Clinical presentation ranges from asymptomatic courses to liver failure, and considerable differences may be observed between patients of distinct races:

• In Caucasians, thrombosis of hepatic veins is the main cause of BCS and because thrombus formation may take several weeks or months, symptoms progressively worsen over time. Consequently, portal hypertension and liver cirrhosis are the hallmarks of severe BCS in these patients. In the rare event of acute occlusion of a major vessel, fulminant liver failure may be induced. Thrombosis-related BCS may be observed in patients of any age, but incidence rates increase with age.
• In Africans and Asians, developmental defects associated with membranous obstruction of hepatic vessels or IVC are much more common. Disease progression is less frequently noted and in case of moderate to severe BCS, symptoms manifest early in life.

Irrespective of the underlying disease, symptoms and signs indicating hepatic insufficiency may be registered:

• Abdominal pain
• Hepatomegaly
• Jaundice
• Ascites and peripheral edema
• Varicose vein and varicose ulcer
• Hepatorenal syndrome

According to the time course of outflow obstruction, symptom onset may be fulminant, acute, subacute or chronic. The latter is most common.

• Splenomegaly

  The Ultrasound scans show no flow in hepatic veins, compressed IVC, enlarged caudate lobe, splenomegaly and varices at the splenic hilum. [emedicine.com]

  […] the hepatic veins (less common) References:[1][2][3][4] Pathophysiology Clinical features Abdominal pain Tender hepatomegaly Ascites and abdominal distention Jaundice Signs of increased perfusion of portocaval anastomoses In severe cases: ankle edema, splenomegaly [amboss.com]

  Causes of Portal Hypertension Prehepatic Portal vein thrombosis Splenic vein thrombosis Massive splenomegaly Intrahepatic Schistosomiasis Congenital hepatic fibrosis Cirrhosis Alcoholic hepatitis Venoocclusive syndrome Posthepatic Constrictive pericarditis [uscmedicine.blog]

  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 similar [radiopaedia.org]

  In acute BCS, the characteristic symptoms are abdominal pain, ascites, hepatomegaly, splenomegaly, and jaundice. However, these symptoms are not usually present in chronic BCS. [casereports.in]

• Fever

  We encountered an APS patient on warfarin therapy who was admitted with fever and acute abdomen. [hindawi.com]

  A 30-year-old female patient with HIV/AIDS and CKD on hemodialysis was admitted to the emergency room for complaints of fever, prostration, and headache in the last six days. She had a tunneled dialysis catheter placed at the left jugular vein. [ncbi.nlm.nih.gov]

  such as Ebola, Lassa fever, and Lábrea fever, may kill in as little as two to five days. [en.wikipedia.org]

  CASE REPORT: A 30-year-old female patient with HIV/AIDS and CKD on hemodialysis was admitted to the emergency room for complaints of fever, prostration, and headache in the last six days. [jbn.org.br]

  Two weeks prior to this, he had noticed progressive fatigue, low-grade fever and passage of dark urine. He had ignored these symptoms and took multivitamins at home. No antecedent history of abdominal trauma or hepatotoxic drug intake was reported. [cancerjournal.net]

• Anorexia

  He additionally notes that his urine was significantly darker with associated nausea/vomiting, anorexia and generalized weakness/fatigue. [uscmedicine.blog]

  The commonest symptoms at diagnosis were abdominal distension, lethargy, and anorexia. Abdominal pain was present in 2 of them. [journals.lww.com]

  […] infections Non infectious complications Nutrition in specific situations Cancer COPD Diabetes mellitus Geriatrics Heart failure HIV IBD Intensive care Liver failure Pancreatitis Renal failure Surgery Miscellaneous B12 Folate deficiency Hypocalcemia Obesity Anorexia [gastrotraining.com]

• Edema of Lower Extremity

  Abdominal distention and edema of lower extremities were most common initial manifestations. Intra- or extrahepatic collaterals on imaging studies were of great importance for differential diagnosis. [ncbi.nlm.nih.gov]

• Dyspnea

  Dyspnea was found in one patient due to pleural effusion and this symptom disappeared after effective drainage. One patient developed acute cerebral infarction during hospitalization, but recovered and was discharged after treatment (Table 3 ). [nature.com]

• Abdominal Pain

  It presents with the classical triad of abdominal pain, ascites, and liver enlargement. The formation of a blood clot within the hepatic veins can lead to Budd– Chiari syndrome. The most common cause is polycythemia vera. [osmosis.org]

  Some of the symptoms are the same as those of gradually developing blockages: fatigue, an enlarged and tender liver, and abdominal pain in the upper abdomen. [merck.com]

  The most common clinical presentation is hepatomegaly, abdominal pain and ascites, but the onset can also be dramatical and life threatening with upper digestive bleeding due to portal hypertension through postsinusoidal blockage. [ncbi.nlm.nih.gov]

• Vomiting

  Vomiting blood is a medical emergency. [diagnose-me.com]

  We report a case of Budd-Chiari syndrome in association with large solitary FNH in a 22-year-old man who was referred to our institution with sudden intermittent right upper quadrant abdominal pain, vomiting, diarrhea with pale stool, decreased appetite [ncbi.nlm.nih.gov]

  Clinical symptoms include hepatomegaly, sudden abdominal epigastric pain with nausea and vomiting, and ascites. [whonamedit.com]

  Additional symptoms include vomiting and a yellow discoloration of the skin and whites of the eyes ( jaundice ). Severe liver failure is rare. [merck.com]

  Other symptoms include: Nausea and vomiting Weight loss Vomiting blood Splenomegaly (an enlarged spleen ) Edema (swelling) of the lower limbs Budd-Chiari Syndrome Diagnosis An internist (a physician who specializes in diseases of the internal organs), [chp.edu]

• Nausea

  Clinical symptoms include hepatomegaly, sudden abdominal epigastric pain with nausea and vomiting, and ascites. [whonamedit.com]

  He additionally notes that his urine was significantly darker with associated nausea/vomiting, anorexia and generalized weakness/fatigue. [uscmedicine.blog]

  While some patients with this liver condition don’t report any symptoms, others experience symptoms that usually worsen or become increasingly apparent over the course of weeks or months, including: Pain in the upper right section of the abdomen Nausea [tgh.org]

  Symptoms include: Ascites, the most common symptom: a build-up of fluid in the abdomen ( peritoneal cavity ) Enlarged, tender liver (blood backs up in the liver, causing it to enlarge) Fatigue Jaundice (yellowing of the skin and eyes) Nausea Vomiting [diagnose-me.com]

  Other symptoms include: Nausea and vomiting Weight loss Vomiting blood Splenomegaly (an enlarged spleen ) Edema (swelling) of the lower limbs Budd-Chiari Syndrome Diagnosis An internist (a physician who specializes in diseases of the internal organs), [chp.edu]

• Abdominal Distension

  A 33-year-old man who presented with abdominal distension, hematemesis, shortness of breath was found to have liver cirrhosis, portal hypertension and Budd-Chiari Syndrome. [ncbi.nlm.nih.gov]

  CC: progressive abdominal distension ID: 70 y/o male with PMH hypertension and hyperlipidemia, previously in excellent health who presents with progressive abdominal distention that started 3 months prior. [uscmedicine.blog]

  > Case Report A 14-year-old male presented with epigastric pain and rapid development of abdominal distension for 1 week. Two weeks prior to this, he had noticed progressive fatigue, low-grade fever and passage of dark urine. [cancerjournal.net]

  The commonest symptoms at diagnosis were abdominal distension, lethargy, and anorexia. Abdominal pain was present in 2 of them. [journals.lww.com]

• Right Upper Quadrant Pain

  Eight months later, she presented with jaundice and right upper quadrant pain. CT revealed multiple focal lesions in the liver, which on biopsy proved to be hepatic hemangioendothelioma (HHE). Her liver disease and ascites progressively increased. [ncbi.nlm.nih.gov]

  Acute obstruction (in about 20%) causes fatigue, right upper quadrant pain, nausea, vomiting, mild jaundice, tender hepatomegaly, and ascites. It typically occurs during pregnancy. Fulminant liver failure with encephalopathy is rare. [merckmanuals.com]

  Presentation can be sudden with right upper quadrant pain and rapidly developing ascites, hepatomegaly, jaundice and acute kidney injury (± fulminant hepatic failure ± hepatic coma). [patient.info]

• Aphthous Ulceration

  Behçet's disease is a chronic inflammatory disease of unknown aetiology, characterized by recurrent oral and genital aphthous ulcerations, uveitis, skin lesions and other multisystem affections associated with vasculitis. [ncbi.nlm.nih.gov]

• Thrombosis

  Leebeek and Moniek P.M. de Maat, Hypercoagulability and Hypofibrinolysis and Risk of Deep Vein Thrombosis and Splanchnic Vein Thrombosis, Arteriosclerosis, Thrombosis, and Vascular Biology, 10.1161/ATVBAHA.110.213371, 31, 3, (485-493), (2011). [doi.org]

  […] of superior vena cava I82.211 Chronic embolism and thrombosis of superior vena cava I82.22 Embolism and thrombosis of inferior vena cava I82.220 Acute embolism and thrombosis of inferior vena cava I82.221 Chronic embolism and thrombosis of inferior vena [icd10data.com]

  He was therapeutically anticoagulated for the cerebral venous thrombosis. [ncbi.nlm.nih.gov]

• Cardiomegaly

  Cardiomegaly and cardiac rhythm abnormalities may be depicted on liver scans. [emedicine.com]

• Hepatomegaly

  Chronic outflow obstruction (developing over months) may cause few or no symptoms until it progresses, or it may cause fatigue, abdominal pain, and hepatomegaly. [merckmanuals.com]

  A 10-month-old boy presented with ascites, right pleural effusion, and hepatomegaly after consumption of herbal drinks for 3 months. His clinical (including imaging) features were compatible with Budd-Chiari syndrome. [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]

• Jaundice

  Budd-Chiari syndrome (BCS) is also rare and presents with ascites, abdominal pain, hepatomegaly, leg swelling, and jaundice. Its etiology is diverse, and it is rarely caused by trauma. [ncbi.nlm.nih.gov]

  Keywords : Angioplasty, Budd-Chiari Syndrome, Dilatation, Jaundice, Warfarin. [casereports.in]

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

• Liver Dysfunction

  dysfunction Membranous obstruction of the vena cava / 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 [pathologyoutlines.com]

  She had no past medical history, including no history of liver dysfunction. On physical examination, yellowish palpebral conjunctiva and pitting oedema were found in both legs. [casereports.in]

  Congestive hepatopathy, is liver dysfunction due to venous congestion, usually due to congestive heart failure. [en.wikipedia.org]

  Shunts also increase the risk of brain function deterioration due to liver dysfunction. Another option is the transjugular intrahepatic portosystemic shunt (TIPS). [puristat.com]

  However, such shunts increase the risk of hepatic encephalopathy (deterioration of brain function due to liver dysfunction). Also, shunts occasionally become blocked, especially in people who have a tendency to form blood clots. [merck.com]

• Neurogenic Claudication

  Most patients with these problems complain of low-back pain, radicular pain, or neurogenic claudication, which are symptoms suggestive of disc herniation or spinal stenosis. [ncbi.nlm.nih.gov]

• Encephalopathy

  Surgical or endovascular treatment for SIPSVS was not carried out because the patient was asymptomatic and remained asymptomatic in terms of encephalopathy at a 1-year follow-up. [ncbi.nlm.nih.gov]

  Four of five patients who died had hepatic encephalopathy and 2/5 were receiving renal support prior to surgery. [doi.org]

• Kidney Failure

  She presented with progressive liver failure, acute kidney failure, acute respiratory failure, and encephalopathy stage IV. Multidisciplinary therapy was initiated with IV anti-infective drugs and optimizing mechanical ventilation. [ncbi.nlm.nih.gov]

  In fulminant BCS, ascites, hepatomegaly and kidney failure can occur with rapid liver failure. Ascites are present in the majority of people because of portal hypertension. [britishlivertrust.org.uk]

• Renal Insufficiency

  Renal Insufficiency. LMWH requires dose adjustment in patients with severe renal impairment (creatinine clearance less than 30ml/minute/1.73m 2 ). [clinicaladvisor.com]

On the one hand, confirmation of a tentative diagnosis of BCS requires the identification and localization of hepatic outflow obstructions. On the other hand, the underlying disease should be determined. As has been indicated above, myeloproliferative and coagulation disorders account for the majority of BCS cases and more than 75% of BCS patients suffers from at least one of those diseases. They may or may not manifest in form of additional symptoms.

Occluded vessels may be depicted by means of diagnostic imaging. Doppler ultrasonography is the technique of choice: It is sensitive, specific, readily available and of low cost [10]. It is not only used for diagnosis, but also for the assessment of response to thrombolytic therapy. However, computed tomography screens or magnetic resonance imaging are carried out in many cases in order to better evaluate the condition of the patient's liver, to identify necrotic or ischemic areas, and to rule out hepatic neoplasms. In contrast, a reliable diagnosis of liver cirrhosis requires the histopathological analysis of a liver biopsy sample.

Furthermore, coagulation tests, laboratory analyses of blood samples and possibly ascites fluid should be realized. The following results are to be expected in BCS patients:

• Elevated serum levels of aspartate and alanine aminotransferases, especially in cases of acute obstruction of hepatic outflow
• Bilirubinemia
• Hypoalbuminemia
• High protein content in ascites fluid (> 25 g/l), but low amounts of leukocytes

Diagnosis of myeloproliferative disorders is often supported by an analysis of bone marrow biopsy specimens. Genetic screens may be required to confirm a suspicion of hereditary disorders.

• Mediastinal Mass

  Esophageal varices can be seen as lobulated posterior mediastinal masses in 5-8% of patients. Silhouetting of the descending aorta and an abnormal convex contour of the azygos-esophageal recess are further signs of portal hypertension. [emedicine.com]

• Posterior Mediastinal Mass

  Esophageal varices can be seen as lobulated posterior mediastinal masses in 5-8% of patients. Silhouetting of the descending aorta and an abnormal convex contour of the azygos-esophageal recess are further signs of portal hypertension. [emedicine.com]

Patients diagnosed with hepatic vein thrombosis and coagulation disorders are often treated by administration of fibrinolytics (e.g., streptokinase, urokinase and recombinant tissue-type plasminogen activator) or anticoagulants (e.g., heparin and warfarin). However, empiric evidence shows that sole drug therapy is frequently insufficient in patients with complete vessel obstruction [11]. Patients diagnosed with partial occlusion of hepatic veins may be cured this way, but it is strongly recommended to assure their response to therapy by means of sonographic follow-ups.

More invasive therapeutic measures need to be applied in the majority of patients. Recanalization may be achieved by means of percutaneous transhepatic balloon angioplasty or stenting. Such measures may need to be repeated. If venous outflow cannot be restored either way, symptoms are likely to progressively worsen. Eventually, patients present with decompensated liver cirrhosis and hepatic failure. An organ transplant may be their only remaining option [10].

For patients suffering from portal hypertension, transhepatic or transcaval placement of a transjugular intrahepatic portacaval shunt (TIPS) should be considered. Liver failure and ascites are often associated with sodium overloads, although these are not necessarily reflected in decreased serum concentrations of sodium. Thus, patients should be advised to maintain a low-sodium diet.

Additionally, appropriate treatment of the underlying disease is necessary.

BCS is a potentially life-threatening disease and if left untreated, obstruction of major hepatic veins or IVC may eventually lead to liver failure. Other patients may remain asymptomatic for years. An individual patient's prognosis depends on the affected vessel and the degree of occlusion. Distinct scoring systems have been developed to predict the outcome [9]. In this context, the presence of encephalopathy or ascites, and - to a lower degree - hypercoagulability, bilirubinemia or old age have been identified as unfavorable prognostic factors. If a patient requires a liver transplant but the transplantation cannot be carried out, patients benefit from surgical shunting. To date, the 5-year-survival rate is between 80 and 90% [10].

Retrospective studies revealed that the vast majority of BCS patients presents with underlying myeloproliferative or coagulation disorders, and this observation may tempt to state that BCS should be classified as a possible complication of any of those diseases [2]. If this is the case, both etiology and pathogenesis of BCS are to be expected to be heterogeneous.

In detail, the following disorders have been related to BCS:

• Hematologic disorders, e.g., paroxysmal nocturnal hemoglobinuria, promyelocytic leukemia, any other form of thrombophilia and polycythemia
• Impaired fibrinolysis [2]
• Coagulation disorders like antiphospholipid syndrome, antithrombin III deficiency, factor II mutation, Leiden factor V mutation, protein C deficiency, protein S deficiency, most of them being genetic diseases
• Drug-induced and physiological hypercoagulable states, e.g., use of oral contraceptives and pregnancy
• Infectious diseases affecting the liver, e.g., amebiasis [3]
• Inflammatory bowel disease
• Abdominal trauma
• Neoplasms infiltrating hepatic veins or IVC

Only minor shares of patients suffering from any one of the aforementioned diseases develops BCS. A person's individual risk of BCS largely increases if more than one risk factor co-occur and indeed, about one fourth of BCS patients is diagnosed with at least two of them. However, in some patients, no underlying disease nor trigger of hepatic vein thrombosis can be identified. Here, either subclinical pathologies or as of yet unknown factors must be involved in BCS etiology.

BCS is considered a very disease. According to a retrospective study conducted in Sweden, the annual incidence is 0.8 per 1,000,000 inhabitants, and prevalence amounts to 1.4 per 1,000,000 people [4]. Here, most BCS patients were found to suffer from myeloproliferative disorders, often in addition to other predisposing diseases. In contrast, developmental defects associated with membranous obstruction of hepatic veins or IVC has been reported to be the most common cause of BCS in patients of African and Asian descent [5].

As per definition, BCS is associated with thrombotic or non-thrombotic obstruction of hepatic veins or IVC.

A variety of conditions predisposes for excess formation of thrombi or reduced fibrinolysis. Diseases like paroxysmal nocturnal hemoglobinuria and promyelocytic leukemia often underly BCS and constitute systemic disorders. Thus, thrombi may form in any vessel, in hepatic as well as extra-hepatic ones [6] [7]. This may explain why only few patients suffering from those diseases develop BCS, despite thrombosis being a dreaded complication of both. However, lesions of hepatic veins inflicted by pathogens, neoplasms or mechanical forces render those vessels particularly susceptible to thrombosis, and it is thus little surprising that many BCS patients are diagnosed with more than one causative pathology.

Irrespective of whether or not hepatic vein thrombosis is triggered by identifiable factors, it does provoke congestion of the liver and portal hypertension. The severity of hepatic damage mainly depends on the affected vessel and on the extent of outflow obstruction. Upon hemorrhagic infarction, i.e., if blood cannot be drained from parts of the liver, affected cells undergo necrosis. Uninvolved lobes of the liver may become hypertrophic in order to compensate for lost tissues, but this process may not protect from liver failure if large parts of the organ are affected.

Over time, thrombi are reorganized and partially replaced by fibrotic tissue. If BCS is not diagnosed before this stage of the disease, fibrous cords or membrane-like structures may be the only recognizable remnants of thrombi in recanalized vessels. Accordingly, conditions like membranous obstruction of the IVC may rather correspond to advanced stages of vein thrombosis than to the result of distinct pathophysiological processes. Nevertheless, the possibility of obliterative hepatocavopathy due to developmental defects should also be considered, particularly in blacks and Asians and if no hypercoagulable state can be detected [8].

No specific measures can be recommended to prevent BCS. However, patients who are diagnosed with any of those diseases predisposing for BCS are strongly advised to comply with treatment regimes to reduce their individual risk for thrombosis of hepatic veins or IVC.

Budd-Chiari syndrome (BCS) is a rare disease of heterogeneous etiology. Accordingly, diagnosis of BCS is based on clinical parameters: Affected individuals present with hepatomegaly, jaundice, ascites and peripheral edema due to thrombotic or non-thrombotic obstruction of hepatic veins; they generally suffer from myeloproliferative or coagulation disorders. Besides hepatic veins, the hepatic portion of the inferior vena cava (IVC) may also be affected [1].

Hepatic outflow obstructions and symptoms associated with BCS have first been described by George Budd, an English internist, and Hans Chiari, an Austrian pathologist, in the late nineteenth century. Since then, more detailed descriptions of different forms of BCS, e.g., thrombotic occlusion of intrahepatic veins or membranous obstruction of the IVC, have become available.

Presumably, both genetic and environmental factors play major roles in BCS pathogenesis. This hypothesis has been deducted from the observation that the majority of BCS patients presents with additional diseases whose etiology is better understood: Leiden factor V mutation and factor II mutation are hereditary disorders, and promyelocytic leukemia as well as infectious diseases can also be ascribed to defined causes. Precise diagnostic guidelines have not yet been established and since BCS generally occurs as a comorbidity, hepatic outflow obstructions may or may not be diagnosed as BCS.

Budd-Chiari syndrome (BCS) is a rare disease caused by hepatic outflow obstructions, i.e., those vessels draining blood from the liver are partially or completely occluded in affected individuals. This condition does provoke congestion, portal hypertension and eventually liver failure.

In most Caucasian patients, blood clots that occlude the respective vessel account for outflow obstructions. Different physiological and pathological conditions may be associated with excess formation of thrombi, e.g., myeloproliferative disorders (paroxysmal nocturnal hemoglobinuria, promyelocytic leukemia, polycythemia), hereditary coagulation disorders (factor II mutation, Leiden factor V mutation, protein C deficiency, protein S deficiency), drug-induced hypercoagulability due to use of oral contraceptives, and pregnancy. In contrast, African and Asian BCS patients often suffer from developmental defects leading to membranous obstruction of hepatic veins.

Irrespective of the underlying pathology, BCS patients experience symptoms of hepatic insufficiency. If small vessels are only partially occluded, these symptoms may not even be noted. In contrast, complete occlusion of major vessels may provoke the sudden onset of symptoms indicating life-threatening, fulminant liver failure. The following may be noted:

• Abdominal pain
• Hepatomegaly
• Jaundice
• Accumulation of fluid in the peritoneal cavity (ascites), swollen ankles and feet (peripheral edema)
• Varicose vein and varicose ulcer

BCS may be diagnosed by means of Doppler ultrasonography. Additional diagnostic measures may be required to assess the condition of hepatic tissue and to identify the underlying disease. To this end, more sophisticated diagnostic techniques, coagulation tests as well as laboratory analyses of blood samples and ascites fluid may be carried out.

In rare, uncomplicated cases, recanalization of the occluded vessel may be achieved by administrating fibrinolytics and anticoagulants. However, most patients require more invasive therapy. Hepatic outflow may be reestablished by means of balloon angioplasty or stenting, and portal hypertension may be diminished by placing a portacaval shunt. Still, not all patients respond to these therapeutic approaches and for some of them, a liver transplant may be their only remaining option.

1. Shin N, Kim YH, Xu H, et al. Redefining Budd-Chiari syndrome: A systematic review. World J Hepatol. 2016; 8(16):691-702.
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10. Martens P, Nevens F. Budd-Chiari syndrome. United European Gastroenterol J. 2015; 3(6):489-500.
11. Klein AS. Management of Budd-Chiari syndrome. Liver Transpl. 2006; 12(11 Suppl 2):S23-28.