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home - Liver - Vascular Problems - Budd Chiari Treatment Written by Dr Sebastian Zeki
Knowledge


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
pharmacological).

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

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

Also...

Knowledge


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

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

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

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

Budd Chiari Treatment

Recommendation — Can use anticoagulation alone if:1. Chronic and well compensated (but always think of other ways to decompress liver)2.Those in whom other types of therapy are not feasible. If only medical treatment, patients should undergo regular endoscopy and liver biopsiesThrombolytic therapy —Useful if recent and well defined Surgical approaches — Side-to-side portacaval, splenorenal, or mesocaval shunts only if patent IVC and no pressure gradient across it (supra vs infrahepatic). Infrahepatic should be >10 mmHg lower than portal pressure. If caudate lobe hypertrophy, direct side-to-side portacaval anastomosis difficult, so need mesocaval shunt.Synthetic shunts can be constructed from the portal-mesenteric system to the R atrium (meso-atrial shunt), which can bypass the IVC if occluded/ big pressure gradient. However, shunts that require artificial graft material are more likely to be complicated by thrombosis.5yr survival following shunt surgery depends upon the extent of liver damage prior to surgery, and the continued patency of the shunt- can be up to 90%Perioperative management — Anticoagulate.Periodic doppler USS to evaluate shunt patency, esp. for synthetic graft shunts.If deteriorate, investigate for stent thrombosis.Liver transplantation — Liver transplantation may be only option esp if have cirrhosis- can cure protein S, protein C, or antithrombin III deficiency10 year survival around 70% TIPS (see TIPS page for details) — Aim: Decompress congested liver segments by creation of an alternative venous outflow tract.Probs: a) May not be technically feasible in many patients b)May only drain a small portion of the liver, c)Associated with a high rate of occlusion d)Many patients would qualify for angioplastyRole: As second line or temporizing measure whilst wiating for a OLT Stenting —Stenting after angioplasty may be useful but likely to still need OLT.Stent permanent so coordinate with liver transplant centre- eg. Placement of a stent above the intrahe-patic IVC,complicates, and can preclude, anastomo-sis of the donor and host IVC required during OLT. Angioplasty — Useful if focal abnormality (eg web)Balloon dilation of hepatic veins may also be effective in some.Angioplasty+ thrombolysis indicated in thrombosis of a single hepatic vein- Reocclusion common Radiologic treatment Options:-Angioplasty alone.-Transjugular intrahepatic portosystemic shunts (TIPS).-Angioplasty with placement of a stent. Budd-Chiari Treatment Surgical therapy — The aim is to shunt portal or mesenteric venous system into the IVC or another systemic vein.It is unlikely to be beneficial in cirrhosis which is best managed by OLT. Medical therapy —Most need chronic anticoagulation but won’t cause recanalization Written by Dr Sebastian Zeki

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