metricas
covid
Buscar en
Archivos de la Sociedad Española de Oftalmología (English Edition)
Toda la web
Inicio Archivos de la Sociedad Española de Oftalmología (English Edition) Crystalline lens alterations in congenital aniridia
Journal Information
Vol. 96. Issue S1.
Aniridia: Update in Diagnosis, Complications and Management
Pages 38-51 (November 2021)
Share
Share
Download PDF
More article options
Visits
6
Vol. 96. Issue S1.
Aniridia: Update in Diagnosis, Complications and Management
Pages 38-51 (November 2021)
Review
Crystalline lens alterations in congenital aniridia
Alteraciones del cristalino en la aniridia congénita
Visits
6
F. D’Oriaa,b, R. Barraquerc, J.L. Alioa,d,
Corresponding author
jlalio@vissum.com

Corresponding author.
a Vissum Innovation, Alicante, Spain
b Department of Basic Medical Science, Neuroscience and Sense Organs, University of Bari, Bari, Italy
c Instituto Universitario Barraquer, Universitat Autònoma de Barcelona, Barcelona, Spain
d División de Oftalmología, Universidad Miguel Hernández, Alicante, Spain
This item has received
Article information
Abstract
Full Text
Bibliography
Download PDF
Statistics
Figures (16)
Show moreShow less
Tables (1)
Table 1. Demographic characteristics, visual outcomes and complications of the main studies on patients with cataract and congenital aniridia.
Special issue
This article is part of special issue:
Vol. 96. Issue S1

Aniridia: Update in Diagnosis, Complications and Management

More info
Abstract

Congenital aniridia is a rare genetic disease associated with mutations in the PAX6 gene. Changes in the lens in aniridia can be alterations of size and shape, of position – which generally reveal zonular weakness and determines subluxation of the lens – and mainly changes in transparency, cataracts, with variable morphology of polar, cortical, subcapsular, lamellar, and more rarely, nuclear cataract. Visual acuity and quality of vision in patients with congenital aniridia complicated by cataracts can be improved by carefully planned surgery, when lack of media transparency justifies surgical indication. Most patients have some improvement in visual acuity and quality of retinal image. Cataract surgery with aniridia is complicated by pathological changes due to the underlying cause of the aniridia. Challenges include corneal opacification, friable capsule and, above all, iris and pupil reconstruction. It can also determine late complications, such as secondary glaucoma or deterioration of pre-existent glaucoma, and corneal endothelial decompensation. After crystalline lens surgery in these patients, either by cataract or dislocation, for visual rehabilitation there are various techniques such as keratopigmentation, prosthetic iris devices or Morcher intraocular lenses with a black diaphragm. An appropriate individualised surgical plan should be selected depending on patient and surgical experience, in order to minimise complications and give the best chance of postoperative success.

Keywords:
Aniridia
Cataracts
Congenital defects
Corneal tattoo
Keratopigmentation
Prosthetic iris devices
Resumen

La aniridia congénita es una enfermedad genética rara asociada a mutaciones del gen PAX6. Las alteraciones del cristalino en aniridia pueden ser de tamaño y forma, de posición – que en general revelan debilidad zonular y posible subluxación del cristalino – y principalmente alteraciones de transparencia. Pueden presentar cataratas con morfología variable desde polares, corticales, subcapsulares y lamelares hasta más infrecuentemente nucleares. La agudeza visual y la calidad de la visión en pacientes con aniridia congénita asociada a cataratas se pueden mejorar mediante una cirugía cuidadosamente planificada cuando la falta de transparencia de los medios justifique la indicación quirúrgica. La cirugía de cataratas con aniridia se complica por alteraciones patológicas asociadas a la aniridia. Los desafíos incluyen opacificación corneal, cápsula friable y sobre todo reconstrucción de iris y pupila; además, pueden aparecer complicaciones tardías como glaucoma secundario o deterioro de glaucoma preexistente y descompensación endotelial corneal. Tras la cirugía cristaliniana en estos pacientes, bien por catarata o luxación, para la rehabilitación visual existen diversas técnicas como la queratopigmentación, los dispositivos protésicos iridianos o las lentes de Morcher con diafragma negro. Debe seleccionarse un método quirúrgico individualizado, según el paciente y la experiencia quirúrgica, que sea apropiado para minimizar las complicaciones y obtener mayores tasas de éxito postoperatorio.

Palabras clave:
Aniridia
Catarata
Alteraciones congénitas
Tatuaje corneal
Queratopigmentación
Dispositivos protésicos de iris

Article

These are the options to access the full texts of the publication Archivos de la Sociedad Española de Oftalmología (English Edition)
Subscriber
Subscriber

If you already have your login data, please click here .

If you have forgotten your password you can you can recover it by clicking here and selecting the option “I have forgotten my password”
Subscribe
Subscribe to

Archivos de la Sociedad Española de Oftalmología (English Edition)

Purchase
Purchase article

Purchasing article the PDF version will be downloaded

Price 19.34 €

Purchase now
Contact
Phone for subscriptions and reporting of errors
From Monday to Friday from 9 a.m. to 6 p.m. (GMT + 1) except for the months of July and August which will be from 9 a.m. to 3 p.m.
Calls from Spain
932 415 960
Calls from outside Spain
+34 932 415 960
E-mail
Article options
es en pt

¿Es usted profesional sanitario apto para prescribir o dispensar medicamentos?

Are you a health professional able to prescribe or dispense drugs?

Você é um profissional de saúde habilitado a prescrever ou dispensar medicamentos

Quizás le interese:
10.1016/j.oftale.2021.01.004
No mostrar más