Abstracts

Rationale: As a rare congenital vascular disease in central nerve system, the main cause for attending of Sturge-Weber syndrome is epileptic seizure onsets and its main surgical treatment is hemispheric or multi-lobus resection. As the main controversial debate is “When is the best time for neurosurgical intervention?” This work aimed to discuss risks and prognosis of early-stage neurosurgical intervention of this disease. Methods: Analyze 23 cases attending to our hospital from Jan. 2014 to Jan. 2016 whom were younger than 5 years old retrospectively. Statistically operation type, surgical time (min), blood loss (ml), corrected blood loss, which is equal with blood loss divide by weight, surgical complication in early-stage and remote stage, pre- and post-operative seizure onset frequency, function of body movement and language in 2 years follow-up were analyzed. Results: The average age of the 23 cases is 15.91±9.96M, in which the youngest is 3M and the eldest is 46M. 13 in this group were boys  and 10 were girls. 2 accepted anatomical hemispherectomy and the others 21 accepted improved hemispherectomy. Average surgical time was 346.69±49.93min, average blood loss was 526.15±153.65ml and corrected blood loss was 49.40±18.05ml/kg. During the surgical procedure in 2 cases whose blood loss is more than 800 ml, occurred serious hypotension. 20 cases accepted continuous lumbar drainage or subdural drainage in 2 weeks postoperatively for hyperpyrexia. Only one case accepted an emergent hematoma aspiration in 24 hours postoperatively. But there was no dead case and hydrocephalus in 2 years follow-up. In all the cases, only two had no seizure-history and all had no seizure onset during the follow-up, in which only 11 cases had interictal epileptiform discharges in remained cerebral regions. Preoperatively in 4 cases there was no obvious abnormality in Denver Developmental Screening Test, 13 suspicious abnormalities mainly in fine motor area and 7 obvious abnormalities mainly in social work and language areas. Postoperatively in 2 years in all cases there was no obvious abnormality in social work and language areas, suspicious abnormalities in rough motor area of contralateral side and obvious abnormalities in fine motor area of contralateral distal extremities. Conclusions: As a rare congenital vascular disease in central nerve system, the main surgical target is termination of seizure onset to improve prognosis. Advantages of early-stage neurosurgical intervention include effectiveness for controlling seizure onset and improvement of social work and language function, while disadvantages include a huge injury and amount of blood loss, which inquire an experienced surgical and anesthetic team. But whether the movement prognosis is better than that of late stage intervention still needs a further study. Funding: No funding