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Background: Ossification of the anterior longitudinal ligament (OALL) of the cervical spine is a relatively rare disease. If patients present with dysphagia, hoarseness, and/or dyspnea, they may require surgery. Case Description: Over a 7-month period, a 55-year-old female with a history of cerebral palsy developed a progressive quadriparesis accompanied by diffuse sensory loss (i.e., clumsiness of the hand/legs and gait disturbance). The cervical spine X-rays showed atlanto-axial subluxation with instability, while the cervical MRI demonstrated "pseudotumor in the retro-odontoid" region. Following an occipital cervical fusion (C0-C2) surgery, her quadriparesis resolved. Nevertheless, she had persistent dysphagia that worsened over 6 months. Video fluoroscopy revealed severe mechanical stenosis of the pharynx, which was attributed to OALL extending from the C3-C6 levels. Following OALL resection through a right anterior approach utilizing diamond burrs and an ultrasonic bone curette, the dysphagia rapidly resolved. Conclusion: We report a rare case of retro-odontoid pseudotumor successfully treated with a posterior C0-C2 cervical fusion. Additional symptomatic C3-C6 OALL, responsible for progressive dysphagia, was later managed with focal anterior OALL resection.

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BACKGROUND: Giant prolactinomas (>4 cm) are a rare entity, constituting less than 1% of all pituitary tumors. Diagnosis can usually be achieved through endocrinological analysis, but biopsy may be considered when trying to differentiate between invasive nonfunctioning pituitary adenomas and primary clival tumors such as chordomas. OBSERVATIONS: The authors presented a rare case of a giant prolactinoma causing significant clival and occipital condyle erosion, which led to craniocervical instability. They provided a review of the multimodal management. Management involved medical therapy with dopamine agonists, and surgery was reserved for acute neural compression or dopamine agonist resistance, with the caveat that surgery was extremely unlikely to lead to normalization of serum prolactin in dopamine agonist-resistant tumors. LESSONS: Adjunctive surgical therapy may be necessary in cases of skull base erosion, particularly when erosion or pathological fractures involve the occipital condyles. Modern posterior occipital-cervical fusion techniques have high rates of arthrodesis and can lead to symptomatic improvement. This procedure should be considered early in the multimodal approach to giant prolactinomas because of the often dramatic response to medical therapy and potential for further craniocervical instability.

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BACKGROUND: Occipital-cervical fusion (OCF) and ventral decompression (VD) may be used in the treatment of pediatric Chiari-1 malformation (CM-1) with syringomyelia (SM) as adjuncts to posterior fossa decompression (PFD) for complex craniovertebral junction pathology. OBJECTIVE: To examine factors influencing the use of OCF and OCF/VD in a multicenter cohort of pediatric CM-1 and SM subjects treated with PFD. METHODS: The Park-Reeves Syringomyelia Research Consortium registry was used to examine 637 subjects with cerebellar tonsillar ectopia ≥ 5 mm, syrinx diameter ≥ 3 mm, and at least 1 yr of follow-up after their index PFD. Comparisons were made between subjects who received PFD alone and those with PFD + OCF or PFD + OCF/VD. RESULTS: All 637 patients underwent PFD, 505 (79.2%) with and 132 (20.8%) without duraplasty. A total of 12 subjects went on to have OCF at some point in their management (PFD + OCF), whereas 4 had OCF and VD (PFD + OCF/VD). Of those with complete data, a history of platybasia (3/10, P = .011), Klippel-Feil (2/10, P = .015), and basilar invagination (3/12, P < .001) were increased within the OCF group, whereas only basilar invagination (1/4, P < .001) was increased in the OCF/VD group. Clivo-axial angle (CXA) was significantly lower for both OCF (128.8 ± 15.3°, P = .008) and OCF/VD (115.0 ± 11.6°, P = .025) groups when compared to PFD-only group (145.3 ± 12.7°). pB-C2 did not differ among groups. CONCLUSION: Although PFD alone is adequate for treating the vast majority of CM-1/SM patients, OCF or OCF/VD may be occasionally utilized. Cranial base and spine pathologies and CXA may provide insight into the need for OCF and/or OCF/VD.

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Malformación de Arnold-Chiari/cirugía , Descompresión Quirúrgica/métodos , Fusión Vertebral/métodos , Siringomielia/cirugía , Malformación de Arnold-Chiari/complicaciones , Niño , Estudios de Cohortes , Femenino , Humanos , Masculino , Siringomielia/complicaciones , Resultado del Tratamiento

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BACKGROUND: Patients with Klippel-Feil syndrome may present with neurologic complaints such as neck pain, radiculopathy and gait instability. Here we describe surgical management of a patient with congenital fusion of the occipital-cervical region and also block circumferential fusion of dens to T3 with spinal cord compression. This report is the first of its kind with such extensive fusion. CASE DESCRIPTION: Our patient was a 56 year-old female, who presented with neck pain and tingling in all extremities. On exam, she had a short neck, prominent jaw with extremely limited range of motion in neck and features of myelopathy. CT showed fusion of the dens to T3 vertebrae. Patient underwent sub-occipital craniectomy, C1 laminectomy and Occiput to T5 posterior fixation and fusion with neurologic improvement. CONCLUSION: This is the first reported case of Klippel-Feil syndrome with fusion of all cervical vertebrae down to T3. We recommend surgery for advanced cases of myelopathy or radiculopathy due to stenosis and spinal instability.

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Vértebras Cervicales/diagnóstico por imagen , Síndrome de Klippel-Feil/diagnóstico por imagen , Estenosis Espinal/diagnóstico por imagen , Vértebras Torácicas/diagnóstico por imagen , Articulación Atlantooccipital/diagnóstico por imagen , Articulación Atlantooccipital/cirugía , Vértebras Cervicales/anomalías , Vértebras Cervicales/cirugía , Craneotomía , Descompresión Quirúrgica , Femenino , Humanos , Inestabilidad de la Articulación/diagnóstico por imagen , Inestabilidad de la Articulación/cirugía , Síndrome de Klippel-Feil/complicaciones , Síndrome de Klippel-Feil/fisiopatología , Síndrome de Klippel-Feil/cirugía , Laminectomía , Imagen por Resonancia Magnética , Persona de Mediana Edad , Procedimientos Neuroquirúrgicos , Hueso Occipital/anomalías , Hueso Occipital/diagnóstico por imagen , Hueso Occipital/cirugía , Apófisis Odontoides/anomalías , Apófisis Odontoides/diagnóstico por imagen , Apófisis Odontoides/cirugía , Fusión Vertebral , Estenosis Espinal/etiología , Estenosis Espinal/fisiopatología , Estenosis Espinal/cirugía , Vértebras Torácicas/anomalías , Vértebras Torácicas/cirugía , Tomografía Computarizada por Rayos X

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The atlas (C1) and axis (C2) have distinct morphologies to support the skull and facilitate head rotation and neck flexion. Congenital defects of C1 posterior arch are rare. We present a case of a 59-year-old man with both an absent C1 posterior arch with concomitant os odontoideum. The patient presented with neck crepitus, moderate neck pain and progressive worsening upper limbs paraesthesia and pain. Computed tomography (CT) revealed non-union between the odontoid process and body of axis as well as absence of C1 posterior arch. An occiput C0-C3 fusion was performed. The patient's symptoms improved significantly, and he is functioning well at 12 months. CT showed solid fusion without implant migration. Concomitant os odontoideum with aplastic C1 posterior arch is rare with limited evidence to guide management. Posterior fixation and fusion may be a potential solution to prevent dynamic compression, thereby preventing further myelopathy and related complications.

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The patient is a 69-year-old woman with a history of atlantoaxial instability and cervical pain who underwent an occipital-cervical fusion at an outside hospital. Five days following the procedure she required a PEG tube due to progressive dysphagia. Compared with preoperative imaging, x-ray shows cervical spine hyperextension with a significant decrease in the occipital-C2 angle. A swallow test confirmed aspiration and pharyngeal phase functional impairment. Two-stage surgery consisted of hardware removal, drilling the fused right C1-2 facet, reinstrumentation, and halo placement. The swallowing test confirmed there is no aspiration. We proceeded with rod placement. The patient recovered completely. The video can be found here: https://youtu.be/YzdJrOm46Y4.

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The complex Chiari, characterized by abnormal craniocervical bony anatomy in addition to Chiari tonsillar herniation, is a relatively recent addition to the concepts surrounding the Chiari literature. The primary findings of complex Chiari include craniocervical kyphosis and retroflexed odontoid, both of which can be described with radiographic measurements. This manuscript will outline the background literature regarding Chiari craniocervical morphometrics and supply an algorithm for the general management of complex Chiari patients.

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Malformación de Arnold-Chiari/diagnóstico por imagen , Articulación Atlantoaxoidea/diagnóstico por imagen , Articulación Atlantooccipital/diagnóstico por imagen , Atlas Cervical/diagnóstico por imagen , Toma de Decisiones Clínicas , Guías de Práctica Clínica como Asunto/normas , Malformación de Arnold-Chiari/cirugía , Articulación Atlantoaxoidea/cirugía , Articulación Atlantooccipital/cirugía , Atlas Cervical/cirugía , Toma de Decisiones Clínicas/métodos , Descompresión Quirúrgica/métodos , Descompresión Quirúrgica/normas , Humanos

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OBJECTIVE: The aim of this study was to create a map of the occipital bone using a cadaveric morphometric analysis. MATERIAL: Twelve heads, from seven male and five female cadavers, were studied. The thickness of the occipital bone was measured with a digital vernier caliper within a coordinate system. RESULTS: The maximum thickness of the occipital bone could be measured at the external occipital protuberance (mean 15.4 mm; range 9-29.3 mm). All male individuals had higher bone thickness around this point. Further lateral a steady decrease of bone thickness could be observed. Same could be observed in craniocaudal direction. However, values above the superior nuchal line were on average thicker than below. CONCLUSION: The measurements demonstrated a great individual variability of bone thickness of the occipital bone. The results emphasize the role of preoperative planning for the feasibility of placement of an occipital screw.

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Hueso Occipital/anatomía & histología , Cadáver , Femenino , Humanos , Masculino , Factores Sexuales

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BACKGROUND: Occipital-cervical fusion (OCF) has been used to treat instability of the occipito-cervical junction and to provide biomechanical stability after decompressive surgery. The specific areas that require detailed morphologic knowledge to prevent technical failures are the thickness of the occipital bone and diameter of the C2 pedicle, as the occipital midline bone and the C2 pedicle have structurally the strongest bone to provide the biomechanical purchase for cranio-cervical instrumentation. The aim of this study was to perform a quantitative morphometric analysis using computed tomography (CT) to determine the variability of the occipital bone thickness and C2 pedicle thickness to optimize screw placement for OCF in a South East Asian population. METHODS: Thirty patients undergoing cranio-cervical junction instrumentation during the period 2008-2010 were included. The thickness of the occipital bone and the length and diameter of the C2 pedicle were measured based on CT. RESULTS: The thickest point on the occipital bone was in the midline with a maximum thickness below the external occipital protuberance of 16.2 mm (±3.0 mm), which was thicker than in the Western population. The average C2 pedicle diameter was 5.3 mm (±2.0 mm). This was smaller than Western population pedicle diameters. The average length of the both pedicles to the midpoint of the C2 vertebral body was 23.5 mm (±3.3 mm on the left and ±2.3 mm on the right). CONCLUSIONS: The results of this first study in the South East Asian population should help guide and improve the safety in occipito-cervical region instrumentation. Thus reducing the risk of technical failures and neuro-vascular injury.

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@#Objective To explore effect of Axis internal fixation system while decompression and fusion for the treatment of timeworn atlantoaxial dislocation with incomplete spinal cord injury.Methods 29 cases of timeworn atlanto-axial dislocation with incomplete spinal cord injury underwent decompression fusion and Axis internal fixation with a 7～46 months (mean 20 months) flowing-up.ResultsAfter treatment, all patients got completed fusion, there was no deterioration of neurofunction, and rate of JOA scores increased was 78.6%.ConclusionAxis internal fixation system can provide immediately a rigid stability and maintain axial correction for the segment ahile decompression and fusion for the treatment of timeworn atlantoaxial deslocation with incomplete spinal cord injury.

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Objective To study the correlation between anatomical measurement and CT measurement of adult occipital thickness so as to provide anatomic evidence for the selection of screw length in occipital-cervical fusion. Methods The occipital thickness was measured on the occipital specimens of 10 normal adults in two ways: direct anatomic measurement and CT measurement. Measurements were made on the basis of the McRac' s line and according to a matrix of 66 points following a grid with one cm spacing. The results of both measurements were statistically analyzed using SPSS 10.0. Results The results of both measurements were highly correlated. The external occipital protuberance was the thickest while the region of cerebellar fossa was the thinnest. The regions two cm lateral to the midline between plane Five and plane Six, one cm lateral to the midline between plane Four and at plane Five, and median between plane Three and plane Four were found to have a thickness of more than eight mm. Conclusions The occipital thickness varies with individuals. CT measurement and direct anatomic measurement are highly correlated. Preoperative CT measurements can be reliable evidence for optimal screw placement before performing occipital-cervical fusions.