Surgical Outcomes in Intracranial Neurenteric Cysts—Retrospective Review of a 15-Year Institutional Experience

Background: Intracranial neurenteric cysts are rare congenital lesions of endodermal origin, estimated to comprise only 0.35% of intracranial lesions.³ They often present with headache, hydrocephalus, and cranial nerve deficits,¹ and can have variable radiographic appearance on magnetic resonance imaging.² The current treatment recommendation is for complete surgical resection when possible.³ However, because of their rarity there is a lack of modern surgical experience with these lesions in the literature.

Objective: The goal of this study was to review our institutional experience with the surgical treatment of intracranial neurenteric cysts.

Methods: This retrospective study was approved by the institutional review board. A database search was performed to identify all histologically confirmed cases of intracranial neurenteric cysts between 2000 and 2014. Demographic, clinical, and radiographic information were obtained. Operative reports, postoperative imaging, and clinical follow-up information were also reviewed to identify the surgical approach, extent of cyst wall resection, postoperative complications, and cyst recurrence.

Results: A total of six cases of intracranial neurenteric cyst were surgically treated over a 13-year period. Median patient age was 39.6 years (range, 10–71 years). One patient was male. Cyst locations included the third ventricle (one), the cerebellopontine angle (one), and the prepontine/premedullary cisterns (four). Radiographically, the cysts ranged from hypo- to hyperdense on computed tomography. On magnetic resonance imaging, the cysts were T1 hypointense (three) and hyperintense (three). On T2 and FLAIR sequences, there were both hyper- and hypointense cysts. Two cysts had a thin rim of enhancement. No cyst demonstrated diffusion restriction. The differential diagnosis considered in these cases varied, but in the posterior fossa it included arachnoid cyst, epidermoid cyst, and racemose neurocysticercosis (in one case). The third ventricular lesion was preoperatively thought to be a craniopharyngioma, colloid cyst, or cystic glia tumor. This cyst wall was subtotally resected via a transcortical, transventricular approach, with no cyst recurrence at 16-month follow-up. The five posterior fossa cysts were approached through a far lateral, transcondylar craniotomy. In one case, a residual cyst wall was left behind. Gross total resection by postoperative MRI was achieved in four cases. Postoperative complications included aseptic meningitis (one), abducens palsy (one), and pseudomeningocele requiring reoperation (three). Mean follow-up time was 6.5 months (range, 0–16 months). There was no imaging evidence of cyst recurrence in three cases for which follow-up imaging was available.

Conclusion: Intracranial neurenteric cysts are rare but should be considered in the differential for cystic posterior fossa lesions. In many cases, complete surgical resection is possible—often via a far lateral, transcondylar approach given their frequent location ventral to the pons and medulla.

Referenzen

References

1 Gauden AJ, Khurana VG, Tsui AE, Kaye AH. Intracranial neuroenteric cysts: a concise review including an illustrative patient. J Clin Neurosci 2012;19(3):352–359

2 Preece MT, Osborn AG, Chin SS, Smirniotopoulos JG. Intracranial neurenteric cysts: imaging and pathology spectrum. AJNR Am J Neuroradiol 2006;27(6):1211–1216

3 Wang L, Zhang J, Wu Z, et al. Diagnosis and management of adult intracranial neurenteric cysts. Neurosurgery 2011;68(1):44–52, discussion 52