History/Chief Complaint

  • 64 year old black male from the Virgin Islands who first noticed difficulty with night vision at about age 9 and difficulty reading and seeing faces at age 18 or so. Both symptoms have been stable for over 4 decades. He reported no family history of vision problems and all of his 10 siblings have no visual complaints. Consanguinity could not be confirmed or ruled out. His general health has always been unremarkable.

Clinical Findings

  • BCVA 20/400 OD and 20/400 OS.
  • Fundus exam: C/D ratio approx .7 with temporal pallor OU in 1997 and perhaps .75 in 2009. Small, uniform yellowish white dots throughout the fundus OU most marked in the arcades and mid-periphery. Round atrophic macula lesions OU that have enlarged slightly in size over the 12 year follow-up period.
  • Color vision with PIP – the only correct response OD and OS was to the demo plate.
  • IOPs In early 1997 12/11 mm but 3 months later 20/18mm. IOPs have never been above 20 mm.
  • Sita fast 30-2: Central scotomas OU and MD -15 OD and MD -20 OS with poor fixation OU.

Electroretinograpy – ERGs in 1997

  • S1 blue-flat (extinguished) rod response after 20 min dark adaptation.
  • Max ERG after 30 min dark adaptation:
    — b wave implicit time (IT) 34 msec and 122 uv in amplitude
  • However, after 3 hrs of dark adaptation:
    — S1 blue: b wave IT 78 msec and amp 105 uv
    — Max ERG response (bright light after dark adaptation) IT 38 msec and amp 169 uv
    — Cone response IT 32 msec and amp 43 uv. Flicker ERG 45 uv in amp.

SD OCT

  • Numerous hyperreflective dots larger than the thickness of the PIL (see pg 14) which “straddle” the PIL.
  • An attenuated macula with loss of the PIL and loss of the RPE as well as overall thinning.
  • Retinal nerve fiber layer (RNFL) with Cirrus™ HD-OCT revealed a pronounced loss OU characteristic of glaucoma.