Applications of the Endoscopic Endonasal Approach in Early Childhood

Introduction: Endoscopic endonasal surgery (EES) has been slower to gain popularity in early childhood due to multiple concerns such as a smaller nasal aperture, poor sinus pneumatization, tighter anatomical intracranial corridors, and reconstructive challenges.

Methods: All patients up to age six who underwent EES at a large Cranial Base Center from 2002 to 2019 were retrospectively reviewed. Demographic information, pathology, operative characteristics, complications, radiographic outcomes, and long-term outcomes were assessed.

Results: Thirty-six patients underwent EES before age seven. Four patients had two-stage EES. Two patients required combined cranial and endonasal approaches. Mean age at the time of initial surgery was 4 years (range: 1–6). Twenty patients were male and 16 were female. There were ten different pathologies, of which encephaloceles/traumatic CSF leak (12), craniopharyngioma (11), dermoid tumor (3), chordoma (3), and rhabdomyosarcoma (2) were most common. Of 21 tumors intended for resection, 11 patients had gross total resections and 10 had near total (>95%) resections. Nine patients had recurrences, of which six were craniopharyngiomas (p = 0.01). One chordoma patient and one rhabdomyosarcoma patient died from tumor recurrence during follow-up. There was no difference in recurrence rates based on degree of resection (p = 0.67). For craniopharyngiomas, two of five gross total resections recurred and four of six near total resections recurred (p = 0.57). Five patients underwent repeat EES for tumor recurrences; two craniopharyngiomas, one chordoma, and one rhabdomyosarcoma. Four craniopharyngiomas and one rhabdomyosarcoma had gamma knife radiosurgery for recurrence. Sixteen patients had unilateral nasoseptal flaps raised. Two patients had vascularized flaps elevated from other sources (one temporalis and one rhinopharyngeal). Eight patients had free mucosal grafts. Ten patients had lumbar drains placed, one had an external ventricular drain, and four patients had shunts placed prior to EES. Three cerebrospinal fluid (CSF) leaks occurred following primary EES (8% rate) and one occurred following surgery for recurrence. All CSF leaks were managed with revision surgery. Following an increase in nasoseptal flap usage (31–52%) and CSF diversion (15–39%) in 2008, there was only one CSF leak out of 23 patients (4 vs. 15%; p = 0.54). There was only one permanent new cranial neuropathy (rhabdomyosarcoma developed a partial third nerve palsy after a second surgery for recurrence). One craniopharyngioma patient developed a permanent field cut postoperatively. Six patients developed permanent postoperative panhypopituitarism, of which all were craniopharyngiomas (p < 0.001). The other craniopharyngioma patients had preoperative panhypopituitarism. One patient required an emergent craniotomy for an epidural hematoma due to a pin site skull fracture. No major vascular injuries occurred. There were no operative mortalities. Mean follow-up was 64 months.

Conclusion: EES can be performed safely under the age of 7 for a variety of skull base pathologies. Nasoseptal flaps are technically feasible and may reduce CSF leak rates. Craniopharyngioma remains a challenging subgroup frequently requiring multiple interventions for tumor control, but patients in this group have competitive outcomes in comparison to adults and superior rates of >95% resection compared with adult data previously published from our center.