A case of Axenfeld-Rieger syndrome and microcornea treated with trabeculotomy

Shino Sato, Tetsuya Baba, Kazuyuki Hirooka, Hirokazu Takenaka, Fumio Shiraga, Haruko Asano

Research output: Contribution to journalArticlepeer-review

Abstract

Background: Several surgeons have reported that trabeculotomy is a successful procedure in cases of glaucoma associated with Axenfeld-Rieger (A-R) syndrome. However, the usefulness of procedures for treating severe goniodysgenesis or other ocular malformations have not been established. We report on a case in which glaucoma in a patient with A-R syndrome with severe microcornea and severe goniodysgenesis was successfully treated with trabeculotomy. Case Report: The patient was a 16-year-old girl with posterior embryotoxon and high insertion of the iris in the right eye. The iris was attached to the Schwalbe line from 3 o'clock to 12 o'clock, and trabecular meshwork was visible only from 12 o'clock to 3 o'clock. She had severe corneal opacity in the left eye and microcornea in both eyes. We diagnosed A-R syndrome based on the presence of a flattened midface and dental hypoplasia. Trabeculotomy was performed from 12 o'clock to 3 o'clock of the angle in the right eye, where goniodysgenesis was mild. Intraocular pressure has been well-controlled over the 7 months since surgery. Conclusion: Trabeculotomy to an area where the angle was open seems to have been effective in this case of A-R syndrome with severe goniodysgenesis.

Original languageEnglish
Pages (from-to)189-192
Number of pages4
JournalFolia Ophthalmologica Japonica
Volume56
Issue number3
Publication statusPublished - Mar 2005

Keywords

  • Axenfeld-Rieger Syndrome
  • Glaucoma
  • Goniodysgenesis
  • Microcornea
  • Trabeculotomy

ASJC Scopus subject areas

  • Ophthalmology

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