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[Occurrence of glaucoma in phakomatosis]
B Koraszewska-Matuszewska1, E Samochowiec-Donocik, E Pieczara
1Kliniku Okulistyki Dzieciecej I Katedry Okulistyki Slaskiej AM w Katowicach.
Insights
Phakomatosis, including Sturge-Weber-Krabbe's syndrome and Recklinghausen's disease, can cause glaucoma in children. Surgical interventions effectively normalized intraocular pressure in 88.9% of cases treated.
Area of Science:
- Ophthalmology
- Genetics
- Pediatrics
Context:
- Phakomatoses are a group of neurocutaneous disorders.
- Glaucoma is a significant complication in certain phakomatoses.
- This study reviews 8 pediatric cases over 10 years.
Purpose:
- To analyze the occurrence of glaucoma in pediatric phakomatosis.
- To evaluate the efficacy of various surgical treatments for glaucoma in these conditions.
- To report outcomes in Sturge-Weber-Krabbe's syndrome and Recklinghausen's disease.
Summary:
- Eight pediatric cases of phakomatosis with glaucoma were treated.
- Six cases involved Sturge-Weber-Krabbe's syndrome, and two involved Recklinghausen's disease.
- Surgical methods included trabeculectomy, cyclo-cryo-application, and cyclo-diathermy, achieving 88.9% intraocular pressure normalization.
Impact:
- Highlights the importance of ophthalmic monitoring in pediatric phakomatosis.
- Demonstrates the effectiveness of surgical management for glaucoma in these rare conditions.
- Provides valuable data for clinicians managing pediatric glaucoma associated with neurocutaneous disorders.
Abstract:
Our clinical material of the last 10 years comprised 8 cases of phakomatosis with glaucoma: 6 children with Sturge-Weber-Krabbe's syndrome and 2 with Recklinghausen's disease. The age of the children was 15 months to 16 years. In 5 children with Sturge-Weber-Krabbe's syndrome glaucoma was found in one eye, in all cases with Recklinghausen disease it was bilateral. The following surgical methods of treatment were applied: trabeculectomy, trabeculectomy with cataract aspiration, cyclo-cryo-application and perforative cyclo-diathermy. The normalisation of the intraocular pressure was achieved in 88.9% of cases.