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

1992-12-01 / 4. szám

Szemészet, 129 (1992) 107

Discussion

Our report shows that angiogliomatosis retinocerebellaris still is a infrequent disease which, however, when not treated makes the eye concerned completely blind.

If angiomas are smaller than two papillas diameter, then as a rule we apply photocoagulation as a therapy. Light coagulation seems to be as equivalent for the treatment as laser coagulation. However, today we mostly prefer laser, because one can better dose its energy.

We do not see any advantage in photocoagulating the surrounding retina (MEYER-SCHWICKERATH, 1956; PÖTZSCH, 1983; SCHÜTTE et ah, 1983). Only the direct treatment of the vessel tumor (WESSING, 1967) can be successful.

Cryotherapy may be in place especially then, when angiomas do not become smaller by photocoagulation because of fibrous changes.

Diathermic coagulation was not applied in our clinic during the recent years because of the danger of bleeding caused by it, and because you are allowed to apply it only once (GERKE et al., 1989).

Secondary retinal changes, which according to LOHSE and FUHRMEISTER (1972) are due to disturbed permeability as shown by them in fluorescent angiography, are considered to have a bad prognosis (MEYER-SCHWICKERATH, 1960 and WESSING, 1967). In our experience this is not true in every case. Successful coagulation cures permeability disturbances. Secondary maculopathies often get better and visual acuity improves.

Radioactive isotopes cause cicatrization of the retina. Therefore, in our opinion this therapy should be used only then, when there is no possibility to apply photocoagulation or cryotherapy. In cases which up to now seemed to be incurable, the treatment with radioactive isotopes, however, was quite successful. The same is valid for vitrectomy, too.

Considering the protracted treatment and the great extent of controlling investigations, it is of special importance to inform the patients, so that they do not interrupt therapy or disregard specialist’s control.

This also applies to family planning. If possible, one should meet the demand of REICH and HOLLWICH (1984) and make an ophthalmological diagnosis with close relatives of the patient, too. In every case the specialist should give humangenetic advice.

Conclusions

1. If not treated, angiogliomatosis retinocerebellaris makes eyes blind.

2. In differential diagnosis, von HIPPEL-LIND AU disease is to be distinguished from morbus COATS, LEBER’s retinitis of miliary aneurysms, morbus EALES, and racemose aneurysms of the retina.

3. The therapy of choice is still photocoagulation. We prefer treating angiomas only directly.

4. Heavy complications can mostly be avoided, when photocoagulation is applied carefully.

5. Further possible therapeutical measures are cryocoagulation, treatment with radioactive isotopes and-if necessary-vitrectomy.

6. Secondary maculopathies can often reduce after sufficient photocoagulation. At the same time visual acuity can improve.

7. It is of great importance to inform the patient about the protracted treatment, extensive controls and family planning.

Literature

1. Apple D J, GOH Naumann: Spezielle Pathologie der Retina in Naumann GOH: Pathologie des Auges Springer: Berlin, Heidelberg, New York, 1980. 2. Colins: zit. bei PÜLHORN und FAUTH. 3. Fuchs: zit. bei PÜLHORN und FAUTH. 4. Gerke E, A Wessing, G Meyer- Schwickerath: Behandlung der retinalen Gefäßerkrankungen in Mackensen G, H Naumann: Augenärztliche Operationen, Bd. 2, Springer: Berlin, Heidelberg, New York, London, Tokyo, 1989. 5. Henkes H E: Erkrankungen der Netzhaut in Francois J, F Hollwich: Augenheilkunde in Klinik und Praxisaid. 3, Teil 1, Thieme: Stuttgart, New York, 1983. 6. Hippel E von: Über eine sehr seltene Erkrankung der Netzhaut. A v. Graefes Arch. klin. exp. Ophthal., 59, 83-106,1904. 7. Hippel E von: Die anatomische Grundlage der von mir beschriebenen, sehr seltenen Erkrankung der Netzhaut. Arch. Ophthal. 79, 350-377,1911.

8. Hoeve J van der: Augengeschwülste bei der tuberösen Hirnsklerose (BOURNEVILLE). A. v. Graefes Arch. klin. exp. Ophthal. 105, 880-898,1921. 9. Hoeve J van der: Augengeschwülste beider tuberösen Hirnsklerose (BOURNEVILLE) und verwandten Krankheiten. A v. Graefes Arch. klin. exp. Ophthal. 111, 1-16, 1923. 10. Huber A, H Wildberger: Erkrankungen der Sehnerven in Francois J, F Hollwich: Augenheilkunde in Klinik und Praxis, Bd. 4, Thieme, Stuttgart, New York, 1991. 11. Lindau A: Zur Frage der Angiomatosis retinae und ihrer Hirnkomplikationen. Acta ophthal. (Kbh.) 4, 193-226, 1927. 12. Lohse K, H Fuhrmeister: Angiomatosis retinae (v. Hippel-Lindau) in Krüger К E, M Tost: Augenheilkunde in Forschung und Praxis Wiss. Beiträge der Martin Luther Universität Halle-Wittenberg, 1972/2 (R 15), Halle/Saale, 1972. 13. Meyer- Schwickerath G: Erfahrungen mit der Lichtkoagulation der Netzhaut und Iris. Doc. opht., Bd. 10, 91-118,1956. 14. Meyer-Schwickerath G: Neue Indikationen der Lichtkoagulation. Ber. Dtsch. Ophth. Ges. 60, 197-200, 1957. 15. Meyer- Schwickerath G: Lichtkoagulation. 33. Beitr. Klin. Mbl. Augenheilk. Enke: Stuttgart 1959. 16. Meyer-Schwickerath G: Die Möglichkeiten zur Behandlung i. o. Tumoren unter Erhaltung des Sehvermögens. Ber. Dtsch. Ophth. Ges. 63, 178-189, 1960. 17. Раи H: Angiomatosis retinae (et papillae). A. v. Graefes Arch. Ophthal. 210, 229-234,1979. 18. Pötzsch D: Ein Fall von Angiomatosis retinae. Klin. Mbl. Augenheilk. 182,603,1983. 19. Pülhorn G, E Fauth: Klinik und Histopathologie der v. Hippel-Lindau’schen Erkrankung. Klin. Mbl. Augenheilk, 167, 884-891, 1975. 20. Reich H, F Hollwich: Zum v. Hippel-Lindau- Syndrom. Klin. Mbl. Augenheilk. 184, 513-519,1984. 21. Schütte E, D Pötzsch, H D Jakumeit: Reversible Sternfigur der Makula bei Angiomatosis retinae. Klin. Mbl. Augenheilk. 183, 205-207, 1983. 22. Spalten D J, R A Hitchings P A Hunter: Atlas der Augenkrankheiten. Thieme: Stuttgart, New York, 1987. 23. Thiel R, F Hollwich: Therapie der Augenkrankheiten. Thieme, Stuttgart, 1970. 24. Vail D: Angiomatosis retinae, eleven years after diathermy coagulation. Transact. Am. Ophth. Soc. 55, 217-238, 1958. 25. Wessing A: 10 Jahre Lichtkoagulation bei Angiomatosis retinae. Klin. Mbl. Augenheilk. 150,57-71,1967.

Address of the authors: Doz. Dr. sc. med. Albrecht Krause Prof. Dr. sc. med. Horst Fuhrmeister

Klinik und Poliklinik für Augenkrankheiten der Martin Luther Universität Leninallee 8.

Halle/Saale D-0-4020