Ciliary and retinal changes in myotonic dystrophy.

We examined 15 patients with myotonic dystrophy by cycloscopy, ophthalmoscopy, and fluorescein angiography. In six cases, we found depigmentation in the relatively short ciliary processes, which may account for ocular hypotony in this disorder. Eight of the patients had retinal lesions, three with butterfly-shaped dystrophy, two with reticular dystrophy, and three with peripheral yellow flecks. These lesions were biomicroscopically at the level of the retinal pigment epithelium or in the deep retinal layer and did not substantially alter visual functions. Psychophysical and electrophysiologic test results were normal or only slightly affected. The butterfly-shaped and reticular dystrophies in myotonic dystrophy may be variant forms of patterned dystrophy of the retinal pigment epithelium. The peripheral yellow flecks in a stone-wall configuration noted in this disorder are particularly unique and, to our knowledge, have not been documented previously.