Our research team presents the case of a 22 years old, male patient, N. S. who had cavernous transformation of the portal vein at 2 years old. Studies show that many cases were caused by embryological malformation, inherited or acquired prothrombotic disorders such as protein C, protein S deficiency, antiphospholipid syndrome was excluded for the patient we report. In the case of our patient, the factor causing the portal vein was idiopathic, although embryological malformation can t be discarded because we don t have a neo-natal abdominal ultrasonography.
He developed portal hypertension, thrombocytopenia and hypersplenism at the age of 3. Our patient was admitted to the hospital in numerous occasions for life threatening hematemesis and melena due to variceal bleeding and underwent several times variceal ligation. Unfortunately endoscopic therapy is used mainly for temporary hemostasis in acute variceal bleeding, moreover without an early portal decompression for a young patient the risk of other biliary complications remains high. So surgical shunts represent a good and rapid alternative to prevent gastroesophageal bleeding, for our patient a Warren shunt was life-saving. At 13year old, our patient underwent surgery and Warren Shunt- (anastomosis of the splenic vein and the left renal vein) was performed in order to lower the PHT. After the surgery, he did not present gastrointestinal bleeding and no other complications due to PHT and his general state was stable.
In the light of this medical history the patient has to monitor his liver disease, every three- six months, all his life.
Although sometimes, a sufficiently large cavernous transformation of PV can encircle the common bile duct at porta hepatis, causing biliary obstruction with subsequent dilatation of the biliary tree, an entity described as portal biliopathy. In the case we report it was no biliary obstruction, our patient total and direct bilirubin were within normal limits despite the large collaterals surrounding the portal vein. Although his portal vein measured 13 cm, he presented splenomegaly (15 cm the long ax), with symptomatic hypersplenism (thrombocytopenia -105 platelets). Luckily N. S has no ascites, which is a condition that we have to close evaluate in order to prevent other decompensations, also the presence of ascites may be of great importance to predict mortality in young patients with CTPV due to its correlation with the deterioration of liver function.
Our patient also continues with 40 mg of Propranolol /day and diuretics. Our patient had to follow up by doppler ultrasonography the function of the distal Spleen-Renal Shunt (DSRS) which is fashioned between the splenic and the left renal vein, for our patient was the best choice to reduce the risk of encephalopathy compared with other nonselective shunts. Selective shunts such as the distal splenorenal developed by Warren have been considered effective in controlling variceal bleeding, also preserves a portion of portal perfusion to the liver and is better in preventing portosystemic encephalopathy when compared with TIPS (transjugular intrahepatic portosystemic shunt ). This is also the preferred surgical procedure when the anatomy is unfavourable for and can be offered to patients who demand a “one-time” treatment and have symptomatic hypersplenism. [5,7,8].
Major acute complications of Warren Shunt include ascites, infection and liver failure. However, DSRS has a lower reintervention rate than TIPS [7].
Taking into consideration that thrombosis may be a long-term complication of the Warren Shunt, this is a condition that has to be prevented and can be which can be easily detected using abdominal Doppler ultrasonography, as it allows direct visualisation of the shunt, and can reveal splenofugal drainage of blood into the left renal vein. [3,7,8]. Doppler sonography is a trustworthy procedure to accomplish the challenge of monitoring a cavernous portal vein transformation in a young patient, due to its non-invasive, generally painless, as well as the fact that it does not use radiation. [4,6,7]. Doppler ultrasound is essential in detecting a recently formed thrombus that is virtually anechoic in a Warren Shunt, a proper follow-up is life saving and can prevent further complications. Abdominal Doppler ultrasonography is the gold standard for long-term monitoring a cavernous portal vein transformation in a young patient. It has a high degree of accuracy in detecting and monitoring portal cavernomas due to its reliable, non-invasive technique, generally painless, and indefinite repetitions, compared to other radiation-based imaging techniques [3,4]. We want to lay emphasis on the importance of colour and /or pulsed Doppler sonography in order to evaluate the blood flow in the cavernous portal vein transformation, in its segmental branches, in the hepatic vein and examine the spleno-renal shunt, and the importance of realizing a Warren shunt as a reliable treatment in order to prevent further decompensations of the liver [4,5].