Unusual Presentation of Bilateral Neurenteric Cysts in the Craniovertebral Junction in a 10-Year-Old Girl: Case Illustration and Review

Introduction: Intracranial neurenteric cysts are rare, benign lesions in the pediatric population consisting of ectopic alimentary tissue residing in the central nervous system. These lesions are typically unilateral cysts located in the posterior fossa and craniocervical junction.

Methods: We report a 10-year-old girl with bilateral intracranial neurenteric cyst at the pontomedullary junction, which was resected via a staged, bilateral far-lateral transcondylar approach. Near-total resection was achieved with a small remnant of cyst wall adherent to the brainstem. The patient was neurologically intact without craniocervical instability. To our knowledge, this is the first reported case of a bilateral presentation of an intracranial neurenteric cyst at the skull base. We also present a brief literature review of intracranial neurenteric cysts described in pediatric patients.

Results: To date, only 26 cases of intracranial neurenteric cysts, including the present case, have been identified in patients younger than 18 years of age. All of the previous cases in the literature included children who presented with unilateral neurenteric cysts, 68% of which represented posterior fossa cysts. Mean age was 7.27 years. MRI and histopathology confirmed the diagnosis. Surgical resection is the current treatment of choice.

Conclusions: A bilateral presentation of intracranial neurenteric cysts, although rare, may occur in children. Surgical resection of these lesions can be safely achieved via a staged, bilateral far-lateral transcondylar approach. Complete excision of the neurenteric cyst is the goal of surgical intervention given the unknown rate of recurrence. Follow-up is crucial in these patients, particularly in cases where complete resection cannot be achieved.