Scimitar Syndrome: Preoperative Incidental Radiographic Finding of an Asymptomatic Rare Congenital Pulmonary Venolobar Anomaly

Scimitar syndrome is a rare congenital anomaly characterized by partial anomalous pulmonary venous return, right lung hypoplasia and dextroposition of the heart. One or more curved pulmonary veins from the right lung drain abnormally into the inferior vena cava (IVC), resulting in a distinctive radiographic appearance resembling a curved scimitar. Patients with the syndrome reaching adulthood are mostly asymptomatic compared to children diagnosed early in life with symptoms.   We report a 60-year-old female who presented with dull, postprandial right upper abdominal pain due to multiple gallstones and was referred for elective cholecystectomy, in whom the Scimitar syndrome was diagnosed. Clinical examination was largely unremarkable except for absent apex beat and reduced breath sounds over the right chest. Chest X-ray revealed a curvilinear shadow along the right cardiac border (scimitar sign), hypoplastic right lung and dextroposition of the heart. CT chest confirmed right lung hypoplasia, anomalous pulmonary venous drainage and compensatory hyperinflation of the left lung. Echocardiography demonstrated rightward displacement of the heart with preserved levocardia, situs solitus, and a structurally  normal heart. Subsequently, she underwent an uneventful cholecystectomy and was scheduled for regular echocardiographic follow-up every three months to monitor potential cardiopulmonary complications. Compared to previously reported cases, our patient was older and asymptomatic. The case report highlights the importance of thorough pre-operative evaluation, particularly when abnormal clinical signs prompt investigation beyond the primary diagnosis.