Intrahepatic portal venous systems in adult patients with cavernous transformation of portal vein: Imaging features and a new classification

Background: Cavernous transformation of the portal vein(CTPV) due to portal vein obstruction is a rare vascular anomaly defined as the formation of multiple collateral vessels in the hepatic hilum. This study aimed to investigate the imaging features of intrahepatic portal vein in adult patients with CTPV and establish the relationship between the manifestations of intrahepatic portal vein and the progression of CTPV. Methods: We retrospectively analyzed 14 CTPV patients in Beijing Tsinghua Changgung Hospital. All patients underwent both direct portal venography(DPV) and computed tomography angiography(CTA) to reveal the manifestations of the portal venous system. The vessels measured included the left portal vein(LPV), right portal vein(RPV), main portal vein(MPV) and the portal vein bifurcation(PVB). Results: Nine males and 5 females, with a median age of 40.5 years, were included in the study. No significant difference was found in the diameters of the LPV or RPV measured by DPV and CTA. The visualization in terms of LPV, RPV and PVB measured by DPV was higher than that by CTA. There was a significant association between LPV/RPV and PVB/MPV in term of visibility revealed with DPV( P = 0.01), while this association was not observed with CTA. According to the imaging features of the portal vein measured by DPV, CTPV was classified into three categories to facilitate the diagnosis and treatment. Conclusions: DPV was more accurate than CTA for revealing the course of the intrahepatic portal vein in patients with CTPV. The classification of CTPV, that originated from the imaging features of the portal vein revealed by DPV, may provide a new perspective for the diagnosis and treatment of CTPV.

How can portal vein cavernous transformation cause chronic incomplete biliary obstruction?

Biliary disease in the setting of non-cirrhotic portal vein thrombosis(and similarly in portal vein cavernous transformation) can become a serious problem during the evolution of disease.This is mostly due to portal biliary ductopathy.There are several mechanisms that play a role in the development of portal biliary ductopathy,such as induction of fibrosis in the biliary tract(due to direct action of dilated peribiliary collaterals and/or recurrent cholangitis),loss of biliary motility,chronic cholestasis(due to fibrosis or choledocholithiasis) and increased formation of cholelithiasis(due to various factors).The management of cholelithiasis in cases with portal vein cavernous transformation merits special attention.Because of a heterogeneous clinical presentation and concomitant pathophysiological changes that take place in biliary anatomy,diagnosis and therapy can become very complicated.Due to increased incidence and complications of cholelithiasis,standard treatment modalities like sphincterotomy or balloon sweeping of bile ducts can cause serious problems.Cholangitis,biliary strictures and hemobilia are the most common complications that occur during management of these patients.In this review,we specifically discuss important issues about bile stones related to bile duct obstruction in non-cirrhotic portal vein thrombosis and present evidence in the current literature.

Transjugular intrahepatic portosystemic shunt for the prevention of recurrent esophageal variceal bleeding in patients with cavernous transformation of portal vein

Background: Treatment options for patients with cavernous transformation of portal vein(CTPV) are limited. This study aimed to evaluate the feasibility, efficacy and safety of transjugular intrahepatic portosystemic shunt(TIPS) to prevent recurrent esophageal variceal bleeding in patients with CTPV. Methods: We retrospectively analyzed 67 consecutive patients undergone TIPS from January 2011 to December 2016. All patients were diagnosed with CTPV. The indication for TIPS was a previous episode of variceal bleeding. The data on recurrent bleeding, stent patency, hepatic encephalopathy and survival were retrieved and analyzed. Results: TIPS procedure was successfully performed in 56 out of 67(83.6%) patients with CTPV. TIPS was performed via a transjugular approach alone( n = 15), a combined transjugular/transhepatic approach( n = 33) and a combined transjugular/transsplenic approach( n = 8). Mean portosystemic pressure gradient(PSG) decreased from 28.09 ± 7.28 mmHg to 17.53 ± 6.12 mmHg after TIPS( P < 0.01). The probability of the remaining free recurrent variceal bleeding was 87.0%. The probability of TIPS patency reached 81.5%. Hepatic encephalopathy occurrence was 27.8%, and survival rate was 88.9% until the end of follow-up. Four out of 11 patients who failed TIPS died, and 4 had recurrent bleeding. Conclusions: TIPS should be considered a safe and feasible alternative therapy to prevent recurrent esophageal variceal bleeding in patients with CTPV, and to achieve clinical improvement.

Sixty-four-slice computed tomography in surgical strategy of portal vein cavernous transformation

AIM:To investigate the role of 64-slice computed tomography(CT) in portal vein cavernous transformation to determine surgical strategy.METHODS:The site of lesions and extent of collateral circulation in 12 pediatric cases of cavernous transformation of the portal vein with surgical treatment were analyzed.RESULTS:Eleven of 12 children had esophageal varices and were treated with lower esophageal and gastric devascularization and splenectomy,and the other case was only treated with splenectomy.There were eight cases with spontaneous spleen/stomach-renal shunt,four with Retzius vein opening,which was reserved during surgery.Three cases of lesions involving the intrahepatic portal vein(PV) were treated with living donor liver transplantation.One patient died from PV thrombosis after liver transplantation,and the rest had no significant complications.CONCLUSION:The PV,its branches and collateral circulation were clearly seen by 64-slice spiral CT angiography,which helped with preoperative surgical planning.

Sonographic features of umbilical vein recanalization for a Rex shunt on cavernous transformation of portal vein in children

BACKGROUND The Rex shunt was widely used as the preferred surgical approach for cavernous transformation of the portal vein(CTPV)in children that creates a bypass between the superior mesenteric vein and the intrahepatic left portal vein(LPV).This procedure can relieve portal hypertension and restore physiological hepatopetal flow.However,the modified procedure is technically demanding because it is difficult to make an end-to-end anastomosis of a bypass to a hypoplastic LPV.Many studies reported using a recanalized umbilical vein as a conduit to resolve this problem.However,the feasibility of umbilical vein recanalization for a Rex shunt has not been fully investigated.AIM To investigate the efficacy of a recanalized umbilical vein as a conduit for a Rex shunt on CTPV in children by ultrasonography.METHODS A total of 47 children who were diagnosed with CTPV with prehepatic portal hypertension in the Second Hospital,Cheeloo College of Medicine,Shandong University,were enrolled in this study.Fifteen children received a recanalized umbilical vein as a conduit for a Rex shunt surgery and were enrolled in group I.Thirty-two children received the classic Rex shunt surgery and were enrolled in group II.The sonographic features of the two groups related to intraoperative and postoperative variation in terms of bypass vessel and the LPV were compared.RESULTS The patency rate of group I(60.0%,9/15)was significantly lower than that of group II(87.5%,28/32)7 d after(on the 8th d)operation(P<0.05).After clinical anticoagulation treatment for 3 mo,there was no significant difference in the patency rate between group I(86.7%,13/15)and group II(90.6%,29/32)(P>0.05).Moreover,3 mo after(at the beginning of the 4th mo)surgery,the inner diameter significantly widened and flow velocity notably increased for the bypass vessels and the sagittal part of the LPV compared to intraoperative values in both shunt groups(P<0.05).However,there was no significant difference between the two surgical groups 3 mo after surgery(P>0.05).CONCLUSION For children with hypoplastic LPV in the Rex recessus,using a recanalized umbilical vein as a conduit for a Rex shunt may be an effective procedure for CTPV treatment.

Application of transmesenteric vein extrahepatic portosystemic shunt in treatment of symptomatic portal hypertension with cavernous transformation of portal vein

Purpose:To evaluate the feasibility and efficacy of a transmesenteric vein extrahepatic portosystemic shunt(TmEPS)for the treatment of cavernous transformation of the portal vein(CTPV).Materials and methods:The clinical data of 20 patients with CTPV who underwent TmEPS between December 2020and January 2022 at Henan Provincial People’s Hospital were retrospectively collected.The superior mesenteric vein(SMV)trunk was patent or partially occluded in these patients.An extrahepatic portosystemic shunt between the inferior vena cava and the SMV was established using a stent graft through an infraumbilical median longitudinal mini-laparotomy.The technical success,efficacy,and complication rates were evaluated,and the preand postoperative SMV pressures were compared.Patients’clinical outcomes and shunt patency were assessed.Results:TmEPS was successfully performed in 20 patients.The initial puncture success rate of the balloon-assisted puncture technique is 95%.The mean SMV pressure decreased from 29.1±2.9 mmHg to 15.6±3.3 mmHg(p<0.001).All symptoms of portal hypertension resolved.No fatal procedural complications occurred.During the follow-up period,hepatic encephalopathy occurred in two patients.The remaining patients remained asymptomatic.All shunts were patent.Conclusions:TmEPS is a feasible,safe,and effective treatment option for patients with CTPV.

Portal ductopathy:Clinical importance and nomenclature

Non-cirrhotic portal hypertension(PHT)accounts for about 20%of all PHT cases,portal vein thrombosis(PVT) resulting in cavernous transformation being the most common cause.All known complications of PHT may be encountered in patients with chronic PVT.However,the effect of this entity on the biliary tree and pancreatic duct has not yet been fully established.Additionally,a dispute remains regarding the nomenclature of common bile duct abnormalities which occur as a result of chronic PVT.Although many clinical reports have focused on biliary abnormalities,only a few have evaluated both the biliary and pancreatic ductal systems.In this review the relevant literature evaluating the effect of PVT on both ductal systems is discussed,and findings are considered with reference to results of a prominent center in Turkey,from which the term"portal ductopathy"has been put forth to replace"portal biliopathy".

Successful liver allograft inflow reconstruction with the right gastroepiploic vein

Portal vein thrombosis is a common complica- tion in cirrhotic patients. When portal vein thrombectomy is not a suitable option, a large collateral vessel can be used for allograft venous inflow reconstruction. We describe an unusu- al case of successful portal revascularization using the right gastroepiploic vein. The patient underwent a cadaveric or- thotropic liver transplantation with end-to-end anastomosis of the portal vein to the right gastroepiploic vein. Six months after liver transplantation the patient is well with good liver function. The use of the right gastroepiploic vein for allograft venous reconstruction is feasible and safe, with a great advan- tage of avoiding the need of venous jump graft.

Portal biliopathy,magnetic resonance imaging and magnetic resonance cholangiopancreatography findings:a case series

Portal biliopathy(PB)is a rare disorder,characterized by biliary ductal and gallbladder wall abnormalities seen in patients with portal hypertension.It most commonly occurs due to idiopathic extrahepatic portal vein obstruction(EHPVO).The abnormalities consist mainly of bile duct compression,stenoses,fibrotic strictures and dilation of both extrahepatic and intrahepatic bile ducts,as well as gallbladder varices.PB may mimic cholangiocarcinoma,sclerosing cholangitis,or choledocholithiasis.Misdiagnosis can be avoided using appropriate imaging modalities to prevent complications.We present the magnetic resonance imaging(MRI)and magnetic resonance cholangiography(MRCP)features of three patients with PB.