Vascular Ehlers-Danlos syndrome is a hereditary disorder of the connective tissue characterized by a reduction in the production of type III collagen. Clinical evolution of the disease is unpredictable because vascular lesions can occur everywhere in the body. Bruising, arterial and bowel fragility, and uterine fragility during pregnancy are the most common symptoms. Thoracic surgeons should take with these patients because, in rare cases, thoracic complications may occur, such as recurrent pneumothorax or spontaneous hemothorax. We report an extra-pleural hematoma in a patient with vascular Ehlers-Danlos syndrome, an unusual complication treated successfully with conservative management.

Successful Conservative Management of a Rare Surgical Complication of Vascular Ehlers-Danlos Syndrome: A Case Report

Mangiameli, Giuseppe;
2021-01-01

Abstract

Vascular Ehlers-Danlos syndrome is a hereditary disorder of the connective tissue characterized by a reduction in the production of type III collagen. Clinical evolution of the disease is unpredictable because vascular lesions can occur everywhere in the body. Bruising, arterial and bowel fragility, and uterine fragility during pregnancy are the most common symptoms. Thoracic surgeons should take with these patients because, in rare cases, thoracic complications may occur, such as recurrent pneumothorax or spontaneous hemothorax. We report an extra-pleural hematoma in a patient with vascular Ehlers-Danlos syndrome, an unusual complication treated successfully with conservative management.
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Utilizza questo identificativo per citare o creare un link a questo documento: https://hdl.handle.net/11699/70906
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