[Retinal angiomatosis- an ophthalmological challenge]

In this report we provide a description of new findings in retinal angiomatosis (RA) and, in particular, of treatment procedures.

A review of relevant publications in the literature has been carried out and remarks on differential diagnosis are provided.

Haemangioblastomas of the retina and the central nervous system are the dominant manifestations in von Hippel-Lindau syndrome (VHL). As in patients with VHL syndrome the danger of new tumours is great, lifelong follow-up examinations are necessary. A genetic counselling with a DNA-based test of index patients and first degree relatives is recommended. The most frequently occurring retinal peripheral small tumours should be treated with the laser, large tumours, however, with kryo. or brachytherapy. Photodynamic therapy was successfully carried out in some patients as described in the literature. Treatment of tumours in the retinal middle periphery may result in central exudates with visual deterioration. Up to now, no sufficient experience exists concerning treatment with VEGF inhibitors, proton therapy, or transpupillary thermotherapy. Large tumours with retinal complications such as retinal detachment should be treated with combined procedures, mainly with vitrectomy.

An early diagnosis and treatment of retinal haemangioblastomas including examination of first-degree family members with a DNA-based test are necessary.