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AJ Aldave, M Mabon, DA Hollander, SD McLeod, W Spencer, RL Abbott; Spontaneous Corneal Hydrops and Perforation in Keratoconus and Pellucid Marginal Degeneration . Invest. Ophthalmol. Vis. Sci. 2002;43(13):3232.
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Purpose: To report two cases of pellucid marginal degeneration and one case of keratoconus associated with rare clinical courses, including spontaneous corneal hydrops and perforation, spontaneous perforation in the absence of hydrops, and spontaneous hydrops and imminent perforation requiring urgent keratoplasty. Methods: A retrospective review of clinical course, including surgical intervention, and pathologic specimens. Results: One patient with keratoconus underwent urgent penetrating keratoplasty for spontaneous central corneal perforation in the absence of hydrops. One patient with pellucid marginal degeneration required three separate administrations of tissue adhesive for spontaneous hydrops complicated by corneal perforation. A second patient with pellucid marginal degeneration developed acute corneal hydrops with a panstromal defect over the Descemet's rupture, leaving only epithelium and Bowman's layer intact. An urgent penetrating keratoplasty was performed for imminent perforation. Conclusion: These cases document the very rare occurrence of spontaneous corneal hydrops and perforation in patients with keratoconus and pellucid marginal degeneration, suggesting that the biomechanical integrity of these ectatic corneas was reduced to the level of spontaneous, atraumatic perforation.
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