Long-term outcomes of ultrashort bowel syndrome due to malrotation with midgut volvulus managed at an interdisciplinary pediatric intestinal rehabilitation center

Abstract

PurposeThe purpose of this study was to describe long-term outcomes of pediatric-onset ultrashort bowel syndrome owing to midgut volvulus managed at an interdisciplinary intestinal rehabilitation center. MethodsPatients with a history of malrotation and pediatric-onset midgut volvulus causing extensive bowel loss (<20% residual small bowel length expected for postconception age) and treated between 2010 and 2017 were reviewed. Data are expressed as median (IQR). ResultsTwenty-three patients had midgut volvulus at age 1 (0–21) day leading to 9 (8–12) percent predicted residual bowel length. Eight (35%) had gastroschisis. Follow-up was 8.5 (6.6–12.2) years from volvulus. Five (22%) patients underwent intestinal/multivisceral transplantation, and all achieved enteral autonomy. Eighteen (78%) patients remained transplant-free, 7 of whom achieved enteral autonomy after 718 (682–1030) days of parenteral nutrition. Transplant-free enteral autonomy was achieved by 0/6 patients with gastroschisis, compared to 7/12 without gastroschisis (p = 0.04). For the overall group, 18 (78%) patients had small bowel bacterial overgrowth, and 7 manifested symptomatic D-lactic acidosis. We observed 2 mortalities, one awaiting transplant and one 4 years following transplantation. ConclusionMidgut volvulus owing to malrotation with extensive bowel loss is associated with favorable long-term survival. Transplant-free enteral autonomy may be feasible, particularly in the absence of gastroschisis. Type of studyPrognosis study. Level of evidenceIIb, retrospective cohort study.