Extrahepatic Portal Obstruction Research Articles

A Rare Case of Portal Vein Thrombosis due to Protein S deficiency in a patient with Decompensated Cryptogenic CLD with Portal Hypertension, Completely Recanalized by Single Oral Anticoagulant-Rivaroxaban

Venous thromboembolic diseases are a group of heterogeneous diseases with different clinical forms and prognosis. Abdominal venous thrombosis may present either as Budd-Chiari syndrome (BCS) caused by hepatic vein or proximal inferior vena cava (IVC) obstruction or as an extra hepatic portal obstruction (EHPVO) caused by Portal vein thrombosis or mesenteric vein thrombosis. Portal vein thrombosis (PVT) is a rare form of venous thrombosis that affects the hepatic portal vein flow, which can lead to portal hypertension. Treatment of PVT includes anticoagulants, thrombolysis, and insertion of shunts, bypass surgery, and liver transplantation. Single anticoagulation therapy can be associated with a reduction in new thrombotic episodes. Here we experienced a 23 year old young lady with history of recent intrauterine death (IUD) diagnosed as PVT provoked by protein S deficiency with newly diagnosed decompensated cryptogenic chronic liver disease with portal hypertension. PVT was completely recanalized with single oral anticoagulant therapy rivaroxaban as initial low molecular weight heparin, enoxaparin administration caused reversible pancytopenia and there is a concern for bleeding and regular monitoring of INR with warfarin in this patient. Keywords: Portal vein thrombosis; Chronic liver disease; Protein S deficiency; Oral anticoagulant; Portal hypertension; Thrombolysis

Japanese periodical nationwide epidemiologic survey of aberrant portal hemodynamics.

AimIdiopathic portal hypertension (IPH), extrahepatic portal obstruction (EHO), and Budd–Chiari syndrome (BCS) are characterized by aberrant portal hemodynamics of unknown etiology. The aim of this study was to explore trends in the descriptive epidemiology of these diseases through periodical nationwide surveys.MethodsNationwide epidemiologic surveys were undertaken in 1999, 2005, and 2015 using the same protocol. The survey targets were selected from all departments of gastrointestinal medicine, surgery, pediatrics, and pediatric surgery in Japan by stratified random sampling according to the number of beds. We asked each department to complete a mail‐back questionnaire on the annual numbers of patients with IPH, EHO, and BCS during the preceding year.ResultsThe estimated number of BCS patients increased from 280 (95% confidence interval, 200–360) in 1999 survey to 410 (300–530) in 2015 survey, whereas the number of IPH and EHO patients has remained largely unchanged during the 15 years (IPH was approximately 1000; EHO was approximately 770 in 2015 survey). The mean age at symptom onset was approximately 45 years for IPH, 30 years for EHO, and 40 years for BCS over the past 15 years. Those who described disease aggravation from the time of diagnosis accounted for approximately 10% of IPH, 15% of EHO, and 20% of BCS patients in each of the three surveys.ConclusionsIn Japan, the prevalence of BCS is increasing, while those of IPH and EHO appear to be stable. Clinical characteristics, including prognoses, have remained largely unchanged in the past 15 years.

Characterization of the coagulation profile in children with liver disease and extrahepatic portal vein obstruction or shunt

ABSTRACTIntroduction: Chronic liver disease causes a disruption of procoagulant and anticoagulant factors, resulting in a fragile state, prone to increased rates of both bleeding and thrombosis. Currently, there is limited literature describing the changes observed in pediatric liver disease and extrahepatic portal vein obstruction or shunt. This study aimed to describe the changes that occur in children with chronic liver disease and extrahepatic portal vein obstruction or shunt. Materials and methods: We measured the concentration and activity of key procoagulant and anticoagulant factors in children with liver disease, children with extrahepatic portal vein obstruction or shunt, and healthy children. Results: Children with severe liver disease had coagulopathic changes, including either decreased concentration or activity of factor II, factor V, and factor VII. Nineteen percent (8/42) of the cohort had significant bleeding. Thrombophilic changes were also observed, including decreased concentration or activity of protein C, protein S, and antithrombin and increased concentration and activity of factor VIII and Von Willebrand factor. Similar coagulation factor changes were observed in children with extrahepatic portal vein obstruction or shunt. There was a trend toward greater changes in coagulation factor activity compared to concentration. Conclusion: This study provides a detailed description of the changes in both the concentration and activity of coagulation factors in pediatric liver disease and extrahepatic portal vein obstruction or shunt. Interestingly, procoagulant and anticoagulant factors were deranged in portal vein obstruction or shunt to a similar degree as in liver disease. An improved understanding of the coagulation profile in the pediatric setting will contribute to the improved management of liver disease and extrahepatic portal obstruction or shunt.Abbreviations: PELD: pediatric end-stage liver disease score; MELD: model for end-stage liver disease score; ELISA: enzyme-linked immunosorbent assay; MCRI: Murdoch Childrens Research Institute; FII: factor II; FV: factor V; FVII: factor VII; FVIII: factor VIII; AT: antithrombin III; A2M: alpha-2-macroglobulin; vWF: Von Willebrand factor; PC: protein C; PS: protein S; ISTH-BAT: International Society on Thrombosis and Haemostasis Bleeding Assessment Tool

Diagnosis and treatment guidelines for aberrant portal hemodynamics: The Aberrant Portal Hemodynamics Study Group supported by the Ministry of Health, Labor and Welfare of Japan.

Idiopathic portal hypertension (IPH), causing aberrant portal hemodynamics, is a disease with an as yet unidentified cause and no established treatment protocol. The Japanese research group on IPH in Japan was set up in 1975 by the Ministry of Health, Labor and Welfare. Extrahepatic portal obstruction and Budd-Chiari syndrome (BCS) have since been added to the group's research subjects. The aims of the research group are to accurately evaluate the current status of the three diseases in Japan, elucidate their etiology and pathogenesis, and develop new treatments. Due to the long-term efforts of the Japanese research group, aberrant portal hemodynamics has been investigated in a variety of aspects, from epidemiological and pathological studies to molecular biology analyses. As a result, it has been shown that there are abnormal genes in the liver, specific for IPH. In addition, pathological findings of BCS were internationally compared and the difference in findings between Japan and Europe (or North America) has been clarified. Furthermore, it was found that complication rates of hepatocellular carcinoma in BCS were higher in Japan. Based on the research, "Diagnosis and treatment of aberrant portal hemodynamics (2001)", including diagnostic criteria for aberrant portal hemodynamics, was published in 2001. In 2013, it was revised to "Diagnosis and treatment guidelines for aberrant portal hemodynamics (2013)" after the incorporation of diagnosis and treatment in accordance with its current status.

3D-CTによる術前計画に基づいて開腹下塞栓術にて止血しえた膵頭十二指腸切除術後肝門部挙上空腸静脈瘤の1例

3D-CTによる術前計画に基づいて開腹下塞栓術にて止血しえた膵頭十二指腸切除術後肝門部挙上空腸静脈瘤の1例

肝外門脈閉塞症を合併した胆嚢皮膚瘻の1例

肝外門脈閉塞症を合併した胆嚢皮膚瘻の1例

Intraductal ultrasonographic anatomy of biliary varices in patients with portal hypertension

Background and Objectives:The term, portal biliopathy, denotes various biliary abnormalities, such as stenosis and/or dilatation of the bile duct, in patients with portal hypertension. These vascular abnormalities sometimes bring on an obstructive jaundice, but they are not clear which vessels participated in obstructive jaundice. The aim of present study was clear the bile ductal changes in patients with portal hypertension in hopes of establishing a therapeutic strategy for obstructive jaundice caused by biliary varices.Materials and Methods:Three hundred and thirty-seven patients who underwent intraductal ultrasound (IDUS) during endoscopic retrograde cholangiography for biliary abnormalities were enrolled. Portal biliopathy was analyzed using IDUS.Results:Biliary varices were identified in 11 (2.7%) patients. IDUS revealed biliary varices as multiple, hypoechoic features surrounding the bile duct wall. These varices could be categorized into one of two groups according to their location in the sectional image of bile duct: epicholedochal and paracholedochal. Epicholedochal varices were identified in all patients, but paracholedochal varices were observed only in patients with extrahepatic portal obstruction.Conclusion:IDUS was useful to characterize the anatomy of portal biliopathy in detail.

直達手術後に慢性骨髄増殖性疾患 (CMPD) が顕性化した肝外門脈閉塞症の1例

直達手術後に慢性骨髄増殖性疾患 (CMPD) が顕性化した肝外門脈閉塞症の1例

Portal Biliopathy in a Human Immunodeficiency Virus (HIV) Positive Patient

Purpose: Portal biliopathy is a rare disorder in the western world. The following case illustrates portal biliopathy in a HIV positive patient with portal vein thrombosis associated with deficiency of protein C, S and antithrombin III. Methods: A 48-year-old white man was referred to our hepatology clinic for persistent elevated liver function tests (LFTs) for 7 months. His medical history was remarkable for HIV on highly active antiretroviral (HAART) therapy. His latest CD4 count was 237/μl. Physical exam was remarkable for splenomegaly and abdominal collateral circulation. Laboratory revealed a total bilirubin (T bil) of 5 mg/dl with direct bilirubin predominance, aspartate aminotransferase (AST) of 49 U/L, alanine aminotransferase (ALT) of 42 U/L, alkaline phosphatase (AP) of 338 U/L, total protein of 6.7 gr/dl, albumin of 2.7 gr/dl, INR 1.6, hemoglobin of 12.2 gr/dl and platelets count of 123,000/μl. Results: An abdominal computed tomography (CT) showed portal vein thrombosis with cavernous transformation, intra-abdominal varices, splenomegaly, and diffuse intra and extrahepatic bile dilation with no stones or obstructing mass. An endoscopic retrograde cholangiopancreatography (ERCP) revealed dilated intrahepatic and extrahepatic bile ducts. The common hepatic duct and the common bile duct measured 1.4 cm and 1.2 cm, respectively. The bile duct walls were irregular and a narrowing in the distal common bile duct was noted. A biliary stent was placed. The hepatic vein pressure gradient and the transjugular liver biopsy were unremarkable. During follow-up, the patient had an improvement of his LFT's with T bil of to 1.3 mg/dl, AP of 112 U/L, AST of 38 U/L, and ALT of 26 U/L. His prothrombotic disorder studies revealed protein C, S and antithrombin III deficiency. Conclusion: Portal biliopathy is associated with extrahepatic portal obstruction. It is a rare disorder in the western world where the most common cause of portal hypertension is cirrhosis. On the contrary, portal biliopathy is seen more commonly in the developing world where 40% of portal hypertension is due to noncirrhotic causes. HIV/acquired immune deficiency syndrome (AIDS) patients are at increased risk of acquiring prothrombotic disorders that can cause portal vein thrombosis. Portal biliopathy should be considered as a differential diagnosis in a patient with dilated biliary ducts and portal hypertension.

Hemorrhage from varices in hepaticojejunostomy in the fifth and tenth year after surgery for hepatic hilar bile duct cancer: a case report

We report a case of a 64-year-old female patient who underwent a right lobectomy of the liver (including total resection of the caudate lobe), dissection of the group 2 lymph nodes, left hepaticojejunostomy (Roux-en-Y fashion), and reconstruction of the portal vein (end-to-end anastomosis between the main portal vein and the left portal branch) for treatment of hepatic hilar bile duct cancer in 1996. In 2001, the anastomotic site of the hepaticojejunostomy was dissected and re-anastomosed due to gastrointestinal bleeding caused by variceal rupture in the jejunal loop. In 2006, splenectomy was performed for recurrence of gastrointestinal bleeding due to another variceal rupture in the jejunal loop. Portal venography performed perioperatively showed a decrease in portal blood flow into the liver via the jejunal varices and an increase in portal blood flow into the liver via the left gastric vein. She had two jejunal variceal ruptures at five-year intervals after extrahepatic portal obstruction and underwent successful treatments.

Transabdominal oesophageal transection for oesophageal varices: experience in 101 patients.

The results of transabdominal oesophageal transection in 101 patients with oesophageal varices are reported. The primary diagnosis was liver cirrhosis and 65 patients, idiopathic portal hypertension in 29 and extrahepatic portal obstruction in 7. Twelve patients died postoperatively. The mortality rate for emergency operations was higher than that for elective and prophylactic procedures. The majority of patients died from hepatic failure. Improvement, judged by radiographic and endoscopic examination, was 100 per cent and 96 per cent, respectively. Post-transection rebleeding was observed in 14 of 89 patients during the follow-up period of 1-13 years. There were 25 late deaths, caused mainly by hepatic failure. The 5-year survival rate, including postoperative deaths, was 72 per cent in idiopathic portal hypertension and 55 per cent in liver cirrhosis. We suggest that transabdominal oesophageal transection is an acceptable operative procedure for oesophageal varices.

Proximal gastric resection in the treatment of bleeding gastro-oesophageal varices in patients with portal hypertension due to extrahepatic obstruction.

Abstract Proximal gastric resection is, in our opinion, the operation of choice in the treatment of recurrent haemorrhage in three groups of patients with extrahepatic portal obstruction leading to portal hypertension:— In children who are too small for splenorenal or mesocaval anastomosis, when the haemorrhage can no longer be controlled by periodic injection of the varices through an oesophagoscope.In patients of any age with no suitable radicle of the portal venous tree available for portal systemic anastomosis.In patients who have had splenectomy alone or with gastric transection, or who have had previous shunt operations which have failed to control haemorrhage. It is the only operation short of total gastrectomy in which the varix-bearing area of the stomach is removed. Twenty-eight patients have had this operation at St. Bartholomew's Hospital in the 16-year period 1949-65. The total and late mortality (4 patients) is 14 per cent during a mean follow-up period of 10 years. Only I patient could remotely be considered as an operative death (3.5 per cent). There was no recurrence of haemorrhage in 53 per cent. Three patients (10.5 per cent) had severe haemorrhage from recurrent varices. Two of these died and are included in the mortality figures. The operative procedure is described.

Idiopathic portal hypertension associated with systemic sclerosis and Sjögren’s syndrome

We report a patient with idiopathic portal hypertension (IPH) associated with systemic sclerosis (SSc) and Sjögren's syndrome. A 72-year-old Japanese woman was admitted to our hospital because of Raynaud's phenomenon, sclerodactyly, and dyspnea. The patient had splenomegaly, esophageal varices in the absence of extrahepatic portal obstruction, and cirrhosis of the liver. Immunological studies revealed positive anti-nuclear antibodies and high titers of anti-Scl-70, anti-SS-A, anti-centromere, and anti-mitochondrial M2 antibodies. Histological examinations of the liver biopsy specimen revealed stenosis and loss of small portal veins without findings of primary biliary cirrhosis. The patient was diagnosed as having IPH associated with SSc and Sjögren's syndrome. These observations suggest an immunological role in the pathogenesis of IPH.

A patient with adult extrahepatic portal obstruction, of which distinction from intrahepatic cholangiocarcinoma was difficult

A 51-year-old Japanese male with chief complaints of slightly high fever and epigastralgia was hospitalized at our facility. The inflammatory response was enhanced, and liver dysfunction was observed. Abdominal ultrasonography demonstrated a hyperechoic lesion occupying the left portal vein, and abdominal plain CT indicated a low density of the lesion with a clear boundary, measuring about 3 cm x 2 cm, between the porta hepatis and segment IV of the liver. Contrast CT showed no enhancement in the arterial and portal phases, but a reduction in the density inside the tumor in the equilibration phase was noted. MRI showed hypointensity by T1-weighted imaging and hyperintensity by T2-weighted imaging. Angiography demonstrated an obstruction of the left portal vein and superior mesenteric vein, and endoscopic retrograde cholangiography revealed a constriction in the left intrahepatic bile duct. Since the possibility of intrahepatic cholangiocarcinoma could not be excluded, extended left hepatectomy combined with caudate lobectomy was performed. The tumor, measuring 31 mm x 21 mm x 20 mm, was pathohistologically diagnosed as an extrahepatic portal obstruction. Extrahepatic portal obstruction is an important disease that is sometimes difficult to rule out oncologic origin.

A case of ruptured esophageal varices caused by secondary extrahepatic portal obstruction followed by thorombosis of splenic vein aneurysm

A case of ruptured esophageal varices caused by secondary extrahepatic portal obstruction followed by thorombosis of splenic vein aneurysm

Endoscopic injection Scleotherapy and anti-platelet therapy in an adult patient with extra-hepatic portal obstruction

Endoscopic injection Scleotherapy and anti-platelet therapy in an adult patient with extra-hepatic portal obstruction

A Case of Adult Primary Extra Hepatic Portal Obstruction

A Case of Adult Primary Extra Hepatic Portal Obstruction

Long-term efficacy of partial splenic embolization in children

Purpose: To elucidate the role of partial splenic embolization (PSE) procedures, long-term outcome was assessed in terms of the recurrence of thrombocytopenia. Methods: A retrospective study was performed after 41 PSE procedures in 36 patients for hypersplenism owing to portal hypertension. The underlying disease was biliary atresia in 32 patients, extrahepatic portal obstruction in 3, and idiopathic cirrhosis in 1. Results: The average volume embolized was 70.1%. The patients were followed up from 20 days to 182 months (average, 70.8 months). Five patients subsequently died, and 6 underwent liver transplantation. The causes of death or the reasons for liver transplantation were not related to hypersplenism. Eleven patients (30.6%) had recurrence of thrombocytopenia (<100,000/mm 3). There was no significant difference in the volume embolized or platelet count before PSE between the patients with and without recurrence of thrombocytopenia. The peak value of platelet count after PSE was significantly lower in the patients with recurrence of thrombocytopenia ( P = .0091). In 17 of 24 survivors without liver transplantation, platelet counts remained normal throughout the follow-up period. Conclusions: PSE is a safe and effective procedure. Hematologic indices improved in all 36 patients after PSE, and its long-term efficacy was shown in 70% of the survivors.

Hemodynamics in extrahepatic portal vein obstruction and its changes during long-term follow-ups

Thirty-six patients with extrahepatic portal vein obstruction (EHO) were studied. Twenty-one patients, whose age at onset was under 20 years old, were thought to be primary EHO. Ten of 15 patients over 21 years old, had a history of abdominal diseases, and were thus considered to be secondary EHO; only 5 were primary EHO. EHO was classified into three categories, based on selective celiac-superior mesenteric arteriography, and intraoperative trans-splenic and superior mesenteric venography: the hilar obstruction of portal vein, the portal trunk obstruction, and the portal trunk–splenic vein obstruction. Both primary and secondary EHO cases were found to have extended their obstructed region over time by progressing from hepatic hilum to portal trunk obstruction followed by splenic vein obstruction. On the other hand, based on retrograde portography, EHO was classified into the absence of intrahepatic portal obstruction (portal trunk type) and the intrahepatic obstruction (portal branch type) and the portal branch type tended to predominate. Changes of intra- and extra-hepatic portal obstruction were examined by comparing various angiographies of the 14 patients available for between 1 and 26 years of long-term follow-up. While EHO tended to progress with time, no patient showed progress of a intrahepatic portal vein obstruction. From these results, EHO patients are expected to show continuous progression of EHOs during long-term follow-ups, and to have high recurrence rates of gastrointestinal varices after various treatments due to the persistent portal hypertension. However, effective hepatic flow is likely to be maintained because collateral circulation to the liver develops, and intrahepatic portal vein obstruction does not progress, resulting in a good prognosis for EHO.

A case report of long term follow-up of duodenal varices associated with extrahepatic portal obstruction.

A case report of long term follow-up of duodenal varices associated with extrahepatic portal obstruction.