Focal macular electroretinogram in X-linked congenital retinoschisis

Invest Ophthalmol Vis Sci. 1993 Mar;34(3):512-5.


Purpose: To study macular function of X-linked congenital retinoschisis (CRS) by focal macular electroretinogram (MERG).

Methods: MERGs were recorded with 5 degrees, 10 degrees, and 15 degrees spots in 20 patients with CRS. Seventeen patients showed foveal schisis with little or no change in foveal fluorescein angiography (Group 1), and three patients showed advanced macular changes with nonspecific macular degeneration (Group 2).

Results: In Group 1, a-wave amplitudes were within the normal range, but b-waves and oscillatory potentials (OPs) had mean amplitudes significantly below those for normal control subjects. The mean b- to a-wave ratios, significantly lower than in normal eyes, decreased significantly with decreasing spot size. The implicit times of a-waves, b-waves, and OPs were significantly delayed. In Group 2, MERGs were nearly nondectable.

Conclusions: The macular pathology of CSR exists mainly in the middle and inner retinal layers, disturbing the fovea more than the perifovea, whereas degeneration of photoreceptors progresses in more advanced stage.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adolescent
  • Adult
  • Child
  • Electroretinography*
  • Fluorescein Angiography
  • Genetic Linkage / genetics*
  • Humans
  • Macula Lutea / physiology*
  • Male
  • Photoreceptor Cells / physiology
  • Retinal Perforations / congenital*
  • Retinal Perforations / genetics
  • Retinal Perforations / physiopathology*
  • X Chromosome