Thoracic Morphology and Bronchial Narrowing Are Related to Pulmonary Function in Adolescent Idiopathic Scoliosis.

BACKGROUND: In adolescent idiopathic scoliosis (AIS), lung function impairment is not necessarily related to the coronal spinal deformity. Recently, right-sided bronchial narrowing has been reported in thoracic AIS. The aim of this study was to describe the relation of chest and spinal deformity parameters, bronchial narrowing, and lung volumes with pulmonary function in preoperative AIS. METHODS: Spinal radiographs, low-dose computed tomographic (CT) scans of the spine including the chest, and pulmonary function tests were retrospectively collected for 85 preoperative patients with thoracic AIS in 2 centers and were compared with 14 matched controls. Three-dimensional lung and airway reconstructions were acquired. Correlation analysis was performed in which radiographic spinal parameters, CT-based thoracic deformity parameters (rib-hump index [RHi], spinal penetration index, endothoracic hump ratio, hemithoracic-width ratio), lung volume asymmetry, and bronchial cross-sectional area were compared with percent-of-predicted spirometry results. RESULTS: Forty-one patients (48%) had a percent-of-predicted forced expiratory volume in 1 second (FEV1%) or percent-of-predicted forced vital capacity (FVC%) of <65%, and 17 patients (20%) had obstructive lung disease. All thoracic deformity parameters correlated significantly with FEV1% and FVC%; RHi was found to be the best correlate (rs = -0.52 for FEV1% and -0.54 for FVC%). Patients with AIS with impaired pulmonary function had hypokyphosis, a larger rib hump, increased spinal and thoracic rotation, a narrower right hemithorax, and increased intrusion of the spine into the chest. Spinal intrusion correlated with right-sided bronchial narrowing, relative right lung volume loss, and decreased FEV1% and FVC%. Multivariate regression including spinal and thoracic deformity parameters, lung volume asymmetry, and airway parameters could explain 57% of the variance in FEV1% and 54% of the variance in FVC%. CONCLUSIONS: Chest intrusion by the endothoracic hump is related to right-sided bronchial narrowing and lung function loss in preoperative AIS. The findings support the theory that ventilatory dysfunction in thoracic AIS is not only restrictive but frequently has an obstructive component, especially in patients with hypokyphosis. RHi is the most predictive chest parameter for lung function loss. LEVEL OF EVIDENCE: Prognostic Level III. See Instructions for Authors for a complete description of levels of evidence.

Long-term follow-up of untreated Scheuermann's kyphosis.

STUDY DESIGN: Long-term cross-sectional study. OBJECTIVES: To investigate the long-term effects of untreated Scheuermann's kyphosis on quality of life, and its relationship to radiographic parameters of spinal deformity. Previous studies reported reduced self-image, increased pain and impaired physical status. Little is known of the long-term impact of sagittal plane deformity in untreated SK. METHODS: One hundred and thirteen consecutive untreated patients with SK were identified from a national service database prior to 2000, when surgery was not offered at this unit. 81 of these patients were available for evaluation; 66 (81%) consented to questionnaire and clinical evaluation, and 47 (58%) consented to additional radiological evaluation. Health-related quality of life (HRQoL) was compared to normative population values. Mean age was 45.1 years (31-65), and mean follow-up was 27 years (16-36). 57 patients had thoracic kyphosis and 9 had thoracolumbar deformity. RESULTS: SRS-22 and SF-36 scores were lower, and ODI was greater in patients with untreated SK compared to normative population values. Kyphosis progressed from mean 66° at skeletal maturity to 78° (p < 0.001) after mean follow-up of 27 years. Long-term progression of untreated SK was 0.45°/year (n = 47). Multilinear regression showed good correlation between increasing SVA and worse ODI scores (r = 0.59; p = 0.001). Increasing SVA also correlated with worse function, pain and mental health scores reported by SRS-22, and with worse physical function and bodily pain scores reported by SF-36. Increasing CL correlated with worse SF-36 physical function scores. Increasing cSVA and increasing TK correlated with worse SRS-22 self-image scores. CONCLUSION: SRS-22 and SF-36 scores were lower, and ODI was greater in patients with untreated SK compared to normative data. Long-term progression of untreated SK was 0.45°/year (n = 47). Increasing SVA correlated with worse SF-36 physical function, SRS-22 function, SRS-22 pain and higher ODI scores. Total kyphosis (TK) and cSVA were independent predictors of low SRS self-image. LEVEL OF EVIDENCE: III.

Health-related quality of life and sagittal balance at two to 25 years after posterior transfixation for high-grade dysplastic spondylolisthesis.

AIMS: High-grade dysplastic spondylolisthesis is a disabling disorder for which many different operative techniques have been described. The aim of this study is to evaluate Scoliosis Research Society 22-item (SRS-22r) scores, global balance, and regional spino-pelvic alignment from two to 25 years after surgery for high-grade dysplastic spondylolisthesis using an all-posterior partial reduction, transfixation technique. METHODS: SRS-22r and full-spine lateral radiographs were collected for the 28 young patients (age 13.4 years (SD 2.6) who underwent surgery for high-grade dysplastic spondylolisthesis in our centre (Scottish National Spinal Deformity Service) between 1995 and 2018. The mean follow-up was nine years (2 to 25), and one patient was lost to follow-up. The standard surgical technique was an all-posterior, partial reduction, and S1 to L5 transfixation screw technique without direct decompression. Parameters for segmental (slip percentage, Dubousset's lumbosacral angle) and regional alignment (pelvic tilt, sacral slope, L5 incidence, lumbar lordosis, and thoracic kyphosis) and global balance (T1 spino-pelvic inclination) were measured. SRS-22r scores were compared between patients with a balanced and unbalanced pelvis at final follow-up. RESULTS: SRS-22r domain and total scores improved significantly from preoperative to final follow-up, except for the mental health domain that remained the same. Slip percentage improved from 75% (SD 15) to 48% (SD 19) and lumbosacral angle from 70° (SD 11) to 101° (SD 11). Preoperatively, 35% had global imbalance, and at follow-up all were balanced. Preoperatively, 63% had an unbalanced pelvis, and at final follow-up this was 32%. SRS-22r scores were not different in patients with a balanced or unbalanced pelvis. However, postoperative pelvic imbalance as measured by L5 incidence was associated with lower SRS-22r self-image and total scores (p = 0.029). CONCLUSION: In young patients with HGDS, partial reduction and transfixation improves local lumbosacral alignment, restores pelvic, and global balance and provides satisfactory long-term clinical outcomes. Higher SRS-22r self-image and total scores were observed in the patients that had a balanced pelvis (L5I < 60°) at two to 25 years follow-up. Cite this article: Bone Jt Open 2021;2(3):163-173.

Combination of Congenital and Deep Penetrating Nevus by Acquisition of ß-Catenin Activation.

Deep penetrating nevus (DPN) is an intradermal, sometimes compound benign melanocytic lesion, which involves the reticular dermis, occasionally reaching the subcutis, which can raise concern for melanoma both clinically and histologically. Recently, it has been genetically defined by the combination of MAPK activating and ß-catenin activating mutations. We sought to investigate genetic alterations in 2 cases of combined nevi of congenital melanocytic and DPN. Case 1 was a 16-year-old woman with a pigmented lesion on the trunk since birth, which was completely excised. Histopathological examination revealed a combined congenital nevus with a DPN. Comparative genomic hybridization showed no major genetic alterations, except for gain of 6q11.1 and point mutation of B-RAF V600E. Case 2 was a 62-year-old woman with a congenital pigmented lesion on the back. The lesion was diagnosed as a combined nevus of congenital and DPN. Comparative genomic hybridization showed no genetic alterations, and the NRAS Q61K was detected in both components. DPN is in most cases part of a combined nevus. Our cases showed strong and uniform nuclear expression of ß-catenin and cyclin D1 in the DPN component suggesting the evolution of the congenital nevus to the DPN clone by acquiring ß-catenin activating mutation.

Effect of idiopathic thoracic scoliosis on the tracheobronchial tree.

INTRODUCTION: High prevalence of obstructive lung disease has been reported in patients undergoing surgical correction of thoracic scoliosis. Airway narrowing due to spine morphology is analysed as a contributing factor. METHODS: Preoperative surgical planning CTs of 34 patients with right-sided thoracic scoliosis (age: 17.6±9.0) were retrospectively analysed and compared with 15 non-scoliotic controls (age: 16.3±5.1). Three-dimensional models of spine and airway lumen were reconstructed. Based on thoracic sagittal profile, patients were divided into hypokyphosis (HypoS: <10°), normal kyphosis (NormS: ≥10° and <40°) and hyperkyphosis (HyperS: ≥40°) groups. Lumen area of bronchi, bifurcation angles and minimum spine-airway distance were measured. Pulmonary function tests were correlated to scoliosis, kyphosis and lumen area. RESULTS: Loss of kyphosis led to proximity between bronchus intermedius (BI) and spine. HypoS (NormS) had lumen area reductions in the right main bronchus of 29% (19%), BI of 45% (23%), right middle lobar bronchus of 46% (32%) and right lower lobe bronchus (RLL7) of 66% (37%), respectively (P<0.05). The lower right superior segmental bronchus was reduced across all scoliotic groups (P<0.05). Airways were displaced caudal by 0.65±0.45 vertebra in patients with scoliosis. Loss of kyphosis correlated negatively with forced expiratory volume in 1 s/forced vital capacity (FEV1/FVC), FVC/(FVC predicted) and FEV1/(FEV1 predicted) (P<0.01). Lumen area of trachea, right upper lobar bronchus, BI and RLL7 correlated negatively with FEV1/FVC. BI and RLL7 narrowing were strong predictors of FVC and FEV1 loss (P<0.001). CONCLUSIONS: Right-sided main stem airways are narrowed in HypoS and NormS. Loss of kyphosis leads to narrowing of BI and its trifurcation. FEV1/FVC correlated negatively with airway narrowing, implying an obstructive element to lung function impairment in patients with scoliosis and hypokyphosis.

Transcranial magnetic stimulation in the semi-quantitative, pre-operative assessment of patients undergoing spinal deformity surgery.

PURPOSE: To compare measurements of motor evoked potential latency stimulated either magnetically (mMEP) or electrically (eMEP) and central motor conduction time (CMCT) made pre-operatively in conscious patients using transcranial and intra-operatively using electrical cortical stimulation before and after successful instrumentation for the treatment of adolescent idiopathic scoliosis. METHODS: A group initially of 51 patients with adolescent idiopathic scoliosis aged 12-19 years was evaluated pre-operatively in the outpatients' department with transcranial magnetic stimulation. The neurophysiological data were then compared statistically with intra-operative responses elicited by transcranial electrical stimulation both before and after successful surgical intervention. MEPs were measured as the cortically evoked compound action potentials of Abductor hallucis. Minimum F-waves were measured using conventional nerve conduction methods and the lower motor neuron conduction time was calculated and this was subtracted from MEP latency to give CMCT. RESULTS: Pre-operative testing was well tolerated in our paediatric/adolescent patients. No neurological injury occurred in any patient in this series. There was no significant difference in the values of mMEP and eMEP latencies seen pre-operatively in conscious patients and intra-operatively in patients under anaesthetic. The calculated quantities mCMCT and eCMCT showed the same statistical correlations as the quantities mMEP and eMEP latency. CONCLUSIONS: The congruency of mMEP and eMEP and of mCMCT and eCMCT suggests that these measurements may be used comparatively and semi-quantitatively for the comparison of pre-, intra-, and post-operative spinal cord function in spinal deformity surgery.

Combined spinal arthrodesis with instrumentation for the management of progressive thoracolumbar kyphosis in children with mucopolysaccharidosis.

PURPOSE: To evaluate the outcome and complications of a novel technique for the treatment of progressive thoracolumbar kyphosis in children with mucopolysaccharidosis (MPS). METHODS: The medical records and spinal imaging of four consecutive paediatric patients who underwent a single stage anteroposterior spinal fusion with segmental pedicle screw instrumentation were reviewed. RESULTS: Patients underwent spinal deformity correction at the mean age of 3 years (2.4-3.7) with mean clinical follow-up of 3.2 years (2.1-4.5) and mean postoperative radiographic follow-up was 2.4 years (0.8-3). Preoperative kyphosis was corrected from a mean angle of 65º (63º-70º) to 6.5º (-12º-13º). Vertebral subluxation at the apex of the deformity was corrected from an average 64% (56-83%) to 12% (0-24%). Spinal cord monitoring with somatosensory evoked potentials (SSEP) was successfully obtained and stable throughout surgery. No instrumentation failure, loss of correction or junctional problems occurred at final follow-up. CONCLUSIONS: Anterior and posterior spinal arthrodesis with segmental pedicle screw instrumentation is a safety and reliable technique for the treatment of severe thoracolumbar kyphosis in children with MPS. This technique achieves excellent correction of the deformity with adequate decompression of the spinal canal. The fusion is limited to the thoracolumbar junction and interferes minimally with the longitudinal growth of the thorax. No neurological complications or intraoperative spinal cord monitoring events occurred. No loss of correction or junctional kyphosis was observed.

Rare true-positive isolated SSEP loss with preservation of MEPs response during scoliosis correction.

STUDY DESIGN: Case report. OBJECTIVE: To report a case of a true-positive isolated somatosensory evoked potential (SSEP) loss with preservation of motor evoked potential (MEP) response during scoliosis correction. SUMMARY OF BACKGROUND DATA: Combined intraoperative monitoring uses SSEPs and MEPs to decrease the probability of observing false-negative events. In combination, SSEPs and MEPs have become a standard of care for spinal deformity surgery. However, literature review reveals several cases of false-negative response with combined SSEPs and MEPs, raising the contention that intraoperative monitoring does not reliably identify all isolated selective spinal cord dysfunction. METHODS: A 15-year-old female patient with a 65° right thoracic adolescent idiopathic scoliosis underwent correction and posterior spinal fusion with segmental pedicle screw instrumentation. After capture and derotation of the left concave rod, left-sided irreversible SSEP loss occurred whereas MEPs remained unchanged. After excluding systemic factors, anesthetic causes, or technical fault, deformity correction was released and instrumentation removed. No cortical breach was reported during pedicle screw removal. RESULTS: Postoperatively, no clinical sensory or motor deficit was present; computed tomography demonstrated a burst left pedicle at T10 with the medial pedicle wall fragment in direct contact with the dorsal spinal cord. Magnetic resonance imaging excluded cord edema or other evidence of injury. Three days after surgery, intraoperative monitoring showed delayed latencies and amplitudes of the left SSEP. An uneventful reinsertion of instrumentation and correction excluding the left T10 pedicle screw was performed 7 days after the initial surgery. CONCLUSION: This case report provides evidence of selective posterior spinal cord dysfunction with sparing of the anterior columns immediately after a correction maneuver and emphasizes the importance of simultaneous SSEP and MEP monitoring. To the authors' knowledge, there is no previous report of a true-positive isolated SSEP loss with preservation of MEP response during scoliosis correction. LEVEL OF EVIDENCE: N/A.

Association between -174 interleukin-6 gene polymorphism and biological response to rituximab in several systemic autoimmune diseases.

Rituximab has become a pivotal treatment for systemic autoimmune diseases. The aim of this study was to determine whether the genetic variant -174 IL-6 contributes to differences in the response to rituximab in patients with systemic autoimmune diseases, including systemic lupus erythematosus (SLE), inflammatory myopathies, anti-neutrophil cytoplasmic antibody-mediated vasculitis, systemic sclerosis, Sjöegren's syndrome, rheumatoid arthritis, and autoimmune hemolytic anemia. DNA samples from 144 Spanish patients with different systemic autoimmune diseases receiving rituximab were genotyped for -174 IL-6 (rs1800795) gene polymorphism using the TaqMan(®) allelic discrimination technology. Six months after the first infusion with rituximab, we evaluated the response to the drug: 60.4% of the patients showed a complete response, partial 27.8%, and 11.8% did not respond to the treatment. The CC genotype frequency was significantly increased in nonresponders with respect to responders (23.5% vs. 7.1%, respectively; p=0.049; odds ratio (OR)=4.03, 95% confidence intervals (CI) 0.78-16.97). According to the genotype distribution, rituximab was effective in 69.2% of the CC carriers, 91.9% of the CG carriers, and 88.4% of the GG carriers. A similar trend was observed when SLE patients were analyzed separately (27.3% carried CC homozygosis in nonresponders and 6.9% in responders; p=0.066; OR=5.10, 95% CI 0.65-31.73). Rituximab was effective in 62.5% of the CC carriers, 88.9% of the GC carriers, and 90% of the GG carriers. These results suggest that -174 IL-6 (rs1800795) gene polymorphism plays a role in the response to rituximab in systemic autoimmune diseases. Validation of these findings in independent cohorts is warranted.

The surgical treatment of congenital kyphosis.

STUDY DESIGN: Retrospective study with clinical and radiologic evaluation of 15 patients with congenital kyphosis or kyphoscoliosis who underwent anterior instrumented spinal fusion for posterolateral or posterior hemivertebra (HV). The management of congenital kyphosis has been described in the literature using a variety of techniques. The presentation of patients at diagnosis is discussed. The question of when to begin treatment is reviewed. The pitfalls in the management and how to avoid these are discussed. The different published techniques are reviewed. We present our own techniques and our results of treatment of congenital kyphosis in very young children. OBJECTIVE: To evaluate the safety and efficacy of early surgical anterior instrumented fusion with partial preservation of the HV in the treatment of progressive congenital kyphosis in children below the age of 3. We discuss the management of patients presenting with neurologic compromise. We aim to systematically review the literature and to present our own experience in the management of these deformities, so that the issues common to treating physicians may be explored. SUMMARY OF BACKGROUND DATA: A variety of treatments have been described in the literature for the treatment of congenital kyphosis due to HV. We report the results of our technique. METHODS: Between 1997 and 2005 we have treated 15 consecutive patients with progressive congenital kyphosis with anterior instrumented fusion and strut grafting. Thirteen patients had a single posterolateral HV and 2 patients had a single posterior HV. Of the 15 patients in the study, 5 were girls and 10 boys. Mean age at surgery was 22 months (range, 8-33). Mean follow-up period was 6.8 years. Thirteen HV were located in the thoracolumbar junction (T10-L2) and 2 in the thoracic spine. RESULTS: The average operating time of procedure was 150 minutes (range, 130-210 minutes). The average blood loss was 180 mL (range, 100-330 mL), equivalent to a mean external blood volume loss of 15% (range, 11%-24%).Preoperative segmental Cobb angle averaging 34 degrees at last follow-up. Compensatory coronal cranial and caudal curves were corrected by 50%. The angle of segmental kyphosis averaged 39 degrees (range, 20 degrees-80 degrees) before surgery and 21 degrees (range, 11 degrees-40 degrees) at last follow-up. This represents a 43% of improvement of the segmental kyphosis, and a 64% of improvement of the segmental scoliosis at last follow-up. One case with initial kyphosis of 80 degrees continued to progress and required revision anterior and posterior surgery. There were no neurologic complications.

Lordoscoliosis and large intrathoracic airway obstruction.

STUDY DESIGN: Case series. OBJECTIVE: We report the treatment of 2 children with right main bronchus obstruction complicating thoracic lordoscoliosis. SUMMARY OF BACKGROUND DATA: The preoperative investigation and treatment of large airway obstruction caused by lordoscoliosis has not been reported in the literature. METHODS: Obstruction of the right main bronchus was confirmed before surgery by ventilation-perfusion scans, bronchogram, and computed tomography scan. Deformity correction was achieved using a submuscular growth rod construct in one child, and posterior spinal fusion in the other. Clinical examination and repeat ventilation-perfusion scans were performed 8 weeks after surgery. RESULTS: In both children, ventilation to the right "convex" lung was reestablished after surgery. Lung function improved in both patients after surgery. CONCLUSION: This is the first report of large airway obstruction associated with thoracic lordoscoliosis in which ventilation was reestablished after spinal deformity correction. Early deformity correction is indicated in such cases because of the risk of irreversible compromise to lung ventilation and perfusion.

Alteraciones de la movilidad ocular en paralisis cerebral / Alterations of ocular motility in cerebral palsy

Introducción y objetivo. El estrabismo es una de las alteraciones oftalmológicas más frecuentes en los pacientes con parálisis cerebral (PC). El objetivo de este estudio fue determinar el tipo, frecuencia y características del estrabismo en pacientes con diangnóstico confirmado de PC. Pacientes y método. Se realizó un estudio prospectivo, longitudinal y descriptivo en pacientes con diagnóstico confirmado de PC, se estudiaron el tipo, frecuencia y características del estrabismo y se correlacionó con el tipo y severidad de la parálisis cerebral. Resultados. Se estudiaron 140 pacientes, el promedio de edad fue de 5.7 años con un recorrido de 1 a 15 años, la alteración topográfica más frecuente fue la cuadriplejia en un 75 por ciento de los casos, la parálisis fue espástica en el 67 por ciento. Se encontró algún tipo de alteración de la movilidad ocular en el 67 por ciento. La alteración predominante fue la exotropia en 58 (62 por ciento), siguiendo en orden de frecuencia las endotropias en 19 (20 por ciento) y cuadros paralíticos en 11 (12 por ciento). Se observó variabilidad en el ángulo de la desviación en el 48 porciento de los casos. No se encontró correlación entre el tipo y la severidad de la PC y las alteraciones de la movilidad ocular. Conclusión. La presencia de exotropia con variabilidad en la magnitud del ángulo de la desviación en los pacientes con PC es la alteración predominante de la movilidad ocular

Comportamiento del estrabismo en pacientes con parálisis cerebral infantil / Behaviour of strabismus in patients with infantile cerebral palsy

Se estudiaron 110 pacientes con diagnóstico confirmado de parálisis cerebral infantil (PCI), en 73 casos (66.3 por ciento) de ellos se encontró alguna alteración en la movilidad ocular. Hubo un claro predominio de la exotropía sobre endotropía, en las exotropías fue más evidente la presencia de variación en la magnitud del ángulo de desviación. Las alteraciones verticales más frecuentes fueron. DVD e hiperfunción de los oblicuos inferiores. Se encontraron alteraciones paralíticas en 13.6 por ciento de los casos, predominando la parálisis de la mirada conjugada. Se exploró a los pacientes un año después, tiempo durante el cual continuaron con rehabilitación integral. No se observó modificación alguna del cuadro, especialmente en la variabilidad de la desviación en 69 de los 73 casos. Se concluye que el estrabismo con variabilidad en el ángulo de la desviación es la alteración oftalmológica característica de la PCI. Quizá sea necesario un largo tiempo de rehabilitación antes de observar mejoría en la variación de la magnitud en el ángulo de la desviación

Método de visión preferencial en niños con parálisis cerebral infantil / Preferential looking in children with cerebral palsy

Se realizó un estudio descriptivo, transversal y prospectivo en 40 pacientes con diagnóstico confirmado de parálisis cerebral infantil (PCI). Se determinó la agudeza visual mediante los tarjetones de Teller a 84 cm. Los pacientes se clasificaron de acuerdo a su ametropía en equivalente esférico. Se correlacionó la agudeza visual obtenida con el defecto de refracción y el patrón de fijación motora. En 37 de los 40 pacientes (72.5 por ciento) fue posible determinar con certeza la agudeza visual, la que se correlacionó en todos los casos con la agudeza visual esperada de acuerdo a la ametropía y el patrón de fijación. Se concluye que el método de visión preferencial es una prueba útil para determinar la agudeza visual en niños con PCI

Protocolo de vacunación antimalárica en fase III municipio Las Majadas Estado Bolívar Venezuela / Antimalaric vaccination protocol III municipio las Majadas Bolivar State Venezuela

El presente proyecto se realizó con el proposito de obtener información sobre la eficacia, seguridad,tolerancia y aceptabilidad de la vacuna sintética SPf66n contra plasmodium falciparum, en una área holoendémica, las majadas del estado Bolívar. La muestra estará representada por 330 individuos, femeninos y masculinos divididos en 2 grupos; uno vacunado y el otro no vacunado, mayores de 12 años y que no presenten criterios de exclusión. La vacuna se administraráen 3 dosis de 2 mgs de la proteina SPf66n por cada inmunización de 0,5 cc subcutaneo. El estudio tendrá un período de observación de un año posrerior ala tercera dosis. Los análisis y resultados se presentarán en un informe final al terminar el estudio