Successful Treatment of Basilar Invagination and Platybasia Associated With Cerebellar Atrophy by Decompression Surgery.
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ABSTRACT:
SUBMITTER: Lee J
PROVIDER: S-EPMC8926774 | biostudies-literature | 2022 Mar
REPOSITORIES: biostudies-literature
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Successful Treatment of Basilar Invagination and Platybasia Associated With Cerebellar Atrophy by Decompression Surgery.
Lee Juhee J Guk Hyung Seok HS Kim Museong M Lee Eung-Joon EJ
Journal of clinical neurology (Seoul, Korea) 20220301 2
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Project description:ObjectiveTo investigate the cervical alignment and the relative range of motion (ROM) in patients with basilar invagination (BI).MethodsA total of 40 BI cases (38.1 years old ± 17.9 years old, 19 male and 21 female) and 80 asymptomatic individuals (33.8 years old ± 10.8 years old, 40 male and 40 female) were included. The Skull-C2 /Skull-BV, Skull-C7 , C2 -C7 /BV-C7 wall angles, C0 -C2 /C0 -BV, C0 -C7 , C1 -C7 , and C2 -C7 /BV-C7 angles were measured in dynamic X-ray images (including neutral, extension, and flexion positions). Correlation between the upper and lower cervical curvatures were analyzed. The total, extension, and flexion ROMs of these angles were calculated, respectively.ResultsThe BI patients had a smaller C0 -C2 /C0 -BV angle (18.2° ± 16.4° vs 30.9° ± 9.3°), but larger C2 -C7 /BV-C7 (32.2° ± 16.1° vs 19.4° ± 10.6°) and C2 -C7 /BV-C7 wall angles (37.8° ± 17.2° vs 23.6° ± 10.2°) than the control group in neutral position. The upper and lower curvatures correlated negatively in neutral (r = -0.371), extension (r = -0.429), and flexion (r = -0.648) positions among BI patients, as well as in extension position (r = -0.317) among control group. The BI patients presented smaller total ROMs in Skull-C2 /Skull-BV (12.3° ± 16.6° vs 19.7° ± 10.9°), C0 -C2 /C0 -BV (8.1° ± 11.1° vs 17.6° ± 10.5°), and C0 -C7 angles (57.8° ± 14.2° vs 78.3° ± 17.9°), but a larger total ROM in C2 -C7 /BV-C7 wall angle (52.8° ± 13.9° vs 27.0° ± 16.1°) than the control group. The BI patients also presented smaller extension ROMs in Skull-C2 /Skull-BV (6.9° ± 9.4° vs 12.5° ± 9.3°), Skull-C7 (24.5° ± 10.9° vs 30.7° ± 12.5°), and C0 -C2 /C0 -BV angles (4.4° ± 7.8° vs 9.9° ± 8.6°) than the control group. Moreover, the BI patients showed smaller absolute values of flexion ROMs in Skull-C2 /Skull-BV (-5.2° ± 9.4° vs -7.3° ± 8.0°), C0 -C2 /C0 -BV (-3.2° ± 8.8° vs -7.7° ± 8.7°), and C0 -C7 angles (-33.2° ± 13.0° vs -52.8° ± 19.2°), but a larger absolute value of flexion ROM in C2 -C7 /BV-C7 wall angle (-33.9° ± 14.8° vs -8.2° ± 15.1°).ConclusionThe cervical spine was stiffer in BI patients than the asymptomatic individuals, especially in the upper cervical curvature. The negative correlation between upper and lower cervical curvatures was more obvious in BI patients.
| S-EPMC8926990 | biostudies-literature
Project description:ObjectiveThis study evaluated the feasibility and efficacy of quantitative reduction and fixation to treat basilar invagination (BI) with atlantoaxial dislocation (AAD).MethodsPosterior occipitocervical angle (POCA), occiput-C2 angle (Oc-C2A), clivusaxial angle (CAA), and C2-7 angle (C2-7A) were considered for quantitative reduction. Twelve patients with BI complicated with AAD received posterior interarticular release and individualized cage implantation to restore vertical dislocation. The POCA was adjusted using cantilever technology to further reduce the horizontal dislocation and adjust lower cervical vertebral angle. All patients received a radiological follow-up for ≥12 months. Improvements in spinal cord function were evaluated using Japanese Orthopedic Association (JOA) score.ResultsAll the patients received successful quantitative reduction for BI-AAD, and bony fusion was achieved without spinal cord injury after surgery for 12 months. The JOA score was improved significantly to 15.2 ± 0.9 twelve months after surgery (p < 0.01). Radiological follow-up revealed that individualized cage and POCA play vital roles in quantitative correction: (1) distance of the dens above McRae's line and atlantodens interval were restored to normal level, respectively; (2) changes in Oc-C2 angle (ΔOc-C2A), C2-7 angle (ΔC2-7A), clivus-axial angle (ΔCAA), and POCA (ΔPOCA) were all caused by changes in axis tilt. Based on the changes of radiological parameter we deduced the formula for quantitative reduction by linear regression analysis: -ΔPOCA = ΔOc-C2A = -ΔC2-7A = ΔCAA.ConclusionQuantitative posterior reduction by individualized cage and adjusting ΔPOCA is feasible for treating BI with AAD.
| S-EPMC7538363 | biostudies-literature
Project description:ObjectiveSurgery is indicated for basilar invagination (BI) in symptomatic patients. In many patients, symptoms and signs occur due to an upward-migrated and malaligned odontoid with fixed or mobile atlantoaxial instability. Posterior distraction and fixation of the atlantoaxial joints has evolved to become the standard of care, but has some inherent morbidity. In this study, we propose that the unilateral anterior submandibular retropharyngeal approach with customized wedge-shaped titanium cages inserted into both atlantoaxial joints and anterior atlantoaxial fixation with a plate screw construct is a safer and easier option in many cases of BI.MethodsFrom February 2014 to February 2019, 52 patients (age range, 15-78 years; 40 males and 12 females) with symptomatic BI with atlantoaxial dislocation and minimal sagittal facetal inclination and only mild Chiari malformation without syringomyelia were offered anterior submandibular retropharyngeal atlantoaxial distraction and fixation surgery.ResultsNeurological improvement occurred in 80% of patients, while the neurological status of 20% remained unchanged. No patients worsened, and no major complications or mortality was observed.ConclusionIn properly selected cases of symptomatic BI, anterior wedge cage distraction with anterior atlantoaxial fixation is a safe and simple option.
| S-EPMC6603832 | biostudies-literature
Project description:ObjectivesTo investigate the diagnostic value of clivopalate angle (CPA) for basilar invagination (BI) at magnetic resonance imaging (MRI).MethodsIn this retrospective case-control study, CPA, clivodens angle (CDA), and clivoaxial angle (CXA) were measured on midsagittal MR images from 112 patients with BI (22 men; mean age, 43.9 years ± 13.1 years; range, 21-79 years) and 200 control subjects (57 men; mean age, 47.1 years ± 13.3 years; range, 20-80 years). Intraclass correlation coefficient (ICC), linear regression, Mann-Whitney U test, binary logistic regression, and receiver operating characteristic (ROC) curve were used for statistical analysis.ResultsClivopalate angle showed better inter-observer agreement (ICC = 0.951) than CXA (0.867) or CDA (0.853). CPA significantly correlated with CXA (R = 0.811, p < 0.001) and CDA (R = 0.716, p < 0.001). Patients with BI had a significantly smaller CPA (45.9° ± 9.9°) than control subjects (61.9° ± 6.2°) (p < 0.001). With the optimal cutoff value of 53.5°, CPA had a sensitivity of 0.839 (94/112) and a specificity of 0.915 (183/200). The area under the ROC curve (AUC) was 0.937 (95% CI, 0.911-0.963) for CPA, which was similar to that of CXA (AUC, 0.957; 95% CI, 0.936-0.978) or CDA (AUC, 0.925; 95% CI, 0.892-0.957). The combination of CPA and CDA or CXA showed a higher diagnostic value than CDA or CXA alone.ConclusionsThe diagnostic performance of CPA was similar to that of CXA or CDA, but CPA might be more reliable in evaluation of BI. CPA provided complementary information to CXA and CDA.Key points• Clivopalate angle has a high diagnostic value for basilar invagination. • Clivopalate angle demonstrates high inter-reader agreement than does clivoaxial angle or clivodens angle. • Clivopalate angle provides complementary information to clivoaxial angle and clivodens angle.
| S-EPMC6554245 | biostudies-literature
Project description:Fibrous dysplasia (FD) is a mosaic disorder of benign fibro-osseous lesions, which may be associated with extraskeletal features as part of McCune-Albright syndrome (MAS). Cranial base abnormalities, including Chiari I malformation (CM1), in which the cerebellum extends below the foramen magnum, and secondary basilar invagination (BI), in which the odontoid prolapses into the posterior cranial fossa, are potentially serious complications of metabolic bone disorders. The purpose of this study was to determine the prevalence, natural history, and risk factors for CM1 and BI in patients with FD/MAS, and to determine mechanisms of cranial base deformities. Clinical and radiographic data from subjects in an FD/MAS natural history study were evaluated and compared to normal controls. In 158 patients with craniofacial FD, 10 (6.3%) cases of CM1 and 12 (7.6%) cases of BI were diagnosed. No cranial base abnormalities were identified in 10 control subjects. Craniomorphometric and volumetric analyses identified cranial constriction and cranial settling as the primary mechanisms of cranial base abnormalities, whereas intracranial hypertension was a contributing factor in a minority of subjects. Longitudinal analyses found progression of odontoid position with age, but no progression of tonsillar position. No endocrinopathies were associated with CM1. MAS endocrinopathies associated with BI included hyperthyroidism (odds ratio [OR] 12.0; 95% confidence interval [CI], 2.9 to 55.6; p < 0.01), precocious puberty (OR 5.6; 95% CI, 1.2 to 26.0; p < 0.05), and hypophosphatemia (OR 7.7; 95% CI, 1.9 to 27.0; p < 0.01). Scoliosis was associated with both CM1 (OR 4.8; 95% CI, 1.1 to 22.8; p < 0.05) and BI (OR = infinity; 95% CI, 4.7 to infinity; p < 0.01). This study successfully characterized cranial base abnormalities in FD/MAS and the pathophysiological connection between them. These findings support routine screening for cranial base abnormalities in patients with craniofacial FD, as well as aggressive management of contributory risk factors. © 2018 American Society for Bone and Mineral Research.
| S-EPMC6218312 | biostudies-literature
Project description:BackgroundEvidence from randomized controlled trials has shown a benefit for endovascular treatment in basilar artery occlusion. We aimed to show the effect of the recanalization result on outcome and determine the role of underlying basilar stenosis in a real-world setting.MethodsA retrospective, single-center study of patients who received endovascular treatment for basilar artery occlusion from March 2008 to June 2022 was conducted. Clinical and outcome characteristics were gathered. Multivariate logistic regression analysis was performed to predict poor outcomes (post-treatment mRS 5 or 6). MRS shift analysis was performed.ResultsThis study includes 210 patients (mean age, 71.4 years +/- 13.3 [standard deviation]; 124 men). The variables age (OR, 1.04; 95% CI: 1.01-1.08; p = 0.014), underlying basilar stenosis (OR: 4.86; 95% CI: 2.15-10.98; p < 0.001), admission NHISS (OR: 1.09; 95% CI: 1.04-1.13; p < 0.001), and TICI (OR: 1.89; 95% CI: 1.09-3.25; p = 0.022) independently predicted a poor outcome. Patients with occlusions due to underlying stenosis had significantly worse recanalization rates. Median post-treatment mRS in all patients with embolic occlusion was 4; IQR, 2-5 (only patients with embolic occlusion: mTICI 0-2a, median: 5 [IQR, 4-5.5]; mTICI 2b, median: 4 [IQR, 2.5-6]; mTICI 3, median: 3 [IQR, 1-5]; p = 0.037). Median post-treatment mRS in all patients with occlusions due to underlying stenosis was 5; IQR, 4-6 (only patients with embolic occlusions: mTICI 0-2a, median: 6 [IQR, 4.5-6]; mTICI 2b, median: 6 [IQR, 4.25-6]; mTICI 3, median: 5 [IQR, 3.5-5.25]; p = 0.059).ConclusionsSuccessful recanalization is essential for preventing poor outcomes in basilar artery occlusion. Underlying basilar stenosis diminishes the effect of successful recanalization on the overall outcome.
| S-EPMC11544911 | biostudies-literature
Project description:ObjectiveDevelopmental bony craniovertebral junction (CVJ) anomalies seem to have a genetic basis and also abnormal joint morphology causing atlantoaxial dislocation (AAD) and basilar invagination (BI).MethodsDNA extracted polymerase chain reaction single-stranded conformation polymorphism (SSCP) performed for mutation screening of FBN1 gene (n = 50 cases+ 50 age/sex-matched normal; total: 100). Samples with a deviated pattern of bands in SSCP were sequenced to detect the type of variation. Computed tomography (CT) scans of 100 patients (15-45 years old) compared with an equal number of age/sex-matched controls (21.9 ± 8.2 years). Joint parameters studied: sagittal joint inclination (SI), craniocervical tilt (CCT), coronal joint inclination (CI).ResultsThirty-nine samples (78%) showed sequence variants. Exon 25, 26, 27, and 28 showed variable patterns of DNA bands in SSCP, which on sequencing gives various types of DNA sequence variations in intronic region of the FBN1 gene in 14%, 14%, 6%, and 44% respectively. CT radiology:SI and CCT correlated with both BI and AAD (p < 0.01). The mean SI value in controls: 83.35° ± 8.65°, and in patients with BI and AAD:129° ± 24.05°. Mean CCT in controls: 60.2° ± 9.2°, and in patients with BI and AAD: 86.0° ± 18.1°. Mean CI in controls:110.3° ± 4.23°, and in cases: 125.15° ± 16.4°.ConclusionThe study showed mutations in FBN1 gene (reported in Marfan syndrome). There is also an alteration of joint morphology, correlating with AAD and BI severity. Hence, we propose a double-hit hypothesis: the presence of weak ligaments (due to FB1 gene alterations) and abnormal joint morphology may contribute to AAD and BI.
| S-EPMC7788422 | biostudies-literature
Project description:INTRODUCTION:Recently, a novel hypothesis has been proposed concerning the origin of craniovertebral junction (CVJ) abnormalities. Commonly found in patients with these entities, atlantoaxial instability has been suspected to cause both Chiari malformation type I and basilar invagination, which renders the tried and tested surgical decompression strategy ineffective. In turn, C1-2 fusion is proposed as a single solution for all CVJ abnormalities, and a revised definition of atlantoaxial instability sees patients both with and without radiographic evidence of instability undergo fusion, instead relying on the intraoperative assessment of the atlantoaxial joints to confirm instability. METHODS:The authors conducted a comprehensive narrative review of literature and evidence covering this recently emerged hypothesis. The proposed pathomechanisms are discussed and contextualized with published literature. CONCLUSION:The existing evidence is evaluated for supporting or opposing sole posterior C1-2 fusion in patients with CVJ abnormalities and compared with reported outcomes for conventional surgical strategies such as posterior fossa decompression, occipitocervical fusion, and anterior decompression. At present, there is insufficient evidence supporting the hypothesis of atlantoaxial instability being the common progenitor for CVJ abnormalities. Abolishing tried and tested surgical procedures in favor of a single universal approach would thus be unwarranted.
| S-EPMC7295832 | biostudies-literature
Project description:The overall aim of this study was to assess the effectiveness of endoscopic decompression for outcomes in patients with lumbar spinal stenosis (LSS). We conducted a retrospective cohort, single-institution study of n = 139 patients from 2019 to 2022 who underwent endoscopic decompression for LSS. The primary outcome was improvement of Oswestry Disability Index (ODI) between baseline and 12-month follow-up. In the present sample (n = 139) the average age was 57.6 years (SD = 17.4, with even distribution of men (49%) vs. women (51%). In patients with LSS, lumbar disc herniation was the most common diagnosis in 49 patients followed by lumbar radiculopathy in 25 patients. Lumbar radicular pain was the 3rd most common diagnosis in 21 patients with all other diagnosis listed in Table S1. There was a significant improvement (i.e., decrease) in ODI following endoscopic decompression (mean change: -8.3, 95% CI: -9.4, -7.2, P < 0.001, Fig. 1). Prior lumbar spine surgery (P = 0.048), BMI (P = 0.053), and age (P = 0.022) were associated with changes in ODI. Nearly half (47%) of the sample had prior lumbar spine surgery. Those with prior lumbar spine surgery (-7.5, 95% CI: -8.3, -6.6) showed less improvement than those without prior lumbar spine surgery (-9.1, 95% CI: -10.9, -7.2, Fig. 2). For BMI, 23% had normal BMI while 24% were overweight and 53% were obese. Patients with normal BMI (-10.3, 95% CI: -13.4, -7.2) showed greater improvements compared to overweight (-7.9, 95% CI: -9.4, -6.4) and obese (-7.6, 95% CI: -9.0, -6.3, Fig. 3) patients. Patients under 40 years old (-10.2, 95% CI: -13.6, -6.8) showed greater improvements in ODI compared to those 40 years and older (-7.8, 95% CI: -8.6, -6.8, Fig. 4). In patients with lumbar spinal stenosis, endoscopic decompression was associated with reduced disability. Patients with no prior lumbar spine surgery, normal BMI, and who were under 40 years old showed greater improvements.
| S-EPMC11373059 | biostudies-literature
Project description:ObjectiveTo investigate the characteristics of functional muscle and muscle size in patients with basilar invagination (BI) and explore the effects of atlantoaxial dislocation.MethodsEighty BI patients (BI group) and 80 age- and sex-matched asymptomatic people (control group) were included. Axial T2 magnetic resonance imaging image was used to measure the cross-sectional area (CSA) and functional CSA (FCSA). The sternocleidomastoid (SCM), longus capitis and longus colli (LCap & LC), trapezius (Trap), splenius capitis (SpCap), splenius cervicis (SpC), semispinalis capitis (SSCap), semispinalis cervicis (SSC), multifidus (MS), levator scapulae (LS) and posterior deep layer muscles (PDLM) were evaluated. Correlations between age, atlantodental interval (ADI), Chamberlain distance and muscles were observed.ResultsBI group (39.4 ± 18.4 years; 33 males/47 females) exhibited significantly lower FCSA/CSA ratios than the control group in all extensor and flexor muscles, and presented smaller CSAs on the right and left Trap, SSC, LS, SCM, and left LCap & LC. FCSA/CSA ratios were significantly lower in BI patients with dislocation on the right Trap, SpCap, SpC, SSCap, MS, LS, LCap & LC, and PDLM, and the left SSCap, MS, and LCap & LC than in patients without deformity. Additionally, functional muscles of all parameters decreased with age in BI patients. Excluding children, the Trap, SpC, MS, and LS muscle sizes of BI patients tended to increase with age. ADI and Chamberlain distance tended to correlate negatively with FCSA/CSA ratio.ConclusionThe BI patients, especially those with atlantoaxial dislocation, had less functional muscles compared with the control group. Moreover, their functional muscles decreased with age more obviously.
| S-EPMC10562225 | biostudies-literature