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.

Midgut Volvulus on an Octogenarian Male: A Case Report

“…In the Eastern world, this condition is more commonly observed in regions with lower socioeconomic status. It is often associated with the practice of consuming large, indigestible meals after periods of fasting, as well as mesentery hypermobility [ 9 ].…”

“…In the Eastern world, this condition is more commonly observed in regions with lower socioeconomic status. It is often associated with the practice of consuming large, indigestible meals after periods of fasting, as well as mesentery hypermobility [9]. Some physical examination findings of a patient with advanced midgut volvulus are concerning and suggest major complications.…”

Recurrent midgut volvulus in an 83-year-old female

“…Overall, it is exceedingly rare to encounter primary midgut volvulus in adult patients with neither evidence of intestinal malrotation nor mechanical causes of volvulus, such as jejunal diverticula [ 4 ]. One case report describes recurrent midgut volvulus in a patient with a diagnosis of Marfan’s syndrome in which the mesentery was noted intraoperatively ‘to appear long with a narrow base and floppy, likely contributing to hypermobility of the small bowel’ [ 5 ]. This description is strikingly similar to the appearance of the mesentery in our case ( Fig.…”

Colonic Volvulus in Patient with Marfan Syndrome: Rare Gastrointestinal Presentation

“…GP is not commonly found in patients with Marfan syndrome compared to higher occurrences of cardiovascular, ocular and soft tissue pathology. GP are infrequent and there are only a few cases reported of differing diagnoses, most of the cases presenting with abnormal anatomy of various segments of the gastrointestinal system such as diverticulosis, hernias, atresias, malrotations and sigmoid volvulus [3].…”