Craniovertebral junction lesions affect the skeleton and the enclosed neuraxis at the junction of the cranium and cervical spine. Up to 25% Chordomas occur at the base of the skull, arising from the clivus. Such lesions are rare, have a difficult anatomical access, are locally invasive and destructive in nature, and are situated in close proximity to the brainstem, thus posing a challenge for both neurosurgeons and anaesthetists.

Hence, it becomes important to formulate an anaesthesia plan, with special regards to intubation and extubation, causing minimal disturbance to the cervicovertebral junction, being prepared for effects of the tumour depending on the location of the tumour, delayed recovery of respiratory function and prolonged ventilation in the postoperative period.

Case in Brief: A 28 years old female, presenting with bilateral upper and lower limb tingling, 2 years after being operated for a clival chordoma. She also had complaints of imbalance while walking for 1 year.

The surgical management was divided in 2 stages; first a posterior occipitocervical fusion was done and then the chordoma was excised via an endoscopic endonasal transsphenoidal approach.

This patient developed cerebrospinal fluid leakage in the immediate postoperative period, which was managed by an endoscopic endonasal surgical repair.

Keywords: recurrent clival chordoma, cervical body erosion, brain stem compression, occipitocervical fixation, endoscopic endonasal skull base surgery.

1. McMaster ML, Goldstein AM, Bromley CM, Ishibe N, Parry DM. Chordoma: incidence and survival patterns in the United States, 1973-1995. Cancer Causes Control. 2001 Jan;12(1):1-11. doi: 10.1023/a:1008947301735. PMID: 11227920.
2. Tzortzidis F, Elahi F, Wright D, Natarajan SK, Sekhar LN. Patient outcome at long-term follow-up after aggressive microsurgical resection of cranial base chordomas. Neurosurgery. 2006 Aug; 59(2):230-7; discussion 230-7. doi: 10.1227/01.NEU.0000223441.51012.9D. PMID: 16883163.
3. Fraser JF, Nyquist GG, Moore N, Anand VK, Schwartz TH. Endoscopic endonasal transclival resection of chordomas: operative technique, clinical outcome, and review of the literature. J Neurosurg. 2010 May;112(5):1061-9. doi: 10.3171/2009.7.JNS081504. PMID: 19698043.
4. Mascarenhas O. Anaesthesia management in craniovertebral junctional anomalies. J Craniovertebr Junction Spine. 2016 Oct-Dec;7(4):193-196. doi: 10.4103/0974-8237.193256. PMID: 27891026; PMCID: PMC5111318.
5. Branch JC, Cunningham’s manual of practical anatomy, London Oxford press, volume 3, pages 258-295.
6. Salisbury JR, Deverell MH, Cookson MJ, Whimster WF. Three-dimensional reconstruction of human embryonic notochords: clue to the pathogenesis of chordoma. J Pathol. 1993 Sep;171(1):59-62. doi: 10.1002/path.1711710112. PMID: 8229458.
7. Singh, Neha & RAO, PARNANDI BHASKAR & Gupta, D. & Ambesh, Sushil. (2013). Anesthetic management of clival chordoma with retropharyngeal extension: Importance of imaging. Anaesthesia, Pain and Intensive Care. 17. 211-212.
8. Stippler M, Gardner PA, Snyderman CH, Carrau RL, Prevedello DM, Kassam AB. Endoscopic endonasal approach for clival chordomas. Neurosurgery. 2009 Feb;64(2):268-77; discussion 277-8. doi: 10.1227/01.NEU.0000338071.01241.E2. PMID: 19190456.
9. Förander P, Bartek J Jr, Fagerlund M, Benmaklouf H, Dodoo E, Shamikh A, Stjärne P, Mathiesen T. Multidisciplinary management of clival chordomas; long-term clinical outcome in a single-institution consecutive series. Acta Neurochir (Wien). 2017 Oct;159(10):1857-1868. doi: 10.1007/s00701-017-3266-1. Epub 2017 Jul 22. PMID: 28735379; PMCID: PMC5590026.
10. Ito E, Saito K, Okada T, Nagatani T, Nagasaka T. Long-term control of clival chordoma with initial aggressive surgical resection and gamma knife radiosurgery for recurrence. Acta Neurochir (Wien). 2010 Jan;152(1):57-67; discussion 67. doi: 10.1007/s00701-009-0535-7. PMID: 19826755.
11. James E Cottrell MD, David Smith MD, PhD. Anaesthesia and Neurosurgery.
12. Hrayer K. Shaninian, M Kabil MD, R Jarrahy MD, MP Thill MD. Endoscopic Skull Base Surgery: A Comprehensive Guide with Illustrative Cases 2008 Edition. Chapter 2, anaesthetic considerations.
13. Soltanpour P, Behnaz F, Azizi Faresani H, Seddighi A, Karimian M. Anesthetic Considerations in a Case With Massive Recurrent Clivus Chordoma. Int Clin Neurosci J [Internet]. 2019Jun.29 [cited 2021Dec.12];6(2):76-8. Available from: https://journals.sbmu.ac.ir/neuroscience/article/view/26258
14. Jellish, W. S., Murdoch, J., & Leonetti, J. P. (2002). Perioperative management of complex skull base surgery: the anesthesiologist's point of view, Neurosurgical Focus FOC, 12(5), 1-7. Retrieved Dec 12, 2021, from https://thejns.org/focus/view/journals/neurosurg-focus/12/5/foc.2002.12.5.6.xml
15. Abrams KJ, Grande CM. Airway management of the trauma patient with cervical spine injury. Current Opinion in Anesthesiology. 1994 Apr 1;7(2):184-90.
16. Bhatnagar V, Karmarkar AA, Dwivedi D, Ray A. Anesthetic management of excision of recurrent C2 chordoma with extension into posterior pharyngeal wall. J Mar Med Soc 2017;19:128-30.
17. Marda, Manish & Pandia, Mihir & Rath, Girija & Bithal, Parmod & Dash, Hari. (2013). Post-operative pulmonary complications in patients undergoing transoral odontoidectomy and posterior fixation for craniovertebral junction anomalies. Journal of anaesthesiology, clinical pharmacology. 29. 200-4. 10.4103/0970-9185.111720.
18. Sreedharan, R., & Farag, E. (2000). Airway Management in a Patient with an “Uncleared” Cervical Spine. In D. Doyle & B. Abdelmalak (Eds.), Clinical Airway Management: An Illustrated Case-Based Approach (pp. 227-231). Cambridge: Cambridge University Press.

Dr Dipti Madan

Bombay Hospital Institute of Medical Sciences, 12 New Marine Lines, Mumbai- 400020