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Related Experiment Videos

Long-segment Hirschsprung's disease.

S W Bickler1, M W Harrison, T J Campbell

  • 1Department of Surgery, School of Medicine, Oregon Health Sciences University, Portland.

Archives of Surgery (Chicago, Ill. : 1960)
|September 1, 1992
PubMed
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Long-segment Hirschsprung's disease, affecting 21 children, presents diagnostic and treatment challenges. The Duhamel procedure offers satisfactory outcomes, but timing of pull-through procedures is crucial for patient continence.

Area of Science:

  • Pediatric Surgery
  • Gastroenterology
  • Medical Genetics

Background:

  • Long-segment Hirschsprung's disease (LSHD) is characterized by aganglionosis extending beyond the rectosigmoid region, often to the ileocecal valve.
  • LSHD poses diagnostic and therapeutic difficulties due to potentially mild symptoms and delayed diagnosis.
  • Common symptoms include abdominal distention, constipation, and delayed meconium passage.

Purpose of the Study:

  • To identify and characterize cases of long-segment Hirschsprung's disease.
  • To evaluate the diagnostic challenges and treatment outcomes for LSHD.
  • To provide recommendations for surgical management, including the timing of pull-through procedures.

Main Methods:

  • Retrospective identification of 21 pediatric patients diagnosed with LSHD.

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  • Review of clinical presentations, diagnostic methods, and surgical interventions.
  • Assessment of long-term outcomes following the Duhamel procedure and pull-through procedures.

    • Main Results:

    • Twenty-one children (14 boys, 7 girls) were diagnosed with LSHD.
    • Radiologic studies were found to be unreliable for diagnosis.
    • The Duhamel procedure yielded satisfactory long-term outcomes, with exceptions for extremely high transition zones.

    Conclusions:

    • LSHD requires careful diagnosis and management due to high morbidity associated with high transition zones and short gut.
    • The standard Duhamel procedure is effective for LSHD, but pull-through procedures should be deferred until the child is older to ensure continence.