Abstract

DO.08.07

Vitrectomy-outcome of capillary hemangiomas in von Hippel-Lindau disease

Mete Gök, Harun Akgül, Michael Freistühler, Eva Biewald, Markus Holdt, Norbert Bornfeld

Hinterer Augenabschnitt, Zentrum für Augenheilkunde, Universitätsklinikum Essen, Essen

Objective
Von Hippel-Lindau syndrome is a rare, autosomal dominant disorder predisposing to a series of benign as well as malignant tumors in various organ systems.
The hallmark tumour lesion in the eye is the retinal capillary angioma which is capable of causing significant visual morbidity through either exsudative or tractional effects on the surrounding retina.
Aim of this study is to describe the indications for surgery, functional outcome and complications of von Hippel-Lindau patients with capillary angiomas.
Methods
A total of 15 patients, 11 female, 4 male, were included in this retrospective case study. Mean age at first surgery was 27,2, years. All patients underwent pars plana vitrectomy due to major complications from their retinal angioma from 1991 to 2008. According to the severity of the disease the vitreous tamponade used was silicone oil, air or gas.
Results
Indication for vitreoretinal surgery was a retinal detachment in six patients of whom four had a complete retinal detachment with PVR. There was a severe macular pucker in two patients, another two had a dense vitreous bleeding, and one was afflicted with a thick preretinal membrane. There was a recurring retinal detachment under silicone oil in two patients after 4,5 years and 1 months respectively leading to further major vitreoretinal surgery. All in all 5 patients were enucleated due to serious complications.
Conclusions
Capillary retinal angioma can represent a sight threatening disease depending on the severity of the retinal involvement. The vitrectomy can be used as a therapeutical option for preserving the eye. However, in our study a globe saving treatment could not be achieved in approximately 33% of the cases. With the development of new treatment options, e.g. anti-VEGF, the prognosis of the course of the disease might be improved in future.

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