Department of Gastroenterohepatology, Istanbul Medical Faculty, Istanbul University, Millet Cad. 34390 Capa, Istanbul, Turkey.
Gastroenterol Res Pract. 2009;2009:479264. doi: 10.1155/2009/479264. Epub 2010 Mar 30.
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.
门静脉动脉瘤（PVA）、肝血窦扩张（PH）和肠淋巴管扩张（IL）均为非常罕见的病变。本文报道了 1 例罕见的 PVA、PH 和 IL 三联征患者，因富含蛋白和淋巴细胞的门脉高压性腹水就诊于我院。PVA 为肝外性，伴冠状静脉动脉瘤。PH 为显微镜下表现。淋巴管扩张见于腹膜和小肠。通过影像学和组织病理学检查明确了这些罕见病变的诊断。患者还存在肾盂积水，由输尿管肾盂连接部狭窄引起。据我们所知，目前尚未有 PVA、PH 和 IL 三联征的相关报道。因此，我们提出 PVAPHIL 综合征来定义这种新的关联。
