Bilateral Papilledema in a Child with Spinal Myxopapillary Ependymoma: A Case Report.

Abstract

BACKGROUND Myxopapillary ependymomas, a subtype of spinal ependymomas, are relatively rare in children and adolescents, primarily affecting the lower spinal region; however, they can also metastasize along the spinal axis, as well as intracranially. The clinical presentation typically involves signs and symptoms of conus medullaris or cauda equina dysfunction, including paralysis and sensory loss, accompanied by lower back pain. Ocular complications are not commonly seen in myxopapillary ependymomas. This case report emphasizes the need to consider spinal tumors in patients presenting with bilateral papilledema, particularly in the presence of neurological symptoms or increased cerebrospinal fluid protein levels. It also stresses the importance of spinal MRI to prevent misdiagnosis during ophthalmic assessments. CASE REPORT A 12-year-old boy, previously diagnosed with neuromyelitis optica at another hospital and initially responsive to intravenous methylprednisolone, returned with relapsed vision loss and neck and back pain. At Shenzhen Children's Hospital, an ophthalmological examination showed bilateral papilledema with poorly defined margins. Due to his neurological symptoms, a consultation was conducted, revealing several positive signs. A lumbar puncture indicated elevated cerebrospinal fluid pressure and protein levels. A subsequent spinal MRI identified a space-occupying lesion in the spinal column. The patient underwent neurosurgical intervention to remove the intradural lesion, and the pathology confirmed a WHO Grade 2 myxopapillary ependymoma. Postoperatively, his right eye vision improved to 20/40, and his left eye vision to 20/20, resolving his diplopia and restoring normal muscle strength and tone in all limbs. However, a tumor recurrence 3 months later necessitated a second surgery. CONCLUSIONS Ocular complications are rare in pediatric patients with myxopapillary ependymoma. Timely recognition of associated symptoms by ophthalmologists, followed by a comprehensive neurological evaluation to uncover potential neurologic issues, can significantly improve outcomes through early intervention for myxopapillary ependymoma.