BILATERAL STURGE-WEBER SYNDROME - A CASE REPORT AND REVIEW OF LITERATURE

M. Gowri, S. Amudhadevi, V. Anurekha, K. S. Kumaravel*, N. Sundareswaran and E. Vidhya

ABSTRACT

A nine years old female child, the second born of a non-consanguineous marriage had multiple episodes of seizures from two years of age. Antenatal history was uneventful. The baby was delivered by Caesarean section, an indication for which was prematurity and fetal distress. The baby was admitted to the Neonatal unit for 15 days for preterm care. The baby did not have any other significant neonatal history. Family history was not significant. Delayed attainment of all the milestones was present. On examination, the child had multiple capillary haemangiomas over the bilateral upper half of the face (Fig: 1). There were no other congenital anomalies noted. The child had an intellectual disability and presented with refractory seizures. Neurological examination revealed increased tone in all four limbs and reflexes were exaggerated. CT brain showed extensive bilateral calcifications over the whole left cerebral hemisphere and right frontal lobe (Fig: 2), features of which were suggestive of Sturge Weber Syndrome. Ophthalmological examination was normal.

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