Hepatic venous outflow tract obstruction are we seeing a change in spectrum; east to west

Author: 
Sunil Chacko Verghese, Rajkumar Solomon T, Venkateswaran Arcot Rajeshwaran, Malarvizhi Murugesan, KaniShaikh Mohamed, Murali Ramamoorthy and Chezhian Annasamy

Background and Aim: Hepatic Venous Outflow Tract Obstruction (HVOTO) is defined as an obstruction predominantly of venous process (thrombosis or phlebitis) or compression or invasion of the hepatic veins and/or the inferior vena cava, independent of the level or mechanism of obstruction, provided the obstruction is not due to cardiac disease, pericardial disease, or sinusoidal obstruction syndrome (veno-occlusive disease).The clinical features and etiology of Budd–Chiari syndrome (BCS) vary from region to region. The aim was to study its presentation at our Center.Methods and Results: 30 consecutive cases of BCS were evaluated during the period from April 2018 till October 2018 at Madras Medical College, Chennai. The diagnosis was made on the basis of MR venography and Doppler ultrasonography. The study population was made up of 16 females (mean age, 37.4 years) and 14 males (mean age, 46.5 years). Nine patients presented with acute disease, while 21 patients had a subacute presentation. Abdominal pain, jaundice, upper gastrointestinal bleed and tender hepatomegaly was the commonest presentation. Ascites and pedal edemawere seen only in 53% of the cases. BCS was caused by a hepatic venous obstruction in 63%, inferior vena cava in 6% and combined obstruction in 31%. Doppler ultrasound accurately detected the site of bock in 24 of 30 patients (80%). Etiologically, five had APLA syndrome, five secondary to extrinsic compression, three had protein C and protein S deficiency, three due to pregnancy and eleven (37%) was idiopathic.Conclusion: Hepatic venous outflow obstruction is a common problem; abdominal pain, tender hepatomegaly and UGI bleed was the common presentation, ascites was seen only in ½ (53%) of the cases showing a changing spectrum compared to various studies. Sub-acute presentation is more frequent and females are at an increased risk. Idiopathic and inherited/acquired hypercoagulable states were the most common etiology.

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DOI: 
http://dx.doi.org/10.24327/ijcar.2019.18449.3526
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