Primary midgut volvulus in a patient with Marfan syndrome

Abstract

Marfan syndrome is a mutation in the fibrillin-1 gene resulting in a connective tissue disorder primarily affecting musculoskeletal, cardiovascular and ocular systems. However, patients with Marfan's rarely manifest gastrointestinal symptoms. Midgut volvulus is abnormal twisting of small bowel around its mesentery that can result in compromising blood flow to the bowel causing intestinal ischemia and obstruction. Primary midgut volvulus is a term used when there is no underlying cause for the volvulus. This case describes an 80-year-old female with Marfan syndrome presenting with primary midgut volvulus, which preoperatively was suspected based on imaging, and later confirmed upon operative exploration. The small bowel mesentery was long with a narrow base twisted around its mesentery 360°. The long narrow base and floppy mesentery likely contributed to hypermobility leading to volvulus and small bowel obstruction. To our knowledge, this is the first reported case of primary midgut volvulus associated with Marfan's syndrome.

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