I型Chiari畸形相关脊髓空洞症治疗后脊髓-椎管比例失调：病例说明

Spinal cord-spinal canal disproportion following treatment for syringomyelia associated with Chiari malformation type I: illustrative case.

作者信息

Nair Arjun, Gupta Vivek P, Kolmetzky Devin, Strahle Jennifer M, Limbrick David D

机构信息

Department of Neurological Surgery, Washington University in Saint Louis School of Medicine, Saint Louis, Missouri.

Philadelphia College of Osteopathic Medicine, Philadelphia, Pennsylvania.

出版信息

J Neurosurg Case Lessons. 2024 Dec 9;8(24). doi: 10.3171/CASE24272.

BACKGROUND

Chiari malformation type I (CM-I) is a common pediatric neurosurgical condition that is often associated with syringomyelia (SM) and spine deformity. The association of these three conditions is well recognized, but the pathophysiology linking them has yet to be fully elucidated.

OBSERVATIONS

This case report describes the unusual course of a 13-year-old male with CM-I, a large holocord syrinx, and progressive scoliosis who developed an angiography-negative subarachnoid hemorrhage (SAH) 4 months after successful posterior fossa decompression with duraplasty (PFDD). Notably, his cervical spinal canal diameter was increased relative to normative data. After presenting with SAH, he was treated with a course of lumbar cerebrospinal fluid drainage, which relieved his symptoms, and he had no further incidents. His syrinx responded to PFDD with a dramatic decrease in spinal cord diameter, in contradistinction to his dilated bony spinal canal. He is presently 4 years out from his SAH and doing well clinically.

LESSONS

This report describes the first case of nonaneurysmal SAH following PFDD for CM-I and SM and explores a possible link between SM and bony spinal canal diameter. https://thejns.org/doi/10.3171/CASE24272.

背景

Ⅰ型Chiari畸形（CM-Ⅰ）是一种常见的儿科神经外科疾病，常与脊髓空洞症（SM）和脊柱畸形相关。这三种疾病之间的关联已得到充分认识，但将它们联系起来的病理生理学尚未完全阐明。

观察结果

本病例报告描述了一名13岁男性患者的不寻常病程，该患者患有CM-Ⅰ、巨大全脊髓空洞症和进行性脊柱侧弯，在成功进行后颅窝减压并硬脑膜成形术（PFDD）4个月后发生了血管造影阴性的蛛网膜下腔出血（SAH）。值得注意的是，相对于正常数据，他的颈椎管直径增大。出现SAH后，他接受了一个疗程的腰椎脑脊液引流治疗，症状得到缓解，此后未再发生此类情况。与他扩张的骨性椎管不同，他的脊髓空洞对PFDD有反应，脊髓直径显著减小。他目前已从SAH发病后度过了4年，临床情况良好。