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; Kelly Austin; Jeffrey R Avansino; Andrea Badillo; Casey M Calkins; Rachel C Crady; Megan M Durham; Megan K Fuller; Ankur Rana; Ron W Reeder; Rebecca M Rentea; Michael D Rollins; Payam Saadai; K Elizabeth Speck; Richard J Wood; Kathleen van Leeuwen; Jason S Frischer; Pediatric Colorectal and Pelvic Learning Consortium

doi:10.1016/j.jpedsurg.2024.03.034

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

J Pediatr Surg. 2024 Mar 18:S0022-3468(24)00186-6. doi: 10.1016/j.jpedsurg.2024.03.034. Online ahead of print.

Authors

Sarah Ullrich¹, Kelly Austin², Jeffrey R Avansino³, Andrea Badillo⁴, Casey M Calkins⁵, Rachel C Crady⁶, Megan M Durham⁷, Megan K Fuller⁸, Ankur Rana⁹, Ron W Reeder⁶, Rebecca M Rentea¹⁰, Michael D Rollins¹¹, Payam Saadai¹², K Elizabeth Speck¹³, Richard J Wood¹⁴, Kathleen van Leeuwen¹⁵, Jason S Frischer¹⁶; Pediatric Colorectal and Pelvic Learning Consortium

Affiliations

¹ Colorectal Center, Cincinnati Children's Hospital, University of Cincinnati, Cincinnati, OH, USA.
² Department of Surgery, Children's Hospital of Pittsburgh, University of Pittsburgh, Pittsburgh, PA, USA.
³ Department of Surgery, Seattle Children's Hospital, University of Washington, Seattle, WA, USA.
⁴ Division of Colorectal and Pelvic Reconstruction, Children's National Hospital, George Washington University, Washington, DC, USA.
⁵ Department of Surgery, Children's Wisconsin, Medical College of Wisconsin, Milwaukee, WI, USA.
⁶ Department of Pediatrics, University of Utah, Salt Lake City, UT, USA.
⁷ Department of Surgery, Children's Healthcare of Atlanta, Emory University, Atlanta, GA, USA.
⁸ Department of Surgery, Boys Town National Research Hospital, University of Nebraska Medical Center, Boys Town, NE, USA.
⁹ Department of Surgery and Perioperative Care, Dell Children's Medical Center, University of Texas Austin, Austin, TX, USA.
¹⁰ Department of Surgery, Children's Mercy Hospital, University of Missouri-Kansas City, Kansas City, MO, USA.
¹¹ Department of Surgery, Primary Children's Hospital, University of Utah, Salt Lake City, UT, USA.
¹² Department of Surgery, UC Davis Children's Hospital, University of California Davis, Davis, CA, USA.
¹³ Section of Pediatric Surgery, C.S. Mott Children's Hospital, University of Michigan, Ann Arbor, MI, USA.
¹⁴ Department of Surgery, Nationwide Children's Hospital, The Ohio State University, Columbus, OH, USA.
¹⁵ Department of Surgery, Phoenix Children's Hospital, University of Arizona, Phoenix, AZ, USA.
¹⁶ Colorectal Center, Cincinnati Children's Hospital, University of Cincinnati, Cincinnati, OH, USA. Electronic address: Jason.Frischer@cchmc.org.

PMID: 38677965
DOI: 10.1016/j.jpedsurg.2024.03.034

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.

Keywords: Functional incontinence; Hirschsprung disease; Postoperative outcomes; Pull-through.