Encephalotrigeminal Hemangiomatosis: Two Case Reports
Journal Title: Journal of Medical Science And clinical Research - Year 2014, Vol 2, Issue 10
Abstract
Encephalotrigeminal Hemangiomatosis also known as sturge- weber syndrome is specifically congenital, non-hereditary, rare condition occurring with a frequency of 1:50,000 live births, with equal predilection for either sex. This syndrome consists of a constellation of clinical features like cutaneous angiomatosis (Port-wine stain), glaucoma and variable neurological manifestation including seizures with or without mental retardation. Two male patients aged 17 and 29 year old reported to our department at different time interval with a complaint of swelling in the upper lip since 4 and 2 years respectively. Similar features of reddish discoloration noted in right side of face since childhood was reported by both the patients; there was no history of trauma or pain. However there was positive history of seizure in one of the patient. This paper reports two cases of this syndrome in detail with classic oral manifestations, radiological features and management. Conclusion:- Dentists play key role in identifying oral manifestations and aid in early recognition of this syndrome and prevent cranial complications thereby improving the prognosis.
Authors and Affiliations
Dr. Muthu Kumar. S
Keywords
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