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Budd chiari syndrome
- 1. BUDD CHIARI SYNDROME DRANANDO SENGUPTA DNB GASTRO 2ND YR 10/4/18
- 2. Introduction • Defined asobstruction of hepatic veins or terminal inferior vena cava (IVC) • Synonymous with Hepatic venous outflow obstruction Obliterative hepatocavopathy
- 3. Epidemiology • 0.2 and2 per 1 million population in general population • Incidence of BCS in Asia may be higher • 4.9% of all portal hypertension patients- Japan • 7-9% of all portal hypertension patients- India • Primary BCS - venous anomaly (Thrombosis, Webs, Endophlebitis • Secondary BCS - initial lesion outside the veins (Tumor, Abscess, Cysts)
- 7. Etiology Infections • Aspergillosis • Filariasis •Hydatid cysts (Echinococcus granulosus or E. multilocularis) • Liver abscess (amebic or pyogenic) • Pelvic cellulitis • Schistosomiasis • Syphilis • TB Malignancies • Adrenal carcinoma • Hepatocellular carcinoma • Leiomyosarcoma • Leukemia • Lung cancer • Myxoma • Renal carcinoma • Rhabdomyosarcoma
- 8. Etiology Miscellaneous • Behçet’s disease •Celiac disease • Dacarbazine therapy • IBD • Laparoscopic cholecystectomy • Membranous obstruction of the vena cava • Polycystic liver disease • Sarcoidosis • Trauma to abdomen or thorax
- 9. Membranous obstruction ofthe IVC • Commoner in east • Congenital or end result of acquired thrombosis (Recent Studies) • Japan, China and other parts of Asia, South Africa • India ( Khuroo & Datta, 1980 ) • Chronic course : Most pts present with fibrosis, cirrhosis, PHTN • Increased risk for HCC
- 10. Classification- Vessel involvement •Type I- IVC with or without hepatic veins occlusion • Type II- major hepatic veins • Type III- small centrilobular veins
- 12.
- 13. Pathophysiology ACUTE STAGE • Portalvein becomes draining vein • Increased hepatic arterial flow CHRONIC STAGE • Necrotic areas replaced by fibrotic tissue • Intrahepatic collateral vessels • Heterogeneous enhancement with a reticular pattern at the periphery of liver
- 14. Pathophysiology • Caudate lobehypertrophy (50%) • Portal vein obstruction - 10-20% • Asynchronous obstruction of hepatic veins Hepatic veins – 66% (Usually two are blocked for disease to be clinically evident) Isolated occlusion of IVC – 10% Combined occlusion – 23% Horton et al Liver International ISSN 1478-3223 , 2007
- 16. Clinical features SYMPTOMS • Abdominalpain & distention • Anorexia • Jaundice SIGNS • Massive ascites (83- 100%) • Hepatomegaly • Collaterals • Splenomegaly • Jaundice • Pedal edema • Wasting Orloff et al Ann Surg 2000
- 19. Clinical suspicion • Ascites,hepatomegaly and upper abdominal pain present simultaneously • FHF with liver enlargement and ascites • Liver disease in known case of prothrombotic disorder • Patients with CLD, but intractable ascites contrasts with mildly altered LFT. • May be few cases of idiopathic CLD.
- 20. Diagnosis • Clinical findings •Biochemical tests – LFT • Imaging – ULTRASOUND ABDOMEN WITH DOPPLER : FIRST STEP – COMPUTED TOMOGRAPHY (CT) – MAGNETIC RESONANCE IMAGING (MRI) – HEPATIC VENOGRAPHY • Infra and supra-hepatic caval pressure • Hyper-coagulable profile, Bone marrow biopsy • Liver Biopsy
- 21. LFT • Bilirubin rise– varying extent • Transaminase rise > 5 times - acute type • ALP rise – varying extent • Serum albumin decreases • High SAAG ascites > 1.1gm/dL
- 22. Doppler USG • Firstinvestigation of choice • Sensitivity & specificity > 85% • CE-USG : superior to Gray scale & CD for detection/characterisation of HV thrombosis • HV patency and size/IVC Thrombosis • Lack of visibility • Reversal of flow • Diffuse narrowing and irregularity • Transformation into a cord-like remnant • Collateral veins- hepatic/ extrahepatic
- 23. Limitations of sonography •Restriction from body habitus, • Intestinal gas or excessive ascites, • Failure to detect fresh thrombus in veins, • Failure to demonstrate patent veins within congested or shrunken cirrhotic liver • Failure to demonstrate retroperitoneal collaterals • Operator-dependency.
- 24. CECT Abdomen • Venousanatomy – patent IVC, PV, HV and non visualisation of HV. • Caudate lobe hypertrophy (Fan shaped enhancement) • Collaterals, Ascites • Liver- Necrotic areas Altered parenchymal perfusion pattern Early homogenous central enhancement Delayed patchy enhancement of periphery of liver Prolonged retention of contrast in the periphery Regenerative nodules • Not useful in showing web in the IVC
- 25. MRI • Particularly beneficialin non-diagnostic USG • Liver morphology and regional perfusional disorders : similar to CECT. • However, some parenchymal lesions such as Benign regeneration nodules (>10 in no,<4 cm & hypervascular), Hemorrhagic necrosis and Perfusion disorders : MRI better than CECT • Useful in differentiating regenerative nodules from HCC • MR Angiography- before selecting a treatment modality
- 26. Angiography and Venography Conventionalangiography • Gold standard • Pressure profile • Anatomy of block IVC venography • Infra-hepatic IVC To Right Atrium Pressure Gradient >15mm Hg - Mesoatrial shunt Hepatic venography/SMA venography • Occluded / narrow hepatic veins • Spider web pattern of venous collaterals • Wedge hepatic venous- IVC gradient >10mm Hg
- 27. Contemporary role ofVenography • TIPS placement, • Catheter-directed thrombolysis, • Mechanical thrombectomy • Balloon angioplasty • Recanalization of an occluded hepatic vein or vena cava with stent placement. • Transjugular liver biopsy. • Use of hepatic venography may be an essential guide and road map for surgical therapy in BCS Erdon, 2007 ; Kamath, 2006
- 28. Role of liverbiopsy • Parenchyma may be affected unevenly • Exclusion of cirrhosis or severe fibrosis • Feasibility of shunt • Differentiates veno-occlusive disease & cirrhosis of other origin Trans-jugular biopsy • Intense centrilobular & sinusoidal congestion, inflammation, cirrhosis and necrosis • Extravasation of red cells in space of Disse. • Limited value in assessment of severity and prognosis Tang T et al, J Hepatol 2001
- 29. Hyper Coagulable states: Workup 1) CBC, prothrombin level, APTT , fibrinogen 2) Red cell mass, plasma volume 3) Bone marrow biopsy, cell culture, karyotype 4) Anti-thrombin III assay 5) Protein-C Assay 6) S antigen assay 7) Lupus anticoagulant 8) Anticardiolipin antibodies 9) Ham’s acid hemolysis test 10) Activated protein –C (resistance and / or factor V Leiden mutation 11) JAK2 mutations : marker is the gain-of-function mutation V617F of the JAK2 gene 12) Plasma homocysteine level 13) β-HCG pregnancy screen 14) Endogenous erythroid colony assay 15) Flow cytometry for blood cells deficient in CD55 and CD59 (PNH) 16) Molecular test for G20210A Prothrombin gene mutation 17) Anti–β 2 -glycoprotein-1 antibodies
- 30. Management • Treatment dependson the cause, the anatomic characteristics and stage of liver disease, the pace of the disease • Underlying disorder can be found in 70% cases & multiple disorders can be present in 25% • Early relief of obstruction may reverse parenchymal abnormalities and improve survival • Remove the cause • Prevention of thrombosis extension • Relieve the high pressure & congestion in liver • Management of massive ascites
- 31. Management • Medical therapy •Minimal invasive interventions • Surgery (Shunt and Non Shunt Surgeries) • Liver transplantation
- 32. Medical Management • Anticoagulants(Heparin/ warfarin) • Systemic thrombolytic therapy • Management of ascites – Sodium restriction (<2gm/day) – Diuretic therapy – Therapeutic paracentesis • Management of hematologic disorders
- 33. Anticoagulants – Recommended routinely –Underlying prothrombotic state – Heparin/ warfarin – INR 2.0-3.0 – Improvement in prognosis Zeitoun G et al, Hepatology 1999 – No reports of severe bleeding Janssen et al, J Hepatol 2003
- 34. Systemic thrombolytic therapy/Local Thrombolytictherapy • No benefit Sharma et al, J Hepatol 2004 • 1/3rd of patients clinical response to treatment for periods of 2 months to 1 year. • Recent series from China, 12 or 13 patients had patent hepatic veins without recurrent thrombosis after a mean follow-up of 24 months. The one initial treatment failure was salvaged by repeat angioplasty Zhang et al, 2013 • Tissue plasminogen activator (tPA) is the direct thrombolytic of choice by catheter route
- 35. Medical therapy alone– Is it enough? • 12/14 died within 6 months McCarthy PM et al, Arch Surg 1985 • A complete response was achieved on medical therapy alone in 9 of 51 patients Plessier A et.al Hepatology 2006 • 8/20 significant improvement clinically and biochemically, 53% survival at 2 years Khuroo et al, J Gastroenterol Hepatol 2005
- 36. Minimal Invasive Interventions •Local thrombolytic therapy • Transluminal angioplasty • Endovascular stenting • TIPS
- 37. Local thrombolytic therapy •Catheter directed • Delivered just proximal or within thrombus • Overall success rate low • Risk of bleeding • Useful in patients with short history of thrombosis (acute disease) Sharma et al, J Hepatol 2004
- 38. Transluminal angioplasty alone •Earlier studies : High re-occlusion rates • 30 patients : Balloon angioplasty : successful in 28 ; restenosis occurred in 4. Kohli V, Nundy S Lancet 1993 • Long-term IVC patency has been achieved in more recent reports. (particularly with using stents) Srinivas et al, 2012 • Stents recommended with angioplasty Sharma et al, J Hepatol 2004
- 39. Angioplasty & Stenting •Retrospective study from China 115 patients 57% of the patients had membranous stenosis Success rate of stenting - 94% in IVC and 87% in HV Patency : 90% at 45 months Zhang CQ et al, World J Gastroenterol 2003 • Useful in- IVC webs, IVC stenosis, Focal HV stenosis, Post liver transplant patients
- 40. TIPS • Decompresses portalsystem • Covered stents- better results Indications- • Bridge to transplant in fulminant BCS • Acute form BCS • Sub acute form BCS with Porta-caval pressure gradient <10mmHg
- 41. • Largest reportedsystematic review and metaanalysis • 29 studies, 2255 patients • Restenosis rate at 1 year in the TIPS group was 12% • 1 year survival in TIPS group : 87.3% • 5 years survival in the TIPS group : 72.1% • OS for any interventional strategy was 92% at 1 year and 76% at 5 years. Zhang et al, 2015 Complications of TIPS • Procedure related mortality – 1-2% • Worsening of encephalopathy- 13-44% • Shunt dysfunction (Portal pressure gradient > 12mm Hg, decreased luminal shunt diameter) 18-78%
- 42. Surgical Management Decompressive surgicalshunts Pre-requisite – Reversible liver injury Indication – Technically difficult TIPS (massive thrombosis) – Porta-caval pressure gradient > 10 mm Hg Radical membrane excision Surgical Removal of Venous Obstruction Liver Transplant
- 44. Quality of lifeafter Surgical Portal decompression • Long-term shunt patency in 97-100%. • Hepatic sinusoidal decompression was maintained. • No ascites or need for diuretics. • Liver function and size returned to normal. • Variceal bleeding decreased • Vascular thrombosis was prevented (anticoagulants). • Encephalopathy did not occur when shunt was patent. • 94-100% returned to work/housekeeping. • 10 year survival was ≥ 91%. • Quality of life was excellent when performed early in the course of disease Orloff MJ et al, Annals of Surg 2000
- 45. Liver transplantation • Fulminantliver failure : rare • Chronic and progressive liver disease (poor liver Synthetic function) • Decompensated cirrhosis • Decompensation after shunt procedures • Shunt failure • Unshuntable portal hypertension : Thrombosis of PV, SV, SMV • Curative in protein C, S, antithrombin III deficiency and factor V leiden mutation Cruz E et al, Clin Transplant 2005 Tan HP et al, Liver Transpl 2000
- 46. Prognostic factors • Age •Severity of liver failure MELD/CTP score • Presence of refractory ascites • Serum creatinine Guy Zeitoun et al, Hepatology 1999 Valla DC et al, Semin Liv Dis 2002
- 47. Summary • Difference inwestern and eastern literature • Different sites and different pathology • Shunt, TIPS and liver transplant- Complimentary modalities • Treatment based on Presentation LFT Anatomy of block Available expertise
- 49. Eapen CE etal, Gut 2005