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Abstract #10091 Published in IGR 6-1
Primary megalocornea: clinical features for differentiation from infantile glaucoma
Ho CL; Walton DSJournal of Pediatric Ophthalmology & Strabismus 2004; 41: 11-17; quiz 46-47
PURPOSE: To describe the ocular findings in megalocornea to assist in its differentiation from infantile glaucoma in the evaluation of children with abnormally enlarged corneas. METHODS: The clinical findings of four boys found to have megalocornea following referral for evaluation of large corneas and suspected glaucoma were reviewed. RESULTS: Three of the four patients had photophobia. Clear and enlarged corneas were observed associated with deep anterior chambers, posterior bowing of the irides, and normal intraocular pressures (IOPs) in all eyes. Transillumination of the irides was found in 6 of 8 eyes and pigment dispersion was seen in four of eight eyes. Pigment dispersion appeared to be acquired over time, and the youngest patient in this series who had pigment dispersion detected on slit-lamp examination was 15 years; the youngest patient with the condition detected on gonioscopy was eight years. No breaks in Descemet's membrane were present. Family history obtained from three of the four patients revealed evidence of sex-linked recessive inheritance. These findings are distinct from the clinical features of infantile glaucoma characterized by elevated IOP, breaks in Descemet's membrane, corneal edema, a generally flat iris profile, less pronounced enlargement of the anterior segment, the absence of iris transillumination and pigment dispersion, and autosomal recessive inheritance. Three patients had corneal size asymmetry, a finding that has not been previously reported. CONCLUSIONS: Hereditary megalocornea has defining clinical findings that help to identify and differentiate it from other causes of enlarged corneas. Asymmetry in corneal size does not preclude its diagnosis.
Dr. C.L. Ho, Massachusetts Eye and Ear Infirmary, Harvard Medical School, Boston, MA, USA
Classification:
10 Differential diagnosis e.g. anterior and posterior ischemic optic neuropathy
