Changing profile of Budd Chiari syndrome in India.

There is increasing evidence that Budd Chiari syndrome occurs when acquired predisposing factor(s) affect a susceptible individual with one or more underlying thrombophilic conditions. Geographical variations in disease pattern of Budd Chiari syndrome exist, which may reflect differing predisposing factors. We review a change in disease profile of Budd Chiari syndrome in India over the past three decades. While earlier studies from India reported isolated inferior vena cava (IVC) obstruction as the commonest disease type, this is a minority in more recent reports where a combination of IVC and hepatic vein obstruction is the commonest type. Longer duration of illness has been shown to be associated with IVC obstruction and the recent change in disease profile in India may reflect earlier diagnosis of Budd Chiari syndrome. Poverty, malnutrition, recurrent bacterial infections and filariasis have been previously suggested as predisposing factors for IVC obstruction. Improvement in hygiene and sanitation may partly explain the recent change in disease profile of Budd Chiari syndrome in India.

Direct intrahepatic cavo-portal shunts in Budd-Chiari syndrome: Role of simultaneous fluoroscopy and trans-abdominal ultrasonography.

BACKGROUND: Transjugular intrahepatic porto-systemic shunt (TIPS) for Budd-Chiari syndrome (BCS) can be inserted from inferior vena cava or hepatic vein to portal vein. The former is performed when hepatic veins are not suitable and is technically more challenging. METHODS: In this retrospective study, 7 patients with chronic BCS needed cavo-portal shunt as hepatic veins were neither amenable to plasty nor provided access for TIPS placement. Simultaneous fluoroscopic and trans-abdominal ultrasound guidance was used at the time of portal vein puncture. RESULTS: Technical success and clinical improvement were obtained in all patients. Median 3 (range 1-4) attempts were needed to puncture the portal vein. There were no significant complications. Uncovered stents were used in six patients and stent occlusion was common, but could be managed by re-intervention. CONCLUSION: Cavo-portal shunt is an effective technique for patients with BCS uncontrolled by medical therapy. Additional trans-abdominal ultrasound in oblique parasagittal plane keeps the procedure safe.