Sturge-Weber syndrome with involvement of chest dermatomes and unilateral buphthalmos: A rare association

Sturge-Weber syndrome is a rare neuro-cutaneous disease presenting as port- wine stain over distribution of trigeminal nerve. There is a wide variability in manifestations of the disease. Associated malformations in the form of buphthalmos is a rare occurrence with this disorder. We report a 2 months old infant who had bilateral port- wine stain over face and extending over the upper part of chest. There were no seizures and developmental milestones were normal. The patient had associated buphthalmos in the right eye. The intraocular pressure in the right eye was raised while it was normal in left eye. Cranial CT scan did not reveal any calcifications. In conclusion, Sturge- Weber syndrome may not present clinically in its typical form. The presence of buphthalmos is to be looked especially when there is extensive bilateral port-wine stains.