Deep Venous Thrombosis and Pulmonary Thromboembolism Associated With Retroperitoneal Hematoma in a Patient With Ehlers-Danlos Syndrome Type VI

Ehlers-Danlos Syndrome (EDS) is a group of genetic diseases of the connective tissue, which is rare and is characterized by joint hypermobility, tissue, and vascular fragility. We present the case of a 38-year-old woman with a known diagnosis of EDS type VI who came to the emergency room, complaining of sudden dyspnea in the context of abdominal pain and pain in the left lower limb with one week of evolution. Computed axial tomography showed the presence of bilateral pulmonary thromboembolism, iliofemoral thrombosis, and a retroperitoneal hematoma. Anticoagulation was started and the patient was admitted to the intermediate care ward. This case highlights the need to know the implications of increased vascular fragility in type VI EDS, with potentially serious consequences. Underlying is the inevitable need for judicious consideration of the decision to undergo anticoagulation, in the context of a retroperitoneal hemorrhagic event and pulmonary thromboembolism.