Management and Outcomes for Long-Segment Hirschsprung Disease: A Systematic Review from the APSA Outcomes and Evidence Based Practice Committee
jpedsurg.org shows its articles on its own site.
Read the article on jpedsurg.org ↗Article · Mar 2021 · 1 min read
In brief
In brief
Systematic review by APSA examining long-segment Hirschsprung disease (LSHD), finding no consensus on optimal surgical approach but identifying increased risk of enterocolitis and long-term GI symptoms compared to short-segment disease. Authors recommend standardized anatomic definitions and highlight stem cell therapy as a potential future treatment avenue.
Written by the GCMD Library team from the article.
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.