nutrition in short bowel syndrome in infancy

Ashrafi M. pediatric surgeon Alzahra Hospital Isfahan University of Medical sciences

 

Introduction: In the past, short-bowel syndrome in infancy was defined as having less than 75 cm of residual small intestine after bowel resection.^[65] Because the newborn small intestine is approximately 200 to 300 cm inlength,^[58] this correlates to a greater than 50% resection. Currently, the definition is a functional one (i.e., the presence of malabsorption following significant small intestinal resection).^[59] The prognosis of infants with significant small bowel resection was poor until the recent advent of parenteral nutrition.^[18] With the increased survival of these neonates, Most pediatric patients develop short-bowel syndrome during the neonatal period. The most common cause is necrotizing enterocolitis.^{[11] [59]} It has been estimated that approximately 20% of patients who have necrotizing enterocolitis and undergo surgical resection develop short-bowel syndrome.^[48] Other causes of short-bowel syndrome include gastroschisis, omphalocele, jejunal or ileal atresia, superior mesenteric artery deformities, and small bowel resections secondary to Hirschsprung's disease or volvulus. A rare entity of congenital short bowel, inwhich there is no evidence of a prenatal intestinal insult, has been reported inabout 30 patients.^[43] 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 inthese 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.

Matherial & Method: A retrospective chart review of infants who received TPN 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.

Results: 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 ina 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.