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Arch Ophthalmol. 2001;119:1855-1856.

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

INTRODUCTION

Wilms tumor, aniridia, genitourinary anomalies, and mental retardation (WAGR syndrome) are caused by the deletion of chromosome 11p13, which includes the Wilms tumor gene (WT1) and the aniridia gene (PAX6) loci (MEM, No. 194070). We report a case of atypical WAGR syndrome with anterior segment anomaly and microphthalmos.

Report of a Case

A 1-month-old boy had microphthalmos bilaterally. A microcornea with a corneal cyst in the right eye (axial length, 14.4 mm) (Figure 1A) and corneal opacity and absent anterior chamber in the left eye (axial length, 21.0 mm) (Figure 1B) seemed to be part of an anterior segment anomaly that includes the Peter anomaly. The vitreous cavities and posterior segments were normal. We examined the right eye with a small contact lens and light stimuli and obtained a normal response on the electroretinogram and in the left eye a subnormal response, suggesting retinal dysfunction. Wilms . . . [Full Text of this Article]

Comment

Corresponding author: Noriyuki Azuma, MD, Department of Ophthalmology, National Children's Hospital, 3-35-31 Taishido, Setagaya-ku Tokyo, 154-8509 Japan (e-mail: nazuma@nch.go.jp).