In addition to today’s resource please review:
- Sturge–Weber syndrome from DermNet, January, 2020.
Today I reviewed and post on the PedsCases podcast, Sturge Weber Syndrome, by Miles Jaques, Jan 21, 2023.
All that follows is from the above outstanding resource.
This podcast on the pathophysiology, clinical presentation and ocular manifestations of Sturge Weber syndrome was developed by Miles Jaques, a fourth-year medical student at the University of British Columbia with Dr. Jennifer Ling, an ophthalmology resident at the University of British Columbia, and Dr. Gardiner, a pediatric ophthalmologist at the University of British Columbia in Vancouver, BC.
Summary
That’s everything we have for you today. Hopefully, you now feel equipped to diagnose a patient presenting for ocular evaluation in the setting of Sturge Weber syndrome, and
understand the fundamentals of management for the potential sequelae of this disease.As a quick recap, Sturge Weber syndrome is a genetic disease of spontaneous origin that causes excess small vascular plexus formations which can manifest in the brain, eyes, and skin.
In the eyes, it most commonly presents as increased intraocular
pressure and can lead to glaucoma. It can also result in choroidal hemangiomas.Indeed, its most classical presentation is a triad of facial port-wine stain, intracranial angiomata, and glaucoma.
Ocular management of the disease is specific to thephenotype of presentation, and includes medical management or surgery for glaucoma,or photocoagulation, photodynamic therapy, or radiation therapy for choroidal hemangiomas.
As well, it is important to adopt a multidisciplinary approach when managing these patients. While their care is usually coordinated by their primary care provider or pediatrician, ophthalmology, dermatology and neurology are often involved.
Thank you for listening today, and we look forward to seeing you again for another episode.
Related content:
- Podcast: Approach to Childhood Glaucoma
