Gastroschisis, atresia, dysmotility: surgical treatment strategies for a distinct clinical entity.

PURPOSE

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.