Schmetterlingsförmige Dystrophie der Makula

Summary

Background The butterfly-shaped dystrophy of the macula is an extremely rare autosomal dominant retinal disorder first described by Deutman in 1970. Two cases are presented.

Case reports The first patient presented to the clinic with slight deterioration of vision OD. Corrected visual acuity was OD 0.63 and OS 0.8. Examination of anterior segments was normal bilaterally. Funduscopically there was a mild disturbance of the foveolar retinal pigment epithelium. Fluorescein angiography, however, disclosed a large patternlike hypofluorescence with hyperfluorescent areas in the macular region. Visual fields and electroretinography were normal. The second patient complained of decreased visual acuity OU. Funduscopic and angiographic findings were similiar to the first patient.

Conclusion Different forms of pattern dystrophies of the macula have been described in the literature with moderate visual impairment. Differentiation is sometimes difficult as some forms have been reported to occur in one family. They may represent different clinical entities of the same disease. Diagnosis is usually made by fluorescein angiography.

Key words

Pattern dystrophy - macular dystorpy - fluorscein angiography

Schlüsselwörter

Musterdystrophie - Makuladystrophie - Fluoreszenzangiographie