97th DOG Annual Meeting 1999



K. Pecold1, A. Krause2, J. Bernardczyk-Meller1, B. Hannemann2, A. Gotz-Wieckowska1, A. Goldhahn2

Introduction: Angiomatosis retinae (von Hippel) is a very rare autosomal dominant transmitted disease of the retina. For the patients affected by it, it is however visus threatening, and as a systemic disease also life threatening (von Hippel-Lindau syndrome). For a long-term observation and review of therapy in a geographical restricted, but sufficient large group of population the results were analysed in the Eye Hospitals of the Karol-Marcinkowski-University Poznan (Poland) and the Martin-Luther-University Halle-Wittenberg.

Patients and Methods: From 1966 to 1997 angiomatosis retinae was diagnosed in 28 patients (40 eyes) and confirmed by angiography (female 13, male 15; age 11-70 years, average: 24,5 years). Follow-up-period: 1 - 18 years and a half, average 8 years and three quarters.The therapy (in 35 eyes still possible) was performed according to the stage of the disease and the location of the angiomata (photocoagulation: 18 eyes, cryocoagulation: 9, Beta-ray-treatment: 1, combinations of the above mentioned operations, including ablatio-operation: 7 eyes).

Results: In 12 patients there existed a positive family-anamnesis (angiomata of the retina and the cerebellum). 5 patients died from angiomata of the cerebellum. In 5 eyes no therapy took place (primary amaurosis, resp. move of the patient). By the above mentioned therapy the visual acuity was unchanged in 17 eyes, in 8 eyes visus improvement, in 5 eyes visus reduction, 5 eyes got blind though therapy took place (mainly by primary visual acuity of 1/50 resp. stage III and IV). After exact lasercoagulation severe complications are rare, but smaller reversible bleedings after photocoagulation are frequent (12 eyes).

Conclusions: In case it is possible, also nowadays the best therapy of angiomas is by photocoagulation. It is possible to improve the results still more by vitrectomy (observation time!). Ophthalmological controls every half year, systemical controls every year. Family diagnostic is made much easier by genetic examination.

Univ.-Augenklinik, ul. Dluga ½ , Pl-61-848 Poznan (Polen)1
Univ.-Augenklinik, Magdeburger Str. 8, 06097 Halle/Saale2