Does Delayed Diagnosis of Hirschsprung Disease Impact Post-operative and Functional Outcomes? A Multi-Center Review

Sarah Ullrich1, Kelly Austin2, Jeffrey R Avansino3

  • 1Colorectal Center, Cincinnati Children's Hospital, University of Cincinnati, Cincinnati, OH, USA.

PubMed

Insights

Delayed diagnosis of Hirschsprung disease (HD) does not affect short-term surgical outcomes. However, later diagnosis increases the need for fecal diversion and interventions for constipation or incontinence after pull-through surgery.

Area of Science:

  • Pediatric Surgery
  • Gastroenterology
  • Clinical Outcomes Research

Background:

  • Hirschsprung disease (HD) is a rare congenital disorder causing intestinal obstruction in children.
  • Diagnosis typically occurs neonatally, followed by pull-through (PT) surgery.
  • Optimal management and outcomes for delayed HD diagnosis remain unclear.

Purpose of the Study:

  • To evaluate the impact of age at diagnosis on post-operative outcomes in children with Hirschsprung disease.
  • To compare complication rates, need for revision surgery, and long-term functional outcomes based on diagnostic timing.

Main Methods:

  • Multi-center retrospective review of 679 pediatric patients with HD.
  • Stratification by age at diagnosis: neonates (<29 days), infants (29 days-12 months), toddlers (1-5 years), and children (>5 years).

Main Results:

  • Age at diagnosis did not influence 30-day complication rates or need for PT revision.
  • Older age at diagnosis correlated with increased fecal diversion post-PT (28% in children vs. 10% in neonates).
  • Delayed diagnosis was associated with higher rates of interventions for constipation/incontinence postoperatively.

Conclusions:

  • Delayed diagnosis of Hirschsprung disease does not adversely affect immediate post-operative outcomes or revision rates.
  • However, delayed diagnosis is linked to a greater need for fecal diversion and subsequent interventions for bowel dysfunction.
Abstract