先天性囊性腺瘤样畸形的病程转归

Review of courses and outcomes in congenital cystic adenomatoid malformations

先天性囊性腺瘤样畸形是先天性肺发育畸形的一种,表现为肺部错构瘤样的病变。大多在产前常规超声检查(孕18~22周)中发现,表现为肺部异常回声团块。在胎儿阶段,部分胸腔内包块减小或消失,极少数病例伴随胎儿水肿,且往往预后不良。新生儿期少数病例发生严重的并发症,需要收入新生儿重症监护室或接受紧急手术。而大部分生后无症状,在随访观察期间,病灶可能会自行消退,也可能出现呼吸道感染等症状,因此需要考虑择期手术治疗。关于先天性囊性腺瘤样畸形的自然转归尚没有大数据统计,现将相关文献总结综述。

Congenital cystic adenomatoid malformation(CCAM)refers to a hamartomatoid lesion in lung.Routinely detected by prenatal ultrasonography during 18-22 weeks of gestation,it presents as an abnormal echogenic mass of lung.During fetal period,some intrathoracic masses diminish or disappear.Few cases are accompanied by fetal hydrops with a poor prognosis.Severe complications are unusual during neonatal period,requiring admission into NICU or emergency surgery.In contrast,most cases present asymptomatic postnatally.During follow-ups,there are self-regression and respiratory infection.Thus elective surgery is considered.Large data are lacking on natural regression of CCAM and the relevant literature is summarized.