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Accepted (AM)Open Access (License Unspecified), Open
Journal article
Management and outcomes for long-segment Hirschsprung disease: A systematic review from the APSA Outcomes and Evidence Based Practice Committee
Journal of pediatric surgery, v 56(9), pp 1513-1523
01 Sep 2021
PMID: 33993978
Featured in Collection : UN Sustainable Development Goals @ Drexel
Abstract
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.
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.
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.
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.
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Details
- Title
- Management and outcomes for long-segment Hirschsprung disease: A systematic review from the APSA Outcomes and Evidence Based Practice Committee
- Creators
- Akemi L. Kawaguchi - The University of Texas Health Science Center at HoustonYigit S. Guner - Children's Hospital of Orange CountyStig Sømme - University of Colorado Anschutz Medical CampusAlexandria C. Quesenberry - University of Tennessee Health Science CenterL. Grier Arthur - St. Christopher's Hospital for ChildrenJuan E. Sola - University of MiamiCynthia D. Downard - University of LouisvilleRebecca M. Rentea - Children's Mercy HospitalPatricia A. Valusek - Children's MinnesotaCaitlin A. Smith - Seattle Children's HospitalMark B. Slidell - Comer Children's HospitalRobert L. Ricca - Naval Medical Center PortsmouthRoshni Dasgupta - Children's Medical CenterElizabeth Renaud - Hasbro Children's HospitalDoug Miniati - Kaiser Permanente Roseville Medical CenterJarod McAteer - AnchorageAlana L. Beres - University of California, DavisJulia Grabowski - Northwestern UniversityShawn D.St Peter - Children's Mercy HospitalAnkush Gosain - University of Tennessee Health Science CenterAmerican Pediatric Surgical Association Outcomes and Evidence-Based Practice (OEBP) Committee
- Publication Details
- Journal of pediatric surgery, v 56(9), pp 1513-1523
- Publisher
- Elsevier
- Number of pages
- 11
- Resource Type
- Journal article
- Language
- English
- Academic Unit
- Pediatrics; Surgery
- Web of Science ID
- WOS:000687303900009
- Scopus ID
- 2-s2.0-85105794803
- Other Identifier
- 991021969474504721
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- Collaboration types
- Domestic collaboration
- Web of Science research areas
- Pediatrics
- Surgery