Surgical Experience and Management of Intracranial Neurenteric Cysts: Single-Center Experience and Review of the Literature

Abstract

Background: Neurenteric cysts (NEC) are rare, congenital lesions lined by endodermal cell-derived columnar or cuboidal epithelium. Based upon previous studies, gross total removal of the capsule has been presumed to be the ideal surgical goal. Objective: This series was undertaken to further understand the risk of recurrence based on extent of capsule resection. Methods: Records were retrospectively reviewed for all patients with radiographic or pathological evidence of intracranial neurenteric cyst from 1996 to 2021. Results: A total of 8 patients were identified; Four of 8 (50%) presented with headache, four had signs of one or more cranial nerve syndromes. One patient (13%) presented with 3rd nerve palsy, one (13%) had 6th nerve palsy, and two (25%) with hemifacial spasm. One patient (13%) presented with signs of obstructive hydrocephalus. MRI demonstrated T2 hyper- or iso-intense lesions. Diffusion-weighted imaging was negative in all patients (100%) and T1 contrast-enhanced imaging demonstrated minimal rim enhancement in two patients (25%). In 3 of 8 (38%), a gross total resection (GTR) was achieved; while in 4 (50%) a near-total resection, and in one (13%) a decompression was performed. Recurrences occurred in 2 (25%) patients, one with decompression and another with near-total resection, among these ½ required repeat surgery after a mean follow-up of 77 months. Conclusion: In this series, none from GTR group demonstrated recurrence, while 40% of those receiving a less than gross total resection recurred, underpinning the importance of maximally safe resection in these patients. Overall patients did well without major morbidity from surgery.