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Hirschsprung's disease

M Dinner

    South African Medical Journal = Suid-Afrikaanse Tydskrif Vir Geneeskunde
    |August 13, 1977
    PubMed
    Summary
    This summary is machine-generated.

    Hirschsprung's disease, a common neonatal intestinal obstruction, involves an aganglionic bowel segment. This case study details the management of total intestinal aganglionosis, a rare form of the disease.

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    Area of Science:

    • Pediatric Surgery
    • Gastroenterology
    • Medical Genetics

    Background:

    • Hirschsprung's disease is a primary cause of intestinal obstruction in newborns.
    • It is characterized by an aganglionic segment of variable length in the bowel.
    • The condition presents in three main types: short-segment, long-segment, and total aganglionosis.

    Observation:

    • Long-segment Hirschsprung's disease accounts for approximately 20% of cases.
    • Total aganglionosis, involving the entire colon and potentially the small bowel, occurs in about 5% of patients.
    • This report focuses on a specific case of total intestinal aganglionosis.

    Findings:

    • The study outlines the management strategies for total aganglionosis.
    • It identifies and discusses challenges encountered during treatment.

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  • The preferred surgical intervention for this severe form is detailed.

    • Implications:

    • Understanding the management of rare total aganglionosis is crucial for pediatric surgeons.
    • Effective surgical approaches can improve outcomes for affected neonates.
    • Further research into the genetic and developmental aspects of Hirschsprung's disease is warranted.