Neurenteric Cyst of the Craniocervical Junction in an Infant -Case Report-

Abstract

A 1-year-old boy presented with an extremely rare neurenteric cyst of the craniocervical junction manifesting as weakness in all extremities, slowly progressing over 2 months with rapid deterioration during the last few days. Neurological examination on admission showed tetraparesis with hyperreflexia in the extremities. Magnetic resonance imaging demonstrated an intradural extramedullary cystic lesion located ventrally to the cord, extending from the craniocervical junction to the C3 level. The cyst wall was not enhanced with contrast medium, and the cyst content was isointense to the cerebrospinal fluid. He underwent total removal of the cyst through a left suboccipital craniectomy with C1 hemilaminectomy. The lesion had not adhered to the surrounding structures except for attachment to the left C2 nerve root outlet. Histological examination of the surgical specimen revealed typical neurenteric cyst with single layer epithelium. His symptoms improved significantly after the surgery, and he suffered no recurrence during the 2-year follow-up period. Neurenteric cyst should be considered in the differential diagnosis of intradural extramedullary cystic lesion located ventrally to the cord at the craniocervical junction in infants. Early surgical removal is recommended.

Intramedullary neurenteric cyst of the conus medullaris without associated spinal malformation: a case report and review of the literature

“…At least 50% of spinal NE cysts are associated with vertebral anomalies such as spina bifida occulta, hemivertebrae, Klippel-Feil syndrome, spinal lipoma, and syringomyelia (1,4,19). These vertebral defects are typically seen in the cervical or upper thoracic spine, (3,22) though these associated abnormalities may be absent in adults as in the present case.…”

“…They are lined with columnar and/or respiratory epithelium (3,7,17) and occur predominantly in intraspinal location, usually ventral to the spinal cord, (3,4,7,8,11,12,13,18,20) and rarely dorsal to the cord (3,20). They are usually single, occurring primarily in the lower cervical and upper thoracic regions (4,7,10,12,14,(17)(18)(19)22). Rarely, NE cysts occur in the lumbar spine (9,12).…”

“…Some patients with NE cysts present with intermittent symptoms like multiple sclerosis (3,7,10,11,17,18,20). Intermittent symptoms are probably due to a thin-walled cyst of the spinal canal (8,9,11,17,18,20) that gradually increase in size by secretion from the cyst wall and osmotic retention of CSF, and decrease in size by small ruptures in to the subarachnoid space (8,9,11,12,(18)(19)(20) and re-absorption of mucin by the cyst wall (8,9,11,18,20). Meningitis, either chemical or bacterial, is a rare presentation of NE cysts which is more common in newborns and infants (4,7,8,11,14,20).…”

“…MRI provides a complete, non-invasive assessment of the bony abnormality, the exact location and extent of the cyst, and the degree of spinal cord or nerve root compression associated with a NE cyst (6,8,11,12,(18)(19)(20)(21). The signal intensity of NE cysts varies depending on the protein content of the cyst fluid or hemorrhage within the cyst (8,20).…”

Staged bilateral far-lateral approach for bilateral cervicomedullary junction neurenteric cysts in a 10-year-old girl

“…13 To our knowledge, only 26 cases of intracranial neurenteric cysts, including the present case, have been identified in patients 18 years of age or younger (Table 1). 5,[8][9][10]12,14,16,17,20,21,[23][24][25][27][28][29][30][32][33][34] All of the pediatric cases previously reported in the literature involved children who presented with unilateral neurenteric cysts. Of the 25 patients, 10 were male and 15 were female; the patients' mean age was 7.27 years (range 1 day-18 years).…”