We sought to evaluate long-term surgical urinary and bowel management in cloacal exstrophy
(CE) in a multi-institutional study.
We performed a cross-sectional study of people with CE and covered variants managed
at five participating institutions. Those with <1 year follow-up or born with variants
without hindgut involvement were excluded. Primary outcomes were methods of urinary
and bowel management. Urinary management included: voiding via urethra, clean intermittent
catheterizations (CIC), incontinent diversion and incontinent in diaper. Bowel management
included: intestinal diversion (colostomy/ileostomy) and pull-through (with/without
MACE). We evaluated three age groups: children (<10 years), older children (10 to
<18) and adults (≥18). We assessed if management varied by age, institution or time
(born≤2000 vs. >2000).
A total of 160 patients were included (40% male). Median follow-up was 15.2 years
(36% children, 22% older children, 43% adults). While 42% of children were incontinent
in diapers, 73% of older children and adults managed their bladder with CIC, followed
by incontinent urinary diversion (21%) (p < 0.001, Table). CIC typically occurred after augmentation (88%) via a catheterizable channel (89%).
Among older children and adults, 86% did not evacuate urine per urethra and 28% of
adults had an incontinent urinary diversion. No child or adult voided per urethra.
Age-adjusted odds of undergoing incontinent diversion was no different between institutions
(p = 0.31) or based on birthyear (p = 0.08).
Most patients (79%) had an intestinal diversion, irrespective of age (p = 0.99). Remaining
patients had a pull-through, half with a MACE. The probability of undergoing bowel
diversion varied significantly between institutions (range: 55–91%, p = 0.001), but
not birth year (p = 0.85).
We believe this large long-term data presents a sobering but realistic view of outcomes
in CE. A limitation is our data does not assess comorbidities or patient-reported
outcomes. Rarity of volitional urethral voiding in CE forces the question of whether
is a potentially unachievable goal. We advocate thoughtful surgical decision making
and thorough counseling about appropriate expectations, distinguishing between volitional
voiding and urinary and fecal dryness.
Summary TableUrinary and bowel management stratified by age.