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Aniridia and WAGR SyndromeA Guide for Patients and Their Families$
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Jill Ann Nerby and Jessca Otis

Print publication date: 2010

Print ISBN-13: 9780195389302

Published to Oxford Scholarship Online: November 2020

DOI: 10.1093/oso/9780195389302.001.0001

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PRINTED FROM OXFORD SCHOLARSHIP ONLINE (oxford.universitypressscholarship.com). (c) Copyright Oxford University Press, 2021. All Rights Reserved. An individual user may print out a PDF of a single chapter of a monograph in OSO for personal use. date: 24 October 2021

Glaucoma Problems Associated with Aniridia

Glaucoma Problems Associated with Aniridia

(p.69) 5 Glaucoma Problems Associated with Aniridia
Aniridia and WAGR Syndrome

Peter A. Netland

Oxford University Press

Glaucoma is a potentially vision-threatening problem that is commonly encountered in aniridia patients. This condition may develop at birth, or shortly thereafter. More commonly, however, glaucoma is acquired later in childhood or even young adulthood. If unrecognized and untreated, glaucoma can result in blindness. For this reason, it is important to be vigilant in watching for this condition in children affected with aniridia. Vision lost due to glaucomatous damage cannot be regained at a later time. In addition to glaucoma, children with aniridia may demonstrate other problems with their vision. They may have refractive errors, corneal or retinal problems, or abnormalities of eye movement. Foveal hypoplasia (lack of development of the retina) may limit vision in some children. In aniridia patients, cataract (opacification or cloudiness of the lens) is seen with approximately the same prevalence as glaucoma. Cataract, however, differs from glaucoma in that the vision loss due to cataract is reversible. Glaucoma is suspected in aniridia patients when there is an increased intraocular pressure. Glaucoma can be definitely diagnosed when changes of the optic nerve occur due to this elevated intraocular pressure. At the later stages of the disease, visual field loss occurs. In the normal eye, the fluid (aqueous humor) in the front of the eye (the anterior chamber) is produced by the ciliary body, which is located behind the iris (see Figure 5.1). The fluid produced from the ciliary body flows forward into the anterior chamber, where it drains from the anterior chamber angle through tissue called the trabecular meshwork. When there is an abnormal situation, the fluid exits the eye poorly or not at all, and the intraocular pressure may be increased. The fluid may be blocked from exiting the eye by a closed angle, or may flow poorly out of the eye even though the angle is open (see Figure 5.2). The angle may be closed in aniridic patients when the stump of residual iris covers the trabecular meshwork in the anterior chamber angle.

Keywords:   Adrenergic agonists, Blepharospasm, Carbonic anhydrase inhibitors, Foveal hypoplasia, Goniotomy, Hypotony, Intraocular pressure (IOP), Laser therapy, Megalocornea, Ophthalmoscopy

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