title: Long-term follow-up of functional outcome in patients with a cloacal malformation: a systematic review.
abstract: BACKGROUND
  Reconstructive surgery is performed in patients with cloacal malformations to achieve anorectal, urological, and gynecological function. The aim of this study was to evaluate the functional outcome of cloacal malformation repair as reported in literature.
  
  
  METHODS
  A systematic literature search was conducted according to PRISMA guidelines using PubMed, EMbase, and Web-of-Science. Records were assessed for the reporting of functional outcomes, which was divided into anorectal, urological, or gynecological function. Studies were used in qualitative (Rangel score) and quantitative syntheses.
  
  
  RESULTS
  Twelve publications were eligible for inclusion. Voluntary bowel movements were reported in 108 of 188 (57%), soiling in 146 of 205 (71%), and constipation in 31 of 61 patients (51%). Spontaneous voiding was reported for 138 of 299 patients (46%). 141 of 332 patients (42%) used intermittent catheterization, and 53 of 237 patients (22%) had a urinary diversion. Normal menstruations were reported for 25 of 71 patients (35%). Centers with limited experience reported similar outcome compared to centers with more experience (≥1 patients/year).
  
  
  CONCLUSIONS
  In this review we present functional outcome of the largest pooled cohort of patients with cloacal malformations as reported from 1993 to 2012. Functional disturbances are frequently encountered in anorectal, urological, as well as gynecological systems. Reporting of functional outcome in these patients should improve to increase knowledge about long-term results in patients with this rare malformation and to reach higher study quality. Especially, sacral and spinal anomalies should always be reported given their impact on functional outcome. Specialized care centers may be of great importance for patients with rare and complex conditions.
authors.affiliation: Department of Pediatric Surgery, Erasmus MC-Sophia Children's Hospital, University Medical Center Rotterdam, Rotterdam, The Netherlands. Electronic address: h.versteegh@erasmusmc.nl.
authors.forename: Hendt P
authors.initials: HP
authors.lastname: Versteegh
authors.forename: Iris A L M
authors.initials: IA
authors.lastname: van Rooij
authors.forename: Marc A
authors.initials: MA
authors.lastname: Levitt
authors.forename: Cornelius E J
authors.initials: CE
authors.lastname: Sloots
authors.forename: Rene M H
authors.initials: RM
authors.lastname: Wijnen
authors.forename: Ivo
authors.initials: I
authors.lastname: de Blaauw
mesh_terms: D000015:Abnormalities, Multiple / Q000196:embryology / Q000601:surgery*; D001003:Anal Canal / Q000002:abnormalities* / Q000196:embryology / Q000601:surgery; D000071056:Anorectal Malformations; D001006:Anus, Imperforate / Q000196:embryology / Q000601:surgery*; D002988:Cloaca / Q000002:abnormalities*; D003248:Constipation / Q000453:epidemiology / Q000209:etiology; D005242:Fecal Incontinence / Q000453:epidemiology / Q000209:etiology; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D008599:Menstruation Disturbances / Q000453:epidemiology / Q000209:etiology; D011183:Postoperative Complications / Q000453:epidemiology / Q000209:etiology*; D019651:Plastic Surgery Procedures / Q000706:statistics & numerical data; D012007:Rectum / Q000002:abnormalities* / Q000196:embryology / Q000601:surgery; D012447:Sacrum / Q000002:abnormalities; D016896:Treatment Outcome; D014547:Urinary Diversion / Q000706:statistics & numerical data; D014555:Urination Disorders / Q000453:epidemiology / Q000209:etiology; D014621:Vagina / Q000002:abnormalities
keywords: Anorectal malformations; Cloacal malformations; Functional outcome; Systematic review