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.

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.