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Gastroenterology Research and Practice
Volume 2009, Article ID 479264, 5 pages
Case Report

Congenital Portal Vein Aneurysm Associated with Peliosis Hepatis and Intestinal Lymphangiectasia

1Department of Gastroenterohepatology, Istanbul Medical Faculty, Istanbul University, Millet Cad. 34390 Capa, Istanbul, Turkey
2Department of Radiology, Istanbul Medical Faculty, Istanbul University, Istanbul, Turkey
3Department of Pathology, Istanbul Medical Faculty, Istanbul University, Istanbul, Turkey

Received 14 August 2009; Revised 3 December 2009; Accepted 23 December 2009

Academic Editor: Juan G. Abraldes

Copyright © 2009 Zeynel Mungan et al. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.


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 imaging techniques and histopathological findings. Patient also had hydronephrosis caused by ureteropelvic junction narrowing. Best of our knowledge, there is no such a case reported previously with the association of PVA, PH and IL. Therefore, we propose PVAPHIL syndrome to define this novel association.