Abstract:
Scimitar syndrome is a rare condition characterized by partial or complete anomalous pulmonary venous drainage of the lung to the inferior vena cava, right lung hypoplasia and dextroposition of the heart. Haemoptysis is uncommon in adults, although the clinical spectrum is wide. We report a case of a 38-year-old male with scimitar syndrome who had low grade haemoptysis persisting over several years secondary to bronchiectatic changes in his hypoplastic right lung. Conservative measures to manage haemoptysis were unsuccessful and the patient proceeded to bronchial artery embolization. The post-procedure course was complicated by lung infarction and the patient ultimately required pneumonectomy. Deformities of the blood vessels and lungs are complex in scimitar syndrome. Bronchial artery embolization should be approached cautiously to protect pulmonary perfusion.
摘要:
弯刀综合征是一种罕见的疾病,其特征是肺部分或完全异常的肺静脉引流到下腔静脉,右肺发育不全和心脏右旋。咯血在成人中并不常见,虽然临床范围很广。我们报告了一例38岁的男性弯刀综合征,该患者因右肺发育不全的支气管扩张改变而持续多年的低度咯血。治疗咯血的保守措施未成功,患者进行了支气管动脉栓塞。术后过程因肺梗死而复杂化,患者最终需要肺切除术。弯刀综合征中血管和肺的畸形很复杂。应谨慎进行支气管动脉栓塞,以保护肺灌注。