Pediatric Endoscopic Endonasal Skull Base Surgery: The Children's Hospital of Philadelphia Experience

Objective: Few reports highlight the use of endoscopic endonasal skull base surgery (EES) in the pediatric population. The authors review the Children's Hospital of Philadelphia experience with endoscopic endonasal approaches to describe surgical outcomes and complications associated with multiple skull base pathologies.

Methods: A retrospective review of 25 pediatric patients who underwent EES at our institution from April 2012 to August 2014 was performed.

Results: A total of 25 pediatric patients underwent 27 EES with a combined neurosurgery–otorhinolaryngology team. Of the 25 patients, 14 patients (56%) were male, and the mean age at the time of surgery was 9.2 years (range, 1.25–19 years). Skull base pathologies included craniopharyngioma (n = 13), germinoma (n = 2), Rathke cleft cyst (n = 2), chordoma (n = 2), optic glioma (n = 2), encephalocele (n = 2), pituitary adenoma (n = 1), and chondromesenchymal hamartoma (n = 1). Mean follow-up period was 13.4 months (range, 0.4–39.5 months). Among the craniopharyngioma patients, gross-total resection (GTR) was achieved in 11 patients (84.6%). One patient had a near-GTR requiring postoperative radiation. Another patient required re-resection of recurrent tumor. Postoperative morbidity included new or stable anterior panhypopituitarism in all but one patient. All patients developed postoperative diabetes insipidus, and cerebrospinal fluid leak occurred in one patient (7.7%). All patients with preoperative visual dysfunction demonstrated stable or improved visual acuity postoperatively, except for one patient in whom recurrence of tumor was noted. Among the patients undergoing endonasal endoscopic biopsy for pituitary stalk lesions consistent with germinoma, one patient required repeat endonasal biopsy due to initial inconclusive pathology. All patients with Rathke cleft cysts, pituitary adenomas, and nasal encephaloceles underwent GTR with no postoperative morbidities. Two patients with clival chordomas underwent initial endonasal endoscopic biopsy. One patient underwent further debulking of tumor resulting in postoperative panhypopituitarism and diabetes insipidus. Two patients without neurofibromatosis1 underwent endonasal endoscopic biopsy for optic pathway glioma, with pathology consistent with pilocytic astrocytoma. One of these patients developed mild diabetes insipidus. Uncomplicated chondromesenchymal hamartoma resection resulted in near-GTR in one patient.

Conclusion: Endoscopic endonasal surgery and/or biopsy is a safe and valuable approach for obtaining a diagnosis and treating a variety of pediatric skull base pathologies. Further studies are needed to characterize the long-term surgical and clinical outcomes of pediatric patients treated with this surgical approach.