Unicentric Series of 82 Consecutive Patients With Ultralong Hirschsprung Disease: How Experience Leads Management

jpedsurg.org

Total Colonic (TCSA), Extended (EA), and Total Intestinal Aganglionosis (TIA) are rare forms of Hirschsprung (HSCR) grouped under the term Ultralong HSCR. We aim at presenting the features of the largest unicentric series reported so far and at sharing the algorithm for management adopted in our Center for Digestive Diseases.

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Article · Mar 2025 · 1 min read

In brief

This study presents the largest single-center series of 82 patients with ultralong Hirschsprung disease variants (total colonic, extended, and total intestinal aganglionosis). The authors share their institutional management algorithm developed through extensive experience treating these rare and complex forms of congenital intestinal aganglionosis.

Ultralong Hirschsprung disease encompasses total colonic, extended, and total intestinal aganglionosis—rare variants requiring specialized management.
This represents the largest single-center series of 82 consecutive ultralong HSCR patients, providing robust evidence for treatment protocols.
Institutional experience is critical for optimizing surgical outcomes in these complex aganglionosis cases.
A standardized management algorithm can guide decision-making for ultralong HSCR at specialized digestive disease centers.
Consecutive patient series allow for systematic evaluation of surgical techniques and long-term outcomes in rare HSCR variants.

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