Surgical Treatment for and Prognosis of Congenital Pulmonary Airway Malformation

Abstract

Congenital pulmonary airway malformation (CPAM) is a congenital disease in which cystic lesions form within the pulmonary parenchyma, because the development of the airway or lung are partially interrupted in the fetal lung development. When considering the indications, timing, procedure, and other aspects of surgery for CPAM, it is imperative to appropriately understand the anatomical and pathophysiological differences between CPAM and other congenital cystic lung diseases (CCLD). The major anatomical characteristic of CPAM is that the cystic lesions are communicated with the normal airways. Since lesions seen in the fetuses with CPAM are generally larger compared with other CCLDs, neonates with CPAM tend to be associated with hydrops and pulmonary hypoplasia and often develop persistent pulmonary hypertension of the newborn (PPHN). Therefore, infants with CPAM have a high risk of severe postnatal respiratory impairment. Surgical treatment during the neonatal period is necessary for the infants with CPAM who reveal any respiratory symptoms after birth. Surgery should be considered even in the asymptomatic cases of CPAM after birth in order to prevent respiratory infection. If all cystic lesions of the CPAM are not treated, the residual cysts could act as a nidus for postoperative infection. Therefore, all affected lesions must be completely resected if the affected lesions are limited in a single lobe. although the affected lesions should be resected as much as possible even in a case of multiple lobes are involved, resection of multiple lobes including total pneumonectomy in a single procedure should be avoided in order to preserve pulmonary functions of the residual lungs.