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目的探讨先天性肺缺如和右位心的发病率、病因及发病机制、临床病理所见及诊断。方法报告经尸检证实的右肺缺如并右位心1例,并复习近期相关文献。结果患儿为出生32天女婴。因口吐白沫、呼吸急促伴口唇发绀1天入院,经抢救无效死亡。尸检主要病变包括:1先天性右肺缺如(包括右肺动脉分支和右肺静脉)并新生儿肺炎(左肺);2伴内脏正位的真性(镜像)右位心。结论婴幼儿肺缺如并右位心是一种极其罕见的先天性畸形。肺缺如临床表现为典型的呼吸道症状,极易误(漏)诊。右位心需与模拟右位心、假性右位心等鉴别。X线、CT、MRI、支气管镜和超声心动图检查有助于肺缺如和右位心的诊断。
Objective To investigate the incidence, etiology and pathogenesis of congenital pulmonary emphysema and right centroid, clinical pathology and diagnosis. Method Reported by autopsy confirmed absence of the right lung and right centrum in 1 case, and review of recent literature. Results The children born 32 days baby girl. Due to vomiting foaming, shortness of breath with cyanosis lips 1 day admitted to hospital, died of rescue invalid. Major autopsy findings include: 1 congenital absence of right lung (including right pulmonary artery branch and right pulmonary vein) and neonatal pneumonia (left lung); 2 true (mirror image) right centimeter with visceral anteroom. Conclusions Infants lacking lung and right locus is an extremely rare congenital malformation. Lack of clinical manifestations of lung as a typical respiratory symptoms, easily missed (missed) diagnosis. Right bit and need to simulate the right centroid, pseudo-right centripetal identification. X-ray, CT, MRI, bronchoscopy and echocardiography contribute to the diagnosis of lung absent and right centroid.