Liver portal vein thrombosis

Hepatic venous thrombosis, also known as Budd-Chiari syndrome, is caused by hypercoagulable disorders precipitated by pregnancy, infection, and birth control medication. An acute painful abdomen, sudden enlargement of the liver, and the presence of ascites make up a triad of clinical symptoms that are important in the diagnosis of this syndrome. Myeloproliferative disorders such as polycythemia vera and paroxysmal nocturnal dyspnea were previously thought to be responsible. Factor V Leiden and prothrombin 20210 mutations are also known to be responsible, Other intraabdominal thromboses include portal vein thrombosis, mesenteric vein thrombosis and renal vein thrombosis. 

Portal vein thrombosis (see chapter 39.3.3) with gradual or incomplete obstruction merely produces anastomoses and develops asymptomatically. After complete obstruction, ultrasonography will showparaportal, angiomatous anastomoses, predominantly in the porta hepatis (= cavernous transformation). (95) Splenomegaly is evidenced with a normal-sized liver. The portal vein is not detectable. Colour Doppler sonography has become of paramount importance in portal vein system diagnostics. 

Tc-DTPA Arterial perfusion accounts for 20%-40% of the circulation in portal hypertension, cirrhosis causes arterial perfusion to increase to over 60%. In portal vein thrombosis, only an arterial curve is visible. Liver metastasis usually displays relatively high arterial perfusion. In (rare) occlusions of the hepatic artery, only a portal venous curve is visible. When a bolus injection of 400 MBq "Tc-diethylenetriamine pentaacetic acid (DTPA) is applied, scintigraphy is able to reveal a bi-phasic time-activity curve. The initial increase of activity is produced by the arterial influence and the second peak by the portal venous inflow. Both curves can be evaluated quantitatively. (36) Perfusion scintigraphy may be useful in the case of liver trauma, TIPS, hyper-vascularized hepatic tumours and partial liver resection as well as after liver transplantation. 

Liver cirrhosis (37), liver tumours, liver echinococcosis, portal vein thrombosis, thrombosis of the splenic vein, right heart failure, Budd-Chiari syndrome, peliosis hepatis (39), etc. 

It is not clearly understood why in some cases oedema without ascites and in other cases ascites without oedema as well as ascites together with oedema or even pleural effusion without ascites occur. Ascites develops most frequently during the course of liver disease (= hepatogenic ascites), in particular in chronic liver diseases with portal hypertension (= portal ascites), (s. tab. 16.7) Various mechanical, biochemical and neural disorders overlap in their effects and pathways, depending on the underlying liver disease. Only rarely is ascites found in diseases with presinusoidal localization of portal hypertension (such as portal vein thrombosis) or with minor restrictions in the synthesis of albumin (as in biliary cirrhosis). Formation of ascites occurs in about 50% of all cirrhotic patients within 10 years of... 

Complications The following complications have been reported (i.) cholangitis, (2.) obstructive jaundice, (i.) intrahepatic cholelithiasis, (4.) sepsis, (J.) portal hypertension (oesophageal varices, portal vein thrombosis, chronic Budd-Chiari syndrome, etc.), (6.) thrombosis of the inferior vena cava, (7.) amyloidosis, (8.) immune complex-associated glomerulonephritis, (9.) metastases, (10.) acute on chronic liver insufficiency or acute liver failure, and (11.) bronchobiliary fistula. 

Okuda, K., Ohnishi, K., Kimura, K., Matsutani, S., Sumida, M., Goto, N., Musha, H., Takashi, M., Suzuki, N., Shinagawa, T., Suzukui, N., Ohtsuki, T., Arakawa, M., Nakashima, T. Incidence of portal vein thrombosis in liver cirrhosis. An angiographic study in 708 patients. Gastroenterology 1985 89 279-286... 

Another alternative HCC staging system is the so-called CLIP score (35, 36). This also combines morphological criteria of the HCC with liver functions (Child-Pugh) and, additionally, with portal vein thrombosis as well as AFP. (s. tab. 37.8) BCLC classification can be recom-... 

Complications Acute liver failure, arterioportal fistula formation, oesophageal varices (15) and pulmonary hypertension have been reported as complications. In most cases, the cause of death is anorexia with tumour cachexia, accompanied by signs of circulatory and renal failure. Occasionally, there is intraperitoneal haemorrhage, portal vein thrombosis (138,146) and tumour rupture with formation of haemorrhagic ascites. (121)... 

Liver involvement in Osier-Vaquez disease is rare or not detectable at all. There is, however, evidence of hepato-splenomegaly due to extramedullary haemopoiesis. Of importance here is the association with Budd-Chiari syndrome and veno-occlusive disease. Polycythaemia vera should be considered in cases of aetiologically unclarified portal vein thrombosis. 

Thromboses in the vessels of the portal system constitute the most common cause of prehepatic portal hypertension. Even in the postpartal phase, portal vein thrombosis may occur due to the obliteration of the umbilical vein spreading to the portal vein or due to an infection of the umbilical vein with subsequent pylephlebitis. In the adult, this clinical picture is most commonly observed in liver diseases that cause the portal blood flow to slow down (or even reverse). All diseases which are accompanied by hypercoagulopathies also have a strong tendency to cause portal vein thrombosis. (106, 110, 112, 119, 121) Patients with liver cell carcinoma due to cirrhosis quite often develop thrombosis. Septic processes (such as appendicitis, diverticulitis, colitis) are a further common cause, particularly in immuno-... 

Yerdel, M.A., Gunson, B., Mirza, D., Karayalcin, E., Olliff, S., Buckels, J., Mayer, D., McMaster, R, I enne, J. Portal vein thrombosis in adults undergoing liver transplantation. Risk factors, screening, management, and outcome. Transplantation 2000 69 1873—1881... 

Previous shunt operations and TIPS need to be removed in order to guarantee that the transplanted liver is sufficiently supplied with portovenous blood. In these cases, the portal system is checked preoperatively for thromboses by means of colour-encoded duplex sonography and X-ray techniques. In any case, the confluence of superior mesenteric vein and splenic vein must be free. (391) The main advantage of portacaval end-to-side anastomosis is its low thrombosis rate of < 5% in addition, there is no need for a distal shunt ligature. In shunts distal to the hilus (mesocaval, distal splenorenal), no preparation of the liver hilus is required however, in 10% of cases, these shunts show portal vein thrombosis (in TIPS, up to 15%). Usually, all surgical shunts are disconnected or ligated before the liver transplantation is completed in order to... 

Hg, Portal hypertension occurs when there is obstruction to portal flow anywhere along its course. The causes of obstruction leading to portal hypertension are classified by site (1) presinusoidal, (2) sinusoidal, and (3) postsinusoidal. Pre-sinusoidal portal hypertension is most commonly caused by portal vein thrombosis or schistosomiasis but may also occur with increased portal flow, such as occurs with Felty syndrome (a combination of chronic rheumatoid arthritis, splenomegaly, leukopenia, pigmented spots on the lower extremities, and sometimes other evidence of hyper-splenism, such as anemia and thrombocytopenia). Sinusoidal hypertension is most commonly caused by cirrhosis but may occur transiently with acute and chronic hepatitis or acute fatty liver. The most important cause of postsinusoidal hypertension is hepatic vein occlusion or Budd-Chiari syndrome, in which sudden obstruction or occlusion of the... 

There are several contraindications for TIPS absolute contraindications consist of right-sided heart failure with elevated central venous pressure, polycystic liver disease, and severe hepatic failure (Shiffman et al. 1995). The latter contraindication is based on the fact that the TIPS shunts blood away from the liver, thus further compromising liver function. Relative contraindications consist of active or systemic infection, as TIPS makes use of a foreign device that could act as a colonization site for bacteria, severe hepatic encephalopathy poorly controlled by medical therapy and portal vein thrombosis. 

Two devices are commercially available. Thera-Sphere (glass microsphere MDS Nordion, Kana-ta, Canada) was approved in 1999 by the Food and Drug Administration (FDA) under a Humanitarian Device Exemption (HDE) for the treatment of unresectable hepatocellular carcinoma (HCC) in patients who can have appropriately positioned hepatic arterial catheters with or without portal vein thrombosis [1]. SIR-Spheres (resin microsphere Sirtex Medical, Lane Cove, Australia) were granted full pre-marketing approval in 2002 by the FDA for the treatment of colorectal metastases in conjunction with intra-hepatic FUDR [2]. Both devices have European approval for liver neoplasia and approvals in various Asian countries. 

A 58-year-old man had bleeding gastric varices injected with a 50/50 mixture of enbucrilate and lipiodol, but a follow-up X-ray showed radiopaque material in the left lobe of the liver and a CT scan showed cyanoacrylate embolization via the right gastric vein into the main and left portal veins [32 ]. A CT scan 2 years later showed atrophy of the left-lateral segment of the liver, with portal-vein thrombosis and little residual lipiodol retention. 

Pentecost MJ, Daniels JR, Teitelbaum GP, Stanley P (1993) Hepatic chemoembolization safety with portal vein thrombosis. J Vase Interv Radiol 4 347-351 Popov I, Lavrnic S, Jelic S, Jezdic S, Jasovic A (2002) Chemoembolization for liver metastases from colorectal carcinoma risk or a benefit. Neoplasma 49 43-48 Ramsey DE, Kernagis LY, Soulen MC, Geschwind JF (2002) Chemoembolization of hepatocellular carcinoma. J Vase Interv Radiol 13 S211-221... 

The procedure is indicated for histologically confirmed inoperable primary HCC (also with portal vein thrombosis) and where are no possibilities for first-line treatment first-line treatment has failed. To date there have been no recommendations for its use in therapy of liver metastases. 

The liver receives a dual blood supply fromboth the hepatic arteries and the portal vein. Although good collateral flow exists, necrosis can occur if enough arterial supply is occluded at the time of embolization. Therefore, it is wise to assess the direction of blood flow in the portal vein because hepatofugal flow may increase the risk of infarction. One should be cautious and superselective when embolizing in the presence of portal vein thrombosis. 

A patient undergoing orthotopic liver transplantation due to acute liver failure experienced consecutive hepatic and portal vein thrombosis and progressive graft failure due to HIT type II, representing a rare case of HIT-associated early liver graft failure [19 ]. 

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