BUDD-CHIARI SYNDROME

                       
                                 BUDD-CHIARI SYNDROME 

             
      The Budd- Chiari syndrome is a heterogeneous group of disorders characterized by hepatic venous outflow obstruction at the hepatic venules, the large hepatic veins, the inferior vena cava, or the right atrium. Hepatic veno-occlusive disease refers to obstruction of hepatic venous outflow at the level of the central or sublobular hepatic veins or both. 

Etiology:

    Most patients with Budd-Chiari syndrome have an underlying one-third of patients, the condition is idiopathic. Causes of Budd-Chiari syndrome include the following: 
. Hematologic disorders
. Inherited thrombotic diathesis
. Pregnancy and postpartum
. Oral contraceptives
. Chronic infections. 
. Chronic inflammatory diseases 
. Tumors
. Congenital membranous obstruction
. Hepatic venous stenosis
. Hypoplasia of the suprahepatic obstruction
.  Postsurgical obstruction
. Posttraumatic obstruction 
. Total parenteral nutrition (TPN): Budd-Chiari syndrome has been reported as a complication of TPN via an IVC catheter in a neonate. 

Clinical manifestations: 

  The classic triad of abdominal pain, ascites, and hepatomegaly is observed in the vast majority of patients with Budd Chiari syndrome, but it is nonspecific. If the liver had developed collaterals and decompress, patients can be asymptomatic or present with few symptoms. As the syndrome progresses, however, it can lead to liver failure and portal hypertension with symptoms.  

The clinic variants of Budd-Chiari syndrome have been described as follows: 
. Acute and subacute forms: Characterized by the rapid development of abdominal pain, ascites, hepatomegaly, jaundice and renal failure. 
. Chronic form: Most common presentation; patients present with progressive ascites, jaundice is absent, approximately 50% of patients also have renal impairment. 
. Fulminant form: Uncommon presentation; fulminant or subfulminant hepatic failure is present, along with ascites, tender hepatomegaly, jaundice and renal failure. 

 Patients with acute onset of obstruction typically present with acute right upper quadrant pain. Abdominal distention can also be a significant symptom because of ascites. Jaundice is rarely observed. 

Evaluation: 

 Physical examination may reveal the following findings: 
. Jaundice
. Ascites
. Hepatomegaly
. Splenomegaly
. Ankle edema
. Stasis ulcerations
. Prominence of collateral veins
. Routine biochemical test results are usually nonspecific in BUDD-CHIARI SYNDROME, which aids in mitigating hepatic congestion and thereby, restoration of hepatic function and alleviation of portal hypertension. 

Imaging is essential for the early identification and evaluation of the disease extent in BUDD-CHIARI SYNDROME, which aids in mitigating hepatic congestion and thereby, restoration of hepatic function and alleviation of portal hypertension. 

Investigation: 

Liver function test:

 Liver function tests may show a mild elevation.

Prothrombin time:

This may be prolonged, which may be confusing if the condition is associated with a hypercoagulability state. 

Ascitic fluid:

This usually has high-protein content but the risks(eg, bacterial peritonitis) and benefits of paracentesis should be considered before this procedure is undertaken. 

Magnetic resonance imaging(MRI): 

MRI may show a prominent caudate lobe. It is more sensitive than CT scan. 

Doppler ultrasound: 

Doppler ultrasound may help to exclude hepatic venous or inferior vena caval thrombosis. Caval venography excludes caval webs and occluded hepatic veins. 

Liver biopsy: 

Liver biopsy often shows centrilobular congestion. 

Homeopathic Therapeutics: 

Aurum- Metallicum: 

Sclerosis of liver arterial system brain. Right hypochondrium hot and painful. Buring heat and cutting pain in right hypochondrium.

Calc-ars:

Hepatosplenomegaly in children. 

cholesterinum: 

Obstinate hepatic engorgement. Buring pain in the side; on walking holds his hand on the side, hurts him. Gallstone. 

Flouric-acid: 

Hob-nailed liver of alcoholics. Soreness over the liver. 

Iodium: 

Liver and spleen sore and enlarged. Jaundice, a region of liver sore to pressure, swelling and hypertrophy of liver.  

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