J Korean Ophthalmol Soc.  1978 Mar;19(1):113-116.

von Hippel-Lindau Disease

• Shin H
• Sohn JO
• Shin NY

Abstract

Although this vascular anomaly, angiomatosis retinae, was initially observed over 85 years ago, and although excellent clinical description of the lesion exist in the literature, treatments for the angiomatosis retinae is difficult and unless the condition is in its early stages, impossible but available symptomatic therapy. Two cases of the angiomatosis retinae, the first, 11 year old girl, von Hippel disease, early stage of vascular dilatation and angiomatous formation, and the second, 15 year old boy, von Hippel-Lindau disease, the final stage of glaucoma and destruction of the eye, are reported. Intravenous fluorescein angiography has been demonstrated, the retinal vascular malformations in von Hippel-Lindau disease are distributed focally and not diffusely, The retinal veins showing greater changes than the arteries are disclosed. This paper presents the fluorscein angiographic findings of von Hippel-Lindau disease and discusses the treatments and the pathologic findings of angiomatosis retinae with literatures related to von Hippel-Lindau disease.

MeSH Terms

Adolescent
Arteries
Child
Dilatation
Female
Fluorescein Angiography
Glaucoma
Humans
Male
Retinal Vein
Retinaldehyde
Vascular Malformations
von Hippel-Lindau Disease*
Retinaldehyde