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颅颈难题;伴有Chiari I畸形的神秘完全性枕颈融合。

Cranio-cervical conundrum; Enigmatic complete atlanto-occipital assimilation with Chairi I malformation.

作者信息

Bhinder Khurram Khaliq, Shah Mariam, Wahla Madiha Saeed, Khan Nasir, Mehboob Amna, Saghir Sabiyal, Ziad Alishbah

机构信息

Radiology Department, Shifa International Hospital, Islamabad, Pakistan.

出版信息

Radiol Case Rep. 2025 May 16;20(8):3832-3835. doi: 10.1016/j.radcr.2025.04.086. eCollection 2025 Aug.

DOI:10.1016/j.radcr.2025.04.086
PMID:40486158
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC12143777/
Abstract

Atlanto-occipital assimilation is extremely rare congenital anomaly, characterized by partial or complete fusion of atlas and occipital bone leading to altered cranio-cervical junction anatomy. In our patient, basilar invagination resulted in acute angulation and narrowing of foramen magnum. Crowding of structures at foramen magnum was seen with tonsillar herniation and compression of brain stem. Edema signal in proximal cervical cord was also seen. Bilateral atlanto-occipital assimilation with increased atlas to dens distance seen suggestive of atlanto-axial instability. Overall findings were suggestive of Chiari I malformation with atlanto-occipital assimilation.

摘要

寰枕融合是一种极其罕见的先天性异常,其特征为寰椎与枕骨部分或完全融合,导致颅颈交界区解剖结构改变。在我们的患者中,基底凹陷导致枕大孔急性成角和狭窄。可见枕大孔处结构拥挤,伴有扁桃体疝和脑干受压。颈髓近端也可见水肿信号。双侧寰枕融合,寰椎至齿突距离增加,提示寰枢椎不稳。总体检查结果提示为合并寰枕融合的Chiari I型畸形。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/c919/12143777/bd7495e79a28/gr2.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/c919/12143777/2715cbe0343b/gr1.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/c919/12143777/bd7495e79a28/gr2.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/c919/12143777/2715cbe0343b/gr1.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/c919/12143777/bd7495e79a28/gr2.jpg

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本文引用的文献

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Paralyzing paradox: Spinal cord infarction, a hidden emergency.麻痹性悖论:脊髓梗死,一种隐匿的急症。
Radiol Case Rep. 2025 Feb 6;20(4):2235-2238. doi: 10.1016/j.radcr.2025.01.054. eCollection 2025 Apr.
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Multi territory ischemic stroke in a patient with Fahr's disease: Report of a rare case.法尔病患者的多灶性缺血性卒中:1例罕见病例报告
Radiol Case Rep. 2024 Nov 9;20(1):661-666. doi: 10.1016/j.radcr.2024.10.100. eCollection 2025 Jan.
3
Atlanto-occipital assimilation: A pictorial review of a commonly missed pathology.
寰枕融合:一种常被漏诊的病理情况的影像学综述
J Clin Imaging Sci. 2024 Jul 10;14:24. doi: 10.25259/JCIS_42_2024. eCollection 2024.
4
Atlanto-occipital assimilation: embryological basis and its clinical significance.寰枕融合:胚胎学基础及其临床意义。
Anat Cell Biol. 2024 Mar 31;57(1):147-151. doi: 10.5115/acb.23.225. Epub 2023 Dec 20.
5
Bilateral basal ganglia ischemia associated with COVID-19: a case report and review of the literature.与 COVID-19 相关的双侧基底节区缺血:病例报告及文献复习。
J Med Case Rep. 2021 Nov 22;15(1):563. doi: 10.1186/s13256-021-03165-x.
6
Traumatic Craniocervical Dissociation in Patients with Congenital Assimilation of the Atlas to the Occiput.先天性寰椎枕骨融合患者的创伤性颅颈分离
Case Rep Orthop. 2019 Dec 16;2019:2617379. doi: 10.1155/2019/2617379. eCollection 2019.
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Cervical Myelopathy Secondary to Atlanto-occipital Assimilation: The Usefulness of the Simple Decompressive Surgery.寰枕融合继发的颈脊髓病:单纯减压手术的有效性
Korean J Spine. 2013 Sep;10(3):189-91. doi: 10.14245/kjs.2013.10.3.189. Epub 2013 Sep 30.
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Chiari I malformation associated with atlanto-occipital assimilation presenting as orthopnea and cough syncope.伴有寰枕融合的Chiari I型畸形,表现为端坐呼吸和咳嗽性晕厥。
J Clin Neurosci. 2014 Feb;21(2):320-3. doi: 10.1016/j.jocn.2013.03.004. Epub 2013 Sep 27.
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J Neurosurg Spine. 2011 Jul;15(1):55-9. doi: 10.3171/2011.3.SPINE10828. Epub 2011 Apr 8.