Rare Congenital Lung Lesions in Even Rarer Locations: A Single-Institution Experience with Extrathoracic Congenital Lung Lesions.

Background: Extrathoracic congenital lung lesions (CLLs) are a rare anomaly of lung development. Management and surgical approach are variable. We present our institutional experience with extrathoracic CLLs for the past 18 years. Methods: We retrospectively reviewed all patients younger than 18 years diagnosed with a CLL from January 2005 to June 2023. Only patients with suspected extrathoracic CLLs on prenatal imaging were included. Results: A CLL was identified in 381 patients and was extrathoracic in 25 patients (6.6%). Six patients (24%) with other congenital anomalies were excluded, and 3 were lost to follow-up. The prenatal course and delivery of the 16 study patients were unremarkable and all were asymptomatic. Larger lesions, both prenatally and postnatally, were more likely to undergo surgical resection (11.8 ± 8.2 cm3 versus 3.9 ± 3.3 cm3, P = .05; 6.1 ± 5.1 cm3 versus 2.5 ± 2.5 cm3, P = .10, respectively). Seven patients (7/16; 44%) underwent resection-5 thoracoscopic and 2 laparoscopic. Thoracoscopic transdiaphragmatic approach was favored for lesions extending >50% above the diaphragm. Five lesions were intradiaphragmatic, one infradiaphragmatic, and one intrathoracic. Four lesions (4/7; 57%) were hybrid lesions on pathology. No malignancy was identified. Nine patients (9/16; 56%) underwent surveillance. Imaging modality for surveillance varied. Duration of follow-up ranged from 0.5 to 83 months. Conclusions: We provide the largest case series to date describing both operative and nonoperative management of extrathoracic CLLs. Surveillance appears to be appropriate for small lesions. If surgical excision is pursued, the thoracoscopic approach is safe and preferred if diaphragmatic involvement is suspected.