The Surgical Management Strategies in Congenital Pulmonary Airway Malformations: According to the Location of the Pulmonary Involvement

Abstract

Aim: Treatment of congenital pulmonary airway malformations (CPAMs) consists of different surgical options. In this study, we aimed to report our surgical management strategy according to the location of pulmonary involvement. Materials and Methods: We retrospectively analyzed the medical records of patients who underwent surgery for CPAMs between 2005 and 2015. The data including patient demographics, pre-operative clinical features, surgical management strategies and postoperative results were reviewed. Results: Twenty patients (14 male, 6 female) with a median age of 4 months (1 day-12 years) were operated on. Antenatal diagnosis was positive in 12 patients (60%). The other patients were admitted with a median age of 3.5 years (1 day-12 years). Respiratory infection was seen in four patients (20%). Nine patients underwent early operation due to severe dyspnea and recurrent respiratory infection (45%). Lobectomy was performed on 17 patients with single lobe involvement (85%). Lobectomy for major lesion and segmentectomy for minor lesion was performed on two patients with unilateral multi-lobar involvement. One patient with bilateral multi-lobar involvement required multiple thoracoscopic wedge resections. Two patients who had severe dyspnea before surgery required mechanical ventilation after the operation, and one of them died. Two postoperative complications, empyema and pneumothorax were seen. Mean postoperative follow-up period was 5.5 years. Conclusion: CPAMs must be excised totally due to the risk of pulmonary infection and malignancy. The resection strategy should be decided according to the number of the affected lobes. Lobectomy should be performed in single lobar involvement. Unilateral multi-lobar involvement requires lobectomy for a major lesion and segmentectomy for a minor one. Thoracoscopic multiple wedge resections should be the option in bilateral multi-lobar CPAMs.

Keywords

• Congenital pulmonary airway malformation
• congenital cystic adenomatoid malformation
• lung cyst
• children