Neurenteric Cyst of the Craniocervical Junction in an Infant

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.