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Peters' anomaly is not an isolated anterior segment abnormality, but occurs as a diverse, phenotypically heterogeneous condition associated with multiple underlying ocular and systemic defects. Surgical treatment of Peters' anomaly is a complex and challenging undertaking. The broad spectrum of disease severity, the lack of uniformity in clinical features, the differences and overlap of preoperative risk factors, and the variability in the numbers and types of intraocular procedures that are performed on individual eyes, as well as postoperative complications all contribute to the difficulties involved in surgical management. This article discloses useful information pertaining to keratoplasty for Peters' anomaly. The study by Yang et al showed that long-term graft clarity could be achieved in 36% of eyes. However, 93% of all clear grafts were first grafts, whereas only 7% were second grafts. All third or subsequent grafts failed. Other important findings were: the significantly greater chance of maintaining a clear graft with initial grafts, compared with subsequent grafts; the disclosure of periods of differential risk for graft failure; and the identification of risk factors for graft failure. Surgical intervention involving one or more procedures is effective in controlling IOP in 32% of eyes with associated congenital glaucoma. Still, multiple procedures and adjunctive medical therapy are often required to achieve and maintain adequate IOP control. The visual outcome is guarded in children with Peters' anomaly. Achieving a satisfactory visual outcome and preventing further visual loss is impeded by the presence of congenital anterior and posterior segment anomalies, structural defects of the CNS, cognitive dysfunction and amblyopia, as well as postoperative complications such as graft failure, cataract, inoperable retinal detachment, and phthisis.
Dr L.L. Yang, Department of Ophthalmology, Emory University, Atlanta, GA, USA
9.1.3 Syndromes of Axenfeld, Rieger, Peters, aniridia (Part of: 9 Clinical forms of glaucomas > 9.1 Developmental glaucomas)