Neurenteric cyst of the lower clivus

Neurenteric cysts (NCs) typically arise as benign ventral intradural extramedullary developmental malformations of the spine which contain heterotopic epithelium resembling the intestinal or respiratory tracts. Intracerebral NCs are extremely rare, though the frequency of symptomatic reports and incidental findings is increasing, perhaps because of advances in neuroimaging. Recognition of the unique radiographic and histopathologic features of this entity is of growing importance in the treatment of cysts of the neural axis. We present an unusual case of an NC arising at the lower clivus.

A 58-year-old man presented with occipitalgia, diplopia, a bilateral hearing deficit, and mild dysphagia. Computed tomography and magnetic resonance imaging demonstrated a 5 x 2 x 3-cm extra-axial cystic midline mass anterior to the brainstem at the lower clivus with posterior cyst wall enhancement.

The patient underwent a left lateral suboccipital total macroscopic resection of the lesion. Microscopic examination and histopathologic findings were consistent with a diagnosis of NC.

We describe the clinical presentation, imaging, and histopathologic characteristics, and discuss the diagnosis and surgical treatment of this rare lesion and related pathologic entities. Because of the remote possibility of delayed recurrence, even in cases of apparent total cyst wall removal, long-term serial imaging and a consideration of reoperation for recurrences is advisable.