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Signs and Symptoms
Microcornea may present in patients of either gender and at any age; however, since it represents a congenital condition, it is typically detected during childhood. The condition may be unilateral or bilateral. In clinical terms, microcornea is defined as a horizontal corneal diameter of less than 11 mm in an otherwise normal-sized eye.1,2 In some cases, the cornea may be as small as 4 mm.3 Isolated cases of microcornea often present with normal visual acuity, though many more cases may demonstrate oculosystemic complications: for example, Ehlers-Danlos syndrome, Waardenburgs craniofacial syndrome, Norrie syndrome, Turner's syndrome and congenital rubella.4-7 Such cases may present with congenital cataract, glaucoma, colobomas or retinal anomalies that significantly compromise vision. Patients with microcornea are also at greater risk for developing secondary glaucomas, and this risk may increase as the patient ages.8,9
Pathophysiology
Microcornea is believed to result from an arrest in corneal development after the fifth month of fetal growth.10 The condition can be a feature of any number of ocular or oculosystemic syndromes, or it may occur as an isolated defect. Microcornea with cataract has been identified as a unique syndrome, associated with a genetic defect for transcription factor MAF, a protooncogene sometimes associated with multiple myeloma.1,11 More commonly,...