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Summary: A congenital intranasal encephalocele is repaired at 23 months of age via a transnasal endoscopic approach. To the best of our knowledge, this is the youngest patient reported in the literature to be treated successfully with this approach. The technique and methods are described. This case highlights the importance of endoscopic approaches to encephaloceles resulting in less morbidity than a traditional craniotomy. Continued advances in technology and skill will increase the feasibility of transnasal endoscopic skull base surgery in younger patients. [Copyright &y& Elsevier]

Endoscopic endonasal skull base surgery (ESBS) is a well-established management strategy for anterior cranial fossa (ACF) encephaloceles in adults. However, its application to the pediatric population has not been evaluated in large-scale studies. This study systematically reviews the safety of ESBS for pediatric ACF encephaloceles. Articles reporting on pediatric patients undergoing purely ESBS for encephaloceles were reviewed from three databases (PubMed, Scopus, and Cochrane Library). Main outcomes were post-operative cerebrospinal fluid (CSF) leak and other surgical complications. Twenty-three articles provided data on 110 eligible patients with a mean age of 4.3 years (range birth – 18 years). Intraoperative CSF leaks occurred in 18.5% of children while post-operative CSF leaks developed in 6.0% of cases. Rates of meningitis and hydrocephalus were each 3.7%. Other complications included alar collapse, nasal stenosis, transient diabetes insipidus, pneumonia, and neurologic injury, collectively occurring in 7.0% of cases. The rate of death was 4.8%. The average duration of follow-up was 25.3 months. Encephaloceles recurred in 5.2% of patients. ESBS for pediatric ACF encephaloceles provides a relatively favorable risk-benefit profile, with a low rate of post-operative CSF leak, other surgical complications, and disease recurrence. [ABSTRACT FROM AUTHOR]