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Case Reference No. CC-1197-01 A 32-year-old man with liver failure, splenomegaly, thrombocytopenia, and elevated bleeding time had undergone transvenous liver biopsy and hepatic vein hemodynamic assessment 2 months prior to this most recent presentation. The biopsy showed focal fatty changes in the liver and a single portal area with fibrosis and chronic inflammation. At that time, pressure measurements had indicated a normal corrected sinusoidal pressure of 4 mm Hg. However, subsequent CT scanning (not shown) and endoscopy had revealed evidence of gastroesophageal varices. We were asked to repeat hepatic venous hemodynamic assessment based on the suspicion that the initial pressure measurements were erroneous. The hepatic venous system was catheterized via a right internal jugular vein approach. Multiple balloon occlusion pressure measurements were taken in various positions in the hepatic venous system (Fig. 1), resulting in variable corrected sinusoidal pressure measurements ranging from 3 to 10 mm Hg. The prior CT scan had suggested the presence of a patent paraumbilical venous collateral. As we had previously had some success at catheterizing such collaterals percutaneously (predominantly as a guide for TIPS placement), we considered attempting portal catheterization via this route to obtain direct portal pressure measurements. The small size of the vein and significant respiratory motion of the abdomen precluded catheterization in the umbilical fascia of the anterior abdominal wall. However, we were able to trace the course of the collateral with ultrasound and note that it joined the right inferior epigastric vein. This in turn was noted to enter (as would be expected) the anterior aspect of the right external iliac vein. Noting this, we catheterized the right common femoral vein and searched for the orifice of the right inferior epigastric vein with an angle-tip catheter and steerable hydrophilic guide wire. Once the orifice was engaged, attempts were made to catheterize the portal vein. Although this proved somewhat arduous, intermittent injections of contrast material and continued manipulation of the steerable wire ultimately proved successful (Figs. 2, 3). A direct portogram showed large varices (Fig. 4). Repeat pressure measurements yielded a significantly elevated gradient of 1822 mm Hg. |
Fig. 1. Balloon occlusion hepatic venogram. Pressure measurements were taken in several positions within the hepatic venous system, including right and left hepatic veins and peripheral and central positions.
Fig. 2. Digital venogram of the pelvis shows the course of the inferior epigastric vein collateral (arrows). Fig. 3. Spot film of the abdomen shows the upper abdominal course of the transfemoral catheter (arrows) once the portal vein was catheterized.
Fig. 4. Transfemoral portogram shows large gastrohepatic ligament varices (arrows). | |
Questions 1) Presumably, this is a case of intrahepatic presinusoidal portal hypertension. It is our understanding that this is typically an idiopathic condition. Does anyone have any experience with this condition, either in the diagnostic or the therapeutic setting? 2) Have other participants had experience in catheterizing the portal vein via percutaneously accessible collaterals? If so, in what setting? Is anyone else looking for such collaterals as a means of localizing the portal vein for TIPS? 3) Why were the second set of wedged hepatic pressure measurements variable enough to yield a corrected sinusoidal pressure of 3 to 10 mm Hg? Does this represent regional variation in whatever pathologic process is involving the liver? Have other participants noted such variability, or is anyone aware of whether this has been reported? Related cases: | ||
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