Abstract
Over a 16-year period, 20 neonates and infants with short-bowel syndrome underwent longitudinal intestinal lengthening and tailoring because of a dysfunctional dilated jejunum. There was no operative mortality, and morbidity was limited to 2 hemiloop anastomotic stenoses and 1 spontaneously resolving air and bile leak. Long-term survival was 45%. Survivors had >40 cm residual jejunum and a greater number also retained their ileocaecal valve and a longer colonic length. They underwent bowel lengthening at a later time and had minimal hepatic dysfunction. 7 of 9 survivors established full enteral nutrition. These children could be regarded as self-selected survivors with residual bowel dysfunction who had come through the hazardous neonatal phase with minimal hepatic injury. Non-survivors often had <40 cm jejunum and limited distal colon. Death was commonly due to end-stage liver failure. It is likely that the severely reduced gut-associated lymphoid tissue contributed to increased bacterial translocation from the dilated bowel and early onset of progressive liver injury. It is possible to conclude that bowel lengthening should be offered only to self-selected survivors with residual bowel dysfunction and minimal liver injury. It seems, however, even more appropriate, to offer early bowel tailoring and lengthening with its recognized reduction in stasis and bacterial translocation, improved absorption and enhanced intestinal adaption, particularly to those high-risk neonates with <40 cm of dilated jejunum with a view to reducing the risk of infection and lethal hepatic injury, thereby improving their chances for quality survival.