Asia Pacific Journal of Pediatric and Child Health

Abstract Ref Number = APCP1116

Poster Presentation

STURGE-WEBER SYNDROME A Case Report

Fandi Argiansya,Masayu Rita Dewi,RM Indra,Syarif Darwin,Rini Gita Sari Department of Child Health Medical School University of Sriwijaya Dr Mohammad Hoesin Hospital Palembang Indonesia Department of Child Health Medical School University of Sriwijaya Dr Mohammad Hoesin Hospital, PalembangIndonesia

Background : Background: Sturge-Weber syndrome (SWS) is a sporadic vascular disorder with a constellation of signs and symptoms including port-wine stain (PWS), leptomeningeal angioma and glaucoma. The incidence of SWS is 1:50,000 infants, with no significant difference between males and females. Complications include transient ischaemic attacks, infarcts, seizure, aneurysmal subarachnoid haemorrhage and parenchymal bleeds. Agenesis or hypoplasia of the internal carotid artery may also occur. The aim of this case report is to describe the diagnosis and management of Sturge-Weber syndrome. Case Presentation Summary : Case: We reported a case of a three-year-old boy, came to Mohammad Hoesin Hospital with frequent involuntary eye blinks of the right eye. The attacks had been occuring since two weeks before admission, with average frequency five times per day, lasting about one until five minutes each episode. The patient had a history of focal seizures since he was three months old. Reddish skin discoloration (port wine stain) was present on the face, neck, wrist, chest and leg since birth and gradual enlargement was noticed. Physical examination revealed port wine nevi, localized in the complete left half of the face, extending to left half of the neck, chest and back. Neurological examination revealed left side hemiparesis. Electroenchepalogram showed slowing of background activity of right hemisphere and epileptiform discharges from right parietal area. Head MRI revealed right cerebral hemiatrophy with an enhancement on the right parietal lobe. We planned CT angiography examination to evaluate larger vessels. Seizures were controlled with valproic acid 20 mg/BW/daily. Learning Points/Discussion : Discussion: Sturge-Weber syndrome is a vascular disorder that necessitate close monitoring and long term treatment for multiple complications that may arise. High index of suspicion should be present in children with facial PWS, neurological and opththalmic symptoms.

Keywords: sturge-weber syndrome port-wine stain agenesis