Background: Scimitar syndrome (SS) is a rare congenital anomaly characterized by anomalous pulmonary venous drainage into the inferior vena cava, producing a characteristic curvilinear shadow on chest x-ray. It is frequently associated with right lung hypoplasia and bronchovascular anomalies. Clinical presentation ranges from asymptomatic to congestive heart failure or recurrent respiratory infections.
Case Presentation Summary: Case 1: A 6-year-old child, with a previous history of recurrent respiratory infections (two requiring hospitalization at 13 and 27 months), presented at the emergency department with fever, cough, and progressive respiratory distress requiring pediatric intensive care admission. Chest x-ray showed a right paracardiac hypotransparency. Echocardiography revealed a single pulmonary vein draining into the left atrium, and thoracic CT angiography identified an anomalous connection between the right upper lobar arterial branch and the inferior vena cava, consistent with an arteriovenous malformation. Cardiac catheterization confirmed SS, and an endovascular device was successfully implanted to occlude the anomalous vessel. The patient remains clinically asymptomatic, with no further infections, normal growth, and preserved exercise tolerance.
Case 2: A 20-month-old infant with recurrent wheezing was admitted with a fever. Chest radiography showed right hemithorax hypotransparency with ipsilateral mediastinal shift. Thoracic CT angiography revealed anomalous drainage of the right inferior pulmonary vein into the right atrium and ipsilateral parenchymal hypoplasia. Echocardiography was suggestive of SS, later confirmed by cardiac catheterization. Surgical correction was performed at 2 years and 7 months without complications. Subsequent multidisciplinary follow-up led to a diagnosis of asthma, well-controlled with as-needed therapy. The patient remains asymptomatic, with normal growth and preserved exercise tolerance.
Learining Points/Discussion: The authors reported two cases that illustrate the wide clinical spectrum of SS: presentation at different ages but both in the context of respiratory infections and asthma-like symptoms. Both patients were successfully treated with different techniques demonstrating that therapeutic approaches must be tailored to the patient’s age, anatomy and clinical course. Also, these cases highlight the importance of clinical suspicion and early diagnosis to prevent cardiopulmonary complications.
