Kurdistan Boar GEH J Club: PVT From Saudi J Gastroenterology.

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  1. 1. Kurdistab Board GEH J Club: Dr. Mohamed Alshekhani Professor in Medicine. MBChB-CABM-FRCP-EBGH. 2015 From Saudi J Gastroenterology 2015. 1
  2. 2. Introduction: (NCPH), a heterogeneous group due to intrahepatic or extrahepatic etiologies. The lesions are vascular in nature classified based on the site of resistance to blood flow as prehepatic, hepatic, &posthepatic. The hepatic causes of NCPH can be subdivided into presinusoidal, sinusoidal, &postsinusoidal PVT was first seen in the late 1860s in a patient with splenomegaly, ascites&variceal dilatation,termed cavernoma to describe spongy appearance of portal vein (PV). Generally a hypercoagulable state, intra-abdominal infection/peritonitis&PV anomaly (PV stenosis &atresia) are considered important predisposing factors of EHPVO; but thr vast majority are due to primary thrombosis of the PV, often with more than one cause. 2
  3. 3. Introduction: 0.05- 0.5% population prevalence. On autopsy 1%. PVT is responsible for 5%10% of PHT., 40% in developing Cs. In children,80%. Incidence of PVT among liver cirrhotics 0.6-64.1%. After cirrhosis, EHPVO is the most common cause of portal hypertension globally. In the India 2030% of all variceal bleeds are due to EHPVO. In Japan, 1020% of variceal bleeds& in the west, 25% of variceal bleeds are due to EHPVO. 3
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  5. 5. PVT: Clinical presentation Different in acute &chronic thrombosis. This depends on development&extent of collateral circulation. Acute PVT: Intestinal congestion&ischemia with abdominal pain, fever, diarrhea, rectal bleeding, distension, sepsis&lactic acidosis with or without splenomegaly are common. Chronic PVT: Can be asymptomatic or could be characterized by splenomegaly, pancytopenia, varices&rarely ascites. 5
  6. 6. PVT: Pathophysiology PV obstruction is usually well tolerated &often asymptomatic. 2 important mechanisms play a role in Pvobstruction: Arterial vasodilationor rescue, can preserve the liver function in acute settings&allows a second mechanism to operate the venous rescue. Venous rescue allows several collaterals to develop, which try to bypass portal vein obstruction, takes around 46 weeks& obstructed portal vein is replaced by collateral network called cavernoma.,bypasses the obstructed portal vein &a thrombosed portal vein turns into a fibrotic cord. The network is seen around structures near the obstructed PV as bile duct, GB, pancreas, gastric antrum & duodenum. The bile duct may be difficult to locate within the network of collaterals on abdominal ultrasonography. 6
  7. 7. PVT: Pathophysiology Biopsy of liver is usually normal except hemosiderosis related to porto systemic shunt. In much advanced states of PVT, hypoperfused cells of the liver die by apoptosis as a result of increased apoptotic signals & enhanced mitotic activity in normally perfused cells,finally leads to reduced synthetic function of the liver in later stages of EHPVO. 7
  8. 8. PVT: Epidemiology Most with PVT are cirrhotics with primary or metastatic cancer. Nontumoral /noncirrhotic PVT is the second most frequent cause of portal hypertension, worldwide,510% in the western world, 40% in the India. Acute PVT: 1025% of all PVTs Chronic PVT/EHPVO: 7590% of all PVTs Overall EHPVO: 510% of portal hypertension In developing countries EHPVO: 3540% of portal HT. In India: 2030% of all variceal bleeds are due to PVT In Japan: 1020% of all variceal bleeds are due to PVT In the West: 25% of all variceal bleeds are due to PVT In children, 70% of all variceal bleeds are due to EHPVO from the India. 8
  9. 9. PVT: Epidemiology A prevalence of 0.626% of PVT in cirrhotics& highest in orthoptic liver transplants. A 6.5% PVT is seen in patients with hepatocellular carcinoma (HCC) at the time of diagnosis, which increases in later stages of HCC. The etiology of liver disease has an influence on prevalence of PVT, being 3.6% in primary sclerosing cholangitis (PSC), 8% in primary biliary cirrhosis (PBC), 16% each in acute liver disease (ALD), hepatitis B virus (HBV)-related cirrhosis& 35% in HCC. The risk of PVT is independently associated with severity of cirrhosis. Cirrhosis listed for liver transplantation, 7.4- 16% of PVT reported in with 12 months prospective follow up. 9
  10. 10. PVT: Etiopathogenesis Thrombophilic conditions are seen in 60% of PVT, while local predisposing factors account for 30%. Usually there is > 1 factor responsible for PVT. Idiopathic 30% of PVT.
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  12. 12. PVT:Ethiopathogesis Factor V Leiden mutation is the most common thrombophilia predisposing factor to PVT followed by PC deficiency. The role of protein S &antithrombin (AT) III deficiency has not yet been confirmed A simple method of screening deficiency of natural anticoagulants in patients with liver disease comprises of the ratio of PS or PC or AT to [(Factor II + Factor X/2)],If 3 days Catheter misplacement Trauma on catheter insertion site Type of solution infused. Abdominal sepsis has been identified as a risk factor in 11% of PVT with strong association between Bacteroides fragilis infection with PVT The transient development of anticardiolipin antibodies has been suggested as a pathophysiological link between this infection and PVT.
  13. 14. Ethiopathogesis:MPD Among the thrombophilic states, primary MPD are more common , frequently occult MPD. 50% of presumed idiopathic PVT suffered from primary MPD,not detected from conventional testing but by spontaneous formation of erythroid colonies in bone marrow culture. An acquired mutation (JAK 2/V617F) testing associated with MPD yields a higher rate of diagnosis, presently considered among the major criteria. Individuals with occult MPD are very often younger than those with full blown MPD who conversely have a relatively low incidence of splanchnic vein thrombosis in their postdiagnosis follow up. These findings reflect that MPD presenting as splanchnic thrombosis has often atypical phenotype, that is, juvenile disorder with high thrombotic risk &typical MPD is less likely to develop in these patients. The diagnosis of occult MPD is not straight forward,requires endogenous erythroid colony assessment. Endogenous erythroid colony assessment (EEC) is spontaneous growth of erythroid colonies in cultures of bone marrow in absence of added erythropoietin, demonstrated in up to 78% of patients of BuddChiari syndrome & 48% of patients with EHPVO.
  14. 15. Ethiopathogesis:MPD Bone marrow morphology is currently included in WHO criteria of MPD &clusters of enlarged, mature, pleiomorphic megakaryocytes is considered to be a diagnostic hallmark of Philadelphia negative MPD. By using bone marrow, underlying MPD demonstrated in 30% of EHPVO. Molecular markers of clonal disease are useful in the diagnostic workup of MPD such as JAK2 (V617F), rare ( in adults with long-standing disease &declining LFs. function. Jaundice result from BD compression because of dilated venous collaterals due to portal biliopathy. Portal biliopathy(abns of extr& intrahepatic BDs with or without abns of the GB wall,includes indentation of paracholedochal collaterals on bile ducts, strictures,angulations, focal narrowing, stones, irregular walls. GB varices are common,but GB contractibility remains intact. Frequency of cholelithiasis is higher in EHPVO. Biliopathy seen in 90100% ; but only few are symptomatic , usually in adult s& reflects advance disease&complicated by GSs, CBD stones, cholangitis, secondary biliary cirrhosis& hemobilia.
  15. 19. Clinical features: Clinical features can be summarized: Recent PVT Asymptomatic Symptomatic Severe nonalcoholic abdominal pain, distention, fever, systemic inflammatory (SIRS) Persistent pain, ascites; ileus should raise suspicion of intestinal infarction Portal pylephlebitis should be suspected if spiky fevers, tenderness, shock, and sepsis-related cholestasis is seen Associated thrombosis in other regions to be investigated.
  16. 20. Clinical features: Clinical features can be summarized: Chronic PVT/EHPVO With portal hypertension Variceal bleed well tolerated Splenomegaly moderate Hypersplenism. Growth retardation in children Jaundice, biliopathy, mild hepatic dysfunction.
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  18. 22. EHPVO DIAGNOSIS: Liver function test (LFT): Normal Endoscopy: Esophageal varices, gastric varices, anorectal varices Doppler: PVT & portal vein cavernoma CECT &CT :angiocollaterals Liver biopsy: Normal but not mandatory. EHPVO imaging characteristic&pattern of obstruction: Color Doppler ultrasound Recent: No color flow or Doppler signal within portal vein, distention of portal vein, absence of cavernoma. Contrast EUS is useful to confirm portal vein thrombosis Chronic: No color flow in portal vein and hepatopetal signal within the cavernoma or varices at gall bladder wall &signs of portal hypertension. 22
  19. 23. EHPVO DIAGNOSIS: Contrast-enhanced CT/MR Recent Nonenhancing material within portal vein & increased hepatic enhancement in arterial phase. Enhancement of thrombus suggests malignant thrombus. CT/MR angiography are more useful. Chronic Cavernomatus transformation of portal vein with splenomegaly, collaterals, and/or no opacification of intrahepatic portal vein. Chronic venous thrombus can manifest as linear areas of calcification within thrombus. Rim enhancement of vessel wall may also be seen &presumed to be due to normal flow in vasa vasorum. Care must be taken to avoid confusion between pseudo thrombus image with true portal vein thrombus. Pseudothrombus appearance occurs during HAP in main portal vein lumen due to mixed flow from enhanced splenic vein re