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Updated: Jun 12, 2025

State of the Art Cranial Ultrasound Imaging in Neonates
Published on: February 2, 2015
Imaging of Craniovertebral Junction Instability, Fixation, and Stenosis in Children
Stephen B Little1, Asha Sarma1, Manish Bajaj1
1From 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.).
Insights
Craniovertebral junction (CVJ) instability, fixation, and stenosis in children require advanced imaging like CT and MRI for diagnosis. Early detection of CVJ abnormalities is crucial for effective treatment and preventing long-term complications.
Area of Science:
- Pediatric Radiology
- Neuroradiology
- Orthopedic Imaging
Background:
- Craniovertebral junction (CVJ) abnormalities in children, including instability, fixation, and stenosis, present diagnostic challenges and significant morbidity.
- High-risk groups for CVJ abnormalities include children with trisomy 21, juvenile idiopathic arthritis, inflammatory conditions, and skeletal dysplasias.
Purpose of the Study:
- To provide a comprehensive overview of imaging evaluation for craniovertebral junction disorders in children.
- To discuss the roles of radiography, CT, and MRI in diagnosing CVJ instability, fixation, and stenosis.
Main Methods:
- Review of imaging modalities including radiography, CT (multiplanar, 3D, dynamic), and MRI.
- Discussion of CVJ morphometry for assessing osseous relationships and neural compression.
- Emphasis on CT angiography for pre-surgical planning and MRI for non-ossified structures.
Main Results:
- CT is valuable for identifying congenital anomalies, atlantoaxial rotatory fixation (AARF) causes, and acquired osseous abnormalities.
- Dynamic CT aids in evaluating refractory torticollis, while CT angiography identifies vascular variations.
- MRI is essential for evaluating the hindbrain, spinal cord, and soft tissues.
Conclusions:
- Integrated imaging approaches using radiography, CT, and MRI are vital for diagnosing and managing pediatric CVJ disorders.
- Early diagnosis and treatment of AARF are critical to prevent chronicity.
- Imaging findings guide treatment considerations for CVJ instability, fixation, and stenosis in children.
Abstract:
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 (a) congenital anomalies associated with instability and/or neural canal narrowing; (b) disorders predisposing to atlantoaxial rotatory fixation (AARF), such as retropharyngeal inflammation in Grisel syndrome; and (c) 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.
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