A rare association between intracranial neuroenteric cyst and Klippel-Feil syndrome: a case report

Abstract

Neuroenteric cysts (NEC) are congenital malformations of endodermal origin, denoting 0.01% of all tumors, where most of these lesions have a spinal location. The authors reported a case that presents an intracranial NEC and Klippel-Feil syndrome (KFS) simultaneously. A 21-year-old male patient with clinical data related with KFS presented to the hospital with obstructive hydrocephalus secondary to an infratentorial lesion, located in the right cerebellum. An urgent ventriculoperitoneal shunt was placed and a midline suboccipital craniectomy with resection of C1 posterior arch and tumor excision was carried out, obtaining a lesion characteristic of NEC through histopathological study.

Keywords

• Neuroenteric cyst. Intracranial. Posterior fossa. Klippel-Feil syndrome. Neurosurgery.