In Reply to: Case 0498_09 --Hepatic vein occlusion posted by Editor on March 31, 1998 at 14:49:30:
As guest editor of Case Club, I have the opportunity to comment on my own case.
My comments here will be focused on timing of TIPS in BCS.
BCS is an uncommon condition, but one that many interventionalists are now seeing with increasing frequency because of the variety of nonoperative therapeutic procedures that we can now offer. At UCSF, for example, we have been referred three patients with BCS in the past two months. The decision to intervene is, I think, fairly straightforward when the disease affects the central hepatic veins or IVC and PTA or stenting is the treatment of choice. Such immediate therapy is very effective and carries with it rather low morbidity. The decision to perform invasive therapy is more difficult, I think, when BCS is due to obliterative disease of the peripheral hepatic vein as in this case and the invasive treatment options include TIPS, surgical shunt or transplantation - all of which are associated with significant immediate morbitity and mortality.
In the case presented, we described a 22 yr. old woman with a 2 week history abdominal pain and a remote history of ascites. She had mild liver disease and would by laboratory and clinical criteria be classified as Child's A. Biopsy revealed a pattern of chronic disease with fibrosis and hepatocyte atrophy. Hematologic workup revealed an underlying hypercoaguable state. All consultants at UCSF felt the underlying hypercoaguable state should be treated and the patient was placed on coumadin. Consultants were divided however on the need for shunt therapy. Many favored close observation and TIPS if LFTs revealed deteriorating liver function. A few consultants argued for more aggressive therapy with immediate TIPS in order to prevent the otherwise certain development of liver failure.
So what do you, the SCVIR internet audience, favor - Coumadin alone, TIPS alone, TIPS and coumadin, or something else?
I must say that I personally find this to be a difficult decision. Many of the patients we see are young and have had a very short history of symptoms, usually mild pain and ascites that is well controlled on diuretics. So when is it appropriate to intervene? Basing a decision on the published literature is pretty difficult since results in fewer than 20 patients have been published.
Much more information is available regarding results of surgical shunts with BCS. Two reports are particularly helpful (Bismuth H. Ann Surg 1991; 214: 581 and Henderson JM. Am J Surg 1990; 159:41). Bismuth bases the decision on clinical presentation. Patients with a fulminant presentation - those with acute portal hypertension and massively elevated transaminases will if untreated die within weeks and he favors immediate intervention. Those with acute presentation - marked ascites, moderate elevation of transaminase will, if untreated, likely die within months - and in these patients he favors immediate shunting. Those with subacute presentation - mild ascites and normal LFTs usually develop progressive liver disease but the course is variable - and in these patients he believes medical therapy (anticoagulation and diuretics) can be attempted but ultimately surgery will be required. Finally, patients with a chronic form with apparent cirrhosis death is inevitable and he favors surgery.
Alternatively, Henderson bases his decision on the results of liver biopsy. If there is no evidence of necrosis or fibrosis, patients are treated conservatively. Patients with centrolobular necrosis are treated by shunt. Patients with severe fibrosis are placed on the transplant list.
So if one applied the Henderson criteria to our case, TIPS should be performed. If one applies the Bismuth criteria, anticoagulation with close clinical follow-up is indicated. In our case, the primary referring service opted to treat the patient conservatively, coumadin and close follow-up.