The Colorectal Quiz Episode 5: Proximal Hirschsprung Disease Surgical Technique

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Topic overview

Surgical decision-making for proximal Hirschsprung disease involving the transverse colon, focusing on intraoperative biopsy interpretation and the choice between immediate pull-through versus staged diversion. Emphasizes the limitations of frozen section pathology and strategies to avoid resecting potentially normal bowel.

Key takeaways

  • Frozen sections can rule OUT Hirschsprung disease but cannot definitively rule it IN—wait for permanent pathology before resecting colon.
  • Hypertrophic nerves are mainly a sacral/rectal finding; in proximal colon biopsies, focus on presence of ganglion cells, not nerve size.
  • For proximal Hirschsprung with uncertain transition zone, consider ileostomy + colonic mapping rather than immediate pull-through.
  • If delaying definitive surgery 4 days for permanent pathology, ensure the infant tolerates rectal irrigations without enterocolitis.
  • Avoid resecting potentially normal colon based on frozen section alone—diversion buys time for accurate histologic diagnosis.

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