Abstract
Aniridia consists in a complex malformation of the eye with congenital absence of iris. This genetic rare disease can cause severe visual impairment occurring from various mechanisms. Ocular clinical signs in aniridia may associate glaucoma, most common complication with limbal insufficiency leading to keratopathy, cataract, ptosis, foveal aplasia or a microphthalmia. The cataract in aniridia must be identified with its specificities in order to adjust the treatment according with other ocular signs and complications of the disease. In aniridia, cataract is usually reduced in infancy to mild opacities or partial lens opacification. The main treatment aims to correct ametropia, potentially induced strabismus, nystagmus and amblyopia in case of anisometropia or cataract asymmetry. When visual acuity becomes low, time of cataract surgery must be discussed. The assessment of the low vision due to an occlusive cataract must be confirmed. Other factors as foveal aplasia, corneal opacities, glaucoma may influence visual acuity and will not be corrected by cataract surgery. Different techniques of cataract surgeries are available and adapted for cataract in aniridia however the surgeon must be aware of high rate of complications as glaucoma, fibrosis and ocular surface impairment. Phacoemulsification and classical intraocular lens, artificial iris or combinated intraocular lens with diaphragm can be performed very carefully. A regular follow-up of the patient must be performed in order to detect complications.
Access this chapter
Tax calculation will be finalised at checkout
Purchases are for personal use only
References
Shaw M, Falls H, Neel J (1960) Congenital aniridia. Am J Human Genet 12:389–415
Eden U, Iggman D et al (2008) Epidemiology of aniridia in Sweden and Norway. Acta Ophthalmol 86:727–729
Nelson LB, Spaeth GL, Nowinski TS, Margo CE, Jackson L (1984) Aniridia. Rev Surv Ophthalmol 28:621–642
Elsas FJ, Maumenee IH, Kenyon KR, Yoder F (1977) Familial aniridia with preserved ocular function. Am J Ophthalmol 83(5):718–724
Layman PR, Anderson DR, Flynn JT (1974) Frequent occurrence of hypoplastic optic disks in patients with aniridia. Am J Ophthalmol 77(4):513–516
Traboulsi E (1998) Genetic disease of the eye. Oxford University Press, New York
Netland PA, Scott ML, Boyle JW 4th, Lauderdale JD (2011) Ocular and systemic findings in a survey of aniridia subjects. J AAPOS 15(6):562–566
Bremond-Gignac D, Gerard-Blanluet M, Copin H, Bitoun P, Baumann C, Crolla JA, Benzacken B, Verloes A (2005) Three patients with hallucalpolydactyly and WAGR syndrome, including discordant expression of Wilmstumor in MZ twins. Am J MedGenet A 134(4):422–425
Bremond-Gignac D, Crolla JA, Copin H, Guichet A, Bonneau D, Taine L, Lacombe D, Baumann C, Benzacken B, Verloes A (2005) Combination of WAGR and Potocki-Shaffer contiguous deletion syndromes in a patient with an 11p11.2-p14 deletion. Eur J Hum Genet 13:409–413
Jastaneiah S, AL-Rajhi AA (2005) Association of aniridia and dry eyes. Ophthalmology 112(9):1535–1540
Brandt JD, Casuso LA, Budenz DL (2004) Markedly increased central corneal thickness: an unrecognized finding in congenital aniridia. Am J Ophthalmol 137:348–350
Schneider S, Osher RH, Burk SE, Lutz TB, Montione R (2003) Thinning of the anterior capsule associated with congenital aniridia. J Cataract Refract Surg 29(3):523–525
Tsai JH, Freeman JM, Chan CC, Schwartz GS, Derby EA, Petersen MR, Holland EJ (2005) A progressive anterior fibrosis syndrome in patients with postsurgical congenital aniridia. Am J Ophthalmol 140(6):1075–1079
Neuhann IM, Neuhann TF (2010) Cataract surgery and aniridia. Curr Opin Ophthalmol 21(1):60–64
Reinhard T, Engelhardt S, Sundmacher R (2000) Black diaphragm aniridia intraocular lens for congenital aniridia: long-term follow-up. J Cataract Refract Surg 26(3):375–381
Aslam SA, Wong SC, Ficker LA, Maclaren RE (2008) Implantation of the black diaphragm intraocular lens in congenital and traumatic aniridia. Ophthalmology 115(10):1705–1712
Author information
Authors and Affiliations
Corresponding author
Editor information
Editors and Affiliations
Rights and permissions
Copyright information
© 2015 Springer International Publishing Switzerland
About this chapter
Cite this chapter
Brémond-Gignac, D. (2015). Clinical and Surgical Management of Cataract in Congenital Aniridia. In: Parekh, M., Poli, B., Ferrari, S., Teofili, C., Ponzin, D. (eds) Aniridia. Springer, Cham. https://doi.org/10.1007/978-3-319-19779-1_5
Download citation
DOI: https://doi.org/10.1007/978-3-319-19779-1_5
Publisher Name: Springer, Cham
Print ISBN: 978-3-319-19778-4
Online ISBN: 978-3-319-19779-1
eBook Packages: Biomedical and Life SciencesBiomedical and Life Sciences (R0)