Giant Craniospinal Intramedullary Neurenteric Cyst in Infant-Case Report and Review of Literature

BACKGROUND: Neurenteric cysts are rare congenital lesions derived from an anomalous connection between the primitive ectoderm and endoderm. CASE DESCRIPTION: A 2-month-old infant presented with an insidious onset of upper limb weakness. Magnetic resonance imaging of the spine revealed a contrast-enhancing solid mass located anterior to the spinal cord at the level of the cervicothoracic junction, which was isointense on T1-weighted images and hyperintense on T2-weighted images. The lesion was associated with marked spinal cord compression and also with a giant intramedullary cyst that extended proximally into the cranium. Spinal dysraphism was noted. The patient underwent cervicothoracic laminotomy, drainage of the cyst contents, and a complete excision of the solid component. Neurologic function recovery was observed postoperatively. CONCLUSIONS: We report and discuss the clinical presentation, pathogenesis, and neuroradiologic findings in an infantile case of a giant craniospinal intramedullary neurenteric cyst. Early recognition is beneficial because of its benign nature and good clinical outcome following total surgical resection.