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.

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.

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.

Anomaly-Related Pathologic Atlantoaxial Displacement in Pediatric Patients.

OBJECTIVE: To analyze clinical and radiologic features of pathologic atlantoaxial displacement (PAAD) in pediatric patients and to compose a treatment algorithm for anomaly-related PAAD. BACKGROUND: Criteria of different types of PAAD and treatment algorithms have been widely reported in the literature but are difficult to apply to patients with odontoid abnormalities, C2-C3 block, spina bifida C1, and children. METHODS: We evaluated results of treatment of 29 pediatric patients with PAAD caused by congenital anomalies of the craniovertebral junction (CVJ), treated in Ilizarov Center in 2009-2017, including 20 patients with atlantoaxial displacement (AAD) and 9 patients with atlantoaxial rotatory fixation. RESULTS: There were 14 males (48.3%) and 15 females (51.7%). We singled out 3 groups of patients: nonsyndromic (6 patients, 20.7%), Klippel-Feil syndrome (13 patients, 44.8%), and syndromic (10 patients, 34.5%). Odontoid abnormalities and C1 dysplasia were widely represented in the syndromic group. Local symptoms predominated in the nonsyndromic and KFS groups. In the syndromic group, all patients had AAD and myelopathy. A pronounced decrease of space available for chord C1 and increase of anterior atlantodental interval were noted compared with other groups. CONCLUSIONS: We present a unified treatment algorithm of pediatric anomaly-related PAAD. Syndromic AAD are often accompanied by anterior and central dislocation and myelopathy and atlantooccipital dissociation. These patients require early aggressive surgical treatment. Nonsyndromic and Klippel-Feil syndrome AAD, atlantoaxial subluxation, and atlantoaxial fixation often manifest by local symptoms and need to eliminate CVJ instability. Existing classifications of symptomatic atlantoaxial displacement are not always suitable for patients with CVJ abnormalities.

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.

Surgical Treatment of Thoracolumbar Segmental Spinal Dysgenesis: Optimal Type of Fusion.

OBJECTIVES: We sought to evaluate long-term results of surgical treatment of thoracolumbar segmental spinal dysgenesis (SSD). METHODS: We analyzed 8 patients with thoracolumbar SSD treated in our institution. Each case was evaluated for specific clinical and radiologic criteria and types and outcomes of treatment. RESULT: The average age of primary surgery was 3.4 years (median 3.4 years, range 1.7-7 years). The average correction of kyphosis was 49.3° (mean 45°, from 25°-75°) and scoliosis 10.6° (mean 10°, from 0°-25°). Average follow-up time was 3.2 years (mean 2.6 years, from 1.8-5.6 years). Neurologic improvement was also achieved in all patients. The Japanese Orthopaedic Association scale score (Benzel's modification) was increased by 2.5 points on average (mean 2.5 points, from 2-5 points). One patient had complications: pseudarthrosis and rod fracture followed by refusion. CONCLUSIONS: Our treatment strategy provides favorable deformity correction and neurologic improvement. It is limited by immature vertebral structures in newborns and infants, who should be carefully monitored from birth with braces until they reach the age when a fixing tool can be used.

Easy method to simplify "freehand" subaxial cervical pedicle screw insertion.

STUDY DESIGN: Technical note. OBJECTIVES: The objective of this study is to check out safety and rationality of standardized and fast tricks to select trajectory of subaxial cervical pedicle screw (SCPS) insertion, based on simple angles to bony landmarks. MATERIALS AND METHODS: Stage 1 - Computed tomography (CT)-morphometric analysis of C3-C7 vertebrae of ten patients with cervical degenerative diseases. Stage 2 - SCPS insertion in 6 cadavers, according to the developed technique (59 pedicle screws). Stage 3 - SCPS insertion in 6 patients, according to the developed technique (32 pedicle screws). RESULTS: CT-morphometric analysis showed that the average length of C3-C7 pedicle channels was 32 mm, the average angle between a pedicle axis and an axis of contralateral lamina - 180°, the average angle between a pedicle axis and plane of a posterior surface of a lateral mass amounted to 90° and the coordinates of an optimal entry point - 2 mm from a lateral edge and 2 mm from an upper edge of the lateral mass posterior surface. During the cadaveric study, 39 screws had a satisfactory position (66.1%), 7 screws permissible (11.9%), and 13 screws unacceptable (22%). During the clinical study, 26 screws (81.25%) had satisfactory position, 4 (12.5%) had permissible position, and 2 (6.25%) unacceptable position. CONCLUSION: Developed and clinically approved a method for simplicity SCPS insertion is relatively safe and cheap. No doubt, it requires further investigation, but the results of primary analysis allow us to recommend it to wide practical application.