AJSP2302 - CME/CMLE - Hirschsprung Disease for the Practicing Surgical Pathologist-Hirschsprung Disease for the Practicing Surgical Pathologist

Hirschsprung Disease for the Practicing Surgical Pathologist

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The document provides an in-depth overview of Hirschsprung disease (HD), a congenital condition involving the absence of ganglion cells in the gastrointestinal tract. It stresses the challenges faced by pathologists in accurately diagnosing HD due to its rarity in biopsies and resections. Emphasizing the importance of a standardized diagnostic approach and communication between surgeons and pathologists, the article discusses techniques like rectal biopsies and histological evaluations using specific stains. Additionally, it details the histologic features of HD and normal bowel, as well as the definitive treatment involving surgical resection in confirmed cases.<br /><br />Furthermore, the document delves into surgical procedures for HD, involving techniques like diverting colostomies and resections. It highlights the significance of intraoperative biopsies to differentiate between ganglionic and aganglionic bowel segments, essential for determining the appropriate surgical approach. The discussion extends to the three primary surgical procedures (Swenson, Soave, and Duhamel) used in correcting HD, stressing the importance of pathology evaluation of resected specimens to confirm the adequacy of resection and diagnose HD accurately.<br /><br />Moreover, the article addresses HD-associated complications like enterocolitis, stressing the role of pathologic evaluation in guiding management decisions. It also discusses advancements in diagnostic tools and understanding the disease's pathophysiology, contributing to improved management and outcomes in HD cases over the years. The overall focus remains on the importance of precise histologic evaluation, effective communication, and following established procedures for successful diagnosis and management of HD.

Keywords

Hirschsprung disease

congenital condition

ganglion cells

gastrointestinal tract

diagnostic challenges

rectal biopsies

histological evaluations

surgical resection

intraoperative biopsies

complications