Abstract
Introduction To assess differences in initial symptoms, treatments, and bowel function between
children with Hirschsprung's disease (HD) with or without a cognitive dysfunction
(CD).
Materials and Methods The study included children with HD who underwent transanal endorectal pull-through.
A retrospective chart review was performed to collect data on patient characteristics,
diagnosis, and treatment. Data on bowel symptoms in children older than 4 years without
a colostomy were compiled during a cross-sectional, patient-reported follow-up.
Results Fifty-three children with HD were included; of these, 12 (23%) had CD. The median
birth weight was lower, frequency of vomiting as the presenting symptom was lower,
and time until the first contact with a pediatric surgeon was higher in children with
CD than in those without (3,295 vs. 3,623 g, p = 0.013; 28 vs. 66%, p = 0.02; and 4 days vs. 1 day, p = 0.048, respectively). At follow-up, 5 (15%) of 33 children aged over 4 years had
CD. More children without CD had some ability to hold back defecation and sense the
urge to defecate than those with CD (p = 0.002 and p = 0.001, respectively).
Conclusion HD children who have CD present with different initial symptoms, have a delay in
the first consultation with a pediatric surgeon, and experience poorer bowel function
outcomes than HD children without CD. Therefore, HD children with CD should receive
special attention in both clinical practice and research.
Keywords
Hirschsprung's disease - pediatrics - bowel function - cognitive dysfunction - transanal
endorectal pull-through - long-term outcome
