Background: Two classifications of retinoschisis were published in the
1960s. A marked expansion of our knowledge of the diseases that give
rise to retinoschisis indicates the need for an updated classification
. Methods: The new classification is based on the authors' clinical ex
perience, an extensive review of the literature, and a survey of 20 vi
treoretinal specialists. Results: There are three types of retinoschis
is: degenerative, hereditary, and secondary. Degenerative retinoschisi
s is very common and has been published extensively. In addition to th
e well known X-linked hereditary retinoschisis, there are less common
pedigrees with autosomal recessive or autosomal dominant patterns. The
re are at least 18 ocular diseases that may show varying degrees of se
condary retinoschisis. Conclusion: The many types of retinoschisis are
divided into these major categories. The multiplicity of types is emp
hasized by the plural form of the term: ''the retinoschises.''