Saudi Journal of Gastroenterology
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Year : 2003  |  Volume : 9  |  Issue : 1  |  Page : 11-14
Budd chiari syndrome: Experience at King Faisal specialist hospital and research centre, Riyadh, Saudi Arabia


Department of Medicine, King Faisal Specialist Hospital and Research Centre, Riyadh, Saudi Arabia

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Date of Submission29-May-2002
Date of Acceptance30-Nov-2002
 

   Abstract 

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

Keywords: Budd Chiari Syndrome, Behcet′s disease.

How to cite this article:
Al Himali SH, Al Osaimi AM, Dahab ST, Khuroo MS, Al Ashgar HI. Budd chiari syndrome: Experience at King Faisal specialist hospital and research centre, Riyadh, Saudi Arabia. Saudi J Gastroenterol 2003;9:11-4

How to cite this URL:
Al Himali SH, Al Osaimi AM, Dahab ST, Khuroo MS, Al Ashgar HI. Budd chiari syndrome: Experience at King Faisal specialist hospital and research centre, Riyadh, Saudi Arabia. Saudi J Gastroenterol [serial online] 2003 [cited 2020 Jul 5];9:11-4. Available from: http://www.saudijgastro.com/text.asp?2003/9/1/11/33361


Budd chiari syndrome (BCS) consists of hepatic venous outflow obstruction. The initial definition of the syndrome was symptomatic occlusion of the hepatic veins [1] . With increasing report in the involvement of the hepatic portion of inferior vena cava associated with hepatic vein occlusion it is then redefined as obstruction to hepatic vein outflow [2] . Clinically characterized by a triad of abdominal pain, hepatomegaly and ascites, the initial studies of BCS described it as an acute disorders characterized by abdominal pain, hepatomegaly, ascites and rapidly progressive hepatic failure. Later on a more insidious presentation characterized by abdominal distension, portal hypertension, and tortuously engorged abdominal vein.

Most of the reports about acute BCS come from Western countries [3] . The etiology cannot be determined in half to two thirds of cases. Causes include trauma, hypercoagulable state such as myeloproliferative disease, pregnancy, oral contraceptive, paroxismal nocturnal hemoglobinemia, neoplasia, Behcet's syndrome, congenital abnormalities [4] . The etiology differed between countries. The etiology in Western countries includes hypercoagulable states, primary myeloproliferatives syndromes, steroidal contraceptives, and tumors [5],[6],[7],[8],[9] . While inferior vena cava or membranous obstruction of the inferior vena cava, pregnancy and infection were implicated in patients from Asian countries. The clinical course varied from survival to death [10],[11],[12],[13],[14],[15],[16] .

BCS remains an uncommon disease and needs a high index of suspicion. Increased awareness of the BCS and combined utilization of percutaneous liver biopsy and hepatic angiography had lead to earlier diagnosis. The routine biochemical determination of aminotransferase and alkaline phosphatase are of little value in the diagnosis of patients with suspected BCS. The characteristic of ascetic fluid is highly variable. Acutely, liver biopsy shows features of centrizonal congestion, cell necrosis and hemorrhage [17] . This lesion evolves into cirrhosis in chronic cases. Doppler ultrasound is the diagnostic procedure of choice. The typical ultrasound feature of BCS include inability to visualize normal hepatic venous connection to the vena cava, comma shaped intrahepatic collateral vessels and absence of any wave form in the hepatic veins. Magnetic resonance imaging and computed tomography may also yield characteristic finding but do not add much to the finding from an adequate ultrasound examination.


   Patients and Methods Top


All patients diagnosed with BCS from May 1990 to May 2000 at KFSH & RC were identified by a computer search of medical records. 43 patients' charts with suspicion of BCS were reviewed and 29 patients' charts were found to have BCS. The diagnosis of BCS was based on presence of obstruction of hepatic vein or inferior vena cava as shown on U/S Doppler, CT scan or MRI abdomen. Two pathologists have reviewed liver biopsy slides. All patients were followed from the date of diagnosis until May 2002. There was some heterogeneity in the investigation and management of those patients and that was mainly due to a long period of follow up (10 years) with different consultants, assistants and fellows.


   Results Top


Twenty-nine patients have fulfilled the criteria, { 18 male and 11 female (M: F 1.6:1)}. Age at diagnosis varied between 17-69 years (mean 35.4). No difference in age between male and female. The duration of symptoms varied between two weeks and two years. All of the patients had abnormal liver function test and ascites at the time of presentation. Twenty-two patients presented acutely with two-week history of abdominal pain and ascites while seven patients have symptoms of abdominal pain and progressive abdominal distention for more than six-week time.

Twenty-two patients had Doppler ultrasound (75%), CT was performed in nine patients (31%), venography in ten '(34%), MRI in three (10%), liver biopsy in ten (17%) and MRA in one patient (3.4%). Thromboses were found in 24 patients (82.7%), and five patients had web (17.2%). The etiology was varied. Behcet's disease was found in nine patients (37.5%). Eight other were treated with steroids­diuretics-anticoagulant and survived, whereas one was severely ill and died with massive thrombosis. Hypercoagulable state was found in nine patients (37.5%), malignancy in seven (24.1%) and no cause was documented in two patients (6.8%). Other associated conditions were drug abuse in one patient and RTA in another. The management was medical in form of anticoagulation in 13 patients (44.8%), streptokinase, heparin, and steroid for those who had Behcet's disease, dilation with balloon was performed in three patients (10.3%), surgical treatment in five patients (17%) and symptomatic treatment in nine patients (37.5%). Those patients who survived were treated according to primary cause, either medically in the form of steroids or diuretics or anticoagulant all together. Those who died were either critically ill or had malignancy and treated conservatively. TIPS was considered in two patients who were waiting for liver transplant as suggested in the literature [18],[19] . Fourteen patients are still following up with regular Doppler ultrasound and doing well. Four patients missed follow up. Death occurred in 11 patients (37.9%), three were related to fulminant hepatic failure (10.3%), six related to malignancy (20.6%) and two while awaiting liver transplant (6.8%).


   Discussion Top


The Budd Chiari Syndrome is an unusual disorder characterized by hepatic venous outflow obstruction either within the liver or in the inferior vena cava between the liver and right atrium. The association between Budd Chiari Syndrome and Behcet's syndrome is well recognized. Behcet's syndrome has been observed all over the world, but the largest number of cases has been reported from the Middle East and Mediterranean countries such as Turkey, Greece, Italy and from Japan [19],[20],[21] .

The peak age of onset of BCS is in the third decade of life. There were two major presentations, the classic presentation of Budd Chiari Syndrome in 24 patients, and five patients present are with upper gastrointestinal bleeding and cirrhosis, and all of the five were found to have malignancy. The inferior versa cava was thrombosed in most patients of Behcet's. The etiology was related to Behcet's and hypercoagulable state in 37.5% each, and this is comparable to studies from Turkey in which 42.4% of cases of BCS were related to Behcet's. The idiopathic form of BCS was found in two of our patients (6.8%), a figure which is low in comparison to worldwide literature [22] .


   Conclusion Top


Budd Chiari Syndrome is rare disease and difficult to diagnose. It needs to be suspected in all patients presenting acutely with hepatomegaly and ascites. A high index of suspicion may hasten diagnosis and management.

 
   References Top

1.Valla DC. Hepatic vein thrombosis (BCS). Seminars in Liver Disease 2002; 22: 5-14.  Back to cited text no. 1  [PUBMED]  [FULLTEXT]
2.Reynolds TB. Budd Chiari Syndrome, in: Schiff L, Schiff ER, eds. Disease of the liver. Philadelphia: Lippincott 1993: 1091-8.  Back to cited text no. 2    
3.Parker RGF. Occlusion of the hepatic veins in man. Medicine 1959,-38: 369-402.  Back to cited text no. 3    
4.Murphy FB, Steinberg HV, Shires III GT, et al: The Budd-Chiari syndrome: A review. AJR 1986; 147: 9­-15.  Back to cited text no. 4    
5.Mitchell MC, Boitnott JK, Kaufman S, et al: Budd-­Chiari Syndrome: Etiology, diagnosis and management. Medicine 1982; 61: 199-218.  Back to cited text no. 5    
6.Valla D. Casadevall N, Lacombe C, et at: Primary myeloproliferative disorder and hepatic vein thrombosis. Ann Intern Med 1985; 103: 329-34.  Back to cited text no. 6    
7.Valla D, Le MG, Poynard T, et al: Risk of hepatic vein thrombosis in relation to recent use of oral contraceptives. Gastroenterology 1986; 90: 807-11.  Back to cited text no. 7    
8.Valla D, Dhurneaux D, Babany C, et al: Hepatic vein thrombosis in paroxysmal nocturnal hemoglobinuria. Gastroenterology 1987; 93: 569-75.  Back to cited text no. 8    
9.Datta DV, Saha S, Samanta AKS, et al: Chronic Budd-Chiari syndrome due to obstruction of the intrahepatic portion of the inferior vena cava. GUT 1972; 13: 372-78.  Back to cited text no. 9    
10.Nakamura T, Nakamura S, Aikawa T, et al: Obstruction of the inferior vena cava in the hepatic portion and the hepatic veins. Angiology 1968; 17: 479-98.  Back to cited text no. 10    
11.Rector WG, Xu Y, Goldstein, et al. Membranous obstruction of the inferior vena cava in the United States. Medicine 1985; 64: 134-43.  Back to cited text no. 11    
12.Simson IW: Membranous obstruction of the inferior vena cava and hepatocellular carcinoma in South Africa. Gastroenterology 1982; 82: 171-8.  Back to cited text no. 12    
13.Khuroo MS, Datta DV: Budd-chiari syndrome following pregnancy. Am J Med 1980; 68: 113-21.  Back to cited text no. 13    
14.Aikat BK, Bhusnurmath SR, Chhuttani PN, Datta DV: Hepatic vein obstruction - A retrospective analysis of 72 autopsies and biopsies. Ind J Med Res 1978; 67: 128-44.  Back to cited text no. 14    
15.Gupta S, Blumgart LH, Hodgson HJF: Budd-chiari syndrome: Long term survival and factors affecting mortality. QJ Med 1986; 60: 781-91.  Back to cited text no. 15    
16.Ludwig J, Hashimoto E, McGill DB, et al. Classification of hepatic venous outflow obstruction: Ambiguous terminology of the Budd-chiari syndrome. Mayo Clin Proc 1990; 65: 51-5.  Back to cited text no. 16  [PUBMED]  
17.Tang TJ, Batts KP, de Groen PC, et al. The prognostic value of histology in assessment of patients with BCS. Journal ofHepatology 2001; 35: 338-43.  Back to cited text no. 17    
18.Seki S, Sakaguchi 1-I, Kobayashi S, et al. Transjugular intrahepataic portosystemic shunt by direct transcaval approach in patients with acute and hyperacute BCS. European Journal of Gastroenterology and Hepatology 2002; 14: 567-71.  Back to cited text no. 18    
19.Gasparini D, Del Forno M, Sponza M, et al. Transjugular intrahepatic portosystemic shunt in combination with oral anticoagulant for BCS. Hepato Gastroenterology 2001; 48: 1447-9.  Back to cited text no. 19    
20.Milkey D, Yocum DE, Oberley TD, et al. Budd­Chiari syndrome and renal failure in Behcet's disease. Am J Med 1983; 75: 541-50.  Back to cited text no. 20    
21.Shimizu T, Erlich GE, Inaba G, Hayashi K. Behcet's disease (Behcet's syndrome). Semin Arthritis Rheum 1979; 8: 223-259.  Back to cited text no. 21    
22.Chajek T, Painaru M. Behcet's disease. Report of 41 cases and a review of the literature. Medicine 1975; 54: 179-196.  Back to cited text no. 22    

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Correspondence Address:
Hamad Ibrahim Al Ashgar
Consultant Hepatologist /Gastroenterologist, Department of Medicine, King Faisal Specialist Hospital and Research Centre P.O. Box 3354, Riyadh 11211
Saudi Arabia
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Source of Support: None, Conflict of Interest: None


PMID: 19861804

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