Hirschsprung disease (gross pathology)

Case contributed by Frank Gaillard
Diagnosis not applicable

Presentation

Distension of abdomen, bloody diarrhea, as well as constipation.

Patient Data

Age: infant
Gender: Male
pathology

The resected specimen measured 20cm in length and 8cm in maximum diameter. The narrow distal end (rectum) is on the left and the proximal dilated end (sigmoid) is on the right. 

Case Discussion

Barium enema was suggestive of Hirschsprung disease involving the anorectal junction (ultra-short segment type). Gross distension of rectum and sigmoid was noted. A frozen section at the time of corrective surgery confirmed the absence of ganglion cells in the submucosal (Meissner) and in the myenteric (Auerbach) plexuses. 

Case reproduced with permission, courtesy of Dr. Sanjay D. Deshmukh, Department of Pathology, Smt. Kashibai Navale Medical College, Pune, India, Webpathology.com (original case is here). All rights reserved; please see original case for copyright information. 

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