Progressive Myelopathy with a Cervical Mass
A 58-year-old man has 4 months of progressive gait unsteadiness, hand clumsiness, and urinary urgency. Examination shows brisk reflexes in all four limbs, bilateral Hoffmann signs, and a sensory level at C5. MRI shows a 2.5 cm intramedullary, expansile, T2-hyperintense lesion at C3–C5 with patchy enhancement and a syrinx above and below. There is no bone destruction. He is otherwise well, with no known primary malignancy.
1. What is your differential diagnosis, and how do you prioritise investigation?
Read the worked answer
The differential for an intramedullary expansile lesion with a syrinx includes ependymoma, astrocytoma, haemangioblastoma, and less commonly metastases, lymphoma, or inflammatory/demyelinating lesions. Age and the presence of a syrinx favour ependymoma or haemangioblastoma; astrocytoma is also possible.
Investigation priorities:
- Full MRI of the entire neuraxis with and without contrast, because ependymomas can seed and haemangioblastomas may be multiple (von Hippel–Lindau).
- Look for a primary elsewhere if metastasis is considered, though intramedullary metastases are uncommon.
- CSF analysis if inflammatory or infectious causes are plausible.
- Baseline neurological assessment and urodynamics if urinary symptoms are prominent.
Biopsy versus resection depends on the imaging pattern: a well-circumscribed lesion with a cleavage plane favours ependymoma and primary resection; a diffusely infiltrative lesion favours astrocytoma and biopsy with limited resection. Do not commit to a surgical plan before full staging.
2. He deteriorates over 48 hours with worsening weakness and new respiratory difficulty. What is your immediate management, and what are the surgical principles if you operate?
Read the worked answer
Acute deterioration with respiratory involvement is a neurosurgical emergency. Immediate steps: airway assessment and consideration of intubation if vital capacity is falling, high-dose corticosteroids for cord oedema, urgent repeat imaging to exclude haemorrhage or cyst expansion, and involvement of the critical care team. Avoid hypotension and hypoxia.
Surgical principles:
- Posterior approach with laminectomy or laminoplasty at the level of the lesion, with intraoperative neuromonitoring (motor and somatosensory evoked potentials).
- Myelotomy through the dorsal midline or via the thinnest part of the cord over the lesion, with meticulous haemostasis.
- For a well-circumscribed ependymoma, aim for gross total resection with preservation of the plane; for an infiltrative astrocytoma, biopsy and decompression rather than aggressive resection to avoid neurological injury.
- Address the syrinx if it is contributing to symptoms, but treat the underlying lesion first.
Postoperatively, monitor for respiratory failure, neuropathic pain, and cerebrospinal fluid leak, and plan rehabilitation and adjuvant therapy based on histology.