Klippel-Feil syndrome with a left Sylvian fissure meningioma: A case report and literature review

Background Klippel-Feil syndrome (KFS), defined by congenital cervical vertebral fusion, is rarely linked to intracranial tumors. In our case, KFS is associated with a Sylvian Fissure Meningioma that was incidentally identified during the evaluation of chronic neck pain in the neurosurgical department. Although KFS is frequently linked to both skeletal and extra-skeletal anomalies, its association with intracranial tumors, particularly meningiomas, remains exceptionally rare. Case Description A 62-year-old female with a long-standing history of neck pain presented to our neurosurgical department with a 6-month history of persistent, mild, non-throbbing headaches. She denied any episodes of seizures, visual disturbances, or loss of consciousness. Magnetic resonance imaging (MRI) of the cervical spine was performed, revealing congenital fusion of the C3–C4, C5–C6, and C7–T1 vertebrae, consistent with Klippel–Feil syndrome. Incidentally, superior slices of the cervical MRI revealed a well-defined lesion in the Sylvian fissure region. Subsequent brain MRI confirmed the presence of a meningioma in the left Sylvian fissure. The tumor was successfully excised via a left pterional approach, with no post-operative complications. Conclusion Intracranial meningioma in patients with Klippel-Feil Syndrome is an exceptionally rare occurrence. Special consideration must be given to head positioning during surgery, particularly for meningioma resection, to mitigate the risk of neurological complications, especially in patients with a degenerative and fused cervical spine.