Liver transplantation for Budd-Chiari syndrome-palliation or cure?

Abstract

This report documents two cases of Budd-Chiari syndrome (BCS) with essential thrombocytosis and antithrombin (AT) III deficiency as underlying etiological factors. Orthotopic liver transplantation was successfully performed in both patients but with different therapeutic intention. In the patient with essential thrombocytosis, hepatic transplantation only relieved the symptoms of the predisposing thrombogenic condition; it did not cure the underlying disorder. Prophylactic long-term anticoagulation, as well as adjuvant therapy for the causative disease, remained necessary. On the other hand, in the patient with AT III deficiency, liver transplantation was curative, resulting in complete reconstitution of serum AT III activity with resolution of the hypercoagulable state postoperatively. Thus, depending on the underlying etiology, liver transplantation for BCS can be considered as palliative, necessitating long-term adjuvant therapy, or as curative, with correction of a metabolic defect.

Budd-Chiari Syndrome: Current Management Options

“…11 Most patients, therefore, irrespective of the primary treatment, require life-long anticoagulation and treatment of the underlying disease.…”

“…Liver transplantation has been reported as effective treatment of BCS by other authors, with survival rates ranging from 69% to 87%. 2,11,17,20,21 Unequivocal indications for liver transplantation as treatment of BCS include inborn errors of metabolism, such as antithrombin III deficiency, which can be cured by transplantation. 11 Selection of other patients with BCS for liver transplantation as a primary therapy is, in contrast, difficult, because characteristics predictive of a poor outcome after surgical shunting are poorly defined.…”

Surgical treatment of Budd-Chiari syndrome

“…1,90 The basis of this recommendation is that shunting usually leads to further hepatic decompensation in these instances. 1,41,91,92 Transplantation also is the treatment of choice in cases of BCS associated with an inborn error of metabolism that can be cured with hepatic replacement, such as antithrombin III, protein S, or protein C deficiencies. 1,91 Liver transplantation for BCS is associated with acceptable morbidity.…”

“…1,41,91,92 Transplantation also is the treatment of choice in cases of BCS associated with an inborn error of metabolism that can be cured with hepatic replacement, such as antithrombin III, protein S, or protein C deficiencies. 1,91 Liver transplantation for BCS is associated with acceptable morbidity. Long-term survival after transplantation, although good, often is dependent on factors not directly related to transplantation itself, such as progression of the underlying hematologic disease.…”

“…Others have reported survival rates between 54% and 87%. 1,3,8,41,55,77,78,[90][91][92][93][94][95][96] Although recurrence of hepatic vein thrombosis occurs infrequently after transplantation, recent series have reported incidences of 0% to 10%. There are no data that suggest a potential immunosuppression-associated exacerbation of underlying malignant or premalignant conditions.…”