Genitourinary Anomaly Guide for Bladder Exstrophy

Mr. Dinabandhu Barad, MSC TUTOR, SNC,SOA,DTU

CONGENITAL
ANOMALY OF
GENITOURINARY
SYSTEM
BY:
Dinabandhu Barad
MSC TUTOR, SNC,SOA,DTU

BLADDER EXSTROPHY
OR
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.
BLADDER EXSTROPHY

INCIDENCE
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 11thweek of pregnancy the embryo undergoes
structural changes including growths of tissue in the lower abdominal wall,
which stimulates development of muscles and pelvic bones.

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.

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.
ASSOCIATED PROBLEMS

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.
ASSOCIATED PROBLEMS

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.
ASSOCIATED PROBLEMS

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.

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 BLADDER CLOSURE
• 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.)

TOTAL RECONSTRUCTION
• It includes closure of the bladder, closure of the abdominal wall, and, in boys,
correction of epispadias.

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 months using cystoscopy
under anesthesia.
• Monitor for thedevelopment of hydronephrosis and infection.

POST OPERATIVE CARE
• 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 CARE
1. ACUTE PAIN
NURSING DIAGNOSIS
Acute pain related to surgery as evidienced by verbalization of pain crying
irritability, restlessness ,distractive behaviour, changes in vital signs
GOAL
Child will experience decreased pain as evidenced by infrequent crying
episodes and exhibit normal sleeping pattern.

NURSING CARE1. ACUTE PAIN
Assess location, characteristics, onset,
duration, frequency, location, and
severity of pain; Observe for verbal and
nonverbal cues.
Provides data about the description of
pain which can be used as a guideline for
analgesic therapy.
Maintain a position of comfort; Properly
set the catheter to avoid tension and
kinking.
Promotes comfort and avoids pain due to
pulling on or manipulating catheter.
Encourage use of relaxation techniques.
Promotes rest and refocus attention thus
decreases discomfort.
RATIONALEINTERVENTION

NURSING CARE1. ACUTE PAIN
RATIONALEINTERVENTION
Administer analgesic (e.g., Tylenol) as
ordered.
Lessens pain and promotes rest which
reduces stimuli and pain.
Educate parents that medications will
prevent pain and restlessness and allow
for healing.
Provides information about the need for
pain medications for child’s comfort.

Impaired Urinary Elimination
Nursing Interventions Rationale
Record input and output; Assess voiding
stream, color and amount of urine on
first flow of urine and each succeeding
void.
Provides data on voiding pattern
after clamping or removal of the
catheter.
Assess for pain, abdominal distention,
inability to void for 8 hours after
catheter.
Indicates urinary dysfunction and
possible obstruction or continuing
edema of the meatus.
Encourage high fluid intake after
catheter removed, offer favored choice
of liquids hourly.
Maintain hydration and free flowing of
urine.

Impaired Urinary Elimination
Support child after the catheter is
removed and provide privacy for voiding.
Avoids embarrassment on the part of an
older child.
Instruct parents to notify the physician
of changes in the urinary pattern or
inability to void.
Allows for early intervention to avoid
further complications.

RISK FOR INFECTION
• Risk for Infection related to inadequate primary defenses
(surgical incision) invasive procedure (catheter)as evidenced by
• Desired Outcomes
• Child will remain free from infection as evidenced by clean and
without redness, edema, odor or drainage and negative urine

Assess wound for redness, swelling,
drainage on dressing.
Provides information on the presence of
infection or impaired healing.
Observe catheter insertion site for
redness, irritation, swelling; Monitor
urine in the catheter bag for cloudiness,
foul odor, sediment.
Indicates infectious process at catheter
site or in the urinary bladder.
Obtain urine specimen for culture
and sensitivities as indicated.
Determines the specific organism and
sensitivity to the antibiotic.
RISK FOR INFECTION

Genitourinary Anomaly Guide for Bladder Exstrophy

Genitourinary Anomaly Guide for Bladder Exstrophy

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