Thoracoscopic surgery for rare congenital pulmonary airway malformations in adults: a decade of retrospective study.

BACKGROUND

Congenital pulmonary airway malformation (CPAM) is a rare benign deformity of the lungs in adults. Our study aimed to evaluate the clinical features and compare the effect of thoracoscopic lobectomy and wedge resection for adult CPAMs.

METHODS

This was a retrospective study including eighteen adults with CPAMs recruited between 2013 and 2023. Radiological scans and pulmonary function test (PFT) were performed before operation. All the patients were treated with thoracoscopic approach, which were categorized into groups of lobectomy and wedge resection. The baseline, preoperative, and operative data were evaluated and analyzed.

RESULTS

Four males and fourteen females were diagnosed with CPAMs at a median age of 57.5 years. Cough was the main symptom, reported by 55.6% of the patients. CPAMs were always initially misdiagnosed as other conditions due to heterogeneous computed tomography (CT) characteristics. The mean of PFT results showed normal (>80% predicted) in forced vital capacity (FVC), forced expiratory volume in 1 s (FEV1), and FEV1 to FVC ratio (FEV1/FVC), but less than 70% predicted in forced expiratory flow (FEF) at 25-75%, 50% and 75% of FVC. All patients underwent video-assisted thoracic surgery (VATS) with a total of nine wedge resections and nine lobectomies. Age at surgery varied statistically between the two groups. Whereas, duration of surgery, blood loss, postoperative drainage, days of drainage, days of hospitalization and postoperative complications showed no statistical difference between the two groups. There were 27.8% of the lesions showing CPAMs mixed with other diseases during histological evaluations.

CONCLUSIONS

CPAM in adults showed a complex presentation in terms of clinical symptoms, imaging performance and pathological findings. Half of the patients were detected with small airway dysfunction preoperatively. Thoracoscopic lobectomy and wedge resection for the treatment can achieve satisfactory short-term outcomes.