Abstracts

Rationale: Sturge-Weber syndrome (SWS) is a neurocutaneous syndrome characterized by facial port wine nevus and leptomeningeal angiomatosis. It is commonly complicated by intractable epilepsy and mental retardation. In spite of broadly affected angiomatosis, spontaneous intracerebral bleeding have never been reported in childhood SWS. In this paper, we report our experience of a patient with SWS who bled in the right thalamus. Methods: We report a girl with SWS who had two epileptic seizures and appropriate development before intracerebral bleeding. At 3-years-old of age, she suddenly developed impairment of consciousness and left hemiplegia. Computed tomography showed intraventricular hemorrhage and acute hydrocephalus. We carried out emergency ventricular drainage, however she had rebleeding in the right thalamus. After continuous ventricular drainage, she gradually recovered consciousness, and rehabilitation induced motor power improvement. Magnetic resonance digital subtraction angiography which performed on day 24 showed poor development of superficial venous drainages, occluded superior sagittal sinus (SSS), and irregular shaped straight sinus. After clearing the intraventricular hematoma, we carried out ventriculoperitoneal shunting with fenestration of the septum pellucidum. Conventional angiography which performed on day 70 showed also poor drainage of the superficial cortical venous system, complete occlusion of the SSS, and prolonged perfusion time. Two years later, she experienced several seizures originated from temporal lobe. Magnetic resonance imaging showed remarkable atrophy in the right temporal lobe. Her psychomotor development also gradually deteriorated. Consequently, we performed the posterior quadrantectomy for her seizure control and maintaining her mental development. Results: We supposed that the hemorrhage may have been caused by venous congestion that occurred in the deep venous system. Additional compromise of the outflow was probably related to the bleedings. We speculate that functional impairment and obtaining epileptogenesis in SWS is due to ischemia mismatched the functional demand. Epileptic seizures also require high cerebral blood flow, therefore induce relative ischemia in SWS with congestive venous drainage, and consequently vicious cycle is formed. Conclusions: The progressive venous restriction leads to bleeding in the congested area and impairs cerebral perfusion with consequent epilepsy deterioration. Surgery can be a promissing way to control this deterioration.