NBEO Systemic Disease Practice Exam

Sturge-Weber syndrome is a neurocutaneous disorder characterized by a facial port-wine stain, which typically follows the distribution of the trigeminal nerve, and is associated with neurological and ocular abnormalities. This syndrome can lead to complications such as seizures, developmental delays, and glaucoma.

The association of Sturge-Weber syndrome with a port-wine stain is well established, as it is often one of the hallmark features of the condition. Unilateral glaucoma is also commonly observed in individuals with this syndrome due to the increased pressure resulting from the involvement of the eye structures.

Iris heterochromia, while not as commonly referenced in the context of Sturge-Weber syndrome, can occur but is not the primary clinical concern associated with the syndrome. It may be linked to other conditions or mechanisms but is not one of the classic findings.

Astrocytic hamartoma, on the other hand, is typically associated with another condition known as tuberous sclerosis, not Sturge-Weber syndrome. Therefore, it does not fit the clinical picture of Sturge-Weber syndrome, making it the correct answer in this context. The presence of astrocytic hamartomas is a key feature of tuberous sclerosis, and their absence in Sturge