Budd–Chiari syndrome
| Budd–Chiari syndrome | |
|---|---|
 |
|
| Budd–Chiari syndrome secondary to cancer, note clot in the inferior vena cava and the metastasis in the liver | |
| Classification and external resources | |
| Specialty | hepatology |
| ICD-10 | I82.0 |
| ICD-9-CM | 453.0 |
| OMIM | 600880 |
| DiseasesDB | 1735 |
| MedlinePlus | 000239 |
| eMedicine | med/2694 ped/296 radio/121 |
| Patient UK | Budd–Chiari syndrome |
| MeSH | D006502 |
Budd–Chiari syndrome is a very rare condition, affecting 1 in a million adults. The condition is caused by occlusion of the hepatic veins that drain the liver. 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 syndrome can be fulminant, acute, chronic, or asymptomatic.
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, elevated liver enzymes, and eventually encephalopathy. The fulminant syndrome presents early with encephalopathy and ascites. Liver cell death and severe lactic acidosis may be present as well. Caudate lobe enlargement is often present. The majority of patients have a slower-onset form of Budd–Chiari syndrome. This can be painless. A system of venous collaterals may form around the occlusion which may be seen on imaging as a "spider's web". Patients may progress to cirrhosis and show the signs of liver failure.
On the other hand, incidental finding of a silent, asymptomatic form may not be a cause for concern.
The cause can be found in more than 80% of patients.
Budd–Chiari syndrome is also seen in Infection such as tuberculosis, congenital venous webs and occasionally in inferior vena caval stenosis.
Often, the patient is known to have a tendency towards thrombosis, although Budd–Chiari syndrome can also be the first symptom of such a tendency. Examples of genetic tendencies include protein C deficiency, protein S deficiency, the Factor V Leiden mutation, hereditary anti-thrombin deficiency and prothrombin mutation G20210A. An important non-genetic risk factor is the use of estrogen-containing (combined) forms of hormonal contraception. Other risk factors include the antiphospholipid syndrome, aspergillosis, Behçet's disease, dacarbazine, pregnancy, and trauma.
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