Does Delayed Diagnosis of Hirschsprung Disease Impact Post-operative and Functional Outcomes? A Multi-Center Review From the Pediatric Colorectal and Pelvic Learning Consortium

Sarah Ullrich, Colorectal Center, Cincinnati Children's Hospital, University of Cincinnati, Cincinnati, OH, USA.
Kelly Austin, Department of Surgery, Children's Hospital of Pittsburgh, University of Pittsburgh, Pittsburgh, PA, USA.
Jeffrey R. Avansino, Department of Surgery, Seattle Children's Hospital, University of Washington, Seattle, WA, USA.
Andrea Badillo, Division of Colorectal and Pelvic Reconstruction, Children's National Hospital, George Washington University, Washington, DC, USA.
Casey M. Calkins, Department of Surgery, Children's Wisconsin, Medical College of Wisconsin, Milwaukee, WI, USA.
Rachel C. Crady, Department of Pediatrics, University of Utah, Salt Lake City, UT, USA.
Megan M. Durham, Department of Surgery, Children's Healthcare of Atlanta, Emory University, Atlanta, GA, USA.
Megan K. Fuller, Department of Surgery, Boys Town National Research Hospital, University of Nebraska Medical Center, Boys Town, NE, USA.
Ankur Rana, Department of Surgery and Perioperative Care, Dell Children's Medical Center, University of Texas Austin, Austin, TX, USA.
Ron W. Reeder, Department of Pediatrics, University of Utah, Salt Lake City, UT, USA.
Rebecca M. Rentea, Department of Surgery, Children's Mercy Hospital, University of Missouri-Kansas City, Kansas City, MO, USA.
Michael D. Rollins, Department of Surgery, Primary Children's Hospital, University of Utah, Salt Lake City, UT, USA.
Payam Saadai, Department of Surgery, UC Davis Children's Hospital, University of California Davis, Davis, CA, USA.
K Elizabeth Speck, Section of Pediatric Surgery, C.S. Mott Children's Hospital, University of Michigan, Ann Arbor, MI, USA.
Richard J. Wood, Department of Surgery, Nationwide Children's Hospital, The Ohio State University, Columbus, OH, USA.
Kathleen van Leeuwen, Department of Surgery, Phoenix Children's Hospital, University of Arizona, Phoenix, AZ, USA.
Jason S. Frischer, Colorectal Center, Cincinnati Children's Hospital, University of Cincinnati, Cincinnati, OH, USA. Electronic address: Jason.Frischer@cchmc.org.

Document Type

Journal Article

Publication Date

7-1-2024

Journal

Journal of pediatric surgery

Volume

59

Issue

7

DOI

10.1016/j.jpedsurg.2024.03.034

Keywords

Functional incontinence; Hirschsprung disease; Postoperative outcomes; Pull-through

Abstract

BACKGROUND: Hirschsprung Disease (HD) is a rare cause of functional bowel obstruction in children. Patients are typically diagnosed in the neonatal period and undergo pull-through (PT) soon after diagnosis. The optimal management and post-operative outcomes of children who present in a delayed fashion are unknown. METHODS: A multi-center retrospective review of children with HD was performed at participating Pediatric Colorectal and Pelvic Learning Consortium sites. Children were stratified by age at diagnosis (neonates <29 days; infants 29 days-12 months; toddler 1 year-5 years and child >5 years). RESULTS: 679 patients with HD from 14 sites were included; Most (69%) were diagnosed in the neonatal period. Age at diagnosis was not associated with differences in 30-day complication rates or need for PT revision. Older age at diagnosis was associated with a greater likelihood of undergoing fecal diversion after PT (neonate 10%, infant 12%, toddler 26%, child 28%, P < 0.001) and a greater need for intervention for constipation or incontinence postoperatively (neonate 56%, infant 62%, toddler 78%, child 69%, P < 0.001). CONCLUSION: Delayed diagnosis of HD does not impact 30-day post-operative outcomes or need for revision surgery but, delayed diagnosis is associated with increased need for fecal diversion after pull-through. LEVEL OF EVIDENCE: III.

Department

Surgery

Link to Full Text

Share

COinS