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Epidemiology and Prenatal Diagnosis

• Incidence: 2.15 per 100K live births
• If prior child with bladder exstrophy, chances of future child are 1:100
• Chances of patient with exstrophy having a child with exstrophy: 1:70
• Prenatal US: may note absence of bladder filling, low-set umbilicus, widened pubic ramus, small genitals, and lower abdominal mass
• Neonatal evaluation: obtain renal US to assess for hydronephrosis, pelvic XR to assess pubic diastasis, and spinal US to assess for spina bifida

Bladder considerations

• Bladder mucosa should be immediately protected with membrane to prevent metaplastic changes
• Bladder neds to be examined under anesthesia to adequately assess depth of bladder extension
• Reflux will always happen in closed bladder and reimplantation is required

Genital considerations

• Corpora cavernosa are shorter and wider than normal
• Testes may be retractile but fertility is normal
• Retrograde ejaculation may occur even after bladder neck reconstruction
• Uterine prolapse more common due to poor pelvic floor support

Other considerations

• Workup: check CBC/BMP, renal US (risk for renal anomalies), and KUB (assess pubic diastasis)
• Orthopedic abnormalities result in waddling gait and outward limb rotation
• Levator ani positioned posteriorly and laterally
• Inguinal hernias present in 82% boys and 11% girls
• Anus positioned more anteriorly, 2% incidence of GI anomalies - imperforate anus, rectal stenosis, rectal prolapse

Repair considerations

• Will require bladder closure, abdominal wall closure, posterior urethral closure, osteotomies, epispadias repair, bladder neck reconstruction, and antireflux procedure
• Osteotomy goals: relaxing tension on GU repairs
• Primary closure: maintain adequate bladder and ureteral drainage
• If primary repair attempts fail, can consider ureterosigmoidostomy or complete urinary diversion

Repair complications

• Penile ischemia: due to corporal injury, more common if osteotomies not performed
• Urethrocutaneous fistula: seen in 5-40%, may spontaneously close if SPT placed, if not may require repeat reconstruction
• Dehiscence: uncommon, may occur with bladder or abdominal wall, requires redo reconstruction
• Urethral obstruction: evaluate with cystoscopy, may require SPT or CIC
• Epididymitis: 19-33%, evaluate for high pressure voiding and strictures
• Malignancy: adenocarcinoma most common, increased risk if colon used for augment
• MSK: increased risk for gait abnormalities and hip/pelvic pain later in life

Longterm issues (into adulthood)

• UTI: common, exacerbated by presence of VUR, can cause longterm renal injury
• Male genital: phallus size/appearance, difficulty with penetration, difficulty with condom sizing, management may require specialized center referrals
• Female genital: increases risk for pelvic prolapse (onset at younger age than general population), C-section recommended
• Urinary: increased risk for stress incontinence after outlet surgery (high dependence on bladder neck for baseline continence)

Cloacal exstrophy

• Will have features of bladder exstrophy, along with exstrophy of terminal ileum, imperforate anus, and omphalocele
• Spinal abnormalities noted in 85-100%
• Uterine duplication seen in > 90% patients