Clinical Features and Outcomes of Adult Congenital Pulmonary Airway Malformation: A 47-Case Retrospective Study

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Abstract

Background Congenital pulmonary airway malformation (CPAM) is a rare developmental anomaly of the lung that is predominantly diagnosed in infancy, whereas adult cases remain uncommon. Comprehensive evidence regarding clinical characteristics, surgical management, and long-term outcomes in adults is limited. Methods We retrospectively reviewed 47 adult patients with pathologically confirmed CPAM who underwent surgical resection at a single tertiary center from 2012 to 2025. Clinical data, radiologic features, operative details, pathological findings, and follow-up outcomes were analyzed. Subgroup analyses were performed according to age and preoperative respiratory infection status. Results The cohort included 20 males and 27 females with a median age of 44 years. Common lesion sites were the left lower lobe (31.9%) and right lower lobe (36.2%). Nineteen patients (40.4%) underwent sublobectomy and 28 (59.6%) lobectomy. Preoperative infection occurred in 17 patients (36.2%), who exhibited more respiratory symptoms, lower pulmonary function, longer operative times, and prolonged chest drainage compared with non-infected patients (all p < 0.05). Younger patients presented more frequently with infections and larger lesions, and lobectomy was more commonly performed in this group. Postoperative complications were infrequent, with no perioperative mortality. During follow-up, 85.1% of patients reported preserved physical strength and activity tolerance. Pathology revealed concomitant pulmonary lesions in 18 cases (38.3%), including one mucinous adenocarcinoma. Conclusions Adult CPAM exhibits heterogeneous clinical manifestations and is frequently complicated by infection or other pulmonary diseases. Surgical resection is safe and effective, providing favorable long-term functional recovery. Personalized surgical planning is essential, and more studies are required to refine management strategies.

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