Zuev A A, Pedyash N V, Epifanov D S, Kostenko G V
Pirogov National Medical Surgical Center, Moscow, Russia.
Zh Vopr Neirokhir Im N N Burdenko. 2016;80(1):27-34. doi: 10.17116/neiro201680127-34.
The rate of Chiari malformation (CM) in a population ranges from 3 to 8 per 100,000 population. In 62-80% of cases, CM is accompanied by the development of syringomyelia (SM) at various levels. The clinical picture in these patients is a combination of CM and SM manifestations; however, SM symptoms often prevail, which creates some problems in the diagnosis of the disease and in the choice of optimal treatment.
On the basis of our own experience of surgical interventions, we aimed to clarify the indications for surgical treatment of SM associated with CM and to determine the optimal amount of surgery and the criteria for evaluation of treatment outcomes.
Two hundred twenty five patients with a combination of syringomyelia and Chiari 1 malformation were examined in the period from 2011 to February 2015. Of them, 125 patients were operated on. The mean age of the operated patients was 56±8 years. The mean time from the appearance of the first signs of the disease to surgery was 75±82 months. All operations were performed by a single surgeon. The operations were carried out in the half-sitting (89.6%) or prone (10.4%) position. The operation included sparing suboccipital craniectomy, C1 arch resection, recovery of the cerebrospinal fluid (CSF) circulation along the posterior surface of the cerebellum, and reconstruction of the dura mater in the craniovertebral junction region.
Exploration of the arachnoid mater of the cisterna magna after dura opening revealed no arachnopathy in 78 (62.4%) patients (Chiari 0 malformation according to Klekamp). The type 1 arachnopathy (by Klekamp) was detected in 31 patients (24.8%), and the type 2 arachnopathy was observed in 16 (12.8%). The condition of 109 (88%) patients was evaluated one year after the surgery. Sixty one (56%) patients had partial or complete regression of preoperative neurological symptoms. The disease stopped progressing in 44 patients (40%). The disease was progressing in 4 (3.7%) patients. No recurrence of a CSF circulation disturbance at the craniovertebral level was observed during follow-up. Early postoperative complications occurred in 4 (3.2%) patients: wound CSF leakage in 1 (0.8%) patient, acute epidural hematoma in 1 (0.8%) patient, and aseptic meningitis in 2 (1.6%) patients. Temporary deteriorations in the condition (headache worsening, meteosensitivity) were detected in 11 (8.9%) patients. These symptoms regressed by the end of the 1st postoperative month. There were no deaths.
The indication for surgery in patients with a combination of CM and SM is the presence of neurological symptoms associated with syringomyelia and their progression as well as headache caused by herniation of the cerebellar tonsils, which significantly deteriorates the patient's quality of life. The main criteria for evaluating the efficacy of treatment include stabilization of the clinical symptoms and/or improvement in the patient condition. Suboccipital craniectomy followed by reconstruction of the dura mater and recovery of the CSF circulation in the craniovertebral region is an effective treatment of syringomyelia associated with Chiari 1 malformation.
Chiari畸形（CM）在人群中的发生率为每10万人中有3至8例。在62% - 80%的病例中，CM伴有不同程度的脊髓空洞症（SM）的发生。这些患者的临床表现是CM和SM表现的组合；然而，SM症状往往更为突出，这在疾病诊断和最佳治疗方案的选择上造成了一些问题。
基于我们自己的手术干预经验，我们旨在明确与CM相关的SM的手术治疗指征，确定最佳手术量以及治疗效果评估标准。
2011年至2015年2月期间，对225例合并脊髓空洞症和Chiari 1畸形的患者进行了检查。其中，125例患者接受了手术。手术患者的平均年龄为56±8岁。从疾病首次出现症状到手术的平均时间为75±82个月。所有手术均由同一位外科医生进行。手术采用半坐位（89.6%）或俯卧位（10.4%）。手术包括保留性枕下颅骨切除术、C1椎弓切除、沿小脑后表面恢复脑脊液（CSF）循环以及颅颈交界区硬脑膜重建。
硬脑膜打开后对小脑延髓池蛛网膜进行探查，78例（62.4%）患者未发现蛛网膜病变（根据Klekamp分类为Chiari 0畸形）。31例（24.8%）患者检测到1型蛛网膜病变（根据Klekamp分类），16例（12.8%）患者观察到2型蛛网膜病变。术后一年对109例（88%）患者的病情进行了评估。61例（56%）患者术前神经症状部分或完全缓解。44例（40%）患者疾病停止进展。4例（3.7%）患者疾病仍在进展。随访期间未观察到颅颈水平脑脊液循环障碍复发。4例（3.2%）患者出现早期术后并发症：1例（0.8%）患者伤口脑脊液漏，1例（0.8%）患者急性硬膜外血肿，2例（1.6%）患者无菌性脑膜炎。11例（8.9%）患者出现病情暂时恶化（头痛加重、对天气敏感）。这些症状在术后第1个月末消退。无死亡病例。
CM和SM合并患者的手术指征是存在与脊髓空洞症相关的神经症状及其进展，以及小脑扁桃体疝引起的头痛，这会显著降低患者的生活质量。评估治疗效果的主要标准包括临床症状稳定和/或患者病情改善。枕下颅骨切除术后进行硬脑膜重建并恢复颅颈区脑脊液循环是治疗与Chiari 1畸形相关的脊髓空洞症的有效方法。
