胎儿先天性肺囊腺瘤样畸形产前超声诊断及临床意义

Congenital cystic adenomatoid malformation of the lung:prenatal ultrasound diagnosis and clinical significance

目的探讨产前超声检查对胎儿先天性肺囊腺瘤样畸形(CCAM)的诊断价值及临床意义。方法对39例产前超声诊断为CCAM胎儿的超声声像图特征进行总结分析;39例胎儿中28例引产,尸体解剖病理证实26例为CCAM;其余11例胎儿均随访至出生后6个月,随访结果证实该11例均为CCAM。结果 (1)产前超声表现:39例胎儿产前超声检查均显示胸腔内异常回声,其中双侧3例,单侧36例(左侧15例,右侧21例);超声分型CCAMⅠ型4例,CCAMⅡ型16例,CCAMⅢ型19例。(2)伴发症状及畸形:36例胎儿(36/39,92.3%)出现伴发症状及畸形,均伴发纵隔移位,羊水过多6例,胸腔积液4例,腹腔积液3例,胎儿全身水肿3例,心包积液2例,肝囊肿2例,胎盘增厚1例,颈部水囊瘤1例;3例胎儿(3/39,7.7%,CCAMⅢ型)未出现其他伴发症状和畸形。(3)产后随访结果及妊娠结局:28例引产胎儿中26例产前超声分型诊断与产后病理诊断符合,2例隔离肺产前超声检查误诊为CCAMⅢ型。其余11例胎儿随访至出生后6个月,超声随访示2例肿块逐渐缩小至消失,余9例胎儿出生后MRI诊断为CCAM,产前超声分型诊断与产后随访诊断结果均相符。产前超声诊断符合率为94.9%(37/39),误诊率为5.1%(2/39)。结论产前超声分型对评估CCAM胎儿预后有重要临床意义。

Objective To explore the clinical significance and the diagnostic value of prenatal ultrasonography for fetal congenital cystic adenomatiod malformation（CCAM）of the lung.Methods A retrospective review was conducted on 39 cases of CCAM diagnosed by prenatal ultrasonography, according to ultrasonographic features about CCAM. All of 39 cases of CCAM were followed up until to the induction of labor or 6 months after birth. The final results were compared with the prenatal ultrasound diagnosis.Results（1）Prenatal ultrasonographic fingdings：39 cases of CCAM diagnosed by prenatal ultrasonography showed the abnormal echo inside the fetal thoracic cavity, inculding 15 cases on the left side, 21 on the right side and 3 on bilateral sides. The prenatal ultrasonography on CCAM were divided into three types： type Ⅰin 4 cases, type Ⅱ in 15, and type Ⅲ in 21;（2）Complicated ultrasound findings：there were 92.3%（36/39） cases of CCAM showed the complicated ultrasound findings, including mediastinal shift（11 cases）, polyhydramnios（6 cases）, hydrops（3 cases）, hydrothorax（4 cases）, ascites（3 cases）, placenta thickening（1 case）, pericardial effusion（2 cases）, hygroma（1 case） , and liver cyst（1 case）. In additional, 3 cases of CCAM（type Ⅲ） were without any other complicated ultrasound findings, 7.7%（3/39）;（3）Pathological diagnosis by autopsy：28 cases were induced abortion. CCAM were confirmed in 26 cases by autopsy and the classification were consistent with the prenatal diagnosis of ultrasonography. Two pulmomary sequestration cases were prenatally misdiagnosed as CCAM type Ⅲ by prenatal ultrasonography;（4）Clinical outcomes in 39 cases：11 cases were followed up until 6 months after birth. Two masses decreased gradually and then disappeared. Nine newborns were confirmed as CCAM by MRI. The prenatal diagnosis of ultrasonography were consistent with the induction of labor and postnatal diagnosis. The accuracy rate of prenatal ultrasonographic diagnosis for CCAM was 94.9%（37/39）, and its misdiagnostis rate was 5.1%（2/39）.Conclusion The diagnosis and classification of prenatal ultrasonography for fetal CCAM play an important role in prognosis assessment.