Abstract
Congenital ocular disorders with a significant potential to develop glaucoma include Axenfeld-Rieger syndrome, Peters anomaly, and aniridia. Other ocular conditions such as microcornea, congenital ectropion uveae, oculodermal melanocytosis, posterior polymorphous dystrophy, congenital iris hypoplasia, and various congenital retinal diseases can also be complicated by glaucoma development. The risk of developing glaucoma in Axenfeld-Rieger syndrome, Peters anomaly, and aniridia is notably high (50-75%) compared to the other condition where this risk is relatively lower (10-15%). Glaucoma secondary to these congenital disorders tends to have a severe clinical course and be more resistant to anti-glaucomatous therapies compared to primary congenital glaucoma. Managing intraocular pressure (IOP) can be challenging after treating concomitant anomalies such as cataract and corneal opacity. Furthermore, IOP-lowering procedures, such as drainage tube implantation, may lead to corneal decompensation and cataract development. Given the complexity of these conditions, a multidisciplinary approach is essential for effective treatment of these diseases. Long-term follow-up is crucial to monitor for the development of glaucoma. While topical anti-glaucoma medications are mostly used as the first-line therapy, many cases ultimately require surgical interventions, such as trabeculectomy and tube implant surgery. Transscleral diode cyclophotocoagulation can be an appropriate treatment option for patients with limited or no visual potential. Often, multiple interventions are necessary to achieve adequate IOP control. In addition to managing IOP, amblyopia rehabilitation is a critical component of the lifelong treatment of these challenging cases. This comprehensive approach ensures that both the ocular and visual development needs of individuals with these challenging conditions are addressed effectively.
| Original language | English (US) |
|---|---|
| Title of host publication | Childhood Glaucoma |
| Subtitle of host publication | Current Trends and Future Prospects |
| Publisher | Springer International Publishing |
| Pages | 53-68 |
| Number of pages | 16 |
| ISBN (Electronic) | 9783031610738 |
| ISBN (Print) | 9783031610721 |
| DOIs | |
| State | Published - Jan 1 2024 |
| Externally published | Yes |
Keywords
- Aniridia
- Axenfeld-Rieger syndrome
- Congenital ectropion uveae
- Congenital glaucoma
- Congenital iris hypoplasia
- Glaucoma associated with retinal disorders
- Microcornea
- Oculodermal melanocytosis
- Peters anomaly
- Posterior polymorphous dystrophy
ASJC Scopus subject areas
- General Medicine