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DM Blackmon, HE Grossniklaus, CD Song; Congenital Corneal Perforation with Secondary Congenital Aphakia in Peters' Anomaly . Invest. Ophthalmol. Vis. Sci. 2002;43(13):1727.
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Purpose: To describe two cases of bilateral Peters' anomaly with congenital unilateral corneal perforation and secondary congenital aphakia. Methods: Retrospective case review of two patients who presented with bilateral corneal opacities. Both patients underwent ultrasonagraphy, penetrating keratoplasty, and histopathologic review of host corneas. Results: Two full-term infants born via spontaneous vaginal delivery presented with dense bilateral central corneal leukomas with ectasia consistent with Peters' anomaly. In addition, Patient 1 had a monocular corneal perforation and Patient 2 had a monocular bulla suspicious for perforation. There is no history of amniocentesis, forceps, or trauma. Systemic evaluations are unremarkable. Ultrasound of Patient 1 revealed aphakia and choroidal detachments. Ultrasound of patient 2 revealed a posterior lens capsule but no definite lens. Both patients underwent penetrating keratoplasty. Intraoperative examination of each patient revealed a perforated cornea and absence of formed lens. Histopathology of both host corneas demonstrated defects in the posterior stroma and Descemet's membrane and no endothelial cells. Both corneas were perforated with adherent posterior capsule and lens remnants. Conclusion: Congenital corneal perforation with secondary congenital aphakia is a rare complication of Peters' Anomaly. To our knowledge these are the only reported cases of Peters' anomaly associated with both corneal perforation and secondary congenital aphakia.
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