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

American Pediatric Surgical Association Outcomes and Evidence-Based Practice (OEBP) Committee

Research output: Contribution to journalReview articlepeer-review

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.

Original languageEnglish (US)
JournalJournal of Pediatric Surgery
DOIs
StateAccepted/In press - 2021

Keywords

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

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health
  • Surgery

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