The optimal treatment of Budd-Chiari syndrome (BCS) remains an open question. It is still a matter of controversial discussion whether venous decompression or liver transplantation is superior. To elucidate the role and prognosis of both surgical options in our own experience, a consecutive series of 50 patients treated between 1981 and 1993 was retrospectively analyzed. Twelve patients had different types of portosystemic shunts or local decompressive procedures, and transplantation was performed in 43 cases, including five with previous conventional surgery. The overall mortality of 18 of 50 was conventional surgery. The overall mortality of 18 of 50 was concentrated within the early postoperative period, with no patient lost after 1 year. In the venous decompression group, the success rate was only 29%, and treatment failure was closely related to the finding of cirrhosis or technical problems like vascular thrombosis. After transplantation, early complications were rejection, primary nonfunction, or graft necrosis, and contributed significantly to the risk of sepsis. Thirty of 43 liver recipients are currently alive, including four rescued after failed decompressive surgery, with 1- and 10-year survival of 69%, and excellent recurrence-free rehabilitation. These results clearly indicate that patient selection plays a dominant prognostic role in the treatment of BCS. Venous decompression and liver transplantation should both be integrated in a common therapeutic concept, and the individual decision for the preferred approach must be based on the leading clinical symptom: portal hypertension or liver failure, together with the assessment of reversibility of hepatic damage, and the potential of cure of the underlying disease.
We analysed our population of renal transplant recipients treated with cyclosporin (CsA) and prednisolone with respect to clinically evident de novo malignancies. Eighteen of 598 patients (mean age 35.6 (1-73) years receiving their first renal graft between 1 May 1981 and 31 December 1986 developed a malignancy at a mean interval of 33.5 months. Types of malignancy were squamous carcinoma of the skin (1), carcinoma of the tonsils (1), urothelioma (5), renal-cell carcinoma (2), adenocarcinoma of colon and liver (3), metastic adenocarcinoma of the lung (1), teratocarcinoma of the testis (1), breast cancer (1), Hodgkin's lymphoma in the renal allograft (1), carcinoma of the uterus (1), and carcinoma of the prostate (1). Six cases were observed in the age group 40-49 years (3%), but only three in age group 20-29 years, and nine cases in patients older than 50 years. No malignancy emerged in children (age group less than 19 years) and in patients with pretransplant malignancies. Five patients with analgesic abuse (n = 21 of 598 patients) developed malignant urotheliomas. It is concluded that de novo malignancies constitute a heterogeneous group with no obvious risk attributable to CsA treatment. As previously reported there is a special risk of malignant urotheliomas in patients with analgesic nephropathy. The risk in children seems to be low. We did not observe the high incidence of lymphomas and skin cancer reported by other groups.
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.
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