Management of giant congenital pulmonary airway malformations requiring pneumonectomy.

Background: Congenital pulmonary airway malformation (CPAM) is a rare congenital anomaly that occurs sporadically throughout fetal development. CPAM is usually a lobar process, but involvement of an entire lung is exceedingly rare. We present a small series of patients with multilobar CPAM who, to the best of our knowledge, are the first reported cases of giant CPAMs treated with pneumonectomy at birth.

Methods: After institutional review board approval, 93 medical records were reviewed for infants 0 to 12 months with congenital cystic adenomatoid malformation (CCAM) from 1990 to 2011. Three patients with entire lung involvement were included.

Results: Two patients were identified prenatally by ultrasonography and 1 was diagnosed at birth. Prenatally, the 2 patients exhibited mediastinal shift and steroids were administered. A pericardial effusion and decreased movement developed in 1 patient, requiring an emergent cesarean section at 31 weeks' gestation. All 3 patients underwent immediate newborn resection. One infant went into cardiac arrest on delivery and was resuscitated and taken for emergent resection. The 2 patients delivered vaginally at term tolerated spontaneous ventilation without positive pressure. All 3 patients underwent an emergent pneumonectomy on the first day of life. The premature infant died intraoperatively after CPAM removal from severe pulmonary hypertension (HTN) and contralateral lung hypoplasia. The hospital course for these patients was characterized by prolonged stays in the intensive care unit (ICU), pneumonia, ventilator dependence, and pulmonary HTN. Bronchoscopy revealed tracheomalacia in 1 patient that was relieved by aortopexy; the other patient had bronchomalacia requiring tracheotomy.

Conclusions: 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.