Division of Thoracic Surgery and Lung Transplantation, Georges Pompidou European Hospital, Assistance Publique-Hôpitaux de Paris, Paris, France.
Perm J. 2021 Mar 9;25:21.021. doi: 10.7812/TPP/21.021.
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.
血管型 Ehlers-Danlos 综合征是一种遗传性结缔组织疾病,其特征是 III 型胶原蛋白生成减少。疾病的临床演变是不可预测的,因为血管病变可能发生在身体的任何部位。瘀伤、动脉和肠道脆弱,以及妊娠期间的子宫脆弱是最常见的症状。胸外科医生应该对这些患者保持警惕,因为在极少数情况下,可能会发生胸部并发症,如复发性气胸或自发性血胸。我们报告了一例血管型 Ehlers-Danlos 综合征患者的胸膜外血肿,这是一种罕见的并发症,采用保守治疗成功治愈。