胎儿先天性肺囊性腺瘤样畸形35例临床分析

Clinical analysis of 35 congenital cystic adenomatoid malformation(CCAM) of the lung in fetal

目的探讨胎儿先天性肺囊性腺瘤样畸形(CCAM)的临床特点和预后。方法回顾性分析2004年2月至2009年7月在复旦大学附属妇产科医院经产前超声诊断为CCAM的35例胎儿的临床资料。超声监测CCAM病灶大小及并发症的变化。结果超声检查提示:Ⅰ型15例,Ⅱ型11例,Ⅲ型8例,Ⅰ型合并Ⅲ型1例。14例合并纵隔或心脏移位,3例合并羊水过多,3例合并颈部透明带增厚,1例合并脑积水,2例合并胎儿生长受限,1例合并胎儿腹水和水肿,1例合并羊水过多和消化道畸形。7例行羊水穿刺染色体检查,未发现异常核型。8例诊断后失访。27例随访病例中,1例伴有水肿和腹水的胎儿孕29周胎死宫内,11例孕妇终止妊娠,15例胎儿分娩。15例婴儿中,6例妊娠期间病灶消失,4例产后病灶消失,2例未消失,3例暂未随访。产后15例婴幼儿身体健康。结论胎儿水肿和腹水是胎儿预后不良的因素。如果胎儿未出现水肿、腹水,未合并其他畸形,胎儿CCAM预后良好,可继续妊娠。

Objective To investigate＇ the clinical feature and prognosis of congenital cystic adenomatoid malformation （CCAM） of the lung in fetal. Methods From February 2004 to July 2009,35 fetuses diagnosed with CCAM by prenatally ultrasonic examinations in Obstetrics and Gynecology Hospital Affiliated to Fudan University were analyzed retrospectively. Ultrasound monitored the changes of the lesion size and the complications. Results The ultrasonic examination indicated that 15 of 35 cases were classified as type Ⅰ , 11 as typeⅡ ,8 as type Ⅲ and 1 was type I combined with type Ⅲ. 14 cases were accompanied with mediastinal or heart shifting, 3 cases with poiyhydramnios, 3 cases with the thickening of nuchal translucency, 1 case with hydrocephalus,2 cases with fetal growth retardation, 1 case with fetal hydrops and ascites, 1 case with polyhydramnios and digestive systerm malformation. 7 of 35 cases underwent amniocentesis and all of the chromosomal karyotype were normal. 8 cases could not be continued to follow up after the first ultrasonic examination. In the follow-up group, one fetus with hydrops and ascites died intrauterine at 29 weeks, 11 women terminated their preg- nancies, 15 cases delievered. Of 15 infants, lesions of 6 cases disappeared at pregnancy, 4 cases disappeared postnatal- ly, lesions of 2 cases still existed, 3 cases were not followed up. 15 infants were healthy. Conclusion Fetal hydrops and ascites are the unfavourable factors for the CCAM prognosis. If fetuses hre not complicated with hydrops, ascites and other malformations, the prognosis of CCAM is good, these women should be advised to continue their pregnancies.