肢端骨发育不全与椎管受累。
Acrodysostosis and spinal canal involvement.
作者信息
Lahoud Georges Abi, Chalouhi Nohra, Jabbour Pascal
机构信息
Department of Neurosurgery, Division of Spine Surgery, Paris Descartes University and Sainte-Anne University Hospital, Paris, France.
Department of Neurosurgery, Thomas Jefferson University and Jefferson Hospital for Neuroscience, Philadelphia, Pennsylvania, USA.
出版信息
World Neurosurg. 2014 Sep-Oct;82(3-4):537.e9-11. doi: 10.1016/j.wneu.2013.03.071. Epub 2013 Mar 30.
BACKGROUND
Acrodysostosis is a rare syndrome characterized by peripheral dysostosis, nasal hypoplasia, and frequently mental retardation. Only two adult cases of acrodysostosis have been reported to have neurological symptoms.
CASE DESCRIPTION
We report one additional adult case that presented with signs of spinal cord compression from spinal stenosis, and make the first histologic description in the literature of the bony anomalies seen in acrodysostosis. The patient had a T3 to T5 laminectomy and experienced a complete recovery.
CONCLUSIONS
Special attention should be given to these patients to detect signs of spinal stenosis, as early decompression can lead to neurological recovery.
背景
肢端发育不全综合征是一种罕见的综合征，其特征为外周骨发育不全、鼻发育不全，且常伴有智力发育迟缓。据报道，仅有两例成年肢端发育不全综合征患者出现神经症状。
病例描述
我们报告了另外一例成年患者，该患者表现出因椎管狭窄导致的脊髓受压症状，并首次在文献中对肢端发育不全综合征中所见的骨骼异常进行了组织学描述。该患者接受了T3至T5椎板切除术，并完全康复。
结论
应特别关注这些患者以检测椎管狭窄的体征，因为早期减压可使神经功能恢复。
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