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Arch Ophthalmol. 1998;116:381-382.

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

We read with interest the article published in the ARCHIVES by Chynn and colleagues¹ who described a full-term male neonate who had bilateral leukocoria, vascularized retrolental membranes, and hemorrhagic retinal detachments. Histopathological examination of 1 eye at the age of 5 weeks revealed elongated ciliary processes, large retrolental vessels, hemorrhagic retinal detachment, and retinal dysplasia—findings indicative of either Norrie disease (ND) or persistent hyperplastic primary vitreous (PHPV). The authors subsequently performed molecular genetic analysis and identified a 1–base-pair deletion in codon 35 of the ND gene, thereby establishing a diagnosis of ND.

As Chynn's study illustrates, the clinical distinction between sporadic ND without systemic manifestations and bilateral PHPV can be difficult and has led to much confusion, with many authors stating that bilateral PHPV occurs in 10% of cases while others believe that bilateral PHPV is extremely rare or may not exist. In addition, it is common in our experience . . . [Full Text of this Article]

Report of Cases

Case 1

Case 2

Comment
Corresponding author: Barkur S. Shastry, PhD, Eye Research Institute, Oakland University, Dodge Hall, Room 431, Rochester, MI 48309.

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