Budd chiari syndrome. ppt

ppt covering all aspects of BCS

1. KING GEORGE’S MEDICAL UNIVERITY LUCKNOW DEPARTMENT OF SURGICAL GASTROENTEROLOGY BUDD CHIARI SYNDROME Dr AMIT DANGI

2. PLAN PART A HISTORY DEFINITION ETIOLOGY PATHOGENESIS CLINICAL PRESENTATION DIFFERENTIAL DIAGNOSIS DIAGNOSIS IMAGING PART B MANAGEMENT MEDICAL THERAPY MINIMAL INVASIVE INTERVENTIONS (MII) SURGERY AND SHUNTS LIVER TRANSPLANTATION LONG TERM OUTCOME PROGNOSIS

3. HISTORY Lambron in 1842 : first case report George Budd (1845) Internist, King’s college Classic triad Abdominal pain Hepatomegaly Ascites G. Budd, London, 1845 Hans Chiari (1895) - Pathologist Histopathological features - obliterating endophlebitis of the hepatic veins Zeitschrift für Heilkunde, Prague, 1898

4. MORE THAN 8000 CASES HAVE BEEN REPORTED IN WORLD LITERATURE

5. History Synonymous Hepatic venous outflow obstruction Obliterative hepatocavopathy Okuda et al, Hepatology 1998 Rokitansky’s disease, von Rokitansky disease, membranous obstruction of the IVC, Hepatic vena cava disease, Budd­Chiari syndrome with occlusion of hepatic vein, or hepatic vein thrombosis Shin N et al . BCS review World J Hepatol 2016 June

6. Definition Budd-Chiari Syndrome Hepatic venous outflow obstruction at Hepatic venules Large hepatic veins Inferior vena cava Right atrium. Sinusoidal obstruction syndrome is excluded from this definition Shin N et al . BCS review World J Hepatol 2016 June European Group for the Study of Hepatic Vascular Diseases 0.2 and 2 per 1 million population in general population Valla, 2009 4.9% of all portal hypertension patients - Japan 7-9% of all portal hypertension patients – India

7. Demographic profile FEATURES WEST (Classical) EAST (HVC-BCS) Obstruction Hepatic veins (HV) (Classical BCS) Inferior vena cava (IVC) (Hepatic vena cava BCS) Sex Females Males Obstruction of IVC Thrombosis Membranous in Japan, Nepal IVC thrombosis in India Presentation Acute (60-85% < 6 months) Greater severity of symptoms Chronic (75-80%) Association Oral contraceptives Myeloproliferative disorders Pregnancy & infection Infection

8. Epidemiology of classical Budd- Chiari syndrome and hepatic vena cava-Budd Chiari syndrome Shin N et al . BCS review World J Hepatol 2016 June

9. a predisposing condition or etiology identified in 30 % of the patients. Parker in 1959 by turn of century , an underlying predisposing condition is identified in 70% of patients Mahmoud et al, 1996 Menon et al, 2004

10. ETIOLOGY– HYPERCOAGUABLE CAUSES Inherited Acquired Antithrombin III deficiency Myelo -proliferative Neoplasms : 53% in western population . POLYCYTHEMIA RUBRA VERA : MC (YOUNG ADULTS, 8.4-24%) ( Orloff and colleagues (2012) , 31% ) Essential thrombocytosis The JAK2V617­F mutation : 26%­-52% of patients with classical BCS Protein C deficiency Paroxysmal nocturnal hemoglobinuria Protein S deficiency Antiphospholipid syndrome Factor V Leiden mutation (MOST SEVERE AND ACUTE FORMS) 25% in western population 5-10 fold increase in the risk of thrombosis if they are heterozygotic and a 50-100 fold increase if they are homozygotic Cancer Prothrombin mutation Pregnancy (poor prognosis) Oral contraceptives

11. Etiology Uncommon causes Tumor invasion ( 8.8-13.4%) Miscellaneous Hepatocellular carcinoma (HCC) Aspergillosis Renal cell carcinoma (RCC) Behcet’s syndrome Adrenal carcinoma Inferior vena cava webs Leiomyosarcoma of IVC Trauma Inflammatory bowel disease Post liver transplant Idiopathic

12. ORAL CONTRACEPTIVES 9.4-25% After use of 2 weeks - 10 years more than 200 cases of BCS in patients taking OCs have been described Janssen et al, 2000 PREGNANCY & POSTPARTUM 9.9-15.2% Mitchell 1982, Khuroo & Dutta 1980 INFECTIONS 3 - 9.9% Amoebic liver abscess Hydatid cyst Schistosomiasis Syphilitic gumma Aspergillosis Filariasis . TRAUMA Blunt and penetrating

13. Connective tissue disorders Behcet's disease Sjogren’s syndrome Sarcoidosis Rheumatoid arthritis Orloff et al, 1999 Membranous obstruction of the IVC Commoner in east Congenital or end result of acquired thrombosis (RECENT STUDIES) More than 600 cases of BCS : Japan ( Okuda , 2002) ; China ( Wang, 1989 ); and other parts of Asia, India ( Khuroo & Datta , 1980 ), and South Africa Chrnic course : Most pts present with fibrosis, cirrhosis, PHTN Increased risk for HCC Okuda 1982, 1995, 2002

14. ETIOLOGICAL CLASSIFICATION Rossle M et al, Surgery 2004, Okuda K et al, J Gastroenterol hepatol 2002 Janssen HL, et alJ Hepatol 2003 ORIGIN EXAMPLES PRIMARY Endoluminal lesion Thrombosis Webs Endophlebitis SECONDARY Outside venous system Invasion or extrinsic compression Tumor Abscess Cysts

15. CLASSIFICATION- VESSEL INVOLVEMENT TYPE I TYPE II TYPE III IVC WITH OR WITHOUT HEPATIC VEINS OCCLUSION MAJOR HEPATIC VEINS SMALL CENTRILOBULAR VEINS

16. Obstruction characteristics: Location, type, and associated findings Shin N et al . BCS review World J Hepatol 2016 June

17. L arge geographic variations even in Asia in the prevalence of HVOTO with IVC , or combined IVC and HV blocks. NEPAL : A prospective survey spanning 3 years of admission to the Liver Unit of Bir Hospital in Kathmandu, disclosed that IVC OR COMBINED IVC BLOCKS accounted for 150 of 866 patients with liver disease (17.3%), PGI CHANDIGARH : As a comparison , it took 14 years to collect 91 cases of IVC or combined block in a Liver Unit at PGI Chandigarh; MADRAS MEDICAL COLLEGE : 13 years to collect 78 cases. AIIMS, NEW DELHI : Of 825 patients admitted for portal hypertension in the years 1978–1992, 51 (6.2%) had IVC or combined block.

18. PATHOPHYSIOLOGY

19. Liver injury becomes irreversible once there is Lobular collapse Diagnosis before lobular collapse will allow the sinusoids to be decompressed, with recoverable liver function Early diagnosis and treatment of sinusoidal congestion is important for good prognosis

20. PATHO-PHYSIOLOGY ACUTE STAGE Portal vein 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

21. Caudate lobe hypertrophy (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

22. CLINICAL PRESENTATION Fulminant Rare (7%) Acute Sub-acute Most common Chronic Onset < 2 months Up to 6 months 6 months- years Months to years Clinical features HE Sudden tense ascites, pain abdomen, hepatomegaly, no collaterals Resistant Ascites, hepatic & portal collaterals Features of chronic liver disease Trans-aminase Levels > 5 times Mild elevation Histo-logy No cirrhosis Little necrosis, no cirrhosis Severe fibrosis or cirrhosis Rossle M et al, Surgery 2004 Dilawari JB et al. Medicine 1994

23. LANGLET’S CLINICO-PATHOLOGICAL CLASSIFICATION Langlet et al, J hepatol 2003 Type-I Acute injury only 7% Best prognosis Type-II Chronic lesions only, sequelae of remote injury 45% Type-III Acute on chronic 48% Worst prognosis

24. Orloff and colleagues (2012) : In 77 patients 12 : advanced cirrhosis, and the remaining 65 patients were referred at a mean 14 weeks after onset of BCS (range, 4-78 weeks). Pavri et al, 2014 (University of Pennisylvania ) : M ost patients presented with advanced disease at diagnosis. 92 % exhibiting ascites and 55 % with cirrhosis Mitchell and colleagues (1982) , which excluded patients with MOVC, 2/3 rd : had symptoms for less than 3 months and 83 % had symptoms for 6 months or less at diagnosis. Parker (1959) : 133 patients, 57 % had symptoms for 3 months or less, and 71 % had been symptomatic for 6 months or less.

25. CLINICAL FEATURES SYMPTOMS ABDOMINAL PAIN & DISTENTION ANOREXIA JAUNDICE SIGNS MASSIVE ASCITES (83-100%) HEPATOMEGALY COLLATERALS SPLENOMEGALY JAUNDICE PEDAL EDEMA WASTING Orloff et al Ann Surg 2000 Young children (<10 years of age) account for 1–7% of all cases of BCS .

26. Signs and symptoms in classical Budd- Chiari syndrome and hepatic vena cava-Budd Chiari syndrome Shin N et al . BCS review World J Hepatol 2016 June

27. IVC OBSTRUCTION HV OBSTRUCTION Geography EAST WEST Etiology Membranous obstruction Hyper coaguable state History Long Short Sex Male Female Ankle edema Gross Absent Ascites Moderate Massive Collaterals Abdominal wall Absent Hepatomegaly Moderate/ cirrhosis Massive, tender Prognosis Good Worse

28. DIFFERENTIAL DIAGNOSIS Cardiac: Tricuspid regurgitation Constrictive pericarditis Right atrial myxoma Hepatitis Cholecystitis

29. 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

30. 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. KV Menon, NEJM 2004

31. LABS LFT Bilirubin rise – varying extent Transaminase rise > 5 times - acute type ALP rise – varying extent Serum albumin decreases High SAAG > 1.1gm/dL

32. IMAGING Role of imaging Site of obstruction Patency of HV, IVC, Portal vein (PV) Details of vascular anatomy Status of liver Caudate lobe Collaterals Differentiates forms of BCS Brancatelli G et al, Am J Roentgenol 2007

33. DOPPLER USG First investigation of choice Sensitivity & specificity > 85% Bolondi L et al,Gastroenterology 1991 CD & pulsed Doppler : complimentary to Gray Scale. CE-USG : superior to Gray scale & CD for detection/ characterisation of HV throbosis . Bolondi Gastroenterology 1991 HV patency and size/IVC Thrombosis Intrahepatic collaterals (90%) Prominent IRHV/ caudate vein (>2mm) The ability to demonstrate the reversal of flow direction may be the most important superiority of Doppler imaging in comparison to contrast-enhanced cross-sectional methods . Reversed flow direction in the main portal vein Caudate hypertrophy (Mean AP > 35 mm)

34. Thrombus in middle hepatic vein. (A) Color Doppler image shows no color signal in lumen (arrow), indicating obstruction of vessel. (B) In another patient, weak intraluminal color signals can be identified consistent with non-occlusive thrombus. Note reverse flow (flow towards to probe) in lumen which is represented by red color. Web in inferior vena cava. Sagittal sonogram of upper abdomen demon- strates echogenic web constricting inferior vena cava (IVC) at subdiaphragmatic level. Chronic thrombosis of right hepatic vein. Gray-scale sonogram shows right hepatic vein (RHV) as hyperechogenic cord-like structure (arrows).

35. Thrombus in middle hepatic vein. (A) Color Doppler image shows no color signal in lumen (arrow), indicating obstruction of vessel. (B) In another patient, weak intraluminal color signals can be identified consistent with non-occlusive thrombus. Note reverse flow (flow towards to probe) in lumen which is represented by red color. Web in inferior vena cava. Sagittal sonogram of upper abdomen demon- strates echogenic web constricting inferior vena cava (IVC) at subdiaphragmatic level. Chronic thrombosis of right hepatic vein. Gray-scale sonogram shows right hepatic vein (RHV) as hyperechogenic cord-like structure (arrows). Inferior RHV (A ) Duplex Doppler reveals enlarged IRHV lying behind right posterior PV branch. The Doppler tracing in IRHV has lost its normal triphasic appearance. (B) Postcontrast fat-suppressed in-phase gradient-echo MR image obtained in axial plane in same patient as in (A) shows IRHV (arrow) draining into IVC at level of porta hepatis . Also note, enlarged veins of vertebral plexus and veins of anterior and lateral abdominal wall . Caudate vein > 2 mm : BCS should be questioned. Intrahepatic collaterals present in 90% of cases

36. US can be used as an appropriate guide to treatment and for surveillance after TIPS placement. B-mode (A) and color Doppler (B) ultrasound images showing patent transjugular intrahepatic portosystemic shunt ( arrows ) with appropriate flow direction and velocity.

37. LIMITATIONS OF SON0GRAPHY R estriction from body habitus , Intestinal gas or excessive ascites, Failure to detect fresh thrombus in veins, Failure to demonstrate patent veins within congested or conversely, shrunken cirrhotic liver, Failure to demonstrate retroperitoneal collaterals unless they are hugely dilated, and Operator-dependency .

38. CROSS SECTIONAL IMAGING CT/MRI scan Venous anatomy – patent IVC, PV, HV and non visualisation of HV. Caudate lobe hypertrophy (Fan shaped enhancement) Collaterals Ascitis Liver The overall accuracy of CT for detecting thrombosis has been reported as 50% . Not found to be useful in showing web in the IVC 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 Ayse Erden European Journal of Radiology 2007

39. Inhomogeneous enhancement of liver. CECT reveals lobular and patchy areas of enhancement separated by linear and reticulated regions of relatively low density. This appearance is probably due to stagna - tion of sinusoidal flow. Also note wedge-shaped peripheral area of diminished attenuation in right lobe. Parenchymal changes in BCS CECT shows peripheral regions with low attenuation due to portal hypoperfusion (black arrows). Such areas are prone to atrophy. Dilated veins of caudate lobe (white arrows) can also be identified.

40. ACUTE BCS : sagittal (A) and coronal (B) computed tomographic images, with subtle thrombosis in the hepatic veins with nonopacification ( black arrow ) and a smooth liver contour. CHRONIC BCS : Prominent intrahepatic vessels can be seen (C) , ascitis , significant caudate hypertrophy ( D, white arrow ), and heterogeneous enhancement is seen with central hyper- and peripheral hypo-enhancement (E) .

41. MRI P reffered as a SECOND LINE OF INVESTIGATION Particularly beneficial in the evaluation of the patients with non-diagnostic ultrasonography L iver morphology and regional perfusional disorders : similar to CECT. However , some parenchymal lesions such as B enign regeneration nodules ( >10 in no,<4 cm & hypervascular ) Hemorrhagic necrosis and Perfusion disorders : characterized better with MR imaging than with a CECT U seful in differentiating regenerative nodules from HCC based on T2-weighted signal characteristics ( Brancatelli et al, 2007 )

42. Benign regenerative nodules. Coronal MIP image obtained at portal venous phase of MR angiography shows multiple enhanced nodules of varying size throughout liver. HCC in BCS : In-phase gradient-echo MR image obtained at late venous phase demonstrates HCC with mosaic pattern. Note delayed enhancement of its fibrous capsule (arrows) and hypointense thrombus material in peripheral branches of right hepatic vein (parallel arrows).

43. ENHANCEMENT PATTERNS ACUTE : B oth early and late gadolinium-enhanced MR images show diminished peripheral enhancement. SUBACUTE : heterogeneously increased enhancement within the liver periphery CHRONIC : Minimal enhancement differences (40% SHOW HOMOGENOUS ENHANCMENT) A trophy of the right lobe of the liver ( especially the peripheral regions), hypertrophy of the left lobe and the presence of regenerative nodules and contour irregularities show the progression to cirrhosis hypertrophy of caudate lobe is found in 60–87% of all cases

44. MULTIPHASIC STUDIES WITH MR ANGIOGRAPHY HA/PV system are examined along with HV-IVC with one injection of contrast material Narrowing , stretching or distortions of arterial and portal vessels : structural changes in parenchyma. In portal venous phase (second phase) of MRA, venous out flow obstruction can be inferred from the patchy enhancement of the liver. L ate venous phase imaging is necessary to visualize the patent HVs and intrahepatic venous collaterals. Hepatic veins with sluggish flow may be missed if inadequate delay time is used. L ater phases of examination : Dilution of intraluminal contrast l/t diminishes contrast difference between the vessels and parenchyma Thus thin collaterals seen on sonography may be inconspicuous at MR angiography.

45. LOCATIONS OF EXTRA- HEPATIC COLLATERAL VEINS IN BCS : Different from those seen in cirrhosis. 4 groups mainly in RP Deep and central tributaries of the systemic circulation Ascending lumber veins, V ertabral venous plexus, A zygos and H emiazygos veins) are the most frequent collateralized routes. These vessels can be observed on MR angiography and CT in 36% and 32% of the cases, respectively O ther collateral vessels : Left renal- hemiazygos pathway and inferior phrenic- pericardiophrenic veins Collateral veins in BCS. (A) Coronal MR image shows (IVC) occluded in its hepatic portion. IRHV is prominent as an intrahepatic collateral whereas azygos vein (AV), hemiazygos vein (HV) and left inferior phrenic vein (LIPV) are noted as extrahepatic collateral vessels. A : aorta, SV: splenic vein, LRV: left renal vein, and SMV: superior mesenteric vein. (B) Inferior venacavogram confirms occlusion in IVC and presence of IRHV and LIPV as collaterals.

46. ADVANTAGES OF MRA OVER DOPPLER N o restriction from body habitus and intestinal gas; Short examination time; No operator-dependency; Better anatomic orientation, and Easy documentation of surgical portacaval shunt patency and portal venous system. It also offers the possibility to evaluate the enhancement patterns of liver parenchyma.

47. Before selecting a treatment modality, the MR angiography as a contrast-enhanced method is advantageous over the other techniques Aysye Erden . European Journal of Radiology 61 (2007)

48. ANGIOGRAPHY AND VENOGRAPHY CONVENTIONAL ANGIOGRAPHY Gold standard Pressure profile Anatomy of block IVC VENOGRAPHY Gradient >15mm Hg - Mesoatrial shunt HEPATIC VENOGRAPHY/SMA VENOGRAPGY Occluded / narrow hepatic veins Spider web pattern of venous collaterals Wedge hepatic venous- IVC gradient >10mm Hg

49. Venogram of the IVC demonstrates compression of the intrahepatic portion of the cava ( A, black arrows ) during placement of a TIPS ( single black arrow ). Most often, one of the occluded veins can be cannulated , often showing thrombus ( B, black arrow ). Further injection after accessing the hepatic vein may demonstrate the classic “ spiderweb ” pattern of small, intrahepatic venous collaterals (C)

50. CONTEMPORARY ROLE OF VENOGRAPHY Platform for interventional radiology procedures. TIPS placement, Catheter-directed thrombolysis, Mechanical thrombectomy and Balloon angioplasty, and Recanalization of an occluded hepatic vein or vena cava with stent placement. T ransjugular liver biopsy. Use of hepatic venography may be an essential guide and road map for surgical therapy in BCS Erdon , 2007 ; Kamath, 2006

51. ROLE OF LIVER BIOPSY Need? 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 Features 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

52. Liver biopsy

53. HYPER COAGULABLE STATES : WORKUP CBC, prothrombin level, APTT , fibrinogen Red cell mass, plasma volume Bone marrow biopsy, cell culture, karyotype Anti-thrombin ill assay Protein-C Assay S antigen assay Lupus anticoagulant Anticardiolipin antibodies Ham’s acid hemolysis test Activated protein –C (resistance and / or factor V Leiden mutation JAK2 mutations : marker is the gain-of-function mutation V617F of the JAK2 gene 12 . Plasma homocysteine level β-HCG pregnancy screen Endogenous erythroid colony assay Flow cytometry for blood cells deficient in CD55 and CD59 (PNH) Molecular test for G20210A prothrombin gene mutation Anti–β 2 -glycoprotein-1 antibodies Baxter et al, 2005 Mahmoud and Elias ( 1996) Hirschberg et al, 2000 ; Valla, 2009 )

54. PART II MANAGEMENT OF BCS

55. MANAGEMENT Treatment depends on the cause the anatomic characteristics and stage of liver disease the pace of the disease

56. Underlying disorder can be found in 70% cases & multiple disorders can be present in 25% Early relief of obstruction may reverse parenchymal abnormalities and improves survival Remove the cause Prevention of thrombosis extension Relieve the high pressure & congestion in liver Management of massive ascites

57. MANAGEMENT OPTIONS Medical therapy Minimal invasive interventions (MII) Surgery (SHUNT AND NON SHUNT SURGERIES) Liver transplantation

58. Is this the sequence? Anticoagulation Angioplasty & stenting Shunt (TIPS or surgical) Liver transplantation Plessier A et.al Hepatology 2006 Seijo S , Hepatology . 2013 May .

59. MEDICAL MANAGEMENT Objectives remove the cause of the venous thrombosis, relieve the high pressure and congestion within the liver, prevent extension of the venous thrombosis, and reverse the massive ascites.

60. MEDICAL MANAGEMENT Anticoagulants (Heparin/ warfarin) Systemic thrombolytic therapy Management of ascites Sodium restriction (<2gm/day) Diuretic therapy Therapeutic paracentesis Management of hematologic disorders

61. Anticoagulants Recommended routinely Underlying prothrombotic state Heparin/ warfarin Improvement in prognosis Zeitoun G et al, Hepatology 1999 No reports of severe bleeding Janssen et al, J Hepatol 2003 Systemic thrombolytic therapy/Local Thrombolytic therapy No benefit Sharma et al, J Hepatol 2004 1/3 rd of patients were believed to have had a 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 T issue plasminogen activator ( tPA ) is the direct thrombolytic of choice by catheter route

62. PCV : Hydroxyurea , (should be started as soon as the disease is discovered and continued for life). Other treaments : serial phlebotomy, anagrelide , interferon alfa-2b , and ruxolitinib . Whatever treatment regimen is used, the disease runs a benign course if treated early. Dramatic responses to intravenous (IV) heparin therapy have been reported, although relapses have been common. Hartmann et al, 1980 PNH : Eculizumab

63. 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

64. Useful Acute form Partial venous occlusion Mild symptoms Mild ascites No ongoing hepatic necrosis Relatively normal LFT Major co-morbidities Menon KV et al, N Engl J Med 2004

65. MINIMAL INVASIVE INTERVENTIONS Local thrombolytic therapy Transluminal angioplasty Endovascular stenting TIPS

66. Local thrombolytic therapy Catheter directed Delivered just proximal or within thrombus Overall success rate low Risk of bleeding Useful in patients Short history of thrombosis (acute disease) Sharma et al, J Hepatol 2004

67. 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

68. 75 patients (Between 1978 and 1992). HV OBSTRUCTION IN 24, IVC OBSTRUCTION in 44, BOTH in 7. 7 PATIENTS : PSRS 2 deaths postoperatively); 4 shunts blocked and only 1 patient became completely symptom free. 2 patients ( partial obstruction): balloon dilatation of the right HV but within 6 months the obstruction recurred. 6 patients : SSPCS 2 died of HE after discharge and 4 are alive and well. 14 pts : Surgical procedures yielding poor results. 30 patients : Balloon angioplasty : successful in 28 ; restenosis occurred in 4. Of the 7 patients with a combined block, 3 have had balloon angioplasty followed by a SSPCS; 1 died, 2 are well, and the remainder have not completed treatment. HV OCCLUSION ( 24) IVC OCCLUSION (44)

69. ANGIOPLASTY & STENTING Retrospective study from China 115 patients 5 7 % 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 Aucejo F et al, Liver Transpl 2006 Wang SL et al, Radiology 2005

70. TIPS TIPS –no prospective trial Decompresses portal system First report – 1993 Benefit Bridge to transplantation Prolonged transplant free survival Cejnu M J et al, Vasc Interv Radiol 2002 Collaterals formation, may be asymptomatic even after stenosis Klein AS et al, Liver transplant 2003

71. Outcome of TIPS ROSSLE M ET AL. SURGERY 2004 Recent larger study 35 patients with BCS (Child 9.2 + 1.9), 11 having cirrhosis (11 acute, 13 subacute , and 11 chronic) Successful TIPS in 33/35 5 year survival : 74% Shunt failure : 7 patients (20%) : 2 (technical), 2 (LT), 3 deaths Transplant free survival 1 year 93% 5 year 74% ROSSLE ET AL, SURGERY 2004 Special issues of TIPS in BCS Technical problem if hepatic vein lumens are completely blocked. Anticoagulation

72. 61 patients TIPS and/or stenting 40 patients required repeated interventions Eapen CE et al, Gut 2006 11 patients No mortality/ major morbidity 73% decrease in pressure gradient, 57% patency rate Required multiple interventions Molmenti et al, Ann Surg 2005 Interpretation of different studies are not comparable Membranous obstruction in Asian population Technology improving day by day

73. Mancuso and associates (2003) 15 patients, 5 deaths and 1 technical failure, 40 % negative outcome. Cejna and colleagues (2002) : 8 patients 2 (25%) died 2 weeks after TIPS, 1 developed TIPS occlusion requiring LT, and 3 others required 2-7 revisions for TIPS stenosis. Only 2 of 8 (25%) had revision-free TIPS patency. Perello and colleagues (2002) : 13 patients 3 shunt failures (23 %) : 1 death, 1 TIPS thrombosis that necessitated a surgical shunt, and 1 patient who required LT. 70% experienced TIPS occlusion.

74. Covered stents Better results European series : 112 pts (17 with BCS) underwent TIPS with covered stents 1 yr failure rate : 10% M ortality rate without and with the patients lost to follow- up was 20% and 30 % respectively Ro ̈ssle and colleagues et al 2006 Two series, 34 patients, PTFE covered stents Improved patency Less dysfunction Hernandez et al, Hepatology 2004 Gandini R et al, Radiology 2006

75. Primary patency : 75% in larger series, Secondary patency of 99% at a mean follow-up of 82 months 72% survival at 10 years.

76. In a multicenter review conducted by Garcia-Pagán and colleagues (2008) , 221 patients with BCS 147 eligible for TIPS 124 (60%) underwent TIPS (14 had technical C/I , 9 failed TIPS) 22 had TIPS related complications including 2 deaths 61 (41%) had TIPS dysfunction : Restenting in 35 Angioplasty in 20 Thrombolysis in 6 patients HE developed in 21% of patients within 1 year 16 (13%) deaths in F/U 8 (6.5%)required LT Actuarial LT-free survival at 1, 5, and 10 years was 88%, 78%, and 69 %, respectively .

77. L argest reported systematic review and meta-analysis 29 studies 2255 patients treated with percutaneous techniques 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 .

78. Indications Bridge to transplant in fulminant Acute form BCS Sub acute form BCS with Porta- caval pressure gradient <10mmHg Menon et al, NEJM 2004 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%, Mostly in 1 st year

79. SURGICAL MANAGEMENT Surgical Options Decompressive surgical shunts Radical membrane excision Surgical Removal of Venous Obstruction LIVER TRANSPLANT

80. SURGICAL SHUNTS Pre-requisite Reversible liver injury Indication Technically difficult TIPS (massive thrombosis) Porta-caval pressure gradient > 10 mm Hg

81. Difficult to compare different groups Surgical vs. medical or minimally invasive techniques Retrospective Significant time bias Sick patients usually managed medically No prospective studies No/ small control group Halff G et al, Ann Surg 1990 - BECAUSE OF THE RARITY OF BCS : NO RANDOMIZED PROSPECTIVE TRIALS COMPARING MEDICAL WITH SURGICAL TREATMENT OF THIS DISEASE Langlet P . Acta Gastro- enterol Belg 2002

82. F rom Orloff MJ, et al: Budd- Chiari syndrome revisited: 38 years' experience with surgical portal decompression. J Gastrointest Surg 16:286-300, 2012. O perative experience reported by Orloff and colleagues (2012)

83. SHUNT OPTIONS Normal IVC (Hepatic Vein Occlusion) Side to side portacaval Shunt (SSPCS) Mesocaval shunt (MCS) Splenocaval shunt (SCS) Proximal splenorenal shunt (PSRS) Distal splenorenal shunt (DSRS)

84. IVC OCCLUSION INFRA-HEPATIC IVC TO RIGHT ATRIUM PRESSURE GRADIENT > 20MM HG Meso -atrial shunts (MAS) Cavo-atrial shunt with SSPCS IVC stenting before PCS/ MCS Cavocaval transflow Splenoatrial shunt Splenojugular shunt Mesojugular shunt

85. Portacaval shunt Mesocaval shunt Mesoatrial shunt Cavoatrial shunt

86. SSPCS Difficult in presence of caudate hypertrophy Makes future liver transplant difficult 31/32 patients alive, good liver functions 3.5-27 years follow up Orloff MJ et al, Ann Surg 2000 Results – not replicated Klein AS, Liver Transpl 2006

87. Valveless PV can act as an outflow tract l/t sinusoidal decompression. Side to Side anastomosis between PV & IVC Shunt patency depends on the pressure gradient between the high pressure portal system & the low pressure IVC Best patency rates Shunt blockage rate is -- 3% at 13 years

88. Measurement of pressures in the I VC and PV with a saline (spinal) manometer by direct needle puncture before performance of portacaval anastomosis. All portal pressures are corrected by subtracting the IVC pressure from the portal pressure. Pressures in the IVC and PV are measured again after completion of the shunt. B, IVC pressure . C, Free portal pressure. D , Hepatic occluded portal pressure, obtained on the hepatic side of a clamp occluding the portal vein. E , Splanchnic occluded portal pressure, obtained on the intestinal side of a clamp occluding the portal vein. Pressure gradient at the end of surgery of > 50 mm of saline is unacceptable and revision may be required

89. PROBLEMS WITH PCS Portal vein thrombosis is an obvious contraindication Dissection at porta- subsequent technical difficulty at transplant Fulminant liver failure and decompensated cirrhosis- Liver transplant

90. Decision making in shunt Sx Documentation of ongoing necrosis Demonstration of intact lobular architecture Status of infrahepatic IVC Status of PV - 20% have associated PVT Status of Caudate lobe DAMAGED LIVER WITH NO LOBULAR COLLAPSE : SHUNT SX SEVERELY DAMAGED LIVER WITH LOBULAR COLLAPSE AND FIBROSIS : LT

91. BLEEDING BEST MANAGED WITH PRESSURE DECOMPRESS PORTAL SYSTEM AS FAST AS POSSIBLE RESECTION OF THE ENLARGED CAUDATE LOBE IS HAZARDOUS PRESSURE GRADIENT AT THE END OF SURGERY OF > 50 MM OF SALINE IS UNACCEPTABLE AND REVISION MAY BE REQUIRED

92. Preop 6weeks post PCS

93. MESOATRIAL SHUNT Higher thrombosis rate : 33% at 5 years Synthetic Dacron or Gore-Tex grafts ranging from 14 to 20 mm in diameter Using autologous internal jugular vein IN MCS ; Patency equal to PCS Easier to manage during future LT Technically easier in caudate hypertrophy In case with infrahepatic IVC obstruction Klein AS, Liver Transpl 2006

94. Long term results of MESO ATRIAL shunt in 8 pts with BCS OPERATIVE MORTALITY 10 AND 15 YEAR MORTALITY OCCLUSION OF SHUNT ASCITES NEED FOR DIURETICS ABNORMAL LIVER FUNCTION TESTS ENCEPHALOPATHY WORKING FULL-TIME % of group 63 63 63 63 63 38 25 UNSATISFACTORY : 63% MORTAILITY FROM LIVER FAILURE d/t SHUNT THROBOSIS : ABANDONED Orloff MJ, et al Arch surg 1992

95. RESULTS OF MESOATRIAL SHUNT IN BCS DUE TO IVC OCCLUSION AUTHOR CASES F/U ( MONTHS SURVIVAL GRAFT PATENCY % STRINGER (1989) 5 9-16 100 100% WANG ( CHINA – 89) 32 2-66 88 ? HENDERNESS - 90 9 1-47 67 33 KLEIN - 90 16 2-80 37 50 ORLOFF 98 8 12-120 37 37 KHANNA (PGI CHANDIGARH – 1992) 13 2- 7.5 YEARS 62% BEHERA (PGI CHANDIGARH -2001) 10 6-71 MONTHS 90% 100% Survival 1 year – 90.7% 3 year – 77.1% 5 year – 61.1% Slakey DP et al, Ann Surg 2001 Wang ZG et al, ANZ J Surg 2005

96. SCS 72 patients 26 SCS, 46 MCS Similar efficacy in decreasing portal pressure, decreasing bleed Dang XW et al,, Hepatobiliary Pancreat Dis Int. 2005

97. COMBINED PCS AND CAS

98. RESULTS OF COMBINED PCS-CAS FOR BCS DUE TO IVC OCCLUSION IN 12 PATIENTS FOLLOWED UP 5-22 YEARS % ASCITES 0 NEED FOR DIURETICS 0 NORMAL LIVER FUNCTION 100 ENCEPHALOPATHY 0 PATENT SHUNT 100 SURVIVAL 5-22 YEARS 100 Orloff MJ, et al Arch surg 1992

99. Cavo-atrial shunt Survival 1 year – 97.2% 3 year – 86% 5 year – 79.8% Wang ZG et al, ANZ J Surg 2005 If done along with SSPCS 10/10 alive at 4-16 years follow up Orloff MJ et al, Ann Surg 2000

100. SURGICAL RESULTS Success rates of portal decompression in BCS after direct SSPCS : 85 % to 97% ; Hemming et al, 1996 ; ; Orloff et al, 1992 67 % success rate after splenorenal shunt Ahn et al, 1987 ; McCarthy et al, 1985 Mesocaval or portacaval interposition grafts with autologous internal jugular vein : success rate of 89% Bismuth & Sherlock, 1991 Panis et al, 1994 Mesocaval or portocaval interposition grafts using synthetic materials, such as Dacron or PTFE (Gore-Tex ), have been successful in approximately 52% of 39 patients with BCS. Hemming et al, 1996 ; Henderson et al, 1990

101. From Orloff MJ, et al: Budd- Chiari syndrome revisited: 38 years' experience with surgical portal decompression. J Gastrointest Surg 16:286-300 , 2012.)

102. From Orloff MJ, et al: Budd- Chiari syndrome revisited: 38 years' experience with surgical portal decompression. J Gastrointest Surg 16:286-300 , 2012.) LIVER BIOPSIES AND ANGIOGRAPHIC OR US STUDIES WERE PERFORMED PERIODICALLY FOR 37 YEARS AFTER SURGICAL SHUNT THERAPY IN THE 38 SURVIVORS. CIRRHOSIS PERSISTED IN THREE PATIENTS WHO HAD ESTABLISHED CIRRHOSIS AT THE SHUNT OPERATION, INCLUDING ONE PATIENT WITH BEHÇET'S DISEASE. IN 97% OF THE SURVIVORS : NO EVIDENCE OF HEPATIC CONGESTION OR NECROSIS WAS FOUND. MILD TO MODERATE FIBROSIS WAS FOUND IN 42% OF PTS. 50% HAD NORMAL LIVER BIOPSY SPECIMENS

103. QUALITY OF LIFE AFTER SURGICAL PORTAL DECOMPRESSION IN BCS 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

104. PREDISPOSING CONDITIONS WERE CONTROLLED. VASCULAR THROMBOSIS WAS PREVENTED (ANTICOAGULANTS). ENCEPHALOPATHY DID NOT OCCUR WHEN SHUNT WAS PATENT. 94-100% RETURNED TO WORK/HOUSEKEEPING. 10 YEAR SURVIVAL WAS ≥ 91 %. Orloff MJ et al, Annals of Surg 2000

105. QUALITY OF LIFE WAS EXCELLENT WHEN PERFORMED EARLY IN THE COURSE OF BCS. Orloff MJ et al, Annals of Surg 2000

106. RESULTS OF LIVER TRANSPLANTATION AFTER SHUNT SURGERY 15 patient case series Worse outcome in patients undergoing PCS Distal splenorenal shunt better in younger patients Brems JJ et al, Ann Surg 1989 58 patient case series (PSRS,DSRS,SSPCS, MCS) DSRS & MCS - better in younger patients, more patent Brems JJ et al, Ann Surg 1989

107. ISSUES Limited technical expertise Peri-operative mortality – 0-50% Langlet Pet al, Acta Gastroenterol Belg 2002 Long term survival – up to 90% (5-14 years) Zimmerman MA et al, Clin Liver Dis 2006

108. MANAGEMENT : NON-SHUNT SURGICAL OPTIONS Transcardiac membranotomy : More than 125 cases have been described with success rate if 70-90 % after brief follow up ( 2-84 months) Splenopneumopexy - P ortopulmonary shunt ( induce collateral circulation between the portal system and the pulmonary veins) Kimura’s finger rupture Foley’s tube dilatation Endovenotomy : Radical membrane excision 12 attempts, 5 success, 7 failures, 3 deaths Surgical Removal of Venous Obstruction. Peritoneovenous Shunt

109. Senning operation D irect method of removing obstruction of the IVC and HV in patients with chronic BCS. Reconstructing the hepatic outflow tract by suturing the RA to the liver capsule 17 patients: 6 deaths within 2 weeks of operation & 4 later. Actuarial 1 year and 3 year survival rates were 76% and 57%, respectively; LT was required for two patients, and three developed recurrent thrombosis. During follow-up of 7 months to 11 years, 10 (67%) of the 15 early survivors had prolonged relief of BCS. Senning (1987) Pasic et al, 1993 Successful in 5 of 7 during follow-up of 2 months to 5 years, Kawashima and colleagues (1991 )

110. SURGICAL OPTIONS BASED ON XU’S CLASSIFICATION OF BCS Type IVC MHV Treatment of choice Ia Membranous obstruction without thrombus Patent/ partly patent Foley’s tube dilation Ib Membranous obstruction with thrombus Patent/ partly patent Radical membrane resection, thrombus extraction II Segmentally narrowed Segmentally obstructed Shunt surgery, splenopneumopexy may be if splenomegaly IIIa <2cm obstruction Obstructed Foley’s dilation, redical excision, IVC tranflow IIIb >2cm obstruction Obstructed Splenojugular,splenoatrial, cavoatrial-mesoaval shunt IV Superior vena cava narrowing/obstruction Xu PQ et al, Hepatobiliary pancreat dis int 2004

111. LIVER TRANSPLANTATION 1974 – first successful Putnam CW et al, JAMA 1976 ~ 20 case series studies 1988-2006 Two national registry analysis European liver transplant registry (248 patients) Adam R et al, Liver Transpl 2003 United Network for Organ Sharing, UNOS : 510 patients Segev DL et al, Liver Transpl 2007

112. A search of the literature indicated that more than 1000 patients with BCS have undergone liver transplantation. AMONG THEM 30 patients with BCS underwent LDLT; all were from Asian countries. Akamatsu N, Sugawara Y, Kokudo N. Budd- Chiari syndrome and liver transplantation. Intractable & Rare Diseases Research . 2015;4(1):24-32. doi:10.5582/irdr.2014.01031

113. INDICATIONS OF LT IN BCS FULMINANT LIVER FAILURE (UNOS IA) : RARE CHRONIC AND PROGRESSIVE LIVER DISEASE (POOR LIVER SYNTHETIC FUNCTION) Reasonable prediction that the patient will die within 1 year—the most common indication for LT and the same used widely in other liver diseases Scharschmidt , 1984 ; Schenker , 1984 Decompensated cirrhosis Decompensation after shunt procedures Shunt failure Unshuntable portal hypertension : Thrombosis of PV, SV, SMV : only if blood vessels are available to vascularize the liverm 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 Compared with surgical shunt LT patients have better synthetic functions. Shaked A et al, Surg Gynecol Obstet 1992

114. REFERENCE NUMBER OF PATIENTS INDIACATION OF LT SHRINIWASAN et 2002 19 FAILED SHUNT, 5 ACUTE LIVER FAILURE, 6 CHRONIC LIVER FAILURE, 8 HEMMING ET AL, 1996 10 FAILED SHUNT, 3 ADVANCED CIRRHOSIS, 4 IVC OBSTRUCTION, 2 RINGE ET AL, 1995 43 UNCLEAR 39 FAILED 4 GALATI ET AL, 1993 32 FAILED 2 HALFF ET AL, 1990 ; IWATSUKI ET AL, 1991 FAILED 5 INTRACTABLE ASCITIS 15 RECURENT VARICEAL BLEEDING 15 HE 15 MENTHA ET AL 2008 248 FHF 47 EUROPEAN LIVER TRANSPLANTATION REGISTRY, 1988-1999 RF 40, PVT 47 FAILED PSS 49 FAILED TIPS 11 FAILED PERCUTANEOS ANGIOPLASTY 18 SEGEV ET AL, 2007 (UNOS REGISTRY, 1987-2006) 510 NOT REPORTED YET Indications for Liver Transplantation in 15 Retrospective Series of Budd- Chiari Syndrome (BCS) Reported in the Literature

115. Liver transplantation for BCS Segev et al – (1987-2006), 510 patients 3 time periods : historical (1987–1997), pre-MELD (1998–2002) and MELD (2002–2006 ), 100 patient from 2002-2006 (MELD ERA) Better graft & patient survival (MELD era) Independent factors for Graft loss & death Life support Prior transplantation Prolonged ischemia time No effect of prior TIPS on final outcome 3 years survival – 85% Underlying etiology not documented in UNOS Segev DL et al, Liver Transpl 2007

116. European experience Adam – European data (1968-2001) 391 patients ( European Liver Transplant Registry : ELTR ) BCS – 1% of all transplants 1 year survival – 73% 5 year survival – 68% 10 year survival – 63% Overall 1-, 5- and 10-year patient survival for all indications during this time period was 83%, 71% and 63% respectively Adam R et al, Liver Transpl 2003

117. Mentha et al 256 patients undergoing LT for BCS in Europe. Pretransplant predictors of mortality Impaired renal function History of surgical shunt and TIPS Mentha G et al, J Hepatol 2006 Yamada et al 9 patients – Living donor liver transplantation 1 year survival – 88% 3 year survival – 71% Yamada T et al, J Hepatol 2006

118. Management after LT Lifelong anticoagulation Heparin, 7500-30000U/day followed by warfarin Janssen HL et al, J Hepatol 2003 Myeloproliferative disorders Hydroxyurea, aspirin, anagrelide Chance of bleeding – 11-44% Mentha G et al, J Hepatol 2006 Recurrence – 27% May require re-transplantation Cruz E et al, Clin Transplant 2005

119. R ecurrence of BCS in the transplanted liver

120. BCS following LT with the piggyback technique is a well-described complication. R eported incidence of 1–7 % ( more common after the piggyback technique). However , two recent retrospective studies were unable to detect a difference in the rate of BCS between the piggyback technique and the standard technique. Usually presents in the early postoperative period . (25% after first week) Technical factors : inadequate graft size and use of two hepatic veins for the venous anastomosis. The rate of BCS after construction of the venous anastomosis with two veins is 2.3% while the use of three veins decreases the rate to 0.7%. C onventional piggy back technique vs the side-to-side variation of the piggyback technique : 2.4 % vs. 0.7% respectively incidence of BCS. Horton et al Liver International ISSN 1478-3223 , 2007

121. Issues with LT Allocation problem Shortage of donors Takes organ away from the patients, whose only chance is liver transplant Long wait times Unpredictable availability of donors Inaccurate MELD score- INR while on warfarin Technical Haemostasis Large caudate lobe- difficult mobilization Diffuse retroperitoneal fibrotic reaction – difficult IVC control Thick, narrow, thrombosed portal vein Other Life long immunosuppression High cost

122. Survival After LT for BCS Reported in the Literature

123. Horton et al. BCS review. Liver International 2007

124. SSPCS and LT are not competing forms of treatment. early and middle stages of BCS : sspcs late stages of BCS, when the liver disease is no longer reversible, and when stabilization of progressive hepatic decompensation is impossible : Olt Most patients who are candidates for LT should be in CTP class C.

125. Prognosis Prognosis depends upon Age Severity of liver failure Presence of refractory ascites Serum creatinine Guy Zeitoun et al, Hepatology 1999 Good prognosis Younger patients with low CTP score Absent or easily controlled ascites Low serum creatinine Valla DC et al, Semin Liv Dis 2002

126. Khuroo analysed 47 consecutive patients with BCS and found the following factors to adversely affect survival: florid clinical presentation, male sex, no TIPS performed and CTP score. Khuroo et al, J Gastroenterol Hepatol 2005 ;

127. Langlet prognostic index (PI) ascites score, Pugh score, age , creatinine and type III presentation (acute on chronic lesions) unable to show a survival benefit to surgical shunts whether performed early (within 2 months after diagnosis) or not.

128. Murad reported the results of an international multi-institutional study. Number of patients : 237 patients (205 included in multivariate analysis) treated with a variety of modalities. T ransplant- free survival rates of 82%, 69% and 62% at 1, 5 and 10 years respectively. The following factors were found by multivariate analysis to be independent predictors of 5-year transplant-free survival: presence of ascites, presence of encephalopathy, INR and bilirubin . MURAD’S BUDD–CHIARI SYNDROME PROGNOSTIC CLASSIFICATION Mural et al. Hepatology 2004

129. The 5-year transplant-free survival was 89%, 74% and 42% for class I, class II and class III respectively. A possible weakness of this study is that it excluded patients who underwent transplant.

130. The Rotterdam score : Excellent in predicting intervention-free survival, and no other variable could significantly improve its prognostic ability. Rotterdam score is the best discrimination index for 3-month mortality in BCS and should be used to determine treatment urgency. Montano- Loza AJ et al. Aliment Pharmacol Ther . 2009 Nov BCS-TIPS prognostic index (PI) score (based on international normalized ratio, Bilirubin , and A ge ) : Strongly associated with survival and had a discriminative capacity, which was superior to the Rotterdam score Seijo S, Hepatology . 2013

131. SUMMARY Difference in western 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

132. Eapen CE et al, Gut 2005

133. Which is the best surgery for Budd- Chiari syndrome: venous decompression or liver transplantation? A single-center experience with 50 patients. Ringe B , Lang H , Klinik fur Abdominal- und Transplantations, Hennover , Germany. The optimal treatment of BCS is still controversial whether venous decompression or LT is superior. 50 patients treated between 1981 and 1993 was retrospectively analyzed . 12 pts : PSS or local decompressive procedures, 43 cases(38+5 ) : LT , including 5 with previous conventional surgery. The overall mortality of 18 of 50 was concentrated within the early postoperative period, with no patient lost after 1 year. In the venous decompression group, the success rate was only 29%, and treatment failure was closely related to the finding of cirrhosis or technical problems like vascular thrombosis . After LT, early complications were rejection, primary non function, or graft necrosis, and contributed significantly to the risk of sepsis. 30 of 43 liver recipients are currently alive, including 4 rescued after failed decompressive surgery, with 1- and 10-year survival of 69%, and excellent recurrence-free rehabilitation. Conclusion P atient selection plays a dominant prognostic role in the treatment of BCS.

134. Portosystemic Shunt Versus OLT For BCS Shaked A , , Department Of Surgery, University Of California, LA 22 Patients Of BCS. Underlying Cause Of BCS : Myeloproliferative Disorders Or The Use Of Birth Control Pills In 18 Of 22 Patients. Liver Biopsy: Centrilobular Congestion And Necrosis : PSS And Fibrosis And Cirrhosis : OLT Indications For PSS : Symptoms Related To PHTN Only And Well-preserved Synthetic Hepatic Function. 10 Patients : 12 Shunt Procedures , Including MAS (8 Patients) & SSPCS (4 Patients). Complications After PSS : FHF And Progressive Hepatic Failure (1 PATIENT EACH) : URGENT OLT One Death Occurred Because Of Pulmonary Sepsis. Indications For OLT (14 PATIENTS) : ESLD Severe PHTN And Variceal Bleeding (4 Of 14 Patients), Progressive HE (7 Of 14 Patients) And Poor Synthetic Function : Bilirubin > 3 MG/DL In Eight Of 14 Patients Albumin < 3.0 GM/L, Or Both, In Ten Of 14 Patients). 14 Patients Were Treated With 16 OLT Three Patients Had Retransplantation For Primary Nonfunction Graft (Two Of 14 Patients) Or Chronic Rejection (One Of 14 Patients). There Were Two Early Deaths In The Group. PMID : 1595020 [PUBMED - INDEXED FOR MEDLINE] With A Follow-up Period Between Two Months To Five Years, 12 Of 14 Patients Undergoing OLT Are Alive, Fully Functional And Have Normal Liver Function Tests. Seven Of Ten Patients Who Had Shunts Are Alive, Six Are Able To Maintain Normal Activity And One Has Progressive ESLD And Is Not A Candidate For OLT.

135. BUDD-CHIARI SYNDROME – SURGICAL CONCLUSIONS IN BCS DUE TO HEPATIC VEIN OCCLUSION, SIDE-TO-SIDE PCS IS MOST EFFECTIVE THERAPY. IN BCS DUE TO IVC OCCLUSION, MESOATRIAL SHUNT HAS A POOR RECORD. IN BCS DUE TO IVC OCCLUSION, COMBINED PCS & CAS IS VERY EFFECTIVE. LIVER TRANSPLANT FOR NEGLECTED BCS WITH PROGRESSIVE LIVER FAILURE OR IN FAILED PCS. EARLY PORTAL DECOMPRESSION IS THE KEY.

136. SIR PETER MANSFIELD (1933-2017) NOBLE PRIZE FOR DEVELOPMENT OF MRI, A BREAKTHROUGH IN MEDICAL DIAGNOSTICS & REASEARCH (MEDICINE AND PHYSIOLOGY NOBEL IN 2003

137. A Nobel laureate who failed his school exams before going on to pioneer body scanning technology has died aged 83 HIS DISCOVERIES OF IMAGING WITH MAGNETIC RESONANCE HAVE PLAYED A SEMINAL ROLE IN THE DEVELOPMENT OF ONE OF THE MOST USEFUL IMAGING MODALITIES IN MEDICINE TODAY …………… RIP ………… .