title: Gastrointestinal ramifications of the cloacal exstrophy complex: a 44-year experience.
abstract: OBJECTIVE
  Cloacal exstrophy is a rare and complex congenital anomaly requiring coordination among multiple pediatric subspecialties. There is currently no consensus regarding the fate and function of the hindgut, which plays an integral role in patients' long-term gastrointestinal health and genitourinary reconstruction.
  
  
  METHODS
  A retrospective chart review was performed evaluating 77 patients with cloacal exstrophy treated during the previous 44 years at our institution.
  
  
  RESULTS
  Seventy-seven patients with cloacal exstrophy were treated between 1965 and 2008. Sixty-five were white, 6 were African American, 3 were Asian, and 3 were Hispanic. Genotypes included 44 XY, 32 XX, and 1 XYY. Fifty-one were reared as females and 26 as males. The hindgut length was 2 to 5 cm in 11 patients, 6 to 10 cm in 18 patients, 11 to 15 cm in 6 patients, 16 to 20 cm in 7 patients, and greater than 20 cm in 2 patients. The hindgut length was unknown in 33 patients. Forty-seven patients had tubularization of the cecal plate with an end colostomy, and 30 patients had an ileostomy placed for bowel diversion purposes. Four patients had short gut syndrome. Thirty-one patients had genitourinary reconstruction, 12 using small bowel and 19 using colon. Eight patients had hindgut pull-through procedures.
  
  
  CONCLUSIONS
  Gastrointestinal ramifications of the cloacal exstrophy complex include the occurrence of short gut syndrome and significant fluid and electrolyte derangements in patients receiving an ileostomy for initial intestinal management. This has caused a paradigm shift of initial intestinal management to tubularization of the cecal plate with end colostomy placement. This shift has eliminated the occurrence of short gut syndrome and enabled patients to be candidates for intestinal pull-through procedure if these patients are able to form solid stool, have a reasonable degree of pelvic neuromuscular development, and are able to comply with a bowel management program.
authors.affiliation: Johns Hopkins Hospital, Baltimore, MD, USA. dsawaya@comcast.net
authors.forename: David
authors.initials: D
authors.lastname: Sawaya
authors.forename: Seth
authors.initials: S
authors.lastname: Goldstein
authors.forename: Rupa
authors.initials: R
authors.lastname: Seetharamaiah
authors.forename: Kristina
authors.initials: K
authors.lastname: Suson
authors.forename: Rosemary
authors.initials: R
authors.lastname: Nabaweesi
authors.forename: Paul
authors.initials: P
authors.lastname: Colombani
authors.forename: John
authors.initials: J
authors.lastname: Gearhart
mesh_terms: D000015:Abnormalities, Multiple / Q000601:surgery; D001006:Anus, Imperforate / Q000601:surgery; D001746:Bladder Exstrophy / Q000601:surgery; D002648:Child; D002675:Child, Preschool; D002988:Cloaca / Q000002:abnormalities* / Q000601:surgery*; D003106:Colon / Q000601:surgery; D039021:Colonic Pouches; D004065:Digestive System Abnormalities / Q000235:genetics / Q000601:surgery*; D013505:Digestive System Surgical Procedures / Q000379:methods*; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007081:Ileostomy / Q000379:methods; D007420:Intestine, Large / Q000002:abnormalities / Q000601:surgery; D008297:Male; D019651:Plastic Surgery Procedures / Q000379:methods; D012778:Short Bowel Syndrome / Q000601:surgery; D016896:Treatment Outcome