Background: Neurenteric cysts are rare endothelium-lined structures. Two patients with symptomatic neurenteric cysts at the craniovertebral junction are presented.
Case presentation: Intermittent progression of neurologic symptoms delayed diagnosis for both patients. In one case, marked enlargement of the cyst was detected on serial imaging studies, and pathological examination of the excised lesion indicated rupture of the cyst. These cysts were totally resected by transoral or suboccipital approaches, as they were not firmly adherent to surrounding neural structures. The diagnosis of neurenteric cyst was confirmed by immunohistochemical analysis of the cyst wall.
Conclusion: In view of the clinical course of these patients, we recommend early surgical resection of neurenteric cysts located at the craniovertebral junction.