PMC4361837_01
The patient, aged 18 years, standing next to his brother, aged 22 years. He has mildfrontal bossing and hypertelorism. His limbs appear symmetric
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F1
Intercanthal distance was normal [Figure 1].
Preoperative X-rays of the chest (a) and upper limbs (b) were unremarkable
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F2
His bone age was between 13 and 16 years, based on assessment of ossification centers of his wrist joint and hands [Figure 2].
Preoperative radiology of the craniovertebral junction. Lateral X-rays in (a) flexion and (b) extension showing the mobile atlantoaxial dislocation. Note the displacement between the posterior arch of C1 and the spinolamellar line of C2. (c) Sagittal reconstruction of spiral CT of the craniovertebral junction showing the osodontoideum. (d) Reconstructed view of the cervical canal showing the lower half of the dens-C2 complex displaced into the canal. CT = Computed tomography
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F3
Flexion and extension X-ray films of cervical spine revealed a partially reducible AAD [Figure 3a and b]. T2hyperintense signal changes in the cord at the C1 leve l[Figure 4]. There was cervical canal compromise due to a posterior displacement of the inferior part of the dens-C2 complex [Figure 3c and d].
Sagittal MRI image showing the severe compression of the cord at the C1 level withan area of T2hyperintense signal change within the cord. MRI = Magnetic resonance imaging
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F4
T2hyperintense signal changes in the cord at the C1 leve l[Figure 4]. There was cervical canal compromise due to a posterior displacement of the inferior part of the dens-C2 complex [Figure 3c and d].
Postop radiology.(a) Lateral X-ray showing the construct. (b) The sagittal reconstruction of the CT scan showing reduction of the atlantoaxial dislocation and an adequately roomy canal
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F5
Postop-rays and computed tomography (CT) of the CVJ showed good reduction of the AAD and proper positioning of the implants [Figure 5].