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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.
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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.
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Anomalías Múltiples , Hernia Diafragmática , Lipoma , Meningocele , Defectos del Tubo Neural , Disrafia Espinal , Niño , Humanos , Columna Vertebral/anomalías , Médula Espinal/patología , Meningocele/patología , Defectos del Tubo Neural/patología , Imagen por Resonancia MagnéticaRESUMEN
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.
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Humanos , Masculino , Femenino , Escoliosis , Médula Espinal , Edad de Inicio , CifosisRESUMEN
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.
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Enfermedades del Desarrollo Óseo , Luxaciones Articulares , Síndrome de Klippel-Feil , Deformidades Congénitas de las Extremidades , Osteocondrodisplasias , Masculino , Humanos , Niño , Luxaciones Articulares/complicaciones , Luxaciones Articulares/cirugíaRESUMEN
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.
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Humanos , Escoliosis , Distrofias Musculares , Enfermedades de la Columna Vertebral , Parálisis CerebralRESUMEN
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.
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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.