Congenital intramedullary spinal ependymal cyst

doi:10.1016/0090-3019(87)90257-6

Surgical Neurology

Volume 27, Issue 5, May 1987, Pages 476-480

https://doi.org/10.1016/0090-3019(87)90257-6 Get rights and content

Abstract

A rare case of congenital intramedullary ependymal cyst of the dorsal cord in a 7-year-old boy associated with kyphoscoliosis and rachidian malformations of the dorsal spine is reported. Myelography suggested an intramedullary lesion. The posterior location and presence of a clear plane of cleavage from the medullary tissue enabled total enucleation of the cyst. Histological differentiation from other similar intradural cysts is discussed and the relevant literature is reviewed. This is the fifth such case reported in the literature.

References (38)

KS Mann et al.
Spinal neurenteric cyst: association with vertebral anomalies, diastematomyelia, dorsal fistula and lipoma
Surg Neurol
(1984)
T Matsushima et al.
Epithelial cells in a so-called intraspinal neurenteric cyst
RD Adams
Case records of Massachusetts General Hospital no. 46122
N Engl J Med
(1960)
A Brun et al.
Intraspinal enterogenous cyst
Acta Pathol Microbiol Scand
(1968)
IS Cooper et al.
Heterotopic glial nests in subarachnoid space: histopathological characteristics, mode of origin and relation to meningeal gliomas
J Neuropathol Exp Neurol
(1951)
SR Dharker et al.
Intramedullary epithelial cyst of the spinal cord
Surg Neurol
(1979)
JF Dorsey et al.
Intraspinal and mediastinal foregut cyst compressing the spinal cord: report of a case
J Neurosurg
(1966)
A Fortuna et al.
Spinal neuroepithelial cysts: report of two cases and review of literature
Acta Neurochir (Wien)
(1978)
A Fortuna et al.
Intradural spinal cysts
Acta Neurochir (Wien)
(1983)
JV Gainer et al.
Ependymal cyst of the thoracic spinal cord
J Neurol Neurosurg Psychiatry
(1974)

B Guidetti et al.

Differential diagnosis of medullary and extramedullary tumours

DGF Harriman

An intraspinal enterogenous cyst

J Pathol Bacteriol

(1958)

A Hirano et al.

An epithelial cyst of spinal cord

CT Hoffman

Cervical arachnoid cyst

GL Holmes et al.

Intraspinal enterogenous cyst

Am J Dis Child

(1978)

I Hyman et al.

Ependymal cyst of the cervicodorsal region of the spinal cord

Arch Neurol Psychiatry

(1938)

S In et al.

A case of intraspinal enterogenous cyst

Neurol Surg

(1979)

RT Johnston

Colloid cyst of cervical canal (abstr)

J Neurol Neurosurg Psychiatry

(1959)

TE Klump

Neurenteric cyst in the cervical spinal canal of a 10-week old boy

Case report. J Neurosurg

(1971)

Cited by (23)

Congenital Spinal Cysts: An Update and Review of the Literature
2021, World Neurosurgery
Citation Excerpt :
The cyst often appears as an intradural extramedullary multilobulated lesion that can produce anterior cord expansion7 or with a widening of the canal.12 On MRI, enterogenous cysts may appear as hypointense or hyperintense lesions, on T1-weighted images, and may appear hyperintense on T2-weighted images7,8,20,33,34,54,101 (Figure 8). Myelography and CT show only an intradural extramedullary mass compressing the cord but are not diagnostic.8,12,43,54
Congenital spinal cysts are rare and encompass a wide variety of diseases including arachnoid, enterogenous, teratomatous, neurenteric, foregut, bronchogenic, epithelial, ependymal, dermoid, and epidermoid cysts. Here, we elucidate the epidemiology, pathology, pathogenesis, and diagnostic findings of the most common congenital spinal cysts, followed by a discussion of their presentation and treatment options. Differentiating the cause of each lesion is crucial for targeted clinical and surgical management for the patient. Our review describes how arachnoid cysts can be observed, fenestrated, percutaneously drained, or shunted; however, the primary goal for neurenteric, dermoid, and epidermoid cysts is removal. Further, we discuss how patient presentation is dependent on the rate of growth and location of compression on the spinal cord and nerve roots. However, although many of these lesions are discovered incidentally on imaging, the spectrum of possible symptoms include pain, weakness, ataxia, bladder incontinence, and progressive or acute neurologic deficits. We present and review the histology and imaging of a variety of cysts and discuss how although the goal of treatment is resection, the risks of surgery must be considered against the benefits of complete resection in each case.
Mobile Myxopapillary Ependymoma with Associated Filum Terminale Cyst
2020, World Neurosurgery
Intradural ependymal cysts are benign, fluid-filled cysts usually situated along the ventral surface of the spinal cord. There are previous reports of 19 intradural cysts in the literature, including one cyst of the filum terminale. Here, we report for the first time the presence of a radiographically occult filum terminale cyst associated with a myxopapillary ependymoma. We propose that mobility of the tumor may provide indirect evidence of the presence of a cyst.
A 65-year-old male patient presented with a homogenously enhancing ovoid mass measuring 25 mm × 10 mm within the thecal sac at the L3 through L4 levels. Repeat magnetic resonance imaging demonstrated migration of the tumor 12 mm rostrally. Following the L2 through L4 laminectomy and resection of the intradural tumor, we identified a filum terminale ependymal cyst superior to the tumor, which was also resected.
Ependymal cysts associated with spinal tumors are rare and may be radiographically occult. The change in cyst size may explain tumor mobility. Complete resection of the cyst and histopathologic analysis is recommended to differentiate between ependymal cyst and cystic tumor tissue.
Discovery of Aquaporin-1 and Aquaporin-4 Expression in an Intramedullary Spinal Cord Ependymal Cyst: Case Report
2017, World Neurosurgery
Citation Excerpt :
Intramedullary spinal cord ependymal cysts are rare cystic lesions thought to be derived from embryologic sequestration of ectopic neuroepithelial tissue.1,2 Unlike their cranial counterparts, which account for approximately 10% of benign intracranial cysts, ependymal cysts of the spinal cord are very rare, with only 32 cases reported in the literature since 1938.1,3-22 Ependymal cysts have the potential to expand and cause a local mass effect, leading to symptom development and neurologic deficit.19
Intramedullary ependymal cysts of the spinal cord are rare, benign, fluid-filled cysts usually situated along the ventral surface of the spinal cord. Only 32 cases have been reported since they were first described. Thus, owing to the rarity at which these cysts are encountered, their management and pathogenesis remain controversial. Whereas some investigators have advocated for cystosubarachnoid shunt placement for symptomatic ependymal cysts, others have argued for complete cyst resection or simple fenestration. Here 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 pathological mechanism of cyst formation by performing immunohistochemistry to detect aquaporin expression in the cyst lining.
A 56-year-old female was found to harbor an enlarging cystic lesion of the conus that was discovered on workup of progressive paraparesis and urinary incontinence. She had lower extremity weakness and progressive myelopathy. Thoracic laminectomy with cyst fenestration arrested her neurologic deterioration. Pathological analysis revealed 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.
Intramedullary ependymal cysts are rare, cystic lesions of the spinal cord. Early cyst fenestration decompresses the cyst and prevents neurologic deterioration. Here we report for the first time that aquaporins are expressed in the cyst wall, which is consistent with a passive, osmotic pathogenic mechanism of cyst formation.
Intramedullary ependymal cyst: Report of a case [4]
2007, Journal de Radiologie
Spinal intramedullary ependymal cyst: A case report
1999, Surgical Neurology
BACKGROUND
Spinal intramedullary ependymal cysts are extremely rare. Only seven pathologically proven cases have been reported in the literature.
METHOD
We present an 18-month-old female with thoracic spinal intramedullary ependymal cyst that was diagnosed pathologically.
RESULTS
Histological diagnosis was made by light microscopy after immunostaining. After partially removing the cyst wall and establishing communication between the cyst and the subarachnoid space, the patient improved neurologically.
CONCLUSIONS
For spinal intramedullary ependymal cyst we recommend diagnosis by MR imaging without myelography, then enucleation of the cyst, if possible. Otherwise, we remove the cyst wall as much as possible and create adequate communication between the cyst and the subarachnoid space.
Intramedullary spinal ependymal cyst: case report
1991, Surgical Neurology

View all citing articles on Scopus

View full text

Congenital intramedullary spinal ependymal cyst

Abstract

Surg Neurol

Case records of Massachusetts General Hospital no. 46122

N Engl J Med

Intraspinal enterogenous cyst

Acta Pathol Microbiol Scand

Heterotopic glial nests in subarachnoid space: histopathological characteristics, mode of origin and relation to meningeal gliomas

J Neuropathol Exp Neurol

Intramedullary epithelial cyst of the spinal cord

Surg Neurol

Intraspinal and mediastinal foregut cyst compressing the spinal cord: report of a case

J Neurosurg

Spinal neuroepithelial cysts: report of two cases and review of literature

Acta Neurochir (Wien)

Intradural spinal cysts

Acta Neurochir (Wien)

Ependymal cyst of the thoracic spinal cord

J Neurol Neurosurg Psychiatry

Differential diagnosis of medullary and extramedullary tumours

An intraspinal enterogenous cyst

J Pathol Bacteriol

An epithelial cyst of spinal cord

Cervical arachnoid cyst

Intraspinal enterogenous cyst

Am J Dis Child

Ependymal cyst of the cervicodorsal region of the spinal cord

Arch Neurol Psychiatry

A case of intraspinal enterogenous cyst

Neurol Surg

Colloid cyst of cervical canal (abstr)

J Neurol Neurosurg Psychiatry

Neurenteric cyst in the cervical spinal canal of a 10-week old boy

Case report. J Neurosurg