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Massive gastrointestinal dilatation in a case of hereditary hollow visceral myopathy
Hereditary hollow visceral myopathy (HHVM) is a rare genetic cause of intestinal pseudo-obstruction described sparingly in the literature . A 21-year-old-male was diagnosed with HHVM based on clinical symptoms, gastroduodenal manometry demonstrating low-amplitude contractions in the stomach and small intestines, and intestinal histopathology demonstrating diffuse fibrous replacement of smooth muscle; his father was also affected, with similar manometric and histopathologic changes. He presented acutely to the Emergency Room with complaints of abdominal pain, recurrent emesis of feculent material, and severe gaseous distent...
Source: Digestive and Liver Disease - July 1, 2013 Category: Gastroenterology Authors: Robert J. Huang, Chohee Yun, Linda Nguyen Tags: Image of the Month Source Type: research