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home - Liver - Vascular Problems - Budd Chiari Aetiology and Management Written by Dr Sebastian Zeki

Understands the risk of variceal bleeding as a complication of with
portal hypertension

Knows risk of variceal haemorrhage in cirrhotics who have not bled

Knows risk of bleeding related to variceal size endoscopic findings
and severity of liver dysfunction

Knows range of therapeutic options (both endoscopic and

Recognises and can treat portal hypertension.
Manages patients with oesophageal varices with skill and

Able to convey the serious risks to patients and their relatives.



Recognises and shows understanding of vascular liver disease
including Budd-Chiari syndrome veno-occlusive disease and
portomesenteric venous thrombosis; understands the underlying
anatomy and physiology of these often complex conditions

Aware of need for investigation for associated myeloproliferative and
procoagulant conditions

Understands the role of anticoagulation and indications for further
intervention including TIPS surgery or transplantation

Can make careful clinical of these conditions and has
heightened awareness of liver vascular disease in differential

Able to make a potentially difficult diagnosis of less common variants
of vascular conditions

Shows ability to keep patient and relatives informed and to refer
appropriately for specialist management

Budd Chiari Aetiology and Management

Budd-Chiari syndrome= thrombosis of the hepatic veins and/or the intrahepatic or suprahepatic inferior vena cava Budd-Chiari Causes:Myeloproliferative disorders - in 50%; Test all for JAK2 mutation if no underlying cause found.Malignancy in 10 %.HCC-most common.Cancer of the adrenal gland or kidney.Sarcomas of the right atrium, IVC or HV.Cancers of the lung, pancreas, and stomach.Infections and benign lesions of the liver — 10%.OCP and pregnancy — in 20%. Includes post-partum women.Other hypercoagulable states.G1691A factor V (Leiden) gene mutation, which produces activated protein C resistance - 25%. Particularly if another prothrombotic state present (eg smoking).G20210A factor II gene mutation.Antiphospholipid syndrome.Antithrombin deficiency.Protein C / S deficiency.PNH. MRI specific the absence or reduction in caliber of hepatic veins and the typical distorted "comma-shaped" intrahepatic collaterals are easily demonstrable.3D MRI is new and developing Magnetic resonance imaging Venography This is the gold standard.Do this if other tests are inconclusive.It shows a "spider web" pattern- collaterals trying to bypass occluded hepatic v. Arteriography This is essential if surgical decompression of congested liver planned as hepatic a. often stretched, arched, and attenuated.It also shows tumours and venous patency. Liver biopsy —Can be diagnostic Centrizonal congestion, necrosis, and hemorrhage.Large regenerative nodules and obstructive portal venopa-thy may also be foundIf shows this only, may benefit from shunt surgery Can develop cirrhosis if chronicTransplant may be better than revascularization IVC Hepatic v. -Thickening, irregularity, stenosis, or dilated hepatic v. walls-Invisible HV-IVC junction-Abnormal HV or IVC flow (on color Doppler) Doppler ultrasonography — As per Doppler USS with...-Patchy flea-bitten liver as inc.central vs peripheral contrast enhancement. This pattern reverses with inhomogeneous parenchymal opacification.Rapid caudate lobe dye clearance .-Delayed/absent HV filling (40-60s after iv contrast)-Narrowing and/or lack of opacification of the IVC IVC Hepatic a. Portal v. Hepatic v. CT scan Portal v. Hepatic a. IVC Hepatic v. IVC Hepatic a. Portal v. Hepatic v. Hepatic a. Portal 14% have assoc. PV thrombo-sis Both 31% Hepatic v. 62% IVC 7% Clinical ManifestationsAverage age of onset is 35.M:F ratio is 1:2. Assoc. with hypertrophy of the caudate lobe of the liver, which has a separate blood supply from rest of the liver.Mild elevations of ALP/ ALT/ BilirubinMay have cirrhosis from chronic congestionEncephalopathy infrequent ALT- 100-600ALP- 300-400Jaundice and ascites may develop rapidly Acute — 20 %Subacute 40%Chronic — 40Ascites (84%)5% asymptomatic. Hepatomegaly and RUQ pain (76 %) Behcet's disease Membranous obstruction (partial or complete) of the inferior vena cava (MOVC)Web-like lesions,cephalad to R hepatic vein entrance into IVC- congenital It is important to diagnose MOVC or so-called short-length hepatic vein stenoses because of the availability of treatment options (eg, anticoagulation, angioplasty, or stenting) that are usually not germane to other causes of the Budd-Chiari syndrome.Miscellaneous SLE;MCTD;Sjögren's syndrome;IBDHypereosinophilic syndrome;Idiopathic granulomatous venulitisSarcoidosis;Protein-losing enteropathy;Minimal change nephrotic syndromeNeurofibromatosis;Alpha-1-antitrypsin deficiency;Trauma (including laparoscopic cholecyste-tomy) Torsion of the liver;Rare familial casesIdiopathic —20% Hepatic a. Portal v. -80% have an underlying disorder. Written by Dr Sebastian Zeki

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