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[Total colonic aganglionosis].

J M Casasa1

  • 1Hospital Materno-Infantil Vall d'Hebrón, Barcelona.

Cirugia Pediatrica : Organo Oficial De La Sociedad Espanola De Cirugia Pediatrica
|April 1, 1992
PubMed
Summary
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Frequency of RET mutations in long- and short-segment Hirschsprung disease.

Human mutation·1997

Total parenteral nutrition (TPN) significantly improved survival rates for Hirschsprung

Area of Science:

  • Pediatric Surgery
  • Gastroenterology
  • Clinical Medicine

Context:

  • Hirschsprung's disease is a congenital disorder affecting the large intestine.
  • Surgical treatment is the primary intervention for Hirschsprung's disease.
  • Total colonic aganglionosis represents a severe form of the disease.

Purpose:

  • To evaluate the impact of total parenteral nutrition (TPN) on surgical outcomes for Hirschsprung's disease.
  • To assess the long-term outcomes and complications in patients treated for Hirschsprung's disease.

Summary:

  • A retrospective analysis of 171 Hirschsprung's disease cases (1966-1990) was conducted.
  • Introduction of TPN (from 1979) correlated with a significant reduction in mortality (1/10 vs. 6/11 deaths without TPN).

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  • Post-operative monitoring revealed good general condition, higher than normal bowel movements, and slightly reduced absorption of iron, vitamin B12, and folic acid.

    • Impact:

    • TPN is a critical supportive measure in managing severe Hirschsprung's disease, improving survival rates.
    • Surgical techniques like Duhamel, Rehbein, and Boley are effective, but long-term nutritional monitoring is essential.