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Arch Ophthalmol. 2002;120:979-984.

	Since this article does not have an abstract, we have provided the first 150 words of the full text and any section headings.

Hereditary X-linked retinoschisis (RS) is the most common cause of juvenile macular degeneration in males^1-2 and may lead to vitreoretinal degeneration characterized by cystic spoke-wheel maculopathy, peripheral retinoschisis, alterations of the vitreous body, and a reduced b wave on the electroretinogram. Its prevalence ranges from 1:5000 to 1:25 000.^3-4 The condition is usually bilateral and affects males only. Males with juvenile RS usually seek treatment because of diminished vision at school age, followed by progressive visual deterioration later in life. Peripheral retinoschisis is found in 50% of patients and may be limited to the inferior temporal quadrant. Breaking of the inner schisis layer may lead to unsupported retinal vessels in the vitreous cavity, called a "congenital vascular veil."⁵ There have been few reports on the histopathologic characteristics of RS.^6-11 The principal feature in all these cases was a large schisis cavity originating from the nerve fiber layer (NFL). Several theories concerning . . . [Full Text of this Article]

Patient, Materials, and Methods


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Corresponding author and reprints: Cornelia M. Mooy, MD, PhD, Pathology Laboratory Dordrecht, Jkvr Van den Santheuvelweg 2A, 3317NL Dordrecht, the Netherlands (e-mail: cmooy@paldordt.com).

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