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Scimitar syndrome or pulmonary venolobar syndrome is a rare, complex and variable cardio-pulmonary malformation, characterized by partial anomalous pulmonary venous connection. It can present in neonatal period as well as later in life. We present a case of 13 year old female who presented with DKA in type 1 diabetes mellitus and was diagnosed with pulmonary venolobar syndrome. Considering the rareness of the syndrome and its unusual form of presentation, this case is reported. Scimitar syndrome is a congenital deformity which consists of abnormal right side pulmonary venous drainage in the inferior vena cava, right lung hypoplasia, dextraposition of heart, and anomalous systemic arterial supply from aorta or one of its branches to the right lung. This syndrome has varied presentations, from an asymptomatic state to severe pulmonary hypertension and/or heart failure. A 13 year old female child presented with vomiting, abdominal pain & breathlessness and was diagnosed with DKA who was a known case of type 1 diabetes mellitus on insulin therapy. Detailed examination and routine work up was done. Chest skiagram (PA) showed scimitar sign formed by anomalous vein with right sided lung hypoplasia and shift of trachea and heart to the right side. Electrocardiography was normal except R/S >1 in V1. Transthoracic echocardiography showed no evidence of any septal defect or other abnormality. HRCT thorax was performed which showed, hypoplastic right lung with complete non visualization of the right upper lobe and anomalous drainage of the right middle & lower lobe veins into the sub-diaphragmatic, supra hepatic portion of the inferior vena cava. No medical management or surgical interventions was required as patient was asymptomatic.
Rosane Cavalcante Fragoso, Brasil
Chief Scientific Officer and Head of a Research Group
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