Congenital Anterior Skull Base Encephaloceles: Long-Term Outcomes After Transnasal Endoscopic Reconstruction

Background: Congenital intranasal encephaloceles (ECs) are rare malformations of the pediatric age, complex to diagnose and treat, above all if associated with genetic syndromes or concomitant dysmorphisms. The aims of the study were to report our experience in managing nasal ECS in children, to evaluate the efficacy and safety of the transnasal endoscopic repair, and to analyze in an overall way the surgical long-term outcomes. Moreover, we sought to contribute to the debate on pathogenesis of ECs, investigating possible related risk factors described in the literature. Methods: A retrospective analysis was performed of pediatric nasal ECs managed with a transnasal endoscopic approach at a tertiary referral center through clinical follow-up and telephone survey. Results: Twenty-three patients with nasal ECs fitted the criteria of the study. Mean age at surgery was 5 years (69 months) and the mean follow-up was 59 months. The clinical presentation is described in detail, with particular emphasis on syndromic cases. Of 23 patients, 17 had an isolated EC, and in 4 patients, a malformation syndrome was associated. EC recurred in 2/23 patients (8.7%) after surgical correction, necessitating a revision procedure. No perioperative complications or long-term sequelae were noted in the entire population. Conclusions: Endonasal endoscopic management of congenital ECs is feasible in children, although regular long-term follow-up is essential. Furthermore, the surgical approach does not seem to affect patients' development and quality of life, although more studies and validated questionnaires are needed. No recurrent risk factors were observed able to justify a certain etiologic relation.