The endoscopic endonasal approach for pediatric craniopharyngiomas: the key lessons learned

The aim of this study is to highlight the role of the endoscopic endonasal approach (EEA) in pediatric craniopharyngiomas by reviewing our experience and the key lessons learned from the application of this approach in children.

Between 1998 and 2017, 12 pediatric craniopharyngiomas were treated via EEA at our institution. Demographic data, preoperative assessment, tumor features, surgical results, complications, and recurrences were analyzed.

Visual defects were the most frequent presenting sign. Seven craniopharyngiomas were infradiaphragmatic, and five were supradiaphragmatic. The EEA was successfully performed in all cases with no complication related to children's sinonasal anatomy. Gross total resection (GTR) rate was of 75%. Endocrinological disturbances improved in one case (20%) and worsened in three (60%). New onset of diabetes insipidus was observed in four (36%) children. Visual defect improved in 91% of cases, with no new postoperative deficit. Postoperative cerebrospinal fluid (CSF) leak occurred in one patient (8%). Three patients (27%) experienced tumor regrowth, and one craniopharyngioma recurred (mean follow-up, 78 months).

The EEA offers a straight route to the sellar-suprasellar, making it the ideal approach for pediatric infradiaphragmatic craniopharyngiomas. In supradiaphragmatic craniopharyngiomas, the extended EEA provides a clearer and close-up visualization of the tumor-hypothalamus interface, which can grant better results in terms of quality of life. The pediatric skull base anatomy should not represent a contraindication for the endoscopic technique. Larger series encompassing a wider spectrum of pediatric craniopharyngiomas are needed to further support the benefits of this surgical approach.