DOI: 10.36347/sjmcr.2017.v05i10.008

Pages: 627-629

A 42-year-old male spontaneously fell down in front of his wife. His past medical history included gastroesophageal reflux disease (GERD) and hypertension without receiving medication. Upon arrival, he was in cardiopulmonary arrest and his initial rhythm was asystole. His chest roentgen revealed a dilated heart and an echocardiogram demonstrated cardiac tamponade. He underwent pericardiocentesis and infusion of 1 mg of adrenalin for three times at 4-minute intervals. Unfortunately, he could not obtain a return of spontaneous circulation. Postmortem computed tomography findings revealed cardiac tamponade, deformity of the contour of the ascending aorta, suggesting Stanford type A aortic dissection, and bilateral patellar subluxation. Bilateral patellar subluxation tends to occur in young females who have an elastic softness. This case was a middle-aged male, thus ligamentous laxity may have existed. Ehlers-Danlos syndrome is a heterogeneous group of heritable connective tissue disorders mainly characterized by joint hypermobility, skin hyperextensibility and tissue fragility. Among congenital or secondary conditions associated with medial degeneration of the aorta, only Ehlers-Danlos syndrome can result in bilateral patellar dislocation. In addition, GERD is common in Ehlers-Danlos syndrome. Accordingly, we strongly suspected that this case was the vascular form Ehlers-Danlos syndrome.