Szemészet, 1992 (129. évfolyam, 1-4. szám)

1992-12-01 / 4. szám

106 Szemészet, 129 (1992)

In one case, a young patient, the cerebellum was involved in the disease, too. Two persons have had several relatives in their family suffering from angiogliomatosis retinae as well. When the diagnosis was made, we found angiomas having most an areal of 1/2 up to more than 2 papillas diameter and capillary hemangiomas in statu nascendi in two cases being the result of arteriovenous shortcircuits.

Formerly, we applied diathermic coagulation to treat angiomas. Today we prefer using photocoagulation (light coagulator 5,000, Zeiss Jena, and argon laser coagulator, Lasertek Helsinki). Photocoagulation was carried out twice up to seven times in intervals from one week to two months always treating one eye only. If there already was an ablatio retinae or if the angiomas were larger than two papillas diameter, then we applied cryotherapy or referred the patient to an ophthalmological tumour centre for radiotherapy. Once, a patient came to our clinic only when she was blind in one eye. Table 1 gives a short survey of all the therapeutical measures taken by us.

Table 1.

therapy of the angiogliomatosis retinocerebellaris:

lightcoagulation

1 eye

lasercoagulation

3 eyes

light- and lasercoagulation

4 eyes

diathermy and lightcoagulation

1 eye

diathermy and ablatio operation

1 eye

radioactive isotopes

2 eyes

without therapy (amaurosis)

1 eye

In half a year up to 16 years (on an average 6 years) of observation we registered the following results of the treatment:

Six eyes are cured. One eye became blind inspite of the therapy. One female patient is still being treated with radioactive isotopes. In one case any therapy was impossible, because the eye was already blind.

At the beginning of the therapy, eight eyes have had maculopathy, which however improved after the treatment. Visual acuity has developed in a similar way: improvement 3x

deterioration 3x

without changes 5x

During ophthalmological controls local recurrence and new angiomas have been found in two eyes each. One of the patients who suffers from angiomatosis retinocerebellaris has already to undergo the third neurosurgical treatment.

Five times, we found a little bleeding on the angioma after having applied photocoagulation. Three times, there was a temporary exudative ablatio. During an external treatment, a large bulb bleeding occurred in one patient (see Table 2).

Six patients used the possibilities of ophthalmological, neurological and internal control. Two patients came unregularly, and the last two patients did not come to any control at all.

Table 2.

complications after photocoagulation: small bleedings on the angioma ablatio fugax

large bleeding in the vitreous body

5 eyes 3 eyes 1 eye

Results

During the recent 25 years (from 1966-1991) 10 patients (two women, eight men) suffering from angiogliomatosis retinae were treated in the Department of Ophthalmology at Martin Luther University in Halle-Wittenberg. The total number of consultations yearly was about 20 thousand.

Those ten patients mentioned above were on an average 23 years old (between 13 and 37) when the diagnosis was made. In four cases the disease cought their right eyes and in three cases the left ones. Three persons were suffering from a HIPPELLINDAU disease in both eyes.

Fig. 1 : Angiomatosis retinae: 28 years old man. right eye. paramacular.

Fig. 2: The same eve. fluorescence angiography.

Fig. 3: The same eye, after lasercoagulation of the angioma