Adult-onset foveomacular vitelliform dystrophy: a study by optical coherence tomography

Am J Ophthalmol. 2003 Mar;135(3):362-7. doi: 10.1016/s0002-9394(02)01946-3.


Purpose: To evaluate the morphology of adult-onset foveomacular vitelliform dystrophy (AFVD) using optical coherence tomography (OCT) and to correlate the OCT findings with those of biomicroscopy and fluorescein angiography (FA).

Design: Prospective observational case series.

Methods: A complete ophthalmologic examination, including visual acuity, fundus biomicroscopy, FA, and OCT was performed in 21 eyes of 14 consecutive patients with AFVD.

Results: Mean age at presentation was 64 years (range, 39 to 84 years), and best-corrected visual acuity ranged from 20/25 to 20/400 (median 20/50). Sixteen of 21 eyes (11 patients) exhibited late staining of lesions on FA. In these 16 eyes, OCT revealed that AFVD material consists of a hyperreflective structure located between the photoreceptor and the retinal pigment epithelium layers. The retinal pigment epithelium layer was linear and was not elevated, unlike what is observed in retinal pigment epithelium detachment. Five other eyes (x4 patients) without late staining in FA showed, by OCT, a hyperreflective area at the level of the retinal pigment epithelium band, with no material visible between the photoreceptor and retinal pigment epithelium layers. In all 21 eyes, the retina overlying the hyperreflective structure was raised by the pseudovitelliform material and was markedly thinned.

Conclusions: Optical coherence tomography is a noninvasive useful tool that provides new information on the morphology of AFVD. It demonstrates, better than biomicroscopy, the location of the yellowish material under the sensory retina but above the retinal pigment epithelium, corresponding angiographically to the late staining. The foveal thinning found by OCT in all cases probably explains the progressive visual loss and possible evolution toward a full-thickness macular hole.

MeSH terms

  • Adult
  • Age of Onset
  • Aged
  • Aged, 80 and over
  • Diagnostic Techniques, Ophthalmological*
  • Female
  • Fluorescein Angiography
  • Fovea Centralis / pathology*
  • Humans
  • Interferometry
  • Light
  • Male
  • Middle Aged
  • Prospective Studies
  • Retinal Degeneration / pathology*
  • Tomography / methods
  • Visual Acuity