J Korean Ophthalmol Soc.  2019 Oct;60(10):999-1005. 10.3341/jkos.2019.60.10.999.

Fundus Albipunctatus Diagnosed in a 9-year-old Female

Affiliations
  • 1Department of Ophthalmology, Gyeongsang National University Changwon Hospital, Changwon, Korea. medcabin@hanmail.net
  • 2Department of Ophthalmology, Gyeongsang National University College of Medicine, Jinju, Korea.
  • 3Institute of Health Sciences, Gyeongsang National University, Jinju, Korea.

Abstract

PURPOSE
We report a case of fundus albipunctatus discovered in a young patient.
CASE SUMMARY
A 7.6-year-old female showed numerous small whitish-yellow flecks in the perimacular area and retinal periphery. Dark adapted 0.01 electroretinography (ERG) and dark adapted 3.0 ERG were profoundly reduced. At 26 months after the first visit, the best-corrected visual acuities were 1.0 right eye and 0.9 left eye. There were no pigmented lesions, atrophic lesions, or vascular abnormalities in the retina. Humphrey and Goldmann visual field tests were performed, but neither of the tests revealed any scotomas or other visual field defect. The number and size of characteristic numerous small whitish-yellow retinal flecks seemed almost unchanged. In spectral domain-optical coherence tomography (SD-OCT), the subretinal hyper-reflective lesions spanned the retinal pigment epithelium and the external limiting membrane. ERG showed improved dark adapted responses (dark adapted 0.01 ERG and dark adapted 3.0 ERG) after prolonged dark adaptation (2.5 hours). No family member showed any abnormal findings.
CONCLUSIONS
Fundus albipunctatus is a rare disease in Koreans. We report a case diagnosed using fundus photography, SD-OCT, visual field tests, and ERG after prolonged dark adaptation (2.5 hours).

Keyword

Dark adaptation; Electroretinography; Fundus albipunctatus; Optical coherence tomography

MeSH Terms

Child*
Dark Adaptation
Electroretinography
Female*
Humans
Membranes
Photography
Rare Diseases
Retina
Retinal Pigment Epithelium
Retinaldehyde
Scotoma
Tomography, Optical Coherence
Visual Acuity
Visual Field Tests
Visual Fields
Retinaldehyde

Figure

  • Figure 1 Fundus photography of this patient. (A, B) At the initial visit. There are many yellow whitish spots on the equator and periphery of retina of both eyes. (C, D) Twenty six months after the initial visit. The spots seemed almost unchanged. (E, F) Ultrawide field fundus photography at 26 months after the initial visit. The size of spots was smaller on the inferior retina compared to superior, nasal and temporal retina.

  • Figure 2 Visual filed test at 26 months after the initial visit. The results of both eyes show generalized reduction of sensitivity. GHT = glaucoma Hemifield test; VFI = visual field index; MD = mean deviation; PSD = pattern standard deviation.

  • Figure 3 Spectral domain-optical coherence tomography at 26 months after the initial visit. Fovea seemed to be normal in both eyes (C, D), but hyper-reflective lesions were observed between retinal pigment epithelium layer and external limiting membrane on superior and inferior area to the fovea (A, B). The size of hyper-reflective lesions was smaller on the inferior area (A, B, E, F).

  • Figure 4 Electroretinography of right eye at 26 months after the initial visit by usual protocol (30 minutes dark adaptation) (A). Scotopic response substantially decreased, but photopic response was normal. After 150 minutes dark adaptation, scotopic response was recovered up to normal range (B). ERG = electroretinography.


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