Management of Giant Congenital Pulmonary Airway Malformations Requiring Pneumonectomy

Emergent total pneumonectomy can be lifesaving, although significant pulmonary HTN can be expected. Management options may include fetal surgical intervention, ex utero intrapartum treatment (EXIT), and expectant management. Extracorporeal membrane oxygenation (ECMO), a prolonged ICU course, and symptomatic tracheomalacia/bronchomalacia may be anticipated. Families can be counseled that survival is achievable with adequate contralateral lung development.