Programm                 "Degeneration und Regeneration– Grundlagen, Diagnostik und Therapie"

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The Marfan-Syndrom – A Retrospective Study

Holland D., Roider J.
Dept. of Ophthalmology, Unversity Clinical Centre Schleswig Holstein, Campus Kiel

Purpose: The Marfan Syndrom (MS) is a mesodermal dystrophy with dominant inheritance. It is combined with abnormal growth, arachnodactyly, thoracic deformation, heart failure and aortic aneurysm. The best known ophthalmological changes are myopia, lens luxation and shärophakia. We performed a study within a Marfan population in Germany for functional, refractiv and other pathological findings.
Method: We developed with the help of the german “Marfan-Hilfe e.V.” a questionnaire which was distributed to the members. 71 questionnaires could be evaluated.
Results: We saw a symmetrical sex distribution in our group. The average age was 35 (median 42). Confirmed was the high number of refractiv errors. Only one patient was emmetropic. 50 patients were myopic, 15 hyperopic and 61 patients had an astigmatism. Anisometropia was found in 13 patients. The average visual acuity was 0,7. Blindness was found in 7 eyes, light perception in 1 eye. Cause for blindness was in all cases a retinal detachment in aphakia. Retinal detachment occured in 11 patients. 38 patients showed lens luxation which was treated operatively in 16 cases. Due to cataract 6 patients were treated operatively. 15 patients were aphakic. An anterior chamber lens was implanted in 11 patients and a anterior chamber lens in one. Glaucoma had to be treated in 7 patients and strabological disorders were found in 10 patients.
Conclusions: Remarkable is with 1

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