Adaptation in short-bowel syndrome: Reassessing old limits

Abstract

The improving survival of patients with severe short-bowel syndrome along with the advent of successful intestinal transplantation have accentuated the need to answer two questions. (1) Is there an intestinal length below which adaptation to full enteral nutrition can not be expected to occur? (2) How much time is necessary to complete intestinal adaptation? We reviewed the outcome of 21 infants with less than 50 cm of small intestine to answer these questions. Patients were divided into three groups based on intestinal length, regardless of ileocecal valve status: group I, <10 cm (n = 3); group II, 10 to 30 cm (n = 11); and group III, 30 to 50 cm (n = 7). Data were collected to assess survival, incidence of adaptation, time to adaptation, and causes of mortality. Infants in group I did not achieve intestinal adaptation to full enteral nutrition. One survived and 2 died, one from varicella pneumonia and the other after intestinal transplantation. Eight of the 11 (73%) patients in group II survived and 5 of 8 (63%) survivors achieved full intestinal adaptation after a mean interval of 320 days (range, 148 to 506 days) on parenteral nutrition. Six of the seven patients (86%) in group III survived and all survivors (100%) achieved complete enteral adaptation after an average of 376 days (range, 58 to 727 days). The overall survival was 71% ( 15 21 ), but survival in patients with > 10 cm was 78%. The incidence of fatal catheter sepsis ( 2 21 ) and fatal parenteral nutrition-associated liver failure ( 1 21 ) was low and accounted for two of the six deaths—one patient died as a consequence of both complications. The 10-cm small intestinal length appears to be a new lower limit for the length necessary to achieve enteral nutrition. Patients with > 10 cm of small intestine should be supported on parenteral nutrition with early institution of enteral feedings to promote intestinal adaptation for a minimum of 2 years before transplantation is considered.