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|LETTER TO EDITOR
|Year : 2021 | Volume
| Issue : 4 | Page : 1076-1077
Mirror Image Dumbell C2 Schwannoma with Subcutaneous Extension without Neuro Fibromatosis in a Young Child: A Rare Entity
Ayusman Satapathy, Sumit Bansal, Rabi Narayan Sahu
Department of Neurosurgery, All India Institute of Medical Sciences, Bhubaneswar, Orissa, India
|Date of Submission||03-Dec-2019|
|Date of Decision||05-Mar-2020|
|Date of Acceptance||31-May-2020|
|Date of Web Publication||2-Sep-2021|
Department of Neurosurgery, All India Institute of Medical Sciences, Bhubaneswar - 751 019, Orissa
Source of Support: None, Conflict of Interest: None
|How to cite this article:|
Satapathy A, Bansal S, Sahu RN. Mirror Image Dumbell C2 Schwannoma with Subcutaneous Extension without Neuro Fibromatosis in a Young Child: A Rare Entity. Neurol India 2021;69:1076-7
|How to cite this URL:|
Satapathy A, Bansal S, Sahu RN. Mirror Image Dumbell C2 Schwannoma with Subcutaneous Extension without Neuro Fibromatosis in a Young Child: A Rare Entity. Neurol India [serial online] 2021 [cited 2022 Jan 23];69:1076-7. Available from: https://www.neurologyindia.com/text.asp?2021/69/4/1076/325386
Bilateral C2 nerve schwannoma is a rare entity, very few number of cases reported in literature. A 4-year-female child presented to our outpatient department with progressive spastic quadriparesis for 8 months duration and subcutaneous swelling at the back of neck for the same duration. On examination, motor power in upper and lower limbs was 2/5 and 1/5 (MRC Grade), respectively, with clasp knife spasticity in all four limbs and brisk deep tendon reflexes. She had also respiratory involvement with respiratory rate 22 per minute, mostly abdominal type, single breath count of 12 and breath holding time of 12 s without any cranial nerve involvement. She did not have any clinical evidence or family history of Neurofibromatosis. No genetic testing carried out as patient was not willing. So cranio-vertebral junction pathology was suspected. Contrast MRI CV junction showed a brilliantly contrast enhancing bilateral dumbell shaped C2 nerve schwannoma compressing the upper spinal cord [Figure 1]a, [Figure 1]b, [Figure 1]c. Preoperative vertebral CT angiography showed bilateral Vertebral artery and CT CV junction showed normal bilateral C1-C2 facet joint [Figure 2]. The lesion has also subcutaneous extension, which was presented like a subcutaneous swelling in the back of neck. She underwent posterior midline approach and complete excision of both the lesions was done [Figure 1]d, [Figure 1]e, [Figure 1]f, [Figure 1]g. Histopathology was suggestive of schwannoma. On follow-up spasticity, power and respiratory function significantly improved. Motor power in all four limbs improved to grade 4/5.
|Figure 1: Preoperative MRI CV junction showing contrast enhancing bilateral C2 tumor, on T-1 weighted image [sagittal (a), coronal (b) axial (c), views]. Postoperative MRI [sagittal (d), coronal (e) axial (f), views] showing good excision of bilateral C2 tumor with left sided residual tumor in the neck. Intraoperative image is showing bilateral C1-2 tumor with widening of C1-2 interlaminar space (g)|
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|Figure 2: Preoperative vertebral CT angiography suggestive of bilateral Vertebral artery having normal course, calibre and equidominant (a). CT CV junction (coronal section) showing normal bilateral C1 C2 facet joint without any evidence of Atlanto axial subluxation or dislocation (b)|
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Among high cervical spinal schwannoma, C2 nerve root originating tumors are predominant. C1-C2 schwannoma are unique as they have a tendency to expand in hourglass fashion. Roomy spinal canal at this level ensures that the tumor has achieved massive dimensions before it is symptomatic. Surgical excision of these tumors raises several challenges like adequate spinal exposure, preservation of involved spinal nerve roots, relationship with vertebral artery, postoperative kyphosis, cervical instability, risk of general anesthesia and high recurrence rates.
Surgical approaches to C1-C2 tumor include the posterior, postero-lateral, anterior, antero-lateral, and extreme lateral approaches. Another important consideration in cervical tumors is their relationship with vertebral artery. This artery is usually displaced antero-medially by the tumor and is always separated from the tumor by a thin layer of periosteum and perivertebral veins. Therefore if dissection is carried out in this plane, there are limited chances of ischemic injury. C1-C2 fixation should be considered in where there is destruction of the facets joint by the tumor directly or indirectly with evidence of AAD or where there is chance of instability after surgery may be iatrogenic C1-C2 instability like excessive bony drilling, facet drilling for tumor removal. However in our case there was no such indication. Proper 3-D anatomical orientation and physiological knowledge, deep neuro-radiological observation, pathological appreciations and micro-neurosurgical skill can make the surgical management of these tumors, simple with gratifying result without extensive bone removal or soft tissue manipulation through a standard midline posterior approach.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.
Financial support and sponsorship
Conflicts of interest
There are no conflicts of interest.
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[Figure 1], [Figure 2]