A RARE CAUSE OF UNILATERAL BRONCHIECTASIS: SWYER-JAMES-MACLEOD SYNDROME

TYPE: Case Report TOPIC: Pulmonary Vascular Disease INTRODUCTION: Swyer-James Mcleod syndrome (SJS) is a rare disease resulting from post-infectious bronchiolitis at young age. Fibrosis of bronchioles and pulmonary vessels hinder growth the lung, causing alveolar hyperinflation hypoperfusion. It can remain undiagnosed for decades. CASE PRESENTATION: A 53-year-old lady with bronchial asthma presented poor response to treatment productive cough. The clinical evaluation did not support diagnosis asthma. CT-Chest showed hypoplastic right lung bronchiectasis changes on mid lower lobes. Air trapping, oligemia, septal thickening were seen upper zone. left was hyperinflated, herniating mediastinal shifting. Contrast CT revealed absence artery peripheral pruning veins. Two arteries descending aorta supplied lung. Echocardiogram normal. Bronchoalveolar lavage grew pseudomonas galactomannan positive. Antibiotics antifungals led improvement. Considering long-standing stable disease, medical management offered bronchiectasis. DISCUSSION: SJS an acquired form unilateral Undetected decades, present as or Congenital mimics are artery, agenesis, aplasia, proximal interruption ventilatory defects like lobar emphysema. When late, radiological differentiation could be challenging. However, late presentation, in resource settings, has similar approaches. CONCLUSIONS: yet important entity that life many mimics. Careful exclude conditions prevent undue surgical interventions. DISCLOSURE: Nothing declare. KEYWORD: Swyer James MacLeod