Management and outcomes for long-segment Hirschsprung disease: A systematic review from the APSA Outcomes and Evidence Based Practice Committee.

Enterocolitis Evidence-Based Practice Hirschsprung Disease / surgery Humans Prospective Studies Quality of Life
Aganglionosis Hirschsprung Level 3 Level 4 Level of Evidence Long-segment Outcome Surgery

Journal

Journal of pediatric surgery
ISSN: 1531-5037
Titre abrégé: J Pediatr Surg
Pays: United States
ID NLM: 0052631

Informations de publication

Date de publication:
Sep 2021
Historique:
received: 21 07 2020
revised: 11 03 2021
accepted: 15 03 2021
pubmed: 18 5 2021
medline: 25 8 2021
entrez: 17 5 2021
Statut: ppublish

Résumé

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.

Identifiants

DOI: 10.1016/j.jpedsurg.2021.03.046 PMID: 33993978 PMC: PMC8552809
pubmed: 33993978
pii: S0022-3468(21)00280-3
doi: 10.1016/j.jpedsurg.2021.03.046
pmc: PMC8552809
mid: NIHMS1748482
pii:
doi:

Types de publication

Journal Article Review Systematic Review

Langues

eng

Sous-ensembles de citation

IM

Pagination

1513-1523

Subventions

Organisme : NIDDK NIH HHS
ID : K08 DK098271
Pays : United States
Organisme : NIDDK NIH HHS
ID : R01 DK125047
Pays : United States
Organisme : NIDDK NIH HHS
ID : R03 DK114543
Pays : United States

Informations de copyright

Copyright © 2021 Elsevier Inc. All rights reserved.

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Auteurs

Akemi L Kawaguchi (AL)

Department of Pediatric Surgery, University of Texas Health Science Center at Houston, Houston, TX, USA. Electronic address: akemi.kawaguchi@uth.tmc.edu.

Yigit S Guner (YS)

Department of Surgery University of California Irvine and Division of Pediatric Surgery Children's Hospital of Orange County, USA.

Stig Sømme (S)

Division of Pediatric Surgery, Children's Hospital Colorado, University of Colorado, Aurora, CO, USA.

Alexandria C Quesenberry (AC)

Health Sciences Library, University of Tennessee Health Science Center, USA.

L Grier Arthur (LG)

Division of Pediatric General, Thoracic, and Minimally Invasive Surgery, St. Christopher's Hospital for Children, Philadelphia, PA, USA.

Juan E Sola (JE)

Division of Pediatric Surgery, University of Miami Miller School of Medicine, Miami, FL, USA.

Cynthia D Downard (CD)

Division of Pediatric Surgery, Hiram C. Polk, Jr, MD Department of Surgery, University of Louisville, Louisville, KY, USA.

Rebecca M Rentea (RM)

Department of Pediatric Surgery, Children's Mercy-Kansas City, Kansas City, MO, USA.

Patricia A Valusek (PA)

Pediatric Surgical Associates, Children's Minnesota, Minneapolis, MN, USA.

Caitlin A Smith (CA)

Division of Pediatric General and Thoracic Surgery, Seattle Children's Hospital, University of Washington School of Medicine, Seattle, WA, USA.

Mark B Slidell (MB)

Section of Pediatric Surgery, Comer Children's Hospital, The University of Chicago Medicine, Chicago, Illinois, USA.

Robert L Ricca (RL)

Division of Pediatric Surgery, Naval Medical Center, Portsmouth, VA, USA.

Roshni Dasgupta (R)

Division of Pediatric General and Thoracic Surgery, Cincinnati Childrens Medical Center, University of Cincinnati, Cincinnati OH, USA.

Elizabeth Renaud (E)

Division of Pediatric Surgery, Alpert Medical School of Brown University, Hasbro Children's Hospital, Rhode Island Hospital, Providence, RI, USA.

Doug Miniati (D)

Division of Pediatric Surgery, Kaiser Permanente Roseville Women and Children's Center, Roseville, California, USA.

Jarod McAteer (J)

Alaska Pediatric Surgery, Anchorage, AK, USA.

Alana L Beres (AL)

Division of Pediatric General, Thoracic and Fetal Surgery, University of California, Davis, Sacramento CA, USA.

Julia Grabowski (J)

Division of Pediatric Surgery, Ann and Robert H. Lurie Children's Hospital, Northwestern University, Chicago, IL long, USA.

Shawn D St Peter (SDS)

Department of Surgery, Children's Mercy Hospital, Kansas City, MO, USA.

Ankush Gosain (A)

Division of Pediatric Surgery, Department of Surgery, University of Tennessee Health Science Center, Children's Foundation Research Institute, Le Bonheur Children's Hospital, Memphis, TN, USA. Electronic address: agosain@uthsc.edu.

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Classifications MeSH

questionsmedicales.fr › Maladies de l'appareil digestif › Maladies gastro-intestinales › Maladies intestinales › Entérocolite › Management and outcomes for long-segment...
questionsmedicales.fr › Professions de santé › Pratique factuelle › Management and outcomes for long-segment...
questionsmedicales.fr › Malformations et maladies congénitales, héréditaires et néonatales › Malformations › Malformations de l'appareil digestif › Maladie de Hirschsprung › Management and outcomes for long-segment...
questionsmedicales.fr › Eucaryotes › Animaux › Chordés › Vertébrés › Mammifères › Eutheria › Primates › Haplorhini › Catarrhini › Hominidae › Humains › Management and outcomes for long-segment...
questionsmedicales.fr › Environnement et santé publique › Santé publique › Méthodes épidémiologiques › Caractéristiques des études épidémiologiques › Études épidémiologiques › Études de cohortes › Études prospectives › Management and outcomes for long-segment...
questionsmedicales.fr › Environnement et santé publique › Santé publique › Mesures épidémiologiques › Démographie › État de santé › Qualité de vie › Management and outcomes for long-segment...