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Progression in juvenile X‐chromosomal retinoschisis
Author(s) -
Forsius H. R.,
Eriksson A. W.,
Damsten M.
Publication year - 1990
Publication title -
acta ophthalmologica
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.534
H-Index - 87
eISSN - 1755-3768
pISSN - 1755-375X
DOI - 10.1111/j.1755-3768.1990.tb01973.x
Subject(s) - retinoschisis , ophthalmology , fundus (uterus) , medicine , sclera , retinal , fundus photography , atrophy , anatomy , retinal detachment , fluorescein angiography , pathology
Forty‐five males and one homozygotic female showing X‐chromosomal juvenile retinoschisis were re‐investigated in 1987 after an average follow‐up study of more than 22 years. Twenty‐two of them had been investigated by fundus‐photography for at least 14 years. With time, the typical radial micro‐cystic macular area flattens out and atrophies. In very old persons only some large choroidal vessels are seen, covering the sclera in the previously radiating area. In young patients with additional peripheral schisis the veil ruptures, the schisis area flattens out, the holes grow and may unite. In the outer retinal layer pigment degeneration develops. In congenitally severe cases the atrophy is marked and rapid. The retinal vessels attenuate and the nerve head becomes pale. Total blindness results in cases of this cathegory. The severity of retinoschisis varies much in patients belonging to the same family and even in the two eyes of the same person.