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Table of Contents    
CASE REPORT
Year : 2012  |  Volume : 60  |  Issue : 6  |  Page : 638-642

The C2 ganglion sectioning epidural approach to craniocervical junction chordoma: A technical case report


1 Department of Neurosurgery, Faculty of Medicine, Saga University 5-5-1 Nabeshima, Saga, Japan
2 Department of Neurosurgery, Hamanomachi Hospital, 3-5-27 Maizuru, Chuo ku, Fukuoka, Japan

Date of Submission17-Jul-2012
Date of Decision05-Aug-2012
Date of Acceptance19-Nov-2012
Date of Web Publication29-Dec-2012

Correspondence Address:
Toshio Matsushima
Department of Neurosurgery, Faculty of Medicine, Saga University 5-5-1 Nabeshima, Saga
Japan
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0028-3886.105201

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 » Abstract 

In chordoma, complete surgical removal of the epidural tumor should be the first choice of treatment. Numerous surgical approaches to clival chordoma have been described: anterior approaches, lateral approaches, and posterolateral approaches. A multistaged operation with a combination of these approaches is generally performed. We used three approaches to remove a clival chordoma extending from the lower clivus anteriorly to the anterior perivertebral space and inferiorly to the C2 level. The epidural posterolateral approach through the vertebral artery (VA)-C2 interval space after resection of the C2 dorsal ganglion was the most effective. To our knowledge, the epidural posterolateral approach below VA, referred to as C2 ganglion sectioning epidural approach has not been reported as an independent approach in detail. We report a two-year-old girl with a lower clival chordoma which has been excised using C2 ganglion sectioning epidural approach.


Keywords: C2 ganglion sectioning epidural approach, craniocervical junction, lower clival chordoma


How to cite this article:
Hagihara N, Matsushima T, Kawashima M, Hikita T. The C2 ganglion sectioning epidural approach to craniocervical junction chordoma: A technical case report. Neurol India 2012;60:638-42

How to cite this URL:
Hagihara N, Matsushima T, Kawashima M, Hikita T. The C2 ganglion sectioning epidural approach to craniocervical junction chordoma: A technical case report. Neurol India [serial online] 2012 [cited 2020 Dec 5];60:638-42. Available from: https://www.neurologyindia.com/text.asp?2012/60/6/638/105201



 » Introduction Top


Chordomas grow slowly, rarely metastasize, and show relatively benign histological behavior. However, clival chordomas have poor prognosis because of their critical deep location and propensity to locally invade surrounding structures, which limits surgical access. [1],[2],[3],[4] These tumors have a high recurrence rate even after the tumor has been surgically resected. The chordomas originate from remnants of the notochord and are essentially epidural lesions. [5],[6] Once chordoma infiltrates and violates the dura, it may rapidly lead to a fatal course. Therefore, epidural removal of the chordoma should be the first choice of treatment. [7] Although numerous surgical approaches have been described, no single surgical approach has emerged as a standard therapy for the removal of clival chordoma. A combination of operations with various surgical approaches are performed. [8],[9],[10],[11],[12] We report a very useful approach for lower clival chordoma that extends inferiorly to the spinal canal at the C2 level.


 » Case Report Top


A two-year-old girl presented with a history of headache over the preceding one month. Neurological examination revealed mild atrophy of the left half of her tongue. Magnetic resonance imaging (MRI) showed a large mass lesion measuring 5 cm × 4 cm × 5 cm involving the lower clivus and extending anteriorly to the anterior perivertebral space and inferiorly to C2. It compressed not only the medulla oblongata but also the upper cervical cord posteriorly. The tumor showed hypointensity on T1-weighted sequence and hyperintensity on T2-weighted sequence. Tumor was solid and not enhanced by gadolinium [Figure 1]a. These features were suggestive of chordoma. We decided to perform a combination of surgical approaches. At first, the transoral approach was performed to resect the tumor in the perivertebral space in front of the vertebral bodies. Pathological diagnosis was chordoma. However, there was still significant compression of the medulla and upper cervical cord [Figure 1]b. While we considered an additional operation, she developed acute right hemiparesis. Because the tumor involving the lower clivus extended inferiorly to the level of C2 [Figure 2], we finally decided that the epidural approach for looking up the clival chordoma from below the vertebral artery (VA) was the best. We planned to use the epidural posterolateral approach to the lower clivus through the VA-C2 interval space after resection of the C2 dorsal ganglion as the second operation. This C2 ganglion sectioning epidural approach gave a sufficient operative field and was very useful in this case.
Figure 1: Preoperative sagittal T2-weighted MRI (a) showing a large tumor measuring 5 cm × 4 cm × 5 cm. The tumor has not only invaded the lower clivus but also surrounded the C1 and C2 vertebral bodies. The medulla oblongata and upper cervical cord are compressed posteriorly. Sagittal T2-weighted MRI after the first operation that used the transoral approach (b) showing significant compression of the medulla and upper cervical cord by the residual large tumor, although the tumor in the anterior perivertebral space in front of the vertebral body was resected

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Figure 2: Sagittal (a) and axial (b, c) T2-weighted MRIs before the second operation that used the left C2 ganglion sectioning epidural approach. The MRIs revealed regrowth of the tumor. The tumor occupied the lower clivus and extended inferiorly to the level of C2. Compression on the medulla oblongata and the upper cervical cord was remarkable. The tumor can be seen extending to the parapharyngeal space (b) and the left hypoglossal canal (c)

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Surgical technique

C2 ganglion sectioning epidural approach (the second operation)

Under general anesthesia with the patient in the prone position, a left hockey stick skin incision and left suboccipital craniotomy over the midline were performed. In addition to the opening of the foramen magnum, the posterior edge of the left occipital bone was sufficiently drilled in the same manner as the far lateral approach. Taking care of left VA, C1 laminectomy and C2 partial laminectomy were performed [Figure 3]a and VA was not mobilized. A part of the epidural tumor was found between the C1 and C2 laminae, which was semitransparent white and solid. C2 dorsal ganglion was raised by the tumor [Figure 3]b. Although we approached from the epidural space between the left VA and C2 lamina, C2 dorsal ganglion was an obstacle. We resected and shifted C2 dorsal ganglion to obtain sufficient surgical space [Figure 3]c. We then looked up the epidural tumor at the craniocervical junction and removed it in pieces without opening the dura mater. Although the tumor was very hard and apt to bleed from the venous plexus of the ligaments, we mainly removed the left side of the tumor in the sufficient operating field [Figure 3]d. Finally, we could clearly see the tectorial membrane anterior to the dura mater of spinal cord and the bony defect of lower clivus. After the second operation, right hemiparesis disappeared and there were no new neurological deficits.
Figure 3: Intraoperative photographs and illustrations of the left C2 ganglion sectioning epidural approach. (a) Midline suboccipital craniotomy, C1 laminectomy, and C2 partial laminectomy were performed. (b) A part of the epidural chordoma can be seen in the epidural space anterolateral to the dura mater after the C2 dorsal ganglion was shifted by the spatula. (c) The C2 dorsal ganglion was incised because it was an obstacle for the approach through the epidural space between the left VA and C2 lamina. The chordoma was removed through the space. (d) Resection of the C2 dorsal ganglion allowed creation of enough surgical space around the lower clivus and provided the ability to look up the tumor and resect without opening the dura mater

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Third operation and postoperative course

Eight months after the second operation, we performed the right C2 ganglion sectioning epidural approach as mentioned in the second operation to remove the residual tumor on the right side of craniocervical junction. A postoperative MRI revealed that most of the tumor existing in the upper spinal canal and involving the lower clivus was sufficiently removed [Figure 4]. Later, cyber knife radiotherapy was given. Recent MRI revealed small residual tumor in the left hypoglossal canal. At seven-year follow-up there is no recurrence of tumor and clinically the patient has only left hypoglossal nerve palsy [Figure 5]. During further follow-up if there is growth of the residual tumor in the left hypoglossal canal, she may require another surgery.
Figure 4: Sagittal (a) and axial (b, c) MRIs after the third operation that used the right C2 ganglion sectioning epidural approach showing successful resection of most of the tumor. The medulla oblongata and the upper cervical spine were decompressed. Residual tumor was present at the parapharyngeal space (b) and the left hypoglossal canal (c)

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Figure 5: Sagittal (a) and axial (b, c) MRIs acquired 7 years after postoperative radiation therapy showing the disappearance of the tumor except for residual tumor in the left hypoglossal canal (c)

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 » Discussion Top


Chordomas originate from remnants of notochord and their location is epidural. [5],[6] Because of this location the clinical course of the disease can be aggressive. [1],[2],[3],[4] These lesions are deep and in midline. Often, by the time the diagnosis is made these lesions are large in size. These factors make it difficult to remove the lesion completely through a single surgical approach. The published data suggests that the initial optimal treatment approach would be surgical removal supplemented with radiotherapy. [13],[14],[15],[16] In the recent reported case series endoscopic endonasal surgery has been shown to be beneficial in small chordomas. [17],[18] However, some limitations exist, particularly for clival chordomas extending too far laterally beyond the width-limiting cavernous carotid arteries and inferiorly to the craniovertebral junction, as in our patient. [19],[20]

For the chordoma at the craniovertebral junction, posterolateral approaches, such as the lateral transcondylar and extreme far lateral approaches, are often used. [21],[22],[23],[24],[25],[26],[27],[28] The posterolateral approaches include opening the foramen magnum, drilling part of the occipital condyle, laminectomy, and mobilizing VA. Using these approaches, tumors have been removed not only epidurally but also intradurally after opening the dura mater. It is very important to remove the chordomas epidurally, if possible, because a residual tumor may rapidly invade intradurally, even when the dura mater is closed after intradural manipulation. [7]

The chordoma in our patient not only involved the clivus but also extended anteriorly to the anterior perivertebral space and inferiorly to C2 level. Although the transoral approach was performed first, a large mass still remained, which compressed the medulla oblongata and upper cervical cord. We considered a different approach as an additional operation, because the patient was only two years old and we did not want to injure the atlanto-occipital joint. We were willing to remove the tumor epidurally and eventually decided on an acceptable posterolateral epidural approach for our patient. As for our cadaver research, when approaching the lower clivus from below VA epidurally, C2 dorsal ganglion was an obstacle. This suggested that resection of C2 dorsal ganglion would gain some surgical space to the lower clivus [Figure 6]. As expected, we were able to obtain sufficient surgical space between the VA and C2 lamina after resection of C2 dorsal ganglion and retraction of VA.
Figure 6: The extradural anatomy at the anterior portion of the craniovertebral junction. (a) The posterior arches of C1 and C2 have been removed to expose the foramen magnum region. Left VA penetrates the dura at the level of the atlanto-occipital joint and ascends through the foramen magnum in front of the dentate ligaments and the 11th cranial nerve. Dorsal roots of the C2 nerve arise from the lateral side of the medulla oblongata and form the ganglion below the C1 epidurally. (b) The lower part of the medulla oblongata and the upper part of the cervical spinal cord have been removed to expose the anterior aspect of the craniovertebral junction. The dura is situated in front of the spinal cord. The vertebral venous plexus can be seen in the dura. (c) The dura with the venous plexus was removed. The tectorial membrane, which extends downward from the clivus to insert onto the upper cervical vertebrae can be seen. In addition to C1 laminectomy, a wide epidural space to the lower clivus without any obstacles (arrow) can be obtained by resection of the C2 ganglion

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Our approach was slightly different from the posterolateral approaches previously reported. It was the only epidural approach below VA that did not involve opening of the dura mater and we were able to look up the clival tumor from below VA and reach middle clivus without any obstacles. In this case, the VA was skeletonized partially and we did not approach lower clivus from above VA. The tumor was visible in the space between C1 and C2. Fortunately, by resection of the C2 dorsal ganglion, we were able to reach with ease the lower clivus and resect the tumor deeply. But we consider that we could remove the tumor through this approach because the tumor itself had opened a space by displacing the brainstem-upper cervical cord. Moreover, there were no complications such as instability of the craniovertebral junction because of partial removal of the occipital condyle and no necessity for fixation of C1-C2. Although there were also no postoperative neurological deficits caused by the sacrifice of the C2 dorsal ganglion, we should consider the necessity of restoration of the anatomical continuity by resuturing the sectioned C2 root.

To the best of our knowledge, there are no reports on epidural approaches below VA after resection of C2 dorsal ganglion as an independent single approach. We would like to emphasize the usefulness of C2 ganglion sectioning epidural approach for craniocervical chordoma. This skull-base approach is neither aggressive nor difficult but provides a sufficient surgical field.

 
 » References Top

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    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6]

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