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[Corneal dystrophies].

¹Université Paris Descartes, Sorbonne Paris Cité, 15, rue École-de-Médecine, 75006 Paris, France; Unité d'ophtalmologie de l'Hôtel-Dieu, service d'ophtalmologie, 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 18, 2017

Summary

This summary is machine-generated.

Corneal dystrophies, affecting 0.09% of people, present diagnostic challenges. Treatment ranges from medical management to advanced keratoplasty techniques, with newer methods often preferred.

Keywords:

Affection héréditaire de cornée Classification Corneal dystrophy Dystrophie de cornée Dystrophie endothéliale Dystrophie granulaire Dystrophie stromale Dystrophie épithéliale Endothelial dystrophy Epithelial dystrophy Genetic Granular dystrophy Génétique Hereditary corneal condition Keratectomy Keratoplasty Kératectomie Kératoplastie Stromal dystrophy

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Area of Science:

Ophthalmology
Genetics
Medical Diagnostics

Background:

Corneal dystrophies are a group of inherited eye diseases affecting approximately 0.09% of the population.
Discriminating between degenerative and hereditary corneal diseases can be challenging.
The International Classification of Corneal Dystrophies (ICD3) provides a framework for diagnosis based on phenotype and genotype.

Purpose of the Study:

To review the diagnostic challenges and management strategies for corneal dystrophies.
To highlight the evolving treatment landscape, including surgical interventions.
To emphasize the need for comprehensive care for rare corneal conditions.

Main Methods:

Review of existing literature on corneal dystrophies.
Discussion of diagnostic criteria based on the ICD3 classification.
Overview of current and emerging treatment modalities.

Main Results:

Corneal dystrophies present with various functional symptoms like visual loss and photophobia.
Treatment options include medical therapies (drops, ointments), less invasive surgeries (phototherapeutic keratectomy), and advanced keratoplasty.
Anterior lamellar and endothelial keratoplasty are increasingly favored over penetrating keratoplasty.

Conclusions:

Accurate diagnosis of corneal dystrophies requires integrating clinical presentation, genetic data, and classification systems.
Management strategies are tailored to symptom severity and disease progression.
Specialized care is crucial for patients with rare corneal dystrophies, with a trend towards less invasive surgical options.