual arterial variations,
especially during surgery, it is of great importance
to look for them. These vessels can be then used Acknowledgement I would like to thank and acknowl-
individually, selectively, or collectively, according edge the hard work done by Prof. Zacharias Zachariou on
the blood supply of the prepuce in the first edition of the
to the anatomical variation present and the choice book and is included in this chapter.
124 A. T. Hadidi
a b c
Fig. 4.21 Incisions for pedicle flaps with intact vascular layer of the prepuce with intact vascular supply; (c) neo-
blood supply: (a) incision at the coronal sulcus where the urethra from the inner prepuce layer and well-vascularized
vessels terminate; (b) dissection of the inner and outer outer prepuce layer to cover the defect
healthy controls. J Pediatr Urol. 2013; https://doi.

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Altered Development of Prepuce
in Hypospadias and Its Clinical 5
Relevance
Ibrahim Ulman
Abbreviations
5.2 Theories of Preputial
COVER Cobra eyes’ ventral reapproximation
Development
MAGPI Meatal advancement and glanulo-
Hunter, in 1935, in his elegant study of human
plasty incorporated
fetuses roughly between 12 weeks to over
MIP Megameatus intact prepuce
21 weeks of gestation, documented preputial
development clearly by serial sections stained
with hematoxylin and eosin and made wax-plate
reconstructions from them [2] (Fig. 5.1).
5.1 Introduction Hunter made wax-plate reconstructions from
histological sections of the developing penis of
Controversy regarding the development of pre- human fetuses between 12 and > 21 weeks of
puce in male fetus starts with its exact time inter- gestational age [2].
val during fetal life. According to Liu et al., He made reconstructions of the penis at differ-
preputial development starts at approximately ent stages of development.
11th week of gestation and is completed at ... the ridge of epithelial tissue behind the groove
17–18 weeks along with the glanular urethra [1]. which demarcates the glans from the body of the
In Hadidi et al.’s study of penile development in penis does not uniformly surround it in the form of
embryos up to 14th week of gestation, none of a circle, but is rather disposed in an oblique direc-
tion, radiating out on either side of the penis from
the specimens showed signs of development of the point of the urethral opening placed well back
the prepuce [2, 3]. The difficulty in identifying on the ventral surface of the organ. It appears as a
exact gestational age at this early stage might kind of "hood" on the dorsum, gradually becoming
have been a reason for this discrepancy [4]. less well marked as its ventrally continued folds
approach the urethral opening [2].
Despite small differences, most studies agree on

the originating site of the prepuce as the dorsal
aspect of the penis. The initial layer of tissue
originating from the dorsal ectoderm grows dor-
I. Ulman (*)
Urology Division, Department of Pediatric Surgery, sally, distally, and laterally beyond the corona
Ege University Faculty of Medicine, Izmir, Turkey over the thick “enigmatic” collection of desqua-
e-mail: ibrahim.ulman@ege.edu.tr mating cells. These cells mentioned frequently in
128 I. Ulman
a b c
Fig. 5.1 The lateral and ventral aspects of wax-plate Vol 70 (Pt1), by Hunter: “Notes on the Development of
reconstructions of three stages in the development of the the Prepuce”, pp68–75, ©1935, with permission from
prepuce: (a) 40 mm CR length; (b) 70 mm CR length; (c) Wiley and Sons [2]).
170 mm CR length. (Reprinted from Journal of Anatomy,
Hunter’s paper may be associated to the tempo- which ultimately forms the frenulum. The cav-
rary urethral plug or the so-called skin tag docu- ernous tissue in the glans is continuous with the
mented by different authors. The skin on the connective tissue of the prepuce, not separated by
dorsum develops “folds” as if it was growing any epithelial or other ectodermal tissue. This is
more rapidly than the differentiating mesoderm probably another area of embryonic epithelio-
into cavernous tissue. Hunter resembled this dor- mesenchymal interaction which is responsible
sal growth to a flow over the dorsum of the glans from the development of nearly every organ of
which at this stage was clearly separated from the the body including the kidneys, teeth, digestive
shaft of the penis by a well-marked sulcus (coro- tract, etc. [5].
nal sulcus). Another finding that most investigators agree
At the ventral midline of the glans, there is a upon is the unequal growth rates between the
synchronous double layer fusion (Fig. 5.2). One dorsal and ventral outer surface of the developing
of them is between sub-glans folds (which resem- penis. This is reflected by the folds on the dorsum
ble the glans wings in hypospadias) enclosing the in the very early phase and the covering of the
abovementioned plug of desquamating cells in glans by the developing prepuce earlier on the
fossa navicularis. Around but not fused to it, and dorsal side than on the lateral and ventral sides.
separated by the continuing desquamating cell The development of the glanular urethra ven-
layer (probably the origin of smegma), the ventrally is in synchrony to dorsal preputial develop-
tral margins of the overflowing prepuce fuse on ment. Hadidi et al. suggested that normal distal
top of the inner fusion. There is also a continuity migration of the glanulo-preputial lamella forms
between these skin folds and sub-glans groove the floor of the glanular urethra [3]. They advo-
5 Altered Development of Prepuce in Hypospadias and Its Clinical Relevance 129
a b
Fig. 5.2 Formation of glanular urethra according to Hunter mating epithelium. Note the continuity of the glans tissue
[2]. (a) Glans region of the penis from human fetus 100 mm with the mesodermal tissue of the prepuce at the frenulum,
CR length. Note the two sub-glans folds closing off a por- and the V-shaped line of fusion between the two sides of the
tion of the desquamating epithelium within the developing prepuce epithelium on its ventral aspect (reprinted from
glans portion of the urethra. (b) Glans region of the penis Journal of Anatomy, Vol 70 (Pt1), by Hunter: “Notes on the
from human fetus 170 mm CR length. The glans urethra is Development of the Prepuce”, pp68–75, ©1935, with per-
here seen closed off enclosing within it a plug of desqua- mission from Wiley and Sons [2]).
cate this theory by documenting the differing epi- Hadidi et al. in their theory have a compelling
thelium between the ventral and dorsal-lateral explanation on glandular urethral developmental
walls of the glanular urethra. They also believe arrest, which is clearly seen in most distal hypo-
that older theories suggesting fusion of “urethral spadias cases as a proximal meatus pointing ven-
labia” or “sub-glans folds” as named by Hunter trally, separated by a bridge of elevated glans
fail to explain megameatus intact prepuce (MIP) tissue (which is cut vertically and sutured hori-
type hypospadias. Nevertheless, separate fusion zontally in MAGPI repair) and a blind groove
of two different growing wings (sub-glanular distally allotting to where normal meatus should
folds and ventral preputial margins) on top of be [3]. So, we postulate that midline fusion in
each other with a failed fusion between them due two layers and migration from proximal to distal
to failed fusion of glanular folds may be a reason- are in progress at the same time, and both con-
able explanation for MIP. Alternatively, failure of tribute to the development of the ventral wall of
fusion on the outer layer (ventral preputial mar- the urethra, frenulum, and ventral prepuce.
gins) with normal fusion of glanular folds may Different types of preputial anomalies can be
also explain clinical cases with normal glans and explained by the arrest of either one or both of
hypospadiac prepuce, which is difficult to inter- these two embryologic events in changing
pret by the migration hypothesis (Fig. 5.3). Yet, degrees.
130 I. Ulman
Fig. 5.3 The migration hypothesis of Hadidi et al. [4] The prepuce plays an important role in the development of
showing the crucial role of ventral penile mesenchyme in the floor of the glanular urethra (from Hadidi personal
the development of the penis, urethra, and penile fascia. communication, courtesy of Ahmed T. Hadidi)
In summary, the glans penis is naked in the puce in hypospadias [6, 7]. To acquire relevance,
early phases of penile development. The ectoder- any classification of prepuce should have grounds
mal tissue grows more rapidly than the differen- on (patho-)embryologic development of the hypo-
tiation of the cavernous tissue, and folds occur on spadiac penis. In normal male external genitalia,
the dorsum of the penis. These folds then flow based on our observations, there are roughly six
over the dorsum of the glans which marks the different types of skin according to their charac-
beginning of the prepuce. The preputial margins teristics that change with their locations. In hypo-
are obliquely disposed along the sides of the spadias, the size, the shape, and the location of
glans toward the urethral opening which is well these different skin types change, but their charac-
back on the ventral surface of the penis at this teristics are preserved. This gives the surgeon a
stage. Arrest of development at this stage may valuable key to solve the puzzle and make appro-
explain typical hood appearance of the prepuce priate skin reconfiguration during hypospadias
together with the commonest form of coronal repair (Fig. 5.5). Management of these different
hypospadias. By further normal development of skin areas without understanding, recognition,
prepuce, its margins flow over the sides of the and proper replacement to their original sites does
glans and are drawn together on ventral surface not only compromise good cosmesis but also
by two sub-glans folds. It is unclear where, how, increases complication rate by risking blood sup-
and when developing preputial margins meet ply and promoting more fibrosis. Typical features
with the distally growing, differentiating, and of different skin types in male genitalia are sum-
migrating ventral penile mesenchyme which marized in the table below (Table 5.1).
hypothetically will form the ventral floor of the
glanular urethra. The line of fusion between the
preputial margins and glans folds, and possibly 5.3 Characteristics of Male
penile ventral mesenchyme form the frenulum, Genital Skin in Different
superficial to the glanular urethra formed around Genital Locations (Table 5.1)
the plug of desquamating cells in the glanular
groove. We all tend to evaluate the abnormal features of
Hence, preputial development is closely hypospadias cases as an arrest of the normal
linked to urethral development within the glans. growth at some point of penile development.
In hypospadiac penis, preputial tissue is absent However, development, although it may be
ventrally and excessive dorsally which gives the arrested at some point, continues at available tis-
typical dorsal hooded foreskin appearance sues. The absence of programmed normal tissues
(Fig. 5.4). does not halt continuing growth of surrounding
Some researchers have documented the differ- structures, but they rather try to compensate the
ences, especially in the prepuce, as variations, defective architecture throughout development,
where others attempted to classify abnormal pre- the end-point of which is obscure.
Fig. 5.4 Typical hood appearance of hypospadiac prepuce
Fig. 5.5 Correct repositioning of different genital skin types in skin reconstruction is an essential part of hypospadias
repair
132 I. Ulman
Table 5.1 Characteristics of male genital skin from different locations in the genital area
Skin type Thickness Color Reflection Hair follicles Creases Amount of subcuticular dartos tissue
Inner prepuce + Red Shiny − − +
Outer prepuce ++ Dark Matte − +++ +
Glans + Red Shiny − − −
Penile body +++ Bright Matte + − +++
Penile base +++ Bright Matte +++ − ++
Scrotum ++ Dark Matte +++ +++ ++
a b c d
Fig. 5.6 The hunchback (hump) appearance is clearly glans and enclosing a layer of desquamating cells between
seen in the dorsal aspect of the developing normal penis it and the glans. (d) LS Penis of human fetus 170 mm CR
where prepuce originates, from Hunter [2]. (a) LS Penis length. This section shows the prepuce covering the whole
of human fetus 40 mm CR length. Note the ridge of ecto- of the glans, with the layer of desquamating cells connect-
derm behind the corona and the glans and the thin layer of ing the two. Note the “plug” of desquamating cells closing
desquamating cells on the surface. (b) LS Penis of human the opening of the urethra (reprinted from Journal of
fetus 70 mm CR length. Note the dorsal ectodermal tis- Anatomy, Vol 70 (Pt1), by Hunter: “Notes on the
sues thrown into loose folds and the distribution of the Development of the Prepuce”, pp68–75, ©1935, with per-
desquamating cells. (c) LS Penis of human fetus 100 mm mission from Wiley and Sons [2]).
CR length. The prepuce is here seen “overflowing” the
The anomaly in hypospadiac penis is not a This excess tissue may well be the remnant of
simple vertical midline separation but an ellipti- developing prepuce programmed to move later-
cal defect of tissues subsequent to arrested or ally and ventrally as explained above. The other
maldevelopment in multiple layers. Simple mid- common feature of hypospadias is the so-called
line approximation of available tissues adjacent cobra eyes or monk’s hood deformity of the dor-
to the defect, as performed in repair techniques sal prepuce. It is not easy to explain the formation
based on Thiersch-Duplay principle, t heoretically of this abnormal characteristic. We advocate that
will never end up with a normal penis because of cobra eyes may serve as landmarks pointing the
the missing structures. On the other hand, avail- dorsal end of the embryo-pathologic folding line
able local tissues can be used to replace the miss- caused by defective ventral development of the
ing ones. In itself, hypospadias anomaly seems to dorsal penile skin. We hypothesize that these two
offer a compensation for the absent ventral tis- lateral suture lines originating from the hypospa-
sues by stocking some extra on the dorsum. This diac meatal level and ending with the cobra eyes
hunchback (hump) appearance in hypospadiac on the dorsum and joining ventrally and proxi-
penis may be explained by the starting site of the mally to continue as normal median raphe are
preputial development on the dorsal aspect of the formed by the absence of bilateral triangular skin
penis in fetal life (Fig. 5.6). fragments on the lateral-ventral walls of the pre-
Fig. 5.7 Paper model depicting defective hypospadiac prepuce with cobra eyes
puce followed by attachment of the free margins for appropriate excision of excess skin in a safe
during fetal development. A paper model was and shapely way. In this method, the very proxi-
developed to illustrate the defective prepuce with mal end of the dorsal vertical midline incision is
cobra eyes (Fig. 5.7). determined by approximating the cobra eyes ven-
During hypospadias repair, incision along trally at the corona in the midline. The dorsal
these lines is safe, because there are no crossing incision usually ends at the transverse line
vessels. The skin distal to cobra eyes is dorsal between the cobra eyes or just proximal to it. If
prepuce. Between cobra eyes, there is thick, this approximation creates a tight ring of skin at
bulky subcuticular dartos tissue that is usually the coronal level, then dorsal incision is extended
raised as a subcuticular tissue flap and utilized as accordingly until a length equal to the circumfer-
a protective covering layer over the neourethra in ence of the mucosal collar is achieved. After fixa-
most current repair techniques. This does not tion of the skin at the end of the dorsal midline
only serve as a protective layer but together with incision to the dorsal mucosal collar with a suture
spongioplasty when available, restores the anat- (Fig. 5.9a), on both sides, a triangle of skin distal
omy to normal as much as possible. A practical to the line between the dorsal fixation suture and
method for skin reconstruction based on this the cobra eye is excised (Fig. 5.9b, c, d). During
hypothesis was developed, the details of which excision of the skin, enough dartos tissue on both
are given below. sides is left as a viable supportive layer to cover
the urethral repair and to correct rotation if neces-
sary (Fig. 5.9e). No attempt is made to separate
5.4 COVER Technique for Skin dartos fascia from the skin, and they are moved
Reconstruction together easily to the ventral side. The low-
in Hypospadias Repair quality dark-colored skin with the most vulnera-
(COVER: Cobra eyes’ Ventral ble blood supply located medial to the line
Reapproximation) between the cobra eye and the former hypospa-
diac meatus is symmetrically excised on both
Classical vertical midline separation of the dorsal sides (Fig. 5.10). This is actually the ventrally
penile skin and moving the wings ventrally either displaced outer prepuce which is normally
as in Byars flap method or midline approxima- excised during circumcision. There is always
tion after trimming with a vertical suture line ample dartos tissue at this stage that one can use
does not always give satisfactory cosmetic results to support, contour, and reshape the hypoplastic
(Fig. 5.8). ventral penile body. Since it is not prepared as a
The abnormal protrusions of dorsal skin mim- bare flap separated from the skin, the blood sup-
icking cobra eyes (or dog-ears) are the main ply of both the dartos and the skin attached to it is
cause of bad cosmesis. Excision of them is an preserved. After the dartos is reconfigured, the
option but that will leave extra scars on both skin is closed vertically at midline as the anatom-
sides. Instead, we use cobra eyes as landmarks ical reconstruction of the median raphe (Figs. 5.9f
134 I. Ulman
Fig. 5.8 Unacceptable cosmesis with cobra eyes and abundant preputial skin left during initial surgery
a b c
d e f
Fig. 5.9 (a–f) The steps of COVER method used for skin reconstruction following urethra repair in hypospadias
surgery
and 5.11). The method can also be used in redo method may be necessary. Preservation of the
cases if the landmarks are identifiable (Fig. 5.12). foreskin is not possible with this method. Patients
This method is applicable in almost all distal who prefer foreskin reconstruction should be
hypospadias cases, and most proximal cases informed about the possible increase in compli-
where the cobra eyes are not located very proxi- cation rates [8].
mally. In cases with asymmetric cobra eyes and/ In conclusion, hypospadias anomaly has
or severe penile torsion, modifications of this numerous problems in different parts of male
Fig. 5.10 Excision of redundant preputial skin in COVER method
Fig. 5.11 Pre- and postoperative appearance with COVER method of skin reconstruction
Fig. 5.12 Reconfiguration of penile skin by COVER method in a redo case

136 I. Ulman
genitalia and prepuce is one of them. Better 4. Hansmann M, Schuhmacher H, Foebus J, Voigt
U. Ultrasonic biometry of the fetal crown-rump length
understanding of the developmental imperfec- between 7 and 20 weeks gestation (author’s transl).
tions and modifying treatment methods accord- Geburtshilfe Frauenheilkd. 1979;39:656–66.
ingly will ultimately improve the results which 5. MacCord K. Mesenchyme. Embryo Proj Encycl ISSN.
are still far from the ideal. 2012:1940–5030.
6. Radojicic ZI, Perovic SV. Classification of prepuce in
hypospadias according to morphological abnormalities
and their impact on hypospadias repair. J Urol. 2004;
References https://doi.org/10.1097/01.ju.0000129008.31212.3d.
7. Fahmy MAB. Congenital anomalies of the penis,
1. Liu X, Liu G, Shen J, Yue A, Isaacson D, Sinclair A, 2017; https://doi.org/10.1007/978-3-319-43310-3.
Cao M, Liaw A, Cunha GR, Baskin L. Human glans 8. Esposito C, Savanelli A, Escolino M, Giurin I, Iaquinto
and preputial development. Differentiation. 2018; M, Alicchio F, Roberti A, Settimi A. Preputioplasty
https://doi.org/10.1016/j.diff.2018.08.002. associated with urethroplasty for correction of distal
2. Hunter R. Notes on the Development of the Prepuce. J hypospadias: a prospective study and proposition of a
Anat 70. PT. 1935;1:68–75. new objective scoring system for evaluation of esthetic
3. Hadidi AT, Roessler J, Coerdt W. Development of and functional outcome. J Pediatr Urol. 2014; https://
the human male urethra: A histochemical study on doi.org/10.1016/j.jpurol.2013.09.003.
human embryos. J Pediatr Surg. 2014; https://doi.
org/10.1016/j.jpedsurg.2014.01.009.
Morphology of Hypospadias
6
Ahmed T. Hadidi
Abbreviations mon that the urethral meatus is on the under

surface of the penis or perineum.
AGD Anogenital distance Van der Meulen (1971) proposed to use the
ASD Anoscrotal distance term “cryptospadias” in order to include other
DSD Disorders of sex development closely related deformities such as congenital
DYG Double Y glanulomeatoplasty chordee without hypospadias (hypospadias sin
MAGPI Meatal advancement and glanulo- hypospadias) and torsion without hypospadias [1].
plasty incorporated The term “dyspadias” is probably an appropri-
MIP Megameatus intact prepuce ate term to use as it would include all the related
DVL Dorsal vertical length anomalies and it refers to the “disorganization
GW Glans width hypothesis” of the pathogenesis of hypospadias
VVL Ventral vertical length and associated anomalies (see Chap. 7:
Pathogenesis of Hypospadias).
There is a triad that influence the outcome of
hypospadias surgery: the patient, the surgeon, and
6.1 Introduction the technique (Fig. 6.1). The patient factors
include the morphology of the hypospadias
Hypospadias (Greek, hypo means under, spadon anomaly and the degree of hypoplasia and differ-
means rent or opening) is classically defined as entiation of the ventral penile structures and skin.
congenital anomaly in which the urethral meatus In this chapter, we are going to discuss the mor-
is situated on the under surface of the penis or phological features associated with hypospadias.
perineum. This simple definition, however, is The hypospadias abnormality usually affects
hardly an adequate description of the complex more than one structure of the male organ. This
nature of hypospadias. Hypospadias is better influences the choice of the most suitable opera-
defined as a wide spectrum of anomalies involv- tion for an individual patient. The task of the
ing the ventral penile structures that have in com- hypospadias surgeon is to try to correct all the
different components of hypospadias spectrum
and not just bring the urethral meatus to the tip of
the glans penis. The surgical technique that is
A. T. Hadidi (*) suitable for a child with a cleft glans and wide,
Department, Sana Klinikum Offenbach, Academic well-vascularized deep urethral plate is totally
Teaching Hospital of the Johann Wolfgang Goethe different from the technique suitable for a patient
University, Offenbach, Frankfurt, Germany with flat glans and fibrotic, nonpliable, narrow
138 A. T. Hadidi
(c) Flat glans: The urethral plate ends short of

the glans penis; no glanular groove.
Generous glans splitting is required to
achieve good cosmetic and functional
results. The new urethra has to be embedded
deep into the glans to have satisfactory cos-
metic appearance.
Fig. 6.1 The triad that influences the outcome of hypo- Avellan in his morphological study on 220
spadias surgery: the patient, the technique, and the sur-
unselected cases of hypospadias described the
geon (©Ahmed T. Hadidi 2022. All Rights Reserved)
glanular groove to be normal (or deep, cleft) in
66/220, shallower than normal (or incomplete
urethral plate. Because of their significance, the cleft) in 67/220, and shallow (or flat) in 87/220
urethral plate and chordee are discussed in Chap. patients [3].
8 and the megameatus intact prepuce (MIP) in From an embryological prospective, the dif-
Chap. 23. ferent forms of glans presentation are probably
Kaufmann presented a detailed description of due to different degrees of vacuolation of the
hypospadias that is still valid today [2]. Avellan glans plate. Absent vacuolation at the solid glans
described in detail the morphological features in plate stage may result in flat glans. Incomplete
his own series of 220 unselected primary cases of vacuolation results in incomplete cleft glans.
hypospadias [3]. The anomalies usually involve Cleft glans suggests normal complete grooving
the penis and scrotum (glans, fascia, prepuce, of the glans plate (see Chap. 3: Embryology).
etc.). However, it may also involve other Another interesting observation is that the
urological organs and even extra-urological tis- glanular wings usually appear as an “open book.”
sues as part of several syndromes. With careful examination of the glans in most
cases, one can usually identify the points that
should come together in order to achieve a near
6.2 The Glans normal-looking glans penis. Mobilization of the
glanular wings off the corpora cavernosa may be
Patients with hypospadias have an abnormal- needed to bring them together in the midline
looking globular glans. The glans is classified (Figs. 6.4 and 6.5).
into three categories based on the degree of cleft- Another observation in some patients with
ing and urethral plate projection [4] (Figs. 6.2 glanular and coronal hypospadias is the pres-
and 6.3). ence of a bridge (or elevation) at the junction
between the urethral plate and the grooved glans
(a) Cleft glans: There is a deep groove in the plate (Fig. 6.6). Embryologically, this suggests
middle of the glans with proper clefting; the failure of fusion of the rapidly growing penile
urethral plate is narrow and projects to the tip urethra with the grooved glans plate forming the
of the glans. An example is the “hidden incomplete glanular urethra. Proximal to the
hypospadias” (megameatus intact prepuce) bridge, there is pseudo-stratified columnar epi-
hypospadias. Tubularized plate technique thelium, and distal to the bridge, there is strati-
without incision or pyramid repair may be fied squamous epithelium. This junction is the
employed with good results. place where fossa navicularis is present in the
(b) Incomplete cleft glans: There is a variable normal urethra [5].
degree of glans split, a shallow glanular This bridge is usually suggestive that the ure-
groove, and a variable degree of urethral thra is mobile and can be stretched to the tip of
plate projection. Inverted Y tubularized plate the glans. The double Y glanulomeatoplasty
or inverted Y meatoglanuloplasty usually (DYG), MAGPI, or meatal advancement tech-
give good results. niques are suitable for such cases.
6 Morphology of Hypospadias 139
a b c
Fig. 6.2 Classification of glans configuration (a) Cleft cleft glans: There is a shallow glanular groove, usually
glans: There is a deep groove in the middle of the glans separate from the urethral orifice. (c) Flat glans: The
with proper clefting to the tip of the glans. (b) Incomplete glans penis is flat, no glanular groove
a b c
Fig. 6.3 Three patients with different glans configura- separate from the urethral orifice. (c) Flat glans: The
tions. (a) Cleft glans: There is a deep groove in the middle glans penis is flat, no glanular groove (©Ahmed T. Hadidi
of the glans with proper clefting to the tip of the glans. (b) 2022. All Rights Reserved)
Incomplete cleft glans: There is a shallow glanular groove,
6.2.1 Glans Dimensions d orsal vertical length (DVL), the ventral vertical
in Hypospadias length (VVL), the maximum glans width (GW),
and flaccid penile length (FPL) [6]. The DVL was
There are wide individual variations regarding found to be equal to the GW in the majority of
the size of the glans and its relation to the size of patients. He also found out that the VVL is half of
the penis. Sennert et al. conducted a study on that of the DVL and DVL is half of the FPL. 45%
1023 patients with hypospadias and measured the of all patients presented with a small glans width.
140 A. T. Hadidi
Fig. 6.4 Diagram showing complete cleft glans. Points B and B' suggest where the glanular wings fuse in normal glans
Fig. 6.5 Distal hypospadias with complete cleft glans. A single stitch at the point of curvature results in approximation
of the glanular wings and a near normal-looking glans (©Ahmed T. Hadidi 2022. All Rights Reserved)
Fig. 6.6 A child with glanular hypospadias showing meatus is mobile and can be pulled to the tip of the glans
grooved glans plate and glanular meatus. The bridge sug- (see Chap. 19: Double Y Glanulomeatoplasty (DYG)
gests failure of fusion of the migrating meatus with the (©Ahmed T. Hadidi 2022. All Rights Reserved)
grooved glans. A toothed forceps would confirm that the
The incidence of small glans size was 36% in fibrous ring that may present with difficulty to
glanular hypospadias, 36% in distal hypospadias, pass urine and needs to be incised during the
57% in proximal hypospadias, and 94% in peri- urethroplasty.
neal hypospadias. 50% of patients with proximal Van der Putte described the histology of the
and perineal hypospadias had DVL less than urethral meatus as similar to that of the urethra,
12 mm and VVL less than 6 mm and were associ- i.e. pseudo-stratified columnar epithelium [7].
ated with less satisfactory cosmetic and func- Often, there may be several openings
tional results (Fig. 6.7). (Fig. 6.12). They may course deep to the urethral
channel for a short distance. They are blind end-
ing and do not communicate in any way with the
6.3 The Meatus urinary stream. Embryologically, these accessory
urethras represent “aborted vacuolations” that
The meatus in hypospadias may present in a vari- would normally form urethral groove. The pres-
ety of configurations in form, diameter and rigid- ence of distal opening may lead the parents and
ity. It can be fissured in transverse or longitudinal the inexperienced practitioner to think that the
directions or can be covered with delicate skin at hypospadias is more distal that it really is or that
times. The more distal locations in particular are the child has two urethrae.
often associated with stenotic meatus.
The common shape of meatus in hypospadias
General Rule: The most proximal orifice is
is the rounded or oval form (Fig. 6.8). In some
the actual urethral orifice connected to the
patients, the meatus may look like a longitudinal
bladder.
fissure but in fact it is circular at the proximal end
of the fissure (Figs. 6.9 and 6.10). Avellan judged
the meatus to be clinically adequate in 50/220
patients [3]. 6.4 The Urethral Plate
The other common type is the pinpoint type of
meatus (Fig. 6.11). The pinpoint opening may be The urethral plate is discussed in detail in Chap.
surrounded by a soft yielding tissue that dilates 8. The urethral plate may be wide and health, nar-
during micturition or may be surrounded by a row or even completely absent (Fig. 6.13).
142 A. T. Hadidi
Fig. 6.7 The dorsal

vertical length (DVL) was
usually equal to the glans
width (GW), average 14
mm, the ventral vertical
length (VVL) was usually
half of the dorsal vertical
length (DVL) or glans
width (GW), average
7 mm (©Ahmed T. Hadidi
Fig. 6.8 The urethral meatus in hypospadias is usually rounded or oval (©Ahmed T. Hadidi 2022. All Rights Reserved)
Fig. 6.10 The urethral meatus is rarely longitudinal

Fig. 6.9 The urethral meatus is rarely transverse
a b c
Fig. 6.11 Three patients with pinpoint meatus. (a) The S-shaped median raphe. (c) The patient has a pinpoint
patient has a midline pinpoint and a wide central median meatus to the right of midline and a narrow left lateral
raphe that bifurcates at the level of the meatus. (b) The raphe that bifurcated 1 cm proximal to the meatus
patient is an adult with a midline pinpoint meatus and (©Ahmed T. Hadidi 2022. All Rights Reserved)
a b
Fig. 6.12 (a) Patient has two orifices. (b) Patient has meatus connected to the bladder (©Ahmed T. Hadidi
multiple orifices. The distal orifices are blind. A general 2022. All Rights Reserved)
rule: The proximal orifice is always the true urethral
144 A. T. Hadidi
Fig. 6.13 The urethral plate may be wide, narrow, or even absent in hypospadias (©Ahmed T. Hadidi 2022. All Rights
Reserved)
6.5 The Prepuce reconstruction following hypospadias repair.

Attempts to excise the two knobs during the pre-
The prepuce forms the anatomical coverings of puce reconstruction may result in a short recon-
the glans penis and clitoris. It is a specialized, structed prepuce, and it is important to inform the
junctional mucocutaneous tissue which marks parents beforehand.
the boundary between mucosa and skin. It is sim- The prepuce itself may accentuate the degree
ilar to the eyelids, labia minora, and lips [8]. The of penile chordee. In many cases, the family doc-
normal anatomy of the prepuce is mentioned in tor reports the presence of penile chordee because
detail in Chap. 5. Embryologically, the prepuce of the appearance of prepuce and the presence of
develops as a result of proliferation of stromal a peculiar line of cleavage (Figs. 6.18 and 6.19)
structures (glanulo-preputial lamella), proximal Radojicic and Perovic classified the hypospa-
to the coronal sulcus and develops first dorsally diac prepuce according to morphology into six
and laterally. Later, it develops ventrally during groups: the Monk’s hood deformity (25%), the
the process of formation of the floor of the glanu- cobra eyes (50%), normal (MIP), flat, V-shaped,
lar urethra [5]. and the collar scarf subtypes. They suggested that
There are two main variants of the prepuce in the Monk’s hood and cobra eye deformity have
hypospadias. In 98% of hypospadiac patients, the the most favorable vascular pattern for flaps
prepuce is longer than normal dorsally and defi- while the flat and V-shaped have the most unfa-
cient ventrally (Figs. 6.14 and 6.15). vorable vascular pattern [9]. This is relevant in
There are two lateral edges that are fixed at the complex flap designs.
lateral borders of the ventral aspect of the pre-
puce. If one draws two lines along the two lateral
edges of the prepuce, this may give an idea about 6.6 The Proximal Penile Urethra
the area of underdevelopment of the urethra and
corpus spongiosum (Fig. 6.15). Posterior Urethral Valves: Routine cystoscopy
A common finding in many hypospadias was performed on 100 consecutive hypospadias
patients is two rounded knobs laterally on the patients in Mannheim-Heidelberg University.
dorsum of the prepuce (like two little eyes), com- Ten patients showed picture suggestive of poste-
monly called “cobra eyes,” “dog ears,” or rior urethral valves. Only two patients had symp-
“whirls”) (Figs. 6.16 and 6.17). These two little toms of urethral obstruction. Both patients had
knobs are visible on the prepuce after preputial distal hypospadias (Hadidi, personal communi-
Fig. 6.14 (a) The

a
classic form of prepuce
associated with
hypospadias. (b) The
prepuce is longer than
normal dorsally and
deficient ventrally and
the median raphe
bifurcates into two
branches just proximal
to the urethral meatus
(©Ahmed T. Hadidi
2022. All Rights
Reserved)
cations). Avellan 1980, and Ericsson and Rudhe Enlarged Utricle: In proximal and perineal
1957, showed similar incidence [3, 10] hypospadias, the surgeon may encounter diffi-
Accessory Urethra: Occasionally, it is difficult culty (proximal to the penoscrotal junction) to
to introduce a catheter through the penile urethra. pass a urinary catheter into the bladder because
This is usually due to the presence of prolapsing of an enlarged utricle (see Chap. 48: Large
orifice(s) of an accessory urethra. It is usually pos- Utricle). Sometimes, passing a thin catheter into
sible to pass the catheter in these instances by using the utricle may help to introduce the catheter into
a Tiemann catheter pressing it against the superfi- the bladder. Rarely, cystoscopy is needed to help
cial urethral wall to avoid the prolapsing orifice of passing a catheter into the bladder and not into
the accessory urethra (Fig. 6.20). the enlarged utricle
146 A. T. Hadidi
Fig. 6.15 A patient with proximal hypospadias. If one pus spongiosum, and ventral fascia (©Ahmed T. Hadidi
draws two lines along the branches of the raphe, this may 2022. All Rights Reserved)
give an idea about the level of disorganized urethra, cor-
rounding the normal proximal urethra is partially

developed (clinically evident by seeing the ure-
thra just under the skin) (Figs. 6.21 and 6.22),
and then it splays out in a V onto the lateral penile
shaft in a rudimentary fashion [11].
Distal to the hypospadiac urethral meatus,
there is abnormal corpus spongiosum [12–14].
Avellan interpreted the laterally penetrating
spongious tissue as underdeveloped or mal-
formed corpus spongiosum [3] (Fig. 6.23). Van
der Putte documented the histological findings of
the corpus spongiosum in embryos with different
grades of hypospadias and demonstrated that this
spongious tissue consists of irregular plexuses of
large veins that normally predominate between
Fig. 6.16 Two little eyes, “cobra eyes,” “dog ears,” or 11 and 13.5 weeks and connect all the primordial
“whirls” commonly seen on the dorsum of the abnormal erectile structures [7] (Figs. 6.24 and 6.25).
prepuce in hypospadias (©Ahmed T. Hadidi 2022. All Baskin suggested that there is increased vascular-
Rights Reserved) ity in the shaft and glans of a hypospadias penis
than in a normal penis [15]. Although these later-
6.7 Corpus Spongiosum ally projecting spongiosus tissues may be full of
blood sinusoids, they are often dysplastic and
The normal anatomy and histology of the cavern- unhealthy and may cause curvature or chordee.
ous spongiosum is mentioned in detail in Chap. Beaudoin et al. examined the pathologic anat-
4. In hypospadias, the corpus spongiosum sur- omy of corpus spongiosum (CS) in 51 cases of
a b
Fig. 6.17 Sagittal section through a “dog ear” on the pre- the difference between the dense fibrovascular stroma of
puce of a newborn baby with coronal hypospadias. In (a) the dog ear and the peculiar loose superficial connective
the low magnification shows the abnormally short pre- tissue of the shaft (3) (from van der Putte [7], reprinted
puce (1) with the dog ear (2), distal shaft (3), glans (4), from J Plast Reconstr Aesthetic Surg, Vol. 60, Issue 1,
and corpus cavernosum (5). In (b) a detail demonstrates pp48-60, © 2007, with permission from Elsevier)
Fig. 6.18 An unusual form of prepuce that aggravates the degree of chordee (©Ahmed T. Hadidi 2022. All Rights
Reserved)
hypospadias and highlighted its relation with Thiersch-Duplay technique to include the “bifur-
other structures and maldevelopment encoun- cated corpus spongiosum” in their repair
tered in hypospadias [16]. With extensive lateral (spongioplasty).
dissection, they could locate the corpus spongio-
sum in all their cases. However, it was always
abnormal in its distal part, diverging like a Y. In 6.8 Bulbospongiosus Muscle
30 cases, the divergence was just proximal to the
meatus, in 5 cases, 1–2 mm proximal to the The bulbospongiosus muscle fibers normally
meatus. In 16 patients, the divergence started at a diverge like the halves of a feather from the
distance proximal to meatus ranging between median raphe to encircle the bulb and corpus
5 mm to the full length of the penis. They attrib- spongiosum (see Chap. 4: Surgical Anatomy of
uted curvature and torsion to the pull of the limbs the Penis and Urethra). In severe forms of hypo-
of corpus spongiosum. They modified the spadias, the bulbospongiosus muscle may be
148 A. T. Hadidi
a b
c d
Fig. 6.19 (a) Bifurcated prepuce, (b) abnormal short pre- unite in the midline ventrally forming the floor of the
puce, (c) abnormal long prepuce, (d) open prepuce with- glanular prepuce and the complete prepuce (©Ahmed
out hypospadias or chordee, and (e) hypospadias with T. Hadidi 2022. All Rights Reserved)
deficient ventral prepuce that appears as if it is about to
Fig. 6.20 (a and b) Prolapsing orifice of lacuna of Morgagni causing difficulty to introduce a catheter into the urethra
Fig. 6.21 Two patients showing thin proximal urethra (zona pellucida) (©Ahmed T. Hadidi 2022. All Rights Reserved)
150 A. T. Hadidi
Fig. 6.22 Localized hypoplasia of corpus spongiosum (the surgeon may think that this is a fistula until he realizes
intraoperatively that the urethral epithelium is intact) (©Ahmed T. Hadidi 2022. All Rights Reserved)
a b c
Fig. 6.23 Abnormal corpus spongiosum: (a) Corpus spongiosum reaching the proximal third of penis, thin ure-
spongiosum bifurcating on either side of the thin urethra thra to the distal third of the penis in a distal hypospadias
in a patient with hypospadias sin hypospadias. (b) Absent (©Ahmed T. Hadidi 2022. All Rights Reserved)
corpus spongiosum in a glanular hypospadias. (c) Corpus
a b
Fig. 6.24 (a) Mid-scrotal hypospadias in a 15-week-old sum (from van der Putte [7], reprinted from J Plast
fetus showing hypoplastic bulbus spongiosum (6). (b) Reconstr Aesthetic Surg, Vol. 60, Issue 1, pp48-60, ©
Magnification showing hypoplasia of the bulbus spongio- 2007, with permission from Elsevier)
a b
a1 b1
Fig. 6.25 Abnormal corpus spongiosum. (a) Transverse plexuses of blood vessels and dense connective tissue
section through the proximal shaft in a normal fetus of 24 mass instead of a corpus spongiosum as seen in (a1) ((a
weeks. (b) Transverse section through the proximal shaft and b) from van der Putte [7], reprinted from J Plast
of a fetus of 32.5 weeks with penile hypospadias. (a1 and Reconstr Aesthetic Surg, Vol. 60, Issue 1, pp48-60, ©
b1) are schematic reproductions. The sections show in 2007, with permission from Elsevier, (a1 and b1) ©Ahmed
(b1) the lack of organization of the shaft with irregular T. Hadidi 2022. All Rights Reserved)
152 A. T. Hadidi
underdeveloped or absent. This may predispose 6.11 Size of the Penis

to the development of diverticulum after correc-
tion of scrotal or perineal hypospadias. The size of the penis is normal in the majority of
hypospadias patients. Patients with disorders of
sex development (DSD) are different category
6.9 he Penoscrotal (Median)
T and not discussed in this book on hypospadias.
Raphe A penis that measures 2–2.5 standard devia-
tions below the mean in stretched length for age
In hypospadias, the penoscrotal raphe usually is called a micropenis [17–19]. In newborns, a
(85%) begins at the perineal body and passes stretched length of less than 2 cm is considered
forward in the midline before it bifurcates just abnormal. Normal urethra and prepuce imply
proximal to urethral meatus into two branches normal first trimester development. Impaired
that end distally into what is called “dog ears.” penile growth usually occurs in the second and
The area between the two branches of the raphe third trimesters [20].
gives an idea about the defective development Hypoplasia of the penis may be associated
of the corpus spongiosum and ventral fascia with hypospadias. Fortunately, these rudimentary
(Fig. 6.26). penises are quite uncommon with hypospadias,
Avellan in his study on 220 hypospadias found and a very careful examination of the suprapubic
straight raphe in 50% of patients with hypospa- fat pad will usually determine a respectable shaft
dias [3]. The raphe was deviant in 40% (Fig. 6.26) buried beneath the skin (see Chap. 45: Buried
and absent raphe in 10% of patients. Penis). Avellan found penile hypoplasia in 3% of
In hypospadias with torsion (15% of hypospa- his patients, half of which had associated chro-
dias), one branch of raphe is longer than the other mosomal anomalies [3].
(Fig. 6.27). It is assumed that distal migration on Some parents (especially in grade III and IV
one side may occur more than the other side of hypospadias) have unrealistic expectations and
the penis resulting in torsion. imagine as if the surgeon is going to replace the
Abnormal raphe may be the only visible indi- child’s penis with a nice big normal penis. It is
cation of maldevelopment of the urethra and the important to make it clear to the parents that
ventral surface of the penis. In some patients, this hypospadias surgery will correct the position of
may be associated with abnormal urethral open- the urethral meatus, should try to make the penis
ing or abnormal intra-urethral valves or rotation straight, and hence a bit longer but will not make
of the penis (Fig. 6.28). the penis bigger.
6.10 Penile Torsion 6.12 The Scrotum
Torsion may occur with or without hypospadias. Normally, the genital tubercle develops cranial to
Interestingly, it is usually anticlockwise (glans the two genital swellings. The penis may be
faces left). Torsion is almost always associated caught between the two scrotal halves and
with abnormal raphe. It is assumed that the become engulfed with fusion of the penoscrotal
abnormal distal migration of the raphe is proba- area causing penoscrotal transposition (Figs. 6.30
bly responsible for the torsion. Rarely, one may and 6.31).
see torsion clockwise and the glans faces right In complete penoscrotal transposition, the
(Fig. 6.29). Penile torsion is discussed in more whole scrotum is located superior to the penile
detail in Chap. 46. shaft, and the scrotal skin may surround the root
Fig. 6.26 Different presentation of abnormal raphe in hypospadias (©Ahmed T. Hadidi 2022. All Rights Reserved)
154 A. T. Hadidi
a b
a1 b1
Fig. 6.27 (a) Normal fetus of 24 weeks. (b) Fetus of 18.5 vessels ((a and b) from van der Putte [7], reprinted from J
weeks’ fetus with proximal penile hypospadias, ventral Plast Reconstr Aesthetic Surg, Vol. 60, Issue 1, pp48-60,
curvature, and torsion. (a1 and b1) are side schematic © 2007, with permission from Elsevier, (a1 and b1)
reproductions. The section in (b) lack of organization in ©Ahmed T. Hadidi 2022. All Rights Reserved)
the shaft with asymmetrical irregular plexuses of blood
of the penis completely. In partial penoscrotal complete separation of otherwise normally posi-
transposition, the scrotum covers two thirds or tioned hemiscrota in patients with severe
part of the root of the penis. The boundary hypospadias or chordee resulting in partially

between the penis and the scrotum may be formed bifid or completely bifid scrotum.
by two oblique raphes that extend from the very Bifid scrotum though rare in hypospadias in
proximal meatus to the dorsal side of the penis. general is often seen in severe forms of hypospa-
Penoscrotal transposition may rarely occur dias or grade IV (less than 10%). This can present
without hypospadias (Fig. 6.31). Avellan reported as a phenotype of the partial androgen insensitiv-
different degrees of penoscrotal transposition in ity syndrome (Fig. 6.32). This syndrome occurs
20% of his group of patients [3]. in various degrees and is caused by mutations in
In the majority of hypospadias patients, the the androgen receptor gene. This gene is located
scrotum is normal (90%). Incomplete fusion of on the X chromosome, and mutations are trans-
the labioscrotal folds may produce a partial or mitted in an X-linked recessive manner.
Fig. 6.28 An abnormal median raphe may be the only the foreskin, one can see that the child had a megameatus
external indication of an abnormal development of the intact prepuce anomaly (MIP) (©Ahmed T. Hadidi 2022.
penis. The child had an abnormal raphe. On retraction of All Rights Reserved)
6.13 Anoscrotal Distance (ASD) scrotum. They concluded that boys with severe
hypospadias have shorter AGD and ASD.
Hsieh et al. in 2012 measured the AGD (anogeni- Wirmer et al. in 2021 on the other hand stud-
tal distance) in 119 Caucasian boys ranging in ied the relation between the severity of chordee,
age from 4 to 86 months. The study included 42 different grades of hypospadias, and the anoscro-
normal and 77 boys with hypospadias [21]. The tal distance (ASD) [23] (Fig. 6.33). They classi-
mean (+SD) AGD of boys with hypospadias was fied hypospadias into four grades: grade I or
67+1.2 versus 73+1 mm for boys with normal glanular, grade II or distal, grade III or proximal,
genitals (P < 0.002). In these age-unmatched and grade IV or perineal hypospadias. They mea-
patient groups, there were also differences in age, sured the anoscrotal distance from the anal verge
height, and weight (P < 0.002, 0.0001, 0.0002, to the beginning of scrotal skin. They classified
and 0.0004, respectively). After age matching, the severity of chordee into three categories: no
boys with hypospadias still featured a shorter chordee, mild chordee (or less than 30°), and
AGD than boys with normal genitals. severe chordee or more than 30°. They concluded
Cox et al. in 2017 studied the relation between that patients with ASD of 2 cm or less are likely
the severity of hypospadias and AGD and ASD in to have severe chordee (more than 30°) that
52 boys with hypospadias [22]. They classified would require division of the urethral plate.
hypospadias into group I or mild forms including
glanular, subcoronal, and distal hypospadias and
group II or severe forms including mid penile, 6.14 Suprapubic Fat
penoscrotal, and perineal hypospadias. They
defined the AGD as the distance from the center The majority of patients with distal forms of
of the anus to the base of the penis and the ASD hypospadias have normal fat distribution.
from the center of the anus to the junction Occasionally, patients with proximal forms of
between the smooth perineal skin and skin of the hypospadias may have exaggerated suprapubic
156 A. T. Hadidi
a b
c d
Fig. 6.29 Torsion of the penis is usually associated with abnormal raphe. Torsion is usually toward the left (a, b, c).
Rarely, it faces right (d) (©Ahmed T. Hadidi 2022. All Rights Reserved)
Fig. 6.30 Penoscrotal transposition associated with proximal hypospadias and chordee (©Ahmed T. Hadidi 2022. All
Rights Reserved)
fat that makes the penis smaller than it actually is, 6.16 The Prostate
similar to buried penis. This is usually a major
concern of parents. Enlarged prostatic utricle is a Müllerian duct
remnant, which is not uncommon with severe
forms of hypospadias [26] (Fig. 6.35). Devine
6.15 The Testis and Shima et al. found an enlarged prostatic utri-
cle in about 11% of the patients with severe
The majority of hypospadias patients have nor- hypospadias [27, 28]. These generally cause little
mal testes in the scrotum. Retractile testes or problem except when a catheter is passed into the
undescended testis may be encountered in 10% bladder and the tip is deflected into the utricle.
of patients with hypospadias usually in proximal Removal of prostatic utricle is rarely necessary in
forms [3, 24, 25] (Fig. 6.34). hypospadias surgery, nor do they cause inconti-
Patients with hypospadias associated with nence or lead to infection. Utricular enlargement
undescended testis should be thoroughly investi- in itself does not indicate intersexuality but may
gated, and chromosomal and hormonal analysis reflect the lack of hormone effect on the urogeni-
and abdominal ultrasound should be performed tal sinus [27]. When removal is indicated, lapa-
to exclude chromosomal anomalies and disorders roscopy or transtrigonal approach may be used
of sex differentiation (DSD). Disorders of sexual (see Chap. 48: Large Utricle).
development is a different category beyond the Avellan reported one perineal hypospadias
scope of this book. patient with underdevelopment of the prostate in
Avellan reported a 15-year-old boy with penile his series [3]. Vasovesiculography showed the
hypospadias, normally developed scrotum and vesicles and ejaculatory ducts longer than normal
prostate, and without testes (bilateral anorchia) and the latter discharged directly into the urethra
[3]. Chromosomal analysis showed normal male which lacked the prostatic colliculus (verumon-
constitution, and urine gonadotropins were ele- tanum). The patients had normal spermatogene-
vated above 96 MU. Bilateral anorchism was sis verified by aspiration biopsy of the testes, but
established after laparotomy. Abdominal a function anomaly in the form of retrograde
aortography revealed bilateral absence of sper- ejaculation was verified by analysis of a two-
matic arteries. glass urine sample after ejaculation.
158 A. T. Hadidi
Fig. 6.31 Two patients with complete penoscrotal transposition without hypospadias (©Ahmed T. Hadidi 2022. All
Rights Reserved)
Fig. 6.32 Different degrees of bifid scrotum that may be associated with penoscrotal transposition (©Ahmed T. Hadidi
a b c
d e
Fig. 6.33 Anoscrotal distance (ASD). (a) glanular hypo- that 3 cm is about 2 finger breadth. (d) Proximal hypospa-
ASD. (c) Mid-penile hypospadias with 3 cm ASD. Notice ASD (©Ahmed T. Hadidi 2022. All Rights Reserved)
160 A. T. Hadidi
a b c
Fig. 6.34 Other anomalies that may be associated with hypospadias. (c) Potter syndrome; minor ear malforma-
hypospadias. (a) Right undescended testis in grade IV tions may be associated with urinary malformations
hypospadias. (b) High imperforate anus associated with (©Ahmed T. Hadidi 2022. All Rights Reserved)
6.17 Other Associated Anomalies
Hydrocele was observed in less than 5% and

inguinal hernia in 3% of hypospadias in Avellan
series. Mild varicocele was found in three patients
(age 11 to 27 years) in Avellan series of 220
hypospadias [3].
Imperforate anus may be associated with
grade IV hypospadias (rare) (Fig. 6.34b).
The majority of hypospadias patients have no
other urological anomalies. Rarely, there may be
vesico-ureteric reflux, double ureter, double renal
pelvis, single kidney, and ectopic kidney.
The online “Mendelian Inheritance in Man”
database lists 50 syndromes in which hypospa-
dias may be a feature and the “London
Dysmorphology Database” lists 186 such syn-
Fig. 6.35 A 12-month-old baby with perineal hypospa- dromes (see Chap. 11: Genetics of Hypospadias).
dias and large prostatic utricle. Urinary catheter entered Single malformations that may be present
the prostatic utricle instead of the bladder. The child pre- include different forms of cleft lip and palate,
sented with meatal stenosis and recurrent urinary tract
hydrocephalus, congenital megacolon, imperforate
infection after the correction of perineal hypospadias. The
urinary tract infection disappeared after correction of the anus, ventricular septal defects, and anomalies of
meatal stenosis (©Ahmed T. Hadidi 2022. All Rights the hand and feet in the form of polydactyly, syn-
Reserved) dactyly, and hypoplasia of the thumb and fingers.
References 16. Beaudoin S, Delaage PH, Bargy F. Anatomical basis

of surgical repair of hypospadias by spongioplasty.
Surg Radiol Anat. 2000; https://doi.org/10.1007/
1. van der Meulen JC. Hypospadias and cryptospa-
s00276-000-0139-7.
dias. Br J Plast Surg. 1971; https://doi.org/10.1016/
17. Feldman KW, Smith DW. Fetal phallic growth
S0007-1226(71)80020-6.
and penile standards for newborn male infants.
2. Kaufmann C. Verletzungen und Krankheiten der män-
J Pediatr. 1975; https://doi.org/10.1016/
nlichen Harnröhre und des Penis. Stuttgart: Deutsche
S0022-3476(75)80969-3.
Chirurgie; 1886.
18. Lee PA, Mazur T, Danish R, Amrhein J, Blizzard
3. Avellán L. Morphology of hypospadias. Scand J
RM, Money J, Migeon CJ. Micropenis. I. Criteria,
Plast Reconstr Surg Hand Surg. 1980; https://doi.
etiologies and classification. Johns Hopkins. Med. J.
org/10.3109/02844318009106717.
1980;146
4. Hadidi AT. Classification of hypospa-
19. Aaronson IA. Micropenis: medical and surgical
dias. Hypospadias Surg. 2004; https://doi.
implications. J Urol. 1994; https://doi.org/10.1016/
org/10.1007/978-3-662-07841-9_7.
S0022-5347(17)32804-5.
5. Hunter R. Notes on the development of the prepuce. J
20. MacDonald M, Barthold J, Kass E. Abnormalities
Anat. 1935;70(Pt 1):68–75.
of the penis and scrotum. Kelalis-King-Belman
6. Sennert M, Wirmer J, Hadidi AT. Preoperative glans
Textb Clin Pediatr Urol Fifth Ed. 2006; https://doi.
and penile dimensions in different hypospadias
org/10.1201/b13795-79.
grades. J Pediatr Urol. 2021. https://doi.org/10.1016/j.
21. Hsieh MH, Eisenberg ML, Hittelman AB, Wilson
jpurol.2021.09.020
JM, Tasian GE, Baskin LS. Caucasian male infants
7. van der Putte SCJ. Hypospadias and associated
and boys with hypospadias exhibit reduced anogenital
penile anomalies: a histopathological study and a
distance. Hum Reprod. 2012; https://doi.org/10.1093/
reconstruction of the pathogenesis. J Plast Reconstr
humrep/des087.
Aesthetic Surg. 2007; https://doi.org/10.1016/j.
22. Cox K, Kyriakou A, Amjad B, O’Toole S, Flett
bjps.2006.05.020.
ME, Welsh M, Ahmed SF, Cascio S. Shorter ano-
8. Cold CJ, Taylor JR. The prepuce. BJU Int.
genital and anoscrotal distances correlate with
1999;83(Suppl 1):34–44.
the severity of hypospadias: a prospective study.
9. Radojicic ZI, Perovic SV. Classification of pre-
J Pediatr Urol. 2017; https://doi.org/10.1016/j.
puce in hypospadias according to morphologi-
jpurol.2016.08.006.
cal abnormalities and their impact on hypospadias
23. Wirmer J, Sennert M, Hadidi AT. Ano-Scrotal
repair. J Urol. 2004; https://doi.org/10.1097/01.
Distance (ASD) Is it a marker for the severity of chor-
ju.0000129008.31212.3d.
dee? J Pediatr Urol. 2021; https://doi.org/10.1016/j.
10. Kjellberg SR, Ericsson NO, Rudhe U. The Lower uri-
jpurol.2021.06.010.
nary tract in childhood. Br J Urol. 1958; https://doi.
24. Kennedy PA. Hypospadias: a twenty year review
org/10.1111/j.1464-410x.1958.tb06239.x.
of 489 cases. J Urol. 1961; https://doi.org/10.1016/
11. Duckett JW, Baskin LS. Hypospadias. In: Gillenwater
S0022-5347(17)65432-6.
JY, Grayhack JT, Howards SS, Duckett JW, editors.
25. Barcat J, Stephan JC. L’hypospadias: Étude clinique
Adult and pediatric urology. 3rd edit. ed. St. Louis,
et thérapeutique; à propos de 140 nouveaux cas. J
MO: Mosby-Year Book; 1996. p. 2549–89.
Chir. 1961;81:551–80.
12. Marshall M, Beh WP, Johnson SH, Price SE,
26. Howard FS. Hypospadias with enlargement of the pros-
Barnhouse DH. Etiologic considerations in peno-
tatic utricle. Surg Gynecol Obstet. 1948;86:307–16.
scrotal hypospadias repair. J Urol. 1978; https://doi.
27. Devine CJ. Embryology of the male external genita-
org/10.1016/S0022-5347(17)57120-7.
lia. Clin Plast Surg. 1980;7:141–8.
13. Smith DR. Hypospadias: its anatomic and therapeutic
28. Shima H, Ikoma F, Terakawa T, Satoh Y, Nagata
considerations. J Int Coll Surg. 1955;24:64–71.
H, Shimada K, Nagano S. Developmental
14. Williams DI. The development and abnormalities of
anomalies associated with hypospadias. J Urol.
the penile urethra. Acta Anat (Basel). 1952; https://
1979;122:619.
doi.org/10.1159/000140742.
studies of hypospadias. J Urol. 1998; https://doi.
org/10.1016/S0022-5347(01)62711-3.
Pathogenesis of Hypospadias: The
Disorganization Hypothesis 7
Ahmed T. Hadidi
Abbreviations troversy and confusion regarding the pathogene-

sis of hypospadias.
ASD Anoscrotal distance This chapter begins with a brief description of
MIP Megameatus intact prepuce the normal penis and urethral development (“the
migration hypothesis”). This is followed by
explaining in brief how “disorganized” develop-
ment may result in the different morphological
7.1 Introduction and histological findings in hypospadias. The
morphological findings came from clinical expe-
Classic theories suggest that hypospadias results rience with more than 8000 hypospadias patients.
from “an arrest in normal development of the ure- The histological findings are from the study and
thra, foreskin and ventral aspect of the penis” [1]. histological sections of 15 human embryos
Others state that “hypospadias results from par- (6–14 weeks of gestation) [10] and 5 human
tial or complete failure of urethral folds to form fetuses and neonates with hypospadias [9] and
throughout their normal length or a failure of the other histological studies available in literature.
fold to close distally if they have already formed” These morphological and histological findings
[2]. These theories cannot explain the variable provide the basis of the “disorganization hypoth-
pattern and depth of the urethral plate, different esis” to explain the pathogenesis of hypospadias
glans configuration, chordee, or torsion. and related anomalies such as short ASD, chor-
Hypospadias is rarely associated with lethal dee, torsion, and penoscrotal transposition.
anomalies of the heart, brain, or kidneys.
Available resources are scarce and histological
sections of hypospadias, available in literature, 7.2 Normal Urethral
were mostly obtained from biopsies during oper- Development (The
ative correction of hypospadias [3–6]. Specimens “Migration Hypothesis”)
of complete hypospadiac penises were rarely
available [7–10]. This explains the current con- The urethral plate appears as an outgrowth from
the anterior walls of the urogenital sinus. This
endodermal solid structure with the underlying
A. T. Hadidi (*) stroma seems to be responsible for urethral devel-
Department, Sana Klinikum Offenbach, Academic opment. It extends from the perineum to the tip of
Teaching Hospital of the Johann Wolfgang Goethe the glans. It passes through the stage of solid
University, Offenbach, Frankfurt, Germany structure, and then vacuolation occurs to form
164 A. T. Hadidi
deep urethral groove. The urethral orifice starts as The solid glans plate is the precursor of the
a longitudinal slit or oval opening (delta) at the glanular urethra and can be seen in histological
perineum, near the anal orifice. The ventral sections of embryos as early as 6 weeks’ gesta-
stroma in the perineum, in front of the anus, plays tional age. The central part of the glans plate grad-
a very important role in the development of the ually becomes vacuolated to form a blind canal,
penile urethra. There is differential growth and and the floor disintegrates and becomes a deep
proliferation of the penile mesenchyme in the groove to form the roof and lateral wall of the
normal development of the penis and urethra. glanular urethra (Fig. 7.3). Failure of grooving of
First, there is dorsal growth and proliferation the glans plate may explain the different clinical
causing “physiological chordee.” Later, there is presentations of the glans in hypospadias.
forward distal migration of the posterior edge of The frequent clinical presentation of a bridge
urethra to reach the tip of the penis resulting in at the junction between the proximal penile ure-
the formation of the floor of the proximal part of thra and glanular urethra (Figs. 7.4 and 7.5)
the penile urethra. The glanular urethra develop- strongly supports concept that the development
ment passes through four stages, a solid plate, a of the glanular urethra is entirely different than
blind canal, and deep groove, thus forming the that of the proximal penile urethra.
roof and lateral walls of the glanular urethra The patient in Fig. 7.6 shows a rare mild form
(pseudo-stratified columnar epithelium). The of hypospadias where the proximal urethra has
floor of the glanular urethra is formed by the dis- terminated to the right of the glanular urethra. In
tal migration of the prepuce (stratified squamous addition, the median raphe is not actually median
epithelium). The penile urethra and glanular ure- but “Z” in shape and ends to the left of the mid-
thra fuse at the fossa navicularis (Fig. 7.1) (see line (1 cm to the left from the formed proximal
Chap. 3: Embryology). penile urethra). This signifies that the glanular
urethra develops separately and has a different
developmental mechanism than the proximal
7.3 The Glans penile urethra. In this patient, the median raphe
lies away from the urethra and confirms that the
Patients with hypospadias have an abnormal- median raphe is not formed due to fusion of the
looking, globular glans with a variable degree of ectodermal edge of the urethral folds as previ-
clefting (Fig. 7.2). The glans appearance has ously described in literature [1].
been classified as cleft glans, incomplete cleft, or Liu et al. in 2018 [12, 13] and Baskin et al.
flat glans [11]. [14, 15] suggested (inaccurately) that the glanu-
Fig. 7.1 The migration hypothesis showing the crucial developing urethra. The prepuce plays an important role
role of ventral penile mesenchyme in the development of in the development of the floor of the glanular urethra.
the penis, urethra, and penile fascia. Differential growth Superficial stroma forms dartos fascia which continues to
and distal migration of the ventral fascia brings the edges grow into the prepuce (©Ahmed T. Hadidi 2022. All
of the urethral groove together which fuse to form the Rights Reserved)
penile urethra. The corpus spongiosum grows around the
7 Pathogenesis of Hypospadias: The Disorganization Hypothesis 165
a b c d
Fig. 7.2 (a–d) Children with different forms of distal the different stages of glanular urethral development
hypospadias and different grades of preputial develop- (©Ahmed T. Hadidi 2022. All Rights Reserved)
ment and depth of the glanular groove that go inside with
a b c
Fig. 7.3 Cut sections through the glans penis showing (c) Complete disappearance of the floor of the glanular
the first three stages of glanular urethra development as canal at the level of the proximal glans turning the glanu-
one proceeds from distal to proximal. (a) A solid epithe- lar canal into a deep glanular groove (©Ahmed T. Hadidi
lial plate at the tip of the glans. (b) A vacuolated epithelial 2022. All Rights Reserved)
plate forming a glanular canal at the level of distal glans.
lar urethra passes through the two stages of a deep glanular groove is documented in Hunter
solid plate that canalizes into a “stand alone ure- [16], Baskin et al. [8], and Hadidi [10] histologi-
thral canal” without further development into a cal sections of the human embryos. Liu et al. sug-
groove that will become a tube. The third stage of gestion does not explain the development of the
166 A. T. Hadidi
a b
Fig. 7.4 (a and b) The bridge between the proximal penile urethra and the grooved glanular urethra suggest different
developmental mechanisms (©Ahmed T. Hadidi 2022. All Rights Reserved)
a b
Fig. 7.5 Two different patients with glanular hypospa- is separated from the penile urethra by a wall or some-
dias. (a) The glanular urethra remained in stage 2 of times by a bridge (©Ahmed T. Hadidi 2022. All Rights
development (canal) that is separated from the penile ure- Reserved)
thra by a bridge. (b) The glanular urethra is different and
frenulum and the continuity of glanular urethra

and the prepuce at the region of the frenulum. If 7.4 Penile Urethra
Liu et al. suggestions were correct, we would
only see either patients with flat glans (solid glan- Kaufmann classified hypospadias into three
ular plate) or with a glanular urethral canal not grades: grade I, grade II, and grade III [17].
connected to the penile urethra. This is not the Hypospadias is currently classified into four
case and most patients with hypospadias have grades: grade I or glanular, grade II or distal,
some degree of glanular grooving. grade III or proximal without deep chordee, and
a b
Fig. 7.6 (a and b) The penile urethra ends to the right of the glanular groove. The median raphe is irregular and ends
to the left of the glanular groove (©Ahmed T. Hadidi 2022. All Rights Reserved).
grade IV or perineal hypospadias or with deep dias or hypospadias sine hypospadias

severe chordee (Fig. 7.7). In other words the (Fig. 7.11).
grade 3 in Kaufmann’s classification is now Chordee is discussed in full detail in Chap. 8.
divided into proximal without severe chordee and
proximal and perineal with severe chordee.
7.7 The Corpus Spongiosum
7.5 The Urethral Plate The corpus spongiosum develops from two small
oval bulbus spongiosa on either side of the primi-
The urethral plate may be wide, narrow, or even tive spongy urethra (see Chap. 6: Morphology).
absent in hypospadias, depending on the degree They approach each other behind the urethra to
of grooving of the original solid urethral plate form the bulb of the penis. Distally, the bulb
(Fig. 7.8). The urethral plate is discussed in detail grows and surrounds the proliferating spongy
in Chap. 8. urethra. Histologically, the corpus spongiosum
consists of regular spongy longitudinal cavernous
sinuses.
7.6 Chordee In most patients with hypospadias, the corpus
spongiosum is abnormal and incompletely devel-
Chordee may be explained to be due to arrested oped. It usually presents as an underdeveloped
disorganized distal migration of the mesenchyme laterally projecting irregular spongious tissue
(including corpus spongiosum) (Figs. 7.9 and dorsal to and on either side of the urethral plate
7.10). (Fig. 7.12) [18–21]. This abnormal development
Superficial and deep chordee may occur of the corpus spongiosum is present to a variable
together. distance proximal to the urethral orifice and may
Another variant is when the arrested disorga- present as a thin urethra under the skin (urethra
nized distal migration may involve the fascia but pellucida) (Fig. 7.13).
with a normal urethral meatus at the tip of the Histological sections showed that the corpus
glans, the so-called chordee without hypospa- spongiosum consists of irregular vascular plex-
168 A. T. Hadidi
Fig. 7.7 The different grade and severity of hypospadias may be explained by arrested distal migration and disorgani-
zation during the different stages of urethral development (©Ahmed T. Hadidi 2022. All Rights Reserved)
a b c
Fig. 7.8 Different presentations and development of the plastic that it must be divided and perhaps excised to bring
urethral plate. (a) The urethral plate may be well devel- healthy vascular tissue to reconstruct healthy neo-urethra.
oped and wide that simple tubularization may be enough. (c) In some cases, the urethral plate may be absent com-
(b) The urethral plate is narrow, disorganized, and hypo- pletely (©Ahmed T. Hadidi 2022. All Rights Reserved)
Fig. 7.9 The “migration hypothesis”: the urethral meatus tal migration resulting in distal and proximal hypospadias
starts at the perineum and migrates gradually until it with variable degrees of curvature (©Ahmed T. Hadidi
reaches the tip of the penis. According to the “disorgani- 2022. All Rights Reserved)
zation hypothesis,” there is this disorganized arrested dis-
a b c
Fig. 7.10 Currently, from a surgical perspective, chordee meatus. (c) Chordee with corporeal disproportion: when
is classified into three categories: (a) Superficial chordee: the chordee is not corrected early enough, the inner circu-
when the arrested disorganized distal migration involves lar layer of the tunica albuginea and corpora cavernosa
tissues superficial and proximal to the urethral meatus. (b) will be involved as well (©Ahmed T. Hadidi 2022. All
Deep chordee: when the arrested disorganized distal Rights Reserved)
migration involves tissues deep and distal to the urethral
170 A. T. Hadidi
Fig. 7.11 Chordee without hypospadias (©Ahmed T. Hadidi 2022. All Rights Reserved)
a b c
Fig. 7.12 The corpus spongiosum is abnormal in most of the penis, the urethral wall becomes gradually thinner
hypospadias patients, even in mild forms. (a) A child with till the end of the urethra. (c) A child with distal hypospa-
hypospadias sine hypospadias; although the meatus looks dias, where the corpus spongiosum ends at the proximal
normal, the corpus spongiosum is bifurcated on either third, the urethra ends at the middle third of the penis
side of the midline. (b) A child with glanular hypospadias (©Ahmed T. Hadidi 2022. All Rights Reserved)
where the corpus spongiosum ends at the proximal third
Fig. 7.13 In some patients, the corpus spongiosum is not developed and the urethral mucosa may lie directly under the
skin (urethra pellucida) (©Ahmed T. Hadidi 2022. All Rights Reserved)
uses between the corpus cavernosum and the ure-

thra (or urethral plate distally) (Fig. 7.14). They 7.9 The Prepuce and Frenulum
may represent bilateral vascular plexuses that
temporarily predominate in normal fetuses Embryologically, the prepuce develops mainly
between 11 and 13.5 weeks (40–70 mm) and from the shaft with a minor component from the
connect all the developing erectile tissues of the glans stroma at the inner side. The solid glanulo-
penis [9]. preputial lamella grows over the surface of the
These irregular vascular plexuses between the glans penis from outside. The median ventral part
urethral plate and corpora cavernosa represent an of the prepuce and frenulum have a different ori-
atretic, abnormal corpus spongiosum and have gin. It is due to rapid forward (distal) prolifera-
been interpreted as “highly vascular urethral tion of the median raphe. In the meantime, the
plate” in some publications [3, 8]. medial ventral part of the prepuce grows more
than the dorsal part. This results in the formation
of a normal complete prepuce, see Chaps. 3 and 5
7.8 The Corpus Cavernosum (Fig. 7.17).
In hypospadias, the median raphe is underde-
In most patients with hypospadias and contrary veloped. It begins at the perineal body and passes
to the corpus spongiosum and other ventral penile forward in the midline before it bifurcates into
structures, the dorsal penile structures and cor- two branches just proximal to the hypospadias
pora cavernosa develop normally. However, in orifice. It does not fuse with the glanular wings to
some instances and particularly if the hypospa- form the floor of the glanular urethra, resulting in
dias is not corrected early in life, the tunica albu- hypospadias. Neither does it fuse with the glanulo-
ginea covering the ventral surface of the corpora preputial lamella to complete the formation of the
cavernosa may be shorter than that of the dorsal prepuce. This results in the ventral deficiency of
surface, necessitating incision and grafting of the the prepuce in most patients with hypospadias.
tunica albuginea. Very rarely, the two corpora The raphe is deviant (in different forms) or absent
cavernosa may be unequal in size (Figs. 7.15 and in about 50% of patients with hypospadias, see
7.16) [9]. Chap. 6: Morphology (Figs. 7.18 and 7.19).
172 A. T. Hadidi
a b
a1 b1
Fig. 7.14 Abnormal corpus spongiosum. (a) Transverse plexuses of blood vessels and dense connective tissue
section through the proximal shaft in a normal fetus of mass instead of a corpus spongiosum as seen in (a1) ((a
24 weeks. (b) Transverse section through the proximal and b) from van der Putte [7], reprinted from J Plast
shaft of a fetus of 32.5 weeks with penile hypospadias. (a1 Reconstr Aesthetic Surg, Vol. 60, Issue 1, pp48-60, ©
and b1) are schematic reproductions. The sections show 2007, with permission from Elsevier, (a1 and b1) ©Ahmed
in (b1) the lack of organization of the shaft with irregular T. Hadidi 2022. All Rights Reserved)
a b
Fig. 7.15 (a) Perineal hypospadias in a 15-week-old sum (from van der Putte [9], reprinted from J Plast
fetus showing hypoplastic bulbus spongiosum (6). (b) Reconstr Aesthetic Surg, Vol. 60, Issue 1, pp48-60, ©
Magnification showing hypoplasia of the bulbus spongio- 2007, with permission from Elsevier)
Fig. 7.16 Severe chordee with corporeal disproportion. correct the chordee. Notice kinking and angle present at
Complete degloving of the penis and division and exci- the level of tunica albuginea and corpus cavernosum
sion of the hypoplastic disorganized urethral plate did not (©Ahmed T. Hadidi 2022. All Rights Reserved)
a b
Fig. 7.17 (a) Normal boys have a complete prepuce. (b) In the majority of patients with hypospadias, the prepuce is
incomplete. It is longer than normal dorsally and deficient ventrally (©Ahmed T. Hadidi 2022. All Rights Reserved)
Fig. 7.18 Incomplete prepuce in hypospadias with the urethral delta in the middle, between the bifurcated raphe and
dog ears (©Ahmed T. Hadidi 2022. All Rights Reserved)
174 A. T. Hadidi
Fig. 7.19 The prepuce in hypospadias is usually incom- abnormal fashion with the ventral penile skin (©Ahmed
plete ventrally and two dog ears are usually seen. The pre- T. Hadidi 2022. All Rights Reserved)
puce distal to the dog ears bends ventrally and fuses in an
a b
Fig. 7.20 A sagittal section through a protuberance fibrovascular stroma of the “dog ear” and the characteris-
(“dog ear”) on the prepuce of a newborn baby with coro- tically loose superficial connective tissue of the shaft (3)
nal hypospadias. In (a) the low magnification shows the (from van der Putte [9], reprinted from J Plast Reconstr
abnormally short prepuce (1) with the “dog ear” (2); distal Aesthetic Surg, Vol. 60, Issue 1, pp48-60, © 2007, with
shaft (3); glans (4); and corpus cavernosum (5). In (b) a permission from Elsevier)
detail demonstrates the difference between the dense
The presence of dog ears on the dorsum of the prepuce has been bent ventrally and fused with
deficient prepuce cannot be explained by failure the ventral penile skin to compensate for the dis-
of fusion or arrested growth. The presence of organized and deficient ventral penile skin
abnormal fusion line suggests that the dorsal (Fig. 7.20).
7.10 Median Raphe 7.11 Torsion and Rotation
Abnormal median raphe may be the only visible Torsion and rotation of the penis may occur with
external indication of abnormal development or without hypospadias (probably presenting a
(e.g., in megameatus intact prepuce or MIP). In mild degree of dyspadias), commonly with chor-
hypospadias, the median raphe is underdevel- dee but may occur without chordee. The torsion
oped. It begins at the perineal body and passes is usually counterclockwise with the glans facing
forward in the midline before it bifurcates into to the left. Rarely, one may see torsion in the
two branches just proximal to the hypospadias clockwise direction with the glans facing to the
orifice. The raphe is deviant (in different forms) right. It is always associated with a deformed
or absent in about 50% of patients with hypospa- raphe.
dias (Fig. 7.21). In hypospadias with torsion (15% of hypospa-
The two branches of the median raphe in dias), one branch of raphe is usually longer than
hypospadias terminate distally in two rounded the other (Figs. 7.22 and 7.23). The abnormal
knobs laterally on the dorsum of the prepuce (like development of the raphe is probably responsible
two little eyes, commonly called “cobra eyes,” for the torsion. Other contributing factors for tor-
“dog ears,” or “whirls”) (Fig. 7.19). These two sion are unequal development of penile fascia
little knobs are visible on the prepuce after prepu- and/or different shape and size of corpora
tial reconstruction following hypospadias repair. cavernosa.
Attempts to excise the two knobs during the pre-
puce reconstruction usually result in a very short
prepuce, and it is important to inform the parents 7.12 egameatus Intact Prepuce
M
beforehand. (MIP)
Histological examination of the median raphe
and its two branches revealed a highly vascular Megameatus intact prepuce (MIP) is a rare pecu-
stroma and stratified squamous epithelium [9] liar form of hypospadias occurring in less than
(Fig. 7.20). 2% of hypospadias. It is characterized by a glan-
Fig. 7.21 The median raphe may be the first indication of abnormal development of the penis. The raphe may be
absent, deviated, or in the form of zigzag (©Ahmed T. Hadidi 2022. All Rights Reserved)
176 A. T. Hadidi
Fig. 7.22 Asymmetric distal migration of the ventral ventral tissues, here right (i.e., the arrested distal migra-
penile structures will result in torsion. The penis will twist tion acting as a “horse rein” (©Ahmed T. Hadidi 2022.
toward the more developed side (here left) as it is pulled All Rights Reserved)
proximally and downward through the more hypoplastic
a b c
Fig. 7.23 (a) Sagittal section in 11 weeks’ gestation an asymmetrical configuration with respect to the midline
male embryo. (b) Transverse section through the proximal ((a) ©Ahmed T. Hadidi 2022. All Rights Reserved, (b, c)
shaft of an 18.5 weeks’ fetus with proximal penile hypo- from van der Putte [9], reprinted from J Plast Reconstr
spadias, ventral curvature, and torsion. (c) Normal fetus of Aesthetic Surg, Vol. 60, Issue 1, pp48-60, © 2007, with
24 weeks. The ventral penile structures are present but in permission from Elsevier)
a b
Fig. 7.24 Megameatus intact prepuce hypospadias. (a) spadias for several years, especially if it is difficult to
The child will have a normal prepuce and the only thing retract the foreskin. (b) After retraction of the foreskin,
that suggests that he may have hypospadias is the abnor- the child has a large (mega) meatus at the base of the glans
mal median raphe. This may delay the diagnosis of hypo- (©Ahmed T. Hadidi 2022. All Rights Reserved)
ular or coronal hypospadias, deeply grooved

glans plate, and normal complete prepuce. 7.13 Penoscrotal Transposition
Usually, the median raphe is irregular or zigzag and Bifid Scrotum
shaped which may give an indication of abnor-
mal urethral development before retraction of the Early in development, the genital tubercle proj-
prepuce (Fig. 7.24). ects caudally and the labioscrotal folds lie lateral
The complete formation of prepuce in associ- to the genital tubercle and urogenital folds (or
ation with glanular deeply grooved (cleft) glans labia). The urethral orifice is bounded laterally by
in MIP is not explained by the failure of fusion the urogenital folds. Deep stromal tissue and dar-
theory, where the prepuce is supposed to develop tos fascia occupy most of the scrotal swellings
entirely from the glans. (see Chap. 3: Embryology).
According to the “disorganization hypoth- With later development, the ventral penile
esis,” the median raphe proliferates and structures proliferate much more than the dorsal
migrates forward (together with the posterior structures of the penis, the penis rotates cranially
edge of the urethral orifice) from the frenulum to form a 90 degree angle, and the migrating tes-
and median ventral part of the prepuce tes – together with two scrotal swellings – move
(Fig. 7.25). closer to the midline and occupy the space under
MIP could occur when the posterior urethral the penis (Fig. 7.26).
edge migrates forward until it reaches the coronal Penoscrotal transposition could be due to dis-
sulcus only and does not continue to grow over organized growth and inadequate proliferation of
the grooved glans penis to form the floor of the the ventral penile structures which hinders the
glanular urethra. In the meantime, the median migration of the two scrotal swellings toward the
raphe and underlying dartos fascia continue to midline under the scrotum.
grow along with the preputial lamella to form a If the disorganization also involves the median
complete prepuce with a normal underlying dar- raphe and is also underdeveloped, the condition
tos fascia but no frenulum. will be associated with bifid scrotum.
178 A. T. Hadidi
a b c
Fig. 7.25 (a) Three different patients with hypospadias attempt of frenulum formation and how the frenulum
with deficient ventral prepuce that appears as if it is about (median part of the prepuce) is gradually incorporated
to unite in the midline ventrally forming the floor of the into the glans forming the floor of the glanular urethra
glanular prepuce and the complete prepuce. (b) Open pre- (©Ahmed T. Hadidi 2022. All Rights Reserved)
puce without hypospadias or chordee. (c) Notice the early
b c d
Fig. 7.26 (a) Penoscrotal transposition may result from position. (c) Classic perineal hypospadias with incomplete
failure of the scrotal folds to migrate caudally and medi- penoscrotal transposition. (d) Classic distal hypospadias
ally at the same time of arrested, disorganized distal with incomplete prepuce (©Ahmed T. Hadidi 2022. All
migration of the ventral mesenchyme. (b) Complete sepa- Rights Reserved)
ration of the scrotal folds with complete penoscrotal trans-
7.14 Anoscrotal Distance (ASD) hypospadias with severe chordee, the ASD may be
as short as 1 or 1.5 cm. This is related to arrested
The anoscrotal distance (ASD) in distal forms of distal migration of the ventral mesenchyme and
hypospadias not associated with severe chordee is may help as a marker to identify the severity of
like in normal boys >3 cm. In proximal forms of chordee in an outpatient setting (Fig. 7.27).
a b c
d e
Fig. 7.27 Anoscrotal distance (ASD): (a) glanular hypo- that 3 cm is about 2 finger breadth. (d) Proximal hypospa-
may facilitate the stretching of this erectile organ.

7.15 Discussion Hardly any information is available about the
microscopic anatomy of these structures in hypo-
Normally, the microscopic anatomy of the penis spadias [9].
demonstrates a subtle structure that is easier to Classic theories suggest that the penile urethra
appreciate during development than after birth. forms as a result of fusion of the medial edges of
In fetuses, histological analysis shows a delicate the endodermal urethral folds like closing a zip in
differentiation of the penile septum, frenulum, the midline [22–27]. The ectodermal edges of the
and the various penile fasciae. For example, a urethral groove fuse to form the median raphe
subepidermal circumferential layer of fine con- [1]. The glanular urethra is supposed to develop
nective tissue replaces the usual dermis, the dar- due to endodermal cellular differentiation or due
tos fascia consists of bundles of smooth muscle to an ingrowth of ectoderm from the tip of the
cells in a loose texture, and the dense deep fascia glans [27–30].
of the penis contains the spongy vascular Clinical findings, intrauterine photos, and his-
trabeculae of the corpora cavernosa and spongio- tological sections do not support the fusion the-
sum. All the penile fascial layers have a trans- ory. Clinically, the variable width of the urethral
verse to oblique orientation of the fibers. This plate in hypospadias, the different glans
180 A. T. Hadidi
c onfigurations, chordee with and without hypo- nations [8], cutaneous deficiency [31, 33] defor-
spadias, and the presence of a midline anocutane- mities of the corpora cavernosa, and fibrosis were
ous fistulous tract in some patients with suggested [18, 19, 21, 32].
imperforate anus are not explained by fusion The capacity of growth of these dysplastic tis-
from lateral to medial. The intrauterine photos sues is questionable as documented by Koff et al.
showed that the urethral opening migrates dis- showing that gonadotrophin stimulation of the
tally along the shaft of the penis. Histological GT affects the dorsum and the segment of GT
sections showed that the penis and its urethra situated proximal to the division of the corpus
develop by rapid growth and elongation of the spongiosum [35, 36].
original urogenital sinus and its related mesen-
chymal structures. There were no signs of regres-
sion in the epithelium covering the urethral folds 7.16 Disorganization Hypothesis
which is mandatory for mesenchymal fusion and (Fig. 7.28)
urethral closure. Histological examination did
not show any change in the microstructure of the Early in development, the cloacal eminence dif-
erectile and fascial structures during the develop- ferentiates into the genital tubercle which proj-
mental process. This indicates a process of dif- ects caudally and makes a 10 degree angle with
ferential growth and is inconsistent with fusion the longitudinal axis of the body. The ventral
from lateral to medial (see Chap. 3: Embryology). stroma and fascia of the penis play a crucial role
The presence of an abnormal distal corpus in the normal development of the penis and ure-
spongiosum has been reported as a regular and thra. Rapid differential proliferation of the ven-
distinctive feature of deficiency with laterally tral stroma and fascia results in progressive
penetrating spongious tissue interpreted as an forward migration of the posterior edge of the
underdeveloped or malformed corpus spongio- urethral orifice and rotation of the penis to make
sum [18–21]. Van der Putte in his study on five a 90 degree angle with the longitudinal body
penises with hypospadias showed that the corpus axis.
spongiosum in hypospadias consisted of irregular Arrested distal migration and disorganized
plexuses of large veins between the urethra and growth of the ventral fascia may explain hypo-
the corpus cavernosum [9]. They represent bilat- spadias and most of its associated anomalies.
eral vascular plexuses that temporarily predomi-
nated in normal fetuses between 11 and • Inadequate proliferation and disorganized
13.5 weeks (40–70 mm CRL) and connected all growth of the dartos fascia and corpus
primordial erectile structures. In three out of the spongiosum may result in chordee without

five specimens, histological examination showed hypospadias with its variable presentations.
distinct disorganization of the penile fascia and • If the disorganization hinders the forward
septum on the ventral aspect of the penis. migration of the posterior edge of the urethral
Chordee has been observed in all variants of orifice, this will result in hypospadias.
hypospadias [18, 19, 21, 31–33]. The traditional Incomplete disintegration of the median part
view that the curvature results from a fibrotic of the glans plate results in the variable degree
cord at the urethral side of the penis was found of glans clefting.
realistic in only very few cases [19, 32]. More • If the inadequate proliferation and disorga-
often a fibrous plate with lateral extensions nized growth involve the ventral part of the
existed [18, 19, 21, 32], but in many other cases, corpus spongiosum and dartos fascia more
remarkably little fibrotic tissue was encountered than the urethra, this will result in hypospa-
during operations [19]. On clinical and theoreti- dias associated with superficial chordee.
cal grounds, relative deficiency of integument of • Hypospadias associated with deep chordee
the urethra [32, 34], absent or abnormal structure may be due to disorganization of the corpus
of the corpus spongiosum and/or deep fascia of spongiosum and fascia dorsal (deep) and dis-
the penis and/or dartos fascia in variable combi- tal to the urethra.
Fig. 7.28 The migration hypothesis and the disorganiza- distal migration will result in hypospadias and the associ-
tion hypothesis. Normal development occurs through dis- ated anomalies (©Ahmed T. Hadidi 2022. All Rights
tal migration of the ventral mesenchyme until the urethral Reserved)
meatus reaches the tip of the glans. Arrested, disorganized
182 A. T. Hadidi
• The median raphe begins normally at the Implications for hypospadias repair. J Urol. 2000;
https://doi.org/10.1016/s0022-5347(05)67233-3.
perineum and grows distally along with the 4. Page RE. Hypospadias revisited. Br J Plast Surg. 1981;
urethra. Arrested migration of the posterior https://doi.org/10.1016/S0007-1226(81)80083-5.
edge of the urethral orifice is associated with 5. Avellán L, Knutsson F. Microscopic studies of
abnormal development of the median raphe curvature-causing structures in hypospadias. Scand
J Plast Reconstr Surg Hand Surg. 1980; https://doi.
which bifurcates proximal to the urethral org/10.3109/02844318009106718.
orifice. 6. Paul M, Kanagasuntheram R. The congenital anoma-
• If one branch of the median raphe grows inad- lies of the lower urinary tract 1. Br J Urol. 1956;
equately and is shorter than the other branch, https://doi.org/10.1111/j.1464-410X.1956.tb04743.x.
7. Rowsell AR, Morgan BDG. Hypospadias
this will result in rotation or torsion of the and the embryogenesis of the penile ure-
glans toward the longer branch. thra. Br J Plast Surg. 1987; https://doi.
• The median raphe is responsible for the devel- org/10.1016/0007-1226(87)90196-2.
opment of the median part of the ventral pre- 8. Baskin LS, Erol A, Li YW, Cunha GR. Anatomical
puce as well as the frenulum. As the median org/10.1016/S0022-5347(01)62711-3.
raphe bifurcates proximal to the urethral ori- 9. van der Putte SCJ. Hypospadias and associated
fice in hypospadias, the prepuce is deficient penile anomalies: a histopathological study and a
ventrally in most forms of hypospadias. reconstruction of the pathogenesis. J Plast Reconstr
Aesthetic Surg. 2007; https://doi.org/10.1016/j.
• The rare and peculiar form of hypospadias bjps.2006.05.020.
known as megameatus intact prepuce (MIP) is 10. Hadidi AT, Roessler J, Coerdt W. Development of
rather complex. There is normal migration of the human male urethra: a histochemical study on
the posterior edge of the urethral orifice until human embryos. J Pediatr Surg. 2014; https://doi.
org/10.1016/j.jpedsurg.2014.01.009.
it reaches the glans and completes the groov- 11. Hadidi AT. Classification of hypospa-
ing of the glans plate. The median raphe grows dias. Hypospadias Surg. 2004; https://doi.
normally but the intimately related fascia and org/10.1007/978-3-662-07841-9_7.
posterior edge of the urethra do not continue 12. Liu G, Liu X, Shen J, Sinclair A, Baskin L, Cunha
GR. Contrasting mechanisms of penile urethral for-
to grow to close the floor of the glanular mation in mouse and human. Differentiation. 2018;
urethra. https://doi.org/10.1016/j.diff.2018.05.001.
• Inadequate proliferation and disorganized 13. Liu X, Liu G, Shen J, Yue A, Isaacson D, Sinclair A,
growth of the ventral fascia may prevent the Cao M, Liaw A, Cunha GR, Baskin L. Human glans
and preputial development. Differentiation. 2018;
migration of the scrotal folds and result in https://doi.org/10.1016/j.diff.2018.08.002.
penoscrotal transposition. 14. Baskin L, Shen J, Sinclair A, Cao M, Liu X,
• Failure of proliferation of the median raphe Liu G, Isaacson D, Overland M, Li Y, Cunha
and penile septum may result in bifid scrotum GR. Development of the human penis and clito-
ris. Differentiation. 2018; https://doi.org/10.1016/j.
which may or may not be associated with diff.2018.08.001.
hypospadias. 15. Baskin LS, Cunha GR. Embryology of the geni-
tourinary tract in Partin A. In: Partin AW, Peters
CA, Kavoussi LR, Dmochowski R, Wein AJ, edi-
tors. Campbell-Walsh-Wein Urology. 12th ed.
Philadelphia, PA: Elsevier; 2020. p. 305.
References 16. Hunter R. Notes on the development of the prepuce. J
Anat. 1935;70(Pt 1):68–75.
1. Baskin L. Basic science of the genitalia. Kelalis- 17. Kaufmann C. Verletzungen und Krankheiten der män-
King-Belman Textb Clin Pediatr Urol Fifth Ed. 2006; nlichen Harnröhre und des Penis. Stuttgart: Deutsche
https://doi.org/10.1201/b13795-72. Chirurgie; 1886.
2. Snodgrass WT, Shukla AR, Canning DA (2007) 18. Marshall M, Beh WP, Johnson SH, Price SE,
Hypospadias. In: Kelalis-King-Belman textbook of Barnhouse DH. Etiologic considerations in peno-
clinical pediatric urology. 5th. Informa Healthcare, scrotal hypospadias repair. J Urol. 1978; https://doi.
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3. Snodgrass W, Patterson K, Plaire JC, Grady R, 19. Smith DR. Hypospadias: its anatomic and therapeutic
Mitchell ME. Histology of the urethral plate: considerations. J Int Coll Surg. 1955;24
20. Williams DI. The development and abnormalities of 29. Altemus AR, Hutchins GM. Development of the
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21. Avellán L. Morphology of hypospadias. Scand J 30. McLachlan J. External genitalia. In: Hogg J, Keany S,
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org/10.3109/02844318009106717. Wesley Publishing Company; 1994. p. 316–59.
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Histologie der männlichen Harnröhre. Arch für dias. In: Stephens FD, Smith ED, Hutson JM (eds)
Mikroskopische Anat. 1903; https://doi.org/10.1007/ Congenital anomalies of the urinary and genital tracts.
bf02978196. Isis Medical Media, Oxford, pp. 80–90.
23. Keibel F, Mall FP. Development of the urogeni- 32. van der Meulen JC. Hypospadias and cryptospa-
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The Urethral Plate and Chordee
8
Ahmed T. Hadidi
Abbreviations 8.1 The Urethral Plate
AAP American Academy of Pediatrics 8.1.1 Introduction

ASD Anoscrotal distance
CEDU Chordee excision and distal There is a lot of controversy about the definition,
urethroplasty histology, morphology of the urethral plate, and
EMC Extracellular matrix component its contribution to congenital penile curvature or
H/E Hematoxylin/Eosin chordee. In this chapter, we will concentrate on
HIS Hypospadias International Society the definition of the urethral plate and its mor-
SIS Small intestinal submucosa phology. We will focus on the histology of the
SLAM Slit-like adjusted Mathieu tissues underneath the mucous membrane and its
SMA Smooth muscle actin relation to chordee (penile curvature).
STAC STraighten And Close
STAG Staged tubularized autograft
TALE Tunica albuginea longitudinal excision 8.1.2 Definition of the Urethral
TAP Tunica albuginea plication Plate
TEM Transmission electron microscopy
TITA Transverse incisions into the outer 8.1.2.1 Medical Dictionary Definition
tunica albuginea The urethral plate is the endodermal lining of the
TVF Tunica vaginalis flap urethral groove that forms the lining of the
UVI Unaided visual inspection spongy urethra [1].
VC Ventral penile curvature
8.1.2.2 Embryological Definition
Glenister was probably the first to introduce the
A. T. Hadidi (*) term the “urethral plate” and defined it as “It is an
Hypospadias Center and Pediatric Surgery outgrowth from the anterior walls of the urogeni-
Department, Sana Klinikum Offenbach, Academic tal sinus” (Fig. 8.1) [2].
186 A. T. Hadidi
a b
Fig. 8.1 (a and b) Embryologically, the urethral plate is Anatomy, Vol 88 (Pt 3), by Glenister: “The origin and fate
the distal anterior end of the urogenital sinus that eventu- of the urethral plate in man”, p424, ©1954, with permis-
ally will form the penile urethra ((a) ©Ahmed T. Hadidi sion from Wiley and Sons [2])
2022. All Rights Reserved, (b) reprinted from Journal of
8.1.2.3 Anatomical Definition of this defect, known as the branches of the

Paul and Kanagasuntheram defined the urethral bifurcated raphe, gradually merge into the
plate as: reverted fold of the divided prepuce. It is a moist
It is the moist pink gutter of mucous membrane pink gutter of mucous membrane with a well-
with well-defined muco-cutaneous line and defined muco-cutaneous line. This gutter usu-
extends from the urethral orifice to the base of the ally extends from the hypospadiac urethral
glans penis [3]. orifice to the base of the glans penis.
• Mouriquand drew two lines from the edges of
8.1.2.4 Surgical Definition the divided prepuce until they meet ventrally
John Duckett wrote: proximal to the urethral meatus and called this
I have used the term “the urethral plate” to define area “the hypoplastic triangle” [7] (Fig. 8.3).
the skin and spongiosal tissue which lies distal to • Holland and Smith differentiated the lateral bor-
the urethral meatus in a hypospadias and goes out ders of the urethral plate from the rest of the penis
onto the ventral glans [4]. by “the glistening appearance of the plate epithe-
lium versus the dull glans and shaft skin” [8].
8.1.2.5 Other Definitions
Snodgrass et al. applied the term “urethral plate”
to “the strip of tissue that extends distal from the 8.1.4 pithelial Lining of the
E
hypospadiac meatus to near the tip of the glans” Normal Urethra and the
[5]. There was no definition of the width or the Urethral Plate
depth of the tissues referred to.
Hematoxylin/Eosin (H/E) staining of the 17
urethral plate specimens [9] showed that the
8.1.3 Morphology of the Urethral intact urethra was lined with pseudostratified
Plate epithelium. At the level of the meatus the
epithelium is non-keratinized stratified squa-
• Distal to the urethral meatus, the skin shows a mous epithelium. At the distal urethral plate it
V-shaped defect, referred to by Van der Meulen is keratinized stratified squamous epithelium
as the urethral delta [6] (Fig. 8.2). The borders (Fig. 8.4).
8 The Urethral Plate and Chordee 187
Fig. 8.2 Van der Meulen (1964) referred to the V-shaped the bifurcated raphe, gradually merge into the reverted
tissue distal to the urethral meatus, as “the urethral delta” fold of the divided prepuce (©Ahmed T. Hadidi 2022. All
[6]. The borders of this defect, known as the branches of Rights Reserved)
Fig. 8.3 Mouriquand (2004) drew two lines from the edges of the divided prepuce until they meet ventrally proximal
to the urethral meatus and called this area “the hypoplastic triangle” [7] (©Ahmed T. Hadidi 2022. All Rights Reserved)
188 A. T. Hadidi
Fig. 8.4 Hematoxylin/Eosin staining of the epithelium in from pseudostratified to a stratified squamous epithelium.
patient with perineal hypospadias. (a) Pseudostratified (b‘) The same section as in (b) magnified. (c) The urethral
epithelium lining the proximal intact urethra. (a‘) the plate is lined with keratinized stratified squamous epithe-
same section as in (a) magnified (b) The epithelial lining lium. (c‘) The same section as in (c) magnified (©Ahmed
of the urethra just proximal to the meatus has changed T. Hadidi 2022. All Rights Reserved)
thral plate at all and the ventral area between the

8.1.5 Quality of the Urethral Plate
hypospadias meatus and the glans is covered with
normal penile skin (Fig. 8.7c).
The urethral plate may be healthy, soft, well vascu-
larized and wide (Fig. 8.5a). On the other hand, the
urethral plate may be hypoplastic, atretic, poorly
8.1.7 Variations of the Urethral
vascularized and narrow especially in perineal hypo-
Plate in a Single Patient
spadias causing severe deep chordee (Fig. 8.5b).
This makes major difference in the management.
The urethral plate may vary in width and depth
For example, the child with healthy, well vascular-
even in the same patient. The variations in depth
ized urethral plate could get his perineal hypospa-
may suggest different stages of vacuolation (see
dias corrected in one operation (Fig. 8.5a). The child
Chap. 3) (Fig. 8.8).
with the hypoplastic atretic urethral plate required 3
operations to correct his severe chordee and recon-
struct neo-urethra (Fig. 8.5b).
8.1.8 Histology of the Urethral Plate
8.1.6 Width of the Urethral Plate • Van der Putte examined the histology of the
urethral delta in five fetuses and neonates
There are many variations of the urethral plate. In with hypospadias [10]. He described the
distal forms of hypospadias, the urethral plate is epithelium of the urethral plate as pale non-
usually wide (more than 0.8 cm), well vascular- cornifying stratified squamous epithelium in
ized, and without severe chordee (Fig. 8.6a, b). In the central area and a more eosinophilic strat-
perineal hypospadias, the urethral plate is usually ified squamous epithelium laterally. He iden-
narrow, inelastic, and associated with severe deep tified in the urethral plate gland-like
chordee (Fig. 8.6c). Rarely, there may be no ure- structures in a row which by position and
Fig. 8.5 Quality of the urethral plate. (a) The urethral urethral plate and excision of all the hypoplastic tissues
plate is wide, healthy, soft and well vascularized and there was not enough to straighten the penis. Single corporot-
is no chordee. (a‘) Urethral reconstruction was performed omy with tunica vaginalis flap was needed. The urethral
in a single operation. (b) The urethral plate is hypoplastic, reconstruction was performed in 3 operations (©Ahmed
short, atretic and poorly vascularized. (b‘) Division of the T. Hadidi 2022. All Rights Reserved)
190 A. T. Hadidi
a b c
Fig. 8.6 (a) The urethral plate is wide and without chor- ciated with severe deep chordee. This is the common form
dee. (b) In the middle, the urethral plate is narrow without in perineal hypospadias (©Ahmed T. Hadidi 2022. All
chordee. (c) The urethral plate is narrow atretic and asso- Rights Reserved)
a b c
Fig. 8.7 (a) The urethral plate may be wide (more than severe deep chordee. (c) A rare form of hypospadias with-
1 cm), well vascularized, and without any associated chor- out urethral plate (©Ahmed T. Hadidi 2022. All Rights
dee. (b) In severe proximal and perineal hypospadias, the Reserved)
urethral plate is narrow, inelastic, and associated with
mixed histology are similar to the lacunae of 8.1.9 ffect of Age on the Urethral
E
Morgagni. Plate
• Paul and Kanagasuntheram described the his-
tological sections of the urethral plate or gut- • Da Silva et al. in an interesting paper showed
ter as stratified squamous epithelium with structural changes that occur in the collagen
pigment in the Malpighian layer [3]. Their structure under the urethral plate with age
sections showed no underlying layer of erec- [13]. They compared the urethral plate of 16
tile tissue. patients (15 with superficial chordee that was
• H/E staining of 17 urethral plate specimens corrected by degloving), age ranged from
[9] showed that the intact urethra was lined 6 months to 53 years and 5 fetuses. They
with pseudostratified epithelium. At the level found significant increase in total collagen
of the meatus the epithelium became a non concentration (type I and III) by age. One
keratinized stratified squamous epithelium. At drawback of the study is that the control spec-
the distal urethral plate it became keratinized imens were obtained from 18 week gesta-
stratified squamous epithelium (Figs. 8.4 and tional age aborted fetuses, i.e. before complete
8.9) [9]. development of the urethra.
a b c
Fig. 8.8 In a single patient, the urethral plate may have variable width (a) or variable depth (b) or both variable width
and depth (c) (©Ahmed T. Hadidi 2022. All Rights Reserved)
Fig. 8.9 The epithelial plate is lined with stratified squamous epithelium with thick keratin layer (©Ahmed T. Hadidi
192 A. T. Hadidi
8.2 Chordee (Penile Curvature) 8.2.2 Definition
8.2.1 Introduction and Origin Chordee is defined as ventral curvature of the

of the Term Chordee penis, whereas other directions of curvature
must be explicitly defined, such as dorsal and
Hypospadias occurs in approximately 1 in 120 lateral chordee (Fig. 8.10). Curvature is most
live male births, and as many as one-fourth of obvious during erection but may also be appar-
those born with hypospadias will have some ent in the flaccid state due to tethering of the
degree of penile curvature [14]. Penile curvature ventral penile structures. There have been
also occurs in 4–10% of males born without attempts in the past to replace the term “chor-
hypospadias [15, 16]. On the other hand, 20% of dee” simply with “penile curvature.” However,
patients with distal hypospadias have superficial the term chordee is also used to describe the
chordee that is usually corrected by ventral ventral dysplastic tissue that causes curvature of
degloving. About 80% of patients with proximal the penis.
forms of hypospadias (grade III and IV) have
deep chordee that required division of the ure-
thral plate. 8.2.3 urgical Anatomy of Penile
S
The significance of chordee — known as Fascia
penile curvature — associated with hypospa-
dias was first appreciated by Galen [129– 8.2.3.1 N ormal Fascial Coverings
199 AD] who noted that penile curvature of the Penis
interfered with sexual intercourse [17]. The The penis is composed of two corpora cavernosa,
term “chordee” was first mentioned in the eigh- glans, and corpus spongiosum where the urethra
teenth-century French medical literature, lies. Each corpus cavernosum is surrounded by a
defined as “painful imperfect erection of the thick, dense fascial layer called the tunica albu-
penis during gonorrhea, with downward incur- ginea. Both the corpora cavernosa and the corpus
vation” [18]. Chordee was a common complica- spongiosum are surrounded by Buck’s fascia,
tion of gonorrhea in the days before the dartos fascia, and skin. The corpora cavernosa
introduction of antibiotics. More details about are closely apposed for three-quarters of their
the history of chordee and the origin of the term length.
are mentioned in Chap. 1: History. From a surgical point of view, underneath the
There is no general consensus in literature penile skin and its areolar connective tissue lie
regarding the precise definition, etiology, classifi- three fascial layers (Fig. 8.11a):
cation, or the surgical management of chordee 1. The dartos fascia consists of bundles of
[19]. This chapter will try to clarify the definition smooth muscle fibers that extend into the skin
and the pathogenesis and pathology of chordee, and Buck’s fascia. The blood supply of the
the classification, and grades of chordee. The penile skin comes from the superficial dorsal
standard protocol for chordee correction used in arteries in the dartos (superficial) fascia, thus
the Hypospadias Center in Frankfurt, Germany, allowing complete degloving of the penis
is mentioned in detail followed by general prin- without damage to the vasculature of the
ciples of chordee management including the dif- skin.
ferent surgical approaches that are common to 2. Buck’s fascia is a dense elastic layer that
correct chordee. encloses the two corpora cavernosa in one
Fig. 8.10 Types of chordee: The term chordee usually fascia, corpus spongiosum, and tunica albuginea. Lateral
refers to ventral chordee. Dorsal or lateral chordee must or dorsal chordee usually involves the lateral or dorsal part
be explicitly defined. It may be superficial, involving the of tunica albuginea (©Ahmed T. Hadidi 2022. All Rights
fascia superficial to the urethra, or deep involving the deep Reserved)
compartment and the corpus spongiosum 3. The tunica albuginea is the deepest covering
(enclosing the urethra) in a separate compart- of the penis. It forms a thick white sheath
ment. It is composed of obliquely running around each corpus cavernosum. Two layers
fibers and is firmly attached to the underlying may be identified (Fig. 8.11a): (1) an outer
tunica albuginea. Superficial to Buck’s fascia longitudinal coat and (2) an inner circular
run the superficial dorsal arteries and veins, coat. The tunica albuginea becomes thicker
while the neurovascular bundles run deep to it ventrally as it forms a groove for the corpus
and dorsolaterally. The neurovascular bundles spongiosum. The tunica condensates in the
consist of two dorsal arteries, nerves, and the middle between the two corpora cavernosa
retrocoronal venous plexus in its distal end. forming an intercavernous septum. The distal
The dorsal nerves run laterally to the arteries part of the septum is incomplete and is called
and then ventrolaterally before entering the the pectiniform septum, being perforated by
glans. The retrocoronal venous plexus is com- openings that provide free vascular communi-
posed of many small veins that leave the glans cation between the two corpora.
dorsolaterally, forming the deep dorsal vein
that runs in a groove at the junction of the two
corpora. Distally, Buck’s fascia is firmly 8.2.4 Morphology of Chordee
attached to the base of the glans at the coronal
sulcus, where it fuses with the ends of cor- Van der Meulen identified the V-shaped defect
pora. Proximally, it is firmly attached to the distal to the urethra meatus as the urethral delta
pubic rami, ischial spines, and ischial (Fig. 8.12) [6]. The borders of this defect, known
tuberosities. as the branches of the bifurcated raphe, gradually
194 A. T. Hadidi
a b
c d
Fig. 8.11 (a–d) The tunica albuginea is composed of TALE). (a, b, d ©Ahmed T. Hadidi 2022. All Rights
outer longitudinal and inner circular layer. In hypospa- Reserved; c (from Baskin et al. “Anatomical studies of
dias, it is possible to dissect the hypoplastic outer longitu- hypospadias”, J Urol, Vol 160, Issue 3 Part 2, pp1108–
dinal layer during the correction of chordee in the 1115, ©1998, with permission from Wolters Kluwer [12])
technique: Tunica albuginea longitudinal excision (or
merge into the reverted fold of the divided 8.2.5 Is There a Chordee Tissue?
prepuce. Mouriquand called this delta “the hypo-
plastic triangle” [7]. Some authors claim that there is no chordee
A normal corpus spongiosum is absent within tissue and penile curvature is due to either
this urethral delta [20–23]. Avellan and van der short ventral penile skin, short urethral plate,
Putte interpreted the laterally penetrating spongy or short urethra. But why is the ventral penile
tissue as an underdeveloped or malformed corpus skin or the urethral plate short? A longitudinal
spongiosum [24, 25]. section examination of a 15-week-old fetus
Fig. 8.12 A patient with proximal hypospadias. If one spongiosum, and ventral fascia (©Ahmed T. Hadidi 2022.
draws two lines along the branches of the raphe, this gives All Rights Reserved)
an idea about the level of disorganized urethra, corpus
8.2.6 Histology of the Chordee
Darewicz et al. excised tunica albuginea samples

from the greater curvature in 15 patients with con-
genital chordee and compared it with control sam-
ples from the lesser curvature of the same patients
[26]. The samples were studied using transmitter
electron microscopy. They found a chaotic disor-
ganized pattern of collagen fibers that formed
bundles with disrupted three-dimensional organi-
zation. The diameter of the fibers differed greatly
on cross section. They observed periodic widen-
ing and fragmentation of collagen fibers with
Fig. 8.13 Sagittal section in a 15-week-old fetus with complete disappearance of striation and transfor-
perineal hypospadias. Notice the corrugated ventral sur- mation into electron dense, fibrous granulated
face of corpus cavernosum (3): (1) wide urethral orifice.
material. Disrupted fibroblasts without cell mem-
(2) Hypoplastic distal corpus spongiosum, (3) corpus cav-
ernosum, (4) glans, (5) longitudinal fibers and vessels of brane and cellular organelles between collagen
perineal raphe, (6) hypoplastic proximal corpus spongio- fibers were also visible (Fig. 8.15).
sum (from van der Putte, reprinted from J Plast Reconstr Avellan and Knutsson in their microscopic
Aesthetic Surg, Vol. 60, Issue 1, ©2006, pp48–60, with
study on 20 hypospadias patients showed abnor-
permission from Elsevier [10])
mal disorganized corpus spongiosum surrounded
by lateral bands formed of hyaline sclerotic con-
with perineal hypospadias confirmed the pres-
nective tissue [27]. In patients with proximal
ence of hypoplastic corpus spongiosum
hypospadias, these hyaline sclerotic bands may
(Fig. 8.13).
correspond to tunica albuginea.
Furthermore, it is obvious intraoperatively
Van der Putte demonstrated that this lateral
that there is dysplastic tissue that gives gritty sen-
spongy tissue consists of irregular plexuses of
sation when divided (like when correcting
large veins and normally predominates between
Dupuytren’s contracture). Excision of this chor-
11 and 13.5 weeks, connecting all the primordial
dee tissue results in considerable lengthening of
erectile structures (see Chap. 5: Morphology)
the penis (Fig. 8.14).
196 A. T. Hadidi
Fig. 8.14 Distal hypospadias with severe chordee (grade IV). Excision of the chordee tissue resulted in lengthening of
the penis 2 cm (©Ahmed T. Hadidi 2022. All Rights Reserved)
a b
c d
Fig. 8.15 Cut sections in tunica albuginea from patients face (greater curvature). It shows collagen fibers of differ-
with penile curvature: (a) Longitudinal section of colla- ent diameters in tunica albuginea. Arrows indicate fibers.
gen fibers on the dorsal surface (lesser curvature) of the (d) Cross section of collagen fibers on the ventral surface
penis. It shows fibers homogeneous in size, periodicity, (from Darewicz et al.: “Ultrastructure of the tunica albu-
and organization, characteristic of type I collagen. (b) ginea in congenital penile curvature”, J Urol, Vol 166,
Cross section of the collagen fibers on the dorsal surface Issue 5, pp1766–1768, ©2001, with permission from
demonstrates even diameter of collagen fibers. (c) Wolters Kluwer [26])
Longitudinal section of collagen fibers on the ventral sur-
a b c
Fig. 8.16 Abnormal corpus spongiosum: (a) Transverse shaft in a normal fetus of 24 weeks (from van der Putte,
section through the proximal shaft of a fetus (32.5 weeks) reprinted from J Plast Reconstr Aesthetic Surg, Vol. 60,
with penile hypospadias. (b) Transverse section through Issue 1, ©2006, pp48–60, with permission from Elsevier
the proximal shaft of a fetus (32.5 weeks) with proximal [10])
hypospadias (c) Transverse section through the proximal
Light microscopy showed the existence of many

massed and intertwined collagen fibers and ves-
sels that resembled those of the cavernous sinus.
TEM showed the existence of many collagen
fibers, capillary vessels, and other structures.
Immunohistochemical staining showed collagen
subtype I in the interfascicular space, and colla-
gen fibers were densely stained. Collagen sub-
type IV was found in the basement membrane of
vessels, but collagen subtype III was not detected.
The same results were obtained by Western blot-
ting. Collagen subtype I usually occurs in the
maturation phase of scar formation, resulting in
Fig. 8.17 Western blotting showing that collagen sub- the formation of large, stiff bands of fibrils,
types I and IV were present but collagen subtype III was
not present in the tissues (from Hayashi et al.: whereas collagen subtype III is usually found in
“Characterization of the urethral plate and the underlying the distensible elastic tissues and is responsible
tissue defined by expression of collagen subtypes and for elasticity and expansion. They concluded that
microarchitecture in hypospadias”, Int J Urol, Vol 18, the tissue beneath the urethral plate was consid-
Issue 4, pp317–322, ©2011, with permission from John
Wiley and Sons) [28]) ered to originate from the corpus spongiosum
penis. The lack of collagen type III in the chordee
[10] (Fig. 8.16). The dysplastic penile fascia and tissue might affect ventral penile curvature.
irregular primitive venous plexuses amalgamate Spinoit et al. examined dartos fascia in patients
together to form a dense connective tissue that with hypospadias and observed poorly devel-
extends proximally from the angle of the urethral oped, abnormally distributed smooth muscle
delta to the base of the glans penis at the coronal fibers with lack of parallel configuration in the
sulcus. dartos fascia (Fig. 8.18) [29].
Hayashi et al. excised the urethral plate from The histology of the urethral plate and chordee
27 hypospadias patients with severe chordee and in 17 patients with perineal hypospadias and severe
examined the excised plate with light microscopy chordee was studied in the Hypospadias Center,
and transmission electron microscopy (TEM), Frankfurt, Germany [9]. Samples were examined
and the presence of collagen subtypes I, III, and with H/E, elastic van Gieson stain, smooth muscle
IV was examined with immunohistochemical actin (SMA), and Factor 8 (Fig. 8.19).
staining and Western blotting (Fig. 8.17) [28]. H/E staining showed that the intact urethra
was lined with pseudostratified epithelium that
198 A. T. Hadidi
a b c
Fig. 8.18 (a) Normal pattern I (normal) demonstrates Reduced from X (from Spinoit et al. “Congenital Penile
SMF (arrow) of DT organized in parallel configuration in Pathology is Associated with Abnormal Development of
subcutaneous tissue. (b) Pattern II reveals poorly devel- the Dartos Muscle: A Prospective Study of Primary Penile
oped and hypotrophic SMF (arrow) of DT. (c) Pattern III Surgery at a Tertiary Referral Center”, J Urol, Vol 193,
shows randomly distributed SMF of DT in subcutaneous Issue 5, pp1620–1624, ©2015, with permission from
tissue, with total absence of parallel configuration. Wolters Kluwer [29])
Fig. 8.19 Horizontal 4-μm sections were taken from cle actin (SMA) showed circular pattern of muscle fibers
proximal (left) to distal (right) in a child with perineal at the proximal end that became flat at the distal urethral
hypospadias. In this child, the hypoplastic intact thin ure- plate. (i–l) Factor 8 staining showed normal regular capil-
thra, proximal to the meatus, was excised until normal laries proximally that became large blood sinusoids at the
urethra was reached. (a–d) H/E showed gradual change distal urethral plate. (m–p) Elastic van Gieson stain
from pseudostratified epithelium to keratinized squamous showed scanty, elastic fibers (©Ahmed T. Hadidi 2022.
epithelium at the distal urethral plate. (e–h) Smooth mus- All Rights Reserved)
Fig. 8.20 Smooth muscle actin (SMA) staining. (a–d) As we progress distally, the U-shaped appearance
The change in the urethral plate from a groove to elevation becomes triangular. (h) The actin layer becomes flat
to a flat plate. (e) At the proximal end (left), the U-shaped (©Ahmed T. Hadidi 2022. All Rights Reserved)
actin rich layer just distal to the meatus can be seen. (f, g)
Fig. 8.21 Factor 8 staining of specimen from patient 12. embryonal blood sinusoids at the distal urethral plate
At the proximal end, there are rather regular, normal sized (©Ahmed T. Hadidi 2022. All Rights Reserved)
capillaries that were replaced with large, dysplastic,
a b c
d e
Fig. 8.22 (a–d) Elastic van Gieson staining showing increase of elastic fibers (©Ahmed T. Hadidi 2022. All
scanty elastic fibers and absence of organized bundles of Rights Reserved)
elastic fibers. (e) At the very lateral end, there was an
200 A. T. Hadidi
Table 8.1 Summary of the histology of the urethral plate and chordee
Intact urethra Meatus Urethral plate
Epithelium Ps. stratified transitional epithelium Non keratinized Keratinized stratified
stratified squamous squamous epithelium
epithelium
Actin/corpus Organized corpus spongiosum Multiple thick bundles Disorganized thick bundles
spongiosum circular around the urethra forming U-shaped forming a diffuse flat mass
sulcus
Von Willibrand Circular organized capillaries High density, large embryonic
factor 8 around the urethra blood sinusoids
Elastic fibres Organized elastic fibers Scanty, disorganized
changed at the meatus level to become a non- capillaries (Fig. 8.21) [9]. (6) Disrupted fibroblasts
keratinized stratified squamous epithelium that without cell membrane, very few elastic fibers and
became keratinized at the level of the urethral lack of organised bundles (Fig. 8.22) [9]. (7) The
plate distally (Figs. 8.4, 8.9 and 8.19). dartos fascia has poorly developed, abnormally
Smooth muscle actin (SMA) staining demon- distributed smooth muscle fibres with lack of par-
strated a regular circular pattern in the proximal allel configuration (Fig. 8.18) [29]. (8) Keratinized
intact urethra forming the corpus spongiosum. stratified squamous epithelium in contrast to pseu-
This changed to a U-shaped pattern at the level of dostratified epithelium in the intact urethra (Figs.
the meatus, to a triangle shaped pattern just distal 8.4 and 8.9) [9]
to the meatus. The distal urethral plate showed an
irregular disorganized, rather flat, thick pattern of
the smooth muscles (Fig. 8.20). 8.2.7 Etiology of Chordee
Factor 8 staining: Showed abnormal vascular-
ization: There was increased density of large The etiology of chordee remains unknown.
blood sinusoids with a disorganized arrangement In 1932, Arthur Cecil reported the experience
and an overall dysplastic impression (Fig. 8.21). of Dr. J Pettit who dissected the genital organs of
Elastic van Gieson staining showed very few a 10-year-old child in 1870 whom he had exam-
elastic fibers in general as compared with normal ined during life and who had penoscrotal hypo-
skin and subcutaneous tissues. No organized spadias [30]. Dr. Pettit inflated the corpora
bundles of elastic fibers were observed. In few cavernosa and then separated the urethra from the
cases, there was a slight increase of elastic fibers corpora cavernosa. He found that the penis still
at the lateral end of the samples in the deeper tis- kept its deformity and he attributed this to a
sues (Fig. 8.22). fibrous band beneath the penis but even division
The findings of the study are summarized in of this band did not straighten the penis. In mak-
(Table 8.1). ing a study of the cells of the corpora cavernosa,
In summary, patients with hypospadias and Pettit found that there was atrophy of these cells
severe chordee may have the following abnormali- on the concave portion. He did not know whether
ties in the urethral plate and the underlying chor- they had always been in this condition or whether
dee tissue (Fig. 8.19): (1) The tunica albuginea this atrophy was secondary to the hindering
showed disorganized pattern of collagen fibers. bands beneath the penis. It should be noted that
The diameter of the fibers differed greatly on cross the autopsy report of Pettit was made on a child
section (Fig. 8.15) [26]. (2) The corpus spongio- 10 years of age.
sum is hypoplastic, atretic and may be laterally Kaplan and Lamm found that ventral penile
displaced (Fig. 8.16) [10]. (3) The smooth muscle curvature persisted in 44% of aborted fetuses and
actin is irregular, flat and thick (Fig. 8.20) [9]. (4) concluded that the development of chordee was
Lack of collagen subtype III (Fig. 8.17) [28]. (5) part of normal development [31]. The severity of
Disorganised high density of irregular embryonal chordee is generally directly proportional to the
blood sinusoids instead of the regular, small blood severity of hypospadias.
Fig. 8.23 (a) Ventral

a b
fascial coverings of the
normal penis. (b) In
proximal hypospadias, the
corpus spongiosum and
ventral fascia are
underdeveloped, are
disorganized, and may fuse
together. The disorganized
hypoplastic fascia and
corpus spongiosum limits
and prevents normal distal
migration of the corpora
cavernosa and glans penis
(©Ahmed T. Hadidi 2022.
Literature review suggests four possible tunica albuginea) are hypoplastic and amalgam-
explanations for the etiology of chordee: ate with the irregular primordial spongiosal
1. Abnormal development of the urethral plate venous plexuses to form a dense connective tis-
[16]. sue (Fig. 8.24). The arrested distal migration of
2. Abnormal condensation of the mesenchymal the mesenchyme results in a shorter anoscrotal
tissue at the urethral meatus forming fibrous distance in severe forms of hypospadias
tissue [31]. (Fig. 8.25).
3. Differential growth between the normally Isolated patients with lateral, dorsal, or com-
formed dorsal corporeal tissue and the abnor- bined (corkscrew) chordee do not have the classic
mal ventral urethral plate [32]. hypospadiac penile appearance.
4. The “disorganization theory”: Arrested distal
migration and hypoplasia of the ventral penile
structures (corpus spongiosum, dartos fascia, 8.2.8 Erection Tests
and tunica albuginea) (Figs. 8.23 and 8.24)
[25, 33]. The classic approach until 1974 was to stretch the
The “disorganization hypothesis”: Based on penis using a stay suture at the glans and examine
the “migration hypothesis” of urethral develop- for tethering or hard tissue on the ventral aspect
ment and the “disorganization hypothesis” for of the penis. In adults, the patients used to take
hypospadias (see Chap. 3: Embryology and photographs in erection and then show it to the
Chap. 6: Pathogenesis), chordee is probably due surgeon. However, it is not very accurate and in
to arrested distal migration and hypoplasia of the some cases can be misleading, as it does not
ventral penile structures [7]. The corpus spongio- detect chordee due to corporeal disproportion.
sum and fascial coverings of the penis (including Many cases of hypospadias were repaired with-
202 A. T. Hadidi
a b c
Fig. 8.24 Abnormal corpus spongiosum. (a) Corpus hypospadias: the corpus spongiosum reaching only the
spongiosum bifurcating on either side of the thin urethra proximal third of the penis and thin urethra to the distal
in a patient with hypospadias sine hypospadias. (b) Absent third of the penis (©Ahmed T. Hadidi 2022. All Rights
corpus spongiosum in a glanular hypospadias. (c) Distal Reserved)
out correcting the co-existing chordee. As a No reports have described damage to the cavern-
result, residual curvature can be observed during ous tissue using the artificial erection test, as
follow-up. long as proper care is taken to ensure that
Today, there are two methods utilized to injectable saline is used. Penile necrosis has
induce erection of the penis intraoperatively: occurred when distilled water or epinephrine
1. The Artificial Erection Test was injected inadvertently into the corporeal
The French anatomist and surgeon, Ambroise bodies [36].
Paré (1510–1590) induced an artificial erec- Care must be taken when interpreting the results
tion of the penis 400 years before the descrip- of the saline erection test. The severity of chor-
tion by Gittes [34]. Gittes and McLaughlin dee (determined by the angle) apparent during
reintroduced artificial erection using heparin- artificial erection varies with the amount of
ized saline for the measurement of the chor- pressure used during injection, and the intra-
dee angle and the point of maximum curvature cavernous pressure varies widely (often not
and also to assess the results of chordee cor- physiological and may reach 280 mmHg). In
rection [35]. A red rubber catheter is used as a addition, it is difficult to place the tourniquet
tourniquet at the base of the penis, and normal low enough in patients presenting with large
saline is injected into the corporal body or suprapubic fat pads or to detect chordee at the
into the glans using a 23-G butterfly needle. base of the penis [37]. The author prefers to
Both corporal bodies are filled and exhibit the apply manual compression at the base of the
extent of the curvature (Fig. 8.26). This is penis rather than using a tourniquet.
repeated after excision of the fibrous chordee 2. Natural Erection Test
or after dorsal plication to assess the amount The natural erection test has been used since
of correction of the chordee before proceed- 2020 in the Hypospadias Center, Frankfurt,
ing with hypospadias repair. Germany, and is under objective evaluation and
a b c
d e
Fig. 8.25 Anoscrotal distance (ASD): (a) glanular hypo- that 3 cm is about 2 finger breadth. (d) Proximal hypospa-
comparison with the standard artificial erection technique and may be used as an alternative to
test (personal communication). artificial erection test. However, it is operator
Technique of Natural Erection Test: Two fingers dependent and requires experience to ensure
of the left hand press against the root of the adequate filling of the corpora cavernosa (per-
penis to stop blood drainage from the penis and sonal communication).
two fingers of the right hand massage the blood 3. Pharmacological Erection Test
from the perineum distally into the penis until The use of pharmacological agents to induce an
it becomes hard. Photos are taken and the angle erection in adults and older children was
of curvature is measured using Angle Meter described by Perovic [38]. He believed that the
App (Fig. 8.27). use of prostaglandin E1 (10–20 g) produced a
Preliminary results suggest that the natural erec- more “natural” erection that could be main-
tion test is a simple, reliable and non-invasive tained during the operation and avoided the use
204 A. T. Hadidi
of a tourniquet during the operation, thereby pharmacological erection was 47 to 70 mmHg,

reducing ischemia and postoperative edema. whereas during saline erection, the intracavern-
Kogan reported the use of alprostadil (14 mcg) ous pressure was 50 to 250 mmHg. Kogan con-
administered intracavernously in 56 boys with cluded that pharmacologically induced
hypospadias, ranging from 6 months to erections in hypospadias repair are effective
13 years [37]. Phenylephrine (40 mcg) was and reliable with no significant complications.
given for detumescence. Erection persisted Disadvantages of the pharmacologically induced
during chordee repair as long as the corpora erection test include increased blood loss dur-
were not opened. Detumescence occurred ing erection, additional cost, and the need for
within seconds in all cases where phenyleph- a reversal agent.
rine was given. There were no cases of pria-
pism, and systemic blood pressure and pulse
did not change. Intracorporeal pressure during 8.2.9 Grades of Chordee
Chordee presents as a wide spectrum of severity.

It may involve the skin, dartos fascia, Buck’s fas-
cia, corpus spongiosum, or the urethra and the
tunica albuginea. These structures may be
replaced by amalgamated disorganized hypoplas-
tic tissue to varying degrees. This disorganized
dysplastic tissue extends proximally from the
angle of the urethral delta distally into the glans
penis.
King wrote an important article stating that
chordee could be produced by skin alone (super-
ficial chordee) [39]. Devine and Horton described
three pathologic types of chordee without hypo-
spadias [40]. In the first type, the spongiosum is
absent in the distal penis and the urethra is often
paper thin. In the second type, the urethra is com-
Fig. 8.26 Artificial erection test pletely developed but Buck’s fascia and the
Fig. 8.27 A child with proximal hypospadias with severe test after degloving (©Ahmed T. Hadidi 2022. All Rights
chordee (a) Natural erection test before degloving (b) Reserved)
Natural erection test after degloving (c) Artificial erection
a c
b
Fig. 8.28 Three grades of chordee: (a) Grade I or super- dee with corporeal disproportion requiring corporotomy
ficial chordee; curvature proximal to the meatus. (b) plus flap or graft or multiple corporotomies (©Ahmed T.
Grade II or deep chordee; curvature distal to the meatus Hadidi 2022. All Rights Reserved)
requiring division of the urethral plate. (c) Grade III; chor-
dartos fascia are abnormal. The third type was • Grade III: Chordee with Corporeal
described as a skin chordee and is due to an Disproportion
abnormality of the superficial dartos fascia. A This occurs when the ventral surface of cor-
fourth type was mentioned by Kramer et al., pora cavernosa is also involved and the ven-
described as the result of disproportional growth tral side is shorter than the dorsal side. This is
of the dorsal aspect of the corporal bodies caus- usually encountered in older children with
ing downward deflection [41]. longstanding uncorrected deep chordee. In
In general, chordee with or without hypospa- this case, the dorsal surface grows normally
dias may be classified into the following grades but the ventral surface cannot grow due to
(Fig. 8.28): hypoplastic tissues ventrally (Fig. 8.32).
• Grade I: Superficial Chordee

This is usually seen in distal hypospadias and
in chordee without hypospadias. It is present 8.2.10 I s the Penile Curvature
when the superficial coverings (i.e., dartos fas- an Angle or an Arc?
cia, Buck’s fascia, and sometimes the corpus
spongiosum) are hypoplastic and disorganized. Careful examination of the corpora cavernosa
The dysplastic fibrous tissue is usually proxi- after excision of the outer layer of the tunica
mal to the meatus. The segment of the penis albuginea confirms that it is an angle and one can
between the meatus and the tip of the glans has clearly see the line where the corpora cavernosa
little or no curvature (Fig. 8.29). Palpation of is kinked (Figs. 8.33 and 8.34).
the ventral aspect of the penis distal to the
meatus between two fingers does not reveal
any hard tissue. 8.2.11 H
ow to Measure the Chordee
• Grade II: Deep Chordee Angle?
This is typically encountered in proximal and
perineal hypospadias. The deep layer of Several authors believe that the “eye ball” test
Buck’s fascia is also involved. Dysplastic tis- underestimates the degree of curvature [19].
sue is usually distal to the meatus. Palpation They recommend to use the orthopedic device
of the ventral aspect of the penis distal to the called the “goniometer” (Fig. 8.35a) and sug-
meatus between two fingers reveals tethering gested that it should be the standard device to
and hard tissue. Excision of this dysplastic tis- measure accurately the degree of curvature and to
sue distal to the meatus is required to straighten allow for standardization and comparison among
the penis (Figs. 8.30 and 8.31). different centers (Fig. 8.35a).
206 A. T. Hadidi
Fig. 8.29 Hypospadias with superficial chordee. The spadias meatus (B) – called the urethral plate – remains
curvature of the penis is proximal to the meatus. the same, while the distance (BC) between the hypospa-
Superficial incision proximal to the meatus and excision dias meatus (B) and the base of the penis (C) becomes
of the bands between the skin and the urethra usually cor- longer as the penis is straightened and the meatus (B)
rects this form of superficial chordee. It is important to moves forward and away from the skin attachment
note that in hypospadias with superficial chordee, the dis- (©Ahmed T. Hadidi 2022. All Rights Reserved)
tance (AB) between the tip of the glans (A) and the hypo-
However, Villanueva [42] compared the “eye as 20° [44] to 30° [45] of ventral curvature may
ball” test or “unaided visual inspection” (UVI) result in significant morbidity for patients, includ-
against goniometry estimations of ventral penile cur- ing difficulties with intercourse and the patient’s
vature (VC) among 25 pediatric urologists. There dissatisfaction of the appearance of the penis.
was no statistical difference between UVI and goni- A survey concerning congenital chordee with
ometry methods for any degree of curvature. The hypospadias was sent to 236 members of the
study concluded that goniometry was not superior to American Academy of Pediatrics (AAP), Section
UVI at estimating VC. Villanueva [43] described the on Urology. Seventy-five respondents indicated
assessment of penile ventral curvature using an that they would not intervene surgically if the
Angle meter App. In a pilot study, he assessed the chordee angle was less than 20%. Significant
penile curvature in 21 patients using an Angle meter chordee was clinically defined as curvature
App, the goniometer and unaided visual inspection greater than 30° and less than 45°. Fifty percent
(UVI). The study concluded that using the App was of respondents indicated that they would perform
superior to goniometer and UVI (Fig. 8.35b). dorsal plication if the angle was more than 20°,
while 99% of respondents would perform dorsal
plication if the angle was >30° and less than 45°.
8.2.12 When to Correct Chordee? Severe chordee was defined as a chordee angle of
>45°. Severe chordee should be approached ven-
Assessment of men with persistent/untreated or trally (Fig. 8.36) [46, 47].
those with Peyronie disease suggests that as little
b
c
Fig. 8.30 (a) Perineal hypospadias in a 15-week-old distance between the tip of the glans and the urethral
fetus showing hypoplastic corpus spongiosum. (b) meatus (distance AB) and may not change the distance
Schematic illustration showing the effect of ventral between the meatus and the base of the penis (distance
arrested migration on the corpora cavernosa and the sub- BC) ((a) from van der Putte, reprinted from J Plast
coronal incision to correct the curvature. (c) After exci- Reconstr Aesthetic Surg, Vol. 60, Issue 1, ©2006, pp48–
sion of the deep chordee, the penis becomes much longer 60, with permission from Elsevier [9], (b, c) ©Ahmed T.
and straight. Here, subcoronal incision will increase the Hadidi 2022. All Rights Reserved)
208 A. T. Hadidi
Fig. 8.31 Perineal hypospadias in a 6-month-old baby. Subcoronal incision and tunica albuginea externa excision was
adequate to correct the deep chordee (©Ahmed T. Hadidi 2022. All Rights Reserved)
a b c
Fig. 8.32 (a) Proximal hypospadias with corporeal dis- is an indication of the corporal disproportion and suggests
proportion in a 14-year-old boy. (b) Dissection of the lon- the persistence of severe chordee with erection test and is
gitudinal layer of the tunica albuginea was not enough to an indication for deep incision of the tunica albuginea and
correct the chordee. Ventral grafting was needed to correct the use of flaps or grafts (©Ahmed T. Hadidi 2022. All
the persistent penile curvature. (c) Notice the “kink” in the Rights Reserved)
middle of the tunica albuginea. The presence of the “kink”
According to the Hypospadias International ciently early in life that the corpora cavernosa will
Society (HIS) classification, chordee is classified fully develop… I can recall but one or two authors
who have suggested that this operation be done
into mild chordee (<20°), moderate chordee within the first two years of life…The operation for
(>20° and < 30°), and severe chordee > 30° (see the correction of the deformity should be done as
Chap. 9: Classification) [48]. soon as the child has been weaned and its nutrition
has been fully established. Some authors have sug-
gested that in some instances it is wise to cut into
corpora cavernosa. It seems to me that this is a bad
procedure on the account of hemorrhage and prob-
8.2.13 F
rankfurt Protocol of Chordee ably uncalled for if the operation of correcting the
Correction deformity is done sufficiently early in life [30].
This is also the author’s experience and in the

8.2.13.1 Timing of Chordee Hypospadias Center we try to correct severe
Correction: Early Correction chordee as early as 6 months. This is particularly
of Chordee at 6 Months important in proximal and perineal hypospadias
As early as 1931, Arthur Cecil wrote: with severe chordee. In those patients, if we do
Clinical experience would seem that if the skin, not correct the severe chordee early enough, there
subcutaneous tissue and fibrous bands extending up is a higher risk that there will be corporeal
between the corpora cavernosa are divided suffi-
Fig. 8.33 Corporeal disproportion in a 15-month-old boy. Note the clear angle of curvature (©Ahmed T. Hadidi 2022.
a b
Fig. 8.34 (a and b) Corporeal disproportion in a 23-year-old man. Note the clear angle of curvature. Also, note that in
a, the ventral outer layer of tunica albuginea was not excised (©Ahmed T. Hadidi 2022. All Rights Reserved)
210 A. T. Hadidi
a b
Fig. 8.35 (a) The goniometer to measure accurately the Ahmed T. Hadidi 2022. All Rights Reserved, (b) courtesy
degree of penile curvature (b). Measurement of the degree of Dr. Braga)
of penile curvature using an app on the mobile phone. ((a)
Fig. 8.36 From a surgical aspect, the angle of chordee is with severe chordee (> 30°) will need division of the ure-
currently classified into mild (< 20°), moderate (between thral plate to correct the severe curvature (©Ahmed T.
20° and 30°), and severe chordee (> 30°). Patients with Hadidi 2022. All Rights Reserved)
angle <20° do not require surgical correction. Patients
d isproportion and hence the need to incise the internal review of 149 grade IV hypospadias
corpora cavernosa and grafting or flaps will be operated on between 2011 and 2017, 2 out of 84
needed. It is impossible to run a prospective ran- patients less than 9 months had corporeal dispro-
domized trial to reach an objective conclusion, portion as compared with 14 out of 65 patients
but this is based on clinical experience. In an operated on after 12 months of age.
8.2.13.2 Intraoperative Assessment combination of superficial and deep chordee). It

and Planning is not uncommon for the preoperative assessment
Surgical correction of chordee remains a major to differ dramatically from intraoperative find-
challenge even for the most experienced sur- ings, leading the surgeon to adopt a technique
geons. There is a vast variability among patients. that differs from the originally planned approach.
Sometimes, the urethral plate is healthy, soft, and Therefore, the surgeon must be prepared to be
mobile over the corpora cavernosa. In other flexible in the course of the operation.
patients, however, the urethral plate may be Under general anesthesia, the first step is to
atretic, hard, and fixed. Occasionally, the patient examine the penis carefully after applying a stay
may have hard rigid bands distal to the meatus, as suture at the glans (Fig. 8.37). Inspection usually
well as skin tethering proximal to the meatus (a reveals the location of penile curvature (distal or
a b c
Fig. 8.37 Proper assessment of the severity of the chor- ture and the presence or absence (and also location) of
dee can only be done under anesthesia after applying a stay tethering, stoutness, and rigid fibrous bands under the skin.
suture to the glans. Inspection usually reveals the location Introduction of a catheter or sound may show paper-thin
of penile curvature (distal or proximal to the meatus) and urethra and transparent overlying skin. (a) No chordee, (b)
the quality of the urethral plate (atretic, healthy, lined by superficial chordee, (c) deep chordee (©Ahmed T. Hadidi
skin, mucous membrane, or narrow white tissue). Palpation 2022. All Rights Reserved)
between two fingers confirms the location of penile curva-
212 A. T. Hadidi
proximal to the meatus) and the quality of the Byars has described the procedure accurately:
urethral plate (atretic, healthy, lined by skin, Traction on the glans demonstrates…the tense
mucous membrane, or narrow white tissue). malformed remnants of the malformed urethra …
Palpation between two fingers may confirm the all strands of limiting tissues are removed after the
location of penile curvature and the presence or manner of operating for Dupuytren’s contracture
of the hand [49].
absence (and also location) of rigid fibrous bands
under the skin. The standard protocol in the Hypospadias Center
is to perform extensive ventral degloving first and
8.2.13.3 Ventral Degloving re-assess the situation and the degree of curvature
of the Penis afterward. If the penis is straight (or the curvature
Several studies have shown that chordee is due is less than 20 degrees), we proceed with the ure-
to arrested distal migration and hypoplasia of the thral reconstruction according to the location of
ventral penile structures (including corpus spon- the urethral meatus.
giosum and ventral fascia) [7] and the dorsal Correction of penile curvature due to superfi-
aspect of the penis is not involved. This means cial chordee involves a ventral subcoronal
that there is no indication to perform dorsal or U-shaped incision proximal to the hypospadias
complete degloving of the penis unless there is meatus and the release of all tethered skin attach-
an associated torsion. Complete degloving ments (Fig. 8.38). This must be performed with
increases the morbidity and edema, reduces vas- extreme care as the urethra may be very thin due
cularity, and reduces the surgical options for the to the absence or involvement of the corpus spon-
surgeon. giosum in the anomalous fibrous tissue. This pro-
Fig. 8.38 Ventral degloving is usually enough to correct the base of the penis becomes longer. This is sometimes
superficial chordee (distal hypospadias). The distance wrongly interpreted that degloving may change proximal
between the urethral meatus and the tip of the glans hypospadias into a distal one (©Ahmed T. Hadidi 2022.
remains the same but the distance between the meatus and All Rights Reserved)
cedure results in the straightening of the penis and

a skin defect proximal to the meatus. If the sur-
geon performs the SLAM technique, the U-shaped
incision must include the meatal-based flap.
It is important to note that in hypospadias
with superficial chordee, the distance between
the tip of the glans and the hypospadias meatus –
called the urethral plate – remains the same,
while the distance between the hypospadias
meatus and the base of the penis becomes longer
as the penis is straightened and the meatus moves
forward and away from the skin attachment. This
has prompted some authors to claim that release
of the skin chordee will change the hypospadias
classification from proximal to distal. It remains
clear, however, that any skin incision proximal to
the hypospadias meatus can never change the
length of the untouched urethral plate between
the tip of the glans and the meatus (Fig. 8.29).
The resulting skin defect can usually be covered Fig. 8.39 The tunica albuginea is formed of inner circu-
by approximating the lateral skin edges. lar and outer longitudinal layer (©Ahmed T. Hadidi 2022.
8.2.13.4 unica Albuginea Externa
T
Excision (TALE) technique using a sharp tenotomy scissor
The principle of the TALE procedure is based on (Fig. 8.41). This is enough in more than 90% of
the anatomical fact that the tunica albuginea is cases presenting less than 2 years of age (Fig. 8.42).
formed of an outer longitudinal layer and an inner
circular layer (Fig. 8.39). Darewicz, using the elec- Assessment of Curvature After the TALE
tron microscope, showed disorganized abnor- Procedure
mal arrangement of the outer longitudinal collagen After ventral degloving and excision of the outer
fibers. The collagen fibers where irregular in shape layer of tunica albuginea (TALE), artificial erec-
and cross section on the ventral aspect of the penis tion test is performed to exclude corporeal dis-
(Fig. 8.15). If the surgeon operates during the first proportion. We prefer to apply pressure in the
9 months of life, there is a good chance that the perineum and not a tourniquet to detect any cur-
child will not have corporeal disproportion and that vature at the base of the penis.
he can correct the chordee by incising (fairy cuts or If there is still curvature less than 20°, we have
superficial corporotomies) or excising the outer lon- experienced that it gets better through fixation to
gitudinal layer (TALE procedure) [50]) (Fig. 8.40). the abdominal wall.
The composite nature of the tunica albu- If the curvature is more than 20°, we proceed
ginea has been utilized by several surgeons to to divide the corpus cavernosum at the angle of
shorten the dorsal convex surface of the penis maximum curvature and put a tunica vaginalis
by incising the outer longitudinal layer of the flap from the scrotum.
tunica albuginea only on the dorsum of the
penis [51, 52]. Tunica Vaginalis Flap (TVF)
If the tethering and stoutness is distal to the The tunica vaginalis flap (TVF) is obtained from
meatus, we divide the urethral plate and excise the the thick, white, outer covering of the testis (pari-
longitudinal layer of tunica albuginea according to etal layer of tunica vaginalis), mobilized with its
the tunica albuginea externa excision (TALE) blood supply and fascia (Fig. 8.43) and used to
214 A. T. Hadidi
Fig. 8.40 Details of the TALE technique to correct the TALE technique (©Ahmed T. Hadidi 2022. All Rights
severe deep chordee in a perineal hypospadias. Notice that Reserved)
the penis has doubled its size after chordee excision using
Fig. 8.41 Technique of excision of the tunica albuginea of the penis. It is possible to identify the right level of dis-
externa excision (TALE). It is useful to use a sharp tenot- section, when you see the glistening corpus cavernosum
omy scissor with serrated edge and excise the longitudinal with the deep furrow in between the two corpora caverno-
layer of tunica albuginea by holding with a fine surgical sum (©Ahmed T. Hadidi 2022. All Rights Reserved)
forceps and having the scissor along the longitudinal axis
Fig. 8.42 A patient with perineal hypospadias and severe procedure was performed. The penis was fixed to the
chordee. The patient received hormonal therapy to enlarge abdominal wall with 3/0 Prolene suture to keep the penis
the penis. Notice the increase in the length of the penis straight and stretched for 3 weeks. The patients do not
from 2.3 cm before chordee correction to 4.5 cm after experience pain with the penis fixation. Proximal urethro-
TALE procedure to correct the chordee. Erection test con- plasty was performed 3 months later (©Ahmed T. Hadidi
firmed the complete straightening of the penis. CEDU 2022. All Rights Reserved)
cover the defect in the corpus cavernosum after cedure, but the same surgeons will refrain from
incision at the angle of chordee (Fig. 8.44). doing so if they plan to perform foreskin recon-
Many surgeons tend to perform a complete struction. It is recommended that hypospadias
degloving of the penis as the first step of the pro- surgeons refrain from performing complete
216 A. T. Hadidi
Fig. 8.43 (a–c) The tunica vaginalis flap. The flap should ented Anatomy”, 5th edn, p223, ©2005, with permission
be at least 1.5 × 1 cm ((a, c) ©Ahmed T. Hadidi 2022. All from Wolters Kluwer Health Inc. [50])
Rights Reserved, (b) from Moore, Dalley: “Clinically ori-
Fig. 8.44 The steps of the tunica vaginalis flap (TVF) for ginea at the point of maximum curvature and application
correction of corporeal disproportion. After extensive of the tunica vaginalis flap (TVF) taken from the scrotum
degloving of the penis and tunica albuginea externa exci- around the testis along with its fascia (©Ahmed T. Hadidi
sion, the curvature persisted 60 degrees. This necessitated 2022. All Rights Reserved)
division of the deeper circular layer of the tunica albu-
218 A. T. Hadidi
degloving as a routine in hypospadias surgery representing approximately 4 to 10% of cases of

before assessing each individual child carefully. congenital chordee [15]. The etiology and man-
agement of this condition remain a subject of
8.2.13.5 Abdominal Wall Fixation debate in the literature.
At the end of the TALE or TVF procedure, the Young advocated that chordee without hypo-
penis is fixed to the anterior abdominal wall with spadias was due to a congenitally short urethra
a 3/0 Prolene stitch for 3 weeks (Fig. 8.45). This and that the urethra should be transected [55].
helps to stretch the penis and keep it straight. At Devine and Horton postulated that chordee with-
the beginning, we were skeptical if the child may out hypospadias resulted from dysgenesis of fas-
experience pain. However, experience in children cia surrounding the urethra, a concept that has
as well as in adults who underwent proximal re-emerged today with the disorganization theory
hypospadias correction showed that the patient [56]. They classified chordee into three grades
does not experience pain in relation to the stay according to the layers affected, namely, grade I
suture. Proximal urethroplasty is performed (with deficient corpus spongiosum, dartos, and
3–6 months later. Buck fascia), grade II (with deficient dartos and
There are two reasons for fixing the penis after Buck fascia), and grade III (with only dartos fas-
correction of the severe chordee under stretch using cia affected). Kramer et al. found that corporal
a 3/0 Prolene suture to the anterior abdominal wall; disproportion was another principal cause of
the first is that the penis was severely curved for chordee, and they classified this condition as
many months, and it is helpful to avoid recurrence group IV chordee without hypospadias [41]. In
of the chordee by keeping the penis under stretch 1982, Kramer et al. recognized that corporeal
until it heals in the stretched straight position. The disproportion was an important cause of isolated
second reason is that when we reconstruct the new chordee. They recommended that dorsal corpo-
urethra, we reconstruct it under stretch with the real plication be performed according to the
stay suture, and it is important to keep the new ure- Nesbit principle to correct this type of chordee
thra under stretch and avoid shrinkage and kinking without hypospadias. However, others suggested
during healing (Fig. 8.46). Interestingly, children that elongation of the ventral corporeal bodies
and adults do not feel any pain or discomfort from with graft material was superior to plication of
the stay suture. It is, however, inconvenient in the dorsal corporeal bodies in severe penile cur-
adults as the urine will go toward the face unless vature. In 1992, Hendren and Caesar reviewed
the man is having a suprapubic catheter. their experience with isolated chordee and found
that 23 of 33 patients required a ventral dermal
graft and/or urethral division and lengthening to
8.2.14 Chordee Without achieve penile straightening [57].
Hypospadias and Paper-Thin The Devine and Horton classification system
Urethra had been widely adopted until 1998 [58], when
Donnahoo et al. presented their series of 87
It occurs when the skin and/or dartos fascia, patients with penile curvature without hypospa-
Buck’s fascia, and corpus spongiosum are dias [59]. In their series, the etiology of isolated
replaced with fibrous dysgenetic tissue. The ure- chordee was evenly distributed among skin tether-
thra is normal unless the corpus spongiosum is ing (32%), fibrotic Buck’s and dartos fasciae
also involved. In such a case, the urethra is paper- (33%), and corporeal disproportion (28%). A con-
thin (the disorganized defected distal migration genitally short urethra rarely caused chordee
involved the fascial coverings rather than the ure- without hypospadias (7%). Cases of a paper-thin
thra). Occasionally, the urethra itself may be hypoplastic distal urethra (type I) were excluded
short as well (Figs. 8.47 and 8.48). from study, since they were considered hypospa-
Isolated chordee without hypospadias was diac variants, and were managed with excision
first described by Siever in 1926 [54]. It is rare, and reconstruction of the abnormal urethral
Fig. 8.45 Fixation of the penis at the end of perineal under stretch. The Prolene suture should remain for 3
hypospadias operation, using 3/0 Prolene. The needle weeks whenever possible (©Ahmed T. Hadidi 2022. All
goes from inside the glans in the midline into the lower Rights Reserved)
abdominal crease (to reduce scarring) to fix the penis
220 A. T. Hadidi
Fig. 8.46 Fixing the penis after correction of severe chordee under stretch using a 3/0 Prolene suture to the anterior
abdominal wall (©Ahmed T. Hadidi 2022. All Rights Reserved)
Fig. 8.47 Chordee without hypospadias: chordee may without hypospadias can be more difficult and challeng-
occur despite having the urethral meatus at the tip of the ing than correcting hypospadias alone (©Ahmed T. Hadidi
glans. However, the prepuce is usually incomplete and the 2022. All Rights Reserved)
median raphe may be abnormal. Correction of chordee
s egment. They proposed a systematic approach to From a surgical point of view, it is difficult
the surgical management of chordee without during surgery to identify if the disorganization
hypospadias and divided this anomaly into four and dysgenesis involves dartos fascia alone or
groups according to structural defect and surgical with Buck’s fascia as the tissues are amalgamated
steps. Their treatment algorithm helps to define together in a single dysplastic tissue. Therefore, it
the cause of isolated chordee in a stepwise fashion seems more appropriate to classify chordee with-
and thus minimizes the need for risky urethral out hypospadias as is the case with hypospadias
replacement. into following grades:
Fig. 8.48 A rare case of chordee without hypospadias under anesthesia, one can see that the urethra is intact
associated with what may be wrongly called “congenital although the corpus spongiosum is missing at a certain
urethral fistula”: The surgeon may think that the child has point on the shaft of the penis (©Ahmed T. Hadidi 2022.
a fistula. Only after introducing a sound inside the urethra All Rights Reserved)
• Grade I: Superficial Chordee: when the dis- 8.2.15 Glanular Tilt

organization and dysgenesis involves only
the tissues superficial to the urethra (man- This is a common finding in patients with chordee.
aged as superficial chordee with ventral It may present with or without hypospadias and is
degloving). due to the abnormal attachment of the amalgam-
• Grade II: Deep Chordee: when the disor- ated fascial and spongiosal tissue to the base of the
ganization involves the tissues deep to the glans. Liberal mobilization of the glanular wings
urethra (managed as severe chordee using (or partial disassembly of the glans) is important in
the TALE technique but preserving the the correction of the glanular tilt (Fig. 8.49).
urethra).
• Grade III: Chordee with corporeal dispro-
portion: when there is corporeal disproportion 8.2.16 General Principles of Chordee
(managed using TAF technique). Correction
• Grade IV: Chordee with short urethra: when
the urethra is also short and needs to be In general there are four approaches to correct
divided. chordee after degloving of the penis; either to (a)
222 A. T. Hadidi
Fig. 8.49 Glanular tilt may be considered a part of the help to correct the chordee in some cases without having
wide spectrum of chordee. It may present as an isolated to divide the urethral plate (©Ahmed T. Hadidi 2022. All
thing (tilt without hypospadias) or in association with Rights Reserved)
hypospadias. Correction of the glanular tilt alone may
a b
Fig. 8.50 There are two approaches to correct chordee cal) and (b) to shorten the dorsal surface of the penis
that persists after extensive degloving of the penis: (a) to (©Ahmed T. Hadidi 2022. All Rights Reserved)
lengthen the ventral surface of the penis (more physiologi-
lengthen the ventral side of the penis, (b) to 1. Techniques That Lengthen the Ventral Side of
shorten the dorsal side of the penis, (c) penile dis- the Penis
assembly technique and urethral mobilization, or (a) Tunica albuginea externa excision
(d) split and roll technique (Fig. 8.50). (TALE).
(b) Fairy cuts and multiple corporotomies. STAG and STAC Procedure
(c) STAG and STAC procedures. Snodgrass et al. suggested the STAG (staged
(d) Incision of tunica albuginea and the use tubularized autograft) where there are three
of grafts or tunica vaginalis flap (TVF). deep transverse incisions deep into the spongy
2. Techniques That Shorten the Dorsal Side of tissue of the corpora cavernosa; the middle is
the Penis at the point of maximum curvature extending
(a) Nesbit procedure. from 4 to 8 o’clock [62]. The other two inci-
(b) Tunica albuginea plication (TAP). sions are made 4 mm proximal and distal to
(c) Heineke-Mikulicz technique. middle incision. In complicated recurrent
3. Penile Disassembly Technique cases, the authors suggested to perform the
4. Split and Roll Technique STAC technique (STraighten And Close) per-
forming three transverse incisions as described
8.2.16.1 Techniques That Lengthen earlier and then cover the open corpora caver-
the Ventral Side of the Penis nosa with penile skin and fascia. However,
these approaches have high risk of herniation,
Tunica Albuginea Longitudinal Excision diverticulum formation, and erectile dysfunc-
(TALE) tion in adulthood as the deep incisions into the
This is the standard technique in the Hypospadias spongy tissue are similar to what happens in
Center in Frankfurt and is already described fracture penis. It is worthy to mention that the
before. principle of “straighten and close” was the
standard approach since Duplay in 1880 [63]
Fairy Cuts and continued through the years and modified
Devine first described fairy incisions (short, by Byars in 1951 [49]. The difference is essen-
superficial transverse cuts) on the ventral surface tially in the technique used for straightening of
of tunica albuginea in an attempt to correct per- the penis.
sistent ventral curvature [58]. It seems that their
aim was to incise the outer longitudinal layer of Incision of Tunica Albuginea and the Use
tunica albuginea, leaving the inner circular layer of Grafts or Flaps
intact; hence there was no need for grafting. Pippi For older children with corporeal disproportion,
Salle et al. applied the same principle and per- there is a need to incise both layers of the tunica
formed three transverse incisions into the outer albuginea and cover the defect with grafts or
tunica albuginea (TITA) between 3 and 9 o’clock tunica vaginalis flap. Horton and Devine experi-
without entering into the spongy tissue of the cor- mented with different methods until they reported
pora cavernosa and then applied a preputial graft the safe use of dermal grafts taken from non-
(Fig. 8.51) [60, 61]. hairy inguinal crease skin [64].
Fig. 8.51 Transverse incisions of tunica albuginea (TITA). The incisions do not go deep into the spongy tissue of the
corpus cavernosum. The patient received androgen hormone therapy before surgery (courtesy of Dr. Pippi Salle)
224 A. T. Hadidi
had severe hypospadias. The dermal grafts were

Important technical tip is to mobilize and harvested from the hairless skin of the inguinal
incise the urethra plate at the subcoronal region and were used to fill a tunical defect cre-
level and excise all the disorganized dys- ated by incising the tunica at the point of maxi-
plastic tissue. The urethral plate will retract mal penile curvature. At a mean follow-up of
proximally, and the tunica albuginea is 27 months, all patients had excellent cosmetic
incised at the level of maximum curvature and functional results, and the investigators con-
between 3 and 9 o’clock. This results in an cluded that in patients with significant residual
oval defect. It is important to have fascia or chordee, a dermal graft provides a straight phal-
local vascularized tissue between the graft lus with a minimal complication rate.
and the tissue used to reconstruct the new
urethra. Dermal grafts, pericardium, dura, 8.2.16.3 he Use of Small Intestinal
T
SIS, buccal mucosa, tunica vaginalis grafts, Submucosa (SIS)
or flaps have been used with variable Several studies report the use of small intestinal
degrees of success. submucosa (SIS). SIS is a commercially available,
acellular, collagen-based, xenogenic graft bioma-
terial derived from the submucosal layer of the
8.2.16.2 The Use of Dermal Grafts porcine small intestine. It has the advantage of
Dermal grafts harvested from non-hair-bearing availability, decreased operative time, and no
inguinal skinfolds were placed in 51 patients donor site morbidity, but it is relatively expensive
during a 5-year period; 36 of these patients had and not available in all countries. The outcome
penoscrotal or perineal hypospadias. The authors varies from excellent investigator satisfaction with
(Lindgren et al.) concluded that a single dermal one-layer SIS to variable satisfaction or even dis-
graft is sufficient in 57% of cases [65]. In cases appointment with four-layer SIS grafts. Leslie
of incomplete straightening, they placed an addi- et al. reported on their experience with SIS, der-
tional graft or performed dorsal plication. They mal, and tunica vaginalis grafts [67]. They reported
believe that additional penile length achieved one SIS case of severe fibrosis at the graft site,
with dermal grafting results in a straight penis requiring excision and repeat grafting. Soergel
and that the cosmetic outcome is preferable to et al. reported two major complications with four-
that with plication alone (Figs. 8.52 and 8.53). layer SIS and stopped using SIS completely [68].
Pope et al. reported their experience with the
use of dermal grafts for the correction of signifi- 8.2.16.4 The Use of Buccal Mucosa
cant residual chordee during a 5-year period [66]. Mokhless et al. reported their preliminary experi-
Fifty-one patients were reviewed, of whom 41 ence with the use of buccal mucosa as a graft to
Fig. 8.52 The technique of dermal grafting. After exten- to use methylene blue to color the epidermis and ensure
sive degloving, the circular deep layer of the corpora cav- complete removal of the epithelium using a sharp scissor
ernosa is incised at the level of maximum curvature and a or scalpel. When possible, suturing the middle of the graft
dermal graft from the groin is sutured in a continuous from inside to the intracorporeal septum may reduce the
watertight manner. It is recommended to de-epithelialize chances of ballooning of the graft (©Ahmed T. Hadidi
the graft before taking the graft off the groin. It is helpful 2022. All Rights Reserved)
a b c
Fig. 8.53 (a) A child with chordee >45. (b) After apply- second stage procedure, the penis is completely straight
ing a large dermal graft. It is important to expect about (courtesy of Dr. Mark Zaontz)
30% shrinkage of the graft after healing. (c) During the
cover the tunica albuginea defect in 12 children stent or suprapubic tube remained in place. A
ranging from 4 to 8 years [69]. The non-fatty side compressive Coban® dressing was wrapped
around the penis for 24–48 h.
was sutured facing the cavernous tissue (i.e., the
smooth surface was toward the urethra). The size They reported the short-term outcome of ven-
of the graft was 25% larger than the defect as is tral penile lengthening using tunica vaginalis
the standard with graft application. The neo- flaps alone for correcting severe chordee with a
urethra was reconstructed as a second stage 95% success rate. Dural grafts were associated
6 months after grafting. They reported excellent with a higher risk of recurrent ventral curvature
results with a straight penis in ten children and compared to tunica vaginalis flaps. The use of a
residual chordee of less than 10 degrees in two tunica vaginalis flap has become their technique
children, requiring no further management. The of choice for correcting severe ventral
follow-up was for 1 year. curvature.
An experimental study in rabbits showed that
8.2.16.5 unica Vaginalis Grafts or
T grafts were associated with necrosis and contrac-
Tunica Vaginalis Flap (TVF) ture in 42% of cases, while no contraction was
Braga et al. reported their experience with inci- observed in any TVF specimens [71].
sion of the ventral tunica albuginea and applying Tunica vaginalis grafts were first used for cor-
grafts (dermis, dura, or pericardium) or tunica recting penile curvature in adults with Peyronie’s
vaginalis flap (TVF) in 38 patients [70]. They disease [72]. It has subsequently been utilized in
recommended that: chordee correction in children with severe hypo-
when using a graft, attention was given to har- spadias [73]. However, the reported results of this
vesting a patch at least 20% to 30% larger than technique have been contradictory. Caesar and
the defect to prevent contracture induced recur- Caldamone reported on 28 patients undergoing
rent curvature. If a TVF was used, the tunica vagi- primary and secondary repairs for chordee [74].
nalis was opened transversely on its distal and
anterior aspect. Two longitudinal incisions were They found that 60% of their patients treated
made on each side of the initial transverse inci- with a TVF had recurrent VC. Additionally,
sion to create a rectangular flap, preserving the Vandersteen and Husmann identified late onset
cord and the posteriorly based blood supply origi- recurrent chordee after prior successful hypospa-
nating from the cremasteric artery. The flap or
graft was then anastomosed to the tunica albu- dias repair using tunica vaginalis grafts [75].
ginea to cover the underlying corporeal defect As mentioned before, TVF is the standard
with a running, locking, double armed 5-zero technique used in the Hypospadias Center in
polydioxanone suture. All patients were given Frankfurt to correct corporeal disproportion
intravenous cephazolin before skin incision. Oral
antibiotics were continued as long as a urethral without a single failure.
226 A. T. Hadidi
Fig. 8.54 Schematic

presentation of different
approaches of dorsal
plication (©Ahmed T.
Hadidi 2022. All Rights
Reserved)
8.2.16.6 Techniques That Shorten In simple plication, excessive infolding of

the Dorsal Side of the Penis tunica can result in the narrowing of the corporal
(Fig. 8.54) lumen, which can lead to decreased rigidity dis-
tally. In some cases, the stitches did not hold long
Nesbit Procedure enough, resulting in recurrence of curvature. In
Nesbit first tried plication without incision on a incisional techniques, edges of incision heal
medical student with lateral chordee. Unfor tightly after closure and remain permanently
tunately, the curvature recurred in less than closed. Here, the tunica tissue can be folded
6 months. Then he excised horizontal ellipses inward, but to a lesser degree. In severe curvature,
with the help of an Allis forceps. He approxi- this can result in decrease of cavernous volume. In
mated the fascial edges with buried sutures of elliptic excision techniques, the tunica does not
heavy silk [76]. infold. If excessive excision of tunica albuginea is
needed, reduction of cavernous volume can be
Tunica Albuginea Plication (TAP) significant and tunica elasticity can be reduced.
Duckett incised Buck’s fascia at 2 and 10 o’clock
in the area of maximum bending to either side of Heineke-Mikulicz Technique
the neurovascular bundle [77]. He then incised Originally, the principle of Heineke-Mikulicz
the tunica albuginea and closed using 5/0 poly- was described for pyloric stenosis where a longi-
propylene burying the knot in what he called the tudinal incision is made and is closed transversely
TAP (tunica albuginea plication) (Fig. 8.55). (pyloroplasty). This principle is now widely
Baskin et al. examined a 33-week fetal penis applied in the correction of strictures in general.
with distal hypospadias and compared it to nor- Yachia applied the Heineke-Mikulicz princi-
mally developing penises and noticed that in all ple to straighten penile curvature instead of using
these specimens, the neurovascular bundle is not the Nesbit corporoplasty technique in 10 patients
present at the 12 o’clock position, rather splaying (Fig.8.56) [78].
out laterally from the 11 and 1 o’clock positions By horizontally closing the longitudinal inci-
ventrally to the spongiosum [12]. They recom- sions, the longer portion of the tunica albuginea
mended positioning the dorsal plicating suture in is made equal in length to the shorter side. He
the midline to avoid injury to the neurovascular believed that this technique would achieve the
bundle. Absorbable sutures can be used but same results as the Nesbit corporoplasty in a sim-
slowly absorbed PDS (polydioxanone) is pler manner. Because of the distance between the
preferable. longitudinal incision and the neurovascular bun-
Fig. 8.55 The TAP

technique of dorsal
plication (©Ahmed T.
Hadidi 2022. All Rights
Reserved)
dle or corpus spongiosum, these structures are cases treated with this technique is presented.
less susceptible to injury during an operation and Yucel et al. used the same principle to correct
need not be mobilized. Our experience with 10 recurrent chordee [79].
228 A. T. Hadidi
elliptical tunica albuginea excisions and geomet-

rical principles for correcting biplanar congenital
penile curvature. They reported 98% success rate
and mean loss of penile length was 0.7 cm (range
0.3–0.9 cm).
8.2.17 D
oes Dorsal Plication Produce
Shortening of the Penis?
In order to answer this question objectively, one

has to measure the length of the penis and degree
of curvature after degloving. Then, he should
apply dorsal placation and measure the length of
the penis again. Then he should undo the dorsal
placation, incise the urethral plate and excise the
hypoplastic tissues on the ventral surface, apply
the erection test, and measure the length of the
penis again. No study has performed such an
Fig. 8.56 The Heineke-Mikulicz technique of dorsal pli-
cation. Here a longitudinal 1 cm incision is made at 12
objective test to examine the degree of shortening
o’clock, and an inverting stitch is used to shorten the dor- caused by dorsal placation. Most available studies
sal surface of the penis. Notice that the total length of the compare the original size of the penis with chordee
shaft of the penis in an infant without the glans is about 2 to the size of the penis after dorsal plication [81].
cm (©Ahmed T. Hadidi 2022. All Rights Reserved)
Dorsal plication was popular in the 1990s and
early 2000s by surgeons who wanted to promote
Nyirády et al. reported their long-term results
techniques that rely on preservation of the ure-
with 3 techniques of dorsal shortening in 87
thral plate. These authors suggest to make 1 cm
patients with a mean age 24 years [80]. Nesbit’s
midline incision and then plicate with a single
procedure was employed in 18, plication in 7, and
stitch [82]. These studies claim that dorsal placa-
the Heineke-Mikulicz technique in 62 men. The
tion reduces the penile length between 2 and
mean follow-up was 89 months. They concluded
5 mm only. The following issues have to be con-
that in their 20-year experience, chordectomy has
sidered when analyzing these studies:
always reduced the curvature but patients could
1. The average length of the penis is 3 cm in
never completely straighten the penis by them-
boys less than 2 years of age (see Chap. 4:
selves. Surgical correction was reported by
Surgical Anatomy). If one deducts the dorsal
patients to be highly successful using the Heineke-
length of the glans of 1.4 cm, it becomes clear
Mikulicz technique, and statistical analysis
that those surgeons shorten more than half the
revealed it to be significantly better than plication
length of the body of the penis.
or the Nesbit procedure in terms of palpable nod-
2. If plication really shortens the dorsal surface
ules, recurrence, and overall satisfaction.
with 2–5 mm only, it can only help in mild
Perdzyński and Adamek described good short-
chordee where no plication is actually needed.
and long-term results of applying the Heineke-
3. Those studies compare the length of the
Mikulicz principle on the external longitudinal
curved penis before plication (already short-
layer of the tunica albuginea [52].
ened by the hypoplastic tissue and chordee)
Kuehhas and Egydio described the superficial
with the length after plication. In order to
tunica albuginea geometric-based excision
measure the true effect of shortening, they
(STAGE) technique in 145 patients [51]. The
should compare the penile length after plica-
technique is based on multiple, small, superficial
tion and compare it with the penile length
Fig. 8.57 A 24-year-old man presenting 20 years after was 8 cm long only. On exploration, one can see the per-
Nesbit procedure with pain with erection and his partner sistent chordee, the hypoplastic Buck’s fascia, and tunica
was complaining of short inadequate penetration. On albuginea and the resultant short urethra (©Ahmed T.
erection test, the penis looked rather straight and the penis Hadidi 2022. All Rights Reserved)
Fig. 8.58 The Perovic technique of penile disassembly. The urethra is completely and freely mobilized including the
glans and the two vascular pedicles and then re-sutured again (courtesy of late Prof. Sava Perovic)
after excising the hypoplastic tissue on the the middle (Fig. 8.58). This disassembly tech-
under surface of the penis. nique seems to be a most effective procedure in
4. An example of the long-term effect of dorsal selected cases of severe curvature of the penile
plication is presented in a 24-year-old man shaft, marked glans tilt, and a small penis. Perovic
who complained of pain with erection and his suggested that penile disassembly combined with
partner complained of inadequate penetration extensive urethral mobilization may correct the
(Fig. 8.57). hypospadiac meatus; therefore, there may be no
need to construct a new urethra.
8.2.17.1 Penile Disassembly Technique Perovic recommended to start the dissection
Perovic et al. described a penile disassembly from the ventral side of the penis over the tips of
technique to avoid penile shortening in curvature the corpora cavernosa toward the dorsal side.
repair [83]. They used the technique in 87 patients This step allows for complete preservation of the
ranging from 12 months to 47 years old (mean neurovascular bundle, which cannot be achieved
4.5 years) between 1995 and 1997. The method by initiating dissection from the dorsal aspect.
consisted of complete disassembly of the penis: Buck’s fascia is released from the underlying
the glans cap with its neurovascular bundle dor- albuginea to lift the urethra, urethral plate, and
sally; the urethra ventrally, or with coexisting neurovascular bundle without injury. These
hypospadias the urethral plate; and the corpora maneuvers provide the maximal potential for cor-
cavernosa, which may be partially separated in recting curvature, especially when the curvature
230 A. T. Hadidi
Fig. 8.59 Another example of the Perovic disassembly technique. Bhat has applied the same principle without dissect-
ing the glans off the corpora cavernosa and called it total urethral mobilization (courtesy of late Prof. Sava Perovic)
exists in the distal one-third of the corpora caver- disassembly, partial ventral separation of the cor-
nosa. After correction of all deformities, the penis pora in the septal area is performed.
is reassembled (Fig. 8.59).
8.2.19 Dissection
8.2.18 Dissection of the Urethral of the Neurovascular Bundle
Plate/Urethra
Dissection is continued close to tunica albu-
Urethral separation begins with dissection of the ginea over the tips of corpora cavernosa toward
urethra along with its adjacent spongy tissue. The the dorsum of the penis, beneath the neurovas-
urethra is dissected from the corporal bodies cular bundle and Buck’s fascia. Therefore, dis-
beginning laterally in the healthy area of Buck’s section must be meticulous and performed as
fascia. Proximally, the urethra (along with its bul- closely to the albuginea as possible. During this
bous portion) is lifted with Buck’s fascia. Since step, injury to the albuginea may occur but does
the distal urethra is wide, thin, and adherent to not compromise the technique or its outcome. It
the cavernous bodies, its dissection is performed is important to mind the course of the neurovas-
laterally and as close as possible to the cavernous cular bundle near the glans in order to avoid
bodies. The next crucial step is the separation of injury (Fig. 8.60).
the distal portion of the urethra (or the urethral
plate in chordee with hypospadias) and lifting of
the glans. The glans cap and tips of the corpora 8.2.20 Total Urethral Mobilization
cavernosa are in close contact with each other in
this region. In order to prevent glans ischemia, it The disassembly principle of Perovic was modi-
is important to preserve both penile arteries that fied by Bhat to be employed only on the ventral
run ventrolaterally before entering the glans. aspect of the penis in what he described as “total
Surgery is performed without a tourniquet, which mobilization of the urethra” [84]. Bhat suggested
also prevents glans ischemia. In cases of chordee total mobilization of the urethra and the urethral
with hypospadias, the urethral plate is lifted, plate from the glans down to the bulbar urethra
along with its vascularized fibrous tissue, in order in an attempt to correct chordee. He reported his
to ensure better blood supply to the plate. Further experience in 34 patients with proximal and
distal dissection includes preservation of the two scrotal hypospadias and suggested 88% success
vascular and fibrous pillars that are inserted into rate. This approach has to be considered with
the undersurface of the glans. At the end of penile extreme caution because of the potential risk of
Fig. 8.60 The technique of dissection and preservation of the neurovascular bundle in the Perovic disassembly tech-
nique (courtesy of late Prof. Sava Perovic)
Fig. 8.61 The split and roll technique (©Ahmed T. Hadidi 2022. All Rights Reserved)
devascularization and necrosis of the urethral area of maximal curvature. Working from the
plate as experienced by Snodgrass [85]. dorsal lateral aspect so that the corporal bodies
are rotated toward the dorsal midline, this method
does not require incision into the corporal sub-
8.2.21 Split and Roll Technique stance, involved no use of grafts, and does not
cause shortening of the phallus. The neurovascu-
Koff first described dorsal corporal rotation as a lar bundle is reported to be preserved and not
technique to correct chordee [86]. Chordee cor- compressed by rotational sutures [88].
rection by corporal rotation (“the split and roll
technique”) was also reported by Kass [87] and
Decter [88] as an alternative approach in the cor- 8.2.22 Recurrent/Persistent Chordee
rection of severe chordee (Fig. 8.61). Division of in Adolescents and Young
the urethral plate and partial splitting of the sep- Adults (Fig. 8.62)
tum between the corpora cavernosa with a ventral
midline incision facilitates corporal rotation. Many of the patients who underwent dorsal plica-
Access is gained to the dorsal aspect of the cor- tion in the 1990s and 2000s presented later at
pora cavernosa by dissecting Buck’s fascia with puberty or early adulthood with recurrent/persis-
its encased neurovascular bundle. After artificial tent chordee [75]. It is not appropriate to correct
erection, non-absorbable sutures are placed in the one pathology by creating another and shorten an
232 A. T. Hadidi
a b
Fig. 8.62 (a) A 14-year-old boy presented with what fistula, and he was also circumcised. The first step of the
looks like distal hypospadias and chordee. Examination operation was to completely excise all the scarred and
under anesthesia confirmed the presence of severe chor- unhealthy tissue. It was clear from the start that the patient
dee. TALE procedure was performed, but there was still would require a two-stage operation with excision of the
corporeal disproportion requiring incision of the corpora scar and application of buccal mucosal grafts from both
cavernosa and grafting. (b) A 21-year-old patient present- inner cheeks in the first operation (©Ahmed T. Hadidi
ing with severe recurrent chordee and proximal urethral 2022. All Rights Reserved)
already short penis. An atretic hypoplastic ure- paper on recurrent chordee and the majority of
thral plate must be excised as it does not grow those patients underwent dorsal plication in com-
along with the growth of the penis and the sutures bination with TIP or onlay flaps for hypospadias
that are used in childhood do not withstand the correction [91].
pressure exerted during erection. Acimi [89] and In the editorial comment after the onlay flap in
Huang [90] believe that the reappearance of cur- the first edition of this book (2004), I mentioned
vature a few years after onlay and other tech- clearly that 80% of patients with proximal hypo-
niques preserving the atretic urethral plate is due spadias who presented to me had severe chordee
to poor growth of the dysplastic urethral plate. requiring division of the urethral plate and exci-
Braga et al. compared ventral penile lengthening sion of the dysplastic tissue.
versus dorsal plication of severe chordee and Penile chordee may present in adulthood.
found the recurrence of chordee after dorsal pli- This may be due to incomplete chordee correc-
cation 36.5% as compared with 0% after ventral tion during childhood, recurrent chordee after
lengthening [47]. Pippi Salle et al. reviewed 140 dorsal plication, or due to severe scarring.
patients with proximal hypospadias who under- Management follows the same protocol that was
went 3 different techniques [61]. About 50% who mentioned before namely degloving and exci-
underwent dorsal plication came back with recur- sion of scarring (this is probably the most impor-
rent chordee. Abosena et al. wrote an excellent tant step and should be done meticulously. Many
patients have been through several operations, 14. Nordenvall AS, Frisén L, Nordenström A,
and the majority would not have abundant Lichtenstein P, Nordenskjöld A. Population based
nationwide study of hypospadias in Sweden, 1973 to
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and two-stage repair. This is an important topic doi.org/10.1016/j.juro.2013.09.058.
and is discussed in more detail in the chapters on 15. Yachia D, Beyar M, Aridogan IA, Dascalu S. The
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https://doi.org/10.1016/S0022-5347(17)35816-0.
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visual inspection at estimating ventral penile curva- 57. Hendren WH, Caesar RE. Chordee without hypospa-
ture on plastic models. 2019; https://doi.org/10.1016/j. dias: experience with 33 cases. J Urol. 1992; https://
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using an app. J Pediatr Urol. 2020;16(4):437.e1–437. 59. Donnahoo KK, Cain MP, Pope JC, Casale AJ, Keating
e3. https://doi.org/10.1016/j.jpurol.2020.04.027. Epub MA, Adams MC, Rink RC. Etiology, management and
2020 May 7. PMID: 32430211. surgical complications of congenital chordee without
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M, Schlomer BJ. Do adult men with untreated S0022-5347(01)62713-7.
ventral penile curvature have adverse outcomes? 60. Leslie B, Weber B, Romao R, Farhat W, Bagli D,
J Pediatr Urol. 2016; https://doi.org/10.1016/j. Lorenzo A, Pippi Salle J. 546 an alternative for ventral
jpurol.2015.09.009. lengthening in children with severe penile curvature
45. Sokolakis I, Hatzichristodoulou G. Current trends that allows graft coverage during staged hypospa-
in the surgical treatment of congenital penile curva- dias repair. J Urol. 2011; https://doi.org/10.1016/j.
ture. Int J Impot Res. 2020; https://doi.org/10.1038/ juro.2011.02.1271.
s41443-019-0177-0. 61. Pippi Salle JL, Sayed S, Salle A, Bagli D, Farhat
46. Bologna RA, Noah TA, Nasrallah PF, McMahon W, Koyle M, Lorenzo AJ. Proximal hypospadias:
DR. Chordee: varied opinions and treatments as a persistent challenge. Single institution outcome
documented in a survey of the American Academy of analysis of three surgical techniques over a 10-year
Pediatrics, Section of Urology. Urology. 1999; https:// period. J Pediatr Urol. 2016; https://doi.org/10.1016/j.
doi.org/10.1016/S0090-4295(98)00656-6. jpurol.2015.06.011.
47. Braga LHP, Lorenzo AJ, Bägli DJ, Dave S, Eeg K, 62. Snodgrass W, Bush N. Staged tubularized auto-
Farhat WA, Pippi Salle JL, Khoury AE. Ventral graft repair for primary proximal hypospadias with
30-degree or greater ventral curvature. J Urol. 2017; 77. Baskin LS, Duckett JW. Dorsal tunica albuginea
https://doi.org/10.1016/j.juro.2017.04.019. plication for hypospadias curvature. J Urol. 1994;
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64. Devine CJ, Horton CE. Use of dermal graft to cor- ment of penile curvature. J Urol. 1990; https://doi.
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S0022-5347(17)59406-9. 79. Yucel S, Sanli A, Kukul E, Karaguzel G, Melikoglu M,
65. Lindgren BW, Reda EF, Levitt SB, Brock WA, Franco Guntekin E. Midline dorsal plication to repair recur-
I. Single and multiple dermal grafts for the manage- rent chordee at reoperation for hypospadias surgery
ment of severe penile curvature. J Urol. 1998; https:// complication. J Urol. 2006; https://doi.org/10.1016/
doi.org/10.1016/S0022-5347(01)62715-0. S0022-5347(05)00186-2.
66. Pope JC IV, Kropp BP, McLaughlin KP, Adams 80. Nyirády P, Kelemen Z, Bánfi G, Rusz A, Majoros
MC, Rink RC, Keating MA, Brock JW. Penile A, Romics I. Management of congenital penile
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setting. Urology. 1996; https://doi.org/10.1016/ juro.2007.11.059.
S0090-4295(96)00097-0. 81. Greenfield JM, Lucas S, Levine LA. Factors affecting
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Misseri R, Rink RC. Corporeal grafting for severe cation for correction of penile curvature. J Urol. 2006;
hypospadias: a single institution experience with 3 https://doi.org/10.1016/S0022-5347(05)00063-7.
techniques. J Urol. 2008; https://doi.org/10.1016/j. 82. Snodgrass W, Prieto J. Straightening ventral curvature
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68. Soergel TM, Cain MP, Kaefer M, Gitlin J, Casale spadias repair. J Urol. 2009; https://doi.org/10.1016/j.
AJ, Davis MM, Rink RC. Complications of small juro.2009.02.084.
intestinal submucosa for corporal body grafting 83. Perovic SV, Vukadinovic V, Djordjevic MLJ,
for proximal hypospadias. J Urol. 2003; https://doi. Djakovic N. The penile disassembly technique in
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69. Mokhless IA, Youssif ME, Orabi SS, Ehnaish org/10.1046/j.1464-410X.1998.00547.x.
MM. Corporeal body grafting using buccal mucosa for 84. Bhat A. Extended urethral mobilization in incised
posterior hypospadias with severe curvature. J Urol. plate urethroplasty for severe hypospadias: a variation
2009; https://doi.org/10.1016/j.juro.2009.03.067. in technique to improve chordee correction. J Urol.
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AJ, Khoury AE. Outcome analysis of severe chordee 85. Snodgrass WT, Granberg C, Bush NC. Urethral stric-
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org/10.1016/j.juro.2007.03.166. J Pediatr Urol. 2013; https://doi.org/10.1016/j.
71. Hafez AT, Smith CR, Mclorie GA, El-Ghoneimi A, jpurol.2013.04.005.
Herz DB, Bägli DJ, Khoury AE. Tunica vaginalis for 86. Koff SA, Eakins M. The treatment of penile chordee
correcting penile chordee in a rabbit model: is there a using corporeal rotation. J Urol. 1984; https://doi.
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doi.org/10.1016/S0022-5347(05)65802-8. 87. Kass EJ. Dorsal corporeal rotation: an alterna-
72. Das S. Peyronie’s disease: excision and autograft- tive technique for the management of severe
ing with tunica vaginalis. J Urol. 1980; https://doi. chordee. J Urol. 1993; https://doi.org/10.1016/
org/10.1016/S0022-5347(17)55682-7. S0022-5347(17)35570-2.
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GF. Tunica vaginalis free graft for the correction the split and roll technique. J Urol. 1999; https://doi.
of chordee. J Urol. 1985; https://doi.org/10.1016/ org/10.1016/S0022-5347(01)68107-2.
S0022-5347(17)47141-2. 89. Acimi S. Commentary: results of onlay preputial flap
74. Caesar RE, Caldamone AA. The use of free grafts for urethroplasty for the single-stage repair of mid-and
correcting penile chordee. J Urol. 2000; https://doi. proximal hypospadias. Front Pediatr. 2018; https://
org/10.1016/S0022-5347(05)67084-X. doi.org/10.3389/fped.2018.00129.
75. Vandersteen DR, Husmann DA. Late onset recurrent 90. Huang LQ, Ge Z, Ma G. Commentary: Recurrent
penile chordee after successful correction at hypo- chordee in 59 adolescents and young adults following
spadias repair. J Urol. 1998; https://doi.org/10.1016/ childhood hypospadias repair. J Pediatr Urol. 2020;
S0022-5347(01)62716-2. https://doi.org/10.1016/j.jpurol.2020.06.005.
76. Nesbit RM. Congenital curvature of the phallus: 91. Abosena W, Talab SS, Hanna MK. Recurrent chordee
report of three cases with description of corrective in 59 adolescents and young adults following child-
operation. J Urol. 1965; https://doi.org/10.1016/ hood hypospadias repair. J Pediatr Urol. 2020; https://
S0022-5347(17)63751-0. doi.org/10.1016/j.jpurol.2019.11.013.
Classification and Assessment
of Hypospadias 9
Ahmed T. Hadidi
Abbreviations dee, the size of the penis and glans, or the degree
of hypoplasia of the undersurface of the penis. To
ASD Anoscrotal distance our knowledge the earliest classification was by
DSD Disorders of sex development the German surgeon Kaufmann in 1886 who
EGS External genitalia score classified hypospadias into the three grades:
EMS External masculinization score grade I or glanular hypospadias, where the ure-
GPPS Genital penile perception score thral meatus lies in the vicinity of the glans;
GPS Genital perception scale grade II or penile hypospadias, where the meatus
HAS Hypospadias assessment score lies in the penile shaft between the glans and the
HIS Hypospadias International Society penoscrotal junction; and grade III or perineal
HOC Hypospadias operative checklist hypospadias, where the meatus lies proximal to
HOPE Hypospadias objective penile the penoscrotal junction and scrotum is usually
evaluation bifid [1].
HOSE Hypospadias objective scoring In Smith’s classification, the first degree
evaluation locates the meatus from the corona to the distal
HPPS Hypospadias penile perception score shaft, the second degree is from the distal shaft to
MCGU Meatus-chordee-glans width and the penoscrotal junction, and the third degree is
shape-urethral plate quality from the penoscrotal junction to the perineum
PPS Penile perception score [2]. Schaefer and Erbes classified the location as
glanular from the subcorona out, penile from the
corona to the penoscrotal junction, and perineal
from there down [3]. The system of Browns
9.1 Introduction (1936) was more specific, with glanular, subcoro-
nal, penile, midshaft, penoscrotal, scrotal, and
The anatomical classifications of hypospadias perineal varieties [4]. Barcat preferred to use the
recognize the level of the meatus without taking new meatus location after the chordee has been
into account associated anomalies such as chor- released [5]. Duckett et al. classified hypospadias
according to meatal location after release of cur-
vature into anterior, middle, and posterior hypo-
A. T. Hadidi (*) spadias [6]. Orkiszewski used pubic bone as a
Department, Sana Klinikum Offenbach, Academic
reference [7]. The GMS score scaled the anomaly
Teaching Hospital of the Johann Wolfgang Goethe based on the properties of glans, location of
University, Offenbach, Frankfurt, Germany meatus, and degree of chordee [8]. Renaux-Petel
238 A. T. Hadidi
et al. classified hypospadias into two groups mated that 62% of the openings were subcoronal
based on the level of bifurcation of the corpus or penile, 22% were at the penoscrotal angle, and
spongiosum [9]. External Genitalia Score(EGS) 16% opened in the scrotum or perineum [14].
looks through a window of Disorders of Sex Juskiewenski and colleagues reported a study of
Development (DSD) [10]. It is a modification of 536 patients with hypospadias [15]. Anterior
External Masculinization Score(EMS), and both hypospadias comprised 71%, middle 16%, and
use a gradual scale from normal male to normal posterior 13%. Juskiewenski subdivided the
female. They aim to distinguish DSD cases from hypospadias of the anterior group of 383 patients
normal variants. into 13% balanic, 43% subcoronal, 38% distal
The Hypospadias International Society rec- shaft, and 6% with prepuce intact. Out of 1286
ommends the MCGU (meatus-chordee-glans cases over 5 years at the Children’s Hospital of
width and shape-urethral plate quality) score Philadelphia, Duckett et al. reported that 49%
incorporating urethral plate and suggests a score were anterior, 21% were middle, and 30% were
from 0 (normal) to 10 (perineal hypospadias with in a posterior location (Fig. 9.1) [6]. Out of 1816
severe chordee, small glans, and narrow urethral cases over a 5-year period from 2016 to 2020
plate) [11]. operated on at the Hypospadias Center, Frankfurt,
Germany, 423 (23%) were grade I (glanular), 967
(53%) were grade II (distal), 261 (14%) were
9.2 Anatomical Classifications grade III (proximal without severe chordee), and
of Hypospadias 165 (10%) were grade IV (perineal or proximal
with severe chordee)(Fig. 9.2, Table 9.1).
From a practical clinical point of view, the
detailed subclassifications may seem rather theo-
retical. Moreover, there are no specific operations 9.3 hy Do We Need to Classify
W
designed for midpenile hypospadias. Depending Hypospadias?
on the actual position of the meatus after curva-
ture correction, one uses either techniques There are several reasons why we need to classify
designed for glanular, distal, proximal, or peri- hypospadias: to avoid mixing “oranges and
neal hypospadias. Techniques to correct glanular apples,” to be able to explain and answer the par-
hypospadias advocate mainly the advancement ents’ questions, for self-assessment, to compare
principle first described by Beck [13]. Techniques different techniques in different centers, for train-
to correct distal hypospadias mainly tubularize ing purposes, and for long-term postoperative
the urethral plate or use proximal penile skin flap. assessment.
Techniques to correct proximal hypospadias When a family with a hypospadias child
without chordee or chordee less than 30 degree comes for consultation, they want answers to
mainly tubularize the urethral plate or use lateral many questions: How severe is the hypospadias
penile skin or preputial onlay flaps. Perineal (grade)? How common is it? Is the penis curved?
hypospadias is a completely different entity How severe is the curvature? When is the best
(actually a syndrome) characterized by severe time to operate? What surgical technique will be
chordee more than 35 degrees necessitating ure- used? How many operations does he need? What
thral plate division and is usually associated with is the success rate? What are the possible compli-
ventral hypoplasia, bifid scrotum, and penoscro- cations and their percentage? Can we correct the
tal transposition. This may fit easier to the inter- complications if they occur? Can we preserve the
national classification of diseases which classifies foreskin? How many catheters will he have after
hypospadias (Q54) into glanular (Q54.0), penile surgery? Transurethral or through the abdomen?
(Q54.1), proximal (Q54.2), and perineal (Q54.3). For how long will he have the catheter/s? How
Welch collected more than 1000 cases of long is the hospital stay? Is the child mobile after
hypospadias from seven separate reports and esti- surgery? Can he sleep on his abdomen? When
9 Classification and Assessment of Hypospadias 239
Fig. 9.1 Different classifications of hypospadias, according to location of meatus [12] (©Ahmed T. Hadidi 2022. All
Rights Reserved)
can he have a shower and bath after surgery?

How to clean after surgery? How often to change
the diapers?
It is difficult in the office without anesthesia
and with a carrying baby to measure the size of the
glans or to measure the degree of curvature with a
goniometer. For such a situation, we need a simple
classification that can help to categorize the condi-
tion and answer the questions of the family.
As surgeons, we need to objectively assess
and evaluate our own performance and results.
Fig. 9.2 Hypospadias is currently classified into four
grades: grade I or glanular hypospadias where the meatus We need to know:
is in the region of the glans and is usually mobile, grade II
or distal where the meatus lies in the distal half of the • Which technique produces the best results in
penis and chordee if present is superficial, grade III or our hands?
proximal where the meatus lies in the proximal half of the
penis and chordee if present is superficial and can be cor- • For which severity of chordee?
rected without dividing the urethral plate, and grade IV or • For which grade of hypospadias?
perineal (a syndrome where the meatus lies proximal to
the penoscrotal junction, usually associated with severe As members of any Hypospadias Scientific
chordee that necessitates division of the urethral plate, the
glans is usually small, the scrotum is bifid, and there is a Society with surgeons in different parts of the
short anoscrotal distance (ASD) (©Ahmed T. Hadidi world and with different approaches and tech-
2022. All Rights Reserved) niques, we need a detailed intraoperative record-
ing system to compare our approaches and results
Table 9.1 Analysis of 1816 patients with hypospadias and to learn tips and tricks from each other, and
operated on in the Hypospadias Center in Frankfurt we need to compare the different techniques to
between 2016 and 2020
see which technique is the best for which grade.
Grade I II III IV
As teachers and trainers, we need to guide our
Number of patients 423 967 261 165
trainees through this complex deformity and help
Percentage 23% 53% 14% 10%
them to decide how to choose the appropriate
240 A. T. Hadidi
technique for each single patient and what param- 9.4.3 Type and Size of the Glans (G)
eters we need to help us make the decision.
Furthermore, hypospadias is a very complex The glans may be classified into 3 types: cleft or
anomaly that may have serious lifelong compli- grooved glans, incomplete cleft, and a flat glans.
cations like recurrent chordee, recurrent hypo- The deeper the groove, the more developed the
spadias, reduced sensation, disfigurement, scars, glans, and the better is the cosmetic result that
late fistula, erection, and ejaculation problems. can be obtained (Fig. 9.3).
Therefore, we need an objective assessment pro- Another influencing factor is the size of the
tocol that follows patients for 15–20 years after glans which again indicates better development
surgery. of the penis. The larger the glans (> 14 mm), the
more developed is the penis, the easier is the
operation, and the better are the cosmetic results.
9.4 actors that Influence
F
Outcome of Hypospadias
Surgery 9.4.4 The Urethral Plate (U)
Several publications have shown that the follow- The urethral plate is the precursor of the penile
ing factors play an important role in the outcome urethra and it is used to reconstruct the new ure-
of hypospadias surgery [16, 17]: thra whenever possible. The more developed the
urethral plate, the easier is the operation and the
less are the complications. However, it is not easy
9.4.1 Site of Meatus (M) to objectively evaluate the degree of development
of the urethral plate. The width of the urethral
In general, the more distal meatus is, the more plate seems to be the available objective indica-
developed is the penis, the easier is the surgical tion of the urethral plate development.
correction, and the less are the complications.
However, there are exceptions to this general rule
(e.g., chordee without hypospadias, distal hypo- In general, a cleft glans is usually large and
spadias with severe chordee). is usually associated with a wider urethral
plate and with mild or no chordee and is
more common in distal hypospadias.
9.4.2 Chordee (C)
The presence of chordee complicates the correc-

tion of the hypospadias. Superficial chordee can 9.5 The Hypospadias
be corrected by extensive degloving; deep chor- International Score (MCGU)
dee requires division of the urethral plate or dor-
sal plication as preferred by some surgeons. The Scientific Committee of the Hypospadias
Corporeal disproportion requires incision of the International Society (HIS) including the author,
corpora cavernosa and flaps or grafts. Mark Zaontz, Luis Braga, Antonio Macedo,
The severity of chordee may also change the Ibrahim Ulman, Grahame Smith, and Chris Long
grade of hypospadias. For example, a proximal have shared their experience to develop the
hypospadias (grade III, one stage urethral recon- Hypospadias International Score as a base to
struction) or even a distal hypospadias with be used and to modify it whenever needed
severe chordee that necessitates division of the (Fig. 9.4).
urethral plate will change the hypospadias into The following figure summarizes the data that
grade IV hypospadias and preferably as a two- we need to collect from every patient with hypo-
stage urethral reconstruction. spadias (Fig. 9.5):
a b c
Fig. 9.3 Classification of glans configuration in hypospa- cleft glans: There is a variable degree of glans split, a shal-
dias: (a) Cleft glans: There is a deep groove in the middle low glanular groove, and a variable degree of urethral
of the glans with proper clefting; the urethral plate is nar- plate projection. (c) Flat glans: The urethral plate ends
row and projects to the tip of the glans. (b) Incomplete short of the glans penis, no glanular groove
9.6 he Hypospadias Operative

T sexual function, and to look as normal as
Checklist (HOC) possible.
Assessment of the Urinary Function of the
During surgery, the data of the patient needs to be penis after hypospadias can be based on the
collected once again under anesthesia with accu- observation of the pattern and caliber of micturi-
rate measurement of the curvature, glans size, tion, straight and strong or deviated, presence of
rotation, and anoscrotal distance (ASD). In addi- spraying, interrupted stream, presence of post
tion, all operative details, including suture mate- micturition drippling of urine or hesitancy, fre-
rial, catheter, and dressing, need to be recorded as quency, or urinary tract infection. Ultrasound is
shown in the following Hypospadias Operative an important noninvasive tool to detect post-
Checklist (HOC) (Fig. 9.6a–b): micturition residual urine volume in the bladder,
bladder wall thickening, reflux, and renal dilata-
tion. Uroflow is helpful in toilet-trained children.
9.7 Postoperative Assessment However, it may be frustrating for children. Peak
flow rates and uroflow curves have unclear mean-
The aim of hypospadias surgery is to have a near ing and are usually flat despite successful symp-
normal functional and cosmetic penis. This tomless urethroplasty. A flat curve with flow rate
means to allow the young boy to micturate nor- less than 5 cc/s may warrant further investigation.
mally, standing with good stream, and to allow Calibration is a term currently used to describe
for normal development of the penis and normal dilation and is very traumatic and painful for
242 A. T. Hadidi
Hypospadias International Score (MCGU)

Name of Patient: (HR No.): Date of Birth:
Family History Hormone Therapy Date of Examination:

Positive Negative Positive Negative Score
1. Meatus
I, Glanular II, Distal Penile III, Proximal IV, Perineal

Hypospadias Hypospadias Hypospadias Hypospadias 1-4
1 2 3 4
2. Chordee
Severe, deep chordee 0-2

or < 15° 15-30° or > 30°
0 1 2
3. Glans Width & Shape
good groove glans poor groove glans flat glans 0-2

≥ 14 mm 12 - <14 mm < 12 mm
0 1 2
4. Urethral
Plate quality
intact divided 0-2
0 2
Glanular Hypospadias with good glans = Score 1

Perineal Hypospadias with poor glans = Score 10 Total score
1-10
©Ahmed T. Hadidi 2022. All Rights Reserved
Fig. 9.4 The MCGU Score that gives an idea of the dif- hypospadias is and the higher the incidence of complica-
ficulty of the hypospadias and the possibility of complications (©Ahmed T. Hadidi 2022. All Rights Reserved)
tions. The higher the score, the more difficult the
1. Family History Hormone Therapy

Date of Examination:
Positive Negative Positive Negative
2. Meatus location Glanular Distal Penile Proximal

Hypospadias
Perineal
Hypospadias
Hypospadias Hypospadias
before chordee
correction 1 2 3 4
3. Meatus location after Glanular Distal Penile Proximal Perineal

Hypospadias Hypospadias Hypospadias Hypospadias
chordee correction
1 2 3 4
0°-15° 15°-30° >30°

4. Chordee No chordee Deep chordee
0 1 2
deep groove poor groove

5. Glans width ≥ 14mm 12- <14mm < 12 mm
0 1 2
6. Urethral plate quality

divided
MCGU Score = ................
0 2
7. Prepuce Complete Incomplete
8. Penile torsion No torsion Left Right
No Incomplete Complete
9. Scrotal transposition transposition transposition transposition
10. Undescended Testis Normal Unilateral Bilateral
3 - 5 cm 2 - 3 cm <2 cm
11. ASD Ano Scrotal Distance
12. Penile length >3 cm 2-3 cm <2 cm
© Ahmed T. Hadidi 2022. All Rights Reserved
Fig. 9.5 The “Hypospadias Initial Score”(HIS) that pro- MCGU in addition to information about the prepuce, rota-
vides the data needed about the hypospadias patient and tion, testis, scrotum, and anoscrotal distance (ASD)
the possible associated malformations. It combines the (©Ahmed T. Hadidi 2022. All Rights Reserved)
244 A. T. Hadidi
a
Hypospadias Operative Checklist (HOC)
Name of the patient:__________________ (HR No.):________Date of birth:____________Date of Surgery:_______________
Experience of operator: in training completed his training in hypospadias
Name of Hospital and Country: _____________________________________________________________________________
Preoperative hormonal stimulation

Topical or Injection: Duration:
Androgens
Dose:
Other:
Size of the penis

Dorsal ongitudinal length of the lans (DLL):
Ventral ongitudinal length of the lans (VLL):
Glans idth at the coronal sulcus (GW):
Level of division of the corpus spongiosum:

Yes No
Distal Mid-shaft Proximal
M eatus Position of the urethral meatus after degloving

1 (Glanular) 2 (Distal) 3 (Proximal) 4 Perineal
C hordee 0: no chordee oder <15 degree 1: 15-30 degree 2: > 30 degree
Glans clefting 0 : Cleft glans 1: incomplete cleft glans

≥ 14 mm 12 - <14 mm < 12 mm
Urethral late quality

intact divided MCGU Score = . . . . . . . . . . . . . . . .
Erection test
hecked not checked
Technique of hordee correction

entral degloving complete degloving Nesbit
TAP plate mobilisation prox urethra mobilisation
corporoplasty corporotomy Tunica Albuginea Longitudinal Excision (TALE)
STAG others plate division
Haemostasis Yes No
Tourniquet Selective tight

Adrenaline swabs injection
ation
No eading glasses loops operating microscope
Technique of urethroplasty
DYG SLAM LABO CEDU Thiersch-Duplay
TIP Mathieu On y Duckett island Bracka (two stage)
Koyanagi one stage grafting two stage MAGPI
GFC Others (mention): grafting
Foreskin Reconstruction Circumcision
©Ahmed Hadidi
Fig. 9.6 (a and b) a) The Hypospadias Operative niques and to learn from tips and tricks used in different
Checklist that provides the accurate hypospadias data centers all over the world (©Ahmed T. Hadidi 2022. All
after degloving and all the necessary intraoperative Rights Reserved)
details. Such information is important to compare tech-
b

246 A. T. Hadidi
HYPOSPADIAS ASSESSMENT SCORE (HAS)
Name: Date of birth:

Pre-operative diagnosis:
Operative technique: Date of surgery:
Hospital stay:
2. Size of urethral meatus
3. Shape of urethral meatus
4. Urethral stenosis No Yes
5. Urine stream single spray
6. Erection straight 10%–30% >30%
multiple
7. Fistula No single and narrow
urethra
8. Diverticulum No distal proximal
normal borderline platue
10. Straining No mild severe
11. Foreskin retractable not retractable
12. Bladder wall normal thick
13. Further surgery ≤2 2–4 >4
14. Parents’ satisfaction borderline
15. Patient’s satisfaction borderline
© Ahmed T. Hadidi 2022. All Rights Reserved
Fig. 9.7 The “Hypospadias Assessment Score” (HAS). This system covers the functional as well as the cosmetic out-
comes of hypospadias surgery (©Ahmed T. Hadidi 2022. All Rights Reserved)
children and is not indicated in symptomless assessed 5 main items: the meatal location,
children. meatal shape, urinary stream, the presence of fis-
Assessment of the sexual function is based tula, and erection and penile curvature.
on observation of straight, hard erection without In 2013 van der Toorn et al. developed the
curvature (chordee), normal sensation of the “Hypospadias Objective Penile Evaluation”
glans and penis, and normal ejaculation without (HOPE) that included six items: the meatal loca-
stagnation of semen in the urethra. tion, the meatal shape, the shape of the glans, the
Assessment of the cosmetic outcome relates shape of the skin, torsion, and curvature in an
to the location of the meatus at the tip of the erect penis [18]. It included standardized photo-
glans, ideally a slit-like meatus, the quality of the graphs and reference pictures.
skin, and the presence of scars or suture tracts in Weber et al. reported the “Pediatric Penile
the glans or the body of the penis. Perception Score” (PPPS) after validation for
The ideal postoperative assessment system prepubertal boys by independent urologists [22].
after hypospadias surgery is an objective, repro- In 2013, after validation of this same instrument
ducible, and validated tool measuring all relevant for post pubertal men, the name was modified to
and surgically correctable aspects of the hypospa- “Penile Perception Score” (PPS) [23]. It includes
dias [18]. Postoperative assessment tools include: the patient self-perception of: (1) the length of
the “Genital Penile Perception Score” (GPPS) the penis, (2) the position and shape of the
[19], the “Hypospadias Objective Scoring meatus, (3) the shape of the glans, (4) the shape
Evaluation” (HOSE) [20], the “Hypospadias of penile skin, (5) the straightness of the penis
Penile Perception Score” (HPPS) [21], the upon erection, and (6) the general appearance of
“Pediatric Penile Perception Score” (PPPS) [22], the penis. The scale ranged from very satisfied,
the “Penile Perception Score” (PPS) [23], the satisfied, dissatisfied, to very dissatisfied.
“Hypospadias Objective Penile Evaluation” In the Hypospadias Center in Frankfurt, we
(HOPE) [18], and the “Genital Perception Scale” are currently using the “Hypospadias
(GPS) for adults [24] and children [25]. Assessment Score” (HAS) with 15 points and
The majority of these tools focused on postop- 3 grades (satisfied, borderline, and unsatis-
erative cosmetic satisfaction, with only one con- fied) that awaits comprehensive validation.
sidering urinary function and no instruments Satisfaction indicates that the patient is con-
evaluating sexual function and psychosocial tent with the outcome of surgery and has no
sequelae. It is important that the surgeon must problem. Borderline indicates that although
spend time with the family and explain that the the patient is not satisfied, he will not seek fur-
child with hypospadias must be followed up until ther surgery. Unsatisfied indicates that the
puberty. In the Hypospadias Center in Frankfurt, patient is unhappy with the surgical outcome
we follow all patients with hypospadias until the and definitely wants surgical correction
age of 15, and we explain that the young adult (Fig. 9.7).
after hypospadias surgery may come back to us
in case they have any concern.
In 1995 Mureau et al. evaluated (parental or References
patient) satisfaction with (different items of) gen-
ital appearance with the use of the validated 1. Kaufmann C. Verletzungen und Krankheiten der män-
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“Genital Penile Perception Score” (GPPS) [19]. Chirurgie; 1886.
The GPPS includes position of the meatus and 2. Smith CK. Surgical procedure for correction of
glanular shape, penile size, penile thickness, and hypospadias. J Urol. 1938; https://doi.org/10.1016/
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3. Schaefer AA, Erbes J. Hypospadias. Am J Surg. 1950;
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5. Barcat J. Current concepts of treatment. In: Horton 17. Bush NC, Villanueva C, Snodgrass W. Glans size is
CE, editor. Plastic and reconstructive surgery of an independent risk factor for urethroplasty complica-
the genital area. Boston, MA: Little Brown; 1973. tions after hypospadias repair. J Pediatr Urol. 2015;
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Adult and pediatric urology. 3rd ed. St. Louis: Mosby- Evaluation)-score: a validation study of an objective
Year Book; 1996. p. 2549–89. scoring system for evaluating cosmetic appearance in
7. Orkiszewski M. A standardized classification of hypospadias patients. J Pediatr Urol. 2013; https://doi.
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org/10.1016/j.jpurol.2011.08.011. 19. Mureau MAM, Slijper FME, van der Meulen JC,
8. Merriman LS, Arlen AM, Broecker BH, Smith EA, Verhulst FC, Slob KA. Psychosexual adjustment
Kirsch AJ, Elmore JM. The GMS hypospadias score: of men who underwent hypospadias repair: norm-
assessment of inter-observer reliability and correla- related study. J Urol. 1995; https://doi.org/10.1016/
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2013; https://doi.org/10.1016/j.jpurol.2013.04.006. 20. Holland AJA, Smith GHH, Ross FI, Cass DT. HOSE:
9. Renaux-Petel M, Mure P-Y, Gorduza D-B, an objective scoring system for evaluating the results
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11. Hadidi AT. Hypospadias International Classification. ment for patient self-assessment and surgeon evalua-
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Epidemiology of Hypospadias
10
Loes F. M. van der Zanden, Iris A. L. M. van Rooij,
and Nel Roeleveld
Abbreviations increasing prevalence in China [17–20], Korea

[21], Australia [22], the USA [2, 8, 23, 24], South
BMI Body mass index America [25], and Europe [26–29], whereas oth-
DES Diethylstilbestrol ers did not find an increase in Australia [30],
EDCs Endocrine disrupting chemicals Canada [31, 32], the USA [5, 7, 12, 33], Europe
hCG Human chorionic gonadotropin [34–37], Japan [38], and Taiwan [39]. The results
ICSI IntraCytoplasmic sperm injection of different studies are difficult to compare
IUGR IntraUterine growth restriction because some are based on hospital discharge
IVF In vitro fertilization registries, including only surgically treated
TDS Testicular dysgenesis syndrome patients or all newborns diagnosed with hypospa-
dias, whereas others are based on birth defects
surveillance systems, including all registered
hypospadias patients or excluding patients with
10.1 Prevalence glandular hypospadias. In addition, the diagnosis
and definition of hypospadias as well as the
Figures on the birth prevalence of hypospadias reporting of patients to registries or surveillance
vary considerably across countries, ranging from systems may have changed over time. Two large
0.04 to 0.8% of male births [1–3]. Hypospadias studies reporting data from multiple registries
occurs most frequently in whites and less fre- concluded that results differ between the regis-
quently in blacks, and rates are lowest among tries and that multiple artifacts may contribute to
Asians and Hispanics [2, 4–16]. There is a debate the upward trends [40, 41], and a systematic
about whether or not the prevalence of hypospa- review concluded that numerous large studies
dias is increasing. Some researchers reported have been performed but that many methodologi-
cal factors influence the calculation of the preva-
lence and the changes in prevalence over time
L. F. M. van der Zanden (*) · Iris A. L. M. van Rooij
N. Roeleveld [42]. However, the most recent large study
Department for Health Evidence, Radboud Institute including data from 27 international programs
for Health Sciences, Radboud University Medical over a 31-year period suggested that the preva-
Center, Nijmegen, The Netherlands
lence of hypospadias is increasing [3]. In our
e-mail: Loes.vanderZanden@Radboudumc.nl;
Iris.vanRooij@Radboudumc.nl; opinion, no firm conclusions can be drawn based
Nel.Roeleveld@Radboudumc.nl on the currently available literature.
250 L. F. M. van der Zanden et al.
10.2 tiology of Hypospadias Is

E lying entity, the testicular dysgenesis syndrome
Multifactorial (TDS) [62]. They were convinced of its existence
because countries with high incidences of testic-
Hypospadias presents as an isolated condition in ular cancer also had high prevalence rates of
the majority of patients, and the etiology remains hypospadias, cryptorchidism, and poor sperm
unknown. However, compared to healthy chil- quality [63]. Other researchers question whether
dren, boys born with hypospadias more often TDS actually exists as there is little evidence of
have additional congenital anomalies [22, 43–46], shared causes [64]; only a few patients display all
an association that appears to be stronger for features, and incidences of the four components
patients with proximal compared to distal hypo- of the syndrome did not increase over time at the
spadias [22, 43, 47]. In particular cryptorchidism same rate [65]. Although the risk of testicular
and other urogenital anomalies are frequently germ cell cancer was increased in patients with
found with hypospadias [22, 48–50]. In 30% of hypospadias or undescended testis, the risk was
the patients with the proximal form of hypospadias, not increased in their family members. This does
a cause can be identified, for example, a complex not support the hypothesis of shared heritability
genetic syndrome, partial androgen insensitivity [66]. More recently, Skakkebæk et al. concluded
related to androgen receptor mutations, or SRD5A that TDS does exist but that it encompasses only
type II deficiency ([51, 52]). a fraction of hypospadias and impaired spermato-
Hypospadias shows familial clustering, with 7 genesis patients [67].
to 22% of patients having affected first, second,
or third degree relatives [53, 54]. Familial occur-
rence seems to be more common for distal and 10.4 Estrogen Hypothesis
middle forms of hypospadias than for proximal
types [53, 55–57]. The chance that a brother of an In 1993, Sharpe and Skakkebæk hypothesized
affected boy will also have hypospadias is 9 to that the increasing incidence of reproductive
17% [58–60]. In two family studies and one abnormalities in males may have a common
small twin study, the heritability of hypospadias cause, namely, increased estrogen exposure in
was estimated to be 57 to 77% [58–60], meaning utero, leading to disturbances in anti-Müllerian
that 57 to 77% of the phenotypic variability can hormone secretion or impairment of Leydig cell
be attributed to genetic variability. Hypospadias development [68]. Ten years after the introduc-
is equally transmitted through the maternal and tion of this hypothesis, Sharpe concluded that the
paternal sides of the family, and recurrence risks evidence for fetal estrogen exposure inducing
for brothers and sons of hypospadias patients are TDS had strengthened [69]. New pathways were
similar [60]. This suggests that genetic rather identified through which estrogens could induce
than shared environmental factors may play a TDS, including suppression of testosterone pro-
principal role in familial hypospadias [60]. duction, androgen receptor expression, and
Segregation analysis, however, suggested that the insulin-like 3 secretion. Whether increased estro-
majority of cases have a multifactorial etiology, gen exposure will turn out to be an important
involving both genes and environmental factors etiologic factor for TDS is not so certain,
[61]. however.
The initial “estrogen hypothesis” was super-
seded by a more refined definition of endocrine
10.3 Testicular Dysgenesis disrupting chemicals (EDCs), suggesting that
Syndrome chemicals may act on the endocrine systems in a
plethora of ways [70]. In 2008, Sharpe and
In 2001, Skakkebæk et al. suggested that poor Skakkebæk highlighted the central role of defi-
sperm quality, testicular cancer, undescended tes- cient androgen production or action during fetal
tes, and hypospadias are symptoms of one under- testicular development in the origin of the down-
10 Epidemiology of Hypospadias 251
stream disorders of TDS [71]. However, the ques- to-pregnancy for parents of patients with hypo-
tion remains whether levels of exposure to EDCs spadias [55, 107, 110, 111], while two other
are sufficient to influence male reproductive studies did not confirm these results [44, 91]. The
health [70], and several reviews and meta- fact that ICSI, rather than IVF, and sperm quality
analyses concluded that there is little evidence are associated with hypospadias supports the idea
for a role of environmental EDCs in hypospadias that paternal fertility problems play a role in
[72–81]. hypospadias instead of hormonal stimulation [55,
84]. Indeed, a trend was found toward increasing
rates of hypospadias from natural conception,
10.5 he Role of Environmental
T IVF, and ICSI with ejaculated sperm to ICSI with
Factors in the Etiology epididymal and testicular sperm, although not
of Hypospadias statistically significant [112], and hypospadias
seemed to be associated with paternal subfertility
10.5.1 Exogenous Exposure [108]. An alternative hypothesis is that assisted
to Estrogens reproductive technologies could increase the risk
of hypospadias by interference with epigenetic
10.5.1.1 Oral Contraceptives regulation, as these technologies may be associ-
Although oral contraceptives provide clear estro- ated with genomic imprinting disorders [113].
gen exposure, an association between hypospa-
dias and use of oral contraceptives for some time
during pregnancy was not found in most studies 10.6 Endogenous Hormone
[55–57, 82–88]. Levels
10.5.1.2 Assisted Reproductive 10.6.1 Endogenous Estradiol Levels

Technology
Assisted reproductive technologies frequently Endogenous levels of free estradiol increase
involve hormonal stimulation, and several studies with increasing body mass index (BMI) and are
showed an increased risk of hypospadias with the elevated in women with an early age at men-
use of these technologies [11, 55, 57, 84, 89–92]. arche [114, 115]. Several studies found associa-
In some studies, hormonal stimulation was asso- tions between hypospadias and mothers being
ciated with hypospadias [57, 87, 93], but this overweight (25 ≤ BMI < 30 kg/m2) [116] or
association was not seen in other studies [82, severely overweight or obese (BMI > 29 or 30
94–96]. Intracytoplasmic sperm injection (ICSI) or 35 kg/m2) [29, 56, 85, 116–119], while others
increased the risk of hypospadias in most [97– did not [55, 57, 120]. One study found an
102] but not all studies ([103, 104]), whereas increased risk for underweight women, but not
in vitro fertilization (IVF) did not increase hypo- for overweight or obese women [121], while
spadias risk in most studies, or the results were another found an increased risk both for under-
inconclusive [82, 98, 102–104], except for two weight and overweight women with a dose-
studies that did find increased risks after IVF response association with increasing BMI [122].
treatment [29, 105]. A systematic review con- The results for early age at menarche were
cluded that ICSI is associated with a slightly inconsistent [82, 118]. Estradiol levels are also
higher risk of hypospadias than IVF, although not higher in first pregnancies and in twin pregnan-
statistically significant [106]. In addition, fathers cies [123, 124], which were both repeatedly
of hypospadias patients were reported to have investigated for their association with hypospa-
lower sperm concentration, sperm count [107, dias. Most studies showed that women in their
108], and sperm motility, as well as a higher pro- first pregnancy [5, 9, 11, 13, 16, 18, 44, 45, 48,
portion of sperm with abnormal morphology 56, 57, 82, 110, 125–128], or with a twin or trip-
[109]. Several studies reported a prolonged time- let pregnancy [11, 13, 16–18, 29, 44, 53, 55–57,
82, 84, 102], were at increased risk of having a insufficiency among newborns being small for
son with hypospadias, but a few studies could gestational age with hypospadias, with the more
not replicate the findings for primiparity or mul- proximal patients having more severe IUGR
tiple pregnancies [5, 45, 48, 126, 129, 130]. The [154]. Jensen et al. concluded that hypospadias is
latter may be caused by overadjustment for birth associated with fetal growth restriction and that
weight in some studies. As only early-onset this association is due to shared causes [155]. The
intrauterine growth restriction (IUGR) could be association with low birth weight seems to be
a risk factor for hypospadias based on the criti- stronger for more proximal forms of hypospadias
cal period during pregnancy, it is more likely [5, 55–57, 129, 156, 157].
that low birth weight and hypospadias share an Nausea in early pregnancy may be caused by
underlying cause rather than low birth weight the early surge of hCG [158], suggesting that pla-
being a risk factor for hypospadias. cental insufficiency may cause absence of nau-
sea. Indeed, vomiting and nausea during early
pregnancy were shown to decrease the risk of
10.6.2 Placental Insufficiency hypospadias [11, 85, 128, 159]. Maternal hyper-
tension during pregnancy ([17, 55, 57, 82, 85,
Placental human chorionic gonadotropin (hCG) 160–163]) and preeclampsia [17, 44, 45, 55, 82,
stimulates fetal testicular steroidogenesis before 126, 128, 134, 140, 163–166] were consistently
the fetus’s own pituitary-gonadal axis is estab- associated with hypospadias, and both factors
lished. Placental insufficiency may result in inad- may be associated with placental dysfunction,
equate fetal hCG provision, possibly explaining possibly by compromising uteroplacental perfu-
the association between hypospadias and low sion [160]. Preterm delivery may be associated
birth weight or being small for gestational age with late placental dysfunction, and several stud-
that has been reported consistently, although not ies demonstrated an association with hypospa-
always statistically significant [5, 16–18, 29, 44, dias [9, 16–18, 50, 85, 102, 111, 118, 128].
45, 48, 53, 55, 56, 82, 84, 85, 102, 111, 118, 125, Others found the association for proximal forms
128–142]. However, because hCG levels in of hypospadias only [57] or could not confirm the
maternal serum samples of hypospadias patients association ([5, 44, 45, 48, 129, 134]), again pos-
and controls were similar [143, 144] or even sibly due to overadjustment for birth weight in
higher in the patients [145, 146], some studies some studies.
suggest that the decreased maternal hCG produc-
tion may not be the mechanism explaining the
relation between hypospadias and placental 10.7 Clinical Factors
insufficiency.
IUGR was found more often in the affected 10.7.1 Pregnancy Complications
twin of same-sex twin pairs discordant for hypo-
spadias [147, 148]. Direct proof of a link between In a few studies, associations were investigated
placental insufficiency and hypospadias was pro- between hypospadias and complications during
vided by research showing an association pregnancy, such as maternal bleeding, which
between hypospadias and low placental weight in seemed to be more prevalent among mothers of
some but not all studies [59, 149, 150], an asso- patients [18, 45]. The amount of weight gain was
ciation between hypospadias and abnormal pla- not associated with hypospadias [9, 82, 128].
cental perfusion [151], an increased frequency of Complications during labor, such as labor induc-
placental infarction among extremely low birth tion and Caesarean section, occurred more fre-
weight boys with hypospadias [152], a higher quently among mothers of hypospadias patients
prevalence of placental lesions in children small [9, 45], indicating that pregnancies affected by
for gestational age with hypospadias [153], and a hypospadias are associated with other difficulties
high rate of early-onset IUGR related to placental that make them prone to these complications.
Diabetes has been another focus of research, but antiretroviral therapy [192], selected antihyper-
most studies were too small to draw conclusions tensive drugs [55, 160, 161, 193], nystatin [168,
or found no associations [17, 45, 55, 57, 82, 125, 194], paroxetine [195], phenobarbital [168, 196],
126, 167, 168]. Nevertheless, some studies lynestrenol [168], and fluconazole [197] during
reported an increased risk for pre-existing but not early pregnancy may increase the risk of hypo-
or less strong for gestational diabetes [7, 169– spadias, but most of these associations were
171], an increased risk for gestational diabetes reported only once or with inconsistent results. In
[128], or an increased risk for any form of diabe- contrast, use of anti-epileptic drugs was linked to
tes [13, 172]. Results were inconsistent for hypospadias in most studies [198–209].
hepatitis B surface antigen positivity [17, 140],
thyroid disorders [45, 168, 173], and fever during
pregnancy [18, 59]. Women with ovarian cysts or 10.7.3 M
aternal Intrauterine DES
benign uterine tumors [135], who had two or Exposure
more previous miscarriages [174], and women
who experience a viral infection or influenza in In 2002, Klip et al. reported a 21 times increased
the first trimester of pregnancy [82, 175] seem to hypospadias risk among sons of women exposed
be at increased risk of giving birth to a son with to diethylstilbestrol (DES) in utero in a cohort
hypospadias, but evidence was derived from only of women with fertility problems [210].
one study each. Urinary infections and anemia do Thereafter, other studies were consistent in
not seem to increase the risk of hypospadias [45, showing increased risks for sons of DES-
128], and the same holds for inflammatory bowel daughters, although less strong [55, 211–214],
disease [176] and endometriosis [177]. but not for sons of DES-sons [215]. The trans-
generational effect may have been related to
genetic or epigenetic changes in primordial
10.7.2 Maternal Drug Use oocytes, which were transmitted to the next gen-
eration, or in somatic cells of the DES-daughter,
Most therapeutic drugs, such as corticosteroids, resulting in a disturbed hormonal balance in
several antihistamines, antibiotics, antipsychot- adult life [210]. Another explanation would be
ics, antifungals, and anti-asthmatic drugs, do not that pathology of the DES-daughter’s reproduc-
seem to be associated with hypospadias, although tive structures interferes with normal fetal
some studies may suffer from underreporting development [212].
[55, 84, 87, 159, 161, 178–183], and recently,
macrolide antibiotics were suggested to be asso-
ciated with hypospadias [184]. Based on data 10.8 Behavioral Factors
from the Swedish Medical Birth Register 1995–
2001, Källén et al. reported 15 hypospadias 10.8.1 Parental Age
patients among 2780 infants born after maternal
use of loratadine, an antihistamine, during preg- Women become pregnant at different ages, but,
nancy [185], but in 2001–2004 only two patients overall, maternal and paternal age at time of con-
were identified among 1911 infants exposed to ception did not seem to increase the risk of hav-
loratadine, indicating that the primary finding ing a son with hypospadias [9, 16, 17, 26, 37, 44,
may have occurred by chance [186]. Other stud- 45, 48, 55, 82, 84, 85, 110, 125, 126, 129, 140,
ies also failed to find an association between 216]. However, some studies reported that a
loratadine and hypospadias [87, 187, 188]. higher maternal age increased the risk of hypo-
Results for progestogens/progestins used for spadias [5, 7, 11, 20, 33, 50, 57, 125, 130, 141,
threatened abortion [58, 168, 189], proton pump 217–220] or the risk of severe hypospadias [221],
inhibitors [87, 159, 190] codeine [87, 175], and while others found lower or higher paternal age
steroids [87, 159] vary. Use of loperamide [191], to increase hypospadias risk [216, 222].
10.8.2 Maternal Diet spadias [9, 57, 84, 125, 238]. For maternal
smoking, most studies showed no association[9,
In 2000, North and Golding reported a five times 44, 55, 57, 82, 84, 85, 125, 140, 239], but two
increased risk of a hypospadias-affected son for large studies and a meta-analysis concluded that
women with a vegetarian diet [175], a finding that smoking reduces the risk of hypospadias [110,
was confirmed in one study [85], but not in others 238, 240]. One study reported increased hypo-
[55, 84, 223, 224]. A meta-analysis failed to reach spadias risk when the mother experienced stress
conclusive results [225]. The suggestion that an due to bereavement before or during pregnancy
increased risk might be related to intake of phytoes- [241], while another study could not confirm
trogens was refuted by a small study involving phy- these results [242]. One small study found mater-
toestrogen-specific questionnaires that did not find nal cocaine use to be associated with hypospadias
an association [111] and by two studies using food [243], while another study found an association
frequency questionnaires that found a decreased for cannabis use [244].
hypospadias risk with phytoestrogen intake ≥90th
percentile and an increased risk with isoflavone
intake ≤10th percentile [226, 227]. Another dietary 10.9 Occupational Factors
factor found to be associated with hypospadias is
vitamin E intake [228], while two small studies 10.9.1 Exposure to Pesticides
found an association with frequent consumption of
fish, possibly associated with the bioaccumulation Occupational exposures have been a major focus
of contaminants in fish [118, 135]. However, two in hypospadias research, especially exposure to
larger case-control studies showed a decreased pesticides, with contradicting results. Paternal
hypospadias risk for frequent fish consumption [85, exposure to pesticides before pregnancy does not
229], and one of these concluded that “health-con- seem to be associated with hypospadias [22, 55,
scious” subjects (with a high frequency of consump- 84, 245], although one small study reported a
tion of fresh fruit and vegetables, dried fruit, fresh or possible increased risk [135]. In addition, an
frozen fish, beans, pulses, soya products, olive oil, increased risk was found among farmers who
and organic food) have a lower risk of hypospadias were indicated as being exposed to pesticides in a
in offspring [229]. The results on the effect of con- register-based study [246] and among New
sumption of organic foods vary [229–231]. Zealand soldiers who served in Malaya and were
Most studies showed no effects of folate on exposed to dibutyl phthalate applied daily to their
hypospadias risk [55, 57, 140, 232–234], although clothing [247]. Most studies showed no associa-
two studies found a reduced risk of folic acid sup- tion with maternal occupational exposure to pes-
plementation [223, 235]. In addition, the propi- ticides [16, 55, 84, 245, 248–250], but being
onylcarnitine concentration (indicative of vitamin involved in agricultural activities [17] or using
B12 deficiency) was higher in newborns with hypo- insect repellents [251, 252] seemed to increase
spadias compared to controls [236]. Iron supple- hypospadias risk in some studies. In addition,
mentation does not seem to influence hypospadias several pesticide concentrations were higher in
risk in most studies [55, 82, 224], but two studies the hair of hypospadias patients and their parents
showed an increased risk of iron supplementation compared to the levels reported for the general
[84, 175]. High maternal dietary glycemic intake population [253].
was not associated with hypospadias [237].
10.9.2 Other Occupational Exposures

10.8.3 Other Lifestyle Factors
Boys conceived to mothers employed in the
Alcohol consumption during pregnancy was con- leather industry [254] and to mothers who
sistently found not to be associated with hypo- served in the Gulf war [255] seemed to have a
higher prevalence of hypospadias. Most other In two studies, the prevalence of hypospadias
maternal occupational exposures were not seemed to be higher in areas of intensive pesti-
associated with hypospadias, although expo- cide use or in agricultural areas [82, 292]. In
sure to hairspray increased the risk in two addition, a study looking at exposure to pesticide
studies and results for exposure to EDCs, application within a 500-m radius of the mother’s
heavy metals and phthalates vary across stud- residential address also found an association with
ies [16, 55, 84, 118, 141, 223, 248, 249, 256– hypospadias [293], while another study did not
258]. A systematic review concluded that there [294]. Another study showed an increased risk
is inadequate evidence in support of an asso- for living in an area where diclofop methyl was
ciation between phthalate exposure and hypo- applied but a decreased risk for alachlor and per-
spadias [259]. For fathers, being a vehicle methrin exposure or for pesticide application in
mechanic or manufacturer [260, 261], police aggregate [9]. Hypospadias occurred more often
officer or firefighter [260], as well as occupa- in areas where exposure to atrazine was higher
tional exposure to dusts from grinding metals [220], but atrazine exposure via drinking water
[55] seemed to increase the risk of having a did not increase the risk of hypospadias [295].
son with hypospadias. Results on heavy met- In some studies, a seasonal trend for hypospa-
als vary [16, 249], but most other paternal dias was identified [296–300], which was attrib-
occupational exposures were not associated uted to factors such as hours of daylight, climate,
with hypospadias [16, 55, 84, 249]. or temperature, whereas other studies did not find
Several studies measured biomarkers in bio- seasonal variation [18, 82, 301].
logical samples, such as heavy metals, organo-
chlorines, chlordane, polychlorinated biphenyls,
phthalates, phenols, and bisphenol A in maternal 10.11 Conclusion
urine or hair, amniotic fluid, placentas, or urine or
plasma of patients, but different substances were Hypospadias affects approximately 0.5% of male
measured in each study and the results vary [118, births. It was suggested that the prevalence of
262–279]. hypospadias is increasing, but whether or not this
is true cannot be concluded based on the cur-
rently available literature. Hypospadias shows
10.10 Living Environment familial clustering, and familial occurrence
seems to be more common for distal and middle
The results for living in rural or (sub)urban areas forms of hypospadias than for proximal types.
are not completely consistent but suggest higher The chance that a brother of an affected boy or
risks in urban areas [17, 20, 22, 39], whereas liv- that a son of an affected father will also have
ing close to a landfill site [280] or in a deprivated hypospadias is approximately 13%. In 30% of
neighborhood also seemed to be associated with the patients with the proximal form of hypospa-
an increased risk of hypospadias [281]. One dias, a cause can be identified, but in the majority
study showed that hypospadias prevalence is of patients, the etiology remains unknown and is
higher in centers on high altitude [282], and likely to be multifactorial.
another study showed that maternal exposure to Development of the male external genitalia is
solvent-contaminated water increases hypospa- dependent on the balance between androgens and
dias risk [283]. Five other studies found an asso- estrogens. The fact that maternal exposure to syn-
ciation with periconceptional exposure to thetic estrogens can induce hypospadias in
different types of air pollution [284–288]. murine models [302] and that antiandrogens act-
Maternal exposure to water disinfection by- ing as inhibitors of steroid hormone synthesis or
products was also suggested to increase hypospa- androgen receptor antagonists can induce male
dias risk, although most studies provided little reproductive abnormalities in animal models
evidence for this association [289–291]. [303] suggests that EDCs have the potential to
induce hypospadias. However, because of con- factors. We found that genetic variants in DGKK
siderable species differences and markedly dif- were associated with hypospadias in the anterior
ferent estrogen levels in humans compared to cases, but the posterior cases showed weaker
rodent pregnancy, it is debatable whether EDCs associations [305]. In addition, familial cluster-
also induce hypospadias in humans and whether ing of hypospadias was much more pronounced
exposure levels in humans are high enough to in the anterior and middle hypospadias cases than
exert this effect. Given that even exposures to in the posterior forms, whereas primiparity, pre-
high levels of exogenous hormones, such as in eclampsia, low birth weight or being small for
case of hormonal stimulation used to induce gestational age, and in utero exposure to oral con-
pregnancy and use of oral contraceptives while traceptives showed a more obvious risk increase
pregnant, do not show consistent associations
with hypospadias, we suggest that exogenous Table 10.1 Clinical, behavioral, occupational, and envi-
hormones and EDCs may not be as important in ronmental factors investigated for their association with
the etiology of hypospadias as has previously hypospadias in more than one study (Table adapted from
van der Zanden et al., 2012 [304])
been assumed.
The consistent association of hypospadias Factors frequently investigated
Factors with consistent results in all studies
with low birth weight, maternal hypertension,
Factors consistently Factors consistently
preeclampsia, and absence of nausea in early associated with hypospadias not associated with
pregnancy suggests that placental insufficiency Low birth weight/being hypospadias
may be a major risk factor for hypospadias, pos- small for gestational age Maternal alcohol
sibly through inadequate provision of hCG to the Placental insufficiency consumption
Maternal hypertension
fetus. A role for endogenous hormones is sug- Preeclampsia
gested by free estradiol levels linked to high Maternal intrauterine
maternal BMI, primiparity, and multiple preg- diethylstilbestrol exposure
nancies that appear to contribute to susceptibility Factors with consistent results in most studies
to hypospadias. In addition, maternal intrauterine Factors associated with Factors not associated
hypospadias in most studies with hypospadias in
DES exposure, use of anti-epileptic drugs, pro- Use of ICSI most studies
longed time-to-pregnancy, and pregnancies Prolonged Use of oral
resulting from ICSI seem to be associated with time-to-pregnancy contraceptives
hypospadias. Other potential environmental risk High maternal BMI during pregnancy
Primiparity Maternal medication
factors were not, or not consistently, associated Multiple pregnancy use:
with hypospadias or studied too infrequently to Maternal medication use: Loratadine
draw conclusions. Table 10.1 provides a sum- Anti-epileptic drugs Maternal vegetarian
mary of environmental factors investigated in Absence of nausea and diet/isoflavone
vomiting in early intake
relation to hypospadias. pregnancy Paternal age
A factor that we found to be potentially of Factors showing inconsistent results
great importance when performing studies on Use of hormonal Maternal smoking
hypospadias was the location of the urethral stimulation Maternal and
opening. The development of the various forms to induce paternal exposure to
pregnancy pesticides
of hypospadias in different time windows, possi- Use of IVF Maternal
bly through disturbances of dissimilar processes, Maternal age occupational
could be an explanation. The labioscrotal swell- Maternal preexisting or exposure to:
ings fuse to form the scrotum, while closure of gestational diabetes Endocrine
Maternal iron disruptors
the urethral folds contributes to the formation of supplementation Heavy metals
the penile urethra. The mechanism behind the Maternal folate Phthalates
development of the glandular portion of the ure- supplementation Maternal stress
thra is not exactly clear yet. Most likely, these Maternal fish Seasonal trend
consumption Preterm delivery
different processes are influenced by specific risk
Table 10.1 (continued) 3. Yu X, Nassar N, Mastroiacovo P, et al. Hypospadias

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Factors frequently investigated
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Genetic Aspects of Hypospadias
11
Loes F. M. van der Zanden
Abbreviations 11.1 Genes Involved

in the Embryology
AMH Anti-Müllerian hormone of the Male External
AR Androgen receptor Genitalia
ATF3 Activating transcription factor 3
BMP Bone morphogenetic proteins Development of the male external genitalia can
CAH Congenital adrenal hyperplasia be divided in three stages:
CAIS Complete androgen insensitivity
syndromes • The indifferent stage.
DGKK DiacylGlycerol kinase κ • The early patterning stage.
DHT DiHydroTestosterone • The masculinization stage.
DSD Disorder of sex development
ESR EStrogen receptors
FGF Fibroblast growth factor proteins 11.1.1 Indifferent Stage
GT Genital tubercle
hCG Human chorionic gonadotropin Early development of the external genitalia is
MAMLD1 MAstermind-like domain contain- similar for males and females. The embryonic
ing 1 cloaca, the far end of the hindgut, is separated
PAIS Partial androgen insensitivity from the amniotic cavity by the cloacal mem-
syndromes brane. Early in the fifth week of development, a
Sf1 Splicing factor 1 swelling develops on both sides of this membrane,
SHH Sonic HedgeHog the cloacal folds, which meet in the midline ante-
SRD5A Steroid-5-alpha-reductase rior to the cloacal membrane, forming the genital
SRY Sex-determining region Y gene tubercle [1]. At the same time, the genital ridges,
Wt1 Wilms tumor 1 the precursors of the gonads, develop. Studies in
mice showed that this process requires Wilms
tumor 1 (Wt1) activity, which activates splicing
factor 1 (Sf1) [2], thus preventing degeneration of
the developing gonads [3]. During the seventh
week of human development, the urorectal sep-
L. F. M. van der Zanden (*)
Department for Health Evidence, Radboud University tum fuses with the cloacal membrane, dividing
Medical Center, Nijmegen, The Netherlands the cloaca into the primitive urogenital sinus and
e-mail: Loes.vanderZanden@Radboudumc.nl the rectum and dividing the cloacal membrane
272 L. F. M. van der Zanden
into the urogenital and the anal membrane. The of NR5A1 (Fig. 11.1) [13]. Testosterone leaves
swellings next to the urogenital membrane are the Leydig cell and is converted into dihydrotes-
then called the urogenital folds, and a new pair of tosterone (DHT) by steroid-5-alpha-reductase
swellings, the labioscrotal swellings, appears on (SRD5A). Testosterone promotes formation of
either side of these folds. In addition, the urogeni- the internal reproductive structures from the
tal membrane breaks down [1]. Wolffian ducts, whereas DHT induces develop-
ment of the external genitalia [1], both through
their effects on the androgen receptor (AR).
11.1.2 Early Patterning Expression of estrogen receptors (ESR) in male
genital tissue during development suggests that
Early patterning of the genital tubercle (GT) is the balance between androgens and estrogens is
androgen-independent. The distal urethral plate important as well [14].
epithelium is the signaling center regulating GT
outgrowth [4]. Fibroblast growth factor proteins
(FGF) play a growth-promoting role in this out- 11.2 Genes Implicated
growth [5], whereas bone morphogenetic pro- in the Etiology of Isolated
teins (BMP) stimulate apoptosis [6, 7]. Sonic Hypospadias
hedgehog (SHH) modulates the balance between
proliferation and apoptosis by regulating the All genes implicated in one of the three stages
expression of the genes encoding these and mentioned above could play a role in the devel-
many other proteins [8]. This way, SHH regu- opment of hypospadias. Therefore, much of the
lates the initiation of GT outgrowth [4]. genetic research on hypospadias has been focused
Immunohistochemical staining of human fetal on these genes.
penises showed expression of SHH, its receptor,
and several of its downstream genes around the
time of urethral closure [9]. 11.3 Study Types
Different types of studies have been performed to

11.1.3 Masculinization examine whether specific genes have an effect on
the occurrence of hypospadias. One example are
Subsequent masculinization relies on hormones the mutation analyses, for which researchers
produced by the testes. Expression of the sex- sequence candidate genes in hypospadias patients
determining region Y gene (SRY) induces a cas- and healthy controls. These studies typically
cade of gene interactions, involving SRY-box 9 included tens to sometimes a little over a hundred
(SOX9) [1], resulting in differentiation of the hypospadias patients and healthy controls.
gonads into the testes [10]. SRY leads to the dif- Sequencing allows complete coverage of the
ferentiation of Sertoli cells [1], which secrete gene, identifying all genetic variants present in
anti-Müllerian hormone. Anti-Müllerian hor- that gene. With this type of studies, researchers
mone causes regression of the Müllerian ducts were aiming to identify the causal variant for
that would otherwise form part of the female hypospadias in part of their patients. Although
genital structures [1]. Human chorionic gonado- several studies identified new and unknown
tropin (hCG), produced by the placenta, controls mutations in hypospadias patients that were not
fetal Leydig cell growth and stimulates fetal tes- present in the healthy controls, it remains unclear
ticular steroidogenesis, the generation of steroids whether these mutations truly have functional
from cholesterol [11]. The enzymatic steps in ste- consequences. Only few studies reported conser-
roidogenesis, mainly taking place in the Leydig vation and function of the region in which the
cell, are well documented, and expression of key mutation is located or predicted a potential influ-
genes in this pathway is dependent on expression ence of the mutation on protein function using
11 Genetic Aspects of Hypospadias 273
Fig. 11.1 Steroidogenesis in the mitochondrium (top) I.A.L.M.: “Aetiology of hypospadias: a systematic review
and smooth endoplasmic reticulum (bottom) of the fetal of genes and environment,” Human Reproduction Update,
Leydig cell. Adapted from van der Zanden et al., 2012 2012, Vol. 18, Issue 3, by permission of Oxford University
[12] (from van der Zanden, L.F.M. and van Rooij, Press)
bioinformatics. Even if the mutations found are spadias patients and healthy controls have been
truly causal, they only explain the occurrence of genotyped for polymorphisms in candidate genes.
hypospadias in a small part of the patients, and If a polymorphism occurs more frequently in the
the majority of mutations were found only once patients compared to the controls, this means that
and were identified in patients with posterior or there is an association and the variant may be a
penile hypospadias. marker for a causal variant that increases hypo-
Another type of study that has been used to spadias risk. However, due to the large amount of
examine whether specific genes have an effect on polymorphisms examined, the risk of false-posi-
the occurrence of hypospadias is the association tive results is high. Therefore, replication of
study. In this type of study, known polymorphisms results is a major issue in association studies,
are genotyped in hypospadias patients and healthy especially in the genome-wide association studies
controls. Polymorphisms are common genetic that aim to cover the whole genome by genotyp-
variants that occur with a frequency of at least ing hundreds of thousands of polymorphisms.
1%. Due to the haplotype block structure of the
human genome, genotyping a specific set of poly-
morphisms in a gene covers much more of the 11.4 Study Results
variation in that gene. Thereby, polymorphisms
function as markers for more rare variants with Table 11.1 shows the candidate genes that were
functional consequences. In association studies, screened for mutations or associations in groups
typically hundreds to sometimes a thousand hypo- of hypospadias patients and healthy controls.
Table 11.1 Result of genetic association studies and mutation studies screening candidate genes in groups of patients
with hypospadias and healthy controls. Genes that were only investigated once and did not show mutations or associa-
tions in that study are not shown
Mutation studies that Association studies that Mutation studies that did Association studies that
found mutations found associations not find mutations did not find associations
Indifferent stage
WT1 Wang et al. [15], Carmichael et al. [17] Nordenskjöld et al.
Diposarosa et al. [16] [18], Kon et al. [19],
Zhang et al. [20]
WTAP Utsch et al. [21] Carmichael et al. [17]
Early patterning
SHH Carmichael et al. [17] Zhang et al. [20]
FGF8 Beleza-Meireles et al. Beleza-Meireles et al. Kon et al. [19], Zhang Carmichael et al. [17]
[22] [22] et al. [20]
FGF10 Carmichael et al. [17] Beleza-Meireles et al.
[22], Zhang et al. [20]
FGFR2 Beleza-Meireles et al. Beleza-Meireles et al. Kon et al. [19], Zhang Carmichael et al. [17]
[22] [22] et al. [20]
BMP4 Chen et al. [23], Zhang Kon et al. [19] Carmichael et al. [17]
et al. [20]
BMP7 Chen et al. [23], Bouty Carmichael et al. [17] Beleza-Meireles et al.
et al. [24] [22], Kon et al. [19],
Zhang et al. [20]
HOXA4 Chen et al. [23] Geller et al. [25] Kon et al. [19], Zhang Carmichael et al. [17],
et al. [20] Kojima et al. [26],
Chen et al. [27]
HOXB6 Chen et al. [23], Kon et Zhang et al. [20] Carmichael et al. [17]
al. [19]
HOXA13 Utsch et al. [21], Zhang Carmichael et al. [17]
et al. [20]
HOXD13 Zhang et al. [20] Carmichael et al. [17]
GLI1 Carmichael et al. [17]
GLI2 Carmichael et al. [17]
GLI3 Zhang et al. [20] Carmichael et al. [17]
ZFPM2 Zhang et al. [20]
CDH7 Zhang et al. [20]
Masculinization
SRY Wang et al. [15], Zhang Carmichael et al. [17]
et al. [20]
SOX9 Wang et al. [15], Zhang
et al. [20]
NR5A1 Köhler et al. [28], Kalfa et al. [32], Kon et Adamovic et al. [30]
Allali et al. [29], al. [19], Zhang et al.
Adamovic et al. [30], [20]
Laan et al. [31]
AR Hiort et al. [33], Alléra Lim et al. [40], Aschim Muroya et al. [46], Muroya et al. [46],
et al. [34], Sutherland et al. [41], Radpour et Radpour et al. [42], Vottero et al. [47],
et al. [35], al. [42], Parada- Kalfa et al. [32] Silva et al. [48]
Nordenskjöld et al. Bustamante et al. [43],
[18], Wang et al. [15], Adamovic and
Thai et al. [36], Nordenskjold [44],
Borhani et al. [37], Kon Adamovic et al. [45]
et al. [19], Yuan et al.
[38], Zhang et al. [20],
Chen et al. [39]

FKBP4 Beleza-Meireles et al. Beleza-Meireles et al.
[49], Zhang et al. [20] [49]
CYP1A1 Kurahashi et al. [50], Kon et al. [19], Zhang Yadav et al. [53]
Carmichael et al. [51], et al. [20]
Mao et al. [52]
CYP1A2 Qin et al. [54]
CYP3A4 Carmichael et al. [51] Qin et al. [54]
CYP11A1 Zhang et al. [20] Carmichael et al. [51]
CYP17A1 Qin et al. [54], Mao et Samtani et al. [55],
al. [52] Yadav et al. [56],
Carmichael et al. [51]
CYP19A1 Qin et al. [54],
Carmichael et al. [51]
HSD3B1 Chen et al. [39] Carmichael et al. [51]
HSD3B2 Codner et al. [57], Kon Zhang et al. [20] Carmichael et al. [51]
et al. [19]
HSD17B3 Sata et al. [58], Thai et al. [36], Kon et
Carmichael et al. [51] al. [19], Yuan et al.
[38], Zhang et al. [20]
SRD5A1 Tria et al. [59] Carmichael et al. [51]
SRD5A2 Silver and Russell [60], Silver and Russell Nordenskjöld et al. van der Zanden et al.
Wang et al. [15], Thai [60], Wang et al. [15], [18], Kalfa et al. [32] [63], Adamovic et al.
et al. [36], Kon et al. Thai et al. [36], Sata et [45]
[19], Rahimi et al. [61], al. [58], Samtani et al.
Yuan et al. [38], Zhang [55], Carmichael et al.
et al. [20] [51], Samtani et al.
[62], Rahimi et al. [61]
STAR Zhang et al. [20] Carmichael et al. [51]
STARD3 Carmichael et al. [51]
STS Carmichael et al. [51]
Other genes
ESR1 Watanabe et al. [64], Beleza-Meireles et al. Beleza-Meireles et al.
van der Zanden et al. [68], Kon et al. [19], [68]
[63], Tang et al. [65] Zhang et al. [20]
Choudhry et al. [66]
Ban et al. [67]
ESR2 Beleza-Meireles et al. Beleza-Meireles et al. Aschim et al. [70]
[68], Beleza-Meireles [68], Kon et al. [19],
et al. [69], Ban et al. Zhang et al. [20]
[67], Choudhry et al.
[66], van der Zanden
et al. [63]
ATF3 Beleza-Meireles et al. Beleza-Meireles et al. Kon et al. [19], Zhang
[71], Kalfa et al. [72] [71], van der Zanden et al. [20]
et al. [63]
MAMLD1 Fukami et al. [73], Chen et al. [75], Kalfa Kalfa et al. [78], Kon et Liu et al. [79]
Kalfa et al. [74], Chen et al. [78], Ratan et al. al. [19], Zhang et al.
et al. [75], Kalfa et al. [77] [20]
[32], Igarashi et al.
[76], Ratan et al. [77]
(continued)

DGKK van der Zanden et al. Kon et al. [19], Zhang Kojima et al. [26],
[80], Carmichael et al. et al. [20] Richard et al. [85]
[81], Geller et al. [25],
Ma et al. [82],
Hozyasz et al. [83],
Xie et al. [84], Chen et
al. [27]
TGFBR2 Han et al. [86]
CTGF Kon et al. [19], Zhang
et al. [20]
BNC2 Bhoj et al. [87], Kon et Zhang et al. [20]
al. [19]
MID1 Zhang et al. [88] Zhang et al. [88] Kon et al. [19], Zhang
et al. [20]
INSL3 El Houate et al. [89],
Zhang et al. [20]
GSTM1 Yadav et al. [53] Kon et al. [19], Zhang Kurahashi et al. [50]
et al. [20]
GSTT1 Yadav et al. [53] Kon et al. [19] Kurahashi et al. [50]
ARNT2 Qin et al. [54]
NR1I2 Qin et al. [54]
AKR1C2 Soderhall et al. [90], Mares er al. [91]
Zhang et al [20] Mares
et al. [91]
AKR1C3 Soderhall et al. [90] Mares et al. [91] Soderhall et al. [90],
Mares et al. [91]
AKR1C4 Soderhall et al. [90], Soderhall et al. [90],
Zhang et al. [20], Mares et al. [91]
Mares et al. [91]
KLF6 Soderhall et al. [82] Soderhall et al. [90]
RYR1 Zhang et al. [92]
PKDCC Geller et al. [25] Kojima et al. [26],
Chen et al. [27]
HAAO Geller et al. [25] Kojima et al. [26],
Chen et al. [27]
DNAH6 Geller et al. [25] Kojima et al. [26],
Chen et al. [27]
EEFSEC Geller et al. [25] Kojima et al. [26],
Chen et al. [27]
PDGFC Geller et al. [25] Kojima et al. [26],
Chen et al. [27]
DAAM2 Geller et al. [25] Kojima et al. [26],
Chen et al. [27]
TAX1BP1 Geller et al. [25] Kojima et al. [26],
Chen et al. [27]
EYA1 Geller et al. [25] Kojima et al. [26],
Chen et al. [27]
CCDC26 Geller et al. [25] Kojima et al. [26],
Chen et al. [27]
KCNMA Geller et al. [25] Kojima et al. [26],
Chen et al. [27]

GREM1 Geller et al. [25] Kojima et al. [26],
Chen et al. [27]
IRX5 Geller et al. [25] Kojima et al. [26],
Chen et al. [27]
IRX6 Geller et al. [25] Kojima et al. [26],
Chen et al. [27]
ZFHX3 Geller et al. [25] Kojima et al. [26],
Chen et al. [27]
EYA1 Geller et al. [25] Kojima et al. [26]
EXOC3 Geller et al. [25]
PROKR2 Zhang et al. [20]
TRIM17 Zhang et al. [20]
SLC25A5 Chen et al. [27]
SP1 Chen et al. [27]
Genes that were only investigated once and did 47, 48, 95–98], while others suggested decreased
not show mutations or associations in that study DHT binding capacity of the AR in genital skin
are not shown. The table shows that only few fibroblasts of patients with hypospadias [34, 99].
studies focused on genes involved in the indiffer- Although not all studies confirmed these results
ent and early patterning stage. Although some [100–103], evidence that a defect in AR or
mutations and associations were found in these SRD5A2 may cause or be a risk factor for hypo-
genes, there is no gene that shows clear evidence spadias is compelling.
of being involved in hypospadias etiology.
Research on genes involved in the masculin-
ization stage has been much more extensive. 11.5 Other Genes
Notably, although expression of the SRY gene,
located on the Y chromosome, is crucial for devel- Not only steroidogenesis but also the balance
opment of the testis from the indifferent gonad between androgens and estrogens appears to be
([10, 93]), there is not much evidence that this important in the development of the male exter-
gene plays a role in the development of hypospa- nal genitalia. The estrogen receptors ESR1 and
dias. Research was especially focused on AR and ESR2 are expressed in the developing human
SRD5A2. SRD5A2 converts testosterone to the male GT [14], and mRNA expression levels seem
more potent androgen DHT, and both testosterone to be decreased in foreskin of hypospadias
and DHT exert their effect through the AR. The patients compared to controls [95]. Although no
AR is expressed in the developing human penis mutations were found in ESR1 or ESR2, associa-
and urethra, and SRD5A2 is expressed during tions have been reported between hypospadias
male genital development around the ventral part and several polymorphisms in the genes encod-
of the remodeling urethra [94]. Multiple studies ing these receptors (Table 11.1), and evidence for
found mutations and associations with polymor- these genes to play a role in hypospadias devel-
phisms in these genes (Table 11.1). In addition, opment is quite strong.
there are other studies that indicated the involve- Other genes for which evidence is quite strong
ment of the AR in hypospadias etiology. For are activating transcription factor 3 (ATF3),
example, some studies indicated different expres- mastermind-like domain containing 1 (MAMLD1),
sion levels in patients compared to controls [39, and diacylglycerol kinase κ (DGKK). ATF3 is an
estrogen-responsive gene showing strong upregu- 11.6.2 46,XY DSD

lation in hypospadias [72, 104–107], and several
studies found mutations in this gene in hypospa- Mutations in genes implied in testosterone
dias patients or polymorphisms in this gene to be biosynthesis (such as 3-beta-hydroxysteroid-
associated with hypospadias (Table 11.1). dehydrogenase, 17-beta-hydroxysteroid-deshy-
MAMLD1, previously known as CXorf6, contains drogenase and 17-alpha hydroxylase) can lead to
the NR5A1 target sequence [108] and mutations, testosterone defects which may present as DSD.
and polymorphisms in MAMLD1 were found in
patients with hypospadias (Table 11.1). Ogata
et al., concluded that MAMLD1 mutations exert 11.6.3 The Dysgenetic Gonad
their effect primarily via compromised testoster-
one production around the critical period for sex The dysgenetic gonad and more specifically the
development [109], and Ratan et al., found lower dysgenetic testis is characterized by insufficient
testosterone levels than the mean for their age in production of testosterone and anti-Müllerian
80% of subjects carrying a polymorphism in hormone (AMH). Although some etiologies are
MAMLD1 [77]. DGKK was identified as a major well identified, the histological definition of the
risk gene for hypospadias in a genome-wide asso- gonadal dysgenesis remains unclear [112] as well
ciation study [80], a result that was confirmed by as the possible underlying genetic anomalies. It is
several other studies (Table 11.1). A study in mice commonly associated with insufficient develop-
revealed that differentiated GT epithelial cells are ment of the GT, impaired testicular descent, and
DGKK positive, while undifferentiated preputial persistence of Müllerian structures. It is most
lamina epithelial cells are DGKK negative, sug- likely that gonadal dysgenesis is a dynamic pro-
gesting that DGKK is a marker or mediator of cess leading to a progressive loss of testicular
squamous cell differentiation [110]. functions. The major concern about dysgenetic or
underdeveloped gonads is the development of
cancers from immature germinal cells [113].
11.6 ommon Clinical Conditions
C
with Gene Defects
11.6.4 Partial and Complete
11.6.1 4
6,XX Disorder of Sex Androgen Insensitivity
Development (DSD) Syndromes
The most frequent example of DSD in the 46,XX 5α-Reductase deficiency is an autosomal reces-
group is congenital adrenal hyperplasia (CAH), sive syndrome. It is a rare disorder in western
mostly resulting from a deficit of 21-hydoxylase countries but frequent in the Dominican Republic,
which causes an abnormal growth of the GT and New Guinea, and Gaza strip due to high consan-
an inappropriate opening of the vagina into the guinity [114]. These 46,XY patients present with
posterior wall of the urethra. quite feminine genitalia at birth which will get
It is important to realize that pubertal and virilized after puberty if the testes are left in
post-pubertal women (46,XX) with CAH develop place. Some newborn babies may present with a
their GT if they are not compliant to their hor- slightly more developed hypospadiac micropenis
monal substitutive treatment. This shows that GT which may grow with topical dihydrotestoster-
target tissues remain responsive to steroids until one. These patients represent one of the most dif-
the organ reaches an ultimate size. ficult situations where gender assignment is an
In western world, most patients with CAH are issue as well as the fate of the testes.
raised as female but severely virilized CAH in Partial and complete androgen insensitivity
male-dominant societies or late diagnosis may syndromes (PAIS/CAIS) are related to impaired
lead to a male assignment [111]. androgen receptors. CAIS does not raise any gen-
der issue as these individuals have normal female 11. Misrahi M, Beau I, Meduri G, Bouvattier C, Atger
M, Loosfelt H, Ghinea N, Hai MV, Bougneres PF,
external genitalia and will be raised as females Milgrom E. Gonadotropin receptors and the control
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12. van der Zanden LFM, van Rooij IALM, Feitz WFJ,
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13. Scott HM, Mason JI, Sharpe RM. Steroidogenesis in
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Hormones and Growth
of the Genital Tubercle 12
Mohamed Fawzy and Ahmed T. Hadidi
Abbreviations MRI Magnetic resonance imaging

NPY Neuropeptide Y
AGD Anogenital distance P Progesterone
AIS Androgen insensitivity syndromes PAIS Partial androgen insensitivity
AR Androgen receptor syndrome
AREs Androgen response elements SHBG Sex hormone binding globulins
CAIS Complete androgen insensitivity
syndrome
cAMP Cyclic adenosine monophosphate
CHH Congenital hypogonadotropic 12.1 Introduction
hypogonadism
CNS Central nervous system The sexual differentiation and growth of male
DHT Dihydrotestosterone genitalia is a complex chromosomal, hormonal,
DSD Disorders of sex development and environmental process [1]. The general the-
E Estrogen ory of sexual determination and sexual differen-
FSH Follicle-stimulating hormone tiation is discussed. The cascades that influence
GH Growth hormone development and differentiation from conception
GnRH Gonadotropin-releasing hormone to death are not well understood. In this chapter,
GT Genital tubercle the role of different hormones involved in the
HPT Hypothalamic-pituitary-gonadal process of sexual differentiation is explained,
IGF-1 Insulin growth factor according to the current knowledge. This may
KNDy Kisspeptin, neurokinin B, and enable the surgeon to understand and identify the
dynorphin different forms of sexual disorders and determine
LH Luteinizing hormone how and when to use hormone replacement
MAIS Mild androgen insensitivity syndrome therapy.
MIH Müllerian inhibiting hormone The final appearance of the external genital at
MPW Masculinization programming window birth is due to interaction between different genes
(SRY and DAX1) as well as different hormones
including GnRH, LH, FSH, testosterone, leptin,
M. Fawzy (*) · A. T. Hadidi growth hormone, thyroid hormones, and insulin.
Hypospadias Center and Pediatric Surgery Any disturbance in these mechanisms may result
Department, Sana Klinikum Offenbach, Academic in different genital disorders. Moreover, environ-
University, Offenbach, Frankfurt, Germany mental factors influence the development and
286 M. Fawzy and A. T. Hadidi
growth of external genitalia. This chapter sum- believed that the inherited inequality of X and Y
marizes the most important factors related to genetic material from the start affects the entire
genital growth and the effect of each factor indi- body including gonads [1, 3].
vidually. Disorders of sexual development are
beyond the scope of this chapter and book.
12.3 Theory of Sexual
Differentiation
12.2 The Genital Tubercle
(a) Sex is determined by the sex hormones. The
The genital tubercle (GT) appears during the different genes carried on sex hormones (XY
fourth gestational week due to condensation of in males, XX in females) are responsible for
mesenchyme at the anterior part of the cloaca sex differentiation [1].
(Fig. 12.1). At the same time, the labioscrotal (b) Males are different from females due to the
folds develop on either side of the urethral folds effect of the Y chromosome. The Y chromo-
(Fig. 12.2). some carries the SRY gene.
(c) The Y chromosome has gonadal and extrago-
nadal effects:
12.2.1 Chromosomal and Genetic –– Gonadal effects: The SRY gene is
Effects on Sexual responsible for the differentiation of the
Determination and Sexual gonads into testis. Likewise, the absence
Differentiation of SRY gene leads to differentiation into a
female with the subsequent hormone pro-
The sexual determination takes place very early, duction according to the gonadal differen-
and it was observed that in some animals, non- tiation [4].
gonadal tissues develop in a different way –– Extragonadal effects of Y genes: This
between males and females even before the could be demonstrated by the effect of
beginning of the gonadal differentiation. It is SRY gene on the brain and is believed to
a b
Fig. 12.1 (a and b) The genital tubercle appears at view of the same embryo (from: An Atlas of Human
6 weeks. The cloaca membrane is delineated by urogenital Prenatal Developmental Mechanics, by Jan E. Jirasek, ©
folds (UF). Anterior is the genital tubercle (GT), and lat- 2004. Reproduced by permission of Taylor & Francis
eral caudally is the anal tubercle (AT). (b) The anterior Group [2])
12 Hormones and Growth of the Genital Tubercle 287
a b
Fig. 12.2 (a and b) The early indifferent external genita- are the labioscrotal folds (LSF) (from: An Atlas of Human
lia are formed by the phallus which is composed of the Prenatal Developmental Mechanics, by Jan E. Jirasek, ©
glans (GL), the corpora cavernosa bodies, and the uro- 2004. Reproduced by permission of Taylor & Francis
genital groove (arrow) with two urogenital folds. Laterally Group [2])
be the reason why male brains function scription rate that the other X chromosome
differently from female brains [5]. [1, 7].
(d) The number of X chromosomes. –– There is evidence of increased hetero-
–– In females: When a cell has more than chromatic activity in XX compared to
one X chromosome, the noncoding RNA XY, which is believed to affect the expres-
Xist is expressed and plays a major role in sion of autosomal genes [1, 8].
the transcription of this X chromosome (e) Sex biasing mechanisms: These are mecha-
rendering the cell different from those nisms that influence sexual differentiation.
carrying only one X chromosome [1, 6]. Some diseases or environmental factors may
–– Overexpression of X escape genes inhi- affect sexual differentiation, e.g., the inheri-
bition in females (XX): When a cell car- tance of mitochondrial DNA from the mother
ries more than one X chromosome, some to the daughter allowing mitochondrial
genes carried on the inactive X chromo- alleles to be selected which are in most cases
some escape the inhibition and start tran- useful to the females but not the males
scription. As a result, the overall (Mothers’ curse) [1, 9].
transcription of X chromosomes in an XX
is more than its rate in an XY, which is
believed to play a role in the female phe- 12.4 he Balance Between Sex
T
notypes [1, 6]. Determination Genes
–– In females (XX) one X chromosome is
inherited from the father (XY) and the The SRY gene is carried on the Y chromosome,
other X chromosome is inherited from the while the DAX1 gene is carried on the short arm
mother (XX). The X chromosome inher- of the X chromosome. When the SRY gene is
ited from the mother has a higher tran- present (in males), it takes the upper hand, and
mediated by (StAR) protein it stimulates the dif- HCG during the first part of gestation and later by
ferentiation of gonads into testis that in turn starts fetal LH. The HCG levels increase gradually
the testosterone production inducing the further from the eighth week to reach the maximum level
differentiation of the male sexual characters. The between tenth and 14th gestational weeks. The
production of StAR is considered the first step in fetal pituitary LH starts to appear at about 14th
steroidogenesis [10]. gestational week and reaches the maximum level
When the SRY gene is absent (as in females), at about 25th weeks of gestation.
the DAX1 genes carried on both XX chromo- In other words, the hormones that influence
somes act without competition and induce the the development of the genital tubercle through
differentiation of the gonads into ovaries due to the hypothalamic-pituitary-gonadal axis can be
the downregulation of StAR protein [10] summarized as follows:
(Fig. 12.3).
• The placenta secretes two groups of hormones
(from eighth week):
12.4.1 H
ormonal Effects on Sexual –– Human chorionic gonadotropin (like LH).
Differentiation and Genital –– Estrogens (E) and progesterone (P).
Growth • The hypothalamus secretes:
–– Gonadotropin-releasing hormone (GnRH).
In males, the presence of SRY gene stimulates • The anterior pituitary (gonadotrope cells)
the differentiation of gonadal cells into testis at under GnRH secretes:
about sixth week of gestation. –– Luteinizing H (LH): stimulates testoster-
The development of the genital tubercle is one H from testis.
under the effect of testosterone secreted by –– Follicle-stimulating H (FSH): stimulates
Leydig cells under the influence of the placental spermatogenesis.
a b
Fig. 12.3 (a) In males (XY), SRY takes the upper hand. (b) In females (XX), DAX1 acts unopposed (©Ahmed
• The fetal testis has two active cells:

–– Sertoli cells secreting Müllerian-inhibiting
H (MIH).
–– Leydig cells secreting testosterone.
12.5 Hypothalamic-Pituitary-
Gonadal (HPG) Axis
Activation (Fig. 12.4)
The hypothalamic-pituitary-gonadal (HPG) axis

is activated three times in life leading to a tripha-
sic hormonal surge that plays a major role in the
process of sexual differentiation and genital
growth [11]:
1. During fetal life: which is responsible for sex

determination and differentiation.
2. Mini-puberty.
3. Puberty.
12.5.1 During Fetal Life (First Surge)
During prenatal period, four main elements influ-

ence sex differentiation: the fetus, the placenta,
the mother, and the environment.
12.5.1.1 The Fetus

The hypothalamus secretes gonadotropin-
releasing hormone (GnRH). This hormone stim-
ulates the gonadotrope cells in the anterior Fig. 12.4 Feedback regulation of the hypothalamic-
pituitary gland at about the ninth gestational pituitary-testicular axis in males. Stimulatory effects are
week to secrete two gonadotropic hormones: shown by plus signs, and negative feedback inhibitory
effects are shown by minus signs. CNS (central nervous
system), FSH (follicle-stimulating hormone), GnRH
–– Luteinizing hormone (LH) is the primary (gonadotropin-releasing hormone), LH (luteinizing hor-
stimulus for testosterone secretion. mone). This figure was published in the “Guyton and Hall
–– Follicle-stimulating hormone (FSH) that stim- Textbook of Medical Physiology,” pp1019–1022, 14th
ulates spermatogenesis. ed., by Hall and Hall, with permission from ©Elsevier
2020 [12]
In the absence of GnRH from the hypothala-

mus, the gonadotrope cells in the pituitary secrete ative feedback of placental estrogens in which the
almost no gonadotropins (LH or FSH) [12]. The gonadotropins show a sex-related pattern [11, 13].
pituitary gonadotropins secretion starts at about The Wolffian ducts develop around the fourth
the ninth gestational week to decline slowly week of gestation, while the Müllerian ducts
toward the end of pregnancy mediated by the neg- develop at the end of the sixth week lateral to the
Wolffian ducts. In males, guided by the presence starts at 8 weeks and reaches the maximum
of SRY gene, the gonadal cells start to differenti- between 10 and 14 weeks.
ate into testis that contains Sertoli and Leydig –– Placental estrogen (E) and progesterone (P):
cells. their peaking during the third trimester leads
The Sertoli cells produce a locally acting to a negative feedback inhibition on LH and
Müllerian-inhibiting hormone (MIH) between FSH production [11, 17, 18].
the eighth and ninth weeks of gestation. The
Müllerian-inhibiting hormone (MIH) is a glyco- The placental HCG influences testosterone
protein responsible for the degeneration of production from the eighth week till about 14th
Müllerian ducts. week of gestation. After that the fetal pituitary
The Leydig cells appear at about the seventh LH takes over and starts to be detectable at
week of the gestation with subsequent testoster- 14–15 weeks to reach its highest value at
one production that acts on the Wolffian duct 25 weeks of gestation [19, 20].
transforming it into the male genital tract till the During fetal life exposure to androgens leads
end of the 11th week of gestation [10]. to expression of Fgfr2, a gene that plays a key
role in the development and tubularization of ure-
Testosterone and Dihydrotestosterone thra, and the deletions in this gene proved to
At a molecular level, the produced testosterone cause different degrees of hypospadias [21]. The
diffuses inside the target cells, where some of it role of androgen was evident when the exposure
is reduced into the more potent form to flutamide (antiandrogen) during pregnancy
5α-dihydrotestosterone under the effect of the leads to the downregulation of transcription of
microsomal enzyme 5α reductase enzyme. Both Fgfr2 gene and subsequent development of hypo-
testosterone and DHT bind to the intracellular spadias [21].
androgen receptors to form a complex that dif- The exposure to testosterone during fetal life
fuses inside the nucleus and work on the DNA is essential to the development of external geni-
androgen-sensitive genes. Testosterone, dihy- talia. Welsh et al. (2008, 2014) identified in rats
drotestosterone, and androstenedione are col- a masculinization programming window (MPW)
lectively called androgens [14]. Both induce that begins with testosterone production.
differentiation of the Wolffian ducts into epi- Sufficient androgen action must occur during
didymis, vas deference, and seminal vesicles. this MPW to permit normal penile development.
The genital tubercle continues to grow after the Any androgen deficiency during this period may
14th week in a linear pattern at a rate of 0.7 mm/ cause hypospadias and/or smaller penis at
week, even after cessation of testosterone peak puberty. This MPW may also occur in humans
suggesting that other factors may play a role between 8 and 14 weeks of gestation. On the
[15]. other hand, when pregnant animals were injected
with large amounts of testosterone, they devel-
12.5.1.2 The Placenta oped male sexual organs even when the fetus
During pregnancy, the placenta secretes: was female; likewise, experimental bilateral
orchidectomy led to the development of female
–– Human chorionic gonadotropin (HCG): acts sexual organs [22].
as an analog to LH producing similar biologi- The classic mechanism of testosterone signal-
cal effects when it binds to LH receptors stim- ing occurs when testosterone diffuses into the
ulating the testis to produce testosterone with cell and binds to androgen receptor (AR). AR is a
subsequent development of male sexual single-copy gene on the X chromosome. Then,
organs [11, 16, 17]. The HCG production the ligand-receptor complex translocated to the
nucleus where it binds to androgen response ele- lack of negative feedback on the GnRH and reac-
ments (AREs) in the regulatory regions of the tivation of the HPG axis.
genes to modify their translation.
The androgen insensitivity syndromes (AIS) 12.5.1.3 The Mother
fall within the generic category of 46,XY DSD Maternal diseases and medications have been
(disorders of sex development) and present as reported to influence the development of the
phenotypes associated with complete or partial penis during the first trimester. A study has found
resistance to the action of androgens. Three cat- an association between pregestational hyperten-
egories are recognized: complete androgen insen- sion and hypospadias [26].
sitivity syndrome (CAIS), partial androgen
insensitivity syndrome (PAIS), and mild andro- 12.5.1.4 The Environment
gen insensitivity syndrome (MAIS). The andro- The role of environmental factors is discussed in
gen receptor (AR) is encoded by an 8 exon gene detail in Chap. 9: Epidemiology.
on the X chromosome long arm. More than 800
mutations in the AR gene have been reported in
AIS patients. 12.5.2 Mini-puberty (Second Surge)
Complete androgen insensitivity syndrome
(CAIS) is an X-linked recessive genetic disor- Minipuberty starts immediately after birth and is
der resulting from maternally inherited or de characterized by elevated levels of LH, FSH, and
novo mutations involving the androgen recep- sex hormones in both males and females. There is
tor gene, situated in the Xq11-q12 region. The reactivation of the HPG axis after cessation of the
diagnosis is based on the presence of female negative feedback inhibition of placental hor-
external genitalia in a 46,XY human individual, mones (estrogen and progesterone).
with normally developed but undescended tes- Hormone levels are similar to the levels dur-
tes and complete unresponsiveness of target tis- ing true puberty, hence the name Minipuberty
sues to androgens. Subsequently, pelvic [28]. The minipuberty is characterized by an
ultrasound or magnetic resonance imaging elevation of LH and FSH during the first week
(MRI) could be helpful in confirming the of age, to reach the highest levels between the
absence of Müllerian structures, revealing the first and third months of age, and slowly
presence of a blind-ending vagina and identify- decreases to almost undetectable levels toward
ing the testes. CAIS management still repre- the sixth month of age [18, 29, 30]. The minipu-
sents a unique challenge throughout childhood berty is evident in both sexes but with different
and adolescence, particularly regarding timing trends in hormone levels. Preterm birth does not
of gonadectomy, type of hormonal therapy, and affect the patterns of hormone levels during
psychological concerns. minipuberty [11].
In addition, growth hormone (GH) may also
play an indirect role in the development of the –– Males: LH peaks more than FSH. The lev-
genital tubercle. There is no difference of GH els of testosterone follow the same patterns
levels between male and female fetuses suggest- as LH levels. The number of Leydig cells
ing that GH may act only in the presence of high corresponds to the LH levels [31], whereas
testosterone level in male fetuses or through a the number of Sertoli cells corresponds to
permissive effect of the action of gonadotropins the FSH levels [32]. This leads to a subse-
on Leydig cells [19, 23–25]. quent increase in the testicular volume dur-
At birth, the placental hormones stop circulating the first months of life till the age of
ing in the neonate’s circulation. This results in a 2 years [33]. The testosterone surges during
Fig. 12.5 Minipuberty of infancy: Schematic presenta- 3 months of age and then slightly decrease but remain
tion of the changes in reproductive hormone levels during higher than in adults until puberty. Penile length and tes-
the first year of life in healthy boys. The peak hormone ticular volume increase, and testicular descent continues
levels are observed between 1 and 3 months of age during minipuberty (modified from Kuiri-Hänninen et at.,
(between the lines). LH and testosterone levels decrease Copyright © 2019, Kuiri-Hänninen, Koskenniemi,
by 6 months of age, but FSH and inhibin B levels remain Dunkel, Toppari, and Sankilampi, CC Attribution Licence
elevated longer. AMH levels increase from birth to (CCBY) [27])
the first 3 months of life lead to penile [11]. Maternal exposure to substances such as
growth [34]. phthalates is associated with low levels of testos-
–– Females: FSH peaks more than LH. The lev- terone during minipuberty [36]; see Chap. 9:
els of estrogen follow the same pattern as FSH Epidemiology.
levels [31].
This hormonal difference is crucial for sexual 12.6 Clinical Application

differentiation and growth [11]. Early exposure and Importance
to androgens during the prenatal period and mini- of Minipuberty
puberty plays a major role in the penile growth
later in life [35]. Anogenital distance (AGD) can The understanding of minipuberty can be helpful
be used as a biomarker that indicates androgen in the early diagnosis of disorders of sex develop-
exposure in the prenatal period. Penile growth in ment (DSD) as follows:
the first 3 months of life is a good biomarker for
androgen exposure in the minipuberty (Fig. 12.5) –– Males with congenital hypogonadotropic
[35]. Both AGD and penile growth can be used as hypogonadism (CHH): Absent first and second
powerful indicators of increased masculine/ surges of LH, FSH, and testosterone with sub-
decreased feminine behavior in early childhood sequent infertility and cryptorchidism [37].
[35]. The highest rate of penile growth (1 mm/ –– Vanishing testis (congenital anorchism): LH
month) was observed during the first months of and FSH levels are elevated during minipu-
life, where serum testosterone levels surge (mini- berty, while testosterone levels are undetect-
puberty) [34]. able [38].
Minipuberty is not only genetically predeter- –– Differentiation between complete and partial
mined but can also be environmentally influenced androgen insensitivity. Depending on the
residual activity of the androgen receptors, penis will continue to grow but slows down after-
these patients can present with a wide spec- wards [44, 45]. This suggests that androgens are
trum of clinical presentation starting from not the only factors that influence penile growth.
complete female phenotype on one side (com- At one year, the penile dorsal radius is approxi-
plete androgen insensitivity) to only undervir- mately 40 mm and may reach 60 mm before
ilization or infertility on the other side (partial puberty. Other hormones such as growth hor-
androgen insensitivity). These patients have mone and adrenal hormones may play a role.
the genotype XY, and gender identity can
sometimes be challenging; thus, karyotyping
is essential in the diagnosis of such patients. In 12.6.1 Puberty (Third Surge)
addition to karyotyping clinical assessment,
measurement of hormone levels (FSH, LH, Starts with the onset of puberty [11]. This is
and testosterone) is important [39]. equivalent to the loss of feedback inhibition on
–– Girls with congenital adrenal hyperplasia are GnRH and the increase of its pulsatile secretion
more exposed to androgen, and they show evi- [46]. Anti-Müllerian factor levels drop, while
dent behavioral changes as preferring boys’ inhibin B levels rise. The volume of testis
toys and activities [40]. increases, and the sensitivity of Leydig cells to
LH hormone increases leading to a rise in testos-
These examples may indicate that the mea- terone levels [47, 48].
surement of serum LH, FSH, and testosterone The neurons of GnRH in the hypothalamus
during first week of life, first, third, and sixth are responsible for the initiation of puberty. What
months of life, may be a helpful early detection triggers this initiation remains unclear. However,
tool in children suspected with DSD [41]. The many theories were developed regarding this
simulation of hormone patterns in minipuberty by increase in GnRH release including body fat
giving children with micropenis and CHH exog- composition, stress, nutritional status, melatonin,
enous short-term recombinant LH and FSH thyroid hormones, exercise, and fitness, but they
instead of the standard treatment with intramus- all remain without a conclusive evidence [46].
cular testosterone proved to be successful as the Recently, it is believed that a higher control from
penile length increased with 50% and testicles the arcuate nucleus mediated by Kisspeptin, neu-
grew 3 times more in size [42]. This response was rokinin B, and dynorphin (collectively called
further demonstrated in a study in which 10 KNDy) may be the reason behind the pulsatile
infants and neonates with micropenis due to manner of GnRH release [49]. The GnRH stimu-
hypogonadotrophic hypogonadism were sub- lates the release of FSH and LH from the anterior
jected to treatment with recombinant LH and FSH pituitary. The LH triggers the release of testoster-
preparations with subsequent normalization of one from Leydig cells. This mechanism is known
penile length of all children after treatment [43]. as hypothalamic-pituitary-gonadal axis [50].
The high levels of testosterone during minipu- At a cellular level, LH binds to a G-protein-
berty lead to a negative feedback inhibition of coupled LH receptor. This leads to an increase in
GnRH in the hypothalamus and LH levels from cAMP (cyclic adenosine monophosphate) that
the pituitary. The drop in LH levels affects the induces the synthetic pathway of testosterone
testosterone-producing Leydig cells. The Leydig from cholesterol (41).
cells undergo apoptosis with gradual decrease in From the second surge till the onset of puberty,
the testosterone levels till the sixth month of life, there is a continuous negative feedback from tes-
and the testosterone levels remain almost non- tosterone, LH, and GnRH release. When the
detectable till the onset of puberty [14]. puberty starts, this negative feedback mechanism
Between minipuberty and puberty: Testicular is no longer dominant, and the levels of GnRH and
androgen production is undetectable after LH start to surge, leading to a subsequent increase
6 months of age. During the first year of life, the in the testosterone levels. This is followed by the
physiological changes that take place during and the release of GnRH. Leptin suppresses
puberty including the increase in testicular volume appetite by inhibiting the release of hypothalamic
and penile length. Further substances like insulin NPY, while it stimulates the release of GnRH
growth factor (IGF-1) and growth hormone (GH) [58]. On the other hand, NPY inhibits the release
show a rise in their levels secondary to testoster- of GnRH and is believed to maintain this inhibi-
one increase and are believed to play a role during tion throughout childhood [59]. The release of
puberty [51]. This negative feedback of testoster- this inhibition is believed to trigger the onset of
one does not appear to affect the FSH levels, which puberty [59]. Optimizing the body weight during
is believed to be controlled by inhibin B. Inhibin B puberty is essential for leptin production and to
is produced by Sertoli cells and acts directly on the avoid puberty dysfunction. Leptin stimulates the
pituitary gland [52]. FSH stimulates the spermato- pulsatile release of GnRH from hypothalamus by
genesis [53]. stimulating the expression of KiSS-1 gene pres-
In addition, the levels of SHBG (sex hormone ent in the Kiss-1 neurons [60].
binding globulins) markedly decrease during
puberty, leading to more circulating testosterone
in free form and further negative feedback inhibi- 12.8 Environmental Influence
tion of SHBGs [54]. on Genital Growth
The role of growth hormone during puberty
has been also discussed, and it is believed that In a population-based cross-sectional study that
many secondary characteristics during puberty included 6200 males between 0 to 19 years mea-
occur directly and indirectly secondary to suring the testicular volume and the penile length,
increased levels of GH in blood because of the there was no increase in the testicular volume
stimulatory effect of androgens. The high levels until the age of 11 years. The penile length
of GH act directly on the target organs together showed gradual increase since birth. The peak
with IGF1. Another mechanism is believed to be growth was demonstrated between 12 and
the relative insulin resistance during puberty. The 16 years in both testicular volume and penile
fact that GH is an anti-insulin results in increased length corresponding to puberty. This study
levels of insulin as well, with relative insulin showed a significant difference between urban
resistance in glucose metabolism during puberty; and rural areas, which can raise the question
however, the more important result of elevated about the environmental influence on the penile
insulin levels is believed to be the increased growth until puberty [61]. In addition, and as pre-
anabolism acting synergistically with IGF1 and viously mentioned, the maternal exposure to dif-
circulating androgens [55, 56]. ferent substances may affect the testosterone
The role of testosterone and gonadotropins levels during and after pregnancy, with subse-
during puberty was evident in treatment of chil- quent effects on genital differentiation and
dren with isolated micropenis (stretched penile growth during fetal life and after birth.
length 2.5 standard deviations less than the mean
for age). Administration of testosterone in chil-
dren less than 11 years and HCG in children 12.9 Tanner Classification
more than 11 years demonstrated a significant
increase on penile length [57]. The changes that occur to the male genital organs
during puberty are classified into five stages
according to Tanner classification as follows [62]
12.7 Role of Leptin (Fig. 12.7):
The proper understanding of these mecha-
Leptin is produced by adipocytes. It antagonizes nisms is essential to understand and treat clinical
the neuropeptide Y (NPY) produced in the hypo- conditions related to hormonal disorders and
thalamus. Both leptin and NYP act on appetite pathological sexual conditions.
Fig. 12.6 The different stages of male sexual function as ages (This figure was published in the “Guyton and Hall
reflected by average plasma testosterone concentrations Textbook of Medical Physiology,” pp1019–1022, 14th
(red line) and sperm production (blue line) at different ed., by Hall and Hall, ©Elsevier 2020, modified [12])
Adulthood and senior years: Although testicu- • Three hormonal surges are responsible for the
lar testosterone production continues until andro- development of male genital organs (prenatal,
pause and beyond (Fig. 12.6), post-pubertal minipuberty, puberty).
androgens do not seem to affect the adult penile • Feedback mechanisms are responsible for reg-
size. Any exogenous testosterone or dihydrotes- ulating different hormonal production in the
tosterone have almost no effect on the penile size hypothalamic-pituitary-gonadal axis.
in adulthood. This is probably due to the decrease • Growth hormone, thyroid hormones, and
of androgen receptors after puberty [63], although leptin also play a role in the genital growth.
this is still controversial [64]. • Many environmental factors affect the rate of
genital growth and the outcome of genital
development during fetal life.
12.10 Summary • Tanner staging can be a useful tool to control
the rate of development during puberty.
• Sexual differentiation and development of • Hormonal alterations early in pregnancy may
male genital organs is influenced by genetic, cause hypospadias. Hormonal alterations after
hormonal, and environmental factors. the second trimester and full development of
• SRY gene carried on chromosome Y is essen- the penis may cause micropenis.
tial for sex differentiation and testicular
development.
Fig. 12.7 Tanner stages in boys showing changes in September 24, 2021, from https://commons.wikimedia.
external genitalia, pubic hair, and testicular volume [62] org/w/index.php?title=File:Tanner_scale-m ale.
File:Tanner scale-male.svg. (2021, June 4). Wikimedia svg&oldid=567207648
Commons, the free media repository. Retrieved 16:17,
ing in human and mouse. Nat Genet. 2015; https://doi.

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The Role of Preoperative
Androgen Stimulation 13
in the Management
of Hypospadias
Fardod O’Kelly and Luis H. Braga
Abbreviations width) is an independent risk factor for post-opera-

tive complications following hypospadias repair by
DHT Dihydrotestosterone a number of institutions; however, recent evidence
hCG Human chorionic gonadotropin has shown the contrary [4–6].
PAS Preoperative androgen stimulation The rationale behind the use of preoperative
testosterone, as well as dihydrotestosterone
(DHT), and human chorionic gonadotropin
(hCG) arises from prior observational studies
13.1 Introduction which demonstrated that topical and parenteral
administration of these products led to a tempo-
Hypospadias is the second most common male rary increase in penile length, glans circumfer-
genital birth defect with an incidence of 1 in every ence, and tissue vascularity and thus would – in
250 male births. The repair of this condition theory – allow for a more successful repair in
requires the availability of good-quality tissue to more technically challenging cases [7, 8]. This
reconstruct a neourethra within the glans penis led to the widespread use of androgen stimula-
which will allow for low pressure voiding within a tion before hypospadias repairs despite a lack of
distensible tube, while trying to minimize potential high levels of evidence to support this practice.
complications such as glans dehiscence, meatal Clinical guidelines published by the European
stenosis, and the development of urethra-cutaneous Urological Association and European Society of
fistulae. Glans width has been shown to be reduced Pediatric Urology have not been able to provide a
in patients with proximal hypospadias compared to clear consensus statement regarding the use of
age-matched controls [1–3]. Furthermore, given preoperative androgen stimulation (PAS) due to
the reconstruction requirements, it has been sug- divergences of opinion about the hormone ther-
gested that the glans width (but not urethral plate apy of choice, time of use, appropriate dose, and
means of application (topical or parenteral) but
F. O’Kelly nonetheless have given it a level 1B recommen-
Departments of Urology and Paediatric Surgery, dation [9–11]. This has been evidenced by a
Beacon Hospital and UCD School of Medicine and number of provider surveys regarding its use.
Medical Sciences, Dublin, Ireland
Malik et al. surveyed members of the American
L. H. Braga (*) Academy of Pediatrics and found that 87% high
Division of Urology, Department of Surgery,
McMaster Children’s Hospital, McMaster University,
volume surgeons (>50 cases per year) regularly
Hamilton, Ontario, Canada used testosterone stimulation for a small appear-
e-mail: braga@mcmaster.ca ing penis, reduced glans circumference, reduced
300 F. O’Kelly and L. H. Braga
urethral plate width, and/or proximal hypospa- versally demonstrate an improvement in penile
dias, with the effect being determined by glans biometrics post-administration. Many of the
appearance [12]. A survey of the use of testoster- studies describe an increase in penile stretched
one was sent to Turkish urologists which was length, cosmesis, and parental penile perception
mirrored by one carried out in Nigeria, both of score [15, 16, 21–23]. The majority of these stud-
which demonstrated highly varied indications for ies are based on proximal hypospadias cases, and
use with no standardized protocol or criteria, and all acknowledge the need for further larger con-
up to 34% administrations are being used in cases trolled studies. Interestingly, a study of the long-
of distal hypospadias [13, 14]. term outcomes of testosterone use for hypospadias
repair failed to demonstrate any difference in
adult penile stretched length or penile cosmesis;
13.2 vidence of Use in Distal
E however, the response rate was 50% and the
Hypospadias authors called for the need of a randomized study
[19]. The most promising outcomes to date
There is little evidence to justify the use of PAS in appear in the sub-cohort of microphallic hypo-
boys with distal hypospadias, as very few ran- spadias patients in which PAS can lead to signifi-
domized controlled trials have been conducted on cant improvements in penile size without a
this topic. In one carried out by Menon et al., the corresponding increase in complications [24].
authors concluded that PAS in these cases should
be used judiciously and that the study itself was
underpowered to demonstrate a meaningful out- 13.4 Adverse Effects
come [15]. Another trial carried out by Babu et al.
demonstrated an improvement in postoperative Concerns have been raised relating to the effects
parental penile perceptions scores with PAS, but of PAS on wound healing and intraoperative
there was nearly a 20% non-response rate [16]. bleeding rates, as well as the temporary growth of
This is not a surprising result given the higher lev- pubic hair. This has been an area of specific con-
els of androgen receptor expression in proximal troversy relating to the use of testosterone and
hypospadias cases [17]. The only randomized one in which opponents quote regularly. The
trial to show a reduction in postoperative compli- results from the literature display significant het-
cations with PAS was conducted by Asgari et al., erogeneity. Mohammadipour et al. described that
but it also showed a non-response to testosterone side effects, such as thick pubic hair growth, fre-
rate of 4.3% [18]. Further non-randomized retro- quent erections, or considerable penis hyper-
spective studies have demonstrated varying sensation, were observed in 27.8%, as well as
degrees of success with the PAS for distal cases decreased glans wing to corporal body thickness,
but at best reached statistical non-inferiority with leading to a more complex intraoperative glans-
proximal cases [19]. Austin et al. demonstrated plasty [25]. Gorduza et al. described statistically
that even in distal cases, elevated serum Müllerian nonsignificant but clinically concerning delayed
inhibiting substance and decreased serum testos- post-operative healing rates with the use of tes-
terone were identified, and therefore this may tosterone in 30% of patients [26]. This was a
explain some of the beneficial effects but does not small series to reach statistical significance, and
constitute any definite recommendations [20]. there was again considerable heterogenicity
regarding the patients, treatment regimen, and
PAS indication. Snodgrass and Bush, analyzing
13.3 Outcomes factors which affected re-operative rates for prior
hypospadias repair, found that PAS increased the
The hypospadias repair outcomes from studies odds of requiring further surgery (OR 2.57, 95%
exploring PAS arise mostly from non-randomized CI 1.53–4.31), which was not affected by age or
trials with small sample sizes. These nearly uni- type of surgery. It should be noted that this may
13 The Role of Preoperative Androgen Stimulation in the Management of Hypospadias 301
also reflect the severity of the initial condition, Zhao et al. identified 13 publications from a
which is in itself a risk factor for future complica- total of 1278, which met inclusion criteria. They
tions [27]. were interested in the concept of androgen resis-
PAS was also used in more severe cases of tance in hypospadias and demonstrated that 25 of
hypospadias (penile length < 25 mm, hypoplasia, 306 patients have this phenomenon, which resulted
and associated undescended testes) [26]. Wong in a prevalence of 7.14% (95% CI: 3.16%–15.31%)
and Braga suggested that these increases in com- following exclusion of heterogenicity [33].
plications may be related to poor study method- Chao et al. analyzed 428 patients from 6 stud-
ology and the lack of testosterone standardization ies that included primary hypospadias repair, of
[28]. It may also be as a result of increased tissue which 171 (39.95%) received some form of PAS,
vascularization and edema. On the other hand, in either with human chorionic gonadotropin, dihy-
the only randomized controlled trial of 75 con- drotestosterone, or testosterone. They found the
secutive boys, transdermal dihydrotestosterone risk ratio for a complication following preopera-
was shown to decrease complications and tive hormone administration at 1.18 (95% CI:
improve cosmetic results, including a reduction 0.7–2.0, p = 0.54), and despite of significant vari-
of penile curvature [29]. An earlier study by ability in the literature, surgical technique, patient
Davits et al. had shown that there were also no age, and study quality were not causes for this
long-term results of preoperative testosterone heterogenicity [34].
stimulation on bone maturation or height [30]. More recently, Chua et al. identified 21 publi-
cations from 16 trials examining the effect of
PAS on postoperative outcomes. Subgroup anal-
13.5 Systematic Reviews ysis based on study design showed no significant
and Meta-Analyses difference in postoperative outcomes (RR 1.31,
95% CI 0.95–1.81) [35].
There have been a total of 5 systematic reviews All of these studies were repetitive in their
and/or meta-analyses published examining the conclusions stating that the published literature is
role of PAS in hypospadias repair. Wright et al. of low quality and lacks standardized reporting of
included 11 studies in their review (622 patients), important patient and surgical details. The effect
of which most were retrospective, and 45% of PAS on hypospadias repair outcomes remains
patients were administered preoperative hor- unclear and requires further investigation.
monal stimulation (most commonly intramuscu- Furthermore, the scarcity of randomized con-
lar testosterone). Four of these studies addressed trolled clinical trials on this topic impairs the
post-operative complication rates, with an odds establishment of evidence bases clinical guide-
ratio for a complication at 1.67 (CI 0.96–2.91, lines. Therefore, although preoperative hormone
p = 0.07, I(2) = 0%). No persistent side effects therapy is currently used before hypospadias sur-
due to PAS were reported [31]. gery, its real benefit in terms of improvement of
Netto et al. identified 14 clinical trials, which surgical results has not been defined. Furthermore,
fit their inclusion criteria. They were not able to more patient-centered risk-stratified approaches
identify an ideal preoperative treatment when are required to determine who might best benefit
taking into account severity of hypospadias, from PAS and those that may exhibit androgen
association with chordee and cryptorchidism, resistance.
type of hormone, route of application, dose and
duration of treatment, penile length before and
after hormone therapy, glans circumference 13.6 odes of Delivery, Dosage,
M
before and after hormone therapy, adverse effects, and Schedule
and surgical complications. They found that side
effects were inconsistently described, and fol- Dosing regimens of PAS for hypospadias remain
low-up was short [32]. inconsistent and author dependent. Some advocate
for the use of intramuscular testosterone based on is still a paucity of good data to support clinical
patient size. The three most common practices are guidelines. Given the high number of pediatric
based on weight (2 mg/kg/dose for 2–3 doses), urologists in the USA that administer testoster-
body surface area (100 mg/m2), or a general fixed one preoperative, further research is needed to
dose (25 mg/dose) given once a month for determine whether PAS improves surgical out-
2–3 months preoperatively [36, 37]. These have come. Furthermore, possible long-term side
been shown to not have any association with effects should be assessed in large prospective
delays in bone maturation [38]. cohorts. Parents should be informed about the
Similar effects can be achieved with topical benefits and risks of PAS.
10% testosterone propionate cream applied twice
daily. If topical dihydrotestosterone is used, its
concentration typically ranges from 2.5% to 3% References
applied daily for up to 3 months [29, 37].
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to Surgery MA. Increased hand digit length ratio (2D:4D) is
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hypospadias: testosterone therapy prior to surgical
Timing of Surgery
14
Ahmed T. Hadidi
14.1 Introduction 14.2 Emotional Development
Several factors influence, directly or indirectly, During the first year of life, a strong and stable
the timing of elective repair of hypospadias in mother-father-child relationship is established.
childhood. The most important of them are the This attachment may be affected by the presence
age-related anesthetic and surgical risks and ben- of a congenital birth defect. From this point of
efits, in relation to the psychological impact of view, the period from 6 months to approximately
the procedure, during the various stages of the 15 months of age may be a relatively less difficult
child’s development [1]. emotional developmental period for surgery, if
The American Academy of Pediatrics, in parental separation is kept to the minimum. In
1975, concluded that hypospadias surgery was that way a non-life-threatening congenital defect
best performed after a child’s third birthday. such as hypospadias can be corrected as early as
Since that time, the operative strategy has been possible, and the family relationships are not
modified as a result of factors such as (1) the irreparably disturbed. The next most appropriate
understanding of the psychological implications time for operative procedure is the period from
of genital surgery in children, (2) the improve- 24 to 36 months, in which the trauma of an opera-
ment in the technical aspects of surgery for hypo- tion is less difficult. However, that period has the
spadias, and (3) the advances in pediatric disadvantage of prolonging the child’s defective
anesthesia [1]. status and crystallizing any disruption in family
The psychological factors include the child’s relationships [2, 3].
emotional development, its sexual development, Another important factor in determining the
and the psychological effects of surgery and impact of hospitalization is the family situation.
anesthesia. This variable is also the most difficult to change.
Normal postoperative and post-hospitalization
reactions have been noted in children from fami-
lies with good relationships among the individual
members. Children from families with no or less
communication face anxiety about separation
and surgery and show an adverse emotional reac-
A. T. Hadidi (*) tion postoperatively [1].
Department, Sana Klinikum Offenbach, Academic The psychological preparation for hospitaliza-
Teaching Hospital of the Johann Wolfgang Goethe tion and operation is of great importance for a
University, Offenbach, Frankfurt, Germany normal reaction. Both the parents and the child
306 A. T. Hadidi
feel insecure and are not sure what is going to be child, characteristics of the illness or the treat-
done. Diagrams, slides, or a movie with details of ment, degree of pain during hospitalization, and
hospitalization and surgery may be helpful for presence or absence of the mother during anes-
parents and for the child and may reduce the thesia [9].
postoperative psychological disturbance. Parents’ The highest incidence of postoperative emo-
presence beside their child before anesthesia and tional disturbance has been noted at the ages of
during recovery is also important [1]. 1–3 years. The most common problems are pro-
longed night terrors, negativism, and various
fears including hysterical reactions, phobias, and
14.3 Sexual Development anxiety reactions. Children at that age are
strongly dependent on their mother, do not have
A child with a birth defect of the external genita- extensive social contacts outside the home, and
lia such as hypospadias may develop distortions show a decreased facility to handle anxiety.
of body image. The defect per se may reflect Because of that, they represent postoperatively a
other people’s communicative evaluations of the greater emotional hazard and develop behavioral
child’s body, and it is also important for paternal changes due to emotional trauma in 10% of cases
response. The repair of hypospadias as early as [10, 11]. Inadequate preoperative sedation in
possible will help achieve a psychologically young children may result in excessive preopera-
healthy body image. On the other hand, a per- tive fear and anxiety in addition to postoperative
son’s sexual body image is largely a function of emotional disturbances [12]. Brief hospitaliza-
socialization [4, 5]. Boys with hypospadias at tion, minor surgical pain, and skillful anesthesia
school age more frequently have gender-atypical result in no residual behavioral effects in the
behavior than boys with normal external genitalia majority of children [13].
[6].
The repair of hypospadias before 30 months
of age seems to be important because the aware- 14.5 Improvement in Technical
ness of the deformity begins at that time. Aspects of Surgery
Furthermore, boys’ socialization at that age and Advances in Pediatric
includes comparison of genitalia, and a boy who Anesthesia
sits to urinate or who has a visible penile defect
will be exposed to the social response of his Technical considerations used to be limiting fac-
peers. Moreover, from the age of 30 months to tors in determining the time for hypospadias sur-
5.5 years, the fear of physical harm is significant. gery in the past. At that time, the reconstructive
Boys who undergo hypospadias repair before the techniques were multistage, with correction of
age of 30 months do not suffer from anxiety that the chordee and urethral reconstruction separated
their sex may be changed or their genitalia muti- by an interval of 6–12 months. The duration of
lated [7, 8]. hospitalization was 5–14 days each time, mostly
without parental rooming-in. Today, increased
surgical experience has changed the operative
14.4 Psychological Effects rules. Single-stage hypospadias repair is per-
of Surgery and Anesthesia formed in most cases of hypospadias (80%), with
the multistage procedure reserved for perineal
The psychological effects of surgery and anesthe- and complicated malformations [14–18]. The use
sia in children depend on the age of the patient, of micro-instrumentation and delicate suture
the duration of hospitalization, and the behavior material and also the routine use of optical mag-
of the anesthesiologist. Factors that are consid- nification have made the operative procedure
ered less significant include birth order, prior technically feasible in small infants [19, 20].
hospitalization, preoperative behavior of the Experienced pediatric urology surgeons can per-
14 Timing of Surgery 307
form early reconstructive surgery with cosmetic for hypospadias [29]. The optimal window rec-
and functional results equal to those achieved in ommended for repair was about 6–15 months of
older children [21, 22]. Furthermore, parental age (Fig. 14.1 and Table 14.1). Delicate surgical
rooming-in is the standard in almost all centers, techniques, optical magnification, and careful
and in that way the psychological effects can be hemostasis have encouraged many surgeons
decreased even more. On the other hand, children (including the author) to operate when the patient
are hospitalized for shorter times despite the fact is between the ages of 3 and 9 months, with very
that the operative techniques have become more satisfactory results [30, 31].
complex and the children are younger at the time
of operation. Hypospadias is performed as an
outpatient procedure or with only overnight hos- References
pitalization in some centers. The most important
positive outcome is the lessening of emotional 1. Kelalis P, Bunge R, Barkin M. The timing of elec-
tive surgery on the genitalia of male children
trauma from parent-child separation [23]. with particular reference to undescended testes
On the other hand, the dramatic improvements and hypospadias. Pediatrics. 1975; https://doi.
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ment of mother-infant interaction. Psychoanal Study
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respiratory problems and cardiovascular Psychoanalysis Developmental Psychology (1985) by
Daniel N. Stern. Int J Early Child. 1987; https://doi.
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of the importance of minimizing the period of girl: the differentiation and dimorphism of gender
parent-child separation [24–27]. identity from conception to maturity. Baltimore:
Johns Hopkins University Press; 1972.
Because a normal penis grows only about 5. Money J, Norman BF. Gender identity and gender
0.8 cm between the ages of 1 and 3 years [28], the transposition: Longitudinal outcome study of 24 male
size of the phallus is not an important technical hermaphrodites assigned as boys. J Sex Marital Ther.
consideration. There can be considerable varia- 1987; https://doi.org/10.1080/00926238708403881.
6. Sandberg DE, Meyer-bahlburg HFL, Aranoff GS,
tion in phallic size, but a penis that is small at Sconzo JM, Hensle TW. Boys with hypospadias: a
6 months will still be a small penis at the age of survey of behavioral difficulties. J Pediatr Psychol.
3 years. One excellent study compared the emo- 1989; https://doi.org/10.1093/jpepsy/14.4.491.
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ety in boys and girls. Ann Arbor, MI: University of
Michigan; 1960.
8. Lane RW. The effect of pre-operative stress on
dreams. Eugene, OR: University of Oregon; 1966.
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J Pers Soc Psychol. 1967; https://doi.org/10.1037/
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Fig. 14.1 Evaluation of risk for hypospadias repair from Fam Physician. 1974:10.
birth to age 7 years. The optimal window is from 3 to 13. Davenport HT, Werry JS. The effect of general anes-
15 months of age (modified from Schultz et al. [29]) thesia, surgery and hospitalization upon the behavior
308 A. T. Hadidi
Table 14.1 Stretched penile length (centimeters) (Data from Feldman and Smith, J Pediatr. 86:895, 1975 [32],
Schonfeld and Beebe, J Urol. 30:554, 1975 [28], Tuladhar et al., J Paediatr Child Health. 34:471, 1998 [33])
Age Mean ± SD Mean ± 2.5 SD
Newborn, 30 weeks’ gestation 2.5 ± 0.4 1.5
Newborn, 34 weeks’ gestation 3.0 ± 0.4 2.0
0–5 months 3.9 ± 0.8 1.9
6–12 months 4.3 ± 0.8 2.3
1–2 years 4.7 ± 0.8 2.6
2–3 years 5.1 ± 0.9 2.9
3–4 years 5.5 ± 0.9 3.3
4–5 years 5.7 ± 0.9 3.5
5–6 years 6.0 ± 0.9 3.8
6–7 years 6.1 ± 0.9 3.9
7–8 years 6.2 ± 1.0 3.7
8–9 years 6.3 ± 1.0 3.8
9–10 years 6.3 ± 1.0 3.8
10–11 years 6.4 ± 1.1 3.7
Adult 13.3 ± 1.6 9.3
of children. Am J Orthopsychiatry. 1970; https://doi. and children: a prospective survey of 40240 anaes-
org/10.1111/j.1939-0025.1970.tb01093.x. thetics. Br J Anaesth. 1988; https://doi.org/10.1093/
14. Duckett JW. The island flap technique for hypospadias bja/61.3.263.
repair. 1981. J Urol. 2002; https://doi.org/10.1016/ 25. Smith RM. Anesthesia for infants and children. 4th
s0022-5347(05)65115-4. ed. St. Louis: Mosby; 1980.
15. Belman AB. The modified Mustarde hypospa- 26. Mayhew JF, Guiness WS. Cardiac arrest due to anes-
dias repair. J Urol. 1982; https://doi.org/10.1016/ thesia in children. J Am Med Assoc. 1986; https://doi.
S0022-5347(17)53619-8. org/10.1001/jama.1986.03380020078023.
16. Kass EJ, Bolong D. Single stage hypospadias recon- 27. Patel RI, Hannallah RS. Anesthetic complica-
struction without fistula. J Urol. 1990; https://doi. tions following pediatric ambulatory surgery:
org/10.1016/s0022-5347(17)39510-1. a 3-yr study. Anesthesiology. 1988; https://doi.
17. Retik AB, Keating M, Mandell J. Complications org/10.1097/00000542-198812000-00044.
of hypospadias repair. Urol Clin North Am. 28. Schonfeld WA, Beebe GW. Normal growth and
1988;15:223–36. variation in the male genitalia from birth to
18. Hadidi AT. Perineal hypospadias: back to the future maturity. J Urol. 1942; https://doi.org/10.1016/
chordee excision & distal urethroplasty. J Pediatr Urol. s0022-5347(17)70767-7.
2018; https://doi.org/10.1016/j.jpurol.2018.08.014. 29. Schultz JR, Klykylo WM, Wacksman J. Timing of elec-
19. Manley CB, Epstein ES. Early hypospadias tive hypospadias repair in children. Pediatrics. 1983;
repair. J Urol. 1981; https://doi.org/10.1016/ https://doi.org/10.1016/s0022-5347(17)51507-4.
S0022-5347(17)55167-8. 30. Duckett JW, Baskin LS. Hypospadias. In: Gillenwater
20. Belman AB, Kass EJ. Hypospadias repair in chil- JY, Grayhack JT, Howards SS, Duckett JW, editors.
dren less than 1 year old. J Urol. 1982; https://doi. Adult and pediatric urology. 3rd ed. St. Louis: Mosby-
org/10.1016/S0022-5347(17)53458-8. Year Book; 1996. p. 2549–89.
21. Culp OS. Experiences with 200 hypospadiacs; evolu- 31. Hadidi AT. Editorial comment. Hypospadias Surgery,
tion of a therapeutic plan. Surg Clin North Am. 1959; an Illus Guide. 2004
https://doi.org/10.1016/S0039-6109(16)35840-6. 32. Feldman KW, Smith DW. Fetal phallic growth
22. Hodgson NB. A one-stage hypospadias and penile standards for newborn male infants.
repair. J Urol. 1970; https://doi.org/10.1016/ J Pediatr. 1975; https://doi.org/10.1016/
S0022-5347(17)61718-X. S0022-3476(75)80969-3.
23. Sipowicz RR. Psychological Responses of Children 33. Tuladhar R, Davis PG, Batch J, Doyle
to Hospitalization. Am J Dis Child. 1965;109:228. LW. Establishment of a normal range of penile length
24. Tiret L, Nivoche Y, Hatton F, Desmonts JM, Vourc’h in preterm infants. J Paediatr Child Health. 1998;
G. Complications related to anaesthesia in infants https://doi.org/10.1046/j.1440-1754.1998.00278.x.
General Principles
15
Ahmed T. Hadidi
Abbreviations scanner in advanced pregnancy. Other genital

anomalies can be detected, such as epispadias,
BP Buried penis ambiguous genitalia, testicular feminization syn-
CEDU Chordee excision and distal drome, and Smith-Lemli-Opitz syndrome [1].
urethroplasty Prenatal detection of anomalies can be helpful
DR Decision regret in the evaluation of fetuses with severe multisys-
DSD Disorders of sex development tem anomalies, as well as lesions more amenable
DYG Double Y glanuloplasty to correction in the neonatal period. Detection is
hCG Human chorionic gonadotropin particularly important in neonates with endocrine
LABO Lateral based onlay flap disorders and in those with complex genitouri-
MCGU Meatus-chordee-glans width and nary and anorectal malformations [2].
shape-urethral plate quality
SLAM Slit-like adjusted mathieu
15.2 The First Consultation
Early diagnosis is recommended. This is usually

15.1 Introduction possible at the maternity unit soon after birth
because of incomplete abnormal foreskin, and
The estimated incidence of hypospadias is 1 in the boys are then referred to a pediatric surgeon
every 120 male children. Incomplete formation or pediatric urologist for early consultation dur-
of the prepuce and excess dorsal hood lead to rec- ing the first 3 months of life.
ognition of the defect soon after birth in about The first consultation is a very important inte-
98% of cases. Prenatal ultrasound may detect a gral part of the hypospadias management and
genital abnormality; a wide distal end of the should be done by a surgeon experienced in
penis corresponds to the excess dorsal prepuce. hypospadias (not a junior inexperienced trainee)
Sonography can be performed with a vaginal because it develops trust between the parents and
probe in early gestation and an abdominal sector the surgeon, it helps to detect the severity of
hypospadias and chordee and associated anoma-
lies, and it draws the road map of treatment pro-
A. T. Hadidi (*) tocol for the child. The parents are usually
Hypospadias Center and Pediatric Surgery anxious, have never heard of the word hypospa-
Teaching Hospital of the Johann Wolfgang Goethe dias before, and have only read horror stories in
University, Offenbach, Frankfurt, Germany the Internet of patients being operated on for up
310 A. T. Hadidi
to 40 times and suffering their whole life because

of hypospadias and its complications. Parents in
general need professional detailed information
by an experienced surgeon who can answer all
their questions satisfactorily. The surgeon should
also detect any anomalies requiring early inter-
vention. For example, meatal stenosis may
require meatotomy at an early age, and severe
chordee should be corrected early to reduce the
chances of corporeal disproportion.
15.3 History
Detailed family history should be obtained.

Previous members of the family with hypospadias
should be recorded. Fourteen percent of male sib-
lings of an index child have hypospadias. If two
members of the family have hypospadias, then the Fig. 15.1 Hypospadias is classified into four grades
risk is 21% in subsequent male children. Maternal according to the location of the meatus and the severity of
the chordee and the Frankfurt Protocol of management of
ingestion of hormone medications during preg-
different grades of hypospadias (©Ahmed T. Hadidi 2022.
nancy must be ascertained. Other anomalies affect- All Rights Reserved)
ing the heart, lungs, brain, kidney, and anus or
syndromes are not uncommon and have important
bearing on anesthesia and the timing of surgery. undescended, a complete genetic and endocrine
examination is recommended.
After history taking and examination, the sur-
15.4 Examination geon should write his notes in the patient file and
fill in the MCGU form (Fig. 15.2). The surgeon
General examination to exclude abnormalities of must be prepared to answer all the parents’ ques-
the ear, anus, sacral sinus, hernia, possible syn- tions. This usually includes:
dromes, and undescended testis is needed. What is hypospadias? What grade of hypospa-
Local examination of the penis should be per- dias does my son have? Is my son’s penis big
formed with special attention to the penile size, enough? Does he need hormone therapy? Is the
the foreskin, rotation, glans clefting, penoscrotal penis curved? How severe? Does my son need sur-
transposition, bifid scrotum, and chordee (penile gery? What is the best time for surgery? Does vac-
curvature) and should exclude buried penis (BP). cination influence timing of operation? What
The penis is lifted to identify the location of the surgical technique are you planning to use? How
meatus (Fig. 15.1) and its relation to the under- long does the operation take? How many opera-
surface of the penis. This is followed by palpa- tions does my son need? What is the success rate?
tion of the penis to feel if there is hypoplasia or What are the possible complications? Can you
hard bands pulling the penis down. This is fol- correct them? When would you correct complica-
lowed by stretching the penis and seeing if there tions if they occur? How long is the hospital stay?
is any tethering of the penile skin or glans titling. How many catheters will my son have? How long
The testes should be palpated in the scrotum will my son have a catheter? Can he sleep on his
or could be brought down to the scrotum. This abdomen? How long will he have dressing? How
should be documented as some patients may often should I change pampers? Also when he is
experience “ascending testis syndrome” after asleep? When and how can I clean my son? When
hypospadias surgery. If one testis (or both) is can he take a shower and bath? Is he going to be
15 General Principles 311
a
Hypospadias International Score (MCGU)
Family History Hormone Therapy Date of Examination:

Positive Negative Positive Negative Score
1. Meatus
I, Glanular II, Distal Penile III, Proximal IV, Perineal

Hypospadias Hypospadias Hypospadias Hypospadias 1-4
1 2 3 4
2. Chordee
Severe, deep chordee 0-2

or < 15° 15-30° or > 30°
0 1 2
3. Glans Width & Shape
good groove glans poor groove glans flat glans 0-2

≥ 14 mm 12- <14 mm < 12 mm
0 1 2
4. Urethral
Plate quality
intact divided 0-2
0 2
Glanular Hypospadias with good glans = Score 1

Perineal Hypospadias with poor glans = Score 10 Total score
1-10
©Ahmed T. Hadidi 2022. All Rights Reserved
Fig. 15.2 (a) Hypospadias International Score (MCGU) description of possible associated anomalies (©Ahmed
(©Ahmed T. Hadidi 2022. All Rights Reserved. (b) T. Hadidi 2022. All Rights Reserved)
Hypospadias International Score (MCGU) together with
312 A. T. Hadidi
b
1. Family History Hormone Therapy

Date of Examination:
Positive Negative Positive Negative
2. Meatus location Glanular Distal Penile Proximal

Hypospadias
Perineal
Hypospadias
Hypospadias Hypospadias
before chordee
correction 1 2 3 4
3. Meatus location after Glanular Distal Penile Proximal Perineal

Hypospadias Hypospadias Hypospadias Hypospadias
chordee correction
1 2 3 4
0°-15° 15°-30° >30°

4. Chordee No chordee Deep chordee
0 1 2
deep groove poor groove

5. Glans width ≥ 14mm 12 - <14mm < 12 mm
0 1 2
6. Urethral plate quality

divided
MCGU Score = ................
2
7. Prepuce Complete Incomplete
8. Penile torsion No torsion Left Right
No Incomplete Complete
9. Scrotal transposition transposition transposition transposition
10. Undescended Testis Normal Unilateral Bilateral
3-5 cm 2- 3 cm <2 cm
11. ASD Ano Scrotal Distance
12. Penile length >3 cm 2-3 cm <2 cm
© Ahmed Hadidi . All Rights Reserved
fixed in bed? Can I take my son for a walk? Does have children when he grows up? Why does my
the operation affect the sensation and function of son have hypospadias? Did I do something wrong
the penis? Will my son’s penis look normal after during pregnancy? When I get pregnant again,
surgery? Can he have a normal sexual life and what are the chances that I may have another son
with hypospadias? What happens if I do not c orrect growth of the penis.

my son’s hypospadias? I want to wait until my son Gonadotropin or testosterone may be used.
reaches puberty and then he can decide for him- Preoperative treatment with gonadotropins has
self, is that okay? Have you seen similar hypospa- not been well supported because of lack of treat-
dias cases before? How many hypospadias ment protocols. Further problems are the wide
operations do you perform per year? How many variation in response to treatment with human
similar hypospadias conditions do you operate per chorionic gonadotropin (HCG), the failure to
year? Can my son keep the foreskin? Can you per- assess androgen receptors or 5-a reductase status,
form foreskin reconstruction? Do I need to do any and the lack of measurement of testosterone lev-
investigations for my son before surgery? How els before and after treatment. However, treat-
soon after surgery can my son resume normal ment with HCG showed decrease in the degree of
activity, like swimming, cycling, and sport? the severity of proximal hypospadias and reduced
The author usually shows Fig. 15.1 to the par- the severity of chordee (see Chap. 13: Preoperative
ents where they can see clearly the different oper- Hormone Therapy) [4, 5].
ative techniques for different grades, how long Defects in androgen receptors and 5-a reduc-
the operation takes, catheter duration, and the tase function and testicular steroidogenesis can
incidence of complications. result in hypospadias. However, gonadotropin
stimulation of testes in patients with defective
testicular steroidogenesis and partial receptor
15.5 Hypoplasia of the Penis abnormality may not be able to increase serum
testosterone level enough to overcome the physi-
The normal penile stretch length in the newborn ological defect, and gonadotropin alone cannot
is 3.9 cm, range 3.1–4.7 cm (see Table 4.1 in enhance penile size, as in cases of hypergonado-
Chap. 4). The response to testosterone stimula- tropic hypogonadism.
tion should be tested. Testosterone has been shown to be more ben-
Reports of long-term testosterone treatment of eficial than HCG in stimulating the growth of the
congenital hypoplasia of the penis are scarce. hypospadiac micropenis; the testosterone may be
Among 66 children with penile hypoplasia of applied locally as testosterone propionate cream
whom 35 had associated hypospadias, the effect of 2% to the penis three times daily for 3 weeks,
IM testosterone injection was evaluated in 40 chil- preferably by the father or by the mother wearing
dren treated under the age of 10 years. The increase gloves to avoid absorption of testosterone. The
in penis length was greater in children with isolated advantages of local application of testosterone
micropenis than when associated with hypospadias propionate cream are good response of increased
and greater when treatment started in the neonatal size of penis, easy application, and lack of pain.
period. The penile length at the last follow-up was However, there has been great variability of
related not to the total dose of testosterone but to response depending on the degree of absorption
the length at the time of first evaluation [3]. and the amount of cream applied.
The advantage of intramuscular application is
that it ensures an increase in serum testosterone
15.6 Preoperative Hormonal level. Koff and Jayanthi conducted a study of 12
Stimulation boys with proximal hypospadias treated preop-
eratively with HCG for 5 weeks immediately pre-
The repair of hypospadias in a child with micro- ceding the repair and concluded that pretreatment
phallus is technically difficult. To enhance the with HCG resulted in disproportional penile
size of the penis and to achieve satisfactory enlargement which advances the meatus distally
repair, preoperative androgen therapy has been to decrease the severity of hypospadias and chor-
suggested before reconstruction. This allows dee [4]. The penile shaft proximal to the urethral
early surgical correction, thereby avoiding the meatus shows greater growth than the proximal
psychological problems that may occur in cases shaft and causes the meatus to move distally
where surgery is delayed to allow sufficient away from the penoscrotal junction.
314 A. T. Hadidi
The most reliable method of administration is [8]. Patient regret and parenteral regret are two
parenteral injections; however, topical testoster- potential types of decision regret following hypo-
one cream may be applied directly to the penis spadias surgery. Either could be immediate or
[6]. Davits et al. found that a dose of 2 mg/kg delayed in relation to the decision to have or
given 5 weeks and again 2 weeks preoperatively decline a repair. The decision-making process in
was effective [7]. Belman used 25 mg testoster- parents/carers of a child with hypospadias is
one enanthate 6 and 3 weeks before repair. Koff shown to sequence through four stages: (1) pro-
and Jayanthi used HCG stimulation 6–8 weeks cessing the diagnosis, (2) synthesizing informa-
prior to repair in 12 boys aged 6–12 months; after tion, (3) processing emotions and concerns, and
a 5-week course, there was significant reduction (4) finalizing the decision. The key factors that
in chordee and an increase in penile length. This may either aid or hinder that process are the
topic is discussed in more detail in Chap. 13: availability and reliability of the relevant infor-
Preoperative Hormone Therapies. mation and the presence of knowledge gaps on
the specifics of the anatomy and the abnormality,
concerns regarding general anesthesia, and the
15.7 Informed Written Consent relationship with the healthcare provider [9].
Decision-making aids have been shown to
Consent, i.e., the agreement of a person (the improve consumer’s knowledge and accurate risk
patient or legal guardian) to go ahead with an perception and assist in making “value-congruent
examination, investigation, or treatment, is valid choices” [10]. There is currently one parent-
only if the appropriate enough information is centered decision aid prototype being developed
given. It must be voluntary, without pressure and and tested for the parents of children with hypo-
sufficient time to decide before the surgical pro- spadias [11, 12].
cedure. It is usually given by parents. Ideally, it Most adult men are content with the results of
should be done by the surgeon performing the the hypospadias repair during childhood with
operation. The doctor has a duty to inform the some lower satisfaction with the cosmetic appear-
parents of the possible risks and benefits of the ance and the length of the penis [13, 14]. Some
proposed procedure in order to obtain valid con- studies report high incidence of parenteral
sent. Parents should be aware that the best proce- decision regret following hypospadias
dure may be better judged when the child is under surgery [15–18]. However, these studies are not
anesthesia, and the surgeon may need to explain necessarily representative due to low response
the possible alternatives in advance. rate and potential misclassification (usually, par-
A brief but clear outline of the outcome, cos- ents who experienced complications or unsatis-
metic results and possible complications, particu- factory results are keen to respond to such
larly in relation to fistula formation, stricture, or surveys). Further objective large population stud-
dehiscence, must be given. Also the parents ies are needed.
should be aware that their child may require a
multistage procedure, a “re-do” operation, or a
minor corrective procedure to trim the foreskin or 15.9 Penoscrotal Transposition
dilate the meatus. There may be a need to repeat
the explanation on admission, after previous dis- Penoscrotal transposition is an infrequent con-
cussion during an outpatient consultation. genital malformation due to a defect in the cau-
dal migration of the scrotum during fetal life.
The anomaly has been classified into a bifid
15.8 Decision Regret scrotum, incomplete or partial penoscrotal trans-
position, complete penoscrotal transposition or
Decision regret (DR) is defined as the psycho- pre-penile scrotum, and lastly ectopic scrotum
logical distress surrounding the diagnosis, opera- (see Chap. 6: Morphology of Hypospadias and
tion, screening procedures, or other intervention Chap. 49).
15.10 The Role of Imaging 15.11 Associated Anomalies
To assess the role of routine radiographic screen- Undescended testicle and hernia are the most
ing of boys with hypospadias, in 1990 Moore common associated anomalies. Urinary tract
conducted a prospective study of 153 boys with anomalies are infrequent as the external genitalia
hypospadias [19]. They were screened by intra- are formed much later. The penile skin may be
venous urography and voiding cystourography; very thin, and this prevents the use of perimeatal
urinary tract anomalies were found in 23.5% of skin flaps for urethral plate repair. The penile skin
cases and significant anomalies in 11% on the may be well developed, elastic, and non-tethering;
initial examination and a further 4.5% on follow- however, it may equally be underdeveloped and
up requiring later surgery. The overall incidence act as a tethering fibrous band that bends the penis
of surgical intervention, not including hypospa- during micturition or erection. Desautel et al. in
dias repair, was 11.7%, and Moore recommended 1999 studied 26 patients ranging from 1 month to
routine screening in boys with hypospadias. 19 years of age with Müllerian duct remnant;
However, similar studies show a low incidence of between 1984 and 1998 ten of these had associ-
anomalies, most not requiring intervention. In ated penoscrotal hypospadias and were treated
view of this, ultrasound has been suggested as a conservatively [24]. However, five patients devel-
screening procedure for boys with hypospadias oped recurrent urinary tract infections and were
and should be considered in severe types of hypo- maintained on long-term prophylactic antibiotics.
spadias as there is a low incidence of upper uri- Utriculus masculinus may be mild, but in severe
nary tract anomalies in mild hypospadias. cases it causes recurrent urinary tract infection
Vesicoureteric reflux was found in 17% of 305 and difficulty in passing a urethral catheter.
boys, in the majority low grade and not associ-
ated with renal damage.
It is generally agreed that upper renal and ure- 15.12 Ambiguous Genitalia
teric anomalies with hypospadias are extremely and Disorders of Sex
rare. Only seven significant renal anomalies were Development (DSD)
found in 360 patients, in 3 of whom renal investi-
gation would have been performed anyway Patients with severe hypospadias, proximal or
because of anorectal malformation [20–22]. perineal (grade IV), with impalpable gonads
Micturating cystourethrography has been sug- should be investigated for disorders of sex devel-
gested only if there is a history of recurrent opment or DSD, as should girls with severe mas-
urinary tract infection and in cases of true ambig- culinization with adrenogenital syndrome. The
uous genitalia to assess the presence of Müllerian presence of both testicles in the scrotal sac indi-
duct remnants. Such remnants are usually asymp- cates male gender. Chromosome abnormalities
tomatic and do not require routine surgical resec- may be suspected in hypospadias with a palpable
tion. Cystoscopy does not add much to our single testicle, particularly if this is associated
knowledge of the child with hypospadias, except with dysmorphic features. Buccal smear and
in severe forms, where it may show utricular karyotyping should be performed, and the internal
enlargement or posterior urethral valves. genitourinary structures should be assessed by
Enlargement of utricle may be noted in 57% of micturating cystourethrography, genitography,
boys with perineal hypospadias, the incidence cystoscopy, and examination under anesthesia.
and severity correlated directly with the degree of
hypospadias [23]. Occasionally, resection of the
utricle may be necessary because of recurrent uri- 15.13 Is Hypospadias Part of DSD?
nary tract infection, urinary dribbling, or stone
formation. A uterus and tubes may be present, During the Chicago Consensus Meeting in 2005,
suggesting a greater degree of disorders of sex disorders of sex development (DSD) were defined
development (DSD). as congenital conditions within which the
316 A. T. Hadidi
development of chromosomal, gonadal, and ana- Only patients with proximal or perineal hypo-
tomic sex is atypical. spadias associated with one or both impalpable
Hypospadias is defined as a congenital anom- gonads (less than 5% of hypospadias) could be
aly where the urethral opening lies under the sur- included in the category of disorders of sex devel-
face of the penis. It may be associated with many opment (DSD).
anomalies of the prepuce, ventral penile fascia Those patients should undergo diagnostic
(chordee), and many others. evaluation that may include history, physical
The incidence of hypospadias in general is examination, serum electrolytes, plasma
1 in 120 live male births or 1 in 250 live births, 17-hydroxyprogesterone, chromosome analysis,
and the incidence of DSD without hypospadias is lymphocyte culture, ultrasound of the abdomen
1 in 5000 live births. Perineal hypospadias and and pelvis, genitography, and, whenever neces-
proximal hypospadias with severe chordee is sary, measurement of plasma concentration of
about 10% of hypospadias, and only half of those testosterone as baseline and after stimulation
are having undescended testis or chromosomal with HCG. Laparotomy may be necessary for
anomalies. In other words, hypospadias is 20 definitive histological examination of the gonads.
times more than DSD without hypospadias In all children with male pseudohermaphrodit-
(Fig. 15.3). Most patients (>95%) with ism or incomplete masculinization consisting of
hypospadias are normal males and can lead a hypospadias, mostly of the perineal type, with
completely normal life. Therefore, it seems com- micropenis and/or bilateral or unilateral cryptor-
pletely unfair to tag 95% with the title DSD just chidism, the male sex is assigned. The most
for less than 5% of patients. In addition it would common cause of ambiguous genitalia is con-
mean unjustified exhaustion of resources for genital adrenal hyperplasia due to 21-hydroxy
investigation and unjustified excessive anxiety deficiency in 46XX cases presenting with
for parents when their children are completely ambiguous genitalia; those with 46XY have a
normal males. wider range of diagnoses (androgen sensitivity
syndrome, gonadal dysgenesis). Despite thor-
ough investigation, 23.5% of children studied
had no definite final diagnosis of genital
ambiguity.
The combined presence of cryptorchidism and
hypospadias often indicates the existence of
DSD. Testicular maldescent and incomplete tubu-
larization of the urethral plate occur in a spec-
trum, with the severity of the two processes
probably dependent on the degree of pathophysi-
ology in the androgenic hormonal access. The
incidence of intersexuality of children with unde-
scended testicle, hypospadias, and otherwise
unambiguous genitalia has been reported to be
27%. A total of 2105 cases of undescended testi-
cles and 1057 cases of hypospadias were studied
between 1982 and 1986. Seventy-nine patients
presented with both undescended testicle and
hypospadias, and a phallus was believed to be a
Fig. 15.3 The incidence of hypospadias is 20 times the possible penis; intersex conditions were identified
incidence of DSD without hypospadias. Only 5% of
patients with hypospadias may have chromosomal anom-
with nearly equal frequency in 44 cases of unilat-
alies and may be under the DSD category (©Ahmed eral and 35 cases of bilateral cryptorchidism. In
T. Hadidi, 2022. All Rights Reserved) the unilateral undescended testicle group, patients
with an impalpable testis were at least three times 15.14 S

hould Hypospadias Surgery
more likely to have intersex [25]. Be Postponed Until the Child
A case-control study of 6177 boys with crypt- Reaches the Age of Consent?
orchidism, 1345 cases with hypospadias, and
23,273 male controls from 1983 to 1992 was car- In recent years, there have been active move-
ried out to determine the relationship between ments trying to prevent any operations on the
cryptorchidism and hypospadias and the pres- genital region including the penis, scrotum,
ence of other anomalies, abnormalities in an and testis until the child reaches the age of
older brother, birth weight, weeks of gestation, consent and she/he should decide for her/
maternal history of stillbirth, parity, twin birth, himself.
parental age, nationality, and professional status. This is quite understandable and absolutely
They found that simultaneous cryptorchidism correct for children/patients with chromosomal
and hypospadias were more common than abnormalities where the gender may not be clear
expected, that there was an increased risk of both or questionable.
entities when the same abnormality was present However, the situation is completely different
in an older brother, and that the risk of cryptor- when chromosomal and genetic examinations
chidism and hypospadias increased with decreas- confirm that the baby/child is a normal 46XY
ing birth weight independent of weeks of male but with undescended testis or hypospadias
gestation. Twins were at a lower risk than single- or both. To explain this, I would like to mention
tons of both entities in all lower birth weight the following points:
groups. An increased risk of hypospadias was
noted in the sons of women with a previous still- 1. There is an evidence-based medicine that
birth. The risk of cryptorchidism and hypospa- shows clearly that leaving the testis inside the
dias slightly increased with decreasing parity abdomen will damage the function of the tes-
[26]. Both cryptorchidism and hypospadias were tis, and the testis may become malignant, and
positively associated with other congenital mal- there will be late diagnosis if it becomes
formations and inversely with maternal parity. malignant.
There is evidence that being born small for age 2. Figure 15.4 shows a classic example of 46XY
and before 33 weeks of gestation have a greater normal male with normal testes, complete
than additive effect with respect to both cryptor- penoscrotal transposition, perineal hypospa-
chidism and hypospadias [27]. McAleer and dias, and severe chordee. Should we leave the
Kaplan set out to determine whether association severe chordee, the complete penoscrotal
of hypospadias and cryptorchidism is associated transposition, and the perineal hypospadias,
with a high incidence of chromosomal abnormal- or should the surgeon do his best to help the
ities and whether they warrant routine karyotype child to lead a normal life?
screening [28]. Complete anatomical, karyo-
typic, pathological, and radiological information Figure 15.5 shows a classic example of 46XY
was available for 48 patients: 8 had chromosomal normal male with bilateral abdominal testes,
abnormalities, 2 had karyotypic intersex disorder, complete penoscrotal transposition, perineal
and 6 had autosomal chromosomal abnormality. hypospadias, and severe chordee. Should we
The authors concluded that most patients who leave the testes in the abdomen, the severe chor-
present for evaluation of hypospadias, chordee, dee, and the perineal hypospadias, or should the
and undescended testicle have normal karyotype, surgeon do his best to help the child to lead a nor-
and routine karyotyping does not seem necessary. mal life?
If karyotypic intersex abnormalities are identi- Following is the story of a young man with
fied, these patients are more likely to have ambig- distal hypospadias where the parents decided not
uous genitalia, especially those with perineal to correct the hypospadias of their son until he
hypospadias and cryptorchidism. decides for himself at the age of 20 years.
318 A. T. Hadidi
a b c
d e
Fig. 15.4 A normal 46 XY male baby with severe peri- healing of the first stage. (d) 1 month after urethroplasty.
neal hypospadias, severe chordee, and complete peno- (e) 1 year after urethroplasty (©Ahmed T. Hadidi 2022.
scrotal transposition. (a, b) Preoperative photos. (c) After All Rights Reserved).
a b c
Fig. 15.5 A normal 46 XY male baby with bilateral Preoperative photo. (b) After healing of the first stage. (c)
abdominal testes, severe perineal hypospadias, severe At the end of urethroplasty (©Ahmed T. Hadidi 2022. All
chordee, and complete penoscrotal transposition. (a) Rights Reserved)
15.15 T
he Story of My Life
with Hypospadias As every normal male kid, I started
being interested in my penis very early, and
15.15.1 An Adult Patient Perspective sometimes I was wondering about how
(let’s say, like nearly all man do) he looks
like, but the day I really recognized that my
To start from the absolute beginning, I was phallus is abnormal was at the school
born in the 1970s with a penile hypospa- examination when I was 9 years old.
dias also named as second degree or distal
hypospadias (the site of urethral meatus
was about 1 cm behind the glans).
We were three boys in the medical room
and she (!) only wrote down a note once,
At this time, it wasn’t common to oper- and this was after having a look at my
ate such classifications of hypospadias. I penis. From this point of time, I found out
just had a small operation to widen the that there’s something with him which is
opening, as it’s usually done (said the doc- not like it should be. I tried to ask the doc-
tors). My parents told me that they went to tor what she wrote down, but of course she
two urologists; one said there would be two didn’t tell me (this was a sad day, also
operations necessary to correct this aberra- because from this day on really every time
tion, and the penis looks like its function is I had a pee I thought about this anomaly
guaranteed so it’s okay to leave this mild and not only at these moments!).
form (in his view!) how it is. The other one
gave my parents the advice that no opera-
tion is needed because the hard-on won’t
show a strong bending down; he also had a
Years passed and playing doctor with
look inside my urethra to look if everything
other boys was inevitable and trust me, I
makes practical things possible; he did this
never will forget those two surprised faces
in front of my parents; well of course I was
of friends (boys) when they saw my penis;
crying out loud (he didn’t use narcotics); I
I felt such ashamed I can’t tell—I still think
was a baby and when my parents saw that
about this situation every day and ask
(especially my mum), the chance of getting
myself very often what did they think in
a normal penis sank to 0%.
this situation, whom did they tell, cause I
grew up with these two guys, and there
were lots of other boys and especially girls
Oh sh..t !! in the neighborhood (children can be very
nasty).
Despite the facts that a single operation

wouldn’t have been a problem for an experi-
enced surgeon and my penis had some kind Now I should be old enough to leave
of practical problems, both urologists didn’t this behind, but when I see one of them I
think a second about the PSYCHOLOGICAL can’t help thinking about this topic because
PROBLEMS which also can lead to FATAL it wasn’t the only time they saw my abnor-
MENTAL DESEASES (especially for a mal mate between my legs (this was a very
man with the third degree of hypospadias) hard experience).
such an anomaly can cause.
320 A. T. Hadidi
Again time passed and by and by the First of all, all the girlfriends I had did a
lookalike of everyone got and still gets great job in giving me self-confidence in
more and more important in our “new” this topic, and they also mentioned all the
society, one point is that we all get more time that this isn’t any problem (I still don’t
and more influenced by lots of different know if they said the truth, because if a fat
kinds of media, and this brings me to the girl asks me if she is fat I would answer
boom of pornography. For every teenager (like every adult would): “no,” to be polite.
like me, it was easy to get and played an Second, as you can imagine from the lines
important role during our puberty. Well I above, THE MAIN PSYCHOLOGICAL
knew that the men in porn movies don’t PROBLEM IS WHAT’S GOING ON IN
represent normal men’s genitals—I was THE MIND OF A MAN WITH
sure that there are a lot of crooked, ugly, or HYPOSPADIAS!!
whatever c..ks, but to see others peeing and
ejaculating from the top of the glans all the
time…. When you got hypospadias and
you see such things, you get some kind of One more example: When I was 17, I had a
depressed, because you start thinking about very pretty girlfriend (she was awesome).
things like: How will the first time be when One day when we were kissing she started
I show my penis to a girl, is there really a to open my jeans, moved her head down-
sense in using a condom, or do other man wards, and said with a very soft and erotic
recognize that I pee from a different part of voice: “I would like to do you a favor with
my d..k at public toilets where you stand my mouth”…at the age of seventeen—get-
side by side and every man does com- ting the feeling of an oral-favor from a
pare—especially at this age? pretty girl one year younger is one of the
greatest moments in a young man’s life
(I’m sure many men will agree)…but I felt
such ashamed because of my penis, that it
Experience with girls: didn’t come to this; I told her that we
should take time for that (what a bl…y lie
;)), and I never had sex with her!
In my case (by the way I’m straight), low to

middle hypospadias second degree, you The penis is by far the most (and nearly the
really can see that the urethra wanted to only) important “part” of a man’s body to
build nearly to the top of the glans, but this show his manliness, so hypospadias can
couldn’t be finished, and this makes my have a big influence on a man’s self-
phallus look horrible (in my hard-self- assurance and is also a very, very personal
skeptical view). Women will see more thing. I never had the chance to talk about
penises in their lives, and when they know this big topic with anyone till 3 months
how a normal one should look like, what do before my first operation. I was too inhib-
they think about mine? ited to talk about this and my parents never
started a conversation, I don’t blame them
for this because other men (e.g., my father)
without this anomaly can’t imagine how it
is to live with it.
Another reason to do hypospadias surgery Now I can say that the decision to have my
is that under certain circumstances, you (I) hypospadias corrected or I prefer to say
feel a little pain at the urethral meatus dur- “erased” was one of the best in my life; my
ing having sex—it’s kind of an open penis looks great (well, he got a scar, but
wound, and too hard friction can lead to an this gives him character ;)), and this has
uncomfortable feeling also with the fact changed now a lot; for example, my self-
that a too short urethra pulls the penis kind confidence raised and life seems to get
of down. sweeter and sweeter ;).
After the operation and an advice: So my personal, subjective advice for par-
ents and surgeons is An operation is neces-
sary to guarantee the BOY a happy,
“NORMAL,” life, and in my view it is not
Well, after many years of living with hypo- a good idea to say “let the boy decide when
spadias, I decided to have a surgery (even he is old enough.” Because then it could be
didn’t know till 3 months before the OP FAR TOO LATE!
that this aberration can be “repaired” by
surgery!); the thought that I can have some
kind of a normal penis made me forget that
there can appear some complications (also References
mental—especially for a grown up).
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sonographic signs of possible fetal genital anoma-
lies. Prenat Diagn. 1995; https://doi.org/10.1002/
pd.1970150303.
So I had a surgery by a very experienced 2. Mandell J, Bromley B, Peters CA, Benacerraf
BR. Prenatal sonographic detection of genital mal-
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made my friend between my legs look P. Hypoplasie de la verge: diagnostic étiologique et
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I also had to take special medication for 4. Koff SA, Jayanthi VR. Preoperative treatment
about 6 weeks after the surgery to avoid with human chorionic gonadotropin in infancy
getting an erection, so the first view erec- decreases the severity of proximal hypospadias
and chordee. J Urol. 1999; https://doi.org/10.1016/
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because of the bad healing, and the erec- 5. Husmann DA. Editorial: microphallic hypospadias -
tile tissues had to get their normal mainly the use of human chorionic gonadotropin and testos-
straight function back again (strong bend- terone before surgical repair. J Urol. 1999; https://doi.
org/10.1016/S0022-5347(05)68334-6.
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the first three months after the surgery Imanaka K. Use of testosterone ointment before
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Keizer-Schrama SMPFDM, Nijman RJM. Effect of decisional satisfaction after hypospadias repair in the
parenteral testosterone therapy on penile development United Kingdom. J Pediatr Urol. 2020; https://doi.
in boys with hypospadias. Br J Urol. 1993; https://doi. org/10.1016/j.jpurol.2020.01.005.
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8. Rehfuss A, Kogan B. Psychological health and AV. Parental decision regret in childhood hypospadias
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juro.2017.07.046. 19. Moore CCM. The role of routine radiographic
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Cain MP, Frankel R. Parental perspectives on decision- tive study. J Pediatr Surg. 1990; https://doi.
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2019; https://doi.org/10.1016/j.jpurol.2019.04.017. 20. Kelly D, Harte FB, Roe P. Urinary tract anomalies in
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C, Cockrum B, Wiehe S. User testing of a hypospadias j.1464-410X.1988.tb04434.x.
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jpurol.2016.11.010. NE. Risk factors for cryptorchidism and hypo-
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Bägli DJ, Braga LHP. Decisional regret after dis- S0022-5347(05)68992-6.
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juro.2015.10.118.
Plastic Surgery Principles
16
Ahmed T. Hadidi
Abbreviations viability, instruments and magnification, suture

material and knots, suturing techniques, diver-
BMG Buccal mucosal graft sion and dressings, are discussed in detail in
SLAM Slit-like adjusted Mathieu Chap. 17.
16.2 Skin
16.1 Introduction
Skin is the largest organ in the body. It comprises
Plastic surgery, although thought of as a 15% of the body weight and is about 21 ft2. The
technique-oriented specialty, is in fact a problem- skin varies in thickness in different parts of the
solving field. The plastic surgery training allows body and ranges from 0.5 to 6.3 mm. The whole
the surgeon to see surgical problems in a different skin is renewed every 28 days and 30,000 dead
light and select from a variety of options to solve cells are shed every minute. Skin has its own
surgical problems. In order to achieve optimum “microbiome” with about 1000 different bacteria
results in hypospadias surgery, one must have full types and 1 × 1023 bacteria. Skin has many essen-
command of the basic principles of plastic sur- tial functions: it protects the body from ultravio-
gery and the potential flaps for use in the recon- let rays, microbes, and padding. It has important
struction of the neourethra. sensory functions for pain, temperature, and
The basic principles of plastic surgery are pressure; it stores fat, glucose, and vitamins; it
careful analysis of the surgical problem, precise controls water balance through evaporation and
planning of procedures, precise technique, and vasodilatation; it excretes waste and salt and pro-
atraumatic handling of tissues. This chapter dis- duces vitamin D.
cusses the skin as an organ and function, princi- The epidermis is made up of 5 cell layers and
ples of wound healing, the difference between varies in thickness from 0.5 to 1.5 mm. The epi-
hypertrophic scar and keloid, specific issues of dermis contains no blood vessels; the dermis, act-
skin grafts and flaps, and skin substitutes. Other ing as the nutrient base of the epidermis, is thus
relevant factors, including tissue handling and indispensable. Keratinocytes make up 95% of the
epidermis cells. Other cells include melanocytes
A. T. Hadidi (*) and Langerhans cells (Fig. 16.1).
Hypospadias Center and Pediatric Surgery The dermis contains the skin adnexa (hair fol-
Department, Sana Klinikum Offenbach, Academic licles, sebaceous glands, sweat glands, and apo-
University, Offenbach, Frankfurt, Germany crine glands). It contains the blood vessels, lymph
324 A. T. Hadidi
Fig. 16.1 Section of the skin to illustrate the comparative tion of the full-thickness graft. Note that the junction
thickness of skin grafts. (A) Line of section of the thin between the dermis and the subcutaneous tissue is irregu-
split-thickness (Thiersch) graft. (B) Line of section of the lar. The protrusions of the subcutaneous fat into the der-
split-thickness graft. (C) Line of section of the thick split- mis are known as the columnae adiposae
thickness or three-quarter thickness graft. (D) Line of sec-
vessels, and nerves; thus, it supplies the epider- 16.3 Wound Healing
mis with nutrient saturated blood. The dermis
regulates the body temperature, protects the body Wound healing is a complex process that can be
from trauma, conserves water, and is responsible summarized into four phases: (1) hemostasis
for skin sensitivity (Fig. 16.2). (seconds to minutes), (2) inflammation
The hypodermis is formed of fat cells embed- (3–5 days), (3) proliferation (4–14 days), and (4)
ded in connective tissue and contains larger blood remodeling (day 8 to 1 year) (Fig. 16.4).
vessels, lymphatics, and nerves. The subcutane- The first step after injury or surgical wound is
ous tissue insulates the body, absorbs trauma, and hemostasis, the coagulation products of fibrin,
reserves energy sources (Fig. 16.3). thrombin, and compliment products (including
16 Plastic Surgery Principles 325
Fig. 16.2 Layers of the epidermis: The epidermis of repository. Retrieved 21:08, September 15, 2021 from
thick skin has five layers: stratum basale, stratum spino- https://commons.wikimedia.org/w/index.php?title=File:502_
sum, stratum granulosum, stratum lucidum, and stratum Layers_of_epidermis.jpg&oldid=548456486)
corneum (copyright: File:502 Layers of epidermis.jpg
(2021, March 31). Wikimedia Commons, the free media
growth factors) that attract inflammatory cells meet and fuse with counterparts migrating from
(leukocytes, macrophages, and fibroblasts) into the other side of the wound. Numerous growth
the wound. The growth factors ideally with Pa O2 factors and cytokines are observed to stimulate
tension 40 mmHg stimulate proliferation of angiogenesis.
fibroblasts. Fibroblasts secrete the collagen and Epithelialization: Epithelial cells respond to
proteoglycans of the connective tissue matrix that the same stimuli and growth factors as fibro-
hold wound edges together. blasts. During wound healing, mitosis appears
Angiogenesis is required for wound healing. It few cells away from the wound edge. The new
is clinically evident about 4 days after injury but cells migrate over the edge of the wound into the
in fact begins earlier when new capillaries sprout unhealed area and anchor to the first un-
from preexisting venules and grow toward the epithelialized matric position encountered.
injury in response to platelets and macrophages. Squamous epithelialization proceeds maximally
In surgical wounds, the budding vessels soon when the surface wound is kept moist.
326 A. T. Hadidi
Remodeling is also a well-regulated pro- Extracellular enzymes (some are Pa O2 depen-

cess. First, fibroblasts replace the provisional dent) quickly polymerize these monomers. The
fibrin matrix with collagen monomers. provisional matrix is gradually replaced with
more mature, better organized, stronger, and
more durable collagen fibers. Healing is suc-
cessful when a net excess of matrix is depos-
ited despite concomitant lysis. During rapid
turnover, wounds normally gain strength and
durability but are vulnerable to contraction or
stretching (important point when performing
foreskin reconstruction). Myofibroblasts
express intracellular actin filaments and dis-
tinct adhesion structures (desmosomes and
maculae adherens). It is important to realize
that contraction is proportional to the
amount of dermis missing.
One important point is that the wound
acquired 70% of its tensile strength in
6 weeks. This has important clinical application
in hypospadias surgery. Adults should not
resume sexual activity or penis manipulation
before 8 weeks after surgery (Fig. 16.5). Several
factors influence wound healing, including isch-
emia, oxygenation, nutritional status, and
infection.
Fig. 16.3 Layers of the dermis: This stained slide shows
the two components of the dermis: the papillary layer and
the reticular layer. Both are made of connective tissue From a practical point of view, the parents
with fibers of collagen extending from one to the other,
making the border between the two somewhat indistinct. must be informed to be careful for the first
The dermal papillae extending into the epidermis belong 6 weeks after the hypospadias operation
to the papillary layer, whereas the dense collagen fiber until the wound has reached 70% of its
bundles below belong to the reticular layer. LM × 10. strength.
(From Anatomy & Physiology, Connexions Website,
©2013, modification of work by “kilbad”/Wikimedia
Commons [1])
Fig. 16.4 Wound healing goes through four stages: (1) hemostasis, (2) inflammation, (3) proliferation, and (4) remod-
eling (©Ahmed T. Hadidi 2022. All Rights Reserved)
Fig. 16.5 Tensile

strength of a healing
skin incision as a
function of time
(reprinted from Surgical
Clinics N Am, Vol. 97,
by Childs, Dylan R. and
Murthy, Ananth S.:
“Overview of Wound
Healing and
Management,”
pp. 189–207, ©2017,
with permission from
Elsevier [2])
Hypospadias wounds are essentially contami- time, spread outside the wound boundaries, are
nated wounds because of the close vicinity to the pruritic and painful, and have high recurrence
anus, but the bacterial count should be low with rate. Silicone gel, corticosteroids, and massage
no replication. Colonization may occur if the are recognized effective measures to prevent and
microflore adhere to the skin and replicate form- improve hypertrophic scars. Substances like vita-
ing colonies. Critical colonization occurs when min E, zinc, or onion extract have unproven effi-
the colonization creates bioburden delaying ciency. There is strong evidence that intralesional
wound healing (signs: increase exudate, friable, injection of steroids or 5 FU may help in dealing
change in color malodor, or pain). Infection with hypertrophic scars.
occurs when there is actual invasion of tissue and
host reaction (edema, swelling, pain, erythema,
purulent drainage). Fortunately, wound infection 16.4 Grafts
is rather rare in hypospadias surgery when stan-
dard surgical rules are observed. A skin graft is skin that has been completely
detached from its original donor site and trans-
ferred to another site, where it will develop a new
16.3.1 Hypertrophic Scarring blood supply. The first recorded successful skin
and Keloid graft was by Sir Astley Cooper in 1817 [3]. Grafts
may be classified according to species or to
Hypospadias wounds usually heal with minimal thickness:
scarring provided general principles are observed.
Dorsal penile skin has more tendencies to hyper- • According to origin, grafts may be classified
trophic scarring than ventral penile skin. I have as autografts, isografts, homografts, or
never seen a keloid on the penis. xenografts:
Hypertrophic scars develop weeks or months –– An autograft is taken from one site and
after surgery, usually improve with time, remain placed on a different site in the same
within the wound boundaries, are pruritic but individual.
rarely painful, and have a low recurrence rate. –– An isograft is taken from one individual
Keloid scars, on the other hand, may develop and placed on another individual with the
many months after surgery, rarely improve with same genotype, e.g., an identical twin.
328 A. T. Hadidi
–– A homograft (or allograft) is taken from may be obtained with a knife or with a mechani-
one individual and grafted to another indi- cal dermatome. Some dermal appendages remain
vidual of the same species but of a different in the original donor bed, including sweat glands,
genotype. sebaceous glands, and hair follicles. From these
–– A xenograft (or heterograft) is grafted to an remaining skin appendages and from the wound
individual of a different species. edges, the donor site heals by epithelialization.
• Skin grafts may either be full thickness or split The thicker the graft, the longer the time required
(Fig. 16.1, Table 16.1): for complete donor site healing. Most split-
thickness graft donor areas heal in approximately
• A full-thickness skin graft consists of epi- 2–3 weeks. With thin grafts, the donor site may
dermis and the full thickness of the heal within 10–14 days and may be used for
dermis. grafting again if necessary.
• Split skin grafts consist of epidermis and a Thin split skin grafts more easily survive
variable thickness of the dermis. They are grafting, and the donor site heals faster than with
described as thin (10–12 thousands of an thick grafts. However, thick grafts contract less,
inch), intermediate (13–17 thousands of an are more like normal skin, resist trauma, and
inch), or thick (18–20 thousands of an inch). esthetically are more acceptable than thin grafts.
Thick graft donor sites heal more slowly with
However, these various categories of graft are more scarring than thin graft donor sites. The
not really completely distinct from one another. most common donor sites are the thigh, buttock,
The real difference in practice is that the full- and abdomen.
thickness skin graft is cut with a scalpel, while Meshed grafts are usually thin or intermediate
the split skin graft of whatever thickness is usu- split-thickness grafts that have been rolled under
ally cut with razor blades, skin grafting knives a special cutting machine to create a mesh pat-
(Blair, Ferris Smith, Humby, Goulian), manual tern. Although grafts with these perforations can
drum dermatomes (Padgett, Reese), and electric be expanded from one and a half to nine times
or air-powered dermatomes (Brown, Padgett, their original size, expansion to one and a half
Hall). The electric and air-powered dermatomes times the unmeshed size is the most useful.
are the most widely used because of their reli-
ability and ease of operation.
16.4.2 Full-Thickness Skin Grafts
16.4.1 Split Skin Grafts Full-thickness skin grafts include the epidermis
and the entire dermis. These grafts provide good
Split grafts include the complete epidermis with coverage because they are full-thickness skin. They
a variable thickness of dermis. The grafts may be resist trauma better, are esthetically more pleasing,
thin, medium, or thick. Donor skin for grafting and contract less than split-thickness skin grafts.
Table 16.1 Summarizes the character of different types of skin grafts

Type of graft Advantages Disadvantages
Thin split-thickness Survives transplantation most easily; donor Fewest qualities of normal skin; maximum
sites heal most rapidly contraction; least resistance to trauma;
sensation poor; esthetically poor
Thick split-thickness More qualities of normal skin; less Survives transplantation less well; donor
contraction; more resistant to trauma; site heals slowly
sensation fair; esthetically more acceptable
Full-thickness Nearly all qualities of normal skin; minimal Survives transplantation least well; donor
contraction; very resistant to trauma; site must be closed surgically; donor sites
sensation good; esthetically good are limited
Full-thickness grafts vascularize more slowly and phase of plasma imbibition. The graft initially
are less likely to survive than split-thickness skin gains weight from the diffusion, absorption,
grafts because of their greater thickness. and capillary action of fluid movement.
Donor sites for full-thickness grafts must be Revascularization of the graft takes place between
closed primarily or with split-thickness grafts. the third and fifth days (inosculation). The revas-
Donor sites are usually areas of thin skin such as cularization may occur by reanastomosis of ves-
the postauricular, supraclavicular, and inguinal sels between the recipient bed and the graft or by
areas. In addition to graft durability and donor the growth of new vessels into the graft. The cir-
site morbidity, color matching is very important, culation in the graft is usually restored by
especially on the face. 4–7 days. Lymphatic circulation usually parallels
the restoration of the blood supply.
In grafted skin, the epidermis initially under-
16.4.3 Cultured Epithelial and goes a period of depressed epidermal cellular
Dermal Grafts function until vascular ingrowth occurs. By
4–8 days, proliferation and thickening of the epi-
Epithelial cells were the first to be cultured thelial cell layers with gross desquamation
in vitro and made to coalesce into sheets that occurs. Fibroblasts migrate into the dermis by the
could be used to cover full-thickness wounds. third day and outnumber those of normal skin by
Although these culture epithelial sheets were 8 days. Most of the collagen in the graft is
used in the treatment of burns, the result was replaced; a split-thickness skin graft replaces less
unsatisfactory because the coverage was very collagen than a full-thickness skin graft.
fragile. More recently, success has been obtained Both split-thickness and full-thickness grafts
with artificial dermis which, when placed in an assume the sensory innervation pattern of the
appropriate bed, will revascularize and can then recipient site. Split-thickness grafts usually have
be covered by a very thin (0.05 cm) split-thickness less return of innervation than full-thickness
skin graft. This artificial dermis is increasingly grafts. Reinnervation follows the neurilemmal
being used in the treatment of burns. Modifications sheaths of the graft. Sensory recovery usually
of this concept have also been applied to the care takes 1–2 years; the sense of pain returns first,
of chronic ulcers, particularly in the leg. The arti- followed by light touch and temperature.
ficial dermis is made out of a collagen matrix and Skin grafts undergo both primary and second-
has very low or no antigenicity [4]. ary contraction. Primary contraction occurs
immediately on harvesting of the graft. Full-
thickness grafts contract by about 40%, split-
16.4.4 Skin Graft Survival thickness grafts by about 10–20% of their original
volumes. Secondary contraction occurs after the
The conditions necessary to ensure free graft sur- graft is vascularized and is related to the amount
vival include (1) a well-vascularized host bed, (2) of dermis in the graft. Full-thickness grafts
rapid onset of imbibition, (3) close apposition of undergo very little secondary contraction, whereas
the graft to the host bed, (4) immobilization of split-thickness grafts tend to contract more.
the graft, and (5) rapid onset of inosculation. Skin grafts may undergo variable pigmentation
Movement of the graft on its bed is likely to inter- changes. Thinner grafts usually exhibit greater
fere with graft survival; consequently, immobili- color changes. Ultraviolet light induces hyperpig-
zation is an important factor for success. mentation in immature grafts. Grafts in darker-
Initially, both split-thickness and full- haired individuals may progressively darken,
thickness grafts adhere to the recipient bed by while in lighter-haired subjects, they may lighten.
fibrin bonding. This usually lasts approximately Several dressings for the donor site have been
72 h. If fibrin is absent or dissolves, the graft described. These include scarlet red ointment,
fails. During the first 2 days, the graft is nour- petroleum jelly gauze, pigskin heterograft,
ished by fluid from the recipient bed. This is the Op-Site, and exposure techniques. Op-Site is
330 A. T. Hadidi
probably associated with less pain, whereas a • When the investing layer of deep fascia is
heterograft has increased infection and scarring. included, the flap becomes fasciocutaneous.
The most common cause of skin graft failure • A flap may consist of a muscle: a muscle flap.
is hematoma or seroma under the graft. This • If the skin overlying the muscle is included, or
inhibits adherence and vascularization of the the bone to which the muscle is attached, a
graft. Another cause of graft failure is inadequate composite flap results, myocutaneous or
immobilization of the graft. After the graft is osteomyocutaneous.
transferred to the recipient site, it should be com-
pletely in contact with the bed and adequately Before the development of microvascular
immobilized and covered with a pressure stent techniques, flaps had to retain a vascular attach-
dressing to decrease shearing forces. If the wound ment to the body throughout their transfer, but it
has a large amount of serous drainage, the graft is now possible to transfer a flap en bloc to a dis-
may be treated open. tant site in a single stage, as a free flap.
Infection is another cause of graft failure. The Blood supply to flaps may arise from seg-
bed should be debrided and well vascularized and mental, anastomotic, and axial vessels that have
should have a bacterial count of fewer than 105 perpendicular muscle perforations terminating
organisms per gram of tissue. If the wound bed is in the dermal-subdermal plexus of the skin.
poorly vascularized, the wound must be debrided Random flaps are based on this blood supply.
until vascularized tissue is obtained, or it must be Random cutaneous flaps are perfused through
covered with a flap. this dermal-subdermal plexus in the dermal and
perifollicular layers of the skin. Myocutaneous
flaps have these perforating musculocutaneous
16.4.5 Buccal Mucosal Graft (BMG) vessels that enter the base of the flap, supplying
a small skin area.
Buccal mucosal graft (BMG) has become the Flap blood may also be supplied by vessels
most popular source of epithelium in hypospa- that penetrate the underlying muscle fascia and
dias repair. The buccal mucosal graft for one- lie in the subcutaneous tissue. These longitudi-
stage repair is covered in full details in Chap. 39 nally oriented vessels supply the dermal-
and for two-stage repair in Chap. 40. subdermal plexus directly. Axial flaps are based
on this blood supply. Axial flaps not only have an
arterial pedicle portion but also distally have a
16.5 Flaps random-pattern portion of the flap.
Grafts are dead tissues that you try to bring

16.5.1 Random Flaps
to life; flaps are living tissue that you try
not to bring to death.
Random flaps are based on perforating vessels.
They have a limited length-to-width ratio because
of their blood supply. In the head and neck area,
A flap is a tissue that is transferred from one random flaps may have a length-width ratio of
area of the body to another with its original blood 2:1 or 3:1. In the rest of the body, length-width
supply intact. Flaps may consist of skin, subcuta- ratios of 1.5:1 or 1:1 may be safely designed. The
neous tissue, muscle, fascia, bone, nerve, omen- dermal-subdermal plexus should be maintained
tum, or other tissues (such as intestine). Flaps when designing these flaps.
may be classified according to their blood supply Random cutaneous flaps may be divided
or to the tissue transferred (Fig. 16.6): according to their design and positioning. Two
types of random flap used to close local defects
• A flap which consists of skin and superficial are rotation flaps, which rotate around a pivot,
fascia is referred to as a skin flap. and advancement flaps.
Fig. 16.6 The different types of flaps
Flaps that rotate are divided into true rotation gular. They are usually used for local coverage of
flaps and transposition flaps, both of which are a triangular defect. The flap should be made lon-
positioned into a defect. They are generally ger than the side of the defect so that the actual
rotated from an area that has enough laxity to diagonal length from the pivot before transfer
allow primary closure of the donor site (second- and the estimated length after transfer are similar.
ary defect). The donor site may require the use of This flap may also require a short back cut across
a back cut (relaxing incision) or skin graft for part of the base to reduce tension. The secondary
closure. Secondary defects should not be closed defect must be closed, and therefore a graft may
under tension. be necessary. The flap donor site may also be
True rotation flaps are semicircular and rotate closed by transposition of a secondary flap from
around an axis into the defect for local closure. the most lax skin at right angles to the primary
Transposition flaps are advanced laterally flap. This technique is known as a bilobed flap.
along an axis that is at an angle to the original Another transposition flap is the Z-plasty
axis of the flap. These flaps are generally rectan- (Fig. 16.7), where two triangular flaps are raised
332 A. T. Hadidi
Fig. 16.7 The diagonals of the Z-plasty, showing how, with transposition of the Z-plasty flaps, the contractural diago-
nal is lengthened and the transverse diagonal is shortened
(undermined) and transposed. The transposition forward. With bipedicle advancement flaps, two
achieves a gain in length along the direction of incisions are made parallel to the defect to cre-
the common limb of the Z, and the direction of ate a flap that is undermined and advanced for-
the common limb of the Z is changed [5]. A ward to close the defect. In the case of V-Y
Z-plasty has one central member or limb and two flaps, another type of advancement flap, a
angled limbs, which together resemble a Z. The V-shaped incision, is made and closed in the
angled limbs are equal in length to the central shape of a Y [6].
limb and are usually angled at 60 degrees. A
greater angle may be selected in a series when
not enough tissue is available for one large 16.5.2 Axial Flaps
Z-plasty and when the esthetic result is of pri-
mary concern. Other transposition flaps include a Axial pattern flaps or arterial flaps are based on
Limberg flap and an interpolation flap. The direct longitudinal vessels lying in the subcuta-
Limberg flap is useful for closure of rhomboid neous tissue. These flaps carry their own direct
defects with angles of 60 and 120 degrees. The artery and vein, which branch into the dermal-
sides are the same length as the short axis of the subdermal plexus. Because these flaps include
rhomboid defect. their own major blood vessels, they can have
An interpolation flap is a skin and subcutane- large length-width ratios, with long, narrow
ous flap that is transposed into a local but not flaps created. If the flap has its artery, veins, and
immediately adjacent defect. The pedicle must skin intact in its pedicle, it is called a peninsular
pass over or under adjacent tissue to be placed flap. If the skin in the pedicle is cut and the flap
into the defect. A common interpolation flap is a is connected to the body by only its intact ves-
deltopectoral flap. sels, it is called an island flap. An island flap in
Advancement flaps are designed and moved which the artery and veins are divided, the flap
directly forward to close a defect. In a single- transferred to a new site, and the vessels re-
pedicle advancement flap, a rectangle of skin is anastomosed to the recipient vessels is called a
incised on three sides, undermined, and moved free flap.
An example of an axial flap maintaining its arteries in the fat, and subfascial arteries from the
longitudinal blood supply is the meatal-based intermuscular septa. Blood vessels extend along
flap (Mathieu and SLAM techniques, see Chaps. the fibrous septa between muscles or compart-
21 and 22). The blood supply is maintained ments and then branch out at the deep fascia to
through the fascia that is elevated with the flap, form plexuses. From the plexuses, branches radi-
and there is no need to observe the length-width ate to the skin. Based on the blood supply, fascio-
ratio. cutaneous flaps may be either pedicle or free
Microsurgical techniques allow for large seg- flaps, depending on the fasciocutaneous system
ments of tissue to be detached and transferred to involved. Some flaps such as forearm free flaps
a recipient site. Using microsurgery, the arterial may also include tendons, muscle, and vascular-
and venous axial vessels are anastomosed to the ized bone. Fasciocutaneous flaps are generally
recipient vessels. Anastomosis of vessels 0.5– very simple and quick to raise and are highly
2.0 mm in diameter can routinely be successfully reliable.
performed. With vessels in the 2.0–4.0 mm range,
the vessel can be anastomosed using a coupling
device. Cutaneous, muscle, musculocutaneous, References
fascial, fasciocutaneous, osteocutaneous, and
osteomusculocutaneous flaps and free vascular- 1. OpenStax_College. Layers of the dermis; Anatomy &
physiology. Connexions web site file, p. 506; 2013.
ized bone grafts are some of the free flaps that 2. Childs DR, Murthy AS. Overview of wound healing
have been successfully constructed and trans- and management. Surg Clin North Am. 2017; https://
ferred. Sensory and motor flaps can restore sen- doi.org/10.1016/j.suc.2016.08.013.
sation and function. These microtechniques have 3. McCarthy JG. Introduction to plastic surgery. In:
Plastic surgery. Philadelphia: Saunders; 1990. p. 2.
also allowed for successful reattachment. 4. Vasconez LO, Vasconez HC. Plastic and reconstructive
surgery. In: Way LW, Doherty GM, editors. Current
diagnosis & treatment: surgery. 11th ed. McGraw-
16.5.3 Fasciocutaneous Flaps Hill; 2003. p. 1230–42.
5. McGregor IA, McGregor AD. Fundamental tech-
niques of plastic surgery and their surgical applica-
Fasciocutaneous flaps are composed of fascia and tions. 9th ed. Edinburgh: Churchill Livingstone; 1995.
skin based on the vascular systems of the deep 6. Slate RK. Principles of plastic surgery. In: Ehrlich
fascia. The deep fascia is supplied by perforating RM, Alter GJ, editors. Reconstructive and plastic
surgery of the external genitalia, adult and pediatric.
arteries from underlying muscle, subcutaneous Philadelphia: Saunders; 1999. p. 1–5.
Principles of Hypospadias Surgery
17
Ahmed T. Hadidi
Abbreviations suture material and knots, suturing techniques,

hemostasis, urinary diversion, and dressing.
CEDU Chordee Excision and Distal
Urethroplasty
DSD Disorders of sex development 17.2 Preoperative Preparation
–– Preoperative investigation: A normal healthy

child with distal hypospadias, without any
17.1 Introduction symptoms, disease, or associated anomaly,
may not require any investigations before sur-
The majority of hypospadias surgery is per- gery. Some centers require routine urine anal-
formed in children. The ideal age is from 3 to ysis, full blood count, and coagulation profile.
9 months [1]. Although a normal penis grows Abdominal ultrasound is useful to screen the
only about 0.8 cm between the ages of 1 and kidneys and the urinary bladder before hypo-
3 years [2], hypospadias surgeons should use spadias surgery. If the hypospadiac child has
magnification and fine plastic surgery instru- an associated undescended testis, a chromo-
ments. Just as the best golf clubs do not make a somal analysis is required to exclude DSD.
professional out of a duffer, however, neither –– Preoperative hormone therapy: The major-
does specialized instruments ensure good opera- ity of surgeons would use preoperative hor-
tive results. mone therapy if the glans penis width is less
This chapter deals with general operative prin- than 12 mm. However, this is a controversial
ciples of hypospadias surgery under the follow- topic and discussed in detail in Chap. 13.
ing categories: preoperative preparations, –– Vaccination: Vaccination impairs the body’s
positioning, magnification, traction and retrac- immune response. It is recommended to have
tion, tissue handling and viability, instruments, the last vaccination at least 1 month before
surgery and the next vaccination, 1 month
after surgery.
–– Shower or bath: Collected evidence recom-
mends that the patient should have a bath or
A. T. Hadidi (*)
shower 1 day before surgery.
Department, Sana Klinikum Offenbach, Academic –– Antiseptic preparation of the genital area:
Teaching Hospital of the Johann Wolfgang Goethe Aqueous-iodophor solutions are the recom-
University, Offenbach, Frankfurt, Germany mended antiseptic solution. It is important to
336 A. T. Hadidi
wait 3 min after application to allow the material leads to a low overall complication rate.
release of free iodine radicles. Magnification, either with loupes or an operating
microscope, helps to preserve blood supply and
allow the operator to perform accurate, meticu-
17.3 Positioning lous anastomosis.
To facilitate the use of magnification, bright
Hypospadias repair is a demanding operation that spot illumination of the operative field is needed
means the surgeon’s head, neck, and shoulders and may need to be augmented by the use of a
are leaning forward for an hour at least. Most sur- head lamp.
geons prefer to bring the patient’s waist near the
edge of the operating table, and the surgeon sits
either on the side or at the foot of the table. Sitting 17.5 Traction, Retraction,
has the advantages that it reduces fatigue and and Tension
enables others to see the procedure clearly.
Sitting alongside the operating table allows The first step in the operation is to apply traction
smooth movement of the operating hand; thus, and maintain stabilization of the penis. This is
precise cutting and suturing can be achieved. achieved by placing a traction suture in the glans
penis using a fine nonabsorbable material (5-0
nylon or silk on a small rounded needle).
17.4 Magnification Although many surgeons prefer a longitudinal
suture, I prefer a transverse one as it is less likely
Magnification has contributed dramatically to to cut through the tissues and may provide more
progress and better results in hypospadias sur- stability and decrease torsion.
gery. The aim is to achieve fine tissue approxima- To minimize trauma to the tissues, the author
tion and fine cauterization of bleeding points. prefers to use stay sutures or skin hooks for trac-
The advantages of optical magnification were tion rather than retractors.
stated quite concisely by Dr. Norman Hodgson Another important point is that one should
[3]: avoid tension of the flaps, as this may interfere
with the blood supply to the flap through the ped-
icle. One should also avoid tubularization of the
This concept (optical magnification) is neourethra under tension, as this will definitely
important since magnification does more interfere with the capillary circulation of the flap
than just make the tissues larger. It devel- tissue and will lead to ischemia and failure.
ops a conscious awareness of fine vessels
and their handling in ensuring the ultimate
best result. With the delicate instrumenta- 17.6 Tissue Handling and Viability
tion and technology now available one can
assure the successful result in the majority The tissues of the penis are very sensitive to
of patients in this early age group. trauma (like the eyelids). A simple common
example is the edema that affects the prepuce
after any rough manipulation. A common finding
Rees et al. wrote that the use of magnification in surgical training centers is that although resi-
helps to avoid piercing the epithelial lining of the dents in training may be strictly supervised and
neourethra when it is being tubed and reduces the perform proper surgical techniques, unless they
chance of fistula formation [4]. Wacksman handle tissues delicately the results may be far
reported the use of optical magnification in hypo- from those desired. One should handle the tissues
spadias surgery (magnification 2–5 times) [3]. as the lioness holds her babies with her teeth,
The use of optical magnification with fine suture without hurting them. This is a very important
17 Principles of Hypospadias Surgery 337
ceps, curved mosquito forceps to hold the stay

sutures, and a standard rounded scalpel with a
number 15 blade. The photo in Fig. 17.1 shows
an example of a small set of five fine instruments
that can be added to a normal pediatric set and
helps a lot in hypospadias surgery. Special atten-
tion may be directed to the serrated scissors.
These scissors have the advantage of being very
sharp, like blades, yet they remain sharp for a
Fig. 17.1 Hadidi set of fine instruments used in hypospa- long time (Fig. 17.2).
dias surgery (©Ahmed T. Hadidi 2022. All Rights
Reserved)
17.8 Suture Material and Knots
In surgery in general, but especially in hypospa-

dias surgery, it is very important to choose the
proper suture material that will meet the needs of
the wound. There are many questions to ask
before choosing a specific suture (Table 17.1):
Fig. 17.2 Super cut scissors with serrated blade, ideal for
For how long is the maximum tensile length
hypospadias surgery (©Ahmed T. Hadidi 2022. All Rights needed? What is the effect of urine on the suture?
Reserved) What is the minimum size of suture that will hold
the tissues in place? What are the knotting char-
point and cannot be overstressed. To achieve this acteristics of the suture material? Does the knot
one should use hooks or very fine toothed instru- tend to form a fistula? Is the suture to be used for
ments to support the tissues rather than grasping skin or deeper structures? Should one use a cut-
the tissues. ting needle or a round-bodied needle [5–9]?
Another important point is tissue dryness and In general, sutures may be divided into absorb-
ischemia. One must constantly ensure adequate able and nonabsorbable. Absorbable sutures are
tissue hydration simply by continuously adding derived from mammals or synthetic polymers.
saline and avoid ischemia by checking the tourni- The material is capable of being absorbed but
quet time, avoiding tight sutures, conducting may be treated to modify its resistance to absorp-
delicate fine cautery of the bleeding points, and tion. Absorbable sutures include plain and chro-
avoiding hematomas. mic catgut, coated polyglactin 910 (Vicryl),
Another helpful tip is to not use dry swabs. I polyglycolic acid (Dexon), polydioxanone
prefer to use wet swabs after being squeezed to (PDS), polygluconate (Maxon), and poliglecap-
be nearly dry. This seems to be more gentle to the rone 25 (Monocryl). Nonabsorbable sutures
tissues and absorbs blood better than dry swabs. include silk, nylon, Prolene, Mersilene, Dacron,
and Ti-cron.
It is generally accepted that nonabsorbable
17.7 Instruments sutures should be excluded from contact with
urine because of the potential for stone forma-
For hypospadias surgery, one does not need a tion. Furthermore, the genitourinary tissues
large number of instruments. However, the instru- regain their tensile strength rapidly, and the pro-
ments must be very fine and delicate, and scissors longed presence of a suture is not necessary.
need to be very sharp. The instruments needed Most hypospadias surgeons tend to use an
include fine serrated scissors, fine needle holder absorbable material to minimize fistula forma-
(for 6/0 and 7/0 sutures), fine toothed tissue for- tion. They tend to use the smallest size that will
338 A. T. Hadidi
Table 17.1 Absorbable sutures commonly used in reconstructive urology

Suture material Trade name Filament Tensile strength loss Absorption time (days)
Plain surgical gut Plain gut Variable up to 7 days, as long as 10 days 70
Natural fiber
Chromic surgical gut Chromic gut Variable up to 14 days, as long as More than 90
21 days
Coated polyglactin Vicryl 75% at 14 days, 50% at 21 days 56–70
910 Synthetic braided
Coated polyglycolic Dexon plus 60% at 7 days, 20% at 15 days 90–120
acid Synthetic braided
Polydioxanone PDS More than 85% at 14 days, 60% at 120–180
Synthetic 28 days
monofilament
Polyglyconate Maxon 75% at 14 days, 65% at 21 days 120–180
Synthetic
monofilament
Poliglecaprone 25 Monocryl 50%–70% at 7 days, 20%–40% at 91–119
Synthetic 14 days
monofilament
hold the tissues together on the most gentle needle both epithelial edges in contact and to avoid ever-
available. My personal preference is to use 6-0 or sion of edges [16]. Eversion of the suture line
7-0 Vicryl on a cutting needle for the urethro- increases periurethral reaction, which can predis-
plasty. I prefer a cutting needle because it allows pose to urine leakage and potential fistula or
one to perform proper subcuticular suturing for diverticulum formation. Ulman et al. (1997) doc-
the neourethra without mutilation of the tissues. umented a statistically significant lower fistula
Nevertheless, many hypospadias surgeons prefer rate (4.9% vs. 16.6%) for subcuticular repair in
to use fine monofilament suture material because comparison with a full-thickness (through and
it passes through the tissues very gently. However, through) technique [17]. Many surgeons prefer to
when monofilament sutures are used for urethral have urine-tight anastomosis. However, one
reconstruction, urinary diversion is often needed should weigh this against the more serious danger
to prevent the potential adverse effects of urine on of tissue ischemia. Spatulated anastomosis is
suture integrity [8, 10–14]. El-Mahrouky et al. important to prevent leakage at the original
showed in his in vitro study that polydioxanone meatus, where most fistulae occur. The suture
(PDS) has more tendency than polyglactin lines of tubularized neourethra can be placed pos-
(Vicryl) to react with urine and may cause more teriorly against the corpora to bury as much of the
fistula formation [10]. Guarino et al. showed that anastomosis as possible.
polydioxanone (PDS) has more tendencies to Interposition of multiple tissue layers between
form suture tracts than polyglactin (Vicryl) [15]. the neourethra and the skin and avoiding the
crossing of suture lines are essential to hypospa-
dias repair [18]. Attention to these points has
17.9 Suturing Techniques helped to improve the results of hypospadias
repair dramatically.
For glans closure, the author prefers to use
Healing occurs in between the sutures (not
interrupted transverse mattress suture. The rea-
where sutures are).
son is that simple interrupted sutures may cut
through the soft glanular tissues, causing glans
For urethroplasty, a subcuticular continuous dehiscence (Fig. 17.3b).
suture using a fine cutting needle is highly recom- For skin closure, many surgeons prefer to use
mended (Fig. 17.3a). The idea is to try to have interrupted subcuticular rapidly absorbable mate-
b c
Fig. 17.3 Suturing techniques: (a) A continuous subcuticular suture for urethroplasty. (b) Interrupted mattress suture
for glans closure. (c) Continuous transverse suture for skin closure (©Ahmed T. Hadidi 2022. All Rights Reserved)
rial to avoid fistula formation. However, this is Tsur et al. in their initial experience in 1979 [20].
rather meticulous and time consuming. I person- They had reported a decline in the incidence of
ally prefer continuous transverse mattress sutur- UCF after application of a thin layer of butyl cya-
ing as this gives very good cosmetic results noacrylate over the suture line.
(Fig. 17.3c). Four different types of sealants are available
Another drawback of interrupted suturing and can be divided into (a) natural, based on
inside the tissues is the presence of knots in the fibrin, albumin, and cryocalcium, and (b) syn-
tissues. These knots, no matter how fine, will thetic, cyanoacrylate based. The fibrin glue con-
cause tissue reaction for absorption and may sists of fibrinogen-rich cryoprecipitate, factor
result in fistula several years after successful ure- XIII, and thrombin. Based on the natural coagu-
thral reconstruction. lation cascade, it can be reabsorbed by the scav-
enging macrophages within 14 days of its
application and thus has poor mechanical
17.10 Tissue Sealants strength. The other significant complications
include risk of infection and anaphylaxis [19]. In
Tissue sealants and adhesives have been utilized comparison, the cyanoacrylate group degrades
for wound closure for more than a century [19]. slowly and has minimal toxicity. The toxicity
Their use in hypospadias was first described by can be further reduced by utilizing the long-
340 A. T. Hadidi
chain forms, i.e., n-butyl cyanoacrylate (nBCA) Elder et al. reported the use of 1% lidocaine
and OCA. Cryocalcium glue is a combination of with epinephrine (1:100,000) to infiltrate the skin
a patient’s cryoprecipitate and calcium gluco- incision and the glans. They wrote that “tissue
nate. It can be prepared within minutes and can ischemia has not resulted, and it is less cumber-
be used immediately. Due to an autologous ori- some than using a tourniquet” [27].
gin, it is free from allergic complications [21]. The other alternative is to use fine bipolar dia-
BioGlue sealant has bovine albumin and glutar- thermy to coagulate single bleeding vessels and
aldehyde as constituents. Although the tensile minimize ischemia to adjacent tissues.
strength of BioGlue is superior to the fibrin glue, The author personally prefers using fine bipo-
its low-viscosity makes the application challeng- lar diathermy only and does not like to inject epi-
ing [22]. nephrine solution as it distorts tissue planes and
Tissue sealants may be used over the suture- prefers not to use tourniquet at all, as it increases
line, skin edges, and the site of fistula. They have postoperative edema and vessels may not rebleed
applied also below the skin flaps to decrease tis- immediately but after wound closure and the
sue edema [23] and on the penile dressings to application of dressing.
provide adequate postoperative compression [24] One area that is particularly prone to bleed a
or as an alternative to dressing [25]. lot and diathermy does not seem to control the
There are controversial reports in literature, bleeding is at the perineum (near the prostatic
and there are no prospective controlled studies plexus). The best way to control bleeding in this
that showed objectively an additional value of the area is to apply pressure for some time using
use of tissue sealants. swabs soaked in adrenaline solution 1 in 100,000.
17.11 Hemostasis 17.12 The Width of the Neourethra

(Fig. 17.4)
Careful hemostasis is a very important factor in
hypospadias repair. Inadequate hemostasis pre- A small change in the lumen diameter has a large
disposes to hematoma formation and infection. effect on the urine flow.
On the other hand, excessive cauterization causes 10 mm lumen = 10 mL/s
tissue necrosis and may result in infection and 8 mm lumen = 5 mL/s
failure of repair.
For distal hypospadias, intraoperative bleed-
ing can be minimized during dissection by the 17.13 Urinary Diversion
intermittent use of an elastic tourniquet for peri-
ods of up to 30 min. The goal of urinary diversion is to prevent ure-
Redman used a rubber band tourniquet to thral edema from obstructing urine flow and to
achieve hemostasis routinely during hypospadias allow the neourethra to heal completely before
repairs [26]. The tourniquet itself consists of a contact with urine. Yet, the chosen device should
length of rubber band held in place by a hemo- be easy to place and remove, comfortable to
static clamp after being placed proximal to the wear, and convenient to care for at home [28].
site of expected bleeding. He preferred a rubber Common forms of diversion include:
tourniquet which is 0.1 cm thick and 1–3 cm
wide. The ischemia time ranged from 20 to
60 min. A common fear of the use of a tourniquet
in penile surgery in children is that prolonged
ischemia may result in thrombosis in the corpora. Fig. 17.4 The urine flow through the neourethra is influ-
Redman stated that ischemia under 50 min is well enced by the diameter of the neourethra according to this
tolerated without complication. equation
• Perineal urethrotomy omy until the neourethra has healed completely

• Natural perineal urethrotomy and then close the natural urethrotomy after
• Suprapubic cystostomy 3–6 months after complete healing of the distal
• Foley catheter drainage neourethra. This is not a new principle as it was
• Transurethral drainage (dripping stent) used by Duplay [29] and Byars [30] and nowa-
days in the “Chordee Excision and Distal
Urethroplasty” technique (CEDU) [31], see
17.14 Perineal Urethrotomy Chap. 33.
The insertion of a catheter into the bladder

through the bulbous urethra is known as a peri- 17.16 Suprapubic Cystostomy
neal urethrotomy. The technique varies with the
type of catheter used. For example, a straight Two methods of suprapubic diversion are fre-
catheter can be inserted directly into the bladder quently used. The first is a standard surgical
and a lubricated guide then passed within the suprapubic cystostomy with a small incision and
lumen of the catheter. An incision is made at the direct placement of a Malecot catheter in the
perineum where the guide presents as a bulge, the bladder.
guide is removed, and the catheter distal to the The recently introduced commercially avail-
urethrotomy is carefully pulled through the inci- able percutaneous trocar cystostomy sets with
sion. The catheter should then be anchored silicone Malecot catheter, or F8 silicone tubing
securely to the adjacent skin, to prevent its dis- has essentially replaced surgical cystostomy.
lodgement. If a Foley catheter is utilized, a simi- Ease of replacement, diminished bladder spasm,
lar procedure is carried out with the perineal and non-incorporation into the dressing have
incision being made over the tip of a pediatric made this form of diversion popular for extensive
sound. hypospadias repairs requiring long periods of uri-
Coran suggested a simplified technique for nary diversion [32].
perineal urethrotomy using a tendon-pulling for-
ceps. The tendon-pulling forceps is passed
though the urethral meatus and pushed out on the 17.17 Urethral Catheterization
perineum against the posterior urethra. After an
incision has been made on the perineum, the for- At the Hospital St. Louis in Paris, August
ceps is pushed through and a Foley catheter is Nélaton (1807–1873) used Goodyear’s vulcani-
grasped. The Foley catheter is then placed into zation process to produce what is still known and
the bladder, and the balloon is inflated. As a fur- used as Nélaton catheter. The catheter was first
ther safety measure, the catheter is also secured made of red rubber and had a solid tip. Nowadays,
to the perineal skin with a silk suture. A suprapu- Nélaton catheters are used for single, short-term
bic cystostomy can be performed employing bladder catheterization. It is manufactured from
either a Foley or a Malecot catheter or the same nontoxic, nonirritant medical grade PVC com-
forceps, by using the same principles. patible with catheter lubricants. The distal end is
coned for nontraumatic introduction with two
lateral eyes for efficient drainage. The proximal
17.15 Natural Perineal end is fitted with a universal funnel-shaped con-
Urethrotomy nector for extension. The funnel-shaped connec-
tors are color coded for instant identification of
Some recent techniques employ a “natural ure- sizes. If the urinary catheter is intended to stay
throtomy” principle. The principle is to recon- more than a couple of days, it is recommended to
struct the distal neourethra, leaving the area of use a silicone catheter to avoid irritation of the
the original meatus intact as a perineal urethrot- urethra.
342 A. T. Hadidi
The French scale system is commonly used to

measure the size of a urinary catheter. It is most
often abbreviated as Fr, F, or CH (after Charrière,
its inventor). The French size is a measure of the
outer diameter of a catheter and is three times the
diameter in millimeters. Thus, the French size is
roughly equivalent to the circumference of a cir-
cular catheter; the true circumference being
slightly larger (circumference = diameter x
3.14285714). For example, a urinary catheter of
8 Fr has an outer circumference of 8 mm and an
external diameter of 8/3 mm. In other words, you
need a urethral plate of 8 mm wide to close
Fig. 17.5 Tiemann catheter transurethral drainage
around an 8F catheter. An increasing size corre-
sponds to a larger external diameter and a larger
outer circumference. postoperative period, as well as affording protec-
The most common nonsurgical form of intu- tion of the glanular portion of the repair from
bated diversion is the self-retaining Foley ure- inadvertent injury from movement of the catheter
thral catheter. Molded silicone catheters, 8, 10, [32]. Baskin et al. reported that quite good suc-
and 12 F in size, are currently available for use in cess was obtained with this technique, which
the hypospadias patient. Because of its molded allows all cases of hypospadias now to be done
nature, the internal diameter of the catheter is on an outpatient basis [33].
maximized, providing adequate drainage. Ease of Some authors do not favor any form of urinary
placement, plus the fact that it serves as a stent diversion (catheter drainage or dripping stent) in
for the repair, represents a significant advantage the repair of distal hypospadias. In 1987,
for this form of diversion [32]. Rabinowitz described his experience in catheter-
Some authors do not favor this type of diver- less Mathieu repair in 59 children with distal
sion [1], citing potential infection from bacterial hypospadias [34]. Rabinowitz cited that since
migration along the catheter or damage to the watertight urethroplasty is performed without
repair by inadvertent removal. Additionally, there overlying suture lines, catheter drainage and uri-
seems to be a diversity of opinion as to whether nary diversion may not be necessary. In 1993,
the occurrence of bladder spasm is attributable to Wheeler et al. supported the conclusion of
the Foley catheter, as the catheter balloon often Rabinowitz and stated that the urethral stent may
rests on the trigone. be associated with significant morbidity without
However, there appears to be a remarkable conferring any advantages in the postoperative
disparity of patient response to an indwelling course or the final results [35].
catheter [32]. A good kind of transurethral cath- In 1996, Hakim et al. reported the results of
eter to use is the Tiemann silicone catheter with a a retrospective study to test the complication
curved soft tip that fits well with the curvature rate for Mathieu repair performed with and
between the penile and bulbar urethra (Fig. 17.5). without a stent and compared the results among
Some surgeons prefer to use a stent which goes groups [36]. Their conclusion was that the fis-
just through the urethra to avoid bladder spasms tula rate and overall complication rate in the
after surgery (Fig. 17.6). stented and unstented groups were not signifi-
If long-term diversion will be required or cantly different.
extensive glandular dissection is carried out in In 1997, Steckler and Zaontz reported the
the course of the repair, a urethral form of diver- findings of a study on stent-free Snodgrass repair
sion with stenting of the repair is recommended. [37]. Stent-free repairs were performed in 33
This allows for a voiding trial at any time in the children. Follow-up was obtained in 31 children.
a b
Fig. 17.6 (a) Zaontz stent, (b) Koyle stent
There was no postoperative urinary retention, fis- totally concealing, partially concealing, and
tulas, or meatal stenosis. No unusual or pro- un-concealing. The most frequently used type
longed discomfort distinguished these children is the partially concealing dressing, in its many
from those who underwent a standard Snodgrass variations. Common dressings include: cotton-
technique. ball dressing [26], the X-shaped elastic dress-
On the other hand, some authors found the ing [40], silicone foam elastomer [41], adhesive
application of urethral stent or even suprapubic membrane dressing [42], Tegaderm dressing
catheter mandatory: In 1994, Buson et al. stated [43], sprays [44], and DermoLite II tape dress-
that application of urethral stent in distal hypo- ing [45].
spadias repair is necessary to reduce the compli- The practical requirements of outpatient man-
cation rate [38]. In 1997, Demirbilek and agement after surgery dictate a simple dressing
Atayurt came to the conclusion that although (Tegaderm or silicon foam) that does not restrict
urethral reconstruction without suprapubic the child’s mobility, or no dressing at all.
diversion allowed early hospital discharge and A bio-occlusive membrane dressing (e.g.,
outpatient treatment, the use of a suprapubic Tegaderm) is wrapped around the glans and
catheter for urinary diversion in hypospadias shaft after the penis has been coated with an
repair is to be recommended because it makes adhesive substance. It is important to keep the
for a lower complication rate and is more com- penis dry during application of the membrane to
fortable for the patient during the postoperative allow complete adhesion. In particular, the pres-
period [39]. ence of dripping urine may prevent a tight seal
to the glans and cause the membrane to be later
dissected off by urine. The membrane is cut to a
17.18 Dressings size that covers from the tip of the glans to the
penoscrotal junction. The penis is then wrapped
The main purpose of a proper dressing is to pro- snugly with two layers. The penis can be
vide immobilization with prevention of hema- wrapped circumferentially or with the two ends
toma and edema. Preference for particular of film adhered to each other as a mesentery or
dressings is as varied as the other fetishes of “dorsal fin.” It is important to include the glans
hypospadias surgery. In fact, there may be as in the dressing to prevent excessive glans
many different types of dressing as there are swelling.
types of repair. In general, hypospadias dress- The major benefit of the membrane dressing is
ings may be divided into three main categories: its simplicity, allowing early mobilization and
344 A. T. Hadidi
minimal home care. There are few complications,

and removal is easy. The vast majority of patients
with this dressing can be treated as outpatients,
without hospital admission. The dressing can
usually be removed easily by slipping the whole
tube upward off the penis. The dressing is typi-
cally left in place for 5–7 days. The dressing not
uncommonly falls off at home but rarely before
the third or fourth day, making its replacement
unnecessary. Because the dressing is imperme-
able, the need for routine early dressing changes
due to soiling is obviated. The transparent nature
of the dressing allows observation during heal-
ing. The simplified nature of the dressing may
also help to relieve parents’ anxiety about taking
care of the child at home. One caution is that if
the dressing begins to roll up or down the penis, Fig. 17.7 A simple dressing that secures the catheter
it may form a tight constricting band, requiring against the abdominal wall for a few days and enables free
immediate removal. mobility of the child
Silicone foam elastomer compound as a dress-
ing in hypospadias surgery was first reported by In the Hypospadias Center, Frankfurt,
De Sy and Oosterlink in 1982 and has since Germany, we use a simple dressing of gauze and
gained wide acceptance [41]. The foam base and fixomull for 2 days (Fig. 17.7). This enables the
catalyst are preheated and then mixed in a child to sleep in prone position and full mobility.
syringe. The mixture is then injected into a mold Hadidi et al., in 2003, having conducted a
(e.g., plastic coffee cup) placed around the penis, prospective randomized study to assess the role
filling upward from the base. The foam sets in of dressing in hypospadias surgery, concluded
approximately 2 min, after which time the mold that repair of hypospadias without dressing
is removed. The cast can then be further anchored offers statistically significantly better results
at the base with adhesive tape. The cast can be than when using dressing (P = 0.014) [48]. The
easily separated and removed after scoring its role of dressing is discussed in further detail in
outer surface. The primary advantage of the foam Chap. 55.
cast is its secure immobilization of the penis,
combined with easy removal with minimal dis-
comfort. It also provides a secure base on which References
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Analgesia and Pain Control
18
Günther Federolf
18.1 Introduction 18.2 hat Difference Does It

W
Make for a Child and Its
The birth of a child with any type of deformity is Family that It Has
a devastating experience for the parents. They Hypospadias?
will naturally be anxious about whether the con-
dition can be corrected with a minimum of dis- A baby is not aware that he is different from other
tress, to the child and family. babies in an organ that is biologically and cultur-
With the treatment of any child, there is ally very important. However, the parents will
involvement of a medical team. The whole family respond to the hypospadias with a wide range of
needs support and reassurance throughout the responses varying from acceptance to guilt, and
different stages of treatment. Hypospadias is their feelings may influence their relationship
sometimes more than a single stage procedure, with their child. They are also often afraid about
and if anything occurs that leads to stress and dis- the outcome of the surgery.
trust in the parents, then subsequent treatments
will be far more difficult to manage [1, 2].
This chapter is written for surgeons, to enable 18.3 Preparation for Surgery
them to have a better understanding of general and Anaesthesia
and regional anaesthesia for healthy children
between three months and five years of age. This Nowadays, the hypospadias is diagnosed at birth
encompasses the vast majority of patients that by the attending paediatrician, due to the presence
undergo hypospadias repair procedures and the of abnormal foreskin. Referral for repair will be
anaesthetic protocols used at Sana Klinikum made to a specialized paediatric surgeon or urolo-
Offenbach, where more than 600 hypospadias gist. During the first consultation, the surgeon
repairs are performed each year. must explain all the relevant details of the opera-
tion and possible complications and how many
operations the child may need. Parents often fear
the anaesthesia more than the surgery, and the sur-
geon may be forced to address the issue of anaes-
thesia. During this first contact, the surgeon can
prepare for the meeting with the anaesthesiolo-
G. Federolf (*) gist, but a definite dialogue about anaesthesia has
Pediatric Anaesthesia Unit, Sana Klinikum Offenbach to be done by an anaesthesiologist. The date for
GmbH, Offenbach am Main, Germany
e-mail: guenther.federolf@sana.de the informed anaesthesia consent is obtained just
348 G. Federolf
prior to the surgery. Normally, being elective, the do not specifically mention possible death, but I
date for the operation may be weeks or months in don’t hide that even with a healthy child, in prin-
the future. This period can be used to give the par- ciple, anything can happen. However, I do stress
ents advice beforehand (What if the child gets that the operation will give the child a better qual-
sick? When should we consult a cardiologist ity of life and that the anaesthesia will improve
when the child has a murmur? What medical the result of the operation.
reports for my preterm baby should we bring or
send upfront?). The anaesthesiologist would like
to see an optimally prepared child not too long 18.5 History and Laboratory Tests
before the surgery. In our institution, this is usu-
ally the day before surgery. To avoid having a sick A careful history of the patient is the main source
child coming to the hospital, we encourage the of information and is normally sufficient without
parents to call an attending physician or a consul- further tests. Age, weight, length, and develop-
tant in charge when in doubt. ment should be known. Exceptions are made for
sick children with a history of cardiac, pulmonary,
neurological, or endocrinological diseases or pre-
18.4 Informed Consent term babies. In these cases, information should be
exchanged in advance. The need for further tests
Parents are often more concerned about the risk should be discussed early, and a perioperative plan
of anaesthesia rather than the risk of surgery should be made. Other exceptions are bleeding
itself. Hypospadias repair for a small child is far disorders, for example, and tests and plans should
less life threatening than a gall bladder procedure be made according to the diagnosed disorder.
for an 80-year-old patient with a history of myo-
cardial infarction.
However, the lower the age, the higher the risk 18.6 pecific Fears and Risks
S
of anaesthesia. The risk for major anaesthetic About Anaesthesia
complications is about 0.4% below 1 year, com-
pared to 0.05% between 1 and 14 years [3]. Most 18.6.1 W
ill My Child Wake Up After
parents are afraid that the child may not wake up Anaesthesia?
after the operation, and they will not be reassured
to be told that hypoxia, not death, is the main It is clear that there is a difference between being
danger during anaesthesia for a small child. The anaesthetized and sleeping. Of course, some chil-
optimal age to operate upon a child should be dis- dren need a longer time for anesthesia emer-
cussed and decided in advance with the parents, gence, but there is a definite risk of hypoxic brain
surgeon, and anaesthetist. damage. The risk for a hypoxic period during an
Informed consent means that parents get the anaesthesia is inversely proportional to the age of
information they need and give legal consent for the patient and the experience of the anaesthesi-
the anaesthesia. I begin by explaining the fasting ologist. In a retrospective analysis, a SaO2 of
rules, premedication, the application of EMLA <90% occurred in 56% of neonates [4]. One
(EMLAR: “Eutectic Mixture of Local should try to explain the measures taken to secure
Anaesthetics”) and transportation to the preopera- a child’s airway and ventilation in a way that the
tive reception area. I try to explain the anaesthetic parents can understand.
procedure, being aware that all details may not be
fully comprehended by many parents. One should
try to avoid increasing the family’s anxiety but 18.6.2 C
an My Child’s Intellect
should be clear about all possible risks. After Be Impaired by Anaesthesia?
explaining the anaesthetic procedures, the anaes-
thetist should ask the parents if they fully under- This question has received much public attention
stood all the information or need further details. I during the last few years. Some laboratory ani-
18 Analgesia and Pain Control 349
mal studies, and retrospective cohort studies, 18.6.3 C

an My Child Become Lame
suggested that repeated anaesthesia in babies From Caudal Anaesthesia?
may result in programmed cell death, apoptosis,
and lead to slightly reduced educational perfor- The risk of a caudal anaesthesia, which is our
mance [5–7]. standard regional anaesthesia, is low.
However, a properly controlled anaesthesia Complications, which are usually minor, occur in
(blood pressure, oxygen saturation, temperature, about 0.1% of cases [13, 14]. Spinal anaesthesia is
etc.) does not result in apoptosis in newborn rarely indicated for hypospadias repair. It wears
monkeys [8]. Controlled studies in humans, off early in children, so there is no postoperative
which are difficult to perform without bias, have analgesic benefit. Early apnoea in patients below
found no difference in anaesthetized groups and 60th week of postmenstrual age is reduced.
controls [9]. One recent cohort study even hints However, late apnoea which may happen after
that anaesthesia (and operation) may be a larger transfer to the ward is not reduced with spinal
developmental disturbance when performed at an anaesthesia compared to general anaesthesia [15].
older age [10]. Other researchers have noticed In longer procedures, epidural anaesthesia
more Medicaid services for learning disabilities may be used. With lumbar epidural anaesthesia,
correlated to anaesthesia (and surgery) before the risk of nerve injury is low, but not zero.
three years of age [11]. The risk of learning dis- Careful research for bleeding disorders of the
ability increased with the number of exposures patient and family should be obtained. If it is
and with the cumulative time of general anaes- positive, I refrain from spinal or epidural (caudal)
thesia [12]. However, the effects and severity of anaesthesia.
the underlying disease cannot be separated from I usually compare regional anaesthesia with
necessary anaesthesia. There are no studies com- an epidural, which is often familiar to the mother,
paring the effects of surgical procedures on the benefits being less postoperative pain and a
developing brain with or without anaesthesia, lighter general anaesthetic. Normally, caudal
and hopefully no ethics committee will allow one anaesthesia is recommended, but the parents
to be performed. must give consent without pressure.
In a Danish nationwide cohort study, the effect Postponing anaesthesia and surgery is often
of surgery and anaesthesia in infancy on academic discussed. Every anaesthesiologist prefers to put
performance was not significant. The difference healthy children to sleep, but during winter many
between boys and girls, favouring the latter sex, children may have running nose or mild cold. I
was larger than the history of early surgery. tend to postpone when a parent tells me that they
Whether a procedure is staged or not seems to think the child is sick, and it is not a normal infec-
be of no importance with respect to apoptosis or tion. Fever and general symptoms, like weakness
how induction and emergence are handled in and lethargy, suggest postponing the operation.
staged procedures; it must be emphasized that These are definite reasons that the child could be
every child has the right to have proper anaesthesia in a better state of health with lesser risk at
without the risk of hypoxia or hypothermia, etc. another date, and the case should be postponed.
The ultimate aim is a better life for the patient, However, if the patient will probably be in a simi-
which means an optimal surgical result, with the lar state of health at another date, there is no rea-
least acceptable risk. No risk means that no treat- son to postpone [16, 17]. The vast majority of
ment is possible. cancellations are done by the parents themselves
I try to explain to the best of my knowledge some days prior to the operation.
how the experts have dealt with the data. A good
anaesthesia should have no influence on the
development of a small child. Performing the 18.7 The Day of the Operation
procedure later may be more problematic, per-
haps because the child is more aware of its Usually, the parents are roughly informed of
environment. the anaesthesia and surgery. The fasting
350 G. Federolf
guidelines vary according to different anaes- 18.9 Mask or IV Induction?

thetic protocols in different countries. The
fasting times in our institution follow the After careful preparation of the equipment, stan-
German guidelines: one hour for clear liquids, dard and emergency medications, ECG, pulse
four hours for breast milk in children younger oximeter, and blood pressure cuff are attached to
than one year, and six hours for other nutri- the patient. Normally, an IV line is placed. A
tion. Parents are encouraged to let the chil- sedated child with EMLAR is not usually aware
dren drink until one hour before the operation of the needle. Mask induction is used when the
[18]. child prefers it or when the veins can only be
Two hours before the operation, EMLAR is punctured successfully when the child is immo-
applied to vein puncture sites. EMLAR is a local bile. Contraindications against mask induction
anaesthetic, but it also has vasoconstrictive prop- are small or sick children or children with an
erties and should be removed 20 min before the upper respiratory infection. After the child is
puncture. Thirty minutes before induction of anesthetized deeply enough, which usually takes
anaesthesia, Midazolam 0.5 mg/kg is given a bit longer than expected, an IV line is placed.
orally. Whether Midazolam is the best premedi-
cation is still under discussion. At our institution,
Be prepared with succinylcholine 5 mg/kg
it is given to most patients older than six months
IM together with atropine (in case the child
of age. More important than a specific dosage is
develops laryngeal spasm and there is no
the timing and whether the child swallows the
IV line established).
whole dose.
Preoperatively, the patients are called to the
preoperative or preparatory area, and all the
patient’s records are checked. When the IV line is in place, the child is given
propofol 5 mg/kg (IV induction, not after mask
induction), rocuronium 0.2 mg/kg (if an intuba-
18.8 resence or Absence
P tion is planned), and alfentanil 20 μg/kg. These
of Parents During medications are used in our hospital. Propofol
Induction use is widespread, and an allergy against soy-
beans is no contraindication. Thiopentone 7 mg/
It is rarely of help to the anaesthesiologist if a kg may be an alternative. Similarly, other muscle
parent is present during induction. An exception relaxants and opioids can be used.
may be made for an older, mentally disabled
child who is focused on a single person. Proper
sedation may benefit a child much more than 18.10 Airway
having an anxious parent around [19]; an anx-
ious child may be calmer when a relaxed parent A good mask airway should be part of the training
is present. Parents feel more comfortable and of every anaesthesiologist, but in operations lasting
relaxed when they are present as their child longer than some minutes, there is no need to
wakes up. occupy one hand when there are better alternatives.
I use an IV access in the recovery and pre- The laryngeal mask is an airway that keeps the
anaesthetic area and give Midazolam together hands of the anaesthesiologist free, which is
with a small dose of ketamine. Having experi- important in times when documentation con-
enced both parental presence and absence during sumes a large part of your time. The laryngeal
induction of anaesthesia, I feel better when I mask seems to be less invasive than a tube, but it
have only the patient to deal with. Almost all is not without airway side effects such as a sore
parents accept that it is better for me to focus on throat. A laryngeal spasm can occur especially in
the child. children with a history of upper respiratory tract
infection, in small children, or in phases of light will be a caudal anaesthesia. The patient is posi-
anaesthesia, e.g. emergency at the end of the tioned on the left lateral side for right-handed
case. I use an LMA for children who are healthy, anaesthesiologists. Care should be taken about
not too small, and for procedures that don’t last the airway, which should stay in place. The punc-
too long. Recently, the use of an LMA in children ture site is slightly caudal to a line between the
has gained more attention [20]. cornua sacralis. One or two 10 cc syringes are
An endotracheal tube is a more invasive filled with ropivacaine 0.2% (1 mL per kg body
choice, but the airway is more secure than with a weight), and clonidine (1–2 μg/kg) is added for a
laryngeal mask. Intubation of children has to be longer-lasting anaesthesia. One syringe is con-
taught. Even after a good preoxygenation, the nected with a short IV line to ensure an immobile
time for desaturation when not ventilating the needle after puncture of the epidural space.
patient will be much shorter than in adults. The This is a sterile procedure. After disinfecting
smaller the child, the quicker the desaturation. the skin, a 25 gauge needle with a stylet is
Intubation is normally easy in the healthy child, directed through the skin in a 45° angle.
but a plan has to be made beforehand how to ven- Advancing the needle for about 1.5 cm (0.5″), a
tilate the child if the second attempt to intubate resistance by the sacrococcygeal ligament is felt.
fails. Children don’t die from not being intubated, Further advance will be followed by loss of resis-
but they do die from repeated unsuccessful tance. The needle is turned down, nearly parallel
attempts to intubate without ventilation. to the skin, and advanced a short distance of
The tube has to be fixed in a way that prevents 2–3 mm (Fig. 18.2). The stylet is removed.
it from moving too much in or out, which is of Aspiration should show no liquor (this may hap-
high importance in a younger child [21]. In smaller pen in smaller children after advancing the nee-
children, nasal intubation is the preferred choice. dle too far) or blood (which may be the result of
bone contact or an epidural vessel). There is no
definite test medication that gives reliable infor-
18.11 Cuffed or Uncuffed Tube? mation as to whether the needle is inside an epi-
dural vessel. One should be prepared that even
The recent trend favours the cuffed tube. One rea- with a perfect caudal placement, part of the
son being the reduced rate of tube change after regional anaesthetic may be placed in a vessel.
intubation [22]. However, the anatomy of the Fortunately, these events are rare, and they have
child’s larynx, with the smallest diameter being no deleterious effects. After the regional anaes-
below the vocal cords, should favour an uncuffed thetic is given, the child is positioned supine and
tube. Also, the useful inner diameter (with respect the operation prepared.
to ventilation resistance) is larger with an Patients above 25–30 kg may not be suitable
uncuffed tube compared to a cuffed tube with the for caudal anaesthesia. Also, parents may not
same outer diameter. give their consent, or there are contraindications
In practice, I use uncuffed tubes, nasally (mostly suspected bleeding disorders). In these
placed, in smaller children, sicker patients, and cases, a penile block is a possible alternative.
longer procedures. In larger children, a cuffed Whether to give it before or after the operation
tube is orally placed, and in shorter operations a depends on the length of the procedure. Penile
laryngeal mask is used. block before the incision will allow for a lighter
anaesthetic, but the patient will suffer earlier
from postoperative pain because the block wears
18.12 Regional Anaesthesia off quicker than with a caudal or with clonidine.
(Fig. 18.1) Giving the penile block after the last suture will
prolong the emergence, but it will prolong the
After securing the airway, a regional anaesthesia postoperative analgesia for a longer time. In rare
is part of the standard procedure. Usually, this cases, a lumbar peridural catheter anaesthesia
352 G. Federolf
Fig. 18.1 The nerve supply of the penis
may be given. Indications are older patients or the needle cephalad, and the Huber point of the
patients whose postoperative pain will be severe needle gives an inadequate change in angle of
for days. insertion of the cannula (Fig. 18.3). The catheter
may also be inserted via the caudal route. I person-
ally do not use this method as I find the catheter
18.13 Epidural Analgesia difficult to fix in place. It is also very difficult to
keep the area of insertion sterile postoperatively.
Epidural analgesia is more complicated to perform When the catheter is in place, a loading dose
but allows for top-ups to be administered. It is of local anaesthesia should be injected, and here
technically slightly more difficult as the catheter the volumes call for 0.5% to be used. This gives a
has to be directed caudally if the lumbar route is dense block which will obtund the reaction to
used. The angle of the lamina will tend to direct surgery with the advantages detailed above.
Fig. 18.2 Caudal analgesia
Fig. 18.4 Penile block
18.14 Penile Block
The dorsal penile nerves are blocked using an

approach slightly off the midline (12:30 and 11:30
o’clock positions), where the dorsal penile nerves
lie on each side of the dorsal penile vein
(Fig. 18.4). A small-gauge, short bevelled needle
is inserted perpendicular to the skin and advanced
to the symphysis pubis. Then, while withdrawing
the needle, the placement is tested by depressing
the plunger of the syringe and noting when resis-
tance is lost. This enables one to locate Buck’s
fascia, where the anaesthetic solution is to be
deposited. Care should be taken not to penetrate
Fig. 18.3 Epidural analgesia the corpora cavernosa just inferior and to avoid
the dorsal penile vein. Doses for the anaesthetic
The best analgesia postoperatively is achieved range from 1 to 5 mL of 0.25% bupivacaine,
by an infusion of 0.125% of bupivacaine. Top-up depending on the age and the weight of the patient.
doses may be used if nursing staff are unfamiliar A potential complication is injection of the cor-
with the infusion technique. This has the advan- pora with the possibility of thrombosis [24].
tage of the presence of an anaesthetist when the
top-up is given to deal with any complications.
The major disadvantage is that the pain will recur 18.15 Local Application
prior to the top-ups and a much bigger dose of
local anaesthesia will be required to maintain Local application of an anaesthetic agent is use-
analgesia in the postoperative period [23]. ful as an adjunct to the other blocks and also post-
operatively prior to any dressing change or bath.
354 G. Federolf
The first bath after this operation can be an 18.17.3 B Breathing

unpleasant experience, and 2% lignocaine half an
hour prior is helpful. Pressure-controlled ventilation is a standard to
begin with: PIP of 13–15 mbar, PEEP of 5 mbar,
respiratory rate of 20–35/min, inspiratory time of
18.16 Summary of Agents Used 0.5–1 s, FiO2 of about 0.3, during induction and
before emergence 0.8. These settings should
• Local anaesthetics: Bupivacaine, ropivacaine, result in a tidal volume of 6–8 mL/kg, an etCO2
chirocaine, lignocaine. of about 40 mmHg (or slightly higher), and a
• Adjuvants: Clonidine, ketamine, methadone. pulse-oximeter reading of 98–100%. These are
• Supplementary pain management: For dress- suggestions, but not rules. Volume-controlled
ing changes, local anaesthetic gel may be ventilation is not standard for paediatric patients,
employed. Another useful technique is to use but there is not sufficient data to suggest that it is
Entonox, which is extremely effective in the contraindicated for operations. Usually, after
older cooperative child [25]. Many other tech- induction, the mode can be switched to assisted
niques have been used for sedation, including ventilation, or even spontaneous breathing, espe-
morphine and temazepam. In general, how- cially with a laryngeal mask. Whether PEEP is
ever, scheduling an anaesthetic for these events indicated when a laryngeal mask is used is not
is preferable to achieving near-anaesthesia by verified.
excessive sedation. Considerable doses may be Patients with lung disease (e.g. bronchopul-
needed to achieve a dressing change, and when monary dysplasia) should be ventilated as nonin-
the stimulus is removed, the child may have a vasively as possible. A low PIP will lead to a
level of sedation that is dangerous from the higher etCO2, which is a normal value for these
point of view of respiration and airway patients. Accordingly, the saturation should not
control. be higher than is normal for the child. A higher
• Sedation agents: Morphine, temazepam, diaz- PEEP may be necessary.
epam, Vallergan, trichlophos.
18.17.4 C Circulation
18.17 Maintenance of Anaesthesia
By minimizing the NPO times, a child rarely
18.17.1 A Airway—See Above presents in the OR with a volume deficit.
Induction is usually tolerated without a decrease
18.17.2 A Anaesthesia in blood pressure; however, after caudal anaes-
thesia is given, a decrease in blood pressure due
Anaesthesia is maintained with sevoflurane 0.8 to vasodilation may take place. In the past, there
MAC. This can usually be reduced to about 0.4 was agreement that decreases in BP are tolerated
MAC when the caudal block is working. When well by a healthy child. However, recent data
meddling with the airway during the case, a MAC shows that in longer operations, care should be
of 0.4 is not sufficient. Desflurane and isoflurane taken to avoid blood pressure decreases [26, 27].
are risky when using a laryngeal mask. Crystalloids are given as a balanced solution.
Propofol can also be used for maintenance. Glucose is added to give a 1% glucose solution in
Necessary doses are higher than in adults, and children younger than one year of age. In larger pro-
there are no validated databases to measure cedures, blood glucose measurements should be
the depth of anaesthesia in small children. I taken to avoid both hypo- and hyperglycaemia [28].
use sevoflurane as a standard and switch to The basic intake of fluids is given according to
propofol if there are contraindications for the 4-2-1 rule, 4 mL/kg/h for the first 10 kg,
sevoflurane. 2 mL/h for every kg between 10 and 20 kg, and
1 mL/h for every additional kg. During an opera- remove a tube is to wait until the child is wide
tion, more fluid is necessary [29]. As a rule of awake, which is not always the most convenient
thumb, 10 mL/kg/h are infused. A blood pressure emergence from anaesthesia. The worst thing to
drop is treated with a bolus of 10 mL/kg of bal- do is to extubate during excitation, which is a
anced crystalloid and another 10 mL/kg if needed. guaranteed way to get experience treating
If the child is not responsive to fluid loading and laryngospasm.
is still hypotensive, vasopressors may be used, Having a light anaesthesia together with a
which is rarely necessary. A false reading in mea- caudal block, the time of excitation is short, but
suring the blood pressure (interference by the once the anaesthesiologist has decided not to
operating team, or a suboptimal adjusted blood extubate in deep anaesthesia, he has to wait until
pressure cuff) has to be ruled out. the excitation phase has passed.
With a laryngeal mask, it is not necessarily an
advantage to let the child become wide awake.
18.17.5 D Drugs During excitation, even small stimuli may lead to
laryngospasm. If spontaneous breathing is rhyth-
Emergency medications should be prepared and mic and strong, I usually remove the LMA early
available quickly. Succinylcholine 5 mg/kg IM is and check to see how the child is doing. I do not
given when an emergency intubation is necessary recommend transferring a spontaneously breath-
or a laryngeal spasm cannot be treated otherwise ing child to the recovery room with an LMA in
and an IV line is not yet established (or lost). place.
Atropine is no longer part of the resuscitation
algorithm, but it is given together with succinyl-
choline to avoid bradycardia. Severe bradycardia 18.19 Recovery Room
or asystole is usually the aftermath of hypoxia.
Epinephrine 10 μg/kg alone will not help the During and after transfer to the recovery room,
patient long term, but it may help to give some the most important feature of the patient is an
time to re-establish an airway and oxygenate the unobstructed airway. Oxygen may be nice, but it
patient. is of no help if the airway is obliterated by the
Additional postoperative pain care is started tongue. Monitoring includes pulse oximetry,
before incision using rectal paracetamol and/or ECG, and blood pressure. These are redundant,
IV Metamizol to reduce local mediators. because an asystolic ECG means that some min-
utes have passed before the saturation has gone to
zero. However, an ECG may be more reliable
18.17.6 E Temperature Management than a pulse oximetry reading. After the child has
(Environment) become stable, the parents are called.
It is better when children sleep and recover in
The patients are kept on a warming blanket. The the recovery room. Some children are definitely
body and the head are covered with sheets, and not calm. This is not necessarily due to pain,
the surgical exposure is minimal. They should especially if the caudal has worked during the
stay warm, even if the OR temperature is operation. However, pain has to be ruled out or
23–26 °C. treated in a restless child. As a backup pain medi-
cation, we use piritramide (analogous to mor-
phine) 50 μg/kg IV which can be repeated.
18.18 Emergence from Anaesthesia If the patient stays in excitement, ketamine
0.5 mg/kg or propofol 0.5 mg/kg may be tried
The decision to remove an endotracheal tube or a [30]. Midazolam seems not to be effective.
laryngeal mask may be more difficult than the Usually, even without response to medical treat-
placement after induction. The safest way to ment, excitement fades after about 30 min.
356 G. Federolf
Vomiting is treated with dexamethasone and surgery during childhood with long-term aca-
demic performance. JAMA Pediatr. 2017; https://doi.
0.15 mg/kg. In children with a history of periop- org/10.1001/jamapediatrics.2016.3470.
erative nausea and vomiting, dexamethasone is 8. Zhou L, Wang Z, Zhou H, Liu T, Lu F, Wang S, Li J,
given prophylactically after induction. If dexa- Peng S, Zuo Z. Neonatal exposure to sevoflurane may
methasone is not effective, ondansetron 0.15 mg/ not cause learning and memory deficits and behav-
ioral abnormality in the childhood of Cynomolgus
kg is added. monkeys. Sci Rep. 2015; https://doi.org/10.1038/
srep11145.
9. Sun LS, Li G, Miller TLK, et al. Association between
18.20 On the Ward a single general anesthesia exposure before age 36
months and neurocognitive outcomes in later child-
hood. J Am Med Assoc. 2016; https://doi.org/10.1001/
A cardiopulmonary stable and pain-free child jama.2016.6967.
that is able to drink is transferred to the ward. 10. Graham MR, Brownell M, Chateau DG, Dragan
Given the limited effective time of a caudal RD, Burchill C, Fransoo RR. Neurodevelopmental
assessment in kindergarten in children exposed
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dren. Br J Anaesth. 1999; https://doi.org/10.1093/ children. A randomized clinical trial. Middle East J
bja/83.1.118. Anesthesiol. 2013;22
24. Chhibber AK, Perkins FM, Rabinowitz R, Vogt
AW, Hulbert WC. Penile block timing for post-
Part II
Operative Techniques
Grade I: Glanular Hypospadias;
Double Y Glanulomeatoplasty 19
(DYG) Technique
Ahmed T. Hadidi
Abbreviations 1898. These include meatal advancement glanu-

loplasty incorporated (MAGPI) [4, 5] and its
DYG Double Y glanulomeatoplasty modifications [6, 7], glans approximation proce-
GAP Glans approximation procedure dure (GAP) [8], and Koff [9], to mention a few.
MAGPI Meatal advancement glanuloplasty Furthermore, there is an increasing interest to
incorporated decrease postoperative patient discomfort, reduce
MEMO Meatal mobilization the risks of complications, and provide cost-
SLAM Slit-like adjusted Mathieu effective therapy.
TIP Tubularized incised plate The double Y glanulomeatoplasty (DYG)
technique is a simple technique, suitable for
patients with glanular hypospadias and a mobile
meatus. This technique helps to achieve a termi-
19.1 Introduction nal, slit-like meatus with minimal complications
(2%). The principle of the technique is to make
Historically, glanular hypospadias was not two incisions: a Y incision along the lower part of
repaired routinely due to the potential risks and the meatus and an inverted Y along the upper part
possible unsatisfactory cosmetic outcome. of the meatus that extends to the tip of the glans
Although the function of the penis may not be penis. Hence, the technique is called “double Y
impaired in some patients with glanular hypospa- glanulomeatoplasty” or DYG. At the end, it will
dias, there has been increasing concern of parents be a rhomboid incision around the meatus that
and older patients about the psychological effects extends in the midline proximally to the coronal
of having an abnormal looking penis [1]. sulcus and distally to the tip of the glans.
A myriad of procedures for glanular hypospa-
dias have been developed during the last 40 years
based on the principle of urethral mobilization 19.2 Selection of Patients
first described by Beck [2] and Hacker [3] in
The double Y glanulomeatoplasty (DYG) tech-
A. T. Hadidi (*) nique is only suitable for selected patients with
Hypospadias Center and Pediatric Surgery glanular hypospadias and mobile meatus and in the
Teaching Hospital of the Johann Wolfgang Goethe absence of deep chordee (Figs. 19.1a and 19.3a).
University, Offenbach, Frankfurt, Germany Those patients usually have a little ridge distal to
362 A. T. Hadidi
a b
Fig. 19.1 A child with mobile meatus that is suitable for firmed under anesthesia using a fine toothed forceps
the DYG technique. (a) Distal ridge that is suggestive of a (©Ahmed T. Hadidi 2022. All Rights Reserved)
mobile meatus. (b) The mobility of the meatus is con-
a b
Fig. 19.2 Patient with glanular hypospadias with an toothed forceps cannot push the meatus to the tip of the
immobile meatus, unsuitable for the DYG technique. (a) glans (©Ahmed T. Hadidi 2022. All Rights Reserved)
No clear ridge is seen distal to the meatus. (b) A fine
meatus. This ridge can be pushed with a fine technique (“slit-like adjusted Mathieu”) for distal
toothed forceps to the tip of the glans (Fig. 19.1b). hypospadias is performed (see Chap. 22). If DYG
If the distal edge of the urethral meatus is is performed when the urethra is not mobile or
immobile (Fig. 19.2a) and cannot be pushed to the meatus is at the coronal sulcus or more proxi-
the tip of the glans (Fig. 19.2b), the child is not mal, there is a higher risk of complications and
suitable for the DYG technique, and the SLAM retraction of the meatus.
19 Grade I: Glanular Hypospadias; Double Y Glanulomeatoplasty (DYG) Technique 363
19.3 Operative Technique to meet the lateral limbs of the inverted Y incision
(Fig. 19.3e).
A 5/0 nylon traction suture is placed on the glans, A 6/0 Vicryl suture approximates the distal
dorsal to the tip of the glans, and chordee is end of the meatus to the tip of the deep incision of
excluded using the artificial erection test. An the glans without making a knot. A couple of
inverted Y incision is outlined on the glans. The deep dorsal midline sutures fix the stretched ure-
center of the inverted Y is in the middle of the thra in the glans and reduce the chances of meatal
distal ridge of the meatus. The longitudinal limb retraction.
extends in the midline to the tip of the glans The upper edges of the two glanular wings are
where the tip of the neo-meatus will be located. fixed to the inside edge of the urethral meatus
Each oblique limb of the inverted Y is about using three Vicryl 6/0 sutures (Fig. 19.3f). Care
0.5 cm long, and the angle between them is must be taken that the urethral meatus is sutured
60 degrees (Fig. 19.3b and 19.4a). The proximal to the inner side of the glanular wings to obtain
Y is drawn on the ventral part of the meatus and good cosmetic results and avoid having suture
extends down to the coronal sulcus. marks at the meatus. The medial edges of the
If the meatus is narrow or pinpoint, it is incised glanular wings are sutured together in the midline
to make it wide enough to accommodate a cath- using two or three Vicryl 6/0 sutures in a trans-
eter F12 or larger according to the age of the verse mattress manner (Fig. 19.4c, d). This helps
patient and size of the penis. A transurethral to avoid the fine sutures cutting through the glans.
Nelaton catheter size F12 or larger is inserted During dissection and mobilization, the thin
into the bladder. distal urethra was injured in 8 patients. The hole
A subcoronal incision (3 mm proximal to the was repaired with 7/0 Vicryl on a rounded needle,
coronal sulcus) along the ventral part of the penis and the repair was covered with thin preputial
is made using a sharp scissor. The incision is dartos fascia as a second protective layer.
deepened to reach the level of the corpora caver- Local gentamycin eye ointment is applied to
nosa. This is followed by going distally in the the wound, and a normal dressing is applied and
midline, along the longitudinal limb of the Y, then adhesive tape fixes the gauze, the catheter, and
around the meatus till the tip of the glans. The the penis against the lower abdominal wall
midline vertical incision of the glans is deepened. (Fig 19.4e). This allows free mobility of the
This will result in a midline meatus and urethra patient and secures the catheter and penis against
and two lateral, freely mobile glanular wings the lower abdominal wall, and the baby can sleep
(like an open book). freely on his abdomen, as most babies prefer.
The meatus and the urethra (with the catheter The transurethral catheter is left for 1–2 days
inside) are mobilized proximally. Extra care depending on the degree of mobilization and
should be taken to avoid injury of the very thin the degree of postoperative edema of the penis.
urethra underneath the skin. The use of sharp Caudal block is used as a routine to reduce
scissors (see Chap. 15) and traction helps to avoid postoperative pain. Although the patient may
injury of the distal urethra. Traction is applied on leave the operating room without a catheter, I
the glanular wings, and the incision is deepened prefer to leave a catheter for one night to reduce
using sharp scissors starting proximally (less the pain and the anxiety of the family when
thin) at the coronal sulcus. The glanular wings they see the child crying when passing urine.
are mobilized off the urethra (Fig. 19.4b). This is The operative time ranges between 35 and
a very important step that helps to wrap the glan- 60 min depending on the experience of the sur-
ular wings around the urethra without any ten- geon, the degree of mobilization, and the age of
sion. The incision is continued around the meatus the patient.
364 A. T. Hadidi
a b c
d
e f
Fig. 19.3 (a) Glanular hypospadias with mobile meatus. are mobilized deep enough to wrap around the urethra and
(b) Inverted Y incision. (c) The three flaps are elevated. are approximated in the midline. The 6 o’clock stitch is a
(d) The apex of the meatus is sutured to the tip of the 3-point stitch that brings the urethra and the two medial
glans. (e) A catheter F12 is introduced inside the urethra, edges of the glanular wings together and is magnified in
and a Y incision is made that surrounds the meatus and the inset (©Ahmed T. Hadidi 2022. All Rights Reserved)
extends down to the coronal sulcus. (f) The glanular wings
a b
c d
Fig. 19.4 Operative steps of the DYG technique: (a) An wings around the urethra. (e) Application of the dressing,
inverted Y incision is outlined on the glans. (b) The glanu- fixing the penis, and catheter against the abdominal wall
lar wings are mobilized, and the distal edge is fixed to the and allowing free mobility of the child (©Ahmed
tip of the incision. (c, d) After closure of the glanular T. Hadidi 2022. All Rights Reserved)
366 A. T. Hadidi
19.4 Postoperative Care There was a learning curve when we tried

the DYG technique in coronal hypospadias, but
The patient is discharged home after he passes that resulted in meatal retraction in three
urine without any dressing (just the diapers). It is patients. Also, we were using catheter size F10
our routine to give oral antibiotics for one week at the beginning. This was associated with
because the wound is very close to the anus, and meatal stenosis in five patients early in the
there is continuous contamination with the stool. study. The use of catheter size F12 has helped
The parents should keep the penis clean and dry to minimize this complication. Meatotomy with
and apply the local gentamycin antibiotic on the a small inverted Y corrected the stenosis in
wound for one week. those five patients. Wound dehiscence occurred
Parents are asked to send an e-mail one month in four patients when the technique was used in
after surgery to report the outcome. Our standard patients with fixed meatus. Total complication
postoperative follow-up protocol includes exami- rate is 3%.
nation after 3 months, one year, three years, and Injury to the thin urethra occurred in eight
then every 5 years until the child is 15 years old. patients early in the series, and that was repaired
The parents can always call or report if there is with Vicryl 7/0 and was covered with a thin layer
any problem (Fig. 19.5). of preputial dartos fascia. None of those eight
patients developed fistula postoperatively.
19.5 Complications
and Follow-Up The important tip is to start mobilization of
the glanular wings proximally at the coro-
The DYG technique has become our standard nal sulcus, where the corpus spongiosum is
technique for glanular hypospadias with mobile better developed.
meatus since 2004 and has withstood the test of
time with experience to date in 546 patients over
the past 17 years. Follow-up ranged between
17 years and 8 months (mean 45 months). At the Ninety-seven patients have maintained
follow-up visits, a good slit-like meatus in a near 10 years follow-up according to our standard pro-
normal looking glans (Fig. 19.6) is considered a tocol with satisfactory results and meatus at the
satisfactory result. tip of the glans (Fig. 19.7).
a b c
Fig. 19.5 Patient with glanular hypospadias: (a) DYG and foreskin reconstruction. Notice that even the
Preoperative picture. (b) The meatus is mobile and can be frenulum can be reconstructed in some cases (©Ahmed
pushed to the tip of the glans. (c) Follow-up 1 year after T. Hadidi 2022. All Rights Reserved)
a b
Fig. 19.6 Patient with glanular hypospadias one year after the double Y glanulomeatoplasty technique (DYG). (a)
Final appearance of the meatus. (b) Passing urine in a stream (©Ahmed T. Hadidi 2022. All Rights Reserved)
complications might leave the patient worse off

than if the anomaly were left untreated. During
the past thirty years, several techniques became
available for the surgical correction of glanular
hypospadias.
The meatal advancement glanuloplasty
(MAGPI) was introduced by Duckett in 1981 [4].
The technique was not always completely satis-
factory, which led the author to modify and refine
it in 1991 [5] to reduce the risk of meatal retrac-
tion. However, meatal advancement with the
Heineke-Mikulicz tissue rearrangement, the
focal point of MAGPI, can lead to an abnormal
meatal and glanular configuration [10] that, even
in the terminal position, may appear round and
abnormally puckered [11]. In fact, Hodgson con-
sidered MAGPI to be an “illusion” because the
glans is incorporated into the meatus and not the
contrary [12].
Fig. 19.7 Long-term follow-up of DYG in an 18-year- The glans approximation procedure or GAP
old after DYG and foreskin reconstruction (©Ahmed
T. Hadidi 2022. All Rights Reserved) [8] procedure is ideal for patients with wide, deep
glanular groove, but it entails suturing of the ure-
thra with the potential risk of fistula formation.
19.6 Discussion Zaontz is describing his technique with tips and
tricks including the routine use of preoperative
For many years, surgical techniques had an unac- testosterone therapy (see Chap. 12).
ceptable morbidity in glanular hypospadias and Redman described a technique for “minimal
did not consistently achieve shift of the meatus to distal balanitic hypospadias” [13]. This tech-
the tip of the glans. Since those patients exhibited nique entails excision of the small triangle distal
a minimal functional impairment, surgeons were to the urethral meatus and suturing the distal
reluctant to undertake an operation because the meatus to the tip of the glans as a form of
368 A. T. Hadidi
Heineke-Mikulicz technique or the first part of pattern outlined will vary in different societies
meatal advancement glanuloplasty (MAGPI) and does vary with the hospital organization and
technique. with the distances from the treatment center that
Seibold et al. described the meatal mobiliza- the patients have to travel.
tion (MEMO) technique for coronal and subcoro- There is usually severe swelling and edema of
nal hypospadias with mobile meatus [14]. In this the glans after hypospadias surgery. Most parents
technique, a transverse incision is made proximal and patients are usually worried immediately
to the meatus, the penis is degloved, and the glan- after surgery and report spraying of urine rather
ular wings are mobilized and sutured together than a single stream. This occurred in more than
proximal to the urethral meatus. 50% of patients included in this study in the
Vallasciani et al. described another modifica- period after removal of the catheter. The urine
tion of MAGPI in the form of a midline incision stream on micturition became steadily more
distal to the urethral meatus [10]. This incision is whorl like within a month after surgery.
deepened for 4–5 mm behind the meatus and is The choice of age of the patients was influ-
left unsutured. This was followed by rotation of enced by the time of referral and ranged from
the glanular wings in a manner similar to 4 months to 5 years. Patients with a narrow urine
MAGPI. The midline distal incision is in fact stream or straining during micturition may bene-
similar to the incision of the tubularized incised fit from earlier surgery. In the author’s experi-
plate procedure. ence, infants have quicker recovery from surgery
Adorisio et al. described a modification of and less complications than children older than
Koff technique where a circumcision incision is 3 years of age.
made distal to the coronal sulcus and the hypo- The author has used the DYG technique
spadias meatus [15]. The penile skin as well as successfully in recurrent, complicated cases
the urethra is mobilized down to the penoscrotal after failed TIP technique, provided the urethra
junction. A V-incision on the distal portion of the was mobile and could be stretched to the tip of
glans associated to a deep incision of the distal the glans. The technique is also suitable in
urethral plate was performed to place the urethra adults with glanular hypospadias and mobile
inside the glans. meatus.
Mollaeian et al., despite using a similar tech-
nique, reported two cases of persistent curvature
owing to inappropriate case selection [16], while References
Roodsari applied the V glanular flap technique to
those patients with anterior hypospadias with 1. Mieusset R, Soulié M. Hypospadias: psychosocial,
sexual, and reproductive consequences in adult life.
good cosmetic results and few complications J Androl. 2005; https://doi.org/10.1002/j.1939-
[17]. 4640.2005.tb01078.x.
The DYG technique is suitable for ambulant 2. Beck C. A new operation for balanic hypospadias.
day case surgery. The DYG technique was used New York Med J. 1898;67:147–8.
3. Hacker V. Zur operativen behandlung der hypospadia
in the first 25 patients without leaving a transure- glandis. Bruns Beitr Klin Chir. 1898:271–6.
thral catheter during 2003–2004. At the end of 4. Duckett JW. MAGPI (meatoplasty and glanu-
the procedure, during anesthesia, the bladder was loplasty). A procedure for subcoronal hypo-
compressed, and urine was emptied from the spadias. Urol Clin North Am. 1981; https://doi.
org/10.1097/00005392-200205000-00059.
bladder. A compressive dressing was applied for 5. Duckett JW, Snyder HM. Meatal advancement
4–6 h, and the child was allowed to pass urine and glanuloplasty hypospadias repair after 1,000
through the penis. This was stressful for the child cases: avoidance of meatal stenosis and regres-
and the parents. It was then found that leaving the sion. J Urol. 1992; https://doi.org/10.1016/
S0022-5347(17)37341-X.
catheter in for 1–2 days relieves the distress of 6. Decter RM. M inverted V glansplasty: a procedure
the child and his parents. The decision to perform for distal hypospadias. J Urol. 1991; https://doi.
the technique as a day case surgery or with the org/10.1016/s0022-5347(17)37881-3.
7. Arap S, Mitre AI, De Goes GM. Modified meatal 14. Seibold J, Boehmer A, Verger A, Merseburger
advancement and glanuloplasty repair of distal AS, Stenzl A, Sievert KD. The meatal mobili-
hypospadias. J Urol. 1984; https://doi.org/10.1016/ zation technique for coronal/subcoronal hypo-
S0022-5347(17)50846-0. spadias repair. BJU Int. 2007; https://doi.
8. Zaontz MR. The GAP (glans approximation proce- org/10.1111/j.1464-410X.2007.06790.x.
dure) for glanular/coronal hypospadias. J Urol. 1989; 15. Adorisio O, Elia A, Landi L, Taverna M, Malvasio V,
https://doi.org/10.1016/S0022-5347(17)40766-X. D’Asta F, Alfredo Danti D. The importance of patient
9. Koff SA. Mobilization of the urethra in the surgical selection in the treatment of distal hypospadias using
treatment of hypospadias. J Urol. 1981; https://doi. modified Koff procedure. J Pediatr Urol. 2010; https://
org/10.1016/S0022-5347(17)55048-X. doi.org/10.1016/j.jpurol.2009.06.015.
10. Vallasciani S, Spagnoli A, Borsellino A, Martini L, 16. Mollaeian M, Sheikh M, Tarlan S, Shojaei H. Urethral
Ferro F. Simplifying the surgical approach to glanular mobilization and advancement with distal triangular
and coronal hypospadias: longitudinal urethral inci- urethral plate flap for distal and select cases of mid
sion and glanuloplasty. J Pediatr Urol. 2007; https:// shaft hypospadias: experience with 251 cases. J Urol.
doi.org/10.1016/j.jpurol.2007.06.004. 2008; https://doi.org/10.1016/j.juro.2008.03.070.
11. Bracka A. Sexuality after hypospadias repair. BJU Int. 17. Roodsari SS, Mulaeian M, Hiradfar M. Urethral
2002;83:29–33. advancement and glanuloplasty with V flap of
12. Hodgson NB. Editorial Comment. J Urol. the glans in the repair of anterior hypospadias.
1984;131:98. Asian J Surg. 2006; https://doi.org/10.1016/
13. Redman JF. Technique for repair of minimal distal S1015-9584(09)60083-X.
balanitic hypospadias. Urology. 2006; https://doi.
org/10.1016/j.urology.2006.05.055.
MAGPI and Modified MAGPI
20
Heather Di Carlo and John P. Gearhart
Abbreviations genital appearance, have changed prevailing atti-

tudes in favor of surgical correction. In 1981 John
MAGPI Meatal Advancement and Duckett described the meatal advancement and
Glanuloplasty Incorporated glanuloplasty incorporated (MAGPI) procedure
MIP Megameatus intact prepuce for the repair of distal hypospadias, renewing
interest in this challenging endeavor [2]. Since its
initial description, the MAGPI operation contin-
ues to be performed worldwide in its original
20.1 Introduction form as well as in multiple variations.
The incidence and severity of hypospadias has

increased over the last several decades [1]. This is 20.2 Selection of Patients
thought to be a result of multiple factors includ-
ing exogenous hormone exposure during gesta- The key to successful outcomes in patients
tion, as well as environmental endocrine treated with the MAGPI procedure is the careful
disruptors. About 50–70% of hypospadias are selection of patients with the appropriate anat-
classified as distal or anterior—when the meatus omy. A good understanding of the nature of this
is located in a glandular, coronal, or subcoronal congenital defect is integral to the patient selec-
location. In the past, surgical correction of these tion process. The MAGPI procedure is applicable
distal defects was not undertaken due to the to many cases of the most distal anterior hypo-
potential morbidity involved with urethroplasties spadias without chordee. A glanular tilt or a
and the risk for complications requiring further minor degree of chordee is not an absolute con-
operations. It was thought that the minimal func- traindication but may make this an inferior tech-
tional and cosmetic defects did not warrant the nique. The insight and experience of the surgeon
potential complications of the corrective proce- should guide the decision-making at the time of
dures. Improved and more numerous surgical surgery.
techniques, as well as the increasing concern
expressed by parents and older patients about
The location and size of the urethral
meatus, quality of parameatal skin, and
glans configuration are the main determi-
H. Di Carlo (*) · J. P. Gearhart
Pediatric Urology, Brady Urological Institute, The nants [3].
Johns Hopkins Hospital, Baltimore, MD, USA
e-mail: hdicarl1@jhmi.edu
372 H. Di Carlo and J. P. Gearhart
A glanular or coronal meatus with a mobile vided the remaining ventral skin is adequate and
distal urethra is the ideal anatomy for the MAGPI the meatus remains mobile enough to be advanced
procedure. A more proximal hypospadias partic- [4].
ularly with chordee is unsuitable. Lifting the skin A circum-coronal incision is made about
below the meatus and pulling it up along with the 8 mm proximal to the urethral meatus, and the
urethra helps the surgeon judge the adequacy of penile shaft skin is degloved to the penoscrotal
urethral mobility. This allows the meatus to be junction. This leaves a mucosal collar below the
positioned at the tip of the glans. glans [5]. Any fibrous tethering bands causing
The size of the meatus determines the extent chordee are released. An artificial erection may
of lateral dissection of the glans. With a large be used to confirm that all bands have been
meatus (as in the “megameatus intact prepuce” released and to exclude any persistent curvature.
(MIP) variant), an adequate glansplasty may not A longitudinal incision is made, starting
be achieved with a MAGPI, and an alternative from inside the dorsal aspect of the meatus and
procedure needs to be considered. extending distally to the end of the glandular
The glans should be of adequate dimensions groove. The incision should be deepened if the
to allow wrapping around the advanced meatus, glandular groove is shallow to accommodate the
providing ventral support as well as a normal urethra. Occasionally, a prominent transverse
conical appearance. A good “ventral cushion” “lip” of tissue in the glans distal to the meatus
prevents fistulas and meatal retraction and causes deflection of the urinary stream. This
enhances aesthetics. may be removed with a wedge excision, if not
Thick and pliable parameatal skin that can be adequately flattened by the initial incision. If the
dissected off the distal urethra is essential. If the glans has a flat configuration, removal of a
skin overlying the urethra is very thin or adher- larger wedge of tissue provides more space to
ent, cutting back to healthy skin would result in a accommodate the advanced urethra, resulting in
more proximal defect which is unsuitable for an elliptical meatus [6]. The edges of this longi-
MAGPI. tudinal incision are brought together in a hori-
zontal Heineke- Mikulicz manner, allowing
advancement of the dorsal aspect of the meatus
20.3 Operative Technique into the distal end of the glans groove with 6-0
(Fig. 20.1) absorbable suture.
20.3.1 Meatoplasty
20.3.2 Glanuloplasty
A polypropylene traction suture is positioned in
the glans for retraction. Some surgeons use a Obtaining good glans coverage ventrally is vital
combination of epinephrine 1:100,000 and 1% for the support of the advanced meatus. To
lidocaine for hemostasis (either injected directly accomplish this, the ventral edge of the meatus is
into the glans tissue or applied topically with a retracted distally toward the glans, using a stay
soaked sponge). The meatus, glans, and perime- suture or a skin hook. This brings the edges of the
atal skin are carefully inspected to determine the glans up toward the meatus in the shape of an
most suitable technique for repair. Constant inverted “V.” The skin edges of the glans wings
awareness of the anatomy is crucial when consid- are trimmed to expose glans tissue (raw surfaces)
ering utilization of the MAGPI procedure for in order to promote good healing. This addition-
repair of the distal hypospadias. If the abovemen- ally serves to tailor the glans wings to provide a
tioned criteria are applicable, then a MAGPI is normal conical configuration. The glans tissue is
performed. When a previous hypospadias repair approximated in the midline in 2 layers with
has failed with a regressed meatus, we do not interrupted absorbable sutures, completing the
consider a repeat MAGPI contraindicated pro- glanuloplasty. While a solid glans approximation
20 MAGPI and Modified MAGPI 373
a b c
d e
f g
Fig. 20.1 The MAGPI procedure: (a) Circumferential groove and transverse Heineke-Mikulicz closure. (e, f)
subcoronal incision 8 mm proximal to the meatus. (b–d) Two-layer closure of the glans edges reconfigures a coni-
Excision of bridge of tissue between meatus and glanular cal meatus. (g) Sleeve reapproximation for skin coverage
prevents meatal retraction, care should be exer- improve the vascularity and substance of local
cised to prevent compression and narrowing of tissues in such cases.
the urethral lumen. Calibration of the urethra and
meatus before and after glansplasty, or intubation
of the urethra during this step, may help avoid a 20.4 Results
tight closure.
The mucosal collar tissue is then closed in two Ten years and more than 1000 patients after the
layers. Routine circumferential penile skin clo- original description of the MAGPI procedure [2,
sure is performed with fine absorbable sutures. If 3] reported an overall incidence of complications
ventral skin is deficient, skin flaps may be rotated requiring a second operation in 1.2% of cases: 5
from the dorsum, as in many hypospadias repair urethrocutaneous fistulas (0.45%), 7 meatal
techniques. For primary repairs, catheters or retractions (0.6%), and 1 persistent ventral curva-
stents are not required. In repeat MAGPI proce- ture (0.09%). No meatal stenosis was noted. The
dures, we leave a stent in for 3–5 days. Various MAGPI repair was adopted widely in the 1980s
dressing techniques have been described based as a simple, same-day procedure for distal hypo-
on surgeon preference, including tegaderm com- spadias. During the same period, others reported
pression dressing, simple topical skin adhesives, generally satisfactory results, albeit in smaller
or coban. groups of patients [10, 11], while higher compli-
cation rates also became evident [12, 13]. Meatal
regression and stenosis were the two commonly
20.3.3 Modifications cited problems. The proponents considered these
complications related to improper selection, so
Many modifications of the MAGPI repair have that those operated on included patients with the
been described. Some were meant to address meatus located too far proximal and with chor-
known complications, while others were aimed at dee. The opponents declared that lesser degrees
expanding the indications of MAGPI. These will of meatal regression were ignored by those
be described in detail in the succeeding chapter reporting excellent results and that their follow-
[7, 8]. up was inadequate. As we all are aware, there is
In the authors’ experience, the MAGPI tech- subjectivity in the evaluation of cosmetic out-
nique works well for the classically described comes in hypospadias repairs.
indications. In more proximal cases where the Several groups have stressed the importance
urethral meatus and overlying skin are mobile, of careful selection of the appropriate patient and
and the urethral plate is of good quality, we have “not stretching the indications” for the MAGPI to
extended the use of the MAGPI for a subcoronal avoid such complications [4, 14, 15]. This is
location. The modification that allows this to suc- underscored by a report from Ghali et al., who
ceed is good lateral mobilization of the glans had a 10% complication rate with 92 MAGPI
wings to ensure adequate glans coverage and repairs [16]. The authors stated that “with increas-
support for the advanced urethra. As previously ing experience of the surgeon, avoidance of the
mentioned, we also use repeat MAGPI proce- use of MAGPI when the meatus was hypoplastic
dures for failed hypospadias repairs with a or fixed to the underlying spongiosum resulted in
retracted meatus, provided the remaining ventral better cosmetic results.” An outcome study sur-
skin is adequate and the meatus is mobile. Similar veyed the parents of a group of 100 children who
experience has been reported by Marte et al. had undergone MAGPI repairs between 1985 and
(2001), with good results in 18 of 21 patients 1990. These repairs were similar to the original
using MAGPI for meatal regression after failed description except in cases of hypoplasia of the
Duplay, Mathieu, Snodgrass, and onlay buccal distal spongiosum, where the lateral Y-shaped
mucosa graft, at a mean follow-up of 3.5 years limbs were mobilized and approximated in the
[9]. Use of preoperative testosterone helps to midline, or a flap of preputial subcutaneous tissue
20 MAGPI and Modified MAGPI 375
was used to cover the spongiosum. Minor com- ance of a sutured urethroplasty (thus, increasing
plications were seen in 6%, and the patient- the desire to utilize this technique).
reported survey demonstrated a high level of
parent satisfaction at a mean interval of 5.7 years
after surgery [17]. 20.6 Conclusion
Moving forward into the twenty-first century,
small modifications in techniques and anesthetic The MAGPI procedure is a valid technique in the
techniques (spinal instead of general anesthesia) surgeon’s armamentarium for the correction of
continue to improve the care of these patients. distal hypospadias. It yields excellent outcomes
with proper patient selection. This technique has
renewed interest in the surgical correction of dis-
20.5 Complications tal hypospadias in the late twentieth century. A
good understanding of the penile anatomy is crit-
The reported incidence of complications after the ical for successful outcomes. The location and
MAGPI procedure ranges from 1.2% [15] to 10% size of the meatus, quality of parameatal skin,
[16]. Meatal stenosis and meatal regression are and glans configuration are the main determi-
the problems most commonly encountered, while nants, and the absence of significant chordee or
other infrequent complications include urethro- glandular tilt, a sufficient glans width, and a
cutaneous fistulas and chordee. mobile urethra are compulsory when considering
Meatal retraction can become pronounced this approach.
with growth of the penis and result in spraying of While it continues to be the bulwark of surgi-
the stream resulting in patients often sitting to cal options for the correction of the most distal
void. Meatal retraction may be a result of local hypospadias, the importance of selecting the
ischemia or inflammation after surgery. right patient for this procedure is vital.
Meticulous attention to tissue handling and
avoidance of undue tension on the repair helps
avoid this troublesome and unsightly complica- References
tion. A tight glans wrap constricting the distal
urethra and meatus should be avoided by mobi- 1. Paulozzi LJ, Erickson JD, Jackson RJ. Hypospadias
trends in two US surveillance systems. Pediatrics.
lizing the glans wings further laterally if neces- 1997; https://doi.org/10.1542/peds.100.5.831.
sary. Such tight wraps exert tension on the 2. Duckett JW. MAGPI (meatoplasty and glanu-
glanular closure resulting in glans separation and loplasty). A procedure for subcoronal hypo-
meatal retraction. The skin edges are trimmed to spadias. Urol Clin North Am. 1981; https://doi.
org/10.1097/00005392-200205000-00059.
bring glans tissue together in the midline for opti- 3. Duckett JW, Snyder HM. The MAGPI hypospadias
mal healing and ventral support of the advanced repair in 1111 patients. Ann Surg. 1991; https://doi.
meatus. Supple perimeatal tissues and a mobile org/10.1097/00000658-199106000-00012.
distal urethra prevent late retraction. 4. Issa MM, Gearhart JP (1989) The Failed MAGPI:
management and Prevention. Br J Urol doi:https://doi.
Strategies to avoid meatal stenosis include a org/10.1111/j.1464-410X.1989.tb05981.x
meatoplasty extending into the dorsal part of the 5. Zaontz MR, Dean GE. Glanular hypospadias repair.
meatus, thus widening its caliber, and a deep inci- Urol Clin North Am. 2002; https://doi.org/10.1016/
sion of a shallow glanular groove. S0094-0143(02)00024-1.
6. Taneli C, Genç A, Günsar C, Sencan A, Arslan OA,
Distal hypospadias is rarely associated with Daglar Z, Mir E. Modification of meatal advance-
significant chordee. Such patients may benefit ment and glanuloplasty for correction of distal hypo-
from either a tubularized incised plate or a spadias. Scand J Urol Nephrol. 2004; https://doi.
meatal-based or an onlay type of flap repair after org/10.1080/00365590310020042.
7. Arap S, Mitre AI, De Goes GM. Modified meatal
chordee correction rather than a MAGPI repair. advancement and glanuloplasty repair of distal
The risk of urethrocutaneous fistulas with hypospadias. J Urol. 1984; https://doi.org/10.1016/
MAGPI is comparatively low due to the avoid- S0022-5347(17)50846-0.
8. Decter RM. M inverted V glansplasty: a procedure dias. Br J Urol. 1989; https://doi.org/10.1111/j.1464-

for distal hypospadias. J Urol. 1991; https://doi. 410X.1989.tb05198.x.
org/10.1016/s0022-5347(17)37881-3. 14. Gibbons MD. Nuances of distal hypospadias. Urol
9. Marte A, Di Iorio G, De Pasquale M. MAGPI Clin North Am. 1985; https://doi.org/10.1016/
procedure in meatal regression after hypospa- s0094-0143(21)00802-8.
dias repair. Eur J Pediatr Surg. 2001; https://doi. 15. Duckett JW, Snyder HM. Meatal advancement
org/10.1055/s-2001-17153. and glanuloplasty hypospadias repair after 1,000
10. Hensle TW, Badillo F, Burbige KA. Experience with cases: avoidance of meatal stenosis and regres-
the MAGPI hypospadias repair. J Pediatr Surg. 1983; sion. J Urol. 1992; https://doi.org/10.1016/
https://doi.org/10.1016/S0022-3468(83)80005-0. S0022-5347(17)37341-X.
11. Livne PM, Gibbons MD, Gonzales ET. Meatal 16. Ghali AMA, El-Malik EMA, Al-Malki T, Ibrahim
advancement and glanuloplasty: an operation for d istal AH. One-stage hypospadias repair. Experience
hypospadias. J Urol. 1984; https://doi.org/10.1016/ with 544 cases. Eur Urol. 1999; https://doi.
s0022-5347(17)50218-9. org/10.1159/000020027.
12. Ozen HA, Whitaker RH. Scope and limitations of the 17. Park JM, Faerber GJ, Bloom DA. Long-term outcome
MAGPI hypospadias repair. Br J Urol. 1987; https:// evaluation of patients undergoing the meatal advance-
doi.org/10.1111/j.1464-410X.1987.tb04586.x. ment and glanuloplasty procedure. J Urol. 1995;
13. Hastie KJ, Deshpande SS, Moisey CU. Long-term https://doi.org/10.1016/S0022-5347(01)67495-0.
follow-up of the MAGPI operation for distal hypospa-
The Meatal-Based Flap Principle
21
Ahmed T. Hadidi
Abbreviations In 1875, Wood presented the “meatal-based

flap” technique which was a prototype of
MAGPI Meatal advancement and glanulo- Mathieu’s procedure [3]. Ombrédanne [4, 5] used
plasty incorporated a perimeatal flap but fashioned the neourethra
MAVIS Mathieu and V incision sutured using a purse-string suture. The repair was too
SLAM Slit-like adjusted Mathieu baggy. Bevan, in 1917, designed a rectangular-
shaped meatal-based flap technique [6]. Mathieu
used a perimeatal flap and constructed the neo-
urethra using two lateral suture lines [7].
21.1 Introduction Mustardé used the same flap but differed in that
he used the perimeatal flap to form the whole
The principle of using a meatal-based flap dates neourethra, not just the ventral surface [9]. This
back even before Thiersch described his tech- bore the advantage of having a single-suture line
nique in 1869 for epispadias [1]. Bouisson is deep to the urethra. He also included a V incision
probably the first to use a meatal-based flap that at the glans to achieve a wider meatus. Barcat
extended down to the scrotum to reconstruct a modified the Mathieu technique by mobilizing
new urethra [2]. Since then, there has been a myr- the urethral plate and making a midline incision
iad of techniques described that used the meatal- to bury the neourethra deeper between the cor-
based principle from the proximal penile skin. pora and the glanular wings. The goal was always
Pioneers including Wood [3], Ombrédanne [4, 5], the advancement of the neourethra to the glans
and Bevan [6] and popularized by Mathieu in tip. Stenosis and fistula were frequently the price.
1932 [7]. Fevre [8], Mustardé [9], Barcat [10, Fevre used a longer meatal-based flap and folded
11], Boddy and Samuel [12], and Hadidi [13, 14] it between the glanular wings [8]. Body and
described techniques using the same principle. Samuel excised a V-shaped piece from the distal
end of the flap to avoid a “fish mouth” meatus
[12]. Hadidi included a Y incision at the tip of the
glans closed as a V to have a wide meatus and
A. T. Hadidi (*) avoid meatal stenosis [13]. He then, in 2012,
Department, Sana Klinikum Offenbach, Academic modified the glans incision in the glans and
Teaching Hospital of the Johann Wolfgang Goethe excised a V from the distal end of the flap to yield
University, Offenbach, Frankfurt, Germany a terminal-wide slit-like meatus.
378 A. T. Hadidi
21.2 The Mathieu Procedure 1931 (Fig. 21.1) [7, 15]. Since then, due to the
successful results achieved, the technique has
In 1928, Mathieu described a single-stage, been popularized by Gonzales et al. [16],
meatal-based flap technique to repair the distal Rabinowitz [15], Rabinowitz and Hulbert [17],
forms of hypospadias. The detail of this opera- Kim and Hendren [18], Wacksman [19], and
tion with its preliminary results was reported in Belman [20–22].
Fig. 21.1 Artist replication of

the original description of the a b
Mathieu technique in 1932 [7].
(a) A U-shaped incision is
made, extending into the tip of
the glans. (b) The parameatal
flap is sutured using
interrupted silk sutures on a
straight needle. (c) The ends of
the silk sutures were brought
out through the lumen of the
new urethra. (d) Notice that he
did not use a catheter and he
used silk sutures with a knot
inside the lumen of the urethra
and going out of the meatus
(©Ahmed T. Hadidi 2022. All
Rights Reserved)
c d
21 The Meatal-Based Flap Principle 379
21.3 The Mustardé Procedure single-suture line that was buried dorsally and
tunneled it through the glans [9]. To avoid
Mustardé described his technique in 1965 in a meatal stenosis, he created a triangular glanu-
provocative article called “One-stage correc- lar flap (Fig. 21.2). The most important prob-
tion of distal hypospadias and other people’s lems were (a) a higher incidence of meatal
fistulae,” where he applied the meatal-based stenosis and (b) technical problems related to
flap principle to reconstruct the whole new ure- the coverage of the large ventral skin defect [9,
thra from the meatal-based flap, thus having a 19, 22–26].
a b c d
Fig. 21.2 (a–e) Mustardé operation (1965) [9]: (a, b) Creation of neomeatus and buttonhole. (e) Transfer of
Preparation and dissection of meatal-based flap and trian- dorsal foreskin to cover the ventral defect
gular glanular flap. (c) Tubularization of neourethra. (d)
380 A. T. Hadidi
21.4 The Barcat Modification degloved, and meticulous chordee release

between the meatus and glans is performed. An
Barcat made an important modification of the erection test is done to make sure that there is no
Mathieu technique. After elevation of the meatal- residual chordee or glanular tilt. Anastomosis
based flap and urethroplasty, he mobilized the between the triangular glanular flap and ventral
neourethra and incised the glans in the midline to flap is completed (Fig. 21.3d). Lateral glanular
bury the neourethra deep into the glans. This wings are reapproximated, and coverage of the
helps to bring the meatus to the tip of the glans penile shaft is completed by ventrally rotated
and may result in a slit-like meatus. This Byars’ flaps (Fig. 21.3e, f). A silicone Foley
technique and its modifications were very popu- catheter is routinely used for urinary diversion
lar in the 1990s. (Baran and Çenetoğlu 1993, unpublished data)
[20, 24, 26–30].
The major advantages of the Horton-Devine
21.5 The Horton-Devine Flip-Flap technique can be stated as follows: (a) the risk
Procedure of meatal stenosis is decreased by the aid of a
triangular glanular flap, and (b) this was a sin-
In 1973, Horton and Devine presented a meatal- gle-stage operation with a reasonable compli-
based flap technique, a modification of Bevan’s cation rate.
and Mustardé’s operation. This single-stage pro- The disadvantages of the technique were (a)
cedure allowed release of chordee in the distal the characteristic snub-nosed appearance of the
shaft and helped to form a glanular meatus (Baran glans and (b) an ugly-looking meatus.
and Çenetoğlu 1993, unpublished data) [20, 22,
27]. The indications for the Horton-Devine tech-
nique are (a) distal hypospadias without chordee 21.6 Selection Criteria
or with moderate chordee; (b) a meatus that is for the Mathieu Technique
still localized on the distal third of the penile
shaft after the chordee release; and (c) well- The Mathieu technique is ideal for distal forms of
vascularized and reasonably thick skin proximal hypospadias. The ideal candidate for Mathieu’s
to the hypospadiac meatus. operation should have a straight penis or with
In this flip-flap procedure, the length of the superficial chordee that can be corrected by
penile flap should be equal to the distance degloving. A deep glanular groove is not
between the hypospadiac urethral meatus and necessary as the surgeon may compensate by
the tip of the penis (Fig. 21.3). After the precise raising a wide flap and hinging or (incising) the
measurement of this distance, a triangular urethral plate [31] or Barcat modification.
meatal-based flap is prepared from the penile Stenotic hypospadiac meatus can be corrected
skin proximal to the meatus (Fig. 21.3a). A tri- by meatotomy.
angular glanular flap is prepared from the mid- A preoperative testosterone injection is not
dle third of the glans (Fig. 21.3b). To prevent usually needed unless the glans is very small
meatal stenosis, a small dorsal meatotomy inci- (width < 11 mm). If there is an indication, testos-
sion is performed in the penile skin flap terone, 25 mg I.M. monthly, twice before the
(Fig. 21.3b, c). Lateral glanular flaps are pre- operation, enlarges the size and vascularity of the
pared with deep dissection in the plane of the penis and foreskin, thus enhancing the success of
corpus cavernosum (Fig. 21.3c). The penis is the operative procedure.
a b c
d e f
Fig. 21.3 (a–f) Horton-Devine operation (1973): (a) triangular glanular flap (d, e). Anastomosis between the
Preparation of triangular meatal-based flap. (b, c) Small glanular and the ventral flap; preparation of Byars’ flaps.
dorsal meatotomy to prevent meatal stenosis; creation of (f) Coverage of ventral defect
382 A. T. Hadidi
a b c d
Fig. 21.4 (a–d) Modified Mathieu technique (1932): (a) wings. (c) Creation of the neourethra by means of subcu-
Lines defining the urethral plate are drawn. (b) Incision ticular running sutures. (d) Prepared Byars’ flaps are
along the marks; preparation and mobilization of glanular sutured in the midline
21.7 The Mathieu Technique r eoperation rate (as much as 18–20%) is reported
and Relevant Modifications [32, 33]. However, there was no incidence of ure-
and Controversies (Fig. 21.4) throcutaneous fistula in the series of Retik et al.
where a dorsal subcutaneous dartos wrap was
The original Mathieu technique was modified by used to cover the neourethra [37].
several authors through the years [20, 26, 28, 29], Wheeler et al. suggested the Mathieu opera-
where the neo-urethra is sutured using continu- tion without a catheter [36]. Although the patients
ous subcuticular sutures. The incision along the could be discharged the same day, the complica-
urethral plate is made deep to create wide glanu- tion rate was around 15%. Gough et al. in 1995
lar wings that are su-tured together in the mid- presented a prospective randomized trial of the
line. Coverage of the penile shaft is completed by Mathieu operation [38]. Their complication rate
ven-trally rotated Byars flaps. The main draw- with 2–5-day urinary diversion and Silastic foam
back of the original Mathieu is the “fish mouth” dressing was less than 5%. They do not recom-
appearance of the meatus (Fig. 21.4). mend a catheterless technique due to the higher
incidence of immediate dysuria and an apparent
increase in late complications.
21.7.1 Stenting In 1996, Hakim et al. presented the results of
four different centers (n = 300 patients) [39].
A modification of the Mathieu repair-eliminating They reported an extremely low complication
stenting or catheterization, described by rate, with no statistically significant difference
Rabinowitz [15], makes the technique more con- between catheterized and non-catheterized
venient for outpatient surgery. Since then Aktug patients. In the author’s experience, a 3–5-day
et al. [32], Buson et al. [33] and Retik et al. [34], catheterization is the policy of choice, at least for
Borer [35], and Wheeler et al. [36] have described the prevention of immediate dysuria syndrome.
a modification of the Mathieu repair using a sub- We think Silastic foam dressing plus a double-
cuticular suture for urethroplasty. No catheter, diaper-free-dripping Silastic catheter method
stent, or suprapubic drainage is used in these does not create much trouble for the family and
patients. A relatively high complication or for children who are not toilet-trained.
21.7.2 Reinforcement 21.7.4 Chordee

of the Neourethra Using Local
Tissues Some surgeons prefer to use dorsal plication
techniques (Nesbit or Baskin type) for glanular
Belman, in 1988, presented his “de-epithelialized tilt and moderate chordee [43, 46]. De Grazia
skin flap coverage” procedure in 84 hypospadias et al. mentioned a 12.5% incidence of distal cur-
patients whose treatment included Mathieu ure- vature and advised the mobilization of the ure-
throplasty [21]. He applied a flap of de- thral plate and extensive removal of ventral
epithelialized preputial skin over the neourethra chordee elements beneath the plate [47]. We
and reported a 3.5% rate of fistula formation. In strongly believe that the hypoplastic atretic tis-
1994, Retik et al. reported the results of a “dorsal sues should be excised and the dorsal plication
dartos subcutaneous flap” to wrap the neourethra procedure should only be applied to a limited
after a Mathieu repair [34]. There was no evi- group of Mathieu cases with less than 20 degrees
dence of urethrocutaneous fistula among 204 of curvature.
patients. Churchill et al. presented excellent
results with a dartos flap in reoperations [40].
Ravasse et al. used a foreskin subcutaneous con- 21.7.5 Hinging of Urethral Plate
nective tissue pedicled flap to reduce the rate of
fistula formation, which for them was 6% [37, Rich and Keating designed “hinging of the ure-
41]. Snow et al. reported a 0% complication rate thral plate” to lower the glanular groove and cre-
in their patients, who were treated with micro- ate a vertical urethral meatus [31]. This
scopic magnification plus a “tunica vaginalis modification enhances the enlargement of a nar-
blanket wrap” technique [42]. Today, many pedi- row glanular groove and thus may change the
atric urologists and pediatric surgeons prefer to spectrum of indications for the Mathieu
use subcutaneous tissue which is attached to the procedure.
flip-flap to cover the neourethra in the Mathieu
technique [20, 21, 37, 41, 43, 44]. If there is not
enough tissue attached to the flap, wrapping the 21.7.6 Mathieu as a Rescue
neourethra with the vascularized dartos tissue Operation
from the inner surface of the preputial foreskin
can prevent fistula formation. There is a myth that if a child had a compli-
cated Mathieu technique, the surgeon cannot
perform a redo Mathieu due to fibrosis and
21.7.3 How to Improve lack of healthy tissues. This is untrue, and a
the Appearance of the Meatus redo Mathieu is a very reliable salvage proce-
in Mathieu Cases dure with very good results. Hayashi et al.
[48], Ravasse et al. [37], and Simmons et al.
In 2000, Boddy and Samuel reported the V [49] reported their experience with the
incision-sutured MAVIS technique to provide a Mathieu operation in secondary cases. Their
cosmetically acceptable natural slit-like meatus in data suggest a success rate exceeding 85%.
Mathieu cases [12]. The MAVIS technique has The author has performed 24 of his 143
been very popular since 1999 [45] as one of the Mathieu operations in secondary, complicated
important steps in the Mathieu operation. This has cases. Mathieu has been used as a rescue oper-
helped to reduce meatal stenosis and the horizon- ation after the complications of MAGPI and
tal bucket-handle meatus problem. All patients distal problems in transverse island flap
have a slit-like meatus with the proper caliber. techniques.
384 A. T. Hadidi
21.7.7 Results and Complications 70–80% of patients with hypospadias. The only
contraindication is the presence of severe chor-
Fistula formation, meatal stenosis, stricture, dee distal to the hypospadiac meatus (a flat-look-
meatal retraction, and the dehiscence of glans ing glans is not a contraindication). In the
sutures are the most frequent complications majority of cases of distal hypospadias, chordee,
of the Mathieu operation [26, 38, 50, 51]. if present, is usually a cutaneous form that is
During the past decade, the improvements almost always released by simply freeing the
seen in suture materials, magnification and proximal skin adequately.
suture techniques, and the application of vas- The Y-V technique has been used successfully
cularized flaps around the neourethra have in recurrent cases of distal penile hypospadias,
reduced the incidence of these complications. provided there is a good quality of skin proximal
A hair-bearing urethra may be the most dis- to the meatus for flap reconstruction (i.e., the
tressing complication of the Mathieu opera- potential flap skin should not be scarred or
tion. Longer flaps are not only at risk for fibrosed). The details of the technique are
de-vascularization with complications of dis- described in the following section.
tal stenosis and stricture but also may incor-
porate hair-bearing skin [20, 22]. The risk of
development of a urethral beard in adoles- 21.8.2 Operative Technique
cence is very high in these patients. “Half-
moon” or “bucket-h andle” meatus is not a The author prefers to use caudal block to avoid
real complication but an important and dis- any change in tissue planes. The patient may be
tressing aesthetic problem [12, 52]. We brought close to the edge of the table, and the
believe that the MAVIS “V excision” modifi- surgeon sits; alternatively, the patient lies in the
cation is a better alternative. traditional middle-of-table position if the surgeon
Murphy reviewed the literature in 1999 and prefers to stand.
investigated the complication rates in a series of Step 1: A 5–0 nylon traction suture is placed on
792 Mathieu operations [53]. There were only the glans. It should be central, transversely applied,
16 complications (9 fistulae and 7 meatal steno- and just dorsal to the site of the “neomeatus.”
ses – a 2% incidence). We have collected a Tourniquet at the base of the penis was used early
group of Mathieu series involving 1060 cases. for hemostasis. Since 2010, I have not used a tour-
The incidence of complications varied between niquet in order to be able to control bleeding prop-
1.5% and 11.2% (Table 21.1) [6, 12, 16, 37, 38, erly and reduce postoperative edema.
41, 50, 54, 55].
Table 21.1 The complication rate in the Mathieu
operation
21.8 The Y-V Glanuloplasty References Number of Complication
Modified “Mathieu” patients rate
Technique [13, 56] (n = 1060)
Boddy and Samuel [12] 64 3%
De Jong and Boemers [43] 116 3.4%
21.8.1 Selection of Patients Dolatzas et al. [54] 180 11.2%
Elder et al. [50] 56 1.8%
The Y-V glanuloplasty modified Mathieu tech- Gonzales et al. [16] 63 8%
nique may be applied to most forms of distal Gough et al. [38] 95 4.7%
hypospadias. By distal we mean all types of Minevich et al. [55] 202 1.5%
hypospadias where the meatus is just distal to Oswald et al. [44] 30 10%
the midpoint of the shaft. The reason is to avoid Ravasse et al. [37, 41] 50 6%
hair-
bearing skin. This will include about Retik et al. [34] 204 0%
Step 2: The erection test is applied and any flap skin edge. This has two advantages: mini-
chordee present is evaluated. mizing flap tissue injury and increasing the
amount of subcutaneous tissue and fascia for a
21.8.2.1 Y-V Glanuloplasty multilayer closure. (e) When the flap is ele-
Step 3: A Y-shaped incision is outlined on the vated, there will be two small dog-ears on either
glans. The center of the Y is exactly at the tip of end of the original meatus. This is better excised
the glans and where the tip of the neomeatus will as it is a common site for fistula formation
be located. Each limb of the Y is 0.5 cm long, and (Fig. 21.5g).
the angle between the upper two limbs of the Y is
60° (Fig. 21.5a). The Y-shaped incision is made 21.8.2.3 Neourethra Reconstruction
deep and results in three flaps, one upper (median) Step 8: I prefer to use a continuous subcuticular
and two lateral (Fig. 21.5b). running Vicryl 6–0 suture on a cutting or taper
Step 4: The three flaps are elevated, and a core needle. I start a few millimeters proximal to the
of soft tissue is excised from the bed of each flap original meatus. This helps to have the knot away
to create a space for the neourethra. from the neourethra. The subcuticular suture is
Step 5: The Y shape is sutured as a V using continued until I reach the tip of the glans, then I
continuous through-and-through Vicryl 6–0 or go back with the same stitch doing a running
similar suture (Fig. 21.5c, d). This means that the stitch approximating the flap fascia to the depth
upper flap is sutured to the lateral limbs, making of the glans and the shaft of the penis (double
sure to keep the “dog-ears” at the upper ends of breasting). Thus, one will have only one knot for
the V suture lines. These dog-ears are important the whole two layers (Fig. 21.5i).
because, when opened, they will enlarge the cir-
cumference of the tip of the glanular wings by 21.8.2.4 Meatoplasty
1 cm at least (Fig. 21.5d). and Glanuloplasty
Step 6: The hypospadiac meatus is evaluated. Step 10: A small V is excised from the apex of the
If the meatus is narrow, it should be dilated to parameatal flap, and the meatus is reconstructed
accommodate a size F10 catheter. If the skin with fine simple Vicryl 6–0 sutures (Fig. 21.5h).
proximal to the meatus is thin, transparent, it This helps to achieve a slit-like meatus. The glanu-
should be excised until we reach a normal healthy lar wings are closed using Vicryl 6–0 transverse
skin. A size 10F catheter is inserted into the mattress-interrupted sutures. The remaining
meatus down to the root of the penis. wound is closed using continuous mattress or
interrupted subcuticular Vicryl stitches (Fig. 21.5j).
21.8.2.2 Parameatal Flap Design
Step 7: The U-shaped incision is made taking 21.8.2.5 Urinary Diversion
the following points into consideration: (a) The Step 11: There is no need to leave a transurethral
length of the flap is slightly longer than the dis- catheter for more than 1 or 2 days.
tance between the meatus and the designed tip
of the neomeatus. (b) When designing the two 21.8.2.6 Dressing
longitudinal incisions, they have to diverge Step 12: A local gentamycin eye ointment is
away from the hypospadiac meatus to allow for applied. A compression dressing is applied for
adequate blood supply to the flap. (c) When the 6 h for hemostasis and then removed. The opera-
U-shaped incision reaches the tip of the glans, tion is performed as an outpatient procedure, and
it will open the dog-ears wide as one opens a the patient is discharged home on the same day
book. (d) When mobilizing the flap, one should on local gentamycin eye ointment and oral
be holding the opposite skin edge and not the sulfamethoxazole-trimethoprim.
386 A. T. Hadidi
a b c
d e f
Fig. 21.5 (a–j) Steps of Y-V glanuloplasty modified serve its fascia. (g) The dog-ear is excised from both lat-
Mathieu technique: (a) Y incision. (b) Elevation of the eral ends of the flap. (h) Urethroplasty is performed in two
three flaps and coring to make a space for the neourethra. layers. (i) A V is excised from the tip of the flap. (j)
(c, d) Y sutured as V with preservation of dog-ears. (e) Meatoplasty and glanuloplasty
U-shaped flap. (f) The flap is elevated, taking care to pre-
g h i

388 A. T. Hadidi
21.8.3 Results and Conclusion
Between January 1992 and July 2002, 384 Y-V

glanuloplasty modified Mathieu procedures were
performed. Early in the study, three patients
developed meatal stenosis that responded to dila-
tation. There were five fistulas, one wound disrup-
tion for severe infection, and one patient came
back 5 days later with secondary bleeding due to
infection (complication rate 2%). There was no
need to leave a catheter inside the urethra for more
than 2 days and no need for calibration or dilata-
tion. The Mathieu procedure combined with Y-V
glanuloplasty is an excellent technique for distal
hypospadias that has stood the test of time.
21.9 The Inverted Y-V

Glanuloplasty Modified Fig. 21.6 Slit-like meatus after inverted Y-V modified
“Mathieu” Technique Mathieu
The Y-V modified Mathieu is an excellent tech-

References
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a ring suture line. The main drawback is that the 1. Thiersch K. Ueber die Enstehungsweise und opera-
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org/10.1097/00005392-199502000-00061. out chordee. J Urol. 1987; https://doi.org/10.1016/
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ized incised-plate urethroplasty (Snodgrass) in pri- 53. Murphy JP. Hypospadias. In: Ashcraft KW, Murphy
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Philadelphia: Saunders; 1994. 55. Minevich E, Pecha BR, Wacksman J, Sheldon
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Surg. 1998;8:98–9. 56. Hadidi AT. The Y-V Glanuloplasty modified
48. Hayashi Y, Sasaki S, Kojima Y, Maruyama T, Tozawa “Mathieu” technique. Hypospadias Surg. 2004;
K, Mizuno K, Kohri K. Primary and salvage urethro- https://doi.org/10.1007/978-3-662-07841-9_19.
The Slit-Like Adjusted Mathieu
(SLAM) Technique 22
Ahmed T. Hadidi
Abbreviations (1992–2002) [4, 5] and inverted Y-V modified

Mathieu (2003–2004) to the slit-like adjusted
MAVIS Mathieu and V incision sutured Mathieu (SLAM) [6] technique, since 2005. The
SLAM Slit-like adjusted Mathieu aim is to try to find the optimum operation that
TIP Tubularized incised plate persistently produces good functional as well as
cosmetic outcome (slit-like meatus) of the penis
with low complication rate.
The SLAM technique is the commonest oper-
22.1 Introduction ation performed in the Hypospadias Center,
Frankfurt, Germany. It is a robust, reliable opera-
The Mathieu [1] technique has withstood the test tion with satisfactory results and constitutes
of time for almost 100 years with a study that about 70% of all hypospadias operations per-
reported a success rate of 100% in 204 consecu- formed either for primary or complicated distal
tive patients [2]. The major drawback of the orig- and mid-penile hypospadias.
inal Mathieu technique is the final appearance of
the meatus (a fish-mouth meatus that is not at the
tip of the glans). The technique has become less 22.2 Selection of Patients
popular during the past 15 years in favor of the
tubularized incised plate (TIP) technique partly The SLAM technique is suitable for all patients
because of the slit-like meatus that could be with hypospadias where the meatus lies distal to
achieved with the TIP technique [3]. the mid-shaft as long as they do not have severe
The author has been performing a modifica- deep chordee (>30 degrees) that requires division
tion of the Mathieu technique since 1986. There of the urethral plate to correct chordee. The reason
has been continuous evolution and modifications is to avoid the hair-bearing scrotal skin. Patients
through the years from Y-V modified Mathieu with proximal hypospadias without severe chor-
dee require a different surgical approach that is
A. T. Hadidi (*) discussed in detail in Chap. 30. Management of
Hypospadias Center and Pediatric Surgery patients with proximal hypospadias and severe
chordee that requires division of the urethral plate
University, Offenbach, Frankfurt, Germany is mentioned in Chaps. 32 and 33.
392 A. T. Hadidi
22.3 Operative Technique The limits of the final meatus are marked with
a pen. A catheter with the appropriate size (size
Under general anesthesia and caudal block, a F12 or larger, according to the caliber of the
traction suture of 4/0 Nylon is placed through the proximal urethra) is introduced into the bladder.
tip of the glans. An erection test is performed to Flap design: The boundaries of the urethral
ensure that the patient does not have severe deep plate are outlined. In patients with globular flat
chordee. glans, special attention was given to create large
Careful assessment of the penis is performed; glanular wings, and the urethral plate could be
if the native urethral meatus is narrow, it is incised outlined as narrow as 3 mm in width (Fig. 22.1a).
proximally to create a wide spatulated meatus At the distal end of the urethral plate, the two
that accommodates catheter size F12. incisions converge toward each other to allow
The approach for patients with thin paper-like free mobility of the glanular wings to wrap
proximal urethra in the Hypospadias Center has around the neo-urethra to produce a slit-like
changed over the years. Until 2010, if the urethra meatus free of sutures (Fig. 22.1a).
proximal to the meatus was thin and paper like, it Incision of the flap begins at the coronal sul-
was incised to reach a healthy urethra covered cus using a sharp scalpel or scissors and contin-
with corpus spongiosum. Since 2010, if the prox- ues distally very deep into the glans in such a way
imal urethra is very thin, we do not incise the thin to create large glanular wings to cover the neo-
paper-like urethra; instead, we design the flap to urethra without tension. Proximally, the flap is
cover the thin paper-like urethra as well as the elevated using sharp scissors taking care to
deficient segment of the urethra. This makes the include dartos fascia and part of the corpus spon-
operation easier and reduces the duration of uri- giosum with the flap (Fig. 22.1b). The redundant
nary diversion. epithelial dog ear is removed from the angle of
One major advantage of the SLAM technique the flap to reduce the chances of fistula formation
is that the width or depth of the urethral plate do at this common fistula site (Fig. 22.1c).
not influence the patient selection. If the urethral Urethroplasty: The flap is sutured to the tip of
plate is narrow, this is compensated for by a the glans, 2 mm from the distal end of the inci-
wider flap. If the glans is flat, one needs to mobi- sion in the glans (Fig. 22.1d). The idea is to keep
lize the glanular wings deeper. the meatal edges free of any sutures and to have a
smooth, slit-like meatus. A continuous subcuticu-
lar running polyglactin 6/0 on a cutting needle is
the standard suture for urethroplasty. The subcu-
It is more important to have larger glanular ticular suture is continued until the distal stitch is
wings than a wide urethral plate. reached, and then the surgeon returns back with
the same suture as a running suture approximating
Fig. 22.1 The SLAM technique: (a) The SLAM tech- excised, maintaining the fascia. (d) Urethroplasty: The
nique design: A U-shaped incision is outlined. The two edges of the flap are fixed to the converging edges of the
parallel incisions at the glans region start along the true urethral plate 2–3 mm from the distal end of the incision.
mucosal urethral plate to have large, wide glanular Urethroplasty is carried out using 6/0 Vicryl on a sharp
wings. At the distal end, the two incisions converge as needle in a continuous subcuticular fashion starting
shown to have a slit-like meatus and to avoid having 3 mm proximal to the angle of the flap. A sealing second
sutures at the meatus. The two lateral incisions diverge suture line in a continuous fashion is carried out. (e) V
near the meatus to produce a wide flap. (b) Flap mobili- excision: A triangle is removed from the tip of the flap.
zation: Using a sharp scissors, the incision is deepened (f, g) This helps to have a slit-like meatus. (h) Glans and
starting near the coronal sulcus; the lateral skin edge skin closure: The glanular wings are mobilized, rotated
(not the flap) is held with fine-toothed forceps and fascia distally, and approximated around the new urethra and
and corpus spongiosum are included with the flap as catheter F12, and the penile skin is closed. Notice that
much as possible. (c) Angle epithelium excision: The the new meatus has one stitch only at 6 o’clock.
epithelium at the proximal two angles of the flap is (©Ahmed T. Hadidi 2022. All Rights Reserved)
22 The Slit-Like Adjusted Mathieu (SLAM) Technique 393
a b c
d e f
g h
394 A. T. Hadidi
the flap fascia to the depth of the glans and the Results are considered satisfactory when the
shaft of the penis (double breasting). Thus, there boy achieves an apical slit-like meatus at the tip
will be a single knot for the whole two layers of the glans penis, single forward stream, unim-
(Fig. 22.1e). The author goes back again with the peded voiding, good cosmesis, and no need for
continuous suturing as a third protective layer secondary surgery for the urethra. Uroflowmetry
approximating the wall of the neo-urethra to the was performed in toilet-trained children. Peak
glanular wings. In this way, we have two protec- flow within 2 standard deviations was consid-
tive layers on top of the urethroplasty. ered normal. Parent feedback was considered
Meatoplasty and glanuloplasty: A small V is adequate in younger children.
excised from the apex of the parameatal flap Early in the study, the preputial dartos fascia
(Fig. 22.1e), and the two edges of the final meatus was used to provide a second protective interme-
are sutured together to the center of the V creating a diate layer for the neo-urethra. Since 2007, this
slit-like meatus using polyglactin 6/0 single stitch was used only when the parameatal flap was thin
(Fig. 22.2e). No other sutures are needed in the and the corpus spongiosum was hypoplastic and
meatus. If the glans is small, release incisions are underdeveloped. The remaining skin of the penis
made from the inside of the glanular wings to make and foreskin was fashioned to provide ventral
it larger and release any tension (insight Fig. 22.1e). skin coverage.
The glanular wings are closed using polyglactin 6/0
transverse mattress-interrupted sutures. The remain-
ing wound is closed using continuous mattress Important Technical Tips
polyglactin 6/0 stitches (Fig. 22.1h). • The caliber of the neo-urethra is designed
Urinary diversion: A transurethral catheter is to be the same size or slightly larger than
left inside the bladder for 1–2 days depending on the normal proximal urethra. The standard
the degree of swelling, the location of the original protocol is to use a catheter size F12 or
meatus, and the age of the patient. If the child has a larger. The aim is to have a wide neo-ure-
mid-penile hypospadias, the author prefers to leave thra and meatus that is less liable to con-
a silicone catheter for 7 days because patients with tract with healing and cause meatal
mid-penile hypospadias develop severe edema after stenosis. This is suitable for most children
surgery that may affect urine drainage. with hypospadias. In six patients included
Dressing: All patients have a standard dress- in the study, the proximal “normal ure-
ing in the form of gentamicin local eye ointment, thra” could only take size F8, and in those
gauze and adhesive tape that compresses the patients, a catheter size 8 was used.
penis, gauze and catheter against the lower • Occasionally the hypospadias meatus is
abdominal wall and allows the free mobility of narrower than the normal proximal ure-
the patient [7]. The dressing is removed at the thra due to the presence of a rather cir-
time of removal of the transurethral catheter, and cular fibrous ring and should be incised
the penis is left exposed. If the dressing becomes to avoid stenosis at the proximal end of
wet with urine or blood, the dressing is changed the neo-urethra as shown in Fig. 22.2b.
using the same standard dressing or removed • Care must be taken to elevate the buck’s
completely. Broad-spectrum antibiotics (second- fascia and part of the corpus spongiosum
generation cephalosporin) are used for 1 week. with the flap as they constitute the main
Paracetamol and ibuprofen suppositories and blood supply to the flap. In patients with
syrup are routinely used for pain relief. proximal bifurcation of the corpus spon-
Our standard postoperative follow-up proto- giosum, part of the corpus spongiosum
col includes examination after 3 months, 1 year, was elevated with the proximal flap. In
3 years, and then every 5 years until the child is patients with hypoplastic or absent corpus
15 years old. Patients living long distances away spongiosum, the author used preputial
or fail to attend for follow-up were contacted by dartos fascia or proximal penile dartos fas-
e-mail and phone. Follow-up period now ranges cia as a protective intermediate layer.
from 22 months to 15 years (mean 8 years).
• When mobilizing the flap, one should be knot (a potential cause of infection and
holding the opposite skin edge and not the fistula) proximal and away from the
flap skin edge. This has two advantages: neo-urethra (Fig. 22.1d).
minimizing flap tissue injury and increas- • As suturing is performed in a continu-
ing the amount of subcutaneous tissue and ous subcuticular manner, a cutting nee-
fascia for a multilayer closure (Fig. 22.1b). dle is easier to go through the dermis as
• The first step in the urethroplasty is to is the case with the classic subcuticular
suture the distal end of the flap to the closure of skin incisions.
end of the glans 2 mm from the tip • The SLAM technique was used success-
(Fig. 22.1d). This helps to have a slit- fully in 130 patients with mid-penile
like meatus and one stitch only at the hypospadias. It is essential to avoid the
meatus at 6 o’clock position. scrotal hair-bearing skin. This was
• The suturing of urethroplasty should achieved by designing the flap with a
start a few millimeters proximal to the curve from the midline to avoid scrotal
original meatus. This helps to have the skin (Fig. 22.3).
a b c
d e f
Fig. 22.2 Operative steps of the SLAM technique: (a) structed around a catheter size F10, and “double-breasted”
Distal hypospadias: A 9-month-old child with distal hypo- urethroplasty was performed. Notice the well-vascularized
spadias and incomplete cleft glans. (b) SLAM technique thick flap with maintained fascia and corpus spongiosum.
design: The SLAM incision is outlined. Notice the con- (e) Glans and skin closure: After closure of the glanular
verging lines distally and the wider flap proximal to the wings and penile skin. Notice that there is only a single knot
meatus. The stenotic meatus was incised to cut a fibrotic at the new meatus (at 6 o’clock). The transurethral catheter
ring to accommodate catheter F10. (c) Flap mobilization: was kept for 2 days. (f) Final appearance: The final shape
After deep incision of the flap, notice the large mobile glan- of the meatus and prepuce 1 year after surgery (©Ahmed
ular wings. (d) Urethroplasty: The new urethral was recon- T. Hadidi 2022. All Rights Reserved)
396 A. T. Hadidi
Fig. 22.3 Operative steps of the SLAM technique in achieve this, the flap is designed in an oblique manner
mid-penile hypospadias: It is important to avoid using the (©Ahmed T. Hadidi 2022. All Rights Reserved)
scrotal hair-bearing skin to reconstruct the neo-urethra. To
22.4 Patients and Methods Table 22.1 Details of 872 patients who underwent the
SLAM technique between January 2005 and December
2009
The SLAM technique was performed on 2679
Location of the meatus Number of patients
patients with distal hypospadias and mid-penile
Coronal hypospadias 289
in the period between January 2005 and
Distal penile hypospadias 508
December 2019 after obtaining the approval of Mid-penile hypospadias 34
the institutional ethics committee. Six hundred Megameatus intact prepuce (MIP) 41
fifty-three patients were lost for follow-up, and Total 872
the remaining 2026 patients constitute the pres-
ent report. The age of patients ranged between
Table 22.2 The details of complications following the
4 months and 30 years (mean 1.5 years). SLAM technique
For the purpose of the study, distal hypospa-
Number of
dias includes all types of hypospadias where the Complications patients
meatus lies distal to the mid-shaft without severe Fistula detected within 2 months 31
chordee >30 degrees. The reason is to avoid the Fistula detected between 2 and 6 20
hair-bearing scrotal skin. The morphology of the months
hypospadiac penis and associated anomalies are Small fistula detected after 6 28
months
summarized in Table 22.1. Patients with severe
Wound disruption 19
deep chordee distal to the hypospadias meatus or Meatal stenosis 30
with complicated distal hypospadias were not Total complications 128 (5%)
included in the present study.
22.6 Discussion
22.5 Results and Complications
In a questionnaire submitted to 35 experienced
Satisfactory results were achieved in 1898 hypospadias surgeons as why they shifted away
patients (95%). Seventy-nine patients developed from the Mathieu technique, the answer included
fistulae. Of those 79 fistulae, 31 were recog- because of fish-mouth meatus that is not at the tip
nized within the first 2 months after surgery, 20 of the glans, high incidence of fistula, or diffi-
developed between 2 and 6 months, and 28 culty to close the glans around the neo-urethra.
small fistulae developed after 2 years. The fis- Several modifications were reported to deal
tula was closed routinely after 6 months, and a with the “fish-mouth meatus”:
second intermediate layer was always used to Barcat in 1969 described the balanic groove
protect fistula closure. Thirty patients developed technique [8]. In addition to the classic Mathieu
meatal stenosis between 2 and 6 months after flap, he made a midline incision into the glans
surgery. Dilatation under anesthesia and and buried the neo-urethra deep into the glans
meatoglanuloplasty was performed in those 30 trench resulting in a slit-like meatus. He reported
patients. Nineteen patients had wound dehis- a complication rate of 20% in 267 patients. The
cence that was detected 2–3 weeks after surgery technique was very popular in the 1980s and
(was associated with severe edema and infec- 1990s with very good results.
tion). These 19 patients underwent redo SLAM Rich et al. in 1989 described hinging of the
after 6–8 months using a wider catheter for urethral plate in combination with the Mathieu
7 days and with 2 intermediate layers flap [9]. This was modified later by Snodgrass as
(Table 22.2). Eight hundred and twenty patients the tubularized incised plate technique [10]. The
maintained long-term follow-up with satisfac- hinging technique was revived again in 2008 by
tory results (Fig. 22.4). Aminsharifi et al. [11].
398 A. T. Hadidi
a b c
Fig. 22.4 Ten-year follow-up after SLAM and (a, b) circumcision and (c) foreskin reconstruction (©Ahmed T. Hadidi
Hadidi in 1996 described the Y-V glanulo- Snodgrass [10]. The technique has become
plasty modified Mathieu. This technique has increasingly popular in recent years partly
helped to use the Mathieu operation in all forms because of its good cosmetic outcome which
of hypospadiac glans in distal hypospadias and can be achieved [3].
gives a wide terminal meatus at the tip of the In the 1990s, a complication rate of less than
glans [4]. He reported a complication rate of 2% 5% was to be expected in distal hypospadias [15].
in 384 patients using this technique between Some studies suggested a complication rate of
1992 and 2002 [5]. However, although the meatus the TIP technique less than 10% is to be expected
was terminal, it was not slit-like. [16]. However, some long-term follow-up studies
Boddy and Samuel described the MAVIS mod- of the TIP technique reported a complication rate
ification of Mathieu technique in 2000 with 3% reaching 35% in distal hypospadias and 66% in
complication rate [12]. In this technique a trian- proximal hypospadias [17].
gle is excised from the apex of the parameatal Holland and Smith showed that natural ure-
flap. This helps to improve the appearance of the thral plate configuration plays an important role
meatus. in the higher complication rate reported with the
Tekant et al. applied the MAVIS modification TIP technique. Narrow and flat urethral plates are
to 32 patients [13]. They reported a slit-like more susceptible to stenosis, despite incision and
meatus and no fistula or stenosis in all the 32 tubularization [18]. Sarhan et al., in a prospective
patients. randomized study on 80 boys, concluded that a
The SLAM technique was associated with urethral plate of 8 mm or more was essential for
long-term follow-up complication rate of 5%. a successful TIP repair [19]. Others suggested
Most complications occurred within the first that the size of the glans rather than the different
6 months after surgery, but some developed com- glans configuration (cleft glans, incomplete cleft,
plications after several years. The SLAM tech- or flat glans) plays an important role in the inci-
nique was performed on 107 patients older than dence of complications [20]. On the other hand,
18 years of age. None of the 107 patients reported these mechanisms are not involved in the Mathieu
problems associated with hair growth inside the procedure. In fact, neo-urethra reconstruction
urethra. Longer follow-up period continuing after with the Mathieu procedure is independent of
puberty is being carried out. urethral plate characteristics or glans configura-
Incision of the urethral plate was first tion [21].
described by Reddy in 1975 [14], Orkiszewski In a prospective randomized study, Aminsharifi
[15], and Rich et al. [9] and popularized by et al. compared the standard TIP technique and
Mathieu technique combined with incision of the 7. Hadidi AT. Double Y glanuloplasty for glanu-
urethral plate (a technique similar to that lar hypospadias. J Pediatr Surg. 2010; https://doi.
org/10.1016/j.jpedsurg.2009.11.019.
described by Rich et al. [9]) [11]. There was a 8. Barcat J. Symposium sur l’hypospadias. 16th meeting
statistical difference between 25% complication of the French Society of Children’s Surgery. Ann Chir
rate with the TIP technique and no complications Infant. 1969;10(287)
with the Mathieu combined with urethral plate 9. Rich MA, Keating MA, Snyder McC H, Duckett
JW. Hinging the urethral plate in hypospadias
incision. meatoplasty. J Urol. 1989; https://doi.org/10.1016/
The SLAM technique was used in 161 patients S0022-5347(17)39161-9.
with megameatus intact prepuce (MIP), and a 10. Snodgrass W. Tubularized, incised plate urethroplasty
second layer from the ventral preputial fascia was for distal hypospadias. J Urol. 1994; https://doi.
org/10.1016/S0022-5347(17)34991-1.
employed. This resulted in a better cosmetic 11. Aminsharifi A, Taddayun A, Assadolahpoor A, Khezri
result and no meatal stenosis nor wound disrup- A. Combined use of mathieu procedure with plate
tion in this series as compared with the pyramid incision for hypospadias repair: a randomized clini-
repair [22]. cal trial. Urology. 2008; https://doi.org/10.1016/j.
urology.2008.02.034.
There is a misconception that the Mathieu flap 12. Boddy SA, Samuel M. A natural glanu-
has a poor blood supply coming from a narrow lar meatus after “Mathieu and a V incision
bridge at the base of the flap and that the Mathieu sutured”: MAVIS. BJU Int. 2000; https://doi.
flap is a graft rather than a flap. This is not the org/10.1046/j.1464-410X.2000.00758.x.
13. Tekant G, Beşik C, Emir H, Büyükünal SNC. Is it
case if the parameatal flap is elevated carefully possible to create a slit-like meatus without incising
with the underlying fascia and the highly vascu- the urethral plate? Pediatr Surg Int. 2002; https://doi.
lar corpus spongiosum. In patients with hypo- org/10.1007/s00383-002-0773-8.
plastic corpus spongiosum, the flap should be 14. Reddy LN. One-stage repair of hypospadias. Urology.
1975; https://doi.org/10.1016/0090-4295(75)90070-9.
augmented with an intermediate vascular layer 15. Orkiszewski M. Urethral reconstruction in skin defi-
from the preputial or penile dartos fascia. Retik cit. Urol Pol/Polish Urol. 1987;40:12–5.
et al. reported a 100% success rate in 204 con- 16. Braga LHP, Lorenzo AJ, Salle JLP. Tubularized
secutive patients with the routine use of a protec- incised plate urethroplasty for distal hypospadias:
a literature review. Indian J Urol. 2008; https://doi.
tive intermediate layer [2]. org/10.4103/0970-1591.40619.
17. Chrzan R, Dik P, Klijn AJ, de Jong TPVM. Quality
assessment of hypospadias repair with emphasis on
References techniques used and experience of pediatric urologic
surgeons. Urology. 2007; https://doi.org/10.1016/j.
urology.2007.01.103.
1. Mathieu P. Traitement en un temps de l’hypospade 18. Holland AJA, Smith GHH. Effect of the depth and
balanique et juxta-balanique. J Chir. 1932;39:481. width of the urethral plate on tubularized incised plate
2. Retik AB, Mandell J, Bauer SB, Atala A. Meatal urethroplasty. J Urol. 2000; https://doi.org/10.1016/
based hypospadias repair with the use of a dor- S0022-5347(05)67408-3.
sal subcutaneous flap to prevent urethrocutane- 19. Sarhan O, Saad M, Helmy T, Hafez A. Effect of sutur-
ous fistula. J Urol. 1994; https://doi.org/10.1016/ ing technique and urethral plate characteristics on
S0022-5347(17)32555-7. complication rate following hypospadias repair: a pro-
3. Cook A, Khoury AE, Neville C, Bagli DJ, Farhat spective randomized study. Randomized Controlled
WA, Pippi Salle JL. A multicenter evaluation of Trial. 2009;182:682–6.
technical preferences for primary hypospadias 20. Snodgrass W, MacEdo A, Hoebeke P, Mouriquand
repair. J Urol. 2005; https://doi.org/10.1097/01. PDE. Hypospadias dilemmas: a round table. J
ju.0000180643.01803.43. Pediatr Urol. 2011; https://doi.org/10.1016/j.
4. Hadidi AT. Y-V Glanuloplasty a new modification jpurol.2010.11.009.
in the surgery of hypospadias. Kasr El Aini Med J. 21. Imamoǧlu MA, Bakirtaş H. Comparison of two meth-
1996;2:223–33. ods—Mathieu and Snodgrass—in hypospadias repair.
5. Hadidi AT. The Y-V glanuloplasty modified “Mathieu” Urol Int. 2003; https://doi.org/10.1159/000072674.
technique. Hypospadias Surg. 2004; https://doi. 22. Duckett JW, Keating MA. Technical challenge of
org/10.1007/978-3-662-07841-9_19. the megameatus intact prepuce hypospadias vari-
6. Hadidi AT. The slit-like adjusted Mathieu technique ant: the pyramid procedure. J Urol. 1989; https://doi.
for distal hypospadias. J Pediatr Surg. 2012; https:// org/10.1016/S0022-5347(17)41325-5.
doi.org/10.1016/j.jpedsurg.2011.12.030.
Megameatus Intact Prepuce (MIP)
Deformity 23
Ahmed T. Hadidi
Abbreviations 23.2 Morphology
DYG Double Y glanulomeatoplasty The megameatus intact prepuce hypospadias

GAP Glans approximation procedure deformity has usually the following important
MIP Megameatus intact prepuce characters:
SLAM Slit-like adjusted Mathieu
TIP Tubularized incised plate • Intact prepuce.
• Wide-mouth meatus.
• Wide, deep proximal glanular groove (fossa
navicularis). However, this deep groove does
23.1 Introduction not reach to the tip of the glans penis. This is
important and a frequent cause of failure of
The term “megameatus intact prepuce” or MIP surgical repair.
was first described by Duckett and Keating in • No ventral chordee.
1989 and is characterized as a wide-mouth • The urethral plate is well vascularized.
meatus with deep glanular groove associated • Abnormal median raphe.
with normal prepuce and no chordee. Because
the prepuce is intact and looks normal (unlike the
classic hypospadias), the deformity is usually not One important tip that may help to sus-
diagnosed at birth. In fact, the MIP anomaly may pect the MIP anomaly even when the nor-
pass undiagnosed for years until the parents try to mal prepuce is not retractable (due to a
retract the foreskin to clean or during a circumci- narrow preputial orifice) is an abnormal
sion procedure. ventral median raphe (Fig. 23.1). An abnor-
mal, tortuous median raphe is highly sug-
gestive of abnormal urethral development
A. T. Hadidi (*)
either in the form of MIP or chordee with-
Hypospadias Center and Pediatric Surgery out hypospadias. Very rarely, it may be
Department, Sana Klinikum Offenbach, Academic associated even with Epispadias anomaly
Teaching Hospital of the Johann Wolfgang Goethe (Fig. 23.2).
402 A. T. Hadidi
a b c d e
Fig. 23.1 (a–e) Different forms of megameatus intact for DYG or an advancement procedure, coronal suitable
prepuce (MIP) deformity. The lower photo shows the for SLAM procedure. Even adults with MIP are unhappy
appearance of the penis after retraction of the foreskin. with the appearance of the glans and penis and seek cor-
One should suspect that the child has MIP deformity if the rection and to have a normal slit-like meatus at the top of
ventral median raphe is abnormal (deviated or S-shaped). the glans (©Ahmed T. Hadidi 2022. All Rights Reserved)
The urethral meatus may be in the glans (Grade I) suitable
Fig. 23.2 A very rare form of MIP deformity with epispadias: When one retracts the normal foreskin, the child has
distal epispadias and continent bladder (©Ahmed T. Hadidi 2022. All Rights Reserved)
23.3 Incidence and Classification see 7–10 MIP per year (700 hypospadias per
year) suggestive of 2% incidence of hypospadias.
The true incidence of MIP is unknown. It varies However, the actual incidence of MIP might be
from 1 to 15% in some publications [1]. In the much higher than what is reported because a sig-
Hypospadias Center in Frankfurt, Germany, we nificant number of children with MIP may not be
23 Megameatus Intact Prepuce (MIP) Deformity 403
detected or remain untreated after diagnosis [2]. incomplete or hooded foreskin. In the MIP vari-
The MIP deformity may be classified into two ant of hypospadias, glanular urethra forms from
grades according to the location of the urethral the ectodermal pit at the glans tip and open end of
meatus; Grade I: the meatus is in the glans the urethral groove. Maldevelopment of glanular
region; Grade II: the meatus is in the coronal sul- epithelial infolding would appear to be the abnor-
cus, subcoronal, or rarely in the distal penile mal process responsible for MIP formation.
region. This classification helps to choose the Complete closure of urethral fold and prepuce
appropriate surgical approach for correction. but canalization of glanular plate is incomplete
that leads to megameatus intact prepuce.
However, all these theories have failed to explain
There is a golden rule for primary care phy- the embryology of MIP.
sicians, obstetricians, mohels, and surgeons According to the recent migration hypothesis
to retract the foreskin first and abandon cir- (supported by Hunter studies in 1935 and Hadidi
cumcision if there is hypospadias or ure- et al. (2014) who suggested that the prepuce
thral anomaly. This important rule must be forms the floor of glanular urethra), the glanular
respected in the case the child has MIP or urethra passes through four stages of develop-
chordee without hypospadias. This is very ment (Chap. 3): (1) solid plate followed by (2) a
important because the prepuce is not only a blind central canal and then (3) deep glanular
source of skin but also of subcutaneous groove [6, 7]. This is followed by (4) fusion of
dartos fascia that is important as a protec- the prepuce with the glanular wings at the coro-
tive intermediate layer. There is no reason nal sulcus and distal migration and development
to increase the risk of complications for the to form the floor (ventral surface) of the glanular
child. urethra. The MIP deformity can be explained by
distal migration of the prepuce without fusion
with the edges of the deep glanular groove.
23.4 Theories of Pathogenesis
The embryologic pathogenesis of MIP remains

23.5 Surgical Management
unclear. Duckett and Keating have suggested that
Because of the peculiar MIP morphology, it is
the foreskin and urethra develop independently
necessary for clinicians to design a suitable surgi-
and are unrelated [3]. Due to excessive division
cal method taking into account the development
of the glans, the distal urethra that has already
of the glans, the width of the urethral plate, the
formed is split to form a large urethral opening,
depth of the urethral groove, and the shape and
while the foreskin develops normally. Nonomura
position of the urethral opening to achieve good
et al. speculated that ischemia and compression
therapeutic effects [8, 9]. It still remains contro-
necrosis may occur after complete formation of a
versial whether patients with partial MIP, but
normal urethra, causing MIP [4]. Stephens and
with normal micturition and an unobstructed
Fortune considered that the ingrowth of the epi-
sexual life and those whose daily life is unaf-
thelium on the top of the glans leads to delayed
fected must undergo surgical intervention. These
connection or failed fusion with the proximal
patients can choose instead a conservative treat-
urethra, which results in temporal high-pressure
ment. In the Hypospadias Center in Frankfurt
blockage of the distal urethra, thus forming MIP
(Germany), we offer surgical correction to all
[5]. It has been postulated that hypospadias
patients with MIP (children or adults) who want
results from incomplete fusion of the urethral
to have their deformity corrected.
folds, resulting in an incomplete urethra and
404 A. T. Hadidi
23.6 Pyramid Repair in 35 patients; the other two had a previous

Thiersch-Duplay repair with resultant malposi-
Duckett and Keating, as they first reported MIP tion of the neourethra on the shaft. A large pro-
in 1989, described also the pyramid repair for the portion of the patients had been circumcised due
MIP correction (Fig. 23.3) where a tennis racket to an apparently normal prepuce, limiting the sur-
incision is made beside the glanular groove and gical options. Hill and colleagues included a de-
around the edges of the megameatus; this section epithelialized perimeatal tissue flap to cover the
is carried out approximately below the corona, ventral suture line. This technique was originally
mobilizing the urethra to the apex of the pyramid described by Smith in the context of a two-stage
and deepening the edges of the glanular groove to repair and resulted in a low complication rate (1
develop glanular wings from the urethral plate. fistula in 51 repairs) [11]. The use of a de-
The distal urethral plate is left wide and intact epithelialized flap has been described by Retik
dorsally. The widened distal urethra is tailored in et al. [12] and Belman [13].
continuity with the urethral plate by removing a The pyramid modification included de-
small wedge of ventral tissue. The urethra and the epithelialization of the Mathieu flap, leaving only
glans strips are tubularized to form the new ure- a proximal horseshoe-shaped skin margin around
thra, and the glanular repair is performed. the meatus. The de-epithelialized Mathieu flap is
Dressings are applied and removed after 48 h by brought over the ventral suture line, effecting a
the parents, and the stent is removed after two-layer closure.
5–7 days. It is important that the glanular plate is ade-
quate to allow tubularization with compliant
glans to cover the neourethra without compres-
23.7 Hill Repair sion. The presence of a silicone stent may exert
tension on the suture line and result in fistula for-
Hill et al., in 1993, described their experience mation. Successful operation without a stent
with pyramid hypospadias repair in 37 patients reduces complications; a second layer of viable
with distal or subcoronal hypospadias [10]. The tissue is very important to prevent fistula
pyramid repair was used as a primary procedure (Fig. 23.4).
a b c
Fig. 23.3 Steps of the pyramid procedure: (a) Tennis racket incision. (b) Mobilization of the distal urethra. (c)
Urethroplasty and glanuloplasty
a b c d
e f
Fig. 23.4 Steps of procedure described by Hill et al. vascularized mesentery. (d) Reconstruction of neourethra.
[10]: (a) Creation of meatal-based flap. (b) The penile (e) Coverage of neourethra suture line by the vascularized
skin is mobilized: glans flaps and perimeter-based flap mesentery. (f) Appearance after approximation of glanular
and development. (c) The skin flap is excised, leaving its wings
406 A. T. Hadidi
23.8 Nonomura Meatal-Based 23.9 MIP Management

Foreskin Flap in the Hypospadias Center,
Frankfurt
Nonomura et al. used a meatal-based foreskin
flap for urethroplasty, harvested from either a In the Hypospadias Center in Frankfurt, Germany,
ventral (Mathieu) or a unilateral site [4]. The we usually use one of two techniques depending
glans is split along the cleft glanular groove to on the grade of MIP and the mobility of the ure-
create glans wings. The flap is laid on the urethral thral meatus:
plate to form the neourethra, and glanuloplasty is
completed by approximation of the glanular • Patients with Grade I MIP with the urethral
wings. Byars flaps are then used to cover the ven- meatus in the glans region (Fig. 23.1a, b, or c)
tral surface of the penile shaft. This is shown in have their deformity corrected using the dou-
Fig. 23.5. ble Y glanulomeatoplasty (DYG—Chap. 19).
Attalla proposed a surgical repair which Other techniques based on the advancement
combines reconstruction of the glanular ure- principle may be used with high success rate.
thra and fashioning of the neomeatus with cir- • Patients with Grade II MIP with coronal, sub-
cumcision [14]. In this procedure, the prepuce coronal, or distal hypospadias undergo a mod-
is divided in the dorsal midline from its free ified slit-like adjusted Mathieu (modified
edge to 5 mm from the coronal sulcus. An SLAM—see Chap. 22). The modification
approximately rectangular flap is created from from the classic SLAM can be summarized in
the ventral external preputial skin and lining. two points: (1) the flap is taken from the ven-
The preputial flap is raised. A vertical glanular tral foreskin (not from the skin of the shaft of
incision is made on each of the two navicular the penis. (2) The flap is narrow (because of
ridges and deepened enough to create thick the deep glanular groove), but the fascia sup-
urethral and glanular lips. The lateral edges of porting the flap is wide to provide a nice well-
the preputial flaps are sutured to the glanular vascularized protective intermediate layer
incisions in two layers; circumcision is then (Figs. 23.6 and 23.7).
completed. Follow-up showed no fistula,
breakdown, or recession of the meatus with In other words, the intact foreskin provides the
satisfactory cosmetic and functional results. epithelium to form the ventral surface of the neo-
Docimo, in 2001, described a technique urethra. The ventral foreskin also provides the
employing a subcutaneous frenulum flap for iat- preputial fascia that is used to cover the urethro-
rogenic or primary megameatus and re-operative plasty as a second layer. To achieve this, ventral
hypospadias repair. Using an inferiorly based preputial fascia should be designed wider than
island flap of the frenulum, the skin is de- the flap to cover the urethroplasty suture line.
epithelialized and advanced over the urethral There were 39 MIP operated on in the
repair, yielding in a satisfactory cosmetic result Hypospadias Center in Frankfurt (Germany) in the
and providing added elasticity in these relatively period from 2014 to 2019. Eight patients under-
unusual cases in which a dorsal or meatal-based went the DYG procedure, and 31 patients under-
flap is not feasible [15]. went the modified SLAM technique without a
Duan et al., in 2020, used a variety of tech- single complication. This is probably because of
niques including MAGPI, GAP, TIP, Mathieu, the wide urethra that was reconstructed around
and Thiersch in 27 patients [2]. They believe that catheter size F12, the well-vascularized urethral
the surgeon should examine each patient and the plate, the protective intermediate layer, and keep-
anatomical variations individually to decide on ing the foreskin widely open until complete heal-
the appropriate technique. ing of the neourethra and the glans.
a b
c d e
Fig. 23.5 Surgical repair of anterior hypospadias with groove. (c) Creation of Mathieu parameatal flap and glans
fish-mouth meatus and intact prepuce as described by wings. (d) Parameatal flap onlaid to the urethral plate. (e)
Nonomura et al. [4]: (a) Appearance of intact prepuce. (b) Final appearance after completion of meatoglanuloplasty
Subcoronal wide-mouth meatus with deep glanular
408 A. T. Hadidi
a b c
d e
Fig. 23.6 MIP with coronal hypospadias (Grade II): (a) wanted foreskin reconstruction that was performed in a
There is an abnormal S-shaped median raphe. (b) After second operation 6 months later. Notice that the repair
retraction of the normal foreskin. (c) The MIP was cor- was done around a catheter size F12, the slit-like appear-
rected using the SLAM technique with skin flap from the ance of the meatus, and the absence of any incisions on
ventral foreskin and protective intermediate layer from the the ventral aspect of the penis. (e) Appearance after a
dartos fascia under the ventral foreskin. (d) Observe that 5-year follow-up reconstruction (©Ahmed T. Hadidi
the foreskin is left widely open because the parents 2022. All Rights Reserved)
a b c
d e
Fig. 23.7 MIP with coronal hypospadias (Grade II): (a) subcuticular continuous sutures. Notice the separate wide
There is an abnormally long intact prepuce. (b) After inci- ventral dartos fascial layer. (e) The final appearance after
sion of the outer layer of the long prepuce. (c) The MIP meatoglanuloplasty. Observe that the foreskin is left
was corrected using the SLAM technique with the skin widely open because the parents wanted foreskin recon-
flap from the ventral foreskin and protective intermediate struction that was performed in a second operation 6
layer from the dartos fascia under the ventral foreskin. months later (©Ahmed T. Hadidi 2022. All Rights
Notice that the fascial layer is much wider than the Reserved)
parameatal skin flap. (d) Urethroplasty was made with
410 A. T. Hadidi
23.10 Important Surgical Tips Front Pediatr. 2020; https://doi.org/10.3389/

fped.2020.00128.
3. Duckett JW, Keating MA. Technical challenge of
1. Although the urethral plate in MIP is well vas- the megameatus intact prepuce hypospadias vari-
cularized and the glanular groove is deep, we ant: the pyramid procedure. J Urol. 1989; https://doi.
get referred many patients with MIP after org/10.1016/S0022-5347(17)41325-5.
4. Nonomura K, Kakizaki H, Shimoda N, Koyama T,
failed pyramid repair. There are two reasons Murakumo M, Koyanagi T. Surgical repair of anterior
for such high complication rate after pyramid hypospadias with fish-mouth meatus and intact pre-
repair: puce based on anatomical characteristics. Eur Urol.
1998; https://doi.org/10.1159/000019742.
• Meatal stenosis: Although the glanular 5. Stephens FD, Fortune DW. Pathogenesis of mega-
lourethra. J Urol. 1993; https://doi.org/10.1016/
groove is deep, it does not reach the tip of S0022-5347(17)36431-5.
the glans, and when the surgeon tries to 6. Hunter R. Notes on the development of the prepuce. J
reach a slit-like meatus at the tip of the Anat. 1935;70(Pt 1):68–75.
glans, he causes meatal stenosis that may 7. Hadidi AT, Roessler J, Coerdt W. Development of
the human male urethra: a histochemical study on
be complicated by wound dehiscence or human embryos. J Pediatr Surg. 2014; https://doi.
fistula. org/10.1016/j.jpedsurg.2014.01.009.
• Foreskin reconstruction: When the sur- 8. Bar-Yosef Y, Binyamini J, Mullerad M, Matzkin H,
geon tries to restore and keep the foreskin Ben-Chaim J. Megameatus intact prepuce hypo-
spadias variant: application of tubularized incised
intact, the resultant incision in the glans is plate urethroplasty. Urology. 2005; https://doi.
always covered with foreskin, and it org/10.1016/j.urology.2005.04.070.
becomes wet and moist with sweat, urine, 9. Chrzan R, Dik P, Klijn AJ, de Jong TPVM. Quality
and stool (in young age). This causes assessment of hypospadias repair with emphasis on
techniques used and experience of pediatric urologic
wound infection and wound dehiscence or surgeons. Urology. 2007; https://doi.org/10.1016/j.
fistula. Therefore, in the Hypospadias urology.2007.01.103.
Center, we never close the foreskin at the 10. Hill GA, Wacksman J, Lewis AG, Sheldon CA. The
same time we correct MIP. modified pyramid hypospadias procedure: Repair
of megameatus and deep glanular groove vari-
ants. J Urol. 1993; https://doi.org/10.1016/
2. The intact ventral foreskin in MIP should be S0022-5347(17)35729-4.
used to provide not only the floor of the neo- 11. Smith D. A de-epithelialized overlap flap tech-
urethra but also the protective intermediate nique in the repair of hypospadias. Br J Plast Surg.
1973;26:106–14.
layer. 12. Retik AB, Keating M, Mandell J. Complications
3. The surgeon should reconstruct the new ure- of hypospadias repair. Urol Clin North Am.
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org/10.1016/s0022-5347(17)42022-2.
14. Attalla MF. Subcoronal hypospadias with com-
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1. Snodgrass W, Bush N. Hypospadiology. Operation org/10.1016/0007-1226(91)90045-L.
Happenis; 2015. 15. Docimo SG. Subcutaneous frenulum flap (SCUFF)
2. Duan SX, Li J, Jiang X, Zhang X, Ou W, Fu M, for iatrogenic or primary megameatus and reopera-
Chen K, Zheng L, Ma SH. Diagnosis and treatment tive hypospadias repair. Urology. 2001; https://doi.
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Thiersch-Duplay Principle
24
Moneer K. Hanna and Mark R. Zaontz
Abbreviations and the asymmetric lateral incisions so that the

urethral suture lines are unopposed (Fig. 24.1).
GAP Glans approximation procedure In 1874, Duplay described the tubularization
MAGPI Meatal advancement and glanulo- of the urethral plate distal to the urethral orifice
plasty technique [3]. His first successful hypospadias repair with
PDS Polydioxanone suture this technique was achieved in five stages. In
TAP Tunica albuginea plication 1880, Duplay observed the capacity of the ure-
TD Thiersch-Duplay thral plate to tubularize over a catheter. He wrote:
TIP Tubularized incised plate Although the catheter is not actually covered
entirely by skin, I am convinced that this has no ill
effect on the formation of the new urethra; stricture
formation does not occur so long as half of the ure-
thral wall is supplied by skin.
24.1 Introduction
This concept was popularized by Denis Browne
In 1869, Professor C. Thiersch reported that in in 1949, when he described the buried skin strip
1857 and 1858, he had tubularized the urethral method for hypospadias repair [4]. However, his-
plate to form a urethral canal in a child born with torically, Liston in 1837 described in his book
epispadias [1]. He credited the technique to Practical Surgery the repair of epispadias in a
August Brauser, his one-time assistant. Thiersch’s 23-year-old man similar to that of Thiersch in
classic article illustrated the design of the flaps 1869 [5].
Mettauer first reported the concept of skin
chordee in 1842 [6], and subsequently D.R. Smith
reported it in 1967 [7]. In 1968, Allen and Spence
M. K. Hanna (*)
utilized the technique of degloving the penile
Institute of Pediatric Urology, New York Weill
Cornell College, New York Hospital-Cornell skin and performed foreskin transposition to cor-
University, New York, NY, USA rect coronal hypospadias with moderate to severe
e-mail: mhanna@mkhanna.com chordee [8]. King (1970) incorporated the con-
M. R. Zaontz cepts of Mettauer and Thiersch-Duplay to correct
Department of Pediatric Urologist, Children’s midshaft hypospadias and brought the meatus to
Hospital of Philadelphia, Philadelphia, PA, USA
the coronal sulcus [9].
Department of Clinical Urology in Surgery, In the early years, glanular and subcoronal
Perelman School of Medicine, University of
hypospadias were often not repaired, because the
Pennsylvania, Philadelphia, PA, USA
e-mail: mark.zaontz@email.chop.edu complications of repair often overshadowed the
412 M. K. Hanna and M. R. Zaontz
a b
Fig. 24.1 (a) Artist reproduction of the original drawing are not symmetrical so that the urethral incision lies to the
from Thiersch for epispadias correction [1]. (b) Artist right of midline and the skin closure lies to the left of the
reproduction of Anger modification of the Thiersch prin- midline (©Ahmed T. Hadidi 2022. All Rights Reserved)
ciple to correct hypospadias [2]. Notice that the incisions
benefits of surgical correction. Duckett’s meatal children were not candidates for this technique of
advancement and glanuloplasty technique glanuloplasty and in situ tubularization for the
(MAGPI) was designed to reduce the risks of for- repair of distal hypospadias. They achieved better
mal urethroplasty in distal hypospadias [10]. cosmetic results than with either the Mathieu or
Time and experience demonstrated that this tech- the MAGPI procedure.
nique could not be universally applied to all In 1997, Stock and Hanna reported the larg-
patients with distal hypospadias. Arap et al. mod- est documented series of Thiersch-Duplay
ified the MAGPI technique to repair a more prox- repairs [16]. Combining the principles of
imal meatus [11]. Decter described the technique Heineke- Mikulicz meatoplasty to widen the
of an “M inverted V” glanuloplasty and utilized meatus dorsally at 12 o’clock with Thiersch-
the principle [12, 13]. Both Arap’s and Decter’s Duplay tubularization of the urethral plate, they
methods were designed to reduce the chance of noted a low complication rate of 2.1% in 512
meatal retraction, which is sometimes seen after children.
conventional MAGPI repair, and to improve the While some were embracing the Thiersch-
cosmetic result of the glans penis. However, nei- Duplay principle, others looked to the meatal-
ther achieved wide acceptance. based flap technique of Mathieu, which also uses
Zaontz, in 1989, applied the Thiersch-Duplay the urethral plate as the dorsal wall of the urethra.
principle to the repair of distal hypospadias [14]. In 1969, Barcat modified the Mathieu technique,
He reported excellent results with the glans using opposing epithelial flaps based at the
approximation procedure (GAP). The operation meatus to create a neourethra and dissecting the
was indicated for patients with coronal or g lanular urethral plate from its bed. He then incised the
hypospadias, deep glanular groove, and a fish- glans penis and placed the urethral tube within
mouth meatus. The GAP eliminated the ventral the newly created sulcus. With the Barcat tech-
glanular tilt, meatal retraction, and splaying of the nique, the configuration of the glans or meatus
urinary stream that was seen at times in meatal did not matter. However, the postoperative inci-
advancement and glanuloplasty procedures. Van dence of fistulae (15–20%) somewhat dampened
Horn and Kass [15] modified King’s [9] approach the enthusiasm for this technique [17]. Koff et al.
by extending the paraurethral incision to the tip of modified the Barcat technique and interposed a
the glans penis and used a two-layer ventral clo- second layer of tissue over the neourethra, thus
sure of the glans penis. They found that very few reducing the fistula rate to 2% [18]. Koff noted
24 Thiersch-Duplay Principle 413
that the only contraindication for this technique 24.2 Preoperative Evaluation
was in cases where a large amount of chordee
was caused by factors distal to the hypospadiac A routine urologic history is taken and physical
meatus, whereby transection of the urethral plate examination conducted. Questions regarding
was required. Barthold et al. reported their expe- maternal prenatal history and family history of
rience with 295 cases in 1996. All of these surgi- hypospadias are also asked.
cal modifications, including the Mathieu On physical examination one notes the posi-
technique, share the inherent potential complication of the urinary meatus and quality of the
tions of raising and rotating a random skin flap. perimeatal skin, the depth of the glanular groove,
Midline incision of the urethral plate was presence and degree of chordee, penile torsion,
first reported by Reddy in 1975 [19]. He per- penoscrotal transposition, and whether the child
formed the incision to excise “fibrous tissue” has been circumcised. The presence or absence
in the midline that he believed to be the cause and the location of the testicles are recorded, and
of the chordee. He then combined this with the if undescended testicle is diagnosed in conjunc-
Thiersch-Duplay principle and tubularized the tion with any degree of hypospadias, intersex
urethral plate. Orkiszewski in 1987 published workup is requested.
the technique of incising the urethral plate and In children with proximal and especially in
called it the dead man jacket as he used this perineo-scrotal hypospadias, voiding cystogra-
principle with his dead uncle when he incised phy is recommended, as we encountered a sig-
his favorite jacket in the back to be able to nificantly large prostatic utricle in approximately
close it in front [20]. Rich et al. described a 25% of children. We also encountered vesicoure-
technique for the creation of a normal vertical teral reflux in 15% of the patients we studied
slit-like meatus by hinging the distal urethral (unpublished data).
plate longitudinally in the midline [21]. This We currently perform surgery when the patient
modification was applied to a variety of is 6 months old. In the child with a small phallus,
meatal-based flaps and achieved excellent cos- 50 mg of testosterone is given intramuscularly
metic results. In 1994, Snodgrass reported on 3–4 weeks preoperatively.
the technique of a tubularized incised plate
urethroplasty (TIP) for distal hypospadias
[22]. A deep sagittal incision of the urethral 24.3 Selection of Patients
plate was made from the meatus to the tip of
the glans penis. This incision facilitates tubu- We began using the Thiersch-Duplay principle in
larization of the plate and results in a normal 1987 for children with a meatus at or just proxi-
slit-shaped meatus. Furthermore, the deep mal to the coronal sulcus. When the meatus was
incision prevents tension on the ventral suture stenotic, or a prominent shelf was encountered,
line, thus acting as an internal relaxing inci- we performed a Heineke-Mikulicz meatoplasty at
sion. Hinging allows the urethral plate to be 12 o’clock. Our experience in 512 consecutive
tubularized even when its width would ordi- cases was reported previously [16]. We have sub-
narily be inadequate to construct a neourethra sequently extended the use of the Thiersch-
of sufficient caliber. Duplay technique to proximal hypospadias with
We find the Thiersch-Duplay procedure offers chordee due to skin tethering or mild chordee
several advantages. It utilizes the tissues that (<30°), due to corporal disproportion, which was
were embryonically destined to form a urethra; corrected by the Nesbit procedure [23] of tunica
there is minimal disturbance of the blood supply albuginea plication (TAP) [24]. Early in our
of the urethral plate, which is often well vascular- series, the absence of a deep glanular groove and/
ized; there is hardly any contracture of the tubu- or the presence of hypoplastic thin skin at the uri-
larized plates; and stricture formation is indeed a nary meatus were considered contraindications to
rare complication. the Thiersch-Duplay technique. The addition of
the Reddy-Rich-Snodgrass incision of the ure- glans penis in the midline at 12 o’clock to avoid
thral plate and distally in the glans penis allowed any tension on the ventral suture line. The feeding
for repair of hypospadias in children with a shal- tube is then removed, and either a silastic urethral
low or minimal glanular groove. Currently in our catheter or a silicone splint of appropriate size is
practice, the only contraindication to the Thiersch- placed and secured. The urethral plate is then
Duplay repair is the presence of moderate or tubularized over the catheter using a running sub-
severe ventral chordee of >30°, due to corporal cuticular 7-0 polydioxanone suture (PDS)
disproportion. In such cases we divide the urethral (Fig. 24.2d). A dartos flap is raised, mobilized
plate and place a dermal graft to lengthen the ven- from the dorsal penile skin, and then rotated and
tral wall of the corpora, rather than shorten the sutured to cover the urethral suture line. The glan-
dorsal walls by the Nesbit or the TAP procedure. ular skin is approximated with horizontal mat-
tress-interrupted 7-0 polyglycolic sutures
(Fig. 24.2e). For resurfacing the penis, we often
24.4 Operative Technique adopt Byars flaps, which create a midline ventral
raphe [26]. However, the available skin and its
A 5/0 proline or silk stay suture is placed in the blood supply dictate the final closure (Fig. 24.2f).
glans penis dorsal to where the future meatus will DuoDerm dressing is applied around the penis
be. If the meatus is stenotic, a longitudinal mid- and secured with Opsite transparent adhesive. The
line incision is made at 12 o’clock. This incision catheter drains into a double diaper, and the child
is closed transversely in a Heineke-Mikulicz fash- is discharged the same day. In distal hypospadias
ion with interrupted 7-0 polyglycolic suture repair, a catheter is seldom used, but for proximal
(Fig. 24.2a). An 8-F feeding tube is inserted in the repairs, the catheter and dressing are necessary
urethra. If the perimeatal skin is thin and hypo- and are removed 3–5 days postoperatively.
plastic, an incision is made ventrally until a Prolonged urinary drainage is frequently associ-
healthy skin is seen. The incised skin edges are ated with catheter obstruction and bladder spasm,
then approximated with interrupted 7–0 polygly- which only serve to complicate matters.
colic sutures (Fig. 24.3). A subcoronal incision is
made dorsally, laterally, and ventrally around the
meatus; the width of the urethral plate is 12 mm, 24.5 Results
leaving 2 mm of skin around the meatus
(Fig. 24.2b, c). The skin and dartos fascia are dis- The Thiersch-Duplay principle with or without
sected circumferentially proximally, and the penis hinging of the urethral plate is now widely
is degloved. In some cases deep dissection of the accepted and practiced by many surgeons.
penoscrotal junction is necessary to completely The main complications in the reported series
straighten the penis. An artificial erection test is are urethrocutaneous fistula and stricture forma-
performed by placing a vessel loop as a tourniquet tion. Table 24.1 summarizes the surgical morbid-
around the base of the penis, then injecting the ity in the largest reported series of cases using the
corpora with normal saline. In the case of residual Thiersch-Duplay principle. It is inevitable that
mild curvature <30°, we used to use the Nesbit there are subtle variations of the surgical tech-
procedure or TAP modification. However, we nique, and these are hard to quantify. However, in
have recently adopted the Baskin et al. method for our experience, the major factors that determine
chordee repair [25]. If the glanular groove is deep, success and complications are primary vs reop-
we find it unnecessary to incise the glans penis on erative repair and distal vs proximal hypospadias.
either side and prefer to denude the skin by excis- In comparing our early series (1987–1994),
ing two wedges of glanular skin on either side, which included 512 children with distal hypospa-
similar to the GAP (Fig. 24.4). If the glanular dias (coronal or juxtacoronal meatus), with our
groove is inadequately shallow, we incise the second series of 265 children with proximal
a b c
d e f
Fig. 24.2 Diagrammatic illustration of the Thiersch- the meatus; the width of the urethral plate is 12 mm, leav-
Duplay repair of distal hypospadias: (a) A longitudinal ing 2 mm of skin around the meatus. (d) The urethral plate
midline incision is made at 12 o’clock. This incision is is tubularized over the catheter using a running suture. (e)
closed transversely in Heineke-Mikulicz fashion. (b) A The glanular skin is approximated with horizontal mat-
subcoronal incision is made around the glans penis. (c) An tress interrupted 7-0 polyglycolic sutures. (f) The avail-
incision is made dorsally, laterally, and ventrally around able skin and its blood supply dictate the final closure
hypospadias (penile, midshaft, and penoscrotal The interposition of a healthy de-epithelialized

meatus), it became apparent that surgical compli- skin flap, as described by Durham Smith, reduces
cations were more often encountered (8.6%) in the incidence of urethrocutaneous fistula [27].
proximal than in distal repairs (2.9%). We often raise a dartos flap from the dorsal penile
a b c
Fig. 24.3 Thiersch-Duplay repair of proximal hypospa- the deep glanular groove. (c) Completed repair: The
dias: (a) Penoscrotal hypospadias and hypoplastic meatal suture line is covered with dartos flap prior to skin
skin: Ventral meatoplasty at 6 o’clock. (b) Lateral skin closure
incisions and denudation of glanular skin on either side of
skin and rotate it to cover the ventral urethral

suture line (Fig. 24.5). 24.7 ditorial Comment by Mark
E
The dartos or tunica vaginalis flaps provide R. Zaontz
excellent support to the suture line, especially
when a ventral meatoplasty is contemplated. The Children’s Hospital of Philadelphia, Philadelphia,
routine use of a dartos flap or tunica vaginalis flap PA, USA
to waterproof the suture line has resulted in a sig- Clinical Urology in Surgery, Perelman School
nificant decrease in our fistula rate from 17% to of Medicine, University of Pennsylvania,
1.8% in a series of 265 proximal hypospadias Philadelphia, PA, USA.
repairs [28]. President of Hypospadias International
Society (HIS).
Email: mark.zaontz@email.chop.edu
24.6 Conclusion
Hypospadias surgery provides ample opportunity 24.8 History

for modification by any surgeon who wishes to
establish his individuality. Some authors claim While preparing to perform a Thiersch-Duplay
originality of idea, but it behooves us all to credit urethroplasty on a child presenting with coronal
those who pioneered current surgical concepts hypospadias with a megameatus appearance, a
and principles, thus preserving our surgical heri- deep wide glanular groove, and noncompliant
tage. We submit that the Thiersch-Duplay prin- meatus, it occurred to me that by simply approxi-
ciple represents the basic foundation for all the mating the glans with my fingers, a normal-
surgical methods that utilize the urethral plate to appearing phallus resulted. Hence at the time, I
construct a urethral tube. believed that by simply de-epithelializing the
a b
c d
Fig. 24.4 GAP procedure: (a) Megameatus with deep sutures. Note the placement of ventral stay sutures aligns
groove. (b) Glanular skin is excised on either side. (c) The the tissues. (d) Skin closure using horizontal mattress
urethral plate is tubularized using continuous subcuticular sutures
Table 24.1 Surgical morbidity in the major reported series of patients operated on using the Thiersch-Duplay
principle
Number Primary vs Location Complications
of secondary
References patients repair Meatal stenosis Fistula
Zaontz (1989): 24 Primary Coronal 11, 0 1
GAP [14] proximal 13
Kass (2000): 308 Primary Coronal/glans 84%, Diverticulum 2 28 (9.1%): Distal
glanuloplasty (no midshaft 9%, prox./ (0.6%) 6.2%, mid. 17.9%,
hinging) [29] penoscrotal 7% proximal 42.9%
Snodgrass et al. 148 Primary Distal 3 Fistula 5, glans
(1996) [30] dehiscence 3
Ross and Kay 18 Primary 15, Distal or midshaft 0 0
(1997) [31] secondary 3
Stock and Hanna 512 Primary Distal 2 Initial 5 (four small
(1997) [16] fistulae diagnosed
after toilet training)
Steckler and 33 Primary Distal 31, midshaft, 0 0
Zaontz (1997) or proximal 2
[32]
Snodgrass et al. 27 Primary Midshaft, 1 Fistula 1, glans
(1998) [34] penoscrotal dehiscence 1
Stock and Hanna 265 Primary Midshaft to Stricture 3, Fistula 16,
(1997) [16] proximal, diverticulum 2 dehiscence 2
penoscrotal
Decter and 197 (156 Primary Glans 52, coronal Meatal retraction 4 Fistula 5
Franzoni (1998) hinged) 101, proximal 44
[33]
Dayanc et al. 25 Primary 23, Distal 20, midshaft Mid-penile 1 Distal 1 (secondary)
(2000) [35] secondary 2 11 (primary), distal 1
(primary)
Hayashi et al. 13 Secondary Distal 5 0 Distal 1 (secondary)
(2001) [36]
Shanberg et al. 13 Secondary Distal 1 Fistula and glans
(2001) [37] dehiscence 1
Smith (2001) [38] 64 Primary Distal 53, midshaft 0 0
11
Yang et al. (2001) 25 Secondary Distal 10, midshaft 13 (three in patients 7 (0 in patients with
[39] 9, proximal 4, with unaltered unaltered urethral
penoscrotal 2 urethral plate and no plate and no
preoperative fistula) preoperative fistula)
glanular skin just lateral and proximal to the ure- The procedure has evolved from its earlier allit-
thral plate and hypospadias meatus, respectively, eration where the initial approximation of the ure-
I could, with minimal dissection, create an ortho- thra was done with a single-layer running closure
topic urethral meatus and cosmetically complete followed by Lambert interrupted second layer. In
what nature had intended. The presence of the later years that was transformed to a double run-
deep and wide urethral plate and the avoidance of ning layer watertight closure as these repairs were
the need for significant glans wing mobilization largely performed without urethral stents. Hence
without significantly increasing tissue bulking the GAP was presented as a tubeless and simple
during glanular approximation made this specific solution to repair coronal and glanular hypospa-
anatomic configuration ideal to proceed with dias in patients with unique anatomy and a non-
what became known as the glans approximation compliant or fish-mouth megameatus not
procedure (GAP) (Fig. 24.6) [14]. conducive to the MAGPI procedure.
Fig. 24.5 Dartos flap and penile resurfacing. (a) The dartos flap is raised from dorsal prepuce and is dissected proxi-
mally to avoid rotational deformity. (b) The flap is rotated and covers the ventral suture line. (c) Completed repair
In my original article, I had performed 24

GAP procedures with a mean age of 1 year, 11 of 24.9 Operative Technique
which were coronal-based and 13 proximal glan-
ular by meatal location over a 20-month time This technique is a modification of the Thiersch-
frame [14]. No urinary diversion was used and Duplay repair without the extensive mobilization
follow-up was a mean of 9 months. There was of the glans wings. As with all tubularization
one complication, a fistula located only 2 mm techniques, care must always be taken to assess
from the distal glanular meatus and corrected in the posterior urethral plate for any transverse
the office via division of what essentially was clefts separating the meatus for the distal glans
treated like a skin bridge. Overall the cosmetic groove. If present this cleft must be incised to
and functional outcomes were excellent. flatten the urethral plate and thus avoid flow aber-
Fig. 24.6 GAP repair by Zaontz (1989) for glanular hypospadias with cleft glans [14]
rations that might otherwise occur. The lateral lar flaps. As noted the skin at the lateral margins
glanular wings just outside the border of the ure- of the urethral groove is simply de-epithelialized
thral plate is marked with a marking pen to denote to permit approximation and healing. The ability
the area to be incised (Fig 24.7a). Tenotomy scis- to do this stentless added to the overall patient
sors are then used to excise only the previously comfort postoperatively.
marked skin, thus leaving a small U-shaped de- While the GAP technique provided overall
epithelialized area of the glans (Fig. 24.7b). This excellent results in my hands with rare complica-
denuded area is then tubularized in a double run- tions, the advent of the Belman pedicle barrier
ning subcuticular fashion with 7-0 polyglycolic flap [41], derived from the work of Durham
acid suture, bringing the neomeatus to the ortho- Smith, made me pause to rethink this. As the
topic distal glans location (Fig. 24.7c). Because Thiersch-Duplay (TD) has been my “go-to” pro-
the glans groove is deep, closure of the neoure- cedure for distal hypospadias with mild to mod-
thra does not significantly increase the bulk of erate chordee, I began to incorporate the Belman
tissue in the glans. Hence in this technique, no flap in all of my TD repairs along with the aggres-
attempt was made to elevate glanular flaps or sive glans wings dissection that I had been per-
excise divots of spongiosum to create a tension- forming for many years since I began training in
free glans closure performed with gentle inter- 1984. This modification of the TD has made the
rupted 7-0 polyglycolic acid suture. The mucosal word fistula virtually almost disappear from my
collar completes the repair. vocabulary. I began to think that it makes more
sense to make sure there is extra coverage over
the GAP surgical candidates to insure low surgi-
24.10 Discussion and Modifications cal complication risks going forward. Hence I
started to change my GAP technique to one simi-
The GAP originally was performed as a simpli- lar to the TD repair with the addition of the
fied version of the Thiersch-Duplay hypospadias aggressive glans wings dissection as well as the
repair without the necessity to create large glanu- de-epithelialized pedicle flap popularized by
a b c
d e
f g
Fig. 24.7 The GAP technique modified to be used in all ized wide urethra. (c, d) After urethroplasty, the prepuce is
forms of distal hypospadias. The principle of the tech- incised dorsally and mobilized ventrally. (e) The preputial
nique: (a) If the glans is small, preoperative hormone fascia is used as a second protective layer. (f) The foreskin
therapy is used to have glans width 14 mm or more. (b) is excised and circumcision is performed as published by
The incision is designed to have a completely epithelial- Firlit [40]. (g) The final appearance after healing
Belman. In cases where the boy had been previ- lous case. Am J Med Sci. 1842; https://doi.
org/10.1097/00000441-184207000-00003.
ously circumcised in the megameatal variant 7. Smith DR. Repair of hypospadias in the preschool
hypospadias cases, I utilize a reverse pedicle flap child: a report of 150 cases. J Urol. 1967;97:723–30.
which essentially is a second U incision equidis- 8. Allen TD, Spence HM. The surgical treatment of cor-
tant from the meatus to the location of the ortho- onal hypospadias and related problems. J Urol. 1968;
https://doi.org/10.1016/S0022-5347(17)62560-6.
topic meatus only done proximally. The shaft 9. King LR. Hypospadias—a one-stage repair with-
skin is de-epithelialized, and the undersurface out skin graft based on a new principle: chordee is
dartos tissue is mobilized superiorly to act as a sometimes produced by the skin alone. J Urol. 1970;
secondary vascularized layer over the neourethra. https://doi.org/10.1016/S0022-5347(17)62023-8.
10. Duckett JW. MAGPI (meatoplasty and glanu-
The end result is a longer suture line down the loplasty). A procedure for subcoronal hypo-
midline of the shaft that blends in with the other- spadias. Urol Clin North Am. 1981; https://doi.
wise normal line seen in the median raphe. org/10.1097/00005392-200205000-00059.
While testosterone use is very common in my 11. Arap S, Mitre AI, De Goes GM. Modified meatal
advancement and glanuloplasty repair of distal
practice at 25 mg, 5 weeks and then 2 weeks pre- hypospadias. J Urol. 1984; https://doi.org/10.1016/
operatively, many of the megameatal variants S0022-5347(17)50846-0.
have glans sizes significantly larger than 14 mm 12. Mathieu P. Treitment en un temps de l’hypospadias
widths that would preclude its use. My experi- balanique et juxta-balanique. J. Chir. 1932;39:481
13. Decter RM. M inverted V glansplasty: a procedure
ence with testosterone over the past 30 years for distal hypospadias. J Urol. 1991; https://doi.
shows that the average glans size increase in my org/10.1016/s0022-5347(17)37881-3.
patients who receive hormone treatment went 14. Zaontz MR. The GAP (glans approximation proce-
from 11 to 17 mm with exceedingly low compli- dure) for glanular/coronal hypospadias. J Urol. 1989;
https://doi.org/10.1016/S0022-5347(17)40766-X.
cation rates. 15. Van Horn AC, Kass EJ. Glanuloplasty and in situ
Hence since the mid 1990s, I went “back to tubularization of urethral plate: simple and reli-
the future” and returned to the Thiersch-Duplay able technique for majority of boys with hypo-
principle, incorporating the vascularized second- spadias. J Urol. 1995; https://doi.org/10.1016/
S0022-5347(01)66916-7.
ary layer which has served me well ever since. 16. Stock JA, Hanna MK. Distal urethroplasty and glanu-
loplasty procedure: results of 512 repairs. Urology.
Acknowledgment The author would like to thank Dr. 1997;49:449–51.
Adam weiß for his valuable contribution in the chapter in 17. Redman JF. The Barcat balanic groove technique for
the first edition of the book. the repair of distal hypospadias. J Urol. 1987; https://
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18. Koff SA, Brinkman J, Ulrich J, Deighton D, Belman
AB. Extensive mobilization of the urethral plate and
urethra for repair of hypospadias: the modified Barcat
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1869:10–20. 20. Orkiszewski M. Urethral reconstruction in skin defi-
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3. Duplay S. De I’hypospade perineo-scrotal et de son JW. Hinging the urethral plate in hypospadias
traitment chirurgical. Arch Gen Med. 1874;1:613–57. meatoplasty. J Urol. 1989; https://doi.org/10.1016/
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1949; https://doi.org/10.1177/003591574904200703. 22. Snodgrass W. Tubularized, incised plate urethroplasty
5. Liston R. Practical surgery: with one hundred and for distal hypospadias. J Urol. 1994; https://doi.
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Cowperthwait; 1838. 23. Nesbit RM. Operation for correction of distal
6. Mettauer JP. Practical observations on those mal- penile ventral curvature with or without hypo-
formations of the male urethra and penis, termed spadias. J Urol. 1967; https://doi.org/10.1016/
hypospadias and epispadias, with an anoma- S0022-5347(17)63105-7.
24. Daskalopoulos EI, Baskin L, Duckett JW, Snyder 33. Decter RM, Franzoni DF. Distal hypospadias repair
HM. Congenital penile curvature (chordee with- by the modified Thiersch-Duplay technique with or
out hypospadias). Urology. 1993; https://doi. without hinging the urethral plate: A near ideal way to
org/10.1016/0090-4295(93)90540-Q. correct distal hypospadias. J Urol. 1998;162:1156.
25. Baskin LS, Erol A, Li YW, Cunha GR. Anatomical 34. Snodgrass W, Keys M, Manzoni G, et al. Tubularized
studies of hypospadias. J Urol. 1998; https://doi. incised plate hypospadias repair for proximal hypo-
org/10.1016/S0022-5347(01)62711-3. spadias. J Urol. 1998;159:2129.
26. Byars LT. A technique for consistently satisfactory 35. Dayanc M, Tan MO, GoKalp A, et al. Tubularized
repair of hypospadias. Surg Gynecol Obstet. 1955; incised plate urethroplasty for distal and mid penile
27. Smith D. A de-epithelialized overlap flap tech- hypospadias. Eur Urol. 2000;37:102–25.
nique in the repair of hypospadias. Br J Plast Surg. 36. Hayashi Y, Kojima Y, Mizuno K, et al. Tubularized
1973;26:106–14. incised-plate urethroplasty for secondary hypospadias
28. Amukele SA, Weiser AC, Stock JA, Hanna MK. Results surgery. Int J Urol. 2001;8:444–48.
of 265 consecutive proximal hypospadias repairs 37. Shanberg AM, Sanderson K, Duel B. Re-operative
using the thiersch-duplay principle. J Urol. 2004; hypospadias repair using the Snodgrass incised plate
https://doi.org/10.1097/01.ju.0000143880.13698.ca. urethroplasty. BJU Int. 2001;87:544–47.
29. Kass EJ, Chung AK. Glanuloplasty and in situ tubu- 38. Smith DP. A comprehensive analysis of a tubular-
larization of the urethral plate: Long-term follow up. J ized incised plate hypospadias repair. Urology. 2001;
urol. 2000;164:991. 57:778.
30. Snodgrass W, Koyle M, Manzoni G, et al. Tubularized 39. Yang SS, Chen SC, Hsieh CH, Chen YT. Reoperative
incised plate hypospadias repair: results of a multi- Snodgrass procedure. J Urol. 2001 Dec; 166(6):2342–5.
center experience. J Urol. 1996;156:839. PMID: 11696781.
31. Ross JH, Kay R. Use of de-epithelialized local skin 40. Firlit CF. The mucosal collar in hypospadias
flap in hypospadias repair accomplished by tubu- surgery. J Urol. 1987; https://doi.org/10.1016/
larization of the incised urethral plate. Urology. S0022-5347(17)43878-X.
1997;50:110. 41. Belman AB. De-epithelialized skin flap cover-
32. Steckler RN, Zaontz MR. Stent free Thiersch-Duplay age in hypospadias repair. J Urol. 1988; https://doi.
hypospadias repair with the Snodgrass modification. J org/10.1016/s0022-5347(17)42022-2.
Urol. 1997;158:1178.
The “Inverted-Y Thiersch” (IT)
Technique 25
Ahmed T. Hadidi
Abbreviations 25.2 Principles of the Inverted Y

Thiersch (IT)
IT Inverted Y Thiersch
LABO Lateral-based onlay flap A modification of the original technique (inverted
SLAM Slit-like adjusted Mathieu Y Thiersch or IT) that changes a flat or incom-
plete cleft glans into a cleft glans is described in
detail. This technique achieves a slit-like meatus
25.1 Introduction at the tip of the glans without incision of the ure-
thral plate and reduces the incidence of compli-
Liston in 1838 and Thiersch in 1869 tubularized cations (Fig. 25.4).
the urethral plate to correct epispadias [1, 2].
Duplay applied the principle to correct hypospa-
dias in 1874 [3]. The use of protective intermedi- 25.3 Patients and Methods
ate layer has helped to reduce the incidence of
fistula and dehiscence. However, the original During the period from January 2005 to February
Thiersch-Duplay principle (Fig. 25.1) and its 2008, 127 boys with hypospadias underwent
recent modifications are ideal in hypospadias single-stage repair using the inverted Y Thiersch
patients with deeply grooved glans (cleft glans) (IT) technique. Patients age at the time of surgery
(Figs. 25.2 and 25.3). One way to be able to use ranged from 4 to 60 months (mean 11 months).
the Thiersch technique in incomplete cleft and The type of hypospadias and associated anoma-
flat glans is to give preoperative testosterone lies are summarized in Table 25.1. There were 96
25 mg twice, 5 weeks and 2 weeks before surgery penile hypospadias and 31 proximal hypospadias
as Dr. Zaontz uses routinely in distal hypospadias without deep chordee. For penile hypospadias,
and described in Chap. 24. preputial dartos fascia was used as a second pro-
tective layer to cover the neourethra. Dartos fas-
cia/tunica vaginalis from the scrotum was used as
the overlying protective layer in patients with
A. T. Hadidi (*)
Hypospadias Center and Pediatric Surgery proximal hypospadias. Patients with coronal and
Department, Sana Klinikum Offenbach, Academic distal hypospadias had a transurethral catheter
Teaching Hospital of the Johann Wolfgang Goethe for less than 2 days depending on the age of the
University, Offenbach, Frankfurt, Germany patient. Patients with proximal hypospadias had a
426 A. T. Hadidi
Fig. 25.1 Artist replication of the original description of opposite side of the midline from the urethroplasty suture
Thiersch for epispadias and the modification by Duplay to line (©Ahmed T. Hadidi 2022. All Rights Reserved)
use in hypospadias. Notice that the skin closure was on the
a b c
Fig. 25.2 Glans configuration in hypospadias. The glans glans: There is a shallow glanular groove. (c) Flat glans:
is globular in shape. The glans may be cleft, incompletely The glans is flat, no groove. The classic Thiersch-Duplay
cleft, or flat. (a) Cleft glans: There is a deep groove in the technique is not suitable for flat glans and incomplete
middle of the glans with proper clefting. This form is cleft glans (©Ahmed T. Hadidi 2022. All Rights
ideal for the classic Thiersch-Duplay and its modifica- Reserved)
tions as point B is sutured to point B′. (b) Incomplete cleft
25 The “Inverted-Y Thiersch” (IT) Technique 427
a b
d e
f g
Fig. 25.3 (a–g) The Thiersch principle can be applied obtained without the need to do circumcision (©Ahmed
successfully in hypospadias with cleft glans. A good pro- T. Hadidi 2022. All Rights Reserved)
tective layer from the preputial dartos fascia can be
428 A. T. Hadidi
Fig. 25.4 The inverted

Y Thiersch (IT)
Technique: (a) Inverted a b c
Y-shaped deep incision
of the glans. (b) Inverted
V median flap is
elevated. (c) Median flap
sutured to the tip of the
glans. (d) A F10 catheter
is used to mark adequate
length of the new
urethra. (e) A U-shaped
incision of the urethral
plate. (f) Urethroplasty.
(g) Mobilization of the
preputial dartos fascia in
distal hypospadias. (h)
In case of proximal
hypospadias without
severe chordee, the
dartos/tunica vaginalis
fascia is mobilized from
the scrotum. (i) Skin d e f
closure (©Ahmed
T. Hadidi 2022. All
Rights Reserved)
g h i
suprapubic catheter for 10–13 days. Compression for patients with proximal hypospadias.
dressing was applied on the penis in all patients Nowadays, it is possible to use Zaontz or Koyle
for 1–2 days postoperatively. Oral third- stents in which there is no need to put a suprapu-
generation cephalosporin antibiotics and local bic catheter. All the operations were performed
application of eye ointment was used for 1 week by one surgeon. Follow-up ranged between 15
in coronal and distal hypospadias and for 2 weeks and 12 years.
Table 25.1 Distribution of the hypospadias patients in urethra. Frequently, the distal urethra is very thin,
the study
dysplastic, and lacks the supportive corpus spon-
Number of giosum. In such cases, the thin urethra is incised
Type of hypospadias anomaly patients
until a healthy thick well-vascularized urethra is
Distal hypospadias 97
reached to minimize the chances of fistula at the
Proximal hypospadias without chordee 31
Total 128 proximal end.
In the region of the glans, the incision has to
go very deep to create large mobile lateral glanu-
25.4 Operative Technique lar wings to wrap around the new urethra. Two or
three sutures are tied along the length of the new
A traction suture of 4/0 Nylon is placed through urethra to reduce tension and help orientation
the tip of the glans. An artificial erection test is (Fig. 25.4f). The new urethra is constructed using
performed. The urethral plate is assessed, and Vicryl 6/0 on a cutting needle in a continuous
superficial chordee, if present, is corrected by subcuticular manner. This manner helps to invert
mobilization of the skin proximal to the urethral the epithelium into the lumen and reduces the
meatus. number of knots which act as a potential source
An inverted Y-shaped incision is outlined on of chemical reaction and fistula formation.
the glans (Fig. 25.4a). The tip of the longitudinal Protection of the new urethra with a protective
limb of the inverted Y is at the tip of the glans and intermediate layer is an essential step to reduce
where the tip of the neo-meatus will be located. the chances of fistula formation. In distal hypo-
The lower two limbs of the inverted Y are about spadias, the protective layer is fashioned from the
0.8 cm long, and the angle between them is 90 preputial fascia under the foreskin (Fig. 25.4g).
degrees. The long vertical limb of the inverted Y In proximal hypospadias without deep chordee,
is 0.5 cm long. The inverted Y-shaped incision is the scrotal dartos/tunica vaginalis fascia is used
deepened to be able to wrap the glanular wings (Fig. 25.4h). The protective layer is sutured to the
around the new urethra. This results in a median glans and corpora cavernosa lateral to midline
inverted V flap and two lateral wings (Fig. 25.4b). suturing of the new urethra. This provides sealing
The two lateral wings are elevated, and the of the new urethra.
median flap is mobilized and sutured to the tip of Closure of the glans follows starting at the tip
the glans penis (Fig. 25.4c). of the glans to ensure a wide meatus. Adequate
The native urethral meatus is calibrated with a mobilization of glanular wings is mandatory to
metal sound, and when narrow, it was dilated or avoid excess tension on the sutures or narrowing
incised proximally to create a wide spatulated of the new urethra. The glans reconstruction is
meatus (63 patients). A catheter size F12 or larger made using transverse mattress sutures using 7/0
(depending on the age of the child) is inserted Vicryl or thicker material depending on the age
through the hypospadias meatus into the bladder. of the child (Fig. 25.4i).
Using two fine surgical forceps, the adequate Closure of the penile skin over the new urethra
diameter of the new urethra is marked around the is always tricky. As part of the ventral penile skin
catheter (Fig. 25.4d). A U-shaped skin incision is is used to reconstruct the new urethra, there is
made using sharp scissors or scalpel size 15. always deficiency of the penile skin to wrap
Special care is taken to incise the skin only and around the new urethra. This deficiency is over-
preserve the fascia underneath carrying blood to come by careful mobilization of the foreskin and
the urethral plate (Fig. 25.4e). bringing it ventrally and proximally. Patients
The transverse limb of the U-shaped skin inci- with proximal hypospadias may benefit from
sion proximal to the meatus is preferably cut Z-plasty at the penoscrotal junction to create the
using sharp scissor after elevating the skin away normal penoscrotal angle.
from the underlying urethra. This should be done Children with distal hypospadias had a trans-
with caution to avoid injury of the underlying urethral catheter for 1–2 days, depending on the
430 A. T. Hadidi
age of the child and the difficulty of the opera- nine patients and are listed in Table 25.2. Three
tion. Oral third-generation cephalosporin were patients developed meatal stenosis; four patients
used for 1 week. A compression dressing was developed urethra-cutaneous fistula occurring at
applied for 1–2 days. the coronal sulcus. Two patients suffered from
Children with proximal hypospadias as a rou- proximal stenosis that responded to dilatation.
tine had a suprapubic catheter for 10–13 days, The remaining three patients required meato-
depending on the degree of swelling. Oral cepha- plasty. Two of the three patients with the meatal
losporins used for 2 weeks. A compression dress- stenosis had severe inflammation of the meatus
ing was applied for 1–2 days. Nowadays, one (Fig. 25.6). These two patients required aggres-
may use a Zaontz or Koyle stent. sive meatotomy and circumcision.
Our standard postoperative follow-up protocol
includes examination after 3 months, 1 year,
3 years, and then every 5 years until the child is 25.6 Discussion
15 years old. Two third of the patients are seen
regularly according to the abovementioned proto- In 1869, Thiersch described tubularization of the
col. Follow-up period ranged from 12 to 15 years. urethral plate for epispadias repair [2]. Duplay
Results were considered satisfactory when the incorporated this technique for a multistage
boy achieved a glanular meatus, single forward hypospadias repair in 1874 [3]. In 1970, King
stream, unimpeded voiding, good cosmesis, and described a single-stage hypospadias repair for
no need for secondary surgery for the urethra.
Table 25.2 Complications encountered with the inverted
Y Thiersch (IT) technique (128 patients)
25.5 Results and Complications Number of
Complication patients
During a 3-year period, 127 patients with hypo- Meatal stenosis 3
spadias underwent treatment using the “inverted Fistula 4
Y Thiersch” or (IT) technique. Satisfactory Proximal stenosis responding to dilatation 2
results were achieved in 93% (118 of 127 Urethral diverticulum 0
patients) (Fig. 25.5). Complications occurred in Total 9 (7%)
Fig. 25.5 Postoperative photo of a 14-month-old boy with distal hypospadias 1 year after the inverted Y Thiersch (IT)
operation and foreskin reconstruction (©Ahmed T. Hadidi 2022. All Rights Reserved)
Fig. 25.6 A 13-month-old boy who developed meatal the operating room, and a wide meatoglanuloplasty was
infection 4 days after removing the catheter, and he expe- performed (©Ahmed T. Hadidi 2022. All Rights Reserved)
rienced pain and difficulty passing urine. He was taken to
patients with coronal or distal hypospadias by reduction in urine flow rate in the majority of
tubularizing the urethral plate [4]. However, the patients included [9].
urethral meatus was placed at the coronal sulcus, Another option is to use the glanular skin to
and the ventral penile skin defect was covered make a wide new urethra and bring it deep inside
with the dorsal prepuce. Stock and Hanna the glans by the inverted Y technique described
reported their experience with Thiersch-Duplay above. This allows the surgeon to have a wide
principle in distal [5] and proximal hypospadias fully epithelialized neourethra and a slit-like
[6]. Kass modified the King technique by adding meatus. This will result in some skin deficiency
a second protective layer and extending the para- on the ventral aspect of the penis. The resultant
urethral incisions into the glans to achieve a glan- skin deficiency is overcome by bringing the fore-
ular meatus with a complication rate of 9.7% [7]. skin ventrally and proximally to replace the defi-
The classic Thiersch-Duplay principle and the cient skin.
abovementioned modifications are ideal for The concept, design, and aim of the inverted Y
patients with cleft glans (Fig. 25.2a). The prob- is totally different from the large V-plasty that
lem exists when the child has a flat or an incom- Horton and Devine described to avoid meatal ste-
plete cleft glans. nosis in hypospadias repair [16]. The aim of the
For patients with flat or incomplete cleft glans, inverted Y is to turn a flat or incomplete cleft
one option is to incise the urethral plate (TIP) as glans into a deep cleft glans. This will allow
was first described by Reddy in 1975 [8], tubularization of the urethral plate without diffi-
Orkiszewski in 1987 [9], and Rich in 1989 [10] culty and results in a slit-like terminal meatus.
and popularized by Snodgrass in 1994 [11]. The use of a protective intermediate layer is an
However, some studies suggested that the actual essential part of the operation to reduce the inci-
circumference of the new urethra after tubular- dence of fistula formation [17]. For distal hypo-
ized incised plate (TIP) procedure is actually the spadias, the protective intermediate layer was
width of the original urethral plate [12]. In addi- obtained from the preputial dartos fascia. For
tion, the long-term complication rate of the TIP proximal hypospadias, the protective intermedi-
procedure reached up to 35% in distal hypospa- ate layer was obtained from the scrotal dartos/
dias and 66% in proximal hypospadias [13]. tunica vaginalis fascia.
Other studies documented reduced uroflow fol- In conclusion, the classic Thiersch-Duplay
lowing the TIP procedure [14, 15]. In a recent principle is ideal for hypospadias patients with
study, long-term follow-up in proximal hypospa- cleft glans and without deep chordee. The
dias showed functional obstruction and marked “inverted Y Thiersch” (IT) technique is suitable
432 A. T. Hadidi
for a selected group of hypospadias patients with follow-up. J Urol. 2000; https://doi.org/10.1016/
s0022-5347(05)67234-5.
flat or incomplete cleft glans. 8. Reddy LN. One-stage repair of hypospadias. Urology.
1975; https://doi.org/10.1016/0090-4295(75)90070-9.
9. Orkiszewski M, Leszniewski J. Morphology and
The technique is not suitable when deep urodynamics after longitudinal urethral plate inci-
chordee is present. sion in proximal hypospadias repairs: long-term
results. Eur J Pediatr Surg. 2004; https://doi.
org/10.1055/s-2004-815778.
Although the IT technique has good results 10. Rich MA, Keating MA, Snyder McC H, Duckett
with complication rate 7%, the author has shifted JW. Hinging the urethral plate in hypospadias
meatoplasty. J Urol. 1989; https://doi.org/10.1016/
to the SLAM (slit-like adjusted Mathieu) tech- S0022-5347(17)39161-9.
nique for distal and LABO (lateral-based onlay 11. Snodgrass W. Tubularized, incised plate urethroplasty
flap) for proximal without severe chordee because for distal hypospadias. J Urol. 1994; https://doi.
the SLAM technique has less incidence of fistula org/10.1016/S0022-5347(17)34991-1.
12. Holland AJA, Smith GHH. Effect of the depth and
and stenosis in his hand (see Chap. 22: SLAM width of the urethral plate on tubularized incised plate
and Chap. 30: LABO). urethroplasty. J Urol. 2000; https://doi.org/10.1016/
S0022-5347(05)67408-3.
assessment of hypospadias repair with emphasis on
References techniques used and experience of pediatric urologic
1. Liston R. Practical surgery: with one hundred and urology.2007.01.103.
thirty engravings on wood. Philadelphia; 1838. 14. Wolffenbuttel KP, Wondergem N, Hoefnagels JJS,
2. Thiersch K. Ueber die Enstehungsweise und opera- Dieleman GC, Pel JJM, Passchier BTWD, de Jong
tive Behandlung der Epispadie. Arch der Heilkd. BWD, van Dijk W, Kok DJ. Abnormal urine flow
1869:10–20. in boys with distal hypospadias before and after
3. Duplay S. De l’hypospade périnéo-scrotal et de son correction. J Urol. 2006; https://doi.org/10.1016/
traitement chirurgical. Arch Gen Med. 1874:1. S0022-5347(06)00614-8.
4. King LR. Hypospadias--a one-stage repair with- 15. Braga LHP, Pippi Salle JL, Lorenzo AJ, Skeldon
out skin graft based on a new principle: chordee is S, Dave S, Farhat WA, Khoury AE, Bagli
sometimes produced by the skin alone. J Urol. 1970; DJ. Comparative analysis of tubularized incised plate
https://doi.org/10.1016/S0022-5347(17)62023-8. versus Onlay Island flap urethroplasty for penoscrotal
5. Stock JA, Hanna MK. Distal urethroplasty and glanu- hypospadias. J Urol. 2007; https://doi.org/10.1016/j.
loplasty procedure: results of 512 repairs. Urology. juro.2007.05.170.
1997;49:449–51. 16. Devine CJ, Horton CE. A one stage hypospa-
6. Amukele SA, Weiser AC, Stock JA, Hanna dias repair. J Urol. 1961; https://doi.org/10.1016/
MK. Results of 265 consecutive proximal hypo- S0022-5347(17)65301-1.
spadias repairs using the thiersch-duplay prin- 17. Hadidi AT. Protective intermediate layer.
ciple. J Urol. 2004; https://doi.org/10.1097/01. Hypospadias Surg. 2004; https://doi.
ju.0000143880.13698.ca. org/10.1007/978-3-662-07841-9_30.
7. Kass EJ, Chung AK. Glanuloplasty and in situ
tubularization of the urethral plate: long-term
Incision of the Urethral Plate
26
Mark Rich
Abbreviations vertical slit-like meatus [2]. Extension of the full-

thickness midline incision concept incorporating
CHOP Children’s Hospital of Philadelphia Thiersch-Duplay (TD) principle [3] of urethro-
LIJ Long Island Jewish Medical Center plasty was a small technical change that allowed
MAGPI Meatal advancement and a flat and rigid urethral plate to become wider and
Glanuloplasty “hinge” in evolution of the tubularized incised
POST Plate Objective Score Tool plate (TIP) repair (Fig. 26.2) [4] that has become
PSARVP Posterior sagittal anorectal the preferred option for midshaft and distal hypo-
vaginoplasty spadias [5–8].
TD Thiersch-Duplay
TIP Tubularized Incised Plate
UP Urethral Plate 26.2 History of the Midline
UPJO Ureteropelvic Junction Obstruction Incision
VCUG Voiding Cystourethrogram
“What has been will be again, what has been
done will be done again, there is nothing new
under the sun” (Ecclesiastes 1:9 NIV). Endorsing
26.1 Introduction the wisdom and ingenuity of the past, Durham
Smith (1997) paraphrased this notable biblical
Hypospadias is a common congenital anomaly passage when stating: “There is nothing new in
that has challenged the surgical skills and creativ- surgery not previously described,” and his oft-
ity of reconstructive surgeons and urologists for quoted dictum may be particularly true for hypo-
centuries [1]. Modern era hypospadias repair spadias surgery. Tracing the historical
strives to restore normal penile form and func- development of midline incision and hinging, the
tion. Midline incision of the urethral plate (UP) urethral plate in the evolution of TIP hypospadias
(Fig. 26.1) was introduced as a modification of repair embodies this concept.
the Mathieu hypospadias repair to improve cos- The modern era of urology in the 1980s was a
metic urethral meatal appearance and create a transformative time of technological advance-
ment. As a urology resident and fellow during the
modern era of hypospadias, Rich conceived the
M. Rich (*) idea of the midline-incised urethral plate from a
Arnold Palmer Hospital for Children, diverse number of mentoring influences, obser-
Orlando, FL, USA
e-mail: mark.rich@orlandohealth.com vations, and a touch of serendipity. During resi-
434 M. Rich
Fig. 26.1 Operative technique illustration of midline incision with meatal-based flap repair
dency from 1983–1988 at Long Island Jewish tubularization of the neourethra with healing over
(LIJ) Medical Center, Rich trained with Arthur a urethral catheter.
Smith, the “Father of Endourology” [9]. In 1989, as a pediatric urology fellow, Rich
Endourology evolved from percutaneous mini- had the privilege of mentorship under John
mally invasive manipulation of the urinary tract. Duckett and Howard Snyder, recognized pio-
Performing percutaneous endopyelotomy for the neers of hypospadias reconstruction at Children’s
management of primary ureteropelvic junction Hospital of Philadelphia (CHOP). Duckett coined
obstruction (UPJO) demonstrated reliable results the term “hypospadiology” as an evolving disci-
of full-thickness incision and widening of a con- pline dedicated to the surgical management and
genital narrow ureteropelvic obstruction incised understanding the art and science of hypospadias
longitudinally and left open to heal over a stent [15]. Rich was exposed to many innovative surgi-
[10, 11]. cal repairs that emphasized preservation of the
As a senior urology resident at LIJ, Rich men- urethral plate in distal hypospadias including the
tored under Alberto Pena, a pediatric surgeon meatal advancement glansplasty/MAGPI proce-
who developed and popularized a revolutionary dure and Mathieu and island onlay preputial flap
midline posterior sagittal approach for anorectal, urethroplasty [16–19].
urogenital, and cloacal malformations [12]. A defining moment occurred when Duckett
Posterior sagittal anorectal vaginoplasty asked Rich’s impression of hypospadias out-
(PSARVP) was a natural midline anatomic comes at CHOP (1988). Rich concluded that
approach for dissection, preservation of anorectal despite reliable functional results of distal hypo-
and urogenital structures, and orthotopic recon- spadias repair, the final cosmetic appearance and
struction. Posterior sagittal dissection of the per- shape of the urethral meatus was not optimal. The
sistent cloaca preserved a distal midline epithelial meatal shape was tacitly described as round; fish-
plate/common channel of variable length that mouth; and Rich’s favorite personal description,
was tubularized to create a urethra [13]. Rich “bouche de canon” (mouth of the canon) [20].
observed in cases of cloaca that the length and Tubularization of the urethral plate was not a
width of the epithelial plate and common channel new concept. Thiersch-Duplay (1869) [3]
varied based on the spectrum of the malformation described the technique to tubularize parallel
[14]. In cases with a narrow plate, Pena would incisions of the UP to create a urethra in distal
unfurl the lateral epithelial plate edges and incise epispadias repair and incomplete epithelialized
the plate in areas that were narrow to facilitate neourethra reconstruction, respectively, Duplay
26 Incision of the Urethral Plate 435
Fig. 26.2 Operative technique illustration of tubularized incised plate (TIP) (©Ahmed T. Hadidi 2022. All Rights
Reserved)
436 M. Rich
(1880) [21]. King (1970) tubularized the urethral demanded more evidence as the technique was
plate for midshaft hypospadias and repositioned not dissimilar from other hypospadias repairs
the meatus to the subcoronal level [22]. Midline incorporating the TD principle [27]. Rich was
incision of the urethral plate was reported by encouraged to submit the technique for publica-
Reddy (1975) to excise fibrous chordee tissue tion by a contemporary pediatric urologist at
beneath the UP and then incorporate TD princi- CHOP, Michael Keating, who rendered the origi-
ple for urethral tubularization [23]. In “hypospa- nal artwork of the midline incision and helped
diology,” Snodgrass (2015) mentions a personal coin the term “hinge” (Fig. 26.3). The technique
communication by Duckett that after completion was ultimately submitted for publication as a
of the hypospadias urethroplasty, Duckett would modification of the Mathieu repair [2, 28] to cre-
blindly incise the distal urethral plate, not for ate an esthetic vertical slit-like meatus. Snodgrass
cosmetic value but to reduce the risk of (1994) observed the cosmetic and technical value
postoperative meatal stenosis [24]. Postoperative of the incised urethral plate after reading the
observation by Rich revealed that urethral meatus report by Rich and subsequently validated and
configuration after blind UP incision was more popularized the TIP repair [4]. Ultimately world-
esthetic, vertical, and slit-like in appearance. At wide adoption and dissemination of TIP was a
CHOP that same year, Duckett and Keating result of similar-minded observations made by
(1989) reported on the technical challenges of the Snodgrass and other reconstructive surgeons ded-
megameatus intact prepuce (MIP) hypospadias icated to the evolving the art and science of hypo-
variant [25]. The pyramid procedure for MIP spadiology [29, 30]. The hinge midline incision
variants was based on the similar TD principle of was a “tipping point” in which an observation
periurethral plate tubularization to create a neo- and a small change caused a major paradigm shift
urethra. Zaontz (1989) also reported success with of operative decision-making in hypospadias sur-
the GAP (glansplasty approximation procedure) gery [31].
repair for patients with distal hypospadias and a
deep-clefted glans groove [26].
Opportunity and observation of these diverse 26.3 Preoperative Assessment
yet similar surgical contributions influenced
Rich’s decision to perform a more extensive mid- Preoperative assessment changes with time as a
line incision of the urethral plate for distal hypo- newborn phallus morphologically changes with
spadias repairs. During fellowship at CHOP in natural postnatal surge of gonadotropins and tes-
1988, Rich performed the first case of full- tosterone [32]. Objective measurement of hypo-
thickness midline incision combined with a spadias anatomic features by GMS score has
Mathieu meatal-based flap urethroplasty, pro- been shown to correlate with severity of hypospa-
gressing to island onlay repair and ultimately dias and risk for postoperative complications [33,
complete urethral tubularization. Extrapolating 34]. Glans (G) size and urethral plate quality, (M)
upon success with full-thickness incision of meatal location, and (S) shaft curvature were
UPJO, urethral reconstruction and tubularization measured with the aim of developing methodol-
of the epithelial plate and common channel in ogy to standardize the assessment and spectrum
persistent cloaca and preservation of urethral of hypospadias. Features besides the location of
plate in hypospadias repair heuristically influ- the hypospadias meatus are classified and mea-
enced Rich to perform full-thickness midline sured to assess the impact on functional and cos-
incision of the urethral plate with healing over a metic outcomes [35, 36]. Glans size less than
urethral stent. The operative technique describing 14 mm was reported as an independent risk factor
UP tubularization was not submitted for publica- for urethroplasty complications including fistu-
tion based on anecdotal small case numbers and las, glans dehiscence, and meatal stenosis/neo-
senior mentor oversight tempering trainee enthu- urethral strictures [34, 37]. The urethral plate
siasm for additional data. Duckett-Snyder objective scoring tool (POST) was developed as
a c
Fig. 26.3 (a) Original drawing of “hinge” midline inci- meatus. (c) Postoperative vertical slit-like appearance
sion. (b) Full-thickness incised urethral plate incision and after a midline incision
folding with glansplasty creating a vertical slit-like
an objective measurement and ratio of urethral 8.34 mm and creates a urethral lumen diameter
plate and glans length to assess the quality of the of 2.67 mm × 3 = 8 French according to the
UP and outcomes of distal hypospadias repair French scale (1 Fr: 1/3 millimeter, e.g., 8 Fr tube
[38]. The urethral plate shape was found to be is 8 × 0.33 = 2.67 mm caliber) [43]. Normal ure-
associated with meatal stenosis in cases described thral width has been measured between 2.3 and
as flat or shallow groove variants [39]. Narrow 3.3 mm [44]. Tubularization of an 8.34-mm-
urethral plate width was implicated as a risk fac- diameter urethral plate does not create a urethra
tor in fistula formation, and a urethral plate diam- with an 8-French lumen because the French
eter less than 8 mm was reported as a risk for scale formula calculates the outer diameter of
complications in TIP urethroplasty [40]. the urethral tube and not the inner lumen caliber.
However, other reviews from a series of TIP have Small millimeter changes in urethral plate width
shown that glans size, urethral plate width, and and thickness increase outer urethral diameter
appearance did not correlate with surgical out- and decrease inner lumen caliber. Suture tubu-
comes [41, 42]. larization of the urethral plate incrementally
Based on personal experience if you do the decreases UP diameter, and the tubularized
math, size does matter. Reconstruction of an anastomosis has been shown to heal with a des-
8-French urethra requires a UP width of moplastic inflammatory response [45]. The
438 M. Rich
physics of urine flow according to Poiseuille’s meatus and supporting spongiosum, urethral
equation dictates urethral resistance is greater plate shape and epithelium thickness, glans diam-
and flow is reduced in a urethra of smaller width eter and configuration, degree of penile chordee,
[46]. It has been demonstrated that most cases and interplay of the components of the hypospa-
of hypospadias repair irrespective of technique dias spectrum. In selection of any operative tech-
have decreased fractionated uroflowmetry rates nique, it is important to highlight anatomic and
in comparison to age-matched controls. developmental morphologic features impacting
Although spontaneous improvement in uroflow- the decision to perform TIP urethroplasty and
metry has been demonstrated over time even in how preoperative assessment and intraoperative
asymptomatic patients, 7 years after surgery, technical modifications can affect surgical
32% of patients demonstrate flow rates below outcomes.
the fifth percentile [47]. Defining the long-term
functional and cosmetic impact of hypospadias
surgery in the pediatric population should con- 26.4 Androgen Stimulation
tinue into adulthood [48, 49].
The length and depth of the midline incision is Testosterone supplementation is controversial,
the critical detail that allows TIP urethroplasty by weighing the purported benefits of increased
widening the UP diameter. Inadequate incision penile and glans size, neovascularization against
depth and excessive distal incision of the UP concerns of poor wound healing, increased risk
have been postulated as possible explanations for of bleeding, and other complications [56]. In my
meatal stenosis [50]. I suspect meatal stenosis experience hormonal stimulation is a valuable
with narrow UP variants is more common adjunct tool for less ideal hypospadias variants
because the distal UP width between the glans with a small, broad, flat, round, or narrow glans
wing tips is narrower in smaller glans, less pli- (<14 mm); poor extension of the urethral plate
able, and not incised. A narrow urethral plate into the glans; and flat, shallow, hourglass shape,
after midline incision creates a larger urethrot- or narrowed urethral plates and width of the UP
omy gap that has been speculated to heal by re- <8 mm (Fig. 26.4a–j). I administer (1–2) doses of
epithelialization and granulation tissue formation testosterone cypionate IM at a dose of 25 mg
with contracture, fibrosis, and stenosis [51]. (2 mg/kg) at 6 weeks and 2 weeks prior to sur-
Some authors recommend placement of a prepu- gery. I have performed TIP in cases with smaller
tial inlay graft (G-TIP) to augment the UP before glans (10–14 mm) and urethral plate diameter
tubularization in these cases with equivalent out- (6–8 mm) when parents decline hormonal stimu-
comes to TIP [52–54]. Combining Mathieu and lation and in hypospadias variants with ideal
TIP techniques (M-TIP) has also been reported to glans configuration and deep-clefted urethral
decrease complications of stenosis in cases with plate without increased rate of complications.
narrow urethral plate compared to standard TIP Greater surgical experience of the hypospadias
repair [55]. Penile curvature greater than 30 spectrum and case volume allows surgeons to
degrees after degloving maneuvers is associated push the envelope of any surgical technique [57,
with an increased risk for complications with TIP 58]. In order to create a reliable, reproducible
urethroplasty and has been reclassified by some operation with successful outcomes across many
authors as a more proximal hypospadias variant levels of surgical experience, there is no shortcut
[24]. The ultimate decision to perform a TIP to the learning curve and decision-making pro-
should not be based on a one-size-fits-all cess to perform TIP and other techniques for dis-
approach or focus on a single anatomic feature. tal hypospadias repair. Evolution and modification
Decision-making should occur at the time of sur- of any surgical technique should account for sur-
gery based on evaluation of the anatomic charac- gical experience, case volume, and utilization of
teristics including urethral meatal location, any adjunct tool with potential to improve patient
urethral caliber, developmental integrity of the and surgeon outcomes.
a b c d e
f g h i j
Fig. 26.4 Less optimal glans and urethral plate variants: meatus (arrow) with grooved glans; (f) stenotic ectopic
(a) small flat glans configuration and shallow urethral meatus position (arrow); (g) poor extension of urethral
plate, paucity of subepithelial plate tissue; (b) small flat plate with shallow hourglass shape plate, paucity of sub-
glans with megalourethra, (c) small round glans configu- plate tissue and small flat glans; (h) meatal stenosis, thin
ration, meatus location on ventral glans, and poor urethral distal urethra, and absent plate; (i) meatal stenosis and
plate development; (d) small glans and ventral poor exten- absent plate; (j) megalourethra identified after
sion of flat urethral plate into glans; (e) stenotic ectopic circumcision
The urethral plate is evaluated in relation to

26.5 Operative Technique the developmental size and configuration of the
and Tips and Tricks glans [34, 38]. A small flat, round glans configu-
ration and UP does not inherently hinge or fold as
26.5.1 Step 1: Operative Assessment naturally as more ideal conical grooved variants
(Fig. 26.5a–e). A flat UP variant requires a deeper
A glans traction suture of 5-0 silk is placed dorsal more extensive midline incision and wider glans
to the urethral pit and proposed urethral neome- wing mobilization to decrease tension of the
atus. The urethral meatus is calibrated with ure- glansplasty closure. Less ideal glans urethral
thral sounds to evaluate the developmental plate variants, i.e., flat glans and shallow UP,
location and integrity of the distal urethral epithe- have less glans wing thickness and distance
lium, spongiosum, dartos, and overlying ventral between the ventral meatal lip and proximal cor-
shaft skin. Distal hypospadias variants with onal edge. Measurement and ratio of the UP over
small, flat, round glans configuration; meatal ste- glans fusion length (POST) has been proposed as
nosis; meatal ectopia; megalourethra; poor ure- an objective assessment of UP quality and out-
thral plate development; and poor extension of comes of hypospadias repair [38]. Decreased
the UP into the glans require contemplation of glans tissue fusion length and plate depth dimin-
alternative hypospadias techniques, modification ishes the glans bridge buttress available for glans-
of TIP repair, or combination of techniques to plasty closure. The inner glans wing edge aligns
optimize functional and cosmetic outcomes at a more obtuse angle to the urethral plate creat-
(Fig. 26.4a–j). ing greater glans separation and tension across
440 M. Rich
a b c d e
Fig. 26.5 Glans and urethral plate variants for TIP urethro- with supporting subepithelial plate tissue, urethral plate diam-
plasty. Glans diameter greater than 14 mm, conical glans con- eter greater than 8 mm, and urethral plate extension into glans
figuration and deep groove variants, healthy urethral plates (a-e) demonstrate hypospadias variants for TIP
the shorter glansplasty bridge. The combined the dorsal aspect of the penis just above Buck’s
anatomic features of a flat glans and UP increases fascia. Ventral dissection is performed in the
the inherent risk for glans dehiscence and meatal superficial plane between the thin distal shaft
stenosis. Performing a TIP procedure in this set- skin and dartos layer to prevent injury to the ure-
ting should accept a subapical meatus positioned thra and preserve a dartos layer for barrier cover-
more proximal on the ventral glans with a shorter age of the urethroplasty. In rare cases a distal
and thinner glans bridge closure. Attempting hypospadias variant is identified with a longer
glansplasty closure to the tip of the TIP is tempt- hypoplastic urethral segment covered by thin
ing for esthetic appearance; however, it is prefer- poorly keratinized shiny ventral skin and defi-
able to have a good functional and cosmetic cient underlying layers of dartos and spongiosum
outcome and avoid the risk of glans dehiscence (Fig. 26.7a–d). A standard or modified TIP can
and iatrogenic meatal stenosis [59]. It also raises still be performed in these cases with meticulous
the question if TIP should be performed with this dissection and preservation of the thin urethra
anatomic variation or would a different operation (Fig. 26.8a, b). Spongioplasty is performed by
such as G-TIP, Mathieu, or a combination of paraurethral mobilization and approximation of
techniques be a better option [53, 55, 60]. the splayed spongiosum as a reinforcing interme-
diate layer of muscular support and fistula pre-
vention (Fig. 26.9a–c) [61].
26.5.2 S
tep 2: Degloving The majority of chordee is superficial and
and Chordee effectively released by shaft skin degloving.
Artificial erection is performed as described by
The urethral plate is outlined as a parabolic “U” Gittes and McLaughlin (1974) [62]. Additional
outline below the meatus with parallel incisions penile torsion and chordee are released after
extending between the lateral edge of the UP and degloving the shaft skin with excision of periure-
glans wings. In cases with a thin distal urethra thral fibrous dartos tissue, release of penoscrotal
deficient in supporting spongiosum, the base of attachments, and partial mobilization of the glans
the “U” should be marked at the transition on either side of the urethral plate from the cor-
between thin and healthy urethra (Fig. 26.6a, b). poral bodies. Residual chordee less than 30
A mucosal collar is outlined as described by Firlit degrees is corrected by excision of a transverse
(1987) and injected with 1:100,000 lidocaine/ 1–2-mm-diameter wedge of the tunica albuginea
epinephrine for local analgesia and hemostasis. from the corpora in the area of maximum dorsal
Skin dissection and shaft degloving begin along and lateral penile curvature [63]. I prefer the
a b
Fig. 26.6 (a, b) Ventral skin incision outlined lateral to the urethra and proximal to the thin urethral segment
a b c d
Fig. 26.7 (a–d) Thin hypoplastic urethral variants with variable spectrum of glans and urethral development
442 M. Rich
a b
Fig. 26.8 (a, b) TIP repair with a distal thin urethral variant
Nesbit technique to a single midline plication as Glans wing dissection and mobilization are
described by Baskin [64]. The tunica albuginea is continued along the deep plane of the corpora
reapproximated with multiple 6-0 PDS to distrib- from medial to lateral and proximal to distal for
ute homogeneous plication of the dorsal and lat- maximum mobilization and partial disassembly.
eral corpora with less chance of corporal tunica Extensive wide and deep mobilization of the
knuckling, palpation of the plication sutures, and glans is a crucial maneuver that was emphasized
recurrence of penile curvature [65]. by Duckett, especially in flat glans variants to
reduce the risk of glans dehiscence (Fig. 26.10c).
The dissection of the glans wing and UP is
26.5.3 S
tep 3: Glans and Urethral extended to the distal lateral tip of the plate out-
Plate Dissection lined within the glans.
After degloving the penis, infiltration of addi-

tional local (1:100,000 lidocaine/epinephrine) is An important technical point to emphasize
injected below the UP and into the glans wing. is that dissection of the urethral plate
The UP dissection is initiated at the proximal should extend to the tip of the glans for
coronal glans urethral plate junction and dis- maximum glans and plate mobilization, but
sected perpendicularly along the lateral edge of tubularization of the UP is not completed to
the urethral plate until reaching the level of the the distal extent of the plate incisions to
corporal body without undermining the urethral prevent iatrogenic meatal stenosis.
plate or skiving the glans wings (Fig. 26.10a–c).
a b c
Fig. 26.9 (a) Proximal thin urethra variant. (b) Preserved giosum, and reconstruction. (c) Postoperative repair of the
thin urethra and splayed paraurethral spongiosum (thick thin urethra variant with M-TIP incorporating thin meatal-
arrows), spongioplasty paraurethral mobilization of spon- based flap (yellow arrow in (b))
a b c d e
Fig. 26.10 (a) TIP hypospadias repair. (b) “U”-based tion. (d) Glansplasty complete. (e) Op-site dressing and
urethral plate incision. (c) Dissection of the urethral plate Zaontz stent for urine diversion
beyond planned tubularization and wide glans mobiliza-
26.5.4 S
tep 4: Incision of the Urethral plate symmetrically with fine 0.5 Castro-Viejo
Plate and Urethroplasty forceps defines the precise midline of the plate.
Full-thickness incision of the UP epithelium and
Midline incision of the UP is performed from the underlying subplate webs of glans tissue releases
meatus to the distal plate at least 2–3 mm proxi- the medial edges of the incised plate, deepens the
mal to the meatal pit (Fig. 26.11a–c). Gently furrow between the plate halves, and allows the
separating and elevating the lateral edges of the plate to extend, widen, and hinge along its entire
444 M. Rich
a b c
Fig. 26.11 (a) Midline separation urethral plate pre-incision. (b) Midline incision urethral plate proximal to the meatal
pit. (c) Full-thickness incision and separation of urethral plate
length. Incision of the plate to the depth of the not wrapped in the glansplasty closure. A two-
corporal body is not necessary when full- layer urethroplasty closure is performed with an
thickness incision adequately widens the plate, initial running inverting first layer of 7-0 PDS II
enabling tensionless tubularization. on a BV-1 taper needle and second interrupted
Full-thickness plate incision is dissected to the imbricating layer 7-0 PDS II suture.
level of the corporal body in cases with a flat
plate that is less pliable, narrow plate with
planned augmentation, and reoperative TIP cases. 26.5.5 S
tep 5: Urethroplasty
A distal plate holding stitch of 6-0 polydioxa- Coverage and Glansplasty
none (PDS) is placed at the distal lateral edges of
the urethral plate at least 2–3 mm below the apex Barrier coverage of the urethroplasty is provided
of the plate incisions to recreate a wide elliptical by mobilization of the preserved ventral dartos
neomeatus. Recreation of the neomeatus by trac- flap, a dartos flap mobilized from the dorsal shaft
tion on the holding stitch ensures adequate meatal skin or a tunica vaginalis flap from the testes. The
caliber and location over an 8–10 French urethral flap is mobilized, trimmed, and thinned to mini-
sound. Additional lateral dissection of the distal mize tissue bulk within the glans channel and is
parameatal plate edge from the glans wings is sutured to the corpora (Fig. 26.12a, b).
performed to maximize meatal mobilization and Glansplasty is performed with 2-3 superficial
confirm esthetic symmetry of the meatus. In horizontal subepithelial 6-0 or 7-0 PDS II sutures.
cases with a flat urethral plate unfurling the The first distal glans stitch is symmetrically
epithelial edges of the proximal parameatal-
placed just proximal to the level of the distal ure-
based “U” and parallel urethral plate edges before thral plate tubularization, to prevent glans con-
the midline incision will facilitate rolling and striction and garroting of the distal urethroplasty
inversion of the urethroplasty. Urethroplasty is and meatus. Traction of the first distal glansplasty
performed with subepithelial inversion starting at suture ensures symmetric alignment of the glans
the proximal parameatal plate edge as an impor- facilitating placement of additional proximal
tant technical point during urethroplasty because superficial glansplasty sutures until reaching the
fistula formation is more common at this location coronal margin. A second layer of interrupted 7-0
where the proximal urethroplasty is exposed and PDS II sutures aligns the epithelial glans wing
a b
Fig. 26.12 (a) Dartos flap mobilized, trimmed, and thinned. (b) Dartos flap barrier layer covers urethroplasty
edges from the ventral submeatal glans edge to caretakers to manage postoperatively with simple
the coronal margin (Fig. 26.10d). The urethro- diaper changes.
plasty and meatus are not sutured to the glans to
prevent traction and distortion of the urethro-
plasty and meatus. After glansplasty the glans 26.5.6 S
tep 6: Foreskin
wings and mucosal collar dartos are sutured to Reconstruction
the corporal body on each side to anchor the
glans and collar. The mucosal collar epithelium is The last step part of hypospadias repair is skin
aligned in the midline with 7-0 PDS II sutures coverage with or without preservation of the
(Fig. 26.10d). foreskin preputioplasty. It has been reported that
A Zaontz urethral stent (Cook Medical) is preputioplasty is possible in most cases of hypo-
inserted one size smaller than the urethroplasty, spadias repair; however other reports suggest
and the larger funneled tip of the stent is sutured increased risk of complications [66, 67]. The
loosely to the glans meatal lip on each side with a decision to perform preputioplasty should be
6-0 Prolene taper needle BV. The distal stent is based on multiple factors including family
funnel shaped to softly dilate and conform to the request, outer foreskin, and inner preputial skin
shape of the neomeatus (Fig. 26.10a, d). The availability and developmental appearance of the
stent is comfortable for patients and easy for foreskin. Insufficient dorsal shaft skin, eccentric
446 M. Rich
torsion of the foreskin, deficient inner preputial (18), Mathieu (10), and Koff urethral mobiliza-
skin with a small hood, or a large glans will tion (2 cases). Two complications occurred in the
potentially increase foreskin complications from TIP group (6%) with one glans dehiscence (3%)
preputioplasty. Most cases of distal hypospadias associated with a flat glans configuration and
are performed with circumcision and have suffi- shallow urethral plate. The complication was
cient circumferential keratinized foreskin for repaired with a meatal-based flap and Barcat
symmetric circumferential coverage without modification [69]. One complication of distal
flaps. I excise a thin keratinized ventral skin that urethral fistula (3%) was repaired by primary
is less resilient and esthetic and prone to irrita- closure.
tion, erythema, chafing, and excoriation. A resil- Postoperatively I remove the urethral stent in
ient thicker keratinized dorsal skin is incised in 7–10 days and follow-up patients after stent
the midline to the level of the submucosal coronal removal in 1 month to assess early complications
edge, and rotational Byars flaps provide adequate and lyse any residual soft filmy penile adhesions.
ventral shaft skin coverage and recreation of the Patients are followed up every 6–12 months until
median raphae if possible. completing toilet training. After toilet training
bladder scans and uroflowmetry are obtained
when possible with follow-up every 2–3 years
26.6 Surgical Dressing through puberty.
The penis is dressed with Opsite Flexifix clear

waterproof biofilm membrane dressing coated 26.8 Reoperative Urethroplasty
with an acrylic adhesive. The clear dressing is
extensible and gently contours to the shape of the The decision to perform TIP after complicated
penis and glans. The dressing is applied without TIP is based on similar surgical principles of
constriction to allow for expected postop swell- evaluation and preservation of the urethral plate
ing and allows drainage of urine and body fluids. [70, 71]. Patients with complications from meatal
The dressing usually loosens within 1–3 days stenosis often present with obstructive voiding
from urine and daily soaking in a warm bath for complaints previously managed as dysfunctional
10–15 min after 3 days (Fig. 26.10e). voiding. Video analysis of the urine stream often
demonstrates a fine, pressured stream typical of
meatal stenosis even in asymptomatic cases [72].
26.7 Results In more severe cases presenting with large post-
void residuals and obstructive uroflowmetry,
Midline incision of the urethral plate and TIP VCUG demonstrates thickened detrusor changes
reliably creates a more esthetic vertical slit-like and urethral meatal stricture (Fig. 26.13a, b). A
meatus and has been adopted worldwide as the urethrotomy is performed to allow normalization
preferred option for midshaft and distal hypospa- of urination until bladder and urethral function
dias. Meta-analysis of TIP reports lower compli- improves before revision of the hypospadias
cation rates in primary distal hypospadias with (Fig. 26.13c). It has been proposed that overzeal-
mean rates of meatal stenosis at 3.6% (range ous distal tubularization of the urethral plate and
1.7–7.4%), urethral stricture rate at 1.3% (range inadequate midline incision may contribute to
0.8–2.2%), and fistula rate at 5.7% (range 4.0– meatal stenosis. In redo cases, I have found evi-
8.2%) and reoperation rate at 4.5% (range 2.7– dence of inadequate midline incisions without
7.5%) [68]. Series incorporating technical ingrowth of epithelium or granulation tissue
modifications with greater volume of TIP have (Fig. 26.13d). In cases of reoperative TIP, the UP
reported lower fistula rates at 2% [24, 30]. From epithelium is thicker with variable underlying
2017 to 2019, I performed 60 primary distal spongiofibrosis. Debridement, thinning, and
hypospadias repairs: TIP (30 cases), MAGPI unfurling the thick UP edges facilitates TIP re-
a b c d
Fig. 26.13 (a) Meatal stenosis. (b) VCUG thick, irregular bladder detrusor, large post-void residual, and distal urethral
stricture. (c) Postoperative urethrotomy. (d) Midline incision (arrows) completely healed from previous TIP
a b c
Fig. 26.14 (a) Redo TIP repair with combined Mathieu-TIP. (b) 2-Week postoperative result and meatus appearance
vertical slit-like. (c) Postoperative repair 3 months
tubularization. If the UP is thick, rigid, and less midline between the tips of the corporal bodies
extensible, it is preferable to perform a buccal creating a deeper furrow to provide greater poten-
graft inlay or combined meatal-based flap to aug- tial space and surface area for inlay graft aug-
ment the urethroplasty (Fig. 26.14a–c) [54, 55]. mentation, less tension with glansplasty closure,
In redo cases more extensive and deeper full- and decreased risk of recurrent stricture and glans
thickness incision of the urethral plate is per- dehiscence (Fig. 26.15).
formed to the level of the corpora, and the
excision of spongiofibrosis prepares a healthy
base for the inlay graft. In flat glans configuration 26.9 Conclusion
with a shallow groove, I have observed the cylin-
drical shape of the distal corporal bodies within Midline incision of the urethral plate and subse-
the glans cap is flattened and blunted. I suspect quent TIP urethroplasty has become widely
the shape of the distal corpora affects the mor- accepted as the preferred technique for repair of
phologic configuration of the flat glans and ure- midshaft and distal hypospadias. No single tech-
thral plate. In redo cases with a flat glans and UP, nique is a universal panacea for all variants of
after excision of spongiofibrosis, I incise in the distal hypospadias, and experience teaches by
448 M. Rich
a b c
Fig. 26.15 (a) Redo tip with buccal graft inlay, harvest poral depth and increase the surface area for graft
buccal graft. (b) Full-thickness incision of the urethral augmentation. Graft sutured to the urethral plate and
plate, excision of subplate spongiofibrosis, midline inci- quilted to depth of furrow between corpora (c) redo TIP
sion dissection between corporal bodies to create intercor- repair complete
failure and success the limitations of any opera- 3. Thiersch K. Ueber die Enstehungsweise und opera-
tion. Sir William Osler stated (1903) “There is no tive Behandlung der Epispadie. Arch der Heilkd.
1869;10:20.
more difficult art to acquire than the art of obser- 4. Snodgrass W. Tubularized, incised plate urethroplasty
vation.” The midline incision and TIP concept for distal hypospadias. J Urol. 1994; https://doi.
arose from knowledge of historical surgical tech- org/10.1016/S0022-5347(17)34991-1.
niques incorporating TD urethral tubularization, 5. Snodgrass W, Bush N. Primary hypospadias repair
techniques: a review of the evidence. Urol Ann. 2016;
modern era distal hypospadias techniques pre- https://doi.org/10.4103/0974-7796.192097.
serving the urethral plate, crossover techniques 6. Cook A, Khoury AE, Neville C, Bagli DJ, Farhat
learned from endourologic and pediatric surgical WA, Pippi Salle JL. A multicenter evaluation of
specialization, and observation that a small technical preferences for primary hypospadias
change could alter the algorithm for distal hypo- ju.0000180643.01803.43.
spadias repair. What is often branded as new is 7. Orkiszewski M. Tubularized incised plate repair, also
nothing more than an amalgamation of similar known as the Snodgrass procedure. J Pediatr Surg.
historical ideas and innovations that have been 2006;41:1786.
8. Wilkinson DJ, Farrelly P, Kenny SE. Outcomes in dis-
molded, or in the case of TIP, hinged into some- tal hypospadias: a systematic review of the Mathieu
thing else. A free flow of ideas is the fundamental and tubularized incised plate repairs. J Pediatr Urol.
essence of “hypospadiology” as we evolve and 2012; https://doi.org/10.1016/j.jpurol.2010.11.008.
build upon the work, knowledge, wisdom, and 9. Anon. Smith’s textbook of endourology; 2019. https://
doi.org/10.1002/9781119245193.
accomplishments of others. 10. Badlani G, Eshghi M, Smith AD. Percutaneous sur-
gery for ureteropelvic junction obstruction (endopy-
elotomy): technique and early results. J Urol. 1986;
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Dorsal Inlay TIP (DTIP)
27
Tariq O. Abbas and Joao Luiz Pippi Salle
Abbreviations However, some pediatric urologists have ques-

tioned this proposed healing process, based on
DIG Dorsal Inlay Graft observations that tubularization of narrow ure-
DTIP Dorsal Inlay TIP thral plates results in higher incidence of urethro-
POST Plate Objective Scoring Tool cutaneous fistulas and stenosis [4–6]. These
TIP Tubularized Incised Plate findings suggest that at least part of the healing
UP Urethral Plate process consists of fibrosis and retraction of the
incised area, leading to progressive tension and
fistula development [6, 7]. Placement of inner pre-
putial or oral mucosa graft (dorsal inlay graft)
27.1 Introduction over the incised area has been suggested to avoid
this physiological mechanism [8–11]. Supporters
Hypospadias remains one of the most challenging of dorsal inlay TIP (DTIP) hypothesize that more
congenital urological abnormalities. This anom- adequate healing occurs in the presence of the
aly presents various associated deformities such graft, resulting in a neourethra with wider caliber,
as ventral curvature, urethral hypoplasia, skin better flow, and less stenosis [9, 12]. We attempted
deficiency, etc. which poses specific difficulties to to test this healing process experimentally, adding
be concurrently corrected. Several surgical proce- oral graft to the TIP urethroplasty, in the rabbit
dures have been introduced, but none fulfills the model, aiming to demonstrate improvement of
ideal criteria for repair; therefore, no single tech- flow and urethral elasticity. Our data, however,
nique can offer the best treatment for all. The although improving both parameters, did not
tubularized incised urethroplasty (TIP), popular- reach statistical significance likely because of the
ized in 1994 by Warren Snodgrass, was quickly number and animal urethral characteristics as
adopted by many surgeons worldwide to repair well as a short postoperative period of observation
distal hypospadias, initially even for proximal [13, 14]. Despite the debatable point that grafting
cases [1, 2]. Snodgrass and others hypothesize the TIP is unnecessary, we believe that, based on
that the incised dorsal urethral plate undergoes our own experience, DTIP is indicated for unfa-
complete epithelialization of the raw area [3, 4]. vorable urethral plates (defined as narrow, inelas-
tic, and with poor spongiosum), adding positively
T. O. Abbas · J. L. Pippi Salle (*) to the repair of hypospadias without or with mild
Division of Urology, Department of Pediatric curvature. In order to standardize our indications,
Surgery, Sidra Medicine, Sidra Medical and Research we developed an objective criterion to define ure-
Centre, Doha, Qatar
e-mail: psalle@sidra.org thral plate characteristics: the plate objective scor-
452 T. O. Abbas and J. L. Pippi Salle
ing tool (POST) score [15]. Intrinsec elements of 27.2 Preoperative Evaluation
the urethral plate (UP) should be assessed. Point
A is the distal muco-cutaneous junction; point B When evaluating a case of hypospadias, a trans-
is at the point is the glanular/coronal junction parent dialogue with the parents should take
(Fig. 27.1). Based on our preliminary results, an place. They should be instructed that the final
AB/BC ratio lower than 1.2 configures an unfa- decision about performing the procedure in one
vorable urethral plate, and, in such cases, we pro- or in stages will be made in the operating table,
pose to perform a DTIP rather than a TIP repair. once the penis is carefully examined under gen-
Our hypothesis is supported by the embryological eral anesthesia as well as an erection test is per-
background of glanular urethral development that formed. If a very small penis is encountered at
suggests that the arrest of distal glanular urethral the initial clinical evaluation, we do not hesitate
closure would be accompanied by flat urethral to administer testosterone, 2 mg/kg, IM, 90, 60,
plate [16, 17]. Herein we describe in detail the and 30 days before the operation. If the patient is
steps of the DTIP technique.
a b
Fig. 27.1 (a) POST unfavorable UP characteristics: ame- ous junction; (B) glanular knob where the mucosal edges
nable to DTIP. (b) POST favorable UP characteristics: of the UP change direction; (C) glanular/coronal junction
amenable to TIP repair. (A) Distal midline mucocutane- was marked and the AB/BC distances ratio calculated
27 Dorsal Inlay TIP (DTIP) 453
healthy and both testes are in scrotal position, vein and whatever other structures found in the
there is no need for preoperative imaging tests. midline are displaced laterally, exposing the
The patient is admitted on the day of surgery. tunica albuginea at 12 o’clock position. Then two
Either a caudal or penile block is performed in all parallel transverse incisions are done in the albu-
patients. Once the IV line is established, IV anti- ginea at the level of maximal curvature. Inverted
biotic is administered (cephalosporin). The goal plication stitches of 5-0 polypropylene are placed
for all patients undergoing hypospadias repair is over the small incisions in order to plicate the
to create a slit-like meatus at an appropriate loca- albuginea in that area [18] (link of midline dorsal
tion on the glans. plication “click here”). In cases of severe curva-
ture (>45 degrees), poor spongiosum (long hypo-
plasia), or very small penis, the urethral plate is
27.3 Operative Technique divided, and a staged repair is planned as
described in another chapter of this book. In such
27.3.1 P
rimary Distal Hypospadias cases ventral lengthening using deep, transverse
Repair incisions of tunica albuginea (DTITA) is per-
formed [19]. The erection test is then repeated to
27.3.1.1 Penile Degloving confirm the resolution of the curvature.
and Preparation
of the Ventral Dartos Flap 27.3.1.3 Urethroplasty
After adequate prepping and draping, a traction Once the curvature is repaired, it is time to pro-
suture of 5-0 polypropylene is placed in the mid- ceed with the DTIP. Xylocaine with epinephrine
line glans, well beyond the expected dorsal end (1:100,000) is injected in the spongiosum of the
of the neomeatus. An 8F feeding tube is passed to glans and sub-coronal area, underlying the
assess the urethra and bifurcation of the spongio- marked incision line. We do not use the tourni-
sum in order to characterize the presence and quet routinely, unless there is significant bleeding
degree of urethral hypoplasia. The penis is that precludes good visualization of the penile
degloved, being sure the correct plane is encoun- structures. In the absence of chordee, or minimal
tered to avoid bleeding (Figs. 27.2a, b and 27.3a). curvature (<30 degrees), a U-shaped incision is
Bipolar cautery is used, if bleeding is encoun- done, starting from the glanular knobs (point B),
tered. An erection test, without the use of a tour- defining the ventral lib of the neomeatus and
niquet, is performed in all patients, infusing extending 1–2 mm proximal to the hypospadiac
normal saline through a large bore butterfly nee- meatus (at a non-hypoplastic territory). A ventral
dle (#21 gauge) (link erection test “click here”). dartos flap is fashioned at this time, if a distal
If chordee is present, it should be measured with repair is anticipated. It should be dissected in the
a goniometer or taking a lateral picture and using exact plane, just above the spongiosum, leaving
an mobile application, to delineate the exact with a wide base to maximize blood supply
degree of ventral curvature (Fig. 27.4). (Fig. 27.2e) (link of the ventral dartos “click
here”).
27.3.1.2 Chordee Correction The urethral plate is colored with a marking
The surgeon then carefully evaluates the situation pen in order to better visualize its mucocutaneous
about performing dorsal plication or a ventral edges. Once the plate is colored and stretched,
lengthening procedure. In cases of distal hypo- the distance between the two glandular knobs,
spadias where the erection test revealed mild cur- where the urethral plate changes direction in the
vature (<30 degrees) or moderate, with normal glans, is measured (link coloring and incising the
size penis and good spongiosum all the way to UP “click here”) and making the decision
the meatus (30–45 degrees), a midline dorsal pli- between TIP and DTIP (Fig. 27.5). A deep mid-
cation is performed. A midline incision on the line incision of the UP, starting at the distal
Buck’s fascia is performed. The dorsal penile mucocutaneous point is made, all the way to the
a b
c d
e f g
Fig. 27.2 (a) Sub-coronal skin incision. (b) Penile pleted urethroplasty. (f) Protective layer from the ventral
degloving. (c) Incision of the UP. (d) Four-point fixation penile dartos fascia. (g) Glanuloplasty and skin closure
of the inner preputial graft into the incised UP. (e) Suturing (courtesy of Ahmed T. Hadidi)
of the inlay graft using subepithelial stitches and com-
corpora cavernosa, similar to the TIP technique. arbitrarily consider that if the ratio of measure-
The UP width is measured again, after the inci- ments before and after incision is less than 0.5,
sion is done, therefore allowing to calculate the there is an indication for DTIP, assuming that a
urethral plate ratio before and after incision. We higher risk of stenosis may occur due to a large
a b c
d e
Fig. 27.3 (a) Distal end of the urethral plate mucocuta- DTIP. (b) Following degloving, preparation of the dartos
neous junction (point A). Glanular knob where the muco- flap, the colored urethral plate was deeply incised to the
sal edges change direction and determine the beginning of level of the corpora. (c, d) The inner preputial graft
the incision (point B). Glanular/coronal junction (point sutured over the incised area. (e) The skin closed with
C). As AB distance is shorter than BC, we performed subcuticular stitches and catheter secured in place
proportion of the neourethra being constructed 27.3.1.4 reputial Graft Harvesting

P
based on raw area, therefore vulnerable to fibro- and Fixation
sis [20, 21]. Such a maneuver helps to standardize A proportional elliptical area of the stretched
the decision-making process to graft or not the inner prepuce is marked and incised, removing
incised area [21]. If the decision is made to per- the dartos found underneath. The graft is then tai-
form DTIP, then we further extend the midline lored adequately to cover the incised raw area
incision of the UP all the way to the vertex of the loosely. To facilitate this part of the procedure, it
glans, where the neomeatus will be located, as is important to initially fix the graft with four
suggested by Jayanthi et al. [22]. The whole raw angular stitches: one at the proximal point of the
area is then covered and quilted with defatted incision, two at the knobs where the glans was
inner preputial graft. Failure to incise and graft initially incised, and one at the vertex. This
the UP above the mucocutaneous junction, all the maneuver immobilizes the graft, expediting
way to vertex of the glans, increases the risk of suturing it to the edges of the raw surface as well
meatal stenosis [9, 12, 23, 24]. as quilting it (Fig. 27.2c, d). The graft does not
27.3.1.5 Glanuloplasty
Glans wings are approximated with subcuticular
sutures of 6-0 or 7-0 polyglactin, producing a
solid bridge of glans below the neomeatus
(Figs. 27.2g and 27.3e). This maneuver is impor-
tant to put the meatus in the “normal” position as
suggested by Babu et al. [25], Dhua et al. [26],
and Abbas et al. [27]. Transcutaneous stitches
should never be used for glans approximation as
they produce cosmetically unappealing marks
that are very difficult to correct later. Catheter
fixation is done with 5-0 Prolene using a tech-
nique that avoids parameatal marks and prevents
its migration (link of catheter fixation, and
removal “click here”).
27.3.1.6 Skin Closure

It is very important to produce a normal-looking
penis without inadequate marks or redundant
skin. Unfortunately, skin closure is often
neglected, despite being one of the most chal-
lenging aspects of hypospadias repair. Sometimes
an adequate skin closure can be very difficult,
especially in cases where there is asymmetry or
hypoplasia. Rotational preputial flaps are mobi-
lized and approximated in the midline whenever
Fig. 27.4 Erection test without the use of tourniquet, possible, creating a median raphe. Subepithelial
infusing normal saline with large bore butterfly #21. stitches are used throughout the closure to avoid
Ventral curvature measured with the application “Angles”R formation of suture tracks. A Tegaderm is applied
over the penis and covered with Coban® in a
necessarily need to be sutured to the mucosal slightly compressed fashion. The stent is left
edges of the incision, but below, in a subepithelial draining freely in diapers and removed approxi-
fashion. This is an essential point of technique in mately 10 days later.
cases of narrow urethral plates (Figs. 27.2d and
27.3d). Placement of penile tourniquet during the
graft suture minimizes blood oozing, optimizing 27.3.2 Reoperations
visualization in this meticulous part of the proce-
dure. The glanular wings are then well mobilized 27.3.2.1 Preoperative Evaluation
laterally, taking care to avoid injury of the muco- Unfortunately, despite all surgeon’s efforts, a
sal collar. An 8F Silastic stent is passed into the small proportion of patients may develop compli-
bladder, and 7-0 PDS running suture is used for cations that require reintervention for correction,
the first layer of the urethroplasty, starting at the most commonly urethra-cutaneous fistulae,
distal corners of the incised plate. An inter- recurrence of ventral curvature, urethral/meatus
rupted second layer using 7-0 Vicryl is then per- stenosis, or unappealing cosmesis. The single
formed. The whole urethroplasty is then covered most important determining factor for the subse-
with previously prepared ventral dartos flap quent redo steps is the degree of curvature found
(Figs. 27.2e and 27.3d). after degloving as described above. If minimal or
Fig. 27.5 Algorithm for primary distal hypospadias repair
no chordee is appreciated, and there is still a pli- gently, avoiding injury to the underlying muscles.
able piece of the urethral plate, a single-stage Upper lip grafts are usually thin and easily defat-
redo is feasible, using a DTIP technique as ted, which is ideal for placement in the glanular
described for primary repairs but using oral area [29]. Hemostasia is secured using cautery,
mucosa graft [28]. If severe chordee is present, a and the donor site is left open, without any suture.
staged hypospadias repair is warranted The graft is then sutured to the incised plate using
(Fig. 27.6), as described in Chapter 40. the same DTIP technique described above. Most
redo cases have redundant ventral dartos that can
27.3.2.2 Urethroplasty be fashioned as a flap to cover the neourethra. If
The choice of oral mucosa donor site will depend this is not available, we do not hesitate to harvest a
on the amount of tissue needed. Most cases of redo tunica vaginalis flap. This is almost always neces-
DTIP are done for complications after TIP repair; sary for proximal cases.
therefore, small segments of graft are needed to
cover the incised are. We prefer to use the upper lip
graft for the majority of such cases. Harvesting of 27.4 Results
the upper lip graft is rather simple and follows
general principles. The upper lip is retracted using There is no clear evidence to prove the superior-
stay 3-0 Prolene sutures to expose the inner ity of DTIP over TIP [30]. Based on principles
mucosa that is infiltrated with lidocaine 1% with introduced above, we believe DTIP allows a
epinephrine. The donor site outlined with marking more orthotopic positioning of the neomeatus
pen, sparing the frenular area. The graft separated that, lined with a graft, is potentially less vulner-
Fig. 27.6 Algorithm for redo hypospadias repair
able to stenosis [31]. However, the indications for cases of unfavorable hypospadias (defined as
DTIP should be defined carefully as the proce- small glans, narrow and flat plates) and 44 redo
dure is technically demanding and time- cases. In this particular population, no compli-
consuming. We tried to define and describe the cations was observed in primary cases although
criteria to serve a road map for decision-making 8/44 (18%) of redo cases developed problems
process. [32]. A recent systematic review by Alshafei
There are few publications presenting the et al. showed a trend toward more complications
outcomes after DTIP. Gupta et al. [31], in an following TIP compared to DTIP, developing
good prospective study of 263 boys, described urethro-cutaneous fistulae in 9.4% (33/350) and
the outcomes of undergoing DTIP aiming to 4.9% (13/267) after TIP and DTIP, respectively.
achieve a orthotopic, slit-like meatus at the tip Meatal stenosis occurred in 21.2% and 4.1%
of the glans. This study indicated that DTIP is after TIP and DTIP, respectively [30]. As
an excellent technique with only 10/263 pts. observed in 17 patients in Gupta’s series [31],
(3.6%) developing urethra-cutaneous fistulae prolapse through the meatus may occur if an
and only one case of distal urethral stenosis oversized and bulky graft is used for inlay;
which resolved with urethrotomy. The authors therefore adequate thinning and re-sizing is rec-
achieved an excellent cosmetic score by HOSE ommended to optimize the cosmesis of the neo-
score in 96% of cases. Ferro et al. reviewed his meatus. A summary of reported outcomes is
experience with DTIP, reporting 18 primary presented in Table 27.1.
Table 27.1 Outcomes of DTIP in primary and redo surgeries

No. of
Author Year Type cases Follow-up UCF Stenosis Total incidents
Kolon et al. [8] 2000 Primary 32 21 months 0 0 0
Singh et al. [24] 2004 Primary 25 Minimum of 9 months 4 (16%) 0 8 (23%)
Gundeti et al. [11] 2005 Primary 14 18 months 1 (7.1) 0 3 (21.4%)
Asanuma et al. [33] 2006 Primary 28 22 months (13–31) 1 (3.6%) 0 1 (3.6%)
Ferro et al. [32] 2009 Primary 18 57 months (6–122) 0 0 0
Redo 44 25 (4–120) 4 (9%) 2 (5%) 8 (18%)
Hayes et al. [34] 2009 Redo 3 1–12 months 0 0 0
Schwentner et al. [28] 2010 Redo 31 78.45–18.18 months 0 0 4 (12.9%)
Shimotakahara et al. [12] 2011 Primary 50 3.6 years 3 (6%) 1 (2%) 4 (8)
Mouravas et al. [9] 2014 Primary 24 3.2 years (2–5) 1 (4.2%) 0 2 (8.3%)
Shuzhu et al. [35] 2016 Primary 160 18 months (6–24) 5 (3.1%) 7 (4.4%) 12 (7.5%)
Gupta et al. [31] 2016 Primary 263 17 months (6–22) 10 (3.7%) 1 (0.3%) 11 (4%)
Helmy et al. [36] 2018 Primary 30 1 year 0 0 2 (6.7%)
27.5 Conclusion 6. Elbakry A, Hegazy M, Matar A, Zakaria

A. Tubularised incised-plate versus tubularisation of
an intact and laterally augmented plate for hypospa-
DTIP is an excellent alternative for hypospadias dias repair: a prospective randomised study. Arab J
repairs with mild or moderate curvature and Urol. 2016;14:163–70.
unfavorable UP. We believe the indications of 7. Hadidi AT. Functional urethral obstruction follow-
ing tubularised incised plate repair of hypospa-
DTIP will expand in the near future as more and dias. J Pediatr Surg. 2013; https://doi.org/10.1016/j.
more surgeons are experiencing similar good out- jpedsurg.2012.10.071.
comes, especially in unfavorable UPs. Hopefully, 8. Kolon TF, Gonzales ET. The dorsal inlay graft
high-quality studies will prove our understand- for hypospadias repair. J Urol. 2000; https://doi.
org/10.1016/S0022-5347(05)67603-3.
ing, providing clear indications for TIP or DIG in 9. Mouravas V, Filippopoulos A, Sfoungaris D. Urethral
the future. plate grafting improves the results of tubularized
incised plate urethroplasty in primary hypospadias. J
Pediatr Urol. 2014;10:463–8.
10. Silay MS, Sirin H, Tepeler A, Karatag T, Armagan A,
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doi.org/10.1016/S0022-5347(17)34991-1. of an inner preputial free graft to extend the indica-
2. Snodgrass WT, Bush N, Cost N. Tubularized incised tions of Snodgrass hypospadias repair (Snodgraft).
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jpurol.2013.11.008.
Urethral Advancement
for Treatment of Distal 28
Hypospadias
Emir Q. Haxhija and Patrick McKenna
Abbreviations appearance of the foreskin, the common down-

ward deviation of the urinary stream, and last but
DYG Double Y Glanulomeatoplasty not least the stenotic appearance of the hypospa-
MAGPI Meatal Advancement and diac meatus, which usually urges parents to seek
Glanuloplasty Technique treatment for their children with glanular, coro-
TIP Tubularized Incised Plate nal, and/or subcoronal hypospadias, responsible
for around 70% of all boys with hypospadias [3].
The correction of distal hypospadias has the
ultimate surgical goals of (1) straightening of the
28.1 Introduction penis by correction of the penile curvature, (2)
reconstruction of the absent most distal part of
Distal hypospadias comprise the largest group of the urethra, (3) formation of the neomeatus at its
children with hypospadias (Fig. 28.1). These normal position at the tip of the glans, and (4)
forms are generally considered as mild forms of circumcision or foreskin reconstruction, depend-
hypospadias because they are not associated with ing on the availability of tissues and parent’s
the physical inability of having a successful sex- wishes. Seldomly also penile rotation must be
ual intercourse and/or being unable to reproduce corrected. These goals seem easy to achieve, but
[1, 2]. Despite the fact that mild penile curvature it is exactly the surgical failure which led to
is occasionally present also in distal hypospadias, despair of both parents and surgeons and there-
the existence of true fibrous chordee is rare, and fore description of many surgical techniques for
the potential need for division of urethral plate correction of distal hypospadias and innumerable
for correction of the curvature would be extremely modifications of those during the past 150 years
unusual. Having stated this, it is the hooded [4–10]. Boys with distal hypospadias often
needed many surgical interventions instead of
only one, and in many cases a worse situation had
E. Q. Haxhija (*) finally to be accepted than was the initial situa-
Department of Pediatric and Adolescent Surgery,
Medical University Graz, Graz, Austria tion prior to the first surgery [5]. Because of this,
e-mail: emir.haxhija@medunigraz.at not all patients with distal hypospadias were
P. McKenna offered surgical correction in the past. However,
Department of Mercyhealth Hypospadias, the abnormally situated urethral meatus and the
Center Javon Bea Womens and Children’s Hospital, hooded appearance of the foreskin often cause
President Societies for Pediatric Urology, psychological stress in boys at latest in their ado-
Rockford, IL, USA
e-mail: pmckenna@mhemail.org
462 E. Q. Haxhija and P. McKenna
Fig. 28.1 Coronal and subcoronal hypospadias examples these examples, no significant chordee is present, and they
with nice distal urethra. Notice in all three cases a good all had a successful hypospadias surgery by urethral
skin coverage of the distal urethra, leaving an impression advancement
of well-developed corpus spongiosum. Furthermore, in all
lescence, leading to later requests for surgical wide, a number of institutions where urethral
correction [6, 7]. advancement was a standard for repair of distal
The main complications of hypospadias repair hypospadias continued to perform urethral
with the most common techniques of tubulariza- advancement on a regular basis [15, 23–27]. The
tion of the local tissues, like Thiersch-Duplay main advantages of urethral advancement were
[8, 9] or Mathieu [10], are fistulas, stenoses, and/or defined as no need for reconstruction of neoure-
repair disruption. The high incidence of such com- thra; minimal risks of stricture, fistula, and/or
plications requiring further surgical interventions meatal stenosis; and often no need for postopera-
have been the basis for the idea of the urethral tive catheter placement. Even though editorial
advancement technique described firstly by Beck comments on the excellent results in these reports
in 1897 [11]. Shortly after the first description of praised the value of the urethral advancement
this technique, other authors reported good results technique, still they were consistently reluctant to
with its use in boys of various ages [12, 13]. support the general use of this technique for boys
Furthermore, excellent results with the use of the with distal hypospadias. The editors pointed out
urethral advancement were repeatedly reported the high potential dangers of inadequate length
throughout the last century and even in the first gain after urethral mobilization due to dysplastic
decades of the twenty-first century, making this urethra, ventral shortening, curvature, and ure-
technique a valuable option in the armamentarium thral injury and that this technique indeed
of surgical procedures published for the correction requires meticulous and extensive dissection of
of distal hypospadias [14–28]. the urethra for a relatively short distance to be
Since the first description of the urethral bridged which is much easier done by other tech-
advancement by Beck [11], this technique has niques applying local skin flaps [14, 17, 25–27].
been postulated to be easy to perform and to In some cases such editorial skepticism was also
deliver excellent results as a single-stage repair based on editor’s personal bad experience with
technique for boys with distal hypospadias. Even the technique of urethral advancement [14, 17].
after the introduction of the tubularized incised In one of the largest recent reports on the
plate urethroplasty [29], which due to its simplic- results of urethral advancement in 158 cases, the
ity and excellent immediate results spread world- authors claimed this technique to be excellent for
28 Urethral Advancement for Treatment of Distal Hypospadias 463
both primary and redo cases, in children and ado- incision of the ventral penile skin up to the
lescents, reporting an overall incidence of post- penoscrotal junction is performed. A subcuta-
operative fistula of 0.6%, incidence of neous injection of a 1:200,000 epinephrine
postoperative chordee of 0.6%, and incidence of solution can be used to facilitate local vaso-
meatal stenosis or retraction of 6% at 2-year fol- constriction and therefore reduce bleeding
low-up [27]. Comparison with other publications during the dissection (Fig. 28.2).
on urethral advancement shows the incidence of • A transverse subglanular and oval submeatal
postoperative chordee of 0–11%, incidence of incision is made, the dartos fascia is mobilized
meatal stenosis and/or retraction of 0–25%, and together with the ventral skin on both sides of
incidence of postoperative fistula of 0–2.1%, the midline, and the superficial chordee is
with the majority of papers reporting the “0” side removed so that the urethra is freely mobilized
of the range [14, 15, 17–27]. to the penoscrotal junction. The meatus is then
Haberlik et al. [15] modified Beck’s technique incised with the scissors in the oval longitudi-
by using a zick-zack opening of the ventral penile nal way, lifting the glanular urethral plate tis-
skin, reducing the possibility of postoperative lon- sue between 11 and 1 o’clock together with the
gitudinal scar formation with excellent long-term meatus. A traction suture is applied to the
cosmetic results. In addition, no tunneling of the meatus. The urethra with the surrounding cor-
glans tissue was performed but formation of two pus spongiosum is then mobilized from the
widely mobilized glanular wings with adequate corpora cavernosum. It is extremely important
excision of glanular tissue in between the wings to to enter immediately the correct plane for ure-
accommodate for mobilized urethra. thral dissection, because that plane is avascular
and allows easy and safe urethral mobilization.
In some cases it is easier to enter the right
28.2 Operative Technique plane by mobilizing the urethra from its sides
as shown by Waterhouse and Glassberg [17].
• At the beginning of the operation, a glans This makes the mobilization of the proximal
suture is placed for retraction, and an urinary urethra to the bulbar part easier (Fig. 28.3).
catheter is put into the bladder. Alternatively, a • Once the urethra is fully mobilized, the erec-
metallic urethral bougie can be placed to facil- tion test can be performed and any deep chor-
itate urethral mobilization. A zick-zack or dee may be easily removed from the ventral
alternatively longitudinal submeatal midline side of the exposed corpora. Glanular wings
Fig. 28.2 A case of recurrent hypospadias with subcoro- the ventral penile skin is performed after a subcutaneous
nal fistula. A metallic urethral bougie is placed to facilitate injection of a 1:200,000 epinephrine solution
urethral mobilization. A zick-zack submental incision of
Fig. 28.3 The meatus is incised ovally, a traction suture is applied, and the urethra is then meticulously sharply mobi-
lized from the corpora in the correct avascular plane
Fig. 28.4 The glanular wings are widely dissected; a part from both sides of the glanular wings to make space for
of the glanular tissue at the tip of the glans is excised to the mobilized urethra. The neomeatus is sutured to the tip
accommodate the neomeatus. A strip of glanular tissue is of the glans
removed longitudinally from the midline of the glans and
are now widely dissected, eliminating any 6-0, attaching the urethra to the glans at 4 and
glanular tilt. A strip of glanular tissue is 8 o’clock, paying attention that the sutures are
removed longitudinally from the midline of on the inner aspects of the glans. In this way
the glans to make space for the urethra and the meatus takes a vertical shape in the glanu-
allow tensionless closure of glanular wings lar groove. Next, 3-4 supportive stitches 7-0
over the urethra. After oblique excision of the can be placed between the spongiosal urethral
most distal part of the mobilized urethra, the tissue and the tunica albuginea in the midline
dorsal part of the urethra is sutured with three to fix the urethra to the corpora. The dartos
interrupted stitches 6-0 to the tip of the glans fascia is reconstructed from proximally to dis-
at 11, 12, and 1 o’clock (Fig. 28.4). tally covering the urethra. The ventral penile
• The glanular wings are wrapped around the skin is reconstructed tensionless, mobilizing
new urethra and sutured together with three the skin laterally if needed so that there is
interrupted or back-and-forth stitches using enough penile skin on the ventral side of the
6–0 sutures. In the most distal glanular suture, straight penis (Fig. 28.5).
spongiosal urethral tissue is incorporated at 6 • After completion of the operation, the penis
o’clock without entering the urethra. The neo- looks almost normal with the meatus at the tip
meatus is completed with two more sutures of the glans and the preputial skin surrounding
Fig. 28.5 The glanular wings are closed over the urethra, the tunica dartos is reconstructed, and the ventral penile skin
is closed
the glans only dorsally. At this stage a circum-

cision can be performed, which makes this
procedure a potential single-step repair. If
parents wish a foreskin reconstruction, and the
tissues left allow for it, we suggest doing it in
a due time in the frame of any other potential
surgery or as a two-stage procedure. We hesi-
tate to cover the fresh glanuloplasty with the
foreskin. The indwelling catheter can be
removed immediately postoperatively or left
in place for up to 5 days depending on the
preference of the surgeon.
28.3 Discussion
One of the characteristics of this technique is the

lack of need for complete degloving of the penis.
One will notice that solely surgical steps on the
ventral penile side are described. A complete
degloving of the penis is of course possible at any
time also during this procedure. However, there
is no need to have a completely degloved penis to
be able to perform a successful erection test, if
Fig. 28.6 Division of the corpus spongiosum (arrows)
that would be the only reason for complete penile
over the most distal part of the urethra (circle) in a patient
degloving. with coronal hypospadias
Careful selection of cases for this technique is
needed. The urethra can be elongated to bridge a
gap of up to 1.5 cm but only in well-developed coronal hypospadias but with a very thin distal
penis (Fig. 28.1). In some cases the corpus spon- urethra with lack of corpus spongiosum over
giosum is divided in the distal part (Fig. 28.6). more than 1 cm, usually presenting clinically
Also, in such cases, the urethra can be nicely with a very thin ventral skin over the distal
completely mobilized and the corpus spongio- urethra, it is better to use another technique for
sum sutured in the midline. In patients with sub- hypospadias repair because one can be con-
fronted with insufficient length gain for intended Rockford, IL, USA
repair due to hypoplastic urethra. Although, also I appreciate the opportunity to comment on
in such cases, a single-step repair is possible by the urethral mobilization technique.
combining urethral mobilization with another As pointed out in the chapter, rarely are there
technique, for example, the preputial skin tubu- new procedures in reconstructive surgery.
larization, as described by Duckett [1]. Urethral mobilization even predates the early
Alternative option would be creating a distal article cited.
hypospadias situation which has to be corrected The urethra is a unique organ, actually made
at the second stage by one of the known flap up of two structures, the urethra and the spongio-
techniques, such as Mathieu [10, 26]. In addi- sum. The characteristics that make it unique are
tion, other alternatives for such situations have its mucosal lining, elasticity, and blood supply.
been described [30, 31]. The mucosal lining is most important because it
The technique of urethral advancement can be holds up to urine exposure for a lifetime, but its
applied as a primary repair technique or after a elasticity and ability to be mobilized while keep-
failure of any other technique applied for correc- ing vascular integrity make it ideal for hypospa-
tion of distal hypospadias. On the other side, also dias surgery. No other structure in the body has
potential failure of urethral advancement can be similar characteristics (Fig. 28.7).
repaired by other techniques for correction of Few would argue against the urethra being the
distal hypospadias, which allows hypospadiolo- best material for urethral reconstruction, because
gists to primarily use the techniques they find that it is what was designed to be a urethra, and results
work best in their hands. in a suture-free repair. The concept of mobiliza-
Nowadays, the majority of hypospadias sur- tion was utilized during my residency for correct-
geons will prefer to use techniques which are ing urethral stricture disease by excision of the
simple to teach, simple to learn, and offer imme- stricture and urethral mobilization, both proximal
diate good results without a need for extensive and distal, followed by tension-free primary end-
urethral mobilization [32]. Only long-term evalu- to-end anastomosis. Over the last two decades, it
ation of boys after hypospadias reconstruction has become part of almost all hypospadias and
can show the success of one technique. The natu-
ral selection of the best techniques for our chil-
dren should win [33–35]. It is amazing to see that
despite of huge improvements in the microsurgi-
cal instruments, magnification, and development
of fine absorbable suturing material, all allowing
much finer tissue handling and much less possi-
bility for failure, the technique of urethral
advancement despite the fact that it has been
described more than 120 years ago still stands
strong.
28.4 ditorial Comment by

E
Dr. Patrick McKenna
Director of the Hypospadias and Urologic

Reconstructive Center Fig. 28.7 The urethra is unique and elastic, and the cor-
Mercy Health Javon Bea Women’s and pus spongiosum is part of the urethra and should be mobi-
Children’s lized with it (courtesy of Dr. McKenna)
chordee without hypospadias repairs that are both ventrally and dorsally, so it is easy to get
done. It is especially advantageous in correcting into with mobilization. The entire atretic area
stenosis from TIP procedures that represent the should be excised.
most common redo procedure in our practice. The chordee is corrected by various combina-
There are as many ways to do a procedure as tions of ventral dissection, corporal body separa-
there are surgeons, and our technique differs sig- tion, fairy cuts, and if required a dermal graft
nificantly from the technique presented: (Fig. 28.10). Dorsal plication is seldom utilized.
In most cases, the penis is degloved. There have been cases where deficits of 5–6 cm
Exceptions would be with very distal hypospa- have been made up with this approach. It can be
dias with no chordee and some redo procedures extended even farther by keeping a vascularized
with stricture and no chordee. flap in the midline of the glans and advance this
One of the key cosmetic refinements is the into the mobilized urethra. This is a composite
development of a 1.5–2-cm preputial collar repair and can result in up to two additional cen-
around the glands. The initial markings should timeters in length.
include two triangular flaps that will become the Once the mobilization has sufficient length, it
frenula repair and complete the collar ventrally. is important to make a space for the new urethra
The preputial dissection is always started on the
dorsum where the avascular plane is identified
easily and carried around ventrally, so clean cuts
can be made ventrally and the urethra avoided.
If an atretic urethra (a urethra above where
the spongiosum splays and is “saran wrap” thin)
is present, it is excised because it does not have
the elastic characteristics of the normal urethra. It
is critical to understand that the spongiosum is
part of a normal urethra. This must be kept intact
to maintain the blood supply and elastic charac-
teristics (Fig. 28.8).
The mobilization should start from the bottom
up, starting below where the spongiosum splays
where there is normal spongiosum (Fig. 28.9).
Fig. 28.9 Dissection should start proximally where the
It is important to keep the spongiosum intact corpus spongiosum is healthy and vascularized to find the
circumferentially. The atretic urethra is atretic proper plane (courtesy of Dr. McKenna)
Fig. 28.8 The distal atretic part of the urethra must be excised because it is nonelastic and not well-vascularized (cour-
tesy of Dr. McKenna)
Fig. 28.11 A part of the glans core must be excised to

create a space for the new urethra (courtesy of Dr.
Fig. 28.10 The chordee is corrected by various methods McKenna)
including corporeal body separation (courtesy of Dr.
McKenna)
Fig. 28.12 The tip of the healthy urethra is spatulated and sutured to the glanular V flap. The proximal urethra is fixed
to the body of the corporeal body to avoid tension (courtesy of Dr. McKenna)
(Fig. 28.11). This includes removing some central The glans is closed by placing a subcuticular
glans erectile tissue and skin where the urethra stitch of a long-lasting absorbable suture over a
will be reconstructed. Wide glandular flaps with a hemostat. The hemostat confirms sufficient space
hockey-stick incision are essential. The corporal in the glans with no pressure on the urethra and
bodies are incised and separated in the midline all prevents glans separation. The pre-planned fren-
the way to the tip to affect a greater upward bend ular flaps are reconstructed to provide the ventral
and provide more space for the new urethra. portion of the glans collar. Dorsal skin is rotated
The distal urethra is attached to the glans. ventrally, adding additional length to the ventral
Another crucial step is to advance the urethra skin and closed in the midline. A drippy tube is
more proximately and fix it to the corporal bodies used because it minimizes any voiding issues
taking all tension off the tip (Fig. 28.12). after surgery. In all cases a tourniquet is avoided.
Fig. 28.13 Final appearance after healing. Observe the slit-like and wide meatus (courtesy of Dr. McKenna)
after adolescence. BJU Int. 2000; https://doi.

After two decades of utilizing this option, the org/10.1046/j.1464-410X.2000.00488.x.
7. Barbagli G, Perovic S, Djinovic R, Sansalone S,
complications associated with distal repairs have Lazzeri M. Retrospective descriptive analysis of
dropped close to zero (Fig. 28.13). 1,176 patients with failed hypospadias repair. J Urol.
2010;183:207–11.
8. Thiersch K. Über die Entstehungsweise und opera-
tive Behandlung der Epispadie. Arch Heitkunde.
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The Glanular Urethral Disassembly
(GUD) Technique: An Alternative 29
to Distal Hypospadias
Antonio Macedo Jr. and Marcela Leal da Cruz
Abbreviations
GUD Glandular Urethral Disassembly

MAGPI Meatal Advancement and
Glanuloplasty Technique
PDS Polydioxanone Suture
TIP Tubularized Incised Plate
UC Urethroplasty Complications
29.1 Introduction
Fig. 29.1 Distal coronal hypospadias (©Ahmed
Distal hypospadias represent the most frequent T. Hadidi 2022. All Rights Reserved)
presentation of hypospadias (Fig. 29.1). The TIP
repair is regarded as the preferred procedure by
most pediatric urologists, but recently the effi- Mitchell and Bagli [3] and Perovic et al. [4]
ciency of this technique has been questioned reported on complete penile disassembly for
based on findings of higher observation of com- epispadias repair as a way to complete release
plications such as coronal fistula and meatal ste- of the rotation of the penis and treat dorsal chor-
nosis. Systematic literature review reports dee, bringing the urethra to a more functional
urethroplasty complications in approximately location. We were inspired by both procedures
7% of distal TIP repairs, but as previously men- to propose an aggressive partial glanular disas-
tioned, many authors report higher complications sembly in association with minor urethral mobi-
rate [1]. lization to treat primary and redo coronal and
We have investigated other alternatives for subcoronal hypospadias forms. The combina-
distal hypospadias repair. Koff et al. have popu- tion of these two steps avoid the need for ure-
larized the urethral mobilization concept [2]. throplasty and suture lines, minimizing the risk
for fistulas. The aggressive glans mobilization
and disconnection from the corpora except for
A. Macedo Jr. (*) · M. L. da Cruz its dorsal component offer great mobility and
Urology Department, AACD Hospital, Sao Paolo capability of refurbishing it. We also noticed
Federal University, São Paulo, Brazil that reconfiguration of glans enabled at the end
e-mail: Antonio.macedo@uropediatria.com.br
472 A. Macedo and M. L. da Cruz
a much more conical aspect. The merit of the carded at the end of the procedure (Figs. 29.2
procedure is the combination of minor mobili- and 29.3).
zation of the urethra with down adjustment and This procedure, differently from the classic
rotation of the glans medially. We have already Koff technique, requires minor urethral mobili-
presented the principles of the procedure in a zation that we believe will avoid urethral
recent short video publication [5]. retraction and compromise of blood supply.
We report the technique in detail and present Laterally to distal spongious tissue at the Y
herein consecutive results with short-term pre- urethra, we incise the Buck’s fascia and super-
liminary results. We have designed this technique ficial tunica albuginea on both sides, releasing
in 2017, and it has passed through minor modifi- the urethra from the corpora and giving mobil-
cations over time. We are convinced that this ity to two flaps of spongiosum tissue that can
technique can be considered an alternative to dis- be joined in midline to reinforce the urethra.
tal hypospadias repair. We follow the lateral plane of dissection to the
urethra cranially on both sides and converge
above the meatus to outline an inverted
29.2 Operative Technique Y-shaped incision. The glans is then entirely
opened in the midline (Fig. 29.3). The glans
The technique consists of subcoronal circumci- disassembled from the corpora and opened in
sion to expose distal dysplastic urethra. In order the midline shows in this moment two glandu-
to avoid damage to distal urethra that frequently lar wings that will embrace the mobilized ure-
is thin and membranous, the U-shaped incision thra subsequently and can be rotated downward
is conducted below to the hypospadiac meatus (Fig. 29.4). The urethra normally can be easily
where the skin is robust enough. After the penile lifted cranially and repositioned distally to the
shaft is degloved, we can observe a bridge of tip of the glans with three posterior anchor stay
skin bound to the thin distal urethra, similar to a sutures with PDS 5.0 at 11, 12, and 1 o’clock.
flip-flap or a Mathieu flap. This minor skin flap We remove at this moment the skin bridge at
is used only to traction the urethra during the the ventral distal urethra used solely to handle
distal mobilization from the corpora and is dis- the urethra and avoid touching the urethra with
Fig. 29.2 Principles of the GUD technique: aggressive The urethra is minimally mobilized and cranially reposi-
glanular disassembly, partially disconnected from corpora tioned in association to caudal and medial rotation of
cavernosa from 2 to 10 o’clock and opened in midline. glanular wings
29 The Glanular Urethral Disassembly (GUD) Technique: An Alternative to Distal Hypospadias 473
Fig. 29.3 The main principles of the GUD: subcoronal is entirely opened in the midline. The two glanular wings
circumcision: the U-shaped incision is conducted below can easily embrace the mobilized urethra subsequently
to the hypospadiac meatus where the skin is robust to pro- and rotate downward. Observe how aggressive the disas-
duce a minor skin flap used only to traction the urethra sembly of the glans is performed to allow mobility and
during the distal mobilization from the corpora. The glans rotation of the glans
forceps. The neomeatus is completed with urethra is centrally displaced (Figs. 29.5, 29.6,
interrupted PDS 6.0, and the glans is recon- and 29.7).
structed embracing the urethra with three sub- A silicone catheter F10 is left indwelling as a
cutaneous PDS 5.0 sutures. The redundant stent for 7–10 days, providing an incontinent
ventral foreskin is discarded; there is no need urinary diversion dropping in a second diaper. As
to create a barrier layer of the dartos flap inter- far as there is not a urethroplasty, the reason to
posing the mobilized urethra to the glans. We keep a stent is to prevent urinary complaints in
use a tourniquet for glanular dissection and the case of glandular edema compressing the ure-
hemostasis control. After glans reconstruction, thra. A bioclusive dressing is applied to the shaft
its new conical conformation is clear, and the of the penis concluding the procedure.
Fig. 29.4 The thoroughly mobilized glans after disas- shape. The urethra moves cranially, and the glans moves
sembly from the corpora can easily embrace the urethra, caudally and medially (courtesy of Ahmed T. Hadidi)
changing the configuration of the glans into a conical
Fig. 29.5 Initial and immediate result after GUD technique. Notice the improvement in the conical aspect of the glans
without the need for urethroplasty
Fig. 29.6 Immediate and early postoperative aspect
29.3 Clinical Data
We have treated 164 patients with distal hypo-

spadias. The median age at surgery was
22.4 months (1–184 months). The meatal posi-
tion after degloving the penis was coronal at 108
cases, subcoronal at 54, and 2 patients presented
with megameatus and an intact foreskin. The
penile curvature evaluated by penoscrotal digi-
tal compression showed that 122 patients had no
curvature, 35 had it but less than 30 degrees, 4
had curvature between 30 and 45 degrees, and 3
had more than 45 degrees. Curvature resolved
Fig. 29.7 One-month postoperatively aspect, notice that completely in all cases by degloving the penis
the urethra has a central position and the glans has a coni-
cal shape format and detaching paraurethral chordee.
Three patients (1.8%) had mild penoscrotal results. Oztorun et al. compared the Mathieu and
transposition in addition to hypospadias. Twenty- TIP techniques and found no statistical differ-
eight patients were treated as a secondary repair ence in a cohort of 492 consecutive cases [11].
(17%), mainly to fix coronal fístulas. These Koff et al. published a modification of the
patients were treated according to the same prin- Barcat technique known as extensive urethral
ciple for primary cases. mobilization and confirmed excellent cosmetic
We found complications in six patients (3.6%) and functional results on 168 patients with only
consisting of five fistulas (3%) and three glans 3.5% of the patients requiring reoperation [2].
dehiscence (1.8%). Two patients had both com- This technique has been also reproduced by sev-
plications. Follow-up was 21 months eral other groups but remained not so popular.
(1–42 months), and the median follow-up time Recently, urethral mobilization started to regain
was 18 months. its position in distal hypospadias repair because
of its simplicity with very favorable results. As
far as there is no suture in the urethra, the fistula
29.4 Discussion rate is expected to be less. However, the attempts
to bring the urethra up should not exceed its limi-
More than 300 methods of surgical repair are tation because excessive mobilization may jeop-
available for hypospadias repair. Distal forms ardize blood supply of the urethra causing tissue
represent more than 60% of them, and over the ischemia and necrosis and then promoting fistula
last years, the tubularized incised plate (TIP) ure- or urethral disruption [12].
throplasty has become the most popular tech- Minor forms of urethral advancement include
nique for this presentation [6, 7]. In a the historical procedure described by Duckett in
meta-analysis study, Pfistermuller et al. reviewed 1981, the “meatal advancement and glanulo-
nine studies of TIP repairs (4675 patients) and plasty incorporated” (MAGPI), which has been
concluded that fistula and reoperation rates were regarded in the past as the most common method
significantly higher in secondary repairs (15.5% for distal hypospadias repair [13]. In this surgical
and 23.3%) compared to primary proximal approach, the hypospadiac meatus is advanced to
(10.3% and 12.2%) and primary distal (5.7% and the tip of glans without tubularization. Its use has
4.5%) [8]. Urethroplasty complications (UC) in been questioned, and very limited indications are
hypospadias commonly reported are fistula, glans seen today due to meatal retraction or regression,
dehiscence, meatal stenosis, urethral stricture, seen in up to 22% of cases [14] and postoperative
and diverticulum [8]. Snodgrass et al. reported glandular chordee [15]. Other attempts to
125 UC diagnosed following 887 TIP operations improve the technique have been proposed but
(14%). These included 54 (43%) fistulas, 59 without wide acceptance [16].
(47%) glans dehiscences, 9 (7%) meatal stenosis The glans approximation procedure (GAP)
or urethral strictures, and 3 (2%) patients with described by Zaontz [17] focuses on the repair of
diverticulum [9]. Interestingly 80/125 UC (64%) glandular and coronal hypospadias with a wide,
were diagnosed at the first postoperative visit, deep glanular groove and noncompliant urethral
and 101 (81%) were diagnosed within the first meatus. It is an excellent alternative to the so-
year after repair. Authors reported a high inci- called non-urethroplasty techniques (no ventral
dence of urethral calibration for the uneventful sutures), but as the author stressed, the indica-
cases (52%) [9]. The same group suggests that if tions are precise and cannot be widespread.
key principles of TIP repair are observed, results We believe that we cannot achieve good
can be reproducible and comparable among men- results only by advancing the urethra without
tor and ex-fellows in junior experience [10]. reconstructing the glans, even by aggressive
In spite of the widespread preference for TIP mobilization as reported by Koff et al. [2]. The
repair for distal repairs, comparison with other glans represents a major component of the hypo-
classical techniques has shown comparable spadiac abnormality, and only by repairing it in
association with mobilization of the urethra a 2. Koff SA, Brinkman J, Ulrich J, Deighton D, Belman
much better result can be obtained (Fig. 29.6) AB. Extensive mobilization of the urethral plate and
urethra for repair of hypospadias: the modified Barcat
[18, 19]. technique. J Urol. 1994; https://doi.org/10.1016/
Mitchell and Bägli [3] and Perovic et al. [4] S0022-5347(17)34992-3.
reported on the complete penile disassembly for 3. Mitchell ME, Bägli DJ. Complete penile disas-
epispadias repair as a way to complete the release sembly for epispadias repair: the Mitchell tech-
nique. J Urol. 1996; https://doi.org/10.1016/
of the rotation of the penis and treat dorsal chor- S0022-5347(01)66649-7.
dee, bringing the urethra to a more functional 4. Perovic SV, Vukadinovic V, Djordjevic MLJ, Djakovic
location. We were inspired by this procedure NG. Penile disassembly technique for epispadias
when we started proposing extensive partial glan- repair: variants of technique. J Urol. 1999; https://doi.
org/10.1016/S0022-5347(01)68122-9.
ular disassembly in association with urethral 5. Macedo A, Ottoni SL, Leal da Cruz M. The GUD
mobilization. The rationale for this procedure is technique: glandular urethral disassembly for distal
to avoid sutures and a formal urethroplasty, hypospadias repair. J Pediatr Urol. 2020; https://doi.
except sutures at the urethra meatus to reposition org/10.1016/j.jpurol.2020.03.017.
6. Steven L, Cherian A, Yankovic F, Mathur A, Kulkarni
the urethra distally without tension. The partial M, Cuckow P. Current practice in paediatric hypospa-
glanular disassembly procedure can also be dias surgery; a specialist survey. J Pediatr Urol. 2013;
regarded as a way to create a more conical and https://doi.org/10.1016/j.jpurol.2013.04.008.
cosmetical glans. We agree that the procedure is 7. Springer A, Krois W, Horcher E. Trends in hypospa-
dias surgery: results of a worldwide survey. Eur Urol.
much more aggressive in the glans, but once the 2011; https://doi.org/10.1016/j.eururo.2011.08.031.
incision follows the lateral Buck’s fascia inci- 8. Pfistermuller KLM, McArdle AJ, Cuckow PM. Meta-
sion, no major bleeding occurs, having a tourni- analysis of complication rates of the tubularized
quet applied. incised plate (TIP) repair. J Pediatr Urol. 2015; https://
doi.org/10.1016/j.jpurol.2014.12.006.
We acknowledge that this procedure is 9. Snodgrass W, Villanueva C, Bush NC. Duration of
intended only to distal hypospadias (coronal and follow-up to diagnose hypospadias urethroplasty
subcoronal). We stress that the GUD procedure complications. J Pediatr Urol. 2014; https://doi.
can be performed irrespectively of any urethral org/10.1016/j.jpurol.2013.11.011.
10. Bush NC, Barber TD, Dajusta D, Prieto JC, Ziada A,
plate “quality” as it does not require a minimum Snodgrass W. Results of distal hypospadias repair after
glans width as the TIP repair. Moreover, there is pediatric urology fellowship training: a comparison
no room for preoperative testosterone treatment. of junior surgeons with their mentor. J Pediatr Urol.
The absence of suture and urethroplasty avoids 2016; https://doi.org/10.1016/j.jpurol.2015.12.007.
11. Oztorun K, Bagbancf S, Dadali M, Emir L, Karabulut
practically the risk of the feared coronal fistula A. A retrospective analysis of Mathieu and TIP ure-
after surgery. throplasty techniques for distal hypospadias repair; a
20 year experience. Arch Esp Urol. 2017;70:679–87.
12. El Darawany HM, Al Damhogy ME. Urethral mobili-
zation as an alternative procedure for distal hypospa-
29.5 Conclusion dias repair. Urology. 2017; https://doi.org/10.1016/j.
urology.2017.03.009.
We have presented the GUD technique in detail 13. Duckett JW. MAGPI (meatoplasty and glanu-
with early clinical results. We believe that this loplasty). A procedure for subcoronal hypo-
spadias. Urol Clin North Am. 1981; https://doi.
procedure may have in the future a significant org/10.1097/00005392-200205000-00059.
role in distal hypospadias repair. 14. Ünlüer ES, Miroglu C, Ozdiler E, Ozturk R. Long-
term follow-up results of the MAGPI (meatal
advancement and glanuloplasty) operations in dis-
tal hypospadias. Int Urol Nephrol. 1991; https://doi.
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hypospadias. J Urol. 1984; https://doi.org/10.1016/ 19. Snodgrass W, MacEdo A, Hoebeke P, Mouriquand
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https://doi.org/10.1016/S0022-5347(17)40766-X.
Proximal Hypospadias With Small
Flat Glans: The Lateral-Based 30
Onlay (LABO) Flap Technique
Ahmed T. Hadidi
Abbreviations cations is reported in patients with small or flat

glans [9, 10].
CEDU Chordee Excision and Distal In this chapter, the LABO flap technique in
Urethroplasty patients with proximal hypospadias, without
LABO Lateral-Based Onlay flap severe chordee (grade III), and small flat glans is
LB Lateral-Based presented. The principle of the technique is to use
TIP Tubularized Incised Plate a narrower base (urethral plate), two deep lateral
incisions in the glans in order to have larger and
more mobile glanular wings. The narrow plate is
compensated for by using part of the lateral fore-
30.1 Introduction skin as an onlay flap.
Proximal hypospadias remains a major challenge

for the hypospadias surgeon. Currently, proximal 30.2 Selection of Patients
hypospadias is subclassified into proximal hypo-
spadias without chordee or with superficial chor- Careful assessment of the penis is performed.
dee <30 (grade III hypospadias) and proximal Patients with proximal hypospadias and deep
and perineal hypospadias with deep chordee >30 chordee that required division of the urethral
that necessitates the division of the urethral plate plate are suitable for CEDU (Chap. 33)
(grade IV hypospadias) [1]. The surgical tech- or two-stage repair (Chap. 40). The degree of
niques for proximal hypospadias when the ure- glans clefting (grooving) is evaluated. Patients
thral plate could be preserved include the with proximal hypospadias with no or superficial
lateral-based onlay (LABO) flap [2], Koyanagi chordee associated with small, flat glans had the
technique [3], Duckett island flap [4], Bracka LABO technique performed. When the native
two-stage repair [5], the onlay flap [6], Thiersch urethral meatus was narrow, it was incised proxi-
technique [7], and tubularized incised plate (TIP) mally to create a wide spatulated meatus.
technique [8]. However, a higher rate of compli-
30.3 Operative Technique

A. T. Hadidi (*)
Hypospadias Center and Pediatric Surgery Under general anesthesia and caudal block, a
Teaching Hospital of the Johann Wolfgang Goethe traction suture of 4/0 Prolene is placed through
University, Offenbach, Frankfurt, Germany the tip of the glans. A tourniquet is applied at the
480 A. T. Hadidi
base of the penis, and an artificial erection test is The LABO flap is turned over the appropriate
performed. size catheter (Fig. 30.1d). The appropriate width
Ventral degloving: As chordee in hypospadias of the LABO flap is confirmed after completing
involves the ventral aspect of the penis only, the left border suture line. Only then is the left
degloving was limited to the ventral aspect of the border of the flap incised. Suturing on the right
penis. This is important to preserve the deep fas- side starts 2 mm proximal to the meatus to have
cia and blood supply to the LABO flap. Patients the knot proximal to the meatus (Fig. 30.1e).
with proximal hypospadias associated with Urethroplasty is completed using 6/0 polyglactin
penile torsion underwent complete degloving on a cutting needle in a continuous subcuticular
after mobilization of the LABO flap. suturing manner (Fig. 30.1f) around catheter size
Flap design: The margins of the urethral plate F12 or larger according to the age of the patient.
are outlined. Attention is given to create large A small V is excised from the apex of the LABO
glanular wings, and the urethral plate can be out- flap (inset).
lined as narrow as 3 mm in width. The narrow Protective intermediate layer: A protective
urethral plate is compensated by a wider flap intermediate layer from the penile shaft or the
from the lateral foreskin. At the distal end of the scrotal dartos/tunica vaginalis is routinely used to
urethral plate, the two borders converge toward cover the urethroplasty (Fig. 30.1g).
each other [11] to allow for more mobility of the Meatoplasty and glanuloplasty: The two
glanular wings to wrap around the neourethra edges of the final meatus are sutured together to
and to have a slit-like meatus free of sutures. the center of the V creating a slit-like meatus
The LABO flap is outlined as shown in using polyglactin 6/0 single stitch. No other
Fig. 30.1. For right-handed surgeons standing on sutures are required for the meatus. The glanular
the right side of the patient, the flap is easier to wings are approximated using polyglactin 6/0
obtain from the left side. The medial border is at transverse mattress-interrupted sutures. The
the left foreskin mucocutaneous junction, adja- remaining wound is closed using continuous
cent to the glans. The width of the flap is designed mattress polyglactin 6/0 stitches (Fig. 30.1h).
according to the width of the urethral plate with The remaining skin of the penis and foreskin
the help of an appropriate-sized catheter and stay was fashioned to provide ventral skin coverage of
sutures. The flap is designed in a way to have a the penis. This is not always easy depending on
wide base. the width of the flap used in the urethroplasty.
Incision in the glans region begins at the coro- Urine drainage: A transurethral silastic cath-
nal sulcus using a sharp scalpel or scissors and eter size F12 is used for 7–10 days.
continued distally very deep into the glans in Dressing: All patients had a standard dress-
such a way to create large, mobile glanular wings. ing in the form of gentamicin local eye oint-
Stay sutures at the foreskin help orientation. ment, gauze, and adhesive tape that compresses
Proximally, the LABO flap is elevated using the penis, gauze, and catheter against the lower
sharp scissors taking care to include the dartos abdominal wall and allows the free mobility of
fascia with the flap. The lateral foreskin is mobi- the patient [12]. The dressing was removed at
lized carefully, maintaining the deep fascia and the time of removal of the transurethral catheter,
blood supply to the designed flap. and the penis was left exposed. If the dressing
Urethroplasty: A stitch fixes the apex (A) of became wet with urine or blood, the dressing
the LABO flap to the urethral plate 2 mm from was changed using a similar standard dressing.
the tip of the incision (A′) to keep the meatal Broad-spectrum antibiotics (second-generation
edges free of any sutures and to have a smooth, cephalosporin) and local application of eye oint-
near normal meatus (Fig. 30.1a) [11]. The medial ment over the wound were used for 1 week.
border of the left foreskin is sutured to the lateral Paracetamol and ibuprofen suppositories and
border of the urethral plate in a continuous mat- syrup were routinely used for pain relief as
tress manner using 6/0 polyglactin (Fig. 30.1b). required.
30 Proximal Hypospadias With Small Flat Glans: The Lateral-Based Onlay (LABO) Flap Technique 481
a b
c d
Fig. 30.1 The lateral-based onlay technique: The prin- (A′). (c) Urethroplasty: The medial border of the LABO
ciple is to use the lateral preputial skin as a lateral meatal- flap is sutured to the left edge of the urethral plate. (d) The
based flap. (a) The LABO technique design: A U-shaped LABO flap is turned over the catheter, like closing a book.
incision is outlined. The two parallel incisions go very (e) The second apex stitch is fixed 2 mm from the tip of
deep into the glans (inset) and converge as shown to have the urethral plate incision. (f) Urethroplasty is completed
a slit-like meatus. The left incision stops at the coronal on the right side. A triangle is removed from the tip of the
sulcus and continues distally in the prepuce at the muco- flap to help having a slit-like meatus. (g) A second inter-
cutaneous junction and constitutes to the medial border of mediate layer is used from the scrotal dartos/tunica vagi-
the LABO flap. The flap is designed to have a wide base nalis. (h) Glans and skin closure: The glanular wings are
as shown in the figure. (b) Flap mobilization: The right approximated around the new urethra and the penile skin
incision is deepened starting near the coronal sulcus. Apex is closed. Notice that the new meatus has one stitch only
suture: The tip of the medial border of the LABO flap (A) at 6 o’clock (©Ahmed T. Hadidi 2022. All Rights
is sutured to the urethral plate 2 mm proximal to the edge Reserved)
482 A. T. Hadidi
e f
g h
Our standard postoperative follow-up protocol and failing to attend for follow-up were contacted
includes examination after 3 months, 1 year, by e-mail and phone using a structured question-
3 years, and then every 5 years until the child is naire [13]. Follow-up period ranged from
15 years old. Patients living long distances away 12 months to 15 years (mean 9 years).
30.4 Important Technical Points follow-up, and the remaining 182 patients consti-
tute the present cohort for this report. Patient age
• The caliber of the neourethra was designed to ranged between 8 months and 2 years (mean
be the same size or slightly larger than the nor- 11 months). All the patients had proximal hypo-
mal proximal urethra. Protocol is to use a spadias with flat or incomplete cleft glans and did
catheter size F12 or larger. The aim is to have not have deep chordee. Cutaneous chordee was
a wide neourethra and meatus that is unlikely corrected by skin mobilization. A transurethral
to contract with healing and cause meatal silastic catheter size F12 is used for 7–10 days.
stenosis. Patients with complicated hypospadias who
• Occasionally the hypospadiac meatus underwent the LABO technique are not included
becomes narrower than the normal proximal in the present study.
urethra due to the presence of a rather circular For the purpose of the study, incomplete cleft
fibrous ring and should be incised to avoid ste- glans is identified when the glanular groove is
nosis at the proximal end of the neourethra. less than 4 mm in depth. Flat glans is considered
• Care must be taken to elevate the Buck’s fas- when there is no glanular groove.
cia with the flap as this constitutes the main
blood supply to the flap.
• When mobilizing the flap, one should be hold- 30.6 Results and Complications
ing the opposite skin edge and not the flap skin (Figs. 30.2, 30.3, 30.4, and 30.5)
edge. This has minimized flap tissue injury
and increases the amount of subcutaneous tis- A hundred and eighty-two patients have been
sue and fascia. followed; the remaining (being from long dis-
• The first step in the urethroplasty is to suture tances) have not responded. Satisfactory results
the distal end of the flap to the end of the glans were achieved in 173 patients (95%). Eighty
2 mm from the tip (A to A′) (Fig. 30.1b). This patients completed 10 years of follow-up or
helps to have only one stitch at the meatus at 6 more. Two patients developed fistulae, four chil-
o’clock position. dren had glans dehiscence, two patients devel-
• The suturing of urethroplasty should start a oped proximal diverticulum, and one child
few millimeters proximal to the original developed prolapsing skin outside the meatus
meatus. This helps to have the knot proximal 2 years after the urethroplasty. The fistula was
and away from the neourethra. closed routinely 6 months later. A second inter-
• The left border of the urethroplasty is sutured mediate layer was always used to protect fistula
first before cutting the lateral border of the closure. The two children with dehiscent glans
flap that will constitute the right border of the had meatoglanuloplasty performed successfully
urethroplasty to ensure having the appropriate 6 months after the first operation. The child with
width of the LABO flap. prolapsed skin had the prolapsed skin excised as
• As suturing is performed in a continuous sub- an ambulant procedure 2 years after the first sur-
cuticular manner, a cutting needle is easier to gery. The two children with diverticula had the
go through the dermis as is the case with the diverticulum excised after 3 and 6 months
classic subcuticular closure of skin incisions. (Table 30.1).
Uroflowmetry was performed in 21 toilet-
trained children, and the peak flow was within 2
30.5 Patients and Methods standard deviations in 16 patients and flat in 5
patients. Those 5 patients are clinically free of
The LABO technique was performed in 214 symptoms, and ultrasound of the urinary bladder
patients in the period between January 2004 and did not show thickened bladder wall suggestive
December 2019. Thirty-two patients were lost to of distal obstruction.
484 A. T. Hadidi
a b c
d e
Fig. 30.2 The lateral-based onlay (LABO) flap tech- tion of the LABO flap. (c) The left suture line of the ure-
nique in a 12 month boy with proximal hypospadias: (a) a throplasty was completed. (d) The appearance after
proximal hypospadias without deep chordee. Notice the completion of urethroplasty around a catheter size F10
incomplete cleft glans, the narrow irregular urethral plate, and skin closure. (e) The appearance 1 year after surgery,
and the smegma mass that was removed at the beginning during the follow-up visit (©Ahmed T. Hadidi 2022. All
of operation. (b) After incision of the plate and mobiliza- Rights Reserved)
30.7 Discussion The onlay flap from the dorsal preputial skin
is a good technique for proximal hypospadias
Surgical correction of proximal hypospadias is without deep chordee that requires incision of the
technically demanding and remains a major chal- urethral plate.
lenge for the experienced hypospadias surgeon. In a retrospective study, Braga et al. [14]
The situation becomes more challenging when reviewed the records of 72 consecutive patients
the child has a small flat glans. with penoscrotal hypospadias and compared the
Common techniques for proximal hypospa- outcome of 35 patients who underwent “tubular-
dias when the urethral plate could be preserved ized incised plate” (TIP) technique and 40
include the onlay flap [6], Thiersch technique [7], patients who underwent an onlay repair. In both
and tubularized incised plate (TIP) technique [8]. groups the repair was done around a catheter size
A higher rate of complications is reported in F8. The overall complication rates were 60% and
patients with small or flat glans [9, 10]. 45% for the TIP and onlay, respectively. A pla-
Fig. 30.3 LABO flap design in a 10-month-old baby urethral plate and incomplete cleft glans (©Ahmed
with penoscrotal hypospadias without deep chordee (con- T. Hadidi 2022. All Rights Reserved)
firmed by erection test). Notice the very narrow irregular
teau uroflow curve vs. normal bell curve was has less complications than the LB flap. The low
observed in 16 of 24 children (66.7%) who complication rate of 5% is probably also related
underwent TIP repair and 7 of 21 (33.3%) who to the careful selection of patients as proximal
underwent onlay repair. Fistula location varied hypospadias without deep chordee usually indi-
significantly between the two groups, with cates a better developed and well-vascularized
proximal fistulae occurring in 11 of 15 TIP
urethral plate.
repairs (73.3%) vs. 2 of 8 onlay repairs (25%). Uroflowmetry was carried out in 21 toilet-
The LABO technique is presented as a tech- trained children following LABO repair. Five
nique of particular value in patients with small, children had a flat curve although they did not
flat glans. It is probably easier to perform because have any clinical symptoms of obstructive void-
the flap is employed from the ventral aspect of ing and ultrasound on the bladder did not show
the penis without the need of extensive mobiliza- increased bladder wall thickness. The signifi-
tion as is the case with the classic transverse pre- cance of uroflow following hypospadias repair
putial onlay flap. remains uncertain [10].
Hadidi reported his experience with the Snodgrass et al. (2011) reported a complica-
lateral-based (LB) flap for proximal hypospadias tion rate of 13% of the TIP technique in proximal
and deep chordee [15]. There is always a search hypospadias [8]. In another publication by the
on how to improve the functional and cosmetic same author, he reported a 15% glans dehiscence
outcome and reduce hospital stay and complica- rate [9]. Eassa et al. reported a 35% complication
tions. One would expect that one suture line of TIP in proximal hypospadias [16]. Long-term
(lateral-based flap) is less liable to complications follow-up studies of the TIP technique reported a
than having two suture lines (LABO flap). This is complication rate reaching 66% in proximal
not the case in clinical practice, and the LABO hypospadias [13]. Other long-term follow-up
486 A. T. Hadidi
a b c
d e f
Fig. 30.4 (a) Patient with distal hypospadias and chor- were taken from the adjacent fascia laterally. (f)
dee. (b, c) After chordee excision LABO technique was Meatoglanuloplasty and skin closure. (g) The penis is
performed around catheter F12. (d) The lateral preputial fixed to the abdominal wall (©Ahmed T. Hadidi 2022. All
skin was sutured to the urethral plate. (e) Protected layers Rights Reserved)
Fig. 30.5 Patient with proximal hypospadias. The chordee was excised, and LABO technique was performed (©Ahmed
488 A. T. Hadidi
Table 30.1 Outcome of lateral-based onlay (LABO) flap out chordee. J Urol. 1987; https://doi.org/10.1016/
in 182 patients S0022-5347(17)43152-1.
7. Amukele SA, Weiser AC, Stock JA, Hanna
Outcome Number of patients
MK. Results of 265 consecutive proximal hypo-
Satisfactory results 173 spadias repairs using the Thiersch-Duplay prin-
Coronal fistula detected between 2 ciple. J Urol. 2004; https://doi.org/10.1097/01.
2 and 6 months ju.0000143880.13698.ca.
Glans dehiscence 4 8. Snodgrass W, Bush N. Tubularized incised plate
Skin prolapsing outside the 1 proximal hypospadias repair: continued evolution and
meatus extended applications. J Pediatr Urol. 2011; https://
Total number of patients 182 doi.org/10.1016/j.jpurol.2010.05.011.
9. Snodgrass W, Cost N, Nakonezny PA, Bush
N. Analysis of risk factors for glans dehiscence after
studies of the TIP technique in proximal hypo- tubularized incised plate hypospadias repair. J Urol.
spadias showed functional obstruction and 2011; https://doi.org/10.1016/j.juro.2010.12.070.
10. Snodgrass W, MacEdo A, Hoebeke P, Mouriquand
marked reduction in urine flow rate in the major- PDE. Hypospadias dilemmas: a round table. J
ity of patients included in the study [17]. Pediatr Urol. 2011; https://doi.org/10.1016/j.
The LABO flap was associated with low mor- jpurol.2010.11.009.
bidity and low complication rate. Long-term fol- 11. Hadidi AT. The slit-like adjusted Mathieu technique
for distal hypospadias. J Pediatr Surg. 2012; https://
low-up is being carried out to assess the functional doi.org/10.1016/j.jpedsurg.2011.12.030.
and cosmetic outcome after puberty. 12. Hadidi AT. Double Y glanuloplasty for glanu-
lar hypospadias. J Pediatr Surg. 2010; https://doi.
org/10.1016/j.jpedsurg.2009.11.019.
References assessment of hypospadias repair with emphasis on
techniques used and experience of pediatric urologic
1. Hadidi AT. History of hypospadias: lost in translation. surgeons. Urology. 2007; https://doi.org/10.1016/j.
J Pediatr Surg. 2017;52:211. https://doi.org/10.1016/j. urology.2007.01.103.
jpedsurg.2016.11.004. 14. Braga LHP, Pippi Salle JL, Lorenzo AJ, Skeldon
2. Hadidi AT. Proximal hypospadias with small flat S, Dave S, Farhat WA, Khoury AE, Bagli
glans: the lateral-based onlay flap technique. J DJ. Comparative analysis of tubularized incised plate
Pediatr Surg. 2012; https://doi.org/10.1016/j. versus onlay island flap urethroplasty for penoscrotal
jpedsurg.2012.06.027. hypospadias. J Urol. 2007; https://doi.org/10.1016/j.
3. Koyanagi T, Matsuno T, Nonomura K, Sakakibara juro.2007.05.170.
N. Complete repair of severe penoscrotal hypo- 15. Hadidi AT. Lateral-based flap: a single stage ure-
spadias in 1 stage: Experience with urethral mobi- thral reconstruction for proximal hypospadias.
lization, wing flap-flipping urethroplasty and J Pediatr Surg. 2009; https://doi.org/10.1016/j.
“glanulomeatoplasty”. J Urol. 1983; https://doi. jpedsurg.2008.08.012.
org/10.1016/s0022-5347(17)51732-2. 16. Eassa W, Jednak R, Capolicchio JP, Brzezinski A,
4. Duckett JW, Baskin LS. Hypospadias. In: Gillenwater El-Sherbiny M. Risk factors for re-operation follow-
JY, Grayhack JT, Howards SS, Duckett JW, editors. ing tubularized incised plate urethroplasty: a compre-
Adult and pediatric urology. 3rd ed. St. Louis: Mosby- hensive analysis. Urology. 2011;77:716–20.
Year Book; 1996. p. 2549–89. 17. Orkiszewski M, Leszniewski J. Morphology and
5. Bracka A. Hypospadias repair: the two-stage alterna- urodynamics after longitudinal urethral plate inci-
tive. Br J Urol. 1995; https://doi.org/10.1111/j.1464- sion in proximal hypospadias repairs: long-term
410X.1995.tb07815.x. results. Eur J Pediatr Surg. 2004; https://doi.
6. Elder JS, Duckett JW, Snyder HM. Onlay island flap in org/10.1055/s-2004-815778.
the repair of mid and distal penile hypospadias with-
The Onlay Island Hypospadias
Repair 31
Howard M. Snyder III
31.1 Introduction simply the skin asymmetry and subcutaneous

tissue in some cases. Controversy grew about
In patients with hypospadias sufficiently severe the significance of the spongiosum, with some
to require a substitution urethroplasty, for many prominent surgeons continuing to recommend
years the most commonly utilized technique at excision. Histologic work by Avellán and
the Children’s Hospital of Philadelphia has been Knutsson in 1980 [5], Baskin et al. in 1998 [6],
the onlay island one-stage hypospadias repair [1]. and then in 2000 by Snodgrass et al. [7], who
Even for the most severe hypospadias, it has been carried out histologic examination of the spon-
this author’s practice to carry out one-stage giosum, indicated that the spongiosum really
repairs. was not dysplastic at all and indeed was a vascu-
The concept of a vascularized preputial larized healthy layer that could be incorporated
island flap was introduced by Hook in 1896 [2]. into a hypospadias repair to provide healthy vas-
It was, however, Asopa and colleagues in Agra, cularized tissue for the anastomosis of a neoure-
India, who developed the first very effective use thra. With experience, we recognized that only a
of inner preputial skin for a substitution urethro- minority of hypospadias cases require division
plasty in hypospadias [3]. Duckett developed of the urethral plate—about 10% of major hypo-
this by describing a transverse island tube repair spadias cases at the Children’s Hospital of
in 1980 [4]. This was an era when it was pre- Philadelphia today [8]. It also was recognized
sumed that if there was a need for a substitution that a minor bend of less than 30 degrees after
urethroplasty, the urethral plate would be abnor- penile degloving can be adequately treated by
mal and require division. In the routine division dorsal midline plication without disturbing the
of the urethral plate, since the plate was of elas- neurovascular supply of the penis. It is, how-
tic tissue, it would retract, supporting the then ever, possible that this treatment is not essential,
held view that the division was required. since in the era prior to the artificial erection
Gradually, it became recognized in the 1980s test, we would never have identified this minor
that chordee in hypospadias could be due to residual bend when we carried out a first-stage
hypospadias repair. It is interesting to see that
hypospadias patients are infrequently seen in
This Chapter is a reprint from the First Edition.
adult sexual dysfunction clinics, suggesting that
we have not undertreated chordee in the past, in
H. M. Snyder III (*)
Department of Pediatric Urology, Children’s Hospital an era when minor residual bend was probably
of Philadelphia, Philadelphia, PA, USA undetected.
490 H. M. Snyder III
31.2 Operative Technique 31.2.3 Incisions
31.2.1 Timing It is our practice to outline the incisions for a penile

hypospadias repair at a subcoronal level, incorpo-
Surgery for hypospadias is generally carried out rating ventrally skirt flaps as described by Firlit in
between 6 and 12 months of age. This is after 1987 [11]. The incisions on either side of the ure-
children reach the age where they will have the thral plate are right at the junction with the normal
same anesthetic risks that they will have through- ventral skin. The incisions ventrally are kept very
out childhood and yet well below the age of geni- superficial, just going through the skin in order to
tal awareness which occurs at about 18 months. avoid injury to the underlying spongiosum. The
This timing follows the recommendations of the incisions continue up on either side of the glanular
Urologic Section of the American Academy of groove to the apex of the glanuloplasty. By the use
Pediatrics. of xylocaine with epinephrine and careful following
of dissection planes away from the vascular supply
(described below), a tourniquet is not required.
31.2.2 Design of Glanuloplasty
The determination of the apex of the glanulo- 31.2.4 Penile Skin Dropped Back
plasty ventrally is the single most important ini-
tial decision that must be made (Fig. 31.1a). This (a) Ventrally: The skin drop back is maintained
point is where the flat ventral surface of the glans just below the intrinsic blood supply of the
begins to curve around the meatus. If holding ventral skin, carefully keeping the scissors’
stitches are placed in the abortive foreskin, in the tip parallel to the skin so as not to injure the
majority of cases, the insertion of the foreskin intrinsic blood supply of the skin (Fig. 31.1b).
into the glans will show where the flat surface It is important to remember that the spongio-
can be traced up to where it begins to curve sum inserts into both the overlying skin and
around the meatus. If the apex is held with for- the corporal bodies. Accordingly, a plane of
ceps on each side, then the amount of epithelium dissection just below the intrinsic blood sup-
that will be within the meatus can be determined. ply must be established laterally and then
It will be readily appreciated that if the apex sharp dissection used to separate the skin
point is picked too high, it will compromise the from the underlying spongiosum. Meticulous
size of the meatus. Conversely, placement of the attention to the plane of dissection avoids
apex too low leaves unsightly bumps on either trauma to the delicate spongiosum and does
side of the meatus after the glanuloplasty is com- not disturb the spongiosum’s insertion into
pleted. If the width and depth of the glanular the glans. Starting the dissection distally and
groove is inadequate, then steps to deepen and working proximally up the shaft has been
widen the groove are appropriate. In infant boys easiest in my experience.
a groove 12–14 mm is appropriate and in a teen- (b) Dorsally: The plane of dissection is kept
ager 25 mm. A dorsal vertical incision is made deep to the four small arteries that constitute
until the width of the glanular groove is adequate the pedicle to the preputial flap. These four
for the meatus [9]. While in the past we closed vessels are the distal end of the pudendal
this in a Heineke-Mikulicz transverse fashion to artery system and reliably provide excellent
create an epithelial-to-epithelial anastomosis, blood flow for the preputial flap.
today it is our practice to follow Snodgrass’ sug- (c) Laterally: The dissection progresses to join
gestion and leave the vertical incision open with- the deep plane of dissection dorsally with the
out closure [10]. Secondary epithelialization superficial plane ventrally lateral to the dor-
occurs very rapidly and satisfactorily and gives a sal preputial pedicle and lateral to the spon-
more normal slit-like configuration to the giosum with an incision running parallel to
meatus. the spongiosum and just outside its border.
31 The Onlay Island Hypospadias Repair 491
a b
c d
Fig. 31.1 The onlay island flap technique: (a) The apex preputial onlay flap with its pedicle is developed and sepa-
of glanuloplasty is identified (marked by dots). The rated from the dorsal penile skin. (d) Suturing the onlay
shaded triangle shows the epithelium to be excised. A flap to the urethral plate incorporates generous bites of the
subcoronal incision encircles the glans and continues spongiosum. (e) The glanuloplasty is the completion of
around the urethral plate. (b) The penile skin is dropped the medial rotation of the glanular wings that has failed
back and the glanular wings are mobilized. (c) The inner during development
(d) The pedicle: The pedicle is then separated 31.2.6 Artificial Erection
from the outer preputial skin in a plane just
below the intrinsic blood supply of the outer An artificial erection is now routinely carried out
prepuce. Holding stitches are placed on the unless the family reports perfectly straight erec-
prepuce, flattening it (Fig. 31.1c). tions. Normal saline for injection and a 23-scalp
vein needle is utilized with a tourniquet at the
base of the penis usually from a rubber band. It is
31.2.5 Elevation of Glans Wings important not to overfill the corporeal bodies.
The elevation of the glans wings will permit them

to be rotated around the urethroplasty. The plane 31.2.7 Design of Onlay Island Flap
of dissection is established laterally just outside
the corporal bodies and then carried medially, pre- In general, the urethral plate after the skin drop
serving an adequate amount of spongiosum with back measures 5–6 mm in width in small infants.
the urethral plate for a healthy reconstructed ure- Accordingly, a maximum of only a 1-cm-wide
thra. Care is taken not to divide the spongiosum onlay flap is required. At each end of the flap, it
insertion into the glans. Glans wing elevation usu- should taper, as there is no need to introduce a
ally continues until approximately the midcoronal new epithelium. Distally, the amount of epithe-
plane of the glans. The blood supply to the glans lium within the urethral meatus will be deter-
in hypospadias is via the dorsal vascularity, and mined by the dorsal tissue rearrangement
no glans jeopardy is created by this mobilization. opposite the apex of the ventral glanuloplasty.
Leaving too much neourethra distally can lead to 31.2.9 Skin Closure
distention and kinking to create the physiology of
an anterior urethral valve which will eventually The dorsal preputial skin is split in the midline,
then lead to the formation of a sizable urethral and then there is a slide-around step to move it
diverticulum. Having designed the onlay flap, it ventrally for a midline closure. In the careful
is sutured into place beginning with the suture dropping back of the ventral skin in the plane just
line underneath the pedicle utilizing running 7-0 below its intrinsic blood supply, one has abun-
polydioxanone suture. The monofilament nature dant protected blood supply to the ventral skin to
of this suture helps to avoid the frustrating drag permit it to be closed in the midline. This creates
through the tissues that is seen with braided the illusion of a normal penile ventral midline
sutures. Generally the stitches are placed from raphe. Having anchored the outer skin carefully,
inside the native urethra outward in order to facil- ventrally, and dorsally, care is taken to be certain,
itate gathering up a good bite of the supporting by precise measurement, that the same amount of
spongiosum (Fig. 31.1d). As the second suture epithelium is left on all sides of the penis.
line is completed distally opposite the pedicle, Asymmetry in residual skin on the shaft will give
the glans should be drawn together setting up the a lopsided appearance and yield an unsatisfactory
first stitch of the glanuloplasty ventrally at its eventual cosmetic result. Having trimmed the
apex. Before the glanuloplasty is begun, redun- excess skin, the closure is completed with subcu-
dant tissue from the pedicle is tacked over the ticular 7–0 polydioxanone suture. Usually the
suture lines with interrupted 7-0 polydioxanone designing of the closure proceeds most smoothly
suture. This creates further vertical displacement if the ventral midline closure is completed first
of suture lines and contributes to avoidance of before trimming the excess skin on either side. A
fistulas. subcuticular closure reliably avoids dermal suture
sinuses later.
31.2.8 Glanuloplasty
31.2.10 Urethral Stenting
Rotation medially of the mobilized glans wings
is carried out with interrupted 6-0 polydioxanone A 15 cm length of F6 medical grade silicone
sutures. The stitches are placed parallel to the tubing obtained from the manufacturer who
surface of the glans and 2–3 mm from the cut fashions this same material into ventriculoperi-
edge of the flat glans surface. This completes the toneal shunts is used for urinary diversion. Extra
rotation of the glans into a conical configuration holes are placed on the bladder end of the tube,
that was aborted embryologically. A second just and it is threaded into place protruding about
immediately subcuticular 7-0 Maxon layer 2 cm outside the urethral meatus. If there is any
ensures a smooth approximation of the glans difficulty threading the stent into place, this may
edge and should result in an almost imperceptible indicate the presence of a utricle, as is fairly
eventual scar (Fig. 31.1e). commonly seen in significant hypospadias.
As the glanuloplasty is completed, it will Either utilizing a small probe to anteriorly direct
bring to the midline the Firlit skirt flaps that the stent or utilizing the slightly stiffer 6-French
were designed with the initial incisions. The Kendal catheter made of polyurethane, which
skirt flaps are sutured into position with subcu- tends to pop over the entrance to the utricle, will
ticular stitches. The soft tissues associated deal satisfactorily with the placement of a cath-
with the skirt flaps may further contribute to eter into the bladder. The diverting stent is
the separation of suture lines and avoidance of secured by a 5-0 Prolene suture placed on the
fistulas. The rotated broad surface of the glans inner aspect of the urethral meatus in order to
brought together in the glanuloplasty achieves avoid any suture marks on the surface of the
the same goal. glans postoperatively.
31.2.11 Dressing 31.3 Outcomes
We continue to utilize Duckett’s “sandwich” In a personal series reported by Cooper, a 2-year

dressing. This consists of pieces of Telfa and experience between February 1997 and 1999
gauze on either side of the penis which is then was summarized [12]. Thirty-six onlay island
compressed against the lower abdominal wall hypospadias repairs were carried out in sequence
aimed at the umbilicus and held down by a piece without one fistula. However, no hypospadias
of Tegaderm. surgeon is immune from that complication, and
patients of mine have had fistulas subsequent to
that series. In this series of 36 consecutive
31.2.12 Aftercare patients, it is of importance to realize that only 4
needed a midline plicating stitch for minor resid-
The child is sent home on the day of the surgical ual bending of the penis. Three in the series,
procedure in all cases. The family is instructed to however, did come to redo surgery for the devel-
use pain medication acetaminophen with codeine opment of a urethral diverticulum. As none had
in a dose of 0.5–1 mg/kg every 4 h. Bladder spasms meatal stenosis, the cause was almost certainly
are minimal with the soft Silastic plastic stent but excess epithelium incorporated into the repair
are dealt with by routine oxybutynin 0.2 mg per distally.
kilo every 8 h for the first 48 h. After that, most
parents find they can reduce the dosage to just one
dose given before the child is put to sleep. We uti- 31.4 Conclusion
lize antibiotic prophylaxis with trimethoprim-
sulfamethoxazole at ½ to 1 teaspoon per day until With our current understanding of hypospadias
the urethral stent has been removed. surgery, the meticulous design of the surgery
Thirty-six hours after the procedure, the fam- from the beginning, paying particular attention to
ily teases the Tegaderm off the skin and then the fine points of the glanuloplasty, will permit an
soaks off the remainder of the dressing in a tub. operation to start out on the right foot. If errors in
Subsequent care comprises merely soaking the design are made initially, it is very difficult to res-
child in a comfortable tub twice a day for cue the situation later. An understanding of the
15–20 min. As long as there is just clean water in vascular anatomy of hypospadias, avoiding entry
the tub, there does not seem to be any adverse into the dorsal pedicle or the spongiosum ven-
effect from having the urethral stent go below the trally, will make for a hemostatic procedure with
surface of the water. Families are instructed to minimal blood loss. With meticulous attention to
avoid straddle-type activities for the child until dissection, there can be consistent preservation of
the urethral stent is removed. They are encour- the spongiosum, which can then be incorporated
aged to give the child extra fluid to drink as it is into the repair. Leaving the spongiosum maxi-
very rare to see these small stents become mally inserted into the tunica albuginea avoids
obstructed unless the child develops significant injury to this delicate vascular structure. The
dehydration and amorphous phosphate crystals spongiosal support contributes to a lower compli-
plug the tube. The child returns at 10–14 days cation rate, particularly the fistula rate, which
after surgery for division of the Prolene suture from our institution had previously been reported
holding the stent in place and its removal. at 6%. For these reasons, if a substitution urethro-
Generally, children void without any discomfort plasty is required, the onlay island operation con-
because epithelialization will have been com- tinues to be this reconstructive surgeon’s
plete by the time the Silastic stent is removed. operation of choice.
References 7. Snodgrass W, Patterson K, Plaire JC, Grady R,

Mitchell ME. Histology of the urethral plate: implica-
tions for hypospadias repair. J Urol. 2000; https://doi.
1. Elder JS, Duckett JW, Snyder HM. Onlay island flap
org/10.1016/s0022-5347(05)67233-3.
in the repair of mid and distal penile hypospadias
8. Hollowell JG, Keating MA, Snyder HM, Duckett
without chordee. J Urol. 1987;138:376. https://doi.
JW. Preservation of the urethral plate in hypospadias
org/10.1016/S0022-5347(17)43152-1.
repair: extended applications and further experience
2. Horton CE, Devine CJJ, Baran N. Pictorial history of
with the onlay island flap urethroplasty. J Urol. 1990;
hypospadias repair techniques. In: Horton CE, editor.
https://doi.org/10.1016/s0022-5347(17)39878-6.
Plastic and reconstructive surgery of the genital area.
9. Rich MA, Keating MA, Snyder H, Duckett
Boston: Little Brown; 1973. p. 237–47.
JW. Hinging the urethral plate in hypospadias
3. Asopa HS, Elhence IP, Atri SP, Bansal NK. One stage
meatoplasty. J Urol. 1989; https://doi.org/10.1016/
correction of penile hypospadias using a foreskin tube.
S0022-5347(17)39161-9.
A preliminary report. Int Surg. 1971;55(6):435–40.
10. Snodgrass W. Tubularized, incised plate urethroplasty
4. Duckett JW. Transverse preputial island flap tech-
for distal hypospadias. J Urol. 1994; https://doi.
nique for repair of severe hypospadias. Urol Clin
org/10.1016/S0022-5347(17)34991-1.
North Am. 1980;7(2):423–30.
11. Firlit CF. The mucosal collar in hypospadias
5. Avellán L, Knutsson F. Microscopic studies of
surgery. J Urol. 1987; https://doi.org/10.1016/
curvature-causing structures in hypospadias. Scand
S0022-5347(17)43878-X.
J Plast Reconstr Surg Hand Surg. 1980; https://doi.
12. Cooper CS, Noh PH, Snyder HM. Preservation of ure-
org/10.3109/02844318009106718.
thral plate spongiosum: technique to reduce hypospa-
dias fistulas. Urology. 2001; https://doi.org/10.1016/
S0090-4295(00)00999-7.
org/10.1016/S0022-5347(01)62711-3.
Perineal Hypospadias:
The Bilateral-Based (BILAB) Skin 32
Flap Technique
Ahmed T. Hadidi
Abbreviations In this chapter, we present the bilateral-based

(BILAB) flap for patients with grade IV hypospa-
BILAB Bilateral-based flap dias (proximal with deep chordee and perineal
CEDU Chordee excision and distal hypospadias). This is the natural gradual evolution
urethroplasty of the lateral-based flap technique [3]. The BILAB
DSD Disorders of sex development technique was the standard technique in the
LAB Lateral-based flap Hypospadias Center in Frankfurt, Germany, from
PIT Preputial island tube 2005 until 2013. Since then we have gradually
SIS Small intestinal submucosa shifted to the CEDU technique as the standard
STAC Straighten and close operation for grade IV hypospadias (see Chap. 33).
TALE Tunica albuginea longitudinal However, nowadays, we still use the BILAB
excision technique in two situations: (1) as a two-stage
procedure when the ventral surface of the penis is
so underdeveloped (usually the child will require
corporoplasty or incision of the corpora caver-
32.1 Introduction nosa and graft or flap) that it is unwise to perform
distal urethroplasty after chordee excision at the
Perineal hypospadias or grade IV hypospadias same procedure and (2) when the ventral surface
(and proximal hypospadias with deep chordee) of the penis looks good after chordee excision
remains a major challenge for the hypospadias and the patient is coming from a long distance
surgeon. The chordee excision and distal urethro- (usually overseas) that it is worthy to perform the
plasty (CEDU) technique [1] (Chap. 33), the chordee excision and complete urethroplasty in
bilateral-based (BILAB) flap [2], the lateral- one operation to save a second long journey with-
based flap [3], Koyanagi technique [4], Duckett out increasing the risks for the patient.
island flap [5], and Bracka two-stage repair [6]
are the current techniques used for perineal hypo-
spadias [7]. 32.2 Selection of Patients
The BILAB technique is suitable for patients

A. T. Hadidi (*) with grade IV hypospadias. For the purpose of
Hypospadias Center and Pediatric Surgery the study, grade IV hypospadias is a severe form
Teaching Hospital of the Johann Wolfgang Goethe of hypospadias defined when the urethral open-
University, Offenbach, Frankfurt, Germany ing lies at or proximal to the penoscrotal junc-
498 A. T. Hadidi
tion, associated with severe deep chordee that sion is deepened to incise the tunica albuginea
requires division of the urethral plate (curvature externa (TALE procedure) and stop at the inner
of 30 degrees or more during erection) and usu- circular layer. The corpora cavernosa is freed off
ally associated with bifid scrotum and occasion- the short urethral plate, the hypoplastic corpus
ally with penoscrotal transposition. spongiosum, and the outer longitudinal layer of
The BILAB technique can be performed as a the tunica albuginea using a sharp scissor. There is
one-stage procedure but usually as a two-stage usually a gritty sensation as if one is cutting
procedure. The indications to perform BILAB as through hard fibrous tissue or cartilage. In some
a two-stage procedure included: unilateral or patients, the TALE procedure is not enough, and
bilateral undescended testis, a narrow skin bridge the surgeon needs to incise the corpora cavernosa
(narrow urethral plate), and poor vascularity after and put a dermal graft, small intestinal submucosa
excision of extensive chordee, corporoplasty, or (SIS), and dura or a tunica vaginalis flap or graft.
incision of the corpora cavernosa and the use of Without excision of any skin, the urethral meatus
flaps or grafts to straighten the penis, small glans, usually retracts proximally to the base of the penis.
and associated medical conditions that favor
short anesthesia. In those patients, orchidopexy
As chordee in hypospadias involves the
(when indicated), chordee excision, and split of
ventral aspect of the penis only, degloving
the glans were performed during the first stage of
is limited to the ventral aspect of the penis
operation. The resultant defect on the glans and
only. This is important to preserve the dar-
penis was covered from the lateral penile skin
tos fascia and blood supply to the BILAB
and foreskin from both sides of the penis. In other
flaps.
words, a new healthy urethral plate is recon-
structed. Urethroplasty is performed 3–6 months
later. A transurethral Silastic catheter is used for
7–10 days depending on the degree of swelling 32.3.2 Glans Split
after surgery. An additional suprapubic catheter
was inserted for 13 days in the 26 patients who The glans is split in the midline from the subcoronal
had one-stage BILAB repair early in the study sulcus to the tip (Fig. 32.1b). The incision is deep-
between 2005 and 2008. Follow-up period ranged ened to allow a large and freely mobile two glanular
from 14 months to 15 years (mean 43 months). wings and to prepare adequate space for the new
urethra. In some patients, the surgeon may need to
excise some tissues deep inside the glans to provide
32.3 Operative Technique enough space for the new urethra. This does not
(Figs. 32.1 and 32.2) mean that small will become smaller as the excised
tissue will be replaced by the new urethra.
Under general anesthesia and caudal block, a trac-
tion suture of 4/0 Prolene is placed through the tip
of the glans. An artificial erection test is performed. 32.3.3 Incision and Mobilization
Careful assessment of the penis and both testes is of the Foreskin
performed. If the child has undescended testis, little
foreskin, or more than 40% curvature, the urethro- A transverse incision is made laterally at the
plasty is performed as a second-stage procedure. mucocutaneous junction of the foreskin on both
sides (Fig. 32.1c, inset, point A′). This simple
step provides adequate outer foreskin to cover
32.3.1 Chordee Excision the glans to the tip without tension. This is dif-
ferent from the Koyanagi technique where the
A sub-coronal skin incision is made 3–5 mm prox- inner mucosal collar is used to form the new
imal to the coronal sulcus (Fig. 32.1a). The inci- urethra.
32 Perineal Hypospadias: The Bilateral-Based (BILAB) Skin Flap Technique 499
a b c
d e f
Fig. 32.1 BILAB operative technique: (a) A transverse the two-stage BILAB repair. Both medial edges are fixed
subcoronal incision is made, and all the hypoplastic tis- together in the midline to the tip of the glans creating a
sues are removed including the outer layer of the tunica new urethral plate that is well fixed to the tunica albu-
albuginea (tunica albuginea longitudinal excision, TALE). ginea in the midline (inset) as well as laterally, and the
(b) The glans is split in the midline like an open book, and child continues to pass urine from the original perineal
some tissue may be excised to provide adequate space for urethral opening. (f) In one-stage repair, a U-shaped inci-
the new urethra (point A). (c) The proximal skin edge usu- sion is made around a catheter size F12. (g) Urethroplasty
ally retracts to the proximal penis, and a transverse lateral is completed with continuous subcuticular sutures, in two
skin incision is made in the prepuce at the junction layers. (h) A third protective layer is mobilized from the
between the outer skin and inner mucosa in both sides scrotal dartos/tunica vaginalis. (i) The tunica vaginalis
(inset, point A′). (d) Both medial edges are fixed together flap is fixed lateral to the urethroplasty in such a way to
in the midline starting at the proximal end (1) and gradu- seal the urethroplasty completely. (j) The remaining
ally until you reach the tip of the glans (2). Notice at the penile skin and foreskin are tailored to cover the penis and
inset how point A′ goes to point A at the tip of the glans in glans (©Ahmed T. Hadidi 2022. All Rights Reserved)
the midline. (e) The appearance at the end of first stage in
500 A. T. Hadidi
g h i j
a b
c d e
Fig. 32.2 The one-stage BILAB: (a and b) Preoperative tion of the new urethral plate. (f) The tailor-step approach
photos. (c) After incision of the short urethral plate and to ensure having the exact adequate diameter of the new
excision of the hypoplastic corpus spongiosum. (d) The urethra. (g) After urethroplasty. (h) One year after surgery
dotted line shows the origin of the flap that will form the (©Ahmed T. Hadidi 2022. All Rights Reserved)
right half of the new urethral plate. (e) After reconstruc-
32.3.4 New Urethral Plate end should be thinned (as one thins a graft to
ensure that it is properly fixed to the glans and
The penile skin and foreskin is mobilized from reduce the chance of mucosal prolapse) and
both sides, preserving its dartos fascia. The distal sutured together in the midline with tunica albu-
f g
ginea (Fig. 32.1d, e, inset). This starts at the prox- bladder, and the adequate width of the new ure-
imal end (1) and continues distally till the surgeon thra is determined by using 2–3 stay sutures
reaches the tip of the penis where point A′ is fixed that allow covering of the catheter with skin.
to point A. Thus a healthy new urethral plate is The flap edges are tailor cut step by step using
formed that is fixed to the tunica albuginea in the a sharp scissor (to preserve the fascia carrying
midline. Additional blanket sutures are taken to blood supply to the flap). It is recommended
fix the new “urethral plate” to the glans and penis not to cut the whole length of the flap at once
as required. If the surgeon decides to perform but rather cut one third, suture, and then cut
urethroplasty at a second operation, he may stop another third then suture. The aim is to avoid
at this stage, insert a transurethral catheter into having a redundant or narrow new urethra
the bladder and apply a dressing for 3–5 days. (Fig. 32.2f). Urethroplasty is performed using
6/0 polyglactin on a cutting needle in a con-
tinuous mattress manner (Fig. 32.1g). The
32.3.5 Urethroplasty remaining dartos tissue laterally is used as a
second layer, and a continuous 6/0 polyglactin
If the surgeon decides to perform a one-stage suture is sewn over the urethroplasty for
BILAB, a catheter size F12 is inserted into the support.
502 A. T. Hadidi
32.3.6 Protective Intermediate Layer sive tape that compresses the penis and catheter
against the lower abdominal wall which allows
A protective intermediate layer from the scrotal the free mobility of the patient [8]. The dressing is
dartos/tunica vaginalis is routinely used to cover removed at the time of removal of the transure-
the urethroplasty (Fig. 32.1h, i). thral catheter, and the penis is left exposed. If the
dressing became wet with urine or blood, the
dressing is changed using a similar standard
32.3.7 Meatoplasty dressing. Broad-spectrum antibiotics (second-
and Glanuloplasty generation cephalosporin) are used for 1 week.
Paracetamol and ibuprofen suppositories and
The two edges of the final meatus are sutured syrup are routinely used for pain relief as required.
together to the center of the protective intermediate Our standard postoperative follow-up protocol
layer at the tip (not to the urethroplasty) to have a includes examination after 3 months, 1 year,
slit-like meatus using polyglactin 6/0 single stitch 3 years, and then every 5 years until the child is
(Fig. 32.1j, inset). The glanular wings are approxi- 15 years old. Patients living long distances away
mated using polyglactin 6/0 transverse mattress- and failing to attend for follow-up were contacted
interrupted sutures. The remaining wound is closed by e-mail and phone using a structured question-
using continuous mattress polyglactin 7/0 stitches. naire [9]. Follow-up period ranged from
The remaining skin of the penis and foreskin 14 months to 15 years (mean 8 months).
is fashioned to provide ventral skin coverage of The results were assessed by an independent
the penis. This is not always easy depending on outpatient nurse who assessed the functional
the width of the flap used in the urethroplasty and results as well as the site, shape of the meatus,
the available penile skin. One patient had severe and the degree of residual curvature if present.
chordee and hypoplasia, and the foreskin was Results were considered satisfactory when the
brought down to cover the ventral penile surface boy achieved a slit-like meatus at the tip of the
through a buttonhole in the prepuce. glans penis, single forward stream, unimpeded
voiding, good cosmesis, and no need for second-
ary surgery for the urethra. Uroflowmetry was
32.3.8 Urine Drainage performed in toilet-trained children (nine
patients). Peak flow within 2 standard deviations
A transurethral Silastic catheter is retained for was considered normal.
7 days. If the BILAB technique is performed as
one-stage procedure, an additional suprapubic cath-
eter was inserted into the bladder for 13 days early 32.4 Patients and Methods
in the series. The aim is to allow adequate time for
the edema to subside before allowing urine to pass The BILAB technique was performed in 68
through the new urethra. Nowadays, we use a trans- patients in the period between January 2005
urethral silicone catheter or stent for 7–10 days. The and December 2011 and 24 patients over the
penis is stretched and fixed to the abdominal wall following 7 years. Twenty patients were lost to
using a single 4/0 Prolene suture. This helps to keep follow-up, and the remaining 72 patients con-
the penis straight and reduces infection. Ideally the stitute the present cohort for this report. Patient
suture should stay for 3 weeks after surgery. age ranged between 8 months and 2 years
(mean 10 months). All the patients had grade
IV hypospadias with severe deep chordee. The
32.3.9 Dressing new urethra was reconstructed during the same
setting in 29 patients (one-stage BILAB). The
All patients had a standard dressing in the form of remaining 43 patients had urethroplasty per-
gentamicin local eye ointment, gauze, and adhe- formed 3–6 months later as a two stage repair.
Patients with bilateral undescended testes (12) traction 6–12 months after surgery. This was cor-
underwent a standard protocol for disorders of rected by excision of scarred intermediate layer.
sexual development (DSD) including chromo- The fistula was closed routinely after 6 months.
somal analysis, ultrasound of the abdomen, A second intermediate layer was always used to
laparoscopy (3 patients), gonadal biopsy (2 protect fistula closure. The two children with dehis-
patients), and cystoscopy (2 patients). cent glans had meatoglanuloplasty performed suc-
cessfully 6 months after the first operation.
32.5 Results and Complications

(Figs. 32.2 and 32.3) 32.6 Discussion
Seventy-four patients maintained regular follow- In literature several publications use the term
up; the remaining (being from long distances) have “severe hypospadias” to describe patients with
not responded. Follow-up period ranged from very proximal hypospadias associated with
18 months to 15 years (mean 8 years). Satisfactory severe deep chordee [10–12]. The problem with
results were achieved in 54 patients (85%). Two this term is that it is unclear [11], and what is
children developed diverticula, two patients devel- “severe” for one surgeon may not be that “severe”
oped glans dehiscence, one child developed fistula, for another. Therefore, the author prefers to use
and one child had complete wound dehiscence the term perineal (or grade IV hypospadias) with
(urethroplasty was successfully reconstructed well-defined location of the meatus proximal to
6 months later). Three patients developed scar con- the penoscrotal junction [13]. The term severe,
a b c
d e
Fig. 32.3 The two-stage BILAB flap technique: (a and b) Preoperative photos. (c) After healing of the first stage. (d)
One month after urethroplasty. (e) One year after urethroplasty (©Ahmed T. Hadidi 2022. All Rights Reserved)
504 A. T. Hadidi
deep chordee is clearly defined as more than dorsal plication alone (p = 0.002) [20]. In the
30 degrees and necessitates division of the ure- present study, division and extensive excision of
thral plate to correct the penile curvature. the hypoplastic corpus spongiosum allowed
Surgical correction of perineal hypospadias is complete penile straightening in 60 of the 63
the most difficult and technically demanding pro- patients (95%). The remaining three patients had
cedure in hypospadias surgery and remains a curvature of 10 degrees, and this was considered
major challenge even for the most experienced acceptable, and no dorsal plication was per-
hypospadias surgeon. Current techniques could formed in any of the patients in the study.
be classified into one- or two-stage repair. The There are advantages and disadvantages of
two-stage repairs entails the use of either flaps or one-stage repair for perineal hypospadias.
grafts (skin or buccal mucosa) to cover the ven- Jayanthi suggested that one-stage repair has the
tral surface of the penis after division of the advantage of saving the child a second operation
urethral plate, and excision of the hypoplastic and also performing urethroplasty in a virgin
ventral structures and urethroplasty is performed field without the risk of having scars [21]. In the
as a second stage. One-stage repairs include the author’s experience, scarring was never a prob-
“preputial island tube” urethroplasty [5], the lem if the urethroplasty was performed as a two-
“lateral-based flap” technique [3], the Koyanagi stage repair.
repair [4], and its modifications [14]. How wide should we reconstruct the new ure-
There has been a major shift in the trends of thra? The distal new urethra should be as wide as
management of perineal hypospadias during the the original urethra; otherwise the new urethra
past few decades. In the 1960s and 1970s, the will be narrower than the proximal one with
two-stage repair was the standard. In the 1980s, increased incidence of fistula and stricture forma-
one-stage repair became popular due to the tion. The author uses catheter F12 as a routine as
increased use of preputial flaps. In the 1990s, long as the proximal urethra accommodates it. In
there has been a shift from preputial island tube patients with small glans, splitting the glans helps
urethroplasty [5] to preputial onlay island flaps to make space for a F12 catheter. Nevertheless,
[15] when the urethral plate could be spared glans dehiscence did occur in two patients with
because it is associated with relatively less com- small glans in the present study.
plications. However, that meant extensive use of Surgical correction of perineal hypospadias
dorsal plication and inadequate excision of the requires meticulous and extensive chordee dis-
ventral hypoplastic tissues and unnecessary section and excision of the ventral hypoplastic
shortening of the penis. In 1995, Bracka pre- structures in addition to division of the urethral
sented a personal series of 600 cases and advo- plate. This results in extensive postoperative
cated for a two-stage repair using full-thickness edema and requires urinary diversion for
skin grafts [6]. 7–10 days if urethroplasty is performed as one-
The pendulum has swung back due to unsatis- stage repair. Early in the series, the author used to
factory results with dorsal plication and extensive use suprapubic catheter if the urethroplasty is
mobilization under the urethral plate. Several performed in one stage.
papers in the 2000s strongly recommended two- Hadidi reported his experience with the
stage repair for severe and perineal hypospadias lateral-based (LAB) flap for proximal hypospa-
[16–18]. Catti et al. reported that division and dias and deep chordee with satisfactory results in
proximal mobilization of the plate allowed them 91% of the patients [3]. When applying the LAB
to achieve complete penile straightening in 51 of technique in perineal hypospadias, it was found
57 cases (89%) [19]. that the very proximal position of the meatus and
Braga et al. reported that none of their patients the severity of chordee to be corrected resulted in
undergoing simultaneous dorsal plication and rotation of the penis, difficulty to mobilize the
urethral plate transection had curvature recur- remaining skin to cover the penis and the new
rence, compared to 19 of 52 (37%) undergoing urethra, and severe postoperative edema.
The BILAB flap is a natural forward develop- tage that it occasionally contracts during healing
ment of the original (LAB) flap technique by which may require excision later as was the case
applying the Koyanagi [4] principle and using the with three patients included in the study.
lateral penile skin from both sides. It differs from It was interesting that the two children, who
the original Koyanagi in the following points: developed diverticula 3–6 months after
urethroplasty, were among the 26 patients who
1. The outer preputial foreskin is used and not had urethroplasty performed as a one-stage
the inner mucosa. This may reduce the possi- repair. There are probably two predisposing fac-
bilities of diverticula as the outer skin is less tors: the lack of rigid supportive tissue and rela-
likely to balloon. tive distal narrowing.
2. The two lateral flaps are sutured together Uroflowmetry was carried out in nine toilet-
with the tunica albuginea in the midline. This trained children following BILAB repair. Two
allows for fixation of the flaps and underly- children had a flat curve although they did not
ing fascia to the midline and creation of a have any clinical symptoms of obstructive void-
new urethral plate. It also reduces manipula- ing and ultrasound on the bladder did not show
tion of the dartos fascia carrying blood sup- increased bladder wall thickness. The signifi-
ply to the flaps. cance of uroflow following hypospadias repair
3. The glans is incised deeply in the midline, and remains uncertain [23].
the two lateral flaps are fixed to the tip of the The use of antibiotics in hypospadias surgery
glans before tubularization. This allows hav- is a controversial issue with different surgeons
ing a slit-like meatus at the tip of the glans. having different protocols [24]. Acknowledging
4. The technique is easily reproducible, and the the fact that hypospadias surgery is performed in
width of the new urethra can be accurately a potentially infected area near the anus and
controlled around a catheter F12 for recon- infection could have a very serious consequence
struction of a new healthy urethral plate. on the patient, the author uses antibiotics rou-
5. Depending on the amount of foreskin avail- tinely after hypospadias surgery as long as the
able, there was a chance to fulfill the wish of patient has a catheter (foreign body).
the parents and do foreskin reconstruction in The BILAB offers the surgeon the flexibility
24 of the 63 patients included in the study. during the operation to decide whether to pro-
6. The BILAB is flexible and can be used as a ceed and perform urethroplasty during the first
one- or two-stage procedure. operation or to defer it to a second operation
3–6 months later. The following factors influence
The BILAB has the advantage over the classic decision-making:
LAB that it is more symmetrical, reduces the
chances of rotation, and allows for equal skin 1. Associated undescended testis: It is better to
flaps from both sides to cover the penis in a more perform orchidopexy, chordectomy, and glans
natural manner. In addition, it provides flexibility split as the first stage and perform urethro-
to the surgeon as it can be done as a two-stage plasty as a second stage.
repair. 2. The severity of chordee: If the surgeon real-
The use of scrotal dartos/tunica vaginalis as a izes that the bridge of fascia and tissues bring-
protective intermediate layer has dramatically ing blood supply to the skin flap is narrow and
reduced the incidence of fistula (1 fistula in 63 compromised, it is wise to postpone urethro-
patients). Fistula used to be the commonest com- plasty to a second stage 3 months later.
plication following hypospadias repair [22]. With 3. Flat small glans: This is a relative indication
the routine use of protective intermediate layer to plan to split the glans in the first stage, line
and the scrotal dartos fascia/tunica vaginalis as a it with skin, and postpone urethroplasty
protective intermediate layer, the incidence of fis- 3 months later.
tula has declined dramatically. However, the 4. Associated medical conditions: Where it is
scrotal dartos/tunica vaginalis has the disadvan- preferable to avoid long anesthesia.
506 A. T. Hadidi
The BILAB technique was associated with and parental perception of urinary symptoms and cos-
metic outcomes among 4 repairs. J Urol. 2013; https://
low morbidity and low complication rate in the doi.org/10.1016/j.juro.2012.11.013.
long-term follow-up (mean 8 years). 13. Hadidi AT. History of hypospadias: lost in transla-
tion. J Pediatr Surg. 2017; https://doi.org/10.1016/j.
jpedsurg.2016.11.004.
14. Hayashi Y, Kojima Y, Mizuno K, Nakane A, Kohri
References K. The modified Koyanagi repair for severe proxi-
mal hypospadias. BJU Int. 2001; https://doi.
1. Hadidi AT. Perineal hypospadias: back to the future org/10.1046/j.1464-410X.2001.02029.x.
chordee excision & distal urethroplasty. J Pediatr Urol. 15. Elder JS, Duckett JW, Snyder HM. Onlay island flap in
2018; https://doi.org/10.1016/j.jpurol.2018.08.014. the repair of mid and distal penile hypospadias with-
2. Hadidi AT. Perineal hypospadias; the bilateral based out chordee. J Urol. 1987; https://doi.org/10.1016/
(BILAB) skin flap technique. J Pediatr Surg. 2014; S0022-5347(17)43152-1.
https://doi.org/10.1016/j.jpedsurg.2013.09.067. 16. Gershbaum MD, Stock JA, Hanna MK, Baskin
3. Hadidi AT. Lateral-based flap: a single stage ure- L, Hanna M, Khoury A. A case for 2-stage repair
thral reconstruction for proximal hypospadias. of perineoscrotal hypospadias with severe chor-
J Pediatr Surg. 2009; https://doi.org/10.1016/j. dee. J Urol. 2002; https://doi.org/10.1016/
jpedsurg.2008.08.012. s0022-5347(05)64399-6.
4. Koyanagi T, Matsuno T, Nonomura K, Sakakibara 17. Lam PN, Greenfield SP, Williot P, Zaontz MR,
N. Complete repair of severe penoscrotal hypo- Snodgrass W. 2-Stage repair in infancy for severe
spadias in 1 stage: experience with urethral mobi- hypospadias with chordee: long-term results after
lization, wing flap-flipping urethroplasty and puberty. J Urol. 2005; https://doi.org/10.1097/01.
“glanulomeatoplasty”. J Urol. 1983; https://doi. ju.0000179395.99944.48.
org/10.1016/s0022-5347(17)51732-2. 18. Johal NS, Nitkunan T, O’Malley K, Cuckow
5. Duckett JW, Baskin LS. Hypospadias. In: Gillenwater PM. The two-stage repair for severe primary hypo-
JY, Grayhack JT, Howards SS, Duckett JW, editors. spadias. Eur Urol. 2006; https://doi.org/10.1016/j.
Adult and pediatric urology. 3rd ed. St. Louis: Mosby- eururo.2006.01.002.
Year Book; 1996. p. 2549–89. 19. Catti M, Lottmann H, Babloyan S, Lortat-Jacob
6. Bracka A. Hypospadias repair: the two-stage alterna- S, Mouriquand P. Original Koyanagi urethro-
tive. Br J Urol. 1995; https://doi.org/10.1111/j.1464- plasty versus modified Hayashi technique: outcome
410X.1995.tb07815.x. in 57 patients. J Pediatr Urol. 2009; https://doi.
7. Hadidi AT. Proximal hypospadias with small flat org/10.1016/j.jpurol.2009.03.010.
glans: the lateral-based onlay flap technique. J 20. Braga LHP, Lorenzo AJ, Bägli DJ, Dave S, Eeg K,
Pediatr Surg. 2012; https://doi.org/10.1016/j. Farhat WA, Pippi Salle JL, Khoury AE. Ventral
jpedsurg.2012.06.027. penile lengthening versus dorsal plication for severe
8. Hadidi AT. Double Y glanuloplasty for glanu- ventral curvature in children with proximal hypo-
lar hypospadias. J Pediatr Surg. 2010; https://doi. spadias. J Urol. 2008; https://doi.org/10.1016/j.
org/10.1016/j.jpedsurg.2009.11.019. juro.2008.03.087.
9. Chrzan R, Dik P, Klijn AJ, de Jong TPVM. Quality 21. Jayanthi VR. The modified Koyanagi hypospa-
assessment of hypospadias repair with emphasis on dias repair for the one-stage repair of proximal
techniques used and experience of pediatric urologic hypospadias. Indian J Urol. 2008; https://doi.
surgeons. Urology. 2007; https://doi.org/10.1016/j. org/10.4103/0970-1591.40617.
urology.2007.01.103. 22. Hadidi AT. Fistula repair. In: Hadidi AT, Azmy AF,
10. Castagnetti M, El-Ghoneimi A. Surgical manage- editors. Hypospadias surgery, an illustrated guide.
ment of primary severe hypospadias in children: 1st ed. Heidelberg: Springer Berlin Heidelberg; 2004.
systematic 20-year review. J Urol. 2010; https://doi. p. 277–82.
org/10.1016/j.juro.2010.06.044. 23. Snodgrass W, MacEdo A, Hoebeke P, Mouriquand
11. de Mattos e Silva E, Gorduza DB, Catti M, PDE. Hypospadias dilemmas: a round table. J
Valmalle AF, Demède D, Hameury F, Pierre- Pediatr Urol. 2011; https://doi.org/10.1016/j.
Yves M, Mouriquand P. Outcome of severe hypo- jpurol.2010.11.009.
spadias repair using three different techniques. 24. Ben Meir D, Livne PM. Is prophylactic antimi-
J Pediatr Urol. 2009; https://doi.org/10.1016/j. crobial treatment necessary after hypospadias
jpurol.2008.12.010. repair? J Urol. 2004; https://doi.org/10.1097/01.
12. Castagnetti M, Zhapa E, Rigamonti W. Primary ju.0000124007.55430.d3.
severe hypospadias: comparison of reoperation rates
Chordee Excision and Distal
Urethroplasty (CEDU) for Perineal 33
Hypospadias
Ahmed T. Hadidi
Abbreviations distal urethroplasty” (CEDU) [3], Koyanagi tech-

nique [4], Duckett island flap [5], and Cloutier-
ASD Anoscrotal distance Bracka two-stage repair [6] are the current
BILAB Bilateral-based flap techniques used for perineal hypospadias [1].
CEDU Chordee excision and distal Recent reports demonstrated that one-stage
urethroplasty repairs of “severe” proximal and perineal hypo-
DSD Disorders of sex development spadias often produce unsatisfactory results, and
PST Penoscrotal transposition the majority of patients require further surgery
TALE Tunica albuginea longitudinal [2, 3, 7]. The principle of the “chordee excision
excision and distal urethroplasty” (CEDU) technique is to
excise the severe chordee, mobilize the foreskin
from both sides ventrally, and reconstruct a new
urethra distally as a first stage repair leaving the
33.1 Introduction perineal orifice intact as a “natural perineal fis-
tula” [3]. Three to six months later, the proximal
Perineal hypospadias or grade IV hypospadias urethra is reconstructed together with the correc-
[1] is a complex deformity consisting of a peri- tion of bifid scrotum.
neal urethral orifice, hypoplastic ventral penile
structures resulting in severe chordee, small sized
glans and penis, bifid scrotum, and often unde- 33.2 Selection of Patients
scended testis and occasionally penoscrotal
transposition [2, 3]. It remains a major challenge The CEDU technique is suitable for patients with
for the experienced hypospadias surgeon. The grade IV hypospadias. For the purpose of the
bilateral-based flap [2], “chordee excision and study, grade IV hypospadias is a severe form of
hypospadias defined when the urethral opening
lies at or proximal to the penoscrotal junction,
associated with severe deep chordee that requires
A. T. Hadidi (*) division of the urethral plate (curvature of 30
Hypospadias Center and Pediatric Surgery degrees or more during erection) and usually
Department, Sana Klinikum Offenbach, Academic associated with bifid scrotum and occasionally
University, Offenbach, Frankfurt, Germany with penoscrotal transposition.
508 A. T. Hadidi
33.3 Operative Technique the tunica albuginea results in the correction of

(Figs. 33.1, 33.2, 33.3, 33.4) deep chordee and penile lengthening of 1–2 cm
in about 70% of patients with severe chordee >30
Under general anesthesia and caudal block, a degrees (Figs. 33.3 and 33.4). As the chordee in
traction suture of 4/0 Prolene is placed through hypospadias involves the ventral aspect of the
the tip of the glans. An artificial erection test is penis only, degloving is limited to the ventral
performed. Careful assessment of the penis and aspect of the penis. This is very important to pre-
both testes is performed. If the child has unde- serve the dartos fascia and blood supply to the
scended testis, orchidopexy is performed as the lateral skin flaps.
first step.
33.3.2 Incision of the Glans

33.3.1 hordee Excision (Tunica
C
Albuginea Longitudinal The glans is split in the midline from the subcor-
Excision (TALE)) onal sulcus to the tip (Fig. 33.1b). The incision is
deepened to prepare two large mobile glanular
A subcoronal skin incision is made 5 mm proxi- wings and to prepare adequate space for the new
mal to the coronal sulcus (Fig. 33.1a). The inci- urethra. Inadequate mobilization of the glanular
sion is deepened to reach the tunica albuginea, wings may leave a degree of glanular tilt and may
and the corpora cavernosa is freed off the short strangulate the new urethra.
urethral plate and hypoplastic corpus spongio-
sum using a sharp scissor (Fig. 33.1b). There is
usually a gritty sensation as if one is cutting 33.3.3 Incision and Mobilization
through hard fibrous tissue or cartilage. Byars of the Foreskin
(1951) described it nicely “as if you are excising
Dupuytren’s contracture of the hand” [8]. The A transverse incision is made laterally at the
outer tunica albuginea longitudinal is also excised mucocutaneous junction of the foreskin on both
(TALE) (Figs. 33.2 and 33.3). The sharp scissor sides (Fig. 33.1c). This simple step provides ade-
cuts along the longitudinal axis of the penis to quate outer foreskin to cover the glans to the tip
avoid injury of the circular layer of tunica albu- without tension. It is important to avoid the inner
ginea. Excision of the outer longitudinal layer of preputial mucosa to reduce the chances of diver-
Fig. 33.1 The CEDU technique: (a) A transverse sub- the tunica albuginea in the midline (inset) as well as later-
coronal incision is made, and all the hypoplastic tissues ally, and the child continues to pass urine from the origi-
are removed down to the tunica albuginea. (b) The glans nal perineal urethral opening. (f) A catheter size F12 is
is split in the midline like an open book, and some tissue inserted into the bladder. Two longitudinal incisions are
may be excised to provide adequate space for the new ure- made that stop 1 cm before the meatus. (g) Distal urethro-
thra. (c) The proximal skin edge usually retracts to the plasty is performed using Vicryl 6/0 around a catheter size
proximal penis, and a transverse lateral skin incision is F12. (h) Two protective layers are mobilized from both
made in the prepuce at the junction between the outer skin sides. (i) A slit-like meatus is created by approximating
and inner mucosa in both sides (inset, point A’). (d) Both the glanular wings at the midline. The remaining skin is
medial edges are fixed together in the midline starting at tailored to cover the glans and penis. The catheter is
the proximal end (1) and gradually until you reach the tip replaced with a Silastic catheter size F10 at the end of the
of the glans (2). Notice at the inset how point A’ goes to procedure. The glans is fixed to the abdominal wall to
point A at the tip of the glans in the midline. (e) Both stretch the penis and provide stability for 2–3 weeks after
medial edges are fixed together in the midline to the tip of surgery (©Ahmed T. Hadidi 2022. All Rights Reserved)
the glans creating a new urethral plate that is well fixed to
33 Chordee Excision and Distal Urethroplasty (CEDU) for Perineal Hypospadias 509
a b c
d
e f
i
g h
510 A. T. Hadidi
ticulum formation. This is different than in the plasty is performed using a continuous subcuticu-
Koyanagi technique where the inner mucosal collar 6/0 polyglactin mattress suture on a cutting
lar is used to form the new urethra. needle. The dartos tissues on both sides are mobi-
lized and sutured over the urethroplasty as two
protective layers using 6/0 continuous polyglactin.
33.3.4 New Urethral Plate When the dartos fascia is thin, another protective
layer can be taken from the scrotal fascia.
The penile skin and outer foreskin are mobilized
from both sides, preserving the dartos fascia proxi-
mally, and are sutured together in the midline with 33.3.6 Meatoplasty
tunica albuginea (Fig. 33.1d, e, inset). The flaps and Glanuloplasty (Figs. 33.3
should be very thin at the glans region (as if you are and 33.4)
preparing a graft) to avoid prolapse of the urethral
mucosa later and to allow closure of the glanular A slit-like meatus is created by approximating the
wings later without tension. Thus a healthy new two glanular wings in the midline, using a single
type of urethral plate is formed that is fixed to the stitch of 6/0 polyglactin (Fig. 33.1i, inset). Care
tunica albuginea in the midline. Additional “blan- must be taken to avoid going through the epithe-
ket” sutures are taken to fix the new urethral plate lium of the neourethra to avoid glanular fistula
to the glans and penis as required. later. Two additional 6/0 polyglactin stitches fix
the neourethra to the inside circumference of the
glans tip at 3 and 9 o’clock. The glanular wings are
33.3.5 Distal Urethroplasty approximated using interrupted 6/0 polyglactin
(Fig. 33.1f–h) transverse mattress sutures. The remaining wound
is closed using a continuous 6/0 polyglactin mat-
A size F12 catheter is inserted into the bladder, and tress stitch. The remaining skin of the penis and
the adequate width of the new urethra is deter- foreskin is fashioned to provide ventral skin cover-
mined by using 2–3 stay sutures that allow coverage of the penis. This is not always easy depending
ing of the catheter with skin without incision of the on the width of the flap used in the urethroplasty
skin (Fig. 33.3). Two parallel incisions are made and the available penile and preputial skin.
guided by the three stay sutures using the sharp
scissor (to preserve the fascia carrying blood sup-
ply to the flap). Proximally, the incisions stop 1 cm 33.3.7 Urine Drainage
distal to the meatus. I do not recommend cutting
the whole length of the flap at once, but rather cut The Nelaton catheter size F12 is replaced with a
one third, suture, and then cut another third and Silastic Tiemann balloon catheter F10 at the end
then suture (Fig 33.3). The aim is to avoid having of the operation for 4 days into a urine bag.
a redundant or narrow new urethra. Distal urethro- Urethral stents can also be used with the same
Fig. 33.2 The technique of severe chordee excision layer. (b) The approach to excise the tunica albuginea lon-
(TALE: tunica albuginea longitudinal excision): In order gitudinal using a sharp scissors and cutting along the lon-
to correct severe chordee in perineal hypospadias, we gitudinal axis of the penis. No skin was excised at all; the
excise all the ventral hypoplastic tissues as well as the skin simply retracted very proximally. (c) The length of
outer longitudinal tunica albuginea layer. (a) After the the penis in a child with perineal hypospadias (2.5 cm).
subcoronal incision, the hypoplastic tissues were excised (d) The length of the same penis after TALE (4 cm)
as well as the tunica albuginea longitudinal distally. (©Ahmed T. Hadidi 2022. All Rights Reserved)
Proximally, you can still see the white fibrous longitudinal
a b
c d
512 A. T. Hadidi
Fig. 33.3 Perineal

hypospadias: (a) A
6-month-old child with
perineal hypospadias,
severe chordee, and bifid
scrotum. (b) The penis
measured 2 cm in length.
The child had a left
undescended testis, and
orchidopexy was done at
the beginning of the
operation. (c) After total
chordee excision using
the TALE technique, the
foreskin was mobilized a b
ventrally and medially,
and a new urethral plate
was reconstructed. (d) A
catheter size F12 was
inserted, and three stay
sutures were put to
accurately determine the
exact width needed. (e)
The skin was incised in a
step manner. (f) At the
end of the operation,
notice that the penis was
fixed with Prolene 4/0 to
help straighten the penis,
allow proper healing, and
avoid coiling of the penis
(©Ahmed T. Hadidi 2022.
c d
e f
a b c
d e
Fig. 33.4 Perineal hypospadias with severe chordee, Z-plasty performed at the penoscrotal junction. (e) One
bifid scrotum, penoscrotal transposition, and high imper- year after closure of the perineal orifice and correction of
forate anus. (a) Before surgery. (b) At the end of operation bifid scrotum. Notice how the scrotum has healed with
of “chordee excision and distal urethroplasty” (CEDU). minimal scarring, and one can hardly see the Z-plasty at
(c) Three months after CEDU. Notice that although the the penoscrotal junction. The penoscrotal transposition
penis looks nice, the scrotum is still bifid and the child still has corrected itself as the bifid scrotum was corrected
passes urine from the perineum. (d) At the end of the (©Ahmed T. Hadidi 2022. All Rights Reserved)
proximal urethroplasty operation. Notice the small
514 A. T. Hadidi
result. After 4 days, no stent or catheter is needed formed as the first step to ensure that the penis is
as urine is diverted away from the new urethra adequately straight. Care is taken to avoid hair-
though the original orifice. bearing scrotal skin. Usually, the perineal mucosa
is wide enough to close over a catheter size F12
using the Thiersch principle. In 14 children, the
33.3.8 Abdominal Wall fixation perineal mucosa was not wide enough, and a skin
flap from the penis was mobilized laterally or dis-
The glans penis is fixed to the anterior abdominal tally to ensure a wide healthy proximal urethra. It
wall with one Prolene 4/0 stay suture for is important to have two thick protective layers
2–3 weeks (Figs. 33.3f and 33.5i, j). from scrotal fascia on both sides to reduce the
chances of diverticulum formation.
A Z-plasty at the penoscrotal junction was
33.3.9 Dressing often required to create an angle between the
penis and scrotum (87 patients). Again, the F12
All patients had a standard dressing in the form of Nelaton catheter is replaced with a F10 silicon
gentamycin local eye ointment, gauze, and adhe- Foley catheter that drains into a urine bag for
sive tape that compresses the penis and catheter 7 days. The glans penis is fixed to the abdominal
against the lower abdominal wall and allows free wall with 4/0 Prolene suture for 2–3 weeks. A
mobility of the patient [9]. The dressing is removed drain in the scrotum was needed in four patients
at the time of removal of the transurethral catheter, after mobilization of the scrotal fascia as a pro-
and the penis is left exposed. If the dressing tective layer to avoid development of scrotal
became wet with urine or blood, the dressing was hematoma.
changed using a similar standard dressing. Broad-
spectrum antibiotics (second-generation cephalo-
sporin) were administered for 1 week. Paracetamol 33.4 Patients, Results,
and ibuprofen suppositories and syrup were rou- and Complications (Figs. 33.2
tinely used for pain relief as required. and 33.3)
The CEDU technique has been our standard tech-

33.3.10 S
tage II: Perineal (Proximal) nique for patients with perineal hypospadias or
Urethroplasty (Fig. 33.4c, d) patients with severe chordee >30 degrees since
2013. Between January 2013 and December
Proximal urethroplasty was routinely performed 2016, the CEDU technique was performed in 126
3–4 months after CEDU after edema subsides patients with perineal hypospadias including 14
and healing is adequate. Adherent scar (if pres- patients from other countries. It is preferred to
ent) was excised, and artificial erection was per- operate on patients with perineal hypospadias as
Fig. 33.5 Preoperative hormone therapy: (a, b) A penile length doubled from 1 to 2 cm as well as the ano-
6-month-old baby with perineal hypospadias, severe chor- scrotal distance. (f) The TALE technique was performed.
dee, bifid scrotum, and very small penis. (c) Dimensions (g) Erection test confirmed the straightening of the penis.
of the glans and penis were very small; glans width was (h) At the end of operation, observe the major change
7 mm, dorsal glans length was 7 mm, ventral glans length from (a) to (h). (i, j)The penis was fixed to the abdominal
was 3 mm, and penile length was 1 cm and ASD was wall for 3 weeks with 4/0 Prolene sutures (©Ahmed
1 cm. (d, e) He received hormone therapy for 3 months; T. Hadidi 2022. All Rights Reserved)
the glans dimensions increased from 7 to 12 mm, and the
a b
c d
e f g
h i j
516 A. T. Hadidi
early as 6 months because it increases the chance rectourethral fistula closure. Three months later,
to correct the severe chordee using the TALE perineal urethroplasty was performed. Closure
technique without the need to incise the corpora of colostomy was performed in a separate oper-
cavernosa. Patients who had persistent chordee ation 6 months after the perineal urethroplasty.
after the TALE procedure underwent tunica vagi- The results were assessed by an independent
nalis flap or dermal graft, and the first operation outpatient nurse who assessed the functional
ends as a two-stage BILAB technique. Patient results as well as the site, shape of the meatus,
age ranged from 6 months to 2 years (mean and the degree of residual curvature if present.
8 months). On presentation, all the patients had Results were considered satisfactory when the
perineal hypospadias (meatus proximal to peno- boy achieved an apical slit-like meatus at the tip
scrotal junction) with severe deep chordee and of the glans penis, single forward stream, unim-
bifid scrotum. At operation, all patients had peded voiding, good cosmesis, and no need for
severe chordee excision, splitting of the glans, secondary surgery for the urethra.
and distal urethroplasty performed in the first
operation. Patients with unilateral or bilateral
undescended testis had the testis fixed during the 33.6 CEDU Technique in Adults
first operation.
The CEDU technique has been also used in three
adults with severe scarring and chordee. It is a
33.5 Hormone Therapy viable option when the patient has had multiple
operations including buccal mucosal grafts, and
It is our standard to use hormone therapy between the surgeon is not sure of the vascularity of the
the CEDU first operation and the second stage of bed in case he applies another graft (Fig. 33.6)
proximal urethroplasty if the glans width is (see Chap. 51).
12 mm or less. Patients with bilateral unde- Our standard postoperative follow-up protocol
scended testes underwent a standard protocol for includes examination after 3 months, 1 year, 3
disorders of sexual development (DSD) includ- years, and then every 5 years until the child is
ing chromosomal analysis, ultrasound of the 15 years old.
abdomen, and laparoscopy (Fig. 33.5). Patients living long distances from the center
One child had associated high imperforate and who failed to attend for follow-up were con-
anus and was referred with sigmoid colostomy. tacted by e-mail and phone using a structured
During the first operation, “chordee excision questionnaire [10].
and distal urethroplasty” (CEDU) were per- Follow-up period ranged from 15 to 84 months
formed in addition to transanal pull-through and (mean 60). Satisfactory results were achieved in
Fig. 33.6 CEDU in adults: (a) An adult with severe scar- design the width of the new urethra around two catheters
ring and chordee following multiple operations including with a total of F30, using four stay sutures before any skin
buccal mucosa graft twice. (b) The penis measured 4 cm incision. (f) Skin incision was made in a step manner. (g)
because of the scarring and curvature. (c) After excision Urethroplasty was performed using 6/0 Vicryl on a cutting
of the scar tissue and TALE procedure, the penis became needle. The urethroplasty was covered with two protective
7 cm in length. (d) After incision of the glans and recon- layers from the surrounding tissue. (h) The final appear-
struction of the new urethral plate, the penis became 8 cm ance at the end of operation (©Ahmed T. Hadidi 2022. All
in length. (e) It was important to accurately plan and Rights Reserved)
a b
c d e
f g h
518 A. T. Hadidi
chordee is defined as “ventral curvature more

than 30 degrees that necessitates division of the
urethral plate to correct the penile curvature with-
out shortening the penis.”
Perineal hypospadias can be considered a
“syndrome” that is usually associated with a bifid
scrotum, small-sized glans and penis, unde-
scended testis, and occasionally penoscrotal
transposition. This subgroup of hypospadias
(grade IV) are challenging and technically
demanding because the surgeon has to deal with
the deep chordee, the hypoplastic ventral struc-
Fig. 33.7 Midterm follow-up: Child with perineal hypo- tures (i.e., lack of healthy vascularized tissues
spadias, severe chordee, penoscrotal transposition, and ventrally), the small-sized glans, the bifid scro-
bifid scrotum 5 years after surgery with normal urine tum, and penoscrotal transposition in addition to
stream (©Ahmed T. Hadidi 2022. All Rights Reserved) reconstructing a new long urethra. Unless we
classify this subgroup of hypospadias separately,
114 patients (90%) (Fig. 33.7). Three patients we will continue mixing apples with oranges.
developed glans dehiscence, two developed a fis- The first challenge the surgeon encounters is
tula, and seven children developed urethral diver- to correct the associated deep chordee. The tim-
ticulum through the 7 years. The fistula was ing of chordee excision to our mind is very
routinely closed after 6 months. A second inter- important. The author operates on perineal hypo-
mediate layer was always used to protect the fis- spadias as early as 6 months in an attempt to cor-
tula closure. The three children with glans rect the curvature before corporeal disproportion
dehiscence had meatoglanuloplasty performed develops as suggested by Cecil in 1932 who
successfully 6 months after the first operation. reported the histological findings and ventral cell
The seven children with diverticula did not atrophy in a 10-year-old boy with uncorrected
have an associated distal narrowing. The classic severe chordee [13].
complaint of the mother is that the child dripples The approach to correct deep chordee is a very
and the urine was smelly. The diverticulum was controversial issue and is discussed in detail in
at the proximal urethral segment. It was excised Chap. 8. Dorsal plication is ineffective and causes
around catheter size F12 and covered with three shortening of an already short penis and is associ-
protective layers, and a transurethral catheter was ated with high incidence of recurrence [12, 14–
used for a week. 16]. The author radically excises the hypoplastic
fascia and corpus spongiosum amalgamated
together as one tissue as the first step. This is fol-
33.7 Discussion lowed by excising the longitudinal layer of tunica
albuginea (TALE) as described in the chordee
The term “severe hypospadias” is often used to excision and shown in Fig. 33.2. This is totally
describe patients with proximal hypospadias different from simple degloving or simple corpo-
associated with severe deep chordee more than rotomies. This approach has been used since
30 degrees. However, this term is ill defined [11], 2005 with satisfactory results [2, 3] and have
and what is considered “severe” for one surgeon resulted so far in a straight penis without the need
may not be that “severe” for another [12]. of ventral grating or the so-called ventral corpo-
Therefore, the author prefers to use the term peri- rotomies. Ventral grafting is usually needed when
neal (or grade IV hypospadias [1]) with well- the child presents later (older than 2 years) when
defined location of the meatus proximal to there is corporeal disproportion. In such cases,
penoscrotal junction, and the term severe or deep the author prefers to use tunica albuginea flaps
taken from around the testis with intact blood perineal hypospadias [11, 12, 14–16]. Catti et al.
supply as described by Braga et al. [17]. In the reported that division and proximal mobilization
case the penis is still curved, the surgeon still has of the plate allowed them to achieve complete
the chance to perform dorsal plication (if he pre- penile straightening in 51 of 57 cases (89%) [21].
fers) during the second stage of proximal ure- Braga et al. reported that none of their patients
throplasty (the dorsal penis is untouched during undergoing simultaneous dorsal plication and
the CEDU procedure). urethral plate transection had curvature recur-
Children who underwent this approach since rence, compared to 19 of 52 (37%) undergoing
2005 are being closely followed, and long-term dorsal plication alone (p = 0.002) [15]. Long
follow-up will be objectively reported within et al. showed that one-stage repair of “severe
2–3 years after adequate numbers reach puberty. proximal” hypospadias is associated with more
Other techniques to correct severe chordee complications and required several redo opera-
include total urethral mobilization, penile disas- tions [7, 16]. In the present study with a 36-month
sembly, fairy cuts, and ventral corporotomies. It follow-up, division and extensive excision of the
is surprising that authors report a straight penis hypoplastic corpus spongiosum allowed com-
after three simple corporotomies without excis- plete penile straightening in all the patients. This
ing the whole hypoplastic amalgamated tissue is probably related to the fact that all the children
[18]. were less than 2 years of age.
As described in the bilateral flap technique Surgical correction of perineal hypospadias
[2], each of the two lateral skin flaps, used to differs from hypospadias correction of other
reconstruct the new urethra, has the advantage of grades in hypospadias because of the associated
receiving dual blood supply, proximally from the severe hypoplasia and severe, deep chordee. This
root of the penis as well as laterally from the pre- necessitates division of the urethral plate and
putial fascia mobilized ventrally. extensive excision of the ventral hypoplastic
Historically, the repairs of the late nineteenth structures including the outer longitudinal layer
century were three stages. Duplay recommended of the tunica albuginea. This results in significant
three steps or stages of repair [19]: (1) excision of postoperative edema and delayed healing.
chordee, (2) neourethra tube reconstruction, and Urinary diversion for 2–3 weeks postoperatively
(3) anastomosis of the neourethra to the proximal is often not enough. In some countries, especially
native urethra. Browne corrected proximal hypo- Germany, parents are not happy to go home with
spadias in two stages but added a perineal ure- a transurethral or suprapubic catheter, and the
throtomy to allow healing of the new urethra standard protocol is to stay in the hospital until
[20]. In fact Byars technique published in 1951 the catheter is removed. This is a major burden on
with a 10-year follow-up is based on the same beds and hospital resources.
principle [8]. In the 1960s and 1970s, the two- The idea behind CEDU is to try to have the
stage repair was the standard. In the 1980s, the best of both worlds, i.e. allowing extensive dis-
one-stage repair became popular due to the section of the hypoplastic structures and free
increased use of preputial flaps. In the 1990s, mobilization of the glanular wings, reconstruct-
there has been a gradual shift from preputial ing the new urethra in a virgin unoperated field,
island tube urethroplasty [5] to preputial island giving the new urethra time to heal without the
onlay flaps when the urethral plate could be potential irritant effect of urine, and early dis-
spared because it is associated with relatively charge of the patient without a catheter or
fewer complications [2, 3]. dressing.
The pendulum has swung back due to unsatis- The CEDU technique also gives the surgeon
factory long-term results with dorsal plication the chance to perform fine adjustment of the
and extensive mobilization under the urethral glans in the second operation as most patients
plate. Several reports in the 2000s strongly rec- with perineal hypospadias have small glans and
ommended the two-stage repair for severe and glans dehiscence was the commonest complica-
520 A. T. Hadidi
tion encountered in this study (three children). gen insensitivity [23]. De Castro Paiva et al. sug-
Surgeons who are keen to correct minor residual gested in a randomized double-blinded controlled
curvature, if present, after the first stage of the trial that local application of 1% testosterone pro-
CEDU procedure, can still perform dorsal plica- pionate ointment on the dorsal and ventral sur-
tion during the second stage of proximal urethro- face of the penis twice daily for 30 days before
plasty. This was not needed in any of the author’s surgery significantly increases the penile length
series during the short-term follow-up. and glans size [24].
One concern that is often encountered is The author has had major concern of the
would the new urethra contract when not used for potential systemic side effects of hormone ther-
3–4 months? If the neourethra is well- apy at an early age and did not use it routinely
vascularized, healthy, and wide, it does not con- early in his series. However, we have observed
tract or stenose. None of the patients included in that it increases the size of the glans and penis
the study developed narrowing of the new considerably. Therefore, we have started to use
urethra. hormone therapy between the CEDU technique
Another controversial issue is how wide and the second stage of proximal urethroplasty.
should the new urethra be? The new urethra This has helped to improve the final appearance
should be as wide as the original proximal ure- of the glans and increase the size of the penis in
thra. If the reconstructed neourethra is narrower general in patients with a very small phallus. The
than the native proximal one, there will be an author uses testosterone cream or 2 mg/kg testos-
increased chance of fistula and stricture forma- terone enanthate for 2–3 months depending on
tion. The author uses a size F12 catheter as a rou- the response. In those children, the increase in the
tine as long as the proximal urethra accommodates size of the penis and glans was permanent and
it. The catheter is replaced at the end of the pro- did not regress with time after withdrawal of the
cedure with a silastic catheter size F10 to avoid hormone therapy. The hypothesis behind using
tension on the suture line. the hormone after excision of the hypoplastic tis-
In our Hypospadias Center in Frankfurt, we sues in the first operation is that the hormone is
do not attempt to correct penoscrotal transposi- expected to function better in a healthy well-
tion (PST) during the first operation. It was the vascularized, straight penis.
author’s observation over the past 6 years with
125 patients with perineal hypospadias that when
we correct the bifid scrotum, the scrotum gradu- 33.8 Value of Abdominal Wall
ally migrated downward and there was no need to Fixation
operate and correct the PST. The disadvantage in
correcting the PST during the first operation is It is our practice now to stretch the penis and fix
that it may jeopardize the blood supply to the it to the anterior abdominal wall with one non-
flaps used for urethral reconstruction and the absorbable (Prolene 4/0) stitch (Figs. 33.3f and
appearance of the root of the penis is cosmeti- 33.5i, j) for 2–3 weeks. This may help straighten
cally less than ideal. the penis after extensive ventral excision of the
The use of testosterone therapy in hypospa- hypoplastic tissues, provides stability of the
dias surgery remains a controversial issue. Asgari penis, and helps healing and cleaning of the
et al. in a prospective randomized controlled wound. At the beginning, there was a concern
study suggested that in distal hypospadias, that it may cause pain and discomfort to the
administration of 2 mg/kg testosterone enanthate patient. Interestingly, none of the children or
once per month for 2 months preoperatively sig- adults who had this stitch complained of pain or
nificantly reduced postoperative complications in discomfort after surgery. The author has used
distal hypospadias with flat plate [22]. Snodgrass this stitch in the past 5 years now in more than
et al., on the other hand, found that 49% of 39 140 patients with proximal and perineal hypo-
patients with proximal hypospadias had andro- spadias without seeing suture tracts which is
rather common with penile skin rather than in cation rate. J Urol. 2017; https://doi.org/10.1016/j.
juro.2016.11.054.
the glans. 8. Byars LT. Functional restoration of hypospadias
The penis is a very important structure, and it deformities; with a report of 60 completed cases.
should function well and look good all through Obstet: Surg Gynecol; 1951.
the man’s life (about 80 years). Our aim as sur- 9. Hadidi AT. Double Y glanuloplasty for glanu-
lar hypospadias. J Pediatr Surg. 2010; https://doi.
geons is to achieve a good-functioning and good- org/10.1016/j.jpedsurg.2009.11.019.
looking penis in each individual patient. The final 10. Chrzan R, Dik P, Klijn AJ, de Jong TPVM. Quality
outcome is much more important than the num- assessment of hypospadias repair with emphasis on
ber of planned operations required to achieve our techniques used and experience of pediatric urologic
goal. With time and increasing experience, the urology.2007.01.103.
author has gradually changed his approach from 11. Castagnetti M, El-Ghoneimi A. Surgical manage-
one-stage procedure [25] to two-stage procedure ment of primary severe hypospadias in children:
[2, 3] to the “one-and-half” stages repair reported systematic 20-year review. J Urol. 2010; https://doi.
org/10.1016/j.juro.2010.06.044.
here. The CEDU technique has become our stan- 12. de Mattos e Silva E, Gorduza DB, Catti M, Valmalle
dard technique in all patients with perineal hypo- AF, Demède D, Hameury F, Pierre-Yves M,
spadias. Penile perception and sexual function Mouriquand P. Outcome of severe hypospadias repair
ultimately relate to the quality of life [26]. The using three different techniques. J Pediatr Urol. 2009;
mean follow-up of the CEDU is 60 months 13. Cecil AB. Surgery of hypospadias and epispadias
(Fig. 33.7). It is clear that we need to follow all in the male. J Urol. 1932; https://doi.org/10.1016/
the patients until after puberty (15-year follow- s0022-5347(17)72734-6.
up) in order to have an objective long-term evalu- 14. Castagnetti M, Zhapa E, Rigamonti W. Primary
severe hypospadias: comparison of reoperation rates
ation of the technique. and parental perception of urinary symptoms and cos-
metic outcomes among 4 repairs. J Urol. 2013; https://
doi.org/10.1016/j.juro.2012.11.013.
15. Braga LHP, Lorenzo AJ, Bägli DJ, Dave S, Eeg K,
References Farhat WA, Pippi Salle JL, Khoury AE. Ventral
penile lengthening versus dorsal plication for severe
1. Hadidi AT. History of hypospadias: lost in transla- ventral curvature in children with proximal hypo-
tion. J Pediatr Surg. 2017; https://doi.org/10.1016/j. spadias. J Urol. 2008; https://doi.org/10.1016/j.
jpedsurg.2016.11.004. juro.2008.03.087.
2. Hadidi AT. Perineal hypospadias; the bilateral based 16. Long CJ, Canning DA. Hypospadias: are we as good
(BILAB) skin flap technique. J Pediatr Surg. 2014; as we think when we correct proximal hypospa-
https://doi.org/10.1016/j.jpedsurg.2013.09.067. dias? J Pediatr Urol. 2016; https://doi.org/10.1016/j.
3. Hadidi AT. Perineal hypospadias: back to the future jpurol.2016.05.002.
chordee excision & distal urethroplasty. J Pediatr Urol. 17. Braga LHP, Pippi Salle JL, Dave S, Bagli DJ, Lorenzo
2018; https://doi.org/10.1016/j.jpurol.2018.08.014. AJ, Khoury AE. Outcome analysis of severe chordee
4. Koyanagi T, Matsuno T, Nonomura K, Sakakibara correction using tunica vaginalis as a flap in boys
N. Complete repair of severe penoscrotal hypo- with proximal hypospadias. J Urol. 2007; https://doi.
spadias in 1 stage: experience with urethral mobi- org/10.1016/j.juro.2007.03.166.
lization, wing flap-flipping urethroplasty and 18. Snodgrass W, Bush N. Staged tubularized auto-
“glanulomeatoplasty.”. J Urol. 1983; https://doi. graft repair for primary proximal hypospadias with
org/10.1016/s0022-5347(17)51732-2. 30-degree or greater ventral curvature. J Urol. 2017;
5. Duckett JW, Baskin LS. Hypospadias. In: Gillenwater https://doi.org/10.1016/j.juro.2017.04.019.
JY, Grayhack JT, Howards SS, Duckett JW, editors. 19. Duplay S. Sur le traitement chirurgical de
Adult Pediatr Urol. 3rd ed. St. Louis: Mosby-Year l’hypospadias et de l’epispadias. Arch Gen Med.
Book; 1996. p. 2549–89. 1880:5–257.
6. Bracka A. Hypospadias repair: the two-stage alterna- 20. Browne D. An operation for hypospadias. J R Soc Med.
tive. Br J Urol. 1995; https://doi.org/10.1111/j.1464- 1949; https://doi.org/10.1177/003591574904200703.
410X.1995.tb07815.x. 21. Catti M, Lottmann H, Babloyan S, Lortat-Jacob
7. Long CJ, Chu DI, Tenney RW, Morris AR, Weiss S, Mouriquand P. Original Koyanagi urethro-
DA, Shukla AR, Srinivasan AK, Zderic SA, Kolon plasty versus modified Hayashi technique: outcome
TF, Canning DA. Intermediate-term follow-up of in 57 patients. J Pediatr Urol. 2009; https://doi.
proximal hypospadias repair reveals high compli- org/10.1016/j.jpurol.2009.03.010.
522 A. T. Hadidi
22. Asgari SA, Safarinejad MR, Poorreza F, Safaei Asl A, of the hypospadic penis after hormone therapy (tes-
Mansour Ghanaie M, Shahab E. The effect of paren- tosterone and estrogen): a randomized, double-blind
teral testosterone administration prior to hypospadias controlled trial. J Pediatr Urol. 2016; https://doi.
surgery: a prospective, randomized and controlled org/10.1016/j.jpurol.2016.04.013.
study. J Pediatr Urol. 2015;11:143.e1–6. 25. Hadidi AT. Lateral-based flap: a single stage ure-
23. Snodgrass WT, Villanueva C, Granberg C, Bush thral reconstruction for proximal hypospadias.
NC. Objective use of testosterone reveals androgen J Pediatr Surg. 2009; https://doi.org/10.1016/j.
insensitivity in patients with proximal hypospa- jpedsurg.2008.08.012.
dias. J Pediatr Urol. 2014; https://doi.org/10.1016/j. 26. Winship BB, Rushton HG, Pohl HG. In pursuit of
jpurol.2013.07.006. the perfect penis: hypospadias repair outcomes.
24. de Castro Paiva KC, Bastos AN, Miana LP, de Souza J Pediatr Urol. 2017; https://doi.org/10.1016/j.
Barros E, Ramos PS, Miranda LM, Faria NM, Avarese jpurol.2017.01.023.
de Figueiredo A, de Bessa J, Netto JMB. Biometry
Preputial Island Flaps
34
Minu Bajpai and Apoorv Singh
Abbreviation penis resulting in a theoretical decrease in penile

torsion. In 1981, Duckett used the same principle
TIP Tubularized incised plate and described the tubularized preputial island
flap as the operation of choice for proximal hypo-
spadias with severe chordee [3]; the Duckett
island flap differed from Asopa’s flap in that
34.1 Introduction Duckett isolated the preputial tube from the
remainder of the dorsal skin on a vascular
In 1971, Asopa et al. had described the use of pedicle.
transverse preputial skin tube as a method of Duckett used a glans channel when he
single-stage correction of proximal hypospadias described the technique. This is no longer recom-
[1]. The flap was based on the superficial dorsal mended as meatal stenosis is liable to occur. It is
vessels of the penis. The authors had postulated preferred to make a deep Y-shaped incision of the
that the inner layer of prepuce was nearest to the glans and freely mobilize the two lateral glanular
distal urethra in its texture and, when used trans- wings.
versely, provides adequate length to form the dis-
tal urethra. However, since the pedicle of the flap
was not dissected and was brought to the ventral 34.2 he Operative Steps
T
surface through one side, there was the problem of Duckett Technique
of penile torsion in these patients. To overcome
the problem of penile torsion, in 1984, Asopa • A deep Y-shaped incision is made on the glans.
et al. proposed another procedure known as the The center of the Y is where the tip of the neo-
“double-faced preputial island flap” [2]. This meatus will be located. The upper two short
procedure, popularly termed “Asopa II,” consists limbs of the Y are each 0.5 cm long. The long
of an adjacent skin flap covering the mucosal flap vertical limb of the Y extends down the whole
of the neourethra. Both these flaps are maintained length of the glans penis to the coronary sul-
by the superficial dorsal vessels. The cutaneous cus (Fig. 34.1a).
surface graft is thus well vascularized and per- • The resultant three flaps are elevated, and a
mits a uniform distribution of the skin around the core of soft tissue is excised to create a space
for the neourethra. A subcoronal circumferen-
tial incision is made 3 mm below the coronal
M. Bajpai (*) · A. Singh
sulcus, and an artificial erection is performed.
Department of Paediatric Surgery, All India Institute
of Medical Sciences, New Delhi, India Meticulous excision of any chordee or fibrous
524 M. Bajpai and A. Singh
bands is carried out. This fibrous tissue is par- flap resulting from the Y incision is sutured to
ticularly heavy in the midline but may extend the upper dorsal end of the tube (Fig. 34.1e).
well laterally (Fig. 34.1b). • A small V is excised from the tip of the neo-
• Note: Some surgeons correct residual penile urethra to obtain a slit-like meatus (Fig. 34.1f).
curvature by dorsal plication. The mobilized glanular wings are rotated
• The urethral meatus is circumcised to free the medially around the neourethra. Three trans-
urethra from the overlying skin. The distal verse mattress sutures maintain a firm approx-
urethra and meatus are trimmed back to good imation of the glanular wings in the midline
spongiosum, and the cutaneous edges are (Fig. 34.1g).
removed. The proximal spatulated meatus and • There is a need to have a protective intermedi-
urethra are fixed to the corpora in a splayed ate layer. Ideally, one should take the tunica
out fashion to receive the proximal end of the vaginalis from the scrotum in all cases of long
obliqued neourethra. urethroplasty. Another option is to use Smith’s
• The penile and preputial skin is dissected free de-epithelialization technique to have a pro-
off the shaft from distal to proximal close to tective intermediate layer (Fig. 34.1h, i) [4, 5].
Buck’s fascia, preserving the arteries that con- • The remaining part of the prepuce is divided
stitute the pedicle to the preputial flap. in the midline and wrapped around the penis.
• A 1.5-cm-wide rectangular flap is prepared.
The length must suffice to bridge the gap
between the meatus and the tip of the glans 34.3 Mixed Interposition
(Fig. 34.1c). Extra length can be obtained by Neourethroplasty
going down into the penile skin in a horseshoe
fashion on either side. This technique was devised to address severe
• The flap is tubularized around a F12 (accord- hypospadias while still preserving the urethral
ing to the patient’s age) catheter and sutured plate when it is wide and viable enough after cor-
into the meatus. The caliber of the new urethra rection of chordee [6]. Here, the urethral plate is
should be F12 when completed. Polyglactin first elevated and then released in the middle by
7-0 continuous subcuticular sutures are used dividing it transversely. The transverse preputial
with a cutting needle. Interrupted sutures are island flap is harvested to retain a wider central
used on the ends so that excess length may be width, enough to be tubularized around a size
excised (Fig. 34.1d). F10 catheter. The length of the tube is determined
• Then the pedicle is separated from the outer by that of the gap created after chordee correc-
preputial skin in a plane just below the intrin- tion. The flaps distal and proximal to this tube
sic blood supply of the outer prepuce down to remain undisturbed to serve as onlay on the
the root of the penis. respective ends of the divided urethral plate. It is
• The flap is usually rotated so that the right side imperative to try to preserve the urethral plate
of the flap is attached to the proximal urethral and its blood supply despite division (Fig. 34.2).
meatus. An oblique anastomosis is made,
freshening up the proximal urethra so that
good tissue along with spongiosum is sutured 34.4 Addressing the Glans
to the tube. The suture line of the neourethra
lies against the corporal groove. The glans being a three-dimensional structure,
• The proximal anastomosis is fixed to the the diagrammatic description of the glans in the
tunica albuginea of the corpora. The neoure- existing literature has limitations in explaining
thra is gently pulled distally toward the glans the practical issues encountered during hypospa-
to avoid redundancy of the tube and kinking of dias surgery. The following factors seem to play
the proximal anastomosis. Any excess length an essential role in cosmesis and function post
of the tube is excised. The upper small median hypospadias repair:
34 Preputial Island Flaps 525
a b
c d
Fig. 34.1 (a–i) Transverse preputial island flap: (a) A Y the meatus, and the upper median flap is sutured to the
incision is made on the glans and a subcoronal circumferen- upper end of the tube. (f) A small V is excised from the tip
tial incision is made, and an artificial erection is performed. of the tube. (g) The mobilized glans wings are rotated medi-
(b) Meticulous excision of any chordee or fibrous bands is ally, and three transverse mattress sutures maintain firm
carried out. (c) A 1.5-cm-wide rectangular flap is prepared. approximation of the glanular wings in the midline. (h, i)
(d) The flap is tubularized. (e) The lower end is sutured to De-epithelialization of the skin to protect the neourethra
e f g
h i

a b c
Fig. 34.2 (a) Division of urethral plate and outlining the transverse island preputial flap. (b) Ventral transfer and tubu-
larization of preputial flap. (c) Mixed interposition neourethral tube with distal and proximal onlay
• There is a variation in glans size in patients of length of the ventral glans closure gets compro-
a similar age group. mised. This results in a mushroom-like effect
• The depth of the fossa navicularis varies from rather than the desired conical glans. This leads
shallow to deep within the same age groups. to an invariable compromise of the lumen of the
• The caliber of the urethra is also distal tube. Several notable attempts have been
age-dependent. made to overcome this but have met with unde-
sirable results. For example, the raw groove cre-
Bush et al. in 2015 published an article show- ated by the vertical incision [8] in the fossa
ing follow-up of their patients following urethro- navicularis to increase the lumen tends to have
plasty for both distal and proximal penile fibrosis with a resultant stricture. However, the
hypospadias [7]. They have shown that small partial thickness skin graft [9] postulated later
glans size (<14 mm) was an independent risk addresses this issue, albeit partially. When sig-
factor for urethroplasty complications, including nificant, the stricture increases the propensity to
fistulas, glans dehiscence, and urethral strictures. urethral fistula, and when less severe, it compro-
However, in this study, the authors had tubular- mises the distensibility and pliability of the glan-
ized the urethral plate over a F6 catheter across ular urethra.
similar age groups regardless of the glans size. It might seem that the demand for creating an
Therefore, to tubularize the urethra to an age- adequate size urethra will be least on bigger glans
specific urethral caliber, there would be more with deep fossa navicularis. In practice, even the
demand on the glans if it is small and when the deepest fossa navicularis in hypospadias is never
fossa navicularis is shallow. Thus, in an attempt sufficient enough to enable an adequate caliber of
to wrap the glans around the urethral tube, the the distal neoglanular urethra.
34.4.1 H
ow Best to Address pora. Thus, the suture line is protected from
the Glans? the risks of fistula formation.
• The ventral side of the distal tube remains
It is, therefore, critical in the repair of hypospa- covered by a “frock” of skin which represents
dias that the glans is addressed in a manner that the inner layer of the prepuce. The latter is the
prevents stricture formation and the resistance it same in color as the glans and provides enough
causes to the urinary outflow. width, precluding the need for approximation
of glans wings to cover the glanular part of the
neourethral tube. It particularly takes away the
demand from the glans wings from being
34.5 Dorsoventral Transfer wrapped around the urethral tube. A more sig-
of Preputial Tube nificant advantage it offers is that, unhindered
Urethroplasty by the glans wrap, the distal tube functions as
a pliable symphysis, thereby eliminating the
This technique attempts to overcome the compli- consequences of strictures of the distal ure-
cations of proximal hypospadias repair such as thra. This is a universal concern frequently
urethracutaneous fistula formation, residual chor- encountered in hypospadias repair.
dee post urethroplasty, meatal stenosis, and
hypospadias cripples. The hallmarks of this This technique essentially represents a single-
approach are the following: stage repair of hypospadias derived from the favor-
able features of previously described techniques,
• The suture line in its entire length is in close including Hodgson, Asopa, and Duckett’s repair
opposition to the ventral surface of the cor- methods (Figs. 34.3 and 34.4) (see Chap. 38).
Fig. 34.3 Dorsal longitudinal island flap: The design and incision of the flap. Notice that the fascia must be mobilized
to the root of the penis. A hole is made, through which the penis is pulled through (courtesy of Ahmed T. Hadidi)
a b
Fig. 34.4 (a–c) Penoscrotal transposition with severe is incised and compensated by a wide transvers island flap.
hypospadias and chordee. Boundaries of incision are indi- (c) The defect in the middle is covered by a graft. Coronal
cated by a dashed line. The penile skin exists only on the parts of glanular wings and inner preputial layer are excised,
dorsal side of the penis as a triangle. Penile and scrotal skin in order to achieve conical shape of the glans. Glanular ure-
are mobilized. There are three possibilities: (a) The urethral thral plate is incised in the midline allowing its folding dur-
plate is preserved. (b) The middle part of the urethral plate ing glanuloplasty (courtesy of Ahmed T. Hadidi)
34.6 Operative Technique tube, and a buttonhole is created through

(Fig. 34.5) which the penis is retrieved (Fig. 34.5e, f),
and the whole tube is simultaneously trans-
• A suture is placed on the glans to allow for ferred onto the ventral surface of the penis
traction (Fig. 34.5a). (Fig. 34.5g, h). The advantage of this maneu-
• The penis is degloved completely, and com- ver is that the suture line now faces the penile
plete release of the chordee is done, followed body leading to a reduced fistula rate. Care is
by Gitte’s test (Fig. 34.5b). Nesbit sutures are also taken to preserve the blood supply of the
placed if the chordee persists (Fig. 34.5c). remaining preputial skin, while transfer, as
• Incisions are given in the center of the fossa this skin, will later be used for penile
navicularis, starting shortly proximal to the tip coverage.
in the midline. The glans wings are lateralized. • The tube is then anastomosed proximally to
• Incisions are given, and a tubularized prepu- the native urethral opening, and distally the
tial flap is created over a F10 feeding tube. It edges of the skin surrounding the tube are
is imperative to note that the distal end of the sutured to the glans wings raised previously,
tube is wider than the proximal end to com- creating a wide meatus (Fig. 34.5i).
pensate for future stenosis (Fig. 34.5d). • The transferred flap’s remaining skin is now
• Following this, an incision is given at the pen- anastomosed with the penile skin to complete
opubic junction proximal to the preputial the skin coverage (Fig. 34.5j).
a b c
d e f
Fig. 34.5 (a) Penoscrotal hypospadias: (b) Complete through which the penis is retrieved. (f) The whole tube is
degloving of the penis and chordee confirmed with the simultaneously transferred onto the ventral surface of the
Gitte’s test. (c) Nesbit sutures are placed to correct chor- penis. (g, h) Creation of preputial tube: The thick arrow
dee. (d) Markings are made on the dorsal preputial and points to the direction which the urethral tube takes for its
penile shaft skin to demarcate a preputial strip of the skin final position. (i) Neourethral tube stitched to the neome-
to be tubularized over a 10F feeding tube. (e) Incision is atus. (j) After the skin cover
given at penopubic junction, and a buttonhole is created
g h
i j
stage hypospadias repair. Br J Plast Surg. 1998;51:

References 542–6.
6. Bajpai M. Mixed interposition neo-urethroplasty (A
1. Asopa HS, Elhence IP, Atri SP, Bansal NK. One stage technique for single stage hypospadias repair): surgi-
correction of penile hypospadias using a foreskin tube. cal craft. Prog Paediatr Urol [Internet] 1997;2(1).
A preliminary report. Int Surg. 1971;55(6):435–40. 7. Bush NC, Villanueva C, Snodgrass W. Glans size
2. Asopa R, Asopa HS. One stage repair of hypospadias is an independent risk factor for urethroplasty com-
using double island preputial skin tube. Indian J Urol. plications after hypospadias repair. J Pediatr Urol.
1984;1:41–3. 2015;11:355.e1–5.
3. Duckett JW. The island flap technique for hypospadias 8. Snodgrass W. Tubularized, incised plate urethro-
repair. Urol Clin North Am. 1981;8:503–11. plasty for distal hypospadias. J Urol. 1994; https://doi.
4. Smith D. A de-epithelialized overlap flap tech- org/10.1016/S0022-5347(17)34991-1.
nique in the repair of hypospadias. Br J Plast Surg. 9. Asopa HS, Garg M, Singhal GG, Singh L, Asopa J,
1973;26:106–14. Nischal A. Dorsal free graft urethroplasty for urethral
5. Telfer JR, Quaba AA, Kwai Ben I, Peddi NC. An stricture by ventral sagittal urethrotomy approach.
investigation into the role of waterproofing in a two- Urology. 2001;58:657–9.
The Modified Asopa (Hodgson XX):
The Procedure to Repair 35
Hypospadias with Chordee
Jeffrey Wacksman
Abbreviations attached skin tube. Subsequent to this, Hodgson

modified this approach by incising the preputial
BXO Balanitis xerotica obliterans skin on a bias, leaving the newly constructed skin
TIP Tubularized incised plate tube attached to the larger surface area of the
incised preputial skin (Fig. 35.1). This modifica-
tion, termed the “Hodgson XX technique,” was
related to me by the late Norm Hodgson as a per-
35.1 Introduction sonal communication. The technique usually has
a lower fistula rate than the Duckett procedure [2]
Midpenile and proximal penile hypospadias is because the neourethra is left attached to the
usually associated with chordee that causes a underneath surface of the preputial skin [3].
ventral deflection of the penile shaft. The chordee Torsion is not a problem as long as the preputial
may be superficial (skin and/or dartos tissue) or skin is totally dissected back to the penopubic
deep (dysplastic urethral remnants and corpus angle. Because of this modification, I have been
spongiosum). With cases of superficial chordee, able to employ this procedure in over 250 patients
this is usually corrected by dividing the skin and with midpenile to penoscrotal hypospadias.
dartos tissue. In many cases, the urethral plate
can be tubularized via the TIP procedure and/or
onlay graft placed to repair this defect. When the 35.2 Operative Technique
chordee is more extreme, then the urethra itself
will need to be constructed utilizing some type of After routine general anesthesia, the patient is
the skin, usually from the prepuce. In 1971, moved to the foot of the operating room table and
Asopa et al. reported on a transverse pedicle flap placed in a frog position. We employ the operat-
utilizing the underneath surface of the prepuce to ing room microscope and usually sit to the side of
replace the urethra [1]. The major problem with the patient. A traction suture of 5-0 silk is placed
this technique was penile torsion due to the trans- through the glans, and a vessel loop is placed at
ference of the entire preputial skin with the the base of the penis to act as a tourniquet. An
incision is made in either transverse or vertical
This Chapter is a reprint from the First Edition. fashion in the dysplastic urethral plate. The dys-
plastic tissue is resected (Fig. 35.1a, b), and an
J. Wacksman (*) artificial erection is accomplished (Fig. 35.1c). If
Emeritus of Pediatric Urology, University of the penis is straight, then no further chordee
Cincinnati College of Medicine, Children’s Hospital
Medical Center, Cincinnati, OH, USA resection is necessary. If all of the chordee tissue
534 J. Wacksman
a b c
d e f
Fig. 35.1 (a–c) Diagram outlining chordee release and outline of neourethra from underneath the surface of foreskin.
(d–f) Neourethra created and rotated onto the ventral surface covered with foreskin
has been resected, but there still exists some Maxon® suture. There are other procedures avail-
remaining chordee, then the dissection is contin- able, but I do feel one has to incise Buck’s fascia
ued onto the dorsum of the penis. The preputial to the corporal tissue as opposed to just plicating
skin is dissected back to the penopubic angle. the tunica albuginea.
The broad-based dorsal neurovascular bundle is Having straightened out the penis, the under-
dissected free, and a modified Nesbit procedure neath surface of the foreskin is tented out with
is accomplished to completely straighten the traction suture of 6-0 chromic. For the neourethra,
phallus. I prefer two vertical incisions in Buck’s we usually construct this over an F8 Silastic stent.
fascia closed transversely with either 5-0 or 6-0 The underneath surface of the foreskin is cut and
35 The Modified Asopa (Hodgson XX): The Procedure to Repair Hypospadias with Chordee 535
rolled loosely over this urethral stent. I usually After completing the anastomosis, we direct
mark out the suture line with several interrupted our attention to the glans. The glans is split in the
6-0 chromic sutures followed by a running inter- midline and two large flaps are created. The neo-
locking 7-0 Maxon® suture. At either end of the urethra is laid in the glanular bed, and the glans
neourethra, interrupted sutures should be placed flaps are brought around the neourethra and
to allow for trimming of the suture line when the sutured in place with two layers: an inner layer of
neourethra is completed (Fig. 35.1d). Next, I usu- 6-0 or 7-0 Maxon® and an outer layer of 6-0 chro-
ally dissect the neourethra for a short distance mic. The neourethra is sutured to the glans with
from the preputial skin but leave the neourethra interrupted 6-0 chromic. The proximal anasto-
well attached to this underneath surface of the mosis is inspected, and a second layer of tissue is
foreskin. Therefore, the skin and neourethra will brought over the urethral anastomosis. The ven-
share a common blood supply, which is totally tral surface is covered with preputial skin, but the
different from the Duckett island pedicle flap pro- left side, which is the thinner side, is brought
cedure. Next, the preputial skin is cut on a bias. around and sutured in the midline on the ventral
Usually, the preputial skin on the right side is aspect of the penis. This will prevent any penile
large and contains the neourethra (Fig. 35.1e). torsion. The ventral skin flaps are closed with a
This is then rotated to the ventral aspect of the running 6-0 chromic catgut suture (Fig. 35.2a).
penis. The proximal end of the neourethra is For dressing we usually employ a single layer of
sutured to the distal end of the proximal urethra Tegaderm®, which will stay in place from 1 to
with 7-0 Maxon® over an F8 Silastic stent. I usu- 5 days. The urethral stent is left indwelling in the
ally spatulate the proximal urethra to assure a bladder to drain for 10–14 days. The patient is
wide-open anastomosis. I will also remove any placed on full-strength trimethoprim sulfa-
devascularized skin of the proximal urethra before methoxazole antibiotics while the stent is in
completing the anastomosis (Fig. 35.1f). place.
a b
Fig. 35.2 (a) Completed repair with penoscrotal transposition repaired as a single stage. (b) Appearance 6 months after
operation
536 J. Wacksman
35.3 Conclusion nary dribbling. It appears that urinary infection is

due to colonization of the skin of the neourethra,
My results in over 250 cases over the past while the urinary dribbling is related to urine col-
15 years show a fairly low complication rate lection in the neourethra. For this, I have recom-
(Fig. 35.2b). We have had a fistula rate of around mended that patients concentrate on stripping the
8%, but not all patients have had follow-up for a urethra following voiding. This usually solves the
long enough time. Occasionally, a patient may problem. Overall, the vast majority of patients
return 10 years after surgery with a small pin- have done quite well, and I still employ this pro-
point fistula. No patient has developed a urethral cedure in the properly selected patient.
stricture at the site of the anastomosis, but one
patient did develop balanitis xerotica obliterans
(BXO) 10 years after a successful repair. Meatal References
stenosis has not been a problem, but we have
repaired three significant urethral diverticula. 1. Asopa HS, Elhence IP, Atri SP, Bansal NK. One stage
correction of penile hypospadias using a foreskin tube.
Although penile swelling may initially be a con- A preliminary report. Int Surg. 1971;55(6):435–40.
cern 6 months to 1 year after surgery, this prob- 2. Duckett JW. The island flap technique for hypospadias
lem has usually resolved. Penile torsion has not repair. Urol Clin North Am. 1981;8:503–11.
been a problem as long as the steps described 3. Wacksman J. Use of the Hodgson XX (modified
Asopa) procedure to correct hypospadias with chor-
above are followed. One late complication has dee: surgical technique and results. J Urol. 1986;
been either an occasional urinary infection or uri- https://doi.org/10.1016/S0022-5347(17)45309-2.
Koyanagi Technique and Its
Modifications in the Management 36
of Proximal Hypospadias
Adham Elsaied
Abbreviation Early in the 1980s, some experts preferred

staged repair in children with proximal hypospa-
OUPF One-stage urethroplasty with paramea- dias [5, 6]. Later Woodard and Parrott developed
tal foreskin flap a one-stage repair combining varieties of urethro-
plasty [7]. They concluded that these patients are
candidates for a one-stage procedure at an early
age with a high degree of success; however
36.1 Introduction around one third will require a secondary proce-
dure for complete correction of the anomaly, ren-
The main goal of hypospadias surgery is creating dering one-stage repair a challenging issue in this
a cosmetically accepted penis with symmetric particular group.
appearance of the glans and shaft, a normal posi- One-stage urethroplasty with parameatal
tion of the meatus, and an adequate uniform cali- foreskin flap (OUPF) was first described by
ber of the urethra, achieving normal erection and Koyanagi as a single-stage technique applica-
voiding [1]. ble to all types of proximal hypospadias. It
Proximal variants of hypospadias have been a appeared to provide an ideal repair for severe
real challenge to all pediatric surgeons; thus hypospadias [8].
many techniques have been devised and modu-
lated to treat those severe forms. The main debate
has been and will continue for a long time to 36.2 The Original Technique
come: one-stage versus staged repair for proxi- [9–11]
mal hypospadias. We used to be totally biased to
one-stage repair for all cases of proximal hypo- 36.2.1 S
tep 1: Outlining the Skin
spadias; however we now believe that each case Incision (Fig. 36.1a, b)
should be dealt with individually and that the sur-
geon should master different techniques so that The glans and the dorsal foreskin are suspended
the patient is offered the best chance [2–4]. by fine traction sutures; the meatus is dilated
using a fine curved mosquito clamp, or ventral
meatotomy may be done wide enough to accom-
A. Elsaied (*)
modate an F6 urethral silicone catheter. The
Pediatric Surgery Department, Mansoura University,
Mansoura, Egypt skin is marked before incision using gentian
e-mail: adhamawe@mans.edu.eg violet. The meatal-based yoke is outlined, tak-
538 A. Elsaied
ing care that the base of the flap proximally is as 36.2.5 Step 5: Glanulomeatoplasty
wide as could be. The lateral incision runs prox-
imal and lateral on both sides along the medial The distal end of the tube is anastomosed to the
edge of the scrotal raphe and then distal to the glans, and the glanular wings are closed in two
lateral part of the penile shaft over the foreskin layers using 6/0 mattress sutures (Fig. 36.1h).
to meet with the other side at 12 o’clock on the
dorsal part of the penis. The inner medial cir-
cumcoronal incision is outlined a few millime- 36.2.6 S
tep 6: Byarization
ters from the coronal sulcus to save a small of the Dorsal Foreskin and Its
collar of the inner skin. Subcutaneous Tissue for Skin
Closure (Fig. 36.1i, j)
36.2.2 S
tep 2: Chordectomy The foreskin flap is incised dorsally at 12 o’clock,
and Creation of Parameatal and the proximal subcutaneous layer is mobilized
Foreskin Flap (Fig. 36.1c) judiciously to create a wide skin flap to enroll the
penile shaft, avoiding tension during skin clo-
Using fine iris scissors, the parameatal foreskin sure. Care should be taken not to injure the axi-
flap is harvested by incising through the outer lat- ally running branches of the superficial dorsal
eral incision first and then the inner circum- arterial system during this step.
coronal incision, releasing it from the dartos
fascia and releasing the ventral and lateral fibrous
chordae starting distally at the coronal sulcus 36.2.7 Step 7: Skin Closure
toward the proximal urethra and extending as far (Fig. 36.1k)
as it takes to establish complete straightening of
the penis. This is done in multiple layers; the subcutaneous
tissue of the proximal part of the wound after
byarization of the foreskin moves ventral and
36.2.3 Step 3: Bisecting the Glans proximal, thus displacing the scrotum proximal
and Creation of Glanular to near normal position rather than the abnormal
Wings overriding position that it assumed before sur-
gery. The distal part of the shaft is covered by the
The glans is incised ventrally in the midline, and ventralized foreskin starting proximal and then
the glanular wings are dissected laterally. In most moving distally to the coronal sulcus. Once clo-
cases this could be a bloodless step if done in the sure of the subcutaneous tissue is done, the skin
right plane. is approximated.
This technique was described and refined by
Koyanagi et al. (1987, 1988, 1993, and 1994)
36.2.4 Step 4: Ventralization without major changes, especially to the vascu-
and Tubularization larity of the neourethra; the results improved but
of the Parameatal Foreskin were still unsatisfactory [12–15].
Flaps (Fig. 36.1g) Emir et al. described a modification of the
original Koyanagi procedure which aimed to
The harvested flaps are ventralized, and the ure- increase the blood supply to the flap, thus causing
thral tube is formed over a F6 catheter by sutur- a significant difference in the results, increasing
ing the medial ends first followed by the lateral the success rate, and decreasing the complica-
edges using 7/0 polydioxanone. The neourethra tions [16]. We took up the modification of Emir
is reinforced by a second layer of ventral and applied it on a series of cases from 2002 to
sutures. 2005. Our results were better than the original
36 Koyanagi Technique and Its Modifications in the Management of Proximal Hypospadias 539
a b
c
d
Fig. 36.1 (a–k) Schematic drawings of the operative tech- along the medial edge of the scrotal raphe and dorsally
nique. The solid lines outline the incisions. The incision along the sulcus coronalis over the foreskin until it meets
encircles proximal to the meatus, runs laterally on both sides with its counterpart at 12 o’clock on the dorsum of the penis
540 A. Elsaied
f g

i j

542 A. Elsaied
Koyanagi but still were not as satisfactory as chordae is started (Fig. 36.2b); this allows the
Emir et al. However the modification was urethral plate to be mobilized sufficiently to
appealing to us, and we used it in another series excise all of the lateral and ventral tissues that
and had much better results [8, 17]. contribute to the chordae (Fig. 36.2c) [8]. The
penis is essentially degloved circumferentially
through the inner incision.
36.3 The Modified Technique The outer incision is then made but only
[8, 16, 17] through the skin, preserving the underlying lat-
eral vascular supply to the skin flaps (Fig. 36.2d).
The lateral part of the meatal based yoke is out- The well-vascularized 7- to 8-mm-wide flaps
lined using a permanent marker pen or a scalpel are ventralized (Fig. 36.2e), and then glanular
cutting only partially through the skin, making cleavage and creation of the glanular wings were
the outer incision always identifiable throughout performed (Fig. 36.2f). The urethra is tubularized
the operation (Fig. 36.2a). using 6/0, 7/0, or 8/0 Vicryl sutures (Fig. 36.2g)
The inner incision is then outlined but incised and is placed at the glans tip.
before the outer one, through which release of
a b
c d
Fig. 36.2 (a) Outlining the outer incision, (b) the inner (g) tubularization of neourethra, (h) tunica wrap, and (i)
incision, (c) excision of chordee, (d) the outer incision, (e) early postoperative view
ventralization of the flap, (f) creation of glanular wings,
e f
g h

544 A. Elsaied
The circumference of attachment between the dorsal junction near the coronal sulcus
meatal skin and glans is made to be wider than (Fig. 36.3d, e). We use the inner circumcoronal
the tube itself to decrease incidence of meatal ste- incision to release the chordae and achieve com-
nosis. The repair is then covered by a tunica vagi- plete straightening of the penis (Fig. 36.3f–h).
nalis wrap (Fig. 36.2h) which acts as an Artificial erection is a must in all cases to detect
intervening layer between the two suture lines. residual chordae (Fig. 36.3i). Corporotomy may
Resurfacing the ventral penile shaft, which be needed in severe cases and when done are cov-
incorporates a multilayer skin and subcutaneous ered by a piece of tunica vaginalis graft
tissue coverage of the urethra, is done without (Fig. 36.3j–l).
compromising the lateral blood supply of the ure- The distal part of the outer incision is devised
thra. An F6 feeding tube is used for diversion of dividing the prepuce in two parts, harvesting
urine and as a stent, and the repair is covered by the bilateral pedicled blood supply to the pre-
sterile dressing. The urethral stent is removed puce and paying attention not to jeopardize the
5–7 days postoperative, and the patient is dis- lateral blood supply to the flap. Keeping the
charged (Fig. 36.2i). two limbs of the flap continuous at 12 o’clock
increases the blood supply to both limbs with-
out compromising its length (Fig. 36.3m, n).
36.4 Tips and Tricks The lateral incision is made cutting only
in the Application through the skin, thus preserving the lateral
of Modified Koyanagi blood supply to the flap (Fig. 36.3o). The flap is
Technique for Proximal ventralized through a buttonhole incision at its
Hypospadias [8, 16, 18] proximal end (Fig. 36.3p, q). The distal part of
the flap is sutured to the complete inner circum-
We have practiced the modified Koyanagi tech- ference of the glans penis (Fig. 36.3r); this we
nique for proximal hypospadias since 2002 and believe to have decreased the incidence of
have adopted a few technical points, some of meatal stenosis [18]. The proximal sound part
which were included in the original modification of the urethral plate is incised as in TIP proce-
by Emir et al. and others we developed during dure (Fig. 36.3s); this added to the width of the
our experience [18]. flap proximally thus makes it easier to avoid the
The outer lateral incision of the flap is out- hair-bearing skin of the scrotum being involved
lined but not incised (Fig. 36.3a, b). The pro- in the proximal design and also decreases the
posed flap is divided schematically into three incidence of stenosis at the site of original
parts: the proximal parameatal part supplied meatus [18]. We use 7/0 or 8/0 Vicryl sutures to
mainly by the random local blood supply of the create the tube from the flap starting with the
flap, the middle part of the flap which takes its dorsal suture line as we anastomose the medial
random blood supply as well as the lateral blood ends of the flap usually using continuous sutur-
supply that we preserve from the subcutaneous ing (Fig. 36.3t, u). Then we anastomose the lat-
dartos tissue, and the distal preputial part that eral edges of the flap to form the ventral part of
has its main pedicled blood supply from the the neourethra (Fig. 36.3v, w). The use of tunica
branches of the dorsal penile artery (Fig. 36.3c). vaginalis as a second layer is essential and we
It is essential to understand the blood supply of believe decreases the incidence of fistula for-
each part and preserve it. mation (Fig. 36.3x). Meticulous closure of the
The lateral flaps of the meatal-based yoke are skin in layers achieves better cosmetic results
designed a least 8 mm wide on each side, taking without compromising the vascularity of the
care not to compromise this width at its ventro- skin (Fig. 36.3y, y1, z, z1).
a b
c d
e f
g h
Fig. 36.3 (a–z1) Modified Koyanagi technique for proximal hypospadias

546 A. Elsaied
i
j
k l m

n o
p q r
t u
v w
x

548 A. Elsaied
y y1
z z1
36.5 Discussion [8, 16]. Snow and Cartwright in 1994 described

the yoke repair which is essentially the same
The original Koyanagi technique was published Koyanagi parameatal-based foreskin flap but
in 1983 for cases of proximal hypospadias and leaving the distal preputial part of the flap in con-
showed discouraging results [10]. This was due tinuity forming a yoke shape and saving its pedi-
to the fact that the parameatal-based flap devised cle [4]. This increased the blood supply to the
by Koyanagi and colleagues had poor vascularity distal part of the flap; however the mid part still
had poor blood supply. They applied this proce- 36.6 Conclusion
dure for four patients with proximal hypospadias
and had complications in two of them which Modified bilateral Koyanagi with its modifica-
were also discouraging [4]. tions have proven simple and successful for fur-
Emir and colleagues in the year 2000 described ther evaluation. Our tips and tricks were found
the modified Koyanagi technique which showed effective in improving the outcome and avoiding
much better results than the original. The secret the previous reported problems of meatal steno-
of attaining better results lies in the rich blood sis, recession, and urethral stricture at the site of
supply of the flap. It attains local parameatal the original meatus.
blood supply proximally, lateral blood supply
from the penile dartos fascia and skin in the mid
part, and distal pedicled blood supply to the distal
preputial part of the flap. They showed a 20%
References
complication rate which was encouraging to take 1. Kass EJ, Bolong D. Single stage hypospadias recon-
up this technique [8, 16]. struction without fistula. J Urol. 1990; https://doi.
We reviewed the literature involving the org/10.1016/s0022-5347(17)39510-1.
Koyanagi technique and its modifications and 2. Snodgrass W, Bush N. Primary hypospadias repair
techniques: a review of the evidence. Urol Ann. 2016;
stated variable results starting from 53% success https://doi.org/10.4103/0974-7796.192097.
for Koyanagi and colleagues in 1994, 50% for 3. Duckett JW. Transverse preputial island flap tech-
Snow and Cartwright (1994) passing to Emir nique for repair of severe hypospadias. Urol Clin
et al. (2000), Hayashi et al. (2001), and Hayashi North Am. 1980;7:423–30.
4. Snow BW, Cartwright PC. Yoke hypospa-
et al. (2007) recording 80%, 70%, and 92% suc- dias repair. J Pediatr Surg. 1994; https://doi.
cess rates, respectively [4, 15, 16, 19, 20]. org/10.1016/0022-3468(94)90091-4.
We adopted the modified Koyanagi technique 5. Hodgson NB. Commentary: review of hypospadias
in the year 2002 and have practiced it for cases of repair. In: Whitehead E, Leiter E, editors. The current
operative urology. 2nd ed. Philadelphia: Harper and
proximal hypospadias. In our earlier experience, Row; 1984. p. 1233–42.
we had around 40% complication rate [17]. Later 6. King LR. Hypospadias repair. In: Whitehead ED,
we mastered the technique, and our results Leiter E, editors. The current operative urology. 2nd
improved marvelously showing only 10% compli- ed. Philadelphia: Harper and Row; 1984. p. 1265–71.
7. Woodard JR, Parrott TS. Management of severe
cations in a series we published in the year 2010 perineal hypospadias with bifid scrotum. J Urol.
[8]. Some of our later cases showed meatal steno- 1991;145:132.
sis and proximal stenosis at the original meatus 8. Elsaied A, Saied B, El-Ghazaly M. Modified
when we avoided any potentially hair-bearing skin Koyanagi technique in management of proximal
hypospadias. Ann Pediatr Surg. 2010;6:22–6.
in our flap. This caused the flap to be narrow at its 9. Koyanagi T, Nonomura K, Kakizaki H. Koyanagi-
proximal end. The tips and tricks we developed Nonomura one-stage repair for severe perineal
with time were found successful in solving the lat- hypospadias. In: Hadidi AT, editor. Hypospadias sur-
ter problems and improving our overall results. gery: an illustrated guide. 1st ed. Berlin-Heidelberg:
Springer Verlag; 2004. p. 191–202.
Modified Koyanagi stands as a tedious opera- 10. Koyanagi T, Matsuno T, Nonomura K, Sakakibara
tion which should be applied only when indicated. N. Complete repair of severe penoscrotal hypo-
Alternatives are staged repair, free graft, pedicled spadias in 1 stage: Experience with urethral mobi-
preputial tube, and augmented Duckett [21–23]. lization, wing flap-flipping urethroplasty and
“glanulomeatoplasty”. J Urol. 1983; https://doi.
Staged repair is preferred by many surgeons in org/10.1016/s0022-5347(17)51732-2.
cases of proximal hypospadias [21, 22]. We used 11. Koyanagi T, Nonomura K, Gotoh T, Nakanishi S,
to apply one-stage repair for all cases of proximal Kakizaki H. One-stage repair of perineal hypospadias
hypospadias; however each case should be dealt and scrotal transposition. Eur Urol. 1984; https://doi.
org/10.1159/000463834.
with individually, and the surgeon should master 12. Koyanagi T, Nonomura K, Imanaka K, Gotoh T,
different techniques and be able to combine them Ameda K. One-stage urethroplasty with paramea-
so that the patient is offered the best chance. tal foreskin-flap (OUPF) for severe proximal hypo-
550 A. Elsaied
spadias associated with bifid scrotum. J Urol. 1987; agement of moderate and severe degrees of hypospa-
https://doi.org/10.1016/s0022-5347(17)75345-1. dias. Mansoura University, Egypt.
13. Koyanagi T, Imanaka K, Nonomura K, Togashi M, 18. Elsaied A. Mansoura modification of modified
Asano Y, Tanda K. Further experience with one-stage Koyanagi technique in management of proximal
repair of severe hypospadias and scrotal transposi- hypospadias. Ann Pediatr Surg. 2015:83.
tion modifications in the technique and its result 19. Hayashi Y, Kojima Y, Mizuno K, Nakane A, Kohri
in eight cases. Int Urol Nephrol. 1988; https://doi. K. The modified Koyanagi repair for severe proxi-
org/10.1007/BF02550668. mal hypospadias. BJU Int. 2001; https://doi.
14. Koyanagi T, Nonomura K, Kakizaki H, Takeuchi org/10.1046/j.1464-410X.2001.02029.x.
I, Yamashita T. Experience with one-stage repair 20. Hayashi Y, Kojima Y, Mizuno K, Nakane A,
of severe proximal hypospadias: operative tech- Kurokawa S, Maruyama T, Kohri K. Neo-modified
nique and results. Eur Urol. 1993; https://doi. Koyanagi technique for the single-stage repair of
org/10.1159/000474274. proximal hypospadias. J Pediatr Urol. 2007; https://
15. Koyanagi T, Nonomura K, Yamashita T, Kanagawa doi.org/10.1016/j.jpurol.2006.06.007.
K, Kakizaki H, Belman AB. One-stage repair of 21. Retik AB, Bauer SB, Mandell J, Peters CA, Colodny
hypospadias: is there no simple method universally A, Atala A. Management of severe hypospadias with
applicable to all types of hypospadias? J Urol. 1994; a 2-stage repair. J Urol. 1994; https://doi.org/10.1016/
https://doi.org/10.1016/S0022-5347(17)32556-9. s0022-5347(17)32697-6.
16. Emir H, Jayanthi VR, Nitahara K, Danismend N, Koff 22. Greenfield SP, Sadler BT, Wan J. Two-stage repair for
SA. Modification of the Koyanagi technique for the sin- severe hypospadias. J Urol. 1994;152:498–501.
gle stage repair of proximal hypospadias. J Urol. 2000; 23. Glassberg KI. Augmented Duckett repair for severe
https://doi.org/10.1097/00005392-200009020-00013. hypospadias. J Urol. 1987; https://doi.org/10.1016/
17. Elsaied A, Sheir M, El-Ghazaly M, Kandil K (2005) S0022-5347(17)43153-3.
Evaluation of modified Koyanagi technique in man-
The Yoke Hypospadias Repair
37
Brent W. Snow
37.1 Introduction 37.2 Operative Technique
Repair of severe hypospadias has been and con- The incisions are initially indicated by marking a
tinues to be a significant challenge to surgeons in circumcising incision. Ventrally at the location of
the field. In the past, surgical repair has relied on the hypospadiac meatus, there is a gentle down-
staging the procedure or combination of proce- ward “V” of the incision toward the meatus. A
dures. Often, the combination of procedures may second incision is marked which begins proximal
add the complication rate of one procedure to that to the hypospadiac meatus and then runs parallel
of another. Procedures were often designed for a along the urethral plate. The width of this inci-
specific length of hypospadias repair, and extend- sion is chosen remembering that the circumfer-
ing the indications may compromise the integrity ence of the neourethra in French measurements
of the original procedure design [1]. In order to equals the millimeters of circumference. Thus,
create a repair that maintains blood supply to the the parallel incisions are made as many millime-
neourethra for severe hypospadias patients, the ters wide as the new urethra will become in
yoke procedure was created [2]. Recognizing that French size plus 1–2 mm that will be taken up by
the most common hypospadias procedures are suturing. As the incisions approach the coronal
based on ventral perimeatal blood supply [3, 4] or margin, the incision is then carried around the
on dorsal island pedicle flap [5] blood supply, the dorsum of the penis. The measurement, then, is
repair was developed to maintain both dorsal and one-half the desired French size in millimeters
ventral supplies and allow a single-stage repair of plus 1–2 mm, since these two sides are going to
complex hypospadias. This repair would avoid be sutured together, both anteriorly and posteri-
combining repairs and obviate the anastomotic orly (Fig. 37.1f). As the incision is marked, the
difficulties that may occur. observer will note that the name “yoke” is derived
from the resemblance of the marked incisions to
the apparatus draped around the neck of working
animals to which implements are attached. Once
This Chapter is a reprint from the First Edition.
the incisions are marked, the initial incision made
is the circumcising incision. The skin of the penis
is degloved along Buck’s fascia, and the urethral
B. W. Snow (*)
Department of Pediatric Urology, Primary Children’s plate is separated from the corpora cavernosa.
Hospital, Salt Lake City, UT, USA Chordee correction can be undertaken according
e-mail: brent.snow@hsc.utah.edu to the surgeon’s preference. Once chordee has
552 B. W. Snow
been corrected, the second incision is made tak- the coronal margin with absorbable suture, and
ing care to incise only the dermis and epidermis, the closure can be made in the ventral midline to
not the underlying vascular pedicle. The shaft mimic the median raphe (Fig. 37.1h, i). The sur-
skin is then separated from the vascular pedicle gical scars, when completed, go around where a
for a distance of 1–2 centimeters away from the circumcision scar would be and in the ventral
neourethra (Fig. 37.1b). It is not necessary to midline along the median raphe, giving a won-
separate the vascular pedicle as far as one would derful cosmetic result (Fig. 37.2). Often, a scro-
during an island flap urethroplasty (Fig. 37.1c). toplasty is necessary to complete the operation. A
Once the outer skin has been separated and the urethral catheter is left in place for 7–10 days,
vascular pedicle developed, a buttonhole is made and the surgeon’s choice of dressing is applied.
near where the shaft skin attaches to the vascular The operation is based on careful dissection
pedicle (Fig. 37.1d). The glans penis is drawn and maintenance of periurethral blood supply
through this buttonhole in the vascular pedicle and the vascular pedicle. These principles and
(Fig. 37.1e), transposing the entire urethra and techniques are familiar to hypospadias surgeons.
vascular pedicle to the ventral aspect of the penis. Those who perform surgery to correct hypospa-
A glans channel is made dissecting over the dias recognize that it is often tedious and techni-
corpora cavernosa to the tip of the glans and mak- cally demanding.
ing an adequate channel of 18–22 F in size. The Experience with this repair is limited since it
neourethra that has been transferred to the ventral has been used in the most severe forms of hypo-
aspect of the penis with the buttonhole technique spadias patients. Some authors have expressed
now has a tennis racquet appearance (Fig. 37.1f). concern that there would be inadequate shaft skin
The distal portion where the glans was has a gap and foreskin to complete this operation; however,
which is sutured together beginning with a 7-0 9 cm of the neourethra was created in one patient,
absorbable suture layer, followed by a second and in no patient has there been inadequate skin
running subcuticular 8-0 absorbable suture layer to perform this operation in its entirety.
to complete the neourethra’s posterior suture line. The limited experience has shown that ure-
Then, a urethral catheter is placed in the urethra, throcutaneous fistula is more common than
and the ventral suture line of the neourethra is desired, and this seems always to happen near the
begun by using an 8-0 absorbable suture subcu- proximal anastomosis or the penoscrotal junc-
ticular stitch. The distal 1 cm of the neourethra is tion. It would be recommended at this point to
sutured in an interrupted fashion, so the length consider a second buttonhole to bring a dartos
can be trimmed without ruining a running suture flap or a tunica vaginalis flap through the neoure-
line. A second layer of 7-0 absorbable suture is thra’s vascular pedicle to cover the neourethra
completed over this, with the last centimeter from the original meatus to the glans channel,
again being sutured in an interrupted manner which would minimize the chance of fistula
(Fig. 37.1f, g). The urethral catheter is removed formation.
before the urethra is passed through the glans Other authors in the past have had similar cre-
channel. The surgeon will note that the distal por- ative suggestions, but none with the dedication to
tion of the neourethra is hooded, which allows for preserving blood supply as the yoke procedure
generous pedicle blood supply. The hooded part does. Russell reported parallel circumferential
of the neourethra is excised by removing the der- incisions that he called a stole procedure [7]. His
mis and epidermis, leaving the vascular pedicle operation, published in 1900 was a two-stage
(Fig. 37.1f, g). This hood is similar to the Turnbull procedure where the part of the urethra that was
modification of ileal conduit that maintains good taken from the dorsum was divided and the blood
blood supply to the meatus and minimizes stomal supply was not preserved. Koyanagi et al. devel-
complications [6]. The meatus is made by sutur- oped similar incisions but divided the dorsal neo-
ing interrupted 7-0 absorbable sutures around the urethra [8]. Long parameatal-based flaps were
tip. The shaft skin of the penis is next sutured to created in a procedure called “wing flap-flipping
37 The Yoke Hypospadias Repair 553
a b
c
d
Fig. 37.1 (a) The coronal margin line can be seen with through the buttonhole, transferring the neourethra to the
the V-shaped ventral incision. The parallel incision is the ventral aspect of the penis. (e) A ventral view at the same
urethral plate. Then go around to the dorsum parallel to stage of the repair: The buttonhole remains as a gap in the
the coronal margin incision. (b) The penis already neourethral plate. (f, g) The gap in the neourethral plate
degloved ventrally with the coronal margin incision made has been closed, and the neourethra is being sutured near
and the parallel urethral plate incisions and incision the tip. The second view shows the hooding that takes
around the dorsal aspect of the coronal margin. (c) The place when the neourethra has been completed and has
vascular pedicle is developed dorsally, but note that it does been passed through the glans channel. The inset shows
not go completely to the base of the penis because in this this being excised. (h, i) Skin coverage of the yoke proce-
procedure that is not necessary. (d) A dorsal view of the dure can easily be accomplished by suturing the circumci-
penis with the buttonhole having been made and a hemo- sion site back together and performing a median raphe
stat used to pull the traction suture and the glans penis closure, as shown
554 B. W. Snow
e f
g h

37 The Yoke Hypospadias Repair 555
Fig. 37.2 Postoperative view showing the excellent cosmetic results after a midscrotal hypospadias repair using the
yoke technique
556 B. W. Snow
urethroplasty.” These are exceedingly long and 4th ed. Philadelphia: Lippincott Williams & Wilkins;
2002. p. 2509–32.
narrow perimeatal-based flaps that did not main- 2. Snow BW, Cartwright PC. Yoke hypospa-
tain pedicle blood supply. dias repair. J Pediatr Surg. 1994; https://doi.
The yoke operation does not cause the torsion org/10.1016/0022-3468(94)90091-4.
that is associated with long-pedicle urethroplas- 3. Mathieu P. Traitement en un temps de l’hypospade
balanique et juxta-balanique. J Chir. 1932;39:481.
ties. Since the urethra is brought ventrally through 4. Mustardé JC. One-stage correction of distal hypo-
with a buttonhole technique, no bulk is added to spadias: and other people’s fistul{high symbol}.
one side of the shaft of the penis with the vascular Br J Plast Surg. 1965; https://doi.org/10.1016/
pedicle nor are there any twisting rotational S0007-1226(65)80068-6.
5. Duckett JW. Transverse preputial island flap technique
effects. The pedicle dissection is minimized com- for repair of severe hypospadias. Urol Clin North Am.
pared with traditional pedicle flap urethroplas- 1980;7(2):423–30.
ties, and this minimizes the damage to the pedicle 6. Turnbull RB, Fazio V. Advances in the surgical tech-
blood supply. The cosmetic results of the yoke nique of ulcerative colitis surgery: endoanal proctec-
tomy and two-directional myotomy ileostomy. Surg
hypospadias repair are excellent. The major fault Annu. 1975;7
of the repair is the frequency of urethrocutaneous 7. Russell RH. Operation for severe hypospadias. Br Med
fistulas near the proximal meatus. It is the author’s J. 1900; https://doi.org/10.1136/bmj.2.2081.1432-a.
recommendation that additional tissue be trans- 8. Koyanagi T, Nonomura K, Gotoh T, Nakanishi S,
Kakizaki H. One-stage repair of perineal hypospadias
posed to this area through a buttonhole to prevent and scrotal transposition. Eur Urol. 1984; https://doi.
this complication and accomplish repair of severe org/10.1159/000463834.
hypospadias in a single procedure.
References
1. Snodgrass WT, Baskin LS, Mitchell ME. Hypospadias.
In: Gillenwater JY, Grayhack JT, Howards SS,
Mitchell ME, editors. Adult and pediatric urology.
Dorsal Longitudinal Penile Skin
Island Flap in One-Stage Repair 38
of Hypospadias with Penoscrotal
Transposition
Rados Djinovic and Sava V. Perovic
38.1 Introduction testicular mobilization with their midline fixation

(inter-orchiopexy) as well as scrotoplasty.
Penoscrotal transposition is a rare anomaly of
external genitalia characterized by malposition of
the penis in relation to the scrotum. It frequently 38.2 Operative Technique
occurs in caudal regression syndrome. Surgical
treatment is based on the severity of transposition 38.2.1 Penile Degloving
and associated anomalies. Several one or multi-
stage procedures have been described in the lit- Step 1: Penile incision starts around the urethral
erature. Repair of isolated penoscrotal plate and continues into the glans as well as
transposition depends on its form and usually is between the inner and outer preputial layer, leav-
performed as one-stage procedure. Penoscrotal ing the dartos fascia with outer layer. Another
transposition associated with hypospadias is incision is made in the scrotum as shown in
more difficult for repair and can be performed as Fig. 38.1; this way the penile and scrotal skin are
one or two-stage procedure. completely mobilized.
Since there is no unique operative approach
for different forms of penoscrotal transposition
with hypospadias, the individual surgical concept 38.2.2 Curvature Correction
is of primary significance. The goal of our treat-
ment is achievement of normal anatomical posi- Step 2: Artificial erection is made using tourni-
tion of the penis and scrotum with simultaneous quet and saline injection or preferably by intra-
correction of hypospadias and all other associ- cavernous injection of prostaglandin E to evaluate
ated anomalies in single stage. penile curvature before and after chordectomy. It
The basic principles of our technique are cor- is also useful for easier dissection of the penile
rection of hypospadias using vascularized longi- entities, permanent checking of the curvature
tudinal dorsal island flap used for neourethra during repair and design of proper size flaps/
creation and two vascularized sliding flaps used grafts for both urethroplasty and penile body
for penile skin reconstruction; transposition of reconstruction. Besides chordectomy, most of the
the penis to its normal supra-scrotal position; and patients require additional corporoplasty for cor-
rection of ventral curvature caused by corporeal
disproportion. Dorsal neurovascular bundle is
R. Djinovic (*) · S. V. Perovic mobilized and residual curvature corrected using
Sava Perovic Foundation, Certified Center of
Reconstructive Urology, Belgrade, Serbia one of the three techniques: (1) simple dorsal pli-
558 R. Djinovic and S. V. Perovic
Fig. 38.1 Penoscrotal transposition with severe hypospa- glanular wings and inner preputial layer are excised, in
dias and chordee. Boundaries of incision are indicated by order to achieve conical shape of the glans. Glanular ure-
dashed line. Penile skin exists only on the dorsal side of thral plate is incised in midline allowing its folding during
the penis as a triangle. Penile and scrotal skin are mobi- glanuloplasty (Courtesy Ahmed T. Hadidi)
lized. The urethral plate is preserved. Coronal parts of
cation of the wounded tunica albuginea created metrical. Three flaps are formed on the mobilized
by numerous superficial net-like incisions, with- penile skin: one island, the best vascularized flap
out penetration into the erectile tissue; (2) dorsal to form the neourethral onlay, and two vascular-
corporoplasty performed by longitudinal incision ized sliding skin flaps to cover the penile shaft
and transversal closure; and (3) ventral transver- defect. If long neourethra is necessary, spiral skin
sal corporotomy and grafting of the tunica flap is formed. In cases where the urethral plate is
albuginea with autologous material. Artificial
divided, the flap should be wider in its middle part
erection is repeated after corporoplasty to evalu- so it could be tubularized later. Additional sagittal
ate the definite chordee correction (Fig. 38.2). skin incision is made toward the mons pubis, and a
hole is made by blunt dissection, avoiding vascular
pedicle injury (Fig. 38.3).
38.2.3 Urethroplasty Step 4: Middle island skin flap is transposed to
the ventral side of the penis by buttonhole maneu-
Step 3: Urethral plate is elongated by its complete ver, positioned as an onlay and anastomosed to
mobilization including a part of normal urethra, the urethral plate using 6 or 7/0 interrupted and/
taking care to preserve all spongiosal tissue during or running absorbable suture; neourethra is fixed
dissection. Glanular urethral plate is incised in to the albuginea with several sutures.
midline allowing its folding during glanuloplasty. Step 5: In cases where a short urethral plate
The new urethra is formed using well-vascularized should be divided, it’s both ends are onlayed with
dorsal penile skin as an onlay. The development island flap, which is tubularized in the middle
and direction of blood vessels of the mobilized where the urethral plate is absent (tube between
penile skin determine flap positioning that may be two onlays) (Fig. 38.4). This way advantage of
oblique or longitudinal, symmetrical, or asym- onlay urethroplasty is maintained. Gap created
38 Dorsal Longitudinal Penile Skin Island Flap in One-Stage Repair of Hypospadias with Penoscrotal… 559
a b
Fig. 38.2 There are three types of ventral curvature ous net-like incisions, without penetrating the tunica albu-
repair: (a) Dorsal corporoplasty by longitudinal corporot- ginea. (c) Ventral transversal corporotomy of the tunica
omy with transversal closure. (b) Simple dorsal plication albuginea and grafting with autologous material (Courtesy
of the wounded tunica albuginea that consists of numer- of Ahmed T. Hadidi)
after division of urethral plate can also be inlayed tioned urethroplasties, neourethral suture lines are
with buccal mucosa graft quilted to the corporeal completely covered with well-vascularized subcu-
bodies; this way formed neourethral plate is taneous tissue of island flap, lowering the risk of
onlayed with dorsal longitudinal penile skin flap fistula formation. Fenestrated urethral stent tra-
as previously described (inlay graft—onlay flap versing the anastomosis is used for 12–14 days. In
urethroplasty). If the urethral plate is poorly devel- patients with the most severe hypospadias with
oped, it should be excised, and tubularized ure- high risk of stenosis/fistula, perineal urethrostomy
throplasty is performed using island flap; long can be left temporarily, whereas a temporary splint
elliptical anastomosis if formed between the hypo- should be placed into the neourethra. Eventually,
spadiac and new urethra. In all the abovemen- urethrostomy should be closed after 3 months.
Fig. 38.3 Vascularized island flap is formed on dorsal vascular pedicle injury. If a long urethra is necessary, spi-
penile skin. Additional incision is made on the skin of ral skin flap is formed. Formation of island flap in cases of
mons pubis. Hole is formed by blunt dissection in the area urethral plate division; the flap is wider in the middle to be
of the mons pubis, avoiding anterior abdominal middle tubularized later (Courtesy of Ahmed T. Hadidi)
38.2.4 Glanuloplasty approach is only occasionally needed. The tes-

tes are fixed to the scrotal skin using 4/0
Step 6: Neourethra is embedded deep into the glans polydioxanone “U” sutures, pulled out, and
forming slit-like meatus. Coronal parts of glanular knotted over the bolster.
wings and inner preputial layer are excised, in Step 8: Medial part of the thin and hairless
order to achieve conical shape of the glans; glanu- hemi-scrotal skin is excised on both sides, and
loplasty is done by closing glans wings in two lay- two oblique scrotal incisions are made, and this
ers, starting at the coronal level to estimate tension way flaps are created for Z-plasty. Drainage is
and avoid possible strangulation of the neourethral placed into the scrotum.
vascular pedicle. If the glans is well shaped, glanu-
lar channel technique could be used.
38.2.6 Penile Skin Reconstruction
38.2.5 Scrotoplasty Step 9: Sub-glanular portion of the penile body

skin is reconstructed from the inner preputial
Step 7: Complete mobilization and fixation of layer using mucosal collar technique. The proxi-
the testes along the midline is performed in all mal penile body skin is reconstructed using two
patients (inter-orchiopexy). In patients with previously created lateral sliding flaps, and the
cryptorchidism orchiopexy with or without her- base of the penile skin is fixed to the corporeal
niotomy done through the scrotal approach in bodies over the bolsters in order to prevent skin
most of the patients, additional inguinal retraction (Fig. 38.5).
Fig. 38.4 Island flap is transposed to the ventral side of hypospadias, thin and poorly developed urethral plate
the penis by buttonhole maneuver. Island flap and urethral must be divided. Both ends of the divided urethral plate
plate are anastomosed using 6 or 7/0 interrupted and/or are onlayed with island flap. Island flap is tubularized in
running absorbable suture. Variant of onlay urethroplasty: the middle where the urethral plate is absent. Advantages
deep glanular grove is tubularized and suture line covered of onlay urethroplasty are maintained (Courtesy of Ahmed
with vascularized tissue of island flap. In cases with severe T. Hadidi)
a b
c d
Fig. 38.5 Penoscrotal transposition with severe hypospa- is placed ventrally. (d) The dorsal longitudinal flap forms
dias and chordee. (a) Dorsal longitudinal flap is mobilized the ventral wall of the new urethra supported by well-
and is about 5 cm long. (b) It is important to mobilize the vascularized fascia without any chance of rotation
fascia adequately to the root of the penis. (c) A buttonhole (Courtesy from Perovic)
is made and the penis is put through the hole; thus the flap
38.2.7 Dressing 38.5 Comment
Step 10: During postoperative course certain pro- One-stage repair of penoscrotal transposition
cedures should be considered indispensable in with hypospadias is a complex and difficult sur-
order to achieve satisfactory final results. Elastic gery that requires a long learning curve as well as
compressive dressing is applied to the penis for great familiarity with all available techniques for
approximately 2 weeks after surgery, to provide hypospadias repair. It has even less complication
support for the new urethra during the healing rate than two-stage repair in the hands of an expe-
process. Suture fixation of elastic compressive rienced hypospadiologist. Otherwise, two-stage
dressing to the penile skin is recommended for repair is recommended.
the prevention of its sliding.
Further Reading
38.2.8 Urinary Diversion
1. Ehrlich RM, Scardino PT. Surgical correction
of scrotal transposition and perineal hypospa-
Step 11: In all patients, including those with tem- dias. J Pediatr Surg. 1982; https://doi.org/10.1016/
porary urethrostomy, the urine is diverted by S0022-3468(82)80205-4.
suprapubic catheter for 14 days. 2. Glassberg KI, Hansbrough F, Horowitz M. The
Koyanagi-Nonomura 1-stage bucket repair of
severe hypospadias with and without peno-
scrotal transposition. J Urol. 1998; https://doi.
38.3 Results org/10.1097/00005392-199809020-00038.
3. Glenn JF, Anderson EE. Surgical correction of
Between 1986 and 2005, 169 patients 2 to incomplete penoscrotal transposition. J Urol.
1973;110:603–5.
29 years old underwent surgery for complete (26 4. Kolligian ME, Franco I, Reda EF. Correction of peno-
patients) and incomplete (143 patients) penoscro- scrotal transposition: a novel approach. J Urol. 2000;
tal transposition with hypospadias. Of those, https://doi.org/10.1097/00005392-200009020-00019.
eight patients had transposition accompanied by 5. Marberger H. Penoscrotale Transposition:
Korrektur des äußeren Genitale. 1981; https://doi.
the caudal regression syndrome. The hypospa- org/10.1007/978-3-642-81706-9_72.
dias was classified as congenital short urethra in 6. Mellin HE. Die penoskrotale Transposition. Aktuelle
4 patients, subcoronal in 11, penile in 27, peno- Urol. 1981; https://doi.org/10.1055/s-2008-1062944.
scrotal in 59, scrotal in 54, and perineal in 14. 7. Miller SF. Transposition of the external geni-
talia associated with the syndrome of caudal
Satisfactory anatomical, aesthetic, and functional regression. J Urol. 1972; https://doi.org/10.1016/
results are obtained in 89% of patients. S0022-5347(17)60878-4.
8. Mori Y, Ikoma F. Surgical correction of incomplete
penoscrotal transposition associated with hypospa-
dias. J Pediatr Surg. 1986; https://doi.org/10.1016/
38.4 Complications S0022-3468(86)80652-2.
9. Pinke LA, Rathbun SR, Husmann DA, Kramer
Complication rate depends on the form of trans- SA. Penoscrotal transposition: review of 53 patients. J
position and associated anomalies, and in our Urol. 2001;166:1865–8.
10. Perovic SV. Penoskrotale transposition. Aktuelle
series was 14%. They included urethral stenosis Urol. 1998;29
at the site of proximal anastomosis, fistula forma- 11. Perović S, Sćepanović D, Sremcević D, Krstić
tion (most of them are closed spontaneously and Z. Einzeitige Korrektur von penoskrotaler
only two required additional surgery) and incom- Transposition mit Hypospadie. Aktuelle Urol. 1990;
https://doi.org/10.1055/s-2008-1060608.
plete correction of the penoscrotal transposition 12. Perović S, Vukadinović V. Penoscrotal transpo-
with a mild form of bifid scrotum that required sition with hypospadias: 1-stage repair. J Urol.
correction. In four patients with tubularized ure- 1992;148:1510–3.
throplasty, diverticulum was developed. Three
patients had partial penile skin necrosis, and two
patients had penile recurvation.
Grafts for One-Stage Repair
39
Ahmed T. Hadidi
Abbreviations Devine and Horton [3] and Hendren and Horton

[4] had excellent results using the inner preputial
BXO Balanitis xerotica obliterans skin after defattening. However, these results
PGA Polyglycolic acid were not duplicated by other surgeons in a uni-
TIP Tubularized incised plate form manner [5, 6].
A wide variety of options has been explored.
Extragenital skin from the groin, the inner arm,
or the postauricular area has been used with vary-
39.1 Introduction ing success [7]. However, a widespread concern
is that free graft skin is associated with fibrosis
The use of a vascularized local penile or prepu- and contracture over many years. The use of the
tial skin has been the mainstay of hypospadias bladder mucosa was therefore embraced enthusi-
repair for a long time. If the reconstruction astically in the 1980s, particularly in view of its
fails, for whatever reason, these precious com- free availability and its compatibility with urine
modities are irretrievably lost, and further [8, 9]. The complication rate proved to be very
reconstruction necessitates the recruitment of high, however, partially because of metaplasia
new tissue from elsewhere, generally in the and stenosis when the mucosa was exposed to air
form of a free graft. at the tip of the penis [10–12]. Such problems
Nove-Josserand in 1897 started the school of were compounded by the thin and relatively weak
urethroplasty utilizing free grafts [1]. A free full- nature of the mucosa itself, resulting in balloon-
thickness skin graft should be used in preference ing and protrusion of the reconstructed urethra
to split-thickness grafts because the former have with only the mildest degree of distal
better growth characteristics in children [2]. obstruction.
In this chapter, the three most popular tech-
niques (i.e., preputial skin graft, buccal mucosa,
and bladder mucosa) will be described in detail.
A. T. Hadidi (*) Whatever technique is used, the preoperative
Hypospadias Center and Pediatric Surgery preparation and postoperative management are
Teaching Hospital of the Johann Wolfgang Goethe the same.
566 A. T. Hadidi
39.2 raft Material and Graft

G surgery have resulted in paucity of usable adja-
Biology cent skin or in those with severe reconstructive
problems.
The free graft survival passes through three
stages: imbibition (first 2 days), inosculation
(2–4 days), and lymphatic drainage (after 39.4 Preputial Skin Graft
4–5 days).
The free graft material initially thrives by Devine and Horton [3, 19] first reported the use
imbibition (diffusion of nutrients between the of the free preputial graft in hypospadias surgery.
donor and recipient sites), and this phase lasts for Since this initial report, the application of the free
48 h. During this period new blood vessels are preputial graft with pedicle flap has been
being formed to nourish the graft. The second extended, and onlay free grafts were performed
phase of graft revascularization or inosculation as frequently as the tubularized repairs. The tech-
occurs between days 2 and 4, and blood recircu- nique was well described by Scherz [20].
lation is re-established to the graft. By days 4–5, A circumferential incision is made approxi-
lymphatic drainage is restored. For this process mately 0.5 cm proximal to the corona, beginning
of revitalization to be successful, the recipient dorsally, and the incision is carried ventrally
site must be well vascularized and immobilized down to the native meatus. After the shaft skin is
in the postoperative period for 7–10 days. Great completely dissected proximally to the penile
care should be taken to obtain excellent skin base, chordee is assessed with an artificial erec-
cover to allow the graft a well-vascularized bed tion. If chordee can be corrected with preserva-
[13]. tion of the urethral plate, as is often possible
using dorsal plication, then an onlay free graft is
used. However, when it becomes necessary to
39.3 ull-Thickness Skin Free
F divide the urethral plate to straighten the penis, a
Graft (Wolfe) tubularized free graft is constructed.
The graft is harvested from the inner face of
The characteristics essential for long-term suc- the prepuce by first unfolding the prepuce and
cess of a free graft are efficient imbibition and then amputating the appropriately sized graft,
inosculation, which ensure successful neovascu- which has been pre-measured (Fig. 39.1a). The
larization and graft uptake. Full-thickness skin graft is prepared by excising the subcutaneous
from non-hair-bearing areas such as (in order of tissue to create a thin, full-thickness strip of skin.
the authors’ preference) inner preputial skin, This step is most easily done by pinning the skin
penile shaft, and postauricular skin has been used strip to a board to stretch it out. To construct the
with success. Groin and upper arm skin have also free graft tube, the skin strip is tubularized over a
been successfully grafted. F10 red rubber catheter using a combination of
Although full-thickness skin grafts have a interrupted and running 7-0 polyglycolic acid
high success rate for urethral reconstruction, (PGA) sutures (Fig. 39.1b). The proximal end of
there are related complications such as stricture the tube is left open as spatulation. The catheter is
formation, graft shrinkage, balanitis xerotica removed and replaced with an F8 silicone Foley.
obliterans (BXO), and hypertrophic scar forma- The native urethra is spatulated, and the Foley is
tion at the donor site [14–18]. Moreover, the pre- introduced into the bladder. The balloon is
putial or penile shaft skin may not be available in inflated, and the catheter is left indwelling. The
patients in whom complications from previous proximal end of the graft is anastomosed to the
39 Grafts for One-Stage Repair 567
a b
Fig. 39.1 Preputial skin graft: (a) elevation of the inner cutaneous flap is developed. (e) The protective intermedi-
preputial skin. The graft is harvested. (b) The graft is ate layer is transformed ventrally and sutured in to cover
tubularized over a F10 catheter. (c) The glans is split and the skin graft
the graft is anastomosed at both ends. (d) The dorsal sub-
568 A. T. Hadidi
d e
native urethra with interrupted 7-0 PGA suture. used to tack down the graft to the corpora
The tube is constructed and anastomosed so that cavernosa.
the seam of the neourethra is positioned against The outer face of the prepuce is then used to
the corpora cavernosa. The glans is then split, and cover the ventral skin defect by fashioning the
the distal end of the neourethra is anastomosed to pedicle flap. An incision is made through the der-
the glans at the glans tip, also with interrupted mis of the outer prepuce at the approximate level
7-0 PGA (Fig. 39.1c). The glans wings are of the corona. This distal skin is mobilized on its
brought together ventrally with several deep 5-0 vascular pedicle by dissecting the more proximal
PGA sutures, and the glans epithelium is closed dorsal skin from the subcutaneous tissue and
and the remainder of the meatus secured with 7-0 continuing the dissection to the base of the penis
PGA. Several additional 7-0 PGA sutures are (Fig. 39.1d). The distal strip of the dorsal skin
that now sits on this vascular pedicle can be to bridge the defect created by incision of the
transposed ventrally by either rotating the flap or tunica albuginea to accomplish penile straight-
bringing the glans through a buttonhole in the ening. In this situation, the host bed may be
vascular pedicle in an avascular space. The latter unsatisfactory to support imbibition and inoscu-
technique may result in less penile torsion. The lation. Patients who have had previous failed
pedicle is secured over the graft with interrupted hypospadias repairs may similarly have an
7-0 PGA, which is also used as skin sutures for unsatisfactory host bed and blood supply to
the remainder of the repair. Finally, the ventral allow graft survival.
skin of the pedicle flap is trimmed to fit, and the
resurfacing of the shaft is completed (Fig. 39.1e).
A Tegaderm and silicone prosthetic foam dress- 39.5 Buccal Mucosa Graft
ing are applied. Both are left in place for 1 week.
When the urethral plate is preserved and an Humby was the first to propose and report the use
onlay free graft is used to reconstruct the urethra, of buccal mucosa for hypospadias repair in 1941
the graft is sutured to the native urethra with [17]. However, the current enthusiasm for the
interrupted 7-0 PGA sutures at the meatus, and technique was promoted by Duckett (1986) [21].
each lateral border is anastomosed with a running The technique has a definitive role for complex
subcuticular 7-0 PGA suture. Distally, we have urethral reconstruction, and indeed, the success
used the Barcat balanic groove technique to rate is so encouraging that the threshold for its use
advance the neourethra farther distally to achieve is becoming lower all the time, to the point where
an improved cosmetic appearance. primary buccal graft surgery may be appropriate
The conditions necessary to ensure successful in some circumstances when skin resources are
free graft survival were enumerated by Devine limited, allowing their preservation for good vas-
and Horton [19]. These include having a well- cularized coverage of the penile shaft.
vascularized host bed, rapid onset of imbibition, Buccal mucosa lines the vestibule of the
close apposition of the graft to the host bed, mouth, which is the space between the teeth and
immobilization of the graft, and rapid onset of the cheek inside the mouth. A cut section of the
inosculation. In imbibition, the graft absorbs cheek consists, from inside out, of mucosa (epi-
nutrients from the host bed, and this phase lasts thelium), submucosa (lamina propria), muscu-
approximately 48 h. Inosculation involves rees- losa (buccinator muscle), subcutaneous fat and
tablishment of vascularization to the graft by fascia (vessels and nerves), and the skin. The
anastomosis of the exposed vessels of the host function of the buccal mucosa is a direct result of
bed with the vessels of the graft. This phase is its structure. A tight spinosum layer and pump-
usually complete by 96 h. functioning cells of the superficial layer provide
Another possible reason for the good success protection from the foreign substances placed in
of free graft with preputial flap may be the result the oral cavity. Elastin and collagen without bony
of improved inosculation because the graft is attachment give the buccal mucosa flexibility and
sandwiched between two well-vascularized beds. the ability to distend and compress. A rapid turn-
The vascular pedicle also covers the repair, and over rate and highly vascularized lamina propria
this is a well-accepted technique in reducing the ensure quick healing after injury. The immune
rate of fistula formation. response is quickly aided by a lamina propria
The use of the free graft repair may be rela- laden with lymphocytes and macrophages. Many
tively contraindicated when chordee is severe, surgeons, after harvesting the buccal mucosa
and a tunica vaginalis or dermal graft is required graft, leave the donor area without closure,
570 A. T. Hadidi
because in reality it is too large to close without mucosa may explain its resilience and ease of
disfigurement. harvest and suturing [25, 26].
Studies on the healing of grafted buccal mucosa Buccal mucosa, unlike the bladder mucosa,
and underlying lamina propria suggest that a simi- does not have the propensity for prolapse. The
lar process occurs as in bladder mucosa graft, neomeatus remains as a vertical slit, providing a
wherein the graft undergoes partial degeneration good esthetic appearance, and functionally there
and desquamation, followed by complete epithe- should be no urinary stream problems. Composite
lial regeneration from the basal layer [22–24]. This grafts of buccal mucosa and free skin graft, espe-
is in contrast to skin grafts, which maintain an cially for the distal meatus, have shown improved
intact epithelial layer throughout graft healing results with regard to the adequacy of the neome-
[22–24]. Hence the basal layer has the capability atus, good cosmesis by retaining the natural ver-
to regenerate the epithelium. The graft connective tical glanular meatus configuration, and a good
tissue is organized by 21 days. The buccal mucosa caliber, straight urinary flow in the forward direc-
is probably similar to the bladder lamina propria tion. The main disadvantage of the buccal mucosa
and urothelium grafts in that it is unstable before graft is the thick epithelial layer, which may
complete epithelialization, which occurs at explain its relative stiffness.
10–14 days, implying that the duration of graft Buccal mucosa may be used as an onlay patch,
stenting is critical and should be at least 7–10 days. as a complete mucosal tube, or in various ways
Immunohistochemistry using antibody to type combined with other free graft tissue (bladder
IV collagen, which stains the basement mem- mucosa) or vascularized skin. It may be harvested
brane, has shown a vascular lamina propria, from the inner surface of the cheek or the inner
which allows efficient angiogenesis and inoscu- surface of the upper or lower lip (Fig. 39.2). For
lation between donor and recipient, explaining a single strip of buccal mucosa for use as an onlay
the excellent uptake of buccal mucosa [22, 23, patch, the adult cheek provides up to 6 cm and
25]. Imbibition is efficient due to the relative the lip 4 cm at 12–15 mm of width.
thinness of the lamina propria [23, 25].
There is abundance of healthy tissue, which
can be harvested with ease from the inner cheeks.
Manzoni and Ransley (1999) did not rec-
However, if a long strip of buccal mucosa is
ommend extending the strip through the
required, it is preferable to harvest two strips,
angle of the mouth to combine both cheek
one from each cheek, and avoid crossing over
and lip segments as this may cause signifi-
the angle of the mouth to the lower lip. Long
cant contracture at the angle of the mouth
buccal mucosa graft harvested from the inner
[27].
cheek to the lower lip usually results in scarring
and deformity of the angle of the mouth. Due to
the lack of luminal support of the corpus spon-
giosum, ballooning of the mucosal neourethra The strips of buccal mucosa obtained cannot
during voiding is a common occurrence. be tubularized lengthwise to create 6-cm tubes,
However, the elastin-rich connective tissue of because the width is insufficient. They can be
the basement tissue of the buccal mucosa is rela- folded lengthwise to provide shorter tubes of up
tively stiff and provides good scaffolding and to 3 cm and 2 cm, respectively, or a second graft
prevents the occurrence of a diffuse diverticu- of similar dimensions can be applied to create a
lum. Increased elastin infiltration of the buccal full-length cylinder of adequate diameter. When
a b
Fig. 39.2 (a–b) Harvesting of buccal mucosa
some vascularized preputial skin is available but If a single strip of mucosa is required, the
is insufficient for tubularization, a strip may be author’s preference is to use the left cheek as the
usefully combined with buccal mucosa to form a mucosal layer because this site is thicker than the
compound neourethra. In very long urethral lip and more robust than the mucosa of the lip,
reconstructions, the bladder mucosa may be used and the donor site is sutured primarily.
successfully for the proximal urethra, reserving It is usually enough to use two stay sutures above
the buccal mucosa for the distal element and and below the donor site to open the mouth. Some
meatus. authors prefer to use a mouth retractor, such as the
Boyle-Davis, with various sizes of tongue depressor
blades. The parotid duct usually is easy to identify
39.5.1 Operative Technique opposite the upper molars, and it may be cannulated
with a short length of 3-0 nylon to identify it through
For a right-handed surgeon standing on the right the dissection and closure although it is usually not
side of the patient, the lower lip and the left cheek necessary. The graft is outlined with a waterproof
are the most convenient donor sites. If the require- pen and the submucosa infiltrated with 1% ligno-
ments for the buccal tissue are clear beforehand, caine containing 1:2000 epinephrine. Two stay
the buccal mucosa may be obtained before com- sutures are placed at the posterior limit to facilitate
mencing penile dissection to allow an elegant traction. After waiting for a few minutes, the paral-
uninterrupted repair that benefits the penile tis- lel upper and lower borders of the graft are incised.
sue. Otherwise, the previous repair must be dis- These incisions are then sharply angled to meet at
mantled, the need for buccal mucosa assessed, the external limit near the angle of the mouth. One
and the graft obtained in mid-procedure. or two stay sutures are then placed to lift the graft
572 A. T. Hadidi
toward the mouth cavity, and the mucosa is dis- 39.7 Onlay Patch
sected away by sharp dissection. Buccinator muscle
inserts directly into the mucosa, and careful dissec- The urethral plate or the previously reconstructed
tion with a knife or sharp-pointed scissors is neces- urethra is retained as a midline strip extending
sary to free the mucosa. Diathermy usually is not forward from the normal native urethra to the
needed, and the defect is closed with an absorbable glans. This strip varies in width from a few mil-
continuous Vicryl 6/0. No further local treatment is limeters to 1 cm. The native urethra is incised for
usually necessary, and patients may eat at once. at least 1 cm proximally to create a spatulated
If the buccal mucosa graft is harvested from junction. The graft is trimmed to an appropriate
the lip, a series of stay sutures is used to evert the length and width and anastomosed to the margins
lower lip and to protect the mucocutaneous junc- of the urethral strip using 6-0 or 7-0 polyglycolic
tion. After epinephrine infiltration and the appro- acid sutures. Glans wings are wrapped around to
priate delay, the parallel incisions are made provide a terminal meatus. A two-layer closure of
transversely, leaving the graft attached at either well-vascularized penile skin is advised.
end. The mucosa (which is a little more delicate
than the cheek and prone to tear) is elevated using
fine tapered scissors in a manner similar to that 39.8 Graft Tube Urethroplasty
used when creating a submucosal tunnel. Finally,
the graft is released at either end and removed. Using either a folded graft or two segments of
The bare area is not sutured but may be packed buccal mucosa as previously discussed, a muco-
with a temporary swab soaked in epinephrine sal tube is created of sufficient length to bridge
until the end of the procedure. Healing is rapid the urethral defect. The proximal anastomosis is
and complete, and the scar is virtually invisible well spatulated, and glans wings are preferable to
within 2–3 weeks. a tunnel for the creation of the terminal meatus. A
The graft tissue is placed in a gauze soaked two-layer skin closure follows.
with normal saline for temporary storage, while
surgery in the mouth is completed. It is then
pinned out, face down, preferrable on a finger, 39.9 Compound Tube
cork, or Silastic block. Excess submucosal tissue
and salivary glands are removed using a sharp A compound tube technique is valuable when
scissor. After a change of gloves, the graft may be some preputial skin remains after earlier surgery
deployed in various ways. but is insufficient for tube urethroplasty on its
own. The preputial skin strip is mobilized on its
own vascular pedicle and the buccal graft anasto-
39.6 Inlay Patch mosed to create the compound tube. This is placed
in position on the ventral surface of the penis, with
This is the commonest approach now for using the vascularized skin lying against the corporal
grafts. The urethral plate is incised, and the surface and the buccal mucosa lying superficially
graft is applied and fixed on the well-vascular- to be covered by a two-layer closure of the penile
ized bed using several Vicryl 6/0 sutures. The skin (Fig. 39.3).
orifice of the proximal native urethra is incised However, this is less favorable than using a
dorsally to avoid proximal meatal stenosis. The preputial graft as an inlay on the dorsal surface of
graft is incised at several points to avoid accu- the new urethra as it is easier to fix and has a bet-
mulation of blood under the graft causing graft ter take than applying the graft in the ventral side
failure. of the neourethra.
a b c d
Fig. 39.3 (a–d) Some methods of urethral reconstruction using buccal mucosa
39.10 Clinical Experience

and Results One Very Important Tip
Given the anaerobic bacterial spectrum of
Buccal mucosa is proving very satisfactory as a the oral mucosa, it is wise to institute
material for urethral reconstruction, particularly appropriate antibiotic prophylaxis such as
in hypospadias cripples who have undergone co-amoxiclav or metronidazole. This
multiple previous procedures. It must be recog- should be commenced prior to harvesting,
nized that only short-term (5 years) follow-up has to ensure effective antibiotic levels in the
been reported. The frequency of meatal problems graft.
and urethral dilatation and ballooning is much
less than with bladder mucosa.
It may be that growth factors within the
mucosa promote rapid healing and that revascu- 39.11 Bladder Mucosal Grafts
larization occurs quickly because of the thin
lamina propria and the originally highly vascu- The use of bladder mucosa for the repair of the
lar bed from which the buccal mucosa graft was urethra in hypospadias was first described by
taken. Memmelaar in 1947 [28]. He discussed the ben-
Most surgeons’ experience indicate a sec- efits of a one-stage hypospadias repair and the
ondary operation rate of approximately 20% favorable characteristics of bladder mucosa in the
[27], mostly due to minor fistulas or the need reconstruction of the male urethra.
for meatal revision. In difficult cases, preopera- The graft biology has been elucidated in the
tive testosterone treatment may improve the New Zealand white rabbit and dogs [22, 24], and
vascularity of the penile skin and thereby the urothelium is well suited for contact with
enhance the chances of success with a free graft urine [25, 29–33]. However, its use in a staged
technique. urethroplasty is not feasible as the bladder
574 A. T. Hadidi
mucosa has the propensity to develop severe ary to fibrosis. The detrusor muscle is then
edema on exposure to air. It is therefore not suit- incised down to the underlying mucosa with a
able for staged urethroplasty, and there are no knife or blunt-tipped scissors. Once dissection
reports of its use in a two-stage hypospadias reveals the bulging blue mucosa; further blunt
repair. dissection allows separation of the detrusor
Bladder mucosal grafts were used exten- muscle off the mucosa for a distance adequate
sively in China in the late 1970s for one-stage to harvest the appropriate-sized graft. If the
hypospadias repairs. During the past decade, mucosa is inadvertently opened before obtain-
bladder mucosal grafts have been used rather ing an adequate graft, the bladder can be
extensively in the one-stage repair of hypospa- opened, and the mucosa dissected off the detru-
dias and urethral stricture disease. Problems sor (Fig. 39.4). However, this is much more
with stricture at the proximal anastomosis, tedious. Once an appropriate-sized area of the
along with fistulas and mucosal glanular pro- bladder mucosa is exposed, it is marked with a
trusion, caused several investigators to devise sterile pencil, stay sutures of 7-0 polyglactin
ways of decreasing the complication rate. are placed at each corner, and the graft is har-
Duffy et al. used a tube of bladder mucosa vested. A suprapubic cystostomy tube is placed,
combined with penile or preputial skin distally and the bladder is closed in one layer with no
to avoid the mucosal protrusion problem [34]. attempt to oppose the mucosa.
Other techniques have been described to The bladder mucosal graft is then tubularized
decrease the incidence of mucosal protrusion over an appropriate-sized catheter using a run-
and meatal stenosis by cutting the bladder ning, inverting 7-0 polyglactin suture with inter-
mucosa flush with the glans or even undercut- rupted sutures on one end to facilitate size
ting the glans epithelium and invaginating it trimming of the tube graft without violating the
into the new meatus. When used as a free tube running suture. Long grafts are augmented with
graft in the posterior or bulbar urethra for ure- a second layer of interrupted sutures.
thral stricture disease, bladder mucosa has Tubularization can be facilitated by placing the
been shown to work well. graft on a polystyrene needle board and immobi-
lizing it at the ends with 30-gage needles. The
Any prior failed tube graft tissue must be

excised, and it may be necessary to freshen the
proximal edge of the native meatus to reach
well-vascularized tissue. Once the bed for the
graft has been prepared and checked for good
vascularity, the gap is then measured from the
native meatus to the tip of the glans tunnel.
Attention is then turned to the harvest of the
bladder mucosal graft. The bladder is distended
with saline so that it is palpable above the sym-
physis pubis. A Pfannenstiel incision is then
performed to expose the underlying bladder. If
the patient has had a previous suprapubic cys-
tostomy, this site is avoided because of Fig. 39.4 Harvesting bladder mucosa for urethral
increased difficulty in obtaining a graft second- reconstruction
graft is kept moist with cool normal saline until little or no treatment being deemed necessary.
reimplantation. A wide, spatulated anastomosis The majority of complications involve protrusion
is performed between the tube graft and recipi- of the graft mucosa at the meatus with or without
ent urethra with 6-0 or 7-0 running polyglactin stricture. This troublesome complication has
suture. The graft suture line is placed dorsally been dealt with in numerous ways, including cut-
against the corpora to avoid overlapping suture ting the graft flush at the glans tip, avoiding any
lines and thus decrease the chance of fistula for- redundancy. Others have proposed undercutting
mation. The graft can be anchored to the corpora the glans epithelium and laying the edge into the
at several points to decrease the risk that shear distal anastomosis. Early and frequent dilatations
effect might compromise graft take. The distal of the neomeatus have also been proposed to help
end of the graft is then brought through the glans, avoid stenosis. The problem with exposed blad-
and the bladder mucosa is cut flush with the der mucosa at the meatus is that it becomes
glans edge while keeping the graft on stretch to hypertrophic, and metaplastic changes occur.
avoid redundancy at the tip. The graft edge is Prevention of mucosal protrusion is the key to
then sutured to the glans with interrupted 6-0 or management. If mucosal protrusion or stenosis
7-0 polyglactin sutures. An appropriate urethral does occur, revision is necessary. Stricture at the
catheter or stent is placed for drainage, along proximal anastomosis or fistula can occur with
with the suprapubic cystostomy tube (Malecot free or vascularized flaps of all types. Urethral
catheter). The graft is covered with at least two diverticulum of the graft tissue can occur if
layers of well-vascularized tissues such as the redundant tissue is not excised or distal obstruc-
tunica vaginalis, including skin. Alternatively, tion is present. Stricture of the proximal or distal
the bladder mucosa at the tip of the glans may be end of the graft may be treated with dilation or
avoided by either tubularizing the distal skin internal urethrotomy. Although bladder mucosa
strip of the glans as described by King [35] or grafts have had a relatively high incidence of
combining the bladder mucosal graft with a strip minor complications in the early postoperative
of penile skin as described by Duffy et al. [34]. period, they have succeeded well in the long run.
The penile or preputial skin may be closed using The bladder mucosa is a good graft tissue for the
Byars flaps. The urethral and suprapubic cathe- urethra and has been shown by many investiga-
ters are connected to gravity drainage, and the tors to work well.
penis is secured to the anterior abdominal wall
with dressing to immobilize it and enhance graft
take. In postpubertal patients, diazepam or estro- 39.14 Editorial Comment
gen can be used to prevent erection in the post-
operative period. The urethral catheter is Dorsal inlay of the preputial inner mucosa after
removed on the 12th to 14th postoperative day incising the urethral plate is gaining popularity in
and the suprapubic tube capped. Once the void- an attempt to benefit from the slit-like meatus
ing trial is deemed successful, the suprapubic achieved with TIP and avoid the common com-
catheter can be removed. plication of stenosis and urethra that is commonly
encountered after the classic TIP procedure (see
Chap. 27: Dorsal Inlay TIP).
39.13 Results The buccal mucosa remains an important
source of urethral epithelium when the patient is
In Keating et al.’s extensive review of the litera- already circumcised or when there is an inade-
ture related to bladder mucosa grafts, an overall quate or scarred foreskin for use in complicated
complication rate of 40% was found [12]. Two hypospadias. It is popular because of its ease of
thirds of these complications were minor, with harvest, thickened epithelium, and thin lamina
576 A. T. Hadidi
propria when compared with the bladder Adult and pediatric urology. 3rd ed. St. Louis: Mosby-
Year Book; 1996. p. 2549–89.
mucosa. Moreover, the fact that the bladder 14. Bracka A. A long-term view of hypospa-
need not be opened makes the morbidity of sur- dias. Br J Plast Surg. 1989; https://doi.
gery for buccal mucosal grafts less than that org/10.1016/0007-1226(89)90140-9.
involved in obtaining bladder mucosa. The blad- 15. Bracka A. Hypospadias repair: the two-stage alterna-
tive. Br J Urol. 1995; https://doi.org/10.1111/j.1464-
der mucosa is rarely used nowadays in hypospa- 410X.1995.tb07815.x.
dias surgery. 16. Bracka A. A versatile two-stage hypospadias repair.
Br J Plast Surg. 1995; https://doi.org/10.1016/
S0007-1226(95)90023-3.
17. Humby G, Higgins TT. A one-stage operation for
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site. J Urol. 1984; https://doi.org/10.1016/ chirurgie infantile. Masson, Paris.
S0022-5347(17)50820-4. 25. Dessanti A, Rigamonti W, Merulla V, Falchetti D,
8. Hendren WH, Reda ER. Bladder mucosa graft for Caccia G. Autologous buccal mucosa graft for hypo-
construction of male urethra. J Urol. 1986; https://doi. spadias repair: an initial report. J Urol. 1992; https://
org/10.1016/s0022-5347(17)44793-8. doi.org/10.1016/S0022-5347(17)37478-5.
9. Mollard P, Mouriquand P, Bringeon G, Bugmann 26. Culp OS, McRoberts JW. Hypospadias. In: Alken CE,
P. Repair of hypospadias using a bladder mucosal graft Dix V, Goodwin WE, editors. Encyclopedia of urol-
in 76 cases. J Urol. 1989; https://doi.org/10.1016/ ogy. New York: Springer; 1968. p. 11307–44.
S0022-5347(17)39159-0. 27. Manzoni GM, Ransley PG. Buccal mucosa graft
10. Ransley PG, Duffy PG, Oesch IL, Hoover for hypospadias. In: Ehrlich RM, Alter GJ, editors.
D. Autologous bladder mucosa graft for urethral Reconstructive and plastic surgery of the external
substitution. Br J Urol. 1987; https://doi.org/10.1111/ genitalia, adult and pediatric. Philadelphia: Saunders;
j.1464-410X.1987.tb04643.x. 1999. p. 121–5.
11. Ransley PG, Duffy PG, Oesch IL, van Oyen P, Hoover 28. Memmelaar J. Use of bladder mucosa in a one-stage
D. The use of bladder mucosa and combined blad- repair of hypospadias. J Urol. 1947; https://doi.
der mucosa/preputial skin grafts for urethral recon- org/10.1016/S0022-5347(17)69518-1.
struction. J Urol. 1987; https://doi.org/10.1016/ 29. Byars LT. A technique for consistently satisfac-
s0022-5347(17)43512-9. tory repair of hypospadias. Surg Gynecol Obstet.
12. Keating MA, Cartwright PC, Duckett JW. Bladder 1955;100(2):184–90.
mucosa in urethral reconstructions. J Urol. 1990; 30. Cloutier AM. A method for hypospadias
https://doi.org/10.1016/S0022-5347(17)39602-7. repair. Plast Reconstr Surg. 1962; https://doi.
13. Duckett JW, Baskin LS. Hypospadias. In: Gillenwater org/10.1097/00006534-196209000-00007.
31. Johnson D, Coleman DJ. The selective use of a single- glans deformity, and the penile urethra. Urol Clin N
stage and a two-stage technique for hypospadias Am. 1979;6:643–55.
correction in 157 consecutive cases with the aim of 34. Duffy PG, Ransley PG, Malone PS, van Oyen
normal appearance and function. Br J Plast Surg. 1998; P. Combined free autologous bladder mucosa/skin tube
https://doi.org/10.1016/S0007-1226(98)80009-X. for urethral reconstruction: an update. Br J Urol. 1988;
32. Klijn AJ, Dik P, De Jong TPVM. Results of pre- https://doi.org/10.1111/j.1464-410X.1988.tb05090.x.
putial reconstruction in 77 boys with distal hypo- 35. King LR. Hypospadias—a one-stage repair with-
spadias. J Urol. 2001; https://doi.org/10.1016/ out skin graft based on a new principle: chordee is
S0022-5347(05)66506-8. sometimes produced by the skin alone. J Urol. 1970;
33. Turner-Warwick R. Observations upon techniques for https://doi.org/10.1016/S0022-5347(17)62023-8.
reconstruction of the urethral meatus, the hypospadiac
Two-Stage Graft Urethroplasty;
Free Full-Thickness Wolfe Graft 40
Ahmed T. Hadidi
Abbreviations urethroplasty [3]. Various authors have introduced

technical modifications of the use of full-thickness
BXO Balanitis xerotica obliterans Wolfe skin graft since 1955 [4–9]. It was Turner-
CEDU Chordee excision and distal Warwick in 1979 who used the two-stage urethro-
urethroplasty plasty for adult salvage surgery and produced
DVL Dorsal vertical length
®EMLA Eutectic Mixture of Local Anesthetics
GW Glans width
PAWG Postauricular Wolfe graft
TALE Tunica albuginea longitudinal
excision
40.1 Introduction
Nové-Josserand in 1897 is credited to be the first

to use skin graft from the thigh for hypospadias
repair (Fig. 40.1) [1]. Humby (1941) was probably
the first to split the glans and use buccal mucosa as
a first stage in the reconstruction of the urethra [2].
Cloutier (1962), however, is the pioneer who first
described the use of full-thickness preputial skin
graft to cover the split glans (Fig. 40.2) at the first
stage and started the modern era of two-stage graft
A. T. Hadidi (*)
Teaching Hospital of the Johann Wolfgang Goethe Fig. 40.1 Nove-Jossérand was the first to use skin graft
University, Offenbach, Frankfurt, Germany from the thigh (1897) [1]
580 A. T. Hadidi
Fig. 40.2 The two-stage repair described by Cloutier, 1962 (from Cloutier: “A method for hypospadias repair,” Plastic
and Reconstructive Surgery, Vol 30, Issue 3, pp. 368–373, ©1962, from Wolters Kluwer Health Inc. with permission)
impressive results. Subsequently, Rabinovitch and 40.2 Selection of Patients

Bracka have resurrected the two-stage hypospa-
dias repair and have popularized this technique not Two-stage graft urethroplasty has become the
only for retrieving failed hypospadias repairs but standard technique in many centers for primary
also as an alternative technique for primary hypo- hypospadias with severe chordee that requires
spadias [6, 10, 11]. division of the urethral plate (grade IV), in compli-
40 Two-Stage Graft Urethroplasty; Free Full-Thickness Wolfe Graft 581
cated hypospadias with scarring that requires exci- glans split too far dorsally. It should be remem-
sion of the fibrotic, scarred urethral plate, and in bered that there is usually a degree of malrotation
patients with balanitis xerotica obliterans (BXO) of the glans wings in a typical hypospadias, and
where the native urethra is severely inflamed and correcting the glans alignment at the second stage
must be excised and replaced with healthy tissue. will naturally place the position of the meatus
In primary hypospadias with severe chordee more distally. Lateral incision lines are drawn
that requires division of the urethra plate (classi- from the midline at a subcoronal level, and these
cally chordee is more than 30 degree), many sur- are continued as far around the shaft as is neces-
geons prefer to use preputial skin as a first-stage sary for full access to, and release of, chordee tis-
inlay graft. sue. Usually this will be in continuity with the
In complicated hypospadias (usually the pre- incisions for the graft harvest and therefore
puce is deficient or absent) and in patients with circumferential. A 4/0 monofilament polypropyl-
BXO or lichen sclerosus (et atrophicus), the ten- ene stay suture is placed deeply through the dor-
dency is to use buccal mucosa because it is sal glans for traction purposes. Surgery is further
thought that BXO has less tendency to occur in facilitated by placing superficial stay sutures on
mucous membranes (see Chap. 61). either side of the midline, approximately at the
proposed ventral limit of the new meatus. These
will later serve the secondary function of “tie-
40.3 Operative Technique over” sutures for fixation of the bolus dressing
over the graft.
Surgery is performed under general anesthesia Skin incisions: A proximal meatotomy is per-
supplemented by caudal block. The author does formed if the meatus does not accommodate
not use a tourniquet to be able to control bleeding catheter size F12 or F14 according to the age of
adequately and avoid ischemia and postoperative the patient and the appropriate catheter is intro-
edema. Local infiltration with vasoconstrictors is duced into the bladder. Incising through the
not used to be able to identify tissue planes marked lines, clefting of the glans and release of
clearly. The aspects of age of repair, magnifica- chordee using a sharp scissors is a relatively
tion, and psychological support have been dis- bloodless procedure (Fig. 40.3a). The glans cleft-
cussed in detail in Chaps. 14 and 17. ing incision should be deep enough to reveal the
heads of the corpora cavernosa as depicted in
(Fig. 40.3b). Release of the glans tilt may require
40.3.1 First Stage dividing any fibrous bands of hypoplastic corpus
spongiosum. Dissection may need to extend to
The first-stage is crucial as it lays the foundation the lateral aspect of the corpora cavernosa to have
for the performance of a straight, esthetic, and freely mobile glanular wings.
normally functioning penis in the second stage. Chordee excision: Excision of the ventral
This stage involves chordee correction, harvesting hypoplastic and amalgamated ventral fascia is an
of the graft, laying down, and anchoring of the essential step of the first stage. Dissection and
graft and immobilization of the graft by an appro- excision should include the outer longitudinal
priate dressing (Figs. 40.3, 40.4, 40.5). layer of the tunica albuginea (TALE procedure)
Markings: An axial line is drawn on the glans, as mentioned in the CEDU technique until we
starting at the proposed dorsal limit of the new reach the inner circular layer of the tunica albu-
meatus and extending proximally down to the ginea (Chap. 33). Despite thorough clearance of
ectopic meatus on the shaft. If there is ventral all restricting tissue, there may still be residual
glans tilt and external rotation present, the glans ventral curvature present on reinflating the penis.
should be pulled up into normal alignment while This may be due to inherent curvature of the cor-
marking the position of the new meatus, as this poral bodies (corporeal disproportion). In such a
will avoid the common error of extending the condition, the author would incise the inner layer
582 A. T. Hadidi
of the tunica albuginea and place a tunica vagina- sions is now harvested from the inner layer of the
lis flap or a dermal graft from the groin. preputial hood. With the prepuce stretched out,
Some surgeons would correct the remaining the incisions are made with scalpel, and the graft
curvature using a dorsal Nesbit or plications at is elevated by sharp scissors dissection. The graft
the second stage (Fig. 40.4). When chordee per- is then carefully thinned over the back of a fin-
sists despite mobilization of the meatus, the skin ger, to remove the remaining areolar tissue and
incision must be extended proximally to the root to leave just a thin translucent membrane. This is
of the penis. This can however be deferred until a practical method than the commonly described
the second stage when the penile skin envelope technique of pinning the graft out onto a board,
will in any case be degloved proximal to the which offers no tactile feedback. The graft is
meatus. stored in a saline moistened swab until required.
Graft harvest and application: Having cre- The flexible nature of inner prepuce means that a
ated a ventral defect to accommodate the new short and wide graft can easily be reconfigured
urethral plate, a skin graft of appropriate dimen- into a longer and narrower graft. Surprisingly
a b
Fig. 40.3 (a) Proximal hypospadias. Excision of the ure- distal ends of the corpora cavernosa as demarcated in the
thral plate and all fibrous tissue. (b) Ventrally, clefting of figure. Note all fibrous tissue is excised to leave a glisten-
the glans should be deep enough to clearly visualize the ing tunica albuginea
b c
Fig. 40.4 (a–c) Various types of dorsal plication for correcting penile chordee
584 A. T. Hadidi
a b
c d
Fig. 40.5 (a) A preputial graft is obtained from the pre- The dimensions of the graft should not be overgenerous;
puce. (b) The ventral aspect of the penis after straighten- the graft should fit snugly into the ventral defect and
ing and excising all the chordee tissue. (c) The preputial should not heap up into folds. (d) Graft immobilized with
graft accurately tailored to the defect with a V-shaped a “tie-over” pressure dressing prior to compressive foam
inset of the graft into the back wall of the urethral meatus. dressing
long grafts can therefore be obtained from the hood will allow the skin to be fed around ven-
circumference of the prepuce. Once experienced trally, to provide tension-free skin length between
in judging the amount of graft that is needed the ectopic meatus and glans. With the lateral
(always erring on surplus), the surgeon may pre- stay sutures holding the glans apart, the graft is
fer to undertake the graft harvesting as the first accurately tailored into the resulting long rectan-
step of the operation, because the prepuce tends gular defect using 6/0 Vicryl Rapide. A V-shaped
to be easier to handle while the penis and skin inset of the graft into the back wall of the urethral
envelope are still intact. meatus is used to reduce the possibility of subse-
Usually the inner prepuce is enough even in quent anastomotic stricture.
severe perineal hypospadias with bifid scrotum The dimensions of the graft should be tailored
(Fig. 40.5). However, if the inner prepuce is defi- a little wider than required to allow for any con-
cient (complicated hypospadias), other donor traction during the healing phase. However, it
sources of skin graft can be used in conjunction should fit on the stretched defect and should not
with, or instead of the prepuce. Scarring, disease, wrinkle when the tension is released. Whenever
or paucity of preputial tissue is therefore not a possible, sutures along the margins of the glans
problem. cleft should be positioned on the inner edge of
Postauricular Wolfe grafts (PAWGs) are rea- the glans to achieve an unmarked neomeatus. The
sonably thin, pliable, relatively non-hairy, adapt depth of the glans cleft should be secured with
well to moist environment, and leave an incon- absorbable quilting stitches. The author prefers to
spicuous donor scar, so they have proved to be an make several “stabs” with a scalpel size 15 in the
excellent alternative source of tissue. They are an graft to allow drainage of underlying blood and
obvious consideration in boys with prominent uses quilting sutures of Vicryl 6/0 to prevent
ears. There is of course potential for donor site shearing between the grafts on the wound bed
morbidity, and hypertrophic or keloid scars and minimize any hematoma or seroma forma-
behind the ears can be a significant problem. tion. Provided that points of contact between the
Other skin donor sites such as upper, inner arm graft and wound bed are not too far apart, then the
and groin have been less satisfactory in the long developing dermal blood supply can bridge over
run, not adapting as well to constant urine expo- small hematomas without any graft loss. This is
sure and more prone to troublesome hair growth particularly important when using relatively
in adult life. The use of these alternative sources bulky buccal mucosa grafts, where plentiful 6/0
of extragenital skin has been abandoned. Vicryl are advised. Adequate removal of areolar
Buccal mucosal grafts have become the tissue from under the graft, a snug fit into the
favored alternative to prepuce. Its use is particu- glans cleft, plus midline quilting sutures to ensure
larly recommended in the presence of balanitis good apposition in depth, and a snug tie-over
xerotica obliterans (BXO) because it is believed dressing should ensure a nice smooth graft.
it has less tendency to develop BXO. The har- Failure to observe these points may result in a
vesting of buccal mucosa is described in Chap. poorly demarcated, blobby glans cleft with
39. excessive mobility of the graft and thereby com-
The lower lip is recommended to cover the promise an ideal outcome at the second stage.
split glans as it is thinner than the cheek mucosa. Dressings and postoperative management: A
The author, however, prefers to use the cheek snug “tie-over” bolus dressing will hold the graft
full-width grafts, particularly in adult patients securely in place, and together with the quilting
where the mouth-to-penis size ratio is less favor- sutures it will prevent shearing between the graft
able than in children. and the wound bed, thereby reducing the risk of
When there has been extensive ventral chor- graft failure due to unstable fixation, hematoma,
dee release and lengthening of the penile shaft, a or seroma collection. For this purpose a roll of
deficiency of ventral skin may result. A midline chlorhexidine tulle, simple tulle gras, or other pli-
axial-releasing incision in the dorsal prepuce able material is used, the choice being down to
586 A. T. Hadidi
user preference. The bolus dressing should be bed rest immediately after removing support
wide enough to keep the lateral edges of the graft from the graft. An antiseptic emollient such as
sufficiently separated to allow for easy subse- chloramphenicol eye ointment is applied daily to
quent suture removal. The two lateral stay sutures the graft junctions with a cotton wool bud during
are used to make the first “tie-over.” Further the ensuing week. This ensures that the glans
looped 4/0 polypropylene sutures pick up the cleft is kept open until the graft has stabilized and
graft/skin margins on the glans and also include a the risk of adherence between the new margins
deeper bite of the tunica once down onto the has passed. A local cortisone cream is applied on
shaft. These sutures are tied over the dressing roll the graft 3 weeks after the operation to help keep
with the knots in the midline to allow for subse- the graft soft and reduce chances of contraction
quent easy removal. and scarring. Massaging the graft with the cream
How tight should the tie-over dressing be? is very important and not just application of the
This is difficult to quantify and comes with expe- cream. Normal physical activities can be resumed
rience. If the sutures are too loose, then there is a 1 month after surgery.
risk of hematoma collecting under the graft, and The author has stopped using “tie-over” dress-
even if the graft viability is not compromised, an ing since 10 years and is satisfied using just mul-
organized hematoma may result in loss of defini- tiple quilting sutures.
tion of the glans cleft. If too tight, there may be Cyproterone acetate, 300 mg daily, is helpful
pressure necrosis evident at the lateral graft mar- in decreasing libido and spontaneous erections. It
gins on removing the dressing. has a slow onset of action and hence must be
The glans traction stitch can now be removed; started 10 days before surgery. Desipramine,
the tourniquet is released, and after hemostasis 150 mg daily, has a rapid action and can be used
with bipolar coagulation, the donor site in the as an adjunct to cyproterone acetate. Application
foreskin hood is loosely closed with a few 6/0 of PR Freeze Spray (©Crookes Healthcare,
Vicryl Rapide sutures. An F10 urinary catheter is Nottingham, UK) can reverse unwanted erections
inserted as a precaution against developing reflex in an acute situation and should be kept nearby in
urinary retention in the immediate postoperative bedside locker for instant access. Other drugs
period. As there is no need to keep urine away such as ketoconazole and procyclidine have been
from the tie-over dressing, the catheter can be recommended as producing more rapid suppres-
removed a day or two later, at which time antibi- sion of erections, but unfortunately they have
otic prophylaxis is usually discontinued. more troublesome side effects. No drug treatment
The “tie-over” dressing is usually removed by can fully suppress erections without producing
the sixth day in children, though it may be left a unacceptable side effects, but cyproterone offers
little longer if the grafting is very extensive, the an acceptable compromise.
ventral tunica breached during chordee release,
or if buccal mucosa has been used. In adult
patients, the “tie-over” is left for around 10 days. 40.3.2 Second Stage (Fig. 40.6)
“Tie-over” removal in children is facilitated if the
patient has been sedated with oral morphine an The second operation should be scheduled
hour beforehand and ®EMLA (Eutectic Mixture 6 months after the first operation. An erection test
of Local Anesthetics) cream applied for at least should confirm that chordee has been fully cor-
15 min beforehand, to provide topical analgesia. rected. This is usefully deferred until after incis-
It is exceptional for a 3-year-old of normal matu- ing the new urethral plate and mobilizing the skin
rity to require a general anesthetic for this proce- cover. If some curvature remains, the surgeon
dure. By contrast, cooperation is generally poor must decide whether to incise the tunica albu-
in younger children or those who are otherwise ginea or incorporate a dorsal plication procedure.
developmentally immature. The sedation has the If the new urethral plate is narrowed due to par-
added benefit of encouraging a few hours of quiet tial graft failure or contraction, the author
a
b
c
e
Fig. 40.6 (a) A U-shaped incision over the graft strip. (b) from the proximal penile shaft and scrotum). (d)
Shaft skin mobilized before tubing of the neourethra. (c) Subcutaneous tissue harvested on its vascular pedicle
Harvesting of subcutaneous tissue on a vascularized pedi- from the penile shaft skin. (e) Penile skin transposed and
cle for “waterproofing” (subcutaneous tissue harvested completed repair of a circumcised penis
588 A. T. Hadidi
augments the neourethra from the neighboring The urethral catheter is usually left for
skin. Other surgeons may incise the narrow new 7–10 days depending on the level of postopera-
plate and put a graft. tive edema. The catheter must be strapped
The second operation is essentially a Thiersch- securely up on the abdominal wall, first ensuring
Duplay operation with a U-shaped incision tai- that the balloon of the Foley is not resting against
lored around a catheter size F12 or larger the bladder trigone. This is an important point as
according to the age of the patient. it minimizes the risk of disrupting the ventral
The incision is deepened to include adjacent glans repair due to erections or accidental pulling
areolar tissue for proper closure of the new on the penis. Also by immobilizing the catheter
urethral tube. Urethroplasty is performed in a and keeping the balloon off the trigone, the likeli-
continuous subcuticular manner using 6/0 hood of bladder spasms is greatly reduced.
Vicryl on a cutting needle. The use of a cutting Antibiotic prophylaxis using second-generation
needle helps to avoid trauma to the tissues and cephalosporin is continued until the catheter is
allow proper approximation of the edges as in removed.
subcutaneous skin closure. A second suture
line is performed using the adjacent areolar tis-
sue as a second protective layer. A third protec- 40.4 Results
tive layer is very important and is preferably
obtained from the scrotal dartos fascia or tunica The chordee excision and distal urethroplasty
vaginalis around one of the testes (see Chap. (CEDU) is the first choice for the author for grade
43). The protective layer should reach up to the IV hypospadias or whenever there is adequate
neomeatus in the glans. The commonest sites preputial or penile skin (see Chap. 33). Aivar
for fistula formation are the corona and the Bracka is the pioneer who resurrected the two-
proximal junction with the native urethra, so an stage graft technique in the 1990s. He kindly pro-
attempt should be made to cover these vided many of the technical tips mentioned above
adequately. and the tricks to handle problems mentioned
The glans is repaired over the flap with 6/0 below (personal communication). He reported
Vicryl (for deep approximation) and 6/0 Vicryl his results in the first 600 consecutive two-stage
Rapide (for the skin). Having tailored the desired repairs undertaken from 1984 to 1995. An audit
shape of skin envelope, the tourniquet is released was carried out and published [12]. At the time of
to allow for hemostasis using a bipolar coagula- the audit, follow-up ranged from 6 months to
tor. Finally the skin is repaired with 6/0 Vicryl 10 years. The high proportion of adult and reop-
Rapide. Parking surplus skin and subcutaneous eration cases is reflected in the rate of complica-
tissue for possible later use in dealing with a tions. His complications in 600 consecutive
complication rarely proves useful. repairs included the following (Table 40.1):
Dressings and postoperative management:
Each surgeon has his favorite way of dressing the • Fistulae: Thirty-four patients (5.7%); how-
penis but provided that the material used is not ever, the majority occurred during the first
unduly rigid and constricting; the choice is a mat- 3 years. The majority were single pinhole
ter of individual preference. The author prefers to fistula.
apply local gentamycin eye ointment and simple • Strictures: Forty-one patients (7%). There
gauze dressing against the abdominal wall. were two distinct groups with two different
Others prefer to wrap the dressing around the etiologies:
penis and adherent stretch dressing (Cuban® or –– Surgical strictures: Thirteen patients (2%)
something similar). There appears to be no ben- developed early strictures due to wound
efit from using expensive materials or complex infection, hematoma, or urine extravasa-
regimes with prolonged compression and tion into the tissues after premature loss of
occlusion. the catheter were likely explanations in
Table 40.1 Complications of two-stage urethroplasty ease proximally down the bulbar urethra,
(1985–2000)
sometimes even as far back as the external
Author (year) Fistula (%) Stricture (%) sphincter. There seemed no solution in
Free full-thickness Wolfe graft sight until, running out of options, Bracka
Rabinovitch [6] 5/35 (14%) 0
started using the buccal and bladder
Bracka [10, 11] 34/600 41/600
(5.7%) (7%) mucosa to substitute the diseased urethra in
Johnson and Coleman 20/122 2/122 (2%) the early 1990s. Disease confined to the
[13] (16%) penile urethra was managed by a two-stage
Bretteville [14] 10/80 0 full-circumference buccal mucosa substitu-
(12.5%) tion. More extensive disease involving also
Moir and Stevenson [15] 2/40 (5%) 0
the bulbar urethra required additional use
of the bladder mucosa.
• Revisional surgery: Twenty-two patients
some cases. The majority were minor anas- (3.7%) underwent revision of their first stage
tomotic narrowings at the proximal junc- of operations, either for further chordee
tion with the native urethra, and nine were release and grafting, meatotomy, or revising a
cured by a single dilatation. shallow or poorly defined glans cleft. With
–– BXO strictures: BXO accounted for the current practice many of these adjustments
other 28 (5%) strictures in the audited would be incorporated into the second stage
series. These were late-onset strictures pre- rather than requiring separate interventions.
senting within 2–5 years after repair. These Thirty-three patients (5.5%) had minor adjust-
were a challenging group of patients ments after completion of their repairs. These
because at the time, little was known about were mostly of a cosmetic nature to tidy up
the optimal way to manage urethral skin irregularities, deroof suture tunnels,
BXO. Dilatations and endoscopic proce- adjustments to the meatus, or correction of
dures provided very short-term relief and residual rotation deformities.
did nothing to prevent progression of the
disease process down the urethra. Surgical
augmentation procedures that left the dis- 40.5 One-Stage Versus Two-Stage
eased urethra in situ eventually failed, irre- Graft
spective of what graft or flap material was
introduced into the stricture. The two-stage The use of buccal mucosa or skin grafts as a one-
complete substitution of the diseased seg- stage tube (Chap. 39) carries significant risks. It
ment with the genital skin almost invari- is difficult to ensure perfect apposition and fixa-
ably restrictured within 2 years. The tion between the graft and the relatively poorly
two-stage substitution with a non-genital vascularized and mobile ventral penile tissues.
skin, in particular using postauricular The likelihood of shearing forces or hematoma
Wolfe grafts, fared somewhat better, with collection leading to incomplete graft survival
some repairs lasting up to 5 years and occa- with resulting contracture, fistula, or pouch for-
sionally even up to 10 years. Ultimately mation is therefore a significant concern. Even if
however almost every one of these also the graft survives, there is an unpredictable ten-
restrictured. Furthermore, the disease pro- dency to contract while healing, making design
cess did not necessarily confine itself to the of the meatus and urethral caliber a somewhat
neourethra, often spreading proximally for haphazard exercise. Furthermore there is a need,
some distance down the native urethra as for these same reasons, to calibrate the urethra
well. Many adults with very long-standing until the scar junctions have matured. For chil-
BXO had involvement of the entire penile dren in particular, this is an undesirable addi-
urethra together with the spread of the dis- tional trauma.
590 A. T. Hadidi
Full-circumference grafting should there- lation of androgen receptors seems

fore be done in two stages, which ensures a unfounded.
more reliable graft “take,” and allows for sub- • Small prepuce (foreskin): Occasionally, the
sequent tubing of tissue that is already stable prepuce is small and does not provide enough
and well vascularized. Because the variable inner prepuce to cover the required defect.
contracture/relaxation phase of graft healing Consider using a composite graft of inner pre-
has already taken place between the first and putial graft plus buccal mucosa or postauricu-
second stages, the dimensions of the new lar full-thickness skin graft. However, try to
meatus and urethral tube are predictable. preserve the thin preputial graft to line the
Furthermore, the resulting repair is mainte- glanular defect.
nance free when the catheter is removed after • Frequent graft take failure: Common causes
the second stage. of graft take failure are as follows.
–– Hematoma: Make multiple-minute incisions
in the graft to drain the blood under the graft.
40.6 Graft Growth and Puberty –– Sheer movement: Use many quilting
sutures with 6/0 or 7/0; some surgeons pre-
One concern about the use of extragenital fer to use tie-over (the author prefers mul-
grafts in childhood is the long-term growth of tiple quilting sutures).
extragenital grafts at puberty. While the genital –– Infection: Ensure a well-vascularized bed.
skin is laden with androgen receptors and Review antibiotic therapy to include
therefore designed to grow rapidly at puberty, against anaerobic bacteria and bacteroides,
the same is not true for the postauricular skin and start antibiotics before the operation to
and buccal mucosa which are already close to ensure a good level of antibiotics in the
adult dimensions by puberty. Do these alterna- graft while it has its blood supply. Consider
tive graft tissues keep pace with the hormon- using hyperbaric oxygen therapy.
ally induced adolescent penile growth spurt? –– Graft contracture: Ensure many quilting
Unfortunately, publications about the use of sutures, and apply local gentamycin eye
buccal mucosa in childhood have so far looked ointment immediately after surgery. Use
at only short- or medium-term outcomes. local steroid creams 3 weeks after grafting
Buccal grafts do seem capable of the rapid with frequent gentle massaging of the graft.
growth that is required at puberty. Interestingly In severe intractable cases, consider using
with these two-stage graft repairs, a well- hyperbaric oxygen therapy.
directed non-spraying urinary stream is a con- • Ill-defined glans cleft after the first stage: Use
sistent feature throughout childhood and into preputial or lip mucosa when possible, and
adult life. use many quilting sutures. The most crucial
place for quilting sutures is deep in the mid-
line. Sometimes it is possible to dissect the
40.7 ips and Tricks in Special
T graft off the glans cleft, making it deeper, and
Situations even remove some glans tissue to create
enough space for the new urethra and refix the
• Small-sized glans: We define small-sized graft deep in the glans cleft.
glans when the glans width (GW) or the dor- • Graft strip is too narrow to reconstruct the neo-
sal vertical length (DVL) is less than 12 mm. urethra in the second stage: May include the
Consider giving testosterone either as intra- adjacent skin whenever needed. Try to avoid
muscular injection (25 mg) once a month or this option when possible in cases with BXO.
topical application daily for a month. Another • Residual severe chordee during the second
option is to give HCGT to stimulate “mini- stage: This is a major problem, especially if
puberty.” Previous concern about downregu- the excision of the chordee was inadequate
during the first stage. If it is mild (less than 30 References

degrees), one may be justified to perform dor-
sal plication. If more, consider tunica albu- 1. Nove-Josserand G. Traitement de l’hypospadias; nou-
velle method. Lyon Med. 1897;85:198.
ginea longitudinal excision (TALE) or incision 2. Humby G, Higgins TT. A one-stage operation for
of the tunica albuginea and tunica vaginalis hypospadias. Br J Surg. 1941; https://doi.org/10.1002/
flap or dermal graft. bjs.18002911312.
• Frequent bladder spasms: The parents often 3. Cloutier AM. A method for hypospadias
repair. Plast Reconstr Surg. 1962; https://doi.
describe this as “the child suddenly has severe org/10.1097/00006534-196209000-00007.
pain or cramps, pull up his legs”; this may 4. Nicolle FV. Improved repairs in 100 cases of penile
continue for a few minutes or longer, and then hypospadias. Br J Plast Surg. 1976; https://doi.
it disappears completely. The best thing is to org/10.1016/0007-1226(76)90042-4.
5. Byars LT. A technique for consistently satisfac-
reposition the catheter so that it does not lie on tory repair of hypospadias. Surg Gynecol Obstet.
the trigone and immobilize the catheter 1955;100(2):184–90.
securely on the abdominal wall together with 6. Rabinovitch HH. Experience with a modifica-
the penis. The author fixes the glans against tion of the Cloutier technique for hypospadias
repair. J Urol. 1988; https://doi.org/10.1016/
the abdominal wall as a routine, and the cath- S0022-5347(17)42754-6.
eter can be fixed using the same suture. 7. Turner-Warwick R. Observations upon techniques for
Antispasmodics like oxybutynin 0.2 mg per reconstruction of the urethral meatus, the hypospa-
kilogram every 8 h is used when needed. diac glans deformity, and the penile urethra. Urol Clin
North Am. 1979;6(3):643–55.
• Glans dehiscence: This is common in proxi- 8. Webster GD, Brown MW, Koefoot RB, Sihelnick
mal forms of hypospadias which normally S. Suboptimal results in full thickness skin graft
have a small penis and small glans. It is our urethroplasty using an extrapenile skin donor
protocol to give hormonal therapy for patients site. J Urol. 1984; https://doi.org/10.1016/
S0022-5347(17)50820-4.
with glans width less than 12 mm. Ensure that 9. Li ZC, Zheng YH, Sheh YX, Cao YF. One-stage ure-
you have created a wide space for the new urethroplasty for hypospadias using a tube constructed
thra in the glans and do not close the glans with bladder mucosa—a new procedure. Urol Clin
tight on the urethra. When the glans is very North Am. 1981;8:463–70.
10. Bracka A. Hypospadias repair: the two-stage alterna-
small, the surgeon may need to settle with the tive. Br J Urol. 1995; https://doi.org/10.1111/j.1464-
meatus closer to the coronal sulcus to avoid 410X.1995.tb07815.x.
stricture and diverticulum complications. 11. Bracka A. A versatile two-stage hypospadias repair.
• Mucosal prolapse, spraying: Usually due to Br J Plast Surg. 1995; https://doi.org/10.1016/
S0007-1226(95)90023-3.
excessive graft dimensions, inadequate 12. Price RD, Lambe GF, Jones RP. Two-stage hypo-
thinning of the graft or inadequate fixation spadias repair: audit in a district general hospital.
of the graft in the glans. Trim off the Br J Plast Surg. 2003; https://doi.org/10.1016/j.
surplus of the graft and perform bjps.2003.08.015.
13. Johnson D, Coleman DJ. The selective use of a single-
meatoglanuloplasty. stage and a two-stage technique for hypospadias
• Post micturition drippling and poor ejacula- correction in 157 consecutive cases with the aim of
tion: This is a common problem when deal- normal appearance and function. Br J Plast Surg. 1998;
ing with proximal forms of hypospadias as https://doi.org/10.1016/S0007-1226(98)80009-X.
14. Bretteville G. Hypospadias: simple, safe and com-
the corpus spongiosum is poorly developed plete correction in two stages. Ann Plast Surg. 1986;
and the neourethra is very thin and only https://doi.org/10.1097/00000637-198612000-00015.
formed of epithelium without the supportive 15. Moir G, Stevenson JH. A modified Bretteville tech-
vascular and spongiosal tissue normally nique for hypospadias. Br J Plast Surg. 1996; https://
doi.org/10.1016/S0007-1226(96)90055-7.
present.
The Cecil-Culp Technique
41
Dana A. Weiss and Douglas A. Canning
Abbreviation tions. In this chapter, we describe a technique

that Cecil developed in the 1950s for proximal
CC Cecil-Culp hypospadias repairs. However, the technique
has evolved over time in its usage both to opti-
mize its potential and to broaden the indica-
tions for its use.
41.1 Introduction As the surgery for proximal hypospadias and
re-operative hypospadias evolved, it became
One of the greatest challenges in complex penile clear that use of local tissue flaps for enhanced
reconstruction is maintaining a vascular tissue vascular support dramatically improved results in
bed for wound healing. A paucity of skin is com- challenging cases. However, as single stage
mon, particularly in re-operative cases and may repairs became more common even for proximal
lead to the need for skin grafting, while a minor hypospadias, reconstructive urologists used
deficit in skin may prompt a skin closure that is Cecil’s concept less and less. Nevertheless,
under tension. This tension can sometimes be Cecil’s repair continued as a tool to provide con-
alleviated by Z-plasties and local skin flaps, but sistent coverage of the penile shaft or the recon-
any wound closed under tension leads to capil- structed urethra in the most challenging cases.
lary vascular compromise during the healing pro- Most often the technique is used for recurrent
cess, which results in superficial skin breakdown fistula or stricture cases. However, we have used
or more seriously, to complete breakdown of the it to provide ventral skin coverage following cir-
underlying repair. cumcision injuries and other types of trauma.
Penile skin deficiency is common in cases While we have used it almost exclusively to pro-
of proximal hypospadias repairs but also vide ventral skin coverage, in this chapter, we
occurs in boys with severe chordee without will describe its use to provide not only skin but
urethral involvement, usually in redo opera- skin and soft tissue coverage to the dorsum of the
penis following a gunshot wound.
D. A. Weiss · D. A. Canning (*)

Division of Urology, Perelman School of Medicine,
Children’s Hospital of Philadelphia, University of
Pennsylvania, Philadelphia, PA, USA
e-mail: canning@email.chop.edu
594 D. A. Weiss and D. A. Canning
a b
Fig. 41.1 (a–c) Steps of first stage. (a) Incision on the ventral skin to allow tubularization. (b) Neourethra constructed
around a catheter. (c) The neourethra buried in the scrotum
41 The Cecil-Culp Technique 595
41.2 Original Description tions….” However there were still challenges

of the Operative Technique regarding the final urethral meatal position and
(Fig. 41.1) meatal stenosis following these procedures [3].
Ormond Culp declared in 1951 [1]:

It is the inalienable right of every boy to be a 41.3 Use in Primary Repairs
“pointer” instead of a “sitter” before he starts
school. Years after these initial reports, Marshall et al.
reported on their series of 234 patients in 1979
The challenges in constructing a durable and [4]. In this group, 26 had severe penoscrotal or
functional neourethra have led to ongoing evolu- scrotal hypospadias for which he used a three-
tion of techniques. staged Cecil approach including the use of a peri-
Cecil originally reported his repair in 1946. He neal urethrostomy. In their series, they waited
described the creation of a neourethra from the 4–6 month before releasing the scrotum. Marshall
urethral plate and surrounding penile shaft skin. and his coauthors found that the more scrotal tis-
This left the ventrum with insufficient skin for sue they had, the better results they enjoyed fol-
coverage; so after closure of the neourethra, he lowing completion of the repair. They believed
buried the entire length of the penis into a scrotal this was due to reduced tension on the penile ure-
incision he made as a “mirror image” of the por- thra in those cases [4].
tion of the penis that lacked appropriate coverage.
A perineal urethrostomy was created for urinary
diversion [2]. In this repair, in the first stage, a 41.4 se of the Cecil Concept
U
wide caliber urethra was formed and buried. In Following Multiple Failed
the second stage, he left a patch of scrotal skin to Repairs
create a tension-free closure when the penis was
divided from the scrotum after about 6 weeks. Following multiple failed hypospadias repairs,
Culp adopted this technique but added some of the penis suffers from lack of healthy ventral
his own modifications [1]. In his series, he per- skin further complicating future reconstruction.
formed a chordee repair at the first stage without Following redo, urethroplasty surgeons have
urethroplasty. In a second stage, he created a neo- used Z-plasties or rotational flaps, transferred
urethra using a Thiersch-Duplay reconstruction or dorsal island flaps, or grafts. Transfer can be
a tissue flap and then buried the penis into the from distant locations in the form of full or
scrotum. Instead of creating a perineal urethros- split-thickness skin grafts or from local skin that
tomy, he left a urethral catheter in place. He per- has been enlarged using tissue expanders [5, 6].
formed his third stage after a median of 2 months Surgeons have also used direct transfer of scro-
by reseparating the penis from the scrotum, at first tal skin, but this approach leaves a large area of
leaving a strip of scrotal skin on the ventrum but ventral hair-bearing skin that may be objection-
later excising this extra skin as he began to notice able to the boy or his parents following
that he could often close the penile shaft skin adolescence.
without tension after a period of healing. In his More recent series of use for redo hypospadias
early series of 19 patients with proximal hypospa- have been reported with good results. In 2001,
dias, which included 14 primary cases and 5 redo, Ehle et al. reported on 15 patients with fistulae
temporary urine leakage occurred in 3; however and limited skin after previous repair. The fistula
all ceased spontaneously with no persistent fistula was closed primarily and then the ventral surface
in any of the 19 patients. In a later update on 400 of the penile shaft was buried in the scrotum. The
patients, Culp reported that “fistulas became average time before release from the scrotum was
extinct after properly executed Cecil opera- 10 ± 3 weeks [7].
We have evolved in our use of the Cecil repair In our 2017 series, 23/39 patients underwent
both in our technique and in whom we use the a Cecil repair for failed hypospadias. Patients
repair. We have used it primarily in redo or sal- had undergone an average of 3.6 surgeries
vage situations, commonly in conjunction with prior to the Cecil. Seven of these 23 suffered
buccal mucosal grafts or other urethral substitu- additional complications of the hypospadias
tions. We wait longer following the initial scrotal repair despite the Cecil, including fistulae,
flap to allow a longer duration of healing from the diverticulum, dehiscence of the urethroplasty,
time the penis is buried into the scrotum until and recurrent stricture [8]. However, because
scrotal release: waiting 9–18 months (sometimes of the rehabilitation of the ventral shaft tissue
longer in older boys) rather than the original due to scrotal tissue transfer, we have success-
6–12 weeks that Cecil described. This allows the fully addressed these complications in most
skin wounds to soften and for the postoperative cases.
edema to resolve as the scrotal dartos integrates
into the soft tissues of the ventral penile shaft. In
our experience, this results in a robust vascular 41.5 Non-hypospadias Uses
layer over the neourethra that we leave behind on
the shaft without its overlying skin when we The concept of the Cecil-Culp (CC) procedure
release the penis from the scrotum. This avoids has a wide range of uses beyond its original
the risk of leaving hair-bearing skin on the ven- description. Surgeons can use the technique in
tral penile shaft. any setting where there is penile skin defi-
The technique for a modern Cecil repair starts ciency to enhance local skin vascularity and
after the urethral reconstruction is complete, for pliability. We have used this procedure at times
example, immediately following either the fis- whenever we have been challenged with the
tula closure or tubularization of a buccal neoure- ventral skin closure following resections for
thra in a previously failed case. We close as lichen sclerosis, following circumcision com-
much of the ventral penile shaft skin in a ten- plications, and following trauma as well as in
sion-free manner as we can with the available boys with hypospadias. In our previously
skin. Once this is complete, we assess the resid- reported experience, we used this method for
ual skin deficit. This usually is present on the ten boys who had penile curvature due to insuf-
distal shaft, although can be along the entire ficient skin after circumcision or due to pri-
penile shaft. We match the size and location of mary severe chordee. In these ten, three had
skin defect to the appropriate location in the direct implantation of the ventral penile skin to
midline of the scrotum and incise the scrotum. the scrotum, while the others had ventral to
We then suture the edges of the scrotum to the dorsal rotational skin flaps to provide for dor-
penile skin edges (Figs. 41.1 and 41.2). We sal penile shaft coverage that was missing, and
divert the urine with a suprapubic or urethral then we marsupialized the ventrum into the
catheter for a duration that is determined based scrotum at the same setting [8].
on the extensiveness of the urethral repair, the The procedure for ventral skin deficiency
health of the native shaft skin, and the age of the includes transfer of any available ventral skin to
boy. After 12–18 months, when we believe the the dorsum, followed by anastomosis of ventral
skin has healed completely and is supple with no skin edges to the scrotum (Fig. 41.3a–c). We wait
associated residual edema, we incise along the to perform the takedown of the Cecil until
original scar, usually leaving a small amount of 12–18 months after the initial repair. During this
scrotal tissue behind that we then trim away as stage, we divide the penile shaft skin from the
we shape the ventral penile shaft skin closure. scrotal skin. We trim any excess scrotal skin on
This ensures that no scrotal skin is left in the clo- the edges of the penile shaft skin before we com-
sure (Fig. 41.2f). plete the closure (Fig. 41.3d, e).
a b
c d
e f
Fig. 41.2 (a–f) CC repair for hypospadias fistula. (a) placement, scar has softened and penile shaft skin has
Initial appearance, with history of penoscrotal hypospa- stretched. (e) Appearance after CC release, with midline
dias, failed two-stage repair followed by buccal graft and anastomosis of penile shaft skin. (f) 3 months postopera-
re-tubularization and again with two coronal fistulae and tive from CC release. Glans appearance has not changed;
irregular glans. (b) Ventral and distal skin defect after fis- however there is no evidence of recurrent fistula (reprinted
tula repair (*) and skin closure of all remaining skin. from Journal of Pediatric Urology, Vol 14, Issue 4, by
Receiving site in the scrotum (white arrow) is prepared for Weiss et al.: “Back to the future: The Cecil-Culp Technique
CC repair. (c) Attachment of penile shaft skin to scrotal for salvage penile reconstructive procedures,” pp. 328.
skin to cover defect. (d) Approximately 1 year after CC E1–328.E7, ©2018, with permission from Elsevier)
a b
c d
e f
Fig. 41.3 (a–f) CC repair for insufficient skin following incision for takedown will be. (e) Immediately after CC
newborn circumcision with glans loss. (a) Initial appear- release with midline ventral incision closing defect. (f)
ance, note paucity of dorsal skin (*). (b) Transfer of skin 1 year postoperative from CC release, note increase in
from ventrum to dorsum for dorsal skin coverage with dorsal skin (*) (reprinted from Journal of Pediatric
resulting ventral skin deficiency (#). (c) Attachment of Urology, Vol 14, Issue 4, by Weiss et al.: “Back to the
penis into scrotum during CC repair. Note the gap left in future: The Cecil-Culp Technique for salvage penile
the scrotum that is not attached to penile shaft (where yel- reconstructive procedures,” pp. 328.E1–328.E7, ©2018,
low vessel loop is). (d) 1 year post-op from CC place- with permission from Elsevier)
ment. Skin well healed and line marking where initial
41.6 Trauma with incisions of the scrotal flap to redirect blood

flow and then complete resurrection of the phal-
Cecil also reported its use following penile lus 10 days later [8].
trauma in his 1952 manuscript [9]. We have also
used the Cecil-Culp technique following trau-
matic penile injury. The goals are the same: to 41.7 Conclusion
provide local skin coverage and to provide a
robust vascular bed for overall tissue healing. The principle of embedding the penis into the
We used the Cecil in a boy who suffered a shot- scrotum for ventral skin coverage has multiple
gun blast injury during a hunting accident. In uses beyond the original description. The Cecil
this case, the entire dorsal skin and corpora were repair can be a useful tool for the most tenacious
lost, while the urethra and ventral skin survived. fistulae and strictures in children with multiple
Rather than a scrotal flap in this setting, we bur- redo hypospadias surgeries, or it can provide
ied the penis into the suprapubic region to cor- ample vascular supply and skin in settings of sig-
rect for a large dorsal skin and soft tissue defect. nificant penile skin and soft tissue loss from
After 8 months, we released the penis in two trauma. The transferred vascular tissue may help
stages, first delaying the flap in order to redirect to avoid split or full-thickness skin grafts which
blood supply to the flap tissue and then tubular- can be painful, leave significant scars, and can
izing the suprapubic flap as a phalloplasty lead to future contractions and penile curvature.
(Fig. 41.4). Ultimately this boy will need a The technique results in soft, supple skin and
penile prosthesis due to lack of corporal tissue, leaves the donor site with minimal scarring. Our
but he has a cosmetically appropriate penile modifications include leaving the marsupialized
shaft [10]. tissue intact for 12–18 months before release that
We have also used the Cecil concept following enhances the benefits of a robust vascular bed for
penile amputation during a circumcision. In this wound healing and provides for local skin
case the amputation occurred at the junction of growth, which avoids the need for transfer of
the middle and distal thirds of the penis. After a hair-bearing scrotal skin to the penile shaft.
failed microvascular attempt to approximate the Although this technique requires additional sur-
too small corporal vessels in this newborn with a gical stages, enhanced wound healing that pre-
relatively distal, yet devastating injury, we recon- vents additional complications may avoid
nected the urethra and corpora bodies. To enhance additional complications in boys who have suf-
blood supply to the replanted segment and to pro- fered multiple failed repairs and may be the only
mote tissue healing, the entire penis was embed- hope in severe cases. This valuable technique
ded into the scrotum after dermabrasion of the should remain a part of every reconstructive sur-
glans. This child also had a staged takedown, first geon’s toolbox.
a b
c d e
f g
Fig. 41.4 (a) The ventral penile skin remained intact, after the initial surgery, after going a primary incision of
while the entire dorsal penile skin was avulsed with the the flap to mature it, he underwent release of the suprapu-
proximal 3 cm of corpora was absent. (b) The black bic flap. (e) The anterior wall skin flap was tubularized
arrows demonstrate the extent of the surviving erectile dorsally in order to create a bulked penile shaft. (f) 5 years
body and the white arrows point to the thrombosed erec- postoperatively from ventral view. (g) 5 years postopera-
tile bodies distally. (c) The dorsal surface of the penis was tively from dorsal/lateral view
embedded into the anterior abdominal wall. (d) 8 months
References coverage for prior failed hypospadias repair. Urol Int.

2016; https://doi.org/10.1159/000444331.
7. Ehle JJ, Cooper CS, Peche WJ, Hawtrey
1. CULP OS. Early correction of congenital chordee and
CE. Application of the Cecil-Culp repair for treat-
hypospadias. J Urol. 1951; https://doi.org/10.1016/
ment of urethrocutaneous fistulas after hypospadias
s0022-5347(17)68481-7.
surgery. Urology. 2001; https://doi.org/10.1016/
2. Cecil AB. Repair of hypospadias and urethral
S0090-4295(00)00997-3.
fistula. J Urol. 1946; https://doi.org/10.1016/
8. Weiss DA, Smith ZL, Taylor JA, Canning DA. Old
S0022-5347(17)69802-1.
tools, old problems, new solution: the use of a modi-
3. Culp OS. Struggles and triumphs with hypospa-
fied Cecil-Culp concept in the trauma setting. Urology.
dias and associated anomalies: review of 400
2017; https://doi.org/10.1016/j.urology.2017.04.023.
cases. J Urol. 1966; https://doi.org/10.1016/
9. Cecil AB. Modern treatment of hypospadias. J Urol.
S0022-5347(17)63266-X.
1952;67:1006.
4. Marshall M, Johnson SH, Price SE, Barnhouse
10. Weiss DA, Long CJ, Frazier JR, Shukla AR,
DH. Cecil urethroplasty with concurrent scrotoplasty
Srinivasan AK, Kolon TF, DiCarlo H, Gearhart JP,
for repair of hypospadias. J Urol. 1979; https://doi.
Canning DA. Back to the future: the Cecil-Culp
org/10.1016/S0022-5347(17)56777-4.
technique for salvage penile reconstructive proce-
5. Sinha CK, Mushtaq I. Penile resurfacing for denuded
dures. J Pediatr Urol. 2018; https://doi.org/10.1016/j.
penis following circumcision. Pediatr Surg Int. 2012;
jpurol.2018.04.026.
https://doi.org/10.1007/s00383-012-3057-y.
6. Zhang S, Zhou C, Li F, Li S, Zhou Y, Li Q. Scrotal-
septal fasciocutaneous flap used as a multifunctional
The Foreskin and Circumcision
42
Ahmed T. Hadidi
Abbreviations 6th Dynasty, 2340 BC at Sakkara, 20 km from

Cairo (Fig. 42.1). The Hebrews extended the
BXO Balanitis xerotica obliterans practice of circumcision (1300 BC): “Every man
FR Foreskin reconstruction among you shall be circumcised” Genesis 17:10
GA Gestational age [2]. Jesus Christ was born as a Jew and thus was
HR Hypospadias repair circumcised on the eighth day after birth. Paul
LABO Lateral based onlay flap wrote on this first Epistle to the Christians: “Is
SLAM Slit-like adjusted Mathieu any man called being circumcised? Let him not
TIP Tubularized incised plate become uncircumcised. Is any called in uncir-
UTI Urinary tract infection cumcised? Let him not be circumcised” [3].
Although circumcision is never mentioned in the
Quran itself but is mentioned in hadith literature
and the sunnah and is a standard practice for all
42.1 Introduction and History Moslem men to be circumcised sometime before
of Circumcision the age of puberty.
Circumcision is one of the oldest planned opera-

tions known to mankind. The art of circumcision 42.2 Surgical Anatomy
was necessitated by balanitis caused by the bite of the Prepuce
of the desert sand fly and other small insects,
leading then to its place as a ritual. Similar rituals The prepuce (or foreskin) has been present in pri-
existed around the globe (black Africans and mates and most mammals for over 65 million
Australian Aborigines) where small biting insects years. It is prolongation of the shaft’s skin,
flourish (between the 30th parallel and under reflecting beyond itself and passing into a muco-
6000 ft elevation) [1]. sal inner surface. This mucous membrane covers
One of the first records in history of circumci- the coronal sulcus as well as the surface of the
sion is a stone wall in the tomb of Ankhmahor, glans. Grossly, the adult foreskin shows a cutane-
ous surface that is dark and wrinkled and a muco-
sal surface that ranges in color from pink to tan.
A. T. Hadidi (*) Circumcision is documented on the Tomb of
Hypospadias Center and Pediatric Surgery Ankhmahor 2340 BC, and circumcision of
Department, Sana Klinikum Offenbach, Academic infants (as described in Genesis 17) has been
University, Offenbach, Frankfurt, Germany practiced for approximately 4000 years.
604 A. T. Hadidi
Fig. 42.1 The first documentation of circumcision in the

tomb of Ankhmahor at Saqqara (near Cairo), Egypt, some
4500 years ago (private photo ©Ahmed T. Hadidi 2022.
Circumcision on the eighth day represents a mark

of the convent between God and the Jewish peo-
ple. Even prior to monotheistic religions, circum-
cision was routinely practiced on young children
and adolescents.
42.3 Histology of the Foreskin
Histologically, the foreskin is formed of five lay-

ers (Fig. 42.2): Fig. 42.2 Anatomy and histology of the prepuce and
blood supply (©Ahmed T. Hadidi 2022. All Rights
1. The epidermis is a keratinized stratified squa- Reserved)
mous epithelium that contains melanocytes,
Langerhans cells, and Merkel cells. The epi- fascia in the penile shaft which is continuous
dermis is thinner than the mucosal epithelium with the scrotal dartos muscle. At the level of
and usually contains a pigmented basal layer. the edge of the foreskin, the fibers are trans-
It exhibits relatively few hairs, sebaceous versely arranged to form a sphincter to close
glands, and sweat glands. this edge over the anterior end of the glans.
2. The dermis consists of finely fibrillar connec- 4. The lamina propria is composed of a vascular
tive tissue with blood vessels and nerve bun- connective tissue that is looser than the lamina
dles. Meissner corpuscles, Vater-Pacini propria of the glans.
corpuscles, and sweat glands can be observed. 5. The mucosal squamous epithelium is usually
3. The dartos consists of smooth muscle fibers thicker with more irregular and broad rete
invested with elastic fibers and is the middle ridges than in the epidermis. Adnexal struc-
layer of the foreskin. It is an extension of the tures are absent.
42 The Foreskin and Circumcision 605
42.4 Embryology rapid proliferation of the epithelium than the mes-

and Development enchyme, in particular on the dorsum, forming a
of the Prepuce “hood” and the proliferation slows toward the
edges of the urethral opening. By further growth of
Hunter (1935) showed clearly in an elegant study the prepuce, its margins are drawn together on its
on embryos between 11 and 22 weeks gestational ventral surface by two subglans folds, so that the
age (GA), the stages of prepuce and frenulum glans is completely surrounded by the prepuce.
development [4]. Until 11 weeks GA, there is equal The line of fusion between the subglans folds and
development of the ectodermal and mesenchymal the margin of the prepuce form the floor of the
tissues and the glans is naked. Then, there is more glanular urethra and the frenulum (Fig. 42.3).
Fig. 42.3 Development of the lateral part of the prepuce from the preputial lamella. The median part and frenulum
from the median raphe and penile septum (©Ahmed T. Hadidi 2022. All Rights Reserved)
606 A. T. Hadidi
a b c
d e
Fig. 42.4 (a–e) The prepuce is abnormal in hypospadias usually (a) with dog ears (two cobra eyes), (b) as a monk
hood, (c) may be double, (d) too short, (e) too long (©Ahmed T. Hadidi 2022. All Rights Reserved)
42.5 Morphology of the Prepuce correction: first to keep preputial skin and the pre-
in Hypospadias (Fig. 42.4) putial fascia as a reserve in case the hypospadias
correction may be complicated and the surgeon
needs skin or healthy vascular fascia and second
to give the parents and the child the option of fore-
42.6 Fate of the Prepuce skin reconstruction if they wish to opt for this
in Hypospadias option. The author tends to keep the foreskin and
deal with it 6 months later in the form of foreskin
Because the prepuce comes in different forms in reconstruction or circumcision (Fig. 42.6).
hypospadias, it is usually difficult to predict
beforehand if it is possible to reconstruct the
foreskin after hypospadias correction. However, 42.7 atient Consent for the Fate
P
it is usually possible to reconstruct the foreskin in of the Prepuce
most cases of distal hypospadias and some cases in Hypospadias
of proximal and perineal hypospadias.
Unfortunately, many surgeons across the globe It is important that the surgeon explains every-
excise the foreskin as a standard procedure during thing to the patient including the potential possi-
the hypospadias correction operation (Fig. 42.5). bility of foreskin reconstruction. If the surgeon
They also do not give the parents the option of does not explain this option to the parents, he
foreskin reconstruction and claim that it is essen- may be liable in case the parents go to court. It is
tial to remove the foreskin during surgery which not acceptable to say that the foreskin must be
is incorrect. There are two major advantages of removed during the operation of hypospadias
preserving the foreskin during the hypospadias without proper detailed explanation.
Fig. 42.5 Failed tubularized incised plate (TIP) repair There are not enough local healthy tissues to reconstruct
combined with circumcision. Unfortunately, the surgeon neo-urethra and a two-stage graft is needed for correction
performed circumcision at the same time of TIP repair. (©Ahmed T. Hadidi 2022. All Rights Reserved)
42.8 Surgical Correction done, he still has this option. The disadvantage of
of the Prepuce foreskin reconstruction is that it may be compli-
in Hypospadias cated by phimosis, there is a higher incidence of
urinary tract infection and BXO (balanitis xerot-
After successful correction of hypospadias, the ica obliterans), and it may not look exactly like a
surgeon must examine and evaluate the remain- normal foreskin.
ing foreskin with regard to the amount, quality,
and form. If there is adequate healthy remaining
foreskin, without scarring and without “monk 42.8.2 Circumcision
hood” deformity, it is possible to perform fore-
skin reconstruction. The surgeon needs to explain The advantages of circumcision are as follows:
the advantages and disadvantages of each no risk of phimosis, less chance of urinary tract
procedure: infection (UTI) and BXO, possible less inci-
dence of sexually transmitted diseases and
HIV (not evidence based), less incidence of
42.8.1 Foreskin Reconstruction cervical cancer (not evidence based), and less
incidence of penile carcinoma (not evidence
The advantages of foreskin reconstruction are based). The disadvantages are as follows: it
that the child’s penis will look like other boy’s does not look like other boys in the European
penises at school, there will be enough skin to community, and once circumcision is
correct complications if they occur, and when the done there is no chance to do foreskin
child grows up and wishes to have circumcision reconstruction.
608 A. T. Hadidi
Fig. 42.6 (a) Normally, the prepuce covers symmetri- to offer the parents and the patients both options of fore-
cally the penis. (b) In hypospadias, the foreskin usually skin reconstruction or circumcision (©Ahmed T. Hadidi
covers the dorsal and lateral sides of the penis. (c) After 2022. All Rights Reserved)
hypospadias correction, in 70% of patients, it is possible
tricks that may be helpful when performing cir-

42.9 Circumcision of the Prepuce cumcision in hypospadias patients:
in Hypospadias
• The dorsal glanular length is double the ven-
The full details of circumcision in normal chil- tral glanular length (i.e., the glans extends
dren is beyond the scope of this book and is men- more dorsally than ventrally) (Fig. 42.7).
tioned elsewhere [5–7]. Nonsurgical techniques Therefore, the incision must be slanting to
including Mogen clamp, the Gomco clamp, and excise more foreskin dorsally than ventrally.
Plastibell cannot be applied in patients with • The dorsal outer incision of the prepuce
hypospadias because of the irregular and incom- should be proximal to the dog ear in order to
plete foreskin. There are some surgical tips and have a normal looking circumcised penis.
Fig. 42.7 The dorsal vertical length (DVL) is usually double the ventral vertical length (VVL) (©Ahmed T. Hadidi
• It is usually better to incise the foreskin dor- c omplications to develop so as to correct them
sally first and leave it attached ventrally to tai- during the second operation if complications
lor the amount of the foreskin removed occur; (3) to give the parents time to think and
ventrally according to the needs after mobili- choose between foreskin reconstruction and cir-
zation and excision of any scar that resulted cumcision; and (4) to use the opportunity to eval-
from the hypospadias operation. uate and calibrate the meatus and the glans, and
• It is recommended to apply the sutures very perform any fine adjustment of the shape of the
close to the suture line or use subcuticular meatus and glans.
sutures to avoid suture tracts and suture mark- Under general anesthesia and caudal block, the
ings after healing. operation starts with assessment of the meatus
• As postoperative hemorrhage is the most com- and urethra and insertion of size F12 catheter (or
mon complication after circumcision, it is larger depending on the size of the penis and the
recommended to coagulate all the bleeding child’s age). The two edges of the foreskin are
vessels (which may be in spasm and bleed few held with fine toothed forceps at the tip and
hours after the end of surgery) meticulously approximated together over the glans to make
using only bipolar diathermy. sure that we do not reconstruct a tight foreskin
(Fig. 42.8). A stay suture is taken to approximate
the chosen points at the edges of the foreskin and
42.10 Foreskin Reconstruction constitutes the distal end of the foreskin recon-
in Hypospadias struction. The free margins of the foreskin are
refreshed using a sharp scissor to have three lay-
In the Hypospadias Center in Frankfurt, Germany, ers: an inner mucosal layer, an intermediate fas-
we usually deal with the foreskin 6 months after cial layer, and an outer skin layer. The free margins
hypospadias repair (HR) (Fig. 42.8). The reasons are sutured together in three separate layers using
are (1) to allow the urethro-glanuloplasty to heal continuous polyglactin (Vicryl, Ethicon, Johnson
completely before being covered with foreskin, & Johnson) 6/0 on a cutting needle. The inner
as when the foreskin covers the glans, there is a mucosal layer should be inverting and the outer
higher incidence of infection and wound dehis- skin layer should be everting. Using this tech-
cence (Fig. 42.9); (2) to give time for most nique of three layers, the incidence of foreskin
610 A. T. Hadidi
Fig. 42.8 Technique of foreskin reconstruction (FR): A The inner layer is sutured in a continuous inverting suture.
stay suture is taken to approximate the two edges of the The second layer approximates the dartos fascia. The third
foreskin before any incision to make sure that it looks layer is an everting continuous suture (©Ahmed T. Hadidi
symmetrical and is not tight. The edges of the foreskin are 2022. All Rights Reserved)
refreshed using a sharp scissor and closed in three layers.
Fig. 42.9 Classic example of foreskin reconstruction at plasty first, wait 6 months until it has healed completely,
the same time of hypospadias repair. The foreskin recon- and then perform foreskin reconstruction which can be
struction has healed well but the urethroplasty has failed done as a day case surgery (©Ahmed T. Hadidi 2022. All
completely. It is recommended to perform the urethro- Rights Reserved)
wound dehiscence was one child in 540 cases. They should be more aggressive to retract the
The foreskin is retracted over the glans to ensure foreskin 3 months after FR. If the parents do not
that it is not narrow, and the catheter is removed at retract the foreskin twice per week on a regular
the end of the procedure and local antibiotic eye basis, there is a high chance to develop phimosis,
ointment is applied and a compression gauze infection, and BXO. These risks are obviously
dressing to be removed after 2 h. less if circumcision is performed.
If the foreskin opening seems narrow at the The following figures (Figs. 42.12, 42.13,
end of procedure, it is possible to do a dorsal slit 42.14, 42.15, 42.16, 42.17, 42.18, and 42.19)
to make the foreskin opening wider (Fig. 42.10). show examples of foreskin reconstruction and
The child should pass urine before being dis- circumcision after hypospadias surgery with
charged home as a day case. If there is an addi- long-term follow-up and possible complications.
tional glanuloplasty or fistula excision, the Antao et al. reported their retrospective experi-
surgeon should decide whether to leave a catheter ence in 408 patients with distal hypospadias using
for a couple of days or not. different surgical techniques with good results and
In some cases, there is no need to suture the 10% complication rate [8]. Castagnetti et al.
foreskin ventrally. Instead, the excess dorsal fore- reviewed the literature of foreskin reconstruction
skin is excised making sure that it is wide enough in an interesting article and showed complication
(Fig. 42.11). rate between 2 and 30% including wound dehis-
The parents are instructed not to retract the cence, BXO, and phimosis [9]. Van den Dungen
foreskin for 1 month after surgery to avoid wound et al. published a comparative study between pre-
dehiscence. Then, the parents should gradually putioplasty and circumcision in distal hypospadias
try to retract the foreskin which may be difficult in 44 patients [10]. There was 22% complication
during the first 3 months after foreskin recon- associated with preputioplasty but good cosmetic
struction (FR) because of the wound healing. and functional results in both groups.
612 A. T. Hadidi
Fig. 42.10 It is important to make sure that the foreskin incision to widen the foreskin opening and avoid postop-
is not narrow after foreskin reconstruction and should be erative phimosis (©Ahmed T. Hadidi 2022. All Rights
tested after completion of the operation. In case it is found Reserved)
narrow or in doubt, it is better to do a dorsal longitudinal
Fig. 42.11 Foreskin reconstruction after hypospadias repair. In some patients, it is only needed to excise the excess
dorsal foreskin (©Ahmed T. Hadidi 2022. All Rights Reserved)
614 A. T. Hadidi
Fig. 42.12 Foreskin reconstruction is usually possible in Mathieu (SLAM) procedure (©Ahmed T. Hadidi 2022.
about 70% of patients with distal hypospadias. This is a All Rights Reserved)
child with distal hypospadias after slit-like adjusted
Fig. 42.13 5 years after SLAM and circumcision for distal hypospadias (©Ahmed T. Hadidi 2022. All Rights Reserved)
Fig. 42.14 5 years after lateral-based onlay (LABO) flap and circumcision for proximal hypospadias without severe
chordee (©Ahmed T. Hadidi 2022. All Rights Reserved)
Fig. 42.15 5 years follow up after LABO and foreskin reconstruction for proximal hypospadias without severe chor-
dee (©Ahmed T. Hadidi 2022. All Rights Reserved)
616 A. T. Hadidi
Fig. 42.16 Foreskin reconstruction may be possible even in some cases of perineal hypospadias with severe chordee
Fig. 42.17 Partial or complete foreskin dehiscence if the spadias repair as the foreskin may heal and the hypospa-
three layers closure is not observed. It is not advisable to dias repair may develop wound dehiscence or fistula
perform foreskin reconstruction at the same time of hypo- (©Ahmed T. Hadidi 2022. All Rights Reserved)
a b
c d
Fig. 42.18 (a) Pyramid repair of MIP, leaving the fore- repair combined with foreskin reconstruction. (d) Upon
skin intact. (b) On retraction of the intact foreskin, the prepuce retraction, the hypospadias repair has failed and
neourethra developed dehiscence and the urethral opening new urethroplasty must be performed (©Ahmed T. Hadidi
remained where it was before surgery. (c) Hypospadias 2022. All Rights Reserved)
618 A. T. Hadidi
2. Waszak SJ. The historic significance of cir-

cumcision. Obstet Gynecol. 1978; https://doi.
org/10.1097/00006250-197804000-00023.
3. Mattelaer JJ, Schipper RA, Das S. The circumcision
of Jesus Christ. J Urol. 2007; https://doi.org/10.1016/j.
juro.2007.03.016.
4. Hunter R. Notes on the development of the prepuce. J
Anat. 1935;70(Pt 1):68–75.
5. Elder JS. Circumcision. BJU Int. 2007;99:1553–64.
6. Bolnick DA, Koyle MA, Yosha A. Surgical guide
to circumcision. Springer; 2012. https://doi.
org/10.1007/978-1-4471-2858-8.
7. Hadidi AT. Penis. Pediatr Surg Dig. 2021; https://doi.
org/10.1007/978-3-030-80411-4.
8. Antao B, Lansdale N, Roberts J, Mackinnon
E. Factors affecting the outcome of foreskin recon-
struction in hypospadias surgery. J Pediatr Urol. 2007;
9. Castagnetti M, Bagnara V, Rigamonti W, Cimador M,
Esposito C. Preputial reconstruction in hypospadias
Fig. 42.19 Phimosis after foreskin reconstruction repair. J Pediatr Urol. 2017; https://doi.org/10.1016/j.
(©Ahmed T. Hadidi 2022. All Rights Reserved) jpurol.2016.07.018.
10. van den Dungen IAL, Rynja SP, Bosch JLHR, de Jong
TPVM, de Kort LMO. Comparison of preputioplasty
and circumcision in distal hypospadias correction:
References long-term follow-up. J Pediatr Urol. 2019; https://doi.
org/10.1016/j.jpurol.2018.08.001.
1. Hodgson NB. History of hypospadias repair. In:
Ehrlich RM, Alter GJ, editors. Reconstructive and
plastic surgery of the external genitalia. Saunders;
1999. p. 13.
Protective Intermediate Layer
43
Ahmed T. Hadidi
Abbreviations with a two-stage procedure [3]. Between 1988

and 1993, they had fistulae in 14 out of 22 patients
ESF External spermatic fascia (i.e., a fistula rate of 63%). Between 1994 and
PRF Platelet rich fibrin 1997, they had a fistula in one out of 22 patients
SLAM Slit-like adjusted Mathieu (fistula rate of 4.5%). They stressed that the only
TIP Tubularized incised plate change in technique was the introduction of a
waterproofing intermediate layer.
Thiersch (1869) was a very clever surgeon.
As early as 1896, when he described his tech-
43.1 Introduction nique for epispadias, he made sure that the
incision was not symmetrical, so that the ure-
In 1973, Horton et al. estimated the incidence of throplasty suture line lies on the right side of
fistula following hypospadias surgery to range the midline and the skin suture line would lie
between 15 and 45% [1]. In 1996, Duckett and on the left side of the midline, thus avoid hav-
Baskin estimated the incidence to be between 10 ing two suture lines on top of each other (see
and 15% [2]. In the new millennium, the accepted Chap. 1: History, Fig. 1.9).
rate of fistula in distal hypospadias is less than Durham Smith was the first to describe the
5%. introduction of an intermediate or interposition
One of the most important factors in reducing layer between the neourethra and the cutaneous
the incidence of fistula and other complications suture in 1973 [4]. Since then a variety of dif-
of hypospadias repair was the introduction of a ferent methods of waterproofing in different
protective intermediate layer between the neo- types of hypospadias have been published and
urethra and the skin layer. In an interesting arti- each author has reported a lower fistula rate
cle, Telfer et al. in 1998 reported their experience which they attribute to the intermediate layer.
Snow and Cartwright (1999) showed that using
tunica vaginalis has reduced the fistula rate
A. T. Hadidi (*) from 20 to 8% [5].
Hypospadias Center and Pediatric Surgery Types of interposition waterproofing layer
Department, Sana Klinikum Offenbach, Academic include the following (Fig. 43.1):
620 A. T. Hadidi
a b c
d e
Fig. 43.1 (a–e) Methods for protective intermediate layer: (a) De-epithelialization of skin. (b) Tunica vaginalis. (c)
Dorsal preputial dartos fascia. (d) Scrotal dartos fascia. (e) External spermatic fascia
–– Durham Smith (1973) reported skin de- –– Cooper et al. (2001) reported their experience
epithelialization and double breasting [4]. with the use of abortive spongiosum as a pro-
–– Snow (1986) described the use of a tunica tective intermediate layer [12].
vaginalis wrap from the testicular coverings –– Furness (2003) described the use of ventral-
[6]. based dartos [13].
–– Retik et al. (1988) was the first to use a dorsal –– Baba et al. (2017) described the use of Buck’s
subcutaneous flap from the prepuce [7]. fascia [14].
–– Motiwala (1993) described the use of a dartos –– Guinot et al. (2014) reported their experience
flap from the scrotum [8]. with platelet-rich fibrin membrane [15].
–– Yamataka et al. (1998) reported the use of an
external spermatic fascia flap [9].
–– Hadidi (1996) reported the use of multiple 43.2 Skin De-epithelialization
layer closure (triple breasting) in the Y-V
modified Mathieu and the slit-like adjusted Durham Smith (1973) described de-
Mathieu (SLAM) technique in 2012 [10, 11] epithelialization of a strip of skin 5 mm wide
(Chap. 22). on one side of the skin edges [4]. This provides
43 Protective Intermediate Layer 621
a raw surface of deep dermis. This is achieved

by cutting two or three fine longitudinal strips
with a pair of small curved-on-the-flat scissors.
The medial edge of the shaved flap is brought
across the neourethra and sutured to fascial tis-
sue beneath the other flap. The latter is then
sutured to the edge of the de-epithelialized
strip (double breasting). Belman (1988, 1994)
reported improved results using this technique
Fig. 43.2 The use of tunica vaginalis is very popular as a
[16, 17].
protective intermediate layer in proximal forms of hypo-
spadias (©Ahmed T. Hadidi 2022. All Rights Reserved)
43.3 The Tunica Vaginalis Flap

is visible. The tunica vaginalis is opened dis-
Snow (1986) described the use of tunica vagina- tally over the testicle near the junction of the
lis to prevent fistula in hypospadias surgery [6]. tunica vaginalis and epididymis, and the tes-
ticle is brought forth. Traction sutures are
placed on the lateral edges of the tunica vagi-
nalis, and incisions are made a few millime-
In 1999, Snow and Cartwright wrote the fol- ters from the edge of the epididymis on either
lowing (Fig. 43.2): “Tunica vaginalis may be side. Proximal to the testicle, a transverse
easily obtained during almost any type of incision through the tunica vaginalis only is
hypospadias repair without risk to the newly then made. This is similar to the dissection of
created urethra. As with all hypospadias sur- the processus vaginalis from the spermatic
gery, the chordee is generally corrected and cord during an orchidopexy at the proximal
the urethroplasty performed according to the end of the spermatic cord. In some cases, the
surgeon’s preference. If the surgeon has cho- tunica vaginalis is generous and easily covers
sen a pedicle-type hypospadias repair in the penis without separating additional tunica
which the pedicle wraps around one side of vaginalis from the spermatic cord. In this sit-
the phallus, the testicle opposite the pedicle uation, incisions parallel to the spermatic
wrap is chosen. This way, if either the neoure- cord suffice. In the usual circumstance, with
thra pedicle or the tunica vaginalis pedicle the proximal transverse tunica vaginalis inci-
exerts any pulling forces on the penis, they sion made, careful dissection elevates the
oppose and thereby neutralize each other. If tunica vaginalis from the spermatic cord. This
the hypospadias procedure does not include a dissection is carried out parallel to the sper-
pedicle, the surgeon may choose either testi- matic cord towards the superficial inguinal
cle to begin the tunica vaginalis procurement. ring as far as vision and retraction allow, tak-
At the base of the penis, where the penis has ing care to leave all of the surrounding tissue
been degloved, two small retractors are and cremaster fibres with the pedicle to
placed. The assistant gently elevates the tes- ensure its rich blood supply. Length of the
ticle into view at the penoscrotal junction pedicle is seldom a problem, and extensive
between the retractors. The scrotal attach- dissection is not regularly needed. As eleva-
ments to the tunica vaginalis and testicle are tion of the tunica vaginalis takes place, the
separated, and the testicle and tunica vagina- spermatic cord often seems to emanate from
lis can be easily delivered into the wound. a sleeve of this pedicle. To allow the testicle
Dissection of the filmy attachments along the to be replaced in the scrotum, the lateral mar-
spermatic cord is completed to the extent that
622 A. T. Hadidi
43.4 The Dorsal

gin of this sleeve may need a longitudinal Subcutaneous Flap
incision to allow the tunica vaginalis to move
medially and the testicle and spermatic cord In 1988, Retik et al. described the use of a dorsal
to fall laterally. The incision is made laterally subcutaneous flap from the foreskin to waterproof
to preserve as much blood supply to the ped- both free graft and meatal-based repairs [7]. They
icle of the tunica vaginalis as possible. Once published a 7-year follow-up of their experience
the dissection is completed, hemostasis is with meatal-based repairs in 1994 [18]. The tech-
obtained with the electrocautery and the tes- nique is very simple. If circumcision is done at the
ticle is replaced into the scrotum in its natural same time, the circumcision incision is marked on
position. the inner layer of prepuce. Then the inner layer is
With the neourethra in place, the penis is excised with sharp scissors, revealing the deep
ready to be covered with the tunica vaginalis subcutaneous layer. This layer is mobilized from
like a blanket. It has not made a difference the dorsum of the penis, taking care to preserve
which side of the tunica vaginalis is placed the dorsal neurovascular bundle. The outer layer
toward the neourethra, and we prefer to of the foreskin is separated from the subcutaneous
place the shiny visceral surface toward the layer and circumcision is completed. The flap of
neo-urethra. Fine absorbable sutures are subcutaneous tissue is mobilized and brought
used longitudinally to suture the tunica vagi- around to the ventral surface. It needs to be suffi-
nalis parallel and lateral to the dorsal neuro- ciently released in order to avoid the possibility of
vascular bundle. The flap, now sutured into causing torsion of the penis.
place on the lateral aspect of the penis, is In the Hypospadias Center in Frankfurt,
drawn across the ventral aspect of the penis Germany, we routinely use the preputial dartos
to the other side, and a matching longitudi- fascia in hypospadias repair in many patients
nal closure is accomplished. This blankets without having to do circumcision (Fig. 43.3).
the neourethra. If a glans-tunnelling tech- This can be achieved by making a 1 cm longitudi-
nique was used during the hypospadias nal incision at the anterior border of the prepuce
repair, the tunica vaginalis may be passed to expose the subcutaneous vascular flap. This
distally through the glans channel with the dartos fascia is held with a fine mosquito forceps.
neourethra or it may be tacked as far into the Then, with sharp scissors, the preputial skin is
glans channel as possible, because coronal mobilized carefully off the subcutaneous vascular
fistulas are among the most common that flap. This is important in Germany where 70% of
develop. If the neourethra has a pedicle, care parents request reconstruction of the prepuce.
should be taken not to confine the pedicle It is worth mentioning that the dorsal subcuta-
too tightly. It is seldom a problem. When neous layer is a very important step in the
this step is completed, the penis is covered Snodgrass tubularized incised plate technique
on two thirds to three fourths of its circum- (TIP). Yet, there is no indication or necessity to
ference with a blanket of tunica vaginalis perform circumcision in order to harvest the pre-
from one dorsal neurovascular bundle mar- putial dartos vascular fascia as shown in Fig. 43.3.
gin to the other and to the tip of the neoure-
thra ventrally. The skin closure of the
surgeon’s preference can then be accom- 43.5 The Scrotal Dartos Flap
plished with ease. There are no special
dressing requirements for the penis or scro- In 1993 Motiwala described the use of a dartos
tum in regard to the tunica vaginalis use. The flap as an aid to urethral reconstruction [8].
familiar dressing and postoperative care that Churchill et al., in 1996, reported their experi-
the surgeon customarily uses will suffice.” ence in eight patients who underwent urethral
reconstruction without a single fistula [19]. They
Fig. 43.3 The dorsal preputial fascia as a protective foreskin or performing circumcision (©Ahmed T. Hadidi
intermediate layer. It is always possible to raise the dorsal 2022. All Rights Reserved)
preputial fascia as a separate flap without excising the
recommended the use of a dartos flap in second-

ary proximal hypospadias and complex urethral
repair. According to their description:
child. When dissection is maintained in the
proper plane, the tunica vaginalis surround-
ing the testicles and skin of the scrotum are
“The penile incision is extended along the
well preserved. The testicle is covered by
median raphe to the mid scrotum. The dar-
visceral and parietal layers of tunica vagi-
tos layer is dissected from the posterolat-
nalis, and the internal spermatic, cremas-
eral surface of either testicle and isolated
teric and external spermatic fasciae. Since
on a pedicle at the penoscrotal junction.
the scrotum consists of skin and the dartos
The pedicle is approximately 1 cm wide in
tunic comprises smooth muscle and fascia,
infants and 2 cm wide in a 10-year-old
624 A. T. Hadidi
43.6 Triple Breasting

care is required to maintain dissection of the Meatal Based Flap
away from the scrotal skin. Thus, all tissue
between the testis and scrotal skin is not Hadidi reported improved results with the use
mobilized. of triple breasting of the meatal-based flap as
The flap contains vessels with fibroadipose part of the Y-V meatoglanuloplasty (1996) [10]
tissue and resembles an omental flap. It is and of the “slit-like adjusted Mathieu” (SLAM)
approximately 1 mm thick and it does not technique (2012) [11]. Using this technique, he
result in difficult penile skin or glans clo- could perform three suture lines not on top of
sure. There is no need for epinephrine and each other with only a single knot. In his expe-
bleeding is minimal. The dartos flap is long rience, he could achieve the same good results
enough to reach the distal glans with no as using a protective intermediate layer as long
tension. The flap is tacked to the subcuta- as the meatal-based flap is thick and
neous tissue of the ventral penile shaft lat- well vascularized (personal communication)
eral to the sides of the urethral repair. (Fig. 43.4). However, if the meatal flap is thin
Scrotal orchiopexy is performed to fix the or not well vascularized he would use a protec-
testicle on the side from which the dartos tive intermediate layer from preputial dartos
flap was isolated. Skin coverage is fascia. In adults, he would use five suture lines
completed. instead of three because there is more chance
We have raised dartos flaps in patients who of wound breakdown due to erection than in
underwent inguinal or scrotal orchiopexy and children.
in those with ambiguous genitalia. It is simple
to isolate and exists in both scrotal compart-
ments. It is distinct from the scrotal skin, or 43.7 External Spermatic
the visceral or parietal layers of the tunica Fascia Flap
vaginalis. Since the dartos flap can be raised
from either side of the scrotum, it may also be In 1998, Yamataka et al. described the use of
used on more than one occasion should the external spermatic fascia (ESF) to protect against
need arise. It is well vascularized with small fistula and recurrence following hypospadias
branches from the superficial and deep exter- repair [9]. The ESF is the outer layer of the cover-
nal pudendal branches of the femoral artery, ings of the spermatic cord. It is the continuation
the superficial perineal branch of the internal of the innominate fascia covering the external
pudendal artery and the cremasteric branch oblique aponeurosis.
from the inferior epigastric artery. These
branches are from the internal and external
iliac arterial trees and, thus, they are more reli- 43.8 The Hypoplastic
able than a flap based on a single end artery. Spongiosum Layer
The dartos flap is mobile since its base is at the
penoscrotal junction and we have also Cooper et al. (2001) reported their experience
achieved good results with primary penoscro- with the use of abortive spongiosum as a protec-
tal hypospadias repair. The dual blood supply, tive intermediate layer [12]. The principle behind
mobility, simplicity and availability of the dar- the technique is that in most cases of distal hypo-
tos flap make it useful for the urethral recon- spadias, the corpus spongiosum is hypoplastic
struction. Limitation of the dartos flap includes and bifurcates proximal to the urethral meatus.
the need for a penoscrotal incision, and so it is They would suture this hypoplastic corpus spon-
not suitable for distal urethroplasty.” giosum together in the midline on top of the
hypospadias repair (Fig. 43.5).
Fig. 43.5 Cooper et al. (2001) mobilized the hypoplastic

bifurcated corpus spongiosum and used it to cover the
urethroplasty (©Ahmed T. Hadidi 2022. All Rights
Reserved)
43.9 The Ventral Dartos Flap
Furness (2003) was the first to use ventral-based

vascularized dartos flap to cover the tubularized
incised plate flap on 111 patients with success
Fig. 43.4 Triple breasting technique in the SLAM tech-
nique for distal hypospadias repair. Using a cutting nee-
rate of 98.2% (Fig. 43.6) [13].
dle, the urethroplasty starts 2 mm proximal to the urethral Soygur et al. (2005) reported the use of
defect in a subcuticular manner until the distal end and ventral-based dartos flap in distal hypospadias
then the surgeon turns back to where he started in a double when performing the TIP technique [20].
breasting manner and then goes back to the tip again as a
triple breasting manner. There should be 2 mm between
However, in their illustrations, it looked as if they
the three layers of closure (©Ahmed T. Hadidi 2022. All are using the preputial dartos flap but mobilizing
Rights Reserved) it from the ventral aspect.
626 A. T. Hadidi
can only be used when the ventral proximal

aspect of the penis is well developed and not
hypoplastic (Fig. 43.7).
43.11 Fibrin Sealants
In 1992 Kinahan and Johnson reported their

experience on the use of Tisseel in hypospadias
repair [22].
43.12 T
he Platelet-Rich Fibrin
Membrane
Guinot et al. (2014) reported the use of platelet-

rich fibrin (PRF) membrane as a protective inter-
mediate layer in 33 patients with distal
hypospadias using the Thiersch-Duplay tech-
nique [15]. At the beginning of the operation,
5–10 mL of the patient’s blood was collected and
immediately centrifuged. A PRF clot was trans-
formed into a dense fibrin membrane with a
particular cell content and architecture. This

membrane was applied and sutured over the ure-
throplasty (Fig. 43.8).
43.13 Protective Intermediate

Layer in the Hypospadias
Center, Frankfurt
Fig. 43.6 The use of ventral dartos fascia as a protective
intermediate layer (©Ahmed T. Hadidi 2022. All Rights
Reserved) In the Hypospadias Center in Frankfurt,
Germany, we always use a protective intermedi-
ate layer in all hypospadias repair. We routinely
Cimador et al. (2013) and others reported that use the triple breasting technique for distal
in their hands, they have better results and less hypospadias and the tunica vaginalis flap for
complications when using double dartos flaps proximal and perineal hypospadias. However,
overlapping each other from both sides rather we have used successfully several of the above
than a single flap from one side [21]. mentioned flaps in specific situations where the
meatal-based flap was thin and not well vascu-
larized or the child is already circumcised and
43.10 The Buck’s Fascia Flap there is no preputial fascia or when the ventral
proximal penis is well developed. Any surgeon
Baba et al. (2017) described the use of the ventral dealing with hypospadias should have knowl-
Buck’s fascia as a protective intermediate layer edge and experience with all forms of protective
from the proximal ventral aspect of the penis intermediate layer and should be ready to tailor
[14]. Although this is a very good fascial flap, it his approach to fit each patient.
Fig. 43.7 Incision of the Buck’s fascia lateral to Y-shaped with glanuloplasty in hypospadias surgery: A simple
spongious tissue define the surgical plane for partial glans approach with excellent outcome,” pp. 631.e1–633.e5,
disassembly (reprinted from Journal of Pediatric Urology, ©2017, with permission from Elsevier [14])
Vol. 13, by Aejaz Ahsan Baba et al., “Buck’s fascia repair
628 A. T. Hadidi
Fig. 43.8 PRF clot in the middle of the tube after cen- Urology, 10 (2), by Guinot A. et al., “Preliminary experi-
trifugation. Tweezers are inserted into the tube to collect ence with the use of an autologous platelet-rich fibrin
the fibrin clot with red cells attached. The PRF patch is membrane for urethroplasty coverage in distal hypospa-
formed by compression of the clot. The PRF patch is dias surgery,” pp. 300–305, ©2014, with permission from
sutured in situ (reprinted from Journal of Pediatric Elsevier)
References coverage. J Urol. 2003; https://doi.org/10.1097/01.

ju.0000058429.18975.30.
14. Baba AA, Wani SA, Bhat NA, Mufti GN, Lone TN,
1. Horton CE, Devine CJJ, Baran N. Pictorial history of
Nazir S. Buck’s fascia repair with glanuloplasty in
hypospadias repair techniques. In: Horton CE, editor.
hypospadias surgery: a simple approach with excel-
lent outcome. J Pediatr Urol. 2017; https://doi.
Boston; 1973. p. 237–47.
org/10.1016/j.jpurol.2017.06.015.
15. Guinot A, Arnaud A, Azzis O, Habonimana E,
Jasienski S, Frémond B. Preliminary experience with
Adult and pediatric urology. 3rd ed. St. Louis: Mosby-
the use of an autologous platelet-rich fibrin membrane
Year Book; 1996. p. 2549–89.
for urethroplasty coverage in distal hypospadias sur-
3. Telfer JR, Quaba AA, Kwai Ben I, Peddi NC. An inves-
gery. J Pediatr Urol. 2014; https://doi.org/10.1016/j.
tigation into the role of waterproofing in a two-stage
jpurol.2013.09.026.
hypospadias repair. Br J Plast Surg. 1998;51:542–6.
16. Belman AB. De-epithelialized skin flap cover-
4. Smith D. A de-epithelialized overlap flap tech-
age in hypospadias repair. J Urol. 1988; https://doi.
nique in the repair of hypospadias. Br J Plast Surg.
org/10.1016/s0022-5347(17)42022-2.
1973;26:106–14.
17. Belman AB. The de-epithelialized flap and its influ-
5. Snow BW, Cartwright PC. Tunica vaginalis wrap.
ence on hypospadias repair. J Urol. 1994; https://doi.
In: Reconstructive and plastic surgery of the external
org/10.1016/s0022-5347(17)31670-1.
genitalia, adult and pediatric. Philadelphia: Saunders;
18. Retik AB, Mandell J, Bauer SB, Atala A. Meatal
1999. p. 109–12.
based hypospadias repair with the use of a dor-
6. Snow BW. Use of tunica vaginalis to prevent fistulas
sal subcutaneous flap to prevent urethrocutane-
in hypospadias surgery. J Urol. 1986;136:861–3.
ous fistula. J Urol. 1994; https://doi.org/10.1016/
7. Retik AB, Keating M, Mandell J. Complications
S0022-5347(17)32555-7.
of hypospadias repair. Urol Clin North Am.
19. Churchill BM, Van Savage JG, Khoury AE, McLorie
1988;15:223–36.
GA. The dartos flap as an adjunct in prevent-
8. Motiwala HG. Dartos flap: an aid to urethral recon-
ing urethrocutaneous fistulas in repeat hypospa-
struction. Br J Urol. 1993;72:260.
dias surgery. J Urol. 1996; https://doi.org/10.1016/
9. Yamataka A, Ando K, Lane GJ, Miyano T. Pedicled
S0022-5347(01)65432-6.
external spermatic fascia flap for urethroplasty in
20. Soygur T, Arikan N, Zumrutbas AE, Gulpinar
hypospadias and closure of urethrocutaneous fistula.
O. Snodgrass hypospadias repair with ventral
J Pediatr Surg. 1998;33:1788–9.
based dartos flap in combination with mucosal col-
10. Hadidi AT. Y-V Glanuloplasty a new modification
lars. Eur Urol. 2005; https://doi.org/10.1016/j.
in the surgery of hypospadias. Kasr El Aini Med J.
eururo.2005.02.022.
1996;2:223–33.
21. Cimador M, Pensabene M, Sergio M, Catalano P,
11. Hadidi AT. The slit-like adjusted Mathieu technique
de Grazia E. Coverage of urethroplasty in pediatric
hypospadias: randomized comparison between dif-
ferent flaps. Int J Urol. 2013; https://doi.org/10.1111/
12. Cooper CS, Noh PH, Snyder HM. Preservation of ure-
iju.12092.
thral plate spongiosum: technique to reduce hypospa-
22. Kinahan TJ, Johnson HW. Tisseel in hypo-
dias fistulas. Urology. 2001; https://doi.org/10.1016/
spadias repair. Can J Surg. 1992; https://doi.
S0090-4295(00)00999-7.
org/10.1016/0022-3468(93)90298-y.
13. Furness PD. Successful hypospadias repair with a
ventral based vascular dartos pedicle for urethral
Procedures to Improve
the Appearance of the Meatus 44
and Glans
Ahmed T. Hadidi
Abbreviations urine and avoid spraying of urine. The cosmetic

appearance of the meatus and glans may play an
BXO Balanitis xerotica obliterans important role in the psychological well-being of
CEDU Chordee excision and distal the person.
urethroplasty The ultimate goal of hypospadias repair is to
DVL Dorsal vertical length have a near-normal looking penis, functionally
DYG Double Y-Glanulomeatoplasty and cosmetically. Until the 1980s, normal appear-
GAP Glans approximation procedure ance of the meatus and the glans was considered
GW Glans width a luxury or a secondary issue. Many surgeons
MAGPI Meatal advancement glanuloplasty were reluctant to operate on patients with glanu-
incorporated lar hypospadias as most of those boys were able
MAVIS Mathieu and V incision sutured to function normally. With modern sophisticated
MIP Megameatus intact prepuce technology, however, most surgeons are now
PL Penile length only satisfied when they achieve a near-normal
SLAM Slit-like adjusted Mathieu appearing penis and glans for the majority of
TALE Tunica albuginea longitudinal excision their patients.
TIP Tubularized incised plate
VVL Ventral vertical length
44.2 Normal Anatomy
of the Glans and Meatus
(Fig. 44.1)
44.1 Introduction
As with every organ in the body, there is a lot of
The glans penis is a very important part of the variation as regards the normal appearance of the
penis. In addition to being the most sensitive part penis. However, the normal glans has the shape
of the penis, it helps to have a long stream of of an obtuse cone (similar to the cap of a mush-
room) and is covered by a pink smooth mucous
membrane. It is molded over and attached to the
A. T. Hadidi (*) blunt extremity of the corpora cavernosa and
extends proximally over the dorsal aspect more
Teaching Hospital of the Johann Wolfgang Goethe than ventrally (an oblique mushroom cap). The
University, Offenbach, Frankfurt, Germany corpora cavernosa encased by the tunica
632 A. T. Hadidi
a b
Fig. 44.1 (a) Normal appearance of the glans penis. glans. The depth of the glans groove may vary from one
Notice that the two glanular wings do not fuse together in patient to another and is classified into cleft glans, incom-
the midline; instead, they are separated by the frenulum. plete cleft, and flat glans. In cleft glans, when B-B’ are
(b) Appearance of the glans in hypospadias. B/B’ is the brought together, we may have a near-normal-looking
angle between the potential meatus and the rest of the meatus (©Ahmed T. Hadidi 2022. All Rights Reserved)
albuginea extend deep into the glans in approxi- In classic anatomy and surgical anatomy text-
mately 77% of cases (Fig. 44.2). The lips of the books, the glans is described as “the distal end”
meatus are smooth and sharp-edged, forming a of the corpus spongiosum. However, as hypospa-
vertical slit; the urethra is rather ventrally placed dias surgeons, we all see patients with hypospa-
within the glans. dias with normal glans, yet the corpus
44 Procedures to Improve the Appearance of the Meatus and Glans 633
Fig. 44.2 Longitudinal section of the distal penis and (the cavernous artery to the corpus cavernosum and the
glans. Notice that the urethral meatus lies rather ventral in bulbourethral artery to the corpus spongiosum and ure-
the glans. The glans penis is like an “oblique mushroom thra) before continuing as the deep dorsal artery to supply
cap” that extends more dorsally. The corpora cavernosa the glans of the penis. It must be appreciated that the dor-
extends deep into the glans in 77% of cases. The penile sal arteries curve ventrally as they approach the coronal
arteries originate from the internal pudendal artery. Each sulcus forming a vascular ring before entering the glans
penile artery gives two branches at the root of the penis (©Ahmed T. Hadidi 2022. All Rights Reserved)
634 A. T. Hadidi
spongiosum is often hypoplastic and short so can be dissected from the corpora cavernosa in a
that it does not reach the tip of the penis. In other relatively bloodless plane.
words, as explained in Chap. 3 (Embryology), The external urethral meatus is the narrowest
the glans penis develops from the penile mesen- part of the urethra and opens at the lower half of
chyme, and it becomes separated from the rest of the tip of the glans as a vertical slit-like meatus
the penis (corpora cavernosa) by the develop- (Fig. 44.4). Hutton and Babu in 2007 examined
ment of septa and tunica albuginea. In fact, as the the normal anatomy of the external urethral
urethral meatus migrates distally, it takes with it meatus in 75 boys (mean age 6.9 years) and con-
the covering of the corpus spongiosum into the cluded that all 75 boys had a vertical slit-like
glans. In the glans region, the corpus spongio- meatus that commenced at the tip of the penis
sum amalgamates with the spongy tissue of the and ran ventrally [2]. Interestingly, they reported
glans after birth. However the distinction can that the mean (SD) vertical meatal length (AB)
still be seen in cut sections of the glans during was 5.4 mm and the mean length of ventral glans
the fetal life. closure (BC) was 4.7 mm. In other words, the
The corona is the base of the conically shaped external meatus is longer than the ventral glans
glans and occupies 80% of the circumferential opposition and should normally accommodate a
head of the glans. Ventrally, the glanular wings F10 catheter. It must be noted that the authors
do not fuse together in the midline as they are described, rather inaccurately, their findings in
separated by the urethral meatus and the frenu- the drawings and photos attached to the paper as
lum (Fig. 44.3). The diameter of the corona is “ventral glans closure” (BC), when they are actu-
wider than the shaft, and the remainder of the ally describing the attachment of the frenulum
glans forms the coronal sulcus. From a surgical and septum glandis (Fig. 44.3). The glanular
standpoint, this means that the glanular wings wings do not fuse together ventrally.
have a long urine stream and avoid spraying of

urine (as in the case of a gun with a long tube).
Özbey believes that the fossa navicularis acts as a
flow control valve that controls the exit of urine in
a wave-like pattern that avoids spraying of urine
flow (Figs. 44.5 and 44.6) [4]. Henle described
condensation of the corpus spongiosum at the
level of the fossa navicularis as “septum glandis”
[5]. The septum glandis is divided into a dorsal
and ventral median septum by the fossa
navicularis.
The glans is covered with partially keratinized
stratified squamous epithelium. Some textbooks
state that the circumcised glans is much thicker
and more keratinized than in uncircumcised men,
but no well-controlled studies support this
statement.
The frenulum is a pyramidal median fold that
passes from the deep surface of the prepuce to the
meatal groove. Current embryological theory
shows that the prepuce starts laterally and dor-
sally and then grows ventrally over the open
glanular groove to form the floor of the glanular
urethra and the frenulum (see Chap. 3:
Embryology). This is supported by the fact that
Fig. 44.3 Full-term fetus. Cut section at the level of
proximal glans penis. The glanular wings do not fuse the frenulum has its own separate blood supply
together; they are always separated by the frenulum and and comes from the frenular branches of the dor-
the septum glandis. Notice that the urethra is separated sal artery that curve around each side of the distal
from the glanular tissue by the pale corpus spongiosum shaft to enter the frenulum and the glans ven-
(from Baskin et al. “Anatomical studies of hypospadias,”
J Urol., Vol 160, Issue 3 Part 2, pp. 1108–1115, ©1998, trally. Cutaneous sensitivity, which is high over
with permission from Wolters Kluwer [1]) the surface of the glans, is accentuated near the
frenulum. Two hollow fossae flank the frenulum.
The lamina propria separates the erectile tis-
44.3 The Fossa Navicularis sue of the glans from the glans epithelium. In the
glans, the penile fascial layers and the fibrous
The fossa navicularis is the dilated segment of sheath of the corpus spongiosum are absent, so
the urethra immediately proximal to the external the lamina propria adheres firmly to the underly-
urethral meatus. It has a larger diameter which ing erectile tissue. The transition between both
extends deep into the glans [3] (Figs. 44.4 and structures is sometimes difficult to determine at
44.5). Embryologically, it is suggested that the medium or high magnifications. However, at low
fossa navicularis is the junction where the penile power or even after careful gross inspection with
urethra fuses with the glanular urethra (see Chap. a magnifying lens, this transition is evident and
3: Embryology). Anatomically, this is where the follows a line corresponding to the geographic
axis of the urethra changes its orientation from limit of the extension of venous sinuses of the
being horizontal into vertical. This may have an corpus spongiosum. A predominance of free
important function to twist the urine stream to nerve endings over the genital corpuscles has
636 A. T. Hadidi
Fig. 44.4 (a, b) The axis of the urethra changes from from “Asklepios Atlas of the Human Anatomy,” illustra-
horizontal in the penile urethral to a large chamber at the tion of the Male Genital System, Penis, Sections, picture
level of fossa navicularis to longitudinal at the level of number C025/6308, https://www.sciencephoto.com/
external urethral meatus (reprinted with permission media/671134/view/male-genital-system-illustration)

638 A. T. Hadidi
Fig. 44.5 The flow of urine increases in pressure and “The mystery of Jacob Henle’s ‘septum glandis.’” Vol
decreases in velocity as it passes through the fossa navicu- 234, pp. 728–729, ©2019, with permission from Wiley
laris (reprinted from Journal of Anatomy by Özbey, H., and Sons)
been described in the glans. A few neurosecre- that the mean ventral vertical length (VVL) is
tory dermal Merkel cells have been identified at 10.1 mm in 7-year-old boys (5.4 mm plus
the end of the free nerves. 4.7 mm) [2]. Bush et al. measured the glans width
In the glans, the erectile tissue has the charac- (GW) in 240 control infants (1.5 months old) and
ter of a dense, venous plexus. Compared to the found the mean GW to be 14.3 mm (Fig. 44.7)
corpora cavernosa, the interstitial fibrous connec- [6]. It must be remembered that all these mea-
tive tissue is more abundant and contains more surements are highly variable among normal
elastic fibers and less smooth muscle bundles. boys and are operator dependent.
44.4 Normal Dimensions 44.4.1 Glans: Penis Ratio

of the Glans and Penis
in Infants and Adults Sennert et al. showed that the dorsal vertical
length of the glans (DVL) is usually equal to the
Hutton and Babu in 2007 examined the normal glans width (GW) with a mean of 14 mm (range
anatomy of the external urethral meatus in 75 10–18 mm) in infants 6–18 months old [7]. The
“normal” boys (mean age 6.9 years) and reported mean ventral vertical length (VVL) measured
Fig. 44.6 An example to show the effect of fossa navicularis on urine flow. The fossa navicularis acts as a flow control
valve (courtesy of Hüseyin Özbey)
Fig. 44.7 In a normal infant, the dorsal vertical length ally equal to the width of the glans (GW) about 14 mm
(DVL) of the glans is 14 mm, the meatus is 6 mm, and the (©Ahmed T. Hadidi 2022. All Rights Reserved)
ventral vertical length (VVL) is 7 mm and the DVL is usu-
640 A. T. Hadidi
from the tip of the external meatus to the proxi- and evaluate the suitable methods of repair.
mal end of the ventral glans opposition is 7 mm Patients with hypospadias have an abnormal-
(range 5–9 mm). In other words, the VVL is half looking globular glans with a variable degree of
the DVL which is equal to the GW in most clefting and urethral plate projection that can be
infants. Also, the DVL of the glans is about half categorized into three main groups (Fig. 44.8):
the flaccid penile length (PL) in infants (1.5 cm:
3 cm or 1:2) and one third of the flaccid PL in • Cleft glans: There is a deep groove in the mid-
adults (glans 3 cm: total flaccid penile length dle of the glans with proper clefting; the ure-
9 cm or 1:3) (personal communication). Again, thral plate is narrow and projects to the tip of
there are large individual variations among nor- the glans. An example is “megameatus intact
mal individuals. prepuce” hypospadias (MIP—Chap. 23).
Some forms of Thiersch-Duplay, pyramid
repair, glans approximation procedure (GAP,
44.5 Classification of the Glans Chap. 24), and slit-like adjusted Mathieu
in Hypospadias (SLAM, Chap. 22) technique can be employed
with good results (Fig. 44.9).
Despite the growing interest in the final postop- • Incomplete cleft glans: There is a variable
erative appearance of the meatus and glans, there degree of glans split, a shallow glanular
was no clear detailed description or classification groove, and a variable degree of urethral plate
of the hypospadiac glans until Hadidi classifica- projection. If the meatus is mobile, meatal
tion in 2004 [8]. This classification was adopted advancement glanuloplasty incorporated
by the Hypospadias International Society in 2018. (MAGPI, Chap. 20), SLAM, dorsal inlay, or
Such classification is important to standardize the tubularized incised plate (TIP, Chap. 27) glan-
different types of hypospadiac glans anomalies uloplasty procedure may give good results.
a b c
Fig. 44.8 (a–c) Classification of the glans configuration cleft glans: there is a shallow groove with mild glans cleft-
in hypospadias: (a) Cleft glans: there is a deep groove in ing. (c) Flat glans: there is no glanular groove
the middle of the glans with proper clefting. (b) Incomplete
Fig. 44.9 In patients with cleft glans, the Thiersch, GAP, or SLAM technique can be employed with good results.
There is no need to incise the urethral plate (©Ahmed T. Hadidi 2022. All Rights Reserved)
• Flat glans: The urethral plate ends short of the through complete vacuolation by macrophages
glans penis; no glanular groove. There may be and scavenger cells.
a variable degree of chordee, especially in
proximal forms of hypospadias. Generous
glans splitting is required to achieve good cos- 44.6 Evolution of Surgery
metic and functional results. Inverted Y-V on the Glans in Hypospadias
(Chaps. 19 and 25), SLAM, or dorsal inlay
graft glanuloplasty give good results. For decades, surgeons were very reluctant to oper-
ate on the glans penis. Understandably so, for the
In fact, this classification has an embryologi- glans is the most sensitive part of the penis and
cal background. As was mentioned and docu- probably they were concerned about glanular scar-
mented in histological sections in Chap. 3 ring, which may be ugly and even painful. Clinical
(Embryology), the glanular urethra passes experience has shown that the glans penis is a very
through four stages of development: (1) a solid forgiving tissue. It is richly vascularized and heals
glanular plate; (2) glanular canal (through vacu- quickly with little scarring, especially on the ven-
olation and canalization of the solid plate); (3) tral surface. Recognition of this observation
glanular groove, when the floor of the glanular resulted in the evolution of glans dissection.
canal disappears by macrophages and scavenger It has also become clear that it is not enough
cell; and (4) complete urethra canal through the simply to dilate and tunnel the neourethra through
prepuce. A flat glans probably represents the the glans. If the surgeon does that, there will be
solid glanular plate stage. An incomplete cleft increased resistance to the flow of urine resulting
through partial vacuolation and cleft glans in recurrent/persistent fistula. Instead, one has to
642 A. T. Hadidi
create an adequate space for the neourethra to channel technique to deliver the urethra to
avoid subsequent stricture formation. the apex of the glans [10]. This method was
used for many years. Bevan (1917) reported
a technique involving creation of a proxi-
If you want to make a road through a moun-
mal penile flap that was converted into a
tain, you have to create a tunnel.
urethral tube and was pulled through a tun-
nel in the glans [11]. Davis [12], Ricketson
The following review shows the gradual histori- [13], Hendren [14], and Duckett [15] all
cal evolution of techniques for glanuloplasty and channeled a full-thickness skin tube
meatoplasty. This has to do with the development of through the glans. For 500 years, surgeons
fine instruments, suture materials, and anesthesia: recommended canalization, tunneling, or
coring. These three methods are essentially
• During the first centuries after Christ, stretch- the same with progressively larger chan-
ing and glue were described by Galen and nels, and glans stenosis was usually the end
were wrongly interpreted as amputation (see result. Wing rotation may be used to mobi-
Chap. 1: History). lize the glanular wings and wrap them
• During the middle ages, Albucasis described around the neourethra (Figs. 44.10, 44.11,
tunneling and leaving a metal tube inside. 44.12, and 44.13).
• During the modern period: –– Glans split or kippering: The glans split has
–– Tunneling, canalization, coring, and wing been used in various reports to move the
rotation: Dieffenbach pierced the glans to meatus to the apex [3, 21–28] (Figs. 44.14,
the normal urethra and allowed a cannula 44.15, 44.16, 44.17, and 44.18).
to remain in position until epithelialization –– Meatoplasty and glanuloplasty incorpo-
occurred [9]; this was not successful rated: Duckett, in 1981, described the
(Fig. 44.10). Russell described the glans “meatal advancement and glanuloplasty
a b
Fig. 44.10 (a) Glans Tunneling was introduced by niques of glanuloplasty. In hypospadias, the glanular
Albucasis 970 AD. The middle age period was dominated wings are classically rotated downward and outward by
by tunneling, canalization, and coring due to lack of fine the atretic splayed corpus spongiosum. This results in the
instruments and suture material and lack of anesthesia. (b) characteristic splayed glans
Wing rotation is a standard step in many modern tech-
into an inverted V (see Chap. 20: MAGPI)

(Figs. 44.19 and 44.20).
–– Incising the urethral plate: Reddy
described incision of the urethral plate in
1975 [37]. Orkiszewski (1987) called it “a
dead man’s jacket” [38]. Rich et al. (1989)
described a urethral plate incision (hing-
ing) as a modification of the Mathieu repair
to improve the cosmetic outcome of the
neomeatus [39]. This helps to achieve a
slit-like vertical meatus. Snodgrass (1994)
extended the concept of urethral plate hing-
ing by incising the whole urethral plate in
the midline from the hypospadiac meatus
distally [40] (see Chap. 26: Incision of the
Urethral Plate) (Fig. 44.21).
–– Modifications of the Mathieu technique:
Barcat incised the glans deep to accommo-
date the meatal-based flap into the glans
and achieve a slit-like meatus [41]. Boddy
and Samuel excised a “V” from the ventral
Fig. 44.11 Devine and Horton (1961) popularized the
perimeatal flap in the MAVIS technique
glans channel procedure and included a dorsal V-flap with
the glans channel [16]. The V-glanular flap is elevated and [19]. Hadidi described different approaches
part of the glanular tissue is excised to produce a wide to achieve a slit-like meatus [42–44]. The
generous tunnel slit-like adjusted Mathieu or SLAM tech-
nique avoided the fish mouth appearance
incorporated” (MAGPI) procedure [33]. with the classic Mathieu through four sim-
The two basic elements of MAGPI are ple modifications: (1) the distal end of the
meatoplasty and glanuloplasty. Meatoplasty U-shaped incision is slanting and not
comprises a generous longitudinal incision straight; (2) when the flap is flipped
that is closed in a Heineke-Mikulicz style upward, it is closed 3 mm from the tip to
of transverse closure. Glanuloplasty is have a smooth, slanting meatus; (3) a “V”
achieved by placing a stitch on the ventral is excised from the ventral upper edge of
meatal edge and approximating the glanu- the flap; and (4) the glanular wings are
lar skin by three vertical mattress sutures. wrapped obliquely around the neourethra
However, Hastie et al. (1989) reported a (Figs. 44.22, 44.23, 44.24, and 44.25).
high incidence of partial meatal regression
in their patients following the MAGPI pro-
cedure [34]. Arap et al. modified the 44.7 ow to Have a Slit-Like
H
MAGPI technique by using two sutures Meatus
instead of one [35]. Decter, in 1991,
described an “M inverted V” technique fea- There is an incorrect impression that incision of the
turing an M-shaped incision and de- urethral plate is necessary to have a slit-like meatus.
epithelialization of the tissue within the This impression has encouraged some surgeons to
confines of that incision [36]. A stitch is incise the urethral plate in the glans in combination
placed on the ventral lip of the meatus and with other techniques in order to have a slit-like
is pulled toward the distal end of the penis. meatus. This is incorrect and unnecessary and may
This reconfigures the M-shaped incision result in urethral and meatal stenosis.
644 A. T. Hadidi
a b
c d
Fig. 44.12 (a–d) Hadidi (2004) described the nique where a “V” is excised from the tip of the flap to
Y-glanuloplasty for the lateral based flap technique [17]. have a slit-like meatus [18, 19]
Boddy and Samuel (2000) described the MAVIS tech-
Fig. 44.13 Mustardé (1965) popularized the glans channel procedure and included a dorsal V-flap with parameatal flap
[20]
It is possible to have a slit-like meatus with In other words:

any surgical technique of hypospadias if three
things are observed (Fig. 44.26): –– The slit-like meatus is achieved easily in the
Thiersch technique, provided that the glans
–– The dorsal tip of the glans is incised deep to has a deep groove.
create a space for the new urethra and the ure- –– If the glans is small and without deep groove,
thral plate or the flap is advanced to the tip of Zaontz reported slit-like meatus with Thiersch
the created groove (as in Y-V plasty). after hormone enhancement (see Chap. 24) [45].
–– The ventral tip of the urethral meatus should –– If the glans is small, Barcat described the
be shorter than the dorsal tip by excising a “V” balanic groove technique to have a slit-like
from the ventral tip of the flap (i.e., the ure- meatus with Mathieu by incising the tip of the
thral meatus should be slanting with the dorsal glans deep [41].
tip slightly longer than the ventral tip). –– Another method is to perform the inverted
–– The glanular wings should be wrapped around Y-technique as described by Hadidi in the
the urethral meatus and approximated at a double Y-glanuloplasty [43].
level about 2–3 mm proximal to the dorsal tip –– Excision of the “V” as in MAVIS technique
in order to maintain the slanting position of has helped to make the ventral tip of the flap
the urethral meatus. shorter and slit-like.
646 A. T. Hadidi
Fig. 44.14 Glans splitting or kippering was described for 1000 years; Byars described splitting the glans and rotated
the prepuce ventrally [29]
44.8 ow to Have a Wide Meatus

H than 12 mm) is to avoid suturing the glanular
and Avoid Meatal Stenosis wings together in the midline and insert some
penile skin in between the glanular wings.
The following steps help to have a wide meatus Please remember that normally the glanular
and avoid meatal stenosis: wings are separated ventrally by the
frenulum.
–– Reconstruct the new urethra around a wide –– Regular calibration (a term introduced in lit-
catheter (F12 or more according to age). erature in recent years to describe regular dila-
–– Deep incision and create a space in the glans tation): In the author’s opinion, if the patient
to avoid compression and ischemia. You may requires more than 1 dilation (or calibration),
even excise part of the glanular tissue as dilatation will not solve his problem.
described by Horton and Devine and using a
V-flap from the glans [16].
–– Use techniques that add healthy epithelium 44.9 ow to Correct Meatal
H
and tissue to the circumference of the urethra Stenosis
(slit-like adjusted Mathieu or SLAM, double
Y-glanuloplasty or DYG, dorsal inlay graft). –– Ventral incision of narrow meatus and urethra
–– When the glans is small (less than 12 mm), (if the incision is short and does not result in
some prefer to give preoperative hormone recurrence of hypospadias).
therapy. Others accept the less perfect solution –– Dorsal incision of the narrow meatus and
and create the meatus near the coronal sulcus. apply a buccal mucosal graft (dorsal inlay
–– One other option in case of small glans (less graft, see Chap. 27).
Fig. 44.15 Asopa [30] and Duckett [31] popularized the use of preputial island flaps to reconstruct the urethra
–– Y-V plasty of the meatus: A “Y” is made to 44.10 Techniques to Improve

widen the narrow meatus and is closed as a the Appearance of the Glans
“V.” This is a very reliable method that is easier (Figs. 44.27, 44.28, 44.29,
to perform on the ventral aspect of the meatus and 44.30)
than the dorsal aspect. However, this may
make the meatus not slit-like. This is a price to Some patients may present at puberty or several
pay but is well accepted by most parents. years after hypospadias surgery complaining of
–– Ventral incision of the whole narrow urethra unsatisfactory glans and meatus appearance.
and reconstruction of a new wide urethra. This Several techniques may be used to improve the
is usually the only solution in order to correct appearance of the meatus and the glans including
complications following incision of the ure- inverted Y-meatoglanuloplasty, Devine and
thral plate or the TIP procedure. Horton (McKenna) ventral V-advancement glan-
–– Exclude BXO (balanitis xerotica obliterans): uloplasty, unilateral SLAM technique, and redo
this is more common in older children and SLAM technique.
may require a more aggressive treatment if
medical treatment fails.
648 A. T. Hadidi
Fig. 44.16 Cloutier [23] and Bracka [32] described splitting the glans and laying down a preputial skin graft
Fig. 44.17 Hadidi

described the “chordee
excision and distal
urethroplasty” (CEDU,
see Chap. 33) [28]
where the severe
chordee is excised using
the “tunica albuginea
longitudinal excision”
(TALE) procedure and
the preputial skin is used
to reconstruct the distal
urethra, leaving the
original meatus intact as
a natural perineal fistula
(©Ahmed T. Hadidi
2022. All Rights
Reserved)
650 A. T. Hadidi
Fig. 44.18 Elder et al. 1987 described the ONLAY requires division of the urethral plate [27]. Part of the pre-
island flap (Chap. 31) [26]. Hadidi (2012) described the puce (A) is mobilized and sutured to the narrow urethral
lateral-based onlay (LABO, see Chap. 30) technique for plate (A′) to have a fully epithelialized neourethra
proximal hypospadias without severe chordee that (©Ahmed T. Hadidi 2022. All Rights Reserved)
Fig. 44.19 Duckett (1981) described the MAGPI technique that was followed by several modifications [33]
Fig. 44.20 Stock & Hanna (1997) combined the Heineke-Mikulicz and the Thiersch-Duplay principles to correct
distal hypospadias (see Chap. 24)
652 A. T. Hadidi
Fig. 44.21 Reddy (1975) was the first to incise the urethra plate [37]. Orkiszewski [38], Rich [39], and Snodgrass [40]
adopted the same principle (©Ahmed T. Hadidi 2022. All Rights Reserved)
Fig. 44.22 Barcat described the balanic groove technique where he added a deep midline incision to the meatal-based
flap and buried it deep into the glans to have a slit-like meatus [41]
Fig. 44.23 Hadidi (1996) described the Y-V glanuloplasty to have a wide slit-like meatus [42]
654 A. T. Hadidi
Fig. 44.24 Hadidi (2010) described the inverted Y in combination to Thiersch-Duplay [43] (Chap. 25) (©Ahmed
Fig. 44.25 Hadidi (2012) described the SLAM (“slit-like adjusted Mathieu”) technique as a simple procedure to
achieve a slit-like meatus with the Mathieu technique [44] (Chap. 22) (©Ahmed T. Hadidi 2022. All Rights Reserved)
656 A. T. Hadidi
Fig. 44.26 In order to have a slit-like meatus, the dorsal proximal to the tip of the glans (©Ahmed T. Hadidi 2022.
tip should be 2–3 mm longer than the ventral tip and the All Rights Reserved)
glanular wings should be approximated at a level 2–3 mm
44.10.1 Double Y-Glanulomeatoplasty
Fig. 44.27 (a, b) The use of DYG technique to improve the appearance of the glans and meatus (©Ahmed T. Hadidi
658 A. T. Hadidi

44.10.2 Unilateral SLAM
Fig. 44.28 The use of unilateral SLAM technique to improve the appearance of the glans and the urethra (©Ahmed
660 A. T. Hadidi
44.10.3 Inverted Y SLAM Technique
Fig. 44.29 The use of inverted Y SLAM technique to improve the appearance of the glans and reconstruct new urethra
44.10.4 Redo SLAM Technique
Fig. 44.30 The use of SLAM technique to correct failed sion of the urethral plate did not help to increase the width
TIP. The child was unfortunately circumcised at the TIP of the urethral plate (©Ahmed T. Hadidi 2022. All Rights
operation and there was little penile skin left. Notice that Reserved)
the TIP operation has healed as a linear scar and the inci-
662 A. T. Hadidi
References 20. Mustardé JC. One-stage correction of distal hypospa-

dias: and other people’s fistulae. Br J Plast Surg. 1965;
https://doi.org/10.1016/S0007-1226(65)80068-6.
21. Beck C. Hypospadias and its treatment. Surg Gynecol
Obstet. 1917;24:511.
org/10.1016/S0022-5347(01)62711-3.
22. Humby G, Higgins TT. A one-stage operation for
2. Hutton KAR, Babu R. Normal anatomy of the
hypospadias. Br J Surg. 1941; https://doi.org/10.1002/
external urethral meatus in boys: implications for
bjs.18002911312.
hypospadias repair. BJU Int. 2007; https://doi.
23. Cloutier AM. A method for hypospadias
org/10.1111/j.1464-410X.2007.06798.x.
3. Turner-Warwick R. Observations upon techniques for
org/10.1097/00006534-196209000-00007.
reconstruction of the urethral meatus, the hypospa-
24. Barcat J. Current concepts of treatment. In: Horton
diac glans deformity, and the penile urethra. Urol Clin
CE, editor. Plastic and reconstructive surgery
North Am. 1979.
of the genital area. Boston: Little Brown; 1973.
4. Özbey H. The mystery of Jacob Henle’s ‘septum glan-
p. 149–263.
dis’. J Anat. 2019; https://doi.org/10.1111/joa.12965.
25. Cronin TD, Guthrie TH. Method of Cronin and
5. Henle J. Eingeweide. Anat Hand-Atlas zum Gebrauch
Guthrie for hypospadias repair. In: Horton CE, editor.
im Secirsaal. 1877; https://doi.org/10.11588/
diglit.3231.
Boston: Little Brown; 1973. p. 304–14.
6. Bush NC, DaJusta D, Snodgrass WT. Glans penis
26. Elder JS, Duckett JW, Snyder HM. Onlay island flap in
width in patients with hypospadias compared to
the repair of mid and distal penile hypospadias with-
healthy controls. J Pediatr Urol. 2013;9:1188–91.
out chordee. J Urol. 1987; https://doi.org/10.1016/
7. Sennert M, Wirmer J, Hadidi AT. Preoperative
S0022-5347(17)43152-1.
glans and penile dimensions in different hypo-
27. Hadidi AT. Proximal hypospadias with small flat
spadias grades. J Pediatr Urol. 2021; https://doi.
glans: the lateral-based onlay flap technique. J
org/10.1016/j.jpurol.2021.09.020.
Pediatr Surg. 2012; https://doi.org/10.1016/j.
8. Hadidi AT. Classification of hypospa-
jpedsurg.2012.06.027.
dias. Hypospadias Surg. 2004; https://doi.
28. Hadidi AT. Perineal hypospadias: back to the
org/10.1007/978-3-662-07841-9_7.
future Chordee Excision & Distal Urethroplasty.
9. Dieffenbach J. Guérison des fentes congénitales de la
verge, de l’hypospadias. Gaz Hebd Med. 1837;5:156.
jpurol.2018.08.014.
10. Russell RH. Operation for severe hypospadias. Br Med
29. Byars LT. Functional restoration of hypospadias
J. 1900; https://doi.org/10.1136/bmj.2.2081.1432-a.
deformities; with a report of 60 completed cases. Surg
11. Bevan AD. A new operation for hypospadias. J Am
Gynecol Obstet. 1951.
Med Assoc. 1917;68:1032.
30. Asopa HS, Elhence IP, Atri SP, Bansal NK. One stage
12. Davis DM. The pedicle tube graft in the surgical
correction of penile hypospadias using a foreskin
treatment of hypospadias in the male. Surg Gynecol
tube. A preliminary report. Int Surg. 1971.
Obstet. 1940;71:790.
31. Duckett JW. Transverse preputial island flap tech-
13. Ricketson G. A method of repair for hypospadias. Am
nique for repair of severe hypospadias. Urol Clin
J Surg. 1958;95:287–93.
North Am. 1980.
14. Hendren WH. The Belt-Fuqua technique for repair of
32. Bracka A. A versatile two-stage hypospadias repair.
hypospadias. Urol Clin North Am. 1981.
Br J Plast Surg. 1995; https://doi.org/10.1016/
15. Duckett JW. The island flap technique for hypospa-
S0007-1226(95)90023-3.
dias repair. Urol Clin North Am. 1981;8:503–11.
33. Duckett JW. MAGPI (meatoplasty and glanu-
16. Devine CJ, Horton CE. A one stage hypospa-
loplasty). A procedure for subcoronal hypo-
dias repair. J Urol. 1961; https://doi.org/10.1016/
spadias. Urol Clin North Am. 1981; https://doi.
S0022-5347(17)65301-1.
org/10.1097/00005392-200205000-00059.
17. Hadidi AT. Lateral-based flap with dual blood supply:
34. Hastie KJ, Deshpande SS, Moisey CU. Long-term
a single-stage repair for proximal hypospadias. In:
follow-up of the MAGPI operation for distal hypospa-
Hadidi AT, Azmy A, editors. Hypospadias surgery. 1st
dias. Br J Urol. 1989; https://doi.org/10.1111/j.1464-
ed. Berlin Heidelberg: Springer; 2004. p. 209–16.
410X.1989.tb05198.x.
18. Boddy SA, Samuel M. A natural glanu-
35. Arap S, Mitre AI, De Goes GM. Modified meatal
lar meatus after “Mathieu and a V incision
advancement and glanuloplasty repair of distal
sutured”: MAVIS. BJU Int. 2000; https://doi.
hypospadias. J Urol. 1984; https://doi.org/10.1016/
org/10.1046/j.1464-410X.2000.00758.x.
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19. Boddy SA, Samuel M. Mathieu and “V” inci-
36. Decter RM. M inverted V glansplasty: a procedure
sion sutured (MAVIS) results in a natural glanular
meatus. J Pediatr Surg. 2000; https://doi.org/10.1016/
org/10.1016/s0022-5347(17)37881-3.
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37. Reddy LN. One-stage repair of hypospadias. Urology. 42. Hadidi AT. Y-V Glanuloplasty a new modification
1975; https://doi.org/10.1016/0090-4295(75)90070-9. in the surgery of hypospadias. Kasr El Aini Med J.
38. Orkiszewski M. Urethral reconstruction in skin defi- 1996;2:223–33.
cit. Urol Pol/Polish Urol. 1987;40:12–5. 43. Hadidi AT. Double Y glanuloplasty for glanu-
39. Rich MA, Keating MA, Snyder McC H, Duckett lar hypospadias. J Pediatr Surg. 2010; https://doi.
JW. Hinging the urethral plate in hypospadias org/10.1016/j.jpedsurg.2009.11.019.
meatoplasty. J Urol. 1989; https://doi.org/10.1016/ 44. Hadidi AT. The slit-like adjusted Mathieu technique
S0022-5347(17)39161-9. for distal hypospadias. J Pediatr Surg. 2012; https://
40. Snodgrass W. Tubularized, incised plate urethroplasty doi.org/10.1016/j.jpedsurg.2011.12.030.
for distal hypospadias. J Urol. 1994; https://doi. 45. Zaontz MR. The GAP (glans approximation proce-
org/10.1016/S0022-5347(17)34991-1. dure) for glanular/coronal hypospadias. J Urol. 1989;
41. Barcat J. Hypospadias. 3. Les uréthroplasties. Leurs https://doi.org/10.1016/S0022-5347(17)40766-X.
résultats. Leurs complications. Ann Chir Infant. 1969.
Buried Penis (BP)
45
Ahmed T. Hadidi
Abbreviations plex” [5, 11–13] techniques described for the

correction of BP. Some “simple” techniques
BP Buried penis have reported excellent results [6, 8, 9]. Complex
CEDU Chordee excision and distal techniques have been reported and some have
urethroplasty had less satisfactory long-term outcome [5, 11–
CMP Congenital mega prepuce 14]. The controversy in the literature suggests
CP Concealed penis that BP is a wide spectrum of anomalies [13]. In
LABO Lateral-based onlay this chapter, we will describe a practical classifi-
LIP Long inner prepuce cation based on morphological findings in
patients with congenital BP and report the con-
stant observation of an abnormally long inner
prepuce (LIP) in all children presenting with BP
45.1 Introduction and describe a versatile surgical approach that
can be used in all grades of BP. This is based on
Buried penis (BP) is an uncommon anomaly first the paper published in the Journal of Pediatric
described by Keyes in 1919 [1] as Surgery in 2014 [15].
…an apparent absence of the penis which exists Buried penis (BP) is now defined as “a rare
when the penis lacks its proper sheath of skin, lies congenital anomaly characterized by the appar-
buried beneath the integument of the abdomen, ent absence of the penis which exists when the
thigh or scrotum. penis lies buried beneath the integument of the
abdomen, thigh or scrotum.”
Since then the terms used included buried
penis [2], concealed penis [3], inconspicuous
penis [4], hidden penis [5], congenital megapre- 45.2 Morphology of Buried Penis
puce [6], trapped penis [7], and webbed penis [5].
Confusion increases with the enormous num- Different authors reported divergent descriptions
ber of reports of “simple” [6, 8–10] and “com- of the morphology of BP. Crawford [2] described
“a distinct fibromuscular layer, tethering the shaft
to the abdominal wall and resembling dartos mus-
A. T. Hadidi (*) cle.” Wollin et al. stressed the abnormal skin
mobility over the shaft of the penis [11]. Others
Teaching Hospital of the Johann Wolfgang Goethe reported divergent observations, ranging from
University, Offenbach, Frankfurt, Germany abnormal attachments of tissue resembling the
666 A. T. Hadidi
tunica dartos, which tethers the shaft to the abdom- The penile skin is freed off the loose dartos
inal wall, to “an insufficient attachment of the dar- fascia down to the penoscrotal junction. The
tos fascia and penile skin to Buck’s fascia” [2, 10, stretch is released and the position of the penis is
11]. Redman on the other hand did not observe in evaluated. If there is any abnormal bands or any
his personal series of 31 boys any abnormalities of evidence of distal attachment of the fundiform
tunica dartos or any tethering bands [9]. and suspensory ligaments (grade II), the penis
The inner mucosal prepuce (or long inner pre- must be freed off any abnormal bands of liga-
puce, LIP) was almost as long as the whole ments that may pull the penis inside. Patients
stretched penile length in all the cases operated with excess suprapubic fat (grade III) will benefit
on by the author (Fig. 45.3b, c). The penile dartos from excision of fat to make the penis more obvi-
fascia was loosely attached to the tunica albu- ous. Care was taken to avoid damage to the
ginea in all the patients. In addition, abnormal nerves and vessels to the penis which enter the
distal attachment of the fundiform and suspen- corpora of the penis at the 2 and 10 o’clock posi-
sory ligaments was observed in some patients. tions (Fig. 45.3).
This was evidenced by the retraction of the penis The tunica albuginea is fixed to the perios-
after the ventral midline incision and freeing of teum at the symphysis pubis with one nonab-
the skin and fascia from the body of the penis. In sorbable (proline 0) suture at 12 o’clock. Two
some patients, the condition was worsened by the more sutures are used to fix the penis to the
presence of excess suprapubic fat. pubic bones at 4 and 8 o’clock. The penile skin
Based on the above-mentioned observations, at the base of the penis is fixed to the tunica
buried penis (BP) can be classified into three albuginea using four sutures at 12, 3, 6, and 9
main grades according to the severity of the con- o’clock (Fig. 45.2h). The excess LIP is excised
dition [15] (Fig. 45.1): and the proximal penile skin is sutured to the
mucosal collar 0.5 cm from the coronal sulcus
Grade I: The abnormal long inner prepuce (LIP): as a circumcised penis.
This is a constant finding in all the patients. A Surgical correction of patients with BP grade I,
subgroup includes children who suffered from associated with intermittent ballooning (previ-
intermittent ballooning when passing urine ously referred to as “congenital megaprepuce” or
due to a sort of valve-like mechanism at the CMP) follows the same principles of surgical cor-
terminal end of the prepuce that prevented free rection of grade I with midline incision, excision
flow of urine. of the abnormal fascial attachment, and excision
Grade II: In addition to LIP, there is an abnormal of the very long inner prepuce (Fig. 45.4).
distal attachment of the fundiform and suspen- Patients with trapped penis (improper circum-
sory ligaments into the midshaft of the penis. cision in BP grade I) require special attention as
Grade III: There is excess suprapubic fat in addi- they usually do not have enough outer skin and
tion to LIP and abnormal distal attachment of the surgeon must try to preserve every millimeter
the fundiform and suspensory attachments. of skin available. In some patients, there is inad-
equate skin and one may have to use skin graft
(Fig. 45.5). A Z-plasty is usually needed at the
45.3 Operative Technique tight preputial mucocutaneous junction to widen
(Fig. 45.2) the tight junction.
Patients with hypospadias and buried penis may
Two stay sutures are used to stretch the ventral sur- have the BP corrected as a second operation after
face of the prepuce. A ventral midline incision is complete correction of hypospadias using the pre-
made that extends from the tip of prepuce down to ferred technique of the surgeon. The author prefers
the penoscrotal junction. A stay suture is inserted the “lateral-based onlay” flap (LABO) or “chordee
into the tip of the glans and a catheter size F12 is excision and distal urethroplasty” (CEDU) tech-
inserted through the urethra into the bladder. niques (see Chaps. 30 and 33) [16, 17].
45 Buried Penis (BP) 667
a b
c d
Fig. 45.1 (a–d) Classification of buried penis: (a) cia, and normal attachment of the fundiform and suspen-
Normal penis with normal length of penile shaft, normal sory ligament, (b) grade I, (c) grade II, (d) grade III
inner prepuce length (less than 1.5 cm), normal dartos fas- (©Ahmed T. Hadidi 2022. All Rights Reserved)
45.4 Patients, Results, Partial retraction occurred in six patients that

Complications, and Follow- required further surgery.
Ups (Figs. 45.3 and 45.4) Fig. 45.3 shows an interesting observation
that with degloving and stretching, the penis had
Between January 2000 and December 2019, the a length of 4.3 cm and the ratio between the
author operated on 96 patients with buried penis. glans and penile shaft was 1:2. One year after
This included 46 patients with grade I, 32 patients surgery with complete healing and without
with grade II that required division to achieve sat- stretch, the glans/penile body ratio was 1:1 in
isfactory initial results, and 18 with grade III that the flaccid state (Fig. 45.3e). With a 10-year
required additional excision of suprapubic fat. follow-up, the penis appeared shorter than
668 A. T. Hadidi
a b c
d e f
g h i
Fig. 45.2 (a–i) Operative steps (©Ahmed T. Hadidi 2022. All Rights Reserved)
a b c
d e f
Fig. 45.3 (a–f) The operative details: (a, b) A 3-years- state, the length of the glans seems to be equal to the
old child with classic buried penis. (c) Shows that the length of the penile shaft. (f) The appearance after
inner prepuce is 3.5 cm long and the glans is 1 cm long. 10-years follow-up. The shaft of the penis does not appear
(d) Shows the fascia and ligaments that pull the penis to be much longer in the flaccid state (©Ahmed T. Hadidi
inward. (e) The very satisfactory results of surgery 1 year 2022. All Rights Reserved)
after surgery (compared to (a)). However, in the flaccid
normal and the glans/penile shaft ratio in flaccid stretched length). The surgical technique used
state remained 1:1 (Fig. 45.3f). for correction depended on the class of the dis-
order. However, it has become internationally
agreed now to limit the term buried penis (BP)
45.5 Discussion to the congenital condition that appears after
birth and that concealed penis should be reserved
Two classification systems of the BP have been to postpubertal patients due to acquired excess
proposed. The classification system by Crawford fat deposition. Micropenis is by definition not a
includes three broad categories: concealed part of BP and webbed penis is a completely dif-
penis, buried penis (partial or complete), and ferent category.
penoscrotal webs [2]. Maizels et al. described a The condition called “congenital megapre-
classification consisting of four categories based puce” (CMP) that was first reported by O’Brien
on the mechanism of concealment [5]: buried et al. [18], Gwin [19], and Philip [20] is now
penis (due to poor skin suspension in a child or included as a subgroup under grade I BP and
a prominent prepubic fat in an adolescent), characterized by intermittent ballooning of the
webbed penis (penoscrotal web), trapped penis genital area after micturition due to an abnormal
(the shaft of the penis is trapped in scarred pre- valve-like mechanism at the terminal end of the
pubic skin usually after circumcision), and prepuce [15]. This is in contrast to Summerton
micropenis (a normally formed penis that is less et al. who suggested that redundant inner prepuce
than 2 standard deviations below mean in is not present in buried penis [21].
670 A. T. Hadidi
a b
Fig. 45.4 (a–d) BP Grade I, referred to as “congenital unstretched) and the ballooned LIP covered by scrotal
megaprepuce (CMP).” (a) Notice the valve-like mecha- skin and abdominal wall skin. (c) After retraction of the
nism at the preputial orifice with ballooned long inner foreskin, observe that the inner mucosal prepuce is 4 cm
prepuce (LIP) and urine drops at the scrotum. (b) Note the long (stretched). (d) There is loose fascial attachment
very little outer prepuce and dorsal penile skin (7 mm (©Ahmed T. Hadidi 2022. All Rights Reserved)
A report of stretched penile length mea- 0–5 months, 4.3 ± 0.8 cm at 6–12 months, and
surements in a usable table has been compiled 4.7 ± 0.8 cm at 1–2 years. Donahoe [23] and
by Elder [22]. Measurements of penile length Radhakrishnan [24] reported that with the bur-
for age have been reported as 3.9 ± 0.8 cm at ied penis anomaly, the corporeal and glanular
a b c
d e
Fig. 45.5 (a–e) Apparent amputation of the penis during outer penile skin during circumcision (©Ahmed T. Hadidi
the circumcision of a buried penis: Exploration showed 2022. All Rights Reserved)
that the penis is intact but the surgeon excised most of the
size and development are normal, and the explanation may be that adult patients with BP
penis is of normal size. Redman observed in learn to live with the condition and do not seek
his study on 31 boys with a mean age of medical advice as does happen with many adults
12 months that the penile shaft itself is short with complicated hypospadias who learn to live
(mean 3.1 cm) [9]. Penile measurements in with the complication and lose hope of surgical
patients with a “visually deficient penis” have correction (personal communication).
been recommended. However, no other series Shenoy et al. presented a comprehensive
of children with a buried penis has included review of the different surgical techniques
these data [25]. reported in literature for the correction of BP [7].
Frank raised a provocative question in an edi- Several surgical options have been proposed for
torial comment on three studies describing the BP correction including multiple Z-plasties [27],
management of the buried penis: “Why do our suction lipectomy [28], fixing of the suprapubic
adult urologist colleagues not see this condition skin to the pubis to define the penile angle [5, 29],
in the older patient?” surmising that the condition preputial unfurling [23], and the use of preputial
corrects itself at puberty [26]. Possible explana- island flaps [11, 28]. Redman used the inner pre-
tions include that the adult urologists probably putial skin (mucosa) to cover the shaft of the
diagnose BP in adults as a congenitally short penis [30]. Frenkl et al. concluded that the num-
penis or as a hidden penis due to excess suprapu- ber of fixation sutures used did not affect the
bic fat or because the condition is very rare and is recurrence rate [8]. However, the surgical proce-
usually corrected during childhood. Another dure used to correct BP did not significantly alter
672 A. T. Hadidi
the patient long-term outcome [14]. Alexander only the inferior penoscrotal junction and
et al. described the ventral-V-plasty technique in the penis is not buried. There is no LIP. The
which they use a V-flap from the inner preputial surgical correction is different than BP and
mucosa to compensate for deficiency in the outer can be achieved by a V-shaped midline
preputial and penile skin to cover the penis [6]. incision that drops the scrotal skin down
The preservation of the entire internal prepu- away from the penis. Circumcision is not
tial layer, congenitally disposed around the entire necessary in this condition as shown in
shaft as described by Redman [9], was consid- Figs. 45.6 and 45.7.
ered risky by Ferro et al. [3] for fear of lymphatic Trapped penis is a term usually used to describe
stasis due to the deep fasciae dissection. The poorly designed circumcision in a child with a
author agrees with Ferro et al. and believes that BP and presents with a buried penis retracted
covering the penis with inner mucosal prepuce is behind the circumcision scar (Fig. 45.5).
cosmetically less satisfactory. Satisfactory results are obtained again through
Experience with hypospadias showed that a ventral midline incision if there is still ade-
ventral midline incision heals with minimal scar- quate penile skin left. Special care has to be
ring and usually looks like the median raphe nor- taken as in those children there is very limited
mally present on the ventral surface of the penis skin and preputial inner mucosa which may be
(personal communication). This is one major needed to cover the penis eventually.
advantage of using this midline incision in
BP. The other advantage is that the surgeon can One controversial issue in literature is the
tailor the amount of excess inner preputial layer ideal age for surgery. Shapiro stated that boys
to be excised according to the need in each indi- with a buried penis are conscious of the problem
vidual child. even before puberty and that with persistent con-
The following are the currently accepted terms cealment of the penis the child’s later psycho-
in literature [15]: logical development would be improved by
correcting the problem earlier rather than later
Buried penis is defined as “a rare congenital [29]. Herndon et al. showed in his long-term
anomaly characterized by the apparent study that the parents thought that surgery is
absence of the penis which exists when the almost uniformly successful in toddlers and less
penis lies buried beneath the integument of the often successful in adolescents [14]. Ferro et al.
abdomen, thigh or scrotum.” It is usually asso- [3] and Philip and Nicholas [20] recommended
ciated with an abnormally long inner prepuce correction as soon as the diagnosis is made, in
(LIP). Buried penis is a wide spectrum that order to resolve both the dysuria and the cosmetic
can be classified into three grades as men- anomaly. Shenoy and Rance asked the families of
tioned before. seven patients with BP and the parents preferred
Congenital megaprepuce (CMP) is a subgroup of to perform surgery at an age when they can dis-
grade I BP presenting with intermittent bal- cuss the problem with the child and before he
looning of the genital area. goes to secondary school [7]. The author prefers
Concealed (hidden or inconspicuous) penis (CP) to correct BP before the child goes to school to
is an acquired condition presenting later in avoid embarrassment by other children.
life due to abnormal excess fat accumulation Another controversial issue is “Should cir-
in the genital area. cumcision be performed as a routine during the
Webbed penis or penoscrotal web is a differ- surgical management of BP?” Based on the con-
ent entity from BP as the anomaly involves stant finding of LIP in all the cases of BP, we rou-
a b
c d
Fig. 45.6 (a–d) Webbed penis is due to abnormal penoscrotal junction. A V-shaped incision is made to bring the scro-
tum downward and is closed with a small Z-plasty (©Ahmed T. Hadidi 2022. All Rights Reserved)
tinely perform circumcision in every child who served in webbed penis which is not associated
requires surgery for BP to excise the excess inner with LIP and requires a different surgical
mucosa. In contrast, the foreskin can be pre- approach.
674 A. T. Hadidi
Fig. 45.7 Webbed penis: The condition can be corrected Z-plasty at the penoscrotal junction helps to have a normal
by a longitudinal incision along the length of the penis penoscrotal angle (©Ahmed T. Hadidi 2022. All Rights
making sure to leave adequate skin to cover the penis. A Reserved)
References 14. Herndon CDA, Casale AJ, Cain MP, Rink RC, Pope
J. Long-term outcome of the surgical treatment of con-
cealed penis. J Urol. 2003; https://doi.org/10.1097/01.
1. Keyes EL. Phimosis, paraphimosis, tumors of the
ju.0000083911.59937.c6.
penis. In: Urology. New York: Appleton; 1919.
15. Hadidi AT. Buried penis: classification surgi-
p. 649.
cal approach. J Pediatr Surg. 2014; https://doi.
2. Crawford BS. Buried penis. Br J Plast Surg.
org/10.1016/j.jpedsurg.2013.09.066.
1977;30:96–9.
3. Ferro F, Spagnoli A, Spyridakis I, Atzori P, Martini
L, Borsellino A. Surgical approach to the congenital
Pediatr Surg. 2012; https://doi.org/10.1016/j.
megaprepuce. J Plast Reconstr Aesthetic Surg. 2006;
jpedsurg.2012.06.027.
https://doi.org/10.1016/j.bjps.2005.12.033.
17. Hadidi AT. Perineal hypospadias: back to the future
4. Bergeson PS, Hopkin RJ, Bailey RB, McGill LC,
chordee excision & distal urethroplasty. J Pediatr Urol.
Piatt JP. The inconspicuous penis. Pediatrics.
2018; https://doi.org/10.1016/j.jpurol.2018.08.014.
1993;92:794–9.
18. O’brien A, Shapiro AMJ, Frank JD. Phimosis or con-
5. Maizels M, Zaontz M, Donovan J, Bushnick PN,
genital megaprepuce? Br J Urol. 1994; https://doi.
Firlit CF. Surgical correction of the buried penis:
org/10.1111/j.1464-410X.1994.tb07570.x.
description of a classification system and a tech-
19. Gwinn JL. Radiological case of the month. Arch
nique to correct the disorder. J Urol. 1986; https://doi.
Pediatr Adolesc Med. 1974;128:835.
org/10.1016/s0022-5347(17)44837-3.
20. Philip I, Nicholas J. Congenital giant prepucial sac:
6. Alexander A, Lorenzo AJ, Salle JLP, Rode
case reports. J Pediatr Surg. 1999;34:507–8.
H. The ventral V-plasty: a simple procedure for
21. Summerton DJ, Mcnally J, Denny AJ, Malone
the reconstruction of a congenital megaprepuce.
PSJ. Congenital megaprepuce: an emerging condi-
J Pediatr Surg. 2010; https://doi.org/10.1016/j.
tion—how to recognize and treat it. BJU Int. 2000;
jpedsurg.2010.03.033.
https://doi.org/10.1046/j.1464-410X.2000.00509.x.
7. Shenoy MU, Rance CH. Surgical correction of con-
22. Elder JS. Abnormalities of the genitalia in boys and
genital megaprepuce. Pediatr Surg Int. 1999; https://
their surgical management. In: Walsh PC, Retik AB,
doi.org/10.1007/s003830050683.
Vaughan EDJ, Wein AJ, editors. Campbell’s urology.
8. Frenkl TL, Agarwal S, Caldamone AA. Results
8th ed. Philadelphia: V. Saunders; 2002. p. 2334–52.
of a simplified technique for buried penis
23. Donahoe PK, Keating MA. Preputial unfurling to cor-
rect the buried penis. J Pediatr Surg. 1986; https://doi.
ju.0000107824.72182.95.
org/10.1016/0022-3468(86)90007-2.
9. Redman JF. Buried penis: a congenital syndrome
24. Radhakrishnan J, Razzaq A, Manickam K. Concealed
of a short penile shaft and a paucity of penile shaft
penis. Pediatr Surg Int. 2002;18:668–72.
skin. J Urol. 2005; https://doi.org/10.1097/01.
25. Bloom DA. Management of the concealed penis.
ju.0000154781.98966.33.
Dialogues Pediatr Urol. 1993;16:1.
10. Cromie WJ, Ritchey ML, Smith RC, Zagaja
26. Frank TD. Editorial comment. BJU Int. 2000;86:526.
GP. Anatomical alignment for the correction of bur-
27. Kubota Y, Ishii N, Watanabe H, Irisawa C, Nakada T,
ied penis. J Urol. 1998; https://doi.org/10.1016/
Chiba R, Fujioka T. Buried penis: a surgical repair.
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Urol Int. 1991; https://doi.org/10.1159/000281777.
11. Wollin M, Duffy PG, Malone PS, Ransley PG. Buried
28. Horton CE, Vorstman B, Teasley D, Winslow
penis. A novel approach. Br J Urol. 1990; https://doi.
B. Hidden penis release: adjunctive suprapu-
org/10.1111/j.1464-410X.1990.tb14667.x.
bic lipectomy. Ann Plast Surg. 1987; https://doi.
12. Boemers TML, De Jong TPVM. The surgical correc-
org/10.1097/00000637-198708000-00004.
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29. Shapiro SR. Surgical treatment of the
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“buried” penis. Urology. 1987; https://doi.
13. Casale AJ, Beck SD, Cain MP, Adams MC, Rink
org/10.1016/0090-4295(87)90435-3.
RC. Concealed penis in childhood: a spectrum of
30. Redman JF. A technique for the correction of peno-
etiology and treatment. J Urol. 1999; https://doi.
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org/10.1016/S0022-5347(01)68114-X.
S0022-5347(17)49008-2.
Penile Torsion (PT)
46
Ahmed T. Hadidi
Abbreviations with hypospadias or chordee. While some reports

describe it as a “rare condition” [5], many other
BP Buried penis reports consider it a rather common condition.
CPP Corporo-periosteo-pexy Sarkis and Sadasivan examined 387 “normal”
DDF Dorsal dartos flap male infants who were referred for routine reli-
DYG Double Y-Glanulomeatoplasty gious circumcision and found 27% had isolated
LABO Lateral-based onlay flap PT after excluding associated anomalies such as
PT Penile torsion hypospadias, chordee, or major medical condi-
SLAM Slit-like adjusted Mathieu tions [3]. The condition is even more common if
we examine patients with hypospadias only. Abu
Zeid and Soliman reported the presence of PT in
32% of patients with distal hypospadias while
46.1 Introduction none of their patients with proximal hypospadias
had penile torsion [6].
Congenital penile torsion (PT) or rotation is the
twist of the penis along its longitudinal axis [1].
It is a common anomaly that may occur alone or 46.2 Etiology
in association with chordee and/or hypospadias
[2]. It is counterclockwise (to the left) in more Several authors have suggested that the underly-
than 95% of cases [3]. ing cause of congenital penile torsion is abnor-
Although the first patient of PT was reported mal skin and dartos fascia attachment [4, 5, 7].
with hypospadias in 1857, Azmy and Eckstein This is supported by the high incidence of penile
were probably the first to publish a systematic torsion with distal hypospadias (32%), while it is
study on the condition [4]. The true incidence of rare in proximal hypospadias cases, where the
PT is unknown probably because many patients ventral skin is completely absent [6]. Others sug-
with mild forms pass unnoticed unless combined gested fibrosis of Buck’s fascia [8] or asymmetric
development of Buck’s fascia, corpus caverno-
sum, and tunica albuginea [4, 5, 9, 10]. This is
because in some cases skin degloving and
A. T. Hadidi (*) realignment is not enough to correct the anomaly.
Asymmetrical development of the corpora caver-
Teaching Hospital of the Johann Wolfgang Goethe nosa around the longitudinal axis of the penis
University, Offenbach, Frankfurt, Germany and/or a fibrous band that tightly attaches the left
678 A. T. Hadidi
corpus cavernosum to the pubic periosteum nization and hypoplasia of the ventral penile
might be another cause of persistent penile tor- structures that result in hypospadias and chordee
sion as described by Zhou et al. [5], who related (Fig. 46.2). If the disorganization is asymmetric,
the penile torsion to an intrinsic longitudinal dis- e.g., more hypoplastic on the right side, the glans
orientation of the corpora cavernosa around the will rotate toward the more developed side, i.e.,
longitudinal axis of the penile shaft. Bauer and to the left (Fig. 46.3). Several papers have
Kogan related penile torsion to fibrosis of Buck’s already shown that the ventral penile structures
fascia, but their finding lacked histological evi- distal to the urethral meatus are hypoplastic and
dence [8]. Sarkis and Sadasivam reported that have different characteristics and proteins than
penile torsion was counterclockwise in 99% of the normal proximal penile structures [14].
affected infants [3]. This phenomenon is not fully
understood [1].
To understand the mechanism of PT, we need 46.3 Classification of Penile Torsion
to consider the normal development of the human
urethra and pathogenesis of hypospadias. According to the degree of rotation, penile tor-
According to the classic fusion theory, the human sion may be classified into three grades:
urethra develops by fusion of the lateral edges of
the urethral plate from lateral to medial and • Grade I with rotation angle <45°
hypospadias is due to failure of fusion [11]. This • Grade II with angle of rotation between 45°
theory does not explain the presence of chordee and 90°
nor PT. • Grade III with rotation more than 90°
Fleishmann [12] and Politzer [13] contra-
dicted the fusion theory as they could not find This is also related to the technique most suit-
evidence of fusion in human embryos. Van der able for surgical correction.
Putte showed in his excellent work on human
embryos the importance of the penile stroma in
the development of cloaca, penis, and urethra 46.4 urgical Correction of Penile
S
[14]. Torsion
According to the “migration hypothesis” [15],
there is differential growth of the penis, first dor- Several techniques have been described to cor-
sally causing “physiologic chordee.” This is fol- rect penile torsion, but none of them has gained
lowed later by distal migration and growth of the consensus as an optimal, ideal, or versatile tech-
ventral penile structures including the urethra to nique. Those techniques vary from simple skin
reach the tip of the penis (Fig. 46.1). degloving and realignment [4], dorsal dartos flap
According to the “disorganization hypothe- (DDF) [2], to more complex techniques involving
sis” (see Chap. 7), hypospadias is due to disorga- the corporeal tissue such as total urethral
Fig. 46.1 The “migration hypothesis” showing the distal migration of the penile ventral structures (©Ahmed T. Hadidi
46 Penile Torsion (PT) 679
a b
Fig. 46.2 (a–c) (a) Sagittal section in 11 weeks gestation fetus of 24 weeks. The ventral penile structures are pres-
male embryo. (b) Transverse section through the proximal ent but in an asymmetrical configuration with respect to
shaft of an 18.5-week-old fetus with proximal penile the midline (©Ahmed T. Hadidi 2022. All Rights
hypospadias, ventral curvature, and torsion. (c) Normal Reserved)
mobilization [16], suturing the tunica albuginea hypospadias correction. Children with glanular
to the periosteum of the pubis (corporopexy) [5], hypospadias underwent the double-Y-
or diagonal corporeal plication sutures parallel glanulomeatoplasty (DYG, see Chap. 19) [19],
to and away from the neurovascular bundle [17, with distal hypospadias underwent the slit-like
18]. However, most of these studies report short- adjusted Mathieu (SLAM, see Chap. 22) [20],
term results. and the children with proximal hypospadias
Herein, we present the protocol of manage- underwent the lateral-based onlay flap technique
ment of PT in the Hypospadias Center in (LABO, Chap. 30) [21].
Frankfurt, Germany, and experience with the Among the 73 patients, there were only two
correction of PT in 73 patients (between 2010 patients with clockwise PT (to the right). Isolated
and 2018) and the midterm follow-up. PT was operated on only when the rotation angle
All children with hypospadias underwent the was more than 90° or grade III PT (3 children).
standard protocol of ventral degloving and mobi- However, extensive degloving was not enough in
lization of glanular wings as the first step of any of the patients included in the study.
680 A. T. Hadidi
Fig. 46.3 Asymmetric distal migration of the ventral ventral tissues (i.e., the arrested distal migration (here
penile structures will result in torsion. The penis will twist right) acting as a “horse rein” (©Ahmed T. Hadidi 2022.
toward the more developed side (here left) as if it is pulled All Rights Reserved)
proximally and downward through the more hypoplastic
46.4.1 Grade I Penile Torsion (<45°) approach for grade I PT (29 patients associated
with hypospadias or chordee).
There is a general agreement that mild forms of
isolated PT do not need surgical correction [1].
However, if the child needs surgery for an associ- 46.4.2 G
rade II Penile Torsion
ated hypospadias or chordee, PT should be cor- (Between 45° and 90°)
rected during surgery. Also, there is a general
agreement that skin degloving and realignment as Some authors report satisfactory results with
described by Azmy and Eckstein are usually degloving and realignment with grade II PT
enough for grade I PT (<45°) [4]. This was our (angle >45° but <90°). Tryfonas and colleagues
reported satisfactory results applying this with grade III PT in the Hypospadias Center in
technique to more severe degrees of penile torsion Frankfurt. However, two of the three patients came
but with suturing the skin in an overcorrected back within 6 months with recurrent PT.
position [22]. Marret et al. reported satisfactory Zhou et al. suggested deep dissection and
results with simple degloving and skin realign- suturing of the affected corpus cavernosum to the
ment in patients with rotation less than 90° [23]. periosteum of the pubis or corpora-pexy [5].
This did not work well in our hands. Some authors were concerned that this technique
In 2004, Fisher and Park described a tech- may carry some risk to the neurovascular bundles
nique utilizing the dorsal dartos flap (DDF) rota- when placing the stitches on the corpora. In a
tion to correct penile torsion [2]. They reported prospective study, Aldaqadossi et al. compared
100% success of this technique; however, 64% the DDF technique with corporopexy [9]. The
achieved complete resolution of penile torsion in results were good and similar in both techniques,
the series of Bauer and Kogan in 2009 [8]. Marret although the authors concluded that the Fisher
et al. used the DDF technique for patients with technique was easier and with no risk to the neu-
rotation more than 90° and reported satisfactory rovascular bundles as may occur in corporopexy,
results [23]. El Darawany et al. suggested that in which all the stitches are placed on the ventral
failure of penile torsion correction by the DDF part of the affected corpora to the periosteum.
flap or its recurrence might be explained by the Elbatarny and Ismail proposed a staged approach
flap becoming unattached or being inadequately starting with degloving and acknowledging the
placed from the start [1]. The DDF technique is fact that skin realignment can only correct about
the standard technique for management of grade 30° of rotation [24]. They proposed to perform
II PT in the Hypospadias Center in Frankfurt DDF if rotation >30° persisted after degloving
with satisfactory results (34 patients) (Fig. 46.4). and to use corporopexy if rotation >30° persisted
after DDF flap. El Darawany et al. started all
cases in their study by degloving and skin realign-
46.4.3 Grade III Penile Torsion (>90°) ment, without or with skin overcorrection [1]. If
that was not adequate, they shifted to DDF and
Marret et al. used the DDF technique for patients they reported using this approach, they could, in
with rotation more than 90° and reported satisfac- their hands, correct all grades of PT including
tory results [23]. The DDF was used in three patients rotation of 180°. They suggested that extensive
Fig. 46.4 The dorsal dartos flap (DDF) for grade II PT (rotation 80° after degloving) (©Ahmed T. Hadidi 2022. All
Rights Reserved)
682 A. T. Hadidi
procedures such as corporopexy or corporeal pli- tudinally, parallel to neurovascular bundle. We

cation were not needed. have also used urethral mobilization to reduce/
In the Hypospadias Center we get referred correct mild forms of chordee and distal hypo-
many cases with grade II and III (or severe) bur- spadias. We have observed that extended urethral
ied penis (BP) that require fixation of the corpora mobilization heals with severe fibrosis and did
cavernosum at the root of the penis to the not give satisfactory results in our hand.
periosteum and skin or corporo-periosteo-pexy The CPP technique was used in seven children
(CPP) as shown in Fig. 46.5. We have very satis- with grade III PT and two children with recurrent
factory results and there was no incidence of PT after DDF with satisfactory results and 4 years
trauma to the neurovascular bundle as the Prolene mean follow-up period. We no longer perform
3/0 sutures are placed at 12 and 9 o’clock longi- the DDF technique for grade III PT.
Fig. 46.5 Diagram to show the technique of corporo- o’clock and a second one at 9 o’clock (©Ahmed T. Hadidi
periosteo-pexy (CPP) for grade III PT (rotation 110° after 2022. All Rights Reserved)
degloving). Two stitches of Prolene 3/0 are used; one at 12
Diagonal plication technique has been orientation. Seven patients with grade II and III
described by some authors [17, 18] who believed had persistent mild rotation (<20°) and required
that diagonal corporal plication suture is much no further management. Two patients with grade
easier to perform than suturing the corpora to III PT who underwent DDF developed recurrent
the periosteum of the pubis. However, long-term rotation. These two children underwent CPP cor-
follow-up is still awaited. Slawin and Nagler rection 1 year later.
described the technique of angular tunica exci-
sion in the direction that can counter rotate the
corpora cavernosa [25]. However, these tech- References
niques did not gain wide acceptance due to the
potential risk of injury to the erectile tissue and 1. El Darawany HM, Al Damhogy ME, Kandil MS,
Elkordi ME, Nagla SA, Taha MR. Procedures
neurovascular bundles. Moreover, these tech- used for correction of isolated penile torsion:
niques might not be effective in severe penile are they competitive or complementary? Int
torsion. Bhat et al. suggested extended urethral Urol Nephrol. 2019; https://doi.org/10.1007/
mobilization as a technique to correct PT [16]. s11255-019-02163-9.
2. Fisher PC, Park JM. Penile torsion repair using dor-
They reported that they could correct “almost sal dartos flap rotation. J Urol. 2004; https://doi.
all cases of PT” (including patients with 180°) org/10.1097/01.ju.0000120148.79867.5c.
by extended urethral mobilization from the 3. Sarkis PE, Sadasivam M. Incidence and predic-
perineum up to the glans. Elbakry et al. on the tive factors of isolated neonatal penile glanular tor-
sion. J Pediatr Urol. 2007; https://doi.org/10.1016/j.
other hand reported their experience in 19 jpurol.2007.03.002.
patients with moderate and severe PT [26]. They 4. Azmy A, Eckstein HB. Surgical correction of torsion
observed that urethral mobilization could only of the penis. Br J Urol. 1981; https://doi.org/10.1111/
reduce the angle of rotation but could not cor- j.1464-410X.1981.tb03202.x.
5. Zhou L, Mei H, Hwang AH, Xie HW, Hardy
rect moderate and severe PT. They concluded BE. Penile torsion repair by suturing tunica albuginea
that corporo-periosteo-pexy might be the most to the pubic periosteum. J Pediatr Surg. 2006; https://
reliable maneuver for complete correction of doi.org/10.1016/j.jpedsurg.2005.10.065.
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looked anomaly with distal hypospadias. Ann Pediatr
All the patients in the Hypospadias Center in Surg. 2010;6(2):93–7.
Frankfurt underwent circumcision at the end of 7. Culp OS. Struggles and triumphs with hypospa-
the procedure. All children had transurethral dias and associated anomalies: review of 400
catheter for 2 days except for the two patients cases. J Urol. 1966; https://doi.org/10.1016/
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with proximal hypospadias who had the catheter 8. Bauer R, Kogan BA. Modern technique for penile
for 7 days. All patients were followed according torsion repair. J Urol. 2009; https://doi.org/10.1016/j.
to the standard hypospadias protocol, i.e., post- juro.2009.02.133.
operative examination after 3 months, 1 year, 9. Aldaqadossi HA, Elgamal SA, Seif Elnasr MK. Dorsal
dartos flap rotation versus suturing tunica albuginea to
3 years, and then every 5 years until the child is the pubic periosteum for correction of penile torsion:
15 years old. a prospective randomized study. J Pediatr Urol. 2013;
10. Corriere JN. Involvement of Buck’s fascia in con-
genital torsion of the penis. J Urol. 1981; https://doi.
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11. Baskin L. Basic science of the genitalia. In: The
The follow-up period of the 73 patients in the Kelalis—King—Belman textbook of clinical pedi-
Hypospadias Center ranged from 22 to 96 months atric urology. 5th ed; 2006. https://doi.org/10.1201/
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15. Hadidi AT, Roessler J, Coerdt W. Development of S, Violaki A, Limas C. Torsion of the penis—a com-
the human male urethra: a histochemical study on parative study between two operative procedures of
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untwist with plication. Urology. 2002; https://doi. tal penile curvature with penile torsion: a new
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Congenital Urethral Duplication
47
Grahame H. H. Smith
Abbreviations 47.2 Classification

and Terminology
ASTRA Anterior sagittal transrectal approach
PADUA Progressive augmentation by dilating A large variety of urethral duplications have been
the urethra anterior described. The fact that the condition is rare and
that the anomalies appear in an almost unlimited
variety of anatomical variants makes a classifica-
tion system a challenge.
47.1 Introduction The commonest used classification system is
that proposed by Effmann in 1976 [5] and it has
Congenital duplication of the urethra is rare stood the test of time. In his original article, he
with 300 cases reported in the English literature described his classification system in text.
by 2008 [1] and a similar number since then. Subsequent authors then illustrated his classifica-
Most cases occur in males, but the condition tion system. A revised illustration of the Effmann
has been reported also in females [2–4]. Reports classification system is presented in Fig. 47.1. Other
of urethral triplication have also been made. classification systems have been proposed [6–8].
Case reports and case series are likely biased Duplications of the urethra can occur dorsally
toward cases treated operatively and to isolated and ventrally or collaterally. They may be isolated,
cases of urethral duplication. The author’s sur- associated with caudal duplications, vertebral
gical specialty training biases the terminology defects, anal atresia, cardiac defects, tracheo-
used. esophageal fistula, renal anomalies (VACTERL),
and limb abnormalities anomalies (including
imperforate anus), or exstrophy and epispadias.
Effmann initially classified duplications into
three main types [5]:
Type Ia is a blind ending dorsal channel, with
a normal ventral urethra. Type Ib opens from the
urethral channel and ends blindly. Type IIa1 has
G. H. H. Smith (*)
Department of Urology, The Children’s Hospital at two separate urethras and in Type IIa2, a second
Westmead, Westmead, NSW, Australia channel arises from the first and courses inde-
Children’s Hospital at Westmead Clinical School, pendently to a second meatus. In Type IIb, two
The University of Sydney, Sydney, NSW, Australia urethras arise from the bladder and join to form
e-mail: Grahame.smith@health.nsw.gov.au a single distal urethra. In Type III, urethral
686 G. H. H. Smith
Ia Ib
IIa1 IIa2 IIa2Y IIb
IIIa IIIb
Fig. 47.1 Effmann Classification, modified. In Type I, (Ia are connected to a single urinary bladder. In Type III (IIIa
and Ib), there is a blind-ended dorsal channel. In Type II and IIIb), there are two separate urethrae ending in two sepa-
(IIa1, IIa2, IIa2Y, IIb), there are two separate urethrae that rate urinary bladders (Courtesy of Ahmed T. Hadidi)
duplications occur as a component of a partial IIIb duplications in a sagittal plane; the latter are
or complete collateral or sagittal duplication. It very rare.
would make sense to separate Type III duplica- The terminology to describe duplications of the
tions into IIIa collateral duplications fitting into urethra versus duplications of the bladder can be
the spectrum of caudal duplication and Type confusing. Sagittal and coronal planes are illustrated
47 Congenital Urethral Duplication 687
in Fig. 47.2. Duplications of the urethra most com- plane. To clarify, using the Abrahamson classifica-
monly occur in the sagittal plane [9], with the more tion, a sagittal duplication of the bladder is two blad-
normal urethra lying ventrally and the abnormal ure- ders sitting side by side and a coronal duplication of
thra lying dorsally. When urethral duplications occur the bladder would be one bladder sitting in front of
side by side, they can be associated with duplica- the other. The terminology ventral and dorsal dupli-
tions of the bladder, the hindgut and/or the caudal cations and collateral duplications is less confusing.
spinal cord. These types of duplications fit into the In ventral and dorsal duplications, the ventral
spectrum of caudal duplication [10]. Bladder dupli- urethra is usually the more normal urethra, both
cations are classified using the Abrahamson classifi- in terms of the caliber and in terms of being asso-
cation [11], as being duplications of the coronal or ciated with a competent sphincter mechanism.
sagittal plane rather than in a coronal or sagittal Urethroanal or perianal accessory urethras can
Fig. 47.2 For anatomical description, the body is classi- (2013) Body Planes. In: Anatomy and Physiology, https://
fied into: sagittal, coronal, and transverse planes (from o p e n s t a x . o rg / b o o k s / a n a t o m y -a n d -p h y s i o l o g y /
OpenStax: Betts JG, Young KA, Wise JA, Johnson E, Poe pages/1-6-anatomical-terminology)
B, Kruse DH, Korol O, Johnson JE, Womble M, DeSaix P
688 G. H. H. Smith
occur and can sometimes break this rule of being are thought to result from faulty fusion of the ure-
the dominant urethra but they are very rare [12]. thral folds [17] or persistence of the embryologi-
Sometimes both channels are abnormal. cal urogenital plate [18]. Paired lateral
Urethral duplications can be accompanied by mesodermal bud fusion may be delayed, leading
duplications of the penis and by duplications of to endoderm capable of forming a urethra both
the bladder, hindgut, and spine. These are usually, above and below the fused genital tubercle [5].
but not always, collateral duplications. These Type Ia cases with a channel opening on the
duplications can be associated with epispadias dorsum of the penis are often associated with a
and exstrophy, sometimes with one duplication widened pubic symphysis and are thought to be
associated with an open bladder and the other part of the exstrophy epispadias spectrum. As
duplication associated with a closed bladder. such a possible etiology is the failure of midline
Ventral and dorsal duplications of the urethra mesodermal fusion [5].
are usually not associated with other anatomical Type IIa2 duplications arise as a result of
abnormalities, such as duplication of the phallus incomplete separation of the cloacal membrane
or the bladder but isolated case reports of such into anal and urogenital membranes [5].
duplications do exist [13]. Side by side duplications of the bladder are
Side by side duplications are thought to be a more common than ventral and dorsal duplica-
result of incomplete twinning and can be associ- tions. They are thought to be the result of partial
ated with duplications of the bladder and the twinning.
phallus. Boissonnat stressed the importance of
separating isolated urethral duplications from
duplications of the urethra associated with dupli- 47.4 Presentation
cations of the bladder and penis [14].
Type IIa2 duplications are also termed Y type A significant number of patients are asymptom-
duplications or H type ano-urethral fistula [12]. atic and do not require treatment for their urethral
They are associated with an imperforate anus up to duplication.
30% of the time. In most cases the penile urethra is Patients can present with an extra opening on
hypoplastic and the ventral urethra that courses to the glans or on the shaft of the penis (Fig. 47.3)
the anus or rectum is a normal caliber and is sur- usually noted at the time of a request circumci-
rounded by a competent sphincter mechanism. sion or when assessing concerns about the fore-
Rarely the ventral component is a fistula and the skin. The differential diagnosis in these patients
dorsal component a normal urethra [1, 15]. The includes hypospadias. In glanular hypospadias,
anomaly may then be described as a congenital there is often a blind ending pit just distal to the
urethroperineal fistula. Variations of the Type urethral meatus. The appearance can suggest the
IIa2 in a Y configuration but with a complete ure- possibility of a urethral duplication, but in the
thral duplication have also been reported [16]. It is great majority of cases this is not the case and the
likely that the terminology used to describe these pit ends blindly after a few millimeters.
cases depends on the specialty of the treating sur- Often in distal hypospadias, there is a small
geon; colorectal surgeons use colorectal terminol- accessory urethra opening distal to the meatus
ogy and urologists use urological terminology. that usually ends blindly after 1 or 2 cm
(Fig. 47.4). By convention these accessory, ure-
thras are not usually included as part of the spec-
47.3 Embryology trum of urethral duplications.
Patients can present with a history of voiding
Not a lot of progress has been made in working with two streams. They can present with urinary
out the embryological etiology of urethra dupli- incontinence, usually stress-type incontinence
cation since Effmann wrote his original paper. associated with an incompetent bladder neck
Ventral and dorsal duplications of the urethra are associated with the accessory urethra. They can
more common than collateral duplications. They present after a urinary tract infection, with a
duplication diagnosed on a voiding cystourethro-

gram. Occasionally an accessory urethra can
become infected and present with a discharge.
Urinary obstruction can occur. Presentation can
be delayed until adult life [19, 20].
Type I duplications are usually an isolated
abnormality.
Type IIa2Y duplications are associated with
upper tract abnormities and with abnormalities in
the VACTERL association. Type III duplications
are associated with colonic duplications and
duplications of the distal spinal column.
Fig. 47.3 Type Ia urethral duplication: There is a blind-

ended opening dorsal to the normal urethra
a b
Fig. 47.4 (a–g) Accessory urethra associated with hypo- long. (e, f) A third patient with accessory glanular urethra
spadias: It is common to find two (a) or three (b) openings and a coronal true urethra. (g) The most proximal (ven-
with hypospadias (due to abnormal vacuolation). (c, d) tral) opening is the normal urethral meatus leading to the
Another patient with accessory urethra that was 0.5 cm bladder (Courtesy of Ahmed T. Hadidi)
690 G. H. H. Smith
c d
e f

47.5 Examination Findings prior to operative correction. The more normal

urethra will have an external sphincter mecha-
For complete urethral duplications and for distal nism and a verumontanum will be visible distal
incomplete duplications, there will be two ure- to the bladder neck.
thral openings. Proximal Y-type duplications are
associated with anorectal abnormities, for exam-
ple, imperforate anus. 47.7 Treatment
Only symptomatic urethral duplications require

47.6 Investigation treatment. Treatment needs to be tailored to
resolving the presenting symptom, for example,
Digital photography is ideal for documenting resolving an accessory urinary stream, correcting
external abnormalities. A combined retrograde incontinence, correcting a discharge, or prevent-
and antegrade urethrogram is a good way to doc- ing infection.
ument the urethral anatomy and to locate the Circumcision should be avoided in children
accessory urethra (Fig. 47.5). Usually both are with a suspected duplication. The foreskin can be
required. A renal and bladder ultrasound is useful for reconstruction.
important, to document a normal bladder and to As a general principle, the ventrally located
exclude upper tract abnormalities. Upper tract urethra is the more normal urethra, containing an
abnormalities are more commonly seen with external sphincter mechanism. It should be pre-
Y-type duplications and in caudal duplications. served. A preliminary cystoscopy should be
MRI scanning is sometimes used, with the undertaken in all cases.
penis taped to the abdomen. Similarly, CT scan- Dorsal accessory urethras (Type Ia or IIa) can
ning has been used in the past. MRI scanning has be excised back to the level of the pubic tubercle.
largely replaced CT scanning since there is no It is not necessary to completely excise the ure-
ionizing radiation with an MRI. thra back to the bladder or bladder neck. These
An examination under anesthetic associated duplications often end blindly before reaching
with urethral calibration, urethroscopy, and cys- the bladder. It is possible to strip out the acces-
toscopy can be helpful in assessing the anatomy sory urethra, in a similar fashion to stripping a
Fig. 47.5 Type IIa1 urethral duplication: The child has two urethrae, both ending in the bladder. (Courtesy of Ahmed
T. Hadidi)
692 G. H. H. Smith
varicose vein [21, 22]. The accessory urethra can the time of anorectoplasty. The patient is placed
be defined with methylene blue or by catheteriz- in a knee-chest position and the rectum is
ing it with a ureteric catheter. It can then be dis- packed with ribbon gauze soaked in betadine. A
sected out from distal to proximal until it ends midline incision is made in the perineum, from
blindly or until the dissection reaches the pubic the anterior anus forward and deepened through
bone, whichever occurs first (Figs. 47.6, 47.7). the perineal body. The anterior wall of the anus
Type Ib duplications are very rare. They fork and rectum can then be seen and the urethra dis-
away from the proximal urethra in the region of sected away. If necessary, the anterior anus and
the bulbar urethra. They travel in a proximal rectum can be opened longitudinally and then
direction. They can be treated in a similar fashion subsequently repaired in layers. A muscle stim-
to a Cowper’s syringocele by endoscopically lay- ulator is useful to make sure the sphincter is
ing the accessory urethra open into the ventral aligned as it is repaired; however the bulk of the
urethra. A resectoscope with a cold knife or with anal sphincter sits posteriorly. The distal ure-
a diathermy knife and cutting current can be thra can then be reconstructed using the same
used. techniques used to reconstruct proximal hypo-
Complete and distal Type II urethral duplica- spadias. Techniques used include a one- or two-
tions can be anastomosed side to side using distal stage foreskin or buccal urethroplasty or a Q
hypospadias repair techniques. If the two urethral flap, or the urethra can be left as a perineal ure-
openings are close together then a simple mea- throstomy [25]. The reason for the latter
totomy may be sufficient to correct problems approach is that attempts at urethral reconstruc-
with the stream [23]. Otherwise the shaft skin of tion are not always successful and often require
the penis is mobilized via a circumferential inci- multiple procedures [26].
sion. The urethras can both be catheterized using Attempts to serially dilate an atrophic dorsal
feeding tubes. The dorsal urethra is then mobi- urethra “progressive augmentation by dilating
lized from distal to proximal over a distance of the urethra anterior” (PADUA) [27] for recon-
2 cm. The ventral urethra is also mobilized along struction are not recommended [26]. This tech-
its dorsal surface. It is sometimes necessary to nique was developed to treat urethra atresia. A
separate the corporal bodies in order to join the guide wire is passed through the atretic urethra
urethras. The two urethras can be joined dorsal and a ureteric JJ stent or ureteric catheter is
urethra end to ventral urethra side or vice versa, passed over the guide wire and secured in place
depending on the quality and location of the dis- for 5–7 days. The catheter is then wired and
tal and ventral urethra. replaced with the next largest size and the pro-
Proximal incomplete urethral duplications cess repeated until a urethra of sufficient caliber
(IIb) are usually asymptomatic and can be left is obtained. In rare situations, where the dorsal
unoperated. urethral is dominant, the ventral fistulous tract
Y-type or H-type duplications with a urethral can be excised using an ASTRA approach.
connection to anus or rectum (Fig. 47.8) are often Collateral urethral duplications associated
associated with anorectal abnormities, and treat- with complete or partial bladder duplication are
ment of both the anorectal and urethral abnor- often associated with other abnormalities, for
mality is required. It is important to determine example, penile duplication, rectal or colonic
which urethra is functional, the dorsal urethra or duplications, and/or spinal duplications. As well
the urethra connecting to the anus; however in as a caudal duplication there may be an associ-
most cases, it will be the latter. ated bladder exstrophy or cloacal exstrophy.
If the ventral urethra is dominant, it can be Upper tract renal abnormalities often coexist. A
disconnected from the anus and/or rectum and duplicated bladder can be joined to its counter-
brought to the perineum using an “anterior sag- part or excised. If two bladder necks exist then
ittal transrectal approach” (ASTRA) [25] or at the less functional one can be closed. Similarly, a
a b c
d e f
Fig. 47.6 (a–g) A child with Type IIa1 urethral duplica- to the pubic bone and ligated there. There is no need to
tion: The accessory dorsal urethra was connected to the continue dissection to the bladder wall (Courtesy of
bladder. The accessory urethra was dissected proximally Ahmed T. Hadidi)
694 G. H. H. Smith
Fig. 47.7 A child with Type IIa1 urethral duplication retracted, no scar is seen on the dorsum of the penis
(same patient as in Fig. 47.6): The appearance of the penis (Courtesy of Ahmed T. Hadidi)
after complete healing. As the penile skin was completely
a b
Fig. 47.8 (a–b). Y type urethral duplications: This group (Modified by G HH Smith and Reprinted with permission
of patients include two categories: (a) Rectourethral from Springer Nature: „Pediatric Surgery“, Editors: Puri
Fistula with a dominant ventral urethra passing to the anus P., Höllwarth M., Chapter 27, “Anorectal Anomalies” by
(b) Rectourethral Fistula with a dominant dorsal urethra Pena A., Levitt M. A, p293, ©2006 [24])
duplicated penis or urethra may be joined to its 12. Lorenz C, Zahn K, Schäfer FM, Möller K, Stehr M,
Stein R. Congenital Y-urethra—a diagnostic and ther-
counterpart or excised [13, 28]. apeutic challenge. J Pediatr Urol. 2020; https://doi.
Treatment of Y-type and collateral urethral org/10.1016/j.jpurol.2020.11.032.
duplications often requires the involvement of a 13. Coker AM, Allshouse MJ, Koyle MA. Complete
multidisciplinary team, with expertise in duplication of bladder and urethra in a sagittal plane
in a male infant: case report and literature review.
hypospadias repair, bladder surgery, and anorec- J Pediatr Urol. 2008; https://doi.org/10.1016/j.
tal abnormalities. jpurol.2008.02.001.
More publications with long-term follow-up 14. Boissonnat P. Two cases of complete double func-
of Y duplications and caudal duplication patients tional urethra with a single bladder. Br J Urol. 1961;
https://doi.org/10.1111/j.1464-410X.1961.tb11642.x.
would be helpful. 15. Dayanc M, Irkilata HC, Kibar Y, Bozkurt Y, Basal S,
Xhafa A. Y-type urethral duplication presented with
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Enlarged Prostatic Utricle
Associated to Hypospadias 48
Mario Lima and Michela Maffi
Abbreviations Utricular anomalies may result from incomplete

regression of the Müllerian system or incomplete
AMH Anti-Müllerian hormone androgen-mediated regression and closure of
MRI Magnetic resonance imaging urogenital sinus due to altered production or sen-
PU Prostatic utricle sitivity to testosterone or AMH [5–7].
TRUS Transrectal ultrasound This explains why prostatic utricle can be associ-
US Ultrasounds ated to other anomalies such as hypospadias, crypt-
VCUG Voiding cysto-urethrogram orchidism, renal agenesis, and intersex disorders [1,
2, 8], while only rarely is an isolated condition.
This anomaly can be classified as grade 0–III
[9]. Grade 0 is limited to verumontanum, grade I
48.1 Introduction is below the bladder neck, grade II is extended
over the bladder neck, and grade III is the open-
Prostatic utricle (PU) is an enlarged diverticulum ing distal to the external sphincter.
localized in the posterior urethra and derived by Most of PUs are asymptomatic but they can
the persistence of Müllerian structures or also be manifested by urinary infections, urinary
decreased androgenic stimulation of the urogeni- retention, epididymitis, stone formation, and
tal sinus. It is a rare condition with an estimated postvoid dribbling. These symptoms are usually
prevalence of 5% in urologic patients and an inci- related to the compressive effect of the PU on
dence of 1% in autopsy [1, 2]. surrounding structures [10].
In males, secretion of anti-Müllerian hormone
(AMH) by fetal testes causes regression of Müllerian
structures. The only remnants of this system are the 48.2 Diagnosis
appendix testis and the prostatic utricle.
PU has a dual histologic origin that consists of Ultrasounds are the first-line imaging method for
an admixture of urogenital sinus and Wolffian PU providing diagnosis in most cases (Fig. 48.1a).
cells caudally and Müllerian cells cranially [3, 4]. VCUG also allows to see the utricular chamber
filled with contrast medium and can be useful to
detect the confluence between urethra and PU
M. Lima (*) · M. Maffi and its extension (Fig. 48.1b). Small utricles can
Pediatric Surgery Department, IRCCS Sant’Orsola
Polyclinic, Alma Mater Studiorum - University of
be missed at ultrasounds and also at VCUG but
Bologna, Bologna, Italy are usually low-grade asymptomatic PU not
e-mail: mario.lima@unibo.it requiring surgical treatment. After first-line diag-
698 M. Lima and M. Maffi
a b c
Fig. 48.1 (a–c) (a) US is the first-line imaging technique medium and it is possible to identify the communication
to diagnose a PU. PU appears as a fluid-filled cavity with the urethra. (c) MRI is a third-line imaging technique
behind the bladder (B Bladder, U Utricle). (b) During that allows a better anatomic definition of the
voiding phase of a VCUG, the PU is filled with contrast malformation
nosis or in case of strong suspicion of PU, we Endoscopic techniques include catheteriza-

recommend MRI which provides a better ana- tion, aspiration, and utricle orifice incision [1, 13]
tomic definition of the PU and the surrounding or, as described by Husmann and Allen [14],
pelvic structures (Fig. 48.1c). Some authors men- electrofulguration of the mucosal layer, allowing
tioned the use of retrograde urethrogram, com- coalescence of the deeper raw tissues to obliter-
puted tomography, and also intravenous ate the cavity.
pyelogram as diagnostic tools [1, 10, 11], but Several open techniques have been described,
radiation exposure should be considered and but the anatomic site of PU remains challenging
avoided when possible or limited in pediatric because of the high risk of injury to the surround-
patients. Since imaging techniques are sensitive ing structures such as ejaculatory ducts, pelvic
enough in diagnosing a PU, urethroscopy is not nerves, rectum, vas deferent, and ureters.
recommended as a routine investigation; however The open approaches reported are extraperito-
in selected cases, it is indicated to detect the neal or transperitoneal, suprapubical extravesi-
coexistence of a uterine cervix [9]. cal, transvesical transtrigonal, perineal, posterior
trans-sacral, parasacral, posterior sagittal tran-
srectal or pararectal, and anterior sagittal with or
48.3 Treatment without rectal splitting [1, 3, 5, 15–20].
The most frequently used approaches will be
Nonsurgical approach can be attempted in case of described below.
UTI associated to PU using antimicrobial treat-
ments but definitive treatment requires an endo-
scopic or surgical approach. Usually, symptomatic 48.4 Open Techniques
cases, grade III or grade II prostatic utricles but
of conspicuous size, are considered an indication Transvesical transtrigonal approach: the anterior
for surgery. Some authors report transrectal ultra- bladder wall is opened longitudinally exposing
sound (TRUS)-guided aspiration with or without the trigone. A midline incision is done in the tri-
injection of sclerosant therapy as an alternative gone revealing the PU behind the bladder. The
[1, 12]. PU is isolated till its confluence with the urethra
48 Enlarged Prostatic Utricle Associated to Hypospadias 699
and its neck is ligated at insertion point. A supra- deepened in the perineal body remaining pre-
pubic tube is left for urinary drainage [3, 20]. cisely in the midline until the retro-urethral space,
Perineal approach: the patient lays in lithot- where the PU lays, is exposed. Two self-retaining
omy position with flexed hips. A midline incision retractors are inserted to provide better exposure.
is made from the caudal end of the scrotum to the The PU is dissected under direct vision till it
anterior margin of the anus with needle tip cau- enters the urethra and excised. The urethral defect
tery and with the help of nerve and muscle stimu- is closed in layers. At the end of the PU removal,
lator. The incision is deepened through the the rectal wall is closed in layers, the patient is
perineal plane till the anterior wall of the rectum turned in a lithotomy position, and a protective
that is retracted posteriorly. Levator muscles are colostomy is created.
mobilized to expose the utricle that is isolated. To avoid protective colostomy, Leite et al.
Low utricles are easy to identify and to encircle introduced the possibility to perform a midline
the insertion point. In high and small ones, it can incision extended from the anterior margin of the
be safer to open the utricle in the midline to see anus to the scrotum without rectal splitting [15].
and close the communication with urethra and The anorectal sphincter can be sectioned if nec-
remove the PU or decide to extirpate only the essary and reconstructed at the end of the
mucosal layer to avoid injuries [5]. procedure.
Posterior sagittal trans-anorectal [17, 21, 22]:
this approach has been widely described by Peña
and is characterized by posterior incision with 48.5 Minimally Invasive
rectal splitting. This approach provides an excel- Techniques
lent exposure but can require protective colos-
tomy in case of insufficient bowel preparation Laparoscopic approach [1, 23, 24]: the patient
and harvesting for 7–10 days postoperatively. lays in supine position. At first, umbilical trocar
Alternatively, a posterior sagittal rectum 10 or 5 mm (according to the age of the patient)
retracting approach has been described [16]: the is placed for the optic. Two further trocars (5 mm
incision is made in the midline from the third or 3 according to the age) are placed at right and
sacral segment to about 1.3 cm posterior to the left flank, respectively. Once entered the abdomi-
anus and deepened dividing the parasagittal mus- nal cavity, the bladder is suspended to the abdom-
cles and elevator ani without cutting the external inal wall to provide a better exposure of the
sphincter with the use of a muscle stimulator retrovesical space. The peritoneum is opened and
until the rectum. The investing fascia is divided the PU is isolated until its communication with
in the midline and the rectum is retracted on the urethra. A ureteric catheter inserted in the utricle
right. The PU is identified thanks to an internal before the procedure helps the localization of the
catheter placed cystoscopically before the proce- malformation. Alternatively, a cystoscope is
dure. It is picked up and isolated carefully start- inserted in the PU, so the light can guide the iden-
ing from the dome to its junction with the tification of the enlarged utricle. The communi-
urethra. cation with urethra can be closed with two
Anterior sagittal: in the transanorectal endoloops or, in case of wide communication,
approach [18, 19], the patient is placed in the with an endostapler (Fig. 48.2).
prone knee-chest position and a ribbon impreg- Robot-assisted approach [25–32]: the child
nated with disinfectant is inserted in the rectum lays supine position with legs stretched out and
to reduce contamination. A midline sagittal inci- abducted in order to allow for concomitant ure-
sion is made from the anterior anal margin onto throcystoscopy which is performed to place a
the perineum with needle tip cautery. Only the urethral catheter inside the utricle and a Foley
anterior rectal wall is opened and the incision is catheter into the bladder to aid in identification.
a b
c
d
Fig. 48.2 (a–d) Laparoscopic removal of PU. (a) The peritoneum is opened above the PU that is indicated by the light
of the cystoscope. (b) Then it is carefully isolated. (c) The neck is closed with an endoloop and is cut (d)
The optical port at the umbilicus is advanced catheter forward and backward, it is possible to
into the peritoneum and two 8 mm working be sure not to have caused a stricture in the
ports are placed on the para-rectal lines under urethra (Fig. 48.3).
direct vision (distance between each port and
umbilicus 7 cm). An additional 5 mm assistant
port is used. The table is put in Trendelenburg 48.6 Outcomes
position so that the bowel glides out of the pel-
vis by gravity; finally the ports are secured to 48.6.1 Endoscopic Techniques
the robotic system. After pneumoperitoneum is
achieved and PU dome identified, also through Schuhrke and Kaplan reported morbidity and
the help of the assistant providing movement of high recurrence rate after endoscopic transure-
the catheter inside the utricle, the peritoneal thral catheterization and aspiration, orifice dilata-
reflection covering the dome is incised to free tion, incision, or unroofing [13]. Coppens et al.
the dome. The PU is grasped with forceps and reported 82% of success rate with transperineal
carefully dissected free of the surrounding tis- or transrectal puncture, simple endoscopic sec-
sues of the retrovesical space with a monopolar tion of the utricle meatus, or large marsupializa-
hook. A complete bloodless dissection is carried tion [33]. Ahmed and Palmer reported successful
out for all the length of the diverticulum. Once transperineal cyst aspiration and sclerotherapy by
completely dissected, the PU neck is secured tetracycline under transrectal ultrasound guid-
with two preformed endoscopic loops and ance [12]. Husmann and Allen had 83% success
resected just a few millimeters above its junc- rate with endoscopic electrofulguration of utricu-
tion with the urethra. By moving the bladder lar mucosal layer [14].
a b
c d
e f
Fig. 48.3 (a–f) Robot-assisted removal of PU. (a) Before peritoneum is opened and the PU is identified with the
the procedure, a ureteric catheter is placed in the PU. (b) help of the catheter in the PU. (d) An endoloop is placed
We used one umbilical trocar for the optic, two operative in the neck. (e, f) The PU is cut and exteriorized
trocars at the flanks, and one accessory trocar. (c) The
48.6.2 Open Techniques the dimension of the stone. Both open and endo-
scopic [37] approaches have been described.
Schuhrke and Kaplan reported 58% of incom- Another issue concerns the possible onset of
plete excision of PU by the suprapubic, retrovesi- tumors in undiagnosed PU. Usually the diagno-
cal, or transvesical approach [13]. Monfort had sis is done in adults, but also an adolescent case
no sequelae after transvesical approach [20], and has been reported [38]. Schuhrke and Kaplan
Desautiel et al. had good results with transvesical reported a 3% incidence of malignancy in a case
transtrigonal approach in their series [3]. Good series of 88 patients with PU [13]. Reported
results with no recurrence have been reported malignancies in the prostatic utricle include uro-
with posterior sagittal approaches both transano- thelial carcinoma, clear cell adenocarcinoma,
rectal and rectum retracting [5, 16, 17]. Also the prostatic duct carcinoma (called endometrial
anterior sagittal approaches have proved effective carcinoma in the past), and squamous cell carci-
as reported by Leite et al. and Rossi et al. [15, noma [38–42].
19].
However, it must be considered that whatever
the approach, the reported case series are quite 48.8 Infertility Issues
small.
The presence of an enlarged PU is associated to
subfertility in adult males. This happens probably
48.6.3 Minimally Invasive Techniques because of compressive effect of the PU on ejac-
ulatory duct [3]. If the PU remains undiagnosed
Minimally invasive technique seems to have less till the adult age, infertility constitutes an indica-
associated morbidity when compared to open tion for treatment.
approaches. Laparoscopic and robotic approaches Müllerian and Wolffian structures have sepa-
give excellent visualization and magnification rate embryologic origin, so there should not be
which allow meticulous dissection of the PU and communication between the two systems; never-
minimal manipulation of surrounding structures. theless in some cases the vas deference has an
In a recent review regarding mainly the laparo- ectopic insertion in the cavity. In these cases,
scopic approach, no intraoperative complications complete resection of the PU requires vasectomy.
are reported nor recurrences, voiding dysfunc- The detached vas can be reimplanted in the pos-
tions, and malignant degeneration with a mean terior urethra or in the bladder [3, 5, 20], but fer-
follow-up of 20.5 months [23]. tility is compromised.
Robotic experience is still limited to case On the other hand, if there is no communica-
reports; nevertheless, all authors report excellent tion between the two systems, the vas deferens
visualization without intra- or postoperative can be separated from the Müllerian derivatives.
complications [25–32]. However, sometimes it is necessary to leave a
small part of the PU wall in close proximity to the
deferens to avoid the risk of damage.
48.7 Complications
of Undiagnosed Prostatic
Utricle References
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H. The prostatic utricle cyst with huge calculus and
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org/10.1159/000354766.
Penoscrotal Transposition
49
Ahmed T. Hadidi
Abbreviation The first published case of penoscrotal trans-

position was described by Broman in 1911 [1].
PST Penoscrotal transposition Forshall and Rickham (1956) reported repair in
three stages [2]: (1) straightening of the penis, (2)
repair of the hypospadias, and (3) later release of
contracture. Campbell (1963) divided the scro-
49.1 Introduction tum, brought the penis forward, and sutured the
scrotum behind [3].
Early in human male genital development
(6 weeks gestational age), the scrotal folds (labial
folds in females) lie lateral to the genital tubercle. 49.2 Presentation and Associated
During normal development under testosterone Anomalies
influence, as the testes descend down, the two
scrotal folds migrate caudally and medially to Penoscrotal transposition (PST) is frequently
fuse in the midline in normal males (Fig. 49.1) associated with up to 90% urogenital (hypospa-
(see Chap. 3). When the labioscrotal folds fail to dias, chordee, renal dysplasia) and/or up to 30%
migrate caudally or migrate only incompletely, gastrointestinal malformations (predominantly
they remain lateral to the genital tubercle and imperforate anus). It may present with a broad
present as penoscrotal transposition. spectrum of anomalies ranging from simple shal-
In severe forms, other anomalies, e.g., peri- low scrotum (doughnut scrotum) to very complex
neal hypospadias, absence of urinary tract, poly- extreme transposition with craniofacial, central
cystic kidney, and imperforate anus may coexist. nervous system, cardiac, gastrointestinal, urolog-
In severe forms, the penis may be barely visible ical, and other genital (undescended testicles,
unless the scrotum is retracted laterally hypospadias, and chordee) malformations.
(Fig. 49.2). Moderate to severe chordee is usually Growth deficiency and intellectual disability may
present and the whole length of the penile urethra also be noticed. PST may be observed in isolation
may need to be reconstructed. or in association with caudal regression syn-
drome [4], Simpson-Golabi-Behmel syndrome
(an X-linked recessive overgrowth disorder char-
A. T. Hadidi (*) acterized by prenatal onset of overgrowth, super-
Department, Sana Klinikum Offenbach, Academic numerary nipples, a grooved tongue or chin,
Teaching Hospital of the Johann Wolfgang Goethe chest wall malformations, cryptorchidism, hypo-
University, Offenbach, Frankfurt, Germany spadias, and penoscrotal transposition) [5], and
706 A. T. Hadidi
Fig. 49.1 The scrotal fold develops lateral to the genital tubercle at about eighth week of gestation. They migrate
downward and medially to fuse in the midline below the penis (©Ahmed T. Hadidi 2022. All Rights Reserved)
Fig. 49.2 Complete penoscrotal transposition (PST). The penis may be only seen when separating the scrotal folds
in Aarskog syndrome (an X-linked inheritance • Complete penoscrotal transposition

syndrome). • Partial penoscrotal transposition (bilateral or
unilateral)
• Penoscrotal transposition associated with bifid
49.3 Classification scrotum in perineal hypospadias
Penoscrotal transposition is classified according Fahmy et al. (2014) added two more catego-
to severity into three grades (Fig. 49.3): ries: central scrotalization of the median raphe
49 Penoscrotal Transposition 707
a b c
Fig. 49.3 (a–c) Penoscrotal transposition may result position. (b) Classic perineal hypospadias with incomplete
from failure of the scrotal folds to migrate caudally and penoscrotal transposition. (c) Classic distal hypospadias
medially at the same time of arrested, disorganized distal with incomplete prepuce (©Ahmed T. Hadidi 2022. All
migration of the ventral mesenchyme. (a) Complete sepa- Rights Reserved)
ration of the scrotal folds with complete penoscrotal trans-
and wide penoscrotal distance or caudal peno- reconstruction is then fashioned 6 months later. If
scrotal transposition [6]. PST persists, it should be corrected as a third
In complete or extreme PST, the penis is com- stage operation to avoid jeopardizing the blood
pletely caudal to the scrotum, and there are no supply to the skin flaps. Mori and Ikoma, in 1986,
scrotal tissues distal to the penis. Major cases are even suggested that a free bladder mucosal graft
usually associated with penoscrotal hypospadias might be applied using tunica vaginalis for addi-
and the scrotal tissues surround the root of the tional cover [9].
penis completely. These cases may or may not Scrotal procedures have been described for
have hypospadias but usually have a short ano- correction of penoscrotal transposition;
genital distance. Minor cases have different Campbell, in 1970, bisected the scrotum and
grades of scrotal tissue creeping to one (unilat- sutured the two halves beneath the penis [10].
eral) or both sides of the penis. Central scrotaliza- McIlvoy and Harris (1955) placed the penis ante-
tion is the condition where the median raphe is rior to the scrotum through a subcutaneous tunnel
replaced completely by the redundant scrotal tis- [11]. Glenn and Anderson (1973) performed
sues, but the whole scrotum still acquire its nor- simultaneous correction of PST and repair of
mal position, another variant described herein hypospadias [12]. They completely mobilized the
where the child had a wide distance between the two halves of the scrotum as rotational advance-
penis and scrotum with a loss of normal continu- ment flaps with relocation of the scrotal compart-
ity and configuration between the penis and ment in a normal dependent position and
scrotum. constructed the neourethra at the same
operation.

49.5 Ehrlich-Scardino Technique
When PST is associated with perineal hypospa-
dias (grade IV), a planned three-stage repair Ehrlich and Scardino’s procedure (Fig. 49.4) is
should be considered, as the preputial skin is similar to the Glenn-Anderson technique, but
rarely sufficient to form the entire urethra during with some modifications [7]. They performed
perineal hypospadias repair [7, 8]. The penis simultaneous chordectomy and scrotoplasty
should be straightened and the skin brought to the for incomplete scrotal transposition, chordee,
ventral surface during the first stage. Urethral and perineal hypospadias. However, the inci-
708 A. T. Hadidi
sion differed in leaving a wide skin bridge con- to facilitate completion of the neourethra at a
necting midline dorsal penile skin with second stage. They felt that simultaneous
suprapubic skin to preserve the vascularity of repair would allow part of the scrotal skin to
the penile shaft skin, and instead of carrying integrate in the neourethra repair and the prob-
the incision distal to the original meatal posi- lem of hair growth might occur.
tion, the incision is made proximal to the
meatus to allow formation of a Thiersch-
Duplay skin tube. Ehrlich and Scardino elected 49.6 Mori-Ikoma Technique
to perform the correction in two stages and
bring the meatus as far distally as possible, The incision for the Mori-Ikoma technique is an
construct the ventral neourethra, and cover it inverted omega around the scrotal skin and the
with Byars flaps; more foreskin was mobilized base of the penis; closure of the flaps is in the
Fig. 49.4 The incision used in the Ehrlich-Scardino technique

form of a zeta [9]. The procedure is shown in The penis is freed from the scrotum along the
Fig. 49.5. The authors stated that the final results line shown in Fig. 49.5. The incision extends to
are better when scrotoplasty is performed after the superior aspect of the scrotum to be swung
repair of hypospadias. inferio-medially below the penis; chordee is
Fig. 49.5 The incision used in the Mori-Ikoma technique

710 A. T. Hadidi
extensively released, splitting the glans. Byars giving the appearance of a bucket. The ure-
flaps are fashioned to cover the defect and extend thral plate is preserved. When a “handle” is
to cover the split glans to create a distal urethra at incised at 12 o’clock between the parallel
the second stage. Diez-Garcia et al. (1995) coronal circumferential incisions, it resem-
reported on ten boys treated during the period bles a long Y. The urethral plate and attached
between 1992 and 1993 at an average age of strips are mobilized with the proximal ure-
10 months [13]; hypospadias was present in nine thra, the penis is degloved, and the chordee
of the patients. The Glenn-Anderson correction is corrected. The medial edges of the two
technique was used in two cases and the Ehrlich- skin flaps are sutured to form the dorsum of
Scardino technique in the others. Correction of the new urethra. The proximal urethra is
the penoscrotal transposition was done during formed by rolling the urethral plate, and
urethroplasty. sutures extend to roll the l ateral edges of the
skin flaps, completing the tubularization.
The glans is split in the midline for place-
49.7 Shanberg-Rosenberg ment of the neourethra and the glanuloplasty
Technique is completed using a silicone tubing stent.
The scrotal skin lying beside and cephalad to
In the Shanberg-Rosenberg technique for treat- the penis is outlined, incised, and tubular-
ment of partial transposition of the penis and ized. Byars flaps are fashioned and may be
scrotum with anterior urethral diverticulum in a crisscrossed for ventral coverage.
child born with caudal regression syndrome, two When simultaneous repair of incomplete scro-
flaps are cut, one with its base on the superior or tal transposition, chordee, and perineal hypospa-
pubic face and the other with its base on the infe- dias is performed, a skin bridge must be left in
rior or scrotal face [14, 15]. continuity with the pubic skin to ensure adequate
They reported their experience with the blood supply and lymph drainage to the penile
Koyanagi-Nonomura one-stage bucket repair skin.
of severe hypospadias with and without The largest series from a single institution,
penoscrotal transposition in 14 boys, the age 53 patients, was published by Pinke et al.
ranged from 10 to 20 months. Severe hypo- (2001) [16]. The patients with penoscrotal
spadias and chordee were associated with the transposition ranged in age from 1 day to
penoscrotal transposition in eight cases. 30 years. Thirteen percent had a family history
Shanberg and Rosenberg carried out simulta- of penoscrotal transposition, one family showed
neous repair of the hypospadias and the inheritance in an X-linked recessive manner,
penoscrotal transposition. In their technique, and 32% showed abnormalities in other organ
an initial circumferential incision around the systems, with the genitourinary system being
corona is followed by a U-shaped incision affected most. Seventy-nine percent had hypo-
with its base proximal to the meatus, spadias and 81% chordee. These anomalies
extended approximately 8 mm proximal to were corrected in a single-stage repair:
the previously made coronal incision. This Thiersch-Duplay urethroplasty in 6 patients
second incision is extended laterally and and complex repair with bladder or buccal
dorsally onto the dorsal hood parallel to and mucosal graft or a staged procedure in 34
approximately 8 mm from the first incision, patients. Correction of penoscrotal transposi-
Fig. 49.6 A 6-month-old baby with complete peno- dragged the scrotum down and medially and corrected
scrotal transposition, perineal hypospadias and imper- the PST. The child did not need further surgery to cor-
forate anus. The hypospadias was corrected during the rect the PST (©Ahmed T. Hadidi 2022. All Rights
first year of life and correction of the bifid scrotum has Reserved)
tion included a Glenn-Anderson technique in sions are continued downward, under the penis,
37 patients, Singapore rotational flaps in 7, and to meet together in the midline, and the penis is
V-Y procedure in 6. Glenn-Anderson repair lifted upward against the abdominal wall. The
produced the best cosmetic results and was two triangular flaps are elevated, freely mobi-
associated with a significantly lower incidence lized, and brought together in the midline under
of complications. the penis. The resultant skin edges are sutured
together. The operative steps are shown in
Fig. 49.7.
49.8 Current Technique Used
for PST in Hypospadias
Center, Frankfurt 49.9 Bifid Scrotum
It was observed that when perineal hypospadias Bifid scrotum may occur as an isolated anomaly
associated with PST was corrected during the but often is associated with hypospadias. The
first year of life, the correction of hypospadias anomaly is corrected by excision of midline strips
and correction of bifid scrotum usually drag the of skin of each hemi-scrotum, approximation of
scrotal transposition down and correct the condi- external spermatic fascia, and skin closed with
tion without the need of surgical correction interrupted stitches after ensuring hemostasis. A
(Fig. 49.6). Z-plasty is usually needed to lengthen the medial
In older children, two triangular incisions edges of the scrotal folds and to have a more nat-
including the scrotal skin are made on either side ural looking penis and scrotum.
of the penis. The medial limbs of the two inci-
712 A. T. Hadidi
Fig. 49.7 Operative steps for penoscrotal transposition repair in the Hypospadias Center, Frankfurt. (©Ahmed
4. Dillon BJ, Alomari AI. Angiographic demonstration

References of an aberrant abdominal umbilical artery in a patient
with penoscrotal transposition and caudal regression.
1. Broman I. Normale und Abnorme Entwicklung Surg Radiol Anat. 2009; https://doi.org/10.1007/
des Menschen. Norm und Abnorme s00276-008-0411-9.
Entwicklung des Menschen. 1911; https://doi. 5. Griffith CB, Probert RC, Vance GH. Genital anoma-
org/10.1007/978-3-642-51340-4. lies in three male siblings with Simpson-Golabi-
2. Forshall I, Rickham PP. Transposition of the penis Behmel syndrome. Am J Med Genet A. 2009; https://
and scrotum. Br J Urol. 1956; https://doi.org/10.1111/ doi.org/10.1002/ajmg.a.33047.
j.1464-410X.1956.tb04764.x. 6. Fahmy MAB, El Shennawy AAA, Edress
3. Campbell MF. Anomalies of the genital tract. In: AM. Spectrum of penoscrotal positional anomalies in
Campbell MF, Harrison JH, editors. Urology. 2nd ed. children. Int J Surg. 2014; https://doi.org/10.1016/j.
Philadelphia: Saunders; 1963. p. 1749. ijsu.2014.08.001.
7. Ehrlich RM, Scardino PT. Surgical correction of 12. Glenn JF, Anderson EE. Surgical correction of
scrotal transposition and perineal hypospadias. incomplete penoscrotal transposition. J Urol.
J Pediatr Surg. 1982; https://doi.org/10.1016/ 1973;110:603–5.
S0022-3468(82)80205-4. 13. Diez García R, Bañuelos A, Marín C, de Tomás
8. Retik AB, Mandell J, Bauer SB, Atala A. Meatal E. Peno-scrotal transposition. Eur J Pediatr Surg.
based hypospadias repair with the use of a dor- 1995;5:222–5.
sal subcutaneous flap to prevent urethrocutane- 14. Shanberg AM, Rosenberg MT. Partial transposi-
ous fistula. J Urol. 1994; https://doi.org/10.1016/ tion of the penis and scrotum with anterior urethral
S0022-5347(17)32555-7. diverticulum in a child born with the caudal regres-
9. Mori Y, Ikoma F. Surgical correction of incomplete sion syndrome. J Urol. 1989; https://doi.org/10.1016/
penoscrotal transposition associated with hypospa- S0022-5347(17)38988-7.
dias. J Pediatr Surg. 1986; https://doi.org/10.1016/ 15. Glassberg KI, Hansbrough F, Horowitz M. The
S0022-3468(86)80652-2. Koyanagi-nonomura 1-stage bucket repair of
10. Campbell MF. Anomalies of the genital tract. In: severe hypospadias with and without peno-
Campbell MF, Harrison JH, editors. Urology. 3rd ed. scrotal transposition. J Urol. 1998; https://doi.
Philadelphia: Saunders; 1970. p. 1576–7. org/10.1097/00005392-199809020-00038.
11. McIlvoy DB, Harris HS. Transposition of the penis 16. Pinke LA, Rathbun SR, Husmann DA, Kramer
and scrotum: case report. J Urol. 1955; https://doi. SA. Penoscrotal transposition: review of 53 patients. J
org/10.1016/S0022-5347(17)67437-8. Urol. 2001;166:1865–8.
Uncommon Conditions
and Complications 50
Ahmed T. Hadidi
Abbreviations 50.2 Genitourinary Injuries

in the Newborn
FH Female hypospadias
LIP Long inner prepuce Genitourinary trauma in the newborn is rare but
SLAM Slit-like adjusted Mathieu often necessitates significant surgical
intervention.
A common cause of injury is circumcision in
a buried penis. If the person performing circum-
50.1 Iatrogenic Hypospadias cision is not experienced with buried penis and
the fact that those children have long inner pre-
Iatrogenic hypospadias may be caused by puce (LIP) and very little outer skin (see Chap.
improper circumcision or by an injury to the ven- 45), he may excise too much outer penile skin
tral male urethra produced by downward pressure (Fig. 50.2).
of an indwelling urethral catheter (Fig. 50.1). Patel et al. (2001) reported seven cases of gen-
Andrews et al. (1998) reported 16 patients itourinary trauma in newborns [2]. Two of these
with this injury during a 9-year period [1]. They infants suffered degloving injury from a Gomco
reconstructed the urethra in six patients and pro- clamp and required skin grafting. Three had a
posed that patients requiring long-term indwell- Mogen clamp, which caused glans injuries: one
ing catheter should be investigated so that an required hemostasis, one a modified meatal
alternative therapy to catheterization can be advancement glanuloplasty, and the third had a
established. formal hypospadias repair. The sixth patient suf-
fered a ventral slit and division of the ventral ure-
thra before placement of a Gomco clamp,
requiring hypospadias repair, and the last suf-
fered a vaginal tear during emergency caesarean
section.
Another cause is the use of unipolar diathermy
A. T. Hadidi (*) during circumcision which may result in necrosis
Hypospadias Center and Pediatric Surgery of the penis (Fig. 50.3). This is a very serious
Teaching Hospital of the Johann Wolfgang Goethe condition and the child may suffer the conse-
University, Offenbach, Frankfurt, Germany quences for the rest of his life.
716 A. T. Hadidi
50.3 Congenital
Urethrocutaneous Fistula
Without Hypospadias
Congenital fistula of the penile urethra without

hypospadias or chordee (Fig. 50.4) is very rare. It
has been postulated to be due to a focal defect in
the urethral plate with normal development of the
glanular urethra, as it develops separately from
the surface ectodermal invagination [3].
50.4 Congenital
Urethrocutaneous Fistula
with Hypospadias
Congenital fistula of the penile urethra with

hypospadias is also very rare (Fig. 50.5).
50.5 Accessory Scrotum
Accessory scrotum or perineal scrotum is an

uncommon anomaly. Balik et al. (1990) traced
Fig. 50.1 Injury to the ventral urethra due to downward five patients reported in the literature and reported
pressure of indwelling catheter (©Ahmed T. Hadidi 2022. a case of accessory scrotum and glanular hypo-
All Rights Reserved) spadias [4].
Fig. 50.2 Circumcision injury where the surgeon excised most of the penile skin (©Ahmed T. Hadidi 2022. All Rights
Reserved)
50 Uncommon Conditions and Complications 717
Fig. 50.3 Necrosis of the penis due to the use of monopolar diathermy (courtesy of Assad Matar)
Fig. 50.4 Congenital fistula without hypospadias (©Ahmed T. Hadidi 2022. All Rights Reserved)
718 A. T. Hadidi
Fig. 50.5 Congenital urethral fistula with hypospadias (©Ahmed T. Hadidi 2022. All Rights Reserved)
50.6 Mole in the Glans resulting in hypospadias fistula if the condition

is not recognized very early. Immediate release
A child may be born with a mole in the glans or is obtained by dividing the constricting hair; if a
the body of the penis. Extra care must be taken fistula occurs, however, it may require formal
when excising the mole as it may result in ugly closure. Fig. 50.7 shows incomplete separation
scar in the glans (Fig. 50.6). When excising the of the glans due to hair coil and its correction.
resultant scar, the surgeon must avoid further dis- The main principle in treatment is complete
figurement of the glans. excision of any unhealthy scarred tissue and clo-
sure with fine sutures; no stenting is carried out.
Interestingly, the penis heals nicely and the inci-
50.7 Hair Coil dence of recurrent fistula is much lower than
with hypospadias fistula as the tissues in this
In occasional cases a hair coil constricts the dis- case are healthy and well vascularized (unlike in
tal penile shaft, causing pressure necrosis and hypospadias).
a b c
Fig. 50.6 (a) Mole on the glans. (b) Ugly scar after excision. (c) After scar excision (©Ahmed T. Hadidi 2022. All
Rights Reserved)
a b
Fig. 50.7 (a) Hair coil that almost amputated the glans penis and distal urethra. (b) After surgery (©Ahmed T. Hadidi
720 A. T. Hadidi
50.8 Congenital Meatal Cyst tion of the corpus spongiosum and is usually
associated with proximal bifurcation of the cor-
A child may present with meatal cyst that may pus spongiosum (Fig. 50.10).
cause disfigurement and maldirection of urine The classic management was to incise the thin
(Fig. 50.8). The etiology is unclear. Excision is urethra until one reaches the healthy normal ure-
simple and the result is satisfactory when using thra as it is almost impossible to dissect the skin
fine absorbable suture material. off the very thin urethra. However, during the
past 10 years, the author has kept the thin adher-
ent skin over the thin urethra and reconstructed
50.9 Lateral Chordee the new urethra, covering the new urethra as well
as the paper thin skin with protective fascial layer
Usually chordee is present on the under surface with excellent results. Thus one can avoid a distal
of the penis due to disorganization and arrested hypospadias into a proximal one.
distal migration of the ventral mesenchyme
(“Disorganization Hypothesis,” see Chap. 7).
Rarely, chordee may be lateral and is called the 50.11 Partial Dissection
“lateral chordee.” Lateral plication may improve of the Epithelium
the condition if it is troubling the child of the Urethral Wall
(Fig. 50.9).
Wall dissection of the neourethra is a rare com-
plication causing severe infravesical obstruction
50.10 Proximal Thin Urethra after hypospadias repair after an initially success-
ful double facial island flap repair. The obstruc-
Proximal paper thin urethra is not uncommon in tion is caused by partial dissection of the
hypospadias and is rare without hypospadias. It is epithelium of the urethral wall, comparable to
probably due to disorganization and distal migra- intima dissection in an aortic aneurysm [5].
a b
Fig. 50.8 (a) Meatal cyst without hypospadias. (b) Meatal cyst with hypospadias (©Ahmed T. Hadidi 2022. All Rights
Reserved)
Fig. 50.9 Lateral chordee and its correction (©Ahmed T. Hadidi 2022. All Rights Reserved)
Fig. 50.10 Thin proximal urethra. One does not need to tective intermediate layer (©Ahmed T. Hadidi 2022. All
incise the thin urethra nor de-epithelialize it. It is possible Rights Reserved)
to leave the thin skin and simply cover it with good pro-
722 A. T. Hadidi
50.12 Lymphedema of the Penis the polyps in four patients in whom the tumor is
After Hypospadias Surgery small. However, severe nephrogenic adenoma
involving most of the reconstructed urethra may
Marked lymphedema of the penis (Fig. 50.11) require complete resection of the graft and cre-
may occur after hypospadias repair due to impair- ation of a new urethra.
ment of lymph drainage, particularly after con-
struction of Byars flaps. It is rare and has been
attributed to postoperative infection. Surgical 50.14 S
quamous Cell Carcinoma
excision of the lymphedematous tissue is required After Hypospadias Repair
to improve the appearance.
Dodd et al. (1996) reported a case of squamous
cell carcinoma of the distal urethra in a patient
50.13 A
denoma After Hypospadias with congenital hypospadias [7].
Repair
Nephrogenic adenoma is an unusual benign 50.15 Hairy Urethra

metaplastic lesion of the urothelium.
Weingartner et al. (1997) reported five chil- Hairy urethra is due to the use of hair-bearing
dren and one adult who developed nephrogenic scrotal skin in cases of severe perineal hypo-
adenoma of the urethra after hypospadias repair spadias. Stones may develop in the hairy seg-
using bladder mucosal grafts [6]. Successful ment of the urethra. A new urethroplasty may
treatment consisted of endoscopic resection of be required to remove the hairy segment and
replace it with non-hair-bearing skin
(Fig. 50.12).
Fig. 50.11 Lymphedema of Byars flaps (courtesy of Fig. 50.12 Hairy urethra 20 years after Cecil-Culp oper-
Amir Azmy) ation (©Ahmed T. Hadidi 2022. All Rights Reserved)
50.16 Ulcer in an Adult observed that atresia of the corpora cavernosa

with Uncorrected was variable [10]. It was concluded that the clas-
Hypospadias sification of megalourethra into scaphoid and
fusiform subtypes was arbitrary, and megaloure-
Rarely, an adult with an uncorrected hypospadias thra is currently recognized as a disorder with a
may come and present with painful ulcer in the spectrum of presentations [11].
glans region. The ulcer was excised and new ure- Although the etiology of megalourethra is
thra was reconstructed to the tip of the glans controversial, mesodermal dysplasia is a poten-
using the “slit-like adjusted Mathieu” (SLAM, tial cause. This view is supported by the observa-
see Chap. 22) technique (Fig. 50.13). tion that megalourethra is often associated with
other congenital anomalies, particularly diffuse
mesenchymal disorders. As many as 50% of
50.17 Congenital Megalourethra patients with scaphoid megalourethra have prune
belly syndrome [12].
Congenital megalourethra is defined as congeni-
tal diffuse enlargement of the anterior urethra. It
was first described by Nesbit in 1955 [8]. This
disorder is characterized by a nonobstructive ure-
thral dilatation associated with deficiency of cor-
pus spongiosum erectile tissue.
In 1963, Dorairajan classified megalourethra
into a scaphoid type and a fusiform variety [9]. In
the more common scaphoid variant, the corpora
cavernosa are intact and a dorsal penile curvature
is often noted (Fig. 50.14). Characteristic bal-
looning of the ventral urethra occurs on micturi-
tion. Fusiform megalourethra is characterized by
the absence of both the corpus spongiosum and
the corpora cavernosa. The penis is elongated and Fig. 50.13 Ulcer in the glans: An adult with uncorrected
saclike, and erections are not possible. In 1986, coronal hypospadias presenting with painful ulcers in the
Appel and colleagues reviewed several cases of glans region (©Ahmed T. Hadidi 2022. All Rights
Reserved)
megalourethra with histological evaluation and
a b
Fig. 50.14 Congenital scaphoid megalourethra (a) Preoperative view. (b) Postoperative view (©Ahmed T. Hadidi
724 A. T. Hadidi
The diagnosis of megalourethra is made by mal postvoid dribbling is the only symptom, the
physical examination of the external genitalia. retained urine can be drained by massage of the
Imaging studies such as retrograde urethrography urethra after urination. Surgical repair of scaphoid
or voiding cystourethrography demonstrate the ure- megalourethra is usually achieved by mobilization
thral dilatation and diagnose any associated lower of the penile shaft skin, ventral vertical incision of
tract anomalies. Upper tract imaging is mandatory. the urethra, excision of redundant urethral tissue,
When instrumentation is performed, prophylactic and suture re-approximation of the urethra over a
antibiotics are indicated to prevent sepsis. stent as originally described by Nesbit. Although
The initial management of megalourethra excision of redundant urethra is the most commonly
includes the initiation of suppressive antibiotics, reported repair [14], Heaton and colleagues (1994)
particularly when upper tract anomalies coexist. described a successful technique using infolding
When the overall prognosis is good, corrective urethral plication rather than excision [15]. The penis
surgical intervention should be considered. With is degloved, and fine absorbable inverted Lembert
fusiform megalourethra, the absence of corporeal sutures are placed in a vertical row approximately
tissue compromises reconstructive repair, and 2–3 mm apart. If necessary, hypospadias repair can
gender reassignment surgery is generally consid- be simultaneously performed. Potential advantages
ered the procedure of choice. The redundant of plication include improved preservation of the
penile skin is excised, gonadal tissue is removed, urethral blood supply and a reduced incidence of
and labia are developed from the scrotal skin. A urethrocutaneous fistula [16, 17].
promising alternative to gender reassignment
may be phallus construction using a forearm free
flap. With this technique, a phallus of adequate 50.18 Suture Tracks
size and acceptable appearance can be con-
structed. The patient can void while standing, Suture tracks (or sinuses) occur when epithe-
achieve erections after placement of a prosthesis lium grows along skin sutures before they dis-
and experience erogenous sensations [13]. solve or are removed postoperatively
Patients with scaphoid megalourethra do not (Fig. 50.15). These sinuses subsequently fill
always require surgery. When hypospadias is absent, with desquamated keratin, producing unsightly
asymptomatic patients can be observed. When mini- marks. The likelihood that wound closure will
Fig. 50.15 Suture tracks (©Ahmed T. Hadidi 2022. All Rights Reserved)
leave suture tracks depends upon a variety of most rapidly absorbed. Because tracks develop
factors, including the size and composition of by epithelial growth along sutures, it is reason-
suture materials and the host conditions influ- able that longer-lasting materials may be more
encing wound healing. likely to produce tracks.
During hypospadias repair, the skin is closed
using absorbable sutures, typically chromic cat-
gut or Vicryl. According to the individual sur- 50.19 Smegma Mass
geon’s preference, interrupted or continuous,
simple, or mattress sutures of either 6-0 or 7-0 Smegma mass is an uncommon complication that
chromic catgut, Vicryl, polyglycolic acid, or PDS may develop years after hypospadias repair using
are commonly used. Bartone et al. [18] compared the foreskin to reconstruct the new urethra
wound healing with catgut, polyglycolic acid, (Fig. 50.16). Interestingly, the smegma mass or
and PDS in baboon foreskins and reported that retention cyst may not be in direct communica-
catgut incited the least inflammation and was the tion with the new urethra and it is often possible
Fig. 50.16 Smegma mass that developed 3 years after smaller in size. There was no communication between the
correction of proximal hypospadias using the LABO tech- smegma mass and the neourethra (©Ahmed T. Hadidi
nique. Notice the slit-like meatus. The child had no prob- 2022. All Rights Reserved)
lem passing urine, but the smegma mass was not getting
726 A. T. Hadidi
to excise it with careful dissection without having Hendren (1998) reported the cases of 40 girls
to injure or open the new urethra. It is very in whom he constructed the female urethra
unlikely to disappear without surgical using a vaginal wall and a buttock flap [21]. The
intervention. underlying pathology included bilateral ectopic
ureters, cloacal malformation, urogenital sinus
anomaly, previous failed surgery of the urethra,
50.20 Female Hypospadias severe trauma, and female hypospadias. The
urethra was reconstructed using tubularized
The natural history of meatal anomalies in girls anterior vaginal wall and covered with a buttock
and the incidence of female hypospadias in the flap in patients with urinary incontinence asso-
normal population are not known. It is thought ciated with short or absent urethra.
that there is an etiological correlation between Gonzalez and Fernandez (1990) described a
the functional voiding disorders and the meatal single-stage feminization genitoplasty which
anomaly. combines the Hendren and Crawford pull-
In fact, the terms “female hypospadias” (FH) through operation and the Kogan clitoroplasty
and “female urogenital sinus” are used inter- [22]. They used a flap of the preputial skin to con-
changeably. Both reflect different degrees of struct the vestibule and anterior vaginal wall and
agenesis of the urethrovaginal septum [19]. Sarin advocated universal use of the urogenital sinus to
and Kumar recommend to reserve the term FH construct the distal urethra and avoid creation of
for very low anomalies that open in the vestibule female hypospadias.
or lower 1/3rd of the vagina, and the term uro-
genital sinus should be used when the urethral
orifice opens in the upper 2/3rd of the vagina References
[19]. This may have prognostic implications. A
urethra that has its orifice close to the vestibule is 1. Andrews HO, Nauth-Misir R, Shah PJR. Iatrogenic
likely to have a normal caliber. The higher the hypospadias—a preventable injury? Spinal Cord.
1998; https://doi.org/10.1038/sj.sc.3100508.
urethral orifice, the narrower the urethra and the 2. Patel HI, Moriarty KP, Brisson PA, Feins
more likely that the patient would have urinary NR. Genitourinary injuries in the newborn. J Pediatr
outflow obstruction. Surg. 2001; https://doi.org/10.1053/jpsu.2001.20062.
Two types of meatal anomalies have been 3. Ritchey ML, Sinha A, Argueso L. Congenital fis-
tula of the penile urethra. J Urol. 1994; https://doi.
recognized: org/10.1016/S0022-5347(17)35182-0.
4. Balik E, Çetinkurşun S, Öztop F. Accessory scrotum:
1. Hypospadias in which the urethral meatus is report of a case. Eur J Pediatr Surg. 1990; https://doi.
displaced dorsally (female hypospadias) org/10.1055/s-2008-1042595.
5. De Gier RPE, Feitz WFJ, De Vries JDM. Wall dis-
2. Meatal web or covered hypospadias in which section of the neourethra: a rare complication after
there is a mucosal web on the dorsal side of hypospadias repair. Urology. 1997; https://doi.
the urethral meatus that deflects the urinary org/10.1016/S0090-4295(97)00229-X.
stream anteriorly 6. Weingartner K, Kozakewich HP, Hendren
WH. Nephrogenic adenoma after urethral recon-
struction using bladder mucosa: report of 6 cases
Hoebeke et al. (1999) studied the cases of and review of the literature. J Urol. 1997; https://doi.
288 girls who were referred for urodynamic org/10.1016/S0022-5347(01)64416-1.
assessment [20]. Eighty-eight had meatal 7. Dodd M, Lawson P, Hayman J. Squamous cell car-
cinoma of the distal urethra in a patient with con-
anomalies, and female hypospadias was genital hypospadias. Pathology. 1996; https://doi.
found in 24 and covered hypospadias in 64 org/10.1080/00313029600169633.
girls. Hoebeke and colleagues concluded 8. Nesbit TE. Congenital megalo-urethra. J Urol.
that girls with meatal anomalies have more 1955;73:839–42.
9. Dorairajan T. Defects of spongy tissue and congeni-
severe dysfunction as assessed by video uro- tal diverticula of the penile urethra. Aust N Z J Surg.
dynamics and proposed correction of the 1963; https://doi.org/10.1111/j.1445-2197.1963.
anomaly. tb03031.x.
10. Appel RA, Kaplan GW, Brock WA, Streit Howards SS, Duckett JW, editors. Adult and pediatric
D. Megalourethra. J Urol. 1986;135:747–51. urology. St. Louis: Mosby; 1996. p. 2411–3.
11. Bloom DA, Ritchey M. Anomalies of the urethra. In: 18. Bartone FF, Adickes ED. Chitosan: effects on wound
Hashmat AI, Das S, editors. The penis. Philadelphia; healing in urogenital tissue: preliminary report. J
1993. p. 35–59. Urol. 1988;140(2):1134–7.
12. Caldamone AA. Megalourethra. In: Seidmon EJ, 19. Sarin YK, Kumar P. Female hypospadias-need for
Hanno PM, editors. Current urologic therapy. clarity in definition and management. J Indian Assoc
Philadelphia: Saunders; 1994. p. 402–3. Pediatr Surg. 2019; https://doi.org/10.4103/jiaps.
13. Gilbert DA, Jordan GH, Devine CJ, Winslow BH, JIAPS_69_18.
Schlossberg SM, Duckett JW. Phallic construction in 20. Hoebeke P, Van Laecke E, Raes A, Van Gool JD,
prepubertal and adolescent boys. J Urol. 1993; https:// Vande Walle J. Anomalies of the external ure-
doi.org/10.1016/S0022-5347(17)36433-9. thral meatus in girls with non-neurogenic bladder
14. Walker RD. Megalourethra. In: Resnick MI, Kursh sphincter dysfunction. BJU Int. 1999; https://doi.
ED, editors. Current therapy in genitourinary surgery. org/10.1111/j.1464-410X.1999x.00929.x.
St. Louis: Mosby; 1992. p. 316–8. 21. Hendren WH. Construction of a female urethra using
15. Heaton BW, Snow BW, Cartwright PC. Repair the vaginal wall and a buttock flap: experience with 40
of urethral diverticulum by plication. Urology. cases. J Pediatr Surg. 1998; https://doi.org/10.1016/
1994;44:749–52. S0022-3468(98)90428-6.
16. Kester RR, Mooppan UMM, Ohm HK, Kim 22. Gonzalez R, Fernandez ET. Single-stage feminization
H. Congenital megalourethra. J Urol. 1990; https:// genitoplasty. J Urol. 1990; https://doi.org/10.1016/
doi.org/10.1016/S0022-5347(17)40228-X. S0022-5347(17)40091-7.
17. Duckett JW, Smith GHH. Urethral lesions in infants
and children. In: Gillenwater JY, Grayhack JT,
Hypospadias Surgery in Adults
51
Ahmed T. Hadidi
Abbreviations However, there are still many adult patients

with unsuccessful outcomes of hypospadias sur-
BXO Balanitis xerotica obliterans gery but are fed up with unsuccessful surgery and
DYG Double Y glanulomeatoplasty they will seek surgical help only if they are confi-
SLAM Slit-like adjusted Mathieu dent that the treating surgeon will be able to help
TIP Tubularized incised plate them based on the word of mouth and the experi-
UTI Urinary tract infection ence of other adult patients.
The interested, experienced surgeon may still
receive adults with no prior surgery and seek sur-
gical help because of their own body image or
51.1 Introduction because of their partners. Abnormal looking and/
or functioning penis may force the person to
The impact of hypospadias and its surgery con- avoid contact with the other sex for fear of embar-
tinues through adulthood. Genital and psycho- rassment and disappointment. The author has
sexual maturation can adversely impact how received adults in their 40s and 50s, successfully
patients perceive the success of their surgery both married, and have children but still, deep inside,
functionally and cosmetically [1]. The majority were unhappy with their body image.
of adults presenting to the surgeon with
hypospadias-related problems have already had
one or several unsuccessful hypospadias opera- 51.2 Adults Are Not Big Children
tions during childhood. Considering the large
number of boys who are operated for hypospa- Although the principles of hypospadias correc-
dias and the small number that present them- tion in adults are similar to the techniques used to
selves in adult life with complications, it seems correct hypospadias in children, adults are differ-
reasonable to assume that the majority of patients ent from children. It is not wise to assume that as
have had a good enough result not to seek revi- the adult penis is bigger, the operation should be
sion surgery. easier and the chances of success should be
higher.
Hollander et al. (1998) showed that wound
A. T. Hadidi (*) healing and skin lacerations in children had a
lower infection rate and more favorable cosmetic
Teaching Hospital of the Johann Wolfgang Goethe outcome than in adults [2]. Bhat et al. (2016)
University, Offenbach, Frankfurt, Germany compared the outcome of tubularized incised
730 A. T. Hadidi
plate (TIP) procedure in 60 adults and 60 chil- 4 weeks) in adults with complicated, proximal
dren and concluded that the complication rate hypospadias with severe scarring.
was higher in adults than in children [3]. • Several protective layers to cover the urethro-
Another important difference is erection. plasty are needed. The author usually uses
Erection will stretch the wound and urethroplasty four protective layers to cover urethroplasty in
suture lines and may cause a gap between the adults as compared to two protective layers in
urethral edges and also between skin edges children.
increasing the chances of fistula and wound • Adults tend to bleed much more than in chil-
dehiscence. dren. If the surgeon decides to use tunica vagi-
The pressure of urine expulsion from the blad- nalis flap as a protective layer, it may be
der is another important factor. The adult bladder advisable to leave a drain in the scrotum for
has a thicker and stronger muscle wall than in 2–3 days to avoid huge hematoma in the scro-
children and the high pressure of urine flow in tum (Fig. 51.1).
adults can be damaging to the new reconstructed • The use of anti-erection medications for
urethra during the healing process. 3 weeks after adult hypospadias operations.
The patient is clearly instructed to refrain
from sexual activity for at least one and half
51.3 Tips and Tricks When months (Chap. 40).
Operating on Adults
Based on the abovementioned observations and 51.4 Classification

experience with hypospadias operations in adults of Hypospadias in Adults
over the past 25 years, the author has modified and Their Management
his approach when operating in adults with regard
to the following points: Ching et al. (2010) [4] proposed a classification
for adult hypospadias based on the status of pre-
• The use of thicker suture material to close the vious surgery and the quality of the tissues
skin and subcutaneous tissues: In adults, the (Fig. 51.2).
author uses polyglactin (Vicryl) 6/0 for ure-
throplasty and polyglactin (Vicryl) 5/0 and 4/0
instead for closure of fascia, protective layers,
and skin.
• The use of very wide catheters for the urethro-
plasty: The author routinely uses catheter size
F12 or F14 in children. In adults he uses cath-
eter size F26–F30. As it is not always avail-
able in most hospitals, he reconstructs the new
urethra over two catheters together inside the
urethra (F12 + F16 or F18) to be replaced by a
catheter size F16 at the end of operation.
• Longer period of urinary diversion: For distal
hypospadias, the author leaves a transurethral
catheter up to 3 days in children or in adults. Fig. 51.1 Huge hematoma in the scrotum following the
use of tunica vaginalis flap as a protective intermediate
For complicated proximal hypospadias in layer without drainage. Immediately after surgery, every-
children, the author may use urinary diversion thing looked good. During the night, a huge hematoma
up to 2 weeks and may double the time (up to developed (©Ahmed T. Hadidi 2022. All Rights Reserved)
51 Hypospadias Surgery in Adults 731
• Category I: Adults who have undergone mul- over 16 years [4] (Fig. 51.3); Myers et al. (2012)
tiple surgeries with significant scarring and reviewed 50 adult patients over 20 years [6]; Hoy
tissue loss and Rourke (2016) reported their experience with
• Category II: Patients who present with delayed 93 adults who presented to their center over a
complications after initially successful child- 10-year period [1]. In other words each referral
hood repair center received less than 10 patients per year. The
• Category III: No previous surgery commonest four presentations are shown in
Table 51.1.
51.5 Presentation
Barbagli et al. (2006) reviewed 60 adult patients
presenting over a 10-year period [5]; Ching et al. In the abovementioned four studies [1, 4–6],
(2010) reported their experience with 55 patients about 50% of patients had their problem solved
in one-stage repair (however, that included fistula
excision, meatotomy, hair ablation among flaps
and inlay grafts). About 50% were corrected as
multiple stage procedure and complication rate
was 25–33%. One may assume that those with
complicated proximal hypospadias are likely to
require two-stage procedures and suffer more
complications.
There were two interesting observations in
those four studies [1, 4–6]: persistent or recurrent
Fig. 51.2 Category system for adult hypospadias patients chordee was the fourth commonest presentation
(from Ching, C.B., Wood, H.M., Ross, J.H., Gao, T. and in two series after stricture, fistula, and residual
Angermeier, K.W. (2011), “The Cleveland Clinic experi- hypospadias. It is worth noticing that the mean
ence with adult hypospadias patients undergoing repair: age of the patients was 30–37 years. This may
their presentation and a new classification system,” BJU
International, 107: 1142–1146, ©2010, with permission have two explanations: the presence of some cur-
from Wiley and Sons) vature was not a major concern for those adults
Fig. 51.3 Initial presenting complaint (from Ching, C.B., hypospadias patients undergoing repair: their presentation
Wood, H.M., Ross, J.H., Gao, T. and Angermeier, K.W. and a new classification system,” BJU International, 107:
(2011), “The Cleveland Clinic experience with adult 1142–1146 ©2010 with permission from Wiley and Sons)
732 A. T. Hadidi
Table 51.1 The number of patients and commonest four presenting symptoms in four major studies on hypospadias
surgery in adults [1, 4–6]. Ching et al. included spraying in voiding symptoms. UTI urinary tract infection (©Ahmed
Barbagli (2006) Ching (2011) Myers (2012) Hoy (2017)
Duration of study 10 years 16 years 20 years 10 years
Number of patients 60 55 50 93
Commonest four Stricture 56% Voiding symptoms Stricture 72% Stricture 53%
presentations Residual hypospadias 82% Fistula 24% Fistula 20%
43% UTI 36% Residual hypospadias Spraying 20%
Fistula 30% Chordee 24% 14% UTI 19%
Meatal stenosis 18% Infertility 24% Chordee 14%
presenting with complications after hypospa-

dias surgery.
51.7 Management of Adult

Hypospadias
in the Hypospadias Center,
Frankfurt, Germany
Between 2014 and 2020, 47 adults (older than

18 years) with hypospadias were operated on in
Fig. 51.4 There were 47 adult patients who required
hypospadias-related surgery in the Hypospadias Center
the Hypospadias Center by the author. This
classified into three categories (©Ahmed T. Hadidi 2022. included 7 with severe scarring after several
All Rights Reserved) unsuccessful operations (category I), 23 who had
previous surgery but still had adequate healthy
and/or that older techniques that incised and skin and tissues (category II), and 17 with no
excised the hypoplastic urethral plate have better prior surgery (category III) (Fig. 51.4).
long-term outcome than the newer approach of Complication rate was 15% in the form of glans
dorsal plication. dehiscence (3), fistula (2), wound dehiscence (2),
The second interesting observation was and meatal stenosis (1).
that the percentage of balanitis xerotica oblit- The following include examples of adult pre-
erans (BXO) in unoperated adults in the Ching sentations and their management (Figs. 51.5, 51.6,
et al. series was much higher than in adults 51.7, 51.8, 51.9, 51.10, 51.11, 51.12, and 51.13).
Fig. 51.5 A 28-year-old presenting with failed distal repair. The patient had adequate skin and the slit-like adjusted
Mathieu (SLAM Chap. 22) technique was used successfully (©Ahmed T. Hadidi 2022. All Rights Reserved)
Fig. 51.6 A 30-year-old patient presenting with failed distal repair. The DYG technique (Double Y Glanulomeatoplasty,
Chap. 19) was used successfully (©Ahmed T. Hadidi 2022. All Rights Reserved)
Fig. 51.7 A 25-year-old man presenting with ugly meatus and spraying of urine. Advancement and meatoglanulo-
plasty (Chap. 28) was used successfully (©Ahmed T. Hadidi 2022. All Rights Reserved)
734 A. T. Hadidi
Fig. 51.8 A 30-year-old patient presenting with grade II rotation (90°). Fisher’s dartos flap was used successfully
(Chap. 46) (©Ahmed T. Hadidi 2022. All Rights Reserved)
Fig. 51.9 A 26-year-old man presenting with failed prox- mal Thiersch urethroplasty (Chap. 24) were used success-
imal hypospadias and urethral diverticulum. A distal fully (©Ahmed T. Hadidi 2022. All Rights Reserved)
SLAM (slit-like adjusted Mathieu, Chap. 22) and proxi-
Fig. 51.10 A 25-year-old presenting with severe chor- performed in the second stage 6 months later (©Ahmed
dee. Chordee excision was performed (Chap. 8) and the T. Hadidi 2022. All Rights Reserved)
available skin was thinned as a graft and urethroplasty was
Fig. 51.11 A 29-year-old man presenting with severe months later urethroplasty was performed around two
stricture, severe scarring, and urine retention which was catheters with total circumference of F30 (©Ahmed
relieved by suprapubic catheter. The scarring was excised T. Hadidi 2022. All Rights Reserved)
and buccal mucosal graft was applied (Chap. 40). Six
Fig. 51.12 An 18-year-old who underwent 30 operations antibiotics 6 months every year. The scarring was excised
including several flaps, bladder mucosa, and buccal and buccal mucosal graft was applied. Six months later
mucosa graft and presenting with severe stricture, severe urethroplasty was performed around catheter F28
scarring, and frequent urinary tract infection. He was on (©Ahmed T. Hadidi 2022. All Rights Reserved)
736 A. T. Hadidi
51.7.1 Failed Distal Hypospadias 51.7.7 A

n Adult with Severe Stricture
After 30 Operations
51.7.2 Ugly Glans
51.7.8 A
n Adult with Chordee
51.7.3 Adult Rotation and Severe Scarring After Six
Operations
51.7.4 F
ailed Proximal Hypospadias
with Diverticulum
51.8 Conclusion
51.7.5 Adult with Severe Chordee
Adults with complications following childhood
51.7.6 A
n Adult with Stricture hypospadias surgery are a major challenge with
and Severe Scarring high failure rate for reoperative surgery. The
Fig. 51.13 A 19-year-old who underwent six operations catheters (F30) with protective layer from tunica vagina-
in different countries including two buccal mucosal graft. lis. Adults tend to bleed a lot in the scrotum; therefore a
It was felt that the urethral plate was hard and fibrous and scrotal drainage was needed for 3 days. The patient still
a third buccal mucosa may be associated with high risk has some curvature to be sorted out later (©Ahmed
due to the unhealthy fibrotic bed. The scar tissue was T. Hadidi 2022. All Rights Reserved)
excised and a Thiersch technique was used around two
surgeon must perform detailed preoperative ment. Pediatr Emerg Care. 1998; https://doi.
org/10.1097/00006565-199802000-00004.
assessment and identify the source of healthy epi- 3. Bhat A, Bhat M, Kumar V, Kumar R, Mittal R, Saksena
thelium as well as the source of healthy, well vas- G. Comparison of variables affecting the surgical out-
cularized fascial coverage. Two-stage repair is comes of tubularized incised plate urethroplasty in
usually required in patients with proximal hypo- adult and pediatric hypospadias. J Pediatr Urol. 2016;
spadias with severe scarring and persistent 4. Ching CB, Wood HM, Ross JH, Gao T, Angermeier
curvature. KW. The Cleveland Clinic experience with adult
hypospadias patients undergoing repair: their presen-
tation and a new classification system. BJU Int. 2011;
https://doi.org/10.1111/j.1464-410X.2010.09693.x.
References 5. Barbagli G, De Angelis M, Palminteri E, Lazzeri
M. Failed hypospadias repair presenting in
1. Hoy NY, Rourke KF. Better defining the spectrum of adults. Eur Urol. 2006; https://doi.org/10.1016/j.
adult hypospadias: examining the effect of childhood eururo.2006.01.027.
surgery on adult presentation. Urology. 2017; https:// 6. Myers JB, McAninch JW, Erickson BA, Breyer
doi.org/10.1016/j.urology.2016.07.057. BN. Treatment of adults with complications from pre-
2. Hollander JE, Singer AJ, Valentine S. Comparison vious hypospadias surgery. J Urol. 2012; https://doi.
of wound care practices in pediatric and adult org/10.1016/j.juro.2012.04.007.
lacerations repaired in the emergency depart-
Flaps Versus Grafts
52
Michael Sennert and Ahmed T. Hadidi
Abbreviations • “Chordee excision and distal urethroplasty”

(CEDU, Chap. 33)
CEDU Chordee excision and distal • “Lateral-based onlay flap” (LABO, Chap. 30)
urethroplasty
LABO Lateral-based onlay flap or urethral plate-based
PAWG Post-auricular Wolffian Graft
SLAM Slit-like adjusted Mathieu • Thiersch-Duplay (Chap. 24)
TIP Tubularized incised plate • Denis Browne (Chap. 40)
• Tubularized incised plate TIP (Chap. 26)
Common pedicled flaps are usually based on

52.1 Flaps preputial vessels and include Hodgson’s/Perovic
flap (Chap. 38), onlay island flap (Chap. 31),
Flaps used in hypospadias repair may be meatal- transverse preputial island flap (Chap. 34), and
based, e.g.,: Asopa’s double-faced island flap (Chap. 35).
Vascularized fascial flaps have also become
• Mathieu popular as a protective intermediate layer (Chap.
• Mustardé and Barcat (Chap. 21) 43). They help to provide a protective interme-
• “Slit-like adjusted Mathieu” (SLAM, Chap. diate layer and minimize complications.
22) Common examples are tunica vaginalis flap,
• Koyanagi (Chap. 36) preputial subcutaneous flap, and dartos flap. The
• Yoke (Chap. 37) routine use of these vascularized fascial flaps
has significantly improved the results of hypo-
spadias repair and reduced the incidence of
complications to less than 5% in distal hypospa-
dias repair [1, 2].
Additionally flaps can be used for corporal
M. Sennert (*) · A. T. Hadidi straightening (Chap. 8). In hypospadias cases
Hypospadias Center and Pediatric Surgery with deep corpus cavernosum-based chordee, an
Teaching Hospital of the Johann Wolfgang Goethe epithelial covered flap can cover the corporal
University, Offenbach, Frankfurt, Germany defect that is achieved by the transverse cut to
e-mail: Michael.sennert@sana.de correct corporal chordee [3].
740 M. Sennert and A. T. Hadidi
52.2 Grafts Duckett believed that flap repair has an advan-

tage over grafts since the normal blood supply to
Grafts commonly used in hypospadias repair the tissues is intact [4]. This again conforms to
include preputial skin (full thickness or partial plastic surgery principles.
thickness), post-auricular Wolffian graft (PAWG) The known advantage of flaps compared to
(full thickness), abdominal skin (full-thickness or grafts is the intact blood supply which results
dermal grafts following chordee excision), blad- in a better survivability. Another important
der mucosa, and oral mucosa (buccal or labial) advantage is that flaps are unlikely to con-
(Chaps. 39 and 40). tract or scar. The published disadvantage is a
higher rate of diverticulum formation com-
pared to grafts, and it requires experience to
52.3 Flaps Versus Grafts plan the flap properly and maintain good
blood supply.
In Chap. 16, the basic principles, advantages, and The advantages of grafts compared to flaps
disadvantages of flaps and grafts were discussed. are the lower rate of diverticulum formation and
A simple practical maxim was mentioned: its availability when there is little local healthy
tissues available and the relatively easy gathering
and availability even in circumcised redo cases.
“A graft is a non-living tissue that you try
The main disadvantages of grafts are failure of
to bring to life and a flap is a living tissue
graft take, scarring or contracture and shrinkage
that you try not to bring to death.”
even after a few years, and failure of graft to grow
as the penis grows.
In a controversial debate of flaps versus grafts,
This is perhaps somewhat biased but nevertheless Cendron stated that a flap in an experienced sur-
conforms to the basic principles of plastic surgery. geon’s hands
a b
Fig. 52.1 (a) A 17-year-old boy, after a failed buccal mucosa graft. (b) A 2-year-old boy after a failed first-stage flap.
Notice the quality of the ventral penis after a failed graft as compared to a failed flap (courtesy of Ahmed T. Hadidi)
52 Flaps Versus Grafts 741
should survive, grow and thrive in opposition to (1980s), and onlay was the preferred technique in
the uncertainty of a graft and thus is the superior
choice in his opinion [5] (Fig. 52.1).
later years (1990s). Two-thirds of the study group
(95 patients) had the repair performed using
On the other hand, Snodgrass reported that he grafts. Two-thirds of the grafts were done as a
had better outcome using buccal mucosal grafts tube (61 vs. 34 patients). In the flap group (47
in proximal hypospadias as compared with flaps patients), 27 patients had a tube flap and 20 had
[6]. It is clear that the surgeon’s experience plays an onlay flap. The authors concluded that there
an important role in the outcome. was no significant difference in the complication
Several studies evaluated the results of differ- rates of flaps and grafts used to repair proximal
ent techniques. However, in an excellent study, hypospadias. However, in the graft group, there
Powell et al. compared the results of flaps and was a significantly higher proximal stricture rate
grafts in the management of proximal hypospa- when a tube rather than an onlay was used. The
dias [7]. In this retrospective investigation, they study also raised a very important issue, namely,
reviewed the records of 142 patients who had that a significant number of complications (67%)
undergone proximal hypospadias repair between presented more than 1 year after surgery.
1981 and 1997. The techniques used in the repair Since then there has been no prospective
were transverse preputial island flaps used as a investigation with a large sample group directly
tube or onlay and preputial skin grafts used as a comparing flaps to grafts.
tube or onlay. One surgeon operated on 93 Table 52.1 shows the complication rate of
patients (65%). Tube urethroplasty was the rou- tubed flaps and tubed grafts in different series,
tine technique in the earlier years of the study while Table 52.2 shows the complication rate of
Table 52.1 Complication rates (% comp.) for tubed flaps and tubed grafts
Tubed flaps Tubed grafts
Study % comp. Study % comp.
Powell et al. (2000) [7] 33 Powell et al. (2000) [7] 34
Monfort et al. (1983) [8] 42 Hendren and Crooks (1980) [9] 8.9
Bondonny et al. (1984) [10] 19.5 De Sy and Oosterlinck (1981) [11] 27
Barraza et al. (1987) [12] 56 Redman (1983) [13] 30
Sauvage et al. (1987) [14] 31 Shapiro (1984) [15] 20
Harris and Jeffery (1989) [16] 24 Vyas et al. (1987) [17] 39.4
Hollowell et al. (1990) [18] 15 Rober et al. (1990) [19] 50
Rickwood and Anderson (1991) [20] 28 Johal et al. (2006) [21] 18
Kass and Bolong (1990) [22] 4.4 Obara et al. (2020) [23] 25
Wacksman (1986) [24] 11 Badawy et al. (2018) [25] 20
Seleim et al. (2017) [26] 16
Liao et al. (2016) [27] 21
Hadidi (2018) [28] 10
Table 52.2 Complication rates (% comp.) for onlay flaps and onlay grafts
Onlay flaps Onlay grafts
Study % comp. Study % comp.
Powell et al. (2000) [7] 30 Powell et al. (2000) [7] 29
Elder et al. (1987) [29] 6 Vyas et al. (1987) [17] 39.4
Baskin et al. (1994) [30] 8.6 Rober et al. (1990) [19] 38
Hadidi (2012) [31] 5
onlay repairs. The data in the two tables permit the team describes a retrospective analysis in 184
no definite conclusions. The retrospective studies proximal hypospadias cases [33]. An onlay flap
were reported at different times, and different urethroplasty was chosen for primary cases with
suture materials and suture techniques were used. a healthy urethral plate. In redo cases with miss-
The surgeons had different levels of experience, ing foreskin, a buccal mucosa graft was gathered
and selection criteria may have differed. Also, the and in the most severe cases the Koyanagi repair
follow-up period varied from one study to was chosen. With these limitations in mind, the
another. complication rates seem rather even between the
There are other factors that need to be techniques.
addressed when comparing flaps and grafts. Grafts are not only used to reconstruct new
These factors include single- or two-stage repair, urethra; sometimes, skin grafts are needed to
the ease or difficulty of flap or graft preparation, cover the penis. Sources for skin graft to cover
the required period of hospitalization, the the penis include the lower abdominal crease,
duration and degree of mobilization, the method post-auricular skin, and even the groin at the
of urinary diversion, if needed, the comfort of the junction of lower abdomen and thigh (Figs. 52.2
child (e.g., hip spica in case of grafts [32]), and and 52.3).
the degree of inconvenience to the family. Further prospective multicenter studies
Generally speaking a hypospadiologist should involving a large number of patients using stan-
be capable of performing both techniques to pro- dardized operation techniques and suturing tech-
vide the optimal care for the hypospadias patients. niques and materials, with a follow-up period of
This can be seen in various publications. An at least 5 years are needed to permit definite
example would be de Mattos e Silva et al., where conclusions.
a b
c d
Fig. 52.2 (a–d) A child with buried penis who undertaken from the groin to cover the skin defect. (c) After
went improper circumcision. (a) Only the glans can be covering the skin defect and before closing the donor area
seen but the rest of the penis is hidden in the abdomen. (b) at the groin. (d) After complete healing of the wound
After mobilization, the penis looks normal in size but (courtesy of Prof. Ibrahim Ulman)
there is lack of skin to cover it. A large skin graft was
Fig. 52.3 A full-thickness skin graft from the groin was used to cover a defect in the ventral penile skin after five
unsuccessful hypospadias operations (courtesy of Ahmed T. Hadidi)
10. Bondonny JM, Barthaburu D, Vergnes

References P. L’hypospadias balanique et penien les elements de
la malformation: implications therapeutiques et resul-
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vent fistulas in hypospadias surgery. J Urol. Ann Urol. 1984;18:21–7.
1986;136:861–3. 11. De Sy WA, Oosterlinck W. One-stage Hypospadias
2. Snow BW, Cartwright PC, Unger K. Tunica vagi- repair by free full-thickness skin graft and island flap
nalis blanket wrap to prevent urethrocutaneous fis- techniques. Urol Clin North Am. 1981; https://doi.
tula: an 8-year experience. J Urol. 1995; https://doi. org/10.1016/s0094-0143(21)01305-7.
org/10.1097/00005392-199502000-00061. 12. Barraza MA, Roth DR, Terry WJ, Livne PM, Gonzales
3. Braga LHP, Pippi Salle JL, Dave S, Bagli DJ, Lorenzo ET. One-stage reconstruction of moderately severe
AJ, Khoury AE. Outcome analysis of severe chordee hypospadias. J Urol. 1987; https://doi.org/10.1016/
correction using tunica vaginalis as a flap in boys S0022-5347(17)44186-3.
with proximal hypospadias. J Urol. 2007; https://doi. 13. Redman JF. Experience with 60 consecutive hypo-
org/10.1016/j.juro.2007.03.166. spadias repairs using the Horton-Devine tech-
4. Duckett JW. The island flap technique for hypospa- niques. J Urol. 1983; https://doi.org/10.1016/
dias repair. Urol Clin North Am. 1981;8:503–11. S0022-5347(17)51948-5.
5. Cendron M. Flap versus graft 2-stage repair of severe 14. Sauvage P, Rougeron G, Bientz J, Cuvelier
hypospadias with chordee. J Urol. 2015; https://doi. G. L’utilisation du lambeau preputial transverse pedi-
org/10.1016/j.juro.2014.12.064. cule dans la chirurgie de l’hypospadias. Chir Pediatr.
6. Snodgrass WT. Flap versus graft 2-stage repair of 1987;28.
severe hypospadias with chordee. J Urol. 2015; 15. Shapiro SR. Complications of hypospadias
https://doi.org/10.1016/j.juro.2014.12.063. repair. J Urol. 1984; https://doi.org/10.1016/
7. Powell CR, Mcaleer I, Alagiri M, Kaplan S0022-5347(17)50475-9.
GW. Comparison of flaps versus grafts in proxi- 16. Harris DL, Jeffery RS. One-stage repair of hypo-
mal hypospadias surgery. J Urol. 2000; https://doi. spadias using split preputial flaps (Harris): the first
org/10.1016/S0022-5347(05)67762-2. 100 patients treated. Br J Urol. 1989; https://doi.
8. Monfort G, Jean P, Lacoste M. Correction des hypo- org/10.1111/j.1464-410X.1989.tb05227.x.
spadias posterieurs en un temps par lambeau pedicule 17. Vyas PR, Roth DR, Perlmutter AD. Experience with
transversal (intervention de Duckett). A propos de 50 free grafts in urethral reconstruction. J Urol. 1987;
observations. Chir Pediatr. 1983;24:71. https://doi.org/10.1016/S0022-5347(17)44071-7.
9. Hendren WH, Crooks KK. Tubed free skin graft for 18. Hollowell JG, Keating MA, Snyder HM, Duckett
construction of male urethra. J Urol. 1980; https://doi. JW. Preservation of the urethral plate in hypospadias
org/10.1016/S0022-5347(17)56163-7. repair: extended applications and further experience
with the onlay island flap urethroplasty. J Urol. 1990; for better outcome. J Pediatr Urol. 2017; https://doi.
https://doi.org/10.1016/s0022-5347(17)39878-6. org/10.1016/j.jpurol.2016.11.016.
19. Rober PE, Perlmutter AD, Reitelman C. Experience 27. Liao AY, Smith GHH. Urethrocutaneous fistulae
with 81, 1-stage hypospadias/chordee repairs with after hypospadias repair: when do they occur? J
free graft urethroplasties. J Urol. 1990;144:526–9. Paediatr Child Health. 2016; https://doi.org/10.1111/
20. Rickwood AMK, Anderson PAM. One-stage hypo- jpc.13102.
spadias repair: experience of 367 cases. Br J Urol. 28. Hadidi AT. Perineal hypospadias: back to the
1991; https://doi.org/10.1111/j.1464-410X.1991. future chordee Excision & Distal Urethroplasty.
tb15171.x. J Pediatr Urol. 2018; https://doi.org/10.1016/j.
21. Johal NS, Nitkunan T, O’Malley K, Cuckow jpurol.2018.08.014.
PM. The two-stage repair for severe primary hypo- 29. Elder JS, Duckett JW, Snyder HM. Onlay island flap in
spadias. Eur Urol. 2006; https://doi.org/10.1016/j. the repair of mid and distal penile hypospadias with-
eururo.2006.01.002. out chordee. J Urol. 1987; https://doi.org/10.1016/
22. Kass EJ, Bolong D. Single stage hypospadias recon- S0022-5347(17)43152-1.
struction without fistula. J Urol. 1990; https://doi. 30. Baskin LS, Duckett JW, Ueoka K, Seibold J, Snyder
org/10.1016/s0022-5347(17)39510-1. HM. Changing concepts of hypospadias curvature lead
23. Obara K, Hoshii T, Hoshino S, Yamana K, Anraku to more onlay island flap procedures. J Urol. 1994;
T, Maruyama R, Kuroki H, Ishizaki F, Yamazaki https://doi.org/10.1016/S0022-5347(17)34915-7.
H, Tomita Y. Free tube graft urethroplasty for 31. Hadidi AT. Proximal hypospadias with small flat
repair of hypospadias. Urol Int. 2020; https://doi. glans: the lateral-based onlay flap technique. J
org/10.1159/000504146. Pediatr Surg. 2012; https://doi.org/10.1016/j.
24. Wacksman J. Use of the Hodgson XX (modi- jpedsurg.2012.06.027.
fied Asopa) procedure to correct hypospa- 32. Cilento BG, Stock JA, Kaplan GW. Pantaloon spica
dias with chordee: surgical technique and cast: an effective method for postoperative immobili-
results. J Urol. 1986; https://doi.org/10.1016/ zation after free graft hypospadias repair. J Urol. 1997;
S0022-5 347(17)45309-2 . https://doi.org/10.1016/S0022-5347(01)64892-4.
25. Badawy H, Orabi S, Hanno A, Abdelhamid 33. de Mattos e Silva E, Gorduza DB, Catti M, Valmalle
H. Posterior hypospadias: evaluation of a paradigm AF, Demède D, Hameury F, Pierre-Yves M,
shift from single to staged repair. J Pediatr Urol. 2018; Mouriquand P. Outcome of severe hypospadias repair
https://doi.org/10.1016/j.jpurol.2017.07.007. using three different techniques. J Pediatr Urol. 2009;
26. Seleim HM, Morsi H, Elbarbary MM. Neo-yoke https://doi.org/10.1016/j.jpurol.2008.12.010.
repair for severe hypospadias: a simple modification
Single-Stage Versus Two-Stage
Repair 53
Ahmed T. Hadidi
Abbreviations (1979) [7], and Duckett (1980) [8], in which the

preputial skin was used in a vascularized fashion
CEDU Chordee excision and distal for correction, further extended the use of one-
urethroplasty stage repairs. By 1988, it was being suggested
DYG Double Y glanulomeatoplasty that all primary hypospadias should be repaired
LABO Lateral based onlay flap with a single-stage approach [9].
MAGPI Meatal advancement and glanulo-
plasty technique
SLAM Slit-like adjusted Mathieu 53.2 Aim of Hypospadias Surgery
TIP Tubularized incised plate
The ultimate goal of hypospadias surgery is to
have a good-functioning and good-looking penis
for 80 years of life and more. It must be stressed
53.1 Introduction that “good-functioning penis” is the first priority
and means the ability to pass a normal straight
In 1941 Higgins wrote, in a prophetic foreword: stream of urine without straining or drippling or
If, however, one could succeed in remodeling the residual urine in bladder as well as normal sexual
urethra in one operation, the gain would be mani- function with normal sensation, erection, and
fest (Humby 1941). ejaculation. Ideally, the penis should look good,
straight with a slit-like meatus at the tip of the
The first one-stage repair was performed “by glans and minimal scarring. It has become clear
mistake” by Russell of Melbourne in 1900 [1]. that achieving the ultimate goal is more impor-
Humby (1941) [2], Broadbent et al. (1961) [3], tant than the number of “planned operations”
Devine and Horton (1961) [4], and DesPrez et al. required to achieve this goal. How is this goal
(1961) [5] were instrumental in popularizing the best achieved; in one stage, two, or three?
one-stage approach for the correction of hypo-
spadias. Work by Asopa (1971) [6], Standoli
53.3 One-Stage Repair
A. T. Hadidi (*) One-stage procedures are undoubtedly attractive
Department, Sana Klinikum Offenbach, Academic and desirable. They are associated with a shorter
Teaching Hospital of the Johann Wolfgang Goethe hospital stay and are more convenient for both
University, Offenbach, Frankfurt, Germany patient and surgeon alike in the short term. This
748 A. T. Hadidi
can be achieved in most distal forms of hypospa- to stenosis and puckering. Several centers
dias with mild or no chordee. For glanular and reported unsatisfactory experience with the oper-
distal hypospadias, the general concept is to per- ative results in such patients managed with a one-
form a single-stage repair [10]. The most popular stage repair [15–20]. Advocates of two-stage
techniques are double Y glanulomeatoplasty repair believe that the quality of the end results is
(DYG, Chap. 19), advancement (Chap. 28), or better both psychologically and sexually than
meatal advancement and glanuloplasty technique with one-stage procedures.
(MAGPI, Chap. 20) for glanular hypospadias. On the other hand, using a two-stage repair, in
For distal hypospadias, popular techniques more than 500 cases, Durham Smith (1981)
include the slit-like adjusted Mathieu (SLAM, reduced his incidence of fistula formation to less
see Chap. 22), Thiersch (Chap. 24), and tubular- than 3% [21]. Similarly, Greenfield and col-
ized incised plate (TIP, Chap. 26). The lateral- leagues (1994) reported a urethral fistula rate of
based onlay flap (LABO, Chap. 30), tubularized 2.5% with a two-stage modified Belt-Fuqua
incised plate (Chap. 26), and onlay island flaps repair for severe hypospadias with chordee [17].
(Chap. 31) are the common techniques used for In conclusion, patients, with severe proximal
proximal hypospadias without severe chordee. hypospadias, chordee, and a small phallus will
Techniques that correct proximal hypospadias benefit from a two-stage procedure. In such chil-
with severe chordee in one operation include the dren, a two-stage repair may offer a better cos-
bilateral-based flap (BILAB, Chap. 32), Koyanagi metic outcome and a lower complication rate
(Chap. 36), and Yoke (Chap. 37). than a one-stage repair with a free, vascularized,
or composite graft.
The Thiersch-Duplay and the Denis Browne
53.4 Two-Stage Repair technique are described in the following section.
Bracka’s modification of the Nove-Josserand
Most surgeons nowadays favor two-stage repair technique was described in Chap. 40.
for hypospadias with severe chordee more than
30°. They base their argument on several points:
Firstly, dorsal plication does not correct severe 53.5 Thiersch-Duplay Technique
chordee adequately, and there is a high incidence as Modified by Byars (1951)
of recurrence/persistence of chordee [11–14]. and Durham Smith (1981)
This necessitates division of the urethral plate
and the use of two-stage grafts or flaps. 53.5.1 First Stage
Also, one-stage methods have their technical
limitations; thus, a specialized surgeon needs to In the first stage, a circumferential incision is
master a number of unrelated repairs and has to made proximal to the coronal sulcus, the chordee
be capable of the decision-making that goes with is excised, and the penile shaft is degloved
them. On the other hand, some two-stage proce- (Fig. 53.1). Failure to create a straight phallus is
dures may be adapted for most deformities. one of the avoidable complications of hypospa-
Although this is true, most hypospadias surgeons dias surgery. Penile straightening and full
agree that there is no place for the “occasional” removal of chordee must be confirmed by means
hypospadias surgeon in the correction of so- of the artificial erection test. After securely plac-
called “minor” hypospadias. Surgeons should ing an elastic band at the base of the penis, or
have a detailed understanding of the various con- using perineal compression, normal saline is
cepts and surgical techniques and maintain a injected with a 25-gauge butterfly needle placed
clinical workload that is sufficient to obtain con- in one corporeal body to fill the entire organ. This
sistently good results. identifies any restraining fibrous bands that
Other points of argument include the follow- remain. Several tests may be necessary to ensure
ing: the circumferential meatus that results from complete excision. If the penis remains bent
some of the one-stage procedures is more liable despite resection of all chordee, an alternative
53 Single-Stage Versus Two-Stage Repair 749
a b
c d
Fig. 53.1 (a–k). Thiersch-Duplay two-stage repair of tubularized with a running subcuticular stitch of 6-0
hypospadias. (a–e) First stage: (a) A circumferential Vicryl all the way to the tip of the glans. (h, i) A strip of
incision is made proximal to the coronal sulcus. (b) The skin (3–5 mm wide) is then de-epithelialized on one
chordee is excised, and the penile shaft is degloved. (c) side to provide a raw surface of deep dermis. The
The glans is divided deeply in the midline to the tip. (d) medial edge of the shaved flap is brought across the
The dorsal foreskin is unfolded carefully and divided in buried urethroplasty and sutured to fascial tissue
the midline. (e) A midline closure is performed, and the beneath the other flap (double breasting). (j, k) The
midline sutures catch a small portion of Buck’s fascia. skin closure is meticulously performed with nylon
(f–k) Second stage: (f) A 16-mm-diameter strip is mea- without strangulation to allow for the expected postop-
sured, extending to the tip of the glans. (g) The strip is erative swelling
750 A. T. Hadidi
e f

h i
j k

752 A. T. Hadidi
approach may be needed, such as insertion of a least two layers. A strip of skin (3–5 mm wide) is
dermal or tunica vaginalis graft into the shaft or then de-epithelialized on one side to provide a
dorsal plication. raw surface of deep dermis (Fig. 53.1h, i). This is
When it has been ascertained that the penis is achieved by cutting two or three fine longitudinal
straight, the glans is prepared. The glans is either strips with a pair of small “curved-on” scissors.
divided deeply in the midline to the tip or, if the The medial edge of the shaved flap is brought
mucosal groove is deep, this is preserved and across the buried urethroplasty and sutured to
incisions are made just lateral to the groove on fascial tissue beneath the other flap (double
each side (Fig. 53.1c). The dorsal foreskin is breasting) (Fig. 53.1j, k). The skin closure is
unfolded carefully and divided in the midline meticulously performed with nylon without
(Fig. 53.1d). The most distal portion of the strangulation to allow for the expected postopera-
foreskin is rotated into the glanular cleft and
tive swelling. Penoscrotal transposition, if pres-
sutured to the mucosa of the glans with inter- ent, is usually repaired during this stage. A penile
rupted sutures of 6-0 chromic catgut. A midline nerve block is used routinely.
closure is performed, and the midline sutures
catch a small portion of Buck’s fascia. This elimi-
nates dead space and helps to create a groove in 53.5.4 Results
preparation for the second stage (Fig. 53.1e). The
bladder is drained with an F8 Silastic Foley cath- In 1994, Retik et al. reviewed their experience
eter for approximately 5–7 days. with two-stage hypospadias repair [15]. From
1986 to 1993, they treated 1437 children with
hypospadias. Of those, 58 patients (4%) had scro-
53.5.2 Second Stage tal or perineal hypospadias with severe chordee
and a small phallus, often resistant to testosterone
The second stage of the procedure is carried out therapy, which was administered preoperatively
6–12 months later, when the tissues have usually as an ointment or parenterally. Those patients
softened sufficiently and healing is complete. underwent a two-stage surgical repair. The use of
The previously transferred preputial skin is used added subcutaneous tissue or tunica vaginalis
to reconstruct the glans and urethra. A 16-mm- [22] over the neourethra in several instances
diameter strip is measured, extending to the tip of achieved the goal of nonoverlapping suture lines
the glans (Fig. 53.1f). The strip is tubularized and may have offered increased vascularity. The
with a running subcuticular stitch of 6-0 Vicryl penile nerve block and transparent biomembrane
all the way to the tip of the glans (Fig. 53.1g). dressing allowed for early postoperative mobili-
Suture-line tension is reduced before closing this zation. The authors reported excellent functional
layer by generous mobilization and undermining and cosmetic results. In their series, only 3 of 58
of adjacent tissues. children (5%) developed a urethrocutaneous fis-
A urine-tight anastomosis is made and inverted tula. They believed that the final cosmetic out-
toward the lumen whenever possible. Eversion of come in these patients was superior to that in
suture lines increases periurethral reaction, which those who underwent a one-stage repair for less
contributes to both urine leakage and potential severe defects.
fistula or diverticulum formation. The new ure-
thra must be wide enough to avoid strictures.
53.6 Denis Browne Technique
53.5.3 Skin Closure In the Denis Browne technique (Fig. 53.2), the
first stage of surgery is as in the Thiersch-Duplay
The lateral skin edges are mobilized, and the technique. The second stage is completed at
remaining tissue is closed over the repair in at about 4–5 years of age. At that time a perineal
a b
Fig. 53.2 (a-c) Denis Browne technique, second stage. tral surface of the penis. No catheter is placed over the
Lateral skin flaps are approximated over the midline lon- buried strip of skin on the penis
gitudinal strip of skin, which remains attached to the ven-
urethrostomy is performed and a peripheral inci- catheter is placed over the buried strip of skin on
sion is made around a midline strip of skin on the the penis. The perineal catheter is usually
ventral shaft of the penis, extending from the ure- removed on the tenth postoperative day, thus
thral meatus to the inferior portion of the glans. A allowing the perineal fistula to heal
small triangle of the ventral glans is denuded on spontaneously.
each side of the midline. By combining wide Several points of surgical technique are impor-
undermining and a dorsal relaxing incision, lat- tant in this operation. First, an adequate urethral
eral skin flaps can then be approximated over the opening must be provided and the first-stage
midline longitudinal strip of skin, which remains meatotomy is essential in most cases. Second, the
attached to the ventral surface of the penis. No dorsal longitudinal relaxing incision on the shaft
754 A. T. Hadidi
of the penis must not extend past the base of the ally, stricture formation. This technique is con-
penis onto the abdominal wall. Incisions on the structed on sound principles and will produce
shaft of the penis heal with wide, flat, pliable scar consistent results when meticulous technique is
unless they encroach upon the abdominal wall. utilized. Sir Denis Browne considered any modi-
At this point, a hypertrophied, keloid-like scar fication of his original procedure to be the cause
will commonly occur and may cause a disap- of fistulae and failure. It is important that the
pointing result. Third, the longitudinal strip of relaxing sutures should not be left in place too
skin which is buried must be wide enough to long. The glass beads should allow at least 0.5 cm
allow circumferential growth of an adequate, new of suture free on each side at the time of applica-
urethra. This may vary with the size of the patient tion, and the beads should be broken and removed
but should never be less than 1 cm in width, even if edema begins to bury them in the skin. Culp
in a child. In an adult, this should measure 1.5 cm (1959) modified this procedure by tying nylon
or more. Fourth, to prevent stenosis at this site mattress sutures over longitudinal bolsters of rub-
during healing, the incision should extend well ber tubing to cut down on necrosis of the skin
around the tube at the distal end of the normal caused by the small pressure points of the sutures
urethra, leaving a circumferential cuff of normal [23]. All sutures should be removed in 7 days.
skin measuring 4–6 mm in all directions. Fifth,
the incisions made on the glans penis should
extend distally as far as possible to elongate the 53.7 One and Half Stages Repair
urethra. This entire technique is simplified by
bringing as much tissue as possible to the distal Hadidi (2018) described the chordee excision
ventral surface of the penis and glans during the and distal urethroplasty (CEDU) technique for
first stage of repair. Sixth, tension-relieving perineal hypospadias with satisfactory results
sutures should be used when the lateral flaps are [24]. In this technique, he corrected the severe
brought over the buried strip of skin. Browne chordee, brought the foreskin ventrally from both
secured these sutures with glass beads and sides, and reconstructed distal new urethra in one
crimped metal sleeves so that if necessary the operation, leaving the original meatus in the
sutures could be loosened from time to time as perineum intact as a “natural perineal fistula.”
edema occurred. The perineal fistula is closed some 3–6 months
later (Chap. 33).
53.6.1 Results
References
The Denis Browne operation, while probably the
most popular method to correct hypospadias in 1. Russell RH. Operation for severe hypospadias. Br Med
J. 1900; https://doi.org/10.1136/bmj.2.2081.1432-a.
the 1950s and 1960s, was not perfect. Culp (1959) 2. Humby G, Higgins TT. A one-stage operation for
reported a series of complications requiring reop- hypospadias. Br J Surg. 1941; https://doi.org/10.1002/
eration in 22–44% of all cases in which the Denis bjs.18002911312.
Browne technique had been used [23]. Most 3. Broadbent TR, Woolf RM, Toksu E. Hypospadias:
one-stage repair. Plast Reconstr Surg. 1961; https://
authors reported a 10–30% incidence of fistula doi.org/10.1097/00006534-196102000-00002.
formation. The usual complications of this opera- 4. Devine CJ, Horton CE. A one stage hypospa-
tion consist of fistula formation, retrogression of dias repair. J Urol. 1961; https://doi.org/10.1016/
the urethral meatus from the tip, and, occasion- S0022-5347(17)65301-1.
5. DesPrez JD, Persky L, Kiehn CL. One-stage 15. Retik AB, Bauer SB, Mandell J, Peters CA, Colodny
repair of hypospadias by island flap tech- A, Atala A. Management of severe hypospadias with
nique. Plast Reconstr Surg. 1961; https://doi. a 2-stage repair. J Urol. 1994. https://doi.org/10.1016/
org/10.1097/00006534-196110000-00007. s0022-5347(17)32697-6.
6. Asopa HS, Elhence IP, Atri SP, Bansal NK. One stage 16. Kroovand RL, Perlmutter AD. Extended urethro-
correction of penile hypospadias using a foreskin tube. plasty in hypospadias. A description and evalua-
A preliminary report. Int Surg. 1971;55(6):435–40. tion of a two-stage technique. Urol Clin North Am.
7. Standoli L. Correzione dell’ipospadia in un unico 1980;7(2):431–6.
tempo: tecnica dell’uretroplastica con lembo ad isola 17. Greenfield SP, Sadler BT, Wan J. Two-stage repair for
prepuziale. Rass Ital Chir Pediat. 1979;21:82. severe hypospadias. J Urol. 1994;152:498–501.
8. Duckett JW. Transverse preputial island flap tech- 18. Bracka A. A versatile two-stage hypospadias repair.
nique for repair of severe hypospadias. Urol Clin Br J Plast Surg. 1995; https://doi.org/10.1016/
North Am. 1980;7(2):423–30. S0007-1226(95)90023-3.
9. Sadove RC, Horton CE, McRoberts JW. The new era 19. Bracka A. Hypospadias repair: the two-stage alterna-
of hypospadias surgery. Clin Plast Surg. 1988; https:// tive. Br J Urol. 1995; https://doi.org/10.1111/j.1464-
doi.org/10.1016/s0094-1298(20)31411-5. 410X.1995.tb07815.x.
10. Borer JG, Retik AB. Current trends in hypospa- 20. Long CJ, Chu DI, Tenney RW, Morris AR, Weiss
dias repair. Urol Clin North Am. 1999; https://doi. DA, Shukla AR, Srinivasan AK, Zderic SA, Kolon
org/10.1016/S0094-0143(99)80004-4. TF, Canning DA. Intermediate-term follow up of
11. Vandersteen DR, Husmann DA. Late onset recurrent proximal hypospadias repair reveals high compli-
penile chordee after successful correction at hypo- cation rate. J Urol. 2017; https://doi.org/10.1016/j.
spadias repair. J Urol. 1998; https://doi.org/10.1016/ juro.2016.11.054.
S0022-5347(01)62716-2. 21. Smith ED. Durham Smith repair of hypospadias. Urol
12. Acimi S. Commentary: results of onlay preputial flap Clin North Am. 1981;8(3):451–5.
urethroplasty for the single-stage repair of mid-and 22. Snow BW. Use of tunica vaginalis to prevent fistulas
proximal hypospadias. Front Pediatr. 2018; https:// in hypospadias surgery. J Urol. 1986;136:861–3.
doi.org/10.3389/fped.2018.00129. 23. Culp OS. Experiences with 200 hypospadiacs; evolu-
13. Huang LQ, Ge Z, Ma G. Commentary: recurrent tion of a therapeutic plan. Surg Clin North Am. 1959;
chordee in 59 adolescents and young adults following https://doi.org/10.1016/S0039-6109(16)35840-6.
childhood hypospadias repair. J Pediatr Urol. 2020; 24. Hadidi AT. Perineal hypospadias: back to the
https://doi.org/10.1016/j.jpurol.2020.06.005. future Chordee Excision & Distal Urethroplasty.
14. Abosena W, Talab SS, Hanna MK. Recurrent chordee J Pediatr Urol. 2018; https://doi.org/10.1016/j.
in 59 adolescents and young adults following child- jpurol.2018.08.014.
hood hypospadias repair. J Pediatr Urol. 2020; https://
doi.org/10.1016/j.jpurol.2019.11.013.
Stenting Versus No Stenting
54
Ahmed T. Hadidi
Abbreviations neourethra, reduce tissue reaction, and provide

patient comfort. On the other hand, disadvan-
CEDU Chordee excision and distal tages of catheter drainage or stenting include the
urethroplasty length of hospital stay (4–8 days), decreased
MAGPI Meatal Advancement Glanuloplasty patient mobility, bladder spasms, and increased
Incorporated potential for infection. Other complications
TIP Tubularized incised plate include migration into the bladder and mechani-
cal catheter problems that may create inadequate
drainage and even result in pressure ischemia of
the neourethra [2].
54.1 Introduction Urinary diversion following hypospadias sur-
gery has gone through different stages: perineal
Many one-stage repairs for hypospadias have urethrostomy, open suprapubic cystostomy, per-
been developed in the past 25 years. The compli- cutaneous suprapubic cystostomy, transurethral
cation rate has remained stable at approximately catheter drainage of the bladder, and urethral
10% despite the use of optical magnification, fine stenting short of the bladder.
instruments, meticulous hemostasis, and broad- Although Mathieu (1932) in his first descrip-
spectrum antibiotics [1]. tion of his wonderful technique in the Archives
des Maladies du Rein [3] did not leave a catheter,
catheter drainage to divert urine has been consid-
54.2 Urinary Diversion ered necessary to allow for healing of the suture
line so that it may become watertight. A catheter
Inherent to all hypospadias repairs has been the helps to immobilize and drain the neourethra.
use of a bladder drainage catheter or a stent, the Catheter drainage and/or urinary diversion has
purpose of which has been to provide temporary been recommended for 2–14 days [4–9]. An
urinary diversion, permit the repair to become informal hand-raising poll at the 1995 AAP
watertight, immobilize the suture line, drain the Section on Urology Meeting suggested that most
pediatric urologists do stent their non-MAGPI
repairs [10].
A. T. Hadidi (*) In 1986, Mitchell and Kulb described the use
Department, Sana Klinikum Offenbach, Academic of a silicon urethral splint instead of a bladder
Teaching Hospital of the Johann Wolfgang Goethe drainage catheter [1]. They suggested deroofing
University, Offenbach, Frankfurt, Germany the silicon stent. Thus, the stent would expand
758 A. T. Hadidi
and contract according to the degree of edema. ing [14]. In addition to the fact that it was a non-
Yet, it permits voiding and drainage of the neo- random study, they used a transurethral stent for
urethra and even catheterization should thus be 2 days even in the group where they did suprapu-
unnecessary. bic drainage. A third drawback was the use of a
Rabinowitz, writing in 1987, reported outpa- suprapubic diversion in 28 cases of Mathieu
tient catheterless Mathieu repair for distal hypo- repair for distal hypospadias. Many surgeons
spadias in 59 boys, with an excellent cosmetic might consider this an unnecessarily invasive
and functional outcome and few complications procedure. However, they concluded from their
[2]. He concluded that urinary diversion was study that “the incidence of complications with
unnecessary. transurethral stenting (26.5%) was significantly
In 1993 Wheeler and colleagues, in a retro- higher than with suprapubic drainage (10.7%;
spective study, compared a group of 10 boys with p < 0.05).” They also recommended “the use of
a bladder stent following Mathieu repair with suprapubic diversion in all hypospadias repair
another group of 11 boys without a stent f ollowing because it provides lower complication rates and
the same procedure [11]. There was no statistical is more comfortable for children during the post-
difference in outcome or in complications. Thus, operative period.” Using a suprapubic drainage in
they supported Rabinowitz’ conclusion that distal hypospadias repair goes a little too far in
stenting is not mandatory in Mathieu repair. the author’s opinion.
In 1994, Buson et al. reported their experience It must be emphasized that suprapubic cathe-
with Mathieu repair for distal hypospadias in a terization is of particular importance when deal-
nonrandom study that included 65 patients with ing with hypospadias in adults. Adults require
stents and 37 patients without stents [12]. They longer time to heal than in children, pass urine
reported a statistically significant difference with greater force that may disrupt the suture
(p < 0.05) between the group with stents (compli- line. Therefore it is important to maintain urinary
cation rate 4.6%) and the group without stenting diversion for a long period to ensure adequate
(18.9%). They concluded that “the use of a mul- healing of the new urethra.
tiperforated silicone urethral stent is advanta- Several large series support the performance
geous for the Mathieu operation.” of distal penile hypospadias repair without the
In 1996 Hakim et al., in a multicenter retro- use of a urethral stent [15]. In one large series of
spective study, reviewed 336 boys who had Mathieu repairs with limited use of urethral
undergone Mathieu repair for distal hypospadias stents, there were no urethrocutaneous fistulas
[13]. There were 114 patients with stents and 222 [16].
boys without stents. Interestingly, there were no Recently, successful stent-free hypospadias
instances of urinary retention in the unstented repair has been reported using the tubularized
group, including those patients who received incised plate technique (TIP) [17].
caudal anesthesia. They found that the complica- Hadidi et al. (2003) conducted a prospective
tion rate in the stented group (2.21%) was not randomized study to evaluate the role of stenting
statistically significantly lower than that in the in hypospadias [18]. The study included 100
non-stented group (3.6%; p = 0.756). They sug- patients suffering from different degrees of hypo-
gested that “successful Mathieu hypospadias spadias. The operations were performed by a
repair is independent of the use of a stent.” single surgeon (A.H.), who was notified only at
In 1997 Demirbilek and Atayurt, in a nonran- the end of the operation whether to insert a stent
dom study of 105 consecutive patients with dif- or not. The incidence of complications in the
ferent types of hypospadias, compared suprapubic form of failure of repair, fistula formation, or
diversion and transurethral drainage with stent- meatal stenosis was higher in group B with
54 Stenting Versus No Stenting 759
stenting (10%) than in group A without stenting 54.4 Natural Perineal

(2%). The difference in complication rate was not Urethrotomy
statistically significant (p = 0.092). However, the
incidence of morbidity in the form of edema, Hadidi, in 2018, described the chordee excision
stent block, or urinary retention was statistically and distal urethroplasty (CEDU, Chap. 33) for the
significantly higher in group B with stenting correction of perineal hypospadias with severe
(44%) than in group A without stenting (18%; chordee. In this operation, the severe chordee is
p = 0.005). excised with the appropriate technique, and fore-
Because hypospadias repair is a surface oper- skin flaps are brought ventrally and sutured
ation that does not violate a body cavity, it lends together in the midline and the distal urethra is
itself readily to the outpatient setting. As such, reconstructed. In this technique, the original ure-
there is no place for a complex postoperative thral opening is left intact as a “natural perineal
regimen that restricts the child’s mobility or
interferes with the parents’ general care of the
child [19].
54.3 ome Modern Catheters

S
and Stents
It is recommended to use Tiemann catheters with

a curved tip that would enable the surgeon to
introduce the catheter into the bladder with mini-
mal injury to the urethra and verumontanum
(Fig. 54.1).
In centers where hypospadias surgery is per-
formed as an ambulant procedure, Zaontz stents
Fig. 54.1 Tiemann catheter for transurethral drainage
and Kole stents seem to be popular (Fig. 54.2).
a b
Fig. 54.2 (a) Zaontz stent. (b) Koyle stent

760 A. T. Hadidi
6. Gonzales ET, Veeraraghavan KA, Delaune J. The

management of distal hypospadias with meatal-
based, vascularized flaps. J Urol. 1983; https://doi.
org/10.1016/S0022-5347(17)51950-3.
7. Devine CJJ, Horton CE, Gilbert DA. Hypospadias. In:
Mustardé JC, Jackson IT, editors. Plastic surgery in
infancy and childhood. 3rd ed. Edinburgh: Churchill
Livingstone; 1988. p. 493–509.
8. Man DWK, Vordermark JS, Ransley PG. Experience
with single stage hypospadias reconstruction.
J Pediatr Surg. 1986; https://doi.org/10.1016/
S0022-3468(86)80198-1.
9. Snodgrass W, Koyle M, Manzoni G, Hurwitz R,
Caldamone A, Ehrlich R. Tubularized incised
plate hypospadias repair: results of a multicenter
experience. J Urol. 1996; https://doi.org/10.1016/
S0022-5347(01)65835-X.
10. Freedman AL. Dressings, stents and tubes. In: Ehrlich
RM, Alter GJ, editors. Reconstructive and plastic sur-
gery of the external genitalia: adult and pediatrics. 1st
ed. Philadelphia: Saunders; 1999. p. 159–62.
11. Wheeler RA, Malone PS, Griffiths DM, Burge
DM. The Mathieu operation. Is a urethral stent man-
datory? Br J Urol. 1993; https://doi.org/10.1111/
j.1464-410X.1993.tb16007.x.
12. Buson H, Smiley D, Reinberg Y, Gonzalez R,
Rubinowitz R. Distal hypospadias repair with-
out stents: is it better? J Urol. 1994; https://doi.
org/10.1016/S0022-5347(17)35180-7.
Fig. 54.3 Natural perineal urethrotomy (©Ahmed 13. Hakim S, Merguerian PA, Rabinowitz R, Shortliffe
T. Hadidi 2022. All Rights Reserved) LD, McKenna PH. Outcome analysis of the modified
Mathieu hypospadias repair: comparison of stented
and unstented repairs. J Urol. 1996; https://doi.
urethrotomy” for 3–6 months until the distal ure- org/10.1016/S0022-5347(01)65834-8.
thra has healed completely. The advantage is to 14. Demirbilek S, Atayurt HF. One-stage hypospadias
maintain skin continuity between the flaps used with stent or suprapubic diversion: which is bet-
ter? J Pediatr Surg. 1997; https://doi.org/10.1016/
for urethroplasty and the normal skin, early mobi- S0022-3468(97)90511-X.
lization of the patient, less pain and discomfort for 15. Borer JG, Retik AB. Current trends in hypospa-
the patient, and avoidance of possible complica- dias repair. Urol Clin North Am. 1999; https://doi.
tions of catheters and stents (Fig. 54.3). org/10.1016/S0094-0143(99)80004-4.
16. Retik AB, Mandell J, Bauer SB, Atala A. Meatal
based hypospadias repair with the use of a dorsal
subcutaneous flap to prevent urethrocutane-
References ous fistula. J Urol. 1994; https://doi.org/10.1016/
S0022-5347(17)32555-7.
1. Mitchell ME, Kulb TB. Hypospadias repair without 17. Steckler RE, Zaontz MR, Hulbert WC, et al. Stent-
a bladder drainage catheter. J Urol. 1986; https://doi. free Thiersch-Duplay hypospadias repair with the
org/10.1016/S0022-5347(17)45626-6. Snodgrass modification. J Urol. 1997; https://doi.
2. Rabinowitz R. Outpatient catheterless modified org/10.1016/S0022-5347(01)64417-3.
Mathieu hypospadias repair. J Urol. 1987; https://doi. 18. Hadidi AT, Abdala N, Kaddah S. Hypospadias
org/10.1016/s0022-5347(17)43506-3. repair: is stenting important? Kasr El Aini J Surg.
3. Mathieu P. Traitement en un temps de l’hypospade 2003;4:37–44.
balanique et juxta-balanique. J Chir. 1932;39:481. 19. Burbige KA, Connor JP. Postoperative management
4. Wacksman J. Modification of the one-stage flip-flap of hypospadias. In: Ehrlich RM, Alter GJ, editors.
procedure to repair distal penile hypospadias. Urol Reconstructive and plastic surgery of the external
Clin North Am. 1981;8:527–30. genitalia, adult and pediatric. 1st ed. Philadelphia:
5. Kim SH, Hardy Hendren W. Repair of mild hypospa- Saunders; 1999. p. 163.
dias. J Pediatr Surg. 1981;16:806–11.
Dressing Versus No Dressing
55
Ahmed T. Hadidi
55.1 Introduction cells at the edges of the wound begin to divide

and migrate across the wound surface (Chap. 16).
How important is the use of dressings in hypo- By 48 h after closure, deeper structures are com-
spadias surgery? Cromie and Bellinger (1981) pletely sealed from the external environment.
gave a questionnaire to 45 hypospadias surgeons
in the USA [1]:
…85 to 95 percent felt the dressing was of impor- “Dressings over closed wounds should be
tance. Its major effect is manifested by immobili- removed on the third or fourth postoperative
zation of the penis and prevention of edema. The day. Dressings should be removed earlier if
ideal dressing would be supple and immobile, but
they are wet because soaked dressings
generally a firm-immobile dressing is acceptable.
increase bacterial contamination of the
Most individuals responding to the question- wound” (Mulvihill and Pellegrini 1994) [2].
naire felt that the dressing should not be changed
and that it was unrelated to the occurrence of skin
slough or infection. Most dressings were left in
place for more than 3 days, and immobilization 55.3 Sources of Dressing
with close attention to postoperative edema was Contamination
strongly recommended by the respondents.
Dressings following hypospadias surgery are
likely to become wet from four different sources:
55.2 ound Healing and Duration
W urine dripping through the urethra (even if diver-
of Dressing sion is used), exudate from wound edges, soiling
following bowel movements (if permeable or
If we review the process of wound healing, within semipermeable dressings are used), and skin
hours after the wound is closed, the wound space sweating, especially if impermeable dressings
fills with an inflammatory exudate. Epidermal are used. In infants and young children, dressing
is frequently contaminated with stool when the
baby passes bowel motion.
A. T. Hadidi (*)
762 A. T. Hadidi
55.4 Advantages Duckett (1996) recommended the use of

and Disadvantages Tegaderm for 48 h in distal forms of hypospadias
of Dressing and for 72 h in other types of hypospadias sur-
gery with urinary diversion [10].
Understandably, dressing limits the degree of In 1965, Hermann published a report of a
edema postoperatively. However, dressing may study in which all dressings from clean wounds
also decrease blood flow to the penis carrying were removed on the second postoperative day
oxygen, nutrients, inflammatory cells, chemotac- with no evidence of an increased incidence of
tic substances, and growth factors which are wound sepsis [11]:
important for healing. Surgeons are usually con- What then is the explanation of our observation
cerned that edema may be that severe to cut that postoperative dressings may be removed from
through the repair and stitches. In practice, the a wound healing per primum on the second day,
author has only experienced this in cases of without increasing the incidence of infection? The
answer must lie in the fact that wound edges, care-
severe infection. Otherwise, edema is unlikely to fully approximated, are sufficiently sealed by
cut through the stitches. coagulum and overlying epithelial regrowth to
resist contamination. Of additional importance, it
would seem, is the fact that an exposed wound is a
dry wound and few bacteria retain their vitality on
55.5 Types of Dressings a dry surface.
If the use of a dressing is indeed important, as Howells and Young (1966) progressed one
held by the many surgeons who advocate it, what step further and introduced the concept of com-
is the best kind of dressing? Unfortunately, the pletely undressed surgical wounds [12]. They
ideal dressing for hypospadias repair remains reported 105 widely varied surgical cases in
elusive, to judge by the many varieties of dress- which, after careful closure of the incision, the
ing currently in use [3, 4]. patient’s gown was simply pulled down over the
Reviewing the type of hypospadias dressings wound. The frequency of wound infection was
described in the literature, a great diversity 4%, comparable with the incidence of infection
becomes apparent with regard to the degree of in clean wounds.
concealment and the type of material and tech- Law and Ellis randomized 170 consecutive
nique used. Horton et al. (1980) are strong advo- patients undergoing either inguinal hernia repair
cates of unconcealing dressing [5]. Falkowski or high saphenous ligation to one of three surgical
and Firlit (1980) favored the totally concealing options: a dry dressing of gauze, a polyurethane
type of dressing [6]. However, the partially con- film dressing (Opsite®), and an immediate expo-
cealing dressing is the most popular type and has sure [13]. There was no difference in dressing
many variations. W. Campbell (1980, personal comfort or dressing preference among the differ-
communication) wrapped the penis with elastic ent groups, and the quality of the final scar was
foam and passed a small intestinal straight needle also not different. There was, however, a higher
transversely through the glans, resting it on top of infection rate in the polyurethane (Opsite®) group,
the foam dressing. Another variation is the use of although the difference did not attain statistical
a styrofoam cup placed over the standard par- significance. This was probably due to the moist
tially concealing dressing; this is secured by the environment beneath the dressing [13].
use of the glandular traction stitch, which is then In hypospadias surgery, the main purpose of a
taped to the sides of the cup. Falkowski and Firlit dressing is to immobilize the penis, minimize
described the X-shaped elastic dressing in 1980 edema, and prevent hematoma formation [14].
[6]. Silicone foam dressings were very popular Van Savage et al., in 2000, conducted a ran-
after they were first described by De Sy and domized prospective study of dressing versus no
Oosterlinck (1982) [7]. Currently, many surgeons dressing for hypospadias repair [15]. They
use simple methods of fixation such as Tegaderm showed that the success rate for hypospadias sur-
[8], Opsite® spray [4], or Dermolite II tape [9]. gery that preserves the urethral plate is indepen-
55 Dressing Versus No Dressing 763
dent of dressing usage. They concluded that 3. Redman JF, Smith JP. Surgical dressing for hypospa-
dias repair. Urology. 1974;4:739–40.
dressings may not be indicated for all types of 4. Tan KK, Reid CD. A simple penile dressing following
hypospadias repairs. hypospadias surgery. Br J Plast Surg. 1990; https://
In 2001 McLorie et al., in a prospective study, doi.org/10.1016/0007-1226(90)90134-L.
evaluated the role of dressing in hypospadias 5. Horton CE, Devine CJ, Graham JK. Fistulas of the
penile urethra. Plast Reconstr Surg. 1980; https://doi.
repair [16]. They showed that the surgical out- org/10.1097/00006534-198066030-00017.
come and rate of complications were not com- 6. Falkowski WS, Firlit CF. Hypospadias surgery: the
promised without a postoperative dressing. They X-shaped elastic dressing. J Urol. 1980; https://doi.
concluded that “an absent dressing simplified org/10.1016/S0022-5347(17)56187-X.
7. De Sy WA, Oosterlinck W. Silicone foam elastomer:
postoperative ambulatory parent delivered home a significant improvement in postoperative penile
care.” They recommended that dressings should dressing. J Urol. 1982; https://doi.org/10.1016/
be omitted from routine use after hypospadias S0022-5347(17)52744-5.
repair. 8. Freedman AL. Dressings, stents and tubes. In: Ehrlich
RM, Alter GJ, editors. Reconstructive and plastic sur-
Hadidi et al. (2003) conducted a prospective gery of the external genitalia, adult and pediatric. 1st
randomized study to assess the role of dressings ed. Philadelphia: Saunders; 1999. p. 159–62.
in hypospadias surgery. The study concluded that 9. Redman JF. A dressing technique that facilitates outpa-
repair of hypospadias without a dressing offers tient hypospadias surgery. Urology. 1991;37:248–50.
statistically significantly better results than when JY, Grayhack JT, Howards SS, Duckett JW, editors.
using a dressing (p = 0.014). This may confirm Adult and pediatric urology. 3rd ed. St. Louis: Mosby-
Hermann’s idea that “an exposed wound is a dry Year Book; 1996. p. 2549–89.
wound and few bacteria retain their vitality on a 11. Hermann RE, Flowers RS, Wasylenki EW. Early
exposure in the management of the postoperative
dry surface” [11]. The study suggested that the wound. Surg Gynecol Obstet. 1965; https://doi.
higher incidence of complications in the dressing org/10.1097/00006534-196507000-00035.
group may be due to the fact that the wound inev- 12. Howells CHL, Young HB. A study of completely
itably becomes wet due to the four potential undressed surgical wounds. Br J Surg. 1966; https://
doi.org/10.1002/bjs.1800530514.
sources, i.e., urine, ooze, sweat, and stools. 13. Law NW, Ellis H. Exposure of the wound—a safe
In the Hypospadias Center in Frankfurt, economy in the NHS. Postgrad Med J. 1987; https://
Germany, we use a simple dressing for 1–2 days doi.org/10.1136/pgmj.63.735.27.
in distal forms of hypospadias and for 7–10 days 14. Gaylis FD, Zaontz MR, Dalton D, Sugar EC, Maizels
M. Silicone foam dressing for penis after reconstruc-
for proximal forms of hypospadias as long as it is tive pediatric surgery. Urology. 1989; https://doi.
dry. The dressing is removed earlier when it org/10.1016/0090-4295(89)90268-9.
becomes wet (personal communication). 15. Van Savage JG, Palanca LG, Slaughenhoupt BL. A
prospective randomized trial of dressings versus no
dressings for hypospadias repair. J Urol. 2000; https://
doi.org/10.1097/00005392-200009020-00015.
References 16. McLorie G, Joyner B, Herz D, McCallum J, Bagli D,
Merguerian P, Khoury A. A prospective randomized
1. Cromie WJ, Bellinger MF. Hypospadias dressings and clinical trial to evaluate methods of postoperative care
diversions. Urol Clin North Am. 1981;8(3):545–58. of hypospadias. J Urol. 2001; https://doi.org/10.1016/
2. Mulvihill SJ, Pellegrini CA. Current surgical diagno- s0022-5347(05)66388-4.
sis and treatment. 10th ed. Connecticut: Appleton &
Lange; 1994.
Editorial Overview of the Current
Management of Hypospadias 56
Ahmed T. Hadidi
Abbreviations There is still no place for the occasional hypo-

spadias surgeon, even for the so-called “minor”
BILAB Bilateral based flap hypospadias. Surgeons must have a detailed
CEDU Chordee excision and distal understanding of the various basic principles and
urethroplasty surgical techniques and maintain a clinical work-
DYG Double Y glanulomeatoplasty load that is sufficient to obtain consistently good
FUO Functional urethral obstruction results.
GUD Glandular urethral disassembly There has been the impression that distal forms
LABO Lateral-based onlay flap of hypospadias have become straightforward and
MAGPI Meatal advancement glanuloplasty easy to correct with minimal or no complications.
incorporated This is unfortunately far from the truth, as we still
MCGU Meatus-chordee-glans width and receive and operate many patients with failed dis-
shape-urethral plate quality tal hypospadias, often operated 3–6 times without
PDS Polydioxanone success and may even have to live with suprapu-
SLAM Slit-like adjusted mathieu bic catheter until referred to a specialized hypo-
TALE Tunica albuginea longitudinal spadias center. Fistula was considered the
excision commonest complication after hypospadias
TIP Tubularized incised plate repair. One is seeing an alarming increase in the
incidence of meatal stenosis and urethral stricture
due to the adoption of some popular techniques.
Part III (Chaps. 57–64) of the book focuses on dif-
56.1 Introduction ferent complications encountered after hypospa-
dias surgery with a chapter dedicated to the newly
When writing this overview, I was wondering encountered complication of functional urethral
how much has changed since we wrote the same obstruction (FUO) (Chap. 59).
chapter some 20 years ago? A lot! Long-term follow-up studies showed that “we
are not as good as we think” [1], and yes, unfortu-
nately “we are not always keeping our promises”
[2]. It was mentioned in the overview in the first
A. T. Hadidi (*) edition that “for proximal hypospadias, the
Department, Sana Klinikum Offenbach, Academic expected incidence of complications is less than
Teaching Hospital of the Johann Wolfgang Goethe 20%” [3]. Long-term follow-up studies showed
University, Offenbach, Frankfurt, Germany that the complication rate for proximal hypospa-
766 A. T. Hadidi
dias is in fact more than 50%. Chap. 66 deals with hypospadias (Chap. 6), the pathogenesis of hypo-
long-term follow-up. Management of hypospadias spadias (Chap. 7), and the mechanism of chordee
in adults is discussed in Chap. 51 and psychologi- (Chap. 8). It became clear that dorsal plication is
cal adjustment in adults is reviewed in Chap. 67. associated with high incidence of recurrence and
Better understanding of the healing process it is only used nowadays in mild curvature. New
and objective analysis of the available data and techniques have evolved including the TALE
follow-up of adults’ functional and cosmetic out- (tunica albuginea longitudinal excision) tech-
comes have contributed to major changes in the nique and multiple corporotomies or fairy cuts
management of hypospadias. These changes (Chap. 8).
include:
1. Hypospadias classification 56.1.4 T

wo-Stage Repair for Severe
2. The urethral plate Proximal and Perineal
3. Chordee management Hypospadias
4. Two-stage repair for severe proximal and per-
ineal hypospadias Objective assessment of surgical management of
5. The use of grafts more than flaps proximal hypospadias with severe chordee and
6. Protective intermediate layers perineal hypospadias (grade IV hypospadias)
7. Preoperative hormone therapy showed the advantages of two-stage correction
8. Technical surgical details using flaps (Chaps. 32 and 33) or grafts as
9. Operative techniques described by Cloutier/Bracka [4–6] (Chap. 40).
56.1.1 Hypospadias Classification 56.1.5 T

he Use of Grafts Rather Than
Flaps
Time has come to use a single classification that
should be used by all hypospadias surgeons that For complicated hypospadias and the previously
should be simple and easy for the surgeon as well called “crippled hypospadias” or grade V, the use
as for the patient and include all important factors of buccal mucosa has proven its superiority over
and not just the location of the meatus. The flaps (Chaps. 27 and 40).
Hypospadias International Classification
(MCGU) may be such a classification (Chap. 9).
56.1.6 Protective Intermediate
Layers
56.1.2 The Urethral Plate
Protective intermediate layers to cover the new
The urethral plate is the basic structure used to urethroplasty have become the standard.
reconstruct the new urethra. It has become clear Preputial dartos fascia is the standard to cover
that the width and quality of the urethral plate distal urethroplasty, and tunica vaginalis flap is
play an important role in the outcome of hypo- now the standard to cover proximal and perineal
spadias and it may be wise to augment a thin or urethroplasty. Classic types of protective inter-
poor urethral plate (Chaps. 8 and 27). mediate layer include Smith’s de-epithelialized
skin, Snow’s tunica vaginalis wrap from the tes-
ticular coverings, Retik’s dorsal subcutaneous
56.1.3 Chordee Management flap from the foreskin, Motiwala’s dartos flap
from the scrotum, and the external spermatic fas-
There has been major advances in the under- cia flap. New protective layers have evolved
standing of the normal development of the human including the use of bifurcated corpus spongio-
male urethra (Chap. 3), the morphology of sum whenever possible, the use of dartos fascia
56 Editorial Overview of the Current Management of Hypospadias 767
from the proximal shaft of the penis, and the use 56.1.9 Operative Techniques
of fibrin sealants (Chap. 43).
Several modifications of the already existing
techniques have emerged during the past
56.1.7 Preoperative Hormone 20 years! We seldom perform any operation the
Therapy same way we used to do some 20 years ago. One
important message is to reconstruct urethra with
There is strong evidence that preoperative hor- wide caliber as this will have a major impact on
mone stimulation increases the size of the penis the urine flow (Chap. 17).
and the glans in the majority of children with
small penis (Chap. 12). One report suggested
Surgery for hypospadias repair is a delicate
that preoperative hormone therapy increases
art. The surgeon should not compromise on
complications [7]. However this report instead
the result just because certain techniques
of comparing the outcome in patients with
are less prone to complications than others.
small glans to those who received hormone to
In other words, the surgeon should use the
increase the size of the glans, they compared
technique that is suitable for the deformity
the outcome of patients who received hormone
rather than making the hypospadiac defor-
stimulation with the outcome in patients who
mity suit the repair he prefers. We do not
have large glans and penis which makes the
believe that one should use a technique that
report nonconclusive. In the Hypospadias
shortens an already short penis just because
Center in Frankfurt, we use preoperative hor-
it has a lower complication rate. By the
mone stimulation routinely in proximal and
same reasoning, one should not shorten the
perineal hypospadias when the glans width is
penis dorsally to correct the ventral chor-
less than 12 mm.
dee and, lastly, one should not leave a raw
surface if a fully epithelialized neourethra
can be achieved.
56.1.8 Technical Surgical Details
The composition of suture material and suturing

technique and the use of magnification are vari- 56.1.9.1 Techniques for
ables that have had a significant impact on the Glanuloplasty and
results. Ulman et al. (1997) documented a statis- Meatoplasty (Grade I)
tically significant lower fistula rate (4.9% vs. The aim of hypospadias surgery today is to
16.6%) for subcuticular repair than for a full- achieve a normal functioning and looking glans
thickness (through and through) technique [8]. and meatus following hypospadias surgery.
Chromic catgut is favored for the skin. Procedures that help to achieve this aim include
Polyglycolic acid (Dexon) or polyglactin 910 the DYG (double Y glanulomeatoplasty) (Chap.
(Vicryl) are favored for urethroplasty. 19), MAGPI (meatal advancement glanuloplasty
Polydioxanone (PDS) may react with urine and incorporated) (Chap. 20), dorsal inlay graft
has not been advised for hypospadias surgery (Chap. 27), advancement (Chap. 28), and GUD
[9–11]. Optical magnification is considered (glandular urethral disassembly) (Chap. 29).
essential by most hypospadias surgeons. The
power of magnification varies, according to the 56.1.9.2 Techniques for Distal
surgeon’s preference, from 1.5× to operative Hypospadias (Grade II)
microscope power. The importance of using fine, For distal hypospadias, the general concept is to
delicate instruments cannot be overemphasized perform a single-stage repair [12]. The most pop-
(Chap. 17). ular techniques are the SLAM technique (slit-like
768 A. T. Hadidi
Fig. 56.1 Algorithm for

primary hypospadias
repair (©Ahmed
T. Hadidi 2022. All
Rights Reserved)
adjusted Mathieu, Chap. 22), Thiersch-Duplay

References
(Chap. 24), and the TIP (tubularized incised
plate) (Chap. 26). 1. Long CJ, Canning DA. Hypospadias: are we as good
as we think when we correct proximal hypospa-
56.1.9.3 Techniques for Proximal dias? J Pediatr Urol. 2016; https://doi.org/10.1016/j.
jpurol.2016.05.002.
Hypospadias Without
2. Long CJ, Canning DA. Proximal hypospa-
Chordee (Grade III) dias: we aren’t always keeping our promises.
The most popular techniques are the LABO F1000Research. 2016; https://doi.org/10.12688/
(lateral-based onlay flap) technique (Chap. 30), f1000research.9230.1.
3. Keating MA, Rich MA. Onlay and tubularized pre-
onlay island flap (Chap. 31), and the TIP (tubu-
putial island flaps. In: Ehrlich RM, Alter GJ, editors.
larized incised plate) technique (Chap. 26). Reconstructive and plastic surgery of the external
genitalia, adult and pediatric. Philadelphia: Saunders;
56.1.9.4 Techniques for Perineal 1999. p. 77.
4. Cloutier AM. A method for hypospadias
Hypospadias (Grade IV)
The common techniques include the BILAB org/10.1097/00006534-196209000-00007.
(bilateral-based flap, Chap. 32), CEDU (chordee 5. Bracka A. Hypospadias repair: the two-stage alterna-
excision and distal urethroplasty, Chap. 33), tive. Br J Urol. 1995; https://doi.org/10.1111/j.1464-
410X.1995.tb07815.x.
Cloutier/Bracka two-stage repair (Chap. 40), and
6. Bracka A. A versatile two-stage hypospadias repair.
Koyanagi (Chap. 36). Other one-stage techniques Br J Plast Surg. 1995; https://doi.org/10.1016/
include tubularized preputial island flap (Chap. S0007-1226(95)90023-3.
34), modified Asopa (Chap. 35), Yoke (Chap. 37), 7. Snodgrass WT, Bush N. Hypospadias. In: Docimo
SG, Canning D, Khoury A, Salle JLP, editors. Kelalis-
and longitudinal flap (Chap. 38).
King-Belman textbook of clinical pediatric urology.
6th ed. London: CRC Press; 2018. p. 1249.
8. Ulman I, Erikçi V, Avanoǧlu A, Gökdemir
56.2 Protocol of Management A. The effect of suturing technique and mate-
rial on complication rate following hypospa-
in the Hypospadias Center, dias repair. Eur J Pediatr Surg. 1997; https://doi.
Frankfurt org/10.1055/s-2008-1071079.
9. Bickerton MW, Duckett JW. Suture material and
To conclude this chapter, the editor would like to wound healing. In: AUA update series, vol. 3.
Houston: Lesson; 1984.
mention the protocol of management in the
10. El-Mahrouky A, McElhaney J, Bartone FF, King L. In
Hypospadias Center, Frankfurt: vitro comparison of the properties of polydioxanone,
For glanular hypospadias with mobile meatus polyglycolic acid and catgut sutures in sterile and
or cleft glans, we prefer to use the DYG tech- infected urine. J Urol. 1987; https://doi.org/10.1016/
S0022-5347(17)43415-X.
nique. For distal hypospadias, we prefer to use
the SLAM technique. We have adopted the JY, Grayhack JT, Howards SS, Duckett JW, editors.
LABO technique for proximal hypospadias with- Adult and pediatric urology. 3rd ed. St. Louis: Mosby-
out chordee. The CEDU technique has become Year Book; 1996. p. 2549–89.
12. Borer JG, Retik AB. Current trends in hypospa-
our standard technique for perineal hypospadias
dias repair. Urol Clin North Am. 1999; https://doi.
since 2013. Figure 56.1 summarizes our recom- org/10.1016/S0094-0143(99)80004-4.
mendations for primary hypospadias repair.
Part III
Complications
Early Complications
57
Ahmed T. Hadidi
Abbreviations Prophylactic antibiotics are of little value in

avoiding wound infections in hypospadias sur-
DDF Dorsal dartos fascia gery. Postoperatively, however, trimethoprim-
DYG Double Y glanulomeatoplasty sulfa or nitrofurantoin suppression may be
LABO Lateral-based onlay flap helpful to prevent cystitis, particularly if the tube
MAGPI Meatal advancement glanuloplasty is left to drain openly into the diaper [1, 2].
incorporated In general, drains are not employed except
MIP Megameatus intact prepuce when a skin graft or buccal mucosal graft is used.
SLAM Slit-like adjusted Mathieu In such cases, a vacuum suction drain is created
TIP Tubularized incised plate by placing a scalp vein needle into the vacuum
tube while the tubing from the needle is placed
under the skin. This helps keep the graft adherent
to the overlying skin. Drains should be removed
57.1 Infection as soon as their purpose has been served, in order
to keep the skin in continuity with the graft (24–
Every effort should be made to prevent wound 48 h) [3] (Figs. 57.1 and 57.2).
infection. Separation of the foreskin adherent to
the glans and removal of desquamated epithelial
debris should be performed under anesthesia 57.2 Meatal Stenosis
prior to skin preparation. When the patient is
postpubertal, skin preparation should be under- The meatus may become stenotic through crust-
taken several days before surgery with daily ing, edema, or synechiae (Fig. 57.3). Leaving a
cleansing. stent in place is expected to help avoid these
Wound infection is a rare problem in hypospa- complications.
dias repair, especially in the prepubertal patient. The best way to avoid meatal stenosis is to use
As long as good viability of tissue is maintained, healthy epithelialized tissue and reconstruct a
infection should be a minor problem. wide urethra with a wide meatus.
Some surgeons recommend placing an oph-
thalmic ointment tube nozzle into the urethral
A. T. Hadidi (*) meatus or gentle bougienage with a straight
Department, Sana Klinikum Offenbach, Academic sound. However, these measures are painful and
Teaching Hospital of the Johann Wolfgang Goethe traumatizing to the little children.
772 A. T. Hadidi
a b c
Fig. 57.1 (a) Meatal infection 3 days after hypospadias surgery. (b) The unhealthy segment (3 mm) was excised. (c)
The urethra was sutured to the tip of the glans meatus (©Ahmed T. Hadidi 2022. All Rights Reserved)
a b
Fig. 57.2 (a) Penile infection after hypospadias surgery new urethra is well vascularized and covered with a
for proximal hypospadias using the “lateral-based onlay” healthy protective intermediate layer, the wound may heal
flap technique (LABO, see Chap. 30). A well-vascularized without complication. (b) Appearance 2 months after sur-
tunica vaginalis flap was used. Although infection is rare gery (©Ahmed T. Hadidi 2022. All Rights Reserved)
after hypospadias surgery, it can happen, but as long as the
57 Early Complications 773
57.3 Loss of Skin Flaps urethra, healing can occur after the sloughed
skin is gone (Figs. 57.4 and 57.5). If a free-graft
Poor wound healing is due primarily to isch- technique has been used, a sloughed skin flap
emic flaps. Appropriate care during the proce- over the neourethra will jeopardize the entire
dure should avoid this problem. If skin flap loss procedure [3].
occurs over a well-vascularized island-flap neo-
Fig. 57.3 Severe stenosis within weeks after hypospadias repair. On pressing of the lower abdomen only urine drops
come out and no stream (©Ahmed T. Hadidi 2022. All Rights Reserved)
a b c d
Fig. 57.4 (a) Ventral skin necrosis 4 days after surgery. (b, c) During healing, the necrotic skin has sloughed. (d)
3 months after rearrangement of the penile skin (©Ahmed T. Hadidi 2022. All Rights Reserved)
774 A. T. Hadidi
bipolar diathermy, it can be kept to a minimum.

Tourniquets or cutaneous infiltrations with dilute
concentrations of epinephrine can be helpful but
they should not replace careful technique [3].
One disadvantage of using tourniquet or vaso-
constrictive injections (adrenaline) is that they
may mask bleeders until the skin is closed and
the patient may bleed hours after surgery.
The author has stopped using tourniquet com-
pletely and prefers to use adrenaline soaked
swaps over injections.
Postoperative bleeding can be usually con-
trolled by the application of a compressive dress-
ing [5]. It is very rare that the surgeon may need
to take the patient to the operating room after
hypospadias surgery to control bleeding.
Evacuation of a hematoma in children is usually
unnecessary unless a free graft is used or when
the scrotal hematoma is huge (Fig. 57.6).
The situation is different when dealing with
adults. In case dissection in the scrotum was per-
formed (e.g., when elevating tunica vaginalis
flap), it is highly recommended to leave a drain in
the scrotal cavity for a few days to avoid accumu-
lation of blood in the scrotal sac.
Fig. 57.5 The child had “megameatus intact prepuce”
(MIP) and severe rotation. The parents wanted to keep the
foreskin. The MIP was corrected using “double Y glanulo
meatoplasty” technique (DYG, see Chap. 19), and the 57.6 Postoperative Erection
grade II rotation was corrected using dorsal dartos fascial
flap (DDF). The mobilization of the dartos fascia and
Postoperative erections do not seem to be a sig-
resuturing of the foreskin caused ischemia and necrosis of
the foreskin only. Circumcision was done 1 month later nificant problem for the small child, but in the
(©Ahmed T. Hadidi 2022. All Rights Reserved) young adult they are at best unpleasant and at
worst may damage the repair. For the adolescent,
the author uses promethazine (®Atosil) drops (15
57.4 Edema drops twice or three times daily) as we are not
supposed to use antiandrogen drugs before the
Edema occurs after most hypospadias repairs and age of 18 years. For adults, the author uses high
can be diminished by means of a compression dosage antiandrogen treatment with cyproterone
dressing. acetate (®Androcur). In decreasing libido and
Edema within the urethra may compromise spontaneous erections, 50 mg daily up to 3 times
healing if a urethral stent is too large. A compress- daily is helpful, and a short course in such
ible stent was described by Mitchell and Kulb [4]. patients is generally very well tolerated. Mood
swings are the commonest complaint and there
is of course temporary disruption of fertility.
57.5 Hemorrhage Most other listed side effects probably pertain to
its long-term use in elderly patients with dis-
Intraoperative hemorrhage can, at times, be trou- seminated prostate carcinoma. Unfortunately a
blesome. With careful attention to the control of slow onset of action means that medication has
bleeding by judicious use of point-tip cautery or to start at least 10 days before admission. Other
57 Early Complications 775
Fig. 57.6 A huge hematoma that developed in the scro- ommended to leave a drain in the scrotum for a few days
tum after tunica vaginalis mobilization. Such a huge after scrotal dissection (©Ahmed T. Hadidi 2022. All
hematoma is rare in children. However, in adults, it is rec- Rights Reserved)
drugs such as ketoconazole and procyclidine Surgery should be postponed for at least
have been recommended as producing more 6 months after the primary operation. The
rapid suppression of erections, but unfortunately author commonly uses either an advancement
they have more troublesome side effects. No technique with a triangular flap from the glans
drug treatment can fully suppress erections with- or unilateral or bilateral “slit-like adjusted
out producing unacceptable side effects, but Mathieu” (SLAM) technique (Chaps. 22 and
cyproterone offers an acceptable compromise. 44). When the glans is less than 12 mm, it may
As an emergency measure, a sharp icy blast of be wise to give a course of hormone stimulation
®
PR Freeze Spray (Crookes Healthcare Ltd. before surgical correction (Fig. 57.7) (see
Nottingham UK) can help reverse unwanted Chaps. 13 and 56).
erections. A can of this is therefore kept on the
bedside locker for instant access, usually during
the night. 57.8 Bladder Spasm
When a catheter is left inside the bladder postop-

57.7 Retrusive Meatus eratively for urinary diversion, bladder spasms
may occur and are sometimes very troublesome.
Occasionally, a meatus heals in such a way that The parents often describe this as “the child
it retracts proximally to the coronal sulcus and suddenly has severe pain or cramps, pulls up his
deflects the urinary stream downward. This legs, this may continue for a few minutes or lon-
problem is more common in older types of ger and then it disappears completely.” The best
hypospadias repair such as “meatal advance- thing is to reposition the catheter so that it does
ment glanuloplasty incorporated” (MAGPI, not lie on the trigone and immobilize the catheter
see Chap. 20) repair. Nowadays, it is a com- securely on the abdominal wall together with the
mon complication if the patient has a small penis. The author fixes the glans against the
glans (less than 12 mm in children less than abdominal wall as a routine in proximal and peri-
2 years). neal hypospadias, and the catheter can be fixed
776 A. T. Hadidi
Fig. 57.7 Meatal retraction after “tubularized incised plate” (TIP) procedure (©Ahmed T. Hadidi 2022. All Rights
Reserved)
using the same suture. Antispasmodics like oxy- References

butynin 0.2 mg per kilogram may be used every
8 h when needed [6]. Valium 1–2 mg given orally 1. Montagnino BA, Gonzales ET, Roth DR. Open cath-
or intramuscularly has been found helpful to eter drainage after urethral surgery. J Urol. 1988;
relieve bladder spasms. https://doi.org/10.1016/s0022-5347(17)42016-7.
2. Sugar EC, Firlit CF. Urinary prophylaxis and post-
operative care of children at home with an indwell-
ing catheter after hypospadias repair. Urology. 1988;
57.9 Catheter Blockage https://doi.org/10.1016/0090-4295(88)90414-1.
Another problem that was common in the postop- Adult and pediatric urology. 3rd ed. St. Louis: Mosby-
erative period in the past was catheter blockage. Year Book; 1996. p. 2549–89.
The catheter may be blocked with urinary crusts 4. Mitchell ME, Kulb TB. Hypospadias repair without
or blood clots. This is less common nowadays a bladder drainage catheter. J Urol. 1986; https://doi.
org/10.1016/S0022-5347(17)45626-6.
with the use of silastic catheters and deroofed 5. Murphy JP. Hypospadias. In: Ashcraft KW, Murphy
stents. If it occurs, however, it can be very trou- JP, Sharp RJ, Sigalet DL, Snyder CL, editors. Pediatric
blesome and will necessitate catheter removal if surgery. 3rd ed. Philadelphia: Saunders; 2000.
deblocking with saline fails. The patient may be p. 763–82.
6. Keating MA, Rich MA. Onlay and tubularized pre-
left to void spontaneously, need another fine putial island flaps. In: Ehrlich RM, Alter GJ, editors.
catheter inserted, or require insertion of a percu- Reconstructive and plastic surgery of the external
taneous suprapubic catheter. genitalia: adult and pediatric. Philadelphia: Saunders;
1999. p. 77.
Meatal Stenosis and Urethral
Strictures 58
Ahmed T. Hadidi
Abbreviations urethral obstruction (FUO) will be discussed in

Chap. 59 and balanitis xerotica obliterans (BXO)
BMG Buccal mucosa graft will be dealt with in Chap. 61.
BXO Balanitis xerotica obliterans
DIG Dorsal inlay graft
FUO Functional urethral obstruction 58.2 Definition
LABO Lateral-based onlay flap
SLAM Slit-like adjusted Mathieu The definition of meatal stenosis varies among
TIP Tubularized incised plate different publications [3]. Recent textbooks
UTI Urinary tract infection define meatal stenosis when the meatal caliber in
infant is < F8 [2, 4].
In reality, the urethral meatus must be as wide
as the normal proximal urethra. If the normal
58.1 Introduction proximal urethra accommodates catheter size
F12 and the meatus accommodates catheter size
Meatal stenosis and urethral strictures are classi- F8 only, the patient is likely to develop symptoms
cally reported in most textbooks as the second of urinary obstruction.
most common complication of hypospadias sur- Meatal stenosis is diagnosed when the patient
gery, after urethrocutaneous fistula [1, 2]. has symptoms of straining, stranguria, requires
However, the incidence of meatal stenosis has long time (more than a minute) to empty the
been increasing steadily with the increasing pop- bladder, narrow and long stream of urine (more
ularity of incision of the urethral plate. In this than 1 m), and residual urine in bladder more
chapter, we will discuss the definition, incidence, than 15 mL after micturition or thickened blad-
etiology, as well as the different clinical presenta- der wall on ultrasound examination. In addition,
tions of meatal stenosis and stricture. Functional meatal stenosis and/or urethral stricture should
be seriously considered when the child develops
urinary tract infection after hypospadias
A. T. Hadidi (*) surgery.
778 A. T. Hadidi
58.3 Incidence through the penis and glans to create new ure-
thra have failed because the surgeon must cre-
As the definition of meatal stenosis varies in dif- ate adequate space for the new urethra.
ferent publications, it is difficult to determine the • Incision of the urethral plate: The incidence
exact incidence of meatal stenosis. However, the of meatal stenosis has increased again fol-
incidence seems to vary according to popular lowing the widespread popularity of the
techniques for hypospadias repair. In 1986, tubularized incised urethral plate technique.
Belman presented data from 21 publications When there was a deep glanular groove
(from 1974 to 1981) and the range was 0–22.7% meatal stenosis was rarely reported as a prob-
[5]. In 2001, Mouriquand and Mure suggested lem, but Elbakry (1999) reported a signifi-
that the incidence of stenosis had significantly cant incidence of early stenosis when the
decreased since surgeons were no longer employ- incised urethral plate was used and recom-
ing circumferential anastomoses [6]. Duel et al. mended a 3-month period of daily meatal
(1998) stated that stricture disease continued to dilatation to avoid the complication [9].
be a significant complication of hypospadias Elbakry (2002) also carried out a randomized
reconstruction, with an incidence of symptomatic controlled trial of patients undergoing tubu-
stricture at their institution of 6.5% in 582 larization of the incised urethral plate and
patients [7]. found a significant increase in stricture dis-
With the increasing popularity of tubulariza- ease in patients not undergoing routine post-
tion of the incised urethral plate, the incidence of operative dilatation [10]. Elbakry et al.
meatal stenosis is on the rise again. In the (2016) conducted a prospective randomized
Hypospadias Center in Frankfurt, Germany, 80% controlled trial comparing the TIP technique
of the patients referred with complicated hypo- versus the classic Thiersch-Duplay technique
spadias had meatal stenosis/stricture or persistent [11]. The study included 370 patients. The
fistula due to distal stenosis and stricture. complication rate was 16.8% in the TIP
group versus 5.6% in the Thiersch group.
Meatal stenosis with or without fistula com-
58.4 Causes of Postoperative prises 80% of the patients referred with
Meatal Stenosis hypospadias complications to the
Hypospadias Center in Frankfurt.
• Circular anastomosis: The incidence of • Balanitis xerotica obliterans (BXO): This is
meatal stenosis has increased when the prac- the commonest cause of meatal stenosis
tice of placing the external urethral meatus at detected on long-term follow-up following
the tip of the glans was routinely adopted in primary hypospadias repair [12]. This is
hypospadias surgery. Duckett (1995) sug- being recognized with increasing frequency
gested that the problem arose as a result of a as a cause of meatal stenosis following hypo-
circular anastomosis [8]. spadias repair and is dealt with in detail in
• Tight glanular closure: Belman (1986) sug- Chap. 61.
gested that stenosis could be due to a tight • Bladder mucosa grafts: Mucosal prolapse and
glans closure and recommended that a glans stenosis were common problems encountered
tunnel of sufficient width should be created to when the bladder mucosal grafts were brought
readily accept the new urethra [5]. Duckett to the tip of the glans [13]. This problem has
(1995) also recommended to excise part of the largely disappeared since buccal mucosa has
glanular tissue to create a space for the new replaced the use of bladder mucosa.
urethra or to perform an oblique anastomosis • Urethral burnout: This is a rare cause of stran-
[8]. This is very important as the glans in guria and meatal stenosis that may occur
hypospadias has no place for the new urethra. 10 years or more after urethral reconstruction
In the middle ages, all attempts of tunneling using skin (Fig. 58.1).
58 Meatal Stenosis and Urethral Strictures 779
gulated, and although this probably minimized

the problem, it still occurred. Garibay et al. (1995)
reported that 5 out of 16 patients with a tubular-
ized preputial island flap developed a stricture but
no patient with an onlay preputial flap did [14].
They concluded that meatal-based and onlay flap
urethroplasties had a low risk for stricture forma-
tion but tubularized preputial flaps frequently
developed anastomotic strictures. Mouriquand
et al. (1995) also recommended avoiding of circu-
lar anastomoses to reduce the risk of stricture for-
mation [15], and Bracka (1995) proposed a
two-stage repair to produce a normal-appearing
external meatus and to reduce the incidence of
urethral stricture and meatal stenosis [16].
When pedicled flaps are used, devasculariza-
tion is always a possible risk, and this can lead to
scarring and urethral stenosis. This has led to the
increased number of procedures employing some
method of tubularizing the native urethral plate,
most of which are dealt with in other chapters of
this book. When there is a deeply grooved and
Fig. 58.1 Urethral burnout: A 12-year-old boy who had
successful proximal hypospadias repair 11 years ago pre- wide urethral plate stricture or stenosis is rarely a
sented with dysuria and narrow stream 11 years after sur- problem, but concern has been expressed that
gery. The affected segment was excised and the healthy problems may arise when shallow and narrow
urethra was mobilized to the tip of the glans. Biopsy urethral plates are tubularized using the Snodgrass
showed chronic nonspecific inflammation. The surgeon
should think of BXO in such cases long time after surgery technique [17]. Holland and Smith (2000) found
(©Ahmed T. Hadidi 2022. All Rights Reserved) that after tubularization of the incised urethral
plate, patients with a shallow urethral plate were
more likely to have a neourethra caliber of F6 or
Most of these causes are technically related, less [17]. In addition they found that these boys
and meatal stenosis could be dramatically had a higher incidence of meatal stenosis, and
reduced when the surgeon reconstructs the new also, when the urethral plate was less than 8 mm
urethra using well-vascularized, fully epithelial- wide, there was a higher incidence of urethral
ized tissues around wide catheter (the author fistula.
using as a standard catheter size F12 in infants) BXO is now being increasingly recognized as
and avoids tight glans closure and circular anas- a cause for late presenting urethral strictures fol-
tomosis. If the surgeon prefers to incise the ure- lowing hypospadias repair (see Chap. 61). Kumar
thral plate, it may be wise to put a dorsal inlay and Harris reported the first series in 1999,
graft (DIG) as mentioned in Chap. 27. describing eight patients whose strictures pre-
sented between 1 and 16 years following their
original surgery [12]. Uemura et al. (2000) also
58.5 Causes of Urethral Stricture described this problem in three patients, with
symptoms developing 3 months to 6 years fol-
Procedures that involved an end-to-end circum- lowing surgery [18]. BXO is a form of lichen
ferential anastomosis led to stricture formation at sclerosus et atrophicus affecting the genital skin
the hypospadiac meatus. It was recommended of the prepuce and glans. It has been widely rec-
that the anastomosis should be spatulated or trian- ognized as a cause of phimosis and meatal steno-
780 A. T. Hadidi
sis [19] and more recently has been recognized as Prolonged and frequent micturition: This occurs
a cause of anterior urethral stricture [20]. It is not when the child has to strain to pass urine and will
surprising, therefore, to see it as a cause of stric- need long time to pass urine and may not empty
ture following hypospadias repair, particularly the bladder completely. This means that the blad-
when genital skin has been used. der will be full again in a short time and he will
have to go frequently to the toilet to pass some
urine.
58.6 Presentation Urinary tract infection (UTI): Straining and
inability to empty the bladder may result in large
Presenting features and means of assessment of residual volume in the bladder. UTI can be
meatal stenosis/urethral stricture: excluded with urine examination. Another cause
of UTI is that severe straining may cause vesico-
ureteric reflux, increasing the risk of infection
Presenting features
and, more importantly, renal scarring.
Spraying stream
Fistula formation: The commonest cause of fis-
Decreased stream
tula formation is distal narrowing and obstruction
Straining to void
(Fig. 58.2). This will be further discussed in
Urinary retention
detail in Chaps. 59 and 60.
Dysuria
Meatal stenosis, breakdown of the repair, and
Urinary tract infection
fistula formation are usually easily recognizable
Urine frequency/urgency
on clinical examination. Sometimes a meatal ste-
Urinary incontinence
nosis is not immediately obvious, as the narrow-
Fistula
ing may just be inside the meatus itself and will
Hypospadias breakdown
be diagnosed only if the meatus is opened and
Reduced urinary flow rate
carefully examined (Fig. 58.3). In the presence of
Assessment of stricture
a distal stenosis, dilatation of the proximal ure-
Examination
thra may occur during micturition and there may
Uroflowmetry
also be terminal dribbling. Therefore it is always
Postvoid ultrasound
valuable to watch the patient void. In the case of
Urethrography
proximal urethral stricture, there may be no phys-
Endoscopy
ical signs (Fig. 58.4).
58.7 Diagnosis of Stenosis

Straining and narrow urine stream: Meatal and Stricture
stenosis and/or urethral stricture usually present
with voiding difficulties (that may be progress to In the absence of physical signs, further assess-
urinary retention) and/or recurrent urinary tract ments will be necessary. These include uroflow-
infections. In meatal stenosis, the child usually metry, ultrasound, and micturating
present with straining and a narrow strong urine cystourethrography. The least invasive form of
stream. With urethral strictures, there is straining assessment is uroflowmetry. There will fre-
and a narrow weak stream. Dysuria may be a fea- quently be a reduced flow rate, but it must be
ture of both meatal stenosis and urethral remembered that even in the presence of a severe
stricture. narrowing there may be a normal flow, providing
the bladder can generate sufficient pressure. It is
Fig. 58.2 A child presenting with fistula after TIP repair. larger than from the tip of the penis (©Ahmed T. Hadidi
It is often possible to see that the fistula is due to distal 2022. All Rights Reserved)
stenosis when the urine stream coming out of the fistula is
Fig. 58.3 The child presented with dysuria and the meatus looks slit-like, normal, and large. Careful examination and
opening the lips of the meatus show very narrow true meatus (©Ahmed T. Hadidi 2022. All Rights Reserved)
782 A. T. Hadidi
of routine urinary flow rate measurements [21].

Garibay et al. (1995) also studied the routine use
of uroflowmetry following hypospadias repair
and concluded that it was an important noninva-
sive tool and helped to detect significant asymp-
tomatic strictures [14].
However, most children show a flat or
“obstructive pattern” after hypospadias surgery.
This is probably related to the fact that the neo-
urethra that we as surgeons reconstruct is totally
different in structure and function from the nor-
mal urethra and as long as the child has no symp-
toms of stranguria, prolonged micturition, narrow
long stream nor signs of increased bladder wall
thickness, or large residual urine volume
(>15 mL) by ultrasound examination, there
should be no need for surgical intervention.
58.8 Treatment
58.8.1 Meatal Stenosis

Fig. 58.4 Dripping of urine due to severe meatal stenosis
• Dilatation: One dilatation is worth trying, as
few patients may require no further treatment
worthwhile combining uroflowmetry with blad- [5, 7, 15]. There is a new term recently intro-
der scanning when the thickness of the bladder duced in literature called “calibration” which
wall can be assessed and postvoid residuals mea- is nothing but dilatation.
sured. Urethrography and micturating cystoure- • Meatotomy: The majority of patients, how-
thrography (if infection has occurred) are also ever, develop recurrent stenosis after dilata-
other useful means of assessing strictures tion, and the next step may be meatotomy
(Fig. 58.5). Finally, it may be necessary to assess which can be carried out by performing a
strictures under anesthetic with urethroscopy and dorsal or ventral incision into the meatal ring
calibration before deciding on the best and advancing the urethra distally (Fig. 58.6).
management. • Y-V Plasty: Kim and King (1992) suggested
However, there continues to be controversy as that conventional meatotomy was frequently
to whether these strictures require treatment, complicated by recurrent meatal stenosis and
although this is recommended by Garibay et al. recommended a V-shaped flap meatoplasty,
(1995) [14] and Hamilton et al. (2000) [21]. with the flap raised from the dorsal aspect of
Patients with asymptomatic strictures require the glans [22]. This is the preferred approach
close follow-up to prevent the late sequelae of by the author in recurrent stenosis (Fig. 58.7).
infection and incontinence. • Incision and Advancement: If the urethra is
Hamilton et al. (2000) reported the detection mobile and stenosis is limited to the tip, it is
of previously unsuspected stenoses with the use sometimes possible to incise the narrow distal
a b
Fig. 58.5 (a) Micturating cystourethrogram in a patient with a distal urethral stricture following hypospadias repair,
demonstrating dilating bilateral vesicoureteral reflux. (b) Distal urethral stricture following hypospadias repair
end and advance the mobile urethra to the tip patients with strictures not related to hypospadias
of the glans as described by Devine and repair, these approaches are palliative rather than
Horton [23] (Chaps. 28 and 44). curative [24]. Duel et al. (1998) reported similar
• Incision and Grafting: If the stenosis is lim- findings managing strictures following hypospa-
ited to the distal 0.5 cm, it may be possible to dias repair [7]. In their series of 29 patients
incise the narrow segment and put a buccal treated initially with dilatation or urethrotomy,
mucosal graft without opening the glans or 79% ultimately required urethroplasty. Therefore
urethra ventrally. it may be worthwhile attempting a one-off ure-
throtomy with dilatation, but if the stricture
recurs this management should be abandoned
58.8.2 Urethral Stricture and an early urethroplasty should be performed.
The traditional treatment of urethral stricture dis- • Incision and SLAM Technique: If the urethral
ease has always started with dilatation or visual stricture is distal to the mid penis and there is
urethrotomy. However, for the majority of adequate skin, the author’s standard approach
784 A. T. Hadidi
Fig. 58.6 A 5-year-old child who underwent TIP repair is less than 5 mm wide. The only solution was to incise the
4 years earlier. Ever since the operation, he went through narrow urethra and reconstruct a wide new urethra
“regular calibration for the past 4 years” without success. (©Ahmed T. Hadidi 2022. All Rights Reserved)
Incision of the narrow urethra showed that the neourethra
Fig. 58.7 A child with severe meatal stenosis. A Y inci- thus widening the stenotic segment and meatoglanulo-
sion is made distal to the stenosis, the longitudinal limb is plasty is performed (©Ahmed T. Hadidi 2022. All Rights
the narrow meatus. The Y is advanced and closed as V Reserved)
Fig. 58.8 The principle of using SLAM in distal urethral stricture. The narrow distal urethra is incised and a meatal-
based flap is advanced (SLAM) technique (©Ahmed T. Hadidi 2022. All Rights Reserved)
is to incise the narrow urethra and perform the scarring and associated chordee, the best
“slit-like adjusted Mathieu” technique approach is the two-stage procedure popular-
(SLAM, Chap. 22). This is a very robust flap ized by Bracka (1995) [16]. In this approach
and the success rate is 90% in the Hypospadias all the scarred ventral tissues, including the
Center in Frankfurt, Germany (Figs. 58.8 and urethra, are excised back to the normal ure-
58.9). thra. A buccal mucosa graft (BMG) [25] is
• Incision and LABO Technique: In patients laid on the raw area and urethroplasty is per-
with long urethral stricture extending to the formed 6 months later (Chap. 40).
proximal penis and there is adequate skin, the
author prefers to perform the “lateral-based
onlay flap” (LABO) technique (Chap. 30) Acknowledgments The author would like to acknowl-
(Figs. 58.10 and 58.11). edge and thank Dr. Pat Malone for his outstanding contri-
bution in the meatal stenosis chapter in the first edition of
• Excision of Scar Tissue and Grafting (Two- the book. Part of the information from the first edition is
Stage Repair): In the presence of extensive included in this chapter.
786 A. T. Hadidi
Fig. 58.9 A child with urethral stricture and fistula that Observe the wide urine stream at the end of surgery
recurred twice. The narrow urethra was incised and the (©Ahmed T. Hadidi 2022. All Rights Reserved)
SLAM technique was done around catheter size F14.
Fig. 58.10 The principle of using LABO in proximal the narrow urethra and a lateral-based flap is advanced
urethral stricture. The narrow urethra is incised and the medially to form a wide urethra (LABO) technique
lateral penile and preputial skin is sutured to the edge of (©Ahmed T. Hadidi 2022. All Rights Reserved)
788 A. T. Hadidi
Fig. 58.11 A child with long urethral stricture following wide new urethra was reconstructed using the LABO
a proximal TIP procedure. The narrow urethra was incised technique (©Ahmed T. Hadidi 2022. All Rights Reserved)
and the lateral edge was sutured to the lateral skin and a
King-Belman textbook of clinical pediatric urology.

References 6th ed. London: CRC Press; 2018. p. 1249.
5. Belman AB. Hypospadias. In: Welch KJ, Randolph
1. Malone P. Meatal stenosis and urethral strictures after JG, Ravitch MM, O’Neill JA, Rowe MI, editors.
hypospadias surgery. In: Hadidi AT, Azmy A, editors. Pediatric surgery. 4th ed. Chicago; 1986. p. 1286–302.
Hypospadias surgery: an illustrated guide. 1st ed. 6. Mouriquand PD, Mure PY. Hypospadias. In: Gearhart
Berlin-Heidelberg: Springer; 2004. p. 295–300. JP, Rink RC, Mouriquand PD, editors. Pediatric urol-
2. Long C, Zaontz M, Canning D. Hypospadias. In: ogy. Philadelphia: Saunders; 2001. p. 713–28.
Partin AW, Dmochowski RR, Kavoussi LR, Peters 7. Duel BP, Barthold JS, Gonzalez R. Management of ure-
CA, editors. Campbell-Walsh urology. 12th ed. thral strictures after hypospadias repair. J Urol. 1998;
Elsevier; 2021. p. 911. https://doi.org/10.1016/S0022-5347(01)63083-0.
3. Wilkinson DJ, Farrelly P, Kenny SE. Outcomes in dis- 8. Duckett JW. The current hype in hypospadiology.
tal hypospadias: a systematic review of the Mathieu Br J Urol. 1995; https://doi.org/10.1111/j.1464-
and tubularized incised plate repairs. J Pediatr Urol. 410X.1995.tb07812.x.
2012; https://doi.org/10.1016/j.jpurol.2010.11.008. 9. Elbakry. Tubularized-incised urethral plate urethro-
4. Snodgrass WT, Bush N. Hypospadias. In: Docimo plasty: is regular dilatation necessary for success?
SG, Canning D, Khoury A, Salle JLP, editors. Kelalis- BJU Int. 1999;84:683–8.
10. Elbakry A. Further experience with the urethroplasty. J Urol. 2000; https://doi.org/10.1016/
tubularized-incised urethral plate technique for S0022-5347(05)67408-3.
hypospadias repair. BJU Int. 2002; https://doi. 18. Uemura S, Hutson JM, Woodward AA, Kelly JH,
org/10.1046/j.1464-4096.2001.01525.x. Chow CW. Balanitis xerotica obliterans with urethral
11. Elbakry A, Hegazy M, Matar A, Zakaria stricture after hypospadias repair. Pediatr Surg Int.
A. Tubularised incised-plate versus tubularisation of 2000; https://doi.org/10.1007/s003830050047.
an intact and laterally augmented plate for hypospa- 19. Bainbridge DR, Whitaker RH, Shepheard
dias repair: a prospective randomised study. Arab J BGF. Balanitis xerotica obliterans and urinary
Urol. 2016;14:163–70. obstruction. Br J Urol. 1971;43:487–91.
12. Kumar MVK, Harris DL. Balanitis xerotica obliter- 20. Venn SN, Mundy AR. Urethroplasty for balani-
ans complicating hypospadias repair. Br J Plast Surg. tis xerotica obliterans. Br J Urol. 1998; https://doi.
1999; https://doi.org/10.1054/bjps.1998.3017. org/10.1046/j.1464-410X.1998.00634.x.
13. Keating MA, Cartwright PC, Duckett JW. Bladder 21. Hamilton SA, Clinch PJ, Goodman PJ. The objective
mucosa in urethral reconstructions. J Urol. 1990; assessment of hypospadias repair (abstract). BJU Int
https://doi.org/10.1016/S0022-5347(17)39602-7. Suppl. 2000;85:45–6.
14. Garibay JT, Reid C, Gonzalez R. Functional evalua- 22. Kim KS, King LR. Method for correcting meatal ste-
tion of the results of hypospadias surgery with uro- nosis after hypospadias repair. Urology. 1992; https://
flowmetry. J Urol. 1995; https://doi.org/10.1016/ doi.org/10.1016/0090-4295(92)90013-m.
S0022-5347(01)67178-7. 23. Devine CJ, Horton CE. A one stage hypospa-
15. Mouriquand PDE, Persad R, Sharma S. Hypospadias dias repair. J Urol. 1961; https://doi.org/10.1016/
repair: current principles and procedures. Br J Urol. S0022-5347(17)65301-1.
1995; https://doi.org/10.1111/j.1464-410X.1995. 24. Andrich DE, Mundy AR. Urethral strictures and
tb07813.x. their surgical treatment. BJU Int. 2000; https://doi.
16. Bracka A. Hypospadias repair: the two-stage alterna- org/10.1046/j.1464-410X.2000.00878.x.
tive. Br J Urol. 1995; https://doi.org/10.1111/j.1464- 25. Duckett JW, Coplen D, Ewalt D, Baskin LS. Buccal
410X.1995.tb07815.x. mucosal urethral replacement. J Urol. 1995; https://
17. Holland AJA, Smith GHH. Effect of the depth and doi.org/10.1016/S0022-5347(01)67497-4.
width of the urethral plate on tubularized incised plate
Functional Urethral Obstruction
(FUO) 59
Ahmed T. Hadidi
Abbreviations the UP often heals by secondary intention result-

ing in a deep contracted groove that does not
FUO Functional urethral obstruction allow free passage of urine but yield to passage of
LABO Lateral-based onlay flap a rigid catheter or bougie.
SLAM Slit-like adjusted Mathieu Functional urethral obstruction or FUO is
TIP Tubularized incised plate defined as persistent obstructive voiding symp-
UP Urethral plate toms (dysuria, pain, and/or persistent straining)
UTI Urinary tract infection and/or clinical signs of back pressure (e.g.,
increased bladder wall thickness, acquired reflux,
and/or residual urine in bladder after micturition)
or recurrent fistula in spite of the ability to pass
59.1 Introduction catheter size F8 through the neo-urethra. The
patients did not have these symptoms and/or
Hypospadias surgery is challenging and may be signs before hypospadias repair [2].
associated with many complications. These com-
plications include fistula, meatal stenosis, ure-
thral stricture, glans and wound dehiscence, 59.2 Patient’s Presentation
diverticulum formation, and persistent/recurrent
chordee, to mention a few. Between 2003 and 2020, 921 children and adults
During the last decade, the “tubularized were referred to the Hypospadias Center,
incised plate” (TIP) technique for repair of hypo- Frankfurt, Germany, with complications follow-
spadias has become a popular procedure [1]. The ing hypospadias repair. These included 290
principle of the TIP repair is to incise the urethral patients presenting with FUO following TIP
plate (UP) deep in the midline and hope that the repair. There were 198 patients who presented
resultant gap will heal by primary intention and with recurrent urethral fistula after closure and
epithelialization resulting in a wide neo-urethra. the remaining 92 patients presented with obstruc-
However, the raw surface following incision of tive symptoms and signs in the form of persistent
dysuria, long duration of micturition, large resid-
ual volume after micturition, and recurrent uri-
A. T. Hadidi (*) nary tract infection (UTI). The majority of
Department, Sana Klinikum Offenbach, Academic patients with recurrent/persistent fistula were
Teaching Hospital of the Johann Wolfgang Goethe passing more urine through the fistula than
University, Offenbach, Frankfurt, Germany through the terminal meatus.
792 A. T. Hadidi
The mean age at the time of corrective surgery were covered with a second protective intermedi-
for the complications was 2.9 years (range ate layer from preputial or penile dartos fascia.
1–4 years). The mean number of procedures per- For patients with complicated proximal hypospa-
formed prior to referral was 2.4 (range 1–4). The dias, scrotal dartos fascia and/or tunica vaginalis
time interval between the initial failed repair and was the standard second protective layer
the salvage urethroplasty was more than employed.
6 months. The mean follow-up period was 6 years Distal forms of hypospadias had a transure-
(range 1–17 years). thral Nelaton catheter for 3 days. Proximal forms
of hypospadias had a single transurethral silastic
catheter for 1 week.
59.3 Diagnostic Investigations All patients had a standard dressing in the
form of gentamicin local eye ointment, gauze,
Ascending urethrography may show a narrow and adhesive tape that compresses the penis and
distal urethra as compared to the original proxi- catheter against the lower abdominal wall which
mal urethra. Uroflow may show a flat curve and allows the free mobility of the patient [5].
prolonged duration. Ultrasound may show large The dressing was removed at the time of
residual volume in the bladder (more than removal of the transurethral catheter and the
12 mL). All these investigations may show penis was left exposed. If the dressing became
obstructive flow that may occur with meatal ste- wet with urine or soaked with blood, the dressing
nosis or urethral stricture. The diagnosis of FUO was changed using the same standard dressing
is confirmed in the operating room under general technique.
anesthesia. Broad-spectrum antibiotics (second-
generation cephalosporin) were used for 1 week
in distal forms of hypospadias and for 2 weeks in
59.4 Management proximal forms.
Our standard postoperative follow-up protocol
Under general anesthesia, the urethral meatus included examination after 3 months, 1 year,
and the availability and quality of foreskin and 3 years, and then every 5 years until the child is
penile skin were assessed. It was possible to pass 15 years old.
a catheter size F8 in all the children with FUO. Mean follow-up period was 7 years (range
The neo-urethra was incised until a normal, 1–17 years). Uroflowmetry was performed in 31
healthy, and wide original urethra was reached. It toilet-trained children. Peak flow within two
was found that the urethral plate incision has standard deviations from mean for age was con-
healed, forming a scarred narrow, deep groove sidered normal.
that yielded temporarily to dilatation and gave a
false impression that the neo-urethra is not nar-
row. The explanation of this paradoxical finding 59.5 Discussion
is illustrated in Fig. 59.1.
Patients with distal hypospadias had the “slit- Incision of the urethral plate was first described
like adjusted Mathieu” technique (SLAM, Chap. by Reddy in 1975 [6], Orkiszewski (1987) [7],
22, [3]) employed (Fig. 59.2). Patients with prox- and Rich et al. (1989) [8] and popularized by
imal hypospadias had a “lateral-based onlay” Snodgrass (1994) [9]. The technique has become
(LABO, Chap. 30 [4]) flap to construct a neo- increasingly popular in recent years partly
urethra (Fig. 59.3). because of the slit-like meatus that can be
The urethroplasty was reconstructed using achieved [1].
Vicryl 6/0 on a cutting needle in a continuous The concept behind the TIP technique is that
subcuticular manner around the appropriate cath- the urethral plate (UP) is a specialized tissue
eter according to the caliber of the proximal designed for the formation of the urethra and
healthy urethra [4]. All distal urethroplasties the new urethra should ideally be reconstructed
59 Functional Urethral Obstruction (FUO) 793
a b
c d
Fig. 59.1 (a–d) Functional urethral obstruction: (a) dilated posterior urethra (the artefact is dye spilling
Failed TIP showing a skin bridge and urethral steno- around the coronal sulcus on the glans). (d) Diagram
sis associated with fistula. (b) Incision of the bridge showing the mechanism of functional urethral
shows that incision of the urethral plate heals as a obstruction where the contracted urethra is causing
deep groove that does not increase the diameter of the narrowing but the insertion of a firm catheter expands
neo-urethra. (c) Urethrography with contrast injected the neo-urethra giving the false impression that the
along the urethra showing the anterior narrow urethra urethra is wide (©Ahmed T. Hadidi 2022. All Rights
which causes functional narrowing and the more Reserved)
794 A. T. Hadidi
Fig. 59.2 “Slit-like adjusted Mathieu” (SLAM) tech- and a meatal-based flap is designed to reconstruct a wide
nique for the correction of narrow urethra after TIP for fully epithelialized new urethra (©Ahmed T. Hadidi 2022.
distal hypospadias (see Chap. 22) [3]. The narrow new All Rights Reserved)
urethra is incised until a healthy wide urethra is reached
only from the urethral plate. In hypospadias, urethra (Figs. 59.1 and 59.4b) or as a linear scar
the UP is usually too narrow to reconstruct the resulting in stenosis (Fig. 59.4c).
new urethra. Therefore, incision of the urethral The standard protocol for surgeons dealing
plate may increase its width to reconstruct a with complicated hypospadias is to insert a cathe-
wide urethra. Figure 59.4 demonstrates the ter or a probe inside the urethra to ensure that the
three possible pathways of healing of the new urethra is not narrow. If the TIP has healed
incised urethral plate. Figure 59.4a demon- with a linear scar and stenosis, the probe or cathe-
strates the desired pathway of healing after ter will not pass through and stenosis can be easily
incision of the urethral plate with a wide new identified. If the new urethra has healed forming a
urethra. However, the incised urethral plate deep narrow groove as was the case in 290 patients
may heal as a deep narrow groove that yields to included in the study (Figs. 59.1 and 59.4b), the
a sound or a catheter passing through the new deep groove will yield to the catheter which will
Fig. 59.3 “Lateral-based onlay” (LABO [4]) for correc- 30). The patient had adequate lateral skin that was sutured
tion of complicated proximal TIP. The narrow urethra is to the incised narrow urethra (A to A’) (©Ahmed T. Hadidi
incised until a healthy wide urethra is reached (see Chap. 2022. All Rights Reserved)
pass through and will give the false impression significantly decreased after complete epitheliali-
that the new urethra is not narrow. After removal of zation and the average gain in urethral width was
the catheter, the deep groove will gradually recoil only 2 mm. Sections of the healed incised plate in
resulting in a narrow passage for urine and recur- rabbits showed the typical deep groove seen in
rence of fistula or dysuria. children with complicated TIP repair presenting
FUO was first reported in 2013 [2]. Those with FUO (Figs. 59.1 and 59.4b).
patients presented with persistent pain, dysuria, Hafez et al. (2003) used the same rabbit model
and/or recurrent fistula. This phenomenon was to study the healing after the TIP technique, and
first identified in a child with persistent pain and they could calibrate the neo-urethra up to F12
dysuria in spite of frequent calibration and dilata- sounds [11]. The disparity between the conclu-
tion under anesthesia in two other centers. sions of the two studies can be explained by FUO
Urethrography confirmed the passage of the dye and shows that “calibration” can be misleading.
through the narrow new urethra. The deep narrow Urethrography is an objective investigation to
grove was seen clearly after incision of the nar- diagnose FUO preoperatively. The dye is injected
row urethra. through a catheter F6. This was used in 14
Is there objective evidence to support the FUO patients early in the study and when the diagnosis
hypothesis? Two experimental studies support was uncertain. It was not justified to perform
this hypothesis: biopsy or expose all patients with persistent pain
Eassa et al. (2011) conducted a well-designed and dysuria to radiation (for urethrography).
objective study on 12 rabbits and made a 3 cm tat- Calibration or cystoscopy is unreliable and
too in the dorsal urethral plate midline before may be misleading as the sound or cystoscope
incising the urethral plate [10]. They measured the will dilate and open the deep narrow groove tem-
stretched width between the incised tattooed edges porarily and will appear as a wide shallow groove.
followed by tubularization of the incised urethral Uroflowmetry is frustrating in children and
plate. The rabbits were sacrificed 2 weeks later, often produces flat curves despite satisfactory
and they measured the distance separating the tat- urethroplasty and the shape of the curve has
too incision after healing and examined sections at uncertain significance [12]. Therefore, it was not
this point histologically. The study concluded that performed preoperatively as a routine in this
the initial width of the midline relaxing incision study.
796 A. T. Hadidi
a c
Fig. 59.4 The three possible pathways of healing of the the new urethra resulting in functional urethral obstruc-
incised urethral plate. (a) The incised urethral plate has tion (FUO). (c) The incised urethral plate has healed as a
healed resulting in the formation of a wide new urethra. linear scar resulting in urethral stricture (©Ahmed
(b) The incised urethral plate has healed as a deep narrow T. Hadidi 2022. All Rights Reserved)
groove that yields to a sound or a catheter passing through
Local skin flaps were the first choice for the 4. Hadidi AT. Proximal hypospadias with small flat
author. “Slit-like adjusted Mathieu” (SLAM) Pediatr Surg. 2012; https://doi.org/10.1016/j.
technique gave satisfactory functional and cos- jpedsurg.2012.06.027.
metic outcome in 94% of the patients with com- 5. Hadidi AT. Double Y glanuloplasty for glanu-
plicated distal hypospadias [3] (see Chap. 22). lar hypospadias. J Pediatr Surg. 2010; https://doi.
org/10.1016/j.jpedsurg.2009.11.019.
“Lateral-based onlay” flap (LABO) gave satis- 6. Reddy LN. One-stage repair of hypospadias. Urology.
factory functional and cosmetic results in patients 1975; https://doi.org/10.1016/0090-4295(75)90070-9.
with complicated proximal hypospadias included 7. Orkiszewski M. Urethral reconstruction in skin defi-
in the study [4] (see Chap. 30). cit. Urol Pol/Polish Urol. 1987;40:12–5.
8. Rich MA, Keating MA, Snyder McC H, Duckett
This group of patients with FUO did not have JW. Hinging the urethral plate in hypospadias
persistent chordee or severe scarring and there meatoplasty. J Urol. 1989; https://doi.org/10.1016/
was adequate local skin to reconstruct a wide S0022-5347(17)39161-9.
neo-urethra and there was no indication to use 9. Snodgrass W. Tubularized, incised plate urethroplasty
grafts or to perform a two-stage repair. org/10.1016/S0022-5347(17)34991-1.
10. Eassa W, He X, El-Sherbiny M. How much does the
midline incision add to urethral diameter after tubu-
References larized incised plate urethroplasty? An experimental
animal study. J Urol. 2011; https://doi.org/10.1016/j.
juro.2011.03.073.
1. Cook A, Khoury AE, Neville C, Bagli DJ, Farhat
11. Hafez AT, Herz D, Bägli D, Smith CR, Mclorie
WA, Pippi Salle JL. A multicenter evaluation of
G, Khoury AE. Healing of unstented tubular-
technical preferences for primary hypospadias
ized incised plate urethroplasty: an experimental
study in a rabbit model. BJU Int. 2003; https://doi.
ju.0000180643.01803.43.
org/10.1046/j.1464-410X.2003.03084.x.
2. Hadidi AT. Functional urethral obstruction follow-
12. Snodgrass W, MacEdo A, Hoebeke P, Mouriquand
ing tubularised incised plate repair of hypospa-
PDE. Hypospadias dilemmas: a round table. J
dias. J Pediatr Surg. 2013; https://doi.org/10.1016/j.
Pediatr Urol. 2011; https://doi.org/10.1016/j.
jpedsurg.2012.10.071.
jpurol.2010.11.009.
3. Hadidi AT. The slit-like adjusted Mathieu technique
Hypospadias Fistula
60
Ahmed T. Hadidi
Abbreviations 60.2 Definition
BILAB Bilateral-based flap A fistula is defined as a tract connecting two epi-

FUO Functional urethral obstruction thelial surfaces, whereas a sinus is a blind tract
LABO Lateral-based onlay flap leading to an epithelial surface. Urethral fistula is
MAGPI Meatal advancement glanuloplasty a tract connecting the urethra and the skin.
incorporated
PDS Polydioxanone suture
SLAM Slit-like adjusted Mathieu 60.3 Incidence of Fistula
TIP Tubularized incised plate Formation
The incidence of fistula formation has decreased

gradually during the 1980s and 1990s. However,
60.1 Introduction it started to rise again associated with meatal ste-
nosis during the 2000s and 2010s. In 1973,
There is no surgery without complications. Barry Horton and Devine estimated the incidence of
O’Donell of Dublin famously commented: fistula following hypospadias surgery to range
Three statements I never believed in my life: “Of between 15 and 45% [1]. In 1984, Shapiro found
course I love you darling!”, “The cheque is in the a urethral fistula rate of 6.25% (11 cases) in a
mail!” and: “I never had a complication or a fistula series of 176 hypospadias repairs including the
after hypospadias repair!” “meatal advancement glanuloplasty incorpo-
rated” (MAGPI, see Chap. 20), flip-flap, island,
Urethrocutaneous fistula was reported as the and free-graft techniques [2]. In 1996, Duckett
most common complication in hypospadias sur- and Baskin estimated the incidence to be between
gery before the popular use of “tubularized 10 and 15% [3]. Nowadays, the incidence of ure-
incised plate” technique (TIP). throcutaneous fistula can be used to judge the
success of hypospadias repair. Some procedures
are more prone to fistula formation than others.
For example, in an extensive review of hypospa-
A. T. Hadidi (*) dias repairs in 1990, Durham Smith noted that
Department, Sana Klinikum Offenbach, Academic the fistula rate for the MAGPI procedure ranged
Teaching Hospital of the Johann Wolfgang Goethe from 0.5 to 10%, whereas the rate for flip-flap
University, Offenbach, Frankfurt, Germany repairs varied from 2.2 to 35% and that for island
800 A. T. Hadidi
pedicle tubed repairs from 4 to 33% [4]. Free- monest cause nowadays is distal urethral narrow-
graft tubed repairs encountered even higher fis- ing due to reconstruction of a narrow incompletely
tula rates of 15–50%. Urethral advancement for epithelialized neo-urethra (Figs. 60.1 and 60.2).
distal hypospadias repair has a variable incidence Normal urethra in most children less than 2 years
of fistula, from less than 1 to 16.7%. It has not of age usually accommodates catheter size F10–
been widely used because of the risk of urethral F12. Other causes include the use of poorly vas-
devascularization with extensive mobilization cularized or hypoplastic tissues in repair, the type
[5]. For the megameatus variety of hypospadias, and size of suture material (e.g., PDS inside the
the pyramid procedure has a reported fistula rate urethra or nonabsorbable material [10]), the
of less than 5%. improper coaptation or eversion of the urethral
Using a two-stage repair, in more than 500 edges in urethroplasty, the suturing technique and
cases, Durham Smith (1981) reduced his inci- infection, the catheter material, and the duration
dence of fistula formation to less than 3% [6]. of the catheterization.
Similarly, Greenfield and colleagues reported a
urethral fistula rate of 2.5% with a two-stage
modified Belt-Fuqua repair for severe hypospa- 60.5 Fistula Prevention
dias with chordee [7].
During the twenty-first century, while some In distal hypospadias, a fistula rate of less than
authors reported the incidence of fistula after 5% is now to be expected [11]. The numerous
hypospadias repair under 10% [8], others reported factors that have accounted for this lower inci-
a fistula rate of 27% after TIP repair [9]. dence of fistula formation today are listed in
Table 60.1. Better and finer suture materials are
available today with greater tensile strength and
60.4 Causes of Fistula Formation less reactivity, including 6-0 and 7-0 polyglactin
(Vicryl) and polyglycolic acid (Dexon). These
Several factors may contribute to a high inci- sutures can be used on the skin (7-0 PDS, 6-0 and
dence of fistula formation. The most common 7-0 polyglactin and polyglycolic acid) as well as
reasons are technical and avoidable. The com- internally. PDS should not be used in the urethra
Fig. 60.1 Meatal stenosis and coronal fistula after failed indicating distal urethral narrowing (©Ahmed T. Hadidi
TIP repair. When the child passes urine more urine comes 2022. All Rights Reserved)
out of the fistula than out of the tip of the penis. Clearly
60 Hypospadias Fistula 801
a b
Fig. 60.2 (a–b) Fistula due to distal stenosis after TIP urethra. Unfortunately, as the first surgeon did circumci-
repair. (a) From the first look, the meatus looks wide sion at the same time of the TIP procedure, buccal or oral
enough. (b) Careful examination shows a very small, pin- mucosa may become the only option available (©Ahmed
point meatus. Here, the surgeon must incise the whole T. Hadidi 2022. All Rights Reserved)
narrow urethra and reconstruct a completely new, wide
Table 60.1 Factors related to a lower incidence of ure- (subcuticular is satisfactory) because it may react
thral fistula formation
with urine and fistula may result [10]. Plastic sur-
1. Quality of tissues Healthy, well vascularized gical principles such as magnification, microvas-
tissues
cular instruments, and delicate tissue handling
2. Technical points Suture material and size
are now used by most hypospadias surgeons.
Fine instruments
Magnification Vascularized pedicle flaps are preferred in the
3. Type of repair SLAM technique various repairs and their modifications (see
LABO technique Chaps. 15 and 16).
Thiersch-Duplay De-epithelialized flaps were first described by
BILAB technique Smith in 1973 [12]. The principle was applied by
4. Protective Preputial dartos flap Belman (1994) to the island pedicle procedures,
intermediate layer Tunica vaginalis flap with only one fistula in 32 onlay repairs [13]. Hill
Ventral dartos flap and colleagues (1993) used the de-epithelialized
Bifurcated corpus
spongiosum
flap to improve results of the pyramid procedure
De-epithelialized skin [14]. Many surgeons use 3.5× optical magnifica-
External spermatic fascia tion or even the microscope. Multiple layer closure
5. Short urethral has a major role in lowering the incidence of fis-
stenting tula formation. The additional layer may be
6. Short dressing obtained from either the fascia underlying the
802 A. T. Hadidi
Mathieu flap, the fascia underlying the preputial Another major factor is the use of different
skin (dorsal subcutaneous flap), or a tunica vagina- types of dressings. In a comparative prospective
lis wrap. Using the dorsal subcutaneous flap, Retik study in Cairo University, the role of dressing has
and associates (1994) performed 204 meatal-based been evaluated. The study concluded that dress-
flap hypospadias repairs without a single urethro- ings in general statistically increase the incidence
cutaneous fistula [15]. Snow and coworkers (1995) of complications and fistula formation in hypo-
used the tunica vaginalis blanket wrap for five dif- spadias repair [18]. Other authors showed that
ferent hypospadias repair procedures in 89 patients dressings should be omitted from routine use
[16]. The fistula rate decreased from 20 to 9% after hypospadias repair [19, 20]. For those sur-
when microscope magnification was combined geons who still prefer to use dressings, modern
with the tunica vaginalis blanket wrap. hypospadias dressings with materials such as
Intraurethral catheters and stents may have an Duoderm (ConvaTec®, Bristol-Myers Squibb®)
important role in fistula formation. In a prospec- or silicone foam have also aided in reducing the
tive comparative study in Cairo University, the incidence of fistula.
role of stenting and catheterization has been eval-
uated. The study included 100 patients divided
randomly into a silicone stent group and a no 60.6 Types and Sites of Fistula
stenting group. Although the difference was not
statistically significant, the incidence of compli- The urethrocutaneous fistula encountered after
cations and fistula formation was higher in the hypospadias repair may be single and simple or it
stented group [17]. may be multiple and complicated. Simple tiny
However, if the surgeon prefers to use cathe- fistulas may occur even when experienced sur-
ters or stents, nonreactive silicone stents or cath- geons use good, delicate techniques. They may
eters are better employed. A stent or catheter one occur anywhere along the neo-urethra and even
size smaller than the urethra should be selected to with urethral advancement (due to
permit voiding around the tube should it become devascularization).
plugged or should bladder spasms occur.
a b c
Fig. 60.3 (a–c) Urethral fistula without stenosis. (a) The using Vicryl 7/0 in two continuous layers. A protective
common, classic fistula location is at the coronal sulcus. layer must be used to seal the fistula either from preputial
The first step is to exclude distal stenosis by inserting dartos fascia or from ventral dartos fascia. (c) The fascia
catheter F12 without difficulty. (b) It is important to open should cover the fistula and extend to the tip of the glans
the glanular wings, excise the genu, and close the fistula (©Ahmed T. Hadidi 2022. All Rights Reserved)
However, the commonest sites for fistula forma- larized tissue, tension, infection, and distal
tion are at the corona sulcus (Fig. 60.3), at the site stenosis.
of the original meatus, or in the glans penis. Fistulas Small fistula may appear many years after
at the corona and the glans are more difficult to successful repair (Fig. 60.4). They are usually
treat and have a higher incidence of recurrence. due to tissue reaction to the suture material that
Multiple and complicated fistulas are less remains in the tissues and eventually causes
common; they usually occur due to technical fac- chemical reaction and opens inside into the ure-
tors such as the use of hypoplastic poorly vascu- thra and outside to the skin.
a b
c d
Fig. 60.4 (a–d) Fistula may appear 5–15 years later. (a) (c) Through a sub-coronal incision, the fistula was exposed
Appearance 5 years after hypospadias surgery and foreskin and enlarged and the genu was excised. This resulted in a
reconstruction. Looks normal but parents report appear- wide defect that was closed primarily with Vicryl 7/0, cov-
ance of fistula 1 month earlier. (b) Injection of Betadine is ered with two protective layers. (d) The skin was closed.
very helpful to identify and locate small fistulae. Betadine The final appearance at the end of the operation (©Ahmed
is better than methylene blue as it does not stain the tissues. T. Hadidi 2022. All Rights Reserved)
804 A. T. Hadidi
60.7 Timing of Fistula Closure The next step is to identify the location of the
fistula and exclude other fistulae. The author pre-
Early after fistula formation, there is usually fers to insert the nozzle of a syringe with diluted
inflammation and/or urine extravasation and ure- Betadine® into the meatus and inject the Betadine
thral tissues quickly become edematous and fria- into the urethra. This has the advantage of identi-
ble. In the past, some surgeons used to insert a fying any additional fistulas and tracing the fistu-
Foley catheter into the bladder for 10–14 days lous tract (Fig. 60.4).
and give appropriate antibiotics until tissue indu- One has to carry out extensive debridement
ration and inflammation disappears. However, it and elaborate excision of the fistulous tract down
is possible that fistulas which heal in this way to healthy thin urethral tissue. The fistulous tract
would have healed in any case, without a cathe- is fibrous and has what can be called a “genu” at
ter. In fact, the catheter (even a silicon one) may the junction between the healthy urethra and the
cause irritation that diminishes the chances of rest of the tract (Figs. 60.5 and 60.6). Most sur-
spontaneous healing. geons are reluctant to perform extensive debride-
There is little point in attempting to close a ment and excision of the fistulous tract for fear of
newly formed fistula by means of secondary making a small fistula larger. This may in fact
sutures, and in fact further inflammation will increase the incidence of recurrence. Sometimes,
inevitably result in a larger fistulous opening [1]. the fistulous tract goes a long way before opening
Recently, some surgeons have recommended the into the skin and there may be multiple tiny tracts
use of fibrin glue to aid the healing of a freshly that are not seen except during elaborate dissec-
formed fistula. The results are not promising as tion. It is very important to dissect and separate
yet. widely between the urethra and the overlying
Once a fistula has formed, it is recommended skin all around the fistula.
to wait at least for 6 months. This is the mini- The same principles that apply to hypospadias
mum time required for complete wound healing repair apply to fistula closure (Chap. 15). Delicate
and to allow for full resolution of scar tissue (see instruments are required. Healthy tissue should
Chap. 16). be incorporated in the repair at all times.
Magnification is important. The type of repair
depends on the size and location of the fistula.
60.8 Guidelines and Steps For a small fistula, simple transverse approxima-
of Fistula Closure tion of healthy urethral tissue is often possible. A
running subcuticular inverting suture of 7-0
The first step after anesthesia is to exclude distal Vicryl is used to close the urethral edges. Some
urethral obstruction. It is simple physics that if surgeons prefer to irrigate the urethra with proxi-
there is distal narrowing, urine must find an eas- mal compression to ensure watertight closure,
ier way to pass through. This is usually done by although it is not really necessary. One or prefer-
inserting a catheter size F12 or larger according ably two protective intermediate layer(s) play an
to the age of the patient. Other options include important part in avoiding recurrence. The details
cystoscopy and/or retrograde urethrography. The of the different intermediate layer techniques are
surgeon needs to consider the possibility of func- discussed in Chap. 43.
tional urethral obstruction (FUO) in case of fis- If the fistula is located in the glans and close to
tula after the TIP procedure where the surgeon the meatus, it is usually sufficient to connect it to
may be able to insert catheter size F12 with some the meatus and refashion the glanular wings
difficulty, but this does not exclude distal narrow- around the new wide meatus.
ing (see Chap. 59).
Fig. 60.5 Excision of the fistula genu is crucial for repair. If it is not excised, there is an increased chance of
recurrence
If the fistula is located at the coronal sulcus, the must dissect the fistula tract carefully until he
surgeon needs to dissect adequately around the fis- reaches the junction with the normal urethra and
tula and exclude distal obstruction. The author pre- excise the genu (Fig. 60.5) before attempting to
fers to close the fistula in two layers and then cover close the urethral defect (Figs. 60.4 and 60.6).
the whole area to the meatus with a dartos fascia Some surgeons prefer to use buccal mucosal
layer (Fig. 60.3). The skin is closed in the usual grafts for repair of fistula and have reported good
fashion. A catheter may be inserted for 1 day or the results [22]. However, most surgeons reserve
operation may be done as a day case [21]. grafts such as buccal mucosa, bladder mucosa, or
The surgeon must be aware of the possibility extragenital skin for repair of strictures. As with
of having a fistula tract (Fig. 60.6). The surgeon the flip-flap or island pedicle repairs, a second
806 A. T. Hadidi
Fig. 60.6 Fistula tract: To avoid the recurrence of fistula, the fistula genu which has tendency to coil outward
the surgeon must dissect the fistula tract completely until (©Ahmed T. Hadidi 2022. All Rights Reserved)
he reaches the junction with the normal urethra and excise
layer of subcutaneous tissue is necessary to cover when local well-vascularized skin is used. With
the edges of the flap or onlay patch. Skin cover is all of these repairs, tension on suture lines must
accomplished by means of a rotation flap from be avoided and narrowing of the urethral lumen
either the lateral skin or the scrotum. must be prevented.
For multiple fistulas, a single-stage procedure Fistulas may recur because of errors in tech-
is still a valid option (Figs. 60.7 and 60.8). Two- nique. Contributing factors include inadequate
stage procedures may be reserved for recurrent layers of closure, ischemic tissue, and overlap-
fistula where there is a deficiency of adequate ping suture lines. Infection, tension such as is
healthy local tissue. The fistulas are converted created by edema or hemorrhage, pressure of
into one large fistula. dressings, and occlusion of catheters with urinary
Figures 60.8 and 60.9 show the different pos- extravasation all contribute to fistula recurrence.
sible techniques for fistula closure and skin cover. The difficulty of fistula closure should not be
Figure 60.10 shows multiple fistulas with fistula underestimated. Nevertheless, most fistulas can
tracts excised from a single patient. Meticulous be repaired on an outpatient basis. Careful atten-
attention to layered closure is essential, and the tion to detail and technique results in ultimate
risk of fistula reformation is substantially lower closure of nearly all fistulas.
Fig. 60.7 A child with mid-penile hypospadias and fascia. Six months later, one proximal fistula was closed
hypoplastic ventral structures and chordee. Extensive ven- primarily, and a composite flap of skin and fascia was
tral degloving and excision of the hypoplastic structures used to repair the two distal fistulae. A third protective
was performed. Urethroplasty using the long lateral “slit- layer was put from the preputial dartos fascia and circum-
like adjusted Mathieu” (SLAM) technique was performed. cision was performed at the same time (©Ahmed
The child developed multiple (3) fistulae. However, the T. Hadidi 2022. All Rights Reserved)
child still had the foreskin intact as well as the underlying
808 A. T. Hadidi
Fig. 60.8 (a–c) Different methods of fistula repair (©Ahmed T. Hadidi 2022. All Rights Reserved)
a b c
d e f
Fig. 60.9 (a) Outline of incisions for fistula closure. (b) lium. (e) The dermal flap is then sutured to subcutaneous
Epithelium is removed from all areas except the central tissues. (f) Skin closure can be accomplished in many
“plug” that is to be used to resurface the urethra. (c) ways, e.g., with flaps or by simple straight-line
Elevation of the dermal flap carrying the “epidermal approximation
plug.” (d) The epidermal plug is sutured to the uroepithe-
810 A. T. Hadidi
Fig. 60.10 Multiple fistula tracks excised from one

patient
10. El-Mahrouky A, McElhaney J, Bartone FF, King L. In

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polyglycolic acid and catgut sutures in sterile and
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CE, editor. Plastic and reconstructive surgery of the S0022-5347(17)43415-X.
genital area. Boston: Little Brown; 1973. p. 397–403. 11. Barry Belman A. Editorial comment. J Urol. 1994;
2. Shapiro SR. Complications of hypospadias https://doi.org/10.1016/s0022-5347(17)34993-5.
repair. J Urol. 1984; https://doi.org/10.1016/ 12. Smith D. A de-epithelialized overlap flap tech-
S0022-5347(17)50475-9. nique in the repair of hypospadias. Br J Plast Surg.
3. Duckett JW, Baskin LS. Hypospadias. In: Gillenwater 1973;26:106–14.
JY, Grayhack JT, Howards SS, Duckett JW, editors. 13. Belman AB. The de-epithelialized flap and its influ-
Adult and pediatric urology. 3rd ed. St. Louis: Mosby- ence on hypospadias repair. J Urol. 1994; https://doi.
Year Book; 1996. p. 2549–89. org/10.1016/s0022-5347(17)31670-1.
4. Smith ED. Hypospadias. In: Ashcraft KW, editor. 14. Hill GA, Wacksman J, Lewis AG, Sheldon CA. The
Pediatric urology. Philadelphia: Saunders; 1990. modified pyramid hypospadias procedure: repair
p. 353–95. of megameatus and deep glanular groove vari-
5. Shapiro SR. Fistula repair. In: Ehrlich RM, Alter GJ, ants. J Urol. 1993; https://doi.org/10.1016/
editors. Reconstructive and plastic surgery of the S0022-5347(17)35729-4.
external genitalia: adult and pediatric. Philadelphia: 15. Retik AB, Mandell J, Bauer SB, Atala A. Meatal
Saunders; 1999. p. 132–6. based hypospadias repair with the use of a dor-
6. Durham Smith E. Durham Smith repair of hypospa- sal subcutaneous flap to prevent urethrocutane-
dias. Urol Clin North Am. 1981;8. ous fistula. J Urol. 1994; https://doi.org/10.1016/
7. Greenfield SP, Sadler BT, Wan J. Two-stage repair for S0022-5347(17)32555-7.
severe hypospadias. J Urol. 1994;152:498–501. 16. Snow BW, Cartwright PC, Unger K. Tunica vagi-
8. Snodgrass W, Bush N. Tubularized incised plate nalis blanket wrap to prevent urethrocutaneous fis-
proximal hypospadias repair: continued evolution and tula: an 8-year experience. J Urol. 1995; https://doi.
extended applications. J Pediatr Urol. 2011; https:// org/10.1097/00005392-199502000-00061.
doi.org/10.1016/j.jpurol.2010.05.011. 17. Hadidi AT, Abdala N, Kaddah S. Hypospadias
9. Chrzan R, Dik P, Klijn AJ, de Jong TPVM. Quality repair: is stenting important? Kasr El Aini J Surg.
assessment of hypospadias repair with emphasis on 2003;4:37–44.
techniques used and experience of pediatric urologic 18. Hadidi AT, Abdaal N, Kaddah S. Hypospadias
surgeons. Urology. 2007; https://doi.org/10.1016/j. repair: is dressing important? Kasr El Aini J Surg.
urology.2007.01.103. 2003;4:37–44.
19. Van Savage JG, Palanca LG, Slaughenhoupt BL. A 21. Duckett JW, Caldamone AA, Snyder HM.
prospective randomized trial of dressings versus no Hypospadias fistula repair without diversion (abstract
dressings for hypospadias repair. J Urol. 2000; https:// 11). Proceedings of the Annual Meeting of the
doi.org/10.1097/00005392-200009020-00015. American Academy of Pediatrics. New York. 1982,
20. McLorie G, Joyner B, Herz D, McCallum J, Bagli D, p. 23–28.
Merguerian P, Khoury A. A prospective randomized 22. Nahas BW, Nahas WB. The use of a buccal mucosa
clinical trial to evaluate methods of postoperative care patch graft in the management of a large urethrocuta-
of hypospadias. J Urol. 2001; https://doi.org/10.1016/ neous fistula. Br J Urol. 1994; https://doi.org/10.1111/
s0022-5347(05)66388-4. j.1464-410X.1994.tb09213.x.
Genital Lichen Sclerosus (Balanitis
Xerotica Obliterans): BXO 61
Christiane Schulze and Alexander Springer
Abbreviations 1887. In 1892, Darier presented the histological

description of LS [3]. In 1928, Stühmer first
AMP Anti-microbial peptides reported about balanitis xerotica obliterans in
BMI Body mass index nine adult patients as a sclero-atrophic inflamma-
BXO Balanitis xerotica obliterans tory chronic progressive constrictive scar-
CP Clobetasol propionate forming disease of the glans and penile prepuce
LS Lichen sclerosus that can involve the frenulum, urethral meatus,
PDT Photodynamic therapy and distal urethra. Many names exist for the dis-
SCC Squamous cell carcinoma order: leukoplakia, lichen albus, lichen sclerosus
et atrophicus (this term seems outdated because
in some cases there is no atrophy but hypertro-
phy), and kraurosis genitalis [4, 5].
61.1 Background LS was regarded as a disease of adulthood
until a case of a 7-year-old boy was documented
Lichen sclerosus (LS), also known as balanitis in 1962 [6]. In a study of the Brooke Army
xerotica obliterans (BXO), is a chronic atrophic Medical Center from 1997 to 1999, the incidence
dermatitis of unknown etiology predominantly of LS in male adults was 0.07% [7]. The real inci-
involving the anogenital area of any age group or dence of LS in childhood is unknown. In the lit-
race, with a of cumulation in Caucasians [1]. erature, the pediatric incidence was estimated to
Both sexes are involved: the female-to-male ratio be 10–19% in the foreskin of circumcised boys
ranges from 3 to 10:1, and LS seems to be undi- [8]. Rossi et al. in 2007 reported about the histo-
agnosed [2]. There are two age peaks: childhood logic evidence of LS of the foreskin in 36.6% of
and, among women, the postmenopausal period. boys with severe phimosis, but no LS of the fore-
In men, LS is mainly involving the glans, pre- skin of boys circumcised because of other reasons
puce, and distal urethra, rarely the proximal ure- [9]. In a prospective study by Kiss et al., LS was
thra. LS can arise spontaneously, but may follow found in 40% of boys with phimosis [10]. The
minor trauma or surgery including hypospadias mean age of patients was 8.7 years, with the high-
repair. The first report of LS was by Hallopeau in est incidence between 9 and 11 years. The young-
est boy with LS was 2 years old. In a 10-year
retrospective study from Boston, a total number
C. Schulze · A. Springer (*) of 41 pediatric patients had histological confirmed
Department of Pediatric Surgery, Medical University diagnosis of LS with a median patient age of
of Vienna, Vienna, Austria
e-mail: Alexander.springer@meduniwien.ac.at 10.6 years. The authors come to the conclusion
814 C. Schulze and A. Springer
that the incidence of LS in pediatric patients may tibodies against the extracellular matrix protein-
be higher than previously reported, with the diag- 1 in 67% of patients with LS [19]. The theory of
nosis rarely made by pediatricians [11]. LS as autoimmune disease fits well into the clini-
cal association with other entities like thyroid
diseases, pernicious anemia, alopecia areata, or
61.2 Etiology vitiligo [20, 21].
61.2.1 Infection
61.2.4 Anatomy, Trauma, and Other
The origin of LS is still unclear. An infectious Causes
genesis has been discussed but not proven. LS
has been thought to be an immunologic response Gross anatomical abnormality like phimosis or
to both external infectious factors (bacteria, hep- hypospadias has an association to LS [22].
atitis C, HPV, and Candida) and endogenous Mechanical irritation and physical or chemical
ones like smegma and urine [1, 12–14]. stimuli on already scarred or damaged skin
Dysregulation of AMP (anti-microbial peptides) (“Koebner phenomenon”) are known to occur, so
has been discussed (as in rosacea: hyperreactive trauma (cycling, horse riding, etc.), injury, instru-
response to microbes induces the inflammation). mentation, and sexual abuse have been suggested
as possible triggers of LS [4, 23, 24]. In adult
men, increased body mass index (BMI) and smok-
61.2.2 Endocrinology ing are negative prognostic factors [25]. Moisture
in the genital area and chronic exposure to urine is
Endocrinological factors in the pathogenesis of also thought to trigger LS [26]. It apparently never
LS have been discussed but not proven. Disturbed occurs in males circumcised at birth [22].
function of fibroblasts with elevated production
of collagen caused by the transformed growth
factor β has been shown [15]. Moreover, LS was 61.3 Epidemiology, Symptoms,
found to have increased fibrogenic cytokines (IL- and Diagnosis
4, IL-6), with a low expression of the collagen
inhibitor, interferon-γ, suggesting an imbalance The real epidemiology of LS is not known.
between Th-1 cells and Th-2 cells [3]. Histopathology of foreskins following circumci-
It is thought that in women lower estrogen lev- sion shows LS in up to 40% of the cases [10]. The
els seem to favor occurrence of LS (including onset of LS is usually without symptoms. Early
lower levels of free testosterone and androstene- symptoms of LS are itching and burning and
dione) seen in affected women [16]. Nevertheless, localized erythematous spots on the foreskin or
treatment with hormones has unfortunately glans (Fig. 61.1). Edema of the foreskin may
proven to be not really effective. appear and progress to sclerosis and chronic
inflammation. The typical clinical presentation of
LS is a ring-like distal sclerosis of the prepuce
61.2.3 Immunology and Genetics with white discoloration or plaque formation [27,
28] (Fig. 61.1). The most common presenting
So far, we do not exactly know the mechanism symptoms in the clinical course are obstruction
that induces the immune response leading to and poor urinary stream, dysuria, hematuria,
inflammation and subsequent sclerosis. There is pain, preputial ballooning, incontinence, history
no specific immune reaction described. However, of balanitis, and urinary tract inflammation [11].
data show that there seem to be a genetic suscep- Urethral obstruction may lead to renal impair-
tibility and a link with autoimmune mechanisms ment [29]. Bunker et al. (2009) point out that the
[17, 18]. A study by Oyama et al. showed autoan- principal presenting scenario of LS in the adult
61 Genital Lichen Sclerosus (Balanitis Xerotica Obliterans): BXO 815
male is rash and difficulty having intercourse feels thickened. Cystourethroscopy may show
(male dyspareunia) [30]. Urethral involvement urethral involvement with areas of pallor of the
starts at the meatus. Although traditionally LS mucosa or even ulceration [31]. If there are uri-
was thought to be localized on the distal urethra, nary symptoms or urinary infection (especially
Depasquale et al. reported of urethral involve- surgery (even many years ago)), the suspicion of
ment proximally as far back as the prostate [31]. LS of the urethra should be raised and subsequent
In 2009, Bochove-Overgaauw et al. conclude that ultrasound and/or referral to the pediatric urolo-
routine biopsy of the foreskin after circumcision gist taken into account.
for pathological phimosis should be carried out, LS may resemble erythroplasia of Queyrat,
because clinical findings underestimate the inci- lichen planus, leukoplakia, scleroderma, or squa-
dence of LS in a high percentage of the cases mous cell carcinoma. However, histology of LS
[32]. Nevertheless, it has not been found in blad- appears unique. It presents with edema of the upper
der mucosa [31]. Clinically, the penile urethra dermis and hyalinization of elastic fibers of the
reticular dermis. The microscopic picture is also
characterized by epithelial hyperkeratosis, hydropic
degeneration of basal cells, subepithelial fibrosis,
and dermal lymphocytic infiltration deep to the hya-
linized band in the upper dermis (Fig. 61.2).
The inflammatory infiltrate and region of scle-
rosis are characterized by CD4- and CD8-positive
lymphocytes, macrophages, and mast cells.
Melanocytes are reduced or missing [4, 33, 34].
Indications for biopsy:
• Candida infection
• Leukoplakia
Fig. 61.1 Foreskin with BXO. There are thickening and • Squamous cell carcinoma
erythematous spots on the foreskin. The foreskin is nar- • Lichen ruber mucosae
row and cannot be retracted (Courtesy of Ahmed
T. Hadidi)
• Lichen nitidus (only for extragenital LS)
Fig. 61.2 The micro-

scopic picture of BXO is
characterized by epithelial
hyperkeratosis, hydropic
degeneration of basal cells,
hyalinization of elastic
fibers of the reticular
dermis, subepithelial
fibrosis, and dermal
lymphocytic infiltration
deep to the hyalinized
band in the upper dermis
(Courtesy of Dr. Anke
Koller, Department of
Pathology, Medical
University of Vienna)
• Balanitis plasmacellularis Table 61.1 Treatment protocol for LS

• Erythroplasia of Queyrat Involvement Treatment
• Morphea/scleroderma Limited to the Circumcision (histology)
• Mucosal, cicatricial pemphigoid foreskin Topical steroids as an alternative in
mild cases
• Balanitis psoriatica
Glans Mild/moderate: circumcision and
• Contact dermatitis observation (± topical steroids)
• Drug reactions Severe: glans resurfacing
• Extramammary Paget Meatus Early: dilatation (± topical steroids)
• Sexual abuse Established: meatotomy/meatoplasty
• Vitiligo (and urethroscopy)
Redo urethroplasty ± buccal mucosa
graft
Biopsy is strongly recommended in: Urethra Excise LS urethra and replace with
buccal mucosa graft
• New papules, nodes, or ulcerations of Short segment: buccal mucosa (inlay)
unknown origin Long segment: buccal mucosa
• Other suspicion of malignancy (staged repair)
• Uncertainty in diagnosis
• Treatment with highly potent corticoid ste-
roids not successful 61.4.1 Medical Treatment
• All surgical specimens (foreskin, penile skin,
glans and meatal region, urethra!) Medical treatment of BXO has been proposed,
but prospective, controlled, multicenter studies
are still lacking. Treatment regimens contain cor-
61.4 Treatment ticosteroids and immunosuppressive and antibi-
otic agents. Considering the chronic course of
Recognition by the general practitioner or pedia- LS, the topic therapy should have three
trician, early diagnosis, and treatment of LS, approaches:
while the lesion is localized, are essential because
extensive involvement of penile tissue compli- 1. To stop the inflammatory process as fast as
cates therapeutic options and worsens prognosis. possible
Especially, in pediatric populations, an alarming 2. To prevent recurrence of LS
incidence of missed diagnoses of LS is reported 3. To avoid side effects of treatment
[11, 35, 36]. LS is treated medically and surgi-
cally. Medical treatment is thought to be less 61.4.1.1 Corticosteroids
curative and more of palliative character in exten- Since the 1960s, corticosteroids have been used
sive disease. Surgery is thought to be more defin- for the treatment of LS [6, 37]. In mild forms
itive and curative and includes circumcision, with erythema, meatal narrowing, and preputial
correcting of meatal stenosis, and various ure- fibrosis, topical application of corticosteroids for
throplasty techniques. Probably, the largest series 4–8 weeks is indicated and may be repeated in
of treatment of LS was published by Depasquale the case of recurrence. In a prospective, placebo-
in 2000 [31]. They suggest a treatment protocol controlled, double-blind, randomized study by
which is shown in Table 61.1. After treatment, Kiss et al. in 2000, it was clearly demonstrated
patients should be kept under long-term that local corticosteroid treatment is effective in
follow-up. LS when the inflammatory mechanism is active
and irreversible tissue damage has not occurred
[38, 39]. Ointments are preferable to creams limus for LS is narrow-sighted, clinically unnec-
because they are subjectively more pleasant, essary and potentially dangerous” [22]. However,
cause less contact allergies in the long run, and Ebert et al. in a prospective phase 2 study could
might be more efficient (occlusive effect). show that tacrolimus 0.1% ointment applied
Clobetasol propionate (CP) 0.05% is effective in immediately after surgery of fully established LS
mild LS. Patients start with application of CP is a tolerable and most probably safe adjuvant
twice a day for 4 weeks and reduce the applica- novel treatment option [46]. The use of topical
tion every second, third, fourth, fifth, and sixth calcineurin inhibitors is still off-label, and they
day and remain for 8–12 weeks with only a should not be applied on the irritated skin,
weekly treatment. If the meatus is affected, CP because it will cause severe burning.
ointment should be applied by meatal dilator or
cotton wool bud for the night. Early treatment of
the affected meatus might prevent progression to 61.4.2 Surgical Treatment
the urethra [40]. Urethral involvement may be
approached by local CP instillation via urethral In minor cases or limited disease, circumcision is
catheter. In mild forms of LS, therapy can be the strategy of choice. In the majority of cases,
switched (after 2–6 weeks) to mometasone circumcision is curative. Since “LS has a predi-
furoate or methylprednisolone aceponate. These lection for the warm, moist, urine-exposed envi-
steroids have a much better therapeutic index and ronment that exists under the foreskin” [31], the
will cause significantly less steroid atrophies and effectiveness of this procedure seems logical.
other side effects [41]. After 2–3 months, the Histology should always be performed to estab-
response to topical treatment should be assessed. lish definitive diagnosis. In severe cases, glandu-
Unfortunately, there is no clear concept for pre- lar resurfacing or extensive preputial adhesions,
venting the recurrence of LS. complete excision of the LS tissue, and skin
grafting are necessary [47].
61.4.1.2 Calcineurin Inhibitors Urethral involvement by LS can be seen in up
(Tacrolimus) to 30% of the cases, and it varies from meatal
In 2003, Assmann et al. reported the successful stenosis to more extensive pan-urethral strictures
treatment of vulvar LS with tacrolimus [42]. [48]. Treatment of these stenotic lesions remains
Tacrolimus (FK506, Protopic®) is a macrolide- a challenge. Factors to be considered when
derived immunomodulator used successfully in choosing the most suitable method of reconstruc-
transplantation medicine and currently has been tion of these strictures are the re-establishment of
licensed for treating atopic dermatitis. Tacrolimus a normal caliber urethra, achievement of a good
is an inhibitor of the second messenger calcineu- cosmesis with a normal slit-like meatus with
rin. The immunosuppressive mechanism lies in long-term stability of the tissue used for recon-
inhibiting the production of interleukin-2 and struction, and avoiding of recurrence of LS [49].
interferon-γ and subsequent T-cell activation. Radical excision of all affected parts of the skin
The drug also has T-cell-independent effects not and urethra is essential (Fig. 61.3).
yet fully understood (inhibition of mast cells and In cases of meatal stenosis, Kumar and Harris
basophil granulocytes) [20, 42, 43]. Tacrolimus advocate meatoplasty followed by regular dilata-
has analogous clinical effects to corticosteroids tion, if necessary [50]. Simple meatotomy in LS
with less side effects. Systemic absorption is regularly is followed by re-stenosis [33]. Searles
minimal. Major side effects are temporary burn- and Mackinnon promote home dilation of meatal
ing and pruritus [44]. The first report of success- stenosis in LS as an easy, successful, and comfort-
ful treatment of LS with tacrolimus was by able method [51]. In 2004, Malone proposed a
Pandher et al. [45]. Controversially, Bunker states modified meatoplasty in distal LS including ven-
that the autoimmune character of LS is not proven tral and dorsal meatotomy with an inverted
and so the advocacy for the use of “topical tacro- V-shaped relieving incision to correct puckering
a b
c d
Fig. 61.3 A 9-year-old child with BXO, 7 years after the involved area was performed. (d) After complete heal-
hypospadias correction. (a, b) The BXO involved the ing, the child has a wide healthy meatus. No further sur-
meatus and the remaining foreskin. (c) Radical excision of gery was performed (Courtesy of Ahmed T. Hadidi)
with surprisingly good long-term results [52, 53]. penile skin flap urethroplasty and a two-stage free
In 2009, Das et al. promote a lingual mucosal ure- graft urethroplasty using non-genital skin [55].
throplasty for anterior urethral strictures with All one-stage procedures failed, and they strongly
comparable complication rates to buccal mucosa recommend a two-stage procedure with non-gen-
with minimal donor site complications without ital skin because of the high recurrence rate of
any functional or cosmetic deficit [54]. In 1998, LS. The strategy of Bracka consists of complete
Venn and Mundy compare a one-stage pedicled excision of LS and substitution [31]. It is pointed
out that substitution urethroplasty using genital or

extragenital skin provides no cure but recurrence.
A two-stage procedure graft from buccal mucosa
and in extensive case bladder mucosa is advo-
cated. In about 100 cases (primary urethroplasty
and recurrence after other procedures), there were
no LS recurrences so far [31]. On the other hand,
Dubey et al. recommend one-stage dorsal onlay
buccal mucosa urethroplasty with excellent cos-
metic and functional results and a low recurrent
stricture rate (12%) [49]. They point out that two-
stage procedures tend to a higher revision rate.
Levine et al. report about a higher recurrence rate
Fig. 61.4 BXO may occur many years after hypospadias
in 13 patients with anterior urethral stricture in LS repair. The typical white appearance of the remaining
using a one-stage ventral or dorsal buccal mucosa foreskin and the meatus (Courtesy of Ahmed T. Hadidi)
onlay urethroplasty and hence advocate a two-
stage urethroplasty [56]. Regarding the material
used in anterior urethroplasty, Trivedi et al. 61.5 LS and Hypospadias
reviewed 153 patients with anterior urethral stric-
ture due to LS [57]. They concluded that a free Astonishingly, LS in patients with hypospadias
graft urethroplasty using non-genital skin is the has not been subject of larger studies so far,
material of choice. Using genital skin had a suc- although LS should be regarded as an important
cess rate of 4% only. prognostic factor in hypospadias surgery. The
exact incidence of LS in hypospadias is not
known. In 1987, Bale et al. reported about an
61.4.3 Other Treatment Options incidence of 4.2% of LS in a series of 166 cases
with hypospadias [60]. In 2002, Mattioli et al.
If the abovementioned treatments fail, the follow- reported the histologic findings of the prepuce in
ing modalities are listed in literature: boys with hypospadias [61]. 46% showed unspe-
cific balanitis; 15% showed LS. The total patient
61.4.3.1 Photodynamic Therapy number in this study was very small. Others
(PDT) report of a very low incidence of LS in hypospa-
PDT is established in the treatment of non- dias: In a consecutive series of more than 500
melanoma skin and bladder cancer. There are distal hypospadias repair, Snodgrass et al.
individual reports on the treatment of LS. In reported 1 case of meatal stenosis due to LS
boys/men, this could be an option to treat areas (0.18%) [62].
difficult to access [58]. Application of a photo- LS after hypospadias repair is a rare but chal-
sensitizer and light in children is challenging. lenging complication that causes significant mor-
bidity, i.e., meatal stenosis (Fig. 61.4), urethral
61.4.3.2 Cryotherapy stricture, or complete breakdown.
Cryotherapy for LS is experimental and should Ragavan et al. call it a rare complication after
only be used as a second-line treatment [1]. hypospadias repair [63]. Hensle et al. report
about the results of secondary hypospadias
61.4.3.3 Systemic Therapies repair using buccal mucosa (tube graft) [64]. All
There are a few reports using systemic acitretin, patients (n = 3) with preoperative diagnosis of
fumarates, methotrexate, corticosteroids, and LS had a significant postoperative complication.
adalimumab [1, 59]. In a study of the management and outcome of
complex hypospadias repair, Amukele et al.
report about 2 cases of postoperative urethral 61.6 Long-Term Outcome

stricture caused by LS in 100 patients (1 after of LS-Related Urethral
free skin graft, 1 after Thiersch-Duplay) [65]. Surgery
Surprisingly, other studies of the treatment of
complicated hypospadias repair do not elaborate A major drawback in pediatric surgery is the lack
on the diagnosis of postoperative LS or LS [66, of long-term follow-up. A recent systematic
67]. Kumar and Harris report about 7 cases of review of long-term outcomes of one-stage aug-
histologically confirmed LS after proximal mentation anterior urethroplasty (buccal mucosa
hypospadias repair using the Harris split prepu- and preputial grafts) showed that the long-term
tial one-stage repair in 257 patients appeared at a success rates of augmentation urethroplasty
later date from 1 year to 16 years after primary appear to be worse than previously expected,
repair [50]. The patients were treated with two- especially following hypospadias repair and LS
stage urethroplasties using postauricular full- [69]. A 7-year online audit in the UK of more than
thickness skin grafts and buccal and bladder 4800 men showed that a significant part of recon-
mucosa. The number of reoperations ranged structive urethral surgery in adulthood is associ-
from two to seven; then the recurrence rate was ated with LS and/or previously repaired
0%. Uemura et al. also report about three cases hypospadias [70]. In a large retrospective series of
of LS after hypospadias repair [8]. Two cases 408 adult men with subsequent urethral strictures
occurred after the repair; the third showed LS of following previous hypospadias repair, there was
the prepuce before the repair and developed ure- correlation between LS and unfavorable outcome
thral stricture afterward. In their series, the inci- [71]. All these data remind us of the importance of
dence of postoperative LS is 0.4%. In the very transitioning hypospadias patients from pediatric
large series of LS reported by Depasquale, they to adult services: hypospadias patients presenting
identified 82 patients with hypospadias and LS to adult urologists do so with a wide range of
problems [31]. 29 patients presented for primary symptoms and problems, including urethral stric-
repair and showed to have LS; 53 patients devel- ture, lower urinary tract symptoms, persisting
oped LS after unsuccessful hypospadias repair. hypospadias, and urinary infection (see Chap.
The majority of these patients were adults with a 65). Up to 13% (range 8–43%) of those patients
long history of recurrent strictures and multiple complain of LS-related problems [72].
operations. After two-stage mucosal urethro-
plasty, there were no recurrences. In a large ret-
rospective study by Sultan et al., it could be 61.7 LS and Penile Cancer
shown that LS is a risk factor for complications:
In 157 boys undergoing redo surgery (fistula Penile squamous cell carcinoma (SCC) may
21.7%, meatal stenosis 28.7%, urethral stricture occur on pre-existing lesions of LS. There are
17.8%, and total dehiscence 31.8%), a signifi- anecdotal reports of men developing penile SCC
cant number of patients had histological proven in association with LS [4]. In the extensive study
LS [68]. In conclusion, LS in hypospadias should by Depasquale et al. of 522 patients with LS,
be treated with thoroughness and accuracy fol- there was only a 2% rate of SCC [31]. On the
lowing the principles mentioned above: com- other hand, in a study by Perceau et al. in 44% of
plete excision of LS skin, urethroscopy in patients with penile carcinoma, there was an
suspicion of LS, consideration of a two-stage association with histological features of LS [73].
procedure, the possibility of using mucosal or In a study by Pietrzak et al., 28% of patients with
extragenital material for reconstruction penile malignancies had histological diagnosis of
(Figs. 61.5, 61.6, and 61.7), and last but not least LS [74]. As a conclusion, they advocate close
long-term follow-up. follow-up of patients with LS.
Fig. 61.5 BXO involving the meatus and distal urethra 6 years after hypospadias repair. The foreskin and the involved
distal urethra were radically excised, and a buccal mucosal graft was applied (Courtesy of Ahmed T. Hadidi)
Fig. 61.6 BXO involving the remaining foreskin and meatus. A trial of medical treatment and then urethrotomy was
performed (Courtesy of Ahmed T. Hadidi)
Fig. 61.7 BXO involving the meatus and distal urethra throtomy, excision of the involved mucosa and buccal
after hypospadias repair and circumcision. The patient mucosal graft was applied. Distal urethroplasty was per-
received medical treatment for 1 month followed by ure- formed 1 year later (Courtesy of Ahmed T. Hadidi)
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65. Amukele SA, Stock JA, Hanna MK, Snodgrass W, M, Lardennois B, Bernard P. Lichen sclerosus is fre-
Amukele SA, Keating M. Management and outcome quently present in penile squamous cell carcinomas
of complex hypospadias repairs. J Urol. 2005; https:// but is not always associated with oncogenic human
doi.org/10.1097/01.ju.0000176420.83110.19. papillomavirus. Br J Dermatol. 2003; https://doi.
66. Snodgrass WT, Elmore J, Snyder H, Mokhless IAS, org/10.1046/j.1365-2133.2003.05326.x.
Kryger J. Initial experience with staged buccal graft 74. Pietrzak P, Hadway P, Corbishley CM,
(Bracka) hypospadias reoperations. J Urol. 2004; Watkin NA. Is the association between bala-
https://doi.org/10.1097/01.ju.0000139954.92414.7d. nitis xerotica obliterans and penile carcinoma
67. Söylet Y, Gundogdu G, Yesildag E, Emir underestimated? BJU Int. 2006; https://doi.
H. Hypospadias reoperations. Eur J Pediatr Surg. org/10.1111/j.1464-410X.2006.06213.x.
2004;14:188–92.
Urethral Diverticula and Acquired
Megalourethra 62
Ahmed T. Hadidi
Abbreviation flap tubes, with only 3% occurring after a single-

faced flap and 13% after double-faced flaps [4];
TIP Tubularized incised plate in another series (1996), Gonzalez reported a 5%
incidence of diverticula among 18 cases of dou-
ble onlay preputial island flap urethroplasty [8].
Castanon reported an incidence of megaurethra
62.1 Introduction and Incidence of 4.9% in 42 patients who underwent the Duckett
technique and no case out of 38 onlay island flap
Urethral diverticula are less common complica- procedures [2].
tions than fistulas and stenoses after hypospadias
repair. Diverticula are reported in 4–12% of
patients after preputial island flap urethroplasty 62.2 Presentation and Diagnosis
[1–3], but are extremely rare after meatal-based
flap urethroplasty and after tubularized incised Urethral diverticula are generally diagnosed at
plate techniques (0–0.6%) according to de long-term follow-up, from a few months to sev-
Badiola et al. (1991) [4] and Kass and Chung eral years after the urethroplasty, suggesting a
(2000) [5]. In a review of 5 years’ experience and process of gradual dilatation of the new urethra.
1022 hypospadias repairs, Baskin reported diver- The diagnosis is easy when ventral urethral
ticula to occur in 7% of the patients treated using “ballooning” of the anterior urethra is seen dur-
the tubularized preputial island flap technique ing voiding. The other common presentation is
and in no case of onlay island flap procedure [6]. postvoid dribbling. When the diverticulum is
Elbakry observed diverticula in 4% of 74 prepu- associated with a distal urethral or meatal ste-
tial island tube urethroplasty [7]. de Badiola et al. nosis, weak stream and dysuria can be the pre-
reported diverticula in 9% of 54 preputial island senting signs. Less often, hematuria, irritative
voiding symptoms, urinary infections, and ure-
thral calculus formation are the principal
complaints.
A. T. Hadidi (*) Voiding cystourethrography permits evalua-
Hypospadias Center and Pediatric Surgery tion of the presence and the size of the diverticu-
Teaching Hospital of the Johann Wolfgang Goethe lum and, if present, a distal urethral stenosis or a
University, Offenbach, Frankfurt, Germany tiny urethral fistula.
828 A. T. Hadidi
62.3 Types of Diverticula 62.4 Etiology and Mechanism

of Diverticulum Formation
• Distal diverticulum: Anterior diverticula are
less common and are usually due to distal • Distal stenosis: This is usually the cause in
stenosis (e.g., after “tubularized incised distal diverticula and can be a contributing
plate” (TIP) repair) (Fig. 62.1). They are easy factor for posterior diverticula. When dealing
to diagnose because of abnormal ballooning with any diverticulum, distal urethral or
of the distal urethra during micturition asso- meatal stenosis must always be ruled out
ciated with narrow stream or dribbling of (Fig. 62.2).
urine. • Lack of supportive tissue around the neoure-
• Proximal diverticulum: The majority of thra: During micturition, the stream of urine
diverticula are located in the newly recon- changes direction from vertically downward
structed proximal urethra. When the dilata- to horizontally forward at the junction between
tion involves the whole length of the the membranous and spongy (bulbous) ure-
neourethra, it has been termed “acquired thra. This is the weakest point, and if the ure-
megalourethra” by Aigen et al. (1987) [1]. thra is thin or new and not supported by the
Less often, if a proximal segment of the ure- corpus spongiosum, there is a good chance to
thra with absent or hypoplastic corpus spon- develop a diverticulum (Fig. 62.3).
giosum is left in place during surgery, a
diverticulum may appear in that tract, involv- The other important factor is the desire to
ing therefore the native urethra proximally to bring the meatus to the tip of the glans in proxi-
the anastomosis. mal hypospadias. It is to be noted that normally,
a b
Fig. 62.1 (a) Diagram to explain the mechanism of ante- of the anterior urethra in a 4-year-old boy who underwent
rior diverticulum development. There are usually distal tubularized island flap urethroplasty at age 1 year
stenosis and poorly developed corpus spongiosum. (b) (©Ahmed T. Hadidi 2022. All Rights Reserved)
Voiding cystourethrogram showing a large diverticulum
62 Urethral Diverticula and Acquired Megalourethra 829
a b
Fig. 62.2 (a) Severe meatal stenosis following TIP procedure. (b) Incision of the huge diverticulum. It was unrealistic
to reconstruct the distal urethra at the same operation (©Ahmed T. Hadidi 2022. All Rights Reserved)
the glanular wings do not meet in midline.

Instead, they are separated by the frenulum (see
Chap. 4: Anatomy). In most modern surgical
repair, the surgeon usually sutures the glanular
wings together in order to have a terminal slit-
like meatus. This increases the resistance against
the urine flow through the urethra and increases
the chances of diverticulum formation.
In addition, the characteristics of the flap used
for the urethroplasty can also play a role. In
double- faced flap urethroplasties, de Badiola
et al. reported a significantly higher incidence of
diverticula when the more elastic inner preputial
layer was used for the urethroplasty [4]. The sub-
sequent use of the outer preputial layer for the
urethroplasty and the inner layer for the coverage
of the shaft resulted in a significant decrease in
the incidence of this complication, according to
de Badiola et al. and Gonzales et al. [4, 8]. An
Fig. 62.3 A diagram to show the mechanism of proximal inadequate vascular supply and the lack of sup-
diverticulum formation. The weakest point is the junction portive periurethral tissue to prevent expansion of
between the membranous and spongy (bulbous) urethra the neourethra are considered significant concur-
where the urine stream changes direction from vertical to
horizontal. If the corpus spongiosum is deficient (in proxi- rent factors. A diverticulum can also result from
mal and perineal hypospadias), there is a higher chance urine extravasation at the site of the anastomosis,
for posterior urethral diverticulum formation (©Ahmed and therefore it may be associated with a urethral
T. Hadidi 2022. All Rights Reserved) fistula.
830 A. T. Hadidi
It is to be noted that urethral diverticula 62.5.3 T

he Use of Grafts Rather Than
were rare when the Denis-Browne technique Flaps in Proximal and Perineal
was popular in the 1950s, mainly because he Hypospadias
did not try to bring the meatus to the tip of the
glans. Many surgeons in the western hemisphere have
shifted away from flaps to two-stage grafts
(Cloutier/Bracka technique) in proximal and per-
62.5 Prevention of Diverticulum ineal hypospadias because two-stage graft ure-
Formation throplasty seems to have less tendency to form a
diverticulum. When grafts are fixed to the under-
62.5.1 F
ormation of Wide Urethra lying tissue in the first stage, they usually heal
and Meatus with some degree of fibrosis, and they become
more hard. This may reduce the incidence of
An important tip is to reconstruct urethra around diverticular formation.
a wide catheter (preferably size F12 or larger). If a free skin patch is necessary, suturing the
This will help to reduce the resistance against the skin graft dorsally rather than ventrally with
urine flow especially in the region of the glans. subsequent fixation of the skin graft to the
Another option is to have the meatus in proxi- tunica albuginea of the corpora has been advo-
mal and perineal hypospadias at the level of the cated by Lenzi, Barbagli, and Stomaci, in order
corona to avoid the high resistance at the region to avoid the development of a urethral divertic-
of the glans as suggested by Mark Zaontz (per- ulum [11].
sonal communication).
A third option that is recommended by
Hüseyin Özbey is to try to mimic the normal 62.6 Management
anatomy and have some skin between the two of Diverticulum
glanular wings and not to suture the two glanular
wings together [9]. 62.6.1 Mild or Small Diverticulum
If the diverticulum is mild, is small, and only

62.5.2 Approximation presents by few drops after passing urine, it is
of the Bifurcated Corpus usually enough to shake the penis and massage
Spongiosum and Surrounding the ventral side of the penis. This is not uncom-
Fascia mon in centers that advocate flaps for perineal
hypospadias.
Whenever possible, the surgeon should try to
bring the bifurcated corpus spongiosum over the
neourethra in the midline to avoid ballooning and 62.6.2 A
nterior or Distal
diverticulum formation. It is usually possible to Diverticulum
bring the surrounding fascia and tissues around
and over the neourethra as a protective intermedi- This is usually due to terminal stenosis. If early
ate layer. This reduces the incidence of fistula diagnosed and managed, meatotomy may be
formation as well as diverticulum formation. enough to correct the terminal stenosis. Otherwise,
Also, reapproximation of the bulbocavernosus formal incision of the narrow segment, excision
muscle over the flap has been suggested [10] in of the diverticulum, and urethroplasty around a
order to increase the periurethral support. wide catheter are warranted (Fig. 62.4).
Fig. 62.4 Large distal diverticulum. The first step was to extra urethral mucosa and urethroplasty around catheter
dissect the skin off the diverticulum. The distal obstruc- F12 (©Ahmed T. Hadidi 2022. All Rights Reserved)
tion was incised. This was followed with excision of the
62.6.3 P
osterior or Proximal an adequate and careful technique, since diver-
Diverticulum ticulectomy can be followed by the same compli-
cations as hypospadias correction, e.g., urethral
Large proximal urethral diverticula usually stenosis and fistula formation.
require surgical correction. Relief of a distal
obstruction, when present, by means of urethral
dilatation, meatotomy, and meatoplasty is of par- 62.7 Surgical Tips and Tricks
amount importance for the ultimate outcome of
surgery. The urethra must be inspected carefully The author prefers to incise the skin and the
for any micro-fistula which might otherwise be urethral diverticulum all through until he
overlooked. In order to avoid overlapping suture reaches normal caliber urethra. The appropriate
lines, degloving of the penis, subsequent diver- caliber catheter is introduced into the bladder. It
ticulectomy, and a “pants over vest” overlapping is very important to use stay sutures to identify
of the closed urethral defect with periurethral tis- the appropriate width for the neourethra before
sue have been advocated by Zaontz et al. (1989) excision of the excess epithelium. Based on the
[12]. Aigen et al. recommended treating the stay sutures, the mucosa or epithelium is
acquired megalourethra in a fashion similar to incised, leaving the rest of the wall to use as
that of primary megalourethra, with a de- covering protective layers after urethroplasty.
epithelialized layer to prevent both fistula forma- Failing to observe these points, the surgeon
tion and diverticulum recurrence [1]. Repair of may face the situation that at some areas he
the urethral diverticulum by plication has also excised more than he should and may cause
been suggested, in order to preserve the urethral another urethra narrowing and complications
blood supply, avoid the urethral opening and (Fig. 62.5).
fistula formation, and guarantee adequate rein- Recurrence of the diverticulum may rarely
forcement of the ventral urethra [13]. The correc- occur after surgical repair, particularly if patho-
tion of any urethral diverticulum always demands genetic factors have not been eliminated.
832 A. T. Hadidi
a b
c d
Fig. 62.5 (a) A huge diverticulum that developed 1 year needed for the urethroplasty and apply stay sutures before
after perineal hypospadias correction. It is important to making any incisions. (c) Only the mucosa (epithelium) is
ensure that there is no distal narrowing (which was the incised at the appropriate distance, leaving the rest of the
case here) and a catheter size F12 went through without wall intact. (d) The remaining wall is used as a protective
any problem. (b) The diverticulum was incised and the and supportive layer after urethroplasty (©Ahmed
dilated segment extended from the coronal sulcus to the T. Hadidi 2022. All Rights Reserved)
perineum. It is important to mark the appropriate width
Acknowledgments The author would like to acknowl- throplasty. J Urol. 1987; https://doi.org/10.1016/
edge and thank Prof. Paolo Caione and Simona Nappo for S0022-5347(17)44185-1.
their excellent chapter on diverticulum in the first edition 2. Castañón M, Muñoz E, Carrasco R, Rodó J, Morales
of the book. Part of the information included here is from L. Treatment of proximal hypospadias with a tubular-
their chapter in the first edition. ized island flap urethroplasty and the onlay technique:
a comparative study. J Pediatr Surg. 2000; https://doi.
org/10.1053/jpsu.2000.16412.
3. Elder JS, Duckett JW. Urethral reconstruction fol-
References lowing an unsuccessful one-stage hypospadias
repair. World J Urol. 1987; https://doi.org/10.1007/
1. Aigen AR, Khawand N, Skoog SJ, Belman BF00326770.
AB. Acquired megalourethra: an uncommon com- 4. de Badiola F, Anderson K, Gonzalez R. Hypospadias
plication of the transverse preputial island flap ure- repair in an outpatient setting without proxi-
mal urinary diversion: experience with 113 9. Özbey H. The mystery of Jacob Henle’s ‘septum glan-
urethroplasties. J Pediatr Surg. 1991; https://doi.
dis’. J Anat. 2019; https://doi.org/10.1111/joa.12965.
org/10.1016/0022-3468(91)90996-7. 10. Mundy AR, Stephenson TP. Pedicled preputial
5. Kass EJ, Chung AK. Glanuloplasty and in situ patch urethroplasty. Br J Urol. 1988; https://doi.
tubularization of the urethral plate: long-term fol- org/10.1111/j.1464-410X.1988.tb09161.x.
low up. J Urol. 2000; https://doi.org/10.1016/ 11. Lenzi R, Barbagli G, Stomaci N. One-stage skin
s0022-5347(05)67234-5. graft urethroplasty in anterior middle urethra: a new
6. Baskin LS, Duckett JW, Ueoka K, Seibold J, Snyder procedure. J Urol. 1984; https://doi.org/10.1016/
HM. Changing concepts of hypospadias curvature lead S0022-5347(17)50563-7.
to more onlay island flap procedures. J Urol. 1994; 12. Zaontz MR, Kaplan WE, Maizels M. Surgical cor-
https://doi.org/10.1016/S0022-5347(17)34915-7. rection of anterior urethral diverticula after hypospa-
7. Elbakry A. Complications of the preputial island dias repair in children. Urology. 1989; https://doi.
flap-tube urethroplasty. BJU Int. 1999; https://doi. org/10.1016/0090-4295(89)90064-2.
org/10.1046/j.1464-410x.1999.00097.x. 13. Heaton BW, Snow BW, Cartwright PC. Repair
8. Gonzalez R, Smith C, Denes ED. Double onlay prepu- of urethral diverticulum by plication. Urology.
tial flap for proximal hypospadias repair. J Urol. 1996; 1994;44:749–52.
https://doi.org/10.1016/S0022-5347(01)65831-2.
Management of Failed
Hypospadias Surgery 63
Ahmed T. Hadidi
Abbreviations Chap. 58, and functional urethral obstruction

(FUO) is discussed in Chap. 59, hypospadias fis-
BILAB Bilateral-based flap tula in Chap. 60, balanitis xerotica obliterans
BXO Balanitis xerotica obliterans (BXO) in Chap. 61, and urethral diverticulum
DYG Double Y glanulomeatoplasty following hypospadias surgery in Chap. 62. The
FUO Functional urethral obstruction use of Cecil-Culp principle in salvage hypospa-
LABO Lateral-based onlay flap dias is mentioned in Chap. 41. Moneer Hanna
SLAM Slit-like adjusted Mathieu describes his experience and personal approach
TALE Tunica albuginea longitudinal in the management of complex and redo hypo-
excision spadias in 402 patients in Chap. 64.
TIP Tubularized incised plate This chapter deals with failed hypospadias
repair and complete wound dehiscence after mul-
tiple operations. Those patients usually suffer
from extensive scarring and lack of local healthy,
63.1 Introduction well-vascularized tissues.
Durham Smith wrote in his foreword to the first

edition of the book: 63.2 hy Complications Are
W
I know of no surgery that can quite humble a sur- Common After Hypospadias
geon as much as hypospadias - It carries a vicious Surgery?
bite with its list of complications, most galling
when they can occur years later. [1] In the 1950s, a perineal urethrotomy was a stan-
dard procedure when performing Denis-Browne
The management of different hypospadias technique for proximal hypospadias (Chap. 53).
complications is covered in several chapters: The perineal urethrotomy used to close spontane-
Early complications are discussed in Chap. 57 ously later without the need of surgical interven-
and meatal stenosis and urethral stricture in tion. It is the author’s experience among many
others that when urethral repair or anastomosis is
performed after hair coil (Chap. 50, Fig. 50.7) or
A. T. Hadidi (*) trauma to the penis, the incidence of fistula or
Hypospadias Center and Pediatric Surgery complications are almost zero, in contrast to the
Teaching Hospital of the Johann Wolfgang Goethe high incidence of complications after hypospa-
University, Offenbach, Frankfurt, Germany dias repair.
836 A. T. Hadidi
Chapter 3 explains the “migration hypothesis” hypoplastic tissues by mobilizing healthy, well-
regarding the development of the male human vascularized tissue or fascia from the prepuce or
urethra. Under the influence of testosterone and the scrotal region and use hyperbaric oxygen if
other hormones, the urethral orifice that is pres- available.
ent at the level of perineum during the 6–7 weeks
of gestation migrates together with the corpus
spongiosum to the tip of the glans penis. 63.3 Emergency Management
Chapter 7 explains the “disorganization
hypothesis” and how, in hypospadias, there are A patient may present as an emergency if he can-
disorganized growth and arrested distal migration not pass urine due to extensive swelling of the
and hypoplasia of all the ventral penile s tructures penis. History of the operation performed is very
to a variable degree. In other words, the ventral important. If the child has a flap repair and fully
penile structures are atretic, hypoplastic, and epithelialized neourethra (like “slit-like adjusted
poorly vascularized. These hypoplastic tissues are Mathieu” (SLAM), Chap. 22), simple insertion
poorly vascularized and do not heal as expected. of a narrow F6 or F8 catheter for 2–3 days
Hence, it is an essential part of hypospadias repair through the urethra is usually enough. If the child
to bring healthy well-vascularized tissues to the had a “tubularized incised plate” (TIP, Chap. 26)
ventral side of the penis. This is usually achieved repair, it is usually difficult to re-insert a transure-
in distal hypospadias repair by mobilizing the thral catheter, and a suprapubic cystostomy is
dorsal preputial fascia and in proximal hypospa- usually needed.
dias by mobilizing the tunica vaginalis fascia In severe cases (Fig. 63.1), when the penis is
from the scrotum. This means that the protective very swollen, it may be necessary to incise the
intermediate layer serves two purposes: most whole new urethra until healthy tissues are
importantly, it brings healthy well-vascularized encountered and wait for 6 months before
tissues ventrally, and it acts as a sealing intermedi- attempting hypospadias repair.
ate layer. Failure to recognize this fact may result Some surgeons may dilate the narrow urethra
in a high incidence of complications. and leave a transurethral catheter for a month
(Fig. 63.2). However, a narrow urethra remains
narrow, and the author had to incise the narrow
Reconstruction of new urethra is like build- urethra and apply a buccal mucosal graft.
ing a house; you need to have the proper The Hypospadias Center in Frankfurt,
building materials or healthy, well- Germany, occasionally receives patients from
vascularized tissues. different countries who had to live with suprapu-
bic catheter for a month or more before referral
(Fig. 63.3).
In Chap. 6, the triad that influences the out-
come of hypospadias surgery (the patient, the
surgeon, and the technique) was discussed in
detail. The patient factors include the morphol- 63.4 Timing of Redo Surgery
ogy of hypospadias, the degree of hypoplasia,
and the degree of differentiation of the ventral The mechanism and duration of healing are dis-
penile structures and skin. Most of us concentrate cussed in Chap. 16. Wound healing is usually
on the technique and may blame the surgeon but complete after 6 months. It is very important that
pay little attention to the patient’s factors. the surgeon should wait at least 6 months or even
It is clear that we cannot alter the patient’s up to a year until tissues are soft and pliable to
tissues, but the surgeon should augment the ensure a high chance of success.
63 Management of Failed Hypospadias Surgery 837
Fig. 63.1 A 12-month-old child presenting with urethral catheter was kept for 2 days. Surgical correction using
obstruction 3 weeks after TIP repair. The child had a trial redo SLAM was performed 6 months later (©Ahmed
of catheterization for a week that did not help. Incision of T. Hadidi 2022. All Rights Reserved)
the whole neourethra was performed, and a transurethral
63.5 Role of Pre-operative 63.7 Management of Persistent/

Hormone Therapy Recurrent Chordee
The role of pre-operative hormone therapy is dis- The extensive use of dorsal plication in the 1990s
cussed in Chap. 13 and in Chap. 56. The author and 2000s has resulted in high incidence of per-
uses pre-operative hormone therapy in failed sistent/recurrent chordee at the age of puberty [8,
hypospadias only if the penis and glans are very 9]. Management of chordee follows the same
small (glans width less than 12 mm) in young guidelines as mentioned in Chaps. 8 and 64.
children (less than 2 years of age). It is our expe-
rience that pre-operative hormone therapy
increases the size of the penis and glans and does 63.8 Different Techniques Used
not increase the incidence of complications. for Failed Hypospadias
63.8.1 T
he Salvage Mathieu
63.6 Role of Nitroglycerine and SLAM Technique
and Hyperbaric Oxygen for Failed Distal Hypospadias
Therapy
In distal urethral breakdowns or in fistulas located
Several studies showed positive effects of the use at the coronal level, a Mathieu urethroplasty is a
of nitroglycerine ointment (vasodilator) and safe procedure if the penile skin proximal to the
hyperbaric oxygen to reduce tissue ischemia and ectopic meatus is healthy [10–12]. Distal stric-
improve healing [2–7]. This topic is covered fur- tures are rare (1%), and the fistula rate is low,
ther by Hanna in Chap. 64. around 5%. Minevich et al. (1999) reported a
838 A. T. Hadidi
Fig. 63.2 A 14-month-old who flew from another coun- incised, and it felt like cutting through cartilage. All the
try with severe dysuria following TIP procedure and came unhealthy hard tissues were removed, and a buccal muco-
with a transurethral catheter for more than a month. As sal graft was applied (©Ahmed T. Hadidi 2022. All Rights
soon as the catheter was removed, the child was unable to Reserved)
pass urine after 2 or 3 days. The whole new urethra was
Fig. 63.3 A failed TIP technique presenting with almost incised till healthy urethra was reached (©Ahmed
absent urethra. The child was flown from another country T. Hadidi 2022. All Rights Reserved)
with suprapubic catheter. The narrow atretic urethra was
840 A. T. Hadidi
a b c
d e f
1.5% reoperation rate with the Mathieu proce- proximal hypospadias with adequate healthy skin
dure in 202 cases with no stricture over a period (see Chap. 30) (Figs. 63.6 and 63.7).
of 5 years [13]. The half-moon-shaped meatus is
avoided by using the SLAM modification [14]
(Chap. 22). In the Hypospadias Center, Frankfurt, 63.8.4 T
he LABO Technique for Failed
Germany, SLAM is the commonest operation Proximal Hypospadias After
used to correct failed distal hypospadias. The Failed Buccal Mucosal Graft
unhealthy scar tissue is excised, and a new ure-
thra is reconstructed using a perimeatal flap. This The LABO technique can also be used after
is usually possible in most cases of failed distal failed buccal mucosal grafting of proximal hypo-
hypospadias even if the child is circumcised spadias (Fig. 63.8).
(Fig. 63.4). It was frequently reported that the Other techniques that were popular before
Mathieu technique or its modifications including include the onlay island flap (Chap. 31) and
SLAM are not suitable for redo distal hypospa- Duckett/Asopa preputial island flaps (Chap. 34).
dias. This is simply incorrect. In the 92 cases of Mollard’s series with onlay
island flaps (Mollard and Castagnola 1994), 15
presented with complications. Elbakry (1999)
63.8.2 Urethral Mobilization reported an overall complication rate of 42% in
and the DYG Technique 74 patients: 5 (7%) had a urethral breakdown, 17
for Failed Coronal (23%) a urethrocutaneous fistula, 7 (9%) ure-
Hypospadias thral strictures, and 3 (4%) urethral diverticula
[18]. The complication rate of Duckett/Asopa
In the case of failed coronal hypospadias and in technique varies between 3.7% (El-Kasaby
the presence of healthy tissue, it is possible to et al., 1986 [19]) and 69% (Parsons and
correct the condition using the “double Y glanu- Abercombie, 1984 [20]). Duckett himself
lomeatoplasty” (DYG) technique (see Chap. 19) reported a complication rate varying between 9
(Fig. 63.5) [15]. Fistula rate is very low as there and 15% [21].
is no urethral reconstruction. The technique is
ideal when the glans is small. The DYG tech-
nique avoids circular anastomosis, thus reducing
the incidence of meatal stenosis reported with the 63.8.5 T
he Use of Two-Stage BILAB
Koff mobilization technique [16]. for Failed Proximal
Hypospadias
63.8.3 T
he LABO Technique for Failed Sometimes, it is still possible to use the local
Proximal Hypospadias penile skin from both sides using the “bilateral-
based flap” (BILAB) technique [22] to recon-
The “lateral-based onlay” flap (LABO) technique struct the new urethra after excising all the
[17] is an excellent technique to correct failed unhealthy scar tissues (see Chap. 32) (Fig. 63.9).
Fig. 63.4 A 2-year-old child after failed TIP procedure. [14], and a catheter size F14 was inserted into the wide
(a) Unfortunately, the surgeon performed circumcision proximal urethra. (e) Urethroplasty was performed using
during the TIP repair. Observe the huge discrepancy the SLAM technique around catheter F14. (f) The final
between the width of the neourethra after TIP incision and appearance at the end of operation. One can see a strong
the original proximal urethra (double the neourethra). (b) wide stream of urine from the tip of the penis and intra-
The width of the neourethra is less than 5 mm. (c) Incision urethral catheter was inserted for 2 days (©Ahmed
of the urethral meatus was made until healthy wide origi- T. Hadidi 2022. All Rights Reserved)
nal urethra (10 mm). (d) The design of the SLAM flap
842 A. T. Hadidi
a b c
Fig. 63.5 A 10-month-old child with failed TIP. The glanular wings freely and create adequate space for the
DYG technique was used to correct the failed hypospadias new urethra. Fine stitches are essential to fix the urethra at
[15]. (a) The glans is small and the tissues looked healthy. the tip of the glans to avoid meatal retraction. (c) The final
(b) An inverted Y was made, and the urethra was mobi- appearance at the end of operation (©Ahmed T. Hadidi
lized to the tip of the glans. It is important to mobilize the 2022. All Rights Reserved)
Fig. 63.6 Failed

proximal hypospadias
with complete wound
dehiscence. The
“lateral-based onlay”
flap (LABO) technique
[17] was used to correct
the failed hypospadias
repair. The lateral
healthy skin on the right
side of the patient was
mobilized and sutured to
the right edge of the
narrow urethral plate. A
wide, healthy, well-
vascularized neourethra
was reconstructed
(©Ahmed T. Hadidi
2022. All Rights
Reserved)
844 A. T. Hadidi
Fig. 63.7 Failed proximal TIP with stenosis and cir- the narrow urethral plate, and a new wide urethra was
cumcision. The narrow neourethra was incised. The lat- reconstructed (©Ahmed T. Hadidi 2022. All Rights
eral skin on the right side was mobilized and sutured to Reserved)
Fig. 63.8 The LABO technique can also be used in failed edge of the remaining urethral plate. It is essential to cover
proximal hypospadias even after the unsuccessful use of the neourethra with a well-vascularized protective layer
buccal mucosal grafts. All the unhealthy scar tissue was using the tunica vaginalis from the scrotum. (©Ahmed
excised, and the available healthy skin is sutured to the T. Hadidi 2022. All Rights Reserved)
846 A. T. Hadidi
Fig. 63.9 A child with failed proximal TIP and narrow months later, the second-stage BILAB (Thiersch princi-
new urethra. The narrow new urethra was incised and all ple) was used to reconstruct the new urethra (©Ahmed
the unhealthy tissues were excised and the lateral penile T. Hadidi 2022. All Rights Reserved)
skin was closed as in first-stage BILAB (Chap. 32). Six
Fig. 63.10 Failed perineal hypospadias. After excision was used to open the glans wide and cover the distal shaft
of all the unhealthy scar tissue, the urethral opening of the penis (©Ahmed T. Hadidi 2022. All Rights
became perineal. The skin from both sides was approxi- Reserved)
mated proximally in the midline. A buccal mucosal graft
848 A. T. Hadidi
63.8.6 Management of Hypospadias 63.8.7 H

ow to Deal with Lack of Skin
with Inadequate Healthy Cover?
Tissues
The first step in the management of complicated
In the late 1980s, Bracka (1995) resuscitated the or failed hypospadias surgery is to get rid of all the
Cloutier (1962) two-stage procedure [23, 24]. In unhealthy scar tissues and to ensure that the penis
the first stage, the penis is straightened, the glans is straight. This usually results in lack of healthy
opened widely, and the ventral radius of the penis tissues to reconstruct the new urethra and to pro-
is grafted with a free graft of preputial mucosa. A vide skin cover of the penis. The lack of healthy
few months later, the graft is tubularized. The tissue to reconstruct the urethra is usually compen-
cosmetic results of this two-stage approach were sated for using buccal or labial mucosa. The lack of
very good, although the urethral stricture rate skin to cover the penis may be compensated for by
was high (7%). using the little skin available after circumcision by
In the Hypospadias Center in Frankfurt, we performing multiple release incisions (Fig. 63.12),
routinely use two-stage buccal mucosal grafts for scrotal flap (Fig. 63.13), or full-thickness skin
patients with inadequate healthy, well-graft from the inguinal region (Fig. 63.14) or from
vascularized tissue (Figs. 63.10 and 63.11). the groin (Fig. 63.15), or occasionally the excess
skin in bifid scrotum may be used as a pedicle flap
(Fig. 63.16). Other options include the use of tis-
sue expanders (Chap. 64).
Fig. 63.11 Failed proximal hypospadias. After excision tration made to avoid hematoma under the graft. Observe
of all the unhealthy tissues and performance of the “tunica how the graft was pink and soft after complete healing.
albuginea longitudinal excision” (TALE) procedure, the New urethra was reconstructed 6 months later (©Ahmed
penis was straight. A buccal mucosal graft was used to T. Hadidi 2022. All Rights Reserved)
cover the ventral penile defect. Notice the extensive fenes-
850 A. T. Hadidi
Fig. 63.12 A patient with failed TIP. The neourethra was dure, there was deficiency of skin to cover the penis. This
reconstructed using the SLAM technique. Because the was compensated for by performing multiple release inci-
surgeon performed circumcision during the TIP proce- sions. (©Ahmed T. Hadidi 2022. All Rights Reserved)
Fig. 63.13 Another patient with failed TIP and lack of skin cover. The SLAM technique was performed, and the lack
of skin was compensated for by using a scrotal flap (©Ahmed T. Hadidi 2022. All Rights Reserved)
Fig. 63.14 A full-thickness skin graft from the groin was used to cover a defect in the ventral penile skin after five
unsuccessful hypospadias operations (©Ahmed T. Hadidi 2022. All Rights Reserved)
852 A. T. Hadidi
a b
c d
Fig. 63.15 (a–d) A child with buried penis who undertaken from the groin to cover the skin defect. (c) After
went improper circumcision. (a) Only the glans can be covering the skin defect and before closing the donor area
seen, but the rest of the penis is hidden in the abdomen. at the groin. (d) After complete healing of the wound
(b) After mobilization, the penis looks normal in size, but (Courtesy of Ibrahim Ulman)
there is lack of skin to cover it. A large skin graft was
Fig. 63.16 The excess scrotal skin available in patients with bifid scrotum can be used as a pedicle flap to cover skin
deficiency at the penoscrotal junction (©Ahmed T. Hadidi 2022. All Rights Reserved)
9. Huang LQ, Ge Z, Ma G. Commentary: recurrent

References chordee in 59 adolescents and young adults following
childhood hypospadias repair. J Pediatr Urol. 2020;
1. Smith ED. Foreword to hypospadias surgery. In: https://doi.org/10.1016/j.jpurol.2020.06.005.
Hadidi AT, Azmy A, editors. Hypospadias surgery, an 10. Mathieu P. Traitement en un temps de l’hypospade
illustrated guide. 1st ed. Berlin-Heidelberg: Springer; balanique et juxta-balanique. J Chir. 1932;39:481.
2004. p. Foreword. 11. Wheeler RA, Malone PS, Griffiths DM, Burge
2. Scheuer S, Hanna MK. Effect of nitroglycerin oint- DM. The Mathieu operation. is a urethral stent man-
ment on penile skin flap survival in hypospadias datory? Br J Urol. 1993; https://doi.org/10.1111/
repair Experimental and clinical studies. Urology. j.1464-410X.1993.tb16007.x.
1986;27:438–40. 12. Retik AB, Mandell J, Bauer SB, Atala A. Meatal
3. Gdalevitch P, Van Laeken N, Bahng S, Ho A, Bovill based hypospadias repair with the use of a dor-
E, Lennox P, Brasher P, Macadam S. Effects of nitro- sal subcutaneous flap to prevent urethrocutane-
glycerin ointment on mastectomy flap necrosis in ous fistula. J Urol. 1994; https://doi.org/10.1016/
immediate breast reconstruction. Plast Reconstr Surg. S0022-5347(17)32555-7.
2015;135:1530–9. 13. Minevich E, Pecha BR, Wacksman J, Sheldon
4. Vania R, Pranata R, Irwansyah D, Budiman. Topical CA. Mathieu hypospadias repair: experience in
nitroglycerin is associated with a reduced mastec- 202 patients. J Urol. 1999; https://doi.org/10.1016/
tomy skin flap necrosis-systematic review and meta- S0022-5347(05)68143-8.
analysis. J Plast Reconstr Aesthetic Surg. 2020; 14. Hadidi AT. The slit-like adjusted Mathieu technique
https://doi.org/10.1016/j.bjps.2020.01.009. for distal hypospadias. J Pediatr Surg. 2012; https://
5. Kindwall EP, Gottlieb LJ, Larson DL. Hyperbaric doi.org/10.1016/j.jpedsurg.2011.12.030.
oxygen therapy in plastic surgery: a review 15. Hadidi AT. Double Y glanuloplasty for glanu-
article. Plast Reconstr Surg. 1991; https://doi. lar hypospadias. J Pediatr Surg. 2010; https://doi.
org/10.1097/00006534-199111000-00029. org/10.1016/j.jpedsurg.2009.11.019.
6. Chang C, White C, Katz A, Hanna MK. Management 16. Koff SA. Mobilization of the urethra in the surgical
of ischemic tissues and skin flaps in re-operative and treatment of hypospadias. J Urol. 1981; https://doi.
complex hypospadias repair using vasodilators and org/10.1016/S0022-5347(17)55048-X.
hyperbaric oxygen. J Pediatr Urol. 2020; https://doi. 17. Hadidi AT. Proximal hypospadias with small flat
org/10.1016/j.jpurol.2020.07.034. glans: the lateral-based onlay flap technique. J
7. Kivisaari J, Niinikoski J. Effects of hyperbaric oxy- Pediatr Surg. 2012; https://doi.org/10.1016/j.
genation and prolonged hypoxia on the healing of jpedsurg.2012.06.027.
open wounds. Acta Chir Scand. 1975;11. 18. Elbakry A. Complications of the preputial island
8. Abosena W, Talab SS, Hanna MK. Recurrent chor- flap-tube urethroplasty. BJU Int. 1999; https://doi.
dee in 59 adolescents and young adults following org/10.1046/j.1464-410x.1999.00097.x.
childhood hypospadias repair. J Pediatr Urol. 2020; 19. El-Kasaby AW, El-Beialy H, El-Halaby R, Nowier
https://doi.org/10.1016/j.jpurol.2019.11.013. A, Maged A. Urethroplasty using transverse penile
854 A. T. Hadidi
island flap for hypospadias. J Urol. 1986; https://doi. 22. Hadidi AT. Perineal hypospadias; The bilateral based
org/10.1016/S0022-5347(17)45002-6. (BILAB) skin flap technique. J Pediatr Surg. 2014;
20. Parsons KF, Abercrombie GF. Transverse pre- https://doi.org/10.1016/j.jpedsurg.2013.09.067.
putial island flap neo-urethroplasty. Br J Urol. 23. Bracka A. Hypospadias repair: the two-stage alterna-
1982; https://doi.org/10.1111/j.1464-410X.1982. tive. Br J Urol. 1995; https://doi.org/10.1111/j.1464-
tb13639.x. 410X.1995.tb07815.x.
21. Duckett JW. Hypospadias. In: Walsh PC, Gittes RF, 24. Cloutier AM. A method for hypospadias
Perlmutter AD, editors. Campbell’s urology. 5th ed. repair. Plast Reconstr Surg. 1962; https://doi.
Philadelphia: Saunders; 1986. p. 1969–99. org/10.1097/00006534-196209000-00007.
Complex and Redo Hypospadias
Repairs: Management of 402 64
Patients
Moneer K. Hanna
Abbreviations In 1970, Horton and Devine [1] coined the

term “hypospadias cripples” to describe these
AE Artificial erection patients; however, this term will be avoided in
BXO Balanitis xerotica obliterans this report since then, significant progress has
GTIP Grafted tubularized incised plate been made in repairing these high morbidity
HAD Human acellular dermis cases. The author’s strategy for the management
HBO Hyperbaric oxygen of these difficult cases has evolved over time, and
HBOT Hyperbaric oxygen therapy the lessons learned from repairing these chal-
NTG Nitroglycerine ointment lenging cases constitute the basis of this chapter.
SIS Small intestinal submucosa There are several surgical techniques for the
STSG Split-thickness skin grafts primary repair of distal and proximal hypospa-
T-D Thiersch-Duplay dias; the latter can be performed in one or two
TIP Tubularized incised plate stages. Regardless of which technique the sur-
geon chooses, there are three principles to the
correction of hypospadias, namely, correction of
the chordee, construction of the urethra, and
64.1 Introduction resurfacing of the urethroplasty by skin. Surgical
complications vary, and some are due to error in
Repeated surgeries for hypospadias leave the the design, and some are due to failure of the
patient with a penis that is scarred, hypovascular, execution of the operation and/or postoperative
and shortened. The multiple operative procedures care. While most complications are found during
result in varying degrees of scarring, and the tis- the first year after the repair, late complications
sue ischemia may result in poor healing. do occur since some well-executed urethroplas-
Frequently, in these cases, the outcome is at best ties deteriorate as children transition to adoles-
more scarring and at worst flap necrosis and cence [2]. Recently, Long et al. indicated that the
breakdown of the repair (Fig. 64.1). published complication rates for proximal hypo-
spadias are much higher than previously reported
[3]. Furthermore, the risk of complications
increased to 40% with three or more redo hypo-
M. K. Hanna (*) spadias repairs [4].
Institute of Pediatric Urology, New York Weill This author has maintained a database since
Cornell College, New York Hospital-Cornell
University, New York, NY, USA 1980, and the approach for creating a neourethra
e-mail: mhanna@mkhanna.com in redo and complex hypospadias repair has
856 M. K. Hanna
a b c
Fig. 64.1 (a) Post 11 operative procedures. (b) 8 prior operations. (c) Urethral stricture and curvature post 5 repairs
evolved over the past four decades. The total skin which is often lacking in these cases. The
experience between January 1980 and January harvested skin graft was made approximately
2020 includes 6039 surgeries, of whom 1142 50% larger than the measured urethral defect
were revision and/or secondary repairs (urethral because of the tendency for free grafts to con-
fistula, persistent or recurrent chordee, urethral tract, a phenomenon known as “primary
stricture, diverticulum, dehiscence, etc.). contracture.”
402/1142 were complex repairs who had had My experience in 22 patients [5] who have
3–23 prior operations, of whom 126 patients undergone full-thickness free skin graft ure-
were previously reported in 2005 [5]. Early in the throplasty developed complications in 7/22
series, the one-stage repair popularized by Horton patients (32%) (Table 64.1). In this series,
and Devine [6] was adopted; however, it became strictures developed in 4/22 (18%) patients,
clear that many patients are better served by and subsequently, balanitis xerotica obliterans
staged operative repair. (BXO) developed in 1 patient. In general, a
free skin graft will cease to contract when it
meets an opposing force equal to its intrinsic
64.2 Urethroplasty force of contraction. Some authors reported
similar high complication rates [8], and
The approach for creating a neourethra in these Webster et al. [9] noted the suboptimal out-
patients has evolved over time. Early in the first come in their series of 22 urethroplasties using
series, construction of a urethra by tubularization extragenital skin. The unfavorable experience
of a full-thickness skin graft which was popular- with use of tubed full- thickness skin grafts
ized by Horton and Devine [6] and Hendren et al. prompted a search for another tissue for ure-
[7] was advocated. When penile skin was not thral substitution.
available, hairless skin from the iliac region or The bladder mucosa, which was first reported
groin was harvested to create a neourethra. by Memmelaar [10], was appealing. It is hairless;
However, the extragenital skin donor has the dis- unlike the skin, there is no encrustation or stone
advantage of greater thickness than the preputial formation [11]. Furthermore, the bladder urothe-
Table 64.1 Shows the morbidity of different methods of urethroplasty
Type of repair No. Major complications No. Minor complications No. Total
One-stage free skin graft urethroplasty 22 Partial breakdown 1 Small fistula 1 7 (32%)
Stricture 2 Skin tethering 1
Stricture and fistula 1
Stricture and BXO 1
One-stage bladder mucosa graft 27 Stricture and prolapse 2 Inclusion cyst 1 10 (37%)
Fistula and prolapse 1 Skin tethering 3
Mucosal prolapse 3
One-stage Thiersch-Duplay repair 28 Distal breakdown 1 Small fistula 1 5 (18%)
Large fistula 1 Skin tethering and chordee 1
Stricture and BXO 1
One-stage buccal mucosa urethroplasty 27 Stricture 1 (tube) Small fistula 1 (onlay) 4 (15%)
Hairy urethra 1 (onlay)
Breakdown 1 (tube)
Two-stage skin flap 28 Distal breakdown 1 Small fistula 2 7 (25%)
64 Complex and Redo Hypospadias Repairs: Management of 402 Patients
Diverticulum and fistula 1 Skin tethering and chordee 1

Hairy urethra 1
Stricture 1
Two-stage buccal mucosa graft 20 Distal breakdown 1 Meatal stricture 1 3 (15%)
Stricture 1
857
858 M. K. Hanna
lium has a high regeneration rate and appears to phic (Fig. 64.3). Furthermore, the pliability of
be suited for the formation of a urinary conduit bladder mucosa resulted in ballooning during
(Fig. 64.2). voiding and frequently post-voiding dripping of
Bladder mucosal grafts were utilized for sec- urine. The high rates of meatal prolapse, the need
ondary urethroplasty in 27 patients who were for a suprapubic incision, and the tendency of the
referred for complex hypospadias repair follow- bladder mucosa to contract resulting in stenosis
ing multiple failed repairs. As described by have led this author to abandon the use of bladder
others, bladder mucosal prolapse per urethra was mucosa.
the most common postoperative complication With bladder mucosa not providing the ideal
[12, 13]. This complication occurred in 6/27 solution for urethral substitution in complex
(22%) of my patients (Table 64.1). The bladder hypospadias repairs coupled with the paucity of
mucosa tended to proliferate, and when exposed local skin, other extragenital sites needed to be
to the air, it became sticky, friable, and hypertro- explored.
Fig. 64.2 Harvesting bladder mucosa graft for a 30-year-old who underwent four prior surgeries
Fig. 64.3 Common complication of tubed bladder mucosa meatal proliferation and proximal anastomotic stricture
64 Complex and Redo Hypospadias Repairs: Management of 402 Patients 859
The mucosa from the oral cavity is one such inner lower or upper labial mucosa and the inner
material. The advantages of this tissue are that it is cheek mucosa are the usual harvest sites. Knowledge
hairless, accustomed to a wet environment, easy of the oral anatomy and specifically where Stensen’s
to harvest, and resilient to infection. Histologically, duct, or parotid duct, is located is essential to avoid
buccal mucosa has a large amount of elastin, a injuring it during harvesting the mucosa from the
relatively thick mucosa with highly vascular and inner cheek. It is typically located opposite the third
thin lamina propria. Studies calculating the actual upper molar. Placement of an oral retractor helps to
number of blood vessels in the dermis of skin and retract the tongue, and the endotracheal tube should
buccal mucosa show that buccal mucosa has a be on the side opposite to the harvest site while the
two- to fourfold greater density of capillaries. mucosa is stretched. Care should be taken not to dis-
These properties may explain the lesser degree of turb the underlying muscle and neurovascular struc-
shrinkage of the graft observed following harvest- tures. Studies evaluating morbidity at harvest site
ing compared to other extragenital sites such as have demonstrated increased postoperative pain and
bladder and skin. It has been clinically recognized persistent numbness in the lower lip harvest site
that skin and buccal mucosa have stiffer mechani- compared to the inner cheek [18, 19].
cal properties than bladder mucosa. This may in The decision to perform the reconstruction
some way prevent the meatal protrusion seen with using the oral mucosa as a single-stage versus
bladder mucosal grafts. two-stage procedure depends upon the quality of
The first report of using buccal mucosa for the urethral plate and the degree of penile curva-
urethroplasty was by Humby in 1941 [14]. ture. When the urethral plate has been excised or
However, no further details of this technique visibly and palpably scarred, two-stage urethro-
were reported in the literature for some time. The plasty was utilized as shown in Fig. 64.4.
relative simplicity of harvesting and concealing When a small graft is needed as an onlay or
the incision, coupled with the disappointing inlay patch (Figs. 64.5 and 64.6), the harvest site
results of bladder mucosal grafts, resurrected the was the lower or upper lip which are more suited
interest in this technique some 40 years later. In for the lining of the glans penis due to the thinness
1986, Duckett [15] described the use of buccal of these grafts. The cheek was used for larger
mucosal graft to replace the mid-urethra at the grafts (Fig. 64.7). When a staged approach was
time of penile lengthening in an exstrophy- indicated, the harvested graft was secured to the
epispadias patient. Other reports followed with ventral side of the penis with absorbable sutures
reasonable success rates [16, 17]. and immobilized with a tie-over dressing. The sec-
Harvesting a buccal mucosal graft is a simple ond stage was performed no sooner than 6 months
procedure with a few important considerations. The and usually 1 year following the first stage.
Fig. 64.4 Recurrent curvature due to short and scarred urethra—excised and oral graft staged repair (18/119 pts.)
860 M. K. Hanna
Fig. 64.5 Mucosal graft onlay for urethral stricture who failed three attempted repairs. The graft must be covered by a
dartos flap (not shown)
Fig. 64.6 Inlay buccal mucosa graft for recurrent urethral stricture
Fig. 64.7 Augmentation of long urethral stricture with residual relatively healthy urethral plate by an oral mucosal graft
In 27 patients selected for one-stage repair, the group of patients who underwent first-stage
buccal mucosa was used as an onlay graft in 22 repair was by a Foley catheter for 7–10 days. For
patients, and it was tubularized in 5 patients. In the second stage of the repair, a urethral stent that
the 20 patients in whom the two-stage approach did not traverse the external sphincter to support
was utilized, the previously placed graft was and drain the urethroplasty, combined with a
tubularized and waterproofed by either a tunica suprapubic catheter for urinary diversion, was
vaginalis or dartos flap. There was no difference utilized for 10–14 days. Postoperatively, all
in the complication rate between the patients patients were kept on bed rest for 3–4 days, and
undergoing a one-stage or two-stage mucosal the post-pubertal patients received ketoconazole
grafts (15%) in each group (Table 64.1). The sin- to avoid/reduce penile erections for 1 week.
gle donor site complication in this series was a Preoperative testosterone was used early in the
mucosa cyst which was subsequently excised. series to improve the vascularity of the recipient
Long-term observation of these patients has dem- site. The patients were seen at follow-up 2 weeks,
onstrated the durability of oral mucosa. 4–6 weeks, 6 months, and 1 year postoperatively.
Adequate diversion of urine following surgi- Subsequently, the patients were encouraged to
cal repair of complex hypospadias is important report periodically, and the majority did. At the
for the healing process. Whether this is accom- office visit, the meatus was calibrated, and the
plished with urethral stents, urethral catheters, or patients were observed voiding, or a uroflow was
suprapubic catheters has been debatable, and performed.
every surgeon has his or her preference.
Nevertheless, the rationale for urinary diversion
is based upon the avoidance of urine extravasa- 64.3 esults of the First 152
R
tion into the subcutaneous tissues and accompa- Patients
nying inflammatory reaction which would be
counterproductive to the epithelialization of the Major complications included partial breakdowns
neourethra and may lead to fibrosis. of the repair, strictures, diverticulum formation,
Intraoperative cephalosporin I.V. injection is and multiple fistulae. Minor complications
given and continued postoperatively until all included a single small fistula, skin tethering,
catheters are removed. Urinary diversion in the inclusion cysts, and glans irregularity.
862 M. K. Hanna
Table 64.1 shows the morbidity of each group. ture, and normal voiding observed by either the
It became clear that the use of a free skin graft patient, the parents, or the physician. The cos-
and/or bladder mucosa for urethral substitution metic results varied from excellent to acceptable,
resulted in a high complication rate of 32% and depending on the complexity of the case and the
37%, respectively, whereas the use of buccal severity of the deformity at the outset.
mucosa resulted in a 15% complication rate.
Further revisions to repair these complications are
summarized in Table 64.2. Some patients declined 64.4 econd Series of 250
S
further surgery and considered the skin tethering Patients
and/or coronal meatus as functionally acceptable.
Those who underwent further surgery achieved Frequently, varying degrees of distress in older
satisfactory outcomes, which were defined as patients and the parents of younger children who
glanular meatus, absent or minimal penile curva- have undergone multiple failed hypospadias
repairs were noted. Having had numerous proce-
Table 64.2 Procedures to correct complications after dures sometimes spanning several years, some of
urethroplasty the patient’s belief in a remedy was exhausted. To
Total no. of No. of re-establish hope, psychological counseling for
Procedure complications (%) revisions (%) these patients can be helpful; in addition, we also
Group I: One stage encourage the patients and/or their parents to
Free skin graft 7 (32) 4 (18)
(22)
speak with other patients and families who have
Bladder mucosa 10 (37) 8 (30) already gone through a definitive procedure. This
graft (27) support group approach has led to patients having
Buccal mucosa 4 (15) 4 (15) appropriate expectations and allows them to cul-
graft (27) tivate an optimistic viewpoint.
Thiersch- 5 (18) 5 (18) In a small group of patients (26/224), the ure-
Duplay (28)
thral plate was remarkably healthy despite mul-
Group II: Two stage
Skin (28) 7 (25) 5 (18) tiple surgeries and was deemed suitable for
Buccal mucosa 3 (15) 2 (10) tubularization first reported by Thiersch [20] and
(20) Duplay [21] (Fig. 64.8).
Fig. 64.8 Glans dehiscence and two urethral fistulas. Thiersch-Duplay repair, closure of fistulas, and waterproofing of
the suture line
64.5 Persistent and Recurrent correction of the chordee. The second group were
Chordee children who underwent two-stage repair, including
ventral lengthening procedures, by dermal or tunica
Residual curvature which is noted during the early vaginalis free grafts. They reported 22% recurrence
postoperative period following proximal hypospa- rate of the ventral curvature in children treated by
dias repair is usually due to either failure to perform one-stage repair (dorsal plication) and no recurrent
an artificial erection (AE) test at the completion of curvature in the second group who underwent
the repair or inadequate correction. However, late staged repair with excision of the urethral plate and
recurrence of the curvature in adolescents following corporal grafting. It can be concluded that a case
AE-proven complete correction of the chordee has can be made for staged repair of proximal hypospa-
been reported (22) and may be caused by periure- dias which is frequently associated with chordee.
thral and skin fibrosis and/or disproportionate Recent review of 119 adolescents from my data-
growth of a relatively hypoplastic ventral corporal base of whom nearly 50% had an AE proved the
wall or the reconstructed urethra (Fig. 64.9). correction of the chordee at the primary repair per-
The role of an intraoperative artificial erection to formed by this author. The rest of the patients were
assess the adequacy of the chordee release has been self-referred. 95/119 had 3 or more surgical proce-
repeatedly emphasized. Gershbaum et al. [22] dures; 24 patients underwent 2 surgeries for hypo-
reported 5–10-year follow-up of two groups of spadias repair. All patients complained of either
proximal hypospadias repaired by this author. The sexual dysfunction or deformity. The patients noted
first group included those who underwent one-stage that the curvature was associated with growth of
repair and dorsal penile shortening procedures for the phallus, and examples are shown in Fig. 64.10.
a b c d
Fig. 64.9 (a–d) Recurrent curvature partly scarring and partly corporal disproportion: Redo dorsal plication (a)
J.G. 1985 first-stage repair AE test. (b) J.G. 2001
a b c d e
Fig. 64.10 (a, b) Initial repair: TIP and dorsal plication. (c) Initial repair: T-D and dorsal plication. (d, e) Initial repair:
island flap onlay and dorsal plication
864 M. K. Hanna
In 36/119 patients, the curvature was due to closed it transversely (Fig. 64.12). Adolescent
skin tethering and subcutaneous scar tissue, patients with mild recurrent curvature less than
which was corrected by skin degloving and radi- 30° were corrected by dorsal replication of the
cal excision of the scar tissue, followed by induc- tunica albuginea. When the penile curvature was
tion of an AE test and Z-plasty of the skin severe (more than 30° plication was deemed to
(Fig. 64.11). Postoperatively, the penis was result in an unacceptable shortening of the penile
immobilized by DuoDERM® wrap and main- length), ventral corporal grafting was utilized. In
tained in the upward position by adhesive tape or cases where the urethroplasty was pliable and
by attaching a stay suture in the glans penis to the healthy, it was mobilized and separated from the
abdominal wall. tunica albuginea, which was incised at the site of
Baskin et al. [23] demonstrated that the penile maximal curvature, and the corporal defect was
nerves are absent at 12 o’clock. We have applied repaired with a dermal graft which was sutured to
this to the repair of adolescents and performed a the edges of the created defect of the tunica albu-
longitudinal incision at 12 o’clock dorsally and ginea with running long-lasting sutures
Fig. 64.11 Curvature due to skin tethering corrected by Z-plasty (36/119 pts.)
a b c d
Fig. 64.12 (a, b) Modified Baskin chordee repair. (c, d) Artificial erection test pre (c) and post (d) repair
(Fig. 64.13). The artificial erection test was site of the curvature, and a dermal graft was placed
repeated to evaluate the correction of the curvature to fill the gap in and lengthen the ventral corporal
and to ensure a water-tight closure of the suture wall (Fig. 64.14). The graft was covered by the
line. In 12/119 patients, the previously recon- intact and mobilized urethra. In cases where the
structed urethroplasty was dissected and separated short and scarred urethra is the cause of the curva-
from the tunica albuginea which was incised at the ture, it was excised and replaced by an oral muco-
Fig. 64.13 Recurrent curvature and scarred urethra (prior four operations). Dermal grafting of the corpora (arrow)
Fig. 64.14 Recurrent curvature following dorsal plication × 2. Dermal corporal graft underneath healthy
urethroplasty
866 M. K. Hanna
sal graft which was subsequently tubularized blood flow to the tissues. Subsequently, six chil-
9 months to 1 year later (Fig. 64.4). The available dren received hyperbaric oxygen treatments (see
materials for filling the corporal wall defect later). Satisfactory outcome was achieved in the
include the dermis [24], tunica vaginalis, and syn- majority of the cases.
thetic material, e.g., small intestinal submucosa
(SIS) [25]. Recently, the human acellular dermis
(HAD) [26] was utilized; however, long-term fol- 64.8 Tissues and Skin Ischemia
low-up of these materials is unavailable. My pref-
erence is to use a dermal graft which is harvested Ischemic skin flaps and tissues occur in two
from non-hair-bearing skin and cover the graft forms: pale ischemic flaps due to arterial insuffi-
with the best available skin flap (Fig. 64.15). ciency or, more commonly, blue and more rigid
flaps with rapid capillary refill due to inadequate
venous drainage (Fig. 64.18).
64.6 Recurrent Fistula Nitroglycerine ointment (NTG) is a potent
vasodilator with a large index of safety. The first
Recurrent urethra-cutaneous fistula following use and application of nitroglycerine to skin flaps
hypospadias repair was frequently a manifesta- in humans was by us in a study of penile skin in
tion of another problem [27], such as urethral children undergoing circumcision and hypospa-
stricture, urethral diverticulum, and curvature dias repairs in 1986 [29]. Quantitative fluorescein
caused by scar tissues. Preoperative examination studies were performed in a group of patients
by cystoscopy and intraoperative distention of who underwent circumcision, and a prospective
the urethra to exclude the possibility of multiple clinical trial in 40 children undergoing hypospa-
fistulae are recommended. dias repair was performed. The NTG-treated skin
flaps had increased uptake of fluorescein, and we
concluded that NTG increased the blood flow to
64.7 Recurrent Glans Dehiscence the skin flaps. Since then, I have applied NTG to
both pale and blue ischemic flaps. It is believed
The glans penis may show a deep groove (surgi- that NTG salvaged many of such flaps over the
cally favorable) or may appear flat with an absent past several decades.
sulcus (unfavorable). Glans dehiscence following Recently, a multi-institutional prospective
hypospadias repair, especially after multiple sur- randomized trial of NTG for post-mastectomy
geries, frequently results in scarred, obliterated, skin flaps was performed [30]. NTG ointment or
or absent urethral plate. Recently, White and placebo was applied to the skin flap at the end of
Hanna reported on salvaging the dehisced glans surgery underneath the dressing. The target sam-
penis of 49 children, the majority of whom had ple size in the study was 400 patients, but the trial
had multiple surgeries [28]. All children under- was stopped at the first interim analysis of the
went two-stage repair. In the first stage, the flat first 165 patients. Mastectomy flap necrosis
glans penis was incised deeply, to visualize but developed in 33.8% of patients who received pla-
spare the corpora. Thereafter, a free graft of oral cebo and in 15.3% of patients who received NTG
mucosa harvested from either the lower or upper ointment. A recent systematic review and meta-
lip, less frequently from the inner cheek or resid- analysis of 6947 patients from different
ual preputial skin, was sutured to the glans cleft. institutions demonstrated that NTG is an ideal
The graft was fenestrated and quilted in the mid- agent to increase the chance of flap survival in
line, and a tie-over dressing was applied. The mastectomy skin flaps [31].
neo-plate was tubularized 6–12 months later Hyperbaric oxygen therapy (HBOT) is an
(Figs. 64.16 and 64.17). In 11/49 patients, the established mode of treatment of ischemic tissues
skin flaps appeared dusky, and nitroglycerine and has several known cellular and biochemical
ointment 2% was applied for 24 h to enhance the benefits which promote wound healing, angio-
Fig. 64.15 A 29-year-old with prior multiple operative procedures. Persistent chordee repaired by dermal grafting of
the corporal wall and coverage by a well-vascularized dartos and skin flap
genesis, and reversal of tissue hypoxia. The most kocyte microbial killing, increased collagen
important effects from the surgeon are enhance- formation, and neovascularization of ischemic
ment of fibroblast replication, stimulation of leu- tissues [32].
868 M. K. Hanna
a b c
Fig. 64.16 (a) Staged repair of recurrent glans dehiscence. (b) Glans splitting. (c) Buccal mucosa graft
Fig. 64.17 Second-stage repair

a b
Fig. 64.18 (a) Pale flaps due to inadequate arterial blood flow. (b) Blue flaps due to venous congestion
In May 2014, we set out to reduce the high treatment protocol as described above. Patients in
complication rate in this group of patients and group II (33 pts.) received the NTG and postop-
instituted a treatment protocol for 82 patients erative local steroids but did not undergo HBOT
(2–24 years old), who had previously undergone for various reasons, namely, failure to obtain
multiple surgeries (3–9 operations) mean of 5.5 insurance approval, social issues, and residence
prior surgeries for hypospadias repair [33]. All too far from the HBOT facility. These patients
patients showed various degrees of skin and sub- served as the control group. The postoperative
cutaneous scarring which was noted either prior complication rates were 10% in group I and 33%
to or during surgery. The protocol included the in group II. The surgical repair and complications
application of NTG at the conclusion of surgery are shown in Table 64.3.
to induce vasodilation and thereby enhance tissue Wound healing is a complex process that is
perfusion. NTG was followed by ten sessions of traditionally divided into inflammatory,
HBO starting the day following the surgery. Each proliferative, and remodeling phases. All pro-

HBO dive included 90 min exposure to 100% cesses occurring in these different phases, includ-
oxygen at 2 atmospheric pressure absolute. ing leukocyte activation, angiogenesis, collagen
4–6 weeks later, local steroidal cream was applied synthesis, and re-epithelialization, are all related
three times daily for 3 months to prevent or to oxygen tension. The demand for oxygen in
reduce scarring and keloid formation. All patients wound healing is the rationale for using HBOT
were informed of the experimental and clinical for compromised tissues where oxygen cannot be
evidence of the benefits of HBOT. They were delivered by the blood. The positive effect of
asked to visit HBO facility and were prepped by HBOT on wound healing can be demonstrated
the physician in charge of the therapy. Group 1 more clearly in ischemic tissues. Kivisaari et al.
(49 pts.) included patients who completed the were able to enhance wound closure in experi-
870 M. K. Hanna
mental wounds with de-vascularized wound ischemic event and a patient from group I are
edges [34]. They compared microvascular perfu- shown in Figs. 64.19 and 64.20.
sion in normal and ischemic tissues. Despite the
reduction in the microvascular response to the
tissue injury, HBOT was able to compensate for 64.9 Penile Resurfacing
the failure of the vascular network, and these
experiments showed that wound healing was Resurfacing the penis after multiple operations
similar in the HBOT group and in those with nor- can be problematic. Early in the series, the use of
mal tissues. Examples of the contribution of the scarred skin for covering the grafted urethra con-
combination of vasodilators and HBOT in acute tributed to some cases of breakdown of the ure-
throplasty. Furthermore, multiple surgeries on
Table 64.3 Single-stage surgeries and complications (45 the penile skin may create skin deficit for the
pts.). Staged repair (4 pts.) lengthened penis after its release from scar tissue
Protocol (NTG + HBOT + No protocol (NTG + and/or correction of the penile curvature. Well-
local steroids) local steroids) planned and well-executed local flaps using vari-
49 patients 33 patients ous techniques including Z-plasty, double
Repair of recurrent glans Repair of recurrent Z-plasty, and rhomboid rotation skin flaps may
dehiscence (13) glans dehiscence (11)
be utilized. After mobilizing the skin flaps and
Re-dehiscence 2/13 Re-dehiscence 4/11
GTIP urethroplasty (7) GTIP urethroplasty degloving the penis, intraoperative skin expan-
Urethral fistula 2/7 (8) sion was achieved by excising the scarred subcu-
Glans dehiscence and taneous dartos fascia which added approximately
fistula 3/8 10–15% surface area.
Urethral mobilization (6) Urethral mobilization
The scrotal skin flap is a random flap nonethe-
No complications (9)
Distal breakdown 2/9 less usually well vascularized. Therefore, we pre-
Repair of recurrent urethral Repair of recurrent fer to rotate a healthy and more reliable scrotal
fistula (10) urethral fistula (5) skin flap despite its hair-bearing quality
Recurrent fistula 1/10 Recurrent fistula 2/5 (Fig. 64.21). In a review of 215 complex and redo
Recurrent chordee and
hypospadias repairs, Fam and Hanna reported
stricture (5) and extensive
skin scarring (4) that 85 did not have adequate healthy local penile
No complications skin to resurface the penis after urethroplasty
Staged repair (4) [35]. Scrotal skin was used to resurface the penis
No complications
in 54/85 patients, 6 patients underwent tissue
Fig. 64.19 Repaired hypospadias (prior four repairs). Six hours later, severe venous congestion treated with topical
nitroglycerine and hyperbaric oxygen
a b c
d e f
Fig. 64.20 (a) Prior multiple surgeries for distal hypospadias. (b) Urethral mobilization. (c) Urethral advancement. (d)
Completed repair. (e) Topical 2% nitroglycerine. (f) Following ten sessions of hyperbaric oxygen
expansion during a 12–16-week period to penile resurfacing. In selected cases where the dorsal
resurfacing, 23 underwent full-thickness skin penile skin was unscarred and remained rela-
grafting when the scrotal skin was unavailable tively healthy and well vascularized, tissue
due to scarring (Fig. 64.22), and another 4 cases expanders ranging from 30 to 60 mL were used
received split-thickness skin grafts (STSG). All in six patients. Successful skin and soft tissue
four patients who underwent fenestrated split- expansion was achieved in all patients [36]. This
thickness skin grafting had 100% graft take, but method provided additional local skin and tissue
secondary contraction and ulceration were noted with congruent texture and color to the native
and therefore are no longer used for penile penis (Fig. 64.23).
872 M. K. Hanna
Fig. 64.21 The use of scrotal flap to cover skin defect on the ventral surface of the penis
64.10 Conclusion construction/widening and is waterproofed with

a flap of tunica vaginalis (Fig. 64.24).
If the urethral plate is severely scarred, a two- If the penile chordee is severe (<30°) and is
stage repair is recommended. In stage 1, the com- due to corporal disproportion, ventral lengthen-
promised plate is excised and replaced with a ing of the tunica albuginea by a free dermal graft,
buccal mucosa graft. In stage 2, at least 6 months, rather than shortening the dorsal wall of the
preferably 12 months later, the graft is tubular- penis, is preferred. In these cases, the dermal
ized to construct a urethra over an appropriate graft is covered by a dartos flap when available
size catheter. However, in some cases, the ure- and a well-vascularized local skin flap.
thral plate appears to be supple and healthy, and The final lesson learned from this experience
it is in these cases that a Thiersch-Duplay is in primary hypospadias repair where a near-
tubularization is adopted [20, 21]. If the urethral perfect functional and aesthetic result represents
plate appears healthy but narrowed, a buccal a successful outcome. However, in patients who
mucosal onlay or inlay graft is placed for urethral have undergone failed surgeries, which compro-
Fig. 64.22 40% skin deficit covered with full-thickness skin graft
Fig. 64.23 Use of tissue expanders in cases of healthy residual dorsal skin and poor aesthetic outcome of resurfacing
by scrotal skin by two-stage Cecil procedure
mised the quality of the genital tissues, a some- even a coronal meatus, but such an outcome
what lower standard is acceptable. For example, would be unacceptable for a primary repair of a
a patient who has endured multiple surgical fail- “virgin” hypospadias.
ures is happy to void through a sub-terminal or
874 M. K. Hanna
Fig. 64.24 Plan of management of complex hypospadias. The first step is to correct chordee and then urethroplasty,
and the last step is to provide adequate skin cover of the penis
3. Long CJ, Chu DI, Tenney RW, Morris AR, Weiss

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Part IV
Long-Term Results
Long-Term Consequences
of Hypospadias Repair 65
Christopher Woodhouse
Abbreviations In adult practice, the majority of presentations

are with the unsuccessful reconstructions, and the
BXO Balanitis xerotica obliterans rest are with hypospadias that has never been
DR Decisional regret treated (Fig. 65.1). For those in either category, it
ED Erectile dysfunction
GPS Genital perception score
HR Hazard ratio
LUTS Lower urinary tract symptoms
UDT Undescended testes
65.1 Introduction
It is now accepted as the standard practice that

babies born with hypospadias should have recon-
structive surgery in infancy, preferably before the
age of 1 year. It would be hoped that the huge
majority have a successful outcome, are cured,
and can be discharged. Recently, there have been
suggestions that minor cases do not need an
operation.
Data on adults who were born with hypospa-
dias are few and often imprecise. Adult patients
who now are available for outcome analysis must
have been operated in the last two decades of the
twentieth century or earlier.
Fig. 65.1 Clinical photograph of an adult with previ-

C. Woodhouse (*) ously unoperated hypospadias. The meatus is almost at
University College London, London, UK the tip of the glans. The hooded prepuce and chordee are
e-mail: christopher.woodhouse@ucl.ac.uk typical features
880 C. Woodhouse
is most important to discover what they hope to Twenty had weak or incomplete ejaculation, and
achieve by surgery—unrealistic expectations are six had difficulty with intercourse [3].
seldom satisfied. Reports of adult outcomes tend to assume that
all the operations are the same. In a meta-analysis
in 2011 of all available papers on adult follow-up,
65.2 Surgical Outcomes the more proximal the hypospadias, the higher the
complication rate whatever technique is used.
65.2.1 Measures of Success There were 1069 patients (48% response rate of
the available men) and 742 controls. Not all papers
Most reports are written by surgeons about their covered all aspects of outcome. Persistent curva-
own results. They tend to be overly optimistic. ture was found in 11%, and 36% were generally
Dennis Browne described all of his first eight dissatisfied with the result. The meatus was on the
cases as “successful” because they healed by first glans in 94% and a vertical slit shape in 70% [4].
intention (Fig. 65.2) [1]. A number of objective scoring systems have
In one surgeon’s personal series of 299 men been proposed. Unfortunately, none is completely
followed for a mean of 18.4 years, he reported a satisfactory. They do not always obtain the opin-
“good” outcome with a straight erection and a ion of the patient or consider function [5].
normal formed stream in all patients [2]. However, a very important observation does
Unfortunately, closer analysis of his data shows arise from the first that was well validated called
that 65% of the men still had a degree of hypo- the Genital Perception Score (GPS). The data
spadias that would, by current standards, require show that the surgeon has a significantly better
correction. view of the outcomes than the patient. However,
Sommerlad may have been the first to point the main difference of opinion was in the size and
out in 1975 that there were many adults who had shape of the penis and glans which are not altered
had hypospadias surgery in childhood who were by surgery and the circumcised appearance. The
dissatisfied. He traced 60 of 113 men operated in opinions on the strictly surgical aspects were
one unit who were 17 years or older. As he had only marginally different [6].
not been involved with the original surgery, he
was able to give an independent opinion. The
meatus was rarely at the tip of the penis. In 13 the 65.3 Hypospadias in Adult Life
penis was not straight when flaccid (though only
10 complained of chordee). Almost none had 65.3.1 General Appearance
normal voiding, and 16 had to sit down to void.
Like other human features, there is a wide varia-
tion in that which is considered normal. Many
men have a poor opinion of the appearance of
their penis even though objectively there is noth-
ing wrong with it. It is a subjective opinion and
difficult to measure.
Patients, parents, and surgeons have a variety
of personal interests in the assessment of out-
comes. The views of lay people give interesting
insights. In a study using 105 women and 70 men
who were shown standardized photographs of
operated hypospadiac penises and normal cir-
cumcised penises (controls), distal forms of
Fig. 65.2 Clinical photograph of an adult who had had a
Denis-Browne repair in childhood. Note the patulous hypospadias were rated similarly to circumcised
meatus which is not in the glans genitals. Penises with corrected proximal hypo-
65 Long-Term Consequences of Hypospadias Repair 881
spadias were viewed less positively, but the lay gen receptors as normal. Progesterone receptors
people did not find the differences troublesome are reduced [15]. Investigations of penile sensa-
or emotionally significant. The opinions of men tion in different groups of circumcised men give
and women were the same. Their sexual orienta- conflicting results [16, 17].
tion was not reported, but as the sample was ran- Whether or not the prepuce is important for
dom, there was probably a normal spectrum [7]. satisfactory sexual function is also controversial
There is a wide range of size of the penis. Its [18]. In the electively circumcised part of the
growth with age has been documented, and a cen- AIDS prevention campaign in Africa, no signifi-
tile growth chart is available [8, 9]. Anxiety about cant sexual harm was reported in the men or their
penile dimensions is primarily due to overestima- partners [19].
tion of the normal size and its range [10]. Danish women have been reported to have
When a group of 105 women were asked to less sexual fulfillment from circumcised men.
rate eight single aspects of the penis in order of Although there were 3000 women in the study,
importance to them, position of the meatus was only 5% of the male partners were circumcised
last. No gay men were included in the scoring which makes it very unbalanced [20].
group, and they may have different opinions. The There is even less information on the conse-
order of the score was [11]: quences in gay men. A study from Australia
showed no significant sexual differences between
General appearance the circumcised and uncircumcised, especially
Skin shape when the procedure was done in infancy [21].
Glans shape
Scrotal appearance
Pubic hair appearance 65.3.3 The External Urinary Meatus
Penile length
Penile girth It has been established that the natural meatus is
Meatal position not always at the tip of the penis. In a study of
500 normal men having a cystoscopic procedure,
13% were found to have a hypospadiac meatus,
65.3.2 The Prepuce and in a further 32%, it was in the middle third of
the glans. Most of these men thought they were
In countries where childhood circumcision is normal, all voided normally, and had sexual
routine, results of childhood hypospadias repair intercourse. Only one had chordee [22].
are reported to be better than in those where it is In hypospadias, a terminal meatus is achieved
uncommon [12]. When the opinions of hypospa- in 44–100%, largely depending on the original
diacs and those circumcised for phimosis are severity and the type of surgery [23]. The best
compared, it has been shown that more of the for- results in this respect have been reported for the
mer are dissatisfied with the outcome (33% vs. Snodgrass repair. As it was first described in
5%) though not necessarily because of the cir- 1994, very few adults on whom it was used are
cumcised appearance [13]. available for follow-up [24].
Hypospadias techniques have been described
to preserve or to reconstruct the foreskin [14].
The results in adulthood will not be available at 65.3.4 Micturition
least until 2024.
The question then arises as to whether the pre- For men, voiding in public can be a competitive
puce has its own sexual function or is just a fold function. They like to be able to void standing up,
of skin useful to hypospadiologists. It has been “through their flies” and with a good stream. This
established that the adult hypospadiac prepuce is probably the most difficult thing to achieve in
has the same vascularity and androgen and estro- hypospadias repair. If a repair has been done and
882 C. Woodhouse
the stream sprays, it is even more difficult to cor- hypospadias operations. Specific predisposing
rect it. factors are advancing age, division of the urethral
There is some evidence that, at least in chil- plate, and ventral corporal grafting. Especially
dren, the patients’ descriptions are very inaccu- significant is the association of ED to the use of
rate. When the volume of sprayed urine is repetitive internal urethrotomy to manage ure-
measured by collecting it with absorbent paper thral stricture disease [30].
placed around the bowl of a flow rate machine, The more proximal the original meatus, the
there is no correlation with the patient or parents’ more scars will be present, and some will be tight
description. Nor did it have any relationship to enough to cause pain on erection. This will usu-
the shape of the meatus. This suggests that some ally require further surgical correction, even if
learn to cope with a spraying stream better than the repair is otherwise satisfactory.
others, but it certainly indicates that the descrip- As might be expected, ejaculation from a
tion by the patient is not reliable [25]. reconstructed urethra which has little or no sur-
In the meta-analysis of adult outcomes, 30% rounding erectile tissue is often slow, and this is
of all patients and 43% of those with severe the main ejaculatory complaint.
hypospadias were said to spray. Nearly all men All aspects of sexual function are reported to
who had penoscrotal hypospadias said that they be less satisfactory than in controls. The control
had a spraying stream [26, 27]. Other lower uri- groups are various and include national norma-
nary tract symptoms (LUTS) occurred twice as tive data, selected as age matched, and men oper-
often in patients than in controls, and they were ated for hernia or phimosis (Table 65.1).
more bothered by them. Nonetheless, more than 80% of men operated for
Measurement of urine flow rates gave a wide hypospadias profess themselves satisfied by their
range of results, from 8.4 to 31 mL/s. The overall sexual activity [26].
mean was 24.4 vs. 30 mL/s for control men It would appear from Table 65.1 that men with
(p < 0.01) [15]. However, in one large series from originally severe hypospadias were more satis-
1997, of men up to 66 years of age, the propor- fied than controls (100% vs. 93%). This may be
tion with a normal peak flow rate increased with because fewer of them developed stable relation-
age [28]. This suggests that the neourethra does ships and intercourse, masturbation being a sub-
grow with age or that the bladder voiding pres- stitute. This does not wholly explain the
sure increases. The patients in this study seem to observation that all of the 27 men concerned
be a reasonable cross section of the hypospadias were satisfied with their sexual function [4].
population although 87% originally had a glanu-
lar or coronal meatus.
Further evidence for neourethral growth
Table 65.1 Sexual activity in men operated for
comes from a study of 18 patients who had been hypospadias
fully assessed for voiding function as children
Hypospadias
and could be re-assessed as adults at a mean of Total Severe cases Controls
10.5 years later. All parameters had improved. Masturbation 85% 100% 93%
The mean flow rate had increased from 10.1 to Erectile dysfunction 19% 9% 1%
15 mL/s, and the spray had reduced from 4.4 to Intercourse 58% 49% 43%
3.2 g. The satisfaction score had significantly Ejaculatory 26% 24% Zero
improved [29]. problemsa
Sexual inhibition 19% N/A 9%
Stable relationship 41% 21% 53%
Sexual satisfaction 81% 100% 93%
65.3.5 Sexuality a
Mainly because of semen retained in the urethra (Data
from a meta-analysis of the literature in Rynja SP et al.:
Early onset erectile dysfunction (ED) in adults Long-term follow-up of hypospadias: functional and cos-
under 40 years is associated with multiple failed metic results. J Urol. 2009;182(4 Suppl):1736–43) [26]
65.3.6 Penile Size 65.4 Surgical Complications
The number of men operated for hypospadias 65.4.1 Late Complications

whose penile size has been recorded is small.
Mureau et al. have shown that with increasing Symptoms from late complications may appear
age from childhood to adolescence, the propor- up to 70 years after apparently satisfactory sur-
tion with a stretched length on or below the 10th gery. Those from stricture, fistula, or chordee are
centile increases, while the proportion above the usually obvious. Other urinary symptoms may
50th centile decreases (Table 65.2). The implica- also be a sign of a late complication.
tion of this finding is that the penis does not Hair in the neourethra should be a thing of the
develop as well as in normal men [12]. past but still appears from time to time. It is due
In the very small number of adults with hypo- to the use of hair-bearing skin for the repair. It
spadias for whom measurements are available, irritates the urethra causing dysuria and forms a
the mean stretched or self-measured erect length nidus for stone formation. Eventually it will form
was 12 cm (SD ± 1.3 cm) which is about 1 cm a hairball (Fig. 65.3).
below the normative 50th centile. Even with Small collections of hair can be dissolved with
proximal or perineal hypospadias the penis has urethral instillation of the standard depilatory
been self-measured when erect at 9.5–17.3 cm cream thioglycolic acid which is sterile (mar-
(mean 13 cm) which is normal [26, 31]. keted as Veet™ Reckitt Benckiser) [32]. Hairballs
Table 65.2 Histogram to show the percentage of patients with stretched penile length by age compared to normal cen-
tiles (Data from Mureau MAM, Slijper FME, Koos Slob A, Verhulst FC, Nijman RJM. Satisfaction with penile appear-
ance after hypospadias surgery: the patient and surgeon view. Journal of Urology. 1996;155:703–6, [6])
80%
70%
60%
50%
40%
30%
20%
10%
0%
9-12yo, n=16
13-15yo, n=10
16-18yo, n
<10th centile mean >90th centile
884 C. Woodhouse
Fig. 65.3 Hairball retrieved from a hypospadias recon-

struction done in the 1970s which had used some hair-
bearing skin (reproduced with permission from
Woodhouse CRJ; Adolescent Urology and Long Term
Outcomes, Chapter 15; 177–189, 2015, Wiley Blackwell,
Oxford)
need open removal with either laser depilation of

the hair follicles or replacement of the graft.
Many of the late presenting problems appear Fig. 65.4 Clinical photograph of “hypospadias cripple”
to be “simple.” Unfortunately, in most cases, the penis. The skin is fibrotic and unhealthy looking. The
whole penis is involved in the failure—truly a meatus is in the corona. This is a difficult situation to
hypospadias cripple (Fig. 65.4). Surgical correc- retrieve and will require import of fresh tissue, probably
buccal mucosa
tion is very difficult and would be at least a two-
stage procedure. Those that, to a hypospadiologist,
could be called simple such as fistulae and chor- puberty would reveal problems not previously
dee can usually be closed in a single stage. apparent.
In a prospective study in which follow-up vis-
its were scheduled at 7, 10, 13, and 16 years old,
65.4.2 Incidence and after puberty if it had not already occurred,
the number of post-puberty complications was
The commonest complications of hypospadias small. From 114 patients, there were four late
surgery are urethral and meatal strictures, resid- strictures. However, only one was found at the
ual chordee, fistulae, and misplaced meatus. post-pubertal visit. Two were present before
Most occur early after surgery and are corrected puberty, and one presented 6 years after the final
(see Chaps. 8, 44, 58, and 60). The pediatric lit- visit. Six men were found to have new curvature
erature is full of reports of incidence and repairs. after puberty but only one needed surgery. Twelve
It would be helpful if we knew how many late of 114 had esthetic concerns but only one wanted
complications developed de novo rather than an operation. There were 12 fistulae, but only two
being a continuation of a childhood problem. It were diagnosed at the final visit, and it is not
may be speculated that the penile growth at clear if they had been previously present [33].
The pickup rate for new complications after Stricturing after hypospadias repair may be
puberty that required surgery was only about 2%. complicated by lichen sclerosus (balanitis xerot-
It could be argued that asking children and ica obliterans—BXO) (Fig. 65.5). The principles
adolescents to attend every 3 years to have an of surgery with BXO are well established and
intimate examination could itself be harmful. apply equally to hypospadias patients (see Chap.
This would especially be the case if they had no 61). Radical excision of the affected tissue must
memory of the operation and thought that the be meticulous. Reconstruction is usually done as
penis was normal. Prospective follow-up as a a two-stage procedure using buccal mucosa.
research exercise is justified and necessary. Penile skin is not suitable because of the near cer-
Routine follow-up of all patients seems excessive tainty of recurrence [37, 39]. Unfortunately, the
but early reconsultation for concerns should be complication rate of around 33% is much higher
encouraged. than that seen in younger patients [40]. Once
again, some men refuse the offer of surgery, a
permanent perineal urethrostomy being accept-
65.4.3 Stricture Repair able [26].
It is tempting to dilate urethral strictures as the

first option. For short, bulbar strictures in other-
wise normal men, up to 50% may be cured [34].
However, in patients who have had previous ure-
thral surgery, particularly for hypospadias, it is
unlikely to be curative and may well make the
stricture longer and denser [35]. Urethroplasty is
the best chance of cure. It is a difficult form of
surgery in any man. In those who have had hypo-
spadias surgery, it is even more difficult.
Current data suggest that proximal and long
strictures (about 50% of cases) should be
repaired in two stages. In the first, the surround-
ing fibrosis should be radically cleared, any
chordee corrected, and a buccal graft applied.
This stage may need revision in up to 20%. In
the second stage, the neourethra is closed, also
with a 20% failure rate, usually due to fistula.
Once successfully repaired, the recurrence rate
is 5–10% [35–37].
Relatively simple strictures confined to the
glans with no complicating factors may be suit-
able for a single stage buccal graft. Even this,
however, may have a 20% complication rate.
Ultimately, about 80% may be cured. The sur-
gical commitment is considerable, and not all Fig. 65.5 Hypospadiac penis with the typical white
men may wish to undertake it. Up to 40% may appearance of BXO around the external meatus and the
stop after the first of a two-stage procedure, and ventrum of the glans (reproduced with permission from
Woodhouse CRJ; Adolescent Urology and Long Term
about 20% may elect to have a permanent peri- Outcomes, Chapter 15; 177–189, 2015, Wiley Blackwell,
neal urethrostomy [35, 38]. Oxford)
886 C. Woodhouse
65.4.4 Chordee
Residual chordee can appear for the first time

during pubertal growth, between 12 and 18 years
old [33, 41]. It is usually due to dense fibrosis in
the reconstructed urethra, and so it is not surpris-
ing to find that it is commonest in penoscrotal
and proximal hypospadias [33].
In a report of redo operations on adults for
hypospadias, late development of chordee made
up 13% of 55 men [37]. There are also some men
who have grown up with chordee but resisted
having further surgery until they encounter phys-
ical or emotional difficulties with intercourse.
Late development of chordee may be at a site
well proximal to the original [41]. It is important
to define the position accurately. Sometimes a
photograph may be sufficient. Otherwise, an arti-
ficial erection is needed. This could be done with
intracorporeal prostaglandin in the clinic or by
infusion under anesthetic (Fig. 65.6). It is impor-
tant not to use a penile tourniquet as the chordee
may be proximal to the position in which it is
applied.
Fig. 65.6 Clinical photograph taken in surgery showing

65.5 New Cases in Adults an artificially induced erection. There is marked ventral
chordee due to tight scarring. The prepuce has been used
From time to time, a man will present with hypo- in the original reconstruction
spadias who has had no previous surgery
(Fig. 65.1). Symptomatic cases most often have 65.6 Nonsurgical Complications
developed BXO—lichen sclerosis. in Adults
Occasionally, a man may present with an
unconnected symptom, and the hypospadias will 65.6.1 Fertility
be a chance finding. Even an uncomplicated
hypospadiac meatus may not be large enough to In studies from Denmark and from the UK, men
accept a conventional cystoscope or resecto- with uncomplicated hypospadias had normal
scope. The distal urethra is often fragile with no semen analysis compared to standards (50–52
supporting corpus spongiosum and may easily million/mL and normal parameters) but slightly
be damaged by instrumentation (Fig. 65.7). It is increased levels of pituitary hormones. Lower
rare for an adult to present just because of the sperm counts (20–32 million/mL) were associ-
appearance of the penis, even when there is chor- ated with other genital anomalies, particularly
dee [37]. undescended testes (UDT). If allowance was
Before deciding on treatment, it is essential to made for the higher incidence of UDT, semen
establish that which the patient hopes to achieve analysis was normal by the WHO criteria of the
from surgery. With limited objectives, such as time [42, 43].
enlargement of the meatus, simple, local surgery Paternity is a more important measure of fer-
will suffice. tility. A population-based cohort study of 1.2 mil-
Men born with hypospadias are usually wor-

ried about the risk of the condition in their sons.
There is a significant familial incidence and a
small effect in babies conceived by ICSI (see
Chaps. 10 and 11).
65.7 Psychological Outcomes

and Consent to Surgery
Although the literature has its conflicts, research

since the 1980s tends to show that men who have
had a good surgical result from hypospadias
repair have no greater incidence of psychological
morbidity than controls [46]. Poor surgical out-
come may be associated with a poor view of total
body image [47] (see Chap. 67).
Men with poor surgical results have a delayed
age of sexual debut. However, many of them will
start to have intercourse before further correction
[43].
In outcome studies in adults, there are cer-
tainly dissatisfied patients, either because of
complications of surgery or because the penile
Fig. 65.7 Clinical photograph of a previously unoper- appearance is displeasing even though the sur-
ated hypospadiac penis. A probe has been inserted into the gery was technically satisfactory. What has not
urethra. Although the external meatus (upper arrow) is been addressed is whether the dissatisfied patients
almost at the tip of the penis, the urethral tissue is poor,
wished that they had never had the surgery in the
and there is no spongiosum around it, down to the bottom
of the probe (lower arrow). This tissue would be damaged first place.
by instrumentation and have to be replaced if a recon- The literature has limited data on the opinions
struction was to be done of men who have not had any surgery for their
hypospadias. In the 65 (of 500) men presenting
lion men born in Sweden between 1964 and 1998 for endoscopic bladder surgery who were found
provides convincing evidence that fertility is to have unoperated hypospadias, 59 thought that
reduced. By three criteria—being the biological they were normal. These patients all had distal
father of a child, requiring fertility treatment, or hypospadias (glanular, coronal, or subcoronal),
having a diagnosis of infertility—men with hypo- and only one had chordee [22].
spadias were less fertile than non-hypospadiacs These are similar to the results of a prospec-
or their unaffected brothers. For the whole hypo- tive study in general urology clinics in which 56
spadiac group, the hazard ratio (HR) of having men with hypospadias were identified prospec-
biological children was 0.87. For those with tively in 2 years. Nine had had incomplete or
proximal hypospadias, the HR was strikingly failed surgery in childhood. The meatus was
worse at 0.59. They also had a higher risk of glanular or subcoronal in 44, penile in 11, and
needing assisted conception or of being diag- proximal penile in 1. Eighteen of 56 (32%) were
nosed with infertility [44]. unaware that they had a congenital abnormality,
Fertility appears to be compromised by and none wished to have corrective surgery [48].
repeated operations for unsatisfactory repairs, Surgery in infancy is done without the consent
presumably because of ascending infection [45]. of the patient. The recommendation of surgery
888 C. Woodhouse
before 18 months of age is based on theoretical 3. Sommerlad BC. A long-term follow-up of hypo-
spadias patients. Br J Plast Surg. 1975; https://doi.
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tion is needed to achieve functional and esthetic in adult men who underwent hypospadias correc-
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43. Bracka A. A long-term view of hypospa- 48. Dodds PR, Batter SJ, Shield DE, Serels SR, Garafalo
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Long-Term Follow-Up
in Hypospadias Repair: 66
What Is It and Are We There Yet?
Christopher J. Long
Abbreviations The literature for hypospadias is vast—100s

of procedures and variations have been described,
EMR Electronic medical record and countless debates on various types of repair
GPS Genital perception scale and aspects surrounding the repair have been
HOSE Hypospadias objective scoring debated for decades. For instance, the complica-
evaluation tion rate associated with the use of a caudal vs.
PPPS Pediatric penile perception score penile block, when to use supplemental testoster-
PPS Penile perception score one, the benefits of antibiotic prophylaxis in
PROMs Patient-reported outcome measures reducing urinary tract infection development, and
STAG Two-stage graft repair the impact of stent duration on complication
TIP Tubularized incised plate development are just a few aspects that have been
examined, yet no consensus has emerged [1–4].
When one closely examines this body of work,
however, it becomes clear that the quality and
Patients, families, and surgeons all have the same duration of follow-up in nearly all of these stud-
goals in mind when a diagnosis of hypospadias is ies are lacking and limit our ability to make prog-
made: the achievement of proper laminar void- ress for these patients [5, 6].
ing, a straight penis of sufficient length for sexual The general consensus is that the overall com-
function, and as near normal appearance estheti- plication rate for distal hypospadias ranges from 5
cally of the penis. Of course given the assump- to 20% [7–11]. This discrepancy in complication
tion that most are diagnosed in infancy, achieving rate varies by institution, surgeon, repair type, and
these goals must be a lifelong endeavor, not a duration of follow-up, factors which are high-
short-term goal. Along the same lines, a success- lighted when these studies are analyzed together.
ful outcome is not merely one in which no fistula A meta-analysis by Pfistermuller et al. in 2015
or glans dehiscence occurs—instead it must looked at 49 studies including 4675 patients that
involve not only the physical aspect of penile underwent a tubularized incised plate (TIP) repair
function but also the mental component that [12]. They identified an 11% complication rate
meets the demands of the patient. with a median follow-up of 16.1 months for distal
hypospadias. Perhaps one of the most important
C. J. Long (*) aspects to come from this paper was the follow-up
Perelman School of Medicine, University of listed for paper: no follow-up was listed in 10/49
Pennsylvania, Children’s Hospital of Pennsylvania, papers, <12 months in 14/49, and 12–24 months
Philadelphia, PA, USA
e-mail: LongC3@chop.edu in 49. In total 84% of the included studies on the
892 C. J. Long
TIP repair included follow-up less than 24 months. complications. Some argue that the majority of
The authors also highlight a significant limitation complications will be identified within the first
of their analysis: variability in the quality of the year of surgery [20]. Many other studies, how-
data and the lack of agreement or standardization ever, indicate that as few as 50% of complica-
of the assessment of complications. These factors tions are detected within the first year after
have been highlighted by others and limit our surgery [8, 9, 11]. Spinoit et al. identified compli-
ability to combine similar patients across institu- cation rates for 474 primary hypospadias repair,
tions in order to perform a high-quality meta- the majority of which (77%) were distal [9]. At a
analysis [13]. median follow-up range of 34 months, 47% of
While one could argue that the ability to col- distal and proximal complications were identified
laborate for distal hypospadias is unnecessary within the first year, while 38% were identified
given its high prevalence, the same cannot be said beyond 24 months post-surgery. Grosos et al.
for proximal hypospadias. Proximal hypospadias examined outcomes for 578 of their patients,
occurs in 20% of patients in most reports, often 90% of whom underwent a distal Thiersch-
resulting in 20–30 new patients yearly at even the Duplay repair [8]. At a median follow-up of
busiest centers in the United States. Prior to 2015 25.6 months, 53% of complications were identi-
most proximal hypospadias repair outcomes fied within the first year, while 47% were detected
were grouped among the much larger cohort of as late as 10 years after the initial operation.
distal hypospadias repairs. Mixing these patient Similarly we recently examined 1280 patients
cohorts results in an overall complication rate operated upon within our institution. We had a
that is more optimistic than it is in reality [14]. median follow-up of 7.9 months, with a total
In 2015, the first of several single institution complication rate of 19.1%, including both prox-
reports focusing on proximal hypospadias imal and distal hypospadias repairs [11]. We
emerged, with disappointing results. Stanasel found that 47% of complications occurred within
et al. included 56 patients that underwent a two- 1 year, 46% within 1–5 years, and 7% more than
stage Byars’ flap repair at a median follow-up of 5 years after surgery. These outcomes suggest
34 months and identified a 68% complication that follow-up that does not extend beyond 1 year
rate [15]. McNamara et al. included 134 patients after surgical intervention will ignore a signifi-
with a two-stage Byars’ flap repair at a median cant number of complications.
follow-up 40 months with a 53% complication Another consideration is the timing of compli-
rate [16]. Pippi Salle et al. included 140 patients cations. In general urethrocutaneous fistula and
undergoing a long TIP, dorsal inlay graft TIP, and glans dehiscence are more common in the early
two-stage repair with a complication rate ranging period of follow-up, while later complications
from 38 to 61% [17]. Long et al. included 168 include meatal stenosis and urethral stricture.
patients undergoing either a single or planned While a fistula can be more noticeable for the
two-stage repair, with a complication rate of 61 patient, a subtle urethral stricture or meatal steno-
ad 43%, respectively [18]. These manuscripts sis may go unnoticed for an extended period of
were important in that they exposed a much time without regular follow-up. More regular
higher complication rate than expected for proxi- follow-up with the surgeon with objective mea-
mal hypospadias. Alternatively the two-stage sures such as witnessing the urinary stream and a
graft repair (STAG) has a reported complication uroflow could help identify concerns in a timely
rate closer to 25% [19]. These high complication manner.
rates warrant attention, and efforts must be made The abnormal anatomy of the hypospadiac
to overcome the challenges of proximal hypospa- penis contributes to complication development.
dias mentioned above. One issue relates to the length of the repair and the
In addition to the variability in quality of the elastic nature of the tissue used for reconstruction.
literature, the duration of follow-up in many stud- Compared to the normal urethra, which is lined and
ies is too short to capture the full complement of reinforced with a robust spongiosum, the hypospa-
66 Long-Term Follow-Up in Hypospadias Repair: What Is It and Are We There Yet? 893
diac urethra lacks a true spongiosum. This remains to extend our efforts into a universal document
true even after urethral reconstruction, as the lack that can now be disseminated across institutions.
of spongiosum places the elastic nature of the skin Using a standard template will facilitate multi-
graft or flap at risk for expansion or bowing in institutional collaboration that is sorely needed in
response to the pressure of a urine bolus [21]. This hypospadias care. This will allow us to generate
effect is further amplified in more proximal repairs comparisons across surgeons, identify the ideal
which result in a longer neourethra in reconstruc- approach to a repair, and figure out how to prop-
tion. Given these factors, one can imagine the effect erly assess outcomes—all with the intent of
that a repeated urine bolus propagated through a improving the care to our patients.
more pliable neourethra would remain at risk for Given the concerns for complication develop-
many years after the initial repair. In the clinical ment and the emerging data on the need for con-
setting, these patients can present with post-void tinued follow-up, emphasis must be placed on
dribbling in its most mild form, require urethral patient and parent education. If one tells the fam-
milking after voiding, or, in its most severe form, ily that a successful outcome can be designated
could result in a large urethral diverticulum with within 6 months of surgery, the family will be
significant urine pooling. It is unclear as to what resistant to commit to further follow-up. Instead,
impact these tissue characteristics will have long just as we outline the etiology and indications for
term and warrants close attention. surgery with the family, the surgeon must spend
Examination of adult men that had a prior hypo- time outlining the need to follow patients as they
spadias repair as a child gives further insight into grow up to monitor them and to ensure proper
the durability of repairs. These patients had a vari- function. The urinary stream cannot be fully
able degree of baseline hypospadias severity, which assessed until the patient is toilet trained and the
can present at any age, and typically require a com- stream can be properly witnessed. It is also
plex surgical reconstruction [22–24]. Although unclear as to whether the reconstructed urethral
concerning, these studies are limited by a high tube will change in pliability over time, placing
degree of loss to follow-up, heterogeneity of data, the patient at risk for altered urinary function
and a lack of use of standardization of outcome with time. In a similar fashion, sexual function
measures. Patients often migrate to institutions and quality of erections cannot be fully assessed
beyond their initial treatment center, limiting until the patient is an adult and sexually active.
access to the technical aspects of the original repair. Follow-up that does not extend to these bench-
Even when this information is available, the previ- marks is likely inadequate and assumes that the
ously mentioned variability in severity assessment repair will maintain its integrity in the setting of
limits the ability to compare outcomes for these the trauma of sexual function. Establishing these
patients. These patients also often have a large gap guidelines with families from the outset will
in their follow-up interval, often with no follow-up guide expectations and increase the adherence to
since their early childhood, leading one to wonder follow-up.
what mitigating factors occurred in the interim. Much of the focus on outcomes related to
Suitable follow-up is not as simple as follow- hypospadias includes surgical measures, such as
ing patients for a longer period of time. The qual- the rates of fistula development. Patient-reported
ity of the assessment and the data collected must outcome measures (PROMs) aim to incorporate
also improve drastically. In 2014 we recognized the patient, parental, and surgeon input into the
the limitations of our own documentation process outcome assessment. There are currently five
and as a result standardized both the office and validated questionnaires in the literature. These
operative notes. Templates were generated in our includes the Hypospadias Objective Scoring
electronic medical record (EMR), allowing us to Evaluation (HOSE), Pediatric Penile Perception
collect intraoperative and office data related to Score (PPPS), Penile Perception Score (PPS),
outcomes. As part of a Society for Pediatric Genital Perception Scale (GPS) for adults, and
Urology task force in hypospadias, we were able the Genital Perception Scale (GPS) for children
894 C. J. Long
Fig. 66.1 Follow-up protocol template
[25–27]. Each is unique in their assessment, but protocol (Fig. 66.1). This would include every
all tend to focus on the cosmetic outcome for patient operated upon from infancy through
their grading system [28]. There is a clear lack of adulthood. In the real world, however, this
focus on the urinary function, sexual function, remains a distant dream. Patients and families
and the psychological element of the repair. tend to avoid follow-up visits if they perceive that
Currently there are no standards for use of PRO their son is doing well, in part due to time
in a follow-up regimen as centers and surgeons restraints and in other due to financial reasons,
are left to their own preferences. As we continue such as co-pays and travel. Families also rarely
to push toward excellence in our field, it is clear stay in one location throughout a full childhood,
that PROM will become a key component of that and once they move, they lose contact with their
effort and must be pursued with regularity. surgeon or treating hospital. Given the benefits of
Another valuable aspect of long-term follow-up long-term follow-up, the onus is on us as sur-
evaluation lies in our current understanding of the geons to drive these efforts and ensure that the
psychological impact of a hypospadias repair. In patient receives comprehensive care.
particular, boys during the adolescent time period Development of hypospadias specific PROMs
are at high risk for poor body image and to have will further refine our interventional strategies to
concerns with their diagnosis and the appearance of correlate anatomic findings to patient function
their penis [29]. Studies have shown that boys with and satisfaction. Finally, reporting on follow up
poor body image are at higher risk for worse out- of all of our patients after surgery, not a select
comes as adults [30]. Examination of men that have few, is important so that we can fully appreciate
had a childhood hypospadias repair shows that they all of the nuances of hypospadias surgery.
have similar age at onset of initiation of sexual con-
tact. Although these men have been found to have
less sexual satisfaction, they are equally active as 66.1 Conclusion
their peers [25]. Patients with proximal hypospadias
are noted to have slightly worse outcome measures Long-term follow-up of boys with hypospadias is
in addition to shorter penile growth at puberty [31]. lacking. It is incomplete due to the lack of quality
In the ideal world, every patient that we oper- data, short duration of follow-up, and lack of
ate upon would adhere to a standard follow-up consistent inclusion of patient-reported out-
66 Long-Term Follow-Up in Hypospadias Repair: What Is It and Are We There Yet? 895
comes. Efforts geared toward improving these cohort analysis. J Urol. 2020; https://doi.org/10.1097/
JU.0000000000000762.
limitations must be made as we seek to improve 12. Pfistermuller KLM, McArdle AJ, Cuckow PM. Meta-
care delivery for our patients. analysis of complication rates of the tubularized
incised plate (TIP) repair. J Pediatr Urol. 2015; https://
doi.org/10.1016/j.jpurol.2014.12.006.
13. Wilkinson DJ, Farrelly P, Kenny SE. Outcomes in dis-
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Hypospadias: Psychosocial
and Sexual Development 67
and Consequences
Anne-Françoise Spinoit and Yakov Reisman
Abbreviations allow urination in the standing position, and to

enable normal sexual function and fertility [7].
DSD Disorders of sex development The evaluation of what seems to be the best
ESPU European Society for Pediatric Urology timing of surgery is based on experts’ opinion,
SPU Society for Pediatric Urology with an evolution over the decades. In 1975 it
was, according to the American Academy of
Pediatrics, assumed that the period after the third
year of age would be the optimal time for surgical
67.1 Introduction intervention [8]. It was based on the available
material for surgery and the lack of microsurgical
Hypospadias is the most common malformation equipment, making the surgery difficult in small
of the penis and one of the most common birth penises. In 1996, the same committee revised its
malformations [1]. Severe forms are associated opinion, with the advent of microsurgical instru-
with a curvature of the penis (chordee) and a ure- ments and the belief that surgery would be better
thral opening that lies closer to the perineum [2]. tolerated on children after potty training. It was at
The etiologies of hypospadias have commonly that moment stated that the best time for surgery
considered genetic, endocrinological, and envi- to treat hypospadias would be between 6 and
ronmental factors [3, 4]. Reports on the incidence 12 months of age [9]. Technical considerations
of hypospadias are inconsistent, but there is evi- were no longer considered the major limiting fac-
dence that it has increased over the last decades tors in determining the timing for the surgery of
[5]. The estimated incidence is approximately one hypospadias. Considerable importance was
per 300 or three per 1000 male births in western instead given to psychological factors such as
countries, and the prevalence seems to be mark- emotional, cognitive, and body image develop-
edly increasing [6]. The surgical goal of hypospa- ment that may be affected by the genital defor-
dias repair is to achieve a typical-looking penis, to mity and the reconstructive surgery. It is also
believed that early genital surgery could mini-
mize disturbances in the patient’s psychological
A.-F. Spinoit (*) development (see Chap. 15: “The Story of my
Department of Urology: Pediatric and Reconstructive
Life”). But it is important to note that none of
Urology, Ghent University Hospital, Ghent, Belgium
these recommendations are evidence-based and
Y. Reisman
rather based on expert opinions.
Sexologist, Andrologist, European Society for Sexual
Medicine (ESSM), Senior lecturer of Clinical Next to expert’s opinions regarding the best
Sexology at the RINO, Amsterdam, Netherlands timing for surgery, there is a growing body of
898 A.-F. Spinoit and Y. Reisman
questioning the necessity itself to operate hypo- behavior patterns by engendering sex-specific
spadias, especially at an age where the child is brain structures. Most of the evidence comes
not in a state to provide his consent. There is from studies of people with disorders of sex
indeed a growing concern that corrective surgery development (DSD), which are exposed to an
performed at a young age might be harming the atypical hormone synthesis [18, 19]. Still, post-
rights of children and their bodily integrity, as the natal androgen receptors seem to be equally pres-
necessity for surgery appears increasingly chal- ent in both hypospadias and control populations
lenged [10]. Institutions like Amnesty and evolve similarly with age [20, 21], triggering
International, the Human Rights Watch, the many questions on the pathways leading to con-
United Nations, and the European Union genital penile defects.
Fundamental Rights Agency all share this con- Publications on the psychological, social, and
cern, considering hypospadias as genital surgery sexual development of patients operated on for
encompassed in the larger spectrum of differ- hypospadias are still rare, and the results are
ences of sexual development. Parents facing sur- somewhat discordant. The possible explanations
gery decisions may find the weight of medical for these discrepancies are mainly methodologi-
opinions and human rights opinions pulling them cal, with too small series, lack of standardized
in opposite directions, making decision-taking questionnaires, and, when available, low rates of
even harder. To guide parents and provide expert’s response to those questionnaires, study popula-
opinion on those statements, the ESPU and SPU tions of different ages, and above all the absence
wrote a conjoined statement comforting the need of a control group, which prevents any compari-
for surgery in many cases [11]. On the other son of the results with those of a reference popu-
hand, the very scarce data about hypospadias lation [22, 23]. Another possible reason for
repair during adulthood showed worse results difficulty in long-term follow-up is that the
compared to surgery performed at an early age patient, after growing up to be an adult, often
[12], comforting the actual recommendation for does not follow up with the initial surgeon [24].
surgery at an early age [13]. In this chapter, some of the knowledge about
From psychological perspectives, psycho- the consequences of hypospadias on psychoso-
logical stress contributing to an increased risk of cial adjustment including behavior and psycho-
behavioral maladjustment and impaired quality sexual development of children and adolescents
of life has probably a various impact according is summarized. It is important to mention that
to the timing of surgery, the number of surgeries health-related quality of life in hypospadiac
undergone by the patient, and the final func- patients during adulthood is very scarcely inves-
tional outcome into adulthood [14]. Stressors of tigated. Important issue is to summarize how
hospitalization and genital surgery are contrib- important is the correction of the hypospadias for
uting, but the boys may also grow up with a cos- the development of the child. The impact of the
metically and functionally impaired penis [15]. deformity extends beyond the realms of a struc-
The parents’ anxieties about the boy might tural defect, by virtue of the diverse functions of
result in an overprotection, which may limit the the penis. To counsel parents and patients appro-
patients’ development of autonomy and self- priately, it is essential to know the effect on sex-
confidence [16]. ual function and reproduction.
Except for psychological influences, there is
also evidence that faulty embryogenesis of the
penile urethra is related to abnormalities in pre- 67.2 ppearance, Satisfaction,
A
natal androgen synthesis and/or androgen recep- and Psychosocial Development
tor defects [17]. There is increasing evidence that
prenatal exposure to androgens does not only There is some controversy on satisfaction about
induce the sexual differentiation of the human penile appearance after penile surgery. Some
genitalia but also influences postnatal sex-typed series observed on one hand that the rate of dis-
67 Hypospadias: Psychosocial and Sexual Development and Consequences 899
satisfaction with penile appearance is higher in anxiety and poorer adjustments compared to
the hypospadias group than in age-matched con- peers [16, 33, 34], while others found no differ-
trols, with results ranging between 25 and 40% ences [27].
dissatisfaction vs. 5 and 34% in the control The findings regarding psychosexual develop-
groups [15, 25, 26]. Scars, penile size, and glan- ment, however, demonstrate in some studies that
dular shape were the most spontaneously boys with hypospadias suffer from negative geni-
reported reasons for dissatisfaction. In some tal appraisal and sexual inhibitions [27], while
studies, it has been found that higher percentage other studies are quite reassuring regarding this
of those with proximal hypospadias were dissat- issue in the long term [35]. Nevertheless, age at
isfied with the penile appearance than those who first sexual experiences and general sexual behav-
were operated for distal hypospadias (40% vs. ior appeared to be less affected by their penile
19.2%) [25, 27, 28]. malformation [15, 36].
A major concern is the penile size. Data varies
about the topic with Mureau [27] and Moriya
[25] showing that the main reason for dissatisfac- 67.3 Hypospadias and Sexuality
tion was penile size. However, a larger study with
healthy controls showed that the dissatisfaction Older studies reported higher frequency of sexual
about penile length was similar in both groups inhibitions (seeking sexual contacts) in patients
[29], suggesting thereby the inherent condition of operated for hypospadias compared to controls
some men who always seem dissatisfied with [37]. The percentage was higher in patients with
their penile length, no matter the condition. proximal hypospadias repair or group with com-
Regarding satisfaction about outcomes, data plications [27]. Hypospadiac patients reported a
are again very variable. Fifty-two adolescents more negative genital appraisal than the controls;
(between 13 and 15 years old) who had hypospa- they did not have a different sexual adjustment.
dias surgery as children were surveyed by Jones Higher patient age at final operation had a nega-
et al. regarding a possible association between tive impact on sociosexual development. More
recollection of surgery and dissatisfaction with recent studies using more reproducible assess-
body appearance [30]. Those who recalled going ments and using a longer follow-up than the pre-
through the process (i.e., those older than 5 years vious ones seem to contradict those initial data,
at the time) reported poorer body image satisfac- showing a very low impact of hypospadias sur-
tion [30]. Unfortunately, the authors lacked body gery [29, 35].
satisfaction scores from a control group of ado- There is some evidence for reduced libido in
lescents with hypospadias who did not undergo patients with hypospadias, but these studies show
surgery, and it is unclear from the retrospective only tendencies without significant differences
data if the memory of the event was modified by when compared to controls [24]. Patients with
other factors or by caregivers. proximal hypospadias and multiple procedures
Data from a few series showed however that differed from those with distal hypospadias and a
parental decision regret regarding surgery was single procedure in all parameters of sexual psy-
correlated with the final outcome of the surgery chology (P < 0.05) [38].
[31] or with the number of surgeries needed [29, Bubanj et al. observed that though the fre-
32]. Interestingly, the hypospadiac patients pro- quency of intercourse during 4 weeks was sig-
vided a better end evaluation score as adults nificantly lesser for those who were operated for
about their penis than the surgeon who performed hypospadias, there were no significant differ-
the surgery [29]. ences between patients with hypospadias and
Concerning depression, anxiety, and psycho- controls regarding inhibition in seeking sexual
social adjustment, there are incoherent results. contacts or patterns of sexual relationships [39].
Some studies noted that adult men operated for Those with distal hypospadias were more satis-
hypospadias more often recalled depression and fied with their sexual life [39]. Other study
found that hypospadiac patients are as active as 67.4 Consequences According

controls but are less satisfied [40]. Patients less to Time of Surgery
often have intimate relationships compared to
controls [40]. In practice, there are three populations of patients
Patients with proximally hypospadias differed with hypospadias: those operated on in childhood,
significantly from distally hypospadiac patients those operated on when adults, and those for whom
also in erectile function. Those proximally hypo- surgery is not considered necessary, although there
spadiac patients complained more of downward is growing debate about who exactly belongs to the
curvature during penile erection and erectile pain latter, as discussed above [47].
[41]. The erectile problems were more in those
who had proximal hypospadias.
Inability to achieve satisfactory ejaculation is 67.4.1 Patients Operated
documented in many publications. Reported inci- on in Childhood
dence ranges from 6 to 37% [15, 36]. Problems
reported include weak or dribbling ejaculation, Psychological and social development, as
having to milk out ejaculate after orgasm, quan- assessed by professional or socioeconomic suc-
tity of semen passing after intercourse, and ane- cess, was not modified in most of the studies
jaculation with or without orgasm [24]. In [48]. However, behavior during childhood was
addition, ejaculation difficulties occurred more more reserved and less outgoing (children’s
frequently in proximally hypospadiac patients timidity or parental overprotection). Lastly, there
than in distally hypospadiac patients (33% vs. was no correlation between the severity of hypo-
17%) [42, 43]. These difficulties presented as spadias and disturbances of psychosocial devel-
spraying, dribbling, or delay in ejaculation. These opment or function. Adolescence is associated
problems were probably because of a higher with difficulties in undressing in public, during
occurrence of postoperative urethral strictures, sports activities, for instance, due to a feeling of
diverticula, insufficient support of the spongio- shame or fear of showing oneself naked (because
sum tissue in the reconstructed urethra, or abnor- of “difference”), which is a corollary of the
mal development of the prostate and seminal patients’ opinion that their genital organs do not
vesicles [39, 42, 44]. have a satisfactory or normal appearance.
Literature is scant on the fertility of men who Although sexual maturation and debut are
had hypospadias. There is indeed an inherent dif- normal in adolescents operated on for hypospa-
ficulty to assess fertility: is there a testicular dys- dias in childhood, on the other hand, greater dif-
genesis syndrome associated with severe ficulty in making contacts with the opposite sex
hypospadias and possible hypofertility [45] or is or even delayed initiation of full sexual activity is
the possible hypofertility associated with obstruc- often reported. Follow-up until late adolescence
tive patterns [46]? Aho et al. found no significant is often demanded by patients operated on in
difference between men who had hypospadias childhood and reviewed as adults, which reflects
during childhood and controls regarding the like- to some extent the psychosocial and sexual diffi-
lihood to live with a partner or regarding the culties that result from hypospadias [16, 27].
number of fathered children [37]. Semen was In adulthood, although some studies conclude
examined in 169 men who were operated for that sexuality is satisfactory overall, there is a
hypospadias in childhood [44]: only one of the 32 high incidence of problems of erection, which
who had fathered a child had sperm count of may be linked to the persistence of penile curva-
<20 million/mL. But 40 of the remaining 137 ture but also to sensitive scars or a “soft” glans.
(29%) whose fertility was not proven had sperm Problems of ejaculation are also often reported,
counts <20 million/mL. such as dribbling, retained or delayed ejaculation,
or absence of ejaculation. These anomalies may the group operated on once (3 [16%] of 19,
be secondary to dilations of the reconstructed ure- P = 0.03), the group operated on at least twice (2
thra, in the case of retained or delayed ejaculation [12%] of 17, P = 0.02), or the men operated on as
[49]. Dribbling ejaculation, which seems to be the a whole (5 [14%] of 36, P = 0.01). Although con-
most frequent anomaly, may be similar to drib- firmation is required in a larger series, the authors
bling antegrade ejaculation, which occurs in men suggest that hypospadias in itself, rather than the
with spinal cord injury, especially since these surgical procedure, may perturb psychosexual
hypospadiac patients also report leaks or drops of development, and they recommend that hypospa-
urine after micturition. Briefly, dribbling ejacula- diac patients be followed up during adolescence
tion suggests abnormal emission of sperm, raising whatever the severity of their genital malforma-
the question of the functional integrity of the tion so that those with psychosexual difficulties
nerve pathways [15, 27, 33]. can be detected as soon as possible [51].
67.4.2 P
atients Operated on During 67.5 Own Clinical Experience
Adulthood
The availability of long-term data about the con-
The treatment of these patients was often delayed sequences of hypospadias repair in the literature
for cultural, religious, or sociodemographic rea- is very limited. Previous attempts to conducts
sons. Hypospadias was usually of distal type surveys among urologist concerning these issues
[50]. None of these studies provide any informa- failed. On the other hand, the treatment
tion on the psychosocial development or sexual approaches in clinical settings have not been
function, whether before or after surgical correc- described. For these reasons, I decided to shortly
tion, of patients operated on during adulthood, summarize my own clinical experience. The
except that before surgery 40% of patients did reader should keep in mind that the data pre-
not have sexual relations [49, 50]. sented here is probably biased as many of the
patients, who are not referred to tertiary institu-
tions, are probably satisfied and without
67.4.3 Non-operated on Patients complaints.
In the last 10 years, I have seen about five
Patients born with hypospadias and in whom sur- adult patients per year with sexual complaints,
gery had not been considered necessary do not years after the hypospadias repair. The majority
seem to differ from patients operated on during of them had multiple operations due to severe
childhood where voiding problems and sexuality hypospadias (mid- and proximal shaft).
are concerned. However, studies more specifi- Important issue is the patient’s concern; the
cally devoted to such patients seem necessary to physician should explore the real patient’s help
confirm these incomplete findings and to investi- request and the main concerns. In general I can
gate fertility. divide them into two patient groups:
Of the 42 hypospadiac patients identified dur-
ing medical and psychological examination of • For the patients with earlier sexual experience
11,649 men aged 18 years during Italian National and sexual partners, the main sexual concerns
Service, 6 (14%) had never been operated on, 19 are about performance. The main complaints
(45%) had undergone a single surgical proce- are about penile deviation or pain and discom-
dure, and 17 (41%) had undergone at least two fort during sexual activity.
operations [51]. Difficulty in initiating contact • The majority of patients, without previous
with the opposite sex was significantly greater in sexual experience, have concerns about
the non-operated on group (4 [67%] of 6) than in appearance, penile length, and “normality.”
The detailed and extensive history taking clinical practice. Based on the findings that boys
should include information about the number of with hypospadias seem in some studies to suffer
operations and in which age, behavioral pattern from negative genital appraisal more often than
with peers and the opposite sex, previous sexual healthy boys, on the other hand, other studies are
experience, and exposure to sexual information reassuring regarding this issue. Anyway, the
and education. hypospadiac patients might profit from psycho-
A physical examination of the genitals is social support for the development of sufficient
obligatory, and it’s often giving a clue about the self-confidence which would possibly encourage
patient’s attitude toward his own genitals. them to better accept their penis.
Measurement of stretch penile size is done in Surgeons must be aware that genital appraisal
case of concerns about appearance, girth, and does not exclusively rely on the quality of surgi-
length. cal outcome, and objective evaluation at the hand
Due to their negative genital appraisal and of standardized questionnaires seems to be the
sexual inhibition, compared to age-matched key to the future for optimization of outcomes.
adults, their attitude and knowledge toward sexu- Thus, optimal medical care for hypospadiac
ality and sexual behavior were limited. Because patients cannot merely be achieved by optimizing
of the genital abnormality and the behavioral surgical results. In order to guarantee a compre-
inhibition, many of them never had the opportu- hensive treatment concept, the incorporation of
nity to compare themself to peers, during the pre- psychological factors such as the anxiety of
vious years. Many of them avoided situations like rejection by eligible sex partners may be equally
taking showers together with peers after sport important. Moreover, it is tenable that genital
classes or swimming pools. appraisal and sexual inhibitions may be affected
The first step in the treatment includes psy- to a higher degree during adolescence than dur-
choeducation. In this stage, information about ing childhood. Hence, it is believed to be essen-
“normality” should be given. The use of images tial that patients with hypospadias will need to be
could be helpful as well. Furthermore, a descrip- followed up until young adulthood. Apart from
tion of the sexual response should be given. For regular urological medical examinations, the
many of the patients, these steps together with patients should be appropriately informed about
reassurance could be sufficient and encourage their penile condition and to be offered psycho-
them in their search for reliable information. logical support. Regular self-assessment and the
In case of deviations, surgical intervention use of standardized patient-reported outcomes
could be considered. Complaints about pain or measurements are the key to the future in hypo-
discomfort could be caused by hyperactive pelvic spadias optimization.
floor; in these patients, consultation of physical For improvement in long-term therapeutic
therapist could be done. In case of neurinoma or outcomes, it is important to choose rational surgi-
scare tissue as cause of pain, the use of local anal- cal procedures, minimize the number of opera-
gesics or corticosteroid injections could be con- tions, prevent complications, perfect
sidered, although the evidence for this method is postoperative penile appearance, and offer post-
missing. Surgical interventions are the last resort operative long-term follow-up and psychosexual
in very persistent complaints. counseling. External factors, such as parents’
opinion, can have more influence on deciding for
a surgery than the objective status of the penis. In
67.6 Conclusion fact, high anxiety can be often observed in rela-
tives of hypospadiac children; this can lead to
The inconsistent results and the lack of knowl- surgery even in mild forms of hypospadias and
edge about the influence of psychosocial factors exert a negative influence on child’s attitude,
on the psychological development of boys with instilling the perception that the malformation is
hypospadias allow only limited implications for more severe than it actually is.
When hypospadias reconstruction is per- Pediatr Urol. 2011; https://doi.org/10.1016/j.

jpurol.2010.11.009.
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49. Hensle TW, Tennenbaum SY, Reiley EA, Pollard M. Hypospadias: incidence and effects on psycho-
J. Hypospadias repair in adults: adventures sexual development as evaluated with the Minnesota
and misadventures. J Urol. 2001; https://doi. multiphasic personality inventory test in a sample of
org/10.1097/00005392-200101000-00019. 11,649 young Italian men. Urol Int. 2002; https://doi.
50. Snodgrass W, Villanueva C, Bush N. Primary and org/10.1159/000048423.
reoperative hypospadias repair in adults—are results
Tissue Engineering and Future
Frontiers 68
Magdalena Fossum
Abbreviations that contain a specific population of autologous

cells, organogenesis is mimicked to replace and
EGF Epidermal growth factor restore tissue or organ function. The term “tissue
GMP Good manufacturing practice engineering,” as currently understood, was intro-
MeSH Medical Subject Headings duced in medicine in 1987 [3]. Tissue engineer-
PAIS Partial androgen insensitivity ing aimed at the action of “generating tissue
syndrome in vitro for clinical applications, such as replac-
PBS Phosphate buffer saline ing wounded tissues or impaired organs.” It was
PEST Penicillin and streptomycin introduced as a MeSH term in PubMed in 2002
and is now a practical reality that has been
accepted as its own discipline.
Advances in cellular biology and biomaterial
68.1 Background sciences have been crucial in combination with
increased interaction between basic science
68.1.1 Tissue Engineering researchers, laboratory staff, and physicians. In
translational research, the key elements for
Tissue engineering can be briefly described as the implementation and success are the communica-
action of replacing tissue structures with cells tions between physicians, who are exposed to
coupled to biological or artificial matrices or patients’ needs and make use of the new meth-
scaffolds that guide the cells during repair and ods, the preclinical researchers, whose scientific
regeneration [1, 2]. By transplanting constructs expertise cannot be sustained by the clinician,
and the biotechnology enterprises that support
M. Fossum (*) good manufacturing, design, logistics, and avail-
Department of Pediatric Surgery, Copenhagen ability to the clinic.
University Hospital, Rigshospitalet,
Copenhagen, Denmark
Laboratory of Tissue Engineering, Rigshospitalet, 68.1.2 Tissue Engineering in Urology
Faculty of Health and Medical Sciences, University
of Copenhagen, Copenhagen, Denmark
Reznikoff and co-workers reported the first suc-
Department of Women’s and Children’s Health,
cessful in vitro cultivation, passage, and charac-
Laboratory of Tissue Engineering, Karolinska
Institutet, Stockholm, Sweden terization of human urothelial cells [4].
e-mail: magdalena.fossum@ki.se Large-scale cell expansion of keratinocytes had
908 M. Fossum
previously been established to treat severe burn with sterile saline via an indwelling catheter
wounds with cultured epidermal autografts [14] (Fig. 68.2a). To the best of our knowledge,
[5–7]. these were the first patients ever to be treated
In general, urothelial cells and keratinocytes with cultured autologous urothelial cells within
have many properties in common. Both are epi- the field of reconstructive urology. At the pres-
thelial cells with a barrier function to maintain ent time, the patients have been followed until
the inner body environment (Fig. 68.1). They after puberty, and they all present with straight
express similar proteins that can be used as cell- erection, good urinary flow, and an adequate
specific markers, and they have similar growth cosmetic appearance [15, 16].
characteristics [8, 9]. The extensive knowledge of Other studies have included tubular tissue-
keratinocyte culturing and transplantation can engineered grafts for patients with long proximal
therefore also be applied to urothelial cells. urethral strictures after pelvic trauma [17]. A bio-
To our knowledge, the earliest penile recon- material alone, without cells, may be usable in
struction using cultured autologous cells was smaller urethral defects; however, the addition of
reported in the early 1990s. In this series, autolo- urothelial cells seems crucial for long-term suc-
gous in vitro cultured keratinocytes, retrieved cess in more complex cases and lowers the risk of
from the fossa navicularis of the meatus, were re-stricture [18, 19].
used to treat ten boys with severe hypospadias, Furthermore, in 2006 a series of seven patients
and the short-term results of the procedure were underwent bladder augmentation with cultured
reported to be successful [10, 11]. autologous urothelial and detrusor muscle cells,
In our own series, we operated six patients using quite different techniques [20]. The study
with severe hypospadias during 2000–2002, all protocol continued in 2007 in a phase II clinical
previously treated with a staged procedure for trial, based on autologous cultured cells on a syn-
correction of chordee and without any remain- thetic tissue scaffold, including ten patients with
ing surplus of local penile skin [12, 13]. The neurogenic bladders at four centers. Although
harvest of urothelial cells was performed with technically success was initially reported, the
minimal invasiveness by washing the bladder trial had to be discontinued in 2011 due to com-
plications, and most patients have now under-
gone conversion to a traditional ileocystoplasty
[21]. Early termination led to a small number of
participants and other problems leading to unreli-
able or uninterpretable data [ClinicalTrials.gov
Identifier: NCT00419120]. A planned similar
study for overactive bladder and urge was there-
fore discontinued before enrollment. Based on
these experiences, we can conclude that animal
experimental models related to tissue regenera-
tion cannot always translate to patients and that
long-term, maybe lifetime, follow-ups will be
crucial when introducing new tissue engineering
techniques to the clinic.
Fig. 68.1 Cross section of a normal mid-penile urethra
of an adult male. Note the transitional epithelium with a
top layer of polynucleated umbrella cells, a middle layer 68.1.3 Impediments
of polygonal intermediate cells with strong intercellular
connections, and a layer of cuboidal cells closest to the
basal membrane. The lamina propria with nerve endings,
Vast implementation of tissue engineering tech-
capillaries, and loose connective tissue is underneath the nologies is currently limited by drawbacks
basal membrane including long incubation times, financial
68 Tissue Engineering and Future Frontiers 909
a b c d
Fig. 68.2 (a–d) Photographs representing one of the ond stage of repair. (c) Immediate postoperative
cases: (a) postoperative appearance after the first stage of appearance after urethral reconstruction with an autolo-
repair with correction of the penile curvature. Note the gous, cultured tissue transplant. (d) Postoperative appear-
indwelling catheter through the perineal meatus for col- ance 3 weeks after reconstruction of the neo-urethra
lection of bladder washes. (b) Phenotype before the sec-
requirements, strict regulations, and often mul- 68.1.4 Expertise and Collaborations
tiple surgical steps. The time required for cell
isolation, cell expansion, and creation of the Collaboration plays a central role in developing a
transplant is laborious and therefore associated successful workflow for tissue engineering pur-
with high costs. In addition, to procure high poses. Specific cell culture facilities require large
quality and safety, European healthcare author- investments, continuous maintenance, and
ities have decided that the same regulations that devoted, well-trained laboratory expertise. To
apply to the production of pharmaceuticals develop good routines and high standard work-
should apply to all in vitro procedures using flows for patient cell expansion in vitro, a certain
autologous cells before transplantation back to turnover has to be reached over time, and dedi-
the patient [http://www.gmp-compliance.org/ cated laboratory personnel must be available also
eca_guideline_1477.html]. The GMP guide- at short notice to handle cells outside office
lines include important considerations such as hours, particularly if cells are harvested in emer-
quality controls, which must be applied not gency situations. After cell expansion, the cells
only to the final product (the cells) but also to have to be prepared for transplantation and trans-
each procedure involved. This applies to cell ported to the surgical unit, sometimes in another
harvesting, processing, cryopreservation, test- hospital or a different town.
ing, and distribution. Traceability has to be Many laboratories are dedicated to work in
established for the entire procedure, including cell culture research and cell expansions for
the materials used and the professionals micromolecular studies. In these cases, labora-
employed, which is accomplished by means of tory routines are usually based on the expansion
standardized registration forms and systems for of fibroblasts for analyzes of genetic material.
packaging, storage, and distribution. Another Other research laboratories might include animal
common drawback impeding current tissue cells or vectors for transmission or transfection of
engineering strategies is the need for an incon- genetic material with plasmids or viruses. It is
venient additional surgical step or procedure therefore of major concern to identify any con-
whereby cells can be harvested. flicts of interest before entering a collaboration
910 M. Fossum
where sharing of resources might be contraindi- At the laboratory, aliquot the bladder wash
cated for the purpose of handling patient tissues. into 50 mL tubes under sterile conditions before
Since keratinocyte cell culture has attained centrifugation (1300 rpm, 10 min). Then, remove
clinical acceptance for the treatment of severe the supernatant and resuspend with 10 mL PBS
burns and other reconstructive interventions, a with PEST using a pipette. Move into 15 mL cen-
good alliance might be achievable within the trifuge tubes. Centrifuge (1500 rpm, 5 min).
field of plastic and reconstructive surgery. Remove the supernatant and add 5 mL PBS with
PEST and resuspend again using a pipette. Pool
to fewer 15 mL tubes. Centrifuge again
68.2 Method for Tissue
A (1500 rpm, 5 min). Repeat the centrifugation
Engineering for Hypospadias steps until the cell solution originated from the
Repair bladder wash can be pooled in only one centri-
fuge tube. Remove the supernatant and add 3 mL
68.2.1 Cell Harvesting and Culture cell culture medium without EGF. Seed into a
laminin-coated culture well in a six-well culture
Insert a urinary catheter through the urethra into dish (approximately 10 cm2). Keep in the 37 °C
the bladder. Perform bladder washings manually cell culture CO2 incubator. After sedimentation
without force with sterile body-tempered saline (usually the day after), change the medium to cell
through a 60 mL syringe. Repeat the procedure culture medium with EGF. Cell culture medium
six to eight times to obtain a minimum volume of should be changed three times a week (i.e.,
250 mL (usually 300–500 mL). Transport the Monday-Wednesday-Friday). Growth of cell col-
resulting liquid directly to the laboratory in ster- onies should be identifiable after 1 week in cul-
ile vials at 5–10 °C (Fig. 68.3). ture, and approximately 500–1000 thousand cells
should be available after 1 month (confluent
25 cm2 monolayer) (Fig. 68.4).
68.2.2 Preparation of Transplants
Approximately 3 weeks before urethroplasty,

thaw the patient urothelial cells and seed them on
the scaffold (acellular dermis) in a culture flask
or in a Petri dish to create the urethral transplants
(Fig. 68.5). The thickness of the carrier should be
approximately 0.3 mm, and the size should be
rectangular with a surface area of approximately
25 cm2 (10 × 2.5 cm). During the last 2 days prior
to surgery, exclude serum from the culture
medium.
Immediately before transporting the urethral
Fig. 68.3 Disposables needed for bladder wash collec- transplants to the operating room, remove the
tion. (a) Catheter for the urinary bladder. (b) Syringe for
instillation and aspiration of saline to the bladder. (c)
original culture medium and replaced it with
Sterile plastic bottle for collection and transportation of transport medium (Leibovitz L-15 Medium,
the bladder wash. (d) 50 mL centrifuge tubes for the first Sigma-Aldrich) to protect the transplant by main-
pooling of the bladder wash. (e) 15 mL centrifuge tubes taining a physiological pH. Under sterile condi-
for washing and pooling of the urothelial cells. (f) Well for
primary culture of the urothelial cells from the bladder
tions, move the transplant from the culture dish
wash. The bottom surface area should be approximately or flask to the surgical table (Fig. 68.6). At all
10 cm2 (one well in a six-well plate) times, it is important to keep the transplant moist
Fig. 68.4 Cells in culture. Left: primary culture of urothelial cells after 5 days. Right: confluent cells in a T25 flask,
4 weeks after initiation of culture
of skin transplants tend to curl toward the dermal

side (urothelial cells on the convex side).
68.2.3 Hypospadias Repair
Urethroplasty should be performed at least

6 months after correction of the ventral penile
curvature. The size of the transplant should be
tailored for an onlay procedure with a sharp scal-
pel or scissors. Incise the penile shaft skin to cre-
ate a 10-mm-wide strip distal to the meatus on
the penile body and mobilize the lateral penile
skin (Figs. 68.7 and 68.8). Suture the transplant,
with the layer of cultured urothelial cells facing
the lumen, over a silicone stent (8Ch Koyle stent,
Fig. 68.5 Cell culture vials. (a) Culture well for initia- Cook®) with inverting interrupted absorbable
tion of primary culture from bladder wash. Bottom sur- braided sutures, 7/0 Dexon® (Tyco Healthcare).
face in one well is approximately 10 cm2. (b) Culture well
Cover the whole neourethra with a layer of sub-
for cell expansion after subculture (passaging). (c)
Cryopreservation tube for low-temperature freezing. (d) cutaneous soft tissue. When possible, create glan-
Acellular dermis for seeding of thawed cells and creation dular flaps to cover the transplant distally to place
of an autologous urothelial transplant the new meatal opening on the glans. If the glans
is small sized, choose a coronal position. Close
(with saline or transport medium) and to monitor the glans and the skin.
the orientation. Since the cells are seeded on the We have used multilayered bandaging with a
basal membrane of the dermis (the former epi- silicon mesh closest to the skin, then acrylic wad-
dermis side), this side should be marked with a ding covered by cotton winding that is sutured to
pen. Also, it should be remembered that the edges the skin with nylon thread in four directions
912 M. Fossum
Fig. 68.6 (a–c) Engineered transplant after culture: (a) surface of the acellular dermal scaffold. (c) Cross section
the transplant is kept moist after lifting from the culture of the biopsy specimen from the same transplant 3 years
flask. (b) Immunostaining with cytokeratin antibodies after surgery (pancytokeratin MNF116, Agilent Dako)
demonstrates a continuous layer of urothelial cells on the
(Ethilon, Ethicon®). Finally, the bandage and the nary flow, and residuals, and tissue regeneration
suprapubic catheter are taped to the skin in four based on macroscopic evaluation at cystoscopy
directions for further immobilization (Fig. 68.9). and micromorphology in biopsies. Artificial erec-
The bandage is changed, and the surgical wound tions were also controlled at both follow-ups,
is cleaned in general anesthesia after 1 week, but which were performed at 3–4 and at 6–8 years
the stent is left in place for 10–14 days. The post-transplantation, according to the study pro-
suprapubic catheter is removed after normal ure- tocol of the clinical trial.
thral voiding, approximately 2.5 weeks after sur- All patients had straight erections, and the
gery, and we have continued with oral antibiotics transplanted neourethra did not show any evi-
up to 1 week after removal of the catheters. dence of contraction or recurrent curvature up to
13 years post-transplantation. All boys were
voiding well through their neourethra without
68.2.4 Follow-Up straining. At the last clinical evaluation, all
patients stated that they could stand up and uri-
Six patients received a tissue-engineered autolo- nate; however, one of them preferred a sitting
gous urothelial transplant for the two-staged pri- position. This patient also had PAIS and a small
mary hypospadias repair between 2000 and 2002 and low-positioned penile body. Urinary flow has
(Fig. 68.2). In our follow-up studies of these been stable at follow-ups; four had bell-shaped
patients, we assessed the cosmetic appearance of urinary flow curves, and two had flatter curves,
the penis, functionality in respect to voiding, uri- but none of them presented with signs of dysuria,
a b c d
Fig. 68.7 (a–d) Illustration of the surgical technique: (a) of autologous transplant sutured over a stent. (d) Skin
Pen marking for incision. (b) Incised penile skin and closed after covering the transplant with a soft tissue flap
debridement of subcutaneous tissue. (c) Onlay placement (courtesy of Ahmed T. Hadidi)
a b c d
Fig. 68.8 (a–d) Photographs illustrating the surgical technique. (a) Measuring. (b) Pen marking. (c) Onlay transplant
over a stent. (d) Skin closed and trans-anastomotic stent is visible
urinary tract infection, residual urine, or second- the corona (Fig. 68.11). The penile skin was soft
ary changes to the bladder, suggesting high void- with no excessive scar formation.
ing pressures (Fig. 68.10). Postoperative biopsies from the transplanted
The appearance of the penis was cosmetically site did not show a uniform picture of the urothe-
satisfactory and well approved by the parents. In lial lining but most frequently a keratinizing
all cases, the opening of the urethral meatus was squamous cell epithelium (Fig. 68.6c). In the first
sufficiently large and located in or just distal to follow-up, the endoscopic evaluation included a
914 M. Fossum
patchy macroscopic appearance, and biopsy hair growth (ventral side) as opposed to the non-
specimens taken from such patchy areas revealed transplanted side (dorsal-penile skin side), and
a keratinizing squamous cell epithelium. By two patients presented with urothelium in biopsy
these means, the taking of biopsy specimens was specimens from the transplanted neourethra.
biased in favor of the squamous epithelium most Only one of the patients showed urothelium at
probably originating from the penile skin. The both evaluations. By these means, altogether, we
histological evaluation revealed a transitional have demonstrated urothelium in four trans-
urothelial lining in three out of six of the patients planted patients up to 8 years after the transplan-
at this time. tation. This finding is important and promising,
At the second endoscopic evaluation considering the small amount of transplanted
(6–8 years after transplantation), the whole trans- urothelial cells in relation to the large amount of
planted neourethra had a smooth lining and no well-vascularized non-transplanted skin that was
also included in the neourethra with this surgical
design.
We have concluded that hypospadias surgery
using cultured cells is safe and reliable. Due to
the novelty of the method and unknown long-
term effects, and in accordance with the initial
ethical committee decisions, we only treated a
few patients within this initial study. It is not yet
known whether the use of urothelial cells might
improve the final outcome, but to the best of our
knowledge, this is the first clinical study in
which cultured urothelial cells were utilized to
create the neourethra in severe hypospadias,
Fig. 68.9 Postoperative bandaging after surgery. The
suprapubic catheter is immobilized and carefully taped to additionally, with a long clinical follow-up
the abdomen, and the penile bandage is sutured and taped period.
to the skin
Fig. 68.10 Standing voiding position and flow curve at follow-up 3 years after urethral reconstruction
Fig. 68.11 Photos demonstrating the coronal position of the urethral meatus in two different patients, 4 years (left) and
8 years (right) after transplantation
68.3 Future Frontiers ation, it could be performed under sterile condi-

tions in any surgical operating room, in contrast to
In the future, the possibility of cell expansion in vitro cell culturing that requires sophisticated
in vivo may circumvent many of the drawbacks cell culture facilities [23–25].
related to in vitro cell processing. In these stud- Preclinical initiatives related to periurethral
ies, the body is used as a bioreactor, and ideally a penile tissues such as the spongiosa, corpora cav-
3D neourethra could be accomplished by ernosa, and glans demonstrate that these are also
enabling cell expansion, remodeling, and reorga- important tissues to regenerate and could be
nization in an orthotopic position. added in combination with autologous urothe-
Animal experimental studies including trans- lium for penile reconstruction and future transla-
plants of small, minced particles of epithelium tion to the clinic [26–28].
demonstrate that these can regenerate around a Other important developments include bio-
cylindrical mold to create 3D channels (Fig. 68.12) printing. In a publication from 2020, Urciuolo and
[15, 22]. Further in vitro experimentation has colleagues demonstrated how a cell- containing
shown that the combination of minced autologous hydrogel could be injected to the subcutaneous
tissues, collagen, and a core biomaterial allows space and then cross-linked in vivo to create 3D
for a 1:6 tissue expansion and good properties for cubes, asterisks, and flower-shaped structures,
surgical handling and 3D modeling. In addition, without even cutting open the skin in mice [29].
the supporting biomaterial could be loaded with By adding time as a fourth dimension, Kim and
growth factors or pharmaceuticals that enhance colleagues demonstrated 4D printing, which is
angiogenesis and wound healing. Further devel- when the shape of a bio-printed tissue transforms
opment of these methods could make them more over time, enabling important implications for
expeditious and inexpensive, and in a clinical situ- tubular structure tissue engineering [30].
916 M. Fossum
a b
Fig. 68.12 (a-b) Illustration of a preclinical in vivo tunnel. The transplanted minced particles have expanded,
model for guided tissue engineering. (a) Transplantation reorganized, and formed a continuous epithelium. When
of minced epithelium onto a tube. (b) Neo-generation of the tube is removed a channel with an epithelial lining has
epithelium covering the inner surface of a subcutaneous formed
In conclusion, even though the translational ISBN: 978-94-007-5689-2 (Print) ISBN: 978-
steps from basic science to clinical application in 94-007-5690-8 (Online)
patients have been cumbersome so far, new • Stacey, G. and Davis, J. (eds) (2007) Front
insights and technological advancements favor Matter, in Medicines from Animal Cell
the future use of tissue engineering techniques Culture, John Wiley & Sons, Ltd, Chichester,
for penile reconstructive surgery. UK. ISBN: 9780470850947 (Print)
9780470723791 (Online)
Recommended Reading • Horsch, R.E., Munster, A.M., Achauer, B.M.
• ECACC Handbook. http://www.sigmaaldrich. (eds.) (2002) Cultured Human Keratinocytes
com/life-science/cell-culture/learning-center/ and Tissue Engineered Skin Substitutes.
ecacc-handbook.html Thieme Medical Publishers, Stuttgart,
• Fundamental Techniques in Cell Culture Germany. ISBN-10: 3131301619 (Print)
Laboratory Handbook—4th Edition. Created ISBN-13: 978-3131301611 (Online)
from the partnership of the European
Collection of Cell Cultures (ECACC) and Acknowledgments I want to express my gratitude to col-
Sigma-Aldrich®. Copyright © 2018 Merck laborators at the Laboratories of Tissue Engineering at
Rigshospitalet and Karolinska Institutet. I also want to
KGaA, Darmstadt, Germany and/or its acknowledge Professors A. Nordenskjöld at Karolinska
affiliates. Institutet and G. Kratz at Linköping University for their
• Steinhoff, Gustav (ed.) (2016) Regenerative innovative thinking and kind support in developing the field
Medicine—From Protocol to Patient 2nd ed. of tissue engineering in reconstructive urology. The Novo
Nordisk Grant NNFSA170030576 supported this work.
2013, XXI. Springer, Dordrecht, Netherlands.
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and children. Scand J Plast Reconstr Surg Hand Surg.
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de Kort LMO, de Graaf P. A systematic review on cell- IBJU.2014.0422.
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Systematic Steps on How to Write
a Scientific Paper on Hypospadias 69
Luis H. Braga
Abbreviations niques, such studies are rare in the hypospadias

field [1, 2]. For these reasons, observational stud-
CB Caudal block ies (particularly case series) represent the major-
DC Decisional conflict ity of the current hypospadias literature [3].
DCS Decisional conflict score Although nonexperimental (observational)
DPB Dorsal penile block research strategies can provide valuable informa-
GD Glans dehiscence tion on surgical outcomes, they are often prone to
PICOT Population/intervention/comparative confounding from unmeasured factors and differ-
intervention/outcome/time horizon ent biases such as selection or recall preconcep-
PTS Preoperative testosterone stimulation tions [2]. Confidence in employing observational
RCTs Randomized controlled trials studies to inform clinical guidelines depends
SoF Summary of findings critically on transparent reporting, so that clini-
TIP Tubularized incised plate cians can independently assess the quality of the
UCF Urethrocutaneous fistula evidence and reliably apply study results to
UP Urethral plate change their practice [4]. Unfortunately, the qual-
VC Ventral curvature ity of reporting of observational studies in all
fields of urology remains suboptimal [5].
In 2007, von Elm et al. designed a 22-item
checklist to assist with clear reporting of observa-
69.1 Introduction tional studies: the “Strengthening the Reporting
of Observational Studies in Epidemiology”
Randomized controlled trials (RCTs) are consid- (STROBE) statement [6]. This checklist includes
ered to be the gold standard for evaluating inter- a description of methodological items and
ventions; however, due to ethical and logistical instructions on how to use them to properly report
challenges imposed by blinding and randomiza- observational studies. In this chapter, we have
tion of surgical participants, the high costs asso- applied the STROBE checklist to hypospadias
ciated with their implementation, and the need studies, aiming to increase transparency in report-
for clinical equipoise between two surgical tech- ing of these studies, allowing pediatric urolo-
gists/surgeons to critically evaluate quality,
L. H. Braga (*) identify bias, and integrate the best available evi-
Division of Urology, Department of Surgery, dence into clinical practice. In addition to improv-
McMaster Children’s Hospital, McMaster University,
Hamilton, Ontario, Canada ing quality, we expect that these recommendations
e-mail: braga@mcmaster.ca will ensure consistency in reporting in the hypo-
920 L. H. Braga
spadias literature, allowing for objective com- size depending on the publication venue). In a
parison between the results of different studies concise fashion, and following the same structure
and development of evidence-based clinical as the manuscript’s text, the introduction should
guidelines. detail the study purpose. The method’s section
This chapter will present itemized recommen- should describe the study design, population, set-
dations for adequate reporting of observational ting, and outcome measures. The result’s section
studies and case series dealing with hypospadias should include the most relevant findings, spe-
repair, ranging from drafting the title and abstract cifically reporting relevant summary measures,
to how to frame a discussion (Table 69.1). and the conclusion should reiterate main findings
Included with each item below is a brief explana- and their clinical importance.
tion of its importance and potential influence on a
study. We have also incorporated examples that
illustrate these recommendations, with pertinent 69.2.2 Introduction Section
discussion on how each specific item should be
reported. Nevertheless, none of the present hypo- 69.2.2.1 Background/Rationale
spadias literature yet conforms entirely to the for- (Item 2)
matting suggested herein. The introduction should explain the purpose of
conducting the research, based on previous
knowledge and publications on the topic, along
69.2 Application of the STROBE with the study’s goals or questions (e.g., what the
Statement Items new surgical technique adds to those previously
described). This section should also highlight
69.2.1 T
itle and Abstract (Item 1a what the hypothesis of the study is and why the
and 1b) research question is clinically important. This
part is not meant to involve a full literature review
69.2.1.1 Title but only a discussion of the relevant evidence,
The title and abstract should clearly indicate the justifying the need for the current study.
study design with commonly used standard
terms. The use of the PICOT format ensures that 69.2.2.2 Objectives (Item 3)
the title clearly describes the study: P = popula- Specific objectives of the study and predeter-
tion, I = intervention, C = comparative interven- mined (i.e., before analysis of the data) hypothe-
tion, O = outcome, and T = time horizon. The ses should be clearly stated. This is customarily
title should explicitly indicate what research described in the last paragraph of the introduction,
method was used to investigate the research as shown in the example below, and allows read-
question. ers formulate their opinion on whether the study
Title example: “Comparative outcome analy- was appropriately designed to test the research
sis of tubularized incised plate versus onlay question(s). In general, simply stating “to review
island flap urethroplasty for penoscrotal hypo- our experience with” a particular technique is not
spadias” [7]. sufficient to justify conducting a study.
Example for background and objectives
69.2.1.2 Abstract [8]:
The abstract should be a brief but detailed sum- Caudal block (CB) is one of the most popular types
mary of the study providing the highlights neces- of regional anesthesia used in children. It is com-
sary to understand the primary outcome, monly applied in pediatric inguinal/genital surgery
methodology, results, and interpretation. It is cus- as an adjunct to general anesthesia. CB provides
reliable postoperative analgesia in the pediatric
tomarily sectioned using specific subheadings population with a favorable safety profile and few
and limited to a brief, strict word count (style and anesthesia related complications. For these reasons
Table 69.1 Strobe summary table
Item Description
Title and abstract 1 Title Use the PICOT format (population, intervention, comparative intervention,
Abstract outcome, time horizon)
Provide a brief but detailed summary of the study. Include methodology, results,
and interpretation
Introduction 2 Background/rationale Explain the purpose of conducting the research
3 Objective Specify the objectives of the study and state the predetermined hypotheses
Methods 4 Study design Present the general study design and indicate the primary and secondary outcomes
5 Setting Description of the location of the study and relevant dates
6 Eligibility criteria Include both the inclusion and exclusion criteria and describe patient selection
7 Variables process
8 Data sources Include all outcome measures, interventions, risk factors, predictors, potential
9 Management confounders, and other applicable variable
10 Bias Describe how each variable was recorded, the source and methods of data, and how
11 Study size the data was collected
Statistical methods Acknowledge and explain how potential sources of bias were addressed
Control/for confounding Describe and explain how sample size was reached
Handling missing data Clear explanation of all statistical methods
Sensitivity analysis Outline the methods used to control for confounding
Identify if these were a priori or post hoc analysis
Evaluate and explain if missing data is random or systematic
Describe any sensitivity analysis
69 Systematic Steps on How to Write a Scientific Paper on Hypospadias
Results 12 No. of participants State the number of subjects included in each stage of the study
13 Reason for nonparticipation State why participants were excluded at each phase
14 Flow diagram Use a flow diagram to display information efficiently
15 Study participants Include baseline pt. characteristics table
Missing data Provide the number of subjects with missing data for each outcome
Outcome data Report the number of outcome events or summary measures
Unadjusted/adjusted estimates Report both adjusted and unadjusted estimates of your main results. Clarify which
confounders were included or excluded and why
Discussion 16 Key results Describe the main results highlighting the original goals and objectives
17 Limitations Acknowledge possible sources of bias and other study limitations
Other information 18 Generalizability Indicate the extent to which study results are generalizable
19 Funding State the funding sources and the role of the funders
20 Summary of findings tables To effectively communicate key study findings
921
922 L. H. Braga
CBs are offered in simple and complex hypospa- 69.2.3.2 Setting (Item 5)
dias cases, allowing for lower requirements of
inhaled and systemic anesthetics.
Provide a description of the location of the study
Although anesthesia related complications have and other relevant information including recruit-
been well described, controversy exists surround- ment, follow-up and data collection periods, as
ing the association between CB and hypospadias well as timing of exposure to interventions.
repair complications. Recently published abstracts
and articles have suggested that receiving a CB
Setting example [9]:
increases the risk of surgical complications in the Following approval by our institutional research
child compared to similar cases without a CB and ethics board, we prospectively offered participa-
with or without a Dorsal Penile Block (DPB). tion to families who were serially referred to a
Others have suggested that the location of the ure- single major pediatric hospital for evaluation of
thral meatus and the use of local epinephrine have distal hypospadias in their sons. A total of 27 fami-
been associated with Urethro-Cutaneous Fistula lies declined to participate without voluntarily cit-
(UCF) formation and not the type of regional anes- ing particular reasons for doing so. Exclusion
thesia used. However, studies to date have been criteria consisted of presentation for surgery past
limited by small sample sizes and patient selection the age of parental informed permission (i.e. able
biases involving indications for CB. As a result, it to participate in the decision-making process, 14
remains difficult to determine whether the reported patients), a history of genitourinary surgical proce-
higher complication rate is correctly attributed to dures (0), and lack of both parents being present at
the regional anesthesia strategy or other important counseling and consent for surgery. Thus, among
confounding factors, such as hypospadias severity, 158 eligible patients seen during a 1-year period
are at play. we enrolled and obtained consent and collected
In an attempt to address these limitations and fur- data from a cohort of 100 couples.
ther explore the possible association between CB
and hypospadias repair complications, we sought
to determine risk factors for UCF and Glans
Dehiscence (GD) after TIP repair in a large cohort
69.2.3.3 Eligibility Criteria (Item 6)
of patients with hypospadias. We hypothesized that Eligibility conditions should encompass both
the severity of hypospadias and not the type of inclusion and exclusion criteria. The population
regional anesthesia used would explain the risk of (true denominator) from which the sample was
postoperative complications.
selected, method of recruitment, and follow-up
should also be included. Report on surgeons’
preferences (selection bias) regarding surgical
69.2.3 Method Section technique, preoperative testosterone stimulation,
catheter insertion, dressings, and prescription of
69.2.3.1 Study Design (Item 4) antibiotics should be clearly stated.
Authors should present the general study design Eligibility criteria example [8]:
early in the article. In the first sentence of the Meatal location was defined as distal (glanular,
methods section, it should be stated whether the coronal and distal penile), midshaft or proximal
study is a retrospective chart review, a retrospec- (proximal penile, penoscrotal) as illustrated in the
tive analysis of a prospective database, a cross- diagram. All patients with scrotal and perineal
hypospadias underwent staged repairs and, thus,
sectional study, a survey, a case-control study, or were effectively excluded from study. For analysis
a prospective study. Indicate the study’s primary purposes, midshaft and proximal cases were
outcome, followed by secondary endpoints. grouped together as these defects are considered
Study Design example [8]: more severe than distal ones. Ventral Curvature
(VC) was segregated at a cutoff of above and
After obtaining Research Ethics Board approval, below 30 degrees.
we conducted a retrospective review of 849 Our regimen for Preoperative Testosterone
patients who underwent hypospadias repair by 2 Stimulation (PTS) included the administration of
pediatric urologists between 2004 and 2015, at a 2 mg/kg Depo-Testosterone (testosterone cypio-
single tertiary children’s hospital. nate) intramuscularly 3 weeks apart to a maximum
The primary outcome of interest was the rate of of 3 doses in all boys with a glans width of less
post-operative complications. For the purpose of than 14 mm from 2009 and thereafter. The interval
this study, the only complications recorded were between the last testosterone dose and surgery was
urethro-cutaneous fistula and glans dehiscence. 4 weeks.
69 Systematic Steps on How to Write a Scientific Paper on Hypospadias 923
Patient selection for the type of regional anesthesia 69.2.3.4 Variables (Item 7)
administered was entirely based on anesthesiolo-
gist discretion after discussion with the parents
All outcome measures, interventions, risk fac-
during the preoperative appointment. The patient tors, predictors, potential confounders, effect
received general anesthesia supplemented by DPB modifiers, and other applicable variables such as
or CB. DPB was performed according to a certain ventral curvature should be clearly defined, high-
technique. The penis was pulled forward with gen-
tle traction to open the plane between the corpora
lighting how each one was measured, by whom,
and the symphysis pubis. A 25 gauge needle was and if any validated or non-validated scales were
inserted under the pubic bone and parallel to the employed. A clear description of the position of
penis, and 0.25% bupivacaine without epinephrine the urethral meatus with or without diagram and
was injected above Buck’s fascia. No ring block
was performed.
the severity of ventral curvature is required, as
All patients underwent TIP urethroplasty accord- shown in the previous example [8]. Explicit
ing to previously published techniques with no description of variables allows the reader to bet-
tourniquet or injection of epinephrine. Briefly, a ter judge the author’s aims and determine appli-
ventral U-shaped skin incision 1–2 mm proximal
to the urethral meatus was created, extending distal
cability of the study to other populations.
into the glans to preserve the urethral plate. For Variables example [10]:
proximal hypospadias, the incision lines were Information regarding recurrent ventral curvature
made adjacent to the corpus spongiosum, follow- and erections was obtained from patient charts.
ing the entire extent of the longer urethral plate. Ventral curvature was assessed by direct history
Tethering tissues lateral to both, the spongiosum via children or parent reports and physical exami-
and the urethral plate were excised as initial steps nation, which was only possible if the child had a
to correct VC. reflex erection during inspection. Straight erec-
The penis was degloved down to the penoscrotal tions were documented by parent reports, patient
junction and into the bulbar urethra in cases of self-assessment or physician observation during
more severe VC. Artificial erection was performed physical examination.
to assess the degree of residual VC which, if pres-
ent, was corrected by dorsal plication with a small
incision in the tunica albuginea at the 12 o’clock 69.2.3.5 Data Sources/Management
position using a 5-zero polypropylene stitch. A
midline relaxing incision was made in the urethral
(Item 8)
plate extending from the glans groove to the most Describe how each variable was recorded, the
proximal point of the plate. The urethral plate was source of data, and how the data were collected.
then tubularized in 2 layers (first layer subepithe- This may include assessment of glans width
lial and running, and second layer subepithelial
and interrupted) over an 8 Fr catheter using 7-zero
(measured with caliper at the widest part), ure-
polydioxanone. Spongioplasty was performed in thral plate width (measured with ruler by stretch-
all cases. The neourethra was covered by a single ing the plate, points where plate was measured
dartos flap layer in lateral or buttonhole fashion. should be described), ventral curvature (measured
Glansplasty was performed with a minimum of 3
subepithelial interrupted 6-zero polydioxanone
with caliper or subjectively by visual inspection,
stitches. Tension-free glans wing approximation utilizing erection test with or without tourniquet),
was achieved after performing extensive glans skin characteristics (asymmetry, torque, peno-
wing mobilization. Midline skin closure was done scrotal transposition), penile length (from the
with interrupted 7-zero epithelial polydioxanone
stitches.
pubic bone to the tip), associated anomalies (such
A urethral catheter was left in place for 7 days in as cryptorchidism), and others. When including
patients with distal defects and 10 to 12 days in specific measurements, it is recommended to have
those with proximal defects. A compressive a photograph or sketch demonstrating how the
dressing (Tegaderm® and Coban® tape) was
applied on the penis for 48 hours. All patients
measurements were taken. The number of peri- or
received antibiotic prophylaxis with trime- postpubertal subjects should be listed, and if pres-
thoprim until catheter removal, anticholinergics ent, sexual function outcomes should be defined
as needed for bladder spasms and morphine for and reported. Detailed measurement of variables
pain control.
924 L. H. Braga
is important as it can affect study validity. The visit). It is very important to clearly indicate who
source of the data and measurement method uti- assessed outcomes, particularly postoperative
lized must be clearly stated to the extent that read- results, and how these outcomes were assessed.
ers could reproduce the results. Diagrams, figures, Vague descriptions, such as “good cosmetic and
or photographs may be a useful way to represent functional result,” are commonly seen in retro-
this information without compromising the word spective chart reviews but should be avoided, as
count of the manuscript. Standardization of mea- their lack of detail does not allow for in-depth
surement methods increases internal validity of analysis of the complications.
the study. On the other hand, a flawed measure- Bias introduces systematic error into a study’s
ment method can introduce systematic error design; thus, biased studies generate results that
(bias), which may invalidate the study results. The are not representative of the truth. Authors should
authors should describe attempts to validate mea- identify potential sources of bias and adjust
sures or adjust analyses for errors whenever appropriately. Transparency is always better than
possible. trying to convey perfection, particularly in this
Data sources/management example [9]: section.
The primary study outcome was DC as measured Bias example [11]:
by the Decisional Conflict Scale 7–9. This open We utilized digital photography and sequentially
access, validated scale measures personal percep- projected 19 digital photographs depicting Urethral
tions of effective decision making and uncertainty Plate (UP)s in children with different degrees of
in choosing options. The scale has been exten- hypospadias. The number of cases was calculated
sively studied with well described properties, based on the maximum that could be projected in
including reliability, test-retest correlations, con- the allotted time. Photographs were chosen from a
struct validity, discrimination and responsiveness. single-institution prospective hypospadias data-
The scale consists of 16 statements and 5 response base, and were selected to include defects with
categories (www.ohri.ca/decisionaid), with out- variable meatal location (8 distal, 6 midshaft and 5
puts adjusted following the recommendation of the proximal). By protocol, all pictures were taken
author, and ranging between 0 (no DC) and 100 immediately before surgery, with the patient anes-
(extremely high DC). For study purposes DC was thetized, and prior to skin marking or surgical inci-
considered to be present when the total DCS score sion. A metric scale ruler was consistently included
was greater than 25 (a score of less than 25 was in all photographs to offer a standardized reference
considered low, as previously related with imple- to dimension, particularly in regard to UP width. It
menting decisions, 25 to 37.5 was intermediate and was placed in a transverse orientation close to UP
greater than 37.5 was high). A score greater than but without obscuring it. All photographs were
37.5 was considered particularly important as it captured under similar light conditions, by a single
has been associated with de-laying decisions and researcher, using a Sonyâ Cyber-shot 7.2 Mega
feeling unsure about implementing them in previ- Pixel digital camera.
ous publications.
69.2.3.7 Study Size (Item 10)

69.2.3.6 Bias (Item 9) Describe how sample size was determined and
Acknowledge and explain how potential sources report any calculations done to reach that number
of bias were addressed. Common possible biases (along with the information to support the
include selection bias, such as mild types of employed numbers). In addition, the timing of the
hypospadias being selected and overrepresented estimate calculation should be disclosed, if a pri-
in the dataset, excluding more severe defects; ori (reflecting a goal figure to reach before pro-
measurement bias (measurement error) or ascer- ceeding with analyses) or a posteriori (aimed at
tainment bias, which occurs when an outcome checking power and adequacy of the available
adjudicator is not blinded to the intervention (sur- data to answer a specific question). To detect a
geon assessing his/her own patients’ hypospadias statistically significant difference between groups,
cosmetic results); and attrition bias, when a sub- the sample size of the study must be sufficiently
stantial loss to follow-up occurs (more than large and the study properly powered. The sample
30–40% of patients do not return for a follow-up size usually depends on the difference of event
rates between the two surgical interventions under We tested 4 logistic regression models and found
that the model including stenting, dartos coverage
investigation. Other factors that may influence and stenting*dartos (checking for interaction) was
sample size should also be mentioned, such as a the most accurate and fitted data the best. Results
limited availability of patients. were analyzed in terms of coefficient and Exp-
Study size example [9]: (OR) with lower and upper 95% confidence inter-
vals [13].
Accepting as a meaningful difference an effect size
of 0.4, following a paired analysis aimed at 80%
power and 5% significance level, we defined a pri- 69.2.3.10 Subgroup Analysis
ori a sample size of at least 100 couples as Subgroup analysis involves searching for rela-
sufficient.
tionships in a subset of study participants.
Subgroup analysis involves searching for rela-
69.2.3.8 tatistical Methods (Item
S tionships in a subset of study participants.
11 a–d) Reporting should include a description of all such
Authors should describe all statistical methods analyses, particularly identifying those that were
employed, paying attention to provide a concise planned a priori or post hoc. It is important to
description of techniques that are not commonly remember that multiple subgroup analyses “look-
used or novel (along with appropriate references). ing for significance” are bound to disclose a spuri-
Thorough understanding of confounding, bias, ously significant association, a fact that highlights
and threats to internal or external validity should the need for analyses to be based on a well-defined
be achieved before study initiation, as statistics hypothesis, and the common overreliance on p
are not meant to correct for inadequacies in study values as a marker for clinical significance.
design or unaccounted biases. Statistical strategy Example of subgroup analysis [8]:
should be ideally planned before data acquisition In addition, we performed a pre-planned subgroup
and analysis. analysis including only distal hypospadias patients
to address the potential selection bias involving CB
69.2.3.9 Control for Confounding preference for proximal cases.
Confounding will obscure the true relationship
of interest in the study and threaten the study 69.2.3.11 Handling Missing Data
validity. Determining potential confounders and Missing data can bias a study and increase both
the optimal method of dealing with the con- type I and type II errors. Authors should detect
founders during study design, before collecting and evaluate the characteristics of missing data
data, is strongly encouraged. The main strategies and determine whether they are missing at ran-
used to control for confounding such as stratifi- dom or not (i.e., systematically missing a particu-
cation and regression should be reported, as lar variable of the dataset). Appropriate methods
demonstrated in the example below. Fistula rate should be utilized to handle the missing data and
between those who had dartos coverage and no be reported in detail. Such methods may include
dartos was stratified according to placement of likelihood-based approaches, weighted estima-
urethral catheter. tion, and imputation analyses.
Control for confounding example: Handling missing data example [9]:
We then used the Mantel-Haenszel test to perform These strategies were reproduced after linear
a comparative analysis between dartos flap cover- imputation techniques to adjust for missing infor-
age in regard to fistula formation, taking into con- mation on dropout partners or couples.
sideration the placement (or not) of a urethral stent.
Following the Mantel-Haenszel method the
adjusted Outcome Results (ORs) of stented and 69.2.3.12 Sensitivity Analyses
non-stented cases with dartos flap coverage were Sensitivity analyses should be performed when-
compared to determine if an effect modification or ever clinically appropriate, disclosing the basis
interaction was indeed taking place between those for the introduced variation and on which vari-
2 variables [12].
ables, and determined a priori.
926 L. H. Braga
69.2.4 Result Section A proper flow diagram should include the

number of participants at each stage of the
69.2.4.1 Participants (Item 12 a–c) study (eligibility, recruitment, enrollment, fol-
low-up, and analysis) and give reasons for non-
Numbers of Participants at Each Stage participation, including ineligibility, loss to
State the number of subjects included in each follow-up, or otherwise. In a retrospective chart
stage of the study. This should include the num- review, it can highlight the number of patients
ber of patients initially screened for eligibility, screened, the selection process, and the ulti-
those deemed eligible, those enrolled in the study, mate group of records evaluated in detail to
those who completed the study, and those generate data.
included in the analyses.
Example [8]: 69.2.4.2 escriptive Data (Item
D
After obtaining research ethics board approval we 13a,b)
retrospectively reviewed the records of 849
patients who underwent hypospadias repair per-
Study Participants
formed by 2 pediatric urologists between 2004 and
2015 at a single tertiary children’s hospital. We Baseline characteristics of study participants,
excluded from the study 13 patients with incom- including age, severity of the condition, and other
plete medical records, 2 with reoperations and relevant variables, should be clearly stated or pre-
316 in whom repairs were done using techniques
sented in a table, allowing readers to easily com-
other than TIP. Ultimately, 518 patients were
included in the analysis. pare study population characteristics with their
own. Relevant to hypospadias repair, the location
of the meatus, presence of curvature, and previ-
Reasons for Nonparticipation ous surgical interventions for the condition are
Clearly state why participants were excluded at universally expected.
each phase of the study.
Example [9]: Missing Data
Following approval by our institutional research Provide the number of subjects with missing
ethics board, we prospectively offered participa- data for every variable measured. Item 11(c)
tion to families who were serially referred to a
details the issues that arise because of missing
single major pediatric hospital for evaluation of
distal hypospadias in their sons. A total of 27 fami- data.
lies declined to participate without voluntarily cit-
ing particular reasons for doing so. Exclusion
criteria consisted of presentation for surgery past 69.2.4.3 Outcome Data (Item 14)
the age of parental informed permission (i.e. able Report descriptive data regarding the study pop-
to participate in the decision-making process, 14 ulation including the number of outcome events
patients), a history of genitourinary surgical proce-
or summary measures. It is important to present
dures (0), and lack of both parents being present at
counseling and consent for surgery (17). Thus, percentages along with a breakdown (numerator
among 158 eligible patients seen during a 1-year and denominator) and limit mean and standard
period we enrolled and obtained consent and col- deviation to data that follow a normal
lected data from a cohort of 100 couples.
distribution.
Flow Diagram 69.2.4.4 Main Results (Item 15)

Use a flow diagram to display information effi-
ciently in an easy-to-read format. A flow diagram Unadjusted and Adjusted Estimates
allows readers to easily see the inclusion and Report both adjusted and unadjusted estimates of
exclusion of participants in each phase of the your main results. Explain which confounders
study and determine potential sources of bias. were included or excluded and why.
69.2.5 Discussion Section tions, our study results must be interpreted in the
context of its limitations. First, being a retrospec-
tive review, it was not possible to collect data on all
69.2.5.1 Key Results (Item 16) potential confounding factors, including intraoper-
Describe the main study results making note of ative factors that may have affected the complica-
original goals and objectives of the study. tion rate, such as glans width, urethral plate quality
and length, as well as slight differences in surgical
Reiteration of study objectives allows the authors technique between surgeons. We attempted to
to discuss whether key results of the study sup- reduce the impact of these factors by including sur-
port their original hypotheses. This can then be geon effect as a variable in our multivariable model,
followed by a pertinent comparison of the results which was constructed to have an outcome/variable
ratio of 7/1. Second, as this is a single center experi-
with available evidence, contrasting the study’s ence including patients treated by only two sur-
findings with previous publications. geons, it has limited generalizability to other
Key results example [8]: institutions. We have included boys with all types
of hypospadias defects in order to allow for repro-
Our single-center retrospective review suggests ducibility of our findings, as we expect that other
that hypospadias severity, analyzed by grouping pediatric urologists see similar type of patients in
meatal location and presence of VC into a single their clinics. Finally, due to differences in practice
variable, is the most important independent risk and missing data, follow-up of patients seen earlier
factor for the development of UCF and GD post- in this series may have been dissimilar to that of
TIP repair, as demonstrated by others. This finding children monitored more recently. It is important to
corroborates our hypothesis that the risk of surgi- note, however that our mean follow-up time was
cal complications is more likely driven by the com- 13.4 months, which was long enough (minimum 6
plexity of the hypospadias rather than the type of months) to determine the vast majority of post-
regional block used. operative complications, as shown by recent stud-
In contrast, some authors have suggested that the ies. Despite these limitations, a post-hoc analysis
type of regional block administered was signifi- revealed that the current study was powered at 82%,
cantly associated with higher complication rates. indicating that our results were robust and valid and
Routh et al. examined 396 cases and found that unlikely to be due to play of chance.
severity of the hypospadias and the type of regional
anesthesia used were independent risk factors for
higher post-operative complications. Univariate
analysis revealed that operative time and surgeon
experience were associated with a greater likeli- 69.2.6 Other Information
hood of UCF.
69.2.6.1 Generalizability (Item 18)
Indicate the extent to which study results are gen-
69.2.5.2 Limitations (Item 17)
eralizable to the greater population. State the dif-
Acknowledge possible sources of bias, inaccura-
ferences and the similarities between the sample
cies, and other limitations of the study. Each ele-
population and the target population. This will
ment of the study design and the type of potential
allow readers to determine the applicability of the
bias should be described and analyzed. The mag-
results to their own patient population.
nitude and direction of bias should also be dis-
Example [14]:
closed, specifically including possible over- or
underestimates of the main results. Attempts to As with any review, clinical heterogeneity of the
studies due to different geographic locations as well
minimize bias or confounding should also be
as singular demographic factors offered some limi-
described. If possible, explain how the results of tations to the comparability of the data. Furthermore,
the study maintain relevance despite acknowl- confounders were not always accessible due to
edged important shortcomings. ambiguity in reporting among authors, who com-
bined distal as well as proximal hypospadias and
Example [8]:
primary and redo cases. Both study variability and
Although we have provided evidence against the confounding factors may have affected the validity
association between CB and surgical complica- and therefore the generalizability of the study.
928 L. H. Braga
69.2.6.2 Funding (Item 19) 3. Borawski KM, Norris RD, Fesperman SF, Vieweg
J, Preminger GM, Dahm P. Levels of evidence in
The funding source and the role of the funders for the urological literature. J Urol. 2007; https://doi.
the present study should be clearly disclosed. If org/10.1016/j.juro.2007.05.150.
applicable, also give funding information for 4. Moher D, Altman DG, Schulz KF, Elbourne
another original study on which the current study DR. Opportunities and challenges for improving the
quality of reporting clinical research: CONSORT
was based. The conclusions and interpretation of and beyond. CMAJ. 2004; https://doi.org/10.1503/
a study can be influenced by the source of finan- cmaj.1040031.
cial support; thus all funding must be clearly 5. Tseng TY, Dahm P, Poolman RW, Preminger GM,
stated, regardless of source. This allows the Canales BJ, Montori VM. How to use a systematic
literature review and meta-analysis. J Urol. 2008;
reader to determine the likelihood of a conflict of https://doi.org/10.1016/j.juro.2008.06.046.
interest or possible influence as a result of the 6. von Elm E, Altman DG, Egger M, Pocock SJ,
funding source. Gøtzsche PC, Vandenbroucke JP. The Strengthening
Funding example [2]: the Reporting of Observational Studies in
Epidemiology (STROBE) statement: guidelines for
The International Pediatric Urology Task Force reporting observational studies. Lancet. 2007; https://
on Hypospadias Meeting was made possible by doi.org/10.1016/S0140-6736(07)61602-X.
a $25,000 Planning Grant from the Canadian 7. Braga LHP, Pippi Salle JL, Lorenzo AJ, Skeldon
Institutes of Health Research (CIHR) S, Dave S, Farhat WA, Khoury AE, Bagli
(#291275). DJ. Comparative analysis of tubularized incised plate
versus onlay island flap urethroplasty for penoscrotal
hypospadias. J Urol. 2007; https://doi.org/10.1016/j.
69.2.6.3 Summary of Findings Table juro.2007.05.170.
(Item 20) 8. Braga LH, Jegatheeswaran K, McGrath M,
Easterbrook B, Rickard M, DeMaria J, Lorenzo
In addition to the creation of this hypospadias- AJ. Cause and effect versus confounding—is there a
specific reporting guide, we recommended that true association between caudal blocks and tubular-
appropriate publications adopt the requirement of ized incised plate repair complications? J Urol. 2017;
a standardized summary of findings (SoF) table https://doi.org/10.1016/j.juro.2016.08.110.
9. Lorenzo AJ, Braga LHP, Zlateska B, Leslie B, Farhat
(Table 69.1) to effectively communicate key find- WA, Bägli DJ, Pippi Salle JL. Analysis of decisional
ings of all recommended items in a standardized conflict among parents who consent to hypospa-
format and to allow for quick comparison of dias repair: single institution prospective study of
studies during a systematic review. The use of a 100 couples. J Urol. 2012; https://doi.org/10.1016/j.
juro.2012.04.022.
standard SoF table will allow authors to present 10. Braga LHP, Pippi Salle JL, Dave S, Bagli DJ, Lorenzo
all relevant study data quickly and easily while AJ, Khoury AE. Outcome analysis of severe chordee
also providing readers the ability to identify per- correction using tunica vaginalis as a flap in boys
tinent information easily and consider a study’s with proximal hypospadias. J Urol. 2007; https://doi.
org/10.1016/j.juro.2007.03.166.
generalizability. The SoF table summarizes both 11. Braga LH, Lorenzo AJ, Suoub M, Bägli DJ. Is sta-
the study methodology and the results and should tistical significance sufficient? Importance of interac-
maintain the same PICOT format noted in the tion and confounding in hypospadias analysis. J Urol.
title and abstract. 2010; https://doi.org/10.1016/j.juro.2010.08.035.
12. El-Hout Y, Braga LHP, Pippi Salle JL, Moore K,
Bägli DJ, Lorenzo AJ. Assessment of urethral plate
appearance through digital photography: do pedi-
atric urologists agree in their visual impressions
References of the urethral plate in children with hypospadias?
1. Welk B, Afshar K, MacNeily AE. Randomized jpurol.2009.09.003.
controlled trials in pediatric urology: room for 13. Weber BA, Braga LHP, Patel P, Pippi Salle JL, Bägli
improvement. J Urol. 2006; https://doi.org/10.1016/ DJ, Khoury AE, Lorenzo AJ. Impact of penile deglov-
S0022-5347(06)00560-X. ing and proximal ventral dissection on curvature cor-
2. Braga LH, Lorenzo AJ, Bagli DJ, Pippi Salle JL, rection in children with proximal hypospadias. J Can
Caldamone A. Application of the STROBE state- Urol Assoc. 2014; https://doi.org/10.5489/cuaj.2337.
ment to the hypospadias literature: report of the 14. Braga LHP, Lorenzo AJ, Salle JLP. Tubularized
international pediatric urology task force on hypospa- incised plate urethroplasty for distal hypospadias:
dias. J Pediatr Urol. 2016; https://doi.org/10.1016/j. a literature review. Indian J Urol. 2008; https://doi.
jpurol.2016.05.048. org/10.4103/0970-1591.40619.
Hypospadias Centers, Training,
and Hypospadias Diploma 70
Johannes Wirmer and Ahmed T. Hadidi
Abbreviation Children with disfigured penis are reluctant to let

others see their penis, avoid sharing common
HIS Hypospadias International Society showers in school, avoid sports, and avoid having
girlfriends. The author had patients who under-
went more than 30 operations during the first
13 years of their lives. After having his problem
70.1 Centralization fixed with satisfactory cosmetic results, one child
in Hypospadias Surgery became top of his class and a champion in
swimming.
Despite being one of the most common congeni- As Twistington said in 1941:
tal malformation [1, 2], the complication rate in The fruits of success are beyond rubies - the grati-
hypospadias surgery remains high [3], reaching tude of a boy has to be experienced to be believed.
36% in distal and 56% in proximal hypospadias
[4, 5]. Several factors influence the outcome of The authors still receive patients in their 50s
hypospadias correction including the age of the and 60s after having families and children, yet
patient, the presence and severity of chordee, the they are not comfortable with their penises and
degree of the ventral hypoplasia, as well as the still want their penises fixed.
location of the meatus. However, the experience One paradox in hypospadias surgery is that:
of the surgeon plays a very important role.
The outcome of the hypospadias operation
“You need a lot of patients to become a
will have an important functional and psycho-
competent surgeon and only competent
logical impact on the quality of life of the person
surgeons get referred a lot of patients.”
that will last for the next 80–90 years (Chap. 68).
Therefore, centralization of hypospadias sur-

gery is essential to have high flow or “quantity”
to achieve high quality. In adults, numerous stud-
J. Wirmer (*) · A. T. Hadidi ies have shown the beneficial effect of centraliza-
Hypospadias Center and Pediatric Surgery tion with the number of complications decreasing
Department, Sana Klinikum Offenbach, Academic with an elevation in the number of treated patients
Teaching Hospital of the Johann Wolfgang Goethe [6, 7]. The situation is still not well defined in the
e-mail: hanno.wirmer@sana.de field of hypospadias: Hisamatsu et al. in a recent
930 J. Wirmer and A. T. Hadidi
as well showed, in an analysis based on a hospi-

tal consortium database for more than 5000
hypospadias repairs at 47 hospitals in the United
States, how the risk of fistula, stricture, or diver-
ticulum decreases with high surgeon volume
[4].
An even bigger study by Wilkinson et al. eval-
uated more than 23,000 operations at 75 centers
in England over a time period of 11 years, show-
ing significantly lower complication rates from
high volume centers compared to low volume
Fig. 70.1 The learning curve of one-stage proximal centers (17.5% vs. 25%, p = 0.01) [12].
hypospadias (reprinted from Journal of Pediatric Urology, Various countries have implemented mini-
published January 2021, Hisamatsu E. et al.: “The learn-
ing curve in proximal hypospadias repair,” ©2021, with
mum volume standards for highly specialized
permission from Elsevier [8]) medical procedures [13]. In Germany, the
Federal Joint Committee (G-BA) determines
minimum volume thresholds for certain proce-
study reviewed 73 cases of proximal hypospadias dures (e.g., 50 cases of knee endoprosthesis a
repair by a single surgeon showing a declining of year or 25 cases of kidney transplantation) [14].
the complications over time with stabilization However, so far, no country has implemented
after 50 cases (Fig. 70.1) [8]. Deschênes-Rompré fixed rules on who or where hypospadias should
et al. (2013) evaluated the results of a pediatric be treated.
urologist after completing his fellowship in 2002
until 2011, doing 303 hypospadias repairs, show-
It is essential to limit hypospadias surgery
ing a steady learning curve as well, with stabili-
to hypospadias centers that perform more
zation after 50–75 cases [9].
than 50 hypospadias operations per year.
In an almost equal setting, Horowitz et al. col-
lected the data of 231 consecutive hypospadias
operations performed by one pediatric urologist
over a 5-year period after completing his This is the only way to reduce complication
fellowship, also showing a steady learning curve rates that reach 60% in proximal hypospadias and
with a statistically significant decline in the fis- to offer proper training and transfer experience.
tula rate in each year of observation [10]. Manzoni et al. expressed similar view [15]:
In the Warwickshire Experience (2007), the the modern surgical repair of hypospadias
direct effect of centralization of hypospadias requires an experienced and dedicated specialist.
repair is demonstrated [11]. Here, all hypospa- […] This is not surgery for the occasional opera-
dias in the county of Warwickshire in the time tor, therefore a volume of at least 40–50 cases per
from July 2000 to August 2004 were sent to a year is desirable.
single surgeon, whereas prior to July 2000, all Some medical health insurance companies
hypospadias repairs were performed by more now in Germany insist on having hypospadias
than one surgeon using a number of techniques. operations in high volume centers to reduce
Comparing the 4 years after July 2000 with 70 costs. Centralization of hypospadias service will
treated patients to the preceding 5 years with 67 not only improve outcome, reduce complica-
treated patients, fistula occurrence was reduced tions, offer adequate training for residents, and
from 35.8 to 6.7% and urethral stricture rates standardize postoperative care but will also
decreased from 4.4 to 0% [11]. reduce operative time and hospital costs, thus
While these studies all have been from single making a major impact on financial resources in
center series with selected surgeons, Lee et al. any health system.
70 Hypospadias Centers, Training, and Hypospadias Diploma 931
70.2 Training of the Hypospadias geon or pediatric urologist is unaware of compli-

Surgeon cations of his operations occurring after puberty.
Hypospadias surgery requires experience in three

different fields of surgery: pediatric surgery, urol- 70.3 Hypospadias Diploma
ogy, and plastic surgeons (Fig. 70.2). Famous
world pioneers that influenced the progress in Hypospadias is a very wide spectrum. The com-
hypospadias originated from all three fields, for petent hypospadias surgeon must have an exten-
example, Horton, Devine, Duckett, Bracka, sive and deep knowledge about all aspects of
Cloutier, Mustardé, Perovic, and many others. hypospadias. This includes: the development and
The author has spent a year training in a pure embryology of the penis, surgical anatomy and
plastic surgery hospital. This year was the most blood and nerve supply, the morphology and
important year in his surgical career as he learnt pathogenesis of hypospadias, the epidemiology
the basics of tissue handling, sound principles of and genetic basis, the principles of plastic sur-
elevating flaps and raising grafts, as well as fac- gery, flaps and grafts, the principles of all tech-
tors that influence wound healing and postopera- niques described for hypospadias correction, and
tive care. all possible complications and how to correct
Therefore, the hypospadias surgeon may orig- them. He should also know several techniques for
inate from any of the three surgical disciplines: all the four grades of hypospadias in depth and all
pediatric surgery, urology, or plastic surgery. related tips and tricks to choose the most suitable
However, he should rotate and gain experience in technique for each single patient.
the other two disciplines. Also, it is important There is no place for the amateur surgeon who
that the hypospadias surgeon should help and is satisfied to handle mild forms of hypospadias
treat hypospadias problems regardless of age. It and will send difficult or complicated forms to a
is not appropriate that the treating pediatric sur- specialized center. Each one of us has encoun-
tered several situations where what seems mild
turns to be very complicated with severe curva-
ture and hypoplastic tissues that require good
experience and deep understanding of
hypospadias.
The Hypospadias Diploma should consist of
theoretical, clinical, and operative parts. The
theoretical part should cover everything related
to the penis from embryology, anatomy, mor-
phology, and plastic surgery principles to surgi-
cal techniques and complications and their
management. For the clinical part, the candidate
should be exposed to about 100 patients at dif-
ferent ages with different forms of hypospadias.
To fulfill the operative part, the candidate should
attend, assist, and be assisted under supervision
in about 100 cases. A written and oral exam
should assess the knowledge, surgical experi-
ence, and judgment of the candidate before being
awarded the certificate of completion of training
Fig. 70.2 Hypospadias surgeons may originate from any
and the title of a Hypospadias Surgeon. This
of the three basic surgical disciplines: pediatric surgery, book may be used as a basis for theoretical
urology, and plastic surgery (courtesy of Ahmed T. Hadidi) knowledge in hypospadias. The HIS website
932 J. Wirmer and A. T. Hadidi
2. Bergman JEH, Loane M, Vrijheid M, et al.

Epidemiology of hypospadias in Europe: a registry-
based study. World J Urol. 2015; https://doi.
org/10.1007/s00345-015-1507-6.
3. Pfistermuller KLM, McArdle AJ, Cuckow PM. Meta-
analysis of complication rates of the tubularized
incised plate (TIP) repair. J Pediatr Urol. 2015; https://
doi.org/10.1016/j.jpurol.2014.12.006.
4. Lee OT, Durbin-Johnson B, Kurzrock EA. Predictors
of secondary surgery after hypospadias repair: a pop-
ulation based analysis of 5,000 patients. J Urol. 2013;
https://doi.org/10.1016/j.juro.2013.01.091.
5. Long CJ, Chu DI, Tenney RW, Morris AR, Weiss
DA, Shukla AR, Srinivasan AK, Zderic SA, Kolon
TF, Canning DA. Intermediate-term follow-up of
Fig. 70.3 The Hypospadias International Society (HIS) proximal hypospadias repair reveals high compli-
website offers lectures, panel discussions, live operations cation rate. J Urol. 2017; https://doi.org/10.1016/j.
and debates about all aspects of hypospadias (https:// juro.2016.11.054.
www.hypospadias-society.org) (courtesy of Ahmed 6. Merrill AL, Jha AK, Dimick JB. Clinical effect of
T. Hadidi) surgical volume. N Engl J Med. 2016; https://doi.
org/10.1056/nejmclde1513948.
7. Urbach DR. Pledging to eliminate low-volume sur-
gery. N Engl J Med. 2015; https://doi.org/10.1056/
offers valuable lectures, live operations, and nejmp1508472.
operative videos that may help prepare the can- 8. Hisamatsu E, Sugita Y, Haruna A, Shibata R, Yoshino
didate for the Hypospadias Diploma. K. The learning curve in proximal hypospadias
repair. J Pediatr Urol. 2021; https://doi.org/10.1016/j.
One important aim of the Hypospadias jpurol.2021.01.005.
International Society (HIS) (Fig. 70.3) is to 9. Deschênes-Rompré M-P, Nadeau G, Moore K, Ajjaouj
establish international curriculum, international Y, Braga LH, Bolduc S. Learning curve for TIP ure-
classification, and standard international training throplasty: a single-surgeon experience. Can Urol
Assoc J. 2013; https://doi.org/10.5489/cuaj.1376.
in hypospadias and to recognize well-established 10. Horowitz M, Salzhauer E. The “learning curve”
hypospadias centers in each and every country in in hypospadias surgery. BJU Int. 2006; https://doi.
the world. However, it remains important to rec- org/10.1111/j.1464-410X.2006.06001.x.
ognize the diversities in hypospadias and to 11. Hardwicke J, Clarkson J, Park A. Centralisation of a
hypospadias repair service—the Warwickshire expe-
acknowledge that what works in one surgeon’s rience. J Plast Reconstr Aesthet Surg. 2007; https://
hand may not work with another surgeon. doi.org/10.1016/j.bjps.2006.02.004.
The Hypospadias International Society web- 12. Wilkinson DJ, Green PA, Beglinger S, Myers J,
site (https://www.hypospadias-society.org) has Hudson R, Edgar D, Kenny SE. Hypospadias sur-
gery in England: higher volume centres have lower
an educational part with lectures by world complication rates. J Pediatr Urol. 2017; https://doi.
pioneers and live surgical procedures performed org/10.1016/j.jpurol.2017.01.014.
by surgeons who originally described the com- 13. Morche J, Renner D, Pietsch B, Kaiser L, Brönneke
mon surgical techniques and live debates on dif- J, Gruber S, Matthias K. International comparison
of minimum volume standards for hospitals. Health
ferent controversial issues in hypospadias. The Policy (New York). 2018; https://doi.org/10.1016/j.
aim is to offer the interested surgeon the chance healthpol.2018.08.016.
to listen, watch, and educate himself. 14. Gemeinsamer Bundesausschuss Regelungen des
Gemeinsamen Bundesausschusses gemäß § 136b
Absatz 1 Satz 1 Nummer 2 SGB V für nach § 108
SGB V zu-gelassene Krankenhäuser(Mindestmengen
regelung, Mm-R) in der Fassung vom 20. Dezember
References 2005. Germany
15. Manzoni G, Bracka A, Palminteri E, Marrocco
1. Springer A, van den Heijkant M, Baumann G. Hypospadias surgery: when, what and
S. Worldwide prevalence of hypospadias. J Pediatr Urol. by whom? BJU Int. 2004; https://doi.
2016; https://doi.org/10.1016/j.jpurol.2015.12.002. org/10.1046/j.1464-410x.2004.05128.x.
Index
A Autologous cells, 907

Abdominal wall fixation, 218, 514 Axial pattern flaps, 332
Abnormally long inner prepuce, 672
Absent vacuolation, 138
Accessory scrotum, 716 B
Accessory urethra, 70, 141, 145 Balanic groove technique, 26, 653
Acquired megalourethra, 828 Balanic hypospadias, 30
Adalimumab, 819 Balanitis xerotica obliterans (BXO), 566, 589, 779, 813,
Adenoma after hypospadias repair, 722 835, 856
Adherence, 73 Barcat, J., 397
Adhesion, 77 Barcat modification, 380
Adolescence, 900 Barcat technique, 412
Advancement flaps, 11, 238, 330, 748 Bifid, 14
Aggressive glans mobilization, 471 Bifid scrotum, 154, 518, 711, 848
Albucasis, 6 Bifurcated corpus spongiosum, 147
5α reductase, 290 Bilateral anorchia, 157
Al-Tasreef, 6 Bilateral based flap (BILAB), 37, 46, 497
Ambiguous genitalia, 309 Bilobed flap, 331
Anaesthesia, 8 Bladder mucosa, 16, 53, 565, 740, 856
Anal folds, 68 Bladder mucosa grafts, 573–575, 778
Anal membrane, 75 Bladder spasms, 591
Androgen insensitivity syndromes, 291 Blood supply, 519
Androgens, 290 Bone morphogenetic proteins, 272
receptors, 65 Bouche de canon, 434
response elements, 291 Browne, D., 754
Angiogenesis, 325 Buccal graft applied, 885
Ankh-mahor at Saqqara, 4 Buccal mucosa, 17, 53, 859
Anogenital distance (AGD), 117 Buccal mucosal graft, 330, 569–572
Anoscrotal distance (ASD), 14, 155 Bucket-handle meatus, 384
Anterior, 66, 830–831 Buck’s fascia, 19, 106, 107, 192, 620
pituitary, 288 Bulb of the penis, 106
sagittal, 699 Bulbi spongiosi, 92
Anterior sagittal transrectal approach, 692 Bulbospongiosus muscle, 92, 109, 147
Anti-asthmatic, 253 Bulbourethral artery, 107, 109
Anti-erection medications, 730 Buried penis, 672
Anti-Mullerian hormone (AMH), 697 Byars flap, 892
Apoptosis, 73, 77
Arterial flaps, 332
Artificial erection, 17, 440 C
Artificial erection test, 202, 363, 414, 748, 865 Calibration, 241
Artificial matrices, 907 Canalization, 87, 642
Ascending urethrography, 792 Catheter blockage, 776
Asopa II, 523 Catheterless Mathieu repair, 758
Autograft, 327 Caudal migration, 314
© The Editor(s) (if applicable) and The Author(s), under exclusive license to Springer Nature 933
Switzerland AG 2022
A. T. Hadidi (ed.), Hypospadias Surgery, https://doi.org/10.1007/978-3-030-94248-9
934 Index
Caudal penoscrotal transposition, 707 COVER technique, 133–136

Caudal regression syndrome, 705 Crimped metal sleeves, 754
Caudal block, 891 Crippled hypospadias, 16–17
Cavernous artery, 109 Critical colonization, 327
Cavernous nerve, 116 Crown-rump length (CRL), 65
Cavernous sinuses, 108 Crura of the penis, 92
Cecil Culp Technique, 52 Cryptospadias, 137
Cellular biology, 907 Cultured epithelial and dermal grafts, 329
Central glanular canal, 88
Centralization in hypospadias surgery, 929–930
Central scrotalization, 706 D
Chordee, 3, 731 Dartos fascia, 19, 94, 107, 192
angle, 205–206 Dartos flap, 419, 861
release, 19 Dead Man Jacket Technique, 13, 643
tissue, 194–195 Decision regret, 314, 888, 899
with corporeal disproportion, 205 Deep arteries, 108
without hypospadias, 218–221 Deep chordee, 28, 205, 518
Chordee excision and distal urethroplasty (CEDU), 20, Deep dorsal artery, 109
37, 46, 497 Deep dorsal urogenital stroma, 92
Circular anastomosis, 778 Deep glanular groove, 88
Circumcision, 4 De-epithelialization, 55
Circumcision injury, 716 De-epithelialized flap, 404, 801
Cleft glans, 33, 138, 426, 640 De-epithelialized preputial skin, 383
Cloaca, 67 De-epithelialized skin, 54
Cloaca eminence, 68 Deep perineal septum, 92
Cloacal groove, 68, 69 Deep urethral groove, 85
Cloacal membrane, 67, 68 Degloved, 748
Cloacal orifice, 69 Deltopectoral flap, 332
Cloacal plate, 68 Denis Browne technique, 36
Cloutier, 16 Dermabrasion, 599
Coagulation, 324 Dermal graft, 516, 865
Cobra eyes, 132, 144 Dermis, 323
Collagen, 190 Dermolite, 762
Collagen subtype III, 197 Development of the prepuce, 605–606
Colles, 107 Diethylstilbestrol (DES), 253
Complete androgen insensitivity syndrome, 291 Differences, 898
Completely bifid scrotum, 154 Differential growth, 89
Complete penoscrotal transposition, 706 Digital photography, 691
Complex penile reconstruction, 593 Dihydrotestosterone, 290
Complication rate, 891 Dilatation, 782
Complications, 729 Disassembly technique, 229–230
Compound tube, 572 Disorders of sex development (DSD), 157, 503, 898
Concealed, 672 Disorganization hypothesis, 137, 163, 836
Concealing type of dressing, 762 Distal diverticulum, 828, 830–831
Congenital hypogonadotropic hypogonadism, 292 Distal hypospadias, 891
Congenital meatal cyst, 720 Distal ligament in the human glans penis, 111
Congenital megalourethra, 723–724 Distal migration, 87
Congenital mega prepuce (CMP), 669, 672 Distal mobilization, 30
Congenital urethrocutaneous fistula, 716 Distal stenosis, 828
Congenital urethrocutaneous fistula with Distal urethroplasty and glanuloplasty (DUG), 33
hypospadias, 716 Diverticulum, 505, 518
Contaminated wounds, 327 Dog ears, 144, 152
Coring, 642 Dorsal, 66
Cornua sacralis, 351 arteries, 109
Corona, 634 corporoplasty, 558
Coronal planes, 686 nerve of the penis, 115
Coronal sulcus, 84, 113, 128, 634 plication, 28, 504, 518
Corpora cavernosa, 18 preputial fascia, 836
Corporoplasty, 497, 557 relaxing, 753
Corpus spongiosum, 28, 146–147, 829 subcutaneous flap, 620, 802
Index 935
Dorsal inlay graft (DIG), 14 Fibrovascular zone, 91, 92

Dorsal inlay TIP (DTIP), 451 Fistula, 35, 799, 835
Dorsal vertical length (DVL), 111, 113, 638 Fistula genu, 805, 806
Double breasting, 394 Fistula rate, 930
Double-faced preputial island flap, 523 Flaccid penile length, 113
Double Y glanulomeatoplasty (DYG), 11, 31, 138, 361, Flat curve, 241
657–659 Flat glans, 138, 426, 640
Dressings, 14, 802 Flutamide, 290
Dribbling ejaculation, 901 Foreskin, 114, 310
Duplication of the urethra, 685 Foreskin reconstruction, 445–446
Duplications of the bladder, 688 Fossa navicularis, 84, 635
Duplications of the penis, 688 Free flap, 332
Dupuytren´s contracture, 195 Free-graft tubed repairs, 800
Duration of follow, 891 Free skin graft, 856
Dysgenetic gonad, 279 Frenulum, 83, 635
Dyspadias, 137 Full-thickness skin graft, 20, 328, 565, 566, 871
Functional urethral obstruction (FUO), 13, 791, 835
Fundiform ligament, 116
E Fusiform, 723
Early period, 3 Fusion theory, 77
Effmann classification, 686
Ehrlich and Scardino’s procedure, 707
Elliptical meatus, 372 G
Eminence, 68, 70, 91 Galen, 6
Emotional development, 305–306 Genetic, 250
Endoscopic techniques, 700 Genital Perception Score (GPS), 880
Enlarged prostatic utricle, 157 Genital tubercle, 68, 71
Environmental, 250 Glandular knobs, 453
Epidermal autografts, 908 Glans, 91
Epidermis, 323 Glans approximation procedure (GAP), 32, 412
Epidural analgesia, 352–353 Glans channel technique, 56, 523
Epithelialization, 325 Glans dehiscence, 503, 518, 591, 732, 866
Epithelial plate, 79 Glans penis, 299
Epithelial seam, 77 Glans plate, 68, 69
Epithelial tag, 70, 76, 89 Glans splitting, 57
Epithelio-mesenchymal interaction, 128 Glans width (GW), 111, 638
Erectile dysfunction, 882 Glans wing, 492
Erectile tissue, 638 Glanular, 11
Erection, 14, 730 groove, 79
Estrogen hypothesis, 250–251 tilt, 221, 380
Estrogen receptors, 881 Glanuloplasty, 372–374
Eutectic Mixture of Local Anesthetics (EMLA), 348 Glass beads, 20, 754
Extensive debridement, 804 Glenister, 78
External Genitalia Score (EGS), 238 Goniometer, 205
External Masculinization Score (EMS), 238 Gonorrhea, 192
External pudendal artery, 106 Grades of chordee, 204–205
External spermatic fascia, 620 Grafts, 16
External spermatic fascia flap, 54 biology, 566
External urethral meatus, 634 graft take failure, 590
Eye ball, 206 growth and puberty, 590
shrinkage, 566
tube urethroplasty, 572
F Greater cavernous nerve, 116
Fairy cuts, 223, 519 Groin, 848
Familial clustering, 250 Growth hormone, 291
Fascial stroma, 91, 92
Fascio-cutaneous flaps, 333
Female hypospadias, 726 H
Fertility, 886–887 Hairball, 883
Fetal testis, 289 Hair coil, 719–720
936 Index
Hairy urethra, 722–723 Infection, 20, 771

Heineke-Mikulicz meatoplasty, 412 Infertility, 702
Heineke-Mikulicz technique, 28, 223 Inflammatory exudate, 761
Hematoma, 775 Informed consent, 348
Hemiscrota, 154 Informed written consent, 314
Hemostasis, 340 Inguinal region, 848
Hepatitis B, 253 Inlay patch, 572
Hernia, 315 Instruments, 6, 337
Hidden, 672 Intact prepuce, 401
Hilum of the penis, 116 Intercavernous septum, 108, 193
Hinge, 433, 643 Internal pudendal artery, 107
Hinging of the urethral plate, 57, 397 Internal urethrotomy, 882
Hinging the distal urethral plate, 413 Interpolation flap, 332
Histology of the foreskin, 604–605 Intra corporeal prostaglandin, 886
Hodgson, N.B., 533 Intraurethral catheters, 802
Hodgson XX technique, 533 Intrauterine growth restriction, 252
Homograft, 328 Inverted Y SLAM technique, 654, 660–661
Hood, 127 Ischial spine, 115
Hooded foreskin, 130 Island flap, 332
Horse hair, 20 Isograft, 327
Hospitalization, 305
Human acellular dermis, 866
Human chorionic gonadotropin, 290, 299 K
Humby, G., 16 Keloid, 327
Hunchback (hump), 132 Keratinized squamous epithelium, 114
Hyperbaric oxygen (HBO), 837 Keratinocytes, 323, 907
Hyperbaric oxygen therapy (HBOT), 866 Kippering, 57
Hyperplasia, 77 Koebner phenomenon, 814
Hypertrophic scar, 327, 566 Koyanagi, T., 14
Hypertrophied, keloid-like scar, 754 Koyanagi technique, 497
Hypodermis, 324
Hypoplasia, 237
Hypoplasia of the penis, 152, 313 L
Hypoplastic, 519 Labioscrotal swellings, 68, 94–96
Hypoplastic corpus spongiosum, 498 Lacuna of Morgagni, 149
Hypoplastic triangle, 186 Lamina propria, 92, 635
Hypospadias, 6 Laparoscopic approach, 699
sexuality and, 899–900 Laryngeal spasm, 350
Hypospadias Assessment Score (HAS), 247 Lateral based flap, 497
Hypospadias cripple, 855, 884 Lateral based onlay flap (LABO), 32, 33, 792
Hypospadias Diploma, 931 Lateral chordee, 720
Hypospadias International Society, 238, 932 Lateral oblique flap, 45
Hypospadias Operative Checklist (HOC), 241 Learning curve, 930
Hypothalamus, 288 Leptin, 285, 294
Hypoxic brain damage, 348 Lesser cavernous nerve, 116
Leucoplakia, 813
Leydig cells, 290
I Lichen albus, 813
Iatrogenic hypospadias, 715 Lichen sclerosus, 885
Ilioinguinal nerve, 116 Littre´s gland, 110
Ill defined glans cleft, 590 Locker room teasing, 888
Imbibition, 566 Longitudinal dorsal island flap, 557
Immobilization, 761 Lymphatic drainage, 566
Immunohistochemical stains, 188 Lymphoedema of the penis, 722
Incision
and advancement, 782
and grafting, 783 M
of the urethral plate, 13, 778 Macrolide, 253
Incomplete cleft glans, 138, 426, 640 Magnification, 336, 801
Inconspicuous, 672 Male dyspareunia, 815
Index 937
Masculinization programming window, 290 Non-keratinized stratified squamous epithelium, 114

Mastermind-like domain containing 1, 278 Normal peak flow rate, 882
Mathieu, P., 837 Nove-Josserand, G., 565
Mathieu and V incision sutured (MAVIS) technique, 57,
383, 645
Mathieu technique, 35, 438 O
M configuration, 31 Oblique mushroom cap, 633
Meatal advancement, 11 One stage repair, 20, 537, 747
Meatal advancement and glanuloplasty incorporated Onlay island flap (OIF), 37, 739
(MAGPI), 11, 32, 138, 642, 799 Onlay patch, 572
Meatal-based flap, 14, 739 Open suprapubic cystostomy, 757
Meatal regression, 374 Open techniques, 702
Meatal retraction, 366 Opsite®, 762
Meatal stenosis, 16, 458, 732, 771, 777, 835 Optical magnification, 307, 336, 757
Meatal strictures, 884 Oral contraceptives, 251
Meatoplasty, 56 Oral mucosa, 740
Meatotomy, 380 Orthoplasty, 27
Meatus, 141 Os penis, 65
Median perineal raphe, 88, 94 Ovarian cysts, 253
Median raphe, 115, 152 Overweight, 251
Megameatus intact prepuce (MIP), 33, 138, 372
Melanocytes, 323
Mendelian Inheritance, 160 P
Merge, 91 Pants over vest, 831
Mesoderm, 78 Parameatal flap, 399
Mesonephric (Wolffian) ducts, 67, 101 Parameatal foreskin flap, 46
Metal pearls, 20 Parameatal skin, 372
Methodus Medendi, 4 Paramesonephric (Mullerian) ducts, 101
Micropenis, 152, 669 Parasympathetic nerves, 116
Microvascular instruments, 801 Partial androgen insensitivity syndrome, 291
Micturating cystourethrography, 315 Partial dissection of the epithelium of the urethral
Midline dorsal plication, 453 wall, 720
Migration hypothesis, 67, 89, 163, 836 Partially bifid, 154
Minimally invasive technique, 702 Partial penoscrotal transposition, 706
Minipuberty, 119, 291–292 Paternity, 886
M inverted V, 412 Patient consent, 606
Modern period, 4 Patient factors, 836
Mole in the glans, 719 Patient self perception, 247
Monk’s hood deformity, 144 Pectiniform septum, 108, 193
Morbidity, 759 Pedicled flaps, 739
Mothers’ curse, 287 Penile amputation, 599
Mucosal collar, 374 Penile block, 891
Mucosal prolapse, 591 Penile crus, 106
Müllerian duct remnant, 157 Penile curvature, 461
Mullerian structures, 697 Penile disassembly technique, 222, 471, 519
Mullerian system, 697 Penile length, 640
Multiple corporotomies, 223 Penile plate, 69
Multiple fistulas, 806 Penile resurfacing, 870–872
Multiple release incisions, 848 Penile size, 310, 899
Mutation analyses, 272 Penile torsion, 152, 677
Penile urethra, 76
Peninsular flap, 332
N Penopubic angle, 534
Narrow urethral plate, 498 Penoscrotal hypospadias, 9
Natural perineal fistula, 754 Penoscrotal transposition, 152, 518, 557, 705
Natural perineal urethrotomy, 341, 759–760 Penoscrotal transposition associated with bifid
Necrosis of the penis, 717 scrotum, 706
Neomeatus, 384 Penoscrotal web, 672
Nesbit procedure, 223 Percutaneous suprapubic cystostomy, 757
Nitroglycerine ointment, 837, 866 Perimeatal flap, 35
938 Index
Perineal approach, 699 Psychological outcomes, 887–888

Perineal body, 92 Pubic bone, 237
Perineal hypospadias, 14–16, 705 Pudendal canal (Alcock`s canal), 115
Perineal nerve, 115 Pudendal nerve, 115
Perineal septum, 94 Pyramid repair, 33
Perineal urethrostomy, 20, 757
Perineal urethrotomy, 341, 835
Persistent curvature, 880 R
Persistent obstructive voiding symptoms, 791 Random flap, 330, 870
Persistent chordee, 231–233, 837, 856 Rectangular flap, 38
Peyronie’s disease, 109, 206 Recurrent chordee, 231–233, 863–866
Pharmacological erection test, 202, 203 Recurrent fistula, 866
Phimosis after foreskin reconstruction, 618 Recurrent urethral fistula, 791
Physiological chordee, 164 Redo SLAM technique, 661–662
Placenta, 288 Redo surgery, 836
Placental estrogen, 290 Regional anaesthesia, 351–352
Platelet-rich fibrin membrane, 620 Remodeling, 326
Politzer, G., 678 Renaissance period, 4
Polydioxanone (PDS), 800 Re-operative, 593
Polymorphisms, 273 Rescue operation, 383
Poor ejaculation, 591 Residual chordee, 440, 884, 886
Positioning, 336 Residual hypospadias, 732
Post-auricular Wolffian graft (PAWG), 740 Residual severe chordee, 590
Post micturition drippling, 591 Retractile, 14
Post puberty complications, 884 Retrocoronal plexus, 116
Posterior, 66 Retrograde and antegrade urethrogram, 691
Posterior sagittal rectum retracting approach, 699 Retrusive meatus, 775
Posterior sagittal trans-anorectal, 699 Rhomboid rotation skin flaps, 870
Posterior urethral valves, 144 Robotic, 702
Postoperative erection, 774–775 Role of imaging, 315
Postoperative oedema, 761 Root of the penis, 105
Postvoid dribbling, 827 Rotation, 310
Practical surgery, 8 Rotation flap, 49, 330
Pregestational hypertension, 291 Running suture, 392
Prenatal ultrasound, 309
Pre-operative hormone therapy, 335, 837
Pre-operative investigation, 335 S
Prepuce, 881 Sagittal, 686
Prepuce reconstruction, 27 Scaffold, 910
Preputial dartos fascia, 366, 394 Scaphoid, 723
Preputial fascia, 519 Schnurnaht Technik, 8
Preputial inlay graft (G-TIP), 438 Scrotal drainage, 736
Preputial skin, 740 Scrotal skin, 49
Prevalence, 249 Scrotal skin flap, 870
Primates, 114 Scrotoplasty, 560
Progesterone receptors, 881 Scrotum, 49
Progressive augmentation by dilating the urethra Semen analysis, 886
anterior, 692 Septum glandis, 635
Proliferation, 73 Sertoli cells, 290
Prostatic colliculus, 157 Severe chordee, 206
Prostatic plexuses, 116 Severe hypospadias, 503
Prostatic urethra, 110 Sex-determining region Y gene (SRY), 285, 286
Protective intermediate layer, 3, 792 Sex hormone binding globulins (SHBG), 294
Protective layers, 730 Sexual development, 306
Proximal diverticulum, 828, 829 Sexual differentiation, 285
Proximal hypospadias, 21, 892 Sexuality, 900
Proximal thin urethra, 720 Sexual maturation, 900
Proximal urethra, 78 Side effects, 300
Psychological factors, 305, 897 Significant chordee, 206
Psychological impact, 894, 929 Silicone foam elastomer, 344
Index 939
Silicon foam, 26 Sympathetic nerves, 116

Silver wire, 20 Syndrome, 14
Sinus, 799
Sinusoidal trabeculae, 108
Skin, 107, 323–324 T
chordee, 411 Tail gut, 67
cover, 848–853 Tanner classification, 120, 294–295
de-epithelialization, 620 Tanner stages, 120
flap loss, 773 Tegaderm, 374
graft survival, 329–330 Tela subfascialis, 107
ischemia, 866–870 Terminal meatus, 881
Sliding skin flaps, 558 Testicular dysgenesis syndrome, 900
Slit-like adjusted Mathieu (SLAM) technique, 35, 655, Testosterone, 285
792, 841 Thick pubic hair growth, 300
Slit-like meatus, 14, 361, 380, 643–645 Thiersch, K., 411
Small flat glans, 479 Thiersch-Duplay urethroplasty, 35
Small intestinal submucosa (SIS), 224, 498 Thin urethra, 366
Small penis, 452 Thyroid disorders, 253
Small prepuce (foreskin), 590 Tiemann silicon catheter, 342
Small size glans, 590 Tie-over, 586
Smegma, 113, 128 Tight glanular closure, 778
Smegma mass, 725–726 “Tin” catheter, 6
Snub-nosed appearance, 380 Tissue engineering, 907
Solid glanular plate, 87, 88 Tissue expanders, 848
Solid urethral plate, 85 Tissue expansion, 870–871
Split and roll technique, 28, 222, 231 Tissue ischemia, 855
Split skin grafts, 328 Tissue sealants, 339–340
Split-thickness, 565 Total mobilization of the urethra, 230
Split-thickness free grafts, 20 Tourniquet, 384
Spongiofibrosis, 447 Training, 931
Spongioplasty, 147 Transitional epithelium, 110
Spongy urethra, 92 Transposition flaps, 331
Spraying, 732 Transverse incisions into the outer tunica albuginea
Squamous cell carcinoma after hypospadias (TITA), 223
repair, 722 Transvesical transtrigonal approach, 698
Staged tubularized autograft (STAG), 223 Trapped penis, 672
Stainless steel, 20 Triangular flaps, 331
Standardized photographs, 880 Triangular glanular flap, 380
Stenosis, 16, 374 Triangularized glans, 11
Stent duration, 891 Triple breasting, 620
Stone retention, 702 True rotation flaps, 331
STraighten And Close (STAC), 223 Tubularization, 11
Streeter Horizon Series, 65 Tubularized incised plate (TIP) techniques, 438, 791, 891
Strengthening the Reporting of Observational Studies in Tubularized incised urethroplasty, 451
Epidemiology (STROBE), 919 Tumors in undiagnosed PU, 702
Stretched penile lengths, 120 Tunica albuginea, 92, 107, 192, 830
Stricture, 16, 732, 885–886 Tunica albuginea externa, 107
Subcoronal circumcision, 472 Tunica albuginea externa excision (TALE), 28, 213–218,
Subcuticular continuous suture, 338 498, 512
Superficial chordee, 27, 28, 205 Tunica albuginea plication (TAP), 223
Superficial dorsal urogenital stroma, 94 Tunica vaginalis, 861
Superficial dorsal vessels, 523 Tunica vaginalis blanket wrap, 383
Superficial perineal septum, 94 Tunica vaginalis fascia, 836
Supplemental testosterone, 891 Tunica vaginalis flap (TVF), 213–218, 516
Suprapubic catheter, 498, 836, 861 Tunica vaginalis free grafts, 863
Suprapubic cystostomy, 341 Tunica vaginalis wrap, 620, 802
Surgical anatomy of the prepuce, 603–604 Tunneling, 6, 642
Suspensory ligament proper, 116 Two stage buccal mucosal, 848
Suture materials, 20 Two stage procedure, 859
Suture tracks, 724–725 Two-stage repair, 35, 800
940 Index
Two suture lines, 9 Ventral, 66

Two triangular flaps, 467 corporal grafting, 882
Tyson’s glands, 113 corporotomies, 519
curvature, 17
degloving, 212–213
U skin coverage, 593
Ulcer in an adult, 723 urethral ballooning, 827
Unconcealing dressing, 762 Ventral-based dartos, 620
Uncuffed tube, 351 Ventral vertical length (VVL), 111, 638
Undescended, 14 Vertebral defects, anal atresia, cardiac defects,
Undescended testicle, 315 tracheo-esophageal fistula, renal anomalies
Unilateral SLAM, 659–660 (VACTERL) anomalies, 685
Upper lip graft, 457 Vertical meatal length/ventral glans closure (AB/BC),
Urethra pellucida, 167 452, 634
Urethral advancement, 462, 800 Verumontanum, 110, 157, 759
Urethral advancement and glanuloplasty procedure Vesical, 116
(UGPI), 11, 31 V-excision, 384
Urethral burnout, 778, 779 V incision, 35
Urethral catheters, 861 Voiding cystourethrography, 827
Urethral delta, 186 V-Y flaps, 332
Urethral diverticula, 841
Urethral endothelium, 87
Urethral labia, 77 W
Urethral lacunae of Morgagni, 110 Weak/incomplete ejaculation, 880
Urethral mobilization, 11, 471 Webbed penis, 672
Urethral plate (UP), 141–144, 501 Whirls, 144
width, 438 Width of the neo-urethra, 340
Urethral plate-based flap, 739 Wilms tumor 1, 271
Urethral splint, 757 Wing rotation, 56, 642
Urethral stents, 757, 861 Wolffian ducts, 290
Urethral strictures, 777, 841 Wound dehiscence, 503, 732
Urethral triplication, 685 Wound healing, 324–327, 729
Urethral tube, 85 Wound infection, 771
Urethroplasty, 9
Urinary diversion, 340–341, 730, 757–759, 861
Urinary function, 241 X
Urinary tract infection, 791 Xenograft, 328
Urine extravasation, 861
Urine flow rates, 882
Uroflowmetry, 438 Y
Urogenital, 68 Y chromosome, 286
folds, 68, 94 Yoke repair, 46
groove, 71 Y type duplications, 688
plate, 68 Y-V glanuloplasty, 653
sinus, 69, 70, 185 Y-V plasty, 782
V Z
Vacuolation, 78 Zona pellucida, 149
Vanishing testis, 292 Z-plasty, 331, 864
Vascularized fascial flaps, 739

Hypospadias Surgery An Illustrated Textbook (Second Edition) Ahmed T. Hadidi 2022 WOPS

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Hypospadias Surgery An Illustrated Textbook (Second Edition) Ahmed T. Hadidi 2022 WOPS

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Hypospadias

ISBN 978-3-030-94247-2 ISBN 978-3-030-94248-9 (eBook)

my Professors in Cairo University: Adel Loutfi and Nabhan

my Mentors: Dan Young in Glasgow and John Duckett in

all my children and their parents all over the world.

Baron Christopher Woodhouse

Mastery of hypospadiology is a lifelong pursuit and is best done with

Everybody is against specialization except the patient.

The purpose of this well-illustrated textbook is to provide a comprehen-

layer to reduce fistulae incidence and preoperative hormone therapy to enlarge

Stage I: READ what others say

Following this rule, I have realized that several things we learn in

I have had the privilege of operating on more than 10,000 hypospadias

I know of no surgery that can quite humble a surgeon as much as hypospadias—it

Reconstruction of a new urethra is like building a house: the surgeon

Offenbach, Frankfurt, Germany Ahmed T. Hadidi

The purpose of this comprehensive, well-illustrated textbook is to help inter-

Section 3 deals with the complications of hypospadias surgery and their

Cairo, Egypt Ahmed T. Hadidi

The challenge of hypospadias correction has always tested the ingenuity of

Sir David Innes Williams

An illness or abnormality that attracts a continuing bibliography of many

Figs. 9.4, 9.5, 9.6, 27.2, 47.1

Part I General and Basic Science

1 History of Hypospadias ������������������������������������������������������������������ 3

13 The Role of Preoperative Androgen Stimulation in the

Part II Operative Techniques

19 Grade I: Glanular Hypospadias;

30 Proximal Hypospadias With Small Flat Glans:

46 Penile Torsion (PT)�������������������������������������������������������������������������� 677

57 Early Complications������������������������������������������������������������������������ 771

63 Management of Failed Hypospadias Surgery ������������������������������ 835

Part IV Long-Term Results

65 Long-Term Consequences of Hypospadias Repair ���������������������� 879

Tariq O. Abbas Division of Urology, Department of Pediatric Surgery,

John P. Gearhart Pediatric Urology, Brady Urological Institute, The Johns

Michael Sennert Hypospadias Center and Pediatric Surgery Department,

Abbreviations To advance, one must acknowledge the work of

1.2 Evolution of Hypospadias

© The Author(s), under exclusive license to Springer Nature Switzerland AG 2022 3

after Christ, the middle or Renaissance period

1.2.1  he Early Period: The Period

Fig. 1.3 (continued)

1.3.2  rade II or Distal Hypospadias

The urethral meatus in distal hypospadias lies in

Fig. 1.7 Artist

A few years earlier, my aunt had called me to say

Fig. 1.10 Artist

Fig. 1.11 Original Mayo technique for distal hypospadias [54]

After Galen and a brief reference by Oribasus

Fig. 1.14 The origin of the word “chordee” dates back to

The first surgeon that I could identify who

b He used the term “congenital chordee” to differ-

Unfortunately these early repairs often resulted 1.6 Suture Materials

58. Toksu E. Hypospadias: one-stage repair. reconstruction. J Urol. 1987; https://doi.org/10.1016/

Abbreviations In an address to the Royal College of Surgeons,

© The Author(s), under exclusive license to Springer Nature Switzerland AG 2022 25

Table 2.1 Short list of men behind principles

Table 2.1 (continued)

1. Abnormal ventral curvature or chordee, by

2.2 Abnormal Ventral Curvature

The history of chordee is mentioned in detail in the

The second type of chordee is commonly a

Offenbach, Frankfurt, Germany Ahmed T. Hadidi

Cairo, Egypt Ahmed T. Hadidi

Part I General and Basic Science

1 History of Hypospadias �� 3

13 The Role of Preoperative Androgen Stimulation in the

Part II Operative Techniques

19 Grade I: Glanular Hypospadias;

30 Proximal Hypospadias With Small Flat Glans:

46 Penile Torsion (PT)�� 677

57 Early Complications�� 771

63 Management of Failed Hypospadias Surgery �� 835

Part IV Long-Term Results

65 Long-Term Consequences of Hypospadias Repair �� 879

1.2 Evolution of Hypospadias

1.2.1 he Early Period: The Period

1.3.2 rade II or Distal Hypospadias

Unfortunately these early repairs often resulted 1.6 Suture Materials

2.2 Abnormal Ventral Curvature

2.3 bnormal Proximal Meatal

2.5 Use of a Protective the use of an intermediate or interposition layer

2.6 Abnormal-Looking Glans rated” (MAGPI) procedure [35]. Arap et al.

3.2.1 Development and Fate

3.2.4 Development of the Male

3.2.4.1 Penile Urethra

3.2.4.2 Glanular urethra

3.3 Discussion Fleishmann [32] in animals and Politzer [33]

Fig. 3.39 Newborn baby with imperforate anus and ano-

4.3.5 The Penile (Spongy) Urethra

4.4 The Glans fibers, but neither osteocytes nor chondrocytes

4.4.2 Glans-Penis Ratio

4.10.3 Penile Length