Multifocal pattern dystrophy (MPD) simulating fundus flavimaculatus is characterized by yellowish flecks over posterior pole and extending beyond the arcades. With the progression of the disease, multiple chorioretinal atrophic patches develop over posterior pole.[1] Good vision is usually maintained till late-stage of the disease. Multimodal imaging features of MPD have not been reported in literature as yet. A 55-year-old male presented to us with gradual loss of vision in both eyes for last 6 months. The best corrected visual acuity was 20/30, N6 in both eyes. Fundus evaluation revealed multiple areas of chorioretinal atrophy over posterior pole along with flecks around vascular arcades. The fovea was spared [Fig. 1]. Infrared reflectance imaging showed hypoautofluroscence corresponding to the atrophic area [Fig. 2]. Fundus autofluorescence imaging showed hypoautofluorescence corresponding to the atrophic areas and hyperautofluorescence corresponding to flecks [Fig. 3]. Fundus fluorescence angiography at late phase showed diffuse transmission defects and staining of the atrophic area. There was no choroidal silence [Fig. 4]. Spectral domain optical coherence tomography (SDOCT) showed ovoid, moderate reflective deposits anterior to the ellipsoid zone and small schitic spaces in the inner retinal layers. Multiple hyperreflective dots were also seen in the inner choroid [Fig. 5].
Figure 1
Color fundus photography of right (a) and left (b) eye showed multiple chorioretinal atrophic patches over the posterior pole just sparing the fovea
Figure 2
Infrared reflectance fundus imaging of right (a) and left (b) eye showed hypoautofluroscence corresponding to the atrophic area (arrowheads)
Figure 3
Fundus autofluorescence imaging of right (a) and left (b) eye showed hypoautofluorescence corresponding to the atrophic areas (arrowheads) and hyperautofluorescence corresponding to the flecks (arrow)
Figure 4
Fundus fluorescence angiography of right (a) and left (b) eye at late phase showed diffuse transmission defects and staining of the atrophic area. (arrowheads) There was no choroidal silence
Figure 5
Spectral domain optical coherence tomography scan of right (a) and left (b) eye through the fovea showed ovoid moderate reflective deposits just anterior to the ellipsoid zone (star) and small schitic spaces in the inner retina (arrowhead). Multiple hyperreflective dots were also seen in the inner choroid in both eyes (long arrow)
Color fundus photography of right (a) and left (b) eye showed multiple chorioretinal atrophic patches over the posterior pole just sparing the foveaInfrared reflectance fundus imaging of right (a) and left (b) eye showed hypoautofluroscence corresponding to the atrophic area (arrowheads)Fundus autofluorescence imaging of right (a) and left (b) eye showed hypoautofluorescence corresponding to the atrophic areas (arrowheads) and hyperautofluorescence corresponding to the flecks (arrow)Fundus fluorescence angiography of right (a) and left (b) eye at late phase showed diffuse transmission defects and staining of the atrophic area. (arrowheads) There was no choroidal silenceSpectral domain optical coherence tomography scan of right (a) and left (b) eye through the fovea showed ovoid moderate reflective deposits just anterior to the ellipsoid zone (star) and small schitic spaces in the inner retina (arrowhead). Multiple hyperreflective dots were also seen in the inner choroid in both eyes (long arrow)
Discussion
Our report utilizing multimodal imaging techniques helps in pinpointing the diagnosis of MPD. Although SDOCT findings in MPD have been described previously, subretinal deposits and schitic spaces are hitherto unreported.[2] Of note was the presence of multiple hyperreflective dots in inner choroid in our case. Similar lesions have been noted in eyes with punctate inner choroidopathy and correspond to inflammatory deposits within choroidal vessels. Their presence in our case might indicate possible subclinical inflammatory pathology in these dystrophies.[3] Multimodal imaging helps in distinguishing this entity from related disorders and provides unique insight into their possible pathogeneses.
Authors: Javier Zarranz-Ventura; Dawn A Sim; Pearse A Keane; Praveen J Patel; Mark C Westcott; Richard W Lee; Adnan Tufail; Carlos E Pavesio Journal: Ophthalmology Date: 2014-05-20 Impact factor: 12.079