Department of Pediatrics, Division of Pulmonology-Allergy, Ege University Faculty of Medicine, Izmir, Turkey.
Eur J Pediatr. 2010 Oct;169(10):1263-5. doi: 10.1007/s00431-010-1153-3. Epub 2010 Feb 16.
Scimitar syndrome is a rare congenital cardiopulmonary malformation characterized by hypoplasia of the right lung and drainage of the right pulmonary veins into the vena cava inferior. It may also be associated with cardiac dextroversion and anomalies of the tracheobronchial system, cardiovascular system, and diaphragm. Some cases are asymptomatic with others diagnosed in early-childhood period with pulmonary hypoplasia and other associated malformations. We present here a patient whose venous return of the middle and lower lobes of the right lung is into the superior vena cava, which is a very unusual finding for this disorder.
弯刀综合征是一种罕见的先天性心肺畸形,其特征为右肺发育不良和右肺静脉引流至下腔静脉。它还可能与心脏右旋和气管支气管系统、心血管系统和膈肌的异常有关。一些病例无症状,另一些则在幼儿期因肺发育不全和其他相关畸形而被诊断。我们在此介绍一例患者,其右肺中、下叶的静脉回流至上腔静脉,这在该疾病中是一种非常罕见的发现。