A Rare Case of Budd Chari Syndrome Theepha Thayalakulasingam, M.D, Rehan Mohammed, M.D, Fred A Lopez, M.D, Arthur Zieske, M.D; David Smith, M.D & Lee Engel, M.D. Louisiana State University Health Sciences Center, New Orleans, LA; Department of Medicine Introduction Discussion Con’t. Budd Chiari syndrome is a rare disorder resulting from hepatic Independent variables predicting a worse prognosis include the presence of venous outflow tract obstruction anywhere from the small hepatic veins encephalopathy, ascites, an International Normalized ratio of > 2.3, older age at to the suprahepatic inferior vena cava. The obstruction can be due to diagnosis, higher bilirubin concentration, more severe liver failure, and presence of various causes, but all of which results in either reduction or refractory ascites. Overall survival was 90% 6 months, 82% at 1 year, 82% at 2 years obstruction of hepatic venous outflow. BCS can be further classified as and 80 % at 5 yrs. The main causes of death for patients with BCS are liver failure, primary BCS, where the obstruction is due to venous disease, such as multiorgan failure, and gastrointestinal bleeding thrombosis or phlebitis or secondary BCS where the compressions or invasion by a lesion originating outside the veins. We are presenting a patient with a primary Budd Chiari Syndrome. Conclusions Budd Chiari syndrome is a rare disorder resulting from hepatic venous outflow Case Presentation tract obstruction anywhere from the small hepatic veins to the suprahepatic inferior A 21 year old woman with tobacco abuse, history of two vena cava. Underlying cause of BCS includes myeloprolifereative disorder, miscarriages presented with flu like illness six week prior followed by two malignancy, infections and benign lesion of the liver, oral contraceptives, pregnancy, week history of abdominal pain and abdominal swelling. Patient during post-partum state and hypercoagulable states. Clinical presentation of BCS can vary the two week period complained of early satiety followed by right upper from absence of symptoms to fulminant hepatic failure. The two most commonly quadrant abdominal pain. The pain was described as dull, non radiating Portal venous phase, axial post contrast image of the Axial postcontrast imaging of the liver after a 5 minute delay associated symptoms of BCS are ascites and abdominal pain. Diagnosis of BCS can and constant 5-8/10 in intensity. The pain was aggravated by movement abdomen shows central hepatic hypertrophy causing mass reveals heterogeneously decreased enhancement (arrows) be established noninvasively using Doppler ultrasonography, Computed Tomography effect on the slit-like IVC (arrow). There is also fatty peripherally and homogenous, increased enhancement and alleviated somewhat by pain medications. The patient was initially infiltration of the liver, ascites, and splenomegaly. centrally. (CT) scan or Magnetic Resonance (MR) imaging. The gold standard is venography. evaluated at another hospital and was found to have ascites. Work-up for Liver biopsy can help diagnose BCS in acute or subacute cases and it can also help her ascites including two paracentesis, and imaging studies failed to decide upon treatment options. The treatment for BCS includes medical, radiological reveal a cause for her ascites thus the patient was transferred to our Discussion and surgical treatments. The treatment is individualized to the particular patient. tertiary facility. Budd Chiari Syndrome (BCS) refers to the pathological resulting from obstruction On physical exam, patient had pale conjunctiva, no icterus or or reduction in normal flow of blood out of the liver. It is a rare condition with incidence lymphadenopathy. Abdominal exam revealed distended abdomen with of 1 in 2.5 million persons per year. Underlying cause of BCS includes normoactive bowel sounds, positive fluid wave and shifting dullness, myeloprolifereative disorder, malignancy, infections and benign lesion of the liver, oral increased liver span and no guarding or rigidity or rebound tenderness. contraceptives, pregnancy, post-partum state and hypercoagulable states. Patient also had bilateral pedal edema without any cyanosis, clubbing. The obstruction of hepatic outflow tracts seen in BCS leads to increase in Genitourinary and pelvic exams were unremarkable. sinusoidal pressure and portal hypertension. The venous stasis leads to hepatic On laboratory analysis, patient was leucopenia with WBC of 3.3 x congestion which decreases hepatic perfusion. This can cause ischemic injury to the 103/UL, anemia with Hemoglobin of 11.2 and hematocrit of 34 with an MCV hepatocytes leading to hepatic necrosis, which eventually leads to hepatic fibrosis and of 80 and RDW of 18%. Patient also had total bilirubin of 2.1 mg/dL and PT cirrhosis. A patient can present at any stage of this continuum, thus clinical Liver Biopsy: sinusoidal congestion and hemorrhage, extensive fibrosis and ductular proliferation with no of 20.8 sec, INR of 1.7, and PTT of 38.9 sec. Evaluating for the cause of presentation of BCS varies depending on the acuteness of presentation and degree of clear cholestasis  suggestive of obstruction of hepatic veins/venules as seen in Budd Chiari Syndrome. ascites numerous other labs were draw: negative acute viral hepatitis, obstruction. These patient can present with acute, even fulminant hepatic failure, negative HIV testing, Negative EBV titers, CMV IgM and IgG, Negative subacute or chronic hepatic failure. BCS present mainly in the third and fourth decade pregnancy test, ANA, SS-A, Anti-smooth muscle antibody, Anti- and more commonly in women than men. Clinical presentation of BCS can vary from mitochondrial Ab, SPEP/UPEP, Negative Gonorrhea and Chlamydia absence of symptoms to fulminant hepatic failure. Up to 20% of the patient may not screening. A CT scan of abdomen and pelvis was done, and it revealed have any symptoms, while very low < 1.5% of all acute liver failure is accounted for by hepatomegaly, ascites, abnormal appearance of intra-hepatic veins, and BCS. The two most commonly associated symptoms of BCS are ascites and abdominal intra-hepatic portion of IVC. A liver biopsy and hepatic venogram was pain. Other classical signs and symptoms include hepatomegay, fever, abdominal pain, done and it showed a clot in the right hepatic vein with spider web lower extremity edema, gastrointestinal bleeding, and hepatic encephalopathy. collaterals and edematous mass effect on the hepatic veins. Patient was Diagnosis of BCS can be established noninvasively using Doppler ultrasonography, Computed Tomography (CT) scan or Magnetic Resonance (MR) Reference stared on anticoagulation with IV heparin therapy. Hypercoagulable work- 1. Marco Cura, MD, Ziv Haskal MD, Jorge Lopera, MD. Diagnostic and Interventional Radiology for Budd Chiari up revealed low protein C of 37 and borderline low anti-thrombin 3 level imaging. The most prominent feature noted on noninvasive imaging is hepatomegaly Syndrome. RadioGraphics.rsnajnls.org. May-June 2009:669-681. of 63 and a heterozygote mutation of Factor V Leiden. Other markers and caudate lobe hypertrophy. Although, Doppler US, CT, MRI are commonly used to 2. DeLeve et al. Vascular Disorders of the Liver. Hepatology. May 2009;49:1729-1761. diagnose BCS, the gold standard is venography. Liver biopsy can help diagnose BCS 3. Marudanayagam, Ravi et al. Aetiolgy and outcome of acute liver failure. International hepato-Pancreato- such as Protein S, Homocysteine level, lupus anticoagulant, Biliary Association. 2009, 11, 429-434. anticardiolipin, and prothrombin gene G20210A mutation, Factor V Leiden in acute or subacute cases and it can also help decide upon treatment options. The 4. Sarwa Darwish Murad et al. Etiolgoy, Management, and Outcome of the Budd Chiari Syndrome. Annals of were all within normal limit. Work up for Paroxysmal nocturnal treatment for BCS includes medical, radiological and surgical treatments. Medical Internal Medicine. 4 August 2009:151(3):168-175. treatments include anticoagulation to prevent recurrent thrombosis and extension of 5. Dominique-Charles Valla. Primary Budd Chiari Syndrome. Journal of Hepatology 50 (2009) 195-203. hemoglobinuria was also negative. Patient’s pathology result showed 6. Andrew L. Singer et al. Successful Liver Transplantation for Budd-Chiari Syndrome in a Patient with sinusoidal congestion and hemorrhage, extensive fibrosis and ductular existing thrombosis. Local thrombolysis can also be used for acute and subacute BCS Paroxysmal Nocturnal Hemoglobinuria Treated with the Anti-Complement Antibody Eculizumab. Liver proliferation with no clear cholestasis. Finding were suggestive of of less than four weeks duration. Ascites can be treated with low sodium diet, and Transplantation 15:540-543, 2009. diuretics. Treating underlying causes whenever possible is also essential to relieve 7. Selcuk Sozer et al. The presence of JAK2V61F mutation in the liver endothelial cells of patients with Budd obstruction of hepatic veins/venules and seen in Budd Chiari Syndrome. Chiari Syndrome. May 21, 2009. 113 (21): 5246-5249. Patient’s abdominal pain and distention improved greatly during this symptoms. Radiological and surgical interventions include angioplasty, Transjugular 8. RP Hasija, A Nagral, S Marar, and Bavdekar. Transjugular Intrahepatic Portosystemic Shunt (TIPSS) for Budd hospital course. She was started on long term anticoagulation with intrahepatic portosystemic shunting (TIPS), Stenting, Shunting procedures and liver Chiari Syndrome. Indian Pediatrics. June 17, 2010; 47: 527-528. Coumadin and was sent home with Hematology and Hepatology and transplant. The treatment is individualized to the particular patient. 9. Dutta TK, Venugopal V. Venous thromboembolism: The intricacies. Journal of Postgraduate Medicine. January 2009:55 (1):55-64. Oncology follow-up.