Outcome and intestinal adaptation in neonatal short-bowel syndrome

We reviewed 52 consecutive patients with short-bowel syndrome (SBS) treated with long-term parenteral nutrition (PN) from 1978 through 1990. The SBS etiologies included necrotizing enterocolitis (NEC) in 26 patients (50%), abdominal wall defects in 11 (22%), jejunoileal atresia in 6 (12%), midgut volvulus in 4 (8%), Hirschsprung's disease in 3 (6%), and segmental volvulus and cloacal exstrophy in 1 (2%) each. The average initial small bowel length was 48.1 cm, and only 31% of the patients retained an ileocecal valve (ICV). The mean duration of PN therapy was 16.6 months, and 39 patients (75%) were successfully weaned from it. Forty-three patients (83%) survived. Significant differences between the initial 20 patients treated from 1978 through 1984 and the next 32 from 1985 through 1990 were duration of PN-therapy (25.1 v 11.4 months; P = .04), incidence of PN-associated jaundice (80% v 31%; P = .001), and survival (65% v 94%; P = .02). NEC patients had a significantly lower mean birth-weight than those with other etiologies (mean, 1,367 v 2,544 g; P < .0001) but did not differ in initial small bowel length, ICV retention rate, duration of PN treatment, incidence of successful PN weaning, or outcome. The presence of an ICV did not correlate with successful PN weaning but did affect the mean duration of PN therapy (7.2 months with ICV v 21.6 months without; P = .03). Patients with 40 cm or less of initial small bowel spent a longer time on PN (24.1 v 10.6 months; P = .04) and had a lower weaning rate from it (57% v 88%; P = .02) than did those with more small bowel. The ratio of initial small bowel length to birthweight did not correlate with the duration of PN therapy in survivors successfully weaned from PN. We conclude that the outcome and morbidity of patients with SBS on long-term PN has improved. In addition, the absence of an ICV prolongs the length of PN therapy but does not determine successful weaning from it.