title: Clinical outcome of cloacal exstrophy, current status, and a change in surgical management.
abstract: BACKGROUND
  Cloacal exstrophy is a complex and rare congenital malformation. Because of improvements in medicine, operative techniques, and perioperative management the survival rates are now approaching 100%. Currently, treatment is focused on improving quality of life. Since 1974, we encountered 20 patients with cloacal exstrophy in our hospital. The aim of this study is to evaluate our clinical experience and outcome during the last 39 years.
  
  
  METHODS
  A retrospective study of the medical records was performed. We evaluated anatomical status, phenotype and genotype at birth, subsequent surgical treatment and current gastrointestinal, urinary, spinal, genital, and gender status and outcome.
  
  
  RESULTS
  The records of all 20 patients who were treated in our center were included in this study. Of the 20 patients, 18 were primary patients, 2 referred. Six patients died within the first year of life. Median age of the surviving 14 patients was 25 years (range, 4-39 years) at time of evaluation. At the last follow-up, 11 patients had an endileostomy or endcolostomy. Three patients had a pull-through; two of them were continent for feces. Two patients were continent for urine; one with a sinus urogenitalis and one after urethra reconstruction. Eleven patients are incontinent; six of them had an incontinent urinary deviation (Bricker deviation). Of the other five incontinent patients, two had a urethral reconstruction, one vesicovaginal anastomosis, one perineotomy, and one epispadias bladder. Urinary continence was unknown in one patient. Of the surviving patients, nine have 46 XY karyotype and five 46 XX karyotype. Initially, six XY patients underwent external genital reconstruction with reassignment to female gender; however, one died postoperatively. One declared to feel like a man at age of 15 years. Remaining four XY patients underwent male external genital reconstruction. All XX patients underwent vagina or vulva reconstruction, except for one who still has aplasia vaginae. All patients were born with varying types of spinal dysraphism.
  
  
  CONCLUSIONS
  Despite medical efforts and development in treatment and support, satisfaction in outcome of cloacal exstrophy remains a challenge. Although reconstruction may be very difficult, male genital reconstruction in 46 XY patients has been given preference for more than a decade. A specialized multidisciplinary team should provide lifelong colorectal, urological, sexual, and psychosocial support to these patients, taking into account their age and phase of life.
authors.affiliation: Division of Pediatric Surgery, Department of Surgery, Radboudumc Amalia Children's Hospital, Radboud University Medical Center, Nijmegen, The Netherlands.
authors.forename: Rob van
authors.initials: Rv
authors.lastname: Vliet
authors.affiliation: Division of Pediatric Urology, Department of Urology, Radboudumc Amalia Children's Hospital, Radboud University Medical Center, Nijmegen, The Netherlands.
authors.forename: Luc A J
authors.initials: LA
authors.lastname: Roelofs
authors.affiliation: Division of Pediatric Surgery, Department of Surgery, Radboudumc Amalia Children's Hospital, Radboud University Medical Center, Nijmegen, The Netherlands.
authors.forename: Roxana
authors.initials: R
authors.lastname: Rassouli-Kirchmeier
authors.affiliation: Division of Pediatric Urology, Department of Urology, Radboudumc Amalia Children's Hospital, Radboud University Medical Center, Nijmegen, The Netherlands.
authors.forename: Robert P E
authors.initials: RP
authors.lastname: de Gier
authors.affiliation: Department of Pediatric Endocrinology, Radboudumc Amalia Children's Hospital, Radboud University Medical Center, Nijmegen, The Netherlands.
authors.forename: Hedi L
authors.initials: HL
authors.lastname: Claahsen-van der Grinten
authors.affiliation: Department of Medical Psychology, Radboudumc Amalia Children's Hospital, Radboud University Medical Center, Nijmegen, The Netherlands.
authors.forename: Chris
authors.initials: C
authors.lastname: Verhaak
authors.affiliation: Department of Orthopedics, Radboudumc Amalia Children's Hospital, Radboud University Medical Center, Nijmegen, The Netherlands.
authors.forename: Allard J
authors.initials: AJ
authors.lastname: Hosman
authors.affiliation: Department of Obstetrics and Gynecology, Radboudumc Amalia Children's Hospital, Radboud University Medical Center, Nijmegen, The Netherlands.
authors.forename: Catharina C M
authors.initials: CC
authors.lastname: Beerendonk
authors.affiliation: Department of Neurosurgery, Radboudumc Amalia Children's Hospital, Radboud University Medical Center, Nijmegen, The Netherlands.
authors.forename: Erik J
authors.initials: EJ
authors.lastname: van Lindert
authors.affiliation: Division of Pediatric Neurology, Department of Neurology, Radboudumc Amalia Children's Hospital, Radboud University Medical Center, Nijmegen, The Netherlands.
authors.forename: Michel A A P
authors.initials: MA
authors.lastname: Willemsen
authors.affiliation: Division of Pediatric Surgery, Department of Surgery, Radboudumc Amalia Children's Hospital, Radboud University Medical Center, Nijmegen, The Netherlands.
authors.forename: Marc H W A
authors.initials: MH
authors.lastname: Wijnen
authors.affiliation: Division of Pediatric Urology, Department of Urology, Radboudumc Amalia Children's Hospital, Radboud University Medical Center, Nijmegen, The Netherlands.
authors.forename: Wout F J
authors.initials: WF
authors.lastname: Feitz
authors.affiliation: Division of Pediatric Surgery, Department of Surgery, Radboudumc Amalia Children's Hospital, Radboud University Medical Center, Nijmegen, The Netherlands.
authors.forename: Ivo
authors.initials: I
authors.lastname: de Blaauw
mesh_terms: D000293:Adolescent; D000328:Adult; D002648:Child; D002675:Child, Preschool; D002988:Cloaca / Q000002:abnormalities*; D005242:Fecal Incontinence / Q000209:etiology; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D008297:Male; D019651:Plastic Surgery Procedures / Q000379:methods* / Q000523:psychology; D012189:Retrospective Studies; D016896:Treatment Outcome; D014549:Urinary Incontinence / Q000209:etiology; D014564:Urogenital Abnormalities / Q000150:complications / Q000523:psychology / Q000601:surgery*; D055815:Young Adult