CHARACTERISTIC INTRADUCTAL ULTRASONOGRAPHIC FEATURES OF PORTAL BILIOPATHY

The term ‘portal biliopathy’ is used to describe cholangiographic abnormalities seen in patients with extrahepatic portal vein obstruction. Portal biliopathy is mainly composed of extrinsic compression of the bile duct caused by enlarged venous collaterals. Herein we report a case of asymptomatic portal biliopathy caused by idiopathic extrahepatic portal vein obstruction. In the present case, intraductal ultrasonography showed normal anatomic layers of the distal common bile duct wall, surrounded by numerous tubular structures which were suspected to be collateral vessels. We suggest that intraductal ultrasonography may be a helpful imaging procedure for detection of this pathological condition.     

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 biliopathy

Biliary ductal changes are a common radiological finding in patients with portal hypertension, however only a small percentage of patients (5%-30%) develop symptomatic bile duct obstruction. The exact pathogenesis is not clear, but an involvement of factors such as bile duct compression by venous collaterals, ischemia, and infection is accepted by most authors. Although endoscopic retrograde cholangiopancreatography was used to define and diagnose this condition, magnetic resonance cholangiopancreatography is currently the investigation of choice for diagnosing this condition. Treatment is indicated only for symptomatic cases. Portosystemic shunts are the treatment of choice for symptomatic portal biliopathy. In the majority of patients, the changes caused by biliopathy resolve after shunt surgery, however, 15%-20% patients require a subsequent bilio-enteric bypass or endoscopic management for persistent biliopathy. There is a role for endoscopic therapy in patients with bile duct stones, cholangitis or when portosystemic shunt surgery is not feasible.     

Thromboelastographic evaluation of coagulation in patients with extrahepatic portal vein thrombosis and non‐cirrhotic portal fibrosis: A pilot study

Background and Aims: Non‐cirrhotic portal hypertension due to extrahepatic portal vein thrombosis (EHO) and non‐cirrhotic portal fibrosis (NCPF) is a major cause of upper gastrointestinal hemorrhage in India. Hypercoagulability has been proposed to explain the thrombosis in the portal vein in EHO and intrahepatic portal vein radicals in NCPF. However, some authors have reported hypocoagulability in these patients. Thromboelastography (TEG), which gives a dynamic assessment of coagulation, has potential for evaluating coagulation in these patients but has not been used so far. It was the aim of this study to evaluate the coagulation status in patients of EHO and NCPF using TEG. Methods: Thirty patients with EHO and 19 patients with NCPF were studied. TEG was done in all patients. R (reaction time), K (constant), α (angle), MA (maximal amplitude), A 60 (width of tracing after 60 min) were recorded and TEG index calculated and compared to controls. Results: Seven patients (23.3%) in the EHO group and eight (42.1%) in the NCPF group had severe thrombocytopenia (platlets, < 50 000 cells/dL). TEG showed significantly shorter R, higher MA and larger A in both EHO and NCPF compared to controls (P < 0.01). Thrombocytopenia normalized A but R continued to be significantly shorter in EHO and NCPF. Overt hypercoagulability (TEG index, > +2.5) was seen in two patients with EHO and one patient with NCPF. Conclusion: A latent hypercoagulable state exists in patients with EHO and NCPF which is partially masked by the thrombocytopenia secondary to splenomegaly and hypersplenism.     

Portal biliopathy

Portal biliopathy refers to cholangiographic abnormalities which occur in patients with portal cavernoma. These changes occur as a result of pressure on bile ducts from bridging tortuous paracholedochal, epicholedochal and cholecystic veins. Bile duct ischemia may occur due prolonged venous pressure effect or result from insufficient blood supply. In addition, encasement of ducts may occur due fibrotic cavernoma. Majority of patients are asymptomatic. Portal biliopathy is a progressive disease and patients who have long standing disease and more severe bile duct abnormalities present with recurrent episodes of biliary pain, cholangitis and cholestasis. Serum chemistry, ultrasound with color Doppler imaging, magnetic resonance imaging with magnetic resonance cholangiopancreatography and magnetic resonance portovenography are modalities of choice for evaluation of portal biliopathy. Endoscopic retrograde cholangiography being an invasive procedure is indicated for endotherapy only. Management of portal biliopathy is done in a stepwise manner. First, endotherapy is done for dilation of biliary strictures, placement of biliary stents to facilitate drainage and removal of bile duct calculi. Next portal venous pressure is reduced by formation of surgical porto-systemic shunt or transjugular intrahepatic portosystemic shunt. This causes significant resolution of biliary changes. Patients who persist with biliary symptoms and bile duct changes may benefit from surgical biliary drainage procedures (hepaticojejunostomy or choledechoduodenostomy).     

Obliterative portal venopathy: A comparative study of 184 cases of extrahepatic portal obstruction and 469 cases of idiopathic portal hypertension

Abstract A total of 184 cases of extrahepatic portal obstruction (EHPO), mostly demonstrated by intraoperative portography and studied at 17 institutes during the period 1957–1983, were compared with 469 cases of idiopathic portal hypertension (IPH) similarly studied. Of the cases of EHPO, there were 101 males and 83 females; 93 were under 20 years of age and the average age was 25.9 years (i.e. much younger than that of IPH cases). There were two age peaks, one before age 19 years and the other at age 40–49 years. One out of three adult cases had a history of abdominal surgery, but otherwise the aetiologic factor was difficult to elicit. Bleeding was the initial symptom in the majority, and splenectomy and haematological findings of hypersplenism were less pronounced compared with IPH. Liver function tests were almost always normal. The liver appeared normal macroscopically in 69% and histologically in 35%. The changes seen in the remainder were similar to those in IPH; they were less frequent in young patients than in cases above age 20 years. Compared with IPH, the wedged hepatic venous pressure in patients with EHPO was lower and the gradient from the portal venous pressure was greater. It is concluded that extrahepatic portal obstruction is less common compared with IPH in Japan, and that there are cases particularly among adults that present clinicopathological features very similar to those of IPH. It is unclear at present whether these two disorders represent two different disease entities, or whether they represent one disorder with differences in the site of involvement along the portal vein system.     

Effect of shunt surgery on spleen size, portal pressure and oesophageal varices in patients with non-cirrhotic portal hypertension

Shunt surgery is considered to be the treatment of choice in patients with non-cirrhotic portal hypertension. There is little data on the effect of side-to-side lieno-renal (SSLR) shunt on oesophageal variceal size, splenic size and splenic pulp pressure (SPP) in patients with non-cirrhotic portal hypertension. We evaluated pre- and postoperatively endoscopic grading of varices, splenic size and SPP for predicting shunt patency in 86 patients with non-cirrhotic portal hypertension: 56 with extrahepatic portal venous obstruction (EHPVO) and 30 with non-cirrhotic portal fibrosis (NCPF). The EHPVO patients with patent shunts (n= 47) showed significant reduction in SPP (pre-operative 43.56±7.9 vs postoperative 29.96±7.7 cm of saline), splenic size (6.5±2.8 vs 4.00±2.6 cm below costal margin) and varices grades (2.96±0.5 vs 0.92±0.8). Patients with blocked shunt (n= 9) did not show significant reduction in SPP and varices grades. However, there was reduction in spleen size (8.6±3.0 vs 6.3±4.3). In the NCPF group, 28 had patent shunts and showed significant reduction in SPP (46.3±13.5 vs 33.8±7.6 cm of saline), splenic size (9.1±3.3 vs 6.8±4.6 cm below costal margin) and varices grades (2.8±0.7 vs 1.05±0.96). As only two patients with NCPF had blocked shunts, no statistical comparison between patients with patent and patients with blocked shunts could be done. In conclusion, following SSLR, there is a significant reduction in SPP and varices grades in patients with patent shunts. Endoscopic grading of varices can be used to predict shunt patency. However, spleen size is not a good criteria for predicting shunt patency.     

Endoscopic treatment of extrahepatic bile duct strictures in patients with portal biliopathy carries a high risk of haemobilia: report of 3 cases

Extrahepatic portal venous obstruction can be associated with bile duct abnormalities, the entity being called portal biliopathy. Three cases are reported of extrahepatic bile duct strictures in patients with portal biliopathy who developed haemobilia during endotherapy. Although endoscopic therapy with stent placement can be successful in patients with portal biliopathy and could also lead to permanent stricture resolution, procedure-related haemobilia is not as uncommon as previously held. Shunt surgery could be a better option in fit patients, since it could provide definitive treatment in a young patient with an otherwise normal life expectancy.     

Extrahepatic portal venous obstruction and obstructive jaundice: approach to management

BACKGROUND: Patients with long-standing extrahepatic portal venous obstruction (EHPVO) develop extensive collaterals in the hepatoduodenal ligament as a result of enlargement of the periportal veins. These patients are also prone to develop obstructive jaundice as a result of strictures and/or choledocholithiasis. Surgical management of obstructive jaundice in such patients becomes difficult in the presence of these collaterals. AIM: To review the approach to management of patients with EHPVO and obstructive jaundice. METHODS: Retrospective review of patients with EHPVO and obstructive jaundice requiring surgical and/or endoscopic management between 1992 and 2002. RESULTS: Thirteen patients (nine males, aged 12-50 years) with EHPVO and obstructive jaundice were evaluated. No patient had underlying cirrhosis or hepatocellular carcinoma. Five patients (group A) had biliary stricture; three (group B) had choledocholithiasis; and five (group C) had biliary stricture with choledocholithiasis. Primary surgical management was performed in group A (portosystemic shunt in four-strictures resolved in three; hepaticojejunostomy in one). In group B (n = 3) endoscopic stone extraction was successful in two patients. One patient underwent staged procedure (portosystemic shunt followed by biliary surgery). In group C, initial endoscopic management failed in four patients in whom it was attempted. All five patients thereafter underwent surgery (staged procedure, one; choledochoduodenostomy, one; devascularization, one; abandoned, two). Repeat postoperative endoscopic management was successful in two of the group C patients. Overall (group B and C), massive intraoperative hemorrhage occurred in three patients (one died). Postoperative hemorrhage occurred in one patient. CONCLUSION: In patients with EHPVO and obstructive jaundice, primary biliary tract surgery has significant morbidity and mortality. Endoscopic management should be the preferred modality. In patients with endoscopic failure, a staged procedure (portosystemic shunt followed by biliary surgery) should be preferred. Strictures alone may resolve after a portosystemic shunt. Endoscopic stenting may be required as an adjunct.     

呼吸对门脉系统血管内径的影响与门脉高压症相关性的临床研究

研究呼吸对门脉系统血管内径的与门脉高压症的相关性。采用Ｂ型超声探测了伴有门脉高压症的肝炎肝硬化病人的门静脉内径,分别记录在吸气末及呼气末两种状态下的测值,并与对照组进行比较分析,在对照组,当深吸气时,胸廓扩张,回心血量增加,同时门脉血流亦增加,门静脉及脾静脉内径增宽,深呼气时正相反,但是,当门脉内压力增高后,呼吸对 门脉内血流及内径的影响明显减弱。通过Ｂ型超声观察呼吸对门脉血管内径的影响程度对于门     

Treatment of a patient with obstructive jaundice and extrahepatic portal hypertension

BACKGROUND:Recurrence of inflammation in the extrahepatic bile duct can lead to bile duct stenosis, obstructive jaundice and cavernous transformation of the portal vein.The latter can develop into extrahepatic portal hypertension(PHT).It is difficult to establish the correct method for treating these conditions. METHODS:At another hospital,a 51-year-old man developed PHT as a result of endoscopic retrograde cholangiopancreatography and endoscopic nasobiliary drainage to relieve cholelithiasis and obstructiv...     

Extrahepatic portal vein thrombosis: aetiology and determinants of survival

BACKGROUND: Malignancy, hypercoagulability, and conditions leading to decreased portal flow have been reported to contribute to the aetiology of extrahepatic portal vein thrombosis (EPVT). Mortality of patients with EPVT may be associated with these concurrent medical conditions or with manifestations of portal hypertension, such as variceal haemorrhage. PATIENTS AND METHODS: To determine which variables have prognostic significance with respect to survival, we performed a retrospective study of 172 adult EPVT patients who were followed over the period 1984-1997 in eight university hospitals. RESULTS: Mean follow up was 3.9 years (range 0.1-13.1). Overall survival was 70% (95% confidence interval (CI) 62-76%) at one year, 61% (95% CI, 52-67%) at five years, and 54% (95% CI, 45-62%) at 10 years. The one, five, and 10 year survival rates in the absence of cancer, cirrhosis, and mesenteric vein thrombosis were 95% (95% CI 87-98%), 89% (95% CI 78-94%), and 81% (95% CI 67-89%), respectively (n=83). Variables at diagnosis associated with reduced survival according to multivariate analysis were advanced age, malignancy, cirrhosis, mesenteric vein thrombosis, absence of abdominal inflammation, and serum levels of aminotransferase and albumin. The presence of variceal haemorrhage and myeloproliferative disorders did not influence survival. Only four patients died due to variceal haemorrhage and one due to complications of a portosystemic shunt procedure. CONCLUSION: We conclude that mortality among patients with EPVT is related primarily to concurrent disorders leading to EPVT and not to complications of portal hypertension.     

Extrahepatic portal vein obstruction in adults detected by ultrasound with frequent lack of portal hypertension signs

Extrahepatic portal vein obstruction (EHPO) was seen in 54 adult patients at the Chiba University Hospital and affiliated hospitals from 1978 to 1991. They were classified according to the background disease (Group A, unknown aetiology; Group B, benign disease; Group C, malignant disease). Among the initial symptoms and signs, abdominal pain was the most frequent in Group A (37%), and symptoms attributable to the primary disease in Groups B (44%) and C (75%). Definite or probable diagnosis was made in 45 of the 54 patients (81.8%) by ultrasound (US) examination carried out because of these symptoms and signs. Signs of portal hypertension were observed in 67% of patients; oesophageal varices were seen in 60%. Extrahepatic portal vein obstruction without portal hypertension signs was characterized by thick extensive hepatopetal collaterals or patency of some intrahepatic portal veins. Extrahepatic portal vein obstruction patients without portal hypertension remained free of its signs for more than 3 years of follow up and, in fact, EHPO without portal hypertension signs was a common occurrence. Emphasis is made on the diagnostic value of US examination which was useful in identifying the relation of clinical manifestation of EHPO to pathophysiology, and on the frequent lack of portal hypertension signs in this disease.     

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...     

Non-cirrhotic portal fibrosis (idiopathic portal hypertension): experience with 151 patients and a review of the literature

BACKGROUND: Non-cirrhotic portal fibrosis (NCPF), the equivalent of idiopathic portal hypertension in Japan and hepatoportal sclerosis in the United States of America, is a common cause of portal hypertension in India. The clinical features, portographic and histological findings, and management of 151 patients with non-cirrhotic portal fibrosis are presented. METHODS: The disease is diagnosed by the presence of unequivocal evidence of portal hypertension in the definite absence of liver cirrhosis and extrahepatic portal vein obstruction (EHPVO). Retrospective analysis of records of 151 patients with NCPF was analyzed for the clinical presentation, physical findings, laboratory tests, radiological and histological findings, and for the outcome of treatment. RESULTS: The disease is characterized by massive splenomegaly with anemia, preserved liver function and benign prognosis in a majority of patients. Splenoportovenography (SPV) showed massive dilatation of the portal and splenic veins, and the presence of collaterals. Twenty-four (15.9%) patients showed evidence of natural/spontaneous shunts (splenorenal 15, umbilical nine) on SPV; these patients had a lower incidence of variceal bleeding. Liver histology demonstrated maintained lobular architecture, portal fibrosis of variable degree, sclerosis and obliteration of small-sized portal vein radicles, and subcapsular scarring with the collapse of the underlying parenchyma. Piecemeal or hepatocytic necrosis was absent in all histology specimens. Three patients showed nodular transformation along with abnormal liver functions, and may represent late manifestation of NCPF where features are similar to those seen in patients with incomplete septal cirrhosis. In the initial part of the study, surgery (side-to-side lieno-renal shunt) was the preferred modality of treatment, however, endoscopic sclerotherapy or variceal ligation has now become the preferred first line of management of variceal bleeding. CONCLUSIONS: The epidemiological and clinical features of NCPF have more similarity to IPH than has previously been documented. The development of spontaneous shunts tends to protect these patients from variceal bleeding.     

Portal hypertensive biliopathy: A single center experience and literature review

Portal hypertensive biliopathy (PHB) is characterized by anatomical and functional abnormalities of the intrahepatic, extrahepatic and pancreatic ducts, in patients with portal hypertension associated to extrahepatic portal vein obstruction and less frequently to cirrhosis. These morphological changes, consisting in dilatation and stenosis of the biliary tree, are due to extensive venous collaterals occurring in an attempt to decompress the portal venous blockage. It is usually asymptomatic until it progresses to more advanced stages with cholestasis, jaundice, biliary sludge, gallstones, cholangitis and finally biliary cirrhosis. Imaging modalities of the biliary tree such as Doppler ultrasound, computed tomography, magnetic resonance cholangiopancreatography and endoscopic retrograde cholangiopancreatography are essential to establish the diagnosis and the need of therapeutical interventions. Once the diagnosis is established, treatment with ursodesoxycholic acid seems to be beneficial. Decompression of the biliary tree to dilate, remove stones or implant biliary prosthesis by endoscopic or surgical procedures (hepato-yeyunostomy) usually resolves the cholestatic picture and prevents septic complications. The ideal treatment is the decompression of the portal system, with transjugular intrahepatic porto-systemic shunt or a surgical porto-systemic shunt. Unfortunately, few patients will be candidates for these procedures due to the extension of the thrombotic process. The purpose of this paper is to report the first 3 cases of PHB seen in a Colombian center and to review the literature.