Abstract

Percutaneous trans-splenic access was used to complete a transjugular intrahepatic portosystemic shunt (TIPS) was used successfully in a nine year old female status post orthotopic liver transplant (OLT) with main portal vein occlusion at the apparent anastomatic site. This patient with biliary atresia, OLT, and subsequent EB virus induced lymphoma developed severe portal hypertension with progressive splenomegaly, diffuse varices and thrombocytopenia, requiring regular blood product administration. Central venous catheter infection unresponsive to antibiotics alone required central line replacement with endotracheal intubation. Numerous attempts to extubate were thrwarted by valsalva induced variceal hemorrhage and hypopnea. Laboratory values at the time included ALT of 422; alkaline phosphatase, 269; BR, 5.3; albumin, 2.9; platelets, 8000; PT 14.7; PTT, 56; with cyclosporin trough levels in normal range. Doppler ultrasound demonstrated hepatopedal flow within a small, patent main protal vein. A calcific ring in the porta hepatis was present on CT scan. Because surgical portosystemic shunt was considered prohibitive, TIPS was attempted with parent's consent. However, transjugular cannulation of the main portal vein failed. Using the techniques of diagnostic splenoportography with a 3F puncture site, a shunt into a collateral of the portal vein was acheived, decompressing the portal system. Venography documented hepatopedal portal flow and disappearance of gastroesophageal varices. Post-procedure doppler demonstrated hepatopedal flow in the shunt at 300-400 cm/sec. Liver function studies improved within days, though platelet counts have rarely risen above twenty thousand. Three months post procedure, ALT is 85; alkaline phosphatase, 298; bilirubin, 1.7; albumin, 3.5. Spleen size and blood product administration remain substantially decreased.

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