title: [Vascular-type Ehlers-Danlos syndrome incidentally diagnosed at surgical treatment for hemothorax; report of a case].
abstract: Vascular-type Ehlers-Danlos syndrome(vEDS) is a rare autosomal dominant inherited disorder of the connective tissue, which often causes arterial ruptures and surgical complications. We report the case of a vEDS patient who was incidentally diagnosed at surgical treatment for hemothorax. A 64-year-old woman with a past history of hysterectomy due to excessive bleeding during childbirth visited our hospital complaining of chest pain. Chest computed tomography revealed right pleural effusion suspected of hemothorax and a high density area behind the right anterior chest wall. Emergency thoracoscopy revealed bloody spots throughout the mediastinal pleura, suggestive of bleeding from the right internal thoracic artery. During thoracoscopy, easy bruising of the tissue by surgical manipulation was noted which led us to suspect connective tissue disease. A biochemical analysis by cultured dermal fibroblasts and molecular biological examination established the diagnosis of vEDS.
authors: Department of Thoracic Surgery, Yokohama Municipal Citizen's Hospital, Yokohama, Japan.
authors: Kazunori
authors: K
authors: Kamiya
authors: Akira
authors: A
authors: Yoshizu
authors: Fumihiro
authors: F
authors: Kashizaki
mesh_terms: D004535:Ehlers-Danlos Syndrome / Q000175:diagnosis*; D005260:Female; D006491:Hemothorax / Q000601:surgery*; D006801:Humans; D033162:Incidental Findings; D008875:Middle Aged