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. 1979 May;63(5):368–373. doi: 10.1136/bjo.63.5.368

Vascular opacification and leakage in X-linked (juvenile) retinoschisis.

J L Green Jr, L M Jampol
PMCID: PMC1043490  PMID: 572698

Abstract

A 22-year-old man had X-linked retinoschisis with extensive vascular sheathing in the posterior pole and dendritic opacified retinal vessels in the periphery. Detailed fluorescein angiographic studies were performed. In the far periphery areas of capillary nonperfusion were seen. Sluggish circulation was present in the majority of dendritic opacified vessels in the areas of schisis. Late staining of the disc and posterior arteries and veins was noted. Scattered intraretinal fluorescein leakage was present even in clinically nonschitic retina, which suggests that the vascular changes may precede the retinoschisis. The patient's half-brother also had X-linked retinoschisis, but the schisis and the vascular changes were much less prominent. Although the retinal vascular changes with X-linked retinoschisis are similar to those seen with the Favre-Goldmann syndrome, a differentiation of the two diseases can be made by family studies, the presence or absence of nyctalopia, examination of the macular areas, measurement of dark adaptation, and electroretinogram determinations.

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Selected References

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  1. Anderson J. R. ANTERIOR DIALYSIS OF THE RETINA: DISINSERTION OR AVULSION AT THE ORA SERRATA. Br J Ophthalmol. 1932 Nov;16(11):705–727. doi: 10.1136/bjo.16.11.705. [DOI] [PMC free article] [PubMed] [Google Scholar]
  2. Burns R. P., Lovrien E. W., Cibis A. B. Juvenile sex-linked retinoschisis: clinical and genetic studies. Trans Am Acad Ophthalmol Otolaryngol. 1971 Sep-Oct;75(5):1011–1021. [PubMed] [Google Scholar]
  3. Cibis P. A. Retinoshchisis--retinal cysts. Trans Am Ophthalmol Soc. 1965;63:417–453. [PMC free article] [PubMed] [Google Scholar]
  4. Constantaras A. A., Dobbie J. G., Choromokos E. A., Frenkel M. Juvenile sex-linked recessive retinoschisis in a black family. Am J Ophthalmol. 1972 Dec;74(6):1166–1178. doi: 10.1016/0002-9394(72)90739-8. [DOI] [PubMed] [Google Scholar]
  5. Ewing C. C., Cullen A. P. Fluorescein angiography in X-chromosomal maculopathy with retinoschisis (juvenile hereditary retinoschisis). Can J Ophthalmol. 1972 Jan;7(1):19–28. [PubMed] [Google Scholar]
  6. Ewing C. C., Ives E. J. Juvenile hereditary retinoschisis. Trans Ophthalmol Soc U K. 1970;89:29–39. [PubMed] [Google Scholar]
  7. Fishman G. A., Fishman M., Maggiano J. Macular lesions associated with retinitis pigmentosa. Arch Ophthalmol. 1977 May;95(5):798–803. doi: 10.1001/archopht.1977.04450050076006. [DOI] [PubMed] [Google Scholar]
  8. Fishman G. A., Jampol L. M., Goldberg M. F. Diagnostic features of the Favre-Goldmann syndrome. Br J Ophthalmol. 1976 May;60(5):345–353. doi: 10.1136/bjo.60.5.345. [DOI] [PMC free article] [PubMed] [Google Scholar]
  9. Harris G. S., Yeung J. Maculopathy of sex-linked juvenile retinoschisis. Can J Ophthalmol. 1976 Jan;11(1):1–10. [PubMed] [Google Scholar]
  10. Helve J. Colour vision in X-chromosomal juvenile retinoschisis. Mod Probl Ophthalmol. 1972;11:122–129. [PubMed] [Google Scholar]
  11. Krause U., Vainio-Mattila B., Eriksson A., Forsius H. Fluorescein angiographic studies on x-chromosomal retinoschisis. Acta Ophthalmol (Copenh) 1970;48(4):794–807. [PubMed] [Google Scholar]
  12. Lewis R. A., Lee G. B., Martonyi C. L., Barnett J. M., Falls H. F. Familial foveal retinoschisis. Arch Ophthalmol. 1977 Jul;95(7):1190–1196. doi: 10.1001/archopht.1977.04450070088006. [DOI] [PubMed] [Google Scholar]
  13. Manschot W. A. Pathology of hereditary juvenile retinoschisis. Arch Ophthalmol. 1972 Aug;88(2):131–138. doi: 10.1001/archopht.1972.01000030133002. [DOI] [PubMed] [Google Scholar]
  14. Tasman W. Macular changes in congenital retinoschisis. Mod Probl Ophthalmol. 1975;15:40–49. [PubMed] [Google Scholar]
  15. Thomson E. MEMORANDUM: Regarding a Family in which Neuro-Retinal Disease of an unusual kind occurred only in the Males. Br J Ophthalmol. 1932 Dec;16(12):681–686. doi: 10.1136/bjo.16.12.681. [DOI] [PMC free article] [PubMed] [Google Scholar]
  16. Yanoff M., Kertesz Rahn E., Zimmerman L. E. Histopathology of juvenile retinoschisis. Arch Ophthalmol. 1968 Jan;79(1):49–53. doi: 10.1001/archopht.1968.03850040051014. [DOI] [PubMed] [Google Scholar]

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