Incipient angiomas of the retina and central nervous system in von Hippel-Lindau disease have been detected by clinical and radiologic examination. Progression of identified lesions and the appearance of new retinal lesions has been observed. Histologically, they are distinctly hemangiomatous tumors. Some of these lesions represent the smallest that have been reported to date. Genetic study of families and surgical therapy of affected individuals is recommended. Greater clinical awareness of the development and potential for progression of incipient lesions and of the genetic manifestations of this disease should reduce the incidence of blindness and the mortality.
Jesberg DO, Spencer WH, Hoyt WF. Incipient Lesions of von Hippel-Lindau Disease. Arch Ophthalmol. 1968;80(5):632-640. doi:10.1001/archopht.1968.00980050634010