DR. TANVEER HUSSAIN FCPS(Medicine) FCPS (Gastroenterology) BUDD CHIARI SYNDROME DR. TANVEER HUSSAIN FCPS(Medicine) FCPS (Gastroenterology)

HISTORY Lambron in 1842 : First case report. George Budd (1845) Internist, kings College. Classic Triad of Abdominal pain Hepatomegaly Ascites Hans Chiari (1895) Pathologist Histopathology features. Obliterative endophlebitis of hepatic veins

HISTORY Okuda et al, hepatology , 1998 Rokitansikys disease. Synonymous: Hepatic venous outflow obstruction. Obliterative hepatocavopathy. Okuda et al, hepatology , 1998 Rokitansikys disease. Von Rokitansikys disease. Membranous obstruction of 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. IVC. Right atrium. Sinusoidal obstruction syndrome is excluded from this definition. Shin N et al.BCS review Worlds J Hepatol 2016 june. European group for the study of hepatic vascular diseases

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

CLASSIFICATION -VESSEL INVOLVEMENT TYPE I TYPE II TYPE III IVC with or without Hepatic veins occlusion. Major Hepatic veins Small Centrilobular Veins.

Categories: based on rapidity of onset and collateralization Acute +/- fulminant: 20%, F>M, Severe RUQ pain, Rapid development of jaundice, ascites, AST/ALT > 5x ULN; ↑↑ bili; +/- encephalopathy Sub-acute: 40%; variable presentation; typical is partial occlusion. asymptomatic to mildly symptomatic. Chronic: 40%; seen as cirrhosis

So then, what are the causes? FIRST THINK Hypercoagulable myeloproliferative diseases Multiple causes in one person often see + JAK2 mutation

DEMOGRAPHIC PROFILE FEATURES WEST (Classical) EAST (HVC-BCS) OBSTRUCTION HV (classical BCS) IVC SEX Females Males OBSTRUCTION OF IVC. Thrombosis. Membranous in Japan, Nepal. IVC thrombosis in India. PRESENTATION Acute (60-85%) Chronic (75-80%) Association OCP and MPD Pregnancy and Infection

CLINICAL PRESENTATION Fulminant Rare(7%) Acute Sub acute Most common Chronic Onset < 2 months Up to 6 months. 6 months- years Months to years CF HE Sudden tense ascites, abdominal pain hepatomegaly. No collaterals. Resistant ascites Hepatic and portal collaterals Features of CLD. Transaminase levels. > five times Mild elevation. Histopathology No cirrhosis Little necrosis Severe fibrosis or Cirrhosis

Signs and Symptoms Abdominal Pain Hepatomegaly Ascites Jaundice Venous collateralization – varices, abdominal/flank collaterals Absence of hepatojugular reflux Laboratory evidence of liver dysfunction

Normal R hepatic vein Normal liver Centrilobular necrosis, dilated sinusoids filled with RBCs No R hepatic v. Rich collaterals ‘Spiders web’

HOW TO DIAGNOSE?

DIAGNOSIS Clinical suspicion. LFTs Imaging Infra and supra hepatic caval pressure. Hypercoagulable profile. Bone Marrow. Liver biopsy.

CLINICAL SUSPICION Classic Triad present simultaneously. FHF with hepatomegaly and ascites. Liver disease in patients with prothrombotic disorders. Patients with CLD, intractable ascites with mildly derranged LFTs. Few cases of idiopathic CLD .. KV menon, NEJM 2004

IMAGING Role of imaging Site of obstruction. Patency of HV , IVC, PV. Details of vascular anatomy. Status of liver. Caudate lobe Collaterals. Differentiates forms of BCS. Brancatelli G et al, AmJ Roentgenol 2007

IMAGING Doppler ultrasound (first step). CT scan MRI Hepatic Venography.

LIVER ULTRASOUND WITH DOPPLER STUDIES Normal right hepatic vein Doppler study showing normal expected biphasic waveform across hepatic vein No hepatic vein, no venous flow

CHARACTERISTIC FEATURES ON VENOGRAPHY AND CT Caudate Hypertrophy and IVC Compression Mottled parenchyma / lack of perfusion Retroperitoneal Varices

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

TREATMENT: Longterm/indefinite anticoagulation and. . .

MEDICAL MANAGEMENT Remove cause of venous thrombosis. Relieve high pressure and congestion with in liver. Prevent extension of thrombosis. Reverse massive ascites.

DECISION MAKING IN SHUNT SX Documentation of ongoing necrosis. Demonstration of intact lobular architecture Status of infra hepatic IVC. Status of PV. Status of caudate lobe. Damaged liver with no lobular collapse. shunt sx. Severe damaged liver with lobular collapse and fibrosis . LT.

BCS– SURGICAL CONCLUSION BCS due to HV occlusion, SSPCS is most effective therapy. BCS due to IVC occlusion , combined PCS and CAS is very effective. LT for BCS with progressive liver failure or in failed PCS. SSPCS and LT are not competing forms of treatment. Early and middle stages of BCS: SSPCS. Fibrosis or Cirrhosis: LT

PROGNOSIS

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