Fryer Robert H, Anderson Richard C, Chiriboga Claudia A, Feldstein Neil A
Division of Pediatric Neurology, Department of Neurology, College of Physicians and Surgeons, Columbia University, New York, NY, USA.
Pediatr Neurol. 2003 Aug;29(2):124-30. doi: 10.1016/s0887-8994(03)00047-x.
Moyamoya disease is a relatively uncommon neurovascular complication of sickle cell anemia. We report a case series of six patients with sickle cell anemia who developed moyamoya disease and underwent encephaloduroarteriosynangiosis procedures. These six patients presented with either cerebrovascular accidents, transient ischemic attacks, or seizures, and subsequent magnetic resonance imaging scans were suggestive of moyamoya-like changes in the cerebral vasculature. Conventional cerebral angiography was used to confirm the diagnosis in all six patients. Four of six patients manifested a cerebrovascular accident before surgery, and two of these patients were compliant on a transfusion protocol at the time of their cerebrovascular accident. Bilateral (n = 4) or unilateral (n = 2) encephaloduroarteriosynangiosis procedures were performed without any complications. The patient who was stroke-free preoperatively had a cerebrovascular accident 2 weeks after the procedure; otherwise, all patients have remained free of neurovascular complications with an average follow-up of 33 months. Collateral anastomoses between external and internal carotid arteries were established by magnetic resonance angiography in three patients. The encephaloduroarteriosynangiosis procedure is a safe and effective treatment option in patients with sickle cell anemia who develop moyamoya disease.
烟雾病是镰状细胞贫血相对罕见的神经血管并发症。我们报告了一组6例镰状细胞贫血并发烟雾病并接受脑硬膜动脉血管融合术的病例。这6例患者均表现为脑血管意外、短暂性脑缺血发作或癫痫发作，随后的磁共振成像扫描提示脑血管系统有烟雾病样改变。所有6例患者均通过传统脑血管造影确诊。6例患者中有4例在手术前发生过脑血管意外，其中2例在发生脑血管意外时遵循输血方案。进行了双侧（n = 4）或单侧（n = 2）脑硬膜动脉血管融合术，无任何并发症。术前无卒中的患者在术后2周发生了脑血管意外；否则，所有患者在平均33个月的随访中均未出现神经血管并发症。3例患者通过磁共振血管造影显示颈外动脉和颈内动脉之间建立了侧支吻合。对于镰状细胞贫血并发烟雾病的患者，脑硬膜动脉血管融合术是一种安全有效的治疗选择。
