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Introduction: Neurofibromatosis (NF) is a cluster of conditions in which tumors grow in the nervous system. Even though the spine is the most common site of skeletal involvement in NF type 1 (NF-1), cervical spinal anomalies are infrequent.
Case Report: The atlantoaxial joint is a critical transitional zone in the cervical spine and is prone to instability. We report the case of a 49-year-old gentleman with atlantoaxial instability due to NF-1 who presented with quadriparesis and was managed by reduction with skull traction followed by C1-C2 Goel-Harms fixation.
Conclusion: In the present case, there was substantial bleeding and significant vertebral dysplasia, which makes the case distinctly unique. Skull traction and C1-C2 Goel-Harms fixation with fusion should be considered for patients with NF-1 complicated by atlantoaxial instability.
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Radiographics
April 2025
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.).
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.
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Atlantoaxial subluxation (AAS) is a neurosurgical condition caused by joint hyperflexion and is characterized by the development of compressive cervical myelopathy. Various ventral fixation techniques have been described to permanently reduce the subluxation by long-term stabilization of the atlantoaxial joint. However, the rate of complications related to implant failure with these techniques remains high.
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Unlabelled: Magerl's technique remains a widely accepted method for achieving C1-C2 fusion. Although two approaches using intermuscular corridors and biplanar X-ray guidance have been reported, there are no published studies dedicated to fully percutaneous techniques employing cannulated screws.
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Asian J Neurosurg
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Department of Neurology, Saiseikai Shiga Hospital, Imperial Gift Foundation Inc., Ritto, Shiga, Japan.
Bow-Hunter syndrome (BHS) includes a form of stroke caused by dynamic factors affecting the extracranial vertebral artery (VA), such as rotation-induced flow disturbance at the atlantoaxial level. BHS is a rare condition that can result from a variety of factors, including abnormal bone compression and vertebral instability. So far, there have been no reports in which both vertebral instability and osteophytes compress the VA at different sites, leading to stroke.
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