A variety of Ehlers-Danlos syndrome type IV presenting with haematemesis and gastro-esophageal reflux.
A case of a variety of Ehlers-Danlos syndrome (EDS) type IV presenting with haematemesis and melaena and symptoms of gastro-esophageal reflux is described. At referral, the manometric esophageal findings similar to scleroderma and the abundant gastroesophageal reflux seemed noteworthy. 3 years after surgery, bleeding and reflux symptoms appeared, perfectly cured by a total biliary diversion operation. The reported case suggests that patients with EDS type IV especially when presenting with haematemesis and melaena should be investigated for reflux, since bleeding might be due to the action of reflux on the fragile-walled vessels of such patients.
D004535:Ehlers-Danlos Syndrome / Q000150:complications* / Q000235:genetics; D005260:Female; D005764:Gastroesophageal Reflux / Q000209:etiology*; D006396:Hematemesis / Q000209:etiology*; D006801:Humans; D008551:Melena / Q000209:etiology; D008875:Middle Aged