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。.

A 70-year-old woman presented with a 6-year history of cognitive dysfunction, neurogenic bladder, constipation and recurrent vomiting, and gradual worsening of symptoms. At the first admission to our department, she was also found to have hepatic encephalopathy due to intrahepatic portosystemic shunt. Head MRI revealed abnormal signal intensity at the corticomedullary junction, the splenium of the corpus callosum, and bilateral middle cerebellar peduncles on DWI. She was diagnosed with intranuclear inclusion disease (NIID) based on skin biopsy and genetic testing of NOTCH2NLC. In a retrospective review of serial head MRI findings for ten years, abnormal signal intensity at the corticomedullary junction and the splenium of the corpus callosum on MRI existed prior to the onset of cognitive dysfunction, and expanded gradually. For early diagnosis of NIID, it is important to focus not only on the characteristic high signal intensity at the corticomedullary junction, but also on the signal at the splenium of the corpus callosum from the early stage.

BACKGROUND: Congenital portosystemic shunt (CPSS) is a rare malformation that leads to hyperammonemia, hypermanganesemia, and various symptoms. CPSSs are divided into intrahepatic and extrahepatic shunts. In patients with persistent CPSS including an intrahepatic portosystemic shunt (IPSS), early intervention to occlude the shunt reverses the associated complications.
METHODS: The patient was a 1-year-and-7-month-old girl. She presented with hypergalactosemia and elevation of blood ammonia level (75 μg/dL) and total bile acid levels (68.2 μmol/L) during the neonatal period. Two IPSSs were detected using ultrasound and enhanced computerized tomography. Magnetic resonance imaging (MRI) at 1 year and 3 months of age showed abnormally high signal intensity in the pallidum of her brain. Spontaneous closure was not observed. We performed a right hepatectomy at 1 year and 7 months of age. The portal vein pressure was 16 mmHg after temporary occlusion of the right portal vein. Blood ammonia and serum manganese levels decreased immediately after the operation. The abnormal signal on brain MRI disappeared. She had a favorable course with no sign of recurrence of IPSS 5 years postoperatively.
CONCLUSIONS: Liver resection for an IPSS to control the symptoms of a portosystemic shunt is reasonable in a child for whom interventional radiological treatment is not indicated.

A young female presented to us with abdominal distension along with pedal edema. She had no prior medical or surgical history apart from a caesarean section done a few years prior. Initial workup showed low hemoglobin, low serum albumin and slight raised ESR. Her LFTs were slightly deranged. Ultrasound abdomen had evidence of portal hypertension along with splenomegaly. While ultrasound hepatic Doppler revealed a portosystemic shunt between the portal vein and the left hepatic vein, with a shunt ratio of 7.1%. CT scan abdomen confirmed these findings and a diagnosis of Type III intrahepatic portosystemic shunt and spleno-renal shunt was made. Since the patient was currently asymptomatic, she was advised regular follow-ups and was managed conservatively.

Vascular malformations are abnormal connections between blood vessels that can have various endothelial characteristics. Although uncommon, these malformations can present challenging diagnostic and therapeutic scenarios. The use of interventional radiology techniques in the management of various vascular malformations is an attractive option because of the ability to treat these malformations at the most appropriate anatomic location and in the most effective manner. Techniques such as coil embolization of intrahepatic portosystemic shunts and liquid embolization of arteriovenous fistulae/malformations have shown tremendous potential as treatments for these challenging diseases.

We analyzed the characteristics of the population with congenital portosystemic shunt diagnosed during the antenatal period and the organization of their perinatal care. This multicentric retrospective study included all the patients with a prenatal diagnosis of congenital portosystemic shunt. Between 1999 and 2015, 12 patients were included. Prenatal diagnosis was done at a median 26.5 weeks of gestation (21-34). All the patients presented intrahepatic CPSS, three of them had associated congenital cardiopathy, and one a Bannayan-Zonana syndrome. Ten patients had simple outcome on conservative treatment, eight of them having a spontaneous closure of their portosystemic shunt within the first 2 years of life. One patient had surgical treatment which failed and he developed a focal nodular hyperplasia. Another patient had radiological interventional closure of his shunt which was complicated by a venal portal thrombosis.
CONCLUSIONS: Outcome of intrahepatic portosystemic shunt diagnosed prenatally is good in the majority of cases. What is known: • Multiples studies exist on congenital porto systemic shunt but when the diagnosis is done after birth. • The evolution, management, and complication are well known. What is new: • There is very few studies with only patients diagnosed in antenatal and it is a large series of cases. • Outcome of intrahepatic portosystemic shunt diagnosed prenatally is good in the majority of cases.

Intrahepatic portosystemic shunts (IHPSS) in dogs are aberrant vascular anomalies that connect the portal and the systemic venous vessels. In most of the patients, the surgical approach is unfavourable due to the difficulties in isolating the IHPSS, making the option of a percutaneous transvenous coil embolization (PTCE) one of the safer occlusive procedures. This study describes the treatment of eight dogs with a single IHPSS using a multimodality imaging approach to guide the modified PTCE procedure. This new technique results in a decrease of 71% of the time of the entire procedure with the reduction of 91% in the time required involved the IHPSS identification and in the fluoroscopy exposure time avoiding the need for iodinated contrast agents during the procedure. Moreover, the placement of the catheter before the caval stent ensures its greater stability, enhancing the procedural safety in the phase when the coils are released and avoiding the risk of their dislocation.

OBJECTIVE: To retrospectively analyze the safety and efficacy of transjugular intrahepatic portosystemic shunting (TIPS) using covered stents in children.
METHODS: We present 6 children (mean age, 10.6 years; mean weight, 33.5kg) who underwent TIPS with 8mm diameter Viatorr(®) covered stents for acute (n=4) or recurrent (n=2) upper digestive bleeding that could not be controlled by endoscopic measures. Five of the children had cirrhosis and the other had portal vein thrombosis with cavernous transformation. We analyzed the relapse of upper digestive bleeding, the complications that appeared, and the patency of the TIPS shunt on sequential Doppler ultrasonography or until transplantation.
RESULTS: A single stent was implanted in a single session in each child; none of the children died. The mean transhepatic gradient decreased from 16mmHg (range: 12-21mmHg) before the procedure to 9mmHg (range: 1-15mmHg) after TIPS. One patient developed mild encephalopathy, and the girl who had portal vein thrombosis with cavernous transformation developed an acute occlusion of the TIPS that resolved after the implantation of a coaxial stent. Three children received transplants (7, 9, and 10 months after the procedure, respectively), and the patency of the TIPS was confirmed at transplantation. In the three remaining children, patency was confirmed with Doppler ultrasonography 1, 3, and 5 months after implantation. None of the children had new episodes of upper digestive bleeding during follow-up after implantation (mean: 8.1 months).
CONCLUSIONS: Our results indicate that TIPS with 8mm diameter Viatorr(®) covered stents can be safe and efficacious for the treatment of upper digestive bleeding due to gastroesophageal varices in cirrhotic children; our findings need to be corroborated in larger series.