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Budd-chiari syndrome

By dr mahmoud jamal

mahmoud114
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Budd-chiari syndrome

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  1. Budd-chiarisyndrome Dr.Mahmoudjamal

  2. Anatomy

  3. Definition.....BCS

  4. Pathophysiology

  5. Etiology Secondary Primary compression or invasion of the hepatic veins and/or the inferior vena cava by a lesion that originates outside of the veinasmalignancy obstruction due to a predominantly venous process (thrombosis or phlebitis), As*PRV *Pregnancy

  6. Features Abdominalpain Triad Asites Hepatomegaly

  7. AcuteBCSwithALF *elevated transaminases *jaundice *hepatic encephalopathy *elevated PT,INR *hepatic encephalopathy (develops within eight weeks after the development of jaundice)

  8. AcuteBCSwithoutALF *Clinical manifestations develop rapidly (over the course of weeks) *intractable ascites *hepatic necrosis.

  9. SubacuteBCS *Insidious onset *patients taking up to three months to develop symptoms *Ascites and hepatic necrosis may be minimal due to decompression of the sinusoids by portal and hepatic venous collaterals.

  10. ChronicBCS complications of cirrhosis ....PH ....Splenomegaly ....OV ....Etc.

  11. Diagnosis

  12. UltrasoundDoppler

  13. Non specificfinding *hepatomegaly *splenomegaly *ascites *intra-abdominal collaterals *caudate lobe hypertrophy *atrophy of other hepatic lobes *compression or narrowing of the inferior vena cava.

  14. Specificfindings Inability to visualize the junction of the major hepatic veins with the inferior vena cava ●Thickening, irregularity, stenosis, or dilation of the walls of the hepatic veins ●A spider-web appearance in the vicinity of the hepatic vein ostia coupled with the absence of a normal hepatic vein ●A hyperechoic cord replacing a normal vein ●Abnormal flow patterns seen on Doppler imaging, including: •A large hepatic vein that appears void of flow-signal or has reversed or turbulent flow •Large intrahepatic or subcapsular collaterals with continuous flow connecting the hepatic veins or the diaphragmatic or intercostal veins •A hepatic wave form that is flat or lacks fluttering

  15. CTorMRIwithcontrast Delayed or absent filling of the three major hepatic veins (which are usually visible within 40 to 60 seconds after rapid intravenous contrast). ●A patchy, flea-bitten appearance of the liver due to increased central contrast enhancement relative to the periphery. ●Rapid clearance of contrast from the caudate lobe. ●Narrowing and/or lack of opacification of the inferior vena cava.

  16. Venography The gold standard for diagnosing Budd-Chiari syndrome is hepatic venography, which is performed by accessing the hepatic venous circulation percutaneously via the internal jugular vein, cephalic vein, or femoral vein. Venography should be performed if noninvasive tests are negative or nondiagnostic, but there is strong clinical suspicion for the disease

  17. Treatment Goals of therapy Treatment for Budd Chiari syndrome aims to: ●Prevent the propagation of the clot ●Restore patency of thrombosed veins ●Decompress the congested liver ●Prevent or manage complications

  18. Initial treatment includes: ●Correcting underlying disorders that predisposed to the development of Budd-Chiari syndrome (when possible) ●Initiating anticoagulation unless there are contraindications ●Treating complications of portal hypertension (if present) *Thrombolysis for patients with acute Budd-Chiari syndrome if a well-defined clot is present. (See 'Categorization' above.) *Angioplasty/stenting for symptomatic patients with venous obstructions that are amenable to angiographic treatment.

  19. If initial treatments fail, options include: ●TIPS placement or surgical shunting to decompress the liver ●Liver transplantation

  20. GoodbyefortheNextlecture......

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