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Chiari Syndrome (chiari + syndrome)

Distribution by Scientific Domains
Medical Sciences100%
Distribution within Medical Sciences
Gastroenterology & Hepatology22%

Selected Abstracts

Is hepatic vena cava disease an endemic type of the Budd,Chiari syndrome?

HEPATOLOGY RESEARCH, Issue 3 2007
Tetsuya Mine
No abstract is available for this article. [source]

Surgical portosystemic shunts and the Rex bypass in children: a single-centre experience

HPB, Issue 3 2009
Sukru Emre
Abstract Objectives:, This study aimed to illustrate the indications for, and types and outcomes of surgical portosystemic shunt (PSS) and/or Rex bypass in a single centre. Methods:, Data were collected from children with a PSS and/or Rex bypass between 1992 and 2006 at Mount Sinai Medical Center, New York. Results:, Median age at surgery was 10.7 years (range 0.3,22.0 years). Indications included: (i) refractory gastrointestinal bleeding in portal hypertension associated with (a) compensated cirrhosis (n= 12), (b) portal vein thrombosis (n= 10), (c) hepatoportal sclerosis (n= 3); (ii) refractory ascites secondary to Budd,Chiari syndrome (n= 3), and (iii) familial hypercholesterolaemia (n= 4). There were 20 distal splenorenal, four portacaval, three Rex bypass, two mesocaval, two mesoatrial and one proximal splenorenal shunts. At the last follow-up (median 2.9 years, range 0.1,14.1 years), one shunt (Rex bypass) was thrombosed. Two patients had died and two had required a liver transplant. These had a patent shunt at last imaging prior to death or transplant. Conclusions:, Portosystemic shunts and Rex bypass have been used to manage portal hypertension with excellent outcomes. In selected children with compensated liver disease, PSS may act as a bridge to liver transplantation or represent an attractive alternative. [source]

Multiple thrombophilic factors in a patient with Budd,Chiari syndrome

INTERNATIONAL JOURNAL OF LABORATORY HEMATOLOGY, Issue 1 2002
V. BRANCACCIO
Myeloproliferative disorders are the main cause of Budd,Chiari syndrome in western countries. Inherited or acquired thrombophilic factors have also been implicated. A novel mutation of the prothrombin gene (G,A20210) has only been described in a few cases of Budd,Chiari syndrome so far. Venous thrombosis is often the result of multiple concomitant thrombophilic factors. We report the case of a patient with essential thrombocythemia and Budd,Chiari syndrome in which heterozygosity for both factor V Leiden and the mutation G20210A of the prothrombin gene were identified. [source]

Cavernous hemangioma of the liver with giant cyst formation: Degeneration by apoptosis?

JOURNAL OF GASTROENTEROLOGY AND HEPATOLOGY, Issue 3 2001
Kazuhiro Hanazaki
Abstract Cavernous hemangioma of the liver with cyst formation is a very rare condition. A case of cavernous hemangioma of the liver with unilocular giant cyst formation undergoing surgical removal is reported. Notably, the patient also had Budd,Chiari syndrome with an obstructing lesion in the inferior vena cava. The cystic degeneration of the hemangioma implied a relationship with apoptosis. This is the first reported case of Budd,Chiari syndrome caused by advanced cystic degeneration of hepatic cavernous hemangioma. [source]

Transjugular intrahepatic cavoportal shunt for Budd,Chiari syndrome

JOURNAL OF MEDICAL IMAGING AND RADIATION ONCOLOGY, Issue 2 2005
T Ul Haq
Summary Budd,Chiari syndrome (BCS) is characterized by obstruction of the hepatic venous outflow tract. Therapeutic options for BCS are limited. We report a case of a 21-year-old woman with protein S and C deficiency with gross ascites. Treatment with transjugular intrahepatic portosystemic shunt (TIPS) was attempted, which revealed occluded hepatic veins, so transcaval TIPS was performed. No serious procedure-related complication occurred. After successful shunt creation, the patient's symptoms subsided and she was discharged and followed up for 6 months. [source]

Rotterdam score predicts early mortality in Budd-Chiari syndrome, and surgical shunting prolongs transplant-free survival

ALIMENTARY PHARMACOLOGY & THERAPEUTICS, Issue 10 2009
A. J. MONTANO-LOZA
Summary Background, Budd,Chiari syndrome carries significant mortality, but factors predicting this outcome are uncertain. Aim, To determine factors associated with 3-month mortality and compare outcomes after surgical shunting or liver transplantation. Methods, From 1985 to 2008, 51 patients with Budd,Chiari syndrome were identified. Results, By logistic regression analysis, features associated with higher risk of 3-month mortality were Rotterdam class III, Clichy >6.6, model for end-stage liver disease (MELD) >20 and Child,Pugh C. Rotterdam class III had the best performance to discriminate 3-month mortality with sensitivity of 0.89 and specificity of 0.63, whereas Clichy >6.60 had sensitivity of 0.78 and specificity of 0.69; MELD >20 had sensitivity of 0.78 and specificity of 0.75 and Child,Pugh C had sensitivity of 0.67 and specificity of 0.72. Eighteen patients underwent surgical shunts and 14 received liver transplantation with no significant differences in survival (median survival 10 ± 3 vs. 8 ± 2 years; log-rank, P = 0.9). Conclusions, Rotterdam score is the best discrimination index for 3-month mortality in Budd,Chiari syndrome and should be used preferentially to determine treatment urgency. Surgical shunts constitute an important therapeutic modality that may help save liver grafts and prolong transplantation-free survival in a selected group of patients with Budd,Chiari syndrome. [source]

Transjugular intrahepatic portosystemic shunt: an analysis of outcomes

ANZ JOURNAL OF SURGERY, Issue 10 2009
Timothy P. Kurmis
Abstract Background:, Transjugular intrahepatic portosystemic shunts (TIPS) are utilized for the management of complications of portal hypertension, particularly diuretic-resistant ascites and recurrent variceal bleeding. It has also been applied in Budd,Chiari syndrome and hepatorenal syndrome. We report the results in a small series, over 9 years, from a single centre, and compare these to those published in the literature. Methods:, A retrospective case note review of 20 consecutive TIPS procedures performed at Flinders Medical Centre from January 1997 to December 2005 was completed. All indications were included in the analysis. Underlying liver disease, peri-procedure complications, relief of symptoms and patient survival were recorded. Data on type of TIPS, shunt patency and method of follow-up were recorded. Results:, Thirty-six TIPS were performed in 20 subjects. All initial TIPS attempts were successful. Indications were: refractory ascites (18), acute variceal bleeding (12) and hepatorenal syndrome (2). There were no peri-procedure deaths, however. Ninety-day mortality was 20%. Outcomes in model of end-stage liver disease score and biochemical characteristics post-TIPS were comparable to those reported. Overall, TIPS dysfunction rate was 35% at 1 year. TIPS follow-up and patency surveillance was an ad hoc combination of Doppler ultrasound and venography. Conclusion:, TIPS procedure outcomes in our centre are similar to those reported in the literature from large centres. TIPS patency rates may be improved with regular monitoring and early intervention when stenosis occurs. [source]