Document Detail


Splenic vascular malformations and portal hypertension in hereditary hemorrhagic telangiectasia: sonographic findings.
MedLine Citation:
PMID:  11180187     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
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:
M Seçil; A Göktay; O Dicle; T Pirnar
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Publication Detail:
Type:  Case Reports; Journal Article    
Journal Detail:
Title:  Journal of clinical ultrasound : JCU     Volume:  29     ISSN:  0091-2751     ISO Abbreviation:  J Clin Ultrasound     Publication Date:  2001 Jan 
Date Detail:
Created Date:  2001-02-22     Completed Date:  2001-04-12     Revised Date:  2004-11-17    
Medline Journal Info:
Nlm Unique ID:  0401663     Medline TA:  J Clin Ultrasound     Country:  United States    
Other Details:
Languages:  eng     Pagination:  56-9     Citation Subset:  IM    
Copyright Information:
Copyright 2000 John Wiley & Sons, Inc.
Affiliation:
Department of Radiology, Dokuz Eylül University Hospital, Inciralti, 35340 Izmir, Turkey.
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MeSH Terms
Descriptor/Qualifier:
Adult
Aneurysm / etiology,  pathology,  ultrasonography*
Humans
Hypertension, Portal / etiology*
Male
Regional Blood Flow
Splenic Artery / pathology,  ultrasonography
Splenic Vein / pathology*,  ultrasonography
Splenomegaly / ultrasonography
Telangiectasia, Hereditary Hemorrhagic / complications*,  ultrasonography*
Ultrasonography, Doppler, Color

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine


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