Budd-Chiari syndrome, also known as hepatic venous outflow obstruction (HVOO), refers to the clinical picture that occurs when there is partial or complete obstruction of the hepatic veins.
There is no clear consensus regarding the number of occluded veins, some authors claim that there should be at least one occluded hepatic vein 7, others state that there are no significant haemodynamic changes if only one vein is occluded therefore clinical manifestations appear when at least two of the three hepatic veins are occluded 8.
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Epidemiology
Budd-Chiari syndrome is rare. A Japanese study estimated the prevalence to be in the region of 2.4 cases/million 4. In Asian countries, there is a male predilection 9. In Western populations, it is most commonly diagnosed in females in the third and fourth decades and the most common cause is thrombosis 9. Membranous webs have been increasingly described in Asian patients as a cause of obstruction.
Clinical presentation
The classic acute presentation is with the clinical triad of ascites, hepatomegaly, and abdominal pain, although this is non-specific. The presentation may be acute or chronic:
acute: results from an acute thrombosis of the main hepatic veins or the inferior vena cava; patients may present with rapid onset ascites
chronic: the chronic form is related to fibrosis of the intrahepatic veins, presumably related to inflammation
Pathology
Aetiology
The aetiology is mixed and varied. The majority of cases result from thrombosis within the hepatic veins. However, 25% arise from external compression that results in obstruction:
idiopathic (one-third of cases)
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congenital
interruption of the diaphragm
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venous thrombosis secondary to
dehydration
pregnancy and the postpartum state
oral contraceptive pill use
thrombocytosis
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injury and/or inflammation secondary to
phlebitis: bone marrow transplant and chemoradiotherapy
autoimmune disease
tumour invasion, e.g. renal cell carcinoma, hepatocellular carcinoma, adrenal carcinoma
It may be associated with concurrent portal vein thrombosis.
Radiographic features
It is characterised on imaging by ascites, caudate hypertrophy, peripheral atrophy, and prominent collateral veins.
Ultrasound
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acute
heterogeneous echotexture
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chronic
peripheral atrophy of affected regions
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regenerative nodules: mostly corresponding to focal nodular hyperplasia-like lesions and therefore sharing similar imaging characteristics of these lesions 5
usually multiple and smaller than 3 cm
Colour Doppler
no flow or inappropriately directed flow in the right hepatic vein or a part of it
discontinuity between the main hepatic vein and the inferior vena cava
reversed flow in hepatic veins
intra- and extrahepatic collaterals
portal vein changes e.g. hepatofugal flow
low or absent flow in the inferior vena cava or balanced bidirectional flow
bland thrombus or tumour thrombus within the inferior vena cava
increased resistive index within the hepatic artery: >0.75
CT
early enhancement of the caudate lobe and central liver around the inferior vena cava
delayed enhancement of the peripheral liver with accompanying central low density (flip-flop appearance)
inhomogeneous mottled liver (nutmeg liver)
peripheral zones of the liver may appear hypoattenuating because of reversed portal venous blood flow
inability to identify hepatic veins
comma-shaped intrahepatic collateral vessels can be seen in sub acute and chronic disease that also known as comma sign 2,11
in the chronic phase, there is caudate lobe enlargement and atrophy of the peripheral liver in affected areas
Angiography (DSA)
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hepatic venography
complete occlusion of hepatic veins
may or may not be associated with a (focal) stenosis of the intrahepatic inferior vena cava
spider web appearance of the intrahepatic collateral veins
Treatment and prognosis
If left untreated, progression to liver failure from fibrosis and eventual death may occur. Management options include 10:
long-term anticoagulation (is usually a first option of treatment)
liver transplantation (definitive treatment)
surgical portosystemic shunting
hepatic venoplasty with stenting
IVC-plasty with stenting
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transjugular intrahepatic portosystemic shunts (TIPS)
as a bridging therapy prior to liver transplantation or surgical portosystemic shunting
as a treatment for patients with contraindications for liver transplantation
Complications
Patients with chronic Budd-Chiari syndrome are at risk for hepatocellular carcinoma (HCC) and therefore should be monitored with serum alpha-fetoprotein (AFP) 5.
History and etymology
Initially described by George Budd (1808-1882) in 1845 before Hans Chiari (1851-1916) lent his first pathological description of "obliterating endophlebitis of the hepatic veins" in 1899.
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