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Case Reports
. 2003 Sep;18(3):191-5.
doi: 10.3904/kjim.2003.18.3.191.

Rapidly progressing Budd-Chiari syndrome complicated by hepatocellular carcinoma

Jeong Won Jang  1 , Seung Kew Yoon, Si Hyun Bae, Jong Young Choi, Kyu Won Chung, Hee Sik Sun
Affiliations
Case Reports

Rapidly progressing Budd-Chiari syndrome complicated by hepatocellular carcinoma

Jeong Won Jang et al. Korean J Intern Med. 2003 Sep.

Abstract

Budd-Chiari syndrome (BCS) is a disorder caused by occlusion of the hepatic vein or inferior vena cava. The clinical presentation include abdominal pain, hepatomegaly, ascites, leg edema, collateral venous dilatation of the body trunk, and portal hypertension. In addition, BCS can cause hepatocellular carcinoma (HCC) in some patients, although its pathogenesis is not yet completely understood. The average reported time lag from diagnosis of BCS to full-blown HCC ranges from several years to several decades. Hepatic carcinogenesis in patients with BCS perhaps reflects a prolonged and persistent liver injury in that it occurs in the primary inferior vena cava obstruction rather than the primary hepatic vein thrombosis. Among patients with BCS, membranous obstruction of the vena cava (MOVC) usually presents an insidious and chronic illness, whereas primary hepatic vein thrombosis presents an acute or subacute illness. We experienced a case of a patient with BCS, which progressed rapidly that HCC developed only nine months after the diagnosis of BCS. The factors causing this rapid progression are still unclear and remain to be investigated.

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Figures

Figure 1.
Figure 1.
(A) CT scan showing narrowed intrahepatic vena cava, hypertrophied caudate lobe, mild ascites, and moderate splenomegaly with focal splenic infarction. (B) MR imaging showing obliterated hepatic veins and diffusely narrowed intrahepatic vena cava.
Figure 2.
Figure 2.
Venogram of the liver showing broad stenosis of the intrahepatic vena cava (arrow) with collateral vessels (arrow head).
Figure 3.
Figure 3.
A and B. Histological findings of the liver showing zone 3 hemorrhage with fibrotic regenerative nodular formation (hematoxylin and eosin stain, ×100), and fibrotic expansion of portal area (Masson trichrome stain, ×100).
Figure 4.
Figure 4.
Liver dynamic CT scan showing multiple, small, and variably-sized nodules in both hepatic lobes. The nodules were enhanced in the early arterial phase (arrows; A), and washed out in the portal phase (arrows; B), suggesting hepatocellular carcinoma.
Figure 5.
Figure 5.
Selective angiography of the hepatic artery showing multiple, variably-sized, and hypervascular lesions in both hepatic lobes (arrows), indicating hepatocellular carcinoma.
See this image and copyright information in PMC

References

    1. Johnson PJ, Williams R. Cirrhosis and the aetiology of hepatocellular carcinoma. J Hepatol. 1987;4:140–147. - PubMed
    1. Schafer DF, Sorrell MF. Hepatocellular carcinoma. Lancet. 1999;353:1253–1257. - PubMed
    1. Okuda H, Yamagata H, Obata H, Iwata H, Sasaki R, Imai F, Okudaira M, Ohbu M, Okuda K. Epidemiological and clinical features of Budd-Chiari syndrome in Japan. J Hepatol. 1995;22:1–9. - PubMed
    1. Takayasu K, Muramatsu Y, Moriyama N, Wakao F, Makuuchi M, Takayama T, Kosuge T, Okazaki N, Yamada R. Radiological study of idiopathic Budd-Chiari syndrome complicated by hepatocellular carcinoma. A report of four cases. Am J Gastroenterol. 1994;89:249–253. - PubMed
    1. Mitchell MC, Boitnott JK, Kaufman S, Cameron JL, Maddrey WC. Budd-Chiari syndrome: etiology, diagnosis and management. Medicine (Baltimore) 1982;61:199–218. - PubMed

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