1例Klippel-Feil综合征合并C1枕化的非类风湿性齿突后假瘤。
A case of nonrheumatoid retro-odontoid pseudotumor in Klippel-Feil syndrome with C1 occipitalization.
作者信息
Sakamoto Kimiya, Uchikado Hisaaki, Hanada Hayatsura, Inoue Ritsuro, Nii Kouhei, Higashi Toshio
机构信息
Department of Neurosurgery, Fukuoka University Chikushi Hospital.
Department of Neurosurgery, Uchikado Neuro-Spine Clinic, Fukuoka, Japan.
出版信息
Surg Neurol Int. 2022 Oct 21;13:480. doi: 10.25259/SNI_891_2022. eCollection 2022.
BACKGROUND
Patients with both nonrheumatoid retro-odontoid pseudotumors (ROPTs) and congenital craniocervical junction (CCJ) abnormalities are rare. Here, a 73-year-old female presented with neck pain and myelopathy due to MR-documented ROPT with intramedullary hyperintensity at the CCJ warranting an occipital-cervical fusion.
CASE DESCRIPTION
A 73-year-old female originally developed occipitalgia and became quadriparetic within the subsequent 7 months. The cervical MR showed a ROPT with intramedullary hyperintensity at the CCJ. Further, the CT demonstrated C1 occipitalization and a congenital C2-3 fusion without radiological instability. After she underwent an occipito-C2 fusion, her symptoms improved.
CONCLUSION
For patients with C1 occipitalization and a Klippel-Feil syndrome, ROPT may occur due to loading of C1-2 complex. These patients typically favorably respond to occipito-C2 fusion.
背景
同时患有非类风湿性齿状突后假瘤（ROPT）和先天性颅颈交界区（CCJ）异常的患者较为罕见。在此，一名73岁女性因磁共振成像（MR）证实的ROPT且CCJ处髓内高信号而出现颈部疼痛和脊髓病，需要进行枕颈融合术。
病例描述
一名73岁女性最初出现枕部疼痛，并在随后7个月内发展为四肢瘫。颈椎MR显示CCJ处有一个伴有髓内高信号的ROPT。此外，CT显示C1枕化和先天性C2-3融合，无影像学不稳定。她接受枕颈2融合术后，症状有所改善。
结论
对于C1枕化和Klippel-Feil综合征患者，ROPT可能因C1-2复合体负荷而发生。这些患者通常对枕颈2融合术反应良好。
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