The Colorectal Quiz Episode 3.5: Proximal Hirschsprung Disease
Topic overview
In this episode, Dr. Levitt and Dr. Frischer discuss the workup and management for a patient with suspected proximal Hirschsprung disease. Listen as they walk you through a their thought process and what to consider in these complex patients.
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Key takeaways
- Proximal Hirschsprung disease requires a different diagnostic approach than typical rectosigmoid disease due to extended aganglionosis.
- Contrast enema findings may be less definitive in proximal cases; rectal biopsy remains the gold standard for diagnosis.
- Surgical planning must account for the extent of aganglionosis to ensure adequate resection and functional outcomes.
- Complex cases benefit from multidisciplinary discussion to optimize timing and technique of definitive pull-through procedures.
- Long-term follow-up is critical as proximal cases have higher rates of postoperative complications including enterocolitis.
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