Anterior segment dysgenesis: current perspectives on management

Elizabeth Bolton, Brenda L. Bohnsack*

*Corresponding author for this work

Research output: Contribution to journalReview articlepeer-review

Abstract

Introduction: Anterior segment dysgeneses are congenital ocular anomalies that involve the cornea, iris, anterior chamber, iridicorneal angle structures, and ciliary body. Management highly varies and often depends on the extent of cornea and lens involvement and glaucoma diagnosis. A coordinated approach between pediatric ophthalmology, cornea, retina, and glaucoma specialists may be required to minimize complications and optimize results. Areas covered: A review of the clinical findings of primary congenital glaucoma, congenital aniridia, Axenfeld-Rieger syndrome, Peters anomaly, sclerocornea, congenital ectropion uvea, and megalocornea/megalophthalmos will be followed by the current management of these diseases. Expert opinion: For optimal outcomes, these diseases often require a multi-specialty approach incorporating glaucoma, cornea, and retina specialists with pediatric ophthalmologists. However, there is a critical shortage of pediatric ophthalmologists and few adult sub-specialists have an interest and desire to incorporate children into their practices. A greater emphasis on pediatric eye diseases during training and exposure to anterior segment dysgeneses is needed to provide the optimal care for these rare conditions.

Original languageEnglish (US)
Pages (from-to)173-186
Number of pages14
JournalExpert Review of Ophthalmology
Volume19
Issue number3
DOIs
StatePublished - 2024

Keywords

  • Anterior segment dysgenesis
  • Axenfeld-Rieger syndrome
  • Peters anomaly
  • aniridia
  • anterior megalophthalmos
  • congenital ectropion uvea
  • primary congenital glaucoma
  • sclerocornea

ASJC Scopus subject areas

  • Biomedical Engineering
  • Ophthalmology
  • Optometry

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