泄殖腔外翻综合征前腹壁异常的产前超声诊断

目的总结胎儿泄殖腔外翻（OEIS）综合征前腹壁畸形产前超声声像图特征。 方法回顾性分析2008年1月至2017年3月湖北省妇幼保健院产前超声诊断的45例（46胎）OEIS综合征前腹壁异常胎儿超声声像图表现，并与引产胎儿标本尸检结果对比分析。 结果46胎OEIS综合征均经产后随访证实，26胎有详细的病理检查资料。OEIS综合征胎儿前腹壁畸形产前超声共同特点：胎儿膀胱未显影、低位的腹壁脐带插入处、胎儿下腹壁膨出物向胎儿两股骨间突起。根据膨出物特征46胎OEIS综合征胎儿产前超声声像图表现为5类畸形：（1）以囊性为主的混合性膨出物12胎。（2）脐下腹壁膨出物伴外翻22胎，伴象鼻征13胎。（3）下腹壁脐带根部膨出6胎。（4）大的腹裂畸形3胎。（5）脐膨出伴外翻3胎。 结论OEIS综合征胎儿前腹壁异常超声声像图表现复杂、多样，以囊性为主的混合性膨出物为OEIS综合征早期特有的超声表现，脐下腹壁膨出物伴外翻是OEIS综合征产前特征性表现。

Prenatal ultrasonographic diagnosis of the anterior abdominal wall abnormalities in omphalocele-exstrophy-imperforate anus-spinal defects complex

ObjectiveTo Summarize the prenatal ultrasonographic characteristics of abdominal wall deformity in omphalocele-exstrophy-imperforate anus-spinal defects (OEIS). MethodsThe prenatal ultrasonic characteristics of anterior abdominal wall abnormalities in 46 fetuses with OEIS complex were retrospectively analyzed and compared with the autopsy examination results. ResultsThe 46 fetuses with OEIS were confirmed by follow-up, among which 26 fetuses had detailed autopsy informations. The prenatal ultrasound performance of fetal abdominal wall malformation included absence bladder, low abdominal wall umbilical cord insertion, and lower abdominal wall bulging between the two legs. The bulgings were divided into 5 types according to the prenatal ultrasound characteristics: (1) Predominantly cystic bulging in the lower anterior abdominal wall in 12 fetuses. (2) The abdominal bulging under the umbilical with exstrophy in 22 fetuses, of which were ″elephant trunk-like″ in 13 fetuses. (3) The lower abdominal wall bulgings in the root of the umbilical cord in 6 fetuses. (4) Large gastroschisis malformation in 3 fetuses. (5) Omphalocele with exstrophy in 3 fetuses. ConclusionsThe prenatal ultrasonographic characteristics of abdominal wall deformity in OEIS complex are diverse and complex. The predominantly cystic bulging is the early prenatal sonographic characteristic of the OEIS syndrome and abdominal bulging under the umbilical with exstrophy is the most common prenatal ultrasonic characteristic of OEIS complex.