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一名54岁出现巨大脊髓空洞症的Chiari畸形罕见病例。

A Rare Course of Chiari Malformation With Large Syringomyelia Presenting at 54 Years Old.

作者信息

Tanaka Masato, Sharma Sneha, Gori Kushal H, Shohidullah Md, Uotani Koji

机构信息

Orthopedic Surgery, Okayama Rosai Hospital, Okayama, JPN.

Orthopedic Surgery, Okayama University Hospital, Okayama, JPN.

出版信息

Cureus. 2025 Feb 2;17(2):e78399. doi: 10.7759/cureus.78399. eCollection 2025 Feb.

Abstract

Chiari malformation type 1 (CM1) is considered a congenital condition. The symptoms include severe headache, hypalgesia, and loss of temperature sensation. It constitutes a significant burden among children and young adults. The onset of symptoms of CM1 is more commonly observed in relatively young children and is very rare in those over 50 years old. This study aims to present a rare surgical case of CM1 associated with a large syringomyelia in a 54-year-old man. A 54-year-old man with low back pain was introduced to our department. He had slight hyperreflexia of the extremities, slight muscle weakness in both legs, and numbness in the right leg (3/10). He also had urinary and bowel incontinence and spastic gait. Cervical magnetic resonance imaging (MRI) showed CM1 with large syringomyelia extending from C1 to T11. The cervical canal was widened because of a long history of spinal cord expansion. The patient was successfully treated surgically by foramen magnum decompression and syringosubarachnoid shunting under the guidance of O-arm navigation. The muscle weakness and sensory function recovered almost entirely on the one-year follow-up. The patient's cervical Japanese Orthopedic Association (JOA) score had improved from 11/17 to 16/17. Gradually enlarging syringomyelia with slight CM1 is rare, but surgeons should consider this condition's possibility. Foramen magnum decompression achieves good results even in cases with a long history of syringomyelia. This new navigation technique provides an excellent result for a large syringomyelia with CM1.

摘要

1型Chiari畸形(CM1)被认为是一种先天性疾病。症状包括严重头痛、痛觉减退和温度感觉丧失。它给儿童和年轻人带来了巨大负担。CM1症状的发作在相对年幼的儿童中更为常见,在50岁以上的人群中非常罕见。本研究旨在介绍一例54岁男性CM1合并巨大脊髓空洞症的罕见手术病例。一名因腰痛前来就诊的54岁男性被收入我科。他四肢有轻度反射亢进,双腿有轻度肌无力,右腿麻木(3/10)。他还存在大小便失禁和痉挛性步态。颈椎磁共振成像(MRI)显示CM1合并从C1延伸至T11的巨大脊髓空洞症。由于脊髓长期扩张,颈椎管增宽。患者在O型臂导航引导下成功接受了枕骨大孔减压和脊髓空洞-蛛网膜下腔分流术。在一年的随访中,肌无力和感觉功能几乎完全恢复。患者的颈椎日本骨科协会(JOA)评分从11/17提高到了16/17。伴有轻度CM1的逐渐扩大的脊髓空洞症很少见,但外科医生应考虑到这种情况的可能性。即使在脊髓空洞症病史较长的病例中,枕骨大孔减压也能取得良好效果。这种新的导航技术为CM1合并巨大脊髓空洞症提供了优异的治疗效果。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/c6f1/11882125/d3ee8cdb8dd7/cureus-0017-00000078399-i01.jpg

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