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Updated: Jun 10, 2026

Diagnosis of Hirschsprung's Disease by Immunostaining Rectal Suction Biopsies for Calretinin, S100 Protein and Protein Gene Product 9.5

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Skip segment Hirschsprung's disease: a systematic review.

Anne-Marie O'Donnell¹, Prem Puri

¹National Children's Research Centre, Our Lady's Children's Hospital, Crumlin, Dublin 12, Ireland.

Pediatric Surgery International

|August 18, 2010

Summary

This summary is machine-generated.

Skip segment Hirschsprung's disease (SSHD) predominantly affects males with total colonic aganglionosis. Identifying these skip segments is crucial for surgical planning in treating this rare condition.

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Published on: September 18, 2013

Area of Science:

Pediatric Surgery
Gastroenterology
Developmental Biology

Background:

Hirschsprung's disease involves congenital aganglionosis of the distal rectum.
Skip segment Hirschsprung's disease (SSHD) presents with a normally innervated segment within aganglionic bowel.
The embryological basis for SSHD remains unclear.

Purpose of the Study:

To systematically review reported cases of SSHD from 1954 to 2009.
To elucidate the clinical characteristics and significance of SSHD.
To understand the prevalence and location of skip segments in SSHD.

Main Methods:

Systematic literature review of SSHD cases.
Data collection from 1954 to 2009 via PubMed.
Analysis of patient demographics, symptoms, and skip segment location.

Main Results:

24 cases of SSHD were identified, with 75% occurring in males.
92% of cases were associated with total colonic aganglionosis (TCA).
Skip segments were most commonly found in the transverse colon (41%) in TCA cases.

Conclusions:

SSHD is more common in patients with TCA.
The presence of a skip segment can inform surgical strategies, potentially allowing preservation of innervated bowel during pull-through procedures.