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Corneal dystrophies.

¹Université Paris Descartes, Sorbonne Paris Cité, 15, rue de l'École-de-Médecine, 75006 Paris, France; Ophthalmology Unit, Ophthalmology Service, Hôtel-Dieu, Hôpitaux Universitaires Paris Centre, Assistance publique-Hôpitaux de Paris, 1, place du Parvis-Notre-Dame, 75004 Paris, France; Équipe 17, Inserm UMRS 1138, Centre de Recherche des Cordeliers, 15, rue de l'École-de-Médecine, 75006 Paris, France.

Journal Francais D'Ophtalmologie

|June 7, 2017

Summary

This summary is machine-generated.

Corneal dystrophies, affecting 0.09% of people, present diagnostic challenges. Management involves symptom relief, medical treatments, and surgical options like keratoplasty, with lamellar procedures often preferred.

Keywords:

Classification Corneal dystrophy Endothelial dystrophy Epithelial dystrophy Genetic Granular dystrophy Hereditary corneal condition Keratectomy Keratoplasty Stromal dystrophy

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Area of Science:

Ophthalmology
Genetics
Medical Science

Background:

Corneal dystrophies are a group of genetic eye disorders.
Distinguishing between degenerative and hereditary corneal diseases can be challenging.
These conditions affect approximately 0.09% of the population.

Purpose of the Study:

To outline the diagnostic and management strategies for corneal dystrophies.
To discuss the classification system (IC3D) based on phenotype and genotype.
To review current and evolving treatment modalities.

Main Methods:

Phenotypic and genotypic analysis for diagnosis using the IC3D classification.
Management of functional symptoms including pain, visual loss, and photophobia.
Review of medical treatments (drops, ointments, bandage lenses) and surgical interventions (keratectomy, keratoplasty).

Main Results:

Corneal dystrophies present with diverse symptoms requiring symptomatic management.
Treatment progresses from conservative medical approaches to surgical options.
Anterior lamellar and endothelial keratoplasty are increasingly favored over penetrating keratoplasty.

Conclusions:

Accurate diagnosis of corneal dystrophies relies on integrated phenotype and genotype data.
Treatment strategies are tailored to symptom severity and disease progression.
Specialized care is essential for managing rare corneal dystrophies effectively.