BuddChiari Syndrome Introduction BuddChiari Syndrome is the obstruction

Budd-Chiari Syndrome Introduction Budd-Chiari Syndrome is the obstruction of hepatic vein/s outflow (Figure 1 & 3) or the extrahepatic inferior vena cava. Thrombosis, stenosis, or membranous web blockages can occur in these areas inhibiting hepatic blood flow (Hagen-Ansert, 2018). When hepatic venous obstruction causes a prolonged increase in pressure within these anastomoses, they can become enlarged (Cai et al. , 2015). There are two classifications of Budd-Chiari syndrome. Primary is a congenital obstruction of the hepatic veins. Secondary is related to the invasion of the IVC and hepatic veins by hepatocellular carcinoma, abscess, cysts, etc. (Grus et al. , 2017). Ultrasound, paired with color Doppler, is the first-choice imaging modality for Budd-Chiari syndrome because of its specificity, sensitivity and lack of harmful ionizing radiation. Figure 1. Visualization of the left hepatic vein stenosis and occlusion with right hepatic vein plaque build-up. Predisposing Factors The common causes of the syndrome are congenital abnormalities, coagulation abnormalities, pregnancy, prolonged oral contraceptive use, tumor extension, infection, malnutrition, and trauma (Hagen. Ansert, 2018) (Mukund & Sarin, 2018). Prothrombic factors and attained conditions attributing to Budd-Chiari diagnosis include; myeloproliferative disease, Behcet disease, antiphospholipid antibodies syndrome, protein S deficiency, thrombocythemia, celiac disease, hepatocellular carcinoma, Gravitz tumor, and previous liver resection. Clinical Presentation Blood Flow Patient presentation depends on the location of the obstruction, and whether it is classified as primary or secondary Budd-Chiari Syndrome. Clinical presentation ranges from asymptomatic thrombosis to liver failure from liver congestion (Iliescu et al. , 2019). In order to be clinically manifested, two or more of the hepatic veins must be blocked. Signs and symptoms are jaundice, upper abdominal pain, increased blood pressure, ascites, hepatomegaly, and splenomegaly (Rautou, 2014). Ultrasonic Doppler is used to visualize thrombosis, stenosis, complete occlusion, anastomoses, reversal of blood flow, and changes in velocity of blood flow. Doppler signals change from normal hepatofugal (Figure 2), phasic flow (Figure 4) to either absent, reversed, turbulent, or continuous (Hagen-Ansert, 2018). The flow will be absent if the hepatic vein becomes completely occluded (Figure 3). Reversal of flow occurs due to hepatic hypertension from hepatic congestion of the sinusoids (Figure 5). Turbulent flow can occur directly after areas of stenosis due to the change in pressure and velocity within the vessel. In areas of stenosis, blood flow velocity increases. Figure 4. Normal, phasic blood flow in the right hepatic vein. Sonographic Evaluation Diagnostic ultrasound is crucial in determining the best treatment options for each patient. Imaging techniques may reveal direct signs of Budd-Chiari syndrome including: dilated veins with absent, inverted or turbulent flow, large intrahepatic or subcapsular venous collaterals, spider-web appearance in vein openings, hypoechoic fibrous cords in place of normal veins, and/or an absent or flat wave-form. Indirect signs of Budd-Chiari syndrome via ultrasound include enlarged caudate lobe and atrophy of affected liver segments. The liver may appear hypoechoic in early stages of thrombosis and progresses to appear hyperechoic and heterogeneous (Hagen-Ansert, 2018). Figure 5. Retrofugal blood flow in the right hepatic vein from flow reversal. Compare to normal flow (Figure 3). Diagnosis Diagnostic methods include ultrasound, magnetic resonance imaging, computed tomographic imaging, liver biopsy, and liver function tests. Ultrasound provides the ability to diagnose the, monitor progression and severity, explore underlying pathology, determine the velocity of blood flow with color Doppler, explore individualized treatment options, and provide follow up post-diagnosis (Iliescu et al. , 2019). Ultrasound has played a major part in improving patient life expectancy with Budd. Chiari syndrome; from nearly always fatal, in the 1980 s, to a 90% fiveyear survival rate in 2019 (Faraoun et al, 2016). Treatment of this disorder includes a multitude of different options based on the severity and presentation of Budd-Chiari Syndrome. The primary medical management is anticoagulation therapy to prevent further venous thrombosis, and to control portal hypertension and ascites (Cai et al. , 2015). Endovascular treatment is used to relieve hepatic congestion and restore hepatocyte perfusion. Surgical methods include resection of membranes with or without inferior vena cava reconstruction, liver transplants, and shunts (Cai et al. , 2015). The transjugular intrahepatic portosystemic shunt is placed to redirect blood flow. Liver transplantation may be performed on patients with a good prognosis as a last resort and results in a 95% survival rate (Narayanan Menon et al. , 2004). References Figure 2. Normal, hepatofugal blood flow. Figure 3. Occlusion of the right hepatic vein with color Doppler. Aydinli, M. , & Bayraktar, Y. (2007). Budd-Chiari syndrome: Etiology, pathogenesis and diagnosis. World of Journal Gastroenterology 12(19), 2693 -2696. doi: 10. 3748/wj g. v 13. i 19. 2693 Cai, S. F. , Gai, Y. H. , Ma, S. , Liang, B. , Wang, G. C. , & Liu Q. W. (2015). Ultrasonographic Visualization of Accessory Hepatic Veins and Their Lesions in Budd-Chiari Syndrome. Ultrasound In Medicine, 41(8), 2091 -8. Faraoun, S. A. , Boudjella, M. E. 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