Spontaneous corneal hydrops and perforation in keratoconus and pellucid marginal degeneration

PURPOSE: To report two cases of pellucid marginal degeneration and one case of keratoconus associated with spontaneous corneal hydrops leading either to perforation or imminent perforation, requiring urgent keratoplasty. METHOD: Retrospective interventional case series of three patients with noninflammatory peripheral corneal degenerations. A retrospective review was done of the clinical courses, surgical interventions, and pathologic specimens, development of spontaneous hydrops, perforation, need for surgical intervention, and final visual outcome. RESULTS: Two patients with pellucid marginal degeneration and one with keratoconus developed spontaneous hydrops followed by aqueous leakage through markedly thinned anterior stroma. In one case, the leak site was successfully sealed after three separate applications of tissue adhesive, although the remaining two cases required penetrating keratoplasty. CONCLUSIONS: These cases document the very unusual occurrence of corneal hydrops leading to spontaneous corneal perforation in patients with keratoconus and pellucid marginal degeneration.