Gastric Submucosal Hematoma and Mucosal Rupture with Ehlers-Danlos Syndrome : A Case Report

概要

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Ehlers-Danlos syndrome (EDS), a rare disease caused by the inadequate production of connective tissue collagen, can carry a high risk of life-threatening aneurysms or intestinal bleeding. Recently, we experienced a case of EDS with very unusual clinical features. A 20-year-old woman presented at our hospital with a sudden onset of hematemesis. She had been previously diagnosed with EDS but had received no specific treatment for this disorder. Results of a gastrointestinal endoscopy suggested duodenal intussusception. We therefore performed an emergent operation, which revealed a submucosal hematoma at the antrum, the mucosal rupture of which had led to hematemesis. The patient underwent a distal gastrectomy and recovered without any complications after the operation. This case highlights the fact that patients with EDS may present with quite unexpected symptoms or images that may still be related to the underlying pathogenesis of this disorder.
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