Bladder Exstrophy and Epispadias

Exstrophy-Epispadias Complex

• The exstrophy-epispadias complex is a spectrum of midline defects that includes classic bladder exstrophy, epispadias, and cloacal exstrophy.
• Incidence: 1 in 40,000 live births, with a male to female ratio of 2:1.
• Recurrence risk is 1 in 100.

Embryology

• Failure of mesenchyme to migrate between the ectodermal and endodermal layers of the lower abdominal wall with subsequent premature rupture of the cloacal membrane.
• Rupture after complete separation of the GU and GI tracts results in classic bladder exstrophy, and prior to descent of the urorectal septum results in cloacal exstrophy.

Antenatal US Findings

• Failure to visualize the bladder.
• Lower abdominal wall bulge and low-set umbilical cord.
• Abnormal genitalia and abnormal widening of iliac crests.

Post-natal Appearance

• The bladder is open on the lower abdomen, with urothelium fully exposed.
• In males, the penis is short and broad with dorsal chordee.
• In females, the clitoris is bifid, anterior labia are displaced laterally, and the mons pubis is absent.
• The pubic symphysis is widely separated in both sexes, with divergent recti muscles.
• Inguinal hernias are frequently associated with exstrophy.

Management

• Objectives of management include:
  • Restoration of urinary continence.
  • Preservation of renal function.
  • Reconstruction of functionally and cosmetically acceptable external genitalia.

Epispadias

• Epispadias is a problem that can occur in both boys and girls.
• In boys, the urethra does not develop into a full tube, and the urine exits the body from an abnormal location.
• In girls, the urethral opening is in the belly area instead of between the clitoris and labia.
• Primary epispadias can be detected in the immediate postnatal examination, but in some boys, it may only become evident later.

Cloacal Exstrophy

• Incidence: 1 in 200,000, with a male to female ratio of 1:1.
• Associated with spinal dysraphism (66%) and short bowel syndrome (33%).
• Post-natal appearance:
  • Bladder is open and separated into two halves by an intestinal plate.
  • Each hemi-bladder may have a ureteric orifice.
  • Nearly all patients have an associated exomphalos.

Preoperative Work-up

• Cover the defect with a moist plastic sheet to prevent epithelium swelling.
• Ultrasonography to exclude other renal malformations.
• Intravenous urography, if anomalies of the upper renal tract are suspected.
• Explain the malformation to the parents and involve them in the decision-making process.

Surgical Intervention

• Bladder exstrophy correction usually requires staged reconstructive procedures.
• Children born with the exstrophy-epispadias complex should only be managed within a limited number of centers with a multidisciplinary team.
• Surgical stages:
  • Bladder closure (first stage) within the first 48 hours of life.
  • Penile reconstruction (Epispadias repair) (second stage) at 12-18 months.
  • Bladder neck reconstruction (third stage) at 4 years.

Postoperative Care

• Antibiotics to prevent urinary infection.
• Bandage application around the hips with inner rotation.
• Ureteral stents for 2 weeks.
• Drainage of the bladder to prevent bladder outlet obstruction.

Complications

• Bladder dehiscence – partial/complete.
• Bladder prolapse.
• Upper urinary tract deterioration due to high outlet resistance and vesicoureteric reflux.
• Incomplete bladder emptying.