Pediatric Endoscopic Skull Base Surgery: Experience at a Tertiary Referral Center and Systematic Review of the Literature

Objective: The frequency of endoscopic skull base surgery in pediatric patients is increasing. This study’s aims are to describe the outcomes in a pediatric cohort ages 0 to 18 years undergoing endoscopic skull base surgery and review the pertinent literature to date.

Study Design: Retrospective cohort, systematic literature review.

Methods: A database of 1,749 patients undergoing skull base surgery at a tertiary referral center was analyzed. Following analysis, 68 patients met inclusion criteria of endoscopic skull base surgery and age ≤18 years. A systematic review of the online literature was also performed using PubMed MeSH terms and SCOPUS searches. Inclusion criteria were as follows: case series or case report, pediatric patients aged ≤18 years, endoscopic skull base surgery, and outcomes reported. Data gathered included demographics, pathology, reconstructive technique, intraoperative findings, and intraoperative and postoperative surgical complications.

Results: Analysis included 68 pediatric patients age 0 to 18 years old who underwent endoscopic (n = 61) or combined endoscopic and open (n = 7) skull base surgery between 2000 and 2018 at a tertiary care center. The average age was 11.5 (15% age 0–4, 31% age 5–12, 54% age 13–18), with 49 male and 19 female patients. Nonsellar benign tumors were the most common pathology (n = 16, 24%) followed by encephalocele (n = 15, 22%). Reconstruction of skull base defects most often utilized a nasoseptal flap (n = 25), followed by a temporalis flap (n = 3), and free mucosal graft (n = 2). All nasoseptal flaps provided 100% defect coverage, and three of the nasoseptal flaps utilized were converted from the rescue flap technique. We observed no major intraoperative or postoperative complications. Of the eight patients who underwent reoperation, three of these patients underwent late reoperation (>6 months) and five underwent early/staged reoperation.

Systematic search of the literature yielded 128 publications between 2000 and 2018. Of these, 18 case series and 6 case reports (22 total) discussing a total of 391 patients aged 0 to 18 years met inclusion criteria for final analysis. In 11 of 18 case series, a single pathology was discussed of which the majority were skull base defects causing CSF leak. The majority of skull base repairs were made with free tissue grafts, and the report of nasoseptal flap use largely came from 2 case series (n = 69 combined). The most common postoperative complication was CSF leak (n = 38). There were 9 cases of meningitis that occurred 1 month to 5 years postoperatively. Two of these episodes of meningitis resulted in death.

Conclusion: Outcomes in skull base surgery in pediatric patients have improved in recent years, making surgery a safe and effective treatment avenue. As advances in endoscopic techniques have evolved, the field has seen a corresponding increase in endoscopic skull base surgery for treatment of pediatric skull base diseases. Overall, skull base disease in the pediatric patient is a unique entity requiring individualized treatment and experience to achieve the best possible outcome.

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