Scimitar syndrome in a four-month-old infant

Abstract

The Scimitar syndrome or pulmonary venolobar syndrome is a rare, complex and variable congenital anomaly of cardiopulmonary development characterised by an abnormal right-sided pulmonary venous drainage in the inferior vena cava, malformation of the right lung, abnormal arterial supply and sometimes cardiac malformations. These serious anomalies notwithstanding, their presentation is varied, ranging from asymptomatic to severe symptoms, particularly in the neonatal and early infantile period. Some symptomatic cases are misdiagnosed as recurrent lower respiratory tract infections or as heart failure, missing the underlying congenital anomaly. Considering associated serious complications such as pulmonary hypertension and a high mortality rate, accurate and timely diagnosis is mandatory. This requires a high index of suspicion, not only by clinicians but also by radiologists who encounter suggestive chest radiographs in asymptomatic children. Still, the classic radiological finding for which the condition is named is seen in only about 50% of all cases and only 10% in affected infants. We highlight this case to heighten clinicians’ and radiologists’ suspicions about Scimitar syndrome.