Background
Intramedullary ependymal cysts of the spinal cord are rare, benign fluid filled cysts usually situated along the ventral surface of the spinal cord. Since they were first described, only 32 cases have been reported. Thus, owing to the rarity at which these cysts are encountered, their management and pathogenesis remain controversial. While some have previously advocated for cysto-subarachnoid shunt placement for symptomatic ependymal cysts, others have argued for complete cyst resection or simple fenestration. Herein we report the case of a 56-year-old female with a T11-T12 ependymal cyst that was successfully managed with cyst fenestration. We further investigated a potential pathologic mechanism of cyst formation by performing immunohistochemistry to detect aquaporin expression in the cyst lining.
Case Description
A 56-year-old female was found to harbor an enlarging cystic lesion of the conus which was discovered upon workup of progressive paraparesis and urinary incontinence. She had lower extremity weakness and progressive myelopathy. Thoracic laminectomy with cyst fenestration was performed, arresting her neurologic deterioration. Pathologic analysis diagnosed an intramedullary ependymal cyst. Immunohistochemistry was subsequently performed for expression of aquaporin-1 and aquaporin-4. There was dense staining of the underlying neuropil with concurrent membranous staining pattern of the cyst lining.
Conclusion
Intramedullary ependymal cysts are rare, cystic lesions of the spinal cord. Early cyst fenestration decompresses the cyst and prevents neurologic deterioration. We identified for the first time that aquaporins are expressed in the cyst wall which is consistent with a passive, osmotic pathogenic mechanism of cyst formation.