Métellus Philippe, Dufour Henry, Levrier Olivier, Grisoli François
Department of Neurosurgery, Timone Hospital, Marseille, France.
Neurosurgery. 2002 Aug;51(2):500-3; discussion 503-4.
A Chiari I malformation associated with syringomyelia and hydrocephalus is a rare condition. We report the successful use of endoscopic third ventriculostomy for the treatment of this pathological entity. The successful use of this technique in such a case has not been previously described, and the results allow us to speculate on the pathophysiological mechanism involved.
A 34-year-old woman presented with headaches, a motor deficit of the right upper limb, and gait dyspraxia. Magnetic resonance imaging scans demonstrated dilation of all ventricles, compression of the retrocerebellar cerebrospinal fluid space, downward displacement of the tonsils, and syringomyelia. Syringomyelia involved the cervicodorsal cord below C3, with a syrinx-free segment between C1 and C3 and no enlargement of the rostral part of the central canal.
Endoscopic third ventriculostomy resulted in prompt improvement of the clinical symptoms. Postoperative magnetic resonance imaging scans demonstrated shrinkage of the syrinx and return of the cerebellar tonsils to their physiological positions.
This experience demonstrates that endoscopic third ventriculostomy, which is a simple, safe technique, may be the treatment of choice for associated Chiari I malformations, hydrocephalus, and syringomyelia (even the noncommunicating type).
Chiari I畸形合并脊髓空洞症和脑积水是一种罕见疾病。我们报告了内镜下第三脑室造瘘术成功用于治疗这一病理实体的情况。此前尚未有在这种病例中成功使用该技术的描述，而其结果使我们能够推测其中涉及的病理生理机制。
一名34岁女性出现头痛、右上肢运动功能障碍和步态失调。磁共振成像扫描显示所有脑室扩张、小脑后脑脊液间隙受压、扁桃体向下移位以及脊髓空洞症。脊髓空洞症累及C3以下的颈胸段脊髓，C1和C3之间有一段无空洞的节段，中央管头端未扩大。
内镜下第三脑室造瘘术使临床症状迅速改善。术后磁共振成像扫描显示空洞缩小，小脑扁桃体恢复到其生理位置。
这一经验表明，内镜下第三脑室造瘘术是一种简单、安全的技术，可能是治疗相关Chiari I畸形、脑积水和脊髓空洞症（甚至是非交通性类型）的首选方法。
