胎儿肝内型门静脉-体静脉异常连接的产前超声诊断及预后分析

Prenatal ultrasound diagnosis and prognosis analysis of abnormal connection of fetal intrahepatic portosystemic shunt

目的探讨产前超声对胎儿肝内型门静脉-体静脉异常连接的诊断价值。方法回顾性分析17例肝内门静脉-体静脉异常连接胎儿的产前超声诊断特征,观察其妊娠结局并追踪随访出生进展状况。结果17例肝内门静脉-体静脉异常连接胎儿Park分型Ⅱ型14例(82.35%),Ⅲ型2例(11.77%),Ⅳ型1例(5.88%);其中1例合并食管闭锁和静脉导管缺失,1例合并双肾发育不良和双足姿势异常,2例合并静脉导管缺失,1例合并迷走右锁骨下动脉,其余均未见其他合并畸形。1例出现宫内生长受限,1例出现心功能不全,其余均未见其他并发症。妊娠结局:2例终止妊娠,1例宫内死亡,14例自然分娩(其中1例异常分流宫内自发闭合)。随访时间6~36个月,9例出生后异常分流自发闭合,2例行介入栓塞治疗,1例分流仍持续性存在,1例失访。结论产前超声对胎儿肝内型门静脉-体静脉异常连接具有较高诊断价值,可为出生后临床干预提供重要参考信息。

Objective To evaluate the value of prenatal ultrasound in the diagnosis of abnormal connection of fetal intrahepatic portosystemic shunt.Methods The prenatal ultrasonographic features of 17 fetuses with abnormal connection of intrahepatic portosystemic shunt were analyzed retrospectively,and the birth progress was followed up with clinical data.Results Seventeen fetuses with abnormal connection of intrahepatic portosystemic shunt were classified according to Park type,including 14 cases(82.35%)of typeⅡ,2 cases(11.77%)of typeⅢand 1 case(5.88%)of typeⅣ.There was 1 case with esophageal atresia and absent ductus venosus,1 case with renal dysplasia and abnormal foot posture,2 cases with absent ductus venosus,1 case with aberrant right subclavian artery,and no other malformations.1 case was complicated with intrauterine growth retardation,1 case with cardiac insufficiency,and the rest had no other complications.Pregnancy outcome:2 cases with termination of pregnancy,1 case with intrauterine death,14 cases with natural delivery(1 case of abnormal shunt spontaneous intrauterine closure).During follow-up period of 6~36 months,9 cases were spontaneously closed postnatal abnormal shunt,and 2 cases were treated by interventional embolization,1 case still found persistent shunt,and 1 case was lost to follow-up.Conclusion Prenatal ultrasonography has high diagnostic value for the abnormal connection of fetal intrahepatic portosystemic shunt,and provides important reference information for clinical intervention after birth.