胎儿支气管肺隔离症的产前评估、治疗与结局——68例临床分析

Prenatal diagnosis, treatment and outcomes of fetal bronchopulmonary sequestration-analysis of 68 cases

目的总结和分析胎儿支气管肺隔离症（bronchopulmonary sequestration，BPS）的产前评估和诊疗结局，提高对胎儿BPS的认识。方法回顾性分析广东省妇幼保健院2009年1月20日至2013年1月31日产前诊断的68例BPS胎儿资料，通过超声检查确诊，结合胎儿肺部肿块体积与胎儿头围比值（volume to head circumference ratio，CVR）测定进行专业评估，出生后选择性行新生儿或婴幼儿期手术治疗并随访。结果68例均经产前超声检查发现胎儿胸部实性或高回声肿块，初次发现平均孕周24周（18～30周），均经2次以上超声检查确诊为BPS。68例胎儿中CVR〈1．6者56例（82．4％）；CVR≥1．6者12例（17．6％），其中7例合并胎儿水肿。除2例CVR≥1．6合并胎儿水肿的孕妇主动要求终止妊娠外，其余66例均继续妊娠至出生并全部存活。66例中，58例（87．9％）出生时无呼吸困难等症状，8例（12．1％）出生时有症状。18例妊娠晚期超声检查肿块消失，但在出生后计算机X射线断层扫描（computed tomography，CT）检查中14例仍可见肿块，另4例则尚未行CT检查。共45例于生后早期行肺叶或隔离肺切除术，其中5例于新生儿期行急诊手术，所有手术病例均全部治愈。45例手术病理分型显示肺叶内型12例；肺叶外型33例，其中6例为膈下BPS。生后3个月～2年随访到的60例新生儿，CT检查其生长发育及肺功能均良好。结论大多数未发生胎儿水肿的BPS胎儿预后良好，胎儿期BPS经产前肺容积专业评估和规范诊疗可以继续妊娠直至分娩。出现胎儿水肿的胎儿，其预后取决于产前评估和新生儿期外科的早期手术干预。CVR是产前评估的重要指标。对于产前超声肿块消失的病例，生后早期的CT检查十分重要。BPS新生儿或婴幼儿早期外科治疗效果满意。

Objective To review and analyze the prenatal diagnosis and treatment of fetal bronchopulmonary sequestration （BPS）. Methods Data of 68 BPS fetuses in the Department of Fetal Medicine, Guangdong Women and Children Hospital and Health Institute from January 20, 2009 to January 31, 2013 were reviewed. All of the 68 fetuses were diagnosed by ultrasound and volume to head circumference ratio （CVR） was measured. Postnatal surgeries at neonatal or infantile period were performed and babies were followed up. Results With antenatal ultrasonography, fetal chest solid or hyper echoic mass was found in all 68 cases at an average of 24 weeks of gestation （18-30 weeks） and were diagnosed as BPS after at least twice ultrasound scans. CVR was measured in these 68 cases, among them 56 cases （82.4%） were 〈1.6, and 12 cases （17.6%） were ≥1.6. Among the 12 cases with CVR≥ 1.6, seven developed fetal hydrops. Except for two terminated pregnancies because of CVR≥ 1.6 with fetal hydrops, all of the rest 66 cases continued their pregnancies till birth and 58 （87.9%） neonates were asymptomatic and eight （12.1%） were symptomatic. The mass was undetectable with ultrasonography in 18 fetuses during the third trimester, however, the mass was identifed again under CT scan after birth in 14 cases （another four cases unexamined）. There were totally 45 cases fully recovered after lobectomy or BPS resection after birth, among which five cases underwent emergency surgery in neonatal period. Pathology results showed that 12 cases were intra-lobar and 33 cases were extraqobar type. Totally, six cases were diagnosed as BPS under diaphragm. Sixty cases were followed up at three months to two years after birth, and all developed well and CT scan indicated normal pulmonary function. Conclusions Most BPS fetuses without hydrops have a good outcome. Fetus with BPS could wait for full term delivery following professional evaluation and standard protocol. However, the prognosis of BPS with fetal hydrops depends on prenatal evaluation and early surgical intervention. And the effect of early surgical intervention in BPS neonates is satisfied. CVR is the most important index for prenatal evaluation of BPS and CT scan after birth is critical for those babies with undetectable mass by prenatal ultrasound.