Wu Haifang, Tao Guowei, Cong Xiang, Li Qi, Zhang Jing, Ma Zhe, Zhang Zhonglu
Department of Ultrasound, Qilu Hospital of Shandong University, Jinan.
Medical School of Shandong University, Jinan.
Medicine (Baltimore). 2019 Jan;98(2):e14125. doi: 10.1097/MD.0000000000014125.
An in-utero re-classification of umbilical-portal-systemic venous shunt (UPSVS) has recently been proposed. We retrospectively reviewed the sonograms of a large cohort of fetuses, identified and analyzed UPSVS cases, and presented the prenatal sonographic characteristics, birth outcomes, and follow-up results following the new classification system.Sonograms and clinical data of all participants who visited our departments from April 2016 to July 2018 were retrospectively reviewed. Identified cases of UPSVS were analyzed according to the new classification: Type I: umbilical-systemic shunt (USS); Type II: ductus venosus-systemic shunt (DVSS); Type IIIa: intrahepatic portal-systemic shunt (IHPSS) and Type IIIb: extrahepatic portal-systemic shunt (EHPSS). Postnatal follow-ups ranged from 3 months to 1 year.A total of 10 UPSVS cases were identified in 61,082 fetuses: 4 with Type I, 3 with Type II and 3 with Type IIIa. All 4 cases of USS had complete agenesis of the portal venous system, and had the umbilical vein drained into the inferior vena cava. Two USS cases also had trisomy 21. Pregnancy was terminated in all cases with a Type I shunt. Two fetuses with DVSS had normal portal venous system and were born full term. The pregnancy of 1 DVSS case was terminated due to the detection of trisomy 21. Three cases were IHPSS with full-term birth. One had chromosomal abnormality and 1 had surgery to repair the shunt 12-days post birth. In the 2 cases that did not receive repair surgery, sonographic examination revealed the portal-hepatic venous shunt was not closed at the 6-month follow-up period. However, the 1 case that had repair surgery appeared healthy at the 3-month follow-up period.UPSVS is extremely rare. Type I shunts have the poorest prognosis, and the presence of the intrahepatic portal venous system is key to live birth in UPSVS regardless of types. Chromosomal abnormalities and other organ anomalies can occur in any types of UPSVS. Therefore, karyotyping and examination of other organs should be performed once UPSVS is detected.
最近有人提出了子宫内脐门静脉系统分流（UPSVS）的重新分类。我们回顾性分析了一大群胎儿的超声图像，识别并分析了UPSVS病例，并根据新的分类系统展示了产前超声特征、出生结局及随访结果。
对2016年4月至2018年7月期间来我院就诊的所有参与者的超声图像和临床数据进行回顾性分析。根据新分类对确诊的UPSVS病例进行分析：I型：脐-体循环分流（USS）；II型：静脉导管-体循环分流（DVSS）；IIIa型：肝内门静脉-体循环分流（IHPSS）；IIIb型：肝外门静脉-体循环分流（EHPSS）。产后随访时间为3个月至1年。
在61082例胎儿中，共确诊10例UPSVS：4例I型，3例II型，3例IIIa型。所有4例USS均存在门静脉系统完全缺如，脐静脉汇入下腔静脉。2例USS还合并21三体综合征。所有I型分流病例均终止妊娠。2例DVSS胎儿门静脉系统正常，足月出生。1例DVSS病例因检测到21三体综合征而终止妊娠。3例IHPSS足月出生。1例有染色体异常，1例出生后12天进行了分流修复手术。在2例未接受修复手术的病例中，6个月随访时超声检查显示门静脉-肝静脉分流未闭合。然而，1例接受修复手术的病例在3个月随访时情况良好。
UPSVS极为罕见。I型分流预后最差，无论何种类型，肝内门静脉系统的存在是UPSVS存活出生的关键。任何类型的UPSVS都可能出现染色体异常和其他器官异常。因此，一旦检测到UPSVS，应进行染色体核型分析和其他器官检查。
