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Table of Contents    
Year : 2021  |  Volume : 69  |  Issue : 1  |  Page : 194-197

Congenital Mobile Atlantoaxial Dislocation with Cervicomedullary Astrocytoma in Pediatric Patient

Department of Neurosurgery, Vivekananda Polyclinic and Institute of Medical Sciences and Hospital, Lucknow, Uttar Pradesh, India

Date of Submission09-Feb-2019
Date of Decision19-Apr-2019
Date of Acceptance22-May-2019
Date of Web Publication24-Feb-2021

Correspondence Address:
Adiba I Sheikh
W 103, DNB Hostel, Vivekananda Polyclinic Institute of Medical Science and Hospital, Lucknow - 226 007, Uttar Pradesh
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0028-3886.310076

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 » Abstract 

Congenital mobile atlantoaxial dislocation with cervicomedullary astrocytoma has never been described. We present a case of a 7-year-old male child who presented to us with gradually progressive spastic quadriparesis following a fall from table. His lateral radiograph and magnetic resonance images showed mobile atlantoaxial dislocation with intramedullary heterogeneously enhancing cervicomedullary mass. The patient underwent suboccipital craniectomy with C1–4 laminectomy. Tumor was pinkish grey, tenacious with ill-defined plane and cyst at poles. C1–C2 fusion was done using C1 lateral mass and C2 pars screw and rod system with onlay bone graft. Histopathology revealed pilocytic astrocytoma. At the time of discharge, the patient showed improvement in spasticity. Postoperative lateral radiograph showed reduced atlantoaxial dislocation with stable construct.

Keywords: Congenital atlantoaxial dislocation, intramedullary tumor, paediatric age
Key Message: This report highlights the extremely rare coincidence of AAD with cervicomedullary astrocytoma. Management of both the entities may be performed in a single sitting.

How to cite this article:
Singh AK, Sheikh AI, Pandey TK, Chabbra DK. Congenital Mobile Atlantoaxial Dislocation with Cervicomedullary Astrocytoma in Pediatric Patient. Neurol India 2021;69:194-7

How to cite this URL:
Singh AK, Sheikh AI, Pandey TK, Chabbra DK. Congenital Mobile Atlantoaxial Dislocation with Cervicomedullary Astrocytoma in Pediatric Patient. Neurol India [serial online] 2021 [cited 2023 Jan 29];69:194-7. Available from: https://www.neurologyindia.com/text.asp?2021/69/1/194/310076

We report a case having congenital/traumatic reducible Atlantoaxial dislocation (AAD), cleft in the anterior and posterior arch of atlas, and hypoplastic odontoid process with cervicomedullary astrocytoma leading to progressive spastic quadriparesis in a child.

 » Case Report Top

A 7-year-old male child presented with history of trauma 6 months back by fall from table in school followed by gradually progressive spastic quadriparesis. On examination, he had wasting of bilateral (left > right) thenar and hypothenar muscle, spastic quadriparesis (Modified Ashworth Grade II), walking with one man support, and hyperreflexia of all deep tendon reflexes with upgoing planter reflexes. Lhermitte's phenomenon was present. Hoffman's sign was seen. Motor power was grade 4/5 in the right upper and lower limb and grade 3/5 in the left upper and lower limb. Sensory examination showed decreased sensation below C4–5 level. His lateral X-ray CV Junction (flexion/extension) showed mobile atlantoaxial dislocation [Figure 1].
Figure 1: Lateral X rays of cevicovertebral junction view showing anterior displaced C1 vertebra in flexion view suggestive of Atlantoaxial dislocation

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The magnetic resonance (MR) images showed craniovertebral junction (CVJ) anomaly in the form of cleft in the anterior and posterior arch of atlas, odontoid process was hypoplastic. T1-weighted images revealed heterogeneously hypointense and T2-weighted revealed heterogeneously hyperintense, heterogeneously enhancing solid cystic lesion extending from cervicomedullary junction upto upper border of C4 vertebra [Figure 2], [Figure 3] and [Figure 4].
Figure 2: Sagittal MRI showing intramedullary tumor

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Figure 3: Sagittal T2WI MRI showing intramedullary tumor

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Figure 4: Axial MRI

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We performed a midline suboccipital craniectomy with C1–4 laminectomy with tumor decompression with bilateral C1 lateral mass and bilateral C2 pars rod screw fixation and fusion [Figure 5] and [Figure 6]. Tumor was pinkish grey, soft to firm, tenacious, with ill-defined plane of cleavage. Decompression of tumor was done. A duraplasty was done. The histopathology revealed pilocytic astrocytoma World Health Organization grade I with fibrillated and elongated (piloid) cells in the myxoid pool of microcytic areas with many rosenthal fibres and occasional mitotic activity [Figure 7], [Figure 8], [Figure 9] and [Figure 10].
Figure 5: Intraoperative image

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Figure 6: Post OP X-ray showing construct in situ

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Figure 7: Histopathology showing intramedullary astrocytoma

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Figure 8: Histopathology

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Figure 9: Histopathology

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Figure 10: Histopathology

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In postoperative period, patient showed improvement in spasticity, and postoperative lxray CV Junction showed reduced atlantoaxial dislocation with construct well in place.

 » Discussion Top

To the best of our knowledge, simultaneous presence of reducible AAD with cervicomedullary pilocytic astrocytoma in pediatric age group has not been previously described in English literature.

The CVJ is a complex transition zone between membranous (skull) and enchondral (occipital and spinal column) ossification, and because of many sclerotomes involved in its development,[1],[2] several congenital anomalies develop in the region.[3],[4],[5]

Cervicomedullary tumors are rare intramedullary tumors; the majority are histologically benign, slow-growing gliomas with long duration of symptoms.[6] Some research has postulated that these are primarily cervical neoplasm with medullary extension.[7] Because of their well-defined tumor plane, resection is feasible in many of these tumors.[8]

In our patient, mobile/reducible AAD was associated with cleft in the anterior and posterior atlas arch with hypoplastic dens. A minor trauma in setting of mobile AAD along with cervicomedullary expansile mass lesion was responsible for his progressive spastic quadriparesis.

Intramedullary mass in setting of irreducible AAD with progressive spastic quadriparesis has been described earlier [Table 1].[9],[10] Behari et al. reported a case of low-grade glioma at C2–5 level which was below the level of coexisting fixed AAD.[10] Chhabra et al.[11] reported C2–3 intramedullary ependymal cyst with irreducible AAD. Goel et al. have reported C1–2 syringomyelia associated with congenital mobile AAD in three infants, and in two of them it was diagnosed as intramedullary tumor.[9] In our case, however, the lesion was extending from medulla to C2–4. It was having both solid and cystic components and heterogeneously enhancing on contrast.
Table 1: Summary of different case reports on Congenital AAD with Intramedullary Tumor

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Low-grade astrocytoma can be confused with ependymoma. However, less intense enhancement and no clear demarcation indicate low-grade astrocytoma as was the case with our patient. Gangliogliomas are difficult to distinguish from astrocytoma; however, Patel et al.[12] described several clinical and imaging findings that are characteristic of ganglioglioma: young patient, long tumor length, tumoral cyst, absence of edema, mixed signal intensity on T1-weighted imaging (resulting from dural cellular element of tumor), patchy tumor enhancement, and cord surface enhancement. They are typically eccentrically located.

Intramedullary spinal cord hemorrhage (hematomyelia) is an uncommon cause of myelopathy and can present as acute, subacute, or chronic fashion. Hematomyelia have varying appearances on T1 and T2 MR imaging (MRI) depending on the age of hematoma. Acute hematomyelia appears as T1 isointense and T2 hyperintense, while late subacute and chronic hematomyelia will appear as T1 and T2 hyperintense.

 » Surgical Consideration Top

Mobile reducible AAD has been categorized into four groups by Behari et al.[13] from management point of view. There are various techniques described for occiput, C1–C2 fusion.[14],[15],[16],[17],[18],[19],[20],[21] However, in our as we had to do C1–4 laminectomy to address intramedullary tumor, the only option available to us was C1–C2 screw fixation. Thus, obviating the possibility of further damage to cord during flexion, extension movement. The bone graft was put inside the decorticated joint to achieve bony fusion.

Subtotal excision of tumor could only be achieved because at some places there was no clear-cut demarcation between tumor and cord tissue. However, intraoperative MRI, ultrasound sonography, and neurophysiological monitoring could help in better surgical resection in cervicomedullary tumor.[21],[22],[23],[24],[25],[26]

As pilocytic astrocytomas are low grade, tumor radiotherapy has been deferred.

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Conflicts of interest

There are no conflicts of interest.

 » References Top

Behari S, Bhargava V, Nayak S, Kirankumar MV, Banerji D, Chhabra DK, et al. Congenital reducible atlantoaxial dislocation: Classification and surgical considerations. Acta Neurochirur (Wien) 2002;144:1165-77.  Back to cited text no. 1
Jain VK, Behari S. Management of congenital atlanto-axial dislocation: Some lessons learnt. Neurol India 2002;50:386-97.3. Joseph H. McAbee, Joseph Modica, Clinton J, et al. Thompson. Cervicomedullary tumors in children. Clinical article. J Neurosurg Pediatr 2015;16:357-366.  Back to cited text no. 2
Cacciola F, Di Lorenzo N. Embryology and development of the craniovertebral junction. In: Goel A, Cacciola F, editors. The Craniovertebral Junction. Stuttgart, Thieme; 2011. p. 14-20.  Back to cited text no. 3
Menezes AH. Craniocervical developmental anatomy and its implications. Childs Nerv Syst 2008;24:1109-22.  Back to cited text no. 4
Pang D, Thompson DNP. Embryology and bony malformations of the craniovertebral junction. Child's Nerv Syst 2011;27:523-64.  Back to cited text no. 5
Young Poussaint T, Yousuf N, Barnes PD, Anthony DC, Zurakowski D, Scott RM, et al. Cervicomedullary astrocytomas of childhood: Clinical and imaging follow-up. Pediatr Radiol 1999;29:662-8.  Back to cited text no. 6
Sridhar K, Sridhar R, Venkatprasanna G. Management of posterior fossa gliomas in children. J Pediatr Neurosci 2011;6(Suppl 1):S72-7.  Back to cited text no. 7
Garcés-Ambrossi GL, McGirt MJ, Mehta VA, Sciubba DM, Witham TF, Bydon A, et al. Factors associated with progression- free survival and long-term neurological outcome after resection of intramedullary spinal cord tumors: Analysis of 101 consecutive cases. J Neurosurg Spine 2009;11:591-9.  Back to cited text no. 8
Goel A, Muzumdar D, Dange N. Syringomyelia in infants secondary to mobile congenital atlantoaxial dislocation. Pediatr Neurosurg 2007;43:15-8.  Back to cited text no. 9
Charley A, Jaiswal AK, Jain M, Behari S. Congenital irreducible atlantoaxial dislocation associated with cervical intramedullary astrocytoma causing progressive spastic quadriparesis. Neurol India 2008;56:477-9.  Back to cited text no. 10
Chhabra R, Bansal S, Radotra BD, Mathuriya SN. Recurrent intramedullary cervical ependymal cyst. Neurol India 2003;51:111-3.  Back to cited text no. 11
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Patel U, Pinto RS, Miller DC, Handler MS, Rorke LB, Epstein FJ, et al. MR of spinal cord ganglioglioma. AJNR Am J Neuroradiol 1998;19:879-87.  Back to cited text no. 12
Behari S, Bhargava V, Nayak S, Kiran Kumar MV, Banerji D, Chhabra DK, et al. Congenital reducible atlantoaxial dislocation: Classification and surgical considerations. Acta Neurochir (Wien) 2002;144:1165-77.  Back to cited text no. 13
Brooks AL, Jenkins EB. Atlantoaxial arthrodesis by the wedge compression method. J Bone Joint Surg (Am) 1978;60:279-84.  Back to cited text no. 14
GeremiaGK, Kim KS, Cerullo L, Calenof L. Complications of sublaminar wiring. Surg Neurol 1985;23:629-34.  Back to cited text no. 15
Lundy DW, Murray HH. Neurological deterioration after posterior wiring of the cervical spine. J Bone Joint Surg (Br) 1997;79:948-51.  Back to cited text no. 16
Wang J, Vokshoor A, Kim S, Elton S, Kosnik E, Bartkowski H. Pediatric atlantoaxial instability: management with screw fixation. Pediatr Neurosurg. 1999;30:70-8.  Back to cited text no. 17
Goel A, Laheri V. Plate and screw fixation for atlantoaxial subluxation. Acta Neurochir (Wien) 1994;129:47-53.  Back to cited text no. 18
Jain VK, Behari S. Posterior occipitoaxial fusion for atlantoaxial dislocation associated with occipitalized atlas. In: Wilkins R, Rengachary S, editors. Neurosurgical Operative Atlas, Volume 7. Illinois: American Association of Neurological Surgeons; 1997. p. 249-56.  Back to cited text no. 19
Jain VK, Takayasu M, Singh S, Chhabra DK, Sugita K. Occipital-axis posterior wiring and fusion for atlantoaxial dislocation associated with occipitalzation of atlas. Technical note. J Neurosurg1993;79:142-4.  Back to cited text no. 20
Cheng JS, Ivan ME, Stapleton CJ, Quinones-Hinojosa A, Gupta N, Auguste KI. Intraoperative changes in transcranial motor evoked potentials and somatosensory evoked potentials predicting outcome in children with intramedullary spinal cord tumors. J Neurosurg Pediatr 2014;13:591-9.  Back to cited text no. 21
Choudhri AF, Klimo P Jr, Auschwitz TS, Whitehead MT, Boop FA. 3T intraoperative MRI for management of pediatric CNS neoplasms. AJNR Am J Neuroradiol 2014;35:2382-7.  Back to cited text no. 22
Choudhri AF, Whitehead MT, Klimo P Jr, Montgomery BK, Boop FA. Diffusion tensor imaging to guide surgical planning in intramedullary spinal cord tumors in children. Neuroradiology 2014;56:169-74.  Back to cited text no. 23
Jallo GI, Biser-Rohrbaugh A, Freed D. Brainstem gliomas. Childs Nerv Syst 2004;20:143-53.  Back to cited text no. 24
Sala F, Krzan MJ, Deletis V. Intraoperative neurophysiological monitoring in pediatric neurosurgery: Why, when, how? Childs Nerv Syst 2002;18:264-87.  Back to cited text no. 25
WinestoneJS, Lin J, Sanford RA, Boop FA. Subepyndemal hemangioblastomas of the cervicomedullary junction: Lessons learned in the management of two cases. Childs Nerv Syst 2007;23:761-4.  Back to cited text no. 26


  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6], [Figure 7], [Figure 8], [Figure 9], [Figure 10]

  [Table 1]

This article has been cited by
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Pediatric Neurosurgery. 2022; 57(3): 149
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