Modified Transoral Transpalatal Approach with Tailored Resection of Hard Palate and Vomer for Clival Chordoma: A Technical Report

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1 Modified Transoral Transpalatal pproach with Tailored Resection of Hard Palate /jp-journals and Vomer for lival hordoma ase Report Modified Transoral Transpalatal pproach with Tailored Resection of Hard Palate and Vomer for lival hordoma: Technical Report 1 Vishwas H Vijayendra, 2 Komal P handrachari, 3 Kiran Mariswamappa, 4 Moni Kuriakose STRT hordomas are rare locally aggressive midline tumors usually seen involving clivus and craniovertebral junction. Safe maximal resection is the mainstay of treatment. We report a case of a young girl with chordoma where a transoral approach was used with limited resection of hard palate and vomer for optimal exposure and maximal safe resection. Good exposure for radical decompression and reduced postoperative morbidity can be achieved using individualized modifications in transoral approach for chordomas involving craniovertebral junction. Keywords: hordoma, raniovertebral junction, Transoral approach. How to cite this article: Vijayendra VH, handrachari KP, Mariswamappa K, Kuriakose M. Modified Transoral Transpalatal pproach with Tailored Resection of Hard Palate and Vomer for lival hordoma: Technical Report. J Spinal Surg 2018;5(1): Source of support: Nil onflict of interest: None INTRODUTION hordomas are rare, slow-growing malignant bony tumors that arise from remnants of the notochord and account for approximately 1% of intracranial tumors. 1 pproximately 35 to 40% of these tumors occur in the skull base, where they typically involve the clivus. 2 Even though they show benign histological behavior, the prognosis is poor due to their deep location, the presence of nearby important structures, and their propensity to invade locally. lso, the very high recurrence rates make it a challenge for the surgeons to achieve 1 onsultant, 2-4 Senior onsultant 1-3 Department of Neurosurgery, Mazumdar Shaw Medical enter Narayana Multispeciality Hospital, engaluru, Karnataka, India 4 Department of Head and Neck Surgery, Mazumdar Shaw Medical enter, Narayana Multispeciality Hospital, engaluru Karnataka, India orresponding uthor: Komal P handrachari, Senior onsultant, Department of Neurosurgery, Mazumdar Shaw Medical enter, Narayana Multispeciality Hospital, engaluru Karnataka, India, drkomalprasad@gmail.com total resection. 3,4 They are conventionally managed by transoral approaches with palatal splitting. 5 If the tumor is extending superiorly, transfacial approach with or without maxillotomy is often needed, resulting in increased morbidity. Most of the time, a combination of operations with various surgical approaches is required. We describe a novel modification for avoiding palatal splitting and thus reducing the morbidities associated with it. SE REPORT 11-year-old girl presented with neck pain, radiating right upper limb pain, progressive weakness of both upper limbs, nasal twang in speech along with difficulty in mixing bolus of food in the mouth. Neurological examination revealed bilateral hypoglossal involvement, wasting of intrinsic muscles of hand, bilateral upper limb weakness, brisk reflexes, and right 5-8 hypoesthesia. Magnetic resonance imaging (MRI) revealed T1 hypointense, T2 hyperintense, heterogeneously enhancing solid clival lesion measuring cm (anteroposterior transverse craniocaudal) compressing on the medulla and upper cervical spinal cord (Fig. 1). These features were suggestive of chordoma. 1 We decided to perform a combination of surgical approaches in a staged manner. In view of her rapid worsening due to cord compression, she initially underwent a posterior approach 1, 2 laminectomy, and decompression of lesion. She showed immediate postoperative clinical improvement in her right upper limb power. Histopathological examination confirmed the diagnosis of chordoma. second surgery, the definitive anterior approach, had to be planned. Traditional transoral approach would not have provided the adequate exposure required. n added maxillotomy would do so but with a high risk of significant postoperative morbidity, such as swallowing difficulty and change in voice. Hence, a modified transoral approach was planned, with tailored resection of posterior hard palate and vomer to provide adequate exposure, which would also reduce the risk of postoperative morbidity (Fig. 2). The Journal of Spinal Surgery, January-March 2018;5(1):

2 Vishwas H Vijayendra et al Figs 1 to : Preoperative MRI images. () T1-weighted sagittal images showing hypointense tumor. The tumor has not only invaded the lower clivus and upper dens but also surrounded the 1 and 2 vertebral bodies. The medulla oblongata and upper cervical cord are compressed posteriorly. (, ) Gadolinium-enhanced contrast studies in axial and sagittal section showing heterogeneously enhancing mass Surgical Technique: Tailored Resection of Hard Palate and Vomer Under general anesthesia, with the patient in supine position, mouth was kept open with retractors. Palatal mucosa was incised and pushed laterally. Posterior hard palate and vomer were excised piecemeal using rongeurs and drill to get a panoramic view of superior aspect of the posterior pharyngeal wall (Fig. 2). Posterior pharyngeal wall was incised. On either side of the apical ligament, a grayish white mass was seen. The tumor was removed piecemeal. It was grayish white, firm in consistency, and moderately vascular. The tumor was seen extending posterior to odontoid process. The tip of the odontoid process was drilled out to reach the posterior component. Piecemeal resection of the tumor was done rostrally up to clivus, posteriorly till the dural tube was seen and inferiorly till the body of 2 vertebra (Fig. 3). Gross total resection of the lesion was achieved. Dura mater was intact. 44 Postoperative imaging revealed no residual lesion (Fig. 4). There was resolution of lower cranial nerve symptoms and improvement in the right upper limb power. She did not receive any radiotherapy. Follow-Up The patient had progression in the size of the tumor after 16 months and underwent posterior approach and decompression. Thirty months after the transoral procedure, she underwent another reexploration posterior decompression for progressive myelopathy and recurrence. She is on regular follow-up for over 5 years now, asymptomatic, and is going to school. DISUSSION hordomas are relatively rare, slow growing, locally aggressive tumors, originating from embryonic remnants of the notochord. 1 These lesions are deep and in midline and most commonly seen in the sacrum and skull base/

3 Modified Transoral Transpalatal pproach with Tailored Resection of Hard Palate and Vomer for lival hordoma Figs 2 to D: Transoral modification: schematic and intraoperative images. (, ) Mucosal incision and flap elevation. () Planned area of hard palate removal. (D) Highlighted area showing the tailored posterior vomer and hard palate removed D Figs 3 to : Intraoperative images. () Incision of posterior pharyngeal wall. () Piecemeal excision of the grayish white, firm tumor. () Dura and the dens after tumor excision The Journal of Spinal Surgery, January-March 2018;5(1):

4 Vishwas H Vijayendra et al Figs 4 to D: Postoperative imaging. () T1-weighted sagittal postoperative image showing good decompression of lower medulla and spinal cord. () Postgadolinium enhanced contrast imaging showing no residual tumor. () T2-weighted axial image showing opened cisternal spaces and decompressed medulla. (D) Sagittal computed tomographic image showing maintained stability D clivus region. They often reach very large sizes. They often require multiple surgical procedures; even then, multiple local recurrences are common. 3,4,6 urrent evidence indicates that initial radical surgical removal supplemented by proton beam radiotherapy is the best mode of management of these lesions. 7 The chordoma in our patient involved the clivus, extended anteriorly into perivertebral space and inferiorly up to the 2 vertebral body. Initially, posterior approach for tumor resection was preferred, as it would relieve rapidly worsening medullary and cord 46 compression symptoms. There was still a significant residual mass remaining. To tackle it, a modified transoral approach was used with limited resection of hard palate and vomer. This provided adequate surgical field to reach the entirety of the tumor and resect it. Thus, we were able to restrict the postoperative morbidity which might have been incurred in cases of more radical approaches. We would like to emphasize that surgical procedures for chordomas should be planned on a case-to-case basis and minor modifications of procedures will not only

5 Modified Transoral Transpalatal pproach with Tailored Resection of Hard Palate and Vomer for lival hordoma provide adequate surgical field for tumor excision but also spare the patient of debilitating postoperative morbidities. REFERENES 1. Erdem E, ngtuaco E, Van-Hemert R, Park JS, l-mefty O. omprehensive review of intracranial chordoma. Radiographics 2003 Jul-ug;23(4): hugh R, Tawbi H, Lucas D, iermann J, Schuetze S, aker L. hordoma: the nonsarcoma primary bone tumor. Oncologist 2007 Nov;12(11): Fischbein N, Kaplan M, Holliday R, Dillon W. Recurrence of clival chordoma along the surgical pathway. m J Neuroradiol 2000 Mar;21(3): Tzortzidis F, Elahi F, Wright D. Patient outcome at longterm follow-up after aggressive microsurgical resection of cranial base chordomas. Neurosurgery 2006 ug;59(2): Hagihara N, Matsushima T, Kawashima M, Hikita T. The 2 ganglion sectioning epidural approach to craniocervical junction chordoma: a technical case report. Neurol India 2012 Nov-Dec;60(6): oriani S, andiera S, iagini R. hordoma of the mobile spine: fifty years of experience. Spine (Phila Pa 1976) 2006 Feb;31(4): Gaki H, Tokuuye K, Okumura T. linical results of proton beam therapy for skull base chordoma. Int J Radiat Oncol 2004 Nov;60(4): The Journal of Spinal Surgery, January-March 2018;5(1):