Budd-Chiari Syndrome Flashcards
Budd-Chiari Syndrome
Budd-Chiari syndrome, or hepatic vein thrombosis, is usually seen in the context of underlying haematological disease or another procoagulant condition
Causes polycythaemia rubra vera thrombophilia: activated protein C resistance, antithrombin III deficiency, protein C and S deficiencies pregnancy oral contraceptive pill
Features
abdominal pain: sudden onset, severe
ascites
tender hepatomegaly
Budd-Chiara Syndrome - Example Question
A 35-year-old woman presents to hospital with a three-day history of acute abdominal pain and distension. Her past medical history includes asthma, coeliac disease and an unprovoked deep vein thrombosis in her left leg a year ago. Her medications include Symbicort 400/12 2 puffs BD and salbutamol PRN. She does not smoke and drinks approximately 12 units of alcohol a week. She works as a secretary in a legal firm. Her parents both suffer from type 2 diabetes mellitus.
On examination, her temperature is 37.7ºC, pulse rate is 110 beats per minute and blood pressure is 130/80 mmHg. There is evidence of icterus in her sclera. Inspection of her abdomen reveals gross distension. There is hepatomegaly on palpation and shifting dullness on percussion. There is marked tenderness on palpation of the right upper quadrant of her abdomen.
Her blood tests show:
Hb 100 g/l Na+ 138 mmol/l Bilirubin 63 µmol/l Platelets 740 * 109/l K+ 5.2 mmol/l ALP 295 u/l WBC 14.2 * 109/l Urea 5.1 mmol/l ALT 230 u/l Neuts 9.5 * 109/l Creatinine 89 µmol/l γGT 210 u/l Lymphs 0.7 * 109/l Albumin 26 g/l INR 1.5 CRP 93 mg/L Amylase 173 U/L
What is the most likely diagnosis?
> Budd-Chiari syndrome Hepatic abscess Portal vein thrombosis Acute pancreatitis Sarcoidosis
The most likely diagnosis, in this case, is Budd-Chiari syndrome (BCS) which is the occlusion of the hepatic veins draining the liver. Patients may present with the classical triad of abdominal pain, ascites and hepatomegaly but some patients with BCS may follow a chronic or asymptomatic course.
BCS can be caused by thrombosis of the hepatic veins (primary) or external compression causing occlusion of the hepatic veins (secondary). It is important to look for a cause for hypercoagulability in patients with primary BCS.
Treatment for BCS involves medical therapy with diuretics and anticoagulants, surgical shunt placement or a TIPSS procedure. In patients with fulminant BCS, liver transplantation should be considered.
Budd- Chiari Syndrome and PRV - Example Question
A 50-year-old woman with a history of polycythaemia rubra vera develops abdominal pain and gross ascites over the past 2 months. Due to her busy work as a lawyer she mentions that her diet is mostly takeaways, however she is a lifelong teetotaler.
On examination: mild jaundice with smooth hepatomegaly. Hepatojugular reflex is negative. Abdominal wall is grossly oedematous with an associated tortuous venous pattern. There is prominent lower limb oedema.
Which of the following is the most likely diagnosis?
> Budd-Chiari syndrome Hepatic cirrhosis Hepatocellular carcinoma Primary sclerosing cholangitis Nonalcoholic steatohepatitis
Budd-Chiari syndrome occurs when hepatic venous outflow is obstructed secondary to thrombosis of the major hepatic veins. The thrombus can extend into the inferior vena cava, leading to the abdominal wall signs and leg oedema as noted in the presentation. The condition is rare, typically occurring in the setting of a coagulopathy due to haematologic disease, which in this cause is polycythaemia rubra vera. The impression from the patient’s social history may lead you towards considering nonalcoholic steatohepatitis, however this does not cause ascities.
Budd-Chiara Syndrome - Presentation
Budd-Chiari syndrome (BCS) is the occlusion of the hepatic veins draining the liver. Patients may present with the classical triad of abdominal pain, ascites and hepatomegaly but some patients with BCS may follow a chronic or asymptomatic course.
BCS can be caused by thrombosis of the hepatic veins (primary) or external compression causing occlusion of the hepatic veins (secondary).
The thrombus can extend into the inferior vena cava leading to abdominal wall signs and leg oedema ie grossly oedematous with an associated tortuous venous pattern.
It is important to look for a cause for hypercoagulability in patients with primary BCS.
Treatment for BCS involves medical therapy with diuretics and anticoagulants, surgical shunt placement or a TIPSS procedure. In patients with fulminant BCS, liver transplantation should be considered.
Portal Vein Thrombosis
Portal Vein Thrombosis
Portal vein thrombosis causes portal hypertension and consequent GI bleeding from varices, usually in the lower esophagus or stomach. Diagnosis is based on ultrasonography. Treatment involves control of variceal bleeding (usually with endoscopic banding, IV octreotide, or both), prevention of recurrence using beta-blockers, and sometimes surgical shunts and thrombolysis for acute thrombosis.
Etiology
Common causes vary by age group
Common Causes of Portal Vein Thrombosis*
Age Group
Neonates
Umbilical stump infection or omphalitis (spread via the umbilical vein to the portal vein)
Congenital portal vein abnormalities (less common)
Congenital abnormalities of the portal vein usually accompany congenital defects elsewhere.
Older children
Pylephlebitis
In acute appendicitis, infection enters the portal system; the vascular infection/inflammation then triggers thrombosis.
Adults
Surgery (eg, splenectomy)
Hypercoagulable states (eg, myeloproliferative disorder, protein C or S deficiency, pregnancy)
Cancer (eg, hepatocellular or pancreatic carcinoma, renal or adrenal cancers)
Cirrhosis
Trauma
Possibly portal hypertension causing congestion and stasis
*The cause is multifactorial in most cases and unknown in about one third of cases.
Symptoms and Signs
Acute portal vein thrombosis is commonly asymptomatic unless associated with another event, such as pancreatitis (the cause), or another complication, such as mesenteric venous thrombosis. Most often, clinical features—splenomegaly (especially in children) and variceal hemorrhage—develop over a period of time secondary to portal hypertension. Ascites is uncommon (10%) in postsinusoidal portal hypertension. Ascites may be precipitated when cirrhosis is also present or when serum albumin (and thus oncotic pressure) deceases after high-volume fluid resuscitation for a major GI bleed.
Diagnosis
Clinical evaluation and liver function tests
Doppler ultrasonography
Portal vein thrombosis is suspected in patients with the following:
Manifestations of portal hypertension without cirrhosis
Mild abnormalities in liver function or enzymes plus risk factors such as neonatal umbilical infection, childhood appendicitis, or a hypercoagulability disorder
Doppler ultrasonography is usually diagnostic, showing diminished or absent portal vein flow and sometimes the thrombus. Difficult cases may require MRI or CT with contrast. Angiography may be required to guide shunt surgery.
Treatment
For some acute cases, thrombolysis
Long-term anticoagulation
Management of portal hypertension and its complications
In acute cases, thrombolysis is sometimes successful, best reserved for recent occlusion, particularly in hypercoagulable states. Anticoagulation does not lyse clots but has some value for long-term prevention in hypercoagulable states despite the risk of variceal bleeding. In neonates and children, treatment is directed at the cause (eg, omphalitis, appendicitis). Otherwise, management is directed at the portal hypertension and its complications; treatment can include octreotide IV (a synthetic analog of somatostatin) and endoscopic banding to control variceal bleeding and nonselective beta-blockers to prevent rebleeding. These therapies have decreased the use of surgical shunts (eg, mesocaval, splenorenal), which can become occluded and have an operative mortality rate of 5 to 50%. Transjugular intrahepatic portosytemic shunting (TIPS) is not recommended. TIPS requires monitoring (including frequent angiography) to assess patency, may become blocked, and may not adequately decompress the liver.
Key Points
Causes of and risk factors for portal vein thrombosis include umbilical cord infection (in neonates), appendicitis (in children), and hypercoagulability states (in adults).
Suspect portal vein thrombosis if patients have manifestations of portal hypertension in the absence of cirrhosis or if they have mild, nonspecific liver abnormalities plus risk factors.
Confirm the diagnosis using Doppler ultrasonography or, if results are inconclusive, MRI or CT with contrast.
Treat the cause of portal vein thrombosis and the complications of portal hypertension
Budd Chiari Syndrome
Budd-Chiari syndrome is obstruction of hepatic venous outflow that originates anywhere from the small hepatic veins inside the liver to the inferior vena cava and right atrium. Manifestations range from no symptoms to fulminant liver failure. Diagnosis is based on ultrasonography. Treatment includes supportive medical therapy and measures to establish and maintain venous patency, such as thrombolysis, decompression with shunts, and long-term anticoagulation.
Etiology
In the Western world, the most common cause is a clot obstructing the hepatic veins and the adjacent inferior vena cava. Clots commonly result from the following:
Thrombotic conditions (eg, protein C or S deficiency, antiphospholipid syndrome, antithrombin III deficiency, factor V Leiden mutation, pregnancy, oral contraceptive use)
Hematologic disorders (eg, myeloproliferative disorders such as polycythemia and paroxysmal nocturnal hemoglobinopathy)
Inflammatory bowel disease
Connective tissue disorders
Trauma
Infection (eg, hydatid cyst, amebiasis)
Tumor invasion of the hepatic vein (eg, hepatocellular or renal cell carcinoma)
Sometimes Budd-Chiari syndrome begins during pregnancy and unmasks a previously asymptomatic hypercoagulability disorder.
The cause of obstruction is often unknown. In Asia and South Africa, the basic defect is often a membranous obstruction (webs) of the inferior vena cava above the liver, likely representing recanalization of a prior thrombus in adults or a developmental flaw (eg, venous stenosis) in children. This type of obstruction is called obliterative hepatocavopathy.
Budd-Chiari syndrome usually develops over weeks or months. When it does develop over a period of time, cirrhosis and portal hypertension tend to develop.
Symptoms and Signs
Manifestations range from none (asymptomatic) to fulminant liver failure or cirrhosis. Symptoms vary depending on whether the obstruction occurs acutely or over time.
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. Aminotransferase levels are quite high.
Chronic outflow obstruction (developing over weeks to months) may cause few or no symptoms until it progresses, or it may cause fatigue, abdominal pain, and hepatomegaly. Lower-extremity edema and ascites may result from venous obstruction, even in the absence of cirrhosis. Cirrhosis may develop, leading to variceal bleeding, massive ascites, splenomegaly, hepatopulmonary syndrome, or a combination. Complete obstruction of the inferior vena cava causes edema of the abdominal wall and legs plus visibly tortuous superficial abdominal veins from the pelvis to the costal margin.
Diagnosis
Clinical evaluation and liver function tests
Vascular imaging
Budd-Chiari syndrome is suspected in patients with hepatomegaly, ascites, liver failure, or cirrhosis when there is no obvious cause (eg, alcohol abuse, hepatitis) or when the cause is unexplained.
Liver function tests are usually abnormal; the pattern is variable and nonspecific. The presence of risk factors for thrombosis increase the consideration of this diagnosis.
Imaging usually begins with abdominal Doppler ultrasonography, which can show the direction of blood flow and the site of obstruction. Magnetic resonance angiography and CT are useful if ultrasonography is not diagnostic. Conventional angiography (venography with pressure measurements and arteriography) is necessary if therapeutic or surgical intervention is planned.
Liver biopsy is done occasionally to diagnose the acute stages and determine whether cirrhosis has developed.
Prognosis
Without treatment, most patients with complete venous obstruction die of liver failure within 3 yr. For patients with incomplete obstruction, the course varies.
Treatment
Supportive care
Restoration and maintenance of adequate venous outflow
Treatment varies according to onset (acute vs chronic) and severity (fulminant liver failure vs decompensated cirrhosis or stable/asymptomatic). The cornerstones of management are
Giving supportive therapy directed at complications (eg, ascites, liver failure, esophageal varices)
Decompressing the congested liver (ie, maintaining venous outflow)
Preventing propagation of the clot
Aggressive interventions (eg, thrombolysis, stents) are used when the disease is acute (eg, within 4 wk and in the absence of cirrhosis). Thrombolysis can dissolve acute clots, allowing recanalization and so relieving hepatic congestion. Radiologic procedures, such as angioplasty, stenting, and/or portosytemic shunts, can have a major role.
For caval webs or hepatic venous stenosis, decompression via percutaneous transluminal balloon angioplasty with intraluminal stents can maintain hepatic outflow. When dilation of a hepatic outflow narrowing is not technically feasible, transjugular intrahepatic portosystemic shunting (TIPS) and various surgical shunts can provide decompression by diverting blood flow into the systemic circulation. Portosystemic shunts are typically not used if hepatic encephalopathy is present; such shunts worsen liver function. Further, clots tend to form in shunts, especially if patients have a hematologic or thrombotic disorder.
Long-term anticoagulation is often necessary to prevent recurrence. Liver transplantation may be lifesaving in patients with fulminant disease or decompensated cirrhosis.
Key Points
The most common cause of Budd-Chiari syndrome (obstruction of hepatic venous outflow) is a clot blocking the hepatic veins and inferior vena cava.
Consider the diagnosis if patients have typical findings (eg, hepatomegaly, ascites, liver failure, cirrhosis) that are unexplained or if they have abnormal liver function test results and risk factors for thrombosis.
Confirm the diagnosis using Doppler ultrasonography or, if results are inconclusive, magnetic resonance angiography or CT.
Restore venous outflow (eg, with thrombolysis, angioplasty, stents), and treat complications
Key differentiating feature between Budd Chiari and Portal Vein thrombosis
Budd Chiari = ASCITES, abdo pain + hepatomegaly
Portal Vein Thrombosis = Ascites uncommon. Splenomegaly +/- Signs of portal HTN
