Congenital pulmonary airway malformation (CPAM) with initial presentation in an adult: a rare presentation of a rare disease

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Abstract

Congenital pulmonary airway malformation (CPAM) is a rare congenital abnormality with unknown exact aetiology or clear genetic association. It is characterised by a failure of bronchial development and localised glandular overgrowth. Typically, it is diagnosed on prenatal ultrasound, only infrequently in children, and even less commonly in adults. We present a case of a 25-year-old man, with no previous lung diseases who presented with right-sided chest pain, fever and cough suggestive of pulmonary infection. Chest imaging, including CT scan, showed a large focal cystic mass within the right lower lobe along with ground glass opacities suggestive of CPAM. He was started on intravenous antibiotics. Bronchoscopy showed a large amount of pus in the right lung and bronchoalveolar lavage confirmed the microbiological diagnosis of methicillin-resistant Staphylococcus aureus. He improved with antibiotic treatment. He was discharged with 6-week course of antibiotics and follow-up afterward.

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Abu Omar, M., Tylski, E., Abu ghanimeh, M., & Gohar, A. (2016). Congenital pulmonary airway malformation (CPAM) with initial presentation in an adult: a rare presentation of a rare disease. BMJ Case Reports , 2016. https://doi.org/10.1136/bcr-2016-216957

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