The relationship between umbilical–portal–systemic venous shunt and outcome in 11 fetuses

Objective: To investigate the relationship between congenital umbilical–portal–systemic venous shunt (UPSVs) and fetal outcome. Methods: The ultrasonographic and genetic characteristics of 11 cases of UPSVS were retrospectively analyzed and followed up to postnatal. Results: Four cases of ductus venosus -- systemic shunt (DVSS), one case of extrahepatic portal -- systemic shunt (EHPSS), and one case of umbilical systemic shunt (USS) combined with intrahepatic portal-systemic shunt (IHPSS), six cases of intrahepatic portal-systemic shunt (IHPSS) were observed. chromosomal abnormalities were observed in 9.1% (1/11), other ultrasonic abnormalities in 54.5% (6/11), cardiothoracic ratio increase in 45.5% (5/11), fetal growth restriction in 36.4% (4/11), edema was in 9.1% (1/11) and live birth was in 72.7% (8/11). Conclusion: The incidence of IHPSS is the highest and the outcome is the best. Shunt of DVSS and IHPSS can be closed spontaneously after birth. When the prenatal diagnosis is congenital UPSVs, chromosomal abnormalities and other ultrasonic abnormalities should be required further examination, and the growth and development of the fetus should be closely monitored.

Ultrasonographic characteristics and outcome of Type III umbilical-portal-systemic venous shunt

Aims: According to a novel in-utero classification termed “umbilical-portal-systemic venous shunt (UPSVS)” recently proposed for an abnormal umbilical, portal and ductal venous system, the portal-systemic shunt belongs to type III UPSVS. This study was designed to examine the ultrasonographic characteristics and outcome of type III UPSVS.Material and methods: All cases of Type III UPSVS diagnosed at our department from April 2016 to December 2020 were retrospectively studied.Results: Seventeen patients with type III UPSVS including 12 type IIIa and 5 IIIb cases were identified. Sonography showed a shunt between the inferior left portal vein and the left hepatic vein in all type IIIa cases. Three cases of type IIIb had a combination of another shunt (2 with type I and one with type IIIa). Integrate intrahepatic portal vein system was not seen in those 2 cases of type IIIb combined with type I UPSVS, leading to termination of pregnancy (TOP). TOP occurred in 4 patients with type IIIa as requested by the parents. Two cases (type IIIa and type IIIb each) underwent surgical procedure for the closure of the shunt. Spontaneous complete closure in 4 type IIIa cases and partial closure in one type IIIb case occurred during a period of 3-16 months.Conclusions: The majority of patients had type IIIa UPSVS presenting a good outcome. The lack of integrate intrahepatic portal vein system was the main reason for TOP in patients with type IIIb UPSVS. These data suggest the UPSVS classification is a useful tool for a prognosis prediction of type III UPSVS.

Lights and Shadows on Hepatic Encephalopathy Diagnosis

Hepatic encephalopathy (HE) is a form of brain dysfunction that is specifically caused by liver insufficiency and/or portal-systemic shunt. The exact nature of HE is debated, so that conflicting uses of the term HE may cause inconsistencies in its detection and, in turn, issues with its management. This review highlights the meaning of the term HE on the basis of both its historical origins and current consensus. It also provides criteria for the diagnosis of the condition, on the basis of its phenotypes and the risk factors for its occurrence. The procedure for differential diagnosis from other conditions which result in similar phenotypes is considered, together with precipitants and confounders. Finally, the current multidimensional approach for the correct clinical recording of HE episodes is discussed.

Method for Calculating the Bending Angle of Puncture Needle in Preoperative Planning for Transjugular Intrahepatic Portal Systemic Shunt (TIPS)

Transjugular Intrahepatic Portal Systemic Shunt is a comprehensive interventional therapy for portal hypertension. During this intervention, puncturing from hepatic vein into portal vein is a difficult step. Selecting puncture needle with a proper bending angle is vital to accurate puncture. Thus, this prospective study provides a method to calculate the angle of the puncture needle using preinterventional contrast-enhanced CT imaging. According to the geometrical characteristics of puncture needle, Bezier curve equation was adopted to describe its bending part. By testing whether each point in a specific region satisfied the equation set of Bezier curves, the possible position of needle tip was obtained. Then, the bending angle of puncture needle was obtained by calculating curvature. The method was evaluated in 13 patients from 2 centers showing now a success rate of 100% and a duration of the procedure of 141 and 161 minutes. The method based on Bezier curve equation for calculating a proper bending angle of puncture needle was proven to be effective. And the clinical study is preliminary and additional work for clinical evaluation is necessary.

Refractory Hepatic Hydrothorax: A Rare Complication of Systemic Sclerosis and Presinusoidal Portal Hypertension

We report on a rare case of refractory hepatic hydrothorax in an individual with Scleroderma/CREST syndrome and noncirrhotic portal hypertension. Portal pressure measurements revealed a normal transjugular hepatic venous portal pressure gradient, mild pulmonary hypertension, and an unremarkable liver biopsy except for mild sinusoidal dilation. Pulmonary hypertension, cardiac diastolic dysfunction, and chronic kidney disease were determined to be the causes of his refractory pleural effusions and ascites. Over the year, he underwent 50 thoracenteses and 20 paracenteses averaging 10–12 liters/week. Repeat pulmonary evaluation determined his pulmonary pressures to be normal and a secondary review of the “unremarkable” liver biopsy noted mild venous outflow obstruction and possibly Nodular Regenerative Hyperplasia (NRH). Repeat portal pressures indirectly and directly confirmed the existence of presinusoidal portal hypertension that has been associated with NRH. A transjugular intrahepatic portal systemic shunt (TIPS) was placed and he has not required thoracentesis or paracentesis over the past 18 months.