SAGES Magazine

THE SOUTH AFRICAN GASTROENTEROLOGY REVIEW 2022 | VOLUME 20 | ISSUE 2 | 20 CASE REPORT following an acute thrombosis. Through the process of neoangiogenesis, a “sponge-like” mass develops at the porta hepatis, whereby the portal vein’s usual anatomy is no longer identifiable within the cavernoma on transabdominal ultrasound. 12-14 Intrahepatic extension of the cavernoma is not uncommon, and leads to intrahepatic shunts. A bypass route is established around an obstructed portal vein to patent intrahepatic branches of the portal vein and displays normal resistance patterns on Doppler interrogation. This newly established network of venous channels acts as a porto-portal shunt and the Colour Doppler flow demonstrated in these vessels is hepatopetal. Despite the development of these intra- and extra hepatic shunts, portal hypertension persists in these patients. 12-13 Clinically, an acute portal vein thrombus presents with nausea, vomiting, and abdominal pain due to intestinal ischemia and congestion, as well as haematochezia. Chronic venous thrombosis tends to be asymptomatic, but patients develop features of portal hypertension e.g. ascites and splenomegaly. 9 The greyscale ultrasound features that support the diagnosis of CTPV are: • A demonstrable filling defect in the portal vein that displays hypoechoic in acute PVT and echogenic in cases of chronic thrombosis. 9 • The absence of flow in the portal vein on Colour Flow Doppler (if the remnant is still visible). 9,12 • Multiple varicose, venous channels in the porta hepatis that displays hepatopetal flow. 12 • Concurrent compensatory changes in the hepatic artery. Normally a vessel that displays low resistance flow and pulsatility, it may now show increased flow and an increased hepatic artery resistance index (HARI) >0.7 in response to the decreased portal venous flow and venous congestion. In established cirrhosis with portal hypertension, the HARI decreases to < 0.55. 15 • Associated ascites and massive splenomegaly. 9,16 Cavernous transformation of the portal vein is deemed incurable. Treatment options are therefore aimed at early diagnosis and management of the thrombosed portal vein. The management of an acute portal vein thrombosis requires systemic anticoagulation. Surgical procedures and portal vein reconstruction is described with variable success in chronic cases. Patients with chronic portal vein thrombosis are at risk of developing portal cavernous cholangiopathy, whereby portosystemic collateral vessels surrounding the common bile duct cause obstruction in 0.5-1% of cases. 9 CTPV is an unfortunate fatal complication of portal venous thrombosis. Invariably, it is associated with portal hypertension and its complications e.g. intractable, massive ascites and ultimately gastro- intestinal variceal bleeds. Ultrasound diagnosis of CTPV relies on the presence of multiple Colour Flow Doppler signals in the area of the porta hepatis indicating collateral venous channels, in the absence of a demonstrable portal vein. 9,12 Acknowledgements We thank Prof. J Ker who provided guidance and assistance. No compensation or commercial funding was received for the purpose of this case report. References 1. Shamim SMS, Higham A. Cavernous transformation of portal vein - a rare cause of massive hepatomegaly. Journal of Postgraduate Medical Institute. 2013; 27:223-7. 2. Chawla YK, Bodh V. Portal vein thrombosis. J Clin Exp Hepatol. 2015; 5(1):22-40. doi:10.1016/j.jceh.2014.12.008 3. Rosu A, Searpe C, Popescu M. Portal vein thrombosis with cavernous transformation in myeloproliferative disorders: Review update. 2012. 4. Bibas M, Biava G, Antinori A. Hiv-associated venous thromboembolism. Mediterr J Hematol Infect Dis. 2011; 3(1):e2011030. doi:10.4084/mjhid.2011.030 5. Ramanampamonjy RM, Ramarozatovo LS, Bonnet F, Lacoste D, Rambeloarisoa J, Bernard N, et al. [portal vein thrombosis in hiv-infected patients: Report of four cases]. Rev Med Interne. 2005; 26(7):545-8. doi:10.1016/j. revmed.2005.04.023 6. Soentjens P, Ostyn B, Van Outryve S, Ysebaert D, Vekemans M, Colebunders R. Portal vein thrombosis in a patient with hiv treated with a protease inhibitor-containing regimen. Acta Clin Belg. 2006; 61(1):24-9. doi:10.1179/ acb.2006.005 7. Edula RG, Muthukuru S, Moroianu S, Wang Y, Lingiah V, Fung P, et al. Ca-125 significance in cirrhosis and correlation with disease severity and portal hypertension: A retrospective study. J Clin Transl Hepatol. 2018; 6(3):241-6. doi:10.14218/jcth.2017.00070 8. Devarbhavi H, Kaese D, Williams AW, Rakela J, Klee GG, Kamath PS. Cancer antigen 125 in patients with chronic liver disease. Mayo Clin Proc. 2002; 77(6):538-41. doi:10.4065/77.6.538 9. 9. Owen C, Meyers P. Sonographic evaluation of the portal and hepatic systems. Journal of Diagnostic Medical Sonography. 2006; 22(5):317-28. doi:10.1177/8756479306293101 10.Ogren M, Bergqvist D, Björck M, Acosta S, Eriksson H, Sternby NH. Portal vein thrombosis: Prevalence, patient characteristics and lifetime risk: A population study based on 23,796 consecutive autopsies. World J Gastroenterol. 2006; 12(13):2115-9. doi:10.3748/wjg.v12.i13.2115 11.Ma J, Yan Z, Luo J, Liu Q, Wang J, Qiu S. Rational classification of portal vein thrombosis and its clinical significance. PLoS One. 2014; 9(11):e112501-e. doi:10.1371/journal.pone.0112501 12.Portal cavernoma. In: Baert AL, editor. Encyclopedia of diagnostic imaging. Berlin, Heidelberg: Springer Berlin Heidelberg; 2008. p. 1520-. 13.Elsayes KM, Shaaban AM, Rothan SM, Javadi S, Madrazo BL, Castillo RP, et al. A comprehensive approach to hepatic vascular disease. Radiographics. 2017; 37(3):813-36. doi:10.1148/rg.2017160161 14.Berzigotti A, Piscaglia F. Ultrasound in portal hypertension - part 1. Ultraschall in der Medizin (Stuttgart, Germany : 1980). 2011; 32:548-68; quiz 69. doi:10.1055/s-0031-1281856 15.McNaughton DA, Abu-Yousef MM. Doppler us of the liver made simple. Radiographics. 2011; 31(1):161-88. doi:10.1148/rg.311105093 16.Scoutt LM, Thorisson H, Hamper U. Ultrasound evaluation of the portal and hepatic veins. 2012. p. 242-74.