Congenital Pulmonary Adenomatoid Malformation in three Infants with Diagnostic and Management Challenges in a Low and Middle-Income Country: A Case Series

Résumé

Congenital Pulmonary Adenomatoid Malformation (CPAM)is a rare disorder consisting of harmatomatous and dysplastic lung tissues. Few cases have been reported in Nigeria. We report CPAM in three infants, to highlight the occurrence of this rare condition in our locality, raise awareness of the high index of suspicion, and explore regional management challenges. The first patient, a 6-month-old female, presented in March 2016, with complaints of recurrent cough, fast breathing, and weight loss of 3 months duration. She was initially diagnosed with lobar pneumonia. A computerized tomographic (CT) scan of the chest showed features in keeping with CPAM (type 1). In April 2019 a 12-day-old male presented with high-grade fever, cough, and fast breathing. Chest radiograph revealed pneumonic changes and macrocystic lesions in the right mid and lower lung zones. Chest CT showed thick and thin-walled, rounded, cystic areas of varying sizes within the right hemithorax, suggestive of CPAM. In June 2019, a 3-month-old female presented with a five-day history of fever and cough and a 4-day history of fast breathing. An initial diagnosis of pneumonia with sepsis was made and the patient was commenced on parenteral antibiotics. Chest CT revealed features highly suggestive of CPAM. Patients' acute symptoms resolved following medical management. All the parents declined surgery. The possibility of an underlying congenital lung anomaly though considered rare should be explored when children present with recurrent respiratory tract infections or show poor response to treatment for respiratory tract infections.