Case of the Week
Section Editors: Matylda Machnowska1 and Anvita Pauranik2
1University of Toronto, Toronto, Ontario, Canada
2BC Children's Hospital, University of British Columbia, Vancouver, British Columbia, Canada
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January 18, 2016
Neurenteric Cyst
- Background:
- Neurenteric cysts (NEC) are benign congenital CNS lesions.
- Foramen magnum NEC is rare, as NECs are usually located in the extramedullary compartment and are ventral to the cord.
- Intramedullary or dorsal lesions are more uncommon. They arise from persistence of the neurenteric canal.
- Clinical Presentation:
- Headache and progressive neurologic deficits are the most common presenting complaints.
- Key Diagnostic Features:
- On CT, they are usually hypo/isodense to spinal cord. Associated bony abnormalities are seen in up to 50% of spinal cases (rare in intracranial NEC). These include scoliosis, spina bifida, and Klippel–Fiel syndrome.
- On MRI, NECs are lobulated masses usually located in the intradural-extramedullary compartment anterior to the cord. Intramedullary location is present in 10–15% of cases. Usually, they are hypo/isointense on T1WI and hyperintense on T2WI. Occassional rim enhancement of the cyst wall may be seen. No restriction is seen on diffusion-weighted sequences.
- Differential Diagnosis:
- Epidermoid cyst
- Arachnoid cyst
- Cystic nerve sheath tumour
- Cystic meningioma
- Cystic metastasis
- Treatment:
- Surgical removal is the treatment of choice. Gross total resection should always be attempted.
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