Seven patients demonstrating the difficulties in diagnosis and management of hepatic vein occlusion are presented. [ncbi.nlm.nih.gov]

The syndrome classically presents with abdominal pain, fluid in the abdomen (ascites) and liver enlargement (hepatomegaly).The syndrome can present all of a sudden or gradually. [veindirectory.org]

Thrombosis of the portal vein is present in 2% of patients. [encyclopedia.lubopitko-bg.com]

Patients with PVT need to know generally about risk factors, clinical presentation, complications and treatment options. [curetoday.com]

[…] is a complex disease with a wide spectrum of aetiologies and presentations. [latunisiemedicale.com]

• Splenomegaly

  Physical examination was remarkable for splenomegaly and mild right upper quadrant tenderness. [members.sirweb.org]

  Ultrasound acute hepatomegaly splenomegaly heterogeneous echotexture chronic hypertrophied caudate lobe peripheral atrophy of affected regions regenerative nodules : mostly corresponding to focal nodular hyperplasia -like lesions and therefore sharing [radiopaedia.org]

  This increased pressure (called portal hypertension ) causes the spleen to enlarge (splenomegaly). It also results in dilated, twisted (varicose) veins in the esophagus (called esophageal varices ) and often in the stomach (called gastric varices). [merckmanuals.com]

  Other causes Increased hepatic blood flow: Increased splenic blood flow - eg, massive splenomegaly. Hepatoportal arteriovenous fistula. Idiopathic (a diagnosis of exclusion). Left-sided (sinistral) portal hypertension Rare. [patient.info]

  • Chronic: This presentation is the result of chronic portal hypertension with or without cirrhosis; ascites, esophageal varices, splenomegaly, coagulopathy, and encephalopathy. [encyclopedia.lubopitko-bg.com]

• Abdominal Pain

  All of them had abdominal pain, abdominal distention secondary to ascites, and massive hepatomegaly. [ncbi.nlm.nih.gov]

  • Acute: Abdominal pain, hepatomegaly, and progressive liver dysfunction. [encyclopedia.lubopitko-bg.com]

  pain Jaundice CT Hepatomegaly and ascites Non-visualization of occluded hepatic veins Also seen on MRI Patchy enhanced appearance with dynamic imaging Inversion of portal blood flow results in inside-out enhancement of liver Caudate lobe is hyperdense [learningradiology.com]

  Budd Chiari classically presents with 3 features which include liver enlargement (hepatomegaly), distended abdomen with fluid (ascites), and abdominal pain. Is the disorder always painful? No. [veindirectory.org]

• Vomiting

  Symptoms include: Abdominal swelling or stretching due to fluid in the abdomen Pain in the right upper abdomen Vomiting blood Yellowing of the skin (jaundice) Treatment varies, depending on the cause of the blockage. [medlineplus.gov]

  The main symptoms indicating the presence of the disease include swelling or stretching of the abdomen and vomiting blood. Pain in the upper part of the abdomen and yellowing of the skin also indicate the presence of the problem. [buddchiarisyndrome.org]

  People then vomit blood. The blood may also pass through the digestive tract, making stools black, tarry, and foul-smelling (called melena). [merckmanuals.com]

  Acute PVT may be marked by abdominal pain, nausea, and/or vomiting, low back pain, and fever in the setting of septic portal vein thrombus (pylephlebitis). [clinicaladvisor.com]

• Nausea

  Acute PVT, which is the sudden formation of a thrombus, can present with severe abdominal pain, ascites, diarrhea, ileus, fever or nausea. [curetoday.com]

  Acute PVT may be marked by abdominal pain, nausea, and/or vomiting, low back pain, and fever in the setting of septic portal vein thrombus (pylephlebitis). [clinicaladvisor.com]

  Additional findings that may be associated with the disorder include nausea, vomiting, and/or an abnormally large spleen (splenomegaly). The severity of the disorder varies from case to case, depending upon the site and number of affected veins. [rarediseases.org]

• Right Upper Quadrant Tenderness

  Physical examination was remarkable for splenomegaly and mild right upper quadrant tenderness. [members.sirweb.org]

• Hepatomegaly

  The syndrome may present in an acute form with upper abdominal pain, abdominal swelling, ascites and tender hepatomegaly or in a chronic form, mimicking cirrhotic ascites. [ncbi.nlm.nih.gov]

  Definition / general Venous outflow obstruction caused by occlusion of hepatic outflow Either acute thrombotic occlusion (usually fatal) or subacute / chronic occlusion with hepatomegaly, ascites, abdominal pain Essential features Thrombotic outflow obstruction [pathologyoutlines.com]

  […] of pancreas Carcinoma of kidneys Metastatic disease Blood dyscrasia Leukemia Sickle cell disease Polycythemia vera Birth control pills Pregnancy Pyrrolizidine alkaloids (senecio) found in Jamaican tea Membranous diaphragm in suprahepatic IVC Acutely Hepatomegaly [learningradiology.com]

• Jaundice

  In other words, severe jaundice is not a contraindication for TIPS in patients with BCS. [nature.com]

  Suddenly she developed jaundice high serum transaminases and mild ascites. A needle liver biopsy was performed. She died 2 weeks later in liver failure in the time before the practice of liver transplantation. [meddean.luc.edu]

  Leukemia Sickle cell disease Polycythemia vera Birth control pills Pregnancy Pyrrolizidine alkaloids (senecio) found in Jamaican tea Membranous diaphragm in suprahepatic IVC Acutely Hepatomegaly and ascites Severe symptoms including shock Abdominal pain Jaundice [learningradiology.com]

  Symptoms include: Abdominal swelling or stretching due to fluid in the abdomen Pain in the right upper abdomen Vomiting blood Yellowing of the skin (jaundice) Treatment varies, depending on the cause of the blockage. [medlineplus.gov]

  […] hepatocellular carcinoma with inferior vena cava occlusion), though 30% of cases are idiopathic Venous obliteration can lead to bridging fibrosis and eventually cirrhosis ( Hepatology 1998;27:488 ) Clinical features Symptoms include painful hepatomegaly, jaundice [pathologyoutlines.com]

• Hepatosplenomegaly

  Patients with acute and sub-acute BCS present with symptoms including digestive discomfort, abdominal distention, abdominal pain, severe jaundice, refractory ascites, hepatosplenomegaly, portal hypertension, gastrointestinal bleeding and even liver failure [nature.com]

Provides all the necessary tools to make a comprehensive diagnostic workup including data from ancillary techniques and molecular findings whenever appropriate. [books.google.com]

WORKUP Physical examination, laboratory analysis, and imaging studies LABORATORY TESTS Assessment of liver function: • Transaminases, prothrombin time, albumin, bilirubin, and platelet count Diagnostic tests: • Viral hepatitis panel, a-1-antitrypsin, [encyclopedia.lubopitko-bg.com]

During a further workup, we obtained previous outside abdominal US images taken 8 years earlier, and they showed diffuse and inhomogeneous echogenicity throughout the liver, suggesting pre-existing diffuse hemangiomatosis ( Fig. 3 ). [e-ultrasonography.org]

• Gastric Varices

  With splenic vein block, oesophageal and gastric varices may result. [patient.info]

  Complications include thrombus extension (which is linked to incomplete recanalization of the vessel), intestinal ischemia and infarction (which occurs when the clot extends to the mesenteric artery), esophageal and gastric varices (typically seen over [curetoday.com]

  It also results in dilated, twisted (varicose) veins in the esophagus (called esophageal varices ) and often in the stomach (called gastric varices). These veins can bleed profusely. Fluid accumulation in the abdomen (called ascites ) is not common. [merckmanuals.com]

  Chronic PVT most often presents with sequelae of portal hypertension, most notably, gastric and esophageal variceal bleeding, and splenomegaly. [clinicaladvisor.com]

• Enlargement of the Spleen

  Signs and symptoms The acute syndrome presents with rapidly progressive severe upper abdominal pain, yellow discoloration of the skin and whites of the eyes, liver enlargement, enlargement of the spleen, fluid accumulation within the peritoneal cavity [ipfs.io]

  Signs and symptoms [ edit ] The acute syndrome presents with rapidly progressive severe upper abdominal pain, yellow discoloration of the skin and whites of the eyes, liver enlargement, enlargement of the spleen, fluid accumulation within the peritoneal [en.wikipedia.org]

It is suggested that early side to side portacaval anastomosis is the current treatment of choice. [ncbi.nlm.nih.gov]

Aims To evaluate the clinical efficacy and safety of HV angioplasty and transjugular intrahepatic portosystemic shunt (TIPS) in the treatment of BCS with HV occlusion. [springermedizin.de]

The symptoms can be lethal without proper treatment 4. Unfortunately, no standard protocol of therapy has been established. The patients barely benefit from conservative treatment. [nature.com]

Intermittent hepatic vein balloon occlusion could potentially be a low-risk adjunctive maneuver for thermal ablation therapy in the treatment of large tumors and tumors adjacent to large vessels. [jhu.pure.elsevier.com]

What is prognosis of patients with hepatic vein thrombosis? If the individual has minimal symptoms and the liver is functioning well, the majority of patients survive for at least 10 years after the diagnosis. [veindirectory.org]

Asymptomatic forms of Budd-Chiari syndrome carry a good prognosis [23]. [latunisiemedicale.com]

Prognosis This depends on the prognosis of the underlying disease, and on the outcome of any complications such as variceal bleeding. [patient.info]

[…] affected areas Angiography (DSA) On hepatic venography: complete occlusion of hepatic vein(s) may or may not be associated with a (focal) stenosis of the intrahepatic inferior vena cava spider web appearance and intrahepatic collateral veins Treatment and prognosis [radiopaedia.org]

[…] therapy can also be used to decompress the portal circulation CHRONIC Rx • Chronic anticoagulation may be needed to maintain shunt patency or in patients with thrombotic BCS • Liver transplantation • Shunt thrombosis is a common complication DISPOSITION Prognosis [encyclopedia.lubopitko-bg.com]

Etiology varies with geography. In the U.S., BCS is more commonly associated with myeloproliferative disease, hypercoagulable states, IVC membranes, and tumors. [encyclopedia.lubopitko-bg.com]

The etiology is mixed and varied. [radiopaedia.org]

Occlusion may occur anywhere from hepatic venules to inferior vena cava Pathophysiology In addition to outflow obstruction, patients often have decreased portal perfusion with eventual compensatory increase in arterial inflow ( Hepatology 2003;37:510 ) Etiology [pathologyoutlines.com]

Pathophysiology and etiology [ ✎ edit | edit source ] The most common cause is hepatic veins thrombosis (75%) [2] in patients with primary predisposition (coagulopathies like Protein C or Protein S deficiency, Leiden mutation...) or other factors like [wikilectures.eu]

In adults, the most common causes of PVT can be divided into localized or systemic etiologies (see History – Part I below). Approximately one-fourth of cases have no identifiable cause. [clinicaladvisor.com]

[…] syndrome technically referred to the triad of painful hepatomegaly, ascites and liver dysfunction Membranous obstruction of the vena cava / obliterative hepatocavopathy likely represents recanalized thrombosis, more commonly seen in developing countries Epidemiology [pathologyoutlines.com]

EPIDEMIOLOGY & DEMOGRAPHICS BCS is a rare disorder. Etiology varies with geography. In the U.S., BCS is more commonly associated with myeloproliferative disease, hypercoagulable states, IVC membranes, and tumors. [encyclopedia.lubopitko-bg.com]

Epidemiologically the causes of primary BCS in western countries include the hepatic venous thrombosis/IVC thrombosis due to systemic disease in contrast to membranous or segmental obstruction of IVC in the Asian countries. [digestive-diseases.imedpub.com]

"Budd–Chiari syndrome in Sweden: epidemiology, clinical characteristics and survival - an 18-year experience". Liver International. 29 (2): 253–9. doi : 10.1111/j.1478-3231.2008.01838.x. [ipfs.io]

"Budd–Chiari syndrome in Sweden: epidemiology, clinical characteristics and survival - an 18-year experience". Liver International. 29 (2): 253–9. doi : 10.1111/j.1478-3231.2008.01838.x. PMID 18694401. [en.wikipedia.org]

Kunio Okuda, Jean-Pierre Benhamou Springer Science & Business Media, ٠٩‏/١١‏/٢٠١٣ - 569 من الصفحات Portal hypertension is the abnormal pathophysiologic state that develops in liver cirrhosis and certain other disorders with characteristic clinical and [books.google.com]

/ obliterative hepatocavopathy likely represents recanalized thrombosis, more commonly seen in developing countries Epidemiology Occurs in roughly 0.001% of the population Sites Occlusion may occur anywhere from hepatic venules to inferior vena cava Pathophysiology [pathologyoutlines.com]

Pathophysiology and etiology [ ✎ edit | edit source ] The most common cause is hepatic veins thrombosis (75%) [2] in patients with primary predisposition (coagulopathies like Protein C or Protein S deficiency, Leiden mutation...) or other factors like [wikilectures.eu]

It can be classified as primary or secondary depending upon the underlying pathophysiology. [digestive-diseases.imedpub.com]

Pathophysiology Posterior abdominal wall, after removal of the peritoneum, showing kidneys, suprarenal capsules, and great vessels. ( Hepatic veins labeled at center top.) [ipfs.io]

Endoscopic sclerotherapy and beta-blockers have come to be widely used in the man agement and prevention of variceal bleeding. [books.google.com]

Acquaintance of practicing radiologists to this phenomenon may be highly useful in the prevention of the diagnostic confusion and potentially unnecessary interventions. [ncbi.nlm.nih.gov]

Antihypertensive drugs, such as beta-blockers and nitrates, reduce pressure in the portal vein and thus prevent bleeding in the esophagus. [merckmanuals.com]

When the hepatic vein is obstructed, blood flow from the liver to the heart is prevented. This situation is very dangerous as it can lead to the liver being damaged. [buddchiarisyndrome.org]