S2804 Brown Bowel Syndrome as a Result of Chronic Bowel Obstruction

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INTRODUCTION: Brown bowel syndrome (BBS) is a rare condition characterized by deposition of lipofuscin in the smooth muscle cells of the muscularis propria and muscularis mucosa. This condition has been attributed to various chronic malabsorptive conditions that result in deficiency in vitamin E. Vitamin E normally acts as an antioxidant which helps stabilize the mitochondrial membranes. Lack of vitamin E leads to mitochondrial degeneration with release of lipofuscin that accumulates in the smooth muscle cells. This results in a form of myopathy with uncoordinated bowel function. There have been case reports of BBS attributed to celiac disease, IBD, chronic pancreatitis, Whipple's disease and malignancy. We present an interested case of BBS attributed to chronic bowel obstruction. CASE DESCRIPTION/METHODS: 56 yo Caucasian female who had been having chronic abdominal pain associated with a 20lb weight loss. CT scan showed a markedly dilated RLQ small bowel with air-fluid levels concerning for ileus vs partial small bowel obstruction (SBO). She subsequently under a small bowel follow through which showed a relatively high-grade partial SBO. She had an EGD and colonoscopy, both of which were unremarkable. Her medical history is notable for a congenital SBO that was repaired at birth as well as a history of ectopic pregnancy requiring removal. She eventually underwent ex-lap with extensive lysis of adhesions. There was one majorly dilated area of the small intestine measuring over 12 cm in diameter. This area was resected and histology was notable for extensive deposition of light brown granular, lipofuscin-like pigment within the smooth muscle cells of the muscularis propria. These pigments were highlighted by special stains for PAS and PAS-D. This was consistent with ceroidosis (BBS). Review of the bowel histology was unremarkable for celiac disease or Whipple’s disease. The patient also had no findings concerning for chronic pancreatitis. DISCUSSION: We describe a case of BBS that was attributed to malabsorption from a chronic partial SBO. Her bowel obstruction was believed to be caused by adhesions related to prior surgeries. While bowel dilation and atresia can occur from the myopathy associated with BBS, this patient's pathologic findings were actually attributed to a series of events that originated from her having chronic SBO. This case illustrates the impact of bowel obstructions on adequate absorption of nutrients as well as the consequences of chronic malabsorption, including the development of BBS.Figure 1.: Gross Pathology.Figure 2.: HE Stain.Figure 3.: PAS-D Stain showing lipofuscin deposition.