Scott R Michael, Smith Jodi L, Robertson Richard L, Madsen Joseph R, Soriano Sulpicio G, Rockoff Mark A
Department of Neurosurgery, The Children's Hospital, Harvard Medical School, Boston, Massachusetts 02115, USA.
J Neurosurg. 2004 Feb;100(2 Suppl Pediatrics):142-9. doi: 10.3171/ped.2004.100.2.0142.
Moyamoya syndrome, a narrowing of the basal intracranial vessels accompanied by the development of a cloud of collateral "moyamoya" vasculature, causes cerebral ischemia and stroke. This study was undertaken to determine if a standardized neurosurgical revascularization procedure, pial synangiosis, conferred long-term benefit in pediatric patients.
The authors reviewed the clinical and radiographic records obtained in a consecutive series of patients with moyamoya syndrome. Patients were 21 years of age or younger and underwent surgery performed by a single neurosurgeon during a 17-year period. There were 143 patients (89 females and 54 males). Sixteen patients were Asian. Neurofibromatosis was present in 16 patients, 13 had undergone therapeutic cranial irradiation, and Down syndrome was present in 10. In 66 there was no known predisposing condition. Stroke had occurred in 67.8% of the population and transient ischemic attacks (TIAs) in 43.4% prior to surgery. Within the first 30 days following 271 craniotomies for pial synangiosis, there were 11 episodes of stroke (7.7% per patient; 4% per surgically treated hemisphere) and three severe TIAs. Follow-up evaluation was performed in all but one patient (mean period 5.1 years). In 126 patients followed for more than 1 year, four suffered a late-onset stroke, one suffered a severe reversible TIA without magnetic resonance imaging-documented evidence of stroke, and two experienced persistent TIAs. In 46 patients followed for more than 5 years in whom the major initial presentation was stroke alone, only two late-onset strokes have occurred. Functional status at the time of surgery determined long-term functional status.
Following pial synangiosis, the majority of pediatric patients with moyamoya syndrome stop having strokes and TIAs, and they appear to experience an excellent long-term prognosis.
烟雾病综合征是一种颅内基底血管狭窄并伴有侧支“烟雾状”血管形成的疾病，可导致脑缺血和中风。本研究旨在确定标准化的神经外科血管重建手术——软脑膜血管吻合术是否能给小儿患者带来长期益处。
作者回顾了一系列连续的烟雾病综合征患者的临床和影像学记录。患者年龄在21岁及以下，在17年期间由一名神经外科医生进行手术。共有143例患者（89例女性和54例男性）。16例患者为亚洲人。16例患者患有神经纤维瘤病，13例曾接受过治疗性颅脑照射，10例患有唐氏综合征。66例患者无已知的诱发因素。67.8%的患者术前发生过中风，43.4%的患者发生过短暂性脑缺血发作（TIA）。在271次软脑膜血管吻合术开颅术后的前30天内，发生了11次中风（每位患者7.7%；每个手术治疗的半球4%）和3次严重TIA。除1例患者外，对所有患者进行了随访评估（平均随访期5.1年）。在126例随访超过1年的患者中，4例发生迟发性中风，1例发生严重可逆性TIA但磁共振成像未记录到中风证据，2例经历持续性TIA。在46例主要初始表现仅为中风且随访超过5年的患者中，仅发生了2次迟发性中风。手术时的功能状态决定了长期功能状态。
软脑膜血管吻合术后，大多数烟雾病综合征小儿患者停止发生中风和TIA，且长期预后似乎良好。
