Hida K, Iwasaki Y, Koyanagi I, Abe H
Department of Neurosurgery, University of Hokkaido, School of Medicine, Sapporo, Japan.
Surg Neurol. 1999 Apr;51(4):383-90; discussion 390-1. doi: 10.1016/s0090-3019(98)00088-3.
Most reports regarding pediatric syringomyelia have focused not only on Chiari malformation, but also on spinal dysraphism. However, the clinical characteristics of syringomyelia with spinal dysraphism are quite different from those of syringomyelia due to Chiari Type I malformation. The objectives of this study were to identify clinical characteristics of pediatric syringomyelia and to determine whether surgery prevents or corrects the scoliosis associated with syringomyelia.
We reviewed the records of 16 pediatric patients with syringomyelia and Chiari Type I malformation. The patients' ages ranged from 3 to 15 years, with mean age of 9.8 years. None of the patients had spinal dysraphism.
Nystagmus was observed in 2 of the 16 patients, motor weakness in 8 patients, sensory disturbance in 10 patients, and scoliosis in 13 patients. As the initial surgical procedure, foramen magnum decompression (FMD) was performed in seven patients and syringo-subarachnoid (S-S) shunting in nine patients. The motor function improved in 7 of the 8 affected patients, and the sensory disturbance improved in 9 of the 10 affected patients. The magnetic resonance images obtained after the surgery revealed marked decrease of the syrinx size in all patients. Of the 13 patients with scoliosis, 5 showed improvement, 5 stabilization, and 3 deterioration.
Compared with adolescent and adult syringomyelia, pediatric syringomyelia shows a much lower incidence of sensory disturbance and pain, but quite a high incidence of scoliosis. Surgery is effective in improving or stabilizing scoliosis in these patients.
大多数关于小儿脊髓空洞症的报道不仅关注Chiari畸形，还关注脊髓发育异常。然而，伴有脊髓发育异常的脊髓空洞症的临床特征与I型Chiari畸形所致的脊髓空洞症有很大不同。本研究的目的是确定小儿脊髓空洞症的临床特征，并确定手术是否能预防或纠正与脊髓空洞症相关的脊柱侧弯。
我们回顾了16例患有脊髓空洞症和I型Chiari畸形的小儿患者的病历。患者年龄在3至15岁之间，平均年龄为9.8岁。所有患者均无脊髓发育异常。
16例患者中有2例出现眼球震颤，8例出现运动无力，10例出现感觉障碍，13例出现脊柱侧弯。作为初始手术方法，7例患者进行了枕骨大孔减压术（FMD），9例患者进行了脊髓蛛网膜下腔（S-S）分流术。8例受影响患者中有7例运动功能改善，10例受影响患者中有9例感觉障碍改善。术后获得的磁共振图像显示所有患者的脊髓空洞大小均明显减小。13例脊柱侧弯患者中，5例改善，5例稳定，3例恶化。
与青少年和成人脊髓空洞症相比，小儿脊髓空洞症感觉障碍和疼痛的发生率要低得多，但脊柱侧弯的发生率相当高。手术对改善或稳定这些患者的脊柱侧弯有效。
