Atlantoaxial dislocation due to os odontoideum in patients with Down's syndrome: literature review and case reports.

PURPOSE: To clarify etiology, clinical features, and diagnostic and treatment options of atlantoaxial dislocation (AAD) due to os odontoideum (OsO) in patients with Down's syndrome (DS). METHODS: We described and analyzed three clinical cases of AAD due to OsO in DS patients and reviewed descriptions of similar cases in the scientific sources. RESULTS: According to literature review, more than 80% of DS patients with odontoid ossicles had atlantoaxial instability (AAI). AAI in DS patients with OsO is more often manifested in childhood and adolescence, rarely in adults when ligament relaxation is reduced. Some patients had acute clinical manifestation after a minor trauma without any precursors; in some of the cases, neurological deterioration increased during several years. We found that the earlier surgical treatment of AAD due to OsO in DS patients carries the higher recovery potential. CONCLUSIONS: Most patients with DS and OsO had AAI. The method of appropriate treatment in such cases is a posterior screw fixation. Preoperative halo traction and posterior fusion have proved to be a very useful tool in the treatment of AAD due to OsO in DS patients. Even if irreducibility of the AAD established preoperatively, it should not be an absolute indication for anterior decompression. In such cases, an attempt to reduce the AAD should be made under general anesthesia during posterior fixation.

Reliability and validity of the pediatric adaptation of the mJOA scale for evaluating cervical spine disorders.

PURPOSE: The study aimed to evaluate the validity and reliability of the pediatric adaptation of the Japanese Orthopedic Association (mJOA) scale in pediatric patients with various cervical spine pathologies. METHODS: Initial assessments were performed by a neurosurgeon, followed by an independent evaluation by a neurologist within 1-2 days to test inter-rater reliability. The same clinician assessed the same group of children using the adapted mJOA scale at different point in time (between 1 month and 1 year after the initial assessment) to test intra-rater reliability. For known-groups validity, the pediatric mJOA scale assessments were compared between two groups of pathologies with different prognosis. Concurrent validity was assessed against the McCormick scale, and convergent validity was tested by reassessing patients using the adult mJOA scale two or more years after the initial assessment by pediatric one. RESULTS: A cohort of 169 pediatric patients aged 6 months to 18 years (mean age: 10 ± 4.6 years) with various cervical spine pathologies was recruited. Pathologies included atlanto-axial rotatory fixation (AARF), Chiari type I anomaly, congenital cervical spine scoliosis, atlanto-axial dislocation (AAD) and instability (AAI), cervical spine stenosis and trauma, and congenital cervicothoracic dislocations. The majority of patients underwent cervical spine surgery and were followed up for an average of 6.9 ± 2.97 years. The pediatric mJOA scale demonstrated high inter-rater reliability (r = 0.99, p < 0.0001) and strong intra-rater reliability (r = 0.82, p < 0.0001). Significant differences in pediatric mJOA scores were observed between patients with expected-intact neurological status and those with expected-pathological neurological status (p < 0.0001). The pediatric mJOA scale showed a strong correlation with the McCormick grading system (r = 0.97, p < 0.001) and good correlation with the adult mJOA scale during long-term follow-up (r = 0.82, p < 0.0001). CONCLUSIONS: The pediatric version of the mJOA scale is a reliable and valid tool for assessing pediatric patients with cervical spine disorders. Its high reliability and validity support its use in both clinical practice and research.

Segmental Spinal Aplasia: Anatomical Variations and Treatment Insights.

STUDY DESIGN: Retrospective Cohort Study. OBJECTIVE: The study aimed to analyze anatomical variants of segmental spinal aplasia (SSA) and investigate factors influencing surgical treatment outcomes, with a specific focus on the incidence of complications. METHODS: The study focused on patients with SSA treated at a single medical center, with over two years of follow-up. Neurological function changes were evaluated using the modified Japanese Orthopedic Scale (mJOA). Functional independence was measured using the Functional Independence Measure (FIM/WeeFIM) scale, and complications, well-being, and reoperation instances were documented. Statistical analyses used ANOVA and Kruskal-Wallis test. RESULTS: The predominant localization of SSA in 36 own cases occurs near or at the level of the thoracolumbar junction, often accompanied by significant spinal cord narrowing and a low position of the conus medullaris. Additionally, it frequently presents with aplasia of the lower ribs. Cervicothoracic SSA was more commonly associated with segmentation disorders (P = .04). The most common early complications were wound problems (17%) and neurological deterioration (17%); the most common late complications were: non-fusion (34%); 38% of patients required one or more revision surgery. The type, age of surgery, level of surgery, and initial neurological deficient did not significantly influence the incidence of complications or neurological and functional outcomes. CONCLUSION: SSA, a range of anomalies appearing early in childhood, progresses gradually. Surgery involves vertebrectomy followed by interbody fusion and screw fixation, guided by neurophysiological monitoring. Surgery is recommended for worsening neurological symptoms, but conservative options like bracing can be considered, due to a high risk of complications.

Optimizing sacral screw fixation in patients with caudal regression syndrome.

PURPOSE: The aim of this study is to evaluate and compare techniques and outcomes associated with two different technique of pelvic screw insertion in patients with caudal spine absence. METHODS: A cohort of patients with varying degrees of caudal structural regression, serves as the focal point of this investigation. Pelvic configurations were classified based on established criteria to facilitate comparative analysis. Each patient underwent spinal surgical interventions, with a follow-up period extending beyond 2 years. The primary surgical interventions predominantly involved spinal stabilization coupled with correction of scoliosis and kyphosis through one or two pairs of pelvic screws. RESULTS: In this study, we investigated a cohort of 22 patients with caudal spine absence, encompassing diverse conditions, such as lumbo-sacral aplasia, hemisacrum, and lumbar absence, with preserved sacrum. Following spinal surgery, notable improvements were observed in scoliosis and pathological lumbar kyphosis, with several patients achieving significant functional milestones such as independent ambulation. There were no significant differences in short-term complications between patients undergoing single versus double pair pelvic screw implantation. Long-term complications, primarily non-fusion, were notably more prevalent in patients undergoing fixation with a single pair of pelvic screws. CONCLUSION: Surgical intervention, particularly spinopelvic fixation, demonstrated promising outcomes in terms of improving spinal deformities. The implantation of two pairs of pelvic screws demonstrates greater reliability compared to the insertion of a single pair, diminishing the risk of non-fusion.

Cervicothoracic dislocation due to congenital and bone-dysplasia-related vertebral malformations.

PURPOSE: To evaluate the approaches to treatment of congenital and bone-dysplasia-related pediatric cervicothoracic dislocations and define the optimal treatment method. METHODS: The publications available in PubMed and Google Scholar data bases were selected following such criteria as the disease in question, pediatric age, the treatment description, and follow-up results. The paper also includes the descriptions of our own six cases of the cervicothoracic dislocations detected in children with different vertebral malformations. RESULTS: Only eight patients meeting the abovementioned selection criteria were found in the publications: three of them had the Klippel-Feil syndrome (KFS), two had one-level vertebral anomaly, one had neurofibromatosis (NF type 1), one had the Larsen syndrome, and one had a variation of VACTERL association. Their treatment was long term, multi stage, and complicated. Among six our own cases, four patients also had KFS, one had a variation of VACTERL association, and one had NF type 1. All the patients suffered from preoperative neurological disorders. Posterior instrumental fixation with posterior vertebral body resection was performed in four cases and one patient underwent a combined surgery. The parents of one of the patients refused the operation, so he was observed while receiving bracing treatment. Since the treatment was long term and complicated by reoperations, the average follow-up period comprised 5 years. CONCLUSION: Congenital cervicothoracic dislocations are an extremely rare pathology that manifests itself in early age and requires an early surgical treatment. Failure to provide the treatment leads to the patient's disability. The surgical tactics for such patients is determined individually, but the published data and our own experience demonstrate that early multi-stage combined treatment has been the best option available so far. The cervicothoracic dislocations due to NF 1 manifest later and have a more favorable forecast.

An alternative way of C1 screwing: Supralaminar C1 lateral mass screws.

STUDY DESIGN: This study involves literature review, technical note, and case series. OBJECTIVES: The objectives were to analyze indications and contraindications, advantages, and disadvantages for C1 lateral mass screw (LMS) insertion above or partially above the arch, to descript technical features, and to give examples of the practical application of this technique and investigated its safety. METHODS: A literature review was carried out in English and Russian in PubMed, Google Scholar, and eLibrary databases. We selected four patients, treated in our clinic, which was carried out partially supralaminar C1 LMS. RESULTS: Only three descriptions of supralaminar C1 LMS were found in the literature. Four adult patients underwent posterior C1-C2 screw fixation with C1 LMS along the superior edge of the C1 arch at our clinic. Partially supralaminar C1 screws were inserted on one of the sides due to the difficulties of using classical techniques. The main reasons for supralaminar screw fixation were narrow C1 lamina, hypertrophied venous plexus, and intraoperative failures of classic techniques application (broken screw trajectory, profuse venous bleeding from the plexus). The average follow-up time for the patients was 2.7 years, no complications were noted, and all had a satisfactory spinal fusion. CONCLUSIONS: The proposed types of C1 LMS above or partially above the C1 arch can be useful alternative method of C1 screwing in selected patients. Indications for the use of the supralaminar C1 LMS method can be narrow C1 posterior arch and pedicle, pronounced C1-C2 venous plexus, some V3 segment anomalies at C1 level, small arthritic inferior part of lateral mass, and intraoperative failures of classic techniques application.

Spectroscopic and theoretical study of the dimeric dicationic fullerene complex [(C70)2]2+ (Ti3Cl13)(-)2.

The first spectroscopic characterization of the dimeric dicationic fullerene complex [(C(70))(2)](2+)(Ti(3)Cl(13))(-)(2) is reported and supported by DFT calculations. The IR spectrum of the dimer is interpreted in terms of the normal modes of the pristine C(70), and the effects of charging C(70) and the intercage bond formation between C(70) units on the IR spectrum are discussed. Analysis of the vibrational spectrum of the anion, Ti(3)Cl(13)(-), is also provided. NIR absorption and fluorescence spectra of the complex are studied, and the dimer is shown to have a small HOMO-LUMO gap of 0.8 eV. The electronic structure of [(C(70))(2)](2+) is studied with the use of DFT and compared to that of the other single-bonded fullerene dimers, including [(C(70))(2)](2-), (C(69)N)(2), and [(C(60))(2)](2-). Characteristic features in the vibrational spectra and electronic structure of all single-bonded fullerene dimers are revealed.

Surgical Management of Myelomeningocele-Related Spinal Deformities.

OBJECTIVE: To evaluate the optimal timing and type of surgical treatment of myelomeningocele (MMC)-related spinal deformities and long-term follow-up of surgical treatment. METHODS: We reviewed and presented clinical pictures, treatment strategies and results of 20 patients with MMC-related spinal deformities treated at our center between 2010 and 2017. RESULTS: The average patient age was 6.3 years. The average preoperative neurologic status according to a modified Japan Orthopedic Association (mJOA) scale was 7.3 points (Benzel's modification). Average functional status was 41 points according to a functional independent measure scale (FIM). The average angle of kyphosis was 83.7°, that of scoliosis was 36.7°, and that of lordosis was 67° (Cobb angles). The average duration of surgery was 234 minutes, and the average total blood loss was 175 mL. The average angle of kyphosis correction was 61°, that of scoliosis correction was 25°, and that of lordosis correction was 25° (Cobb angles). The average duration of hospitalization was 16.6 days, and the average follow-up was 34.5 months. The total number of complications was 13. Reoperation was required in 9 cases. Neurologic status according to the mJOA scale improved by 0.6 point on average. Functional status according to the FIM increased by 6.6 points on average. CONCLUSIONS: Early surgical correction of MMC-related spinal deformities improves body balance and quality of life. The dual growing rod technique is safe and effective in cases of moderate neuromuscular spinal deformities at an early age. Kyphectomy is a challenging procedure with high complication rates, especially skin problems, but there are no alternative procedures for cases of heavy rigid kyphosis.