Aim: Budd–Chiari syndrome, or Hepatic venous outflow obstruction (HVOO) is defined as the obstruction of hepatic venous outflow regardless of its causative mechanism or level of obstruction. Indian studies on BCS date back to the 1970s. It was suggested that in contrast to western world where hepatic vein (HV) obstruction is a commoner IVC obstruction is a commoner in the far east. This study was done to evaluate the aetiology-based prevalence, clinical spectrum with pattern of obstruction, outcome and non-surgical treatment in patients with BCS from southern part of India.

Methods: All the consecutive cases of Budd Chiari syndrome (BCS) or Hepatic Vein Outflow Obstruction (HVOO) in the study period from September 2018 to July 2021 were prospectively evaluated. Diagnosis was based on angiographic evidence of HVOTO (i.e., obstruction of IVC and/or HV). All the patients with BCS were subjected to tests available for hypercoagulable state before starting any treatment and if required was confirmed in the first degree relative for any hypercoagulable state. After the confirmation of the diagnosis patients were taken up for appropriate radiological intervention after discussion with the intervention radiology and anaesthesia team.

Results: Seventy-eight patients, forty-three female and thirty-five males had radiologically confirmed BCS. The mean age at presentation was 35.86 years (range 10–65 years). Twenty-seven patients had acute presentation and fifty-one had chronic illness. Abdominal pain and jaundice were more common in patients with acute disease than the chronic patients but distended veins were more commonly seen in the later. Right upper quadrant pain or epigastric pain (65.38%), distension of the abdomen (75.64%), pedal oedema (41%), UGIB (24.35%) were the primary symptoms and distended veins (66.67%), hepatomegaly (69.2%) were the most common presenting signs. Hypercoagulable state was the etiology in 14 (18%) patients. 48 (61.53%) patients had both IVC and HV obstruction. Associated portal venous thrombosis was seen in 8 (10.25%) patients. Out of the 27 patients that presented with the acute onset of the disease, 2 (7.4%) patients underwent balloon angioplasty of the IVC obstruction.3 (11.1%) patients underwent angioplasty with stenting and they had pure hepatic vein obstruction. 5 (18.5%) patients underwent TIPS procedure. Out of the 51 patients that presented with chronic disease, two third of the patients had radiological features suggestive of established cirrhosis. 12 (23.5%) patients with combined IVC and hepatic vein obstruction underwent angioplasty with stenting.

Conclusion: Combined obstruction is more common in India in contrast to the west. Hypercoagulable states are a common cause of etiology in Indian patients which was reported earlier in very few studies. Combined IVC and HV obstruction can be treated with angioplasty with stenting as demonstrated in our study which is usually difficult to treat. Significant mortality seen with surgical procedures is not seen in patients treated with non-surgical methods. TIPS has been useful in patients with complete HV obstruction with IVC obstruction.    

Keywords: Budd Chiari syndrome; HVOO; TIPS in BCS; Angioplasty in BCS; Acute BCS; Chronic BCS.

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Dr Arnab Dey

DM Hepatology Postgraduate, Institute of Hepatobiliary Sciences, Madras Medical College, Chennai, Tamil Nadu