2009
DOI: 10.1155/2009/479264
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Congenital Portal Vein Aneurysm Associated with Peliosis Hepatis and Intestinal Lymphangiectasia

Abstract: Portal vein aneurisym (PVA), peliosis hepatis (PH) and intestinal lymphangiectasia (IL) all are very uncommon entities. Herein, we presented a unique patient with these three rare entities who was admitted to our hospital because of portal hypertensive ascites rich in protein and lymphocyte. PVA was extrahepatic and associated with coronary vein aneurysm. Peliosis hepatis was of microscopic form. Lymphangiectasia was present in peritoneum and small intestine. Diagnoses of these rare entities were made by imagi… Show more

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Cited by 10 publications
(3 citation statements)
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“… 15 More uncommon associations include malignancy such as HCC, cystic fibrosis, celiac disease, intestinal telangiectasia, portal vein aneurysms, Hodgkin's lymphoma, X-linked myotubular myopathy, hereditary haemorrhagic telangiectasia, and idiopathic thrombocytopaenic purpura. 15 27 28 29 Despite the multiple reported associations, no reported association / causative factors have been found in up to 50% of cases.…”
Section: Discussionmentioning
confidence: 99%
“… 15 More uncommon associations include malignancy such as HCC, cystic fibrosis, celiac disease, intestinal telangiectasia, portal vein aneurysms, Hodgkin's lymphoma, X-linked myotubular myopathy, hereditary haemorrhagic telangiectasia, and idiopathic thrombocytopaenic purpura. 15 27 28 29 Despite the multiple reported associations, no reported association / causative factors have been found in up to 50% of cases.…”
Section: Discussionmentioning
confidence: 99%
“…Its pathogenesis is not completely clear, except for SOS-dependent peliosis, and regardless of the cause, sinusoidal damage has been generally proposed as being critical to outflow blockage and dilation of the sinusoids/central vein of the hepatic lobule[ 120 , 125 ]. Moreover, hepatocellular necrosis may represent another possible mechanism with the subsequent formation of blood-filled lacunae[ 126 , 127 ]. Two different histological forms of peliosis can be identified: the parenchymal type, usually associated with hemorrhagic parenchymal necrosis and characterized by a lack of endothelial lining within the blood-filled lacunae, and the phlebectatic type, with a dilated central vein, showing an endothelial lining within the cystic spaces[ 128 ].…”
Section: Blue Livermentioning
confidence: 99%
“…[5][6][7][9][10][11][12][13][14][15][16][17][18] Extrahepatic portal venous aneurysms have been described in the literature. [19][20][21][22][23] However, peripheral intrahepatic portosystemic venous shunts with aneurysms are extremely rare [1][2][3][4][5]13,24 and incidentally discovered. 2,13 In a study of nine cases involving intrahepatic portosystemic venous shunts, De Gaetano et al 4 found that 56% of the cases, without any clinical evidence of liver disease, were incidentally discovered.…”
Section: Discussionmentioning
confidence: 99%