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.     

Antiphospholipid antibodies and lipoprotein (a) as etiologic or contributory factors in patients with idiopathic cavernous transformation of portal vein.

BACKGROUND/AIMS: Antiphospholipid antibodies consisting of anticardiolipin antibodies and lupus anticoagulant are strongly associated with thrombosis in adult patients. It is also well known that there is a close relationship between antiphospholipid antibodies and lipoprotein (a) in thrombous formation. The aim of this study was to determine whether antiphospholipid antibodies and lipoprotein (a) have any effect on the formation of thrombosis in the portal vein of patients with 'idiopathic' cavernous transformation of the portal vein. METHODS: Twenty seven patients with idiopathic cavernous transformation of the portal vein (Group 1) seen at Hacettepe University Hospital were identified and prospectively studied. All were investigated for antiphospholipid antibodies and lipoprotein (a). Anticardiolipin antibodies, lupus anticoagulant and lipoprotein (a) were measured using commercially available kits. The findings of these 27 patients were compared with those of 20 healthy control subjects (Group 2). RESULTS: Anticardiolipin antibodies, especially ACA Ig G and lipoprotein (a) levels were found to be higher than of healthy controls and statistically significant differences were documented in two of these parameters, which seems to play an important role in thrombous formation in the portal vein. There was no correlation for lupus anticoagulant between the two groups. CONCLUSIONS: Anticardiolipin antibodies and lipoprotein (a) are strongly associated with thrombosis in the portal vein, producing a favorable medium for and acting as contributory factors in thrombous formation. It is suggested that these factors should be evaluated carefully in patients with 'idiopathic cavernous transformation of the portal vein'.     

Extrahepatic portal venous obstruction of different pathogenesis in pancreatic diseases: reports of 4 autopsy cases with chronic pancreatitis and pancreatic carcinoma

Four autopsy cases of extrahepatic portal venous obstruction associated with pancreatic diseases, 1 case of pancreatitis and 3 cases of pancreatic carcinoma, are presented. The pathogenesis of portal obstruction was different in each case; old thrombosis with recanalization due to chronic pancreatitis with pseudocysts formation in 1 case, fresh thrombosis due to intraportal venous catheterization for pancreatic carcinoma in 1 case, fresh thrombosis probably due to pancreatitis accompanying pancreatic carcinoma in 1 case, and direct invasion of pancreatic carcinoma into the portal vein in the remaining 1 case. Morphologic evidence for portal hypertension was present in each case. In the pancreatitis case and one pancreatic carcinoma case with portal tumor invasion, both of which had chronic portal obstruction, there were many thin-walled vascular channels (cavernous transformation) around the occluded portal vein. Their endothelia were positive for factor VIII-related antigen and Ulex europaeus lectin I, implying that these vessels were hepatopetal blood vascular collaterals. It was shown that pancreatic diseases resulted in portal venous obstruction by several different mechanisms and chronic portal obstruction in pancreatic diseases led to the formation of hepatoperal blood vascular collaterals.     

Bile Duct Varices or "Pseudo-Cholangiocarcinoma Sign" in Portal Hypertension Due to Cavernous Transformation of the Portal Vein

A total of 832 patients with portal hypertension resulting from different etiology was studied by ultrasonograph as a screening test. In 17 of the 832 patients, cavernous transformation of the portal vein was detected by means of ultrasonography. We have prospectively studied these 17 patients, and the diagnosis of cavernous transformation was confirmed by portography in all patients. To evaluate how much biliary tract has been affected from cavernous transformation of the portal vein, and to explain the cause of mildly increased alkaline phosphatase and serum bilirubin levels, endoscopic retrograde cholangiopancreatography (ERCP) was performed in 16 of the 17 patients. There were narrowing, irregularity, undulation and nodular extrinsic defects resulting from compression of thrombosis of the portal vein and the collateral vessels, mimicking cholangiocarcinoma spreading along the common bile duct on the extrahepatic biliary tract in all 16 patients who underwent ERCP. Similar ERCP findings were not found in six patients with portal hypertension due to liver cirrhosis. The ultrasonographic, portographic, and ERCP findings corresponded to surgical findings in six patients who had undergone splenectomy for either hypersplenism or bleeding from esophageal varices. The results indicate that cavernous transformation of the portal vein cause the above-mentioned radiographic findings that we propose to call "pseudo-cholangiocarcinoma sign."     

螺旋CT诊断肝癌合并门静脉海绵样变性及其在介入治疗中的意义

目的分析门静脉海绵样变性（CPTV）患者的CT表现和特征,探讨螺旋CT诊断该病的价值及临床意义。方法回顾性分析28例经彩色多普勒、MRI或手术证实的CTPV,所有病例均行上腹部螺旋CT平扫及动态增强扫描。结果肝癌合并CTPV的螺旋CT主要表现为：门静脉主干或其分支扩张增粗,门静脉走行区可见迂曲扩张、紊乱的静脉血管影;肝实质动脉期一过性异常灌注;显示原发病灶。结论肝癌合并CTPV有特征性螺旋CT表现,螺旋CT可以诊断门静脉海绵样变性。正确认识其CT表现对肝癌的介入治疗具有重要指导意义：对于肝癌合并门静脉癌栓患者,在有CTPV形成时,经皮肝动脉栓塞治疗是安全、可行的。     

Portal vein resection without reconstruction during Appleby operation in a patient with pancreatic body carcinoma with cavernous transformation.

The prognosis of pancreatic body carcinoma has been poor due to cancerous invasion of major vessels. Resection of the involved vessels may improve resectability and prognosis. We report a patient who had a pancreatic body carcinoma with cavernous transformation of the portal vein, in whom the portal vein was resected without reconstruction during an Appleby operation. A 67 year-old man was admitted for evaluation of back pain. Enhanced computed tomography showed no main trunk of the portal vein but a developed collateral circulation. Celiac angiography revealed encasement of the common hepatic, splenic and celiac artery. Venous angiography revealed obstruction of the portal and splenic veins with cavernous transformation surrounding these veins. Pre-operative diagnosis was carcinoma in the pancreatic body, which invaded the portal vein, the celiac and common hepatic arteries. The Appleby operation combined with resection of the portal vein without reconstruction could be performed, by preserving collateral vessels and monitoring hepatic venous oxygen saturation (ShvO2) to prevent hepatic ischemia caused by occlusion of the portal vein. The post-operative course was uneventful.     

A case of hemorrhage from varices of an interposed jejunum after choledochojejunostomy treated successfully by endoscopic injection using α-cyanoacrylate monomer

A 73-year-old woman was admitted with gastrointestinal bleeding. She had undergone pylorus-preserving pancreaticoduodenectomy, hepaticojejunostomy and pancreatojejunostomy for pancreatic cancer a year earlier. Gastrointestinal endoscopy revealed bleeding from varices in an interposed jejunum. Enhanced CT showed an extrahepatic portal venous obstruction and cavernous transformation of the portal vein, which were complications of these operations. We performed endoscopic injection using α-cyanoacrylate monomer for the varices. After 4 treatments, the bleeding stopped. We concluded that endoscopic injection using α-cyanoacrylate monomer was effective and useful treatment for bleeding from hepatopetal varices, including cavernous transformation of the portal vein. This method is also useful in emergency situations.     

特发性门静脉高压症的彩色多普勒超声诊断与临床价值

目的探讨特发性门静脉高压症（IPH）的声像图特征,评价彩色多普勒对IPH的临床诊断价值。方法对25例IPH患者进行彩色多普勒超声检查,观察肝脏表面、内部回声、脾脏大小及肝内外门静脉系统等。结果25例患者中,25例均见门静脉肝内分支管壁增厚、回声增强、管腔狭窄甚至闭塞,15例实质回声增粗,门静脉海绵样变性22例,门静脉系统血栓5例,均为门静脉主干、脾静脉及肠系膜上静脉血栓,15例伴有胆道系统的异常。结论临床上不明原因的门脉高压及脾功能亢进患者均应进行彩色多普勒超声检查,肝内门静脉分支管壁增厚、管腔狭窄甚至闭塞的特征性改变及门静脉海绵样变性可提示IPH。     

Cavernous transformation of the portal vein in nephrotic syndrome

We report a 19-year-old man with thrombosis of the portal vein associated with a nephrotic syndrome. A computed tomography showed obstruction of the portal vein with prominent collaterals and cavernous transformation. This case is noteworthy as a report of nephrotic syndrome accompanied by extensive abdominal venous thrombosis and was cured successfully.     

Membranous obstruction of the portal vein. A case report

Membranous obstruction of the portal vein has not been previously reported. This 56-yr-old Indonesian man with a history of a tropical fever 5 yr earlier presented with variceal bleeding. Transhepatic obliteration of the varices was attempted, but the catheter placed in the right portal branch would not pass beyond the porta hepatis. Another catheter was inserted into the portal trunk under ultrasound guidance. Simultaneous opacification through the two catheters demonstrated a complete membranous obstruction of the portal vein at the porta hepatis and a portal-superior mesenteric-venous axis that had lost communication with incoming veins and the left portal branch, most likely because of multiple thrombosis. Other angiographic procedures also revealed marked hepatopetal collaterals (cavernous transformation) entering the liver through the hilum. Liver biopsy showed acute posttransfusion hepatitis superimposed upon portal fibrosis. The possible mechanism for membrane formation in relation to thrombosis is discussed.     

Portal and mesenteric vein and inferior vena cava thrombosis associated with antiphospholipid syndrome

We report a 48-year-old man with thrombosis of the portal and superior mesenteric vein and inferior vena cava associated with primary antiphospholipid syndrome (APS). Primary APS was diagnosed by a positive reaction with anticardiolipin antibody (aCL) and the absence of any evidence suggesting the presence of other disease states known to be associated with aCL. A coeliac angiography showed obstruction of the portal and superior mesenteric vein with prominent collaterals and cavernous transformation. Femoral vein angiography showed total obstruction of the external iliac vein and inferior vena cava, and dilation of the pelvic veins, with contrast medium in the lumbar vein. This case is noteworthy as a report of primary APS accompanied by extensive abdominal and pelvic venous thrombosis.     

Transjugular intrahepatic portosystemic shunt for palliative treatment of portal hypertension secondary to portal vein tumor thrombosis

AIM: To evaluate the palliative therapeutic effects of transjugular intrahepatic portosystemic shunt (TIPS) in portal vein tumor thrombosis (PVTT) complicated by portal hypertension. METHODS: We performed TIPS for 14 patients with PVTT due to hepatocellular carcinoma (HCC). Of the 14 patients, 8 patients had complete occlusion of the main portal vein, 6 patients had incomplete thrombosis, and 5 patients had portal vein cavernous transformation. Clinical characteristics and average survival time of 14 patients were analysed. Portal vein pressure, ascites, diarrhoea, and variceal bleeding and circumference of abdomen were assessed before and after TIPS. RESULTS: TIPS was successful in 10 cases, and the successful rate was about 71%. The mean portal vein pressure was reduced from 37.2 mmHg to 18.2 mmHg. After TIPS, the ascites decreased, hemorrhage stopped and the clinical symptoms disappeared in the 10 cases. The average survival time was 132.3 d. The procedure failed in 4 cases because of cavernous transformation in portal vein and severe cirrhosis. CONCLUSION: TIPS is an effective palliative treatment to control hemorrhage and ascites due to HCC complicated by PVTT.     

Extrahepatic portal obstruction in adults--report of an autopsy case

We report the case of a 31-year-old female with extrahepatic portal vein obstruction. At the age of 16 she had developed hematemesis from esophageal varices. Trans-splenic portography failed to demonstrate the portal trunk, but showed instead well developed collateral circulation. She suffered from repeated hematemesis and ascites despite mesocaval shunt operation and resection of the esophagus for varices. Death resulted from gastrointestinal hemorrhage. Her family history revealed a 30-year-old sister with Takayasu's disease. On autopsy, the portal trunk and the portal vein at the hilus were indistinguishable due to proliferation of hyalinized connective tissue. Cavernous transformation was observed at the hilus. Histologically the presence of the portal trunk and the portal vein was confirmed, but they showed marked sclerosis, and were almost occluded by possible organized thrombus. The cavernous transformation consisted of numerous thin-walled vessels, which were also observed in the medium-sized portal tracts in the liver. The peripheral portal tracts showed no obvious narrowing nor occlusion of the portal vein branches, which are factors usually considered to play an important role in the genesis of portal hypertension in idiopathic portal hypertension. Even though the etiology is not clear, the portal trunk obstruction is concluded to be a significant factor in this case contributing to the development of protracted portal hypertension.     

脾切除贲门周围血管离断术治疗晚期血吸虫病并门静脉海绵样变

目的 目的 探讨晚期血吸虫病门静脉高压症合并门静脉海绵样变的外科治疗方式。 方法 方法 对湖南省血吸虫病防治 所附属湘岳医院18例晚期血吸虫病合并门静脉海绵样变患者的临床资料进行回顾性分析。结果 结果 16例患者行脾切除贲 门周围血管离断术; 2例患者先行内镜食管曲张静脉套扎术, 后因再次食管静脉曲张破裂出血, 分别于术后32、 40个月行脾 切除贲门周围血管离断术。术后随访6～72个月, 未再发现食管胃底静脉曲张。结论 结论 脾切除贲门周围血管离断术适用 于晚期血吸虫病门静脉高压合并门静脉海绵样变的治疗。     

Portal vein thrombosis: real-time sonographic demonstration and follow-up

Twelve patients with portal vein thrombosis (PVT) associated with various disorders were examined. In 11 the diagnosis of PVT was made primarily by ultrasound. Endovenous lesions that presented with a mainly homogenous texture pattern of different echodensity could be clearly displayed, but failed to disclose specific echographic features that allow conclusive discrimination between blood clots (n = 7; mean diameter 13 +/- 1.7 mm) and venous tumor invasion (n = 5; mean diameter 24 +/- 12 mm). Thrombus resolution on therapy as well as cavernous transformation of the portal vein following acute PVT may be visualized by serial sonograms. Secondary findings in PVT that can be displayed by sonography include splenomegaly and superior mesenteric vein obstruction with intestinal ischemia. Real-time sonography has proved to be a valuable noninvasive method for the early diagnosis and follow-up of PVT.     

超声检查门静脉海绵样变特征表现

目的 探讨门静脉海绵样变(CTPV)及其相关并发症的超声表现特征,并分析了超声漏诊的原因。方法 2017年6月～2020年6月首都医科大学附属北京地坛医院诊治的101例CTPV患者,经CT和MRI增强扫描诊断,接受腹部超声检查。总结CTPV及其相关并发症超声图像表现特征,并分析超声漏诊的原因,提出解决策略。结果 在本组101例CTPV患者中,超声诊断82例(82.2%),漏诊19例(17.8%);在超声诊断病例中,82例(100.0%)患者门静脉主干及其分支周围出现广泛或局部蜂窝状粗细不等的血管结构,70例(85.4%)出现门静脉管壁增厚,76例(92.7%)在迂曲扩张的蜂窝状血管结构内部呈红蓝相间的丰富的血流信号,脉冲多普勒测得门静脉样低速血流频谱,10例(12.2%)门脉周围迂曲扩张蜂窝状血管压迫胆管,导致胆管扩张,8例(9.6%)出现胆囊增大,6例(7.4%)出现胆囊结石,5例(6.1%)出现胆管结石,5例(6.1%)出现门体分流。结论 超声是诊断CTPV的首选影像学方法,超声漏诊的原因主要为海绵样变血管腔细、病变范围小,腹部胀气,假性胆管肿瘤征,肋间隙或胸骨下角窄。利用腹腔积液和胆囊等声窗扫查肝脏,采用超声造影技术或结合其他影像学技术检查可减少漏诊。     

Is portal vein cavernous transformation a component of congenital hepatic fibrosis？

Congenital hepatic fibrosis （CHF） is an autosomal recessive disorder that belongs to the family of fibropolycystic liver diseases. This family includes a spectrum of disorders which are usually found in combination with each other and are usually inherited. Clinically fibropolycystic diseases have three effects being present in different proportions, those of a space occupying lesion, of portal hypertension and of cholangitis. In most patients, the first manifestations of CHF are signs and symptoms related to portal hypertension such as splenomegaly and varices. Portal hypertension in these patients has been attributed to the hypoplasia or compression of the portal vein radicles in the fibrous bands. Cavernous transformation of the portal vein （CTPV） is a relatively rare condition resulting from extrahepatic portal vein obstruction with recanalization or collateral vein formation to bypass the obstruction. It has been found that patients with CHF having an accompanying CTPV have relatively large splenomegaly and suffers more frequent episodes of bleeding from esophageal varices.We believe that CTPV is a congenital component of CHF and also one of the important causative factors of portal hypertension in these patients.     

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 eig...     

Endoscopic management of biliary obstruction caused by cavernous transformation of the portal vein

Background

Symptomatic biliary obstruction caused by cavernous transformation of the portal vein isan extremely rare disorder for which there is no consensus as to optimal treatment. The results of endoscopic treatments in a small group of patients is reviewed.

Methods

A total of 10 patients (5 men, 5 women; mean age 36.1 years, range 17-48 years) with severe biliary strictures were treated between 1995 and 2001. Biliary sphincterotomy was performed in all patients. Four patients also underwent balloon dilation, nasobiliary drainage, and stone or sludge extraction by using a balloon. All patients had stent insertion.

Observations

The mean duration of therapy was 3.3 years (range 1-7 years). There was no complication directly related to the endoscopic procedures except for hemobilia that occurred in one patient during stent removal. Cholangitis developed in 5 patients during the therapy period and was treated endoscopically. In 4 patients, significant improvement in the biliary stricture was observed and stents were removed in 3. These patients were followed without stent insertion for one year.

Conclusions

Endoscopic management of biliary stricture caused by cavernous transformation of the portal vein appears to be effective and safe.     

孤立性胃静脉曲张的病因和临床特征

目的 分析孤立性胃静脉曲张的病因和临床特征.方法 选择2003年1月至2008年1月收治的31例孤立性胃静脉曲张患者,回顾性分析其病因、临床表现、影像学检查及治疗情况.结果 31例占同期胃食管静脉曲张患者的7.38%(31/420).病因依次为左侧门脉高压(14例,45.2%)、肝硬化(8例,25.8%)、不明原因(6例,19.4%)、原发性肝癌(2例,6.5%)、门静脉海绵样变性(1例,3.2%).曲张静脉破裂出血21例(67.7%).脾肿大21例(67.7%),其中脾功能亢进10例(32.3%).18例患者有明确的受侵静脉,其中脾静脉阻塞9例(50%),门静脉受侵9例(50%).外科手术8例,硬化剂治疗3例,其余20例内科保守治疗.结论 孤立性胃静脉曲张大部分由左侧门脉高压引起,部分病因是肝硬化.手术是治疗出血和预防再出血的有效措施.