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Budd-Chiari syndrome: blocked veins, open mind
  1. D PATCH,
  2. A BURROUGHS
  1. Liver Unit,
  2. Royal Free Hospital School of Medicine,
  3. Pond Street,
  4. London NW3 2QG, UK

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See article on page 568

The article by Fisher et al(see page 568) on the various techniques that may be used in the management of Budd-Chiari syndrome is timely, and demonstrates some important points. This condition must be considered as part of the differential diagnosis of ascites in all patients, though the mode of presentation may be protean and hence may trap the unwary. The classic syndrome of ascites, hepatomegaly and abdominal pain occurs with obstruction of hepatic venous outflow at any level—from the hepatic venules (e.g. veno-occlusive disease) to the suprahepatic vena cava. Constrictive pericarditis may also mimic the presentation. The syndrome may run a number of clinical courses which also partly determine treatment. The applicability of any treatment is also influenced by the fact that the inferior vena cava may be blocked in 20% of cases, and portal vein thrombosis evident in 10%.

The patient may present with fulminant hepatic failure, with notable coagulopathy, encephalopathy and renal impairment. A more indolent presentation is also well recognised, with ascites with a high protein content, mildly impaired hepatic function, and a variable degree of fibrosis on biopsy. Finally, well compensated patients with hepatic vein obstruction and no ascites have been described; the development of numerous intrahepatic collaterals with time is said to account for this difference.1 A thrombophilic disorder will be found in up to 75% of patients. This may have additional implications with respect to family screening. Primary myeloproliferative disorders are the most common, but all of the inherited prothrombotic tendencies have been shown to be causal, particularly in association with the oral contraceptive pill and the puerperium. Paroxysmal nocturnal haemoglobinuria, lupus anticoagulant, Behçet’s syndrome, vasculitides, ulcerative colitis, and mechanical obstruction of the hepatic veins by tumour or cysts are also possible associations or causes. Not uncommonly, two or more prothrombotic tendencies may co-exist, and thus bone marrow biopsy is mandatory.

Having considered the diagnosis, and when initial imaging suggests abnormal venous outflow, hepatic venography and visualisation of the inferior vena cava with pressure measurements above and below the liver is the next step, as outlined by Fisher and colleagues. This will confirm:

  • The site of the hepatic vein blockage. Short segment hepatic vein stenosis/occlusion may be amenable to local angioplasty and stenting.

  • Whether there is significant caval compression secondary to caudate lobe hypertrophy. If present, a standard portocaval (infrahepatic) shunt will not reduce hepatic congestion, and a mesoatrial (suprahepatic) shunt is required, unless the cava is stented beforehand.

  • Liver histology. A transjugular liver biopsy should be performed and will establish the degree of fibrosis. In our experience, it is rare that this is technically unsuccessful, even with extensive proximal hepatic vein occlusion. A small “beak” arising from the inferior vena cava may indicate hepatic vein origin, and with care a biopsy can be performed as the cava is within the liver.

At this point our experience differs from that of the Birmingham group. We do not see short segment hepatic vein occlusion. Caval webs are well described in Japan and South Africa, but the authors comment that most of their patients were from Western Europe. We are unable to explain this anomaly.

Clearly a focal occlusion may be amenable to angioplasty and stenting, and the authors document a good technical and overall success rate, and this technique can be added to the other various treatments used in this syndrome. There are, however, some important caveats. The right and middle hepatic veins are relatively easy to visualise at hepatic venography, whereas the left hepatic vein may be difficult to gain access to. Standard teaching advises that all of the liver should be decompressed and one could envisage that left hepatic vein occlusions could be missed, unless very proximal. In this respect, wedge pressure measurements are an important indicator of therapeutic success. As Budd-Chiari syndrome is a postsinusoidal condition, a successful shunt should reduce the wedged hepatic venous gradient to less than 6 mm Hg. In the paper by Fisher et al, only seven of 21 patients had data on wedge pressure responses, and gradients were not given. Theoretically, incomplete restoration of normal hepatic venous blood flow, as reflected by failure to normalise wedge pressure, could allow the fibrotic process to continue. Moreover, using the technique of percutaneous hepatic vein angioplasty, the patients require, according to the Birmingham protocol, annual follow up venography, and to compare their results with the group of patients who had surgical shunts may not be entirely valid as, by their own admission, these patients had more proximal occlusion and therefore perhaps greater overall intrahepatic thrombosis.

None the less, the paper demonstrates the heterogeneity of this patient population and some of the difficulties encountered, as well as a sensible clinical algorithm, and the potential clinical role of a relatively non-invasive radiological technique. For patients with a fulminant presentation, hepatic transplantation is the treatment of choice, and overall success rates of 70% have been recorded.2 3 For those patients without fulminant failure, treatment will be influenced by the presence of fibrosis on liver biopsy, and the length of history. Text books and several papers4 5 suggest that if there is an acute presentation (history less than 16 weeks), with little fibrosis, no portal vein thrombosis or significant caval gradient, then a side to side portocaval shunt is the treatment of choice, with a five year survival of 95% in one series.5 One could argue that the Birmingham results should be viewed in this context. However, these patients may be more fragile than the textbooks would have one believe (for some, the procoagulant process will have been present since birth) and acute liver failure may be precipitated by shunting, despite the correct investigational pathway. This has been the experience of ourselves,6 and others. Ringe et al performed four emergency transplants on four patients with Budd-Chiari syndrome shunted 11–22 days previously, in whom shunt induced hepatic failure developed.3 In this respect transjugular intrahepatic portosystemic stent shunt is an attractive proposition—it bypasses the caval obstruction and does not compromise the surgical field should transplantation be necessary.7 8 Whichever mode of treatment is chosen, it is our opinion that these patients should be referred to a transplant unit as hepatic decompensation may not be predictable.

The final point, well made by Fisher et al, is the need for life long, full anticoagulation. Mesh metal stents notoriously block in patients with cirrhosis—this occlusion rate may well be higher in patients who have a prothrombotic state.

See article on page 568

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