Spontaneous regression of retinal angiomatous lesions in v. Hippel-Lindau disease (VHL).

BACKGROUND: Very little documentation of spontaneous regression of an angiomatous retinal lesion in v. Hippel-Lindau disease (VHL) exists. It is commonly believed that a spontaneous change of hemangiomas into fibrotic lesions occurred. PATIENTS/METHODS: Follow-up examinations of four patients with VHL in the Freiburg VHL study were carried out. RESULTS: A 16-year-old girl revealed a vascular lesion at the border of the optic disc. Control examination nine years later revealed complete spontaneous regression of the retinal vascular changes. A slight retinal vascular change at the superior border in her right eye was found in a 36-year-old woman. A control examination 20 years later revealed regression of the lesion. A 41-year-old woman showed in the retinal periphery a small fibrotic white hemangioma with a pigmented feeder vessel as sign of spontaneous tumor regression. A 12-year-old boy had a retinal microaneurysm inferior to the optic disc that disappeared several years later. CONCLUSION: Documentations of spontaneous regression of minor angiomatous retinal lesions in VHL exist. Such vascular changes are rare. Every retinal lesion should be controlled by follow-up examination and documentation. In case of retinal lesion growth, treatment is necessary.