• The term hypospadias refers to a urethral

opening that is on the ventral surface of the

penile shaft, proximal to the end of the glans.
The meatus may be located anywhere along
the shaft of the penis, from the glans to the
scrotum, or even in the perineum.
• Some boys with hypospadias, particularly
those with proximal hypospadias, have
chordee, in which there is ventral penile
curvature during erection.
Incidence
• Hypospadias occurs in up to 4 in 1,000
newborn boys. • The reason why the penis
does not develop properly is still not clear. •
The development of the penis whilst the baby
is growing in the womb is partly dependent
on the male sex hormones such as
testosterone. • The effects of testosterone on
the growing penis may be blocked in some
way. • Although it is not a genetic condition,
hypospadias can run in some families
Causes
• present at birth (congenital). The exact
reason this defect occurs is unknown. • A
defect in the androgen stimulation of the
developing penis • the deficient androgen
production by the testes and placenta.
Risk factors
• Age and weight: Mothers who were age 35
years or older and who were considered
obese had a higher risk of having a baby with
hypospadias. • Fertility treatments: Women
who used assisted reproductive technology to
help with pregnancy had a higher risk of
having a baby with hypospadias.
• Certain hormones: Women who took
certain hormones just before or during
pregnancy were shown to have a higher risk
of having baby with hypospadias. • Exposure
to smoking and chemicals: There is some
speculation about an association between a
mother's exposure to pesticides and
hypospadias. • Family history: This condition
is more common in infants with a family
history of hypospadias.
Classification
by Duckett in 1996. He divided them into
anterior (50%), middle (30%) and posterior
(20%) hypospadias. –The anterior form:
glandular, coronal and distal penile. – The
middle form: "midshaft” and proximal penile.
–The posterior form: penoscrotal, scrotal and
perineal
Glanular hypospadias : the urethra is within
the head of his penis (glans). Coronal:
The opening of the urethra is just below the
head of his penis. –Distal penile: the opening
of the urethra is in the distal portion of the
shaft of the penis
• Midshaft: The opening of the urethra is
located along the shaft of the penis. •
Proximal penile: the opening of the urethra is
in the proximal portion of the shaft of the
penis • Penoscrotal: The opening of the
urethra is located where the penis and
scrotum meet.
usually cleft.
Clinical manifestations
• Mild hypospadias usually does not cause
symptoms, especially in newborns and young
children. • This condition may cause a
downward curve of the penis during an
erection. • Erections are common in infant
boys.
Other symptoms include: • Abnormal
spraying of urine • Having to sit down to
urinate • Foreskin that makes the penis looks
like it has a "hood" • The farther the opening
is from the tip of the glans, the more likely
curvature in the penis (chordee) is present.
Diagnostic measures • prenatal ultrasound •
Physical exam of a newborn: upon
examination, the foreskin is usually
incomplete and the misplaced urethral
opening is located. • Excretory urogram: This
test uses X-rays to provide pictures of the
urinary tract, and other congenital anomalies
Treatment • Medical management • Minor
cases of hypospadias, in which the meatus is
located up toward the tip of the glans, may
not require surgical repair and may simply be
managed with observation.
Adjuvant hormonal therapy
• Presurgical treatment with testosterone
injections or creams, as well as HCG
injections, to promote penile growth, and for
the improvement in chordee with lessening
in the severity of the hypospadias.
Surgical management
• Management begins in the newborn period.
• Circumcision should be avoided, because
the foreskin often is used in the repair. • The
ideal age for repair in a healthy infant is 6–12
months –There is no greater risk of general
anesthesia at this age compared to 2–3 yr –
Penile growth over the next several years is
slow –The child does not remember the
surgical procedure –Postoperative analgesic
needs are less than in older children
DIFFERENT SURGERIES
• Glandular hypospadias requires a glandular
meatotomy • Coronal hypospadias requires a
meatal advancement and glanduloplasty
(MAGPI operation) • Proximal hypospadias
without a chordee can be treated by a skin
flap advancement • If chordee present it
should be excised and an island flap
urethroplasty performed
EPISPADIASIS
• Epispadias is a congenital malformation in
which the opening of the urethra is on the
dorsum of the penis. • In boys with
epispadias, the urethra generally opens on
the top or side of the penis rather than the
tip. However, it is possible for the urethra to
be open along the entire length of the penis.
• In girls, the opening is usually between the
clitoris and the labia, but may be in the belly
area.
Incidence
• Epispadias occurs in 1 in 117,000 newborn
boys and 1 in 484,000 newborn girls.
Causes
• Unknown • Related to improper
development of the pubic bone • Failures of
abdominal and pelvic fusion in the first
months of embryogenesis • Epispadias can be
associated with bladder exstrophy, an
uncommon birth defect in which the bladder
is inside out, and sticks through the
abdominal wall • Also occur with other
defects
Classification Classification of epispadias is
based on the location of the meatus the
penis. It can be positioned: • On the glans
(glanular) • Along the shaft of the penis
(penile) • Near the pubic bone (penopubic).
• The position of the meatus is important
because it predicts the degree to which the
bladder can store urine (continence). The
closer the meatus is to the base of the penis,
the more likely the bladder will not hold urine
Symptoms
In males: • Abnormal opening from the joint
between the pubic bones to the area above
the tip of the penis • Backward flow of urine
into the kidney (reflux nephropathy) • Short,
widened penis with an abnormal curvature •
Urinary tract infections • Widened pubic bone
In females:
• Abnormal clitoris and labia • Abnormal
opening where the from the bladder neck to
the area above the normal urethral opening •
Backward flow of urine into the kidney (reflux
nephropathy) • Widened pubic bone •
Urinary incontinence • Urinary tract infection

Diagnostic measures
• Prenatal diagnosis - rare • Blood test to
check electrolyte levels • Intravenous
pyelogram (IVP), a special xray of the kidneys,
bladder, and ureters • MRI and CT scans,
depending on the condition • Pelvic x-ray •
Ultrasound of the urogenital system
Surgical technique in males:
• The modified Cantwell technique It
involves partial disassembly of the penis and
placement of the urethra in a more normal
position.
Treatment
The primary goals of treatment of epispadias
are to: • maximize penile length and function
by correcting dorsal bend and chordee; and •
create functionality and cosmetically
acceptable external genitalia with as few
surgical procedures as possible. • If the
bladder and bladder neck are also involved,
surgical treatment is required to establish
urinary continence and preserve fertility.
• The second technique is the Mitchell
technique. »It involves complete disassembly
of the penis into its three separate
components. »Following disassembly, the
three components are reassembled such that
the urethra is in the most functional and
normal position and dorsal chordee is
corrected.

Surgical technique in females • The urethra

and vagina may be short and near the front of
the body and the clitoris is in two parts. • If
diagnosed at birth, the two parts of the
clitoris can be brought together and the
urethra can be placed into the normal
position. • If repaired early enough, lack of
urinary control (incontinence) may not be a
problem.
• If the diagnosis is missed or if early repair is
not performed, then incontinence can be
surgically corrected at the time of diagnosis. •
If the vaginal opening is narrow in older girls
or younger women, reconstruction can be
performed after puberty.
UNDESCENDED TESTIS
Definition • Undescended testis is the failure
of one or both testes to reach the normal
position in the scrotal sac through the
inguinal canal.
Incidence
• Cryptorchidism is the most common genital
abnormality in boys, affecting approximately
30% of baby boys born prematurely and
about 4% born at term. • Around 1 in 20 male
babies born at term also has cryptorchidism.
Many of these will become descended in
time. However, for around 1 in 70 cases, the
testis remains undescended after the child is
1 year old.

Etiological factors
• The exact cause of an undescended testicle
isn't known. • A combination of genetics,
maternal health and other environmental
factors might disrupt the hormones, physical
changes and nerve activity that influence the
development of the testicles.
contd..
• Impairment of the hypothalamic pituitary
gonadal axis: block in the hormonal axis to
stimulate the testes to descend or the testes
may fail to respond o the stimulus due to
some inherent defects • Anatomical
obstruction: there may be an obstruction in
the pathway of descend or failure of intra
abdominal pressure to rise • Heredity or
chromosomal anomalies: absence of one or
both testes • Short spermatic cord and artery
mechanically prevent the descend • Ectopic
attachment of the testes
Risk Factors
• Factors that might increase the risk of
undescended testicle in a newborn include: •
Low birth weight • Premature birth • Family
history of undescended testicle or other
problems of genital development •
Conditions of the fetus that can restrict
growth, such as Down syndrome or an
abdominal wall defect • Alcohol use by the
mother during pregnancy
Contd….
• Cigarette smoking by the mother or
exposure to secondhand smoke • Obesity in
the mother • Diabetes in the mother — type
1 diabetes, type 2 diabetes or gestational
diabetes • Parents' exposure to some
pesticides
Pathogenesis. • The process of testicular
descent is regulated by an interaction
between hormonal and mechanical factors. •
The testis develops at 7–8 wk of gestation. At
10–11 wk, the Leydig cells produce
testosterone, which stimulates differentiation
of the wolffian (mesonephric) duct into the
epididymis, vas deferens, seminal vesicle, and
ejaculatory duct. • At 32–36 wk, the testis,
which is anchored at the internal inguinal ring
by the gubernaculum, begins its process of
descent. The gubernaculum distends the
inguinal canal and guides the testis into the
scrotum. Following testicular descent, the
patent processus vaginalis (hernia sac)
normally involutes.
Types
• Retractile or pseudo cryptorchidism: This is
NOT an undescended testicle but is often
mistaken for one. It is caused byoveractive
muscles that pull the testicle(s) out of the
scrotum. In this type the testicles can be
placed in the scrotum manually and stay
there for a short period of time. This is a type
of normal and does not need treatment. •
Palpable (80%): In this type, (also called
prepubic or inguinal) the testicle is located
anywhere from just above the scrotum to
high in the groin.
Contd….
• Nonpalpable (15%): This means the testicle
is in the boy’s abdomen or is absent and not
felt in the scrotum or inguinal canal. • Ectopic
(5%): In this case, the testicle has taken the
wrong path and ended in an unusual locat
Ectopic (5%): In this case, the testicle has
taken the wrong path and ended in an
unusual location in the groin area.
Clinical features
• A nonpalpable testis (unable to feel on
examination) is the most common symptom
of cryptorchidism. • However, each child may
experience symptoms differently. •
Symptoms of cryptorchidism may resemble
other conditions or medical problems.
Diagnostic measures
• Soon after the baby is born or during a
routine check-up when they are six to eight
weeks old. • Physical examination • The first
stage in diagnosing undescended testicles is
to carry out a physical examination to see
whether the testicles can be felt near the
scrotum (palpable) or if they cannot be felt at
all (unpalpable).
Contd……
• Further tests • These tests may include an
ultrasound scan, CT scan, a diagnostic
laparoscopy, hormonal assay and urine and
blood tests. • Open surgery. • Direct
exploration of the abdomen or groin through
a larger incision may be necessary in some
cases.
Treatment
• In most cases, the testicle will descend
without treatment during the child’s first
year. If this does not occur, treatment may
include: • Hormone injections (B-HCG or
testosterone) to try to bring the testicle into
the scrotum. It also helps in the enlargement
of the testis. • Surgery (orchiopexy) to bring
the testicle into the scrotum. This is the main
treatment. If there is an associated hernia,
the herniotomy along with orchidoplexy is
indicated.
• Having surgery early may prevent damage
to the testicles that can cause infertility. An
undescended testicle that is found later in life
may need to be removed. This is because the
testicle is not likely to function well and could
pose a risk for cancer.
Complications
• In order for testicles to develop and
function normally, they need to be slightly
cooler than normal body temperature. The
scrotum provides this cooler environment.
Until a boy is 3 or 4 years old, the testicles
continue to undergo changes that affect how
well they function later.
• Complications of a testicle not being located
where it is supposed to be include: •
Testicular cancer • Fertility problems • Other
complications related to the abnormal
location of the undescended testicle include:
• Testicular torsion. • Trauma • Inguinal
hernia.
BLADDER EXSTROPHY/ ECTOPIA VESCICA
• Exstrophy of the bladder or ectopia vesicae
is a congenital malformation of the urinary
bladder in which a part of the wall of the
bladder in front is absent and the inner part
of the bladder is exposed.
Incidence
• It is a rare occurrence. • The incidence of
bladder exstrophy is approximately 2.07 per
100,000 live births and is slightly more
common in males than females. • The risk of
a family having more than one child with this
condition is approximately one in 100, • And
children born to a parent with exstrophy have
a risk of approximately one in 70 of having
the condition
Etiology
• There is no known cause for this condition
but there are many theories. • Some experts
believe during the 11th week of pregnancy
the embryo undergoes structural changes
including ingrowths of tissue in the lower
abdominal wall, which stimulates
development of muscles and pelvic bones. •
Up to this point the primitive bladder and
rectum are contained within tissue called the
cloacal membrane.
• The rectum then separates from the
bladder, and if migration of tissue towards
the midline over the primitive bladder fails
ion in the the cloacal membrane may rupture,
creating an exstrophied bladder. • The exact
timing of premature rupture of the
membrane determines whether the child is
born with isolated epispadias, classic bladder
exstrophy or cloacal exstrophy.
Risk factors
• The disorder has no known risk factors,
although it is more common in boys and in
infants of mothers who had tobacco exposure
or were young when giving birth. Children
whose parents were also born with bladder
exstrophy have a 1 in 70 chance of having it
themselves.
Associated problems
• Bladder: The bladder is turned inside out
and exposed on the abdominal wall. The
bladder neck has not developed properly and
the bladder itself is usually small.
Contd… • Epispadias: The urethral opening,
which is the hollow tube that drains urine
from the bladder to the outside of the body,
is in an abnormal location. In males, the
urethral opening is usually on the topside of
the penis and not the tip. In girls, the urethral
opening may be positioned further up
between the divided clitoris and labia minora.
Contd… • Widening of the pubic bones: The
pubic bones usually join to protect and
support the bladder, urethra and abdominal
muscles. In children with exstrophy, the pubic
bones do not join, leaving a wide opening.
This causes the hips to be outwardly rotated.
Contd…
• Vesicoureteral reflux (VUR): Normally the
kidneys make urine and drain down the
ureters (drainage tubes) into the bladder.
VUR is a condition where urine travels back
up into the kidneys. This may develop after
the bladder is reconstructed.
• Contd……
Abnormal Development of Genitalia:
–Boys: The penis may appear shorter and
curved in an upward direction. The testicles
may not be in a normal position in the
scrotum and a hernia may be seen. –Girls: The
clitoris and labia minora are separated and
spread apart; the vagina and urethra are
shorter. The uterus, fallopian tubes and
ovaries are generally normal.
–Contd…..
• Displacement of the umbilicus and/or an
umbilical hernia.
Clinical features
• Constant urinary dribbling through the
defect • Skin excoriation • Infection •
Ulceration of bladder mucosa • Ambiguous
genitalia • Waddling unsteady gait • Urinary
tract infections • Growth failure
Diagnostic measures
• fetal ultrasound • cystoscopic examination
• X ray • USG • IVP • Urodynamic studies • All
these will help to determine the extent of
anomaly and other associated problems
Treatment

• Management of bladder exstrophy should

start at birth. • Cover the bladder with
plastic wrap to keep the bladder mucosa
moist. • Avoid application of gauze or
petroleum-gauze to the bladder mucosa.

SURGICAL MANAGEMENT
Prompt closure of the exstrophic bladder •
During this procedure the abdominal wall is
mobilized and the pubic rami are brought
together in the midline. • (If the bladder
closure is performed during the first 48 hr of
life, often there is sufficient mobility of the
pubic rami to allow approximation of the
pubic symphysis. If the procedure is delayed,
the pelvic bones must be broken (pelvic
osteotomy) to allow the pubic rami to be
brought together and create a pubic
symphysis to support the bladder closure.)
Early bladder closure
• It can be applied to almost all neonates with
classic bladder exstrophy. Treatment should
be deferred in selected situations when
surgical therapy would be excessively risky or
complex, such as in a premature baby or
when it would have to be performed by
inexperienced surgeons.

Total reconstruction • It includes closure of

the bladder, closure of the abdominal wall,
and, in boys, correction of epispadias using a
technique of penile disassembly
Creation of a sphincter muscle for bladder
control and correction of the vesicoureteral
reflux
• The final stage of reconstruction involves
creation of a sphincter muscle for bladder
control and correction of the vesicoureteral
reflux.
Post operative care
• Bladder capacity is monitored every 12–24
mo using cystoscopy under anesthesia. •
Monitor for the development of
hydronephrosis and infection. • Most infants
with bladder exstrophy have vesicoureteral
reflux and should receive antibiotic
prophylaxis. • In boys, if the epispadias is not
corrected at birth, epispadias repair usually is
performed between 1–2 yr of age
NURSING MANAGEMENT FOR CONGENITAL
GENITOURINARY MALFORMATIONS
Preoperative
• It is important to address parents’ concerns
at the time of birth. Preoperative teaching
can relieve some of their anxiety about the
future appearance and functioning of the
penis.

Postoperative nursing diagnoses

• Pain (acute / chronic) related to physical
factors like damage to the skin / tissue
(incision) • Impaired skin integrity related to
surgical trauma • Impaired urinary
elimination related to groin area. to surgical
diversion, tissue trauma