Format

Send to

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

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

Author information

1
Center for Advanced Intestinal Rehabilitation, Department of Surgery; Boston Children's Hospital and Harvard Medical School, Boston, MA, USA.
2
Department of Surgery; Boston Children's Hospital and Harvard Medical School, Boston, MA, USA.
3
Center for Advanced Intestinal Rehabilitation, Center for Nutrition, Division of Gastroenterology, Hepatology, and Nutrition; Boston Children's Hospital and Harvard Medical School, Boston, MA, USA.
4
Center for Advanced Intestinal Rehabilitation, Department of Surgery; Boston Children's Hospital and Harvard Medical School, Boston, MA, USA. Electronic address: tom.jaksic@childrens.harvard.edu.

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.

KEYWORDS:

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

PMID:
30826119
DOI:
10.1016/j.jpedsurg.2019.01.025
[Indexed for MEDLINE]

Supplemental Content

Full text links

Icon for Elsevier Science
Loading ...
Support Center