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

Abstract

Abstract. Sturge-Weber syndrome is a rare neuro-cutaneousdisease presenting as port- wine stain over distribution of trigeminal nerve.There is a wide variability in manifestations of the disease. Associatedmalformations in the form of buphthalmos is a rare occurrence with thisdisorder. We report a 2 months old infant who had bilateral port- wine stainover face and extending over the upper part of chest. There were no seizuresand developmental milestones were normal. The patient had associatedbuphthalmos in the right eye. The intraocular pressure in the right eye wasraised while it was normal in left eye. Cranial CT scan did not reveal anycalcifications. In conclusion, Sturge- Weber syndrome may not presentclinically in its typical form. The presence of buphthalmos is to be lookedespecially when there is extensive bilateral port-wine stains.Key words: Bilateral port-winestains, buphthalmos

Keywords

• Bilateral port-wine stains, buphthalmos

References

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