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This year-old male patient experienced a significant deterioration for his neurological condition several months and presented with neck pain and dysesthesia and was unable to walk properly. Treatment of craniocervical extramedullary tumor is challenging as CCJ presents a unique, complex, biomechanical interface between the cranium and the upper cervical spine [ 15 ]. Various surgical approaches have been advocated for the extramedullary tumor in CCJ, depending on the anatomical location of the tumor relative to the spinal cord [ 12 ].

Reconstruction of Upper Cervical Spine and Craniovertebral Junction

Tumors situated posterior or posterolateral to the spinal cord or the brainstem can be safely resected via a posterior midline suboccipital approach combined with C1 laminectomy. The optimal surgical approach to the anterior and anterolaterally located tumors still remains debatable [ 4 , 16 ]. Theoretically, the transoral route is considered as the best approach for ventral tumors, which provided a direct and natural approach to the CCJ [ 17 ]. However, this approach has several drawbacks, including CSF leak, subsequent infections, velopharyngeal insufficiency, and limited lateral access [ 18 ].

The lateral approaches to extramedullary tumor in CCJ include the anterolateral or the extreme lateral approach and the posterolateral or the far lateral approach. Several authors reported that these approaches provided an adequate exposure for tumor resection in comparison with a conventional anterior approach [ 19 , 20 ]. However, the lateral approaches might be technically challenging as risk of accessory nerve or vertebral artery injury and venous plexus hemorrhage. Recently, Fong [ 21 ] and Bydon [ 22 ] reported that patients with tumor could be successfully treated with endoscopic endonasal and others minimally invasive approaches, which offer the potential benefits of less blood loss and quicker recovery, and it prevents postoperative instability from extensive bone resection while maintaining spinal structural integrity.

However, the major shortcomings of minimally invasive approaches are the limited space for manipulation and the restricted view [ 23 ]. Additionally, minimally invasive technique needs to evolve over time, and the learning curve may be quite steep. Noticeably, Ahn and colleagues in a retrospective study of patients that underwent surgical resection of spinal extramedullary tumors via a posterior approach reported no difficulties in removing ventrally located extramedullary tumors [ 24 ].

Actually, complete excision in ventral spinal extramedullary tumors is also not easy via an anterior approach because it seems difficult to use this approach for a tumor outside the intervertebral foramen in the upper cervical cord. To overcome these difficulties, the lateral facet joint must be excised by posterior approach, and an adequate visual field of the ventrally located tumors must be maintained when approaching them. Thus, a single posterior approach was used in our patients by this method, regardless of the location of the tumor relative to the spinal cord.

Consistent with the previous findings, we found it was safe and effective for resecting ventrally located tumors through a purely posterior approach. Regardless of the surgical approach, complete resection of the spinal extramedullary tumors is one of the primary surgical goals. Numerous studies have shown a positive association between gross total resection and favorable outcomes [ 12 , 25 ]. In the current series, five patients had schwannoma, three patients had meningioma, and one patient had neurofibroma.

Several factors may be responsible for this condition. First, the vertebral artery involvement was all widely accepted as an important factor against radical resection. Fortunately, our patients had no artery involvement which may significantly affected radical resection. Second, the length and extent of laminectomy or the removal of facet joint of C1 are directly associated with the safety and effectiveness of complete resection. The concomitant fusion procedures may permit more radical resections without concern for iatrogenically induced spinal instability.

Postoperative clinical and radiographic improvements were maintained during the follow-up period. The rate of neurological recovery was At the final follow-up, no evidence of recurrence was observed in our patients. The result of the present study confirmed the previous findings that gross total resection was the most important treatment variable influencing long-term outcomes and recurrence rates in patients with spinal extramedullary tumors [ 24 , 27 ].

In order to provide a surgical window and expose the extramedullary tumor at the CCJ, it is necessary to perform a limited craniectomy of the inferior aspect of the occiput and resection of posterior elements and facet joint of C1. Concomitant spinal fusion and stabilization may be required depending on the amount of bony resection.

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Sciubba [ 7 ] and Mcgirt [ 8 ] reported that patients who undergo laminectomy at high stress regions like the CCJ will probably have an increased risk for postoperative CCJ instability as biomechanical mechanism. Furthermore, spinal cord lesions alone, without surgical destabilization, can lead to spinal instabilities and deformities in both children and adults as involvement of the anterior horn cells causes muscle denervation and weakness [ 30 ].

For these reasons, spinal stability should be evaluated in cases of spinal tumor extirpation from the CCJ requiring C1 facetectomy. Fusion procedures are recommended to perform if instability is evident. Nevertheless, it is cautious to determine whether OCF is necessary in these patients with craniocervical extramedullary tumor as it may sacrifice the motion of the occipital and C complex. The ndication of OCF have documented by several surgeons, however, which is still controversial [ 31 , 32 ]. If the integrity of occipital-C1 facet capsule is possible, we usually perform C fusion so that occipital-C1 motion is preserved.

Pediatric Craniovertebral Junction Surgery

In other words, we recommend OCF for instability if removal of the C1 facet joint, either unilateral or bilateral, and C1 is not suitable for placement of screws. In our study, the mean C angle and the mean C angle before surgery were No evidence of instability at the lower adjacent motion segment and kyphosis of the upper cervical spine were observed until a mean follow-up of The results demonstrate that OCF is valuable for use in patients who require the extramedullary tumor resection and occipito-cervical stabilization by one-stage posterior approach.

There are several limitations in our study. First, sample size is small, which are related to the extremely rare and unusual patients with such conditions. Second, the mean follow-up period Our results will need to be confirmed in larger series with longer follow-up. Guidelines for OCF stabilization after resection of extramedullary tumor at the CCJ have been still unclear, and the literature includes very few reports of clinical experience in this area.

On the basis of our findings in the clinical series, we recommend that OCF be performed in cases of tumor extirpation from the CCJ requiring C1 facetectomy, which may prevent the postoperative kyphosis of the upper cervical spine. Written informed consent was obtained from all patients enrolled in the investigation. The study protocol conformed to the ethical guidelines of the Declaration of Helsinki and the guidelines of the regional ethical committees of Zurich, Switzerland, and Basel, Switzerland.

Spinal meningiomas in patients younger than 50 years of age: a year experience. J Neurosurg. Spinal meningiomas: surgical management and outcome.

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Neurosurg Focus. Outcome predictors and complications in the management of intradural spinal tumours. Eur Spine J.

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Management of benign craniovertebral junction tumors. Acta Neurochir Wien. Surgical strategies in the management of spinal nerve sheath tumors. World Neurosurg. Surgical outcomes of craniocervial junction meningiomas: a series of 22 consecutive patients. Clin Neurol Neurosurg. Factors associated with cervical instability requiring fusion after cervical laminectomy for intradural tumor resection. J Neurosurg Spine. Incidence of spinal deformity after resection of intramedullary spinal cord tumors in children who underwent laminectomy compared with laminoplasty.

J Neurosurg Pediatr. C2 lamina reconstruction using locking miniplate for the intradural tumor of the craniocervical junction two case reports. Posterior occipitocervical reconstruction using cervical pedicle screws and plate-rod systems. Spine Phila Pa Long-term results of computer-assisted posterior occipitocervical reconstruction. Anatomical location dictating major surgical complications for intradural extramedullary spinal tumors: a year single-institutional experience.

Basic Sciences - Cranio-cervical junction

Roentgenographic findings of the cervical spine in asymptomatic people. Patients were followed by the same surgeon AFJ. Complications were described in details. The images were reconstructed in 0. Presence or absence of facet joints subluxation of the occipital-lateral masses of the atlas and the atlanto-axial joints; diagnosis of clivus hypoplasia: Adopted when the clivus had less than Thirty patients with congenital CVJ malformations were operated from to by the senior author AFJ at our institution. A total of ten patients had complete dynamic exams and were included according our inclusion criteria.

The mean age of our population was Table 2 summarizes the clinical data of the patients. All patients had some degree of tonsillar herniation mean of Treatment consisted in posterior fossa decompression with removal of the posterior arch of C1 in all cases. In all cases, duroplasty was performed, but only two cases had also tonsillar resection due to the severity of the tonsillar herniation.

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Two patients underwent concomitant occipito-cervical fixation because they presented a mobile atlantoaxial subluxation on dynamic images, as explained below. The main bone anomaly associated with BI was clivus hypoplasia. Five patients had clivus hypoplasia, four patients had atlas assimilation and two patients had a C2-C3 fusion Table 3.

Four patients had some facet joints subluxation at the atlanto-axial region. Two of these patients underwent a cranio-cervical fixation due to their mobile subluxation: One patient had an increased facet subluxation in extension, from 4 to 7 mm, without changing the ADI distance and the other had an increased ADI in flexion, from 2.

The other two cases were treated initially without occipito-cervical fixation because their facet joints did not change on flexion and extension CT scan Figure 2. During the study follow-up, another patient who had BI and a congenital C2-C3 fusion developed latter cervical pain and dizziness when flexing the neck.