title: The association of the severity of anorectal malformations and intestinal malrotation.
abstract: BACKGROUND
  Intestinal malrotation is a known association of anorectal malformations (ARM). Exact incidence, prognosis and surgical implications related to ARM are unknown. The aim of this study was to identify relevant associations between ARM and the presence of malrotation.
  
  
  METHODS
  Records of patients from two referral centers were retrospectively analyzed looking for malrotation associated to ARM and its management, as well as factors for functional prognosis.
  
  
  RESULTS
  40 patients out of 2572 with ARM (1.6%) were found to have malrotation. Females were more commonly affected, and severe malformations were more frequent (cloaca, covered cloacal exstrophy in females and rectoprostatic and rectobladder neck fistula in males). Factors significantly associated with malrotation included Müllerian or Wolffian duct anomalies (P<0.05), while fecal continence status, presence of constipation, and use of laxatives or enemas were not. Detecting and correcting malrotation early on or at the time of colostomy creation represented a protective factor against additional surgeries for bowel obstruction and volvulus (P<0.001). Removal of the appendix during malrotation treatment required constructing a neoappendicostomy using a cecal flap in 9 out of 14 patients needing antegrade enema administration.
  
  
  CONCLUSIONS
  Malrotation presence in patients with ARM has the same frequency as in the general population, but it is more common in severe malformations. Surgeons treating these patients should address the malrotation at the time of colostomy opening if detected. The appendix should be preserved for potential future use as an appendicostomy for antegrade administration of enemas.
authors.affiliation: Colorectal Center for Children, Pediatric Surgery Division, Cincinnati Children's Hospital Medical Center, 3333 Burnet Avenue, MLC 2023, Cincinnati, OH, 45229, U.S.A.. Electronic address: bruno.martinez.leo@cchmc.org.
authors.forename: Bruno
authors.initials: B
authors.lastname: Martinez-Leo
authors.affiliation: Reconstructive Pelvic Medicine Program, Seattle Children's Hospital. Department of Surgery, University of Washington, 4800 Sand Point Way NE OA.9.256, Seattle, WA 98105-0371, U.S.A.. Electronic address: patrick.chesley@seattlechildrens.org.
authors.forename: Patrick
authors.initials: P
authors.lastname: Chesley
authors.affiliation: Pediatric Urology, New York-Presbyterian Morgan Stanley Children's Hospital, 3959 Broadway, New York, NY 10032. Electronic address: sa3168@cumc.columbia.edu.
authors.forename: Shumyle
authors.initials: S
authors.lastname: Alam
authors.affiliation: Colorectal Center for Children, Pediatric Surgery Division, Cincinnati Children's Hospital Medical Center, 3333 Burnet Avenue, MLC 2023, Cincinnati, OH, 45229, U.S.A.. Electronic address: jason.frischer@cchmc.org.
authors.forename: Jason S
authors.initials: JS
authors.lastname: Frischer
authors.affiliation: Center for Colorectal and Pelvic Reconstruction, Nationwide Children's Hospital, 700 Children's Drive Columbus, OH 43205. Electronic address: marc.levitt@nationwidechildrens.org.
authors.forename: Marc A
authors.initials: MA
authors.lastname: Levitt
authors.affiliation: Reconstructive Pelvic Medicine Program, Seattle Children's Hospital. Department of Surgery, University of Washington, 4800 Sand Point Way NE OA.9.256, Seattle, WA 98105-0371, U.S.A.. Electronic address: jeffrey.avansino@seattlechildrens.org.
authors.forename: Jeffrey
authors.initials: J
authors.lastname: Avansino
authors.affiliation: Colorectal Center for Children, Pediatric Surgery Division, Cincinnati Children's Hospital Medical Center, 3333 Burnet Avenue, MLC 2023, Cincinnati, OH, 45229, U.S.A.. Electronic address: belinda.dickie@cchmc.org.
authors.forename: Belinda Hsi
authors.initials: BH
authors.lastname: Dickie
mesh_terms: D000328:Adult; D000071056:Anorectal Malformations / Q000145:classification / Q000150:complications*; D001065:Appendix / Q000601:surgery; D002675:Child, Preschool; D003125:Colostomy / Q000009:adverse effects; D003248:Constipation / Q000209:etiology; D005242:Fecal Incontinence / Q000209:etiology; D005260:Female; D006801:Humans; D015994:Incidence; D045822:Intestinal Volvulus / Q000150:complications*; D008297:Male; D011379:Prognosis; D012189:Retrospective Studies; D012720:Severity of Illness Index
keywords: Anorectal malformations; Appendix; Imperforate anus; Intestinal malrotation