title: Gastroschisis, atresia, dysmotility: surgical treatment strategies for a distinct clinical entity.
abstract: OBJECTIVE
  Intestinal atresia (IA) occurs in 10% to 20% of infants born with gastroschisis (GS). We describe a distinct subset of these children with severe dysmotility, without mechanical intestinal obstruction, and with adequate intestinal length for enteral nutrition and propose possible treatment strategies.
  
  
  METHODS
  A total of 177 patients with GS managed at a single institution between 1993 and 2007 were retrospectively reviewed.
  
  
  RESULTS
  Twenty-one (12%) patients had IA. Six (29%) did well, with gradual progression to full diet; 1 died at birth because of complete midgut infarction; 1 died of necrotizing enterocolitis; 4 with short bowel syndrome (SBS) (small bowel length, 30-41 cm) died of liver failure because of total parenteral nutrition (3) or sepsis (1). Nine (43%) were felt to have gastroschisis/atresia/dysmotility, defined as adequate small bowel length for survival as measured at time of laparotomy (mean, 146 cm; range, 66-233 cm), massive intestinal dilatation, and stasis. Of 9 patients with gastroschisis/atresia/dysmotility, 5 (56%) survived. All had surgery to "rescue" their dysfunction intestine, at mean age 128 days (range, 52-271 days): 4 had tapering enteroplasties of mean 37 cm (range, 5-115 cm)-all 4 survived; 3 had diverting stomas created for intestinal decompression, followed by stoma closure-1 survived; 1 nonsurvivor had a Kimura "patch"; 1 nonsurvivor underwent redo anastomosis. Survivors weaned off total parenteral nutrition at mean age 331 days after rescue surgery (range, 42-814 days).
  
  
  CONCLUSIONS
  More than one third of patients with GS/IA appear to have significant intestinal dysmotility without true SBS nor obstruction. Successful treatment of these infants may be achieved with the use of tapering enteroplasty and/or temporary diverting stomas.
authors.affiliation: Division of Pediatric Surgery, Department of Surgery, School of Medicine, University of North Carolina at Chapel Hill, Chapel Hill, NC 27599, USA. duncan_phillips@med.unc.edu
authors.forename: J Duncan
authors.initials: JD
authors.lastname: Phillips
authors.forename: Mehul V
authors.initials: MV
authors.lastname: Raval
authors.forename: Courtney
authors.initials: C
authors.lastname: Redden
authors.forename: Timothy M
authors.initials: TM
authors.lastname: Weiner
mesh_terms: D004750:Enteral Nutrition; D004766:Enterostomy; D005260:Female; D005769:Gastrointestinal Motility; D020139:Gastroschisis / Q000150:complications / Q000401:mortality / Q000601:surgery*; D017052:Hospital Mortality; D006801:Humans; D045823:Ileus / Q000209:etiology*; D007231:Infant, Newborn; D007234:Infant, Premature; D007235:Infant, Premature, Diseases / Q000401:mortality / Q000601:surgery; D007409:Intestinal Atresia / Q000209:etiology / Q000401:mortality / Q000601:surgery*; D007813:Laparotomy; D017093:Liver Failure / Q000209:etiology / Q000401:mortality; D008297:Male; D010289:Parenteral Nutrition, Total / Q000009:adverse effects; D012189:Retrospective Studies; D012778:Short Bowel Syndrome / Q000209:etiology / Q000401:mortality; D013577:Syndrome; D016896:Treatment Outcome