Congenital Pulmonary Venolobar Syndrome: Neurodevelopmental Follow-up

Abstract

Congenital pulmonary venolobar syndrome or infantile scimitar syndrome is a rare congenital cardiopulmonary anomaly and has variable presentation, especially in the neonatal period. It is characterized by partial or total anomalous pulmonary venous return from the right lung along with pulmonary hypoplasia. The infantile form is associated with significant mortality and has a worse prognosis due to severe respiratory insufficiency, cardiac failure, and pulmonary infections. We report an infantile scimitar syndrome diagnosed within few hours of life, which has been followed up for 4 years for the presence of intact survival and without neurodevelopmental disorder in the domains of motor, cognition, communication, personal-social, vision, and hearing.