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Chiari syndrome


Chiari syndrome, often called Budd-Chiari syndrome, is a rare disease, secondary to the occlusion of the veins exiting the liver, called hepatic veins. There are three hepatic veins. They join the inferior vena cava which brings a very large part of deoxygenated blood to the heart. Two main reasons are behind this obstruction: it may be a thrombosis, i.e. a clot blocking normal blood flow, but sometimes also the compression of the vein, most often by a tumor, developed at the expense of the liver or kidney. Budd-Chiari syndrome defines the set of symptoms resulting from the obstruction. Note that Budd-Chiari syndrome has nothing to do with Arnold-Chiari syndrome, a rare neurological abnormality of the newborn.


The Budd-Chiari syndrome is characterized by:
  • portal hypertension syndrome, the symptoms of which result from the increase in pressure within the system carrier (all veins located before arrival in the liver). The clinical signs are:
    • an increase in the size of the spleen, called splenomegaly;
    • the accumulation of abnormal fluid in the abdominal cavity, called ascites;
    • a very visible venous network on the abdomen, which is called collateral venous circulation.
  • abdominal pain;
  • an increase in the size of the liver;
  • sometimes a yellow discoloration of the skin and mucous membranes, called jaundice;
  • the presence of lower limb edema, increased volume of the legs;
  • a gradual destruction of liver cells that can lead to cirrhosis.


Given these symptoms, the evocation of the Budd-Chiari syndrome is not necessarily the first diagnosis mentioned, because of its rarity. After a series of generally non-specific laboratory tests, imaging tests will be done including a liver ultrasound, CT scan or MRI. They can show the existence of a tumor compressing the veins. Frequently, a Doppler ultrasound to study the blood flow through the vessels near the liver shows the absence of blood flow in one of the hepatic veins, confirming the diagnosis.


To treat Budd-Chiari syndrome, one must first have identified the cause. If a tumor is responsible, surgery will probably be necessary. If the cause is obstruction by a clot, the use of anticoagulants, direct destruction of thrombus, angioplasty with stenting to restore the vein to its normal size, or sometimes a bypass of the obstacle with shunt are possible alternatives.

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