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THE SOUTH AFRICAN GASTROENTEROLOGY REVIEW 2022 | VOLUME 20 | ISSUE 2 | 18 CASE REPORT Introduction Cavernous transformation of the portal vein (CTPV) is characterized by the formation of periportal venous channels within or around a thrombosed portal vein. It is usually a sign of a chronic venous portal vein thrombosis. It was first described in 1869 by Balfour and Stewart as “thrombosis and varicose dilation of the portal vein leading to splenomegaly and ascites.” 1 Acute portal vein thrombosis can occur secondary to portal pyemia, complicated intra-abdominal infections, myeloproliferative disorders, pancreatitis, cirrhosis, malignancy, trauma, medication, e.g., combined oral contraceptives, hypercoagulable disorders like HIV- associated thrombophilia, as well as prolonged venous stasis in cases of constrictive pericarditis. 2-4 Case A 33-year-old female patient was referred to Internal Medicine from the gynaecological department for work-up of massive ascites. The patient gave a history of progressive abdominal swelling, weight loss and dyspnoea over a period of four years. The working diagnosis at that stage was that of a possible ovarian mass or malignancy, based on an elevated serum Ca- 125 level of 515 kU/L (0-35). Repeated ascites taps were performed that showed no malignant or mesothelial cells, only lymphocytes and red blood cells. On admission to the Internal Medicine Department the blood results showed the following: Na 140 mmol/l, K 3 mmol/l ,Urea 4.6 mmol/l, Creatinine 58 μmol/l, total protein 76 g/L, albumin 12 g/L, total bilirubin 3 μmol/L, conjugated bilirubin 3 μmol/L, ALT 28 U/L, AST 28 U/L, ALP 218 U/L, GGT 94 U/L, LDH 551 U/L. Alpha feto- protein levels were 4.2 μg/L, CA 125 1281 kU/L. The full blood count showed a haemoglobin of 9 g/dL with an MCV of 120 fL. The platelet count was 120 x 10 9 /L and INR 2. The ammonia level was 77μmol/L, cholinesterase 3848 U/L. A urine protein: creatinine ratio within normal limits excluded nephrotic syndrome as a cause of the low albumin and ascites. The serum ascites albumin gradient on a repeat tap was more than 11g/L and in keeping with ascites related to portal hypertension. Ascites fluid adenosine deaminase levels (2.4 U/L), as well as Mycobacterium tuberculosis cultures and Gene-Xpert analysis of the ascites fluid were negative. The transabdominal ultrasound findings showed a liver surrounded by ascites. The surface of the liver appeared irregular. The hepatic parenchyma was coarse and heterogeneous and no hypoechoic liver nodules were demonstrated. The gallbladder wall was thickened and oedematous, most likely related to the extensive ascites. Splenomegaly was present. The portal vein could not be demonstrated. In the porta hepatitis, several small blood vessels were visualized on Colour Flow Doppler in the area where the portal vein is usually demonstrated. This finding was in keeping with cavernous transformation of the portal vein. Thrombus was also noted in the inferior vena cava (IVC). These findings were confirmed on CT scan, with no demonstrable ovarian mass. The CT scan findings were also positive for pulmonary embolism. Oesophageal varices were demonstrated on gastroscopy. An extensive workup for causes of portal venous thrombosis was performed. Protein C was 74 IU/dL, free protein S levels were 67 %, ANF negative, Lupus anticoagulant and anti-cardiolipin antibodies negative as well as hepatitis serology. Factor V Leiden PCR and Prothrombin G 20210A PCR were negative. JAK-2 PCR was negative as well as flow cytometry (CD 55/59) for Paroxysmal Nocturnal Haemoglobinuria. Human immunodeficiency virus serology was positive with a CD4 count of 155 with a viral load 1245. HIV-associated L Bösenberg, P Thomas Department of Internal Medicine, Steve Biko Academic Hospital, University of Pretoria, Pretoria, South Africa Cavernous transformation of the portal vein a rare phenomenon Abstract: Cavernous transformation of the portal vein is a complication of a prior portal vein thrombosis and carries a very poor prognosis. It is associated with portal hypertension, and the massive ascites that develops as a result, mimics other clinical conditions that may puzzle physicians. Elevated levels of Ca-125 is often seen in patients with associated underlying chronic liver disease, which in turn may lead to a wild goose chase for ovarian carcinoma. Portal vein thrombosis in HIV-positive patients, most likely as a result of HIV-associated thrombosis, must be considered in all HIV patients who present with features of portal hypertension and ascites. Keywords: portal vein thrombosis, cavernous transformation of portal vein, Ca-125 levels, HIV-associated thrombosis Correspondence L Bösenberg email: liesel.bosenberg@up.ac.za