CONGENITAL

GENITO-URINARY
DISORDER
Ms ELLEN ANGELLIN
 HYPOSPADIAS

• 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.
 DEFINITION

Hypospadias is defined as Congenital (at birth)

malformation in which the opening of the urethra is on
the ventral surface (underside) of the penis.
 
HYPOSPADIAS
 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.
• Scrotal hypospadias:
hypospadias with the urethral
opening on the scrotal surface.
• perineal hypospadias: the urethral
meatus opens in the perineum near
the anus; the scrotum is 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.
 DEFINITION

Epispadias Is defined as Congenital (at birth)

malformation in which the opening of the urethra (from
whence comes the urinary stream) is on the dorsum
(topside) of the penis
 
 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 x-
ray 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
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 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
• Urod
• yna
mic
studi
es
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
Nursing Assessment
History.
Obtain a thorough history and physical examination,
including any history of a familial pattern of hypospadias,
any past medical history or comorbidity, and a meatal
location, glans configuration, skin coverage, and
chordee.
Physical examination. 
Although the diagnosis of hypospadias has been made
with both antenatal fetal ultrasonography and magnetic
resonance imaging (MRI), the diagnosis is generally
made upon examination of the newborn infant
 Nursing Diagnoses

Based on the assessment data, the major nursing

diagnoses are:
Acute pain related to physical factors: damage to the
skin/tissue.
Impaired skin integrity related to surgical trauma.
Impaired urinary elimination related to mechanical
trauma from surgery.
Anxiety related to threat to self-concept; change in
health status and environment.
Risk for infection related to inadequate primary
defenses.
 
The major nursing care plan goals are:
Child will experience decreased pain as evidenced by
infrequent crying episodes and exhibit normal sleeping
pattern.
Child will experience improved urinary elimination.
Parent will experience less anxiety.
Child will remain free from infection as evidenced by
clean and intact wound without redness, edema, odor or
drainage and negative urine culture.
 Nursing Interventions

Nursing interventions for the child are:

RELIEF FROM PAIN.
 Assess location, characteristics, onset, duration,
frequency, location, and severity of pain; Observe for
verbal and nonverbal cues.
Maintain a position of comfort.
Properly set the catheter to avoid tension and kinking.
Encourage use of relaxation techniques. .
Apply ice compress as indicated.
Administer analgesic (e.g., Tylenol) as ordered.
Educate parents that medications will prevent pain and
restlessness and allow for healing.
IMPROVE URINARY ELIMINATION
Record input and output;
Assess voiding stream, color and amount of urine on
first flow of urine and each succeeding void.
Assess for pain, abdominal distention, inability to void
for 8 hours after catheter.
Encourage high fluid intake after catheter removed,
offer favoured choice of liquids hourly.
Maintain hydration and free flowing of urine.
Support child after the catheter is removed and provide
privacy for voiding.
Instruct parents to notify the physician of changes in the
urinary pattern or inability to void.
LESSEN ANXIETY
Assess source and level of anxiety and need for
information that will relieve anxiety include the type of
procedure and appearance of penis after surgery;
whether the penis will be sexually adequate; possibility
that correction may need to be done in stages if the child
is old enough
Encourage verbalization of concerns and allow time for
parents and child to ask questions about condition,
procedures, recovery.
Encourage parents to stay with the child during
hospitalizations and to assist in care.
Provide parents an opportunity to make decisions on
care and common routines.
Answer questions calmly and honestly; use pictures,
drawings, and models for information.

Inform parents regarding the type, and purpose of

surgery, the appearance of the penis post op and
cosmetic results to expect; inform older child that penis
will not be cut off and that procedure is not a form of
punishment.

Teach parents about postoperative care

(indwelling meatal or suprapubi catheter or stents will
be in place; restraints maybe in place).
PREVENT INFECTION.
Assess wound for redness, swelling, drainage on
dressing.
Observe catheter insertion site for redness, irritation,
swelling;
Monitor urine in the catheter bag for cloudiness, foul
odour, sediment.
Obtain urine specimen for culture
and sensitivities as indicated..
Note urinary output of at least 1 ml/kg/hr and report if
less.Indicates that catheter obstruction may be present
with urinary retention which results in infection.
Encourage to increase fluid intake according to age
needs.
Apply sterile technique during dressing changes,
catheter care or draining urine bag
Maintain catheter and collection bag below level of the
bladder and a closed drainage system free of kinks in the
tubing then maintain catheter and collection bag—
marked in red.
Reinforce dressing as needed, and secure catheter to
penis with dressing and tape, and to leg or abdomen with
tape.
Immobilize arms and legs with restraints, remove
periodically; use a bed cradle following surgery
Inform parents to avoid allowing the child to straddle
toys, play in a sandbox, swim, or engage in rough
activities until advised by the physician.
Teach parents to sponge bathe the child and use loose-
fitting clothing, disallowing the contact of feces with the
wound, and instruct in cleansing after each bowel
elimination.
Educate parents in signs and symptoms of infection
Teach parents on catheter care, irrigation, emptying of
urine bag or using a diaper for urine drainage, securing
the catheter with a tape.
Apply sterile technique during dressing changes,
catheter care or draining urine bag.
 Evaluation

Goals are met as evidenced by:

Child will experience decreased pain as evidenced by

infrequent crying episodes and exhibit normal sleeping
pattern.
Child will experience improved urinary elimination.
Parent will experience less anxiety.
Child will remain free from infection as evidenced by
clean and intact wound without redness, edema, odor or
drainage and negative urine culture.
THANK YOU