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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.
Insights
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.
- 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.
Abstract:
We identified 21 children (14 boys and seven girls) with long-segment Hirschsprung's disease defined as aganglionosis extending proximal to the ileocecal valve. Long-segment Hirschsprung's disease is difficult to diagnose and treat; symptoms may be mild, and diagnosis delayed. Abdominal distention and constipation or delayed passage of meconium are the most common symptoms. Radiologic studies are unreliable in establishing the diagnosis. The morbidity rate is high because of the high transition zone and short gut. The long-term outcome of patients after the standard Duhamel procedure is satisfactory, except with extremely high transition zones. Pull-through procedures should not be performed in the small infant, but should be delayed until patients are old enough to be continent.