Liver Images (continued) __________________________________________________________ Budd-Chiari syndrome secondary to cancer, note clot in the inferior vena cava and

the metastasis in the liver

________________________________________________________________________ _________________________________________________________ Case ReferenceNo. CC-0200-08 A 60-year-old gentleman, mildly symptomatic with epigastric discomfort was referred for abdominal ultrasound. Ultrasound showed large tortuous vessels in the porta-hepatis and in the liver parenchyma (Fig. 1) No focal parenchymal liver mass was seen. CT of the abdomen demonstrated dilated tortuous vessels (Fig. 2) Selective angiography of celiac artery and superior mesenteric artery demonstrated grossly dilated hepatic artery. A large tangle of abnormal vessels replaced the entire liver parenchyma causing arteriovenous shunting. No abnormal parenchymal staining of the liver was present (Figs. 3,4)Pertinent clinical history was negative for any known malignancy, weight loss or steroid use. On examination, the patient had a prominent abdominal bruit. No visceromegaly was present. There was no clinical evidence of cardiac failure or portal hypertension. Patient was mildly hypertensive and had normal serum creatinine. LFTs were performed and were

normal. There were no signs of cutaneous or visceral vascular malformations other than in the liver.

Fig. 3.Celiac artery injection shows multiple dilated tortuous vessels with severe arteriovenous shunting and filling of hepatic veins in early to mid arterial phase, note the absence of parenchymal staining.

Fig. 4. Superior mesenteric arterial injection shows shunting of blood to the hepatic artery though pancreato-duodenal arcade. Injection of 45 cc of contrast into SMA failed to opacify portal vein because of shunting of blood to hepatic artery. Portal vein was however patent on ultrasound. Questions: 1) Does this condition represent a large isolated AVM of the liver (Not part of Hereditary Hemorrhagic Telangiectasia) or is it Peliosis hepatis? Has any one seen the entire liver replaced by an AVM? 2) What are other differential possibilities? 3) How can a definitive diagnosis be reached short of biopsy? 4) Should this patient be treated? Although this he is not in cardiac failure, if his heart does decompensate, should he be treated at that time? ________________________________________________________________________ _________________________________________________________

 CC0200-08 Fig.1Sagittal scan though the liver shows multiple tortuous vessels in the liver parenchyma

CC0200-08 Fig.2 Multiple dilated hepatic arterial and venous channels are seen the axial image through the level of celiac artery which is also dilated. ________________________________________________________________________ _________________________________________________________

diagnosis Classic Pseudomyxoma Peritonei ________________________________________________________________________ _________________________________________________________