胎儿先天性肺囊腺瘤样畸形超声声像图特征及预后分析

目的：探讨胎儿先天性肺囊腺瘤样畸形（CCAM）超声声像图特征，为胎儿临床结局评估提供依据。方法分析21例产前超声诊断为胎儿CCAM的超声声像图特征，并与产后CT、手术及随访结果进行对照。结果（1）21例胎儿CCAM产前超声声像图特征：CCAMⅠ型2例（10%，2/21），CCAMⅡ型13例（62%，13/21），CCAMⅢ型6例（28%，6/21）；位于一侧胸腔20例（95%，20/21），双侧胸腔1例（5%，1/21）；首次检出胎儿CCAM的孕周为18＋4~25＋4周，中位孕周为22＋6周；胎儿CCAM体积与头围比（CVR）＜1.6者16例（76%，16/21），CVR＞1.6者5例（24%，5/21）；动态观察病灶达最大体积后维持不变8例（38%，8/21），达最大体积后逐渐缩小13例（62%，13/21）（其中消失5例）。（2）合并症：19例（90%，19/21）出现伴发症状或合并其他结构畸形，均伴发纵隔移位，羊水过多6例，膈疝1例，肺隔离症1例，胎儿水肿1例。（3）随访结果：16例产前超声检查与出生后胸部CT结果相符，其中9例经手术及病理证实；1例引产者与尸体解剖病理诊断符合；余4例产前超声动态复查提示肿块缩小，无合并症，出生后未行检查及治疗。（4）临床预后：19例（90%，19/21）预后良好，1例合并胎儿水肿者孕妇选择性终止妊娠，1例合并膈疝者出生后1d死亡。结论产前超声检查是诊断胎儿肺囊腺瘤的首选和可靠的方法，产前动态超声检查是评估及预测胎儿CCAM预后的关键，单纯CCAM预后良好。

Prenatal ultrasonographic diagnosis and prognosis of congenital cystic adenomatoid malformation

Objective To investigate the ultrasound characteristics of congenital cystic adenomatoid malformation （CCAM）. Methods The ultrasonographic features of 21 cases of fetal CCAM confirmed by prenatal ultrasound were analyzed. And ultrasound ifndings were compared with the postnatal thoracic computed tomography （CT）, surgical result and follow-up data. Results （1） Prenatal ultrasonographic ifndings：2 cases of CCAM typeⅠ（10%, 2/21）, 13 cases of typeⅡ（62%, 13/21）, and 6 cases of typeⅢ（28%, 6/21）. Of them, 95%（20/21） lesions were located in unilateral thorax, while 5%（1/21） in bilateral thorax. CCAM was ifrst detected at 18th-25th gestational week with a median of 22nd week. The CVR was〈1.6 and〉1.6 in 16 （76%, 16/21） and 5 （24%, 5/21） cases respectively. After reaching the maximum volume, the size of mass remained stable in 8 cases and regressed in 13 cases with even complete remission in 5 cases. There were mediastinal shift in 19 fetuses （90%, 19/21）, polyhydramnios in 6, and concomitant structural abnormalities in 3 cases such as pulmonary sequestration, diaphragmatic hernia and fetal hydrops. （2） Clinical outcomes：15 newborns were conifrmed as CCAM by CT, in which 9 cases underwent surgical resection. One case was conifrmed by autopsy. Nineteen cases showed good prognosis. Whereas 1 pregnancy with fetal hydrops was terminated, and 1 fetus with diaphragmatic hernia died after birth. Conclusions Dynamic prenatal ultrasound assessment is the key factor in prognosis prediction of fetal CCAM. CCAM without other structural abnormalities has a good outcome.