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

J Pediatr Surg. 2019 May;54(5):964-967. doi: 10.1016/j.jpedsurg.2019.01.025. Epub 2019 Feb 2.


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.

Keywords: Intestinal failure; Intestinal rehabilitation; Intestinal transplantation; Midgut volvulus; Parenteral nutrition; Ultrashort bowel syndrome.

MeSH terms

  • Adolescent
  • Child
  • Child, Preschool
  • Female
  • Follow-Up Studies
  • Gastroschisis / complications*
  • Humans
  • Infant
  • Infant, Newborn
  • Intestinal Volvulus / complications*
  • Intestines / transplantation
  • Male
  • Parenteral Nutrition
  • Pediatrics
  • Prognosis
  • Rehabilitation Centers
  • Retrospective Studies
  • Short Bowel Syndrome / etiology*
  • Short Bowel Syndrome / therapy*
  • Time Factors