Khan Sabeen Abid, Ahmad Zaheer
Pediatrics Department, Shifa College of Medicine, Shifa Tameer e Millat university, Islamabad.
Consultant Pediatrics Cardiologist, Shifa International Hospital, Islamabad.
J Pak Med Assoc. 2019 Mar;69(3):423-425.
Scimitar syndrome is a rare, congenital malformation. It is characterized by partial anomalous pulmonary venous connection to inferior vena cava, hypoplastic right lung along with dextroposition of the heart. Depending on the severity of the defect it can present as early as in neonatal period or incidentally later in life. Its incidence is 1-3 per 100,000 patients. We present a case of a two-year-old girl who presented with recurrent fever, cough and was later diagnosed as scimitar syndrome.
弯刀综合征是一种罕见的先天性畸形。其特征为部分肺静脉异常连接至下腔静脉、右肺发育不全以及心脏右旋。根据缺损的严重程度,其症状最早可在新生儿期出现,或在生命后期偶然发现。其发病率为每10万名患者中有1 - 3例。我们报告一例两岁女童病例,该女童反复发热、咳嗽,后来被诊断为弯刀综合征。