BACKGROUND: Budd-chiari syndrome (BCS) is rare disorder, varied in etiology, inconsistent in presentation, unpredictable in progression, and challenging in therapy. AIM OF STUDY: The aim of this study is to review experience of Budd-Chiari syndrome at KFSH, Riyadh; over a ten-year period. METHODS: In a retrospective study using a computer data search of the medical records from May 1990 to May 2000, 43 patients' charts suspected of BCS were reviewed. 29 patients were found to have BCS, diagnosed by Doppler ultrasound, venography, MRI, or CT with or without liver biopsy. FINDINGS: Of the 29 patients, 18 were male (62.1%) and I 1 were female (37.9%); mean age was 35.4 (range 17-69 years). Nine patients had Behcet's disease; seven patients had malignancy (five HCC, one melanoma and one hypernephroma). Eight patients had antiphospholipid syndrome, two patients had no known cause and three (one related to trauma, the other related to protein C deficiency and the third related nephrotic syndrome). Nineteen patients presented acutely with abdominal pain, progressive ascites and hepatomegaly. Ten patients had subacute presentation mimicking cirrhosis of liver. Five patients had web in the intrahepatic IVC or ostium HV and the remaining 24 patients had thrombosis of hepatic veins. Five patients received surgical shunts, three had balloon dilatation of webs, 11 had anticoagulant therapy and seven patients had symptomatic treatment. Eleven patients died, six of them had malignancy, two died while awaiting liver transplantation and three died with fulminant hepatic failure. Fourteen patients are still on follow-up. CONCLUSION: Budd Chiari syndrome in Saudi Arabia is related to a defined cause in majority of patients (92%). Behcet's disease is a dominant cause of BCS in Saudi Arabia. This is in contrast to world literature where around 50% of patients are of unknown etiology.
BACKGROUND:Budd-chiari syndrome (BCS) is rare disorder, varied in etiology, inconsistent in presentation, unpredictable in progression, and challenging in therapy. AIM OF STUDY: The aim of this study is to review experience of Budd-Chiari syndrome at KFSH, Riyadh; over a ten-year period. METHODS: In a retrospective study using a computer data search of the medical records from May 1990 to May 2000, 43 patients' charts suspected of BCS were reviewed. 29 patients were found to have BCS, diagnosed by Doppler ultrasound, venography, MRI, or CT with or without liver biopsy. FINDINGS: Of the 29 patients, 18 were male (62.1%) and I 1 were female (37.9%); mean age was 35.4 (range 17-69 years). Nine patients had Behcet's disease; seven patients had malignancy (five HCC, one melanoma and one hypernephroma). Eight patients had antiphospholipid syndrome, two patients had no known cause and three (one related to trauma, the other related to protein C deficiency and the third related nephrotic syndrome). Nineteen patients presented acutely with abdominal pain, progressive ascites and hepatomegaly. Ten patients had subacute presentation mimicking cirrhosis of liver. Five patients had web in the intrahepatic IVC or ostium HV and the remaining 24 patients had thrombosis of hepatic veins. Five patients received surgical shunts, three had balloon dilatation of webs, 11 had anticoagulant therapy and seven patients had symptomatic treatment. Eleven patients died, six of them had malignancy, two died while awaiting liver transplantation and three died with fulminant hepatic failure. Fourteen patients are still on follow-up. CONCLUSION: Budd Chiari syndrome in Saudi Arabia is related to a defined cause in majority of patients (92%). Behcet's disease is a dominant cause of BCS in Saudi Arabia. This is in contrast to world literature where around 50% of patients are of unknown etiology.