Splenic vascular malformations and portal hypertension in hereditary hemorrhagic telangiectasia: Sonographic findings

Hereditary hemorrhagic telangiectasia, or Osler-Rendu-Weber disease, is an autosomal dominant disorder in which a variety of vascular dysplasias occur throughout the organ systems. We report the gray-scale and color Doppler s onographic findings in a case of hereditary hemorrhagic telangiectasia. Gra y scale sonographic examination revealed massive splenomegaly, multiple dil ated intrasplenic vascular structures (some with adjacent punctate calcific ations), an aneurysmal dilatation of the splenic vein, dilated intrahepatic portal branches, and marked atrophy of the right hepatic lobe. Color Doppl er sonography showed dilatation of the truncus coeliacus and high-velocity flow in the splenic artery. There were significant aliasing in the splenic hilum and an abnormal, arterialized flow in intrasplenic branches of the sp lenic vein. The splenic vein was massively enlarged with increased flow vel ocity and contained an isolated aneurysmal dilatation in the hilum. There w ere multiple serpiginous retroperitoneal collateral vessels, and the left g astric vein was dilated with hepatofugal flow. The patient had portal hyper tension that developed secondary to the increased portal flow. (C) 2000 Joh n Wiley & Sons, Inc.