title: Splenic vascular malformations and portal hypertension in hereditary hemorrhagic telangiectasia: sonographic findings.
abstract: 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 sonographic findings in a case of hereditary hemorrhagic telangiectasia. Gray-scale sonographic examination revealed massive splenomegaly, multiple dilated intrasplenic vascular structures (some with adjacent punctate calcifications), an aneurysmal dilatation of the splenic vein, dilated intrahepatic portal branches, and marked atrophy of the right hepatic lobe. Color Doppler 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 splenic vein. The splenic vein was massively enlarged with increased flow velocity and contained an isolated aneurysmal dilatation in the hilum. There were multiple serpiginous retroperitoneal collateral vessels, and the left gastric vein was dilated with hepatofugal flow. The patient had portal hypertension that developed secondary to the increased portal flow.
authors.affiliation: Department of Radiology, Dokuz Eylül University Hospital, Inciralti, 35340 Izmir, Turkey.
authors.forename: M
authors.initials: M
authors.lastname: Seçil
authors.forename: A
authors.initials: A
authors.lastname: Göktay
authors.forename: O
authors.initials: O
authors.lastname: Dicle
authors.forename: T
authors.initials: T
authors.lastname: Pirnar
mesh_terms: D000328:Adult; D000783:Aneurysm / Q000000981:diagnostic imaging* / Q000209:etiology / Q000473:pathology; D006801:Humans; D006975:Hypertension, Portal / Q000209:etiology*; D008297:Male; D012039:Regional Blood Flow; D013157:Splenic Artery / Q000000981:diagnostic imaging / Q000473:pathology; D013162:Splenic Vein / Q000000981:diagnostic imaging / Q000473:pathology*; D013163:Splenomegaly / Q000000981:diagnostic imaging; D013683:Telangiectasia, Hereditary Hemorrhagic / Q000150:complications* / Q000000981:diagnostic imaging*; D018615:Ultrasonography, Doppler, Color