case_images: PMC10032463_01
case_images: Fundus imaging of both eyes of a patient with end-stage Sjogren-Larsson
  syndrome (SLS). Fundus photographs of the (a) right and (b) left eyes show
  depigmented, pale areas in the central macula associated with faint yellow
  parafoveal crystalline deposits more numerous in the left than the right
  eye. Fluorescein angiography reveals staining of the inclusions in the (c)
  right and (d) left eyes. Fundus autofluorescence images illustrate
  hyperfluorescence with a speckled hypo-autofluorescence pattern in the
  parafoveal region of both the (e) right and (f) left eyes.
case_images: 10.1177_26330040221122496-fig1.jpg
case_images: PMC10032463_01_10.1177_26330040221122496-fig1.jpg
case_images: fig1-26330040221122496
case_images: Dilated fundus examination revealed depigmented, pale areas of atrophy of the central macula in both eyes associated with glistening crystal-like deposits consistent with a crystalline maculopathy (Figure 1(a) and (b)). Fluorescein angiography revealed stippled hyperfluorescence in the parafovea with staining of the crystalline macular deposits (Figure 1(c) and (d)). Fundus autofluorescence demonstrated parafoveal hyperfluorescence with a speckled hypo-autofluorescence pattern in both eyes (Figure 1(e) and (f)).
case_images: Imaging of the macula of a patient with end-stage Sjogren-Larsson syndrome
  (SLS). Horizontal high-definition (4096 A-scans) 6 mm spectral domain
  optical coherence tomography (SD-OCT) scans centered on the fovea of the
  right (a) and left (b) eyes (Cirrus software version 11.5.2.54532; Carl
  Zeiss Meditec, Inc. Dublin, CA). Outer retinal loss involves the parafovea
  of both eyes associated with cystoid macular degeneration. There is
  vitreomacular traction in both eyes with vitreous syneresis. A thin serous
  detachment of the macula is evident in the right eye. A distinct lack of
  reverse shadowing demonstrates the relative sparing of the retinal pigment
  epithelium layer. En face macular thickness maps (colored)
  derived from the 6 mm macular cube (512 x 128 macular cube) overlying the
  corresponding near-infrared reflectance fundus image (36  x 30 ) centered on
  the fovea of the right (c) and left (d) eyes, respectively. Central macular
  thickness was significantly decreased to 118 microm on the right and 119 microm on
  the left.
case_images: 10.1177_26330040221122496-fig2.jpg
case_images: PMC10032463_01_10.1177_26330040221122496-fig2.jpg
case_images: fig2-26330040221122496
case_images: Optical coherence tomography (OCT) demonstrated central macular atrophy with ellipsoid zone (EZ) band loss associated with cystoid degeneration and vitreomacular traction in both eyes (Figure 2).