Abstracts

Rationale: Some patients with Sturge-Weber syndrome (SWS) need epilepsy surgery for good seizure control and prevention of psychomotor deterioration. Most frequent type of SWS has leptomeningeal angioma distributed temporal, parietal and occipital lobe. We indicate the posterior quadrant disconnection surgery for those patients. In this paper, we report the surgical procedure of the posterior quadrantectomy and the seizure and developmental outcome in patients with SWS.Methods: We surgically treated 9 patients with SWS whose leptomeningeal angioma located in temporal, parietal and occipital lobe using the posterior quadrantectomy. After the craniotomy, we dissect the sylvian fissure to observe the inferior insular sulcus from the limen insulae to the distal end. Next step is disconnecting the temporal stem to the inferior horn of lateral ventricle. We can see hippocampus from the head to tail at this point. Corticotomy of parietal lobe is made to the trigon of lateral ventricle and the falx. Final step is the posterior corpus callosotomy and the disconnection of the fornix. Using this method, we can reduce blood loss and shorten surgical time. Results: Seven in 9 patients with SWS who were operated using this method resulted seizure free and improved psychomotor development. We carried out the second surgery for the resting 2 patients with residual seizures, and finally resulted seizure free. Early surgery is better to seizure control and psychomotor development.Conclusions: The posterior quadrantectomy for patients with SWS is suitable for good seizure control and psychomotor development. This method can reduce surgical insult in infant.