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목적 : To describe clinical features in children detected with vesicle- or band-like endothelial lesions characteristic of posterior polymorphous corneal dystrophy (PPCD) in their first or second decade of life.
방법 : Seven unrelated Korean pediatric patients who were diagnosed by the presence of characteristic vesicular or band lesions at the level of Descemet’s membrane were included. Thorough ocular examinations were performed, including best-corrected visual acuity, intraocular pressure, and refractive measurements, slit-lamp biomicroscopy, and specular microscopy.
결과 : Slit-lamp examinations revealed vesicular lesions in a 3-year-old boy and horizontally parallel band-like endothelial lesions in 6 patients. Four patients displayed unilateral corneal involvement, yielding 10 eyes with characteristic deep corneal lesions. Specular microscopic examinations revealed a reduced endothelial cell density (ECD, 1662.8±601.1 cells/mm2) composed of enlarged cell (average cell area, 637.8±172.9 μm2/cell) with no significant changes in the hexagonality (HA, 63.2±12.6 %) and coefficient of variation (CV, 29.6±5.5) in the PPCD-affected eyes.
결론 : Children with early-onset PPCD can gain good visual function by early appropriate refractive correction with occlusion therapy. Long-term monitoring of corneal endothelium should be required in pediatric patients with early-onset PPCD.
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