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  • Title: Long-term outcomes of ultrashort bowel syndrome due to malrotation with midgut volvulus managed at an interdisciplinary pediatric intestinal rehabilitation center.
    Author: Hong CR, Han SM, Staffa SJ, Carey AN, Modi BP, Jaksic T.
    Journal: J Pediatr Surg; 2019 May; 54(5):964-967. PubMed ID: 30826119.
    Abstract:
    PURPOSE: The 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. METHODS: Patients 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). RESULTS: Twenty-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. CONCLUSION: Midgut 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 STUDY: Prognosis study. LEVEL OF EVIDENCE: IIb, retrospective cohort study.
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