A neonate presents with a port-wine stain involving the left side of the face in the V1 distribution, seizures on day 2 of life, and a history of gestational diabetes. Which ocular complication is most strongly associated with this condition?

• A Cataracts
• B Retinal detachment
• C Glaucoma ✓
• D Optic nerve hypoplasia

Explanation

This describes Sturge-Weber syndrome (encephalotrigeminal angiomatosis) with facial port-wine stain in the trigeminal V1 distribution. Glaucoma is the most common and serious ocular complication, occurring in up to 50% of patients, particularly when the upper eyelid is involved. Seizures result from leptomeningeal angiomatosis. Cataracts are not characteristic. Retinal detachment and optic nerve hypoplasia are less associated.

Reference: Nelson Textbook of Pediatrics, 21st ed.

High-yield for: NEET PGINI-CETNExTFMGEUSMLEPLABMRCP

Written and medically reviewed by the StethoPrep medical team.