Michell Hans, Chopra Prajna, Bhave Anant, Ali Naiim, Parkinson William, Shields Joseph, Scriver Geoffrey, Morris Christopher
Section of Interventional Radiology, Department of Radiology, University of Vermont Medical Center, Burlington, Vermont, United States.
Department of Radiology, University of Vermont Medical Center, Burlington, Vermont, United States.
BJR Case Rep. 2020 Jul 29;6(4):20200082. doi: 10.1259/bjrcr.20200082. eCollection 2020 Dec 1.
Ehlers-Danlos syndrome (EDS) refers to a rare group of genetic disorders that makeup part of the connective tissue disorders consortium. It is characterized by clinical features such as skin hyperextensibility, joint hypermobility, and tissue fragility. A vascular subtype (EDS IV) exists, that predisposes affected patients to vascular injury and is well-known and documented. However, other manifestations of EDS IV are less commonly understood and reported. Though spontaneous pneumothorax has been described in several cases, formation of traumatic air cysts/pneumatoceles with little to no inciting factors has not. This can eventually lead to pulmonary hemorrhage or hemopneumothorax. We present a case of spontaneous formation of a traumatic air cyst with ensuing large-volume hemopneumothorax occurring in a time period of under 3 minutes, between pre- and post-contrast-media administration during CT angiography of the chest.
埃勒斯-当洛综合征(EDS)是指一组罕见的遗传性疾病,属于结缔组织疾病联盟的一部分。其临床特征包括皮肤过度伸展、关节活动过度和组织脆弱。存在一种血管亚型(EDS IV),使受影响的患者易发生血管损伤,这是众所周知且有文献记载的。然而,EDS IV的其他表现较少被理解和报道。虽然在几例病例中已描述过自发性气胸,但在几乎没有或没有诱发因素的情况下形成创伤性气囊/肺大疱的情况尚未见报道。这最终可能导致肺出血或血气胸。我们报告一例在胸部CT血管造影术造影剂注射前和注射后不到3分钟的时间内,自发性形成创伤性气囊并随后发生大量血气胸的病例。