Congenital pulmonary airway malformation in a 5 month old boy, complicated by pneumonia

Congenital cystic lesions of the lung are rare. The most common congenital anomaly among them, involving the lower respiratory tract, is congenital cystic adenomatoid malformation (CCAM), currently referred to as congenital pulmonary airway malformation (CPAM). They may be incidentally detected on prenatal or postnatal imaging. They usually present within two years of life, rarely in adults. CPAMs may be asymptomatic at birth. Some may opt for observation alone due to lack of evidence on the incidence of long-term complications. However, rarely has a CPAM remained asymptomatic throughout life and complications eventually develop. Pneumonia is most common, which is not amenable to medical treatment alone. CPAMs are notorious for their known malignant potential and they may also lead to pneumothorax, hemoptysis and hemothorax. Computed Tomography Thorax is the investigation of choice. Surgical resection is known to be safe and is the mainstay of treatment. For patients who are diagnosed prenatally, surgery is recommended at 3 to 6 months, so that compensatory lung growth can occur. A five-month old boy from Bhutan, with cough, intermittent fever, respiratory distress and a history of recurrent lower respiratory tract infections, was diagnosed with CPAM, complicated by pneumonia and underwent surgery at our centre, following optimal medical management viz. antibiotics and supplemental oxygen. The post-operative course was relatively uneventful and he was discharged from hospital in 7 days.