Glaucoma in Children with Facial Port Wine Stain

What is it about?

To report on the clinical presentation and surgical treatment (procedure and outcome(s)) of glaucoma in children with facial portwine stain (PWS), the charts of 22 children (44 eyes) with facial PWS referred to Alexandria University pediatric ophthalmology practice from 2005 to 2016 were reviewed with the aim of stratifying the eyes into glaucoma, glaucoma suspects and no glaucoma at initial and final presentations. The average age of presentation was 18.2(±33.9) months. After a follow up over 16.1 (±24.8) months, there were almost equal thirds ( 34%, 30% and 36%) of the study eyes diagnosed as glaucoma, glaucoma suspects and no glaucoma respectively with mean±SD of pressure inside the eye of 20.6±5.1, 13.6±5.4, 7.5±1.7 mmHg. The majority (91%) of eyes presenting with glaucoma had clear corneas. Eleven eyes were operated upon for glaucoma. Recorded success rate was 91%. Two eyes developed an abnormal accumulation of fluid inside the eye after the surgery (postoperative exudative choroidal detachment), one resolved spontaneously and the other was successfully managed by (intravitreal) gas injection inside the eye. The study demonstrated that glaucoma is a significant ocular hazard in children with facial PWS that may not be evident on initial presentation. The presentation is usually with a clear cornea and surgical intervention is associated with a high success rate and a low rate of complications.

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Why is it important?

Facial portwine stain is a rather common birthmark. Glaucoma is a potentially blinding disease and is relatively silent in such children. This study demonstrates that almost one third of these children develop that potentially blinding disease over time. This study highlights the importance of continued medical supervision of children with facial portwine stain.

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