Budd-Chiari Syndrome is a very rare condition, affecting one in a million adults. This condition is caused by the occlusion of hepatic vein that is drying the liver. It comes with a classic triad of abdominal pain, ascites, and liver enlargement. The formation of blood clots inside the hepatic vein can cause Budd-Chiari syndrome. This syndrome can be fulminant, acute, chronic, or asymptomatic.
Video Budd-Chiari syndrome
Signs and symptoms
Acute syndrome develops with rapid, progressive upper abdominal pain, yellow discoloration of the skin and whites of the eyes, enlarged liver, enlarged spleen, fluid buildup in the peritoneal cavity, elevated liver enzymes, and finally encephalopathy. Fulminant syndrome occurs earlier with encephalopathy and ascites. Liver cell death and severe lactic acidosis can also occur. Enlarged caudate lobes are often present. The majority of patients have a slower form of Budd-Chiari syndrome. It can be painless. A venous collateral system can form around the occlusion that can be seen in imaging as a "spider web". Patients can develop into cirrhosis and show signs of liver failure.
On the other hand, the incidental findings of the silent, asymptomatic form may not be of concern.
Maps Budd-Chiari syndrome
Cause
The cause can be found in more than 80% of patients.
- Main Budd-Chiari Syndrome (75%): hepatic venous thrombosis
- The veins of the hepatic veins correspond to the following in decreasing frequency sequence:
- Polycythemia vera
- Pregnancy
- Postpartum state
- Oral contraceptive use
- Paroxysmal nocturnal hemoglobinuria
- Hepatocellular carcinoma
- Lupus anticoagulant
- Secondary Budd-Chiari syndrome (25%): hepatic venous compression by external structures (eg tumors)
Budd-Chiari syndrome is also seen in tuberculosis, congenital venous tissue and occasionally in inferior vena cava stenosis.
Often, patients are known to have a tendency towards thrombosis, although Budd-Chiari syndrome can also be the first symptom of such a trend. Examples of genetic predispositions include lack of protein C, protein S deficiency, Factor V Leiden mutation, hereditary anti-thrombin deficiency and prothrombin G20210A mutation. An important non-genetic risk factor is the use of hormonal contraceptive forms containing estrogen (combined). Other risk factors include antiphospholipid syndrome, aspergillosis, BehÃÆ'§et disease, dacarbazine, pregnancy, and trauma.
Many patients have Budd-Chiari syndrome as a complication of polycythemia vera (myeloproliferative disease of red blood cells). Patients suffering from paroxysmal nocturnal hemoglobinuria (PNH) appear to be particularly at risk for Budd-Chiari syndrome, more than any other form of thrombophilia: up to 39% develop venous thrombosis and 12% may acquire Budd-Chiari.
Related conditions are veno-occlusive disease, which occurs in bone marrow transplant recipients as a complication of their treatment. Although the mechanism is similar, it is not considered a form of Budd-Chiari syndrome.
Other toxicological causes of veno-occlusive disease include plant & amp; source of pyrrolizidine alkaloid herbs such as Borage, Boneset, Coltsfoot, T'u-san-chi, Comfrey, Heliotrope (sunflower seeds), Gordolobo, Germander, and Chaparral.
Pathophysiology
Any venous heart vein obstruction is called Budd-Chiari syndrome, from the venule to the right atrium. This leads to an increase in portal vein and sinusoidal liver pressure as stagnant bloodstream. The increased portal pressure causes increased vascular fluid filtration with the formation of ascites in the abdomen and collateral venous flow through alternative veins leading to esophageal, stomach and rectal varices. Obstruction also causes centrilobular necrosis and peripheral lipid lobe changes due to ischemia. If this condition continues chronically what is known as liver nutmeg will develop. Kidney failure may occur, perhaps because the body feels "underfill" and subsequent activation of the renin-angiotensin pathway and excess sodium retention.
Diagnosis
When Budd-Chiari syndrome is suspected, measurements are made from the levels of liver enzymes and other organ markers (creatinine, urea, electrolytes, LDH).
Budd-Chiari syndrome is most often diagnosed using abdominal ultrasound and retrograde angiography studies. Ultrasound may indicate obliteration of the hepatic vein, thrombosis or stenosis, spiderweb vessels, large collateral vessels, or hyperechoic wires replacing normal veins. Computed tomography (CT) or magnetic resonance imaging (MRI) is sometimes used although this method is generally insensitive. Liver biopsy is not specific but it is sometimes necessary to distinguish between Budd-Chiari syndrome and other causes of hepatomegaly and ascites, such as galactosemia or Reye's syndrome.
Treatment
A small percentage of patients can be medically treated with sodium restriction, diuretics to control ascites, anticoagulants such as heparin and warfarin, and general symptomatic management. The majority of patients require further intervention. A milder form of Budd-Chiari can be treated with a surgical shunt to divert blood flow around the obstruction or the liver itself. Shunt should be placed early after diagnosis for best results. TIPS are similar to a surgical shunt: it accomplishes the same goal but has a lower related death rate procedure - a factor that has led to growth in its popularity. If all hepatic veins are blocked, the portal vein can be approached via the intrahepatic portion of the inferior vena cava, a procedure called DIPS (direct directional portocaval shunt). Patients with stenosis or obstruction of the vena cava may benefit from angioplasty. Studies limited to thrombolysis with direct infusion of urokinase and tissue plasminogen activator to the obstructed veins indicate success in treating Budd-Chiari syndrome; However, this is not routinely tried.
Liver transplantation is an effective treatment for Budd-Chiari. It is generally reserved for patients with fulminant liver failure, shunt failure or cirrhosis developments that reduce life expectancy to 1 year. Long-term survival after transplantation ranges from 69-87%. The most common complications of transplantation include rejection, arterial or venous thrombosis and anticoagulation bleeding. Up to 10% of patients may experience recurrence of Budd-Chiari syndrome after transplantation.
Prognosis
Several studies have attempted to predict the survival of patients with Budd-Chiari syndrome. In general, nearly 2/3 patients with Budd-Chiari are alive at 10 years. Important negative prognostic indicators include ascites, encephalopathy, increased Child-Pugh score, increased prothrombin time, and changes in serum levels of various substances (sodium, creatinine, albumin, and bilirubin). Survival is also highly dependent on the cause of Budd-Chiari syndrome. For example, a patient with an underlying myeloproliferative disorder may develop into acute leukemia, regardless of Budd-Chiari syndrome.
Eponyms
Named after George Budd, a British doctor, and Hans Chiari, an Austrian pathologist.
References
External links
directory Merck Manual Diagnosis and Therapy Home Edition Syndrome 10-138d.Budd-Chiari
Source of the article : Wikipedia
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