Rapid Communication
Copyright ©2008 The WJG Press and Baishideng. All rights reserved.
World J Gastroenterol. Jan 14, 2008; 14(2): 278-285
Published online Jan 14, 2008. doi: 10.3748/wjg.14.278
Changing spectrum of Budd-Chiari syndrome in India with special reference to non-surgical treatment
Deepak N Amarapurkar, Sundeep J Punamiya, Nikhil D Patel
Deepak N Amarapurkar, Nikhil D Patel, Department of Gastroenterology, Bombay Hospital and Medical Research Centre, Mumbai, India
Sundeep J Punamiya, Department of Interventional Radiology, Bombay Hospital and Medical Research Centre, Mumbai, India
Correspondence to: Dr. Deepak N Amarapurkar, D-401 Ameya Society, New Prabhadevi Road, Mumbai 400025, India. amarapurkar@gmail.com
Telephone: +91-22-22067676
Fax: +91-22-24368623
Received: July 19, 2007
Revised: October 13, 2007
Published online: January 14, 2008
Abstract

AIM: To evaluate patterns of obstruction, etiological spectrum and non-surgical treatment in patients with Budd-Chiari syndrome in India.

METHODS: Forty-nine consecutive cases of Budd-Chiari syndrome (BCS) were prospectively evaluated. All patients with refractory ascites or deteriorating liver function were, depending on morphology of inferior vena cava (IVC) and/or hepatic vein (HV) obstruction, triaged for radiological intervention, in addition to anticoagulation therapy. Asymptomatic patients, patients with diuretic-responsive ascites and stable liver function, and patients unwilling for surgical intervention were treated symptomatically with anticoagulation.

RESULTS: Mean duration of symptoms was 41.5 ± 11.2 (range = 1-240) mo. HV thrombosis (HVT) was present in 29 (59.1%), IVC thrombosis in eight (16.3%), membranous obstruction of IVC in two (4%) and both IVC-HV thrombosis in 10 (20.4%) cases. Of 35 cases tested for hypercoagulability, 27 (77.1%) were positive for one or more hypercoagulable states. Radiological intervention was technically successful in 37/38 (97.3%): IVC stenting in seven (18.9%), IVC balloon angioplasty in two (5.4%), combined IVC-HV stenting in two (5.4%), HV stenting in 11 (29.7%), transjugular intrahepatic portosystemic shunt (TIPS) in 13 (35.1%) and combined TIPS-IVC stenting in two (5.4%). Complications encountered in follow-up: death in five, re-stenosis of the stent in five (17.1%), hepatic encephalopathy in two and hepatocellular carcinoma in one patient. Of nine patients treated medically, two showed complete resolution of HVT.

CONCLUSION: In our series, HVT was the predominant cause of BCS. In the last five years with the availability of sophisticated tests for hypercoagulability, etiologies were defined in 85.7% of cases. Non-surgical management was successful in most cases.

Keywords: Budd-Chiari syndrome; Interventional radiology; Ascites; Hepatic vein thrombosis; Percutaneous transluminal angioplasty; Stent; Transjugular intrahepatic portosystemic shunt; Thrombophilia