Investigative Ophthalmology & Visual Science Cover Image for Volume 61, Issue 7
June 2020
Volume 61, Issue 7
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ARVO Annual Meeting Abstract  |   June 2020
Surgical Management for Glaucoma in Sturge-Weber Syndrome: Experience from a Tertiary Referral Paediatric Hospital
Tanya Karaconji; Eugene Ting; Sophia Zagora; Simone Arden Holmes; Robyn Jamieson; John R Grigg
Author Affiliations & Notes
  • Tanya Karaconji
    Discipline of Ophthalmology, The University of Sydney, Save Sight Institute , Sydney , New South Wales, Australia
    Ophthalmology, The Children's Hospital Westmead, Sydney, New South Wales, Australia
  • Eugene Ting
    Discipline of Ophthalmology, The University of Sydney, Save Sight Institute , Sydney , New South Wales, Australia
  • Sophia Zagora
    Discipline of Ophthalmology, The University of Sydney, Save Sight Institute , Sydney , New South Wales, Australia
  • Simone Arden Holmes
    TY Nelson Department of Neurology and Neurosurgery, The Children’s Hospital at Westmead, The Sydney Children’s Hospitals Network, Australia., Sydney, New South Wales, Australia
  • Robyn Jamieson
    Eye Genetics Research Group Children’s Medical Research Institute, The Children’s Hospital at Westmead and Eye Genetics Clinics, Sydney, New South Wales, Australia
  • John R Grigg
    Discipline of Ophthalmology, The University of Sydney, Save Sight Institute , Sydney , New South Wales, Australia
  • Footnotes
    Commercial Relationships   Tanya Karaconji, None; Eugene Ting, None; Sophia Zagora, None; Simone Arden Holmes, None; Robyn Jamieson, None; John Grigg, None
  • Footnotes
    Support  None
Investigative Ophthalmology & Visual Science June 2020, Vol.61, 977. doi:
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      Tanya Karaconji, Eugene Ting, Sophia Zagora, Simone Arden Holmes, Robyn Jamieson, John R Grigg; Surgical Management for Glaucoma in Sturge-Weber Syndrome: Experience from a Tertiary Referral Paediatric Hospital. Invest. Ophthalmol. Vis. Sci. 2020;61(7):977.

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Abstract

Purpose : The optimal surgical technique for the management of glaucoma in Sturge-Weber Syndrome (SWS) is controversial. The aim of our study was to describe the treatment outcomes in SWS in children presenting to a tertiary referral Paediatric Ophthalmology Department

Methods :
A retrospective study of patients with SWS referred to the Department of Ophthalmology at The Children's Hospital Westmead (CHW) between 2003 to 2016 with at least 2 years follow up were identified and information was collected from the clinical notes of all subjects

Results : 27 patients with SWS were evaluated for glaucoma in which 9 were excluded due to transfer of care. In total 18 patients with SWS were included in this study in which glaucoma was diagnosed in 15 patients and 19 eyes. Glaucoma surgery was required in 13 eyes. A total of 21 surgical procedures were performed with a median follow up of 85 months.

The median age of glaucoma diagnosis was 14 months (IQR 44). Of these, 11 were unilateral (73%) and 4 (26%) had bilateral disease giving a total of 15 patients and 19 eyes affected with glaucoma. Port wine stains (PWS) were ipsilateral to the glaucoma-affected eye in 100% of eyes. Choroidal vascular malformations were present in 8 (50%) of the glaucoma-affected eyes.

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, 4 achieved success (2 complete and 2 qualified) and 2 had failed, one of which required removal of the intraluminal stent. 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 (Figure 1).

Conclusions : Glaucoma affects a significant proportion of patients with SWS and is associated with the presence of an ipsilateral PWS in most cases. In our study, Trabeculectomy was the most efficacious procedure for controlling IOP and reducing the burden of ongoing treatment in SWS associated glaucoma.

This is a 2020 ARVO Annual Meeting abstract.

 

Figure 1: box plots of IOP measurements pre-operatively and at post-operative time points for each surgical procedure.
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Figure 1: box plots of IOP measurements pre-operatively and at post-operative time points for each surgical procedure.

Figure 1: box plots of IOP measurements pre-operatively and at post-operative time points for each surgical procedure.

Figure 1: box plots of IOP measurements pre-operatively and at post-operative time points for each surgical procedure.
View OriginalDownload Slide

This work is licensed under a Creative Commons Attribution-NonCommercial-NoDerivatives 4.0 International License.
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Copyright © 2025 Association for Research in Vision and Ophthalmology.
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