Cavernous Transformation Research Articles

Biliary Obstruction following Transjugular Intrahepatic Portosystemic Shunt Creation in Patients with Variceal Bleeding

This report describes an unusual complication after creation of a transjugular intrahepatic portosystemic shunt (TIPS). Biliary obstruction developed in two patients with portal hypertension accompanied by portal vein thrombosis, one patient with and the other without portal cavernous transformation. The biliary obstruction was thought to be secondary to compression of the bile duct by the stent graft placed in the TIPS. Awareness of this possible complication is important for its early diagnosis.

Abstract No. 638 - Creation of percutaneous mesocaval shunt as well as other techniques for management of GI bleed in patient with cavernous transformation of the portal vein

Abstract No. 638 - Creation of percutaneous mesocaval shunt as well as other techniques for management of GI bleed in patient with cavernous transformation of the portal vein

Rex shunt in pediatric prehepatic portal hypertension: a clinical analysis of 5 cases

Objective To study the therapeutic efficacy of Rex shunt in treating pediatric patients with prehepatic portal hypertension (PHPH). Methods Five children with PHPH who were admitted from October 2014 to May 2015 were reviewed. There were three boys and two girls, with age ranging from 50 to 95.5 months [(75.8±1.9) months]. They all suffered from recurrent upper gastrointestinal (GI) blee-ding. Their red blood cell (RBC), white blood cell (WBC) and platelet (PLT) counts were decreased, but laboratory findings revealed no liver dysfunction. Ultrasound and CT scan diagnosed cavernous transformation of portal vein (CTPV) and splenomegaly. The mean splenic length was (42.8±8.2) cm. Indirect portal venography revealed patent left portal vein. All patients underwent Rex shunt and were followed up for 3~7 months. Results The mean duration of operation was (566.0±39.7) min. Intraoperative bleeding varied from 10 to 50 ml. The portal pressure significantly decreased after surgery from [(25.6±1.5) mmHg, 1 mmHg=0.133 kPa] preoperatively to (19.2±3.3) mmHg postoperatively (P<0.05). Portal venography indicated patent left portal vein after the Rex shunt. The postoperative course was uneventful in the five patients with a mean hospital stay of (26±9.3) days. There was no further GI bleeding. The RBC, WBC and PLT counts increased. Ultrasound indicated patent anastomotic stomas and decreased splenic size. Conclusion A Rex shunt in treating patients with PHPH is safe, feasible and efficacious. Key words: Rex shunt; Prehepatic portal hypertension; Cavernous transformation of portal vein; Children; Superior mesenteric vein; Left branch of portal vein

Cavernous transformation of the portal vein

A male patient with an unknown medical history sustained injuries that were deemed incompatible with life. He became an organ donor. At the time of donor liver harvest, periportal collaterals were seen around the liver (A), with webs visualized inside the portal vein (B) consistent with cavernous transformation of the portal vein (CTPV). This made the liver unsuitable for transplantation, and the procedure was aborted.

New surgical approach for advanced hepatoceluler carcinoma with cavernous transformation of portal vein

New surgical approach for advanced hepatoceluler carcinoma with cavernous transformation of portal vein

Imaging and radiological interventions in extra-hepatic portal vein obstruction.

Extrahepatic portal vein obstruction (EHPVO) is a primary vascular condition characterized by chronic long standing blockage and cavernous transformation of portal vein with or without additional involvement of intrahepatic branches, splenic or superior mesenteric vein. Patients generally present in childhood with multiple episodes of variceal bleed and EHPVO is the predominant cause of paediatric portal hypertension (PHT) in developing countries. It is a pre-hepatic type of PHT in which liver functions and morphology are preserved till late. Characteristic imaging findings include multiple parabiliary venous collaterals which form to bypass the obstructed portal vein with resultant changes in biliary tree termed portal biliopathy or portal cavernoma cholangiopathy. Ultrasound with Doppler, computed tomography, magnetic resonance cholangiography and magnetic resonance portovenography are non-invasive techniques which can provide a comprehensive analysis of degree and extent of EHPVO, collaterals and bile duct abnormalities. These can also be used to assess in surgical planning as well screening for shunt patency in post-operative patients. The multitude of changes and complications seen in EHPVO can be addressed by various radiological interventional procedures. The myriad of symptoms arising secondary to vascular, biliary, visceral and neurocognitive changes in EHPVO can be managed by various radiological interventions like transjugular intra-hepatic portosystemic shunt, percutaneous transhepatic biliary drainage, partial splenic embolization, balloon occluded retrograde obliteration of portosystemic shunt (PSS) and revision of PSS.

Is Post-TIPS Anticoagulation Therapy Necessary in Patients with Cirrhosis and Portal Vein Thrombosis? A Randomized Controlled Trial.

Purpose To determine whether posttransjugular intrahepatic portosystemic shunt (TIPS) placement anticoagulation therapy could benefit patients with cirrhosis and portal vein thrombosis (PVT) from the perspective of a change in portal vein patency status and clinical outcomes. Materials and Methods The study was approved by the institutional review board, and informed consent was obtained from each patient. From October 2012 to February 2014, patients with cirrhosis and PVT who underwent TIPS placement were randomly assigned to the anticoagulation therapy or control group. All patients were followed at 1, 3, 6, and 12 months after the TIPS procedure. Outcome measures were a change of portal vein patency status and clinical measures including gastrointestinal rebleeding, shunt dysfunction, hepatic encephalopathy, and survival. Student t test, χ(2) test, Fisher exact test, Mann-Whitney U test, and logistical regression were applied where appropriate. Results A total of 64 patients were enrolled in the study, with 31 allocated to the anticoagulation group and 33 allocated to the control group. Overall, thrombi were improved in 61 patients (96.8%) after the procedure. PVT recanalization (ie, complete disappearance; reconstruction of cavernous transformation) was achieved in 26 patients (83.9%) in the anticoagulation therapy group and in 23 (71.8%) patients in tthe control group (P = .252). The presence of a superior mesenteric vein thrombus may help predict recanalization failure (unadjusted relative risk = 0.243; 95% confidence interval: 0.070, 0.843; P = .026). Clinical outcomes were also similar between the two groups. Conclusion Anticoagulation therapy may not be necessary in certain patients with PVT because TIPS placement alone can achieve a high persistent recanalization rate. (©) RSNA, 2015.

Rare disorders can be an underlying cause of cyclic vomiting: Familial Mediterranean fever, Helicobacter pylori gastritis, and cavernous transformation of the portal vein.

Considering the etiology of cyclic vomiting syndrome (CVS) in childhood, a variety of underlying organic causes has been clearly identified in the literature. The aim of this study was to emphasize that endoscopic evaluation in the first step may help diagnosis and treatment in patients with CVS, unlike the CVS-related "North American Society for Pediatric Gastroenterology, Hepatology and Nutrition" (NASPGHAN) consensus statement in 2008. The medical files of patients with vomiting complaints admitted to our tertiary center between the years 2007 and 2012 were analyzed retrospectively. Patients were identified according to the International Classification of Diseases (ICD) codes at their initial presentation, including vomiting. A total of 815 patients with vomiting complaints were evaluated. Of the 379 patients who presented with vomiting only, 336 patients were already being followed for chronic vomiting. Cyclic vomiting was detected in 31 out of 336 patients. In our series, familial Mediterranean fever (FMF), cavernous transformation of the portal vein, and Helicobacter pylori (HP) gastritis presented with CVS for the first time in the pediatric age group. We emphasize that endoscopic evaluation in patients with CVS should be performed as the first step for appropriate diagnosis and treatment.

Endovascular Reconstruction of a Chronic Non-cirrhotic Extra-hepatic Portal Vein Thrombosis by “Neo-Porta” System: A Case Report

Introduction: Chronic portal thrombosis can present itself with symptoms that fail to respond to conventional treatments, in this scenario management with transjugular intrahepatic portosystemic shunt (TIPS) might be an option. Nevertheless in presence of portal cavernoma this procedure is generally thought to be contraindicated due to technical difficulties. We describe the case of a symptomatic patient with noncirrhotic chronic portal thrombosis in which with porto-mesenteric stent and TIPS placement portal axis reconstruction was achieved. Case Report: 57 year-old white man was refer to our center with chronic portal thrombosis secondary to abdominal surgery. He received medical treatment however he kept complaining of post-prandial severe abdominal pain. Abdominal tenderness was remarkable and all laboratory data was normal. CT scan showed permeability of right intra-hepatic portal vein (IHPV) and its segmental branches, cavernous transformation of the extra-hepatic portal vein (EHPV) and the ending of the native EHPV forming a fibrous cord until its communication with the superior mesenteric vein. Considering these findings we decided to perform an endovascular approach in order to re-establish portal flow. A percutaneous micropuncture aiming for the right IHPV was done followed by native EHVP canalization all the way to the superior mesenteric vein restoring in this manner portal permeability (Figure 1, A-E). Once the porto-mesenteric stent was placed, flow redistribution from the cavernoma was achieved (Figure 2) therefore portal pressure was reduce and patient symptomatology was resolved. Three months later the patient developed ascites due to hyperdynamic flow on the right IHPV. A TIPS was placed using the “neo porta” system with successful resolution of portal hypertension, patient improvement and discharge (Figure 1, G, H).Figure 1Figure 2Discussion: In this case, as in many others, cavernous transformation reduces treatment options; based on the patient clinical and radiological findings we propose this endovascular approach hypothesizing that reconstruction of the portal axis with porto-mesenteric stent and TIPS could be successful. In selected patients with chronic thrombosis of the portal axis this approach can be considered as therapeutical option. Conclusion: Endovascular treatment in this particular scenario should not be considered contraindicated with adequate patient selection this approach can be successful with improvement of patient's life quality.

Advanced Combined Hepatocellular Carcinoma and Cholangiocarcinoma (cHCC-CC) in a Patient With Alcoholism and Chronic Hepatitis C

Introduction: A combined hepatocellular and cholangiocarcinoma (cHCC-CC) is rare subtype of primary liver cancer accounting for 0.87% of primary liver tumors. It is a distinct type of primary liver cancer with aggressive behavior, which is morphologically and phenotypically intermediate between HCC and CC. Although few studies have shown the increased occurrence of cHCC-CC in chronic hepatitis B patients, there is a lack of association with chronic hepatitis reported in US studies. We present a patient with advanced cHCC-CC and history of alcoholism and chronic hepatitis C(CHC). Case: A 64-year-old man with history of alcohol abuse and CHC, treatment naive, was referred to hepatology for jaundice for 2 weeks (blood work in table 1). Ultrasound of the abdomen showed a 6.6cm mass in left hepatic lobe extending to the hilum causing intrahepatic ductal dilatation(IHD), a 5.5cm mass in right hepatic lobe and scattered small liver lesions. MRI of the abdomen with Eovist revealed an 8.6cm mass in segment 4 extending to the hilum resulting in right(RT) and left (LT) IHD dilation and a 6cm mass in posterior right hepatic lobe. There were also smaller satellite nodules in both lobes, occlusion of the main portal vein with extensive cavernous transformation, and thrombus within the RT and LT intrahepatic portal veins. Liver was non-nodular while enhancement suggested CC. Liver biopsy showed CK7 positive, poorly differentiated CC with rare glandular architecture(Image 1). It also showed poorly differentiated HCC with giant cell features consistent with cHCC-CC (Image 2,3). Jaundice worsened and total bilirubin(TB) trended up to 30. A biliary drain was placed and TB decreased to 18. After the drain was capped 5 days later, he developed acute renal failure, leukocytosis and encephalopathy. Antibiotics, rifaximin, and albumin were given and his renal function and mental status improved. Palliative care was consulted for advanced cHCC-CC and the patient was discharged to home hospice. Conclusion: The diagnosis and treatment of cHCC-CC can be challenging due to clinical, imaging and histological features that overlap with pure HCC and CC. Our patient, although did not have ascites, had advanced stage disease with satellite lesions, multiple tumors, biliary, vascular and regional lymph node involvement. Further studies are needed to evaluate the risk of CHC in development of cHCC-CC. This may ultimately help us to screen for early disease and to improve prognosis.Table 1: LabsFigure 1Figure 2Figure 3

Hepatobiliary Quiz (Answers)—15 (2015)

Hepatobiliary Quiz (Answers)—15 (2015)

Spontaneous Regression of HCC: A Case Report

The spontaneous regression of HCC is extremely rare. This event could be explained by a strong and persistent activation of the immune system directed against the neoplastic cells or a sudden change in vascular inflow resulting in autoinfarction. We report herein a case of 51-year-old man who presented with a diagnosis of HCV induced cirrhosis. The initial liver ultrasound revealed an ill defined right lobe lesion approximately 7 x 8 cm in diameter, involving segments VII and VIII with malignant portal vein thrombosis. Baseline laboratory tests included: FBS 72 mg/dl, 2-hour postprandial sugar was 166 mg/dl, Hb 12.1 g/dl, WBC 3 000/cmm, neutrophils 1500/cmm, platelet count 70 000/cmm, ALT 85 U/L, AST 100 U/L, total serum bilirubin 1.8 mg/dl, serum albumin 3.3 g/dl, prothrombin time 13.1 seconds (70%) with calculated Child A compensated cirrhosis according to Child Pugh scoring. The initial AFP was 19,309 ng/ml. The triphasic CT (Figure 1) revealed a large invasive right lobe mass in segment IV,V,VI,VIII with an arterial blush and rapid washout pattern diagnostic for HCC, with associated with thrombosis of the right branch of portal vein with a porta hepatis lymphe node consistent with metastatic HCC. The patient had an ECOG performance status of 0, and according to the BCLC staging for HCC, Stage C (advanced stage) disease. Sorafenib was not available and the patient did not receive any other treatment. A triphasic CT, performed 4 months later, demonstrated (Figure 2) a marked decrease in the size of the lesion, with 4 x 3.8 cm in maximum dimensions. The thrombosed right portal vein branch showed cavernous transformation and no malignant neovascularization. The AFP was 1,923 ng/ml, laboratory tests: FBS 206 mg/dl, urea 22 mg/dl, creatinine 0.7 mg/dl, ALT 63 U/L, AST 77 U/L, total serum Bilirubin was 1.7 mg/dl, serum albumin 3.3g/dl. The patient was at a good general condition and remained at an ECOG performance status of 0. In conclusion, we report here a rare event in a patient with HCC, wherein the HCC underwent a spontaneous event that resulted in a marked decrease in the tumor size and area of neovascularization. This rare spontaneous event, which can change a patient's prognosis substantially, needs to be recognized by clinicians who monitor patients with HCC, both on and off treatment as well as in clinical trials.Figure 2View Large Image Figure ViewerDownload Hi-res image Download (PPT)

Multimodality imaging of primary extrahepatic portal vein obstruction (EHPVO): what every radiologist should know.

Portal vein thrombosis (PVT) is a frequent complication of liver cirrhosis, but it can also occur as a primary vascular disorder amid absent liver disease. Extrahepatic portal vein obstruction (EHPVO) refers to the obstruction of the extrahepatic portal vein with or without involvement of the intrahepatic portal vein branches, splenic and/or superior mesenteric vein. It is a distinct disorder that excludes PVT occurring in concurrence with liver cirrhosis or hepatocellular carcinoma. The term "EHPVO" implies chronicity and is principally reserved for a long-standing condition characterized by cavernous transformation of the portal vein. The most characteristic imaging manifestation is the formation of portoportal collaterals (via the venous plexi of Petren and Saint) that allow hepatopetal flow. However, this collateral circulation is insufficient resulting in clinically significant pre-hepatic portal hypertension, wherein the liver function and structure remain preserved until late. Although the long-term (more than 10 years) survival with controlled variceal bleeding is up to 100%, affected individuals have an impaired quality of life owing to portal cavernoma cholangiopathy, hypersplenism, neurocognitive dysfunction and growth retardation. Imaging diagnosis is not always straightforward as the collaterals can also present as a tumour-like solid mass that can be inadvertently biopsied. Moreover, EHPVO has its implications for the biliary tree, arterial circulation, liver/splenic volumes and stiffness, which merit proper understanding but have not been so well described in literature. In this review, we present the complete spectrum of the vascular, biliary and visceral changes with a particular emphasis on what our medical/surgical hepatology colleagues need to know from us in the pre-operative and post-operative settings.

Biliary collateral veins and associated biliary abnormalities of portal hypertensive biliopathy in patients with cavernous transformation of portal vein

PurposeThe purpose was to investigate magnetic resonance imaging (MRI) features of biliary collateral veins and associated biliary abnormalities of portal hypertensive biliopathy (PHB). Materials and methodsThirty-six patients including 18 patients with abnormal biliary changes and 18 patients as control group were involved in this study. MRI features of biliary collateral veins were analyzed. ResultsStenosis with dilated proximal bile ducts occurred in 33.3% of patients, 27.8% of patients had irregular ductal walls, 22.2% of patients had thickened ductal walls, 16.7% of patients had angulated ductal walls, and 44.4% of patients had thickened gallbladder walls. ConclusionsBiliary collateral veins and associated biliary abnormalities of PHB can be detected by MRI.

COMPUTED TOMOGRAPHIC AND ULTRASONOGRAPHIC CHARACTERISTICS OF CAVERNOUS TRANSFORMATION OF THE OBSTRUCTED PORTAL VEIN IN SMALL ANIMALS.

In humans, the process of development of collateral vessels with hepatopetal flow around the portal vein in order to bypass an obstruction is called "cavernous transformation of the portal vein." The purpose of this retrospective, cross-sectional, multicentric study was to describe presumed cavernous transformation of the portal vein in small animals with portal vein obstruction using ultrasound and multidetector-row computed tomography (MDCT). Databases from three different institutions were searched for patients with an imaging diagnosis of cavernous transformation of the portal vein secondary to portal vein obstruction of any cause. Images were retrieved and reanalyzed. With MDCT-angiography, two main portoportal collateral pathways were identified: short tortuous portoportal veins around/inside the thrombus and long portoportal collaterals bypassing the site of portal obstruction. Three subtypes of the long collaterals, often coexisting, were identified. Branches of the hepatic artery where involved in collateral circulation in nine cases. Concomitant acquired portosystemic shunts were identified in six patients. With ultrasound, cavernous transformation of the portal vein was suspected in three dogs and one cat based on visualization of multiple and tortuous vascular structures corresponding to periportal collaterals. In conclusion, the current study provided descriptive MDCT and ultrasonographic characteristics of presumed cavernous transformation of the portal vein in a sample of small animals. Cavernous transformation of the portal vein could occur as a single condition or could be concurrent with acquired portosystemic shunts.

English

Portal biliopathy (PB) is defined as biliary obstruction associated with enlarged collaterals in the setting of chronic extrahepatic portal vein occlusion (portal vein thrombosis EHPVO) or cavernous transformation of the portal vein. Although patients with portal biliopathy normally have asymptomatic biliary dilatation or an increase in liver enzyme levels, they may rarely experience jaundice, cholangitis or choledocholithiasis. The present case is of 17 year old male presenting with obstructive jaundice, malena, cholelithiasis secondary to portal venous thrombosis and cavernous transformation of portal vein due to CBD stricture secondary to Portal Hypertensive Biliopathy (PHB).

Cavernous transformation of the portal vein in children: about 11 cases

Le cavernome portal est la conséquence d'une occlusion chronique, du système porte extra-hépatique formé d'un réseau de veines dont le calibre est augmenté et au sein desquelles chemine un sang portal hépatopéte. Chez l'enfant, est une cause majeure d'hypertension portale dite «pré ou infra-hépatique» ou encore «extra-hépatique». Onze cas de cavernome porte parmi 78 cas d'hypertension portale ont été colligés au service sur une période allant du Janvier 2003 au Septembre 2012. L’âge de nos patients variait entre 2 et 15 ans et le sexe ratio est de 1,75. Tous nos patients étaient admis au stade d'hypertension portale avec la splénomégalie SMG (100% des cas); hémorragies digestives (63%); ascite (36%); la circulation veineuse collatérale CVC et l'hépatomégalie HMG (27%). L'exploration endoscopique a objectivé la présence de varices ‘sophagiennes dans tous les cas avec une gastropathie hypertensive dans 27% et des varices ectopiques chez 36%. Les perturbations biologiques étaient dominées par la pancytopénie. Le bilan de thrombophilie était demandé pour tous les malades mais réalisé mais n'est réalisé que chez trois revenus normaux chez deux et a objectivé une baisse de protéine S chez le troisième. L’échographie abdominale était le moyen de diagnostic positif et l’écho-doppler a confirmé l'HTP chez tous nos patients. Aucun de nos malades n'a pu être opéré pour le moment. La ligature a été réalisée chez 54,5%. Dix patients ont nécessité une transfusion sanguine. L’évolution globale de nos patients est favorable. La durée moyenne d’évolution du cavernome porte chez nos patients est de quatre ans.

Rational classification of portal vein thrombosis and its clinical significance.

Portal vein thrombosis (PVT) is commonly classified into acute (symptom duration <60 days and absence of portal carvernoma and portal hypertension) and chronic types. However, the rationality of this classification has received little attention. In this study, 60 patients (40 men and 20 women) with PVT were examined using contrast-enhanced computed tomography (CT). The percentage of vein occlusion, including portal vein (PV) and superior mesenteric vein (SMV), was measured on CT image. Of 60 patients, 17 (28.3%) met the criterion of acute PVT. Symptoms occurred more frequently in patients with superior mesenteric vein thrombosis (SMVT) compared to those without SMVT (p<0.001). However, there was no significant difference in PV occlusion between patients with and without symptoms. The frequency of cavernous transformation was significantly higher in patients with complete PVT than those with partial PVT (p<0.001). Complications of portal hypertension were significantly associated with cirrhosis (p<0.001) rather than with the severity of PVT and presence of cavernoma. These results suggest that the severity of PVT is only associated with the formation of portal cavernoma but unrelated to the onset of symptoms and the development of portal hypertension. We classified PVT into complete and partial types, and each was subclassified into with and without portal cavernoma. In conclusion, neither symptom duration nor cavernous transformation can clearly distinguish between acute and chronic PVT. The new classification system can determine the pathological alterations of PVT, patency of portal vein and outcome of treatment in a longitudinal study.

Transjugular intrahepatic portosystemic shunting with covered stents in children: A preliminary study of safety and patency

ObjectiveTo retrospectively analyze the safety and efficacy of transjugular intrahepatic portosystemic shunting (TIPS) using covered stents in children. Material and methodsWe 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. ResultsA 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). ConclusionOur 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.

Tratamiento de la trombosis portal no tumoral en la cirrosis

Portal vein thrombosis in cirrhosis is a relatively common complication associated with the presence of an accompanying prothrombotic phenotype of advanced cirrhosis. The consequences of portal vein thrombosis are relevant because it can be associated with impaired hepatic function, might contraindicate hepatic transplantation and could increase morbidity in the surgical procedure. There is controversy concerning the most effective treatment of portal vein thrombosis, which is based on information that is seldom robust and whose primary objective is to achieve a return to vessel patency. Various studies have suggested that starting anticoagulation therapy early is associated with portal vein repatency more frequently than without treatment and has a low rate of complications. There are no proven data on the type of anticoagulant (low-molecular-weight heparins or dicoumarin agents) and the treatment duration. The implementation of TIPS is technically feasible in thrombosis without cavernous transformation and is associated with portal vein recanalization in a significant proportion of cases. Thrombolytic therapy does not appear to present an adequate balance between efficacy and safety; its use is therefore not supported for this indication. The proper definition of treatment for portal vein thrombosis requires properly designed studies to delimit the efficacy and safety of the various alternatives.