Multiple bur hole surgery for the treatment of moyamoya disease in children

Object. The authors' aim in this study was to review their experience in the use of indirect revascularization alone in a series of 14 children with moyamoya disease, in which numerous bur holes and arachnoid openings were made over each affected hemisphere. Methods. Revascularization through multiple bur holes and arachnoid openings was performed in 14 children (mean age at diagnosis 6.5 years [range 3-15 years]) who suffered from progressive moyamoya disease. The authors performed surgery in a total of 24 hemispheres during 18 procedures. Ten children underwent bilateral multiple bur hole procedures, three underwent a unilateral procedure in the more severely affected hemisphere, and one child had previously undergone an encephaloduroarteriomyosynangiosis on the contralateral side. Ten to 24 bur holes were made in the frontotemporoparietooccipital area of each hemisphere, depending on the site and extent of the disease. Early postoperative perfusion magnetic resonance imaging studies, performed in the five most recent cases, showed restoration of cortical perfusion as early as 3 months, which was confirmed on subsequent angiography studies (performed between 8 and 12 months postoperatively) that showed excellent revascularization of the ischemic brain by external carotid artery collateral vessels. None of the children sustained further ischemic attacks postoperatively. Motor improvement was noted in those who had presented with paresis. A single seizure episode occurred in two patients at 2 weeks and 5 months after surgery; both children had presented with epilepsy. There were no postoperative deaths, and only one complication (an infected lumbar shunt in the patient who required cerebrospinal fluid [CSF] drainage). Five of the 18 procedures were complicated by subcutaneous CSF collections, which resolved with tapping and compressive head dressings; a transient lumbar drain was necessary in one case. Conclusions. The results obtained in this series suggest that in children with moyamoya disease this simple technique is both effective and safe. Furthermore, it is effective as a sole treatment without supplementary revascularization procedures.