Sturge Weber Syndrome
An infant presented to clinic as a referral due to a cloudy right cornea and port wine birthmark. The parents did not notice any abnormal behavior from the infant. The patient’s intraocular pressure was 28 and 14, but limited exam was possible in the office setting. An exam under anesthesia was performed which revealed diffuse corneal edema, elevated intraocular pressure, and optic nerve cupping on the right side. The left side was normal. Trabeculectomy and stage 1 placement of Baerveldt drainage device was performed. The patient was then monitored over the next 5 years with serial exams under anesthesia and in-office examinations with stable appearance. The patient’s right eye was stable until about 5 years after the first procedure when increased axial length and increased intraocular pressure was noted. A stage 2 placement of the Baerveldt tube was performed with prophylactic sclerotomies. On post op day 1, the patient was noted to have a large inferior exudative retinal detachment. Intravitreal propranolol was administered. By post op day 30, the exudative retinal detachment was resolved. The patient is now stable with controlled intraocular pressure.
Presentation Date: 07/25/2024
Issue Date: 08/09/2024
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