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Abstract

P 057

Therapeutical course of retinal angioma in von Hippel Lindau disease

Claudia Zorn, Matthias M. C. Zorn, Mathias Maier, Chris P. Lohmann

Augenklinik, Klinikum rechts der Isar, Technische Universität München, München

Objective
The VHL disease belongs to the phacomatosis syndroms. Angioma are seen in the retina and cerebellum, other tumors may occur in the brain stem, kidney, adrenal gland and pancreas. Approaches to treat retinal angioma are laserkoagulation, kryokoagulation, radiotherapy, photodynamic therapy, off label use of anti-VEGF injcetion and vitreoretinal surgery.
Methods
A 27 year old woman suffering from VHL with associated carcinoma of the pancreas and kidney and a phechromocytoma was seen in our clinic in March 2007. A standardized examination including best corrected visual acuity (BCVA), slit.lamp examination, fluorescencangiography and OCT showed a bilateral BCVA of 20/20. The flourescencangiography showed bilateral mulitple angioma next to the vascular arches and in the periphery with associated leakage. Angioma with macula affecting leakage were treated with laserkoagulation using a fd:Nd YAG laser. In March 2008 the standardized examination showed a bilateral BCVA of 20/20. An additional lasertreatment was performed due to  persisting leakage of the angioma in both eyes and due to central retinal edema. In February 2009 no further lasertreatment was needed because the BCVA was unchanged and the angioma presented without any clinically significant leakage.
Results
Lasertreatment of retinal angioma with associated leakage and with affection of the macular region seemed to be a safe and effecitve procedure. Small angioma without associated leakage did not require any treatment. Follow up examinations are important to detect an increse in growth and leakage of the retinal angioma. Under these circumstances retreatment would be indicated.
Conclusions
In cases with ocular manifestation of a VHL disease a thorough work up of the angioma is important. According to localisation and tumor size lasertreatment may be a safe, reasonable and appropriate therapy.

 
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