What parents need to know about Hirschsprung disease

Semin Pediatr Surg. 2022 Apr;31(2):151159. doi: 10.1016/j.sempedsurg.2022.151159. Epub 2022 Apr 8.

Abstract

Hirschsprung's disease (HD) is a complex colorectal disorder that can be challenging to understand and overwhelming to many parents. Counseling parents is critical for ensuring they understand their child's condition, how it must be treated, pitfalls that can occur during treatment, and how they will do in the long term. This article breaks down this process into a series of informally worded questions and reviews evidence-based discussion points around the most common concerns parents will have: survival, pathophysiology, surgical correction and complications, etiology and genetic basis, associated issues, outcomes, and familial nature. Where evidence is unclear, expert opinion is included. Discussion is geared towards the parents of a newly diagnosed and otherwise healthy neonate, with a brief section on complex situations including associated syndromes and late diagnosis. Embarking on the longitudinal care of a child with HD is a complex undertaking and requires significant parental education to establish a proper parent-surgeon relationship, an essential factor for optimizing outcome and family satisfaction.

Keywords: Aganglionosis; Complications; Enterocolitis; Hirschsprung; Outcomes; Pull-through.

MeSH terms

  • Child
  • Hirschsprung Disease* / diagnosis
  • Hirschsprung Disease* / surgery
  • Humans
  • Infant, Newborn
  • Parents