Primary craniovertebral anomalies and the hindbrain herniation syndrome (Chiari I): data base analysis.
Primary craniovertebral anomalies and the hindbrain herniation syndrome (Chiari I): data base analysis.
复制标题
原发性颅椎异常和后脑疝综合征（Chiari I）：数据库分析。
DOI：
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发表时间：
1995
影响因子：
0.7
通讯作者：
Arnold H. Menezes
中科院分区：
文献类型：
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作者：
Arnold H. Menezes
这项前瞻性研究分析了100例基亚里畸形和原发性颅颈交界（CVJ）异常患者（3-66岁）。神经诊断调查采用断层扫描，气体脊髓造影，CT和CT脊髓造影，和MRI。考虑的因素包括复位、侵犯方式、脑脊液动力学和脊髓积水。66名患有不可复性病变的患者接受了腹侧或腹外侧减压和背侧稳定。在还需要背侧减压的可复位病变中进行背侧枕颈固定（n = 34）。前寰椎残留8例，寰椎同化92例。20例存在旁正中内陷，46例存在脊髓积水，66例存在椎体分割缺陷。20例2-14岁患者中有16例发现完全可复性异常，14-20岁患者中有4例发现部分可复性异常，20-40岁患者中有8例发现部分可复性异常，40-60岁患者中有6例发现部分可复性异常。枕骨大孔处的临界矢状径为19 mm。22例患者既往接受过后路减压术，27例患者既往接受过空洞至蛛网膜下腔分流术，延迟恶化。在腹侧或腹外侧减压后，所有病例均得到改善，脊髓积水消退，脑脊液流量正常化。我们的结论是：（1）后脑疝综合征多见于第四枕部硬结异常，（2）基亚里畸形伴颅颈畸形，椎管直径< 19 mm时出现症状，（3）腹侧骨质异常在寰椎同化时可复位，以后变为不可复位的内陷，建议早期手术融合;（4）腹侧减压可缓解脑干、小脑症状和脊髓水肿;（5）CSF电影MRI研究显示腹侧CVJ减压后颅脊髓CSF分离逆转;（6）与基亚里畸形相关的颅颈畸形必须早期适当处理。
This prospective study analyzes 100 patients with Chiari malformation and primary craniovertebral junction (CVJ) anomalies (3-66 years). Neurodiagnostic investigations employed tomography, gas myelography, CT and CT myelography, and MRI. Factors considered were reducibility, mode of encroachment, cerebrospinal fluid (CSF) dynamics and syringohydromyelia. Sixty-six patients with irreducible pathology underwent ventral or ventrolateral decompression and dorsal stabilization. Dorsal occipitocervical fixation was performed in reducible lesions that also required dorsal decompression (n = 34). Proatlas remnants were identified in 8 and atlas assimilation in 92 patients. Paramesial invagination was present in 20, syringohydromyelia in 46, and vertebral segmentation defects in 66 others. Completely reducible abnormalities were identified in 16 of 20 patients aged 2-14 years, and partially reducible abnormalities in 4 of 16 patients aged 14-20 years, 8 of 48 patients aged 20-40 years and 6 of 16 patients aged 40-60 years. The critical sagittal canal diameter at the foramen magnum was 19 mm. Twenty-two patients had previous posterior decompression and 27 had previous syrinx to subarachnoid shunt with delayed deterioration. Improvement occurred in all after ventral or ventrolateral decompression with resolution of the syringohydromyelia and normalization of CSF flow. We conclude that: (1) hindbrain herniation syndrome is frequently seen with fourth occipital sclerotome abnormalities; (2) Chiari malformation with craniovertebral abnormalities become symptomatic with a canal diameter of < 19 mm; (3) abnormal ventral bony pathology is reducible in children wit atlas assimilation and later becomes irreducible invagination, therefore early operation with fusion is recommended; (4) ventral decompression relieves brain stem, cerebellar symptoms and syringohydromyelia; (5) CSF studies with cine MRI shows reversal of craniospinal CSF dissociation after ventral CVJ decompression and; (6) craniovertebral anomalies associated with Chiari malformations must be addressed early and appropriately.