Clinical features and outcomes of adult congenital pulmonary airway malformation: a 47-case retrospective study

Abstract Background Congenital pulmonary airway malformation (CPAM) is predominantly diagnosed prenatally or in early childhood, whereas adult-presenting cases remain uncommon and are often identified incidentally or after the development of respiratory symptoms. However, clinical evidence regarding adult CPAM remains relatively limited, particularly concerning surgical management and long-term outcomes. 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 48 years. Common lesion sites were the left lower lobe (31.9%) and right lower lobe (36.2%). Nineteen patients (40.4%) underwent sublobar resection and 28 (59.6%) lobectomy. Preoperative infection occurred in 17 patients (36.2%), who more frequently presented with dry cough and fever and had lower DLCO, 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 additional pathological findings in 18 cases (38.3%), including infection-related secondary pathological changes and associated lesions, as well as one case of mucinous adenocarcinoma. Conclusions Adult-presenting CPAM is an uncommon but clinically relevant condition with heterogeneous manifestations. Secondary infection, chronic inflammatory changes, and other pathological alterations were frequently observed. Surgical resection appears to be safe and feasible, with favorable postoperative outcomes.