Biomaterial Database

BILATERAL CHOROIDAL VASCULAR MALFORMATIONS IN HEREDITARY HEMORRHAGIC TELANGIECTASIA. 2023

Kenneth J Taubenslag, and Sara Al Hussein Al Awamlh, and Paul Sternberg

Department of Ophthalmology, Vanderbilt University Medical Center, Nashville, Tennessee.

OBJECTIVE To describe a case of bilateral choroidal vascular malformations in a patient with hereditary hemorrhagic telangiectasia. METHODS Case report. RESULTS A 78-year-old man with hereditary hemorrhagic telangiectasia was incidentally noted to have focal, large deep choroidal vessels on optical coherence tomography with corresponding elevation of the overlying retina. Indocyanine green angiography revealed dilated, intensely fluorescent vessels in arterial phase emptying into massively dilated choroidal veins consistent with choroidal arteriovenous malformation. CONCLUSIONS This case presents multimodal imaging findings of choroidal arteriovenous malformations in hereditary hemorrhagic telangiectasia. Choroidal vascular malformations may represent an underrecognized clinical feature of this syndrome.

UI	MeSH Term	Description	Entries
D008297	Male		Males
D002829	Choroid	The thin, highly vascular membrane covering most of the posterior of the eye between the RETINA and SCLERA.	Choriocapillaris,Haller Layer,Haller's Layer,Sattler Layer,Sattler's Layer,Choroids
D004396	Coloring Agents	Chemicals and substances that impart color including soluble dyes and insoluble pigments. They are used in INKS; PAINTS; and as INDICATORS AND REAGENTS.	Coloring Agent,Dye,Dyes,Organic Pigment,Stain,Stains,Tissue Stain,Tissue Stains,Organic Pigments,Pigments, Inorganic,Agent, Coloring,Inorganic Pigments,Pigment, Organic,Pigments, Organic,Stain, Tissue,Stains, Tissue
D006801	Humans	Members of the species Homo sapiens.	Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D000368	Aged	A person 65 years of age or older. For a person older than 79 years, AGED, 80 AND OVER is available.	Elderly
D000792	Angiography	Radiography of blood vessels after injection of a contrast medium.	Arteriography,Angiogram,Angiograms,Angiographies,Arteriographies
D001165	Arteriovenous Malformations	Abnormal formation of blood vessels that shunt arterial blood directly into veins without passing through the CAPILLARIES. They usually are crooked, dilated, and with thick vessel walls. A common type is the congenital arteriovenous fistula. The lack of blood flow and oxygen in the capillaries can lead to tissue damage in the affected areas.	Arteriovenous Malformation,Malformation, Arteriovenous,Malformations, Arteriovenous
D013683	Telangiectasia, Hereditary Hemorrhagic	An autosomal dominant vascular anomaly characterized by telangiectases of the skin and mucous membranes and by recurrent gastrointestinal bleeding. This disorder is caused by mutations of a gene (on chromosome 9q3) which encodes endoglin, a membrane glycoprotein that binds TRANSFORMING GROWTH FACTOR BETA.	Osler-Rendu Disease,Rendu-Osler-Weber Disease,Weber-Osler Disease,Weber-Osler Syndrome,Hereditary Hemorrhagic Telangiectasia,Osler's Disease,Osler-Rendu-Weber Disease,Osler-Weber-Rendu Syndrome,Telangiectasia, Hereditary Hemorrhagic, Type 1,Telangiectasia, Hereditary Hemorrhagic, of Rendu, Osler, and Weber,Hemorrhagic Telangiectasia, Hereditary,Osler Disease,Osler Rendu Disease,Osler Rendu Weber Disease,Osler Weber Rendu Syndrome,Rendu Osler Weber Disease,Weber Osler Disease,Weber Osler Syndrome