Budd chiari syndrome. ppt

KING GEORGE’S MEDICAL UNIVERITYLUCKNOW

DEPARTMENT OF SURGICAL GASTROENTEROLOGY

BUDD CHIARI SYNDROME

Dr AMIT DANGI

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

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

MORE THAN 8000 CASES HAVE BEEN

REPORTED IN WORLD

LITERATURE

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, – Budd Chiari syndrome with occlusion of hepatic vein, or – hepatic vein thrombosis

Shin N et al . BCS review World J Hepatol 2016 June

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

definitionShin N et al . BCS review

World J Hepatol 2016 JuneEuropean Group for the Study of Hepatic Vascular Diseases

■ 0.2 and 2 per 1 million population in general populationValla, 2009

■ 4.9% of all portal hypertension patients - Japan■ 7-9% of all portal hypertension patients – India

Demographic profileFEATURES 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, NepalIVC thrombosis in India

Presentation Acute (60-85% < 6 months)Greater severity of symptoms

Chronic (75-80%)

Association Oral contraceptivesMyeloproliferative disorders

Pregnancy & infectionInfection

Epidemiology of classical Budd-Chiari syndrome and hepatic vena cava-Budd Chiari

syndrome


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

ETIOLOGY– HYPERCOAGUABLE CAUSESInherited 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 thrombocytosisThe JAK2V617 F mutation : 26% -52% of patients with classical BCS

Protein C deficiency Paroxysmal nocturnal hemoglobinuria

Protein S deficiency Antiphospholipid syndromeFactor V Leiden mutation (MOST SEVERE AND ACUTE FORMS) 25% in western population5-10 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

EtiologyUncommon 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 diseasePost liver transplantIdiopathic

■ 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

INFECTIONS3 - 9.9%Amoebic liver abscessHydatid cystSchistosomiasisSyphilitic gummaAspergillosisFilariasis.TRAUMABlunt and penetrating

■ 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

ETIOLOGICAL CLASSIFICATION

Rossle M et al, Surgery 2004,Okuda K et al, J Gastroenterol hepatol 2002

Janssen HL, et alJ Hepatol 2003

ORIGIN EXAMPLESPRIMARY Endoluminal lesion Thrombosis

WebsEndophlebitis

SECONDARY Outside venous system

Invasion or extrinsic compression

TumorAbscessCysts

CLASSIFICATION- VESSEL INVOLVEMENT

TYPE I TYPE II TYPE III

IVC WITH OR WITHOUT HEPATIC VEINS OCCLUSION

MAJOR HEPATIC VEINS

SMALL CENTRILOBULAR VEINS

Obstruction characteristics: Location, type, and associated findings


■ Large 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.

PATHOPHYSIOLOGY

Hepatic venous outflow obstruction

Increased sinusoidal &

portal pressure

Extravasation of fluid from

sinusoids

Ascites

Venous stasis and congestion

Hypoxic damage to hepatocytes

Hepatocyte necrosis (Centrilobular) l/t lobular collapse

Fibrosis, Nodular regenerative hyperplasia

Cirrhosis

■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

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

■ 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

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 cirrhosisRossle M et al, Surgery 2004

Dilawari JB et al. Medicine 1994

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

■ Orloff and colleagues (2012) : In 77 patients12 : 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) :

Most patients presented with advanced disease at diagnosis.

92% exhibiting ascites and 55% with cirrhosis

■ Mitchell and colleagues (1982) , which excluded patients with MOVC,

2/3rd : 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.

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 2000Young children (<10 years of age) account for 1–7% of all cases of BCS.

Signs and symptoms in classical Budd-Chiari syndrome and hepatic vena cava-Budd Chiari

syndrome


IVC OBSTRUCTION

HV OBSTRUCTION

Geography EAST WEST

Etiology Membranous obstruction

Hyper coaguable state

History Long ShortSex Male FemaleAnkle edema Gross AbsentAscites Moderate MassiveCollaterals Abdominal wall AbsentHepatomegaly

Moderate/ cirrhosis Massive, tender

Prognosis Good Worse

DIFFERENTIAL DIAGNOSIS

■Cardiac: – Tricuspid regurgitation– Constrictive pericarditis– Right atrial myxoma■Hepatitis■Cholecystitis

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

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

LABS

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

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

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 ThrombosisIntrahepatic 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)

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 demonstrates 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).

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 demonstrates 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

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.

LIMITATIONS OF SON0GRAPHY

■ Restriction 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.

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

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.

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) .

MRI■ Preffered as a SECOND LINE OF INVESTIGATION ■ Particularly beneficial in the evaluation of the patients with non-

diagnostic ultrasonography ■ 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 :

characterized better with MR imaging than with a CECT■ Useful in differentiating regenerative nodules from HCC based on T2-

weighted signal characteristics ( Brancatelli et al, 2007 )

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).

■ ENHANCEMENT PATTERNSACUTE : Both 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)

Atrophy 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

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.

■ Late 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.

■ Later 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.

LOCATIONS OF EXTRA- HEPATIC COLLATERAL VEINS IN BCS :

Different from those seen in cirrhosis. 4 groups mainly in RPDeep and central tributaries of the systemic circulation

Ascending lumber veins, Vertabral venous plexus, Azygos and Hemiazygos 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 Other 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.

ADVANTAGES OF MRA OVER DOPPLER

■ No 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.

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)

ANGIOGRAPHY AND VENOGRAPHY■ CONVENTIONAL ANGIOGRAPHY

Gold standardPressure profileAnatomy of block

■ IVC VENOGRAPHY Gradient >15mm Hg - Mesoatrial shunt

■ HEPATIC VENOGRAPHY/SMA VENOGRAPGY

Occluded / narrow hepatic veinsSpider web pattern of venous collateralsWedge hepatic venous- IVC gradient >10mm Hg

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)

CONTEMPORARY ROLE OF VENOGRAPHYPlatform 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.■ Transjugular liver biopsy. ■ Use of hepatic venography may be an essential

guide and road map for surgical therapy in BCS

Erdon, 2007 ; Kamath, 2006

ROLE OF LIVER BIOPSYNeed?■ 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

Liver biopsy

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 ill assay

5. Protein-C Assay6. S antigen assay7. Lupus

anticoagulant8. Anticardiolipin

antibodies9. Ham’s acid

hemolysis test

9. Activated protein –C (resistance and / or factor V Leiden mutation

10. 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 assay15. 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

Baxter et al, 2005Mahmoud and Elias (1996)

Hirschberg et al, 2000 ; Valla, 2009 )

PART IIMANAGEMENT OF

BCS

MANAGEMENT

■Treatment depends on the

causethe anatomic

characteristics and stage of liver diseasethe 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 improves survival

■Remove the cause■Prevention of thrombosis extension ■Relieve the high pressure &

congestion in liver■Management of massive ascites

MANAGEMENT OPTIONS

■Medical therapy■Minimal invasive

interventions (MII)■Surgery (SHUNT AND NON

SHUNT SURGERIES)■Liver transplantation

Is this the sequence?

Anticoagulation

Angioplasty & stenting

Shunt (TIPS or surgical)

Liver transplantationPlessier A et.al Hepatology 2006Seijo S, Hepatology. 2013 May.

MEDICAL MANAGEMENT

Objectives (1) remove the cause of the venous thrombosis, (2) relieve the high pressure and congestion within

the liver, (3) prevent extension of the venous thrombosis, and (4) reverse the massive ascites.

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

■ 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 benefitSharma et al, J Hepatol 2004

- 1/3rd 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■ Tissue plasminogen activator (tPA) is the direct thrombolytic of choice by catheter

route

■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

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

UsefulAcute form Partial venous occlusionMild symptoms Mild ascites No ongoing hepatic necrosis Relatively normal LFTMajor co-morbidities

Menon KV et al, N Engl J Med 2004

MINIMAL INVASIVE INTERVENTIONS

■Local thrombolytic therapy

■Transluminal angioplasty

■Endovascular stenting

■TIPS

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

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

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 : SSPCS2 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)

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

Aucejo F et al, Liver Transpl 2006

Wang SL et al, Radiology 2005

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

stenosisKlein AS et al, Liver transplant 2003

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

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

■ 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.

Covered stents– Better results European series : 112 pts (17 with BCS)

underwent TIPS with covered stents 1 yr failure rate : 10% Mortality rate without and with the patients lost to follow- up was 20% and 30% respectively

Rossle and colleagues et al 2006■ Two series, 34 patients, PTFE covered

stents– Improved patency– Less dysfunction

Hernandez et al, Hepatology 2004Gandini R et al, Radiology 2006

Primary patency : 75% in larger series,

Secondary patency of 99% at a mean follow-up of 82 months

72% survival at 10 years.

■ In a multicenter review conducted by Garcia-Pagan and colleagues (2008) ,

221 patients with BCS147 eligible for TIPS124 (60%) underwent TIPS (14 had technical C/I , 9

failed TIPS)22 had TIPS related complications including 2 deaths61 (41%) had TIPS dysfunction : Restenting in 35

Angioplasty in 20 Thrombolysis in 6 patients

HE developed in 21% of patients within 1 year16 (13%) deaths in F/U8 (6.5%)required LT Actuarial LT-free survival at 1, 5, and 10 years was

88%, 78%, and 69%, respectively.

■ Largest reported systematic review and meta-analysis

29 studies2255 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.

Indications■ Bridge to transplant in fulminant■ Acute form BCS ■ Sub acute form BCS with Porta-caval pressure gradient

<10mmHgMenon 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 1st year

SURGICAL MANAGEMENT■Surgical Options

– Decompressive surgical shunts– Radical membrane excision– Surgical Removal of Venous

Obstruction

LIVER TRANSPLANT

SURGICAL SHUNTS

■Pre-requisite– Reversible liver injury

■Indication– Technically difficult TIPS (massive

thrombosis)– Porta-caval pressure gradient > 10 mm Hg

■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

From Orloff MJ, et al: Budd-Chiari syndrome revisited: 38 years' experience with surgical portal decompression. J

Gastrointest Surg 16:286-300, 2012.

Operative experience reported by Orloff and colleagues (2012)

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)

■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

Portacaval shunt

Mesocaval shunt

Mesoatrial shunt

Cavoatrial shunt

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

■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

Measurement of pressures in the IVC 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

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

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

• 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

Preop 6weeks post PCS

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

Long term results of MESO ATRIAL shunt in 8 pts with BCS

OPERATIVE MORTALITY10 AND 15 YEAR MORTALITYOCCLUSION OF SHUNTASCITESNEED FOR DIURETICSABNORMAL LIVER FUNCTION TESTSENCEPHALOPATHYWORKING FULL-TIME

% of group0

63636363633825

UNSATISFACTORY : 63% MORTAILITY FROM LIVER FAILURE d/t SHUNT THROBOSIS : ABANDONED

Orloff MJ, et al Arch surg 1992

RESULTS OF MESOATRIAL SHUNTIN 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%

Survival1 year – 90.7%3 year – 77.1%5 year – 61.1%

Slakey DP et al, Ann Surg 2001Wang ZG et al, ANZ J Surg

2005

SCS– 72 patients■ 26 SCS, 46 MCS■ Similar efficacy in decreasing portal pressure,

decreasing bleedDang XW et al,, Hepatobiliary Pancreat Dis Int. 2005

COMBINED PCS AND CAS

RESULTS OF COMBINED PCS-CAS FOR BCS DUE TO IVC OCCLUSION IN 12

PATIENTS FOLLOWED UP 5-22 YEARS%

ASCITES 0NEED FOR DIURETICS 0NORMAL LIVER FUNCTION 100ENCEPHALOPATHY 0PATENT SHUNT 100SURVIVAL 5-22 YEARS 100

Orloff MJ, et al Arch surg 1992

■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

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

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

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 BEHCET'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

QUALITY OF LIFE AFTER SURGICAL PORTAL DECOMPRESSION IN BCS

1. LONG-TERM SHUNT PATENCY IN 97-100%.

2. HEPATIC SINUSOIDAL DECOMPRESSION WAS MAINTAINED.

3. NO ASCITES OR NEED FOR DIURETICS.4. LIVER FUNCTION AND SIZE RETURNED

TO NORMAL.5. VARICEAL BLEEDING DECREASED

6. PREDISPOSING CONDITIONS WERE CONTROLLED.

7. VASCULAR THROMBOSIS WAS PREVENTED (ANTICOAGULANTS).

8. ENCEPHALOPATHY DID NOT OCCUR WHEN SHUNT WAS PATENT.

9. 94-100% RETURNED TO WORK/HOUSEKEEPING.

10. 10 YEAR SURVIVAL WAS ≥ 91%. Orloff MJ et al,

Annals of Surg 2000

QUALITY OF LIFE WAS EXCELLENT WHEN

PERFORMED EARLY IN THE COURSE OF BCS.

ORLOFF MJ ET AL, ANNALS OF SURG 2000

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

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

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- Portopulmonary 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

Senning operation■ Direct 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)

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

LIVER TRANSPLANTATION

■ 1974 – first successfulPutnam 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

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

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 mutationCruz 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

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 39FAILED 4

GALATI ET AL, 1993 32 FAILED 2

HALFF ET AL, 1990 ; IWATSUKI ET AL, 1991 FAILED 5INTRACTABLE ASCITIS 15RECURENT VARICEAL BLEEDING 15HE 15

MENTHA ET AL 2008 248 FHF 47EUROPEAN LIVER TRANSPLANTATION REGISTRY, 1988-1999

RF 40, PVT 47FAILED PSS 49FAILED TIPS 11FAILED 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

Liver transplantation for BCSSegev 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

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

■ 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

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

Recurrence of BCS in the transplanted liver

■ BCS following LT with the piggyback technique is a well-described complication.

■ Reported 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%.

■ Conventional 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

Issues with LTAllocation 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 warfarinTechnical ■ Haemostasis■ Large caudate lobe- difficult mobilization■ Diffuse retroperitoneal fibrotic reaction – difficult IVC control■ Thick, narrow, thrombosed portal veinOther■ Life long immunosuppression■ High cost

Survival After LT for BCS Reported in the Literature

Horton et al. BCS review. Liver International 2007

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.

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

■ 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;

■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.

■ 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.

■ Transplant- 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

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.

■ 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 Age) :

■ Strongly associated with survival and had a discriminative capacity, which was superior to the Rotterdam score

Seijo S, Hepatology. 2013

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

Eapen CE et al, Gut 2005

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.

► ConclusionPatient selection plays a dominant prognostic role in the treatment of BCS.

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.

BUDD-CHIARI SYNDROME – SURGICAL CONCLUSIONS1. IN BCS DUE TO HEPATIC VEIN OCCLUSION, SIDE-

TO-SIDE PCS IS MOST EFFECTIVE THERAPY.2. IN BCS DUE TO IVC OCCLUSION, MESOATRIAL

SHUNT HAS A POOR RECORD.3. IN BCS DUE TO IVC OCCLUSION, COMBINED PCS

& CAS IS VERY EFFECTIVE.4. LIVER TRANSPLANT FOR NEGLECTED BCS WITH

PROGRESSIVE LIVER FAILURE OR IN FAILED PCS.5. EARLY PORTAL DECOMPRESSION IS THE KEY.

SIR PETER MANSFIELD (1933-2017)NOBLE PRIZE FOR DEVELOPMENT OF MRI, A

BREAKTHROUGH IN MEDICAL DIAGNOSTICS & REASEARCH (MEDICINE AND PHYSIOLOGY NOBEL IN 2003

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………….

Education

Budd chiari syndrome. ppt