Congenital intrahepatic portosystemic shunts: a potential cause for early-onset neonatal cholestasis

2018 ◽  
Vol 7 (1) ◽  
Author(s):  
Radhika Narang ◽  
Minal Patel ◽  
Neelesh Ajit Tipnis ◽  
Sajani Matai Tipnis
Keyword(s):  
Hepatic Encephalopathy ◽  
Early Onset ◽  
Biliary Obstruction ◽  
Metabolic Disorders ◽  
Alternative Explanation ◽  
Liver Tumors ◽  
Portosystemic Shunt ◽  
Neonatal Cholestasis ◽  
Portosystemic Shunts ◽  
Intrahepatic Portosystemic Shunt

Abstract Cholestasis in the first days of life is uncommon in neonates. Neonatal cholestasis is usually associated with shock, sepsis, alloimmunity, metabolic disorders or biliary obstruction. A congenital intrahepatic portosystemic shunt results from failed involution of primordial liver vessels during the first days of life. Resulting shunts can lead to hepatic encephalopathy or liver tumors. A congenital intrahepatic portosystemic shunt should be considered when an alternative explanation cannot be found. In most cases, congenital intrahepatic portosystemic shunts will involute spontaneously by 1–2 years of age; however, surgical or radiologic closure may be needed.

scholarly journals Spontaneous portosystemic shunts in liver cirrhosis: new approaches to an old problem

2020 ◽  
Vol 13 ◽  
pp. 175628482096128 ◽  
Author(s):  
Judit Vidal-González ◽  
Sergi Quiroga ◽  
Macarena Simón-Talero ◽  
Joan Genescà
Keyword(s):  
Liver Cirrhosis ◽  
Hepatic Encephalopathy ◽  
Portosystemic Shunt ◽  
Venous System ◽  
Variceal Hemorrhage ◽  
Portal Venous System ◽  
Portal Flow ◽  
Portosystemic Shunts ◽  
Intrahepatic Portosystemic Shunt

Portal hypertension is the main consequence of liver cirrhosis, leading to severe complications such as variceal hemorrhage, ascites or hepatic encephalopathy. As an attempt to decompress the portal venous system, portal flow is derived into the systemic venous system through spontaneous portosystemic shunts (SPSSs), bypassing the liver. In this review, we aim to provide an overview of the published reports in relation to the prevalence and physiopathology behind the appearance of SPSS in liver cirrhosis, as well as the complications derived from its formation and its management. The role of SPSS embolization is specifically discussed, as SPSSs have been assessed as a therapeutic target, mainly for patients with recurrent/persistent hepatic encephalopathy and preserved liver function. Furthermore, different aspects of the role of SPSS in liver transplantation, as well as in candidates for transjugular intrahepatic portosystemic shunt are reviewed. In these settings, SPSS occlusion has been proposed to minimize possible deleterious effects, but results are so far inconclusive.

scholarly journals Endovascular Management of Hepatic Encephalopathy

2021 ◽  
Vol 5 (02) ◽  
pp. 106-115
Author(s):  
Hieu Le ◽  
Siddhant Thukral ◽  
A. Michael Devane ◽  
Souheil Saddekni ◽  
Rakesh K. Varma
Keyword(s):  
Liver Disease ◽  
Hepatic Encephalopathy ◽  
Vascular Anatomy ◽  
Portosystemic Shunt ◽  
Endovascular Management ◽  
Endovascular Techniques ◽  
Factors Associated ◽  
Endovascular Interventions ◽  
Portosystemic Shunts ◽  
Intrahepatic Portosystemic Shunt

AbstractTransjugular intrahepatic portosystemic shunt (TIPS) and spontaneous portosystemic shunts (SPSS) may lead to new or worsening hepatic encephalopathy (HE), especially in patients with chronic liver disease. Patients with medically refractory HE (rHE) may benefit from endovascular interventions. In this review, we briefly describe the post-TIPS and SPSS vascular anatomy, pathophysiology, classification, factors associated with HE, and the medical management of HE. In addition, we will discuss current endovascular techniques for HE management, their advantages, disadvantages, and review of the current literature.

Risk Factors for Hepatic Encephalopathy after Transjugular Intrahepatic Portosystemic Shunt in Patients with Hepatocellular Carcinoma and Portal Hypertension

2015 ◽  
Vol 24 (3) ◽  
pp. 301-307 ◽  
Author(s):  
Jiannan Yao ◽  
Li Zuo ◽  
Guangyu An ◽  
Zhendong Yue ◽  
Hongwei Zhao ◽  
...  
Keyword(s):  
Risk Factors ◽  
Hepatocellular Carcinoma ◽  
Portal Hypertension ◽  
Hepatic Encephalopathy ◽  
Pressure Gradient ◽  
Transjugular Intrahepatic Portosystemic Shunt ◽  
Meld Score ◽  
Portosystemic Shunt ◽  
Portal Flow ◽  
Intrahepatic Portosystemic Shunt

Aims: This study aimed at assessing the risk factors for hepatic encephalopathy (HE) after transjugular intrahepatic portosystemic shunt (TIPS) in patients with hepatocellular carcinoma (HCC) and portal hypertension. Method: Consecutive patients (n=279) with primary HCC who underwent TIPS between January 1997 and March 2012 at a single institution were retrospectively reviewed. Patients were followed up for 2 years. Pre-TIPS, peri-TIPS and post-TIPS clinical variables were reviewed using univariate and multivariate analyses to identify risk factors for HE after TIPS. Results: The overall incidence of HE was 41% (114/279). Multivariate analysis showed an increased odds for HE in patients with: >3 treatments with transcatheter arterial chemoembolization (TACE) and/or trans-arterial embolization (TAE) (odds ratio [OR], 4.078; 95% confidence interval [95%CI], 1.748-9.515); hepatopetal portal flow (OR, 2.362; 95%CI, 1.032-5.404); high portosystemic pressure gradient (OR, 1.198; 95%CI, 1.073-1.336) and high pre-TIPS MELD score (OR, 1.693; 95%CI, 1.390-2.062). Odds for HE were increased 1.693 fold for each 1-point increase in the MELD score, and 1.198 fold for each 1-mmHg decrease in the post-TIPS portosystemic pressure gradient. Conclusion: The identification of clinical variables associated with increased odds of HE may be useful for the selection of appropriate candidates for TIPS. Results suggest that an inappropriate decrease in the portosystemic pressure gradient might be associated with HE after TIPS. In addition, >3 treatments with TACE/TAE, hepatopetal portal flow, and high MELD score were also associated with increased odds of HE after TIPS. Key words:  –  –  – .

scholarly journals Transsplenic portal vein recanalization and direct intrahepatic portosystemic shunt placement to optimize liver transplantation

2020 ◽  
Vol 3 (1) ◽  
Author(s):  
Osman Ahmed ◽  
Abhijit L. Salaskar ◽  
Steven Zangan ◽  
Anjana Pillai ◽  
Talia Baker
Keyword(s):  
Liver Transplantation ◽  
Portal Vein ◽  
Splenic Vein ◽  
Portosystemic Shunt ◽  
Refractory Ascites ◽  
Hepatic Veins ◽  
Sonographic Guidance ◽  
Portosystemic Shunts ◽  
Intrahepatic Portosystemic Shunt ◽  
End To End

Abstract Background Percutaneous trans-splenic portal vein recanalization (PVR) has been reported for facilitation of transjugular intrahepatic portosystemic shunts (TIPS), however has not been applied to patients undergoing direct intrahepatic portosystemic shunt (DIPS). We report the utilization of trans-splenic-PVR with DIPS creation in a patient with chronic portal and hepatic vein occlusions undergoing liver transplantation evaluation. Case presentation A 48-year-old male with decompensated alcoholic cirrhosis complicated by refractory ascites, hepatic encephalopathy, and variceal bleeding underwent CT that demonstrated chronic occlusion of the hepatic veins (HV), extrahepatic portal vein (PV), and superior mesenteric vein (SMV). Due to failed attempts at TIPS at outside institutions, interventional radiology was consulted for portal vein recanalization (PVR) with TIPS to treat the portal hypertension and ascites and also facilitate an end-to-end PV anastomosis at transplantation. After an initial hepatic venogram confirmed chronic HV occlusion, a DIPS with trans-splenic PVR was planned. The splenic vein was accessed under sonographic guidance using a micropuncture set and subsequently upsized to a 6 French sheath over a stiff guidewire. A splenic venogram via this access confirmed occlusion of the PV with drainage of the splenic vein (SV) through gastric varices. The thrombosed PV was then recanalized and angioplastied to restore PV flow via the transsplenic approach. A transjugular liver access kit with a modified 21-gauge needle was advanced into the IVC through the internal jugular vein (IJV) sheath and directed towards the target snare in PV. The needle was used to subsequently puncture the PV through the caudate lobe and facilitate placement of a wire into the SV. The initial portosystemic gradient (PSG) was 20 mmHg. The IJV sheath was advanced through the hepatic parenchymal tract into the main-PV and a stent-graft was placed across the main PV and into the IVC. A portal venogram demonstrated brisk blood flow through the DIPS, resolution of varices and a PSG of 8 mmHg. One month after the procedure, the patient had a significant reduction in ascites and MELD-NA score. Patient is currently listed and awaiting transplantation. Conclusions In the setting of chronically occluded portal and hepatic veins, trans-splenic PVR DIPS may serve as an effective bridge to liver transplantation by facilitating an end to end portal vein anastomosis.

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