Contributed by Nour Yadak, MD and Michael Nalesnik, MD
A 41 year old male presented with rectal bleeding. He had a past medical history of omphalocele, with colostomy at birth and subsequent extensive small bowel resection requiring total parenteral nutrition from the age of 12-13. On admission he was found to have colonic ulcers at the ileo-colonic anastomosis with recurrent small bowel obstruction and required small bowel/anastomotic resection with GI reconstruction.
Received is a 24.0 cm length of small bowel with attached 1.8 cm segment of large bowel with serosal adhesions. Opening the bowel reveals edematous, pale tan-brown discoloration of the mucosa with fine nodularity throughout. No masses or lesions identified grossly.
Representative sections of the small bowel show thickening of the muscularis propria with smooth muscle cells containing a marked accumulation of granular gray-brown pigment resembling lipofuscin that is highlighted by PAS and PAS diastase.
Some increase in fibrous tissue is also noted particularly involving the outer longitudinal segment (given the recurrent history of bowel obstruction). Also present is a mild increase in chronic inflammatory cells both within and between the two muscle layers. Submucosa and mucosa do not show these changes. Moderate predominantly plasmacytic lamina propria inflammation is seen in the mucosa with vascular congestion.