Case Report: Postpartum diagnosis and microsurgical management of a cervicothoracic intramedullary spinal ependymoma clinically manifesting during the third trimester of pregnancy.

BACKGROUND: Intramedullary spinal cord ependymomas are the most common intramedullary tumours in adults. However, clinical manifestation during pregnancy is exceptionally rare, and diagnosis may be delayed because pain, sensory disturbances, and muscle weakness are often mistakenly attributed to the common musculoskeletal changes associated with pregnancy. CASE DESCRIPTION: A 29-year-old multiparous woman developed progressive back pain, numbness, and weakness in both lower extremities at 30 weeks of gestation. She was initially managed conservatively. Following an uncomplicated spontaneous delivery at 38 weeks of gestation, her neurological condition deteriorated rapidly, with the development of severe spastic paraparesis, sensory impairment below the T1 level, urinary retention, and constipation. Magnetic resonance imaging revealed a centrally located contrast-enhancing intramedullary tumour extending from C7 to T5, accompanied by fusiform expansion of the spinal cord. On postpartum day 38, the patient underwent C7-T5 laminectomy, midline myelotomy, and microsurgical gross total resection of the tumour under multimodal intraoperative neurophysiological monitoring. Despite intraoperative deterioration of left-sided motor responses, no postoperative worsening of the neurological deficit or other surgery-related complications were observed. Following multidisciplinary tumour board review, adjuvant proton beam therapy was delivered to the tumour bed at a total dose of 54 Gy. Сontrast follow-up MRI performed at 6 months showed no convincing evidence of disease progression; Initial histopathological examination classified the tumour as an ependymoma, NOS, CNS WHO grade 3. Molecular testing revealed no mutations in the IDH1 or IDH2 genes; a heterozygous deletion of the MYB gene was additionally identified. The tumour specimen was referred for further specialist neuropathological review. At 6-month follow-up, lower-extremity muscle strength had improved from Medical Research Council grade 2/5 to 3-4/5, functional status had improved from grade IV to grade III on the modified McCormick scale, and partial recovery of bladder function was observed. No clinical or radiological evidence of disease progression was identified. CONCLUSION: The development of progressive motor deficit, a defined sensory level, pyramidal signs, or sphincter dysfunction during pregnancy or the postpartum period warrants urgent neurological assessment and MRI of the spine and spinal cord.