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

Akemi L Kawaguchi; Yigit S Guner; Stig Sømme; Alexandria C Quesenberry; L Grier Arthur; Juan E Sola; Cynthia D Downard; Rebecca M Rentea; Patricia A Valusek; Caitlin A Smith; Mark B Slidell; Robert L Ricca; Roshni Dasgupta; Elizabeth Renaud; Doug Miniati; Jarod McAteer; Alana L Beres; Julia Grabowski; Shawn D St Peter; Ankush Gosain; American Pediatric Surgical Association Outcomes and Evidence-Based Practice (OEBP) Committee

doi:10.1016/j.jpedsurg.2021.03.046

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

J Pediatr Surg. 2021 Sep;56(9):1513-1523. doi: 10.1016/j.jpedsurg.2021.03.046. Epub 2021 Mar 28.

Authors

Akemi L Kawaguchi¹, Yigit S Guner², Stig Sømme³, Alexandria C Quesenberry⁴, L Grier Arthur⁵, Juan E Sola⁶, Cynthia D Downard⁷, Rebecca M Rentea⁸, Patricia A Valusek⁹, Caitlin A Smith¹⁰, Mark B Slidell¹¹, Robert L Ricca¹², Roshni Dasgupta¹³, Elizabeth Renaud¹⁴, Doug Miniati¹⁵, Jarod McAteer¹⁶, Alana L Beres¹⁷, Julia Grabowski¹⁸, Shawn D St Peter¹⁹, Ankush Gosain²⁰; American Pediatric Surgical Association Outcomes and Evidence-Based Practice (OEBP) Committee

Affiliations

¹ Department of Pediatric Surgery, University of Texas Health Science Center at Houston, Houston, TX, USA. Electronic address: akemi.kawaguchi@uth.tmc.edu.
² Department of Surgery University of California Irvine and Division of Pediatric Surgery Children's Hospital of Orange County, USA.
³ Division of Pediatric Surgery, Children's Hospital Colorado, University of Colorado, Aurora, CO, USA.
⁴ Health Sciences Library, University of Tennessee Health Science Center, USA.
⁵ Division of Pediatric General, Thoracic, and Minimally Invasive Surgery, St. Christopher's Hospital for Children, Philadelphia, PA, USA.
⁶ Division of Pediatric Surgery, University of Miami Miller School of Medicine, Miami, FL, USA.
⁷ Division of Pediatric Surgery, Hiram C. Polk, Jr, MD Department of Surgery, University of Louisville, Louisville, KY, USA.
⁸ Department of Pediatric Surgery, Children's Mercy-Kansas City, Kansas City, MO, USA.
⁹ Pediatric Surgical Associates, Children's Minnesota, Minneapolis, MN, USA.
¹⁰ Division of Pediatric General and Thoracic Surgery, Seattle Children's Hospital, University of Washington School of Medicine, Seattle, WA, USA.
¹¹ Section of Pediatric Surgery, Comer Children's Hospital, The University of Chicago Medicine, Chicago, Illinois, USA.
¹² Division of Pediatric Surgery, Naval Medical Center, Portsmouth, VA, USA.
¹³ Division of Pediatric General and Thoracic Surgery, Cincinnati Childrens Medical Center, University of Cincinnati, Cincinnati OH, USA.
¹⁴ Division of Pediatric Surgery, Alpert Medical School of Brown University, Hasbro Children's Hospital, Rhode Island Hospital, Providence, RI, USA.
¹⁵ Division of Pediatric Surgery, Kaiser Permanente Roseville Women and Children's Center, Roseville, California, USA.
¹⁶ Alaska Pediatric Surgery, Anchorage, AK, USA.
¹⁷ Division of Pediatric General, Thoracic and Fetal Surgery, University of California, Davis, Sacramento CA, USA.
¹⁸ Division of Pediatric Surgery, Ann and Robert H. Lurie Children's Hospital, Northwestern University, Chicago, IL long, USA.
¹⁹ Department of Surgery, Children's Mercy Hospital, Kansas City, MO, USA.
²⁰ 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.

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.

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

Publication types

Review
Systematic Review

MeSH terms

Enterocolitis*
Evidence-Based Practice
Hirschsprung Disease* / surgery
Humans
Prospective Studies
Quality of Life

Abstract

Publication types

MeSH terms

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