We present a case of isolated intrahepatic portosystemic shunts with an aorto- hepatic-umbilical connection; prenatally diagnosed with 3D Doppler flow in a 27-year-old patient at 27 weeks of gestation. Fetal karyotyping was normal, detailed evaluation of the venous and cardiovascular system showed a patent ductus venosus and no other abnormalities. These fetuses with isolated intrahepatic shunts are at risk for intra-uterine growth restriction (27%), all cases had a good prognosis with live births. Postnatal follow-up to control shunt closure is vital as persistent shunts may lead to subsequent complications.

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.

A transjugular intrahepatic portosystemic shunt is the standard of care for complications of portal hypertension, such as variceal bleeding, refractory ascites, and hepatic hydrothorax. Hepatic encephalopathy, hepatic insufficiency, and right heart failure are the major complications after shunt creation. If medical management is unsuccessful, the interventionalist is consulted to close/revise the shunt. Closure of the shunt results in a dangerously abrupt increase in portal pressure, increasing risk for life-threatening variceal bleeding. Methods for revising these shunts are reported, which result in coarse adjustments in shunt diameter, causing rapid changes in portosystemic gradients. Our method for shunt revision utilizes carefully sized covered stents deployed in a controlled \"onion skin\" fashion to produce a narrowing within the hepatic venous limb of the shunt to precisely calibrate the desired portosystemic gradient.

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.