Congenital lobar emphysema (CLE) is a rare congenital abnormality characterized by over inflation of a pulmonary lobe. It often presents a diagnostic and therapeutic dilemma. No case has been described in Uganda in the previous 10 years. We describe a case of a 2 months old infant who presented with severe respiratory distress. A diagnosis of congenital left upper lobar emphysema was established on diagnostic imaging. Both his vascular anatomy and the bronchial wall were normal. He improved dramatically after surgery. The SaO2 normalized within 12 hours of Surgery (lobectomy) and the postoperative period was uneventful.
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