弥漫性颅骨肥厚致获得性Chiari Ⅰ畸形一例并文献复习  

Acquired Chiari Ⅰ malformation secondary to generalized hyperostotic skull： a case report and literature review

作　　者：尹绍雅[1] 陈祎阳[1] 杨国军[1] 王宏[1] 

机构地区：[1]天津市环湖医院神经外科,300060

出　　处：《中华神经外科杂志》2011年第11期1151-1153,共3页Chinese Journal of Neurosurgery

摘　　要：目的探讨弥漫性颅骨肥厚致获得性ChiariⅠ畸形个体化治疗方案及机制。方法报告1例弥漫性颅骨增厚致颅内压增高和小脑扁桃体下疝，并复习相关文献。结果颅骨肥厚造成颅内容积减小，部分患者可合并小脑扁桃体下疝，可继发于Camurati—Engelmann病、颅骨一骨干发育不良、Worth—type骨内膜肥厚和硬化性骨化病等。作者所报告病例即属于常染色体显性遗传性骨硬化症。治疗上宜采用幕上开颅减压术，而禁忌采用单纯枕下减压术。结论弥漫性颅骨肥厚引起获得性ChiariⅠ畸形很少见，治疗上应行幕上开颅减压术。Objective Acquired Chiari Ⅰ malformation associated with generalized hyperostotic skull is a rare condition. The management of this condition is far more different from congenital and other kind of acquired Chiari Ⅰ malformation. This study tried to establish an individual management plan for these patients. Method A 22 -year old female patient was admitted for headache and papilledema. Generalized hyperostotic skull and secondary ChiariⅠ malformation were found after examination. This case was reported and related literatures were reviewed. Results Most cases of generalized hyperostosis of skull are associated with Camurati - Engelmann disease, craniodiaphyseal dysplasia, Worth - type endosteal hyperostosis, sclerosteosis, or other conditions. Infrequently, a Chiari malformation may also be described. This patient was a Worth - type endosteal hyperostosis and the surgical approach of subtemporal decompression was taken. Conclusions Acquired Chiari Ⅰ malformation is rarely associated with hyperostosis of skull. For a patient with symptom, a procedure of subtemporal decompression should be taken and suboccipital approach should be prohibitive.

关 键 词：获得性Chiari畸形 骨肥厚 颞下减压术 

分 类 号：R651.1[医药卫生—外科学]