Combined Transoral, Transnasal Endoscopic Surgery with Immediate Craniocervical Fusion for Giant Chordomas of the Clivus and Craniocervical Junction—State of the Art Team Work

Introduction: Chordoma is a rare aggressive, locally invasive tumor, arising from remnants of notochord. Complete surgical resection followed by radiation offers the best chance of long-term local control and survival. Treatment of clival chordomas is exceptional with emphasis on vascular protection and preservation of neurological function, typified by a concept of maximally aggressive safe surgery and advanced radiation techniques, such as proton beam. The craniocervical stability should be addressed during surgery of giant tumors.

Methods: Review of the operative technique and surgical results of giant clival chordomas requiring combined trans-oral, trans-nasal endoscopic surgery with immediate craniocervical fusion in the past 2 years.

Results: Three giant clival/craniocervical junction chordoma cases underwent a combined trans-oral, trans-nasal endoscopic surgery with immediate posterior craniocervical fusion. There were one male and two females in the ages of 30, 16, and 17 years. Surgery was staged as required and lasted up to a total of 30 hours in 3 days. Complete surgical excision was achieved in all with no vascular sequela. A hypoglossal nerve, involved by the tumor in two cases, was damaged. There were no other neurological deficits. The 6 to 8 cm skull base defect was reconstructed using multilayer closure with one post-operative CSF leak managed conservatively. Craniocervical fusion was uneventful in all cases, demanding 3 months of fixation in a rigid collar. The third, most complicated and demanding case, will be presented.

Conclusion: Endoscopic surgery of giant clival/craniocervical junction chordomas is very challenging but feasible. It should be performed by an experienced endoscopic skull base team.