PMC3642751_01
(a, b) Radiographs of the cervical and thoracic spine demonstrating fused vertebral bodies from C2 down to T6. No instability was documented on flexion-extension studies, (c) Significant kyphosis at the higher thoracic spine
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F1
No instability was documented on flexion-extension studies throughout her spine, notably not at occiput-C1 or C1-C2 [Figure 1].
(a-d) Pre-operative cranio-cervico-thoracic CT scan showing a mass in the posterior fossa behind the fourth ventricle and below the cerebellum extending down behind the spinal cord to the posterior thoracic vertebra to T1. In the posterior fossa, it obstructed the foramen of Magendie, thus resulting in secondary obstructive hydrocephalus. In addition, fusion of the cervical and thoracic vertebral bodies from C2 to T6, involving the anterior spinal column and posterior elements is seen
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F2
Computed tomography (CT) of the head, cervical, and thoracic spine confirmed congenital Klippel-Feil fusion of the cervical and thoracic vertebral bodies from C2 to T6, involving the anterior spinal column and posterior elements which were fused 'en bloc' [Figures 2 and 3].
(a-d) Preoperative CT scan with 3D-reconstruction showing the osseous anomalies, including C2 to T6 'en bloc' fusion
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F3
Computed tomography (CT) of the head, cervical, and thoracic spine confirmed congenital Klippel-Feil fusion of the cervical and thoracic vertebral bodies from C2 to T6, involving the anterior spinal column and posterior elements which were fused 'en bloc' [Figures 2 and 3].
(a-c) CT-angiography showing the vertebral arteries located anterior to the lesion in addition to a low-lying venous confluence
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F4
A CT angiogram showed that both vertebral arteries were located anterior to the lesion [Figure 4].
Pre-operative magnetic resonance imaging, T1WI sagittal (a), T2WI sagittal (b), and Stir axial (c) sections documenting an expansive midline lesion extending from the cranio-cervical junction down to T1. The dermal sinus tract, extending from the dermis to the suboccipital bone below the occipital protuberance is identified on T1WI and T2WI (arrow)
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F5
Cerebral and cervical magnetic resonance imaging (MRI) documented a craniocervical mass that measured 3.2 cm anteroposteriorly, by 8.4 cm rostrocaudally [Figure 5]. Furthermore, a sinus tract was suspected on the T2 weighted images involving the suboccipital region extending from the dermis to the suboccipital bone [Figure 5c]. During preparation of the sub-occipital region, a small dimple located below the occipital protuberance and connecting to a sinus tract during preparation of the sub-occipital region, a small dimple located below the occipital protuberance and connecting to a sinus tract was identified [Figure 5c] was clearly identified.
Intraoperative image of the dermoid cyst
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F6
After performing a laminectomy from C1 to C4 and opening the dura, a large midline cystic mass filled with thick yellowish fluid, hair and keratin debris was found [Figure 6].
Postoperative magnetic resonance imaging, T1WI sagittal (a), T2WI sagittal (b) sections and T1WI axial
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F7
The MRI performed 4 days after surgery documented complete resection of the DC [Figure 7].
Postoperative plain lateral cervicothoracic radiograph demonstrating no new-onset deformity at 6-year follow-up
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F8
Six years postoperatively, the patient is doing very well, and remains symptom-free, without evidence of deformity on plain radiographs [Figure 8] or tumor recurrence on follow-up MRIs studies [Figure 9].
Postoperative magnetic resonance imaging, T2WI sagittal (a) T2WI axial (b) T1WI post-gadolinium axial sections demonstrating no recurrence at 6 years after surgery (c) Demonstrating no recurrence at 6-year follow-up
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F9
Six years postoperatively, the patient is doing very well, and remains symptom-free, without evidence of deformity on plain radiographs [Figure 8] or tumor recurrence on follow-up MRIs studies [Figure 9].