Search

Osteogenesis Imperfecta (OI) is a genetic disease characterized by osteopenia and bone fragility in which the craniocervical junction is also affected. This is of special relevance due to the high prevalence in anomalies described in the literature as follows: basilar invagination, basilar impression, and platybasia. Methods. We analyzed 19 lateral skull radiographs and 14 magnetic resonance images (MRIs) of 28 patients with OI in which eight linear and five angular measurements were plotted to determine the existence of craniocervical junction anomalies and compare them with 38 lateral skull radiographs and 28 MRIs performed on age-matched healthy controls. Results. From the reference values obtained from the control sample for each age group, we established the limit value at which pathology could be suspected. Some of the variables studied showed a clear trend associated with growth. More than half of the patients (60.71%) presented an anomaly in the skull base. Conclusions. According to the diagnostic criteria used and taking +2.5 SD as the limit value, 10.71% of the patients had basilar invagination, 35.71% had basilar impression, and 39.29% had platybasia, the latter being the most common finding, Depto. de Especialidades Clínicas Odontológicas, Fac. de Odontología, TRUE, pub

Osteogenesis Imperfecta (OI) is a disease that causes bone fragility and deformities, affecting both the cranial base and the craniocervical junction, and may lead to other neurological disorders. A retrospective cross-sectional study was carried out based on cephalometric analysis of the cranial base in a sample of patients with OI, in lateral skull radiographs and magnetic resonance imaging (MRI), comparing them with a sample of age-matched controls. When the different variables of the craniocervical junction were analyzed, significance was found in comparisons with the different age groups. All measurements of the variables studied stabilized as growth progressed. For most of the variables, the severity of the disease influences the measurements of the skull base, with statistically significant differences. Both age and severity of the disease are factors that directly influence the anatomy of the craniocervical junction in these patients and may serve as indicators in the early detection and prevention of other derived alterations., Depto. de Especialidades Clínicas Odontológicas, Fac. de Odontología, TRUE, pub

[Objective] Malformations in the craniocervical junction (CCJ) are rare in the pediatric population but often need surgical treatment. We present a pediatric case series of patients treated with a 2-stage surgical approach with a halo vest and occipitocervical fusion and review complications and outcomes., [Methods] A retrospective analysis of a single-center case series was performed. Pediatric patients affected by congenital craniocervical junction anomalies and treated with a 2-stage approach were included. A halo vest was implanted in the first surgery, and ambulatory progressive reduction was performed. When a favorable anatomic situation was observed, arthrodesis was performed. Safety analysis was undertaken by analyzing the incidence of complications in both procedures. Effectivity analysis was carried out analyzing radiologic and clinical outcome (Goel grade and modified Japanese Orthopaedic Association score). Student t test was used for statistical analysis., [Results] Sixteen cases were included. Mean age of patients was 9.38 years. Safety analysis showed 2 halo loosenings, 1 pin infection, 2 wound infections, 1 cerebrospinal fluid leak, and 2 delayed broken rods. No major complications were observed. Radiologic analysis showed an improvement in the tip of the odontoid process to the McRae line distance (from −3.26 mm to −6.16 mm), atlantodental interval (from 3.05 mm to 1.88 mm), clival-canal angle (from 134.61° to 144.38°), and cervical kyphosis (from 6.39° to 1.54°). Clinical analysis also showed improvement in mean Goel grade (from 1.75 to 1.44) and modified Japanese Orthopaedic Association score (from 15.12 to 16.41)., [Conclusions] The 2-stage approach was a suitable and effective treatment for craniocervical junction anomalies in pediatric patients.

Rationale: Atlantoaxial subluxation (AAS), caused by congenital factors, inflammation such as rheumatoid arthritis, infection, neoplasia, or trauma, is rare and severely erodes and subluxates atlantoaxial (AA) joints. For these patients, surgical reduction, and stabilization are difficult. Surgery, including anterior transoral decompression and posterior fixation, anterior endonasal decompression and fixation, and posterior decompression with AA or occipitocervical fixation, is often the only treatment available. However, there have only been 2 reports of C1-C2 facet spacer use in treating AAS. Here, we report the case histories of 3 patients with severely damaged and subluxated AA joints and symptomatic basilar invagination (BI), malalignment, or C2 root compression. Patient concerns: The cases included 2 women with rheumatoid arthritis and 1 man with spondyloarthropathy secondary to ulcerative colitis. Diagnosis: Radiographic imaging revealed severely damaged and subluxated AA joints. Their symptoms included worsening pain in the neck or occiput with or without myelopathy and neuralgia. Interventions: After realignment with C1-C2 spacers and posterior C1-C2 screw fixation, the patient symptoms were resolved. Outcomes: Of note, 2 of the 3 patients were healed without complications. One patient who underwent secondary revision surgery because of rod breakage and obvious nonunion at C0-C2 was determined to be healed at 1-year follow-up after the revision surgery. Lessons: We confirmed that C1-C2 facet spacers both reduced BI and occipitocervical coronal malalignment as well as releasing C2 root compression. Therefore, surgical restoration and fixation should be a required treatment in this very rare group of patients.

Rationale: Atlantoaxial subluxation (AAS), caused by congenital factors, inflammation such as rheumatoid arthritis, infection, neoplasia, or trauma, is rare and severely erodes and subluxates atlantoaxial (AA) joints. For these patients, surgical reduction, and stabilization are difficult. Surgery, including anterior transoral decompression and posterior fixation, anterior endonasal decompression and fixation, and posterior decompression with AA or occipitocervical fixation, is often the only treatment available. However, there have only been 2 reports of C1-C2 facet spacer use in treating AAS. Here, we report the case histories of 3 patients with severely damaged and subluxated AA joints and symptomatic basilar invagination (BI), malalignment, or C2 root compression. Patient concerns: The cases included 2 women with rheumatoid arthritis and 1 man with spondyloarthropathy secondary to ulcerative colitis. Diagnosis: Radiographic imaging revealed severely damaged and subluxated AA joints. Their symptoms included worsening pain in the neck or occiput with or without myelopathy and neuralgia. Interventions: After realignment with C1-C2 spacers and posterior C1-C2 screw fixation, the patient symptoms were resolved. Outcomes: Of note, 2 of the 3 patients were healed without complications. One patient who underwent secondary revision surgery because of rod breakage and obvious nonunion at C0-C2 was determined to be healed at 1-year follow-up after the revision surgery. Lessons: We confirmed that C1-C2 facet spacers both reduced BI and occipitocervical coronal malalignment as well as releasing C2 root compression. Therefore, surgical restoration and fixation should be a required treatment in this very rare group of patients.

Rationale: Atlantoaxial subluxation (AAS), caused by congenital factors, inflammation such as rheumatoid arthritis, infection, neoplasia, or trauma, is rare and severely erodes and subluxates atlantoaxial (AA) joints. For these patients, surgical reduction, and stabilization are difficult. Surgery, including anterior transoral decompression and posterior fixation, anterior endonasal decompression and fixation, and posterior decompression with AA or occipitocervical fixation, is often the only treatment available. However, there have only been 2 reports of C1-C2 facet spacer use in treating AAS. Here, we report the case histories of 3 patients with severely damaged and subluxated AA joints and symptomatic basilar invagination (BI), malalignment, or C2 root compression. Patient concerns: The cases included 2 women with rheumatoid arthritis and 1 man with spondyloarthropathy secondary to ulcerative colitis. Diagnosis: Radiographic imaging revealed severely damaged and subluxated AA joints. Their symptoms included worsening pain in the neck or occiput with or without myelopathy and neuralgia. Interventions: After realignment with C1-C2 spacers and posterior C1-C2 screw fixation, the patient symptoms were resolved. Outcomes: Of note, 2 of the 3 patients were healed without complications. One patient who underwent secondary revision surgery because of rod breakage and obvious nonunion at C0-C2 was determined to be healed at 1-year follow-up after the revision surgery. Lessons: We confirmed that C1-C2 facet spacers both reduced BI and occipitocervical coronal malalignment as well as releasing C2 root compression. Therefore, surgical restoration and fixation should be a required treatment in this very rare group of patients.

Rationale: Atlantoaxial subluxation (AAS), caused by congenital factors, inflammation such as rheumatoid arthritis, infection, neoplasia, or trauma, is rare and severely erodes and subluxates atlantoaxial (AA) joints. For these patients, surgical reduction, and stabilization are difficult. Surgery, including anterior transoral decompression and posterior fixation, anterior endonasal decompression and fixation, and posterior decompression with AA or occipitocervical fixation, is often the only treatment available. However, there have only been 2 reports of C1-C2 facet spacer use in treating AAS. Here, we report the case histories of 3 patients with severely damaged and subluxated AA joints and symptomatic basilar invagination (BI), malalignment, or C2 root compression. Patient concerns: The cases included 2 women with rheumatoid arthritis and 1 man with spondyloarthropathy secondary to ulcerative colitis. Diagnosis: Radiographic imaging revealed severely damaged and subluxated AA joints. Their symptoms included worsening pain in the neck or occiput with or without myelopathy and neuralgia. Interventions: After realignment with C1-C2 spacers and posterior C1-C2 screw fixation, the patient symptoms were resolved. Outcomes: Of note, 2 of the 3 patients were healed without complications. One patient who underwent secondary revision surgery because of rod breakage and obvious nonunion at C0-C2 was determined to be healed at 1-year follow-up after the revision surgery. Lessons: We confirmed that C1-C2 facet spacers both reduced BI and occipitocervical coronal malalignment as well as releasing C2 root compression. Therefore, surgical restoration and fixation should be a required treatment in this very rare group of patients.

Rationale: Atlantoaxial subluxation (AAS), caused by congenital factors, inflammation such as rheumatoid arthritis, infection, neoplasia, or trauma, is rare and severely erodes and subluxates atlantoaxial (AA) joints. For these patients, surgical reduction, and stabilization are difficult. Surgery, including anterior transoral decompression and posterior fixation, anterior endonasal decompression and fixation, and posterior decompression with AA or occipitocervical fixation, is often the only treatment available. However, there have only been 2 reports of C1-C2 facet spacer use in treating AAS. Here, we report the case histories of 3 patients with severely damaged and subluxated AA joints and symptomatic basilar invagination (BI), malalignment, or C2 root compression. Patient concerns: The cases included 2 women with rheumatoid arthritis and 1 man with spondyloarthropathy secondary to ulcerative colitis. Diagnosis: Radiographic imaging revealed severely damaged and subluxated AA joints. Their symptoms included worsening pain in the neck or occiput with or without myelopathy and neuralgia. Interventions: After realignment with C1-C2 spacers and posterior C1-C2 screw fixation, the patient symptoms were resolved. Outcomes: Of note, 2 of the 3 patients were healed without complications. One patient who underwent secondary revision surgery because of rod breakage and obvious nonunion at C0-C2 was determined to be healed at 1-year follow-up after the revision surgery. Lessons: We confirmed that C1-C2 facet spacers both reduced BI and occipitocervical coronal malalignment as well as releasing C2 root compression. Therefore, surgical restoration and fixation should be a required treatment in this very rare group of patients.

Presented in part at the AAPMR 71st Annual Assembly Meeting, Seattle, WA, November 4-7, 2010. Atlantoaxial instability results from cartilaginous destruction, periarticular erosions, and ligament and tendon attenuation. Instability affects 19%-70% of patients, and basilar invagination from vertical odontoid subluxation through the foramen magnum occurs in 38% of patients. This phenomenon occurs twice as often in women than men, whose age at diagnosis typically ranges from 30-50 years. Along with bony compression, the pannus further decreases the space available for the cord by 3mmor more in approximately 66% of patients (Figures 1-7). The earliest and most common symptom of cervical subluxation is pain radiating up into the occiput with associated headaches. Episodes of medullary dysfunction that represent severe but less common patterns of progressive myelopathic symptoms provide an even more grim prognosis. When cervical myelopathy is established, 50% of these patients die within 1 year.