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Urinary and bowel management in cloacal exstrophy: A long-term multi-institutional cross-sectional study

Published:October 07, 2022DOI:https://doi.org/10.1016/j.jpurol.2022.10.003

      Summary

      Background

      We sought to evaluate long-term surgical urinary and bowel management in cloacal exstrophy (CE) in a multi-institutional study.

      Methods

      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).

      Results

      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).

      Summary

      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.

      Conclusions

      Summary TableUrinary and bowel management stratified by age.
      Younger children (0–9.9 years old, n = 57) Older children (10.0–17.9 years old, n = 35) Adults (≥18 years old, n = 68) p-value Overall (n = 160)
      Urinary management
      Clear intermittent catheterizations (CIC) 18 (32%) 29 (83%) 46 (68%) <0.001 93 (58%)
       Per channel 16 (89%) 28 (97%) 39 (85%) 83 (89%)
       Per urethra 2 (11%) 1 (3%) 7 (15%) 10 (11%)
       Augmentation 12 (67%) 25 (86%) 45 (98%) 82 (88%)
      Incontinent diversion 15 (26%) 3 (9%) 19 (28%) 37 (23%)
      Incontinent into diaper 24 (42%) 3 (9%) 2 (3%) 29 (18%)
      Empties per urethra with Valsalva maneuver 0 (0.0%) 0 (0%) 1 (1%) 1 (0.6%)
      Overall: empties urine via urethra (CIC, incontinent, Valsalva) 26 (46%) 4 (11%) 10 (15%) <0.001 40 (25%)
      Bowel management
      Intestinal diversion 45 (79%) 27 (77%) 54 (79%) 0.99 126 (79%)
      Pull-through without a MACE 7 (12%) 4 (11%) 7 (10%) 18 (11%)
      Pull-through with a MACE 5 (9%) 4 (11%) 7 (10%) 16 (10%)

      Keywords

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