Brittle Cornea Syndrome (Brittle Cornea, Blue Sclera and Red Hair Syndrome; Blue Sclera Syndrome) 174
General: Autosomal recessive; rare.
Ocular: Spontaneous perforation of cornea (brittle cornea); blue sclera; acute hydrops; microcor 454h72e nea; sclerocornea; cornea a; keratoconus; keratoglobus.
Clinical: Red hair; associated with Ehlers-Danlos syndrome, osteogenesis imperfecta, and Marfan syndrome.
Cameron JA, et al. Epikeratoplasty for keratoglobus associated with blue sclera. Ophthalmology 1991; 98:446-452.
Cameron JA. Corneal abnormalities in Ehlers-Danlos syndrome type VI. Cornea 1993; 12:54-59.
Mehrotra AN, Bhatia RP. Syndrome of blue sclera, fragilitas ossium and otosclerosis. Ind J Ophthalmol 1974; 22: 42-44.
Osama O, et al. Brittle cornea syndrome. Presented at the American Academy of Ophthalmology, Atlanta, Georgia, 1995: 128(abst).
Paul AK, et al. Blue sclera with fragilitas ossium. Ind J Ophthalmol 1974; 22:38-39.
Stein E, et al. Brittle cornea. Am J Ophthalmol 1968; 66:67-69.
Ticho U, et al. Brittle cornea, blue sclera and red hair syndrome. Br J Ophthalmol 1980; 64:175-l77.
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