NEURENTERIC CYST OF THE CRANIOVERTEBRAL JUNCTION

Neurenteric cysts are rare malformations that lead to brain stem and spinal cord compression. These cysts are derived from endoderm that is fused with the developing notochord during the third week of gestation. A 3-year-old boy presented with a 2-month history of torticollis and had developed paraparesis 2 weeks before admission. Examination showed that he was quadriparetic. Plain radiography films revealed widening of the cervical canal and pedicle erosion, while a hypointense lesion on T1, at the craniovertebral junction, with a compression effect on the anterior aspect of the brain stem and spinal cord were noted on magnetic resonance imaging. C1 to C3 laminectomy as well as a suboccipital craniectomy were performed; upon opening the dura, a cystic swelling was found to be protruding. Fluid was aspirated from the cyst and after shrinkage, the cyst was removed completely. On the first day after the surgery, the patient’s motor power improved significantly. This report describes a rare form of neurenteric cyst in terms of both its location and unusual presentation (torticollis).