Clinical Characteristics and Postoperative Functional Outcomes in Children With Mowat-Wilson Syndrome and Hirschsprung's Disease: A Single-center Study

jpedsurg.org

Mowat-Wilson Syndrome (MWS) is a rare autosomal dominant genetic disorder. Approximately half of individuals with MWS present with Hirschsprung's Disease (HSCR). There is no nested case–control study that correlates with its prognosis. This study aimed to compare the mid-to-short-term postoperative prognosis between children with MWS-associated with HSCR and those with isolated HSCR.

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Article · Jan 2025 · 1 min read

In brief

This single-center study examines postoperative functional outcomes in pediatric patients with Mowat-Wilson Syndrome-associated Hirschsprung disease compared to isolated Hirschsprung cases. The research addresses a gap in prognostic data for this rare genetic condition affecting approximately half of MWS patients.

Mowat-Wilson Syndrome is autosomal dominant; ~50% of MWS patients present with Hirschsprung's Disease
This single-center study compares postoperative outcomes in MWS+HSCR vs isolated HSCR patients
First nested case-control study examining prognosis correlation in this patient population
Focus on mid-to-short-term functional outcomes following surgical intervention

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