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.
|Number of pages||4|
|Journal||Folia Ophthalmologica Japonica|
|Publication status||Published - Mar 2005|
- Axenfeld-Rieger Syndrome
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