Morbidity and mortality of short-bowel syndrome in infants with abdominal wall defects.

Total parenteral nutrition (TPN) has made survival beyond infancy possible for many infants who have sustained small intestinal loss as a result of gastroschisis or omphalocele. The length and quality of life in these patients have often been limited by the development of late sequelae secondary both to the protracted use of TPN and the long-term complications of a shortened gut. This study was undertaken to determine what factors influence the morbidity and mortality of short-bowel syndrome (SBS) due to gastroschisis or omphalocele. A retrospective chart review of 850 infants who received TPN from January 1977 through December 1999 was carried out. All infants were treated at one academic medical center; those who had received > or =3 months of TPN were further segregated and their diagnosis, surgical procedures, length of bowel, ability to wean from TPN, follow-up weight and height, and developmental progress were recorded. Seventeen children were identified with SBS and either gastroschisis or omphalocele. Tight primary or secondary closure of the abdominal wall was believed to be a major cause of bowel necrosis and SBS in at least ten of the 17 patients. Overall survival was 76 per cent (13/17); survival was correlated with length of remaining bowel and was 86 per cent in patients having more than 15 cm of small bowel remaining but only 33 per cent in patients with less than 15 cm of small bowel remaining (P = 0.05). A longer length of residual small bowel resulted in a significantly shorter duration of TPN with a mean duration of 1.0 year for survivors having >38 cm and 10.0 years for survivors with <38 cm of bowel remaining (P = 0.03). Hepatic dysfunction with progressive failure resulting from TPN was related to death in three of the four nonsurvivors. The presence or absence of an ileocecal valve appeared unrelated both to the success of TPN weaning and to the length of time on TPN (P > 0.2). Eight of the 13 survivors have no ileocecal valve; five have undergone >50 per cent colonic resection. Nine of the survivors have adapted to enteral feedings (mean 36 +/- 60 months) during which time weaning from TPN occurred. The mean age of survivors is 7.9 +/- 5.1 years. Near-normal weights (defined as exceeding the fifth percentile for weight) were achieved for 92 per cent of the patients (12/13) with 46 per cent of the patients (6/13) exceeding the 50th percentile. Near-normal heights (exceeding the fifth percentile) were achieved for 77 per cent of the patients (10/13) with 15 per cent of the patients (2/13) exceeding the 50th percentile. Quality of life was measured on the basis of return to public school: nine of ten school-age survivors attend school and eight are normal without signs of developmental delay. Tight abdominal closure of gastroschisis or omphalocele may cause bowel necrosis and SBS. TPN has improved the long-term survival and quality of life in infants with SBS.