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[Total colic aganglionosis (author's transl)]

J M Casasa, J Boix-Ochoa, A Blanco

    Anales Espanoles De Pediatria
    |November 1, 1981
    PubMed
    Summary

    This study reviewed eleven cases of total colonic aganglionism in Hirschsprung's disease patients. The Duhamel-Roviralta technique showed advantages in surgical outcomes for these complex cases.

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    Area of Science:

    • Pediatric Surgery
    • Gastroenterology
    • Medical Genetics

    Background:

    • Hirschsprung's disease is a congenital disorder characterized by the absence of ganglion cells in the distal bowel.
    • Total colonic aganglionism represents a severe form of Hirschsprung's disease, posing significant surgical challenges.
    • This study focuses on a series of 130 patients diagnosed with Hirschsprung's disease, specifically examining eleven cases of total colonic aganglionism.

    Observation:

    • Eleven cases of total colonic aganglionism were identified within a cohort of 130 Hirschsprung's disease patients.
    • Surgical interventions included the Duhamel-Roviralta technique and the Duhamel procedure.
    • Outcomes varied, with five patients surviving the surgical procedures.

    Findings:

    • Four survivors underwent the Duhamel-Roviralta technique, and one survived the Duhamel procedure.
    • Mortality was observed in six patients; two died postoperatively from septic processes, one from hepatitis, and two after ileostomy.
    • Delayed biopsy results impacted the timely application of surgical solutions for some patients, contributing to mortality.

    Implications:

    • The Duhamel-Roviralta operation is highlighted as a preferred technique for total colonic aganglionism due to its speed and simplicity.
    • Effective management of total colonic aganglionism requires prompt diagnosis and surgical intervention.
    • Further research may explore strategies to mitigate postoperative septic complications and optimize surgical timing in complex Hirschsprung's disease cases.

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