Introduction

Epispadias is a rare congenital anomaly characterized by a dorsal urethral defect where it is replaced
by a broad mucosal strip lining the dorsum of the penis extending toward the bladder with the
potential incompetence of the sphincter mechanism. In contrary to hypospadias, the current entity is
not frequently discussed in the society of urology, plastic and paediatric surgery, and it is because of
its uncommon occurrence. Associated anomalies in patients with epispadias are deformed external
genitalia, diastasis of the pubic symphysis and urinary incontinence. Nevertheless, a carefully
constructed and well-planned approach is the prerequisite to the management of these patients. The
objectives of repair must include achievement of urinary continence, cosmetically acceptable
genitalia, normal genital function, and preservation of fertility potential in most cases. Here, we are
interested to report such cases not only for its rarity but also to refresh our memory with respect to its
surgical repair and outcome.
Corresponding Author:
Dr. Bijit Lodh
Email: drblodh@yahoo.co.in
1. During the first 2 months of gestation, the embryo (germinal disc) is subjected
to a complex process (delimitation) [1] of cranio-caudal tubularization
resulting in the cavitation and connection of the pelvic organs to the pelvic
floor. This leads to the creation of urinary, genital and intestinal cavities
independently connected to the perineal surface by distinct conduits and their
corresponding sphincter. If the delimitation process is interrupted early, the
distal bowels, the bladder and the urethra are not individualized and appear
as contiguous and often duplicated plates. This is a cloacal exstrophy which is
rare (1:250 000 births) and often associated with other abnormalities. If the
process halts a bit later in the gestation, the bowels are properly formed and
connected but the bladder and urethra are both widely open. This results in a
classical bladder exstrophy which is the most common presentation of the EEC
although still quite rare (1:10-50 000 births). Finally, when the process stops
late, only the urethra and the urethral sphincter are open and incompetent.
This results in an epispadias which is much rarer than the classical bladder
exstrophy (1:117 000 births) [2]. Depending on the timing of the cavitation
failure, one may distinguish posterior epispadias involving the sphincteric
mechanisms and various degrees of incontinence, from anterior epispadias
where the continence mechanisms are completely or partially respected but
the genital tubercle remains abnormal. Epispadias exists both in boys and girls
with a clear predominance in the male gender.
Complex embryonic hypotheses have been described to explain these
abnormalities. They involve the formation and positioning of the cloacal
membrane with is situated at the caudal end of the germinal disc and occupies
the infra-umbilical abdominal wall. Between the 2 layers (ectoderm and
endoderm) initially forming the cloacal membrane comes a mesenchymal
ingrowth [3] which will result in the formation of the lower abdominal muscles
and the pelvic bones [4]. The surrounding mesoderm will be at the origin of
the genital tubercle. The 3D development of the embryo progresses from the
cephalic to the caudal extremity, and from the dorsum to the ventrum of the
embryo. When the caudal delimitation is aborted, the mesenchymal ingrowth
between the ectodermal and endodermal layers fails to progress and the
overstretched cloacal membrane becomes fragile and subject to a premature
rupture leading to exstrophy (or non-cavitation) of the pelvic organs [5]. This
zip down process [2] explains the progressive closure of the pelvis and the
cavitation of the pelvic organs from the back to the front and from the top to
the bottom of the embryo. It is possible that the middle period of the cloacal
delimitation is more vulnerable than the early and late stages. This would
explain why bladder exstrophy is mEpidemiological data confirmed that the
incidence of bladder exstrophy is between 2.15 and 3.3 per 100 000 births [11,
12]) whereas epispadias was 2.4 per 100 000 with a sex ratio quite variable
from almost even [11] to a marked male predominance of 5:1 [13]. White
children, in vitro fertilization [14] and socioeconomic status seem to be
interacting factors. Significant genetic predisposition remains uncertain
considering the small reported cohorts [15-17]. The recurrence risk varies
from 0.3% [18] to 2.3% [16] [17] [15].ore frequent than cloacal exstrophy and
epispadias [6]. Other embryonic hypotheses have been reported [7] [8-10].
Anatomical and clinical presentation:
The description given hereunder is limited to the epispadiac penis itself. Associated urologic and
non-urological pelvic anomalies often found with the exstrophy patient are detailed in another
chapter.
In boys: The urethral plate is wide open along the dorsum of the penis on a
variable length. The extent of exposed urethral mucosa can vary from the
dorsum of the glans for anterior epispadias to the whole urethra and
bladder neck for posterior epispadias. The penis may look short and is
almost always bent dorsally due to the malrotation of the corpora
cavernosa. In isolated epispadias, the pelvic bony cavity is usually normal
although an interpubic gap may exist which shows that the EEC anomalies
are in fact a continuum. When associated with a bladder exstrophy, the
epispadiac penis appears foreshortened because of the wide separation of
the corporal insertions [19, 20]. The displacement of each hemipelvis
leads to a malrotation of each corpus, explaining the dorsal curvature of
the erect penis; the flaccid epispadiac penis does not dangle. The corpora
cavernosa are also shorter than in normal men especially in the exstrophy
group [21]. The erection mechanisms are preserved but sexual intercourse
is rendered impossible by the size and shape of the erect penis. The
hooded foreskin is only present on the ventral side of the penis although a
complete foreskin may be seen in epispadiac penis. The corpus
spongiosum is only present under the urethral plate which is itself often
wide and healthy contrary to what is often found in hypospadiac penis
where it can be hypoplastic [2]. Up to recently, it was thought that the
nerves leading to the glans were essentially represented by 2
neurovascular bundles running along the dorso-lateral sides of the corpora
cavernosa. The work done by Baskin [22] demonstrated that the
neurological fibres leading to the glans (and the clitoris) are spread around
a large surface covering the lateral aspects of the corpora and are
probably less individualized than previously thought. In the epispadiac
penis, the routes taken by the nerves are a bit more lateral due to the
malrotation of the corpora. A better understanding of the anatomy of the
nerves has significantly changed the way these nerves are separated from
the corpora and the urethral plate. No vascular anastomosis exist between
the epispadiac corpora which allows their full separation during repair.
The veru montanum and the seminal tracts are normal [23], provided they
are not injured iatrogenically. In posterior epispadias, retrograde
ejaculation may occur as the sphincteric mechanisms are in essence
defective. In epispadias following exstrophy closure, the prostate is only
present posterior to the urethral plate. The testicles are normal although
frequently located near the pubic tubercles, above the corresponding
scrotum which usually appears wide and stretched. Duplicated urethra
and penis have been reported [24] with exstrophy.
In girls, the clitoris is bifid. The urethral plate is
wide open up to the level of the bladder neck. The
vagina is normal in isolated female epispadias as
well as the bony pelvis. A hairless area separates the
two hemimons pubis. The labia are divergent. The
uterus, fallopian tubes and ovaries are normal except
for occasional uterine duplication. In case of
exstrophy where the vagina opening is anteriorly
positioned, often stenotic, the vaginal conduit is
horizontal.
2. Surgery of epispadias

Surgical repair of these genital anomalies are usually
performed during the first two years of life.
1. In boys: The aims of this surgery are 1) to
correct the dorsal curvature of the penis by
rotating the corpora cavernosa to obtain a
dangling penis; 2) to relocate the
reconstructed urethra on the ventral side of the
penis; 3) to redistribute the skin around the
penis to compensate the dorsal skin
defect. Although many procedures have been
described over the years [25, 26] [27], the
techniques of Ransley [28] ,Mitchell [29] and
Kelly [30] are probably the most commonly
used these days. Preoperative androgen
stimulation of the genital tubercle makes
surgery easier.
1. In Ransleys technique, the incision
begins in the midline above the urethral
opening and is extended far enough
upwards to provide good access to the
proximal corpora for
mobilization.Figure 1 It continues
down on each side of the urethral plate
(backed by the corpus spongiosum) and
sweeps ventrally around the coronal
sulcus separating the prepuce and
ventral skin from the corpora. Once the
ventral aspects of the corpora are fully
exposed. The fascia covering each
corpus is vertically opened all along
the ventral aspect of each corpus. This
maneuver allows exposure of the
albuginea of each corpus. Dissection
continues under the fascia around the
albuginea of each corpus medially to
separate the vascular tissues
(spongiosum) leading to the urethral
plate, and externally to separate the
wide strip of neurological fibers
innervating the glans. Each
neurovascular bundle and the urethral
plate with its spongiosal tissue are then
separated from adjacent
structures. Figure 2. The urethral plate
is tubularized and a MAGPI type plasty
(IPGAM) of its distal end allows
ventralization of the future urethral
meatus. The corpora are then rotated
medially by approximately 90 and
maintained in this new configuration by
a proximal caverno-
cavernostomy. This new anastomosis
between the corpora keeps the urethra
in its ventral position and gives the
penis a dangling position when flaccid.
The skin shaft cover is performed with
a transverse flap of ventral skin
dissected with its pedicle and transfer
to the dorsal side of the penis (reverse
Duckett).Figure 3
2. Mitchells technique [29, 31] is based
on a complete disassembly of the penile
structures which allows a tubularization
and ventralization of the entire urethra,
and a more complete release of the
corporal rotation. The corpora
cavernosa are completely separated
from each other with their
corresponding hemi glans. The urethral
plate is dissected off of the corporeal
bodies respecting its blood supply
coming from underneath. It is then
tubularized and transferred ventrally.
The corpora entirely separated and
independent are rotated to correct the
dorsal chordee and sutured together.
The glans halves are subsequently
brought together.
3. The Kelly procedure or radical soft
tissue mobilization is used by some
[32, 33] for exstrophy closure. The
technique is described elsewhere[30]
and may be a valuable alternative for
primary or secondary penile
reconstruction in EEC patients. The
periostium on the inner side of the
ischium and pubis with the attachment
of the sphincter muscles, the pudendal
vessels and nerves are mobilized on
both sides so that the outstretched
muscles can help to reconstitute the
membranous urethra without
osteotomy. Others [34] have suggested
a mobilization of the crura from the
pubic rami to get more penile length
without the complete Kelly
mobilization
.
2. In girls, the open urethral plate extending
from the bladder neck to the medial aspect of
both hemi-clitoris anteriorly, and to the
anterior vaginal edge posteriorly, is separated
from the adjacent structures up into the
perineal muscles and subsequently tubularized
(Figure 4 ). The triangular hairless area
separating both hemi-pubis is excised (Figure
5). The perineal muscles located in front of
and between the neo-urethral conduit and the
vaginal orifice are both sutured together
which significantly increases the bladder
outlet resistance and provides social
continence in most cases(Figure 6). A
secondary plasty of the mons venus may be
needed at puberty if the cosmetic appearance
of this area remains unsatisfactory. It is
recommended not to dissect and suture
together both medial aspects of the two hemi-
clitoris to avoid any damage of the clitoral
sensitivity [35].

Epispadic penis classification a. glanular b. mids shaft c. penopubic