Clinical picture and treatment of von Hippel-Lindau disease (cerebroretinal angiomatosis)

Purpose. To analyze the course and treatment of Hippel-Lindau angiomatosis on clinical cases. Material and Methods. We analyzed retrospectively 9 case histories (11 eyes) of patients with von Hippel-Lindau angiomatosis. All patients underwent the routine ophthalmic examination. J. Schlingemann’s classification was used to determine the stage of the disease. Results. The rare von Hippel-Lindau disease was described. Laser or surgical treatment was performed depending on the stage of the disease. Conclusions. Timely treatment of Hippel-Lindau angiomatosis in patients on early stages allow to maintain high visual functions and prevent the transition of the disease to a far-gone stage with the development of severe complications (exudative retinal detachment, neovascular glaucoma, hemophthalmos, uveitis). In the advanced stages of the disease without timely treatment it is possible to preserve visual functions and stabilize the pathological process only by complex surgical treatment.