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

Authors:
Charles R Hong
Charles R Hong
Center for Advanced Intestinal Rehabilitation
Boston | United States
Sam M Han
Sam M Han
Center for Advanced Intestinal Rehabilitation
Alexandra N Carey
Alexandra N Carey
Cincinnati Children's Hospital Medical Center (CCHMC)
Cincinnati | United States
Biren P Modi
Biren P Modi
Boston Children's Hospital and Harvard Medical School
United States
Tom Jaksic
Tom Jaksic
Harvard Medical School
United States

J Pediatr Surg 2019 May 2;54(5):964-967. Epub 2019 Feb 2.

Center for Advanced Intestinal Rehabilitation, Department of Surgery; Boston Children's Hospital and Harvard Medical School, Boston, MA, USA. Electronic address:

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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Source
http://dx.doi.org/10.1016/j.jpedsurg.2019.01.025DOI Listing
May 2019
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