Pattern retinal dystrophy in a case of myotonic dystrophy

Abstract

Myotonic dystrophy is an autosomal dominant disease which can present with various ocular manifestations. A case of myotonic dystrophy presented with limited visual recovery postcataract surgery. Multimodal imaging analysis, including fundus photography, spectral domain optical coherence tomography (SD-OCT), and fundus fluorescein angiography, were performed showing pattern dystrophy of the pigment epithelium. Fundus photography revealed mild pigmentary alteration over the macular area, whereas fluorescein angiography showed hyperfluorescent staining from the disc to macular area with intervening hypofluorescent linear streaks. On SD-OCT, an epiretinal membrane was seen over the foveal area with mild puckering of inner retinal layers. Furthermore, intraretinal hyperreflective foci were seen along with intermittently disrupted photoreceptor layer. Annual follow-up with fundus photography, SD-OCT, and fluorescein angiography is required to avoid associated complications of scarring and choroidal neovascularization. High suspicion of retinal involvement is warranted in a case of myotonic dystrophy with limited visual recovery postcataract surgery.