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

Authors

• 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.

APA Citation

Ullrich, Sarah; Austin, Kelly; Avansino, Jeffrey R.; Badillo, Andrea; Calkins, Casey M.; Crady, Rachel C.; Durham, Megan M.; Fuller, Megan K.; Rana, Ankur; Reeder, Ron W.; Rentea, Rebecca M.; Rollins, Michael D.; Saadai, Payam; Speck, K Elizabeth; Wood, Richard J.; van Leeuwen, Kathleen; and Frischer, Jason S., "Does Delayed Diagnosis of Hirschsprung Disease Impact Post-operative and Functional Outcomes? A Multi-Center Review From the Pediatric Colorectal and Pelvic Learning Consortium" (2024). GW Authored Works. Paper 5390.
https://hsrc.himmelfarb.gwu.edu/gwhpubs/5390

Department

Surgery