胎儿期囊肿型胆道闭锁的临床特点及转归并文献复习

Clinical characteristics and prognosis of cystic biliary atresia in prenatal fetuses with a literature review

目的探讨胎儿期囊肿型胆道闭锁的临床特点及其转归。方法回顾性分析2012年10月至2017年10月10例胎儿期囊肿型胆道闭锁产前产后的超声、MRI等特点，根据术中造影分为A组：Ⅰ型树枝状胆道闭锁，B组：Ⅰ型云雾状胆道闭锁，C组：Ⅲd型胆道闭锁，比较三组术前、术后生化结果，并进行统计分析，并追踪自体肝生存时间及随访转归，评估其手术疗效，并检索Pubmed、Embase数据库、中国知网、万方数据和维普数据中2017年10月以前报道的囊肿型胆道闭锁病例文献复习。结果产前产后超声：三组均表现为胎儿肝门部类圆形、规则、张力较高的囊性包块，囊肿大小不随孕周改变，Ⅰ型和Ⅲd型囊肿大小差异无统计学意义（P〉0.05）。产前产后MRI：Ⅰ型表现为肝门部类圆形、较规则、张力较高囊性包块、囊壁信号增强；Ⅲd型表现为肝门部类圆形、规则、张力高的囊性包块、囊壁信号无增强。术中胆道造影：A组3例，B组3例，C组4例。A组术后1个月丙氨酸转氨酶、天冬氨酸转氨酶有波动，远期逐渐恢复正常；B组术后半年后好转，术后一年半复诊显著升高（P〈0.05）；C组术后半年逐渐好转，一年半复诊较术前显著升高（P〈0.05）。A组胆红素逐渐下降，至术后1个月基本恢复正常；B组术后3个月下降，术后1年半复诊明显升高（P〈0.05） ；C组术后3个月至半年有下降，术后1年半开始显著升高（P〈0.05）；检索文献：2017年10月前检索纳入文献13篇，共89例囊肿型胆道闭锁，加上本组病例共99例。结论产前MRI、超声可初步鉴别囊肿型胆道闭锁与胆总管囊肿，早期诊断治疗2个月具有重要意义，随访三组胎儿期囊肿型胆道闭锁在退黄时间、自体肝生存时间、术后转归，Ⅰ型树枝状胆道闭锁好于Ⅰ型云雾状胆道闭锁，Ⅲd型胆道闭锁较差。

ObjectiveTo explore the clinical characteristics and prognosis of cystic biliary atresia （BA） in prenatal fetuses.MethodsFrom October 2012 to October 2017, retrospective analysis was performed for 10 patients with cystic BA were traced from antenatal, including Doppler ultrasound, magnetic resonance imaging （MRI） in pernatal period and clinical manifestations and Doppler ultrasound, MRI and intraoperative cholangiography in postnatal period.Based upon the findings of interoperative cholangiography, the clinical types were Ⅰ （treelike）, Ⅰ （cloudiness） and Ⅲd.Blood biochemical results, native liver survival and prognosis were collected and analyzed at preoperation and postoperation.SPSS 17.0 was employed for evaluating operative effects.And 89 cases of cystic BA were retrieved from the databases of PubMed, Embase, CNKI, Wangfang and VIP prior to October 2017.ResultsPrenatal ultrasonography： All cases showed cystic lesions of cycloidal, inerratic and high tension in gestation and nearly equirotal during gestation.Pre- and post-prenatal MRI： Type Ⅰ showed cystic lesions of oval, relatively inerratic and high tension in gestation with a high signal of cyst wall; Type Ⅲd showed cystic lesions of cycloidal, inerratic and high tension in gestation with cyst wall without a high signal.Interoperative cholangiography and exploration of biliary tract： the clinical types were Ⅰ （treelike） （n=3）, Ⅰ （cloudiness） （n=3） and Ⅲd （n=4）. All patients were operated.Biochemistry： AST/ALT： Biochemical results of type Ⅰ （treelike） gradually declined to normal.Biochemical results of type Ⅰ （cloudiness） gradually improved after 6 months and 18-month follow-up outcome was unfavorable （P〈0.05）. Biochemical results of Ⅲd declined gradually within 6 months and increased again within 18 months （P〈0.05）. TBIL/DBIL： Bilirubin results of type Ⅰ BA （treelike） gradually declined to normal within 1 month.Type Ⅰ （cloudiness） gradually improved within 3 months and 6-month follow-up showed a marked increase （P〈0.05）. Ⅲd declined within 3-6 months and increased again within 18 months （P〈0.05）. A total of 13 studies involving 89 patients were diagnosed as cystic BA and 10 patients of our study were also included.ConclusionsDoppler ultrasound and MRI in pernatal may preliminarily differentiate cystic BA and choledochal cyst.Early diagnosis and prompt treatment two months are of vital importance.Based upon jaundice disappearance time and native liver survival, type Ⅰ （treelike） has the best prognosis.