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患有广泛长节段严重C1至C6节段椎管狭窄的成年软骨发育不全患者:独特表现

Adult Achondroplasia Patient with Extensive Long Segment Severe C1 to C6 Segment Spinal Canal Stenosis: Unique Presentation.

作者信息

Satyarthee Guru Dutta, Grag Kanwaljit

机构信息

Department of Neurosurgery, Neurosciences Centre, AIIMS, New Delhi, India.

出版信息

Asian J Neurosurg. 2019 Jul-Sep;14(3):883-885. doi: 10.4103/ajns.AJNS_273_17.

Abstract

Achondroplasia represents the common variety of skeletal dysplasia causing short-limb dwarfism. It may be associated with congenial craniovertebral junction anomaly, including atlantoaxial dislocation, Os odointoideum or basilar invagination or acquired as a result of the previous foramen magnum region surgery. However, long segment cervical compression from the first cervical vertebra region to sixth cervical region, such extensive compression producing myelopathy is not reported, and current case represents first case in the western literature. He was managed surgically with a good outcome. Perioperative management along with brief review of pertinent literature along with the management of such case is discussed in brief. Anesthetic management of such patient with coexisting extensive cervical compression presents unique challenges in view of the multisystem involvement along with preexisting anatomical and physiological constraints.

摘要

软骨发育不全是导致短肢侏儒症的常见骨骼发育异常类型。它可能与先天性颅颈交界区异常有关,包括寰枢椎脱位、齿状突骨或基底凹陷,或者是既往枕骨大孔区手术导致的后天性异常。然而,从第一颈椎区域到第六颈椎区域的长节段颈椎受压,产生如此广泛的压迫并导致脊髓病的情况尚未见报道,本病例是西方文献中的首例。他接受了手术治疗,效果良好。本文简要讨论了围手术期管理以及相关文献的简要回顾和此类病例的处理。鉴于多系统受累以及既往存在的解剖和生理限制,对患有并存广泛颈椎压迫的此类患者进行麻醉管理面临独特的挑战。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/b50e/6703022/f3b4d55e898a/AJNS-14-883-g001.jpg

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