SKIP SEGMENT HIRSCHSPRUNG DISEASE: UNICENTRIC EXPERIENCE WITH FOUR CASES AND SYSTEMATIC LITERATURE REVIEW

jpedsurg.org

Skip lesion aganglionosis is characterized by intestinal aganglionosis interrupted by portions of normally ganglionated bowel that is located in between segments of aganglionosis. Even though this is a well-known entity, only scattered cases have been reported so far. The aim of this manuscript is to present our series of patients and perform a systematic literature review, to offer a critical overview of possible management.

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Article · Feb 2026 · 1 min read

In brief

This article examines skip segment Hirschsprung disease, a rare variant where aganglionic bowel segments are interrupted by normally innervated intestine. The authors present four cases from their institution and conduct a systematic literature review to evaluate diagnostic approaches and surgical management strategies for this challenging condition.

Skip segment Hirschsprung disease involves non-contiguous aganglionic bowel segments separated by normally ganglionated intestine.
This variant is rare with only scattered case reports in literature, making evidence-based management challenging.
Systematic review of existing cases is essential to guide surgical planning and avoid missed aganglionic segments.
Intraoperative frozen section biopsies at multiple levels are critical to identify all affected bowel segments.

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