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一名先天性无痛觉患者的脊柱表现

Spinal manifestations in a patient with congenital insensitivity to pain.

作者信息

Tsirikos Athanasios I, Haddo Omar, Noordeen Hilali H

机构信息

Great Ormond Street Hospital for Children, London WC1N 3JH, UK.

出版信息

J Spinal Disord Tech. 2004 Aug;17(4):326-30. doi: 10.1097/01.bsd.0000096271.75373.04.

Abstract

Spinal manifestations in congenital insensitivity to pain are relatively uncommon and easily misdiagnosed. We report on a patient with absent protective pain sensation, who developed spinal neuropathic arthropathy. At age 11 years, he presented with a destructive lesion at the L1-L2 level, causing him tingling sensation in both lower limbs. He was treated with combined anteroposterior spinal fusion from T12 to L3 and had full recovery. Five years later, he presented with a long history of clicking in his low back, muscle weakness and paresthesia in both lower extremities during walking, and evidence of Charcot arthropathy at the L4-L5 level, resulting in junctional kyphosis and canal narrowing. Posterior spinal arthrodesis from L3 to the sacrum was performed, due to lack of patient and parental consent for combined anterior decompression/posterior fusion. The patient resumed normal muscle function and his previous level of activities. Spinal complications should be anticipated in this condition and create diagnostic and therapeutic dilemmas. However, surgical management can produce favorable clinical results.

摘要

先天性无痛觉患者的脊柱表现相对少见且易被误诊。我们报告一例无保护性痛觉的患者,其发生了脊柱神经性关节病。11岁时,他出现L1-L2水平的破坏性病变,导致双下肢刺痛感。他接受了从T12到L3的前后路联合脊柱融合术,术后完全康复。五年后,他出现长期下腰部弹响、行走时双下肢肌肉无力和感觉异常,以及L4-L5水平的夏科关节病证据,导致交界性后凸和椎管狭窄。由于患者及其家长不同意进行前路减压/后路融合联合手术,遂行从L3到骶骨的后路脊柱融合术。患者恢复了正常肌肉功能和之前的活动水平。在这种情况下应预见到脊柱并发症,并会产生诊断和治疗难题。然而,手术治疗可产生良好的临床效果。

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