A Multi-disciplinary management of a rare case of a complete lung congenital pulmonary airway malformation in a newborn from a developing country

Document Type

Case Report


Paediatrics and Child Health; Neurosurgery


Congenital pulmonary airway malformation (CPAM) is a pulmonary malformation in which parts of the lung are replaced by hamartomatous, cystic, or non-functional abnormal tissue. Herein, we present a case of life-saving pneumonectomy in an infant with type II CPAM which affected entire right lung. A 1-day-old male infant who was a known case of CPAM presented with progressive respiratory distress requiring intubation. There is a paucity of evidence regarding the management protocol of entire lung CPAM. After multi-disciplinary input, right-sided pneumonectomy was performed. Thereafter, he had a prolonged, complicated hospital stay due to prolonged intubation, complicated by pneumonia, sepsis, and metabolic abnormalities. In this report we discuss the etiology, surgical procedure, management strategies, challenges faced in decision making of management, as well as potential complications, which make this case report a valuable asset to the scientific literature. Our case report contributes towards the scare literature present with regards to management of CPAM in the presence of respiratory distress. After receiving appropriate post-operative care, the patient was eventually discharged home in a stable condition. With the advancements in modern obstetric practice, CPAM is detected prenatally and early surgical resection of the affected area is considered the best management option in symptomatic cases of CPAM. A multidisciplinary approach with CPAM diagnosed prenatally and early surgical resection of the affected area is the gold standard evidence-based management protocol that exists.


Pagination are not provided by the author/publisher

Publication (Name of Journal)

International journal of Pulmonary & Respiratory Sciences