Bilateral Sturge-Weber syndrome with soft tissue hypertrophy and trichomegaly.

Abstract

Sturge-Weber syndrome (SWS), also known as encephalotrigeminal angiomatosis, is a rare congenital neurocutaneous syndrome characterised by a triad of cutaneous, ocular and central nervous system findings. In the majority of the patients, it manifests as unilateral facial port-wine stain (PWS), which is a capillary malformation frequently affecting the first branch (ophthalmic division) of the trigeminal nerve. The ocular manifestations involve glaucoma and choroidal haemangiomas, whereas intracranial leptomeningeal venous angiomas form part of the central nervous system involvement. There are a few reports of bilateral PWS involving the trigeminal nerve's ophthalmic and maxillary branches. We hereby report a case of bilateral SWS associated with soft tissue hypertrophy of the lip and trichomegaly.