先天性肝内门体分流是一种极为罕见的血管畸形，其异常分流道连接肝内门静脉与肝静脉或下腔静脉，导致部分血液绕过肝脏直接进入体循环从而引发高氨血症、高半乳糖血症等，严重者并发肝肺综合征、肺动脉高压、肝性脑病，治疗较为棘手。现报道1例以肝肺综合征为首发表现的先天性肝内门体分流V型患儿，应用Amplatzer血管塞Ⅱ型成功介入栓塞的案例，术后1d复查血氨降至正常，术后6个月患儿缺氧症状消失，临床指标改善。以期加深对该病的认识，提示介入治疗可作为部分患儿的首选治疗方案。.

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BACKGROUND: Congenital portosystemic shunt (CPSS) is a rare congenital portal malformation. 35.5% of CPSS is intrahepatic. Certain intrahepatic CPSS cases can be spontaneously closed within 2 years after birth. However, the intrahepatic CPSS with clinical symptoms or large shunt requires immediate treatment. In the present study, we used a laparoscopic ligation of portosystemic shunt to treat one newborn case.
METHODS: An abnormal shunt between the portal and the hepatic vein was initially detected by a prenatal ultrasound in one newborn infant. The postnatal ultrasound and CT indicated a connection between the left portal and hepatic vein and a venous cyst at the anterior edge of the left liver. Owing to the hyperammonemia and the large shunt from the portal into the hepatic vein, the infant was treated with a laparoscopic ligation of the shunt. During the operation, the left portal vein and its branches were dissected and the anterior branch of the left portal vein was clamped using a Hem-o-lok. The venous cyst was sutured and ligated using a prolene suture.
RESULTS: The surgery was completed successfully. The duration of the operation time was 60 min. The postoperative hospital stay was 3 days. The preoperative portal angiography indicated an intrahepatic shunt between the left portal and the left hepatic vein and a venous cyst. Postoperative portal angiography indicated the disappearance of the abnormal shunt and the venous cyst. The portal pressure was increased from the preoperative 6 cmH2O to the postoperative 12 cmH2O. The duration of the follow-up was 22 months. Following surgery, the level of serum ammonia returned to normal. The postoperative CT demonstrated that the abnormal shunt had disappeared. No symptoms of portal hypertension were noted following surgery, such as splenomegaly, hypothrombocytopenia, and upper gastrointestinal bleeding.
CONCLUSIONS: The laparoscopic ligation of portosystemic shunt is suitable for the treatment of the intrahepatic CPSS in the newborn patient.

This study aimed to investigate the feasibility and effectiveness of surgical ligation in the treatment of a congenital extrahepatic portosystemic shunt (Abernethy malformation) in children.
Twelve children (aged 10 days to 13.3 years; six boys and six girls) with Abernethy malformation were admitted to the Capital Institute of Pediatrics (Beijing, China) from May 2014 to November 2019 owing to hyperammonemia. Among them, nine suffered from hematochezia, two had elevated liver transaminase levels or jaundice, and one had hypoxemia. Their surgical procedures and outcomes were retrospectively analyzed. Portal pressure measurement and angiography of the portal vein were performed before and after the occlusion of the portosystemic shunt during the surgery. Six patients underwent a single-stage ligation of the portosystemic shunt, five underwent a two-stage ligation of the portosystemic shunt, and one was treated with a partial ligation of the portosystemic shunt.
Each of the operations was successfully completed with an operative time ranging from 60 to 240 minutes and with an intraoperative blood loss ranging from 50 to 200 mL. Three children had a blood transfusion. The postoperative portal pressure was significantly higher than that before the surgery in all children. The patients were followed for 5 to 70 months. The symptoms of hematochezia were relieved in nine patients after the surgery. The levels of blood bilirubin and transaminase returned to normal after the surgery in two patients. The oxygen saturation returned to normal in one patient. The level of blood ammonia was normal after the surgery in all patients.
The surgical ligation of the portosystemic shunt was an effective method to treat a type II Abernethy malformation. According to the portal pressure after the occlusion of the portosystemic shunt, a single or staged procedure was selected.

OBJECTIVE: To review our experience with the prenatal diagnosis of congenital portosystemic shunt (CPSS).
METHODS: This is a retrospective study of CPSS cases examined at an ultrasonographic tertiary referral center from 2013 to 2019. The anatomical origin and drainage of the shunt were assessed. Feto-maternal clinical characteristics and long-term outcomes were investigated via medical files and telephone interviews with the mothers.
RESULTS: Eleven cases were reviewed. Based on the anatomical origins, before or after portal vein division, cases were classified into extrahepatic portosystemic shunt (EHPSS, n = 3, 27.3%) and intrahepatic portosystemic shunt (IHPSS, n = 8, 72.7%). Additional abnormalities were also observed in the EHPSS (n = 2, 66.7%) and IHPSS (n = 3, 37.5%) groups. Intrauterine growth restriction was the most common abnormality (n = 4, 80%). The median age of the pregnant women was 31.9 years (range 26 ~ 43 years). Most cases (n = 8, 72.7%) were diagnosed in the third trimester, and the median gestational age was 31+5 weeks (range 24 ~ 36+6 weeks). Three cases underwent karyotype examinations, and one had trisomy 13. The median time after birth was 2 years (range 0.7 ~ 5.7 years). The overall postnatal live-birth rate was 60% (6/10), not including one case with no data on pregnancy outcome. The mothers of the six live births indicated that their children were in excellent health.
CONCLUSIONS: This study indicates that prenatal CPSS diagnosis is feasible, especially in the third trimester. IHPSS is more common than EHPSS. Complicated cases most often occur with EHPSS. Intrauterine growth restriction is the most common concomitant abnormality. The prognosis of most cases is good.

BACKGROUND: The clinical presentation of congenital portosystemic shunt is variable and gastrointestinal bleeding is an uncommon presentation.
OBJECTIVE: To describe the imaging features of congenital portosystemic shunt as it presented in 11 children with (n = 6) and without gastrointestinal bleeding (n = 5).
METHODS: We performed a retrospective study on a clinical and imaging dataset of 11 children diagnosed with congenital portosystemic shunt.
RESULTS: A total of 11 children with congenital portosystemic shunt were included in this study, 7 with extrahepatic portosystemic shunts and 4 with intrahepatic portosystemic shunts. Six patients with gastrointestinal bleeding had an extrahepatic portosystemic shunt, and the imaging results showed that the shunts originated from the splenomesenteric junction (n = 5) or splenic vein (n = 1) and connected to the internal iliac vein. Among the five cases of congenital portosystemic shunt without gastrointestinal bleeding, one case was an extrahepatic portosystemic shunt and the other four were intrahepatic portosystemic shunts.
CONCLUSIONS: Most congenital portosystemic shunt patients with gastrointestinal bleeding had a shunt that drained portal blood into the iliac vein via an inferior mesenteric vein. This type of shunt was uncommon, but the concomitant rate of gastrointestinal bleeding with this type of shunt was high.