Lonsane Abhijeet Rameshwar, Wankhede Vaibhav Vishwambhar, Tirpude Sneha, Rout Abhipsa, Kekre Geeta
Department of Respiratory Medicine, Ruby Hall Clinic Pune, Maharashtra, India.
Department of General Surgery, Ruby Hall Clinic Pune, Maharashtra, India.
Lung India. 2023 Jul-Aug;40(4):349-352. doi: 10.4103/lungindia.lungindia_437_22.
Congenital pulmonary airway malformation (CPAM) is most common congenital lung anomaly of lower respiratory tract accounting for approximately 25% of all congenital pulmonary malformations. It is usually unilateral and involves single lobe of lung. It is usually diagnosed prenatally; rarely found in children and adults. We report a rare case of 14-year-old male presented with sudden onset breathlessness secondary to right sided pneumothorax associated with right lower lobe cystic lesion; successfully managed with multidisciplinary approach involving tube thoracostomy and non-anatomical wedge resection of right lower lobe cystic lesion (using VATS). Adults diagnosed with CPAM usually present with breathlessness, fever, recurrent pulmonary infection, pneumothorax, and haemoptysis. For definitive treatment of symptomatic CPAM cases, surgical resection at the time of diagnosis is recommended in view of possible risk of malignant transformation and recurrent respiratory tract infections. Considering the mild but definitive risk of malignancy, it is advocated to closely monitor the individuals with CPAM even after the surgical resection.
先天性肺气道畸形（CPAM）是下呼吸道最常见的先天性肺异常，约占所有先天性肺畸形的25%。它通常为单侧，累及单肺叶。通常在产前诊断；在儿童和成人中很少发现。我们报告一例罕见病例，一名14岁男性因右侧气胸继发突然发作的呼吸困难，伴有右下叶囊性病变；通过多学科方法成功治疗，包括胸腔闭式引流术和右下叶囊性病变的非解剖性楔形切除术（使用电视辅助胸腔镜手术）。诊断为CPAM的成人通常表现为呼吸困难、发热、反复肺部感染、气胸和咯血。鉴于可能存在恶性转化和反复呼吸道感染的风险，对于有症状的CPAM病例，建议在诊断时进行手术切除以进行确定性治疗。考虑到存在轻度但明确的恶性风险，即使在手术切除后，也主张密切监测CPAM患者。
