Abstract
Sturge-Weber syndrome is a rare neurocutaneous syndrome characterized by port -wine stain, seizures and intracranial calcifications.The present case had bilateral port-wine nevus, generalised tonic-clonic seizures and right sided intracranial calcifications involving right temporo-parieto-occipital lobe regions with prominent choroid plexus.
Highlights
Sturge-Weber syndrome is a rare disorder that occurs with a frequency of 1: 50,000 [1]
It is a sporadic neurocutaneous disease characterized by facial port-wine stain, ocular abnormalities and leptomeningeal angioma most often involving occipital and posterior parietal lobes [2]
Glaucoma develops in up to 42% of patients whose facial cutaneous angioma is adjacent to eye [11]
Summary
Sturge-Weber syndrome is a rare disorder that occurs with a frequency of 1: 50,000 [1] It is a sporadic neurocutaneous disease characterized by facial port-wine stain, ocular abnormalities (glaucoma and choroidal haemangioma) and leptomeningeal angioma most often involving occipital and posterior parietal lobes [2]. This syndrome consists of constellation of symptoms and signs including a facial nevus, seizures, hemiparesis, intracranial calcification and mental retardation [3]. Physical examination revealed a febrile child with pulse rate of 126/minute, respiratory rate of 32/minute and his blood pressure was 76/50 mmHg. There was port-wine stain involving bilateral face involving ophthalmic, maxillary and mandibular division of trigeminal nerve (Figure 1). Based on the presence of seizure, port wine stain over bilateral and calcification, the diagnosis of Sturge Weber syndrome (SWS) was considered
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