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Von-Hippel-Lindau Syndrome: Case Report

Tanasescu I.,
Bayerische Julius-Maximilians-Universität Würzburg, Universitäts-Augenklinik (Würzburg)

Background: The Von-Hippel-Lindau (VHL) Syndrome is a hereditary disease, which is characterized by multiple tumors, i.e. retinal haemangiomas which occur in 50% of cases.
Case: A 14-year old boy presented in our department with reduced visual acuity of 0.1 in the left eye. Funduscopically, the left eye showed multiple exsudations extending from the temporal midperiphery to the macula, accompanied by extensive dilatated blood vessels and serous retinal detachment. Fluorescein angiography detected two capillary retinal haemangiomas with multiple satellites. Differential diagnosis included VHL-Syndrome and the cranial MRI was normal. Due to lacking compliance, the patient did not follow up for staging and therapy plan until 1.5 years later. At this point, the visual acuity was reduced to light perception. The intraocular pressure was 48 mmHg, the sonography showed a 5,2x5,7 mm vascularized tumor and complete retinal detachment. Due to the decompensation of secondary glaucoma cyclocryocoagulation was performed. After surgery the intraocular pressure was 30mmHg. The right eye was normal. Following staging exstirpation of two left-sided cerebellar haemangioblastomas was performed in the Dept. of Neuro-Surgery. In spinal MRI multiple haemangioblastomas were detected in the thoracic spine, which await exstirpation. Topical irradiation therapy with a ruthenium applicator has been scheduled to physically preserve the eye because of tumor exsudation. A genetic blood analysis of the family will follow. Meanwhile the mother was diagnosed with spinal haemangioblastomas.
Discussion: Regular examinations of VHL affected patients and carriers of the gene mutation improves long-term outcome by the possibility for early intervention, such as retinal argon laser treatment. Conclusion. Early-on genetic diagnostics of VHL affected families and stringent staging controls with short follow-up intervals may significantly improve prognosis.