Aim of the study
The diagnosis of congenital pulmonary airway malformations (CPAM) is largely antenatal, due to improvements in antenatal imaging. These lesions are mostly asymptomatic at birth and their management remains controversial. Infection and malignant potential are the commonly sited reasons for resection. This study reviews our evolving experience of CPAM management and quantifies the rate of indolent infection.
We conducted a retrospective review over a 16 year period (2000-2016) of patients with CPAM. Details regarding antenatal diagnosis, preoperative symptoms, imaging, and surgery were reviewed. All patients underwent resection. Thoracoscopic surgery was introduced from 2006.
During the study period, 66 patients had a diagnosis of CPAM of which 59 were detected antenatally. The mean gestational age at diagnosis was 22 weeks. 31/66 patients (47%) had postnatal changes visible on chest radiography. All children with an antenatal diagnosis underwent CT scanning (mean age 5 months), with 3-D reconstruction. 10 (15%) patients had clinical evidence of symptoms relating to CPAM, mostly chest infections. All patients underwent operative resection (mean age 14 months). Overall 25 (38%) had thoracoscopic surgery, the incidence of which has significantly increased recently (28% pre-2013, 57% post-2013), reflecting a cohort of initial older inherited patients, where infections caused operative difficulty, and the new technique learning curve. Operative time in the later group was 131 minutes (105-150), reflecting increased proficiency. 31 resections demonstrated histological evidence of infection (47%). There were no deaths/major complications. Mean followup: 3 months.
Thoracoscopic resection is preferable in CPAM and at a younger age before infection causes hilar fibrosis resulting in conversion as in our earlier cases. It can be safely introduced with minimal complications. Histological analyses of resections shows sub-clinical infection in almost half the cases. The clinical significance of that is however not fully clear.