Encephaloduromyosynangiosis (EDMS) on a Pediatric Patient with Cerebral Proliferative Angiopathy

Cerebral proliferative angiopathy (CPA) is a rare cerebral vascular lesion characterized by diffuse vascular abnormalities with intermingled normal brain parenchyma. Cerebral proliferative angiopathy has a complex clinical presentation, and many patients present with ischemic symptoms. We described a case of a 6 year old boy presenting with transient dizziness with weakness of left limbs. MRI showed the presence of a diffusely dilated extensive vascular lesion mainly located in the corpus callosum, right frontal and temporal lobes. Digital subtraction angiography (DSA) revealed a large hypervascular lesion with a diffuse arterial supply. 99mTc-ECD SPECT cerebral blood flow perfusion image revealed decreased perfusion in right frontal and parietal lobe. A surgical procedure of encephaloduromyosynangiosis (EDMS) was performed on the patient, and the patient exhibited disappearance of TIAs during the follow-up period. There is no consensus on treatment of cerebral proliferative angiopathy, but the hemodynamic characteristics of CPA were more similar to moyamoya disease in the PWI-MR study. Furthermore, we speculated that increased regional cerebral blood flow perfusion may relieve clinical symptoms and improve prognosis of patient with CPA. Therefore, we performed encephaloduromyosynangiosis (EDMS) on this pediatric CPA patient with ischemic symptoms. 99mTc-ECD SPECT obtained 6 months after operation showed significant improvement of right frontal and parietal blood perfusion. MR perfusion at follow-up period demonstrated the range and blood flow of vascular lesion decreased significantly compared with preoperative MR perfusion. Follow-up angiography studies showed well compensation by profound neovascularization through the superficial temporal artery, and vascular malformation disappeared around the region supplied from the ECA. EDMS procedure may be a reasonable and effective treatment for patients with ischemia associated with CPA.