Delayed surgical management of extensive bilateral multilobar congenital pulmonary airway malformation

Management of bilateral congenital pulmonary airway malformations (CPAM) distributing multiple lobes is challenging since it requires complicated surgical decision-making regarding when and how much abnormal lung parenchyma should be resected with minimal risk of postsurgical respiratory failure.We present a case of antenatally diagnosed bilateral CPAM distributed almost to the entire lungs, which had a favourable outcome with optimised conservative and surgical management. The management was started with maximal ventilatory support, and when and how much of the lesions should be resected was carefully assessed with serial CT and lung perfusion and ventilation scintigraphy. Growth of healthy parenchyma was observed during the conservative management, and then sufficient functioning lung parenchyma could be preserved.