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Liver Images (continued)

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Budd-Chiari syndrome secondary to cancer, note clot in the inferior vena cava and the

metastasis in the liver

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Case ReferenceNo. CC-0200-08

A 60-year-old gentleman, mildly symptomatic with epigastric discomfort was referred forabdominal 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 theabdomen 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 arteriovenousshunting. 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 wasmildly hypertensive and had normal serum creatinine. LFTs were performed and were

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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 severearteriovenous 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 toopacify 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 liverreplaced 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 heartdoes decompensate, should he be treated at that time?

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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.

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diagnosis

Classic Pseudomyxoma Peritonei

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