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Surgical Treatment for SWS Glaucoma: Experience From a Tertiary Referral Pediatric Hospital
Tanya Karaconji1,2, Eugene R Ting1, Sophia L Zagora1,2
1Discipline of Ophthalmology, Save Sight Institute.
Insights
Trabeculectomy is an effective surgical treatment for glaucoma in children with Sturge-Weber syndrome (SWS). This study found it successfully controlled intraocular pressure, reducing the need for further interventions in SWS-associated glaucoma.
Area of Science:
- Ophthalmology
- Pediatric Ophthalmology
- Medical Genetics
Background:
- Sturge-Weber syndrome (SWS) is a rare congenital disorder often associated with glaucoma.
- Glaucoma in SWS typically requires surgical management.
- Early diagnosis and intervention are crucial for managing SWS-associated glaucoma.
Purpose of the Study:
- To evaluate the surgical outcomes of glaucoma in children with Sturge-Weber syndrome.
- To determine the efficacy of different surgical procedures in managing SWS-associated glaucoma.
- To provide insights into the long-term management of pediatric glaucoma in SWS patients.
Main Methods:
- Retrospective study of pediatric patients with SWS and glaucoma.
- Data collection from clinical notes between 2003 and 2016.
- Analysis of surgical interventions including trabeculectomy, trabeculotomy, and Baerveldt tube shunt implantation.
Main Results:
- 19 SWS patients with glaucoma were analyzed, with 13 eyes requiring surgery.
- Trabeculectomy was performed in 6 eyes, achieving complete success in 4 and qualified success in 2, with no further surgery needed.
- Other procedures like trabeculotomy and Baerveldt tube shunt implantation were also assessed, with varying success rates.
Conclusions:
- Trabeculectomy is the most effective surgical procedure for controlling intraocular pressure in SWS-associated glaucoma.
- Surgical intervention is essential for managing glaucoma in a significant proportion of SWS patients.
- Effective surgical management can reduce the burden of ongoing treatment for SWS-associated glaucoma.
Precis:
Glaucoma associated with Sturge-Weber syndrome (SWS) often requires surgical intervention. Our study shows that trabeculectomy is efficacious in treating this condition.
Purpose:
The purpose of this study was to describe the surgical outcomes of glaucoma associated with SWS in children presenting to the tertiary Paediatric Ophthalmology Department at The Children's Hospital at Westmead.
Materials And Methods:
A retrospective study of patients with SWS referred to the Department of Ophthalmology at The Children's Hospital at Westmead between 2003 and 2016 with at least 2 years of follow-up were identified, and information was collected from the clinical notes of all subjects.
Results:
A total of 27 patients with SWS were evaluated for glaucoma in which 8 were excluded due to inadequate follow-up. In total, 19 patients with SWS were included in this study in which glaucoma was diagnosed in 15 patients and 19 eyes, of which 13 eyes required glaucoma surgery. A total of 21 surgical procedures were performed with a median follow-up of 85 months. A primary trabeculotomy was performed in 5 eyes of which 4 required re-do trabeculotomy, and 3 of these eyes underwent a Baerveldt tube (BVT) shunt as a third procedure. One eye with a primary trabeculotomy underwent a BVT as a secondary procedure. A BVT was inserted in a total of 6 eyes in which it was a primary procedure in 2 eyes. Of the 6 eyes undergoing a BVT insertion, 5 achieved success (2 complete and 3 qualified), and 1 failed. One case underwent intraluminal stent removal. Six eyes underwent a primary trabeculectomy and needed no further surgical intervention. In the trabeculectomy group, 4 eyes achieved complete success and 2 eyes achieved qualified success.
Conclusions:
Glaucoma affects a significant proportion of patients with SWS and is associated with the presence of an ipsilateral port-wine stain in most cases. In our study, trabeculectomy was the most efficacious procedure for controlling intraocular pressure and reducing the burden of ongoing treatment in SWS-associated glaucoma.
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