Management and outcomes for long-segment Hirschsprung disease: A systematic review from the APSA outcomes and evidence based practice committee.

Document Type

Article

Publication Date

9-1-2021

Identifier

DOI: 10.1016/j.jpedsurg.2021.03.046

Abstract

OBJECTIVE: Long-Segment Hirschsprung Disease (LSHD) differs clinically from short-segment disease. This review article critically appraises current literature on the definition, management, outcomes, and novel therapies for patients with LSHD.

METHODS: Four questions regarding the definition, management, and outcomes of patients with LSHD were generated. English-language articles published between 1990 and 2018 were compiled by searching PubMed, Scopus, Cochrane Central Register of Controlled Trials, Web of Science, and Google Scholar. A qualitative synthesis was performed.

RESULTS: 66 manuscripts were included in this systematic review. Standardized nomenclature and preoperative evaluation for LSHD are recommended. Insufficient evidence exists to recommend a single method for the surgical repair of LSHD. Patients with LSHD may have increased long-term gastrointestinal symptoms, including Hirschsprung-associated enterocolitis (HAEC), but have a quality of life similar to matched controls. There are few surgical technical innovations focused on this disorder.

CONCLUSIONS: A standardized definition of LSHD is recommended that emphasizes the precise anatomic location of aganglionosis. Prospective studies comparing operative options and long-term outcomes are needed. Translational approaches, such as stem cell therapy, may be promising in the future for the treatment of long-segment Hirschsprung disease.

Journal Title

Journal of pediatric surgery

Volume

56

Issue

9

First Page

1513

Last Page

1523

Keywords

Aganglionosis; Hirschsprung; Level 3; Level 4; Level of Evidence; Long-segment; Outcome; Surgery

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