title: 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 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.
authors.affiliation: Department of Gastroenterohepatology, Istanbul Medical Faculty, Istanbul University, Millet Cad. 34390 Capa, Istanbul, Turkey. munganz@gmail.com
authors.forename: Zeynel
authors.initials: Z
authors.lastname: Mungan
authors.forename: Binnur
authors.initials: B
authors.lastname: Pinarbasi
authors.forename: Baris
authors.initials: B
authors.lastname: Bakir
authors.forename: Mine
authors.initials: M
authors.lastname: Gulluoglu
authors.forename: Bulent
authors.initials: B
authors.lastname: Baran
authors.forename: Filiz
authors.initials: F
authors.lastname: Akyuz
authors.forename: Kadir
authors.initials: K
authors.lastname: Demir
authors.forename: Sabahattin
authors.initials: S
authors.lastname: Kaymakoglu