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We have analyzed the indications and results of shunt operation versus orthotopic liver transplantation (OLT) in 22 patients with Budd-Chiari syndrome (BCS). The underlying cause of the syndrome was similar between the two groups and was related to myeloproliferative disorders or the use of birth control pills in 18 of 22 patients. The results of biopsies of the liver showed centrilobular congestion and necrosis in all candidates who underwent shunting and the presence of fibrosis and cirrhosis in the OLT candidates. The indications for shunts included symptoms related to portal hypertension only and well-preserved synthetic hepatic function. Ten patients were treated with 12 shunt procedures, including mesoatrial (eight patients) and side to side portacaval shunt (four patients). Significant complications after shunt procedure included fulminant (one of ten patients) and progressive (one of ten patients) hepatic failure requiring urgent OLT; one death occurred because of pulmonary sepsis. Indications for OLT were signs of end stage liver expressed by severe portal hypertension and variceal bleeding (four of 14 patients), progressive encephalopathy (seven of 14 patients) and poor synthetic function (bilirubin greater than 3 milligrams per deciliter in eight of 14 patients and albumin less than 3.0 grams per liter, or both, in ten of 14 patients). Fourteen patients were treated with 16 OLT, three patients had retransplantation for primary nonfunction graft (two of 14 patients) or chronic rejection (one of 14 patients). There were two early deaths in the group. With a follow-up period between two months to five years, 12 of 14 patients undergoing OLT are alive, fully functional and have normal liver function tests. Seven of ten patients who had shunts are alive, six are able to maintain normal activity and one has progressive end stage hepatic disease and is not a candidate for OLT. However, the hepatic function continues progressively to be abnormal. Various options are available for the treatment of the syndrome. Portosystemic decompression is effective and should be considered at the early stage of the disease, prior to the development of significant hepatic failure. However, few of the patients will continue to have slow, but progressive hepatic failure and may require OLT. The only effective treatment for end stage hepatic disease secondary to the BCS is OLT.
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PMID:Portosystemic shunt versus orthotopic liver transplantation for the Budd-Chiari syndrome. 159 20

Renal vein thrombosis in early infancy is a complication of dehydration and prolonged hypotension. The onset is usually acute and the most common clinical signs are uni- or bilateral frank masses, hematuria, proteinuria and thrombocytopenia. In most cases, with conservative management, the late outcome is favorable. In the adult, renal vein thrombosis is often a silent complication of the nephrotic syndrome, the hypercoagulability of which may be an important factor in the pathogenesis of the thrombosis. Clinically, the presentation of a sudden complete occlusion is that of severe abdominal and lumbar pain with hematuria and loss of function of the kidney that suffers hemorrhagic infarction. Physical examination often reveals an enlarged kidney. With gradual occlusion, renal function is preserved. The initial diagnostic approach is with ultrasound studies and computed tomography; definitive diagnosis is established by renal venography or by selective renal arteriography. In general, a conservative approach including the use of anticoagulant treatment is preferred to surgical intervention. Priapism is a persistent painful penile erection due to ischemic or non-ischemic causes; therapeutic intracavernosal injection of papaverine is becoming the most common cause. In early and mild stages, aspiration of blood from the corpora cavernosa supplemented with intracavernosal irrigation with alpha-stimulating agents is the procedure of first choice; in late and severe ischemia, a shunt procedure may become necessary. Hepatic vein thrombosis occurs in association with a number of conditions considered predisposing factors including the use of oral contraceptives. The clinical picture may be that of an acute illness with abdominal pain, hepatomegaly, ascites and hepatic failure as well as early death. More often, the onset is insidious with slowly developing ascites and wasting. For the diagnosis, hepatic scintigraphy may be helpful but, at present, ultrasonography, computed tomography and magnetic resonance scanning are procedures of choice. There is, as yet, no adequate treatment. A fatal outcome may be prevented by surgical decompression of the congested liver and, in recent years, liver transplantation has been employed. Portal vein thrombosis, in children, is usually considered a complication of umbilical sepsis or a result of a congenital abnormality of the portal vein. In adults, the most frequent causes are hepatic cirrhosis and neoplasia. Clinically, there may be a sudden appearance of ascites with resolution in a symptom-free interval until the onset of other features of portal hypertension occur. Currently, ultrasound real-time imaging supplemented with Doppler capability, computed tomography and magnetic resonance scanning provide the necessary diagnostic information. Variceal hemorrhage is often the first major complication requiring treatment.(ABSTRACT TRUNCATED AT 400 WORDS)
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PMID:Thrombosis in particular organ veins. 268 Aug 53

From January 1978 to August 1987, 21 patients received a peritoneovenous shunt using the Le Veen valve (LVV). The indications criteria were the long-term diuretic therapy failure (mean time = 24.4 months) or resistence to medical therapy during hospital internment. The 21 patients underwent 36 surgeries, being 4 valve position review and 11 changes of LVV. The mean age was 51.6 years. Fifteen patients had alcoholic cirrhosis, 3 postnecrotic cirrhosis, one Budd-Chiari syndrome, one mansoni Schistosomiasis, and one malignant ascites. Ten were Child B and 9 Child C patients. Eight patients with history of previous esophageal varices bleeding (EVB) underwent endoscopic sclerotherapy (EE) before LVV implantation. Seven patients died in the early postoperative period (3 Child B and 4 Child C patients). Three patients died due to EVB and the others as consequence of hepatic failure (one), cardiac insufficiency (one), sepsis (one), and bronchopneumonia (one). The mean follow-up was 19.9 months (1-61). Early LVV occlusion occurred in 4 patients and late valve occlusion in others 4 patients. The LVV changes were done at ambulatorial preceeding. Ten patients (47.6%) died in late follow-up and in these cases death was related to the main disease course. It is concluded that: 1) LVV is a useful therapy in patients with intractable ascites, since it is not the terminal manifestations of disease; 2) early mortality is related to liver function and late mortality to main disease course; 3) ascitic patients with EVB should undergo endoscopic sclerotherapy before LVV implantation.
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PMID:[Use of the Leveen shunt in the treatment of clinically intractable ascites]. 325 81

A patient with Budd-Chiari syndrome who underwent orthotopic liver transplantation and developed recurrent disease is described. The immediate postoperative period was complicated by multiple thrombotic episodes, followed by a period of apparent remission associated with the initiation of coumadin and persantine therapy. After discontinuation of such antithrombotic therapy in order to biopsy the liver, the patient experienced another series of clinically overt vascular thromboses and ultimately died of sepsis 15 mo posttransplantation after a prolonged and complicated terminal hospital course. At autopsy, recurrent Budd-Chiari syndrome as well as thromboses in numerous other organs was demonstrated.
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PMID:Budd-Chiari syndrome recurring in a transplanted liver. 633 73

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.
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PMID:Which is the best surgery for Budd-Chiari syndrome: venous decompression or liver transplantation? A single-center experience with 50 patients. 773 40

A retrospective study was performed on 11 patients who underwent orthotopic liver transplantation for Budd-Chiari syndrome (BCS), 3 of whom had fulminant type BCS and 8, chronic type BCS. Both the 3- and 5-year actuarial survival rates were 64%, after one patient with fulminant, and three with chronic disease died of sepsis or multiple organ failure following transplantation. Anticoagulation therapy in the early postoperative period was tailored to each individual patient. Most of the patients received heparin for several days and were then converted to Coumadin therapy, although some were not given heparin in the immediate postoperative period but were instead commenced on oral Coumadin after the prothrombin time had recovered to within the normal range. All the long-term survivors had received Coumadin therapy and there was no recurrence of BCS and no early thrombotic or hemorrhagic event. One patient developed late thrombosis of the portal vein despite having received apparently adequate Coumadin therapy. It was thus concluded that liver transplantation is an effective therapy for both fulminant and chronic BCS, and that immediate postoperative heparinization is not mandatory for all patients.
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PMID:Liver transplantation for Budd-Chiari syndrome: a retrospective study. 805 75

Budd Chiari syndrome is a rare disorder resulting from occlusion of hepatic venous drainage by hepatic vein thrombosis or by a membranous web in the inferior vena cava. In western countries the commonest causes are myeloproliferative disorders and hypercoagulable states. Presentation may be acute with rapid accumulation of ascites and hepatic failure, or subacute with symptoms developing over a few months. A chronic progressive form has also been described. On presentation there is usually abdominal pain, ascites, and hepatosplenomegaly; hepatic encephalopathy is found in about a third. Noninvasive, ultrasound-Doppler is recommended in diagnosis, and has a high correlation with hepatic venography. Liver biopsy is required for therapeutic decisions. Those with advanced hepatic failure or severe fibrosis on liver biopsy are referred for hepatic transplantation. When biopsy shows only hepatic congestion and inflammatory infiltrates, portosystemic shunting is recommended. We present a 61-year-old woman with ascites and hepatosplenomegaly that had developed over the courses of a few months. Budd-Chiari syndrome with chronic myelofibrosis and congenital protein C deficiency were diagnosed. Portosystemic shunt was performed but death from sepsis followed shortly.
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PMID:[Budd-Chiari syndrome]. 933 72

Budd-Chiari syndrome (BCS) is an uncommon form of portal hypertension caused by obstruction of the hepatic venous outflow. From 1969 to 1997 we treated 19 patients (7 men, 12 women; mean age 37.6 years) affected by primary BCS. In most of the cases no etiologic factors were identified; in the remaining cases the etiology was associated with polycythemia vera, use of oral contraceptives, presence of endoluminal membranes, and repeated episodes of sepsis. Three patients with membranous occlusion of the major hepatic veins were treated by percutaneous placement of a self-expanding metallic stent inserted via a transjugular or transhepatic approach. The remaining 16 patients underwent a side-to-side portacaval shunt, which required interposition of a graft in five cases. In two patients with a significant caval obstruction, a metallic vascular stent was placed in the narrowed tract of the inferior vena cava, before shunting, by means of a transfemoral venous approach. One patient died within the first 30 postoperative days. The 18 survivors were followed for a mean of 66.7 months. The 5-year survival was 83%. Primary BCS requires different therapies depending on the stage of the disease. The fulminant or chronic forms with irreversible hepatic damage require definitive treatment, such as orthotopic liver transplantation. For the acute or subacute forms, characterized by reversible hepatic injury, a portasystemic shunt represents the most effective treatment. The patients at poor hepatic risk can be treated by interventional radiology. In both cases preliminary caval stenting is necessary if the syndrome is complicated by significant obstruction of the inferior vena cava.
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PMID:Surgical and radiologic treatment of primary Budd-Chiari syndrome. 946 61

Over the past decade we have reported excellent outcomes in pediatric living-donor liver transplantation (LDLT) with recipient survival exceeding 90%. Principles established in these patients were extended to LDLT in adults. To compare outcomes in donors and recipients between adult and pediatric LDLT in a single center, we reviewed patient records of 45 LDLT performed between 1/98 and 2/01: 23 adult LDLT (54 +/- 6.5 yr) and 22 pediatric LDLT (33.7 +/- 53.5 months). Preoperative liver function was worse in adults (International Normalized Ratio [INR] 1.5 +/- 0.4 vs. INR 1.2 +/- 0.5; p = 0.032). 4 adults (17%) met criteria for status 1 or 2A. Only 1 child was transplanted urgently. Analysis included descriptive statistics and Kaplan-Meier estimation. Donor mortality was 0% with 1 re-exploration, 2.4%. Median hospital stay (LOS) was 6.0 days (range, 4-12 days). Donor morbidity and LOS did not differ by sex, extent of hepatectomy, or adult and pediatric LDLT ( p = 0.49). In contrast, recipient outcomes were worse for adults. Adult 1 year graft survival was 65% (3 retransplants [ReTx], 5 deaths) vs. 91% for children (1 ReTx, 1 death) p = 0.02. Graft losses in adults were due to sepsis (n = 3), small for size (n = 2), suicide, and hepatic artery thrombosis (HAT), whereas in children graft losses were due to portal thrombosis and total parenteral nutrition (TPN) liver failure. Biliary leaks occurred in 22% of adults and 9% of children. Hepatic vein obstruction occurred in 17% of adults and in none of the children. Median LOS was comparable (adult, 16.5 days (range, 7-149 days); child, 17 days (range, 10-56 days), p = 0.2). Graft function (total bilirubin (TBili) < 5mg/dl, INR < 1.2, aspartate aminotransferase (AST) < 100 U/l) normalizing by day 4 in children and by day 14 in adults. Adults fared worse, with an array of problems not seen in children, in particular, hepatic vein obstruction and small-for-size syndrome. Biliary leaks were diagnosed later in adults and were lethal in 3 cases; this was later avoided with biliary drainage in adult recipients. Finally, use of LDLT in decompensated adults led to death in 3 of 4 patients, and should be restricted to elective use.
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PMID:Analysis of failure in living donor liver transplantation: differential outcomes in children and adults. 1260 66

Cryptococcus neoformans is an important pathogen in immunocompromised patients. We report 2 cases of spontaneous C. neoformans peritonitis in patients with liver cirrhosis, a condition not previously reported in Taiwan. Patient 1, a 59-year-old man with alcoholic liver cirrhosis, had primary C. neoformans peritonitis with fungemia. The patient recovered completely after prolonged fluconazole therapy without relapse. Patient 2, a 51-year-old woman with liver cirrhosis due to Budd-Chiari syndrome, had C. neoformans isolated from ascites, cerebrospinal fluid, and blood culture. In spite of adequate antifungal treatment, the patient died of fulminant sepsis. Information about the interaction and relation between liver cirrhosis and cryptococcal peritonitis is rare in the literature. The experience of these cases may help facilitate the diagnosis and treatment of cryptococcal peritonitis.
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PMID:Cryptococcus neoformans peritonitis in two patients with liver cirrhosis. 1566 Jan 76


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