Özçelik Neslihan, Özyurt Songül, Yılmaz Kara Bilge, Şahin Ünal
Department of Chest Diseases, Faculty of Medicine, Recep Tayyip Erdogan University, Rize, Turkey.
Tuberk Toraks. 2019 Mar;67(1):71-76. doi: 10.5578/tt.68095.
Congenital pulmonary airway malformation (CPAM), is a rare disease known as a developmental abnormality of the lower respiratory tract. It may occur in the neonatal period due to respiratory distress and patients usually die in the first few months of life. Rarely, it may remain asymptomatic until adulthood. In this study we describe an adult case and present a review of the literature. A 19-year-old male with no relevant medical history was admitted to our clinic with cough and wheeze. The patient had a history of frequent lower respiratory tract infection during childhood. Chest radiograph revealed a diffuse opacity and volume loss in the right hemithorax. High resolution tomography showed hypoplasia of the right hemithorax, multiple cysts in all of the lobes and segments on the right side, ground glass opacity and interlobular septal thickening of the whole right lung parenchyma. Right pneumonectomy was performed with the pre-diagnosis of congenital pulmonary airway malformation and the pathological examination was compatible with CPAM. CPAM is a rare disease in adulthood. We should consider CPAM in the differential diagnosis of patients with frequent recurrent pulmonary infection and cystic lung lesions. In order to prevent infections and to eliminate the risk of malignancy, surgical treatment should be applied for definite diagnosis and treatment.
先天性肺气道畸形（CPAM）是一种罕见疾病，被认为是下呼吸道的发育异常。它可能在新生儿期因呼吸窘迫而出现，患者通常在生命的头几个月死亡。极少数情况下，它可能直到成年都无症状。在本研究中，我们描述了一例成人病例并对文献进行了综述。一名无相关病史的19岁男性因咳嗽和喘息入住我们的诊所。该患者童年时有频繁下呼吸道感染史。胸部X线片显示右半胸弥漫性模糊影和肺容积减小。高分辨率断层扫描显示右半胸发育不全，右侧所有肺叶和肺段有多个囊肿，整个右肺实质有磨玻璃影和小叶间隔增厚。在术前诊断为先天性肺气道畸形的情况下进行了右肺切除术，病理检查结果与CPAM相符。CPAM在成年期是一种罕见疾病。对于频繁反复肺部感染和囊性肺病变的患者，我们在鉴别诊断时应考虑CPAM。为了预防感染并消除恶性肿瘤风险，应进行手术治疗以明确诊断和治疗。
