"Portal double ductopathy sign" in patients with portal vein cavernous transformation

BACKGROUND/AIMS: The aim of this study was to determine to what extent portal vein cavernous transformation affects the biliary tract and pancreatic duct system concurrently. METHODOLOGY: Patients who had liver cirrhosis, liver malignancy, history of chronic alcohol use, chronic pancreatitis, pancreatic cancer, or surgery of upper abdomen were excluded. Endoscopic retrograde cholangiopancreatography was performed on all patients. The radiographic images from the biliary and pancreatic systems obtained at the same session were evaluated. RESULTS: In 36 patients, both systems were evaluated. In 2 out of 36 patients, the biliary system was normal, while in 5 patients (13.9%) the pancreatic duct was found to be normal. Thus, 31 patients (12 men, 19 women) had successful visualization. Additionally, 31 out of 36 patients had luminal narrowing throughout the pancreatic duct, local atrophy at head of the pancreas with moderate dilatation behind the narrowed segment. These findings were not found in control patients. In 4 patients who underwent splenectomy for severe hypersplenism, radiological findings were consistent with surgical findings. CONCLUSIONS: The above mentioned morphological findings which are demonstrated in biliary and pancreatic duct are novel findings which the authors propose to call "portal double ductopathy sign" when both ducts are affected concurrently.     

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...     

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。     

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.     

Disappearance of the Pseudo-cholangiocarcinoma Sign after TIPSS

Bile duct varices, also named the "pseudo-cholangiocarcinoma sign," are frequently encountered by ERCP in portal hypertension cases due to cavernous transformation of the portal vein. There is documentation that, in three cases, this sign disappeared after placement of a transjugular intrahepatic portosystemic shunt.     

Effects of Esophageal Transection Combined with Splenectomy on Portal Hemodynamics

Portal hemodynamics were studied in 69 patients with cirrhosis and 29 patients with idiopathic portal hypertension to investigate the effects of an operative procedure for varices that consists of transabdominal esophageal mucosal transection, paraesophagogastric devascularization, pyloroplasty, and splenectomy. Portal venous flow measured by the pulsed Doppler flowmeter in 14 patients with cirrhosis and nine patients with idiopathic portal hypertension, who underwent operation 2-5 yr earlier, was significantly reduced compared with that in unoperated 49 patients with cirrhosis and 17 patients with idiopathic portal hypertension who had esophageal varices (410 +/- 158 versus 660 +/- 263 ml/min in cirrhosis; 443 +/- 185 versus 912 +/- 189 ml/min in idiopathic portal hypertension). In nine patients (six cirrhosis, three idiopathic portal hypertension), portal venous flow and portal vein pressure were measured before and after operation. In patients with cirrhosis, portal vein pressure did not change significantly postoperatively even though portal venous flow was reduced. In contrast, portal vein pressure decreased in two patients with idiopathic portal hypertension in whom portal venous flow was reduced. Portal vein pressure was elevated in one patient with idiopathic portal hypertension in whom portal venous flow was increased postoperatively as a result of resection of a large gastro- and splenorenal shunt conducted additionally.     

Cavernous transformation of the portal vein associated to multiorgan developmental abnormalities.

Initial diagnosis of cavernous transformation of the portal vein (portal cavernoma) is rarely made in adults. Its main clinical manifestation is upper gastrointestinal hemorrhage due to variceal bleeding. More rarely, diagnosis is made from obstructive jaundice. In children, this condition is frequently associated to prehepatic portal hypertension and congenital anomalies, the most frequent of which are atrial septal defects or malformations of the biliary tract or of the inferior vena cava. We describe here a case of a 23-year-old female presenting with massive hematemesis due to the presence of esophageal and small intestinal varices. She had a cavernous transformation of the portal vein with prehepatic portal hypertension associated with heretofore unreported malformations such as right pulmonary hypoplasia, cardiac dextroposition, and right renal ectopia. A unifying hypothesis (e.g. an intrauterine vascular insult) to explain the pathogenesis of these defects seems unlikely. Appropriate tests failed to identify specific functional abnormalities in these organs. Although she bled more than once, the combination of sclerotherapy and beta-blockers has been, thus far, able to control the major clinical consequences of this disease.     

Portal hemodynamics in primary biliary cirrhosis as evaluated by per-rectal portal scintigraphy with Tc-99m pertechnetate

BACKGROUND/AIMS: Portal circulation can be evaluated in a relatively noninvasive manner by per-rectal portal scintigraphy. We used this method to evaluate portal hemodynamics in patients with primary biliary cirrhosis and idiopathic portal hypertension. We did the procedures simultaneously in some patients to examine the relation between portal circulation and hepatic functional reserve in these diseases. METHODOLOGY: Per-rectal portal scintigraphy with Tc-99m pertechnetate was done in 17 healthy subjects, 154 patients with chronic hepatitis, 447 patients with cirrhosis, 40 patients with primary biliary cirrhosis, and 20 patients with idiopathic portal hypertension. Eighty-three patients (14 with hepatitis, 48 with cirrhosis, 16 with primary biliary cirrhosis, and 5 with idiopathic portal hypertension) also underwent scintigraphy with Tc-99m galactosyl human serum albumin with 2 weeks. A solution containing Tc-99m pertechnetate was instilled into the rectum, and serial scintigrams were taken while radioactivity curves for the liver and heart were recorded sequentially. The per-rectal portal shunt index was calculated from the curves. A receptor index was calculated by dividing the radioactivity of the liver region of interest by that of the liver-plus-heart region of interest 15 min after the injection of Tc-99m galactosyl human serum albumin. The index of blood clearance was calculated by dividing the radioactivity of the heart region of interest at 15 min by that of the heart region of interest at 3 min. RESULTS: The shunt index was higher for more severe disorders, increasing in the order of chronic hepatitis, cirrhosis without varices, and cirrhosis with varices. The shunt indices in patients with primary biliary cirrhosis and idiopathic portal hypertension were higher than that in patients with chronic hepatitis. In terms of receptor index, the standard residuals were more than 0 in 10 of 16 patients with primary biliary cirrhosis and 4 of 5 patients with idiopathic portal hypertension. In terms of index of blood clearance, the standard residuals were more than 0 in 10 of 16 patients with primary biliary cirrhosis and 4 of 5 patients with idiopathic portal hypertension CONCLUSIONS: Abnormalities of portal hemodynamics in patients with primary biliary cirrhosis or idiopathic portal hypertension occur while hepatic functional reserve is still satisfactory as compared with patients who have chronic hepatitis or cirrhosis.     

Portal hypertension in primary biliary cirrhosis

Evidence of portal hypertension was found in 50 out of 109 patients (47%) with primary biliary cirrhosis, and of these 32 bled from oesophageal varices. In four patients portal hypertension was the initial manifestation of the disease and this complication was recognized in a further 17 within two years of the first symptom of primary biliary cirrhosis. The development of portal hypertension was associated with a poor prognosis and death could frequently be attributed to variceal bleeding; the mean duration of survival from the time that portal hypertension was recognized was 14.9 months. Portal decompression operations may have improved the immediate prognosis in some patients but did not otherwise influence the progression of the disease. In 47 patients the histological findings in wedge biopsy or necropsy material were correlated with the presence or absence of varices. An association between nodular regeneration of the liver and varices was confirmed, but, in the absence of nodules, no other histological cause for portal venous obstruction could be found.     

Formation of hilar collaterals or cavernous transformation after portal vein obstruction by hepatocellular carcinoma. Observations in ten patients

A total of 155 patients with hepatocellular carcinoma were studied by celiac and superior mesenteric angiography. Complete (9 patients) or near complete (1 patient) obstruction of the portal vein and formation of hepatopetal collateral veins in the porta hepatis, or the so-called cavernous transformation of the portal vein, were seen in 10 patients. In 4 patients, the first angiogram did not show cavernous transformation, but on the follow-up angiograms cavernous transformation was present. The suggested interval between obstruction of the portal vein and formation of cavernous transformation was no more than 5 wk. The mechanism of cavernous transformation and its clinical implications are briefly discussed.     

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 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.     

Portal hypertensive gastropathy with portal thrombosis successfully treated with partial splenic embolization

A 60-year-old man with alcoholic liver cirrhosis was admitted to our hospital with severe anemia and tarry stool. Upper gastrointestinal endoscopy revealed grade 4 esophageal varices without bleeding and severe portal hypertensive gastropathy (PHG) of the fornix of the stomach with oozing. These findings suggested that PHG was the cause of progression of anemia. Abdominal computed tomography demonstrated no enhancement of the main portal vein and its first branches, indicating portal thrombosis and cavernous transformation. The patient underwent partial splenic embolization (PSE) to reduce portal hypertension. Two months after PSE was performed, upper gastrointestinal endoscopy showed improvement of PHG and endoscopic variceal ligation was performed to treat the esophageal varices. Contrast-enhanced CT revealed partial enhancement of the main portal vein indicating improvement of portal thrombosis. One year after PSE, hemoglobin had increased from 6.0 to 11.0 g/dl without blood transfusion. Moreover, albumin level had risen from 2.8 to 3.7 g/dl, cholinesterase from 51 to 150 IU/l, and prothrombin time from 47% to 66%. PSE can be an effective alternative for the management of severe PHG with portal vein thrombosis, and it might also be effective in improving liver function.     

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。     

门静脉高压症少见成因及其诊断

门静脉高压症是指由不同原因引起的门静脉血流受阻或者血流异常增多而导致门静脉系统压力增高和广泛侧支循环形成的临床综合征,最常见于肝硬化.而非硬化性门脉高压常见于特发性门静脉高压(idiopathic portal hypertension,IPH)、胰源性门脉高压(pancreatic sinistral portal hypertension,PSPH)、门静脉海绵样变性(cavernous transformation of the portalvein,CTPV)、先天性肝纤维化(congenital hepatic fibrosis,CHF)、Budd-Chiari综合征(Budd-Chiari syndrome,BCS)、肝窦阻塞综合征(hepatic sinusoidal obstruction syndrome,SOS)、门静脉血栓形成(portal vein thrombosis,PVT)、骨髓增生性疾病(myeloproli ferative disease,MLD)、肝淀粉样变(hepatic amyloidosis)、血色病(hemochromatosis,HC)等.本文就这几种门脉高压的...     

Portal biliopathy

In patients with portal hypertension, particularly with extrahepatic portal vein obstruction, portal biliopathy producing biliary ductal and gallbladder wall abnormalities are common. Portal cavernoma formation, choledochal varices and ischemic injury of the bile duct have been implicated as causes of these morphological alterations. While a majority of the patients are asymptomatic, some present with a raised alkaline phosphatase level, abdominal pain, fever and cholangitis. Choledocholithiasis may develop as a complication and manifest as obstructive jaundice with or without cholangitis. Endoscopic sphincterotomy and stone extraction can effectively treat cholangitis when jaundice is associated with common bile duct stone(s). Definitive decompressive shunt surgery is sometimes required when biliary obstruction is recurrent and progressive.     

Portal hemodynamics in idiopathic portal hypertension (Banti's syndrome). Comparison with chronic persistent hepatitis and normal subjects

A comparative study of portal hemodynamics was made in 17 patients with idiopathic portal hypertension, 5 patients with chronic persistent hepatitis having no portal hypertension, and 21 healthy adults who served as the control for certain measurements. Venous pressures were measured by portal and hepatic vein catheterizations, blood flow by the pulsed Doppler flowmeter, organ volume by computed tomography, and intrahepatic shunt index by 99mTc-macroaggregated albumin instilled in the portal vein. The patients with idiopathic portal hypertension were divided into two groups: group A (n = 8) and group B (n = 9), consisting of those who respectively had portal venous flow per liver volume above and below the mean + 2 SD of healthy adults. In group A, portal vein pressure was moderately elevated, portal venous flow was significantly increased compared with the control, and portal vascular resistance was not much altered. In group B, portal vein pressure was markedly elevated above that of control, portal venous flow was comparable, and portal vascular resistance was significantly elevated. Splenic venous flow measured in the splenic vein between the left and short gastric veins was markedly increased in groups A and B, the increase being greater in the former. It was concluded that in some patients with idiopathic portal hypertension, increased portal venous flow, partly a result of increased splenic venous flow secondary to splenomegaly of an undetermined process, is the main contributor initially to the elevation of portal vein pressure; in others, possibly later, increased portal vascular resistance plays an important role.     

Cavernous Transformation of the Portal Vein: A Common Manifestation of Behcet's Disease

Behcet's disease (BD) is a chronic, multisystem inflammatory disorder of unknown etiology, which is characterized by recurrent aphthous ulcers of the mouth and genitalia, uveitis with hypopyon, and a diffuse vasculitis that involves the arterial and venous systems.
From January 1968 to July 1993, 66 of 844 patients with BD seen at the Hacettepe University Hospital, Ankara, Turkey, experienced a vascular complication other than peripheral thrombophlebitis. The vascular complication in each case was identified based upon a combination of clinical data, digital subtraction angiography, CT, and ultrasonography findings. Six of these 66 (9.1%) had cavernous transformation of the portal vein. Five of these six had additional large vein involvement resulting in the Budd-Chiari syndrome with or without inferior vena caval obstruction.
Based upon this experience, it can be concluded that portal vein thrombosis is not a rare complication of BD. When patients with BD are found to have or develop splenomegaly, portal vein thrombosis should be suspected and investigated. If hepatomegaly and ascites are detected, Budd-Chiari syndrome due to hepatic vein thrombosis should be suspected. Finally, if hepato-splenomegaly, ascites, and dependent edema of the lower body are present, thrombosis of the inferior vena cava should be suspected.     

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.     

Caroli disease: computed tomographic diagnosis

Computed tomographic (CT) scan findings in patients with cavernous ectasia of the biliary tract (Caroli disease) have been described. Our patient had the isolated form of Caroli disease characterized by saccular dilatation of intrahepatic bile ducts and no evidence of portal hypertension. CT scan of the liver showed tiny dots with strong contrast enhancement within dilated intrahepatic bile ducts (the central dot sign). This finding when correlated with sonogram indicated portal radicles surrounded by dilated intrahepatic bile ducts.