Thick keratoconic cornea associated with posterior polymorphous corneal dystrophy. 2017

K Zaarour, and E Slim, and J Antoun, and N Waked
Ophthalmology department, Hôtel-Dieu de France, Saint-Joseph University (USJ), faculty of medicine, Alfred Naccache Street, Achrafieh, Beirut, Lebanon.

BACKGROUND We herein report a case of bilateral unusually thick non-edematous keratoconic corneas with associated endothelial features of posterior polymorphous corneal dystrophy (PPCD). METHODS We report the case of a 27-year-old myopic woman who presented for refractive surgery. Slit lamp exam showed bilateral corneal protrusion with diffuse deep stromal and endothelial vesicular opacities and small paracentral bands. Topography showed generalized advanced corneal steepening in both eyes with increased anterior and posterior central corneal elevations in comparison to the best fit sphere. Ultrasound pachymetry showed central corneal thickness of 605μm (RE) and 612μm (LE). On specular biomicroscopy, cell density of 2503 cells/mm2 RE and 1526 cells/mm2 LE with significant cellular pleomorphism and polymegathism were noted. CONCLUSIONS Clinical and paraclinical findings together suggest the presence of simultaneous keratoconus and PPCD. The literature has suggested an association between PPCD and steep cornea. Moreover, many reports have also described cases of associated PPCD and keratoconus with characteristic thinning and ectasia, in comparison to the unusual thick corneas noted in our patient, despite the absence of edema. Identification of genetics factors is further needed to clarify this association. CONCLUSIONS This case describes a patient whose corneas present features of both keratoconus and PPCD and is unique due to the presence of increased corneal thickness despite the absence of edema.

UI MeSH Term Description Entries
D007640 Keratoconus A noninflammatory, usually bilateral protrusion and thinning of the CORNEA, the apex being displaced downward and nasally. It occurs most commonly in females at about puberty. Two closely related noninflammatory corneal ectasias are pellucid marginal degeneration and keratoglobus. Keratoglobus,Pellucid Marginal Corneal Degeneration,Pellucid Marginal Degeneration,Degeneration, Pellucid Marginal,Marginal Degeneration, Pellucid,Pellucid Marginal Degenerations
D003315 Cornea The transparent anterior portion of the fibrous coat of the eye consisting of five layers: stratified squamous CORNEAL EPITHELIUM; BOWMAN MEMBRANE; CORNEAL STROMA; DESCEMET MEMBRANE; and mesenchymal CORNEAL ENDOTHELIUM. It serves as the first refracting medium of the eye. It is structurally continuous with the SCLERA, avascular, receiving its nourishment by permeation through spaces between the lamellae, and is innervated by the ophthalmic division of the TRIGEMINAL NERVE via the ciliary nerves and those of the surrounding conjunctiva which together form plexuses. (Cline et al., Dictionary of Visual Science, 4th ed) Corneas
D003317 Corneal Dystrophies, Hereditary Bilateral hereditary disorders of the cornea, usually autosomal dominant, which may be present at birth but more frequently develop during adolescence and progress slowly throughout life. Central macular dystrophy is transmitted as an autosomal recessive defect. Corneal Dystrophies,Granular Dystrophy, Corneal,Groenouw's Dystrophies,Macular Dystrophy, Corneal,Stromal Dystrophies, Corneal,Corneal Dystrophy,Corneal Dystrophy, Hereditary,Corneal Granular Dystrophies,Corneal Granular Dystrophy,Corneal Macular Dystrophies,Corneal Macular Dystrophy,Corneal Stromal Dystrophies,Corneal Stromal Dystrophy,Dystrophy, Corneal,Dystrophy, Corneal Granular,Dystrophy, Corneal Macular,Dystrophy, Corneal Stromal,Dystrophy, Hereditary Corneal,Groenouw Dystrophies,Groenouws Dystrophies,Hereditary Corneal Dystrophies,Hereditary Corneal Dystrophy,Stromal Dystrophy, Corneal
D005260 Female Females
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D000328 Adult A person having attained full growth or maturity. Adults are of 19 through 44 years of age. For a person between 19 and 24 years of age, YOUNG ADULT is available. Adults
D063171 Corneal Pachymetry Measurement of the thickness of the CORNEA. Corneal Pachymetric Measurement,Corneal Thickness Measurement,Corneal Pachymetric Measurements,Corneal Thickness Measurements,Measurement, Corneal Pachymetric,Measurement, Corneal Thickness,Measurements, Corneal Pachymetric,Measurements, Corneal Thickness,Pachymetric Measurement, Corneal,Pachymetric Measurements, Corneal,Pachymetry, Corneal,Thickness Measurement, Corneal,Thickness Measurements, Corneal

Related Publications

K Zaarour, and E Slim, and J Antoun, and N Waked
January 1999, American journal of ophthalmology,
K Zaarour, and E Slim, and J Antoun, and N Waked
January 1985, Cornea,
K Zaarour, and E Slim, and J Antoun, and N Waked
October 2011, Cornea,
K Zaarour, and E Slim, and J Antoun, and N Waked
March 1968, The British journal of ophthalmology,
K Zaarour, and E Slim, and J Antoun, and N Waked
November 2015, Optometry and vision science : official publication of the American Academy of Optometry,
K Zaarour, and E Slim, and J Antoun, and N Waked
October 2013, Journal of pediatric ophthalmology and strabismus,
K Zaarour, and E Slim, and J Antoun, and N Waked
January 2003, Klinika oczna,
K Zaarour, and E Slim, and J Antoun, and N Waked
January 1982, Birth defects original article series,
K Zaarour, and E Slim, and J Antoun, and N Waked
January 2009, Klinika oczna,
K Zaarour, and E Slim, and J Antoun, and N Waked
November 1960, Bollettino d'oculistica,
Copied contents to your clipboard!