Little Stephen B, Sarma Asha, Bajaj Manish, Dennison John, Brahma Barunashish, Pruthi Sumit
From the Departments of Radiology (S.B.L., M.B., J.D.) and Neurosurgery (B.B.), Children's Health Care of Atlanta, Emory University, Atlanta, Ga; and Department of Radiology, Vanderbilt University Medical Center, Monroe Carell Jr. Children's Hospital, 2200 Children's Way, Nashville, TN 37323 (A.S., S.P.).
Radiographics. 2025 Apr;45(4):e240075. doi: 10.1148/rg.240075.
Craniovertebral junction (CVJ) instability, fixation, and stenosis in children are closely related conditions that are often challenging to diagnose and are associated with significant morbidity. Groups at higher risk for CVJ abnormalities include children with trisomy 21, juvenile idiopathic arthritis, upper respiratory infection or other inflammatory conditions of the head and neck, and certain skeletal dysplasias. Radiography, CT, and MRI play complementary roles in the evaluation of pathologic conditions of the CVJ. CVJ morphometry is helpful in characterizing osseous relationships and suggesting potential instability and/or neural compression. CT with multiplanar and three-dimensional volume-rendered reconstructions may be helpful in identifying congenital anomalies associated with instability and/or neural canal narrowing; disorders predisposing to atlantoaxial rotatory fixation (AARF), such as retropharyngeal inflammation in Grisel syndrome; and acquired osseous abnormalities associated with irreducibility in children with chronic AARF (eg, facet deformity or new bone formation). Dynamic CT is particularly helpful for evaluating children with persistent torticollis that is refractory to initial conservative therapy. Early diagnosis and treatment of AARF are essential in reducing the likelihood of progression to chronic AARF. Performing CT angiography before C1-C2 fixation may help identify vascular variations that increase surgical risk and provide an opportunity for modification of the surgical plan. MRI is preferred for assessment of the hindbrain; upper cervical spinal cord; and nonossified structures such as cartilage, ligaments, and paravertebral soft tissues. The authors discuss normal development and anatomy, imaging evaluation, and disorders associated with CVJ instability, fixation, and stenosis in children. Imaging-related treatment considerations are also discussed. RSNA, 2025 Supplemental material is available for this article.
儿童颅颈交界区（CVJ）不稳定、固定及狭窄是密切相关的病症，诊断往往具有挑战性，且会导致严重的发病率。CVJ异常风险较高的群体包括21三体综合征患儿、幼年特发性关节炎患儿、上呼吸道感染或头颈部其他炎症性疾病患儿以及某些骨骼发育异常患儿。X线摄影、CT和MRI在CVJ病理状况评估中发挥着互补作用。CVJ形态测量有助于描述骨质关系，并提示潜在的不稳定和/或神经受压情况。具有多平面和三维容积再现重建功能的CT可能有助于识别与不稳定和/或神经管狭窄相关的先天性异常；易导致寰枢椎旋转固定（AARF）的疾病，如格里斯尔综合征中的咽后炎症；以及慢性AARF患儿中与不可复位性相关的后天性骨质异常（如关节面畸形或新骨形成）。动态CT对评估初始保守治疗无效的持续性斜颈患儿特别有帮助。早期诊断和治疗AARF对于降低进展为慢性AARF的可能性至关重要。在进行C1-C2固定之前进行CT血管造影可能有助于识别增加手术风险的血管变异，并为修改手术方案提供机会。评估后脑、上颈段脊髓以及软骨、韧带和椎旁软组织等非骨化结构时，首选MRI。作者讨论了儿童CVJ不稳定、固定及狭窄的正常发育和解剖结构以及影像学评估和相关疾病。还讨论了与影像学相关的治疗考虑因素。RSNA，2025 本文提供补充材料。
