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Limited surgery for lower-segment Hirschsprung's disease

R Sawin1, E Hatch, R Schaller

  • 1Department of Surgery, Children's Hospital and Medical Center, University of Washington, Seattle.

Archives of Surgery (Chicago, Ill. : 1960)
|September 1, 1994
PubMed
Summary

Rectal myectomy effectively treated older children with Hirschsprung's disease, improving constipation and bowel function. This less-extensive surgery offers a viable option for managing this condition in pediatric patients.

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Area of Science:

  • Pediatric Surgery
  • Gastroenterology
  • Genetics

Background:

  • Hirschsprung's disease is a congenital disorder characterized by the absence of ganglion cells in the distal bowel.
  • Chronic constipation and abdominal distension are common symptoms in affected children.
  • Diagnostic challenges exist, particularly in cases with nondiagnostic barium enema studies.

Purpose of the Study:

  • To evaluate the efficacy of posterior rectal myectomy as a treatment for Hirschsprung's disease in children presenting with chronic constipation and inconclusive diagnostic findings.
  • To assess the long-term outcomes and patient-reported improvements following rectal myectomy.

Main Methods:

  • A retrospective case series involving 14 pediatric patients (aged 2-14 years) with Hirschsprung's disease.

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  • All patients underwent posterior rectal myectomy.
  • Outcomes were assessed over a mean follow-up period of 37.1 months, evaluating factors like laxative use, stool frequency, soiling, incontinence, and overall patient satisfaction.

    • Main Results:

    • All 14 patients demonstrated clinical improvement after rectal myectomy, with assessments categorized as excellent (6), good (6), or fair (2).
    • Improvements were observed irrespective of the presence or absence of ganglion cells in the surgical specimen.
    • No patients experienced soiling or incontinence post-operatively, and seven required only intermittent laxative use.

    Conclusions:

    • Posterior rectal myectomy is a safe and effective surgical intervention for selected pediatric patients with Hirschsprung's disease.
    • This less-invasive procedure should be considered for older children experiencing chronic constipation due to Hirschsprung's disease.
    • The procedure yields favorable functional outcomes, including improved bowel regularity and absence of incontinence.