胎儿先天性肺气道畸形的临床病理分析

Congenital pulmonary airway malformation of lung in fetus： a clinicopathological analysis

目的探讨先天性肺气道畸形（CPAM）的病因学、发病机制、临床病理特征及预后、治疗等。方法对18例胎儿CPAM的尸体解剖资料、免疫组织化学结果、临床病史及相关文献进行回顾性分析。结果依照Stocker组织病理学分型，18例CPAM中I型2例，Ⅱ型12例，Ⅲ型4例。证实5例为双侧病变，余13例均为单侧病变和累及单叶。12例为单发畸形，6例合并其他器官和／或系统畸形，合并其他畸形为心脏畸形，颈部水囊状淋巴管瘤伴胸、腹腔积液，1例合并双侧多囊肾、胃肠道闭锁。结论CPAM是一种少见的肺部病变，病因不明，并非真正的肿瘤。影像学检查是发现该病的有效手段，确诊依据病理组织学检查。其预后取决于是否伴发畸形、存在胎儿水肿、肺发育不全。

Objective To study the etiology, pathogenesis, clinicopathologic characteristics, prognosis and treatment of congenital pulmonary airway malformation （CPAM）. Methods Eighteen cases of CPAM were enrolled into the study. The clinical history, autopsy findings and immunohistoehemical results were evaluated, with review of literature. The pathogenetic mechanism, pathologic features and differential diagnosis of CPAM were studied. Results Histologic examination showed that 2 cases were classified as Stoeker type I , 12 cases as type ]l , and 4 cases as type m. The lesion was unilateral and involved single lobe in 13 cases. The remaining 5 cases had bilateral diseases. Of the 18 cases studied, 12 cases showed single organ involvement and 6 cases had malformations affecting muhiple organs. The associated malformations included cardiac anomalies （4 cases ）, polyeystic kidney with gastrointestinal atresia （1 case） and nuchal cystic hygroma with hydrothorax （1 case）. Conclusions CPAM is a rare pulmonary disorder. The etiology of this non-neoplastic condition is unknown. Imaging analysis is a valuable tool to suggest CPAM, while definite diagnosis requires pathologic examination. The overall prognosis is determined by the presence of associated malformations, fetal hydrops and pulmonary hypoplasia.