The BuddChiari syndrome is a disorder that is extremely uncommon, affecting only one adult in one million. The illness is brought on by a blockage in the hepatic veins, which are responsible for draining the liver. Abdominal discomfort, ascites, and an enlarged liver are the three basic symptoms that accompany this condition. The BuddChiari syndrome is a condition that can be caused when a blood clot forms inside the veins of the liver. The condition can present itself in a variety of ways, including being fulminant, acute, chronic, or asymptomatic. The subacute type is by far the most typical manifestation.
The primary manifestation of BuddChiari syndrome, which accounts for 75% of cases, is thrombosis of the hepatic vein.
There is a strong correlation between hepatic vein thrombosis and the disorders listed below.
The vera form of polycythemia
Pregnancy Postpartum state
Utilization of oral contraceptive methods
Hemoglobinuria with paroxysmal nighttime episodes
Hepatocellular carcinoma
Lupus anticoagulants
The acute syndrome manifests itself clinically as fast growing severe upper abdominal pain, yellow coloring of the skin and whites of the eyes, enlargement of the liver and the spleen, fluid buildup inside the peritoneal cavity, increased liver enzymes, and finally encephalopathy. Early symptoms of fulminant syndrome include encephalopathy and the development of ascites. In addition, there is a potential for severe lactic acidosis and the loss of liver cells. Caudate lobe hypertrophy is commonly observed. The majority of patients suffer from a variant of BuddChiari syndrome that has a more gradual start. This might not even be uncomfortable. Imaging may reveal the formation of a “spider’s web” pattern caused by the formation of a network of venous collaterals surrounding the blockage. Patients run the risk of developing cirrhosis and developing symptoms associated with liver failure.
It is known as BuddChiari syndrome when there is any blockage of the venous vasculature of the liver, which can occur anywhere from the venules to the right atrium. Because of this, the pressure in the portal vein and the hepatic sinusoid rises as the blood flow becomes obstructed. Higher portal pressure leads to increased filtration of vascular fluid, which results in the development of ascites in the abdominal cavity and collateral venous flow through alternative veins, which can induce varices in the esophagus, stomach, and rectal areas. As a result of ischemia, obstruction is also responsible for the development of centrilobular necrosis and peripheral lobule fatty alteration. If this situation is allowed to exist for an extended period of time, nutmeg liver will eventually develop. Failure of the kidneys may develop, possibly as a result of the body’s perception of a “underfilled” situation, which leads to the activation of the renin-angiotensin pathways and the retention of excessive salt.
This video depicts all the key features of the Budd Chairi Syndrome with clarity
I am Dr Shashikant, a pathologist,professor and medical writer by profession. Please contact-ruc.isha@gmail.com for medical writing, Pathology online tutoring for usmle,meetpg,plab,fmge,mbbs,md
