Early Diagnostics and Treatment of Scimitar Syndrome in Infancy

Background: Scimitar (Yataghan) syndrome is a rare congenital pathology and occurs from 1 to 3 cases in 100,000 newborns, predominantly in girls. It belongs to a group of velobular syndromes associated with lung sequestration and congenital heart disease.

Objective: The aim of this study is to show that the clinical picture of patients with the Scimitar syndrome is extremely diverse. It may cause many difficulties by diagnostics and making decision on treatment (surgical or medical) for children with mild Scimitar syndrome, at the asymptomatic course and having a random diagnosis.

Methods: The research of the clinical case of Scimitar syndrome in a child, who is 3 years old. A diagnosis for a baby at infancy was made due to an aspiration episode. The child was diagnosed with the Scimitar syndrome in combination with aortic coarctation and aberrant right subclavian artery. Balloon dilation of aortic coagulation was performed at the age of 2.5 months. At the age of 2 years, surgical correction was performed – elimination of abnormal drainage of the pulmonary veins and the aortic arch plastic.

Conclusion: Some patients do not actually have symptoms characteristic of the Scimitar syndrome because there are no signs of heart failure, they do not have respiratory disease. Atelectasis and mesocardia prevent visualization of the characteristic radiological feature of the Scimitar syndrome. Therefore, a complete study using contrast-enhanced CT is mandatory for such patients.