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.

Association of spinal cord abnormalities with vertebral anomalies: an embryological perspective.

PURPOSE: To analyze the relationship between spinal cord and vertebral abnormalities from the point of view of embryology. METHODS: We analyzed the clinical and radiological data of 260 children with different types of spinal cord malformations in combination with vertebral abnormalities. RESULTS: Among 260 individuals, approximately 109 presented with open neural tube defects (ONTDs), 83 with split cord malformations (SCMs), and 83 with different types of spinal lipomas. Pathological spina bifida emerged as the most frequent vertebral anomaly, affecting 232 patients, with a higher prevalence in ONTD. Vertebral segmentation disorders, including unsegmented bars, butterfly vertebrae, and hemivertebrae, were present in 124 cases, with a higher prevalence in SCM. The third most common spinal anomaly group consisted of various forms of sacral agenesis (58 cases), notably associated with blunt conus medullaris, spinal lipomas, and sacral myelomeningocele. Segmental aplasia of the spinal cord had a typical association with segmental spinal absence (N = 17). CONCLUSION: The association between SCM and neuroenteric cyst/canal and vertebral segmentation disorders is strong. High ONTDs often coincide with pathological spina bifida posterior. Type 1 spinal lipomas and focal spinal nondisjunction also correlate with pathologic spina bifida. Segmental spinal absence or dysgenesis involves localized spinal and spinal cord aplasia, sometimes with secondary filar lipoma.

Hidden neuraxial pathology in idiopathic scoliosis: original research / Patologia neuroaxial oculta na escoliose idiopática: pesquisa original / Patología neuroaxial oculta en la escoliosis idiopática: investigación original

ABSTRACT Objective: To evaluate the prevalence of hidden neuraxial pathology (NAP) revealed in idiopathic scoliosis (IS) in neurologically normal patients. Methods: We selected 401 patients with IS who visited our clinic. We identified patterns of NAP and its frequency. In addition to the main part of the study, we assessed the reliability of Magnetic Resonance Imaging (MRI) measuring of the pedicles to plan screw width and trajectory. Results: Among the 401 patients, 53 (13%) presented NAP. The proportion of males in this group was higher (42% vs. 21%, p=0.004), the age of onset of the deformity was lower (8.9±3.77 vs. 9.9±3.93 years old, p=0.045), the left-sided thoracic curve was more frequent (21% vs. 8%, p=0.016), thoracic kyphosis was more pronounced (p=0.070), and the percentage of revision surgeries for deformity progression or non-fusion was higher (13% vs. 5%, p=0.147). Conclusions: The spine MRI should be performed in the early stages of IS, as in some cases of NAP (Chiari, tethered spinal cord), there is the possibility of an early neurosurgical operation that will prevent the development of scoliosis. The main signs of hidden NAP in IS are early-onset IS, IS with left-sided thoracic curve, male gender, and thoracic kyphosis > 40°Cobb. Level of Evidence II; Retrospective Study.

Resumo: Objetivo: Avaliar a prevalência das patologias neuroaxiais ocultas (PNO) reveladas na escoliose idiopática (EI) em pacientes neurologicamente normais. Métodos: Foram selecionados 401 pacientes com EI que visitaram nossa clínica. Identificamos padrões de PNO e sua frequência. Além disso, avaliamos a confiabilidade da medição por ressonância magnética (RM) dos pedículos para planejar a largura e a trajetória do parafuso. Resultados: Entre os 401 pacientes, 53 (13%) apresentaram PNO. A proporção de homens neste grupo foi maior (42% contra 21%, p=0,004), a idade de início da deformidade foi menor (8,9±3,77 contra 9,9±3,93 anos, p=0,045), a curva torácica do lado esquerdo foi mais frequente (21% contra 8%, p=0,016), a cifose torácica foi mais pronunciada (p=0,070) e a porcentagem de cirurgias de revisão para progressão da deformidade ou não fusão foi maior (13% contra 5%, p=0,147). Conclusões: A ressonância magnética da coluna deve ser realizada nos estágios iniciais da EI, pois em alguns casos de PNO (Chiari, medula espinhal amarrada) existe a possibilidade de uma operação neurocirúrgica precoce que impedirá o desenvolvimento de escoliose. Os principais sinais de PNO oculta na EI são: EI de início precoce, EI com curvatura torácica à esquerda, sexo masculino e cifose torácica > 40°Cobb. Nível de Evidência II; Estudo Retrospectivo.

Resumen: Objetivo: Evaluar la prevalencia de las patologías neuroaxiales ocultas (PNO) reveladas en la escoliosis idiopática (EI) en pacientes neurológicamente normales. Métodos: Se seleccionaron 401 pacientes con EI que visitaron nuestra clínica. Se identificaron patrones de PNO y su frecuencia. Además, evaluamos la fiabilidad de la medición por resonancia magnética (RM) de los pedículos para planificar la anchura y la trayectoria del tornillo. Resultados: Entre los 401 pacientes, 53 (13%) presentaron PNO. La proporción de hombres en este grupo fue mayor (42% vs 21%, p=0,004), la edad de aparición de la deformidad fue menor (8,9±3,77 vs 9,9±3,93 años edad, p=0,045), la curva torácica del lado izquierdo se encontró con más frecuencia (21 % frente a 8 %, p=0,016), la cifosis torácica fue más pronunciada (p=0,070) y el porcentaje de cirugías de revisión por progresión de la deformidad o falta de fusión fue mayor (13% vs 5%, p=0,147). Conclusiones: La resonancia magnética de la columna debe realizarse en las primeras etapas de la EI, ya que en algunos casos de PNO (Chiari, médula anclada) existe la posibilidad de una operación neuroquirúrgica temprana que prevendrá el desarrollo de la escoliosis. Los principales signos de PNO oculta en EI son: EI de inicio temprano, EI con curvatura torácica izquierda, sexo masculino y cifosis torácica > 40°Cobb. Nivel de Evidencia II; Estudio Retrospectivo.

Treatment of spinal deformity with diastematomyelia type I: one-stage, two-stage surgery and new technique (vertebral column resection through wide bony septum).

STUDY DESIGN: Case series, literature review, and technical note. OBJECTIVES: To compare two different approaches to treat the spinal deformity with split cord malformation type I (SCM I). To present a new method of one-stage surgical treatment of congenital spinal deformity with wide bony septum (SCM I). METHODS: Analysis of the literature on the different types of combined surgical treatment of spinal deformities with SCM I was performed. We have provided our own data on 27 patients treated for congenital spinal deformity and SCM I, one of which underwent Schwab IV type osteotomy at the apex of the deformity through the bony septum and pedicles. Inclusion criteria were presence of spinal deformity in combination with SCM 1, performed surgery to correct spinal deformity, and follow-up period of at least 2 years. RESULTS: The result of the literature review was controversial and requires additional research. The average age of patients was 8.8 ± 6.6 years old. One-stage treatment of SCM I and spinal deformity was performed in 10 patients (group I) and two-stage in 14 patients (group II). Three patients with severe myelodysplasia, SCM I, and congenital kyphoscoliosis underwent correction of spinal deformity without SCM I removing (group III). The group I had the longest surgery duration (mean 289 ± 75 min) and largest blood loss (mean 560 ± 386 ml), a high percentage of deformity correction (mean 69.6%), and the highest rate of complications (60%). The most optimal was the two-stage treatment with the mean surgery duration 191 ± 137 min, mean blood loss 339 ± 436 ml, mean correction rate 63%, and frequency of complications 21%. The average follow-up time was 6.0 ± 2.6 years. CONCLUSIONS: One stage surgery associated with a large surgical invasion and a large number of complications. It can be used in some cases, for example when the wide bony septum (SCM I) is localized at the apex of the congenital scoliosis or kyphosis. In all other cases, it is worth adhering to a two-stage treatment. Many new works demonstrate the relative safety and effectiveness of deformity correction without removing the SCM. In our opinion, indications for treatment of spinal deformity without SCM I removing can be the need to perform a shortening ostetomy outside the SCM zone. The remaining cases require a thorough assessment and a balanced decision.

Frontal and sagittal patterns of neuromuscular spinal deformities based on nosological profile / Padrões frontais e sagitais das deformidades neuromusculares da coluna vertebral, segundo o perfil nosológico / Patrones frontal y sagital de las deformidades neuromusculares de la columna vertebral según el perfil nosológico

ABSTRACT Objective: Reveal frontal and sagittal patterns of spinal deformity depending on neuromuscular nosology for surgery and outcome planning. The characteristics of spinal deformity vary according to the pathology. In cerebral palsy, muscular dystrophies, and spinal muscular atrophy, specific features of deformities are poorly written, especially in the sagittal profile. Methods: The evaluation criteria were age, gender of the patients, the volume of blood loss, duration of hospitalization, measurement of the deformity curve, thoracic and lumbar kyphosis (Cobb angle), pelvic obliquity concerning the horizontal line, the percentage of curve correction. Cobb angle was measured preoperatively before hospital discharge (up to 21 days postoperatively) and one year after surgery. Results: The cohort of 71 patients with spinal deformities due to neuromuscular diseases included four groups: muscular dystrophy (MD), spinal muscular atrophy (SMA), Duchenne muscular dystrophy (DMD), and cerebral palsy (CP). The most characteristic deformity in the frontal plane was C-shaped thoracolumbar scoliosis with rotation of the pelvis; rotation of the vertebrae increased according to the magnitude of scoliosis. Lumbar hyperlordosis was common in patients with PD, whereas decreased thoracic kyphosis or even thoracic lordosis occurs more frequently in patients with DMD. Moderate correction of scoliosis was observed in all groups. There was no significant improvement in functional status, according to the FIM. Conclusion: The findings showed that rigid hyperlordosis is the main problem of spinal deformities in neuromuscular patients. Scoliosis and pelvic obliquity can be well corrected in NMS by pedicle screw construction with standard maneuvers and pelvic screw fixation. Level of Evidence IV; Lesser quality prospective study.

RESUMO Objetivo: Revelar padrões frontais e sagitais de deformidade espinhal depende da nosologia neuromuscular para cirurgia e planejamento de resultados. As características da deformidade espinhal variam de acordo com a patologia. Na paralisia cerebral, nas distrofias musculares e na atrofia muscular espinhal, as características específicas das deformidades estão mal escritas, especialmente no perfil sagital. Métodos: Os critérios de avaliação foram: idade, sexo dos pacientes, volume de perda de sangue, duração da internação hospitalar, medida da curva de deformidade, cifose torácica e lombar (ângulo Cobb), obliquidade pélvica em relação à linha horizontal, a porcentagem da correção da curva. O ângulo Cobb foi medido no pré-operatório antes da alta hospitalar (até 21 dias de pós-operatório) e um ano após a cirurgia. Resultados: A coorte de 71 pacientes com deformidades espinhais devido a doenças neuromusculares incluiu quatro grupos: distrofia muscular (DM), atrofia muscular espinhal (AME), distrofia muscular de Duchenne (DMD) e paralisia cerebral (PC). A deformidade mais característica no plano frontal era a escoliose toracolombar em forma de C com a rotação da pélvis; a rotação das vértebras aumentou de acordo com a magnitude da escoliose. A hiperlordose lombar era comum em pacientes com DP, enquanto que a diminuição da cifose torácica ou mesmo a lordose torácica ocorre com maior frequência em pacientes com DMD. A correção moderada da escoliose foi observada em todos os grupos. Não houve melhora significativa no status funcional, de acordo com a FIM. Conclusão: Os achados mostraram que a hiperlordose rígida é o principal problema das deformidades espinhais em pacientes neuromusculares. A escoliose e a obliquidade pélvica podem ser bem corrigidas no NMS através da construção de parafusos pediculares com manobras padrão e fixação de parafusos pélvicos. Nível de Evidência IV; Estudo prospectivo de menor qualidade.

RESUMEN Objetivo: La revelación de los patrones frontal y sagital de la deformidad de la columna vertebral depende de la nosología neuromuscular para la planificación de la cirugía y los resultados. Las características de la deformación de la columna vertebral varían según la patología. En la parálisis cerebral, las distrofias musculares y la atrofia muscular espinal, las características específicas de las deformidades están mal escritas, especialmente en el perfil sagital. Métodos: Los criterios de evaluación fueron la edad, el sexo de los pacientes, el volumen de pérdida de sangre, la duración de la hospitalización, la medición de la curva de deformación, la cifosis torácica y lumbar (ángulo de Cobb), la oblicuidad pélvica en relación con la línea horizontal, el porcentaje de corrección de la curva. El ángulo de Cobb se midió antes del alta hospitalaria (hasta 21 días después de la operación) y un año después de la misma. Resultados: La cohorte de 71 pacientes con deformidades espinales debidas a enfermedades neuromusculares incluía cuatro grupos: distrofia muscular (DM), atrofia muscular espinal (AME), distrofia muscular de Duchenne (DMD) y parálisis cerebral (PC). La deformación más característica en el plano frontal era la escoliosis toracolumbar en forma de C con rotación de la pelvis; la rotación de las vértebras aumentaba según la magnitud de la escoliosis. La hiperlordosis lumbar fue común en los pacientes con EP, mientras que la disminución de la cifosis torácica o incluso la lordosis torácica ocurre más frecuentemente en los pacientes con DMD. Se observó una corrección moderada de la escoliosis en todos los grupos. No hubo una mejora significativa del estado funcional según el FIM. Conclusión: Los resultados mostraron que la hiperlordosis rígida es el principal problema de las deformidades de la columna vertebral en los pacientes neuromusculares. La escoliosis y la oblicuidad pélvica pueden corregirse bien en el SMN mediante la construcción de tornillos pediculares con maniobras estándar y la fijación de tornillos pélvicos. Nivel de evidencia IV; Estudio prospectivo de menor calidad.

Final fusion in a complex of surgical treatment for early onset scoliosis.

INTRODUCTION: Surgical treatment of early onset scoliosis (EOS) is one of the most challenging problems of spine surgery and includes staged distraction and final fusion at the end of skeletal maturity that remains debatable. AIM: The objective of the review is to evaluate the efficacy of final fusion following staged distraction with VEPTR instrumentation in patients with EOS. MATERIALS AND METHODS: Outcomes of multi-staged operative treatment of 37 patients with EOS of different etiology were reviewed. Medical records and radiographs of the patients were retrospectively analyzed. Standing postero-anterior and lateral spine radiographs were used for the spinal radiologic assessment before and after each stage of distraction-based treatment, before and after final fusion and at the last follow-up. RESULTS: The mean age of patients at baseline was 5.2 years and the mean age at final fusion was 13.9 years. All patients demonstrated decrease in the angle of primary (from 81.5° to 51.6°) and secondary (from 59.3° to 37.8°) curves, increase of the height and normalized body balance. The mean height increased from 104.8 cm to 141.0 cm, and the mean weight increased from 15 kg to 35 kg throughout the treatment period. The height of the thoracic and lumbar vertebra (Th1-S1) increased from 245 mm to 340 mm, and that of the thoracic vertebra - from 136 mm to 193 mm. There was a mean of 2.3 complications per patient during distraction performed in a staged manner, and they were arrested during elective procedures. There were 7 (19%) complications after final fusion that required 6 (16%) unplanned revisions. Radiologic evidence of spontaneous autofusion was seen in the lumbar spine of the patients with the inferior anchor at the lumbar vertebra. CONCLUSIONS: Multi-staged pediatric surgeries performed in the first decade of life facilitate radical changes in the natural history of progressive scoliosis and ensure satisfactory functional and cosmetic results despite multiple difficulties and complications. The VEPTR instrumentation used for the thoracic curve is unlikely to result in the spinal fusion of the major arch and this is the cause for the use of third-generation instrumented final spinal fusion in the patients.

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.