Vigneul Eric, Del Gaudio Nicole, de Nijs Loïc, Raftopoulos Christian
Department of Neurosurgery, Cliniques universitaires Saint-Luc, Université Catholique de Louvain, Brussels, Belgium.
Laboratory of Neural Differentiation (NEDI), Animal Molecular and Cellular Biology Group, Louvain Institute of Biomolecular Science and Technology, Université Catholique de Louvain, Louvain-La-Neuve, Belgium.
Childs Nerv Syst. 2024 Jun;40(6):1943-1947. doi: 10.1007/s00381-024-06327-6. Epub 2024 Feb 19.
Chiari malformation type 1 (CM1) is a congenital hindbrain malformation characterized by herniation of the cerebellar tonsils below the foramen magnum. The term Chiari type 1.5 is used when herniation of the brainstem under the McRae line and anomalies of the craniovertebral junction are also present. These conditions are associated with several symptoms and signs, including headache, neck pain, and spinal cord syndrome. For symptomatic patients, surgical decompression is recommended. When radiographic indicators of craniovertebral junction (CVJ) instability or symptoms related to ventral brainstem compression are present, CVJ fixation should also be considered.
We report the case of a 13-year-old girl who presented with severe tetraparesis after posterior decompression for Chiari malformation type 1.5, followed 5 days later by partial C2 laminectomy. Several months after the initial surgery, she underwent two fixations, first without and then with intraoperative cervical traction, leading to significant neurological improvement.
This case report underscores the importance of meticulous radiological analysis before CM surgery. For CM 1.5 patients with basilar invagination, CVJ fixation is recommended, and C2 laminectomy should be avoided. In the event of significant clinical deterioration due to nonadherence to these guidelines, our findings highlight the importance of traction with increased extension before fixation, even years after initial destabilizing surgery.
1型Chiari畸形(CM1)是一种先天性后脑畸形,其特征为小脑扁桃体疝入枕骨大孔以下。当脑干在McRae线以下疝出且存在颅颈交界区异常时,使用Chiari 1.5型这一术语。这些情况与多种症状和体征相关,包括头痛、颈部疼痛和脊髓综合征。对于有症状的患者,建议进行手术减压。当存在颅颈交界区(CVJ)不稳定的影像学指标或与腹侧脑干受压相关的症状时,也应考虑CVJ固定术。
我们报告了一名13岁女孩的病例,她在接受1.5型Chiari畸形后颅减压术后出现严重四肢轻瘫,5天后接受了C2部分椎板切除术。初次手术后数月,她接受了两次固定手术,第一次未进行术中颈部牵引,第二次进行了术中颈部牵引,神经功能得到显著改善。
本病例报告强调了CM手术前细致的影像学分析的重要性。对于伴有基底凹陷的CM 1.5患者,建议进行CVJ固定术,应避免C2椎板切除术。如果由于未遵循这些指南而导致显著的临床恶化,我们的研究结果突出了在固定前进行增加伸展度牵引的重要性,即使是在初次失稳手术数年之后。