X-linked congenital retinoschisis

  • Ulrich Kellner
  • Stefanie Brümmer
  • Michael H. Foerster
  • Achim Wessing
Clinical Investigations


The natural history and electrophysiological findings of 52 patients with X-linked congenital retinoschisis with a follow-up of up to 26 years are described. The mean visual acuity was reduced to 0.24±0.2 and remained unchanged in most patients during this time. If visual loss occurred, it usually happened in the first decennium. The complications were retinal detachments in 11% and vitreous hemorrhages in 4% of the eyes. In general, the vitreous hemorrhages resolved spontaneously. Retinal detachments were treated successfully with conventional buckling procedures. Redetachments occurred in about 40%. Prophylactic laser coagulation was of no use because it was complicated by detachment in 43% of our series. The electro-oculogram was usually normal. In addition to the known electroretinographic findings of normal a-wave and reduced b-wave amplitudes, we found prolonged b-wave latencies and implicit times, as well as a reduced 30 Hz flicker response.


Public Health Visual Acuity Natural History Visual Loss Retinal Detachment 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.


Unable to display preview. Download preview PDF.

Unable to display preview. Download preview PDF.


  1. 1.
    Bengtsson B, Linder B (1967) Sex-linked hereditary juvenile retinoschisis. Acta Ophthalmol 45:411–423Google Scholar
  2. 2.
    Condon GP, Brownstein S, Wang NS, Kearns AF, Ewing CC (1986) Congenital hereditary (juvenile X-linked) retinoschisis. Histopathologic and ultrastructural findings in three eyes. Arch Ophthalmol 104:576–583PubMedGoogle Scholar
  3. 3.
    Constantaras AA, Dobbie JG, Choromokos EA, Frenkel M (1972) Juvenile sex-linked recessive retinoschisis in a black family. Am J Ophthalmol 74:1166–1178PubMedGoogle Scholar
  4. 4.
    Denden A (1975) X-chromosomale vitreoretinale Degeneration ERG- und EOG-Untersuchungsergebnisse. Klin Monatsbl Augenheilkd 166:35–43PubMedGoogle Scholar
  5. 5.
    Deutman AF (1971) The hereditary dystrophies of the posterior pole of the eye. Gorcum, Assen, The NetherlandsGoogle Scholar
  6. 6.
    Ewing CC, Ives EJ (1969) Juvenile hereditary retinoschisis. Trans Ophthalmol Soc UK 89:29–39Google Scholar
  7. 7.
    Forsius H, Krause U, Helve J, Vuopala V, Mustonen E, Vainio-Mattila B, Fellman J, Eriksson AW (1973) Visual acuity in 183 cases of X-chromosomal retinoschisis. Can J Ophthalmol 8:385–393PubMedGoogle Scholar
  8. 8.
    Haas J (1898) Über das Zusammenvorkommen von Verände-rungen der Retina und Chorioidea. Arch Augenheilkd 37:343–348Google Scholar
  9. 9.
    Hirose T, Wolf E, Hara A (1977) Electrophysiological and psychophysical studies in congenital retinoschisis of X-linked recessive inheritance. Doc Ophthalmol Proc Ser 13:173–184Google Scholar
  10. 10.
    Knave B, Nilsson SE, Lunt T (1973) The human electroretinogram: DC recordings at low and conventional stimulus intensities. Acta Ophthalmol 51:716–726Google Scholar
  11. 11.
    Lisch W (1983) Hereditary vitreoretinal degenerations. Dev Ophthalmol 8:19–32Google Scholar
  12. 12.
    Manschot WA (1972) Pathology of hereditary juvenile retinoschisis. Arch Ophthalmol 88:131–138PubMedGoogle Scholar
  13. 13.
    Miller RF, Dowling JE (1970) Intracellular responses of the Müller (glial) cells of the mudpuppy retina: Their relation to b-wave of the electro-retinogram. J Neurophysiol 33:323–341PubMedGoogle Scholar
  14. 14.
    Miyake Y, Miyake S, Yanagida K (1981) X-chromosomal congenital retinoschisis. Acta Soc Ophthalmol Jpn 83:97–112Google Scholar
  15. 15.
    Peachey NS, Fishman GA, Derlacki DJ, Brigell MG (1987) Psychophysical and electroretinographic findings in X-linked juvenile retinoschisis. Arch Ophthalmol 105:513–516PubMedGoogle Scholar
  16. 16.
    Rohde N, Täumer R, Pernice D (1977) Vorschlag eines verbesserten klinischen EOG-Testes. Ber Dtsch Ophthalmol Ges 74:747–750Google Scholar
  17. 17.
    Sorsby A, Sheridan M, Leary GA, Benjamin B (1960) Vision, visual acuity, and ocular refraction of young men. Br Med J 1:1394–1405Google Scholar
  18. 18.
    Tanino T, Katsumi O, Hirose T (1985) Electrophysiological similarities between two eyes with X-linked recessive retinoschisis. Doc Ophthalmol 60:149–161PubMedGoogle Scholar
  19. 19.
    Thaler A, Heilig P, Slezak H (1973) Elektroretinogramm und Elektrookulogramm bei juveniler Retinoschisis. Klin Monatsbl Augenheilkd 163:699–703PubMedGoogle Scholar
  20. 20.
    Yanoff M, Rahn EK, Zimmerman LE (1968) Histopathology of juvenile retinoschisis. Arch Ophthalmol 79:49–53PubMedGoogle Scholar

Copyright information

© Springer-Verlag 1990

Authors and Affiliations

  • Ulrich Kellner
    • 1
  • Stefanie Brümmer
    • 1
  • Michael H. Foerster
    • 1
  • Achim Wessing
    • 1
  1. 1.Zentrum für Augenheilkunde der Universität EssenEssen 1Germany

Personalised recommendations