Sahin Sinan, Celebi Ahmet, Yalçin Yalim, Saritaş Mustafa, Bilal Mehmet S, Celik Levent
Department of Radiology, Dr. Siyami Ersek Thoracic and Cardiovascular Surgery Hospital, Istanbul, Turkey.
Cardiovasc Intervent Radiol. 2005 Mar-Apr;28(2):249-53. doi: 10.1007/s00270-004-0046-0.
Scimitar syndrome is a rare congenital disorder. It is characterized by partial or total abnormal venous drainage of the right lung into the inferior vena cava, which is often associated with anomalous systemic arterial supply to the right lung, congenital cardiac anomalies, hypoplasia of the right lung and bronchial anomalies. Symptoms depend on the degree of the shunt and severity of the associated anomalies, which determine the treatment. We present a 6-year-old boy who was diagnosed as having the adult form of scimitar syndrome during evaluation for recurrent pulmonary infections, and underwent embolization with a detachable silicon balloon of the anomalous systemic arterial supply from the abdominal aorta to the right lower lung lobe. Successful elective surgery was performed 6 months later, in which right pulmonary veins were directed to the left atrium using a Gore-Tex patch by creating an intra-atrial tunnel. The patient has been symptom-free period during 6 months of follow-up, which supports the idea that recurrent pulmonary infections can be eliminated by embolization of the anomalous arterial supply.
弯刀综合征是一种罕见的先天性疾病。其特征是右肺部分或全部静脉血异常引流至下腔静脉，常伴有右肺异常体循环动脉供血、先天性心脏畸形、右肺发育不全和支气管畸形。症状取决于分流程度和相关畸形的严重程度，这些因素决定了治疗方法。我们报告一名6岁男孩，在因反复肺部感染进行评估时被诊断为成人型弯刀综合征，并接受了经可脱卸硅胶球囊栓塞来自腹主动脉至右下肺叶的异常体循环动脉供血。6个月后成功进行了择期手术，术中使用Gore-Tex补片通过创建心房内隧道将右肺静脉引流至左心房。患者在6个月的随访期间无症状，这支持了通过栓塞异常动脉供血可消除反复肺部感染的观点。
