The advent of total parenteral nutrition (TPN) has made survival beyond infancy possible for large numbers of patients who have sustained massive small intestinal loss due to a variety of intraabdominal catastrophes. However, the quantity and quality of life have been limited by the development of late sequalae due both to the protracted use of TPN and the long-term complications of foreshortening of the gut. To determine to what extent the morbidity and mortality of short-bowel syndrome (SBS) may have improved over the last 10 years, we reviewed our experience since 1973 with patients losing more than 50% of total small intestinal mass in infancy. The etiologies of SBS in the 16 study patients were necrotizing enterocolitis (6), midgut volvulus (5), multiple atresias (3), gastroschisis (1), and congenital SBS (1). Overall survival was 81%; total small intestinal length (SIL) at the time of diagnosis was 44.2±7.9 cm in survivors and 30.3±7.8 cm in nonsurvivors, probability values not significant. Although no patient survived without an ileocecal valve whose total SIL was greater than 20 cm, the three deaths in this series were not related directly to the SIL, but to end-stage liver disease resulting from TPN-associated cholestasis. Among the survivors, adaptation to enteral feedings required 13.8±2.5 mo, during which time weaning from TPN occurred; weight at adaptation was 6.87±1.32 kg. A mean follow up of 3.3±0.8 years permitted assessment of quality of life; the mean score was 3.0±0.3 on an arbitrary scale (excellent=4, good=3, fair=2, poor=1), while the mean number of stools per diem was 3.1±0.7. Finally, near-normal somatic growth was achieved in survivors weaned from TPN in that mean weight (W), height (H), and weight-for-height (W/H) all approached or exceeded the 25th percentile (W=26th percentile, H=24th percentile, W/H=41st percentile). We conclude that the long-term outlook for most patients with SBS resulting from massive small intestinal loss in infancy is good, although TPN-associated cholestasis may lead to end stage liver disease and death in a minority of patients.
- Short bowel syndrome
- total parenteral nutrition
ASJC Scopus subject areas
- Pediatrics, Perinatology, and Child Health