Megalocornea, anterior megalophthalmos, keratoglobus and associated anterior segment disorders: A review
Autor: | Andrea L Vincent, Charles N J McGhee, Jie Zhang, Aaron Pin Chien Ong |
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Rok vydání: | 2021 |
Předmět: |
0301 basic medicine
Keratoconus medicine.medical_specialty genetic structures Anterior megalophthalmos 030105 genetics & heredity Cornea 03 medical and health sciences Elevated intraocular pressure Megalocornea 0302 clinical medicine Corneal thinning Ophthalmology Ciliary ring medicine Humans Eye Abnormalities business.industry Hydrophthalmos Eye Diseases Hereditary Genetic Diseases X-Linked medicine.disease eye diseases Buphthalmos 030221 ophthalmology & optometry sense organs business Keratoglobus |
Zdroj: | Clinical & Experimental Ophthalmology. 49:477-497 |
ISSN: | 1442-9071 1442-6404 |
DOI: | 10.1111/ceo.13958 |
Popis: | Megalocornea and anterior megalophthalmos (megalocornea spectrum) disorders are typically defined by corneal diameter > 12.5 mm in the absence of elevated intraocular pressure. Clinical features overlap with keratoglobus but are distinct from buphthalmos and severe (globus) keratoconus. Megalocornea spectrum disorders and keratoglobus are primarily congenital disorders, often with syndromic associations; both can present with large and thin corneas, creating difficulty in diagnosis, however, only keratoglobus is typically progressive. Molecular genetics provide significant insight into underlying aetiologies. Nonetheless, careful clinical assessment remains intrinsic to diagnosis. Surgical management can be challenging due to the enlarged ciliary ring and weakened zonules in megalocornea spectrum disorders and the extreme corneal thinning of keratoglobus. In this review, the established literature on measurement of corneal diameter, diagnosis of megalocornea, anterior megalophthalmos and keratoglobus, differentiation from severe keratoconus, recent molecular genetics research and key surgical modalities in the management of these rare disorders are outlined and discussed. |
Databáze: | OpenAIRE |
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