Research Progress of Congenital Scoliosis / 罕见病研究

Congenital scoliosis is an early-onset spinal deformity cauded by somitogenesis abnormalities. This disease is characterized by rapidly progressing in deformities, often accompanied by concurrent organ malformations. Current treatments include conservative treatment and surgical treatment. Various imaging technology-X-ray, CT, and MRI are used in the diagnosis of the disease. The majority of congential scoliosis deformities has a progressive nature so that close observation is vital and effective at the beginning. In cases of moderate congential scoliosis, non-invasive interventions, such as bracing and traction are effective. In surgery, factors such as age, growth potential and the specific location and type of deformity of individual patients are the basis for using such surgical procedures as epiphysiodesis, hemivertebra resection, growth-friendly techniques, and hybrid techniques in treatment. This paper makes a summery of the etiology pathogenesis, diagnosis and treatment of the congential scoliosis.

Diagnóstico prenatal ultrasonográfico de hemivértebra / Prenatal Ultrasonographic Diagnosis of Hemivertebrae

Introducción: La hemivértebra es un raro defecto congénito de la columna vertebral fetal en la que solo se desarrolla el cuerpo vertebral de un lado, lo cual provoca su deformidad. Objetivo: Presentar un caso con diagnóstico ecográfico tridimensional prenatal de hemivértebra, como único defecto. Método: Se realizó evaluación ecográfica prenatal y examen anátomo patológico y radiológico posmortem al feto con escoliosis congénita provocado por hemivértebra. Se revisó la literatura sobre este defecto congénito, su diagnóstico prenatal y otros aspectos genéticos que deben tenerse en cuenta para el asesoramiento a la familia. Presentación de caso: Gestante de 28 años remitida a la consulta provincial de Genética Médica en la ciudad de Camagüey, Cuba, el 25 de septiembre del 2018, por sospecha ultrasonográfica de hemivértebra fetal con 20 semanas de gestación. Se confirma diagnóstico a esta instancia, mediante ultrasonografía tridimensional. Con el consentimiento familiar informado se realiza interrupción de la gestación y se comprueba el diagnóstico prenatal realizado por estudios radiológicos y anátomo patológico de la región dorso lumbar. Conclusiones: Se concluye como un defecto congénito múltiple, aislado, de posible etiología multifactorial. Se destacó el valor de la ecografía tridimensional, vista sagital coronal, como método no invasivo más empleado para el diagnóstico prenatal(AU)

Introduction: The hemivertebrae is a rare congenital defect of the fetal spine in which only the vertebral body develops on one side, resulting in deformity. Objective: To present a case with three-dimensional prenatal ultrasound diagnosis of hemivertebrae, as the only defect. Method: Prenatal ultrasound evaluation, postmortem anatomopathological and radiological examination were performed in a fetus with congenital scoliosis caused by hemivertebrae. The literature on this congenital defect, the prenatal diagnosis and other genetic aspects that should be taken into account for family counseling was reviewed. Case report: A 28-year-old pregnant woman referred to the provincial office of Medical Genetics in Camagüey, Cuba, on September 25, 2018, due to ultrasonographic suspicion of fetal hemivertebrae. She was 20 weeks of gestation. Diagnosis is confirmed by three-dimensional ultrasonography. After the informed family consent, the pregnancy was interrupted. The prenatal diagnosis was verified by radiological and pathological studies of the lumbar back region. Conclusions: It is concluded as a multiple congenital defect, isolated, of possible multifactorial etiology. The value of three-dimensional ultrasound, coronal sagittal view, was highlighted as the most commonly used, non-invasive method for prenatal diagnosis(AU)

One-stage posterior approach surgical treatment for congenital scoliosis caused by lumbosacral spine deformity in children / 中华实用儿科临床杂志

Objective To explore the treatment method and clinical effect of congenital scoliosis caused by lumbosacral spine deformity in children.Methods From October 2000 to October 2015,a consecutive series of 21 congenital scoliosis due to lumbosacral spine deformity were treated in Department of Pediatric Orthopedics,the Third Affiliated Hospital of Zhengzhou University,including 12 male and 9 female,and the age was (63.1±47.3) months(3-144 months).The hemivertebrae,bone bridge,and the mixed type were found in 18 cases,2 cases,and 1 case,respectively.Hemivertebraes were on the left in 10 cases,on the right in 9 cases;involved L5 in 8 cases,S1 in 6 cases,multiple vertebrae malformation in 4 cases,wedged-shaped vertebrae involved L5 associated with contralateral bar in 1 case,unilateral unsegmented bar from L4 to S1 in 1 case,from L2 to L5 in 1 case.There were 4 cases combining with thoracic hemivertebrae,3 cases with ribs fusion and contralateral bar,16 cases(76%) with spinal cord malformations,3 cases(17%) with urinary system malformations,no congenital heart malformation.All the 21 patients were operated with one-stage posterior approach.Compared the scoliosis Cobb angle,trunk shift,operative time,blood loss volume,and complications among preoperation,post-operation and final follow-up.Results In this study,operative time was (278.9±83.1) min,blood loss volume was (355.3±249.0) mL,follow-up was (5.1±2.7) years.There were 18 cases operated with pedicle screw fixation,aged (72.9±44.1) months(16-144 months),operative time was (296.2±74.2) min,blood loss volume was(422.1±238.2) mL;the mean coronal Cobb angle of malformation area at preoperation,post operation and the final follow-up were(27.3±10.2)°,(11.0±5.9)°,(9.8±4.2)°,while the correction rate of post-operation and the final follow-up were 59.7%,64.1%.There were 3 cases operated by hemivenebra resection without pedicle screw fixation,aged 7.7 months(3-15 months),operative time was 168.3 min,blood loss volume was 103.3 mL.The mean coronal Cobb angle of malformation area at preoperation,post-operation and the final follow-up were 26.0°,13.6°,12.5°,while the correction rate of post-operation and the final follow-up were 47.7%,51.9%.The follow-up period was (5.1±2.7) years.The coronal Cobb angle of lumbosacral curve at preo-peration,post-operation and the final follow-up were (27.7±10.0)°,(11.3±5.4)°,(10.0±5.0)°,compared with preoperation,the differences of post-operation and the final follow-up were statistically significant (t=6.600,7.230,all P<0.05),with the correction rate of 59.2%,63.9%;the compensatory head side Cobb angle were(25.0±12.8)°,(11.9±8.2)°,(10.3±6.9)°,compared with preoperation,the differences of post-operation and the final follow-up were statistically significant (t=3.934,4.626,all P<0.05),with the correction rate of 52.4%,58.8%.Trunk shift was significantly improved.One case appeared fracture fixation rods breakage after 9 years,1 case appeared iliac screw breakage after 2 years but no complication such as infection,nerve injury.Conclusions The formation of obstacles and vertebral segmentation defects will lead to lumbosacral spine deformity,and scoliosis.Early one-stage posterior hemivertebra resection,breakage the bone bridge combined with pedicle screw fixation can obtain satisfactory clinical outcome.

Manejo quirúrgico de la deformidad vertebral en un paciente con síndrome de Escobar: revisión de la literatura / Surgical management of spinal deformity in a patient with Escobar syndrome

Resumen: Antecedentes: El síndrome de Escobar o de pterigium múltiple en su variante no letal es una entidad con tipo de herencia autosómica recesiva ligada al cromosoma X; se caracteriza por presentar múltiples pterigiones -de ahí su nombre-, principalmente localizados en cuello (95%) y axilas (55%), así como otras malformaciones de tipo ortopédico como astrágalo vertical, luxación congénita de cadera y escoliosis congénita. Objetivo: Dar a conocer una técnica quirúrgica opcional para el manejo de deformidades vertebrales severas en pacientes con este síndrome. Caso clínico: Femenina de 12 años de edad con diagnóstico de síndrome de Escobar con escoliosis severa que condiciona malformaciones de la caja torácica con compromiso pulmonar, produciendo restricción de la mecánica ventilatoria e incrementando el riesgo de infección severa de vías aéreas inferiores. Se realiza instrumentación posterior con manos libres y sistema PASS LP más osteotomías de Smith-Petersen. Conclusiones: Mejora del ángulo de Cobb de 62° a 23°, así como del balance sagital de 125 mm a 73 mm.

Abstract: Background: The non-lethal variant of the Escobar or multiple pterygium syndrome is an entity of autosomal recessive inheritance linked to the X chromosome; it is characterized by multiple pterygia (hence its name) located mainly in the neck (95%) and armpits (55%), as well as other orthopedic malformations such as a vertical talus, congenital hip dislocation, and congenital scoliosis. Objective: To present an optional surgical technique for the management of severe spinal deformities. Case report: Twelve-year-old female diagnosed with Escobar syndrome with severe scoliosis which conditions malformations of the chest with lung involvement, producing mechanical ventilatory restriction and increasing the risk of severe lower respiratory tract infection. We performed a hands-free posterior instrumentation with PASS LP system and Smith-Petersen osteotomies. Conclusions: The Cobb angle improved from 62° to 23° and the sagittal balance from 125 mm to 73 mm.

Comparative analysis on influence of congenital scoliosis and idiopathic scoliosis in pulmonary function / 吉林大学学报(医学版)

Objective:To compare the differences of lung function parameters of the patients with congenital scoliosis (CS)or idiopathic scoliosis (IS),and to clarify their influence in respiratory function.Methods:Sixty-one patients of scoliosis undergoing operation were selected.According to the cause they were divided into 25 patients with CS and 36 patients with IS.The changes of lung function parameters and the differences in the patients with scoliosis were analyzed. Results:The ages,body weights, Cobb angles of the patients in two groups had no significant differences.There were 6 cases and 12 cases of ventilation disturbance in CS and IS groups,and 15 cases and 25 cases of ventilation dysfunction.The levels of residual volume (RV),ratio of residual volume to total lung capacity (RV/TLC),RV/TLC percent predicted (RV/TLC% pred )in two groups were higher than the normal. The percentage of forced vital capacity predicted (FVC%pred)and FEV1/FVC in CS group were both lower than those in IS group (P<0.05).Some pulmonary function parameters such as vital capacity (VC%pred),FVC%pred,forced expiratory volume in one second (FEV1%pred), maximal mid expiratory flow (MMEF%pred), maximal voluntary ventilation (MVV%pred),TLC%pred and diffusion capacity for carbon monoxide of the lung (DLCO%pred)were negtively correlated with Cobb angle (r=-0.54--0.35,P<0.05)in IS group and the TLC%pred was negtively correlated with Cobb angle (r=-0.047,P<0.05)in CS group.Conclusion:Different types of scoliosis have influence in the pulmonary function of the patients.The degree of pulmonary function injury of the patients with IS is worse than that of the CS patients.The IS patients need treatment in time.

Early clinical observation of posterior approach operation for rigid scoliosis caused by lumbosacral hemivertebra in adolescencts / 重庆医学

Objective To evaluate the early clinical outcome of one‐stage posterior approach operation for rigid scoliosis caused by lumbosacral hemivertebra in adolescencts .Methods Eighteen adolescencts with rigid scoliosis caused by lumbosacral hemivertebra were surgically treated by the posterior hemivertebral resection and long segment fixation .Hemivertebrae were sited in L5 (14 cases) ,S1 (4 cases) .There were 12 cases of full‐segmented hemivertebrae and 5 cases of semi‐segmented hemivertebrae and 1 case of wedge vertebral body ,10 patients had coronal trunk imbalance .Results The patients were successfully completed sur‐gery .The operation time was 4 .5-8 .5 h ,averaged 6 .3 h ;and the perioperative bleeding was 600-2 800 mL ,averaged 1 180 mL . 2 patient suffered from nerve root injury who received neuro nutrition drugs and Prednisolone after operation and recovered com ‐pletely after one weeks .All cases were follow ed up for 12-38 months ,averaged 21 .6 months .The coronal Cobb angles of lumbo‐sacral curve and proximal lumbar after one week of operations and at the time of the last follow‐up were much better than those be‐fore the operations ,the differences were statistically significant (P<0 .05) .Postoperative radiography demonstrated that no implant failure was observed .Coronal trunk shift was improved in 17 patients after surgery ,1 patient had coronal trunk decompensation . Conclusion The posterior surgery with hemivertebrae resection and long segment fixation is an effective procedure in the treatment of rigid scoliosis caused by lumbosacral hemivertebra in adolescencts .The reconstruction of the coronal plane is the key to the oper‐ation .

Vertebral and Intraspinal Anomalies in Indian Population with Congenital Scoliosis: A Study of 119 Consecutive Patients

STUDY DESIGN: Retrospective case study by clinical and radiological data analysis. PURPOSE: To analyze different types of vertebral anomalies and the incidence of associated intraspinal anomalies in the Indian population. OVERVIEW OF LITERATURE: This is the largest study of congenital scoliosis and associated intraspinal anomalies in Indian population. Incidence of intraspinal anomaly in this series is 47% which is higher than previous literature. Hemivertebra was the most common anomaly as seen in previous studies. METHODS: A total of 119 patients with congenital scoliosis who underwent surgery between December 2006 and December 2012 were studied. Data was reviewed with medical records, plain radiographs, and magnetic resonance imaging (MRI) scans. RESULTS: Thoracolumbar curve was most common, seen in 43.6% of patients. In addition to scoliotic deformity, kyphosis was seen in 26% of patients. Failure of formation, the most common vertebral anomaly, was seen in 51.2% of patients, failure of segmentation was seen in 19.3% of patients, and there were 29.4% patients having both formation and segmentation anomalies. Hemivertebra was the most common vertebral anomaly seen in 66.3% of patients and for whom 63.2% were in thoracic spine. Intraspinal anomalies were associated with 47% of patients with congenital scoliosis. Tethered cord was the most common intraspinal abnormality and was found in 48.2% patients with intraspinal anomalies. The patients with failure of segmentation and mixed deformities were found to have a significantly higher incidence of intraspinal anomalies (65% and 57%, respectively) than those with failure of formation (34%). Out of 31 patients with kyphotic deformity 29% had intraspinal anomalies, and amongst them tethered cord was the most common anomaly seen in 66% patients. Out of 12 patients with neurocutaneous markers, 83% patients had intraspinal anomaly. CONCLUSIONS: Intraspinal anomalies were seen in 47% of patients with congenital scoliosis in the Indian population. Tethered cord was the most common intraspinal anomaly, seen in 48% cases of congenital scoliosis. Hemivertebra was seen in 66% cases and was the most common vertebral defect. Hence MRI imaging of whole spine should be done in all cases of congenital scoliosis before any surgical intervention. Special attention should be given to physical examination and visualization of any neurocutaneous markers, which are associated with a higher incidence of intraspinal anomalies; absence of a neurocutaneous marker, however, does not rule out intraspinal anomaly.

A Retrospective Study of Congenital Cardiac Abnormality Associated with Scoliosis

STUDY DESIGN: Retrospective study. PURPOSE: To identify the incidence of congenital cardiac abnormalities in patients who had scoliosis and underwent surgical treatment for scoliosis. OVERVIEW OF LITERATURE: Congenital and idiopathic scoliosis (IS) are associated with cardiac abnormalities. We sought to establish and compare the incidence of congenital cardiac abnormalities in patients with idiopathic and congenital scoliosis (CS) who underwent surgical treatment for scoliosis. METHODS: Ninety consecutive scoliosis patients, who underwent surgical correction of scoliosis, were classified as CS (55 patients, 28 female [51%]) and IS (35 patients, 21 female [60%]). The complete data of the patients, including medical records, plain radiograph and transthoracic echocardiography were retrospectively assessed. RESULTS: We found that mitral valve prolapse was the most common cardiac abnormality in both patients with IS (nine patients, 26%) and CS (13 patients, 24%). Other congenital cardiac abnormalities were atrial septal aneurysm (23% of IS patients, 18% of CS patients), pulmonary insufficiency (20% of IS patients, 4% of CS patients), aortic insufficiency (17% of IS patients), atrial septal defect (11% of IS patients, 13% of CS patients), patent foramen ovale (15% of CS patients), dextrocardia (4% of CS patients), bicuspid aortic valve (3% of IS patients), aortic stenosis (2% of CS patients), ventricular septal defect (2% of CS patients), and cardiomyopathy (2% of CS patients). CONCLUSIONS: We determined the increased incidence of congenital cardiac abnormalities among patients with congenital and IS. Mitral valve prolapse appeared to be the most prevalent congenital cardiac abnormality in both groups.

A Retrospective Study of Congenital Cardiac Abnormality Associated with Scoliosis

STUDY DESIGN: Retrospective study. PURPOSE: To identify the incidence of congenital cardiac abnormalities in patients who had scoliosis and underwent surgical treatment for scoliosis. OVERVIEW OF LITERATURE: Congenital and idiopathic scoliosis (IS) are associated with cardiac abnormalities. We sought to establish and compare the incidence of congenital cardiac abnormalities in patients with idiopathic and congenital scoliosis (CS) who underwent surgical treatment for scoliosis. METHODS: Ninety consecutive scoliosis patients, who underwent surgical correction of scoliosis, were classified as CS (55 patients, 28 female [51%]) and IS (35 patients, 21 female [60%]). The complete data of the patients, including medical records, plain radiograph and transthoracic echocardiography were retrospectively assessed. RESULTS: We found that mitral valve prolapse was the most common cardiac abnormality in both patients with IS (nine patients, 26%) and CS (13 patients, 24%). Other congenital cardiac abnormalities were atrial septal aneurysm (23% of IS patients, 18% of CS patients), pulmonary insufficiency (20% of IS patients, 4% of CS patients), aortic insufficiency (17% of IS patients), atrial septal defect (11% of IS patients, 13% of CS patients), patent foramen ovale (15% of CS patients), dextrocardia (4% of CS patients), bicuspid aortic valve (3% of IS patients), aortic stenosis (2% of CS patients), ventricular septal defect (2% of CS patients), and cardiomyopathy (2% of CS patients). CONCLUSIONS: We determined the increased incidence of congenital cardiac abnormalities among patients with congenital and IS. Mitral valve prolapse appeared to be the most prevalent congenital cardiac abnormality in both groups.

Posterior spinal osteotomy of severe and rigid congenital scoliosis with diastematomyelia / 中国综合临床

Objective To evaluate the effectiveness and safety of one stage posterior vertebral osteotomy for correction of severe and rigid congenital scoliosis associated with Ⅰ, Ⅱ type of diastematomyelia.Methods According to the diastematomyelia packet,52 patients were divided into type Ⅰ group performed with mediastinum resection combined with spinal osteotomy, group Ⅱ without treatment of diastematomyelia direct spinal osteotomy.Group Ⅲ spinal osteotomy directly without diastematomyelia.Results The mean operation time was (548.6±113.2) min,the average amount of bleeding was (3 728.6±1 436.5) ml.In group Ⅰ,the mean operation time was (608.6± 123.2) min, significantly longer than those of group Ⅱ ((521.3 ±102.4) min,t=2.787,P＜0.01).In group Ⅰ the average amount of bleeding was (5 018.3 ±2 174.2) ml, significant more than that of group Ⅱ((2 615.3± 1 132.8) ml,t=5.182,P＜0.01).Patients with preoperative Cobb angle measurement for (95.2± 14.3) degrees, postoperative for (35.2± 14.8) degrees, follow-up of 2 years for (37.6± 16.1) degrees, group Ⅰ included preoperative (92.3 ± 12.8) degrees, postoperative (32.6 ± 15.8)degrees, 2 years later (35.8 ± 17.2) degrees;group Ⅱ before operation (99.2 ± 17.3) degrees, postoperative (37.3±14.3)degrees, 2 years later (40.2± 15.3) degrees.The postoperative Cobb angle correction rate and correction loss rate showed no significant difference between two groups (P ＞0.05), a posterior spinal osteotomy for the treatment of type Ⅰ and type Ⅱ with diastematomyelia severe rigid congenital scoliosis has good correction effect.This group of patients, the complication rate was 21.2％ (11/52);where in Ⅰ group the incidence rate of 36.4％ (8/22) was significantly higher than that of Ⅱ group 10.0％ (3/30) (P =0.021).Conclusion One stage posterior vertebral osteotomy for severe rigid with diastematomyelia of congenital scoliosis with the feasibility, effectiveness and safety, patients with type Ⅰ diastematomyelia should first bony mediastinum resection, Ⅱ type of diastematomyelia there is no need for treatment of diastematomyelia.

Aspectos quirúrgicos en deformidades congénitas de la columna vertebral: clínico y radiológico / Surgical aspects in congenital spinal deformities: clinical and radiological

El tratamiento quirúrgico de las deformidades congénitas de la columna vertebral ha evolucionado en los últimos años desde fusiones in situ, hemiepifisiodesis hasta hemivertebrectomía y sustracción pedicular. En la actualidad no hay concenso cual es el procedimiento idóneo. Objetivo: Determinar la eficacia y utilidad de la Hemivertebrectomía y Sustracción Pedicular de los pacientes con Escoliosis Congénita y Cifoescoliosis. Métodos: se estudiaron 18 pacientes intervenidos en el período de enero 2013- diciembre del 2014, en un análisis estadístico retrospectivo. En 12 (66%) pacientes se realizó hemivertebrectomía y a 6 (33%) pacientes sustracción pedicular. Se evaluó edad, sexo, tipo de defecto, grado de corrección, niveles de resección y fusión, evolución neurológica, pérdida de sangre, tiempo quirúrgico y complicaciones. Resultados: se observó un ángulo de Cobb preoperatorio de 46º con escoliosis y 8 casos con ángulo de cifosis de 75º. Los porcentajes de corrección promedio de la escoliosis y la cifosis fueron 59 y 60% respectivamente, y el ángulo en el postoperatorio fue de 23º y 45º. La resección de la hemivertebra fue en un nivel (75%), dos niveles (33%). Se presentaron complicaciones en 8 pacientes, fístula de LCR, infección de la herida, pseudoartrosis, con pérdida de sangre de 528 ml, tiempo quirúrgico de 8 horas, y fusión 70%. Conclusiones: la hemivertebrectomía y la sustracción pedicular, constituyen una alternativa que permiten la corrección de la deformidad y estabilidad de la columna(AU)

Surgical treatment of congenital deformities of the spine has evolved in recent years from mergers in situ, hemiepiphysiodesis to hemivertebrectomy and pedicle subtraction. At present there is no consensus on the appropriate procedure. Objective: To determine the effectiveness and usefulness of the Hemivertebrectomía and Subtraction Pedicular of patients with congenital scoliosis and Kyphoscoliosis. Methods: in a retrospective statistical analysis 18 patients treated in the period January 2013-December 2014, were studied. In 12 (66%) patients a hemivertebrectomy was performed and a pedicle subtraction was performed on the other 6 (33%) patients. Age, sex, type of defect, degree of correction, resection and fusion levels, neurologic outcome, blood loss, operative time and complications were evaluated. Results: The preoperative Cobb angle of 46º with scoliosis and 8 cases with kyphosis angle of 75 ° were observed. The percentages of average correction of scoliosis and kyphosis were 59% and 60% respectively, and the postoperatively angle was 23º and 45º. Hemivertebra resection was on a level (75%); two levels (33%). There were complications in 8 patients, fistula LCR, wound infection, nonunion, with blood loss of 528 ml, operating time of 8 hours, and 70% merger. Conclusions: hemivertebrectomy pedicle subtraction are an alternative that allows the correction of the deformity and column stability(AU)

Comparative Study of Paravertebral Muscles in Congenital Scoliosis and Idiopathic Scoliosis Patients / 中国康复理论与实践

@#Objective To identify the role of paravertebral muscles in the pathogenesis of scoliosis.MethodsParavertebral muscles were gotten from the 37 patients(12 congenital scoliosis patients and 25 idiopathic scoliosis patients) during the operations.Cryostat sections were cut by 10 μm nd stained with H&E,m-GT,NADH-TR,ATPase.ResultsMyogenic changes,incuding muscle fibrosis,fiber necrosis,etc,were common in paravertebral muscles of scoliosis patients,however regenerating fibers were quite rare.Diffuse fibrosis and remarkablely disorganized fiber directions presented in most of congenital scoliosis patients,while focal fibrosis without necrosis in most of idiopathic scoliosis patients.Neurogenic changes were found in one congenital scoliosis patient and 4 idiopathic scoliosis patients,however four of the five patients had undergone orthopedics.Thickened capsule wall of muscle spindles and connective tissue infiltration in muscle spindles were found in both kinds of scoliosis.ConclusionThere are some differences on pathological changes of paravertebral muscles between congenital scoliosis and idiopathic scoliosis,which indicates that paravertebral muscles may play a special role in the pathogenesis of idiopathic scoliosis.

Posterior Hemivertebra Excision in Congenital Scoliosis / 대한척추외과학회지

STUDY DESIGN: Retrospective study OBJECTIVES: To evaluate the surgical outcome of congenital scoliosis with a hemivertebra treated by posterior hemivertebra excision and pedicle screw instrumentation. SUMMARY OF LITERATURE REVIEW: Posterior hemivertebra excision can be accomplished through a single posterior approach, and excellent correction and outcome may be achieved. MATERIALS AND METHODS: Forty patients with one fully segmented hemivertebra treated by posterior hemivertebra excision with pedicle screw instrumentation were retrospectively analyzed after a minimum follow-up of 2 years (range 2~7.7 years). The mean age at surgery was 15.9 years (range 2.6~37.9 years). Preoperative and postoperative standing radiographs were used to assess radiographic parameters. RESULTS: The average number of vertebrae in the major curve was 4.2 (range 3~8), and the average flexibility was 29% (range 8~59%). The average length of fusion was 3.5 segments (range 1~6). The number of fused vertebrae had a positive correlation with age at the index surgery (r=0.345, p<0.05). Mean preoperative scoliosis of 48+/-12degrees was corrected to a mean of 17+/-10degrees (65% correction), and mean preoperative kyphosis of 46+/-18degrees was corrected to a mean of 12+/-12degrees at the most recent follow-up. The compensatory curve had a mean of 25+/-10degrees preoperatively and spontaneously corrected to a mean of 8+/-8degrees (70% correction) at the most recent follow-up. The mean operating time was 233+/-81 min, with an average blood loss of 2904 ml. There was neither crankshaft phenomenon nor iatrogenic spinal stenosis in 6 patients under the age of 5 years after an average follow-up of 6 years. CONCLUSIONS: Posterior hemivertebra excision using pedicle screw instrumentation in congenital scoliosis due to a hemivertebra is a safe and effective procedure. Posterior hemivertebra excision at an early age may reduce the fusion length while avoiding the induction of iatrogenic spinal stenosis during follow-up.

Comparative Study of Paravertebral Muscles in Congenital Scoliosis and Idiopathic Scoliosis Patients / 中国康复理论与实践

@#Objective To identify the role of paravertebral muscles in the pathogenesis of scoliosis.MethodsParavertebral muscles were gotten from the 37 patients(12 congenital scoliosis patients and 25 idiopathic scoliosis patients) during the operations.Cryostat sections were cut by 10 μm nd stained with H&E,m-GT,NADH-TR,ATPase.ResultsMyogenic changes,incuding muscle fibrosis,fiber necrosis,etc,were common in paravertebral muscles of scoliosis patients,however regenerating fibers were quite rare.Diffuse fibrosis and remarkablely disorganized fiber directions presented in most of congenital scoliosis patients,while focal fibrosis without necrosis in most of idiopathic scoliosis patients.Neurogenic changes were found in one congenital scoliosis patient and 4 idiopathic scoliosis patients,however four of the five patients had undergone orthopedics.Thickened capsule wall of muscle spindles and connective tissue infiltration in muscle spindles were found in both kinds of scoliosis.ConclusionThere are some differences on pathological changes of paravertebral muscles between congenital scoliosis and idiopathic scoliosis,which indicates that paravertebral muscles may play a special role in the pathogenesis of idiopathic scoliosis.

LumbarWedgeResectionOsteotomyforCongenitalScoliosis duetoaSacralMalformation: A CaseReport / 대한정형외과학회잡지

Congenital scoliosis due to a sacral malformation is quite rare. To the best of our knowledge, most wedge resection osteotomies have been performed to correct a kyphotic deformity in ankylosing spondylitis. However, there is no report of a trapezoidal lumbar wedge resection osteotomy of the vertebral body in the surgical treatment of congenital scoliosis due to a sacral malformation. This paper reports a 41-year-old female with a 25-year history of lower back and buttock pain combined with radiating pain to the lower extremities. The coronal imbalance was 3.8 cm and the scoliosis angle using the Cobb method was 22 degrees. A trapezoidal wedge resection osteotomy of the L5 body was performed, and the scoliosis was corrected. We detail this modification of a vertebral osteotomy technique and show that a fixed coronal deformity could be corrected effectively using this technique.

Analysis on operative effect of posterior instrumentation and fusion for the surgical treatment of congenital kyphoscoliosis / 中国矫形外科杂志

[Objective]To evaluate the surgical effect of posterior correction and instrumentation on the treatment of congenital kyphoscolosis deformity. [Methods]From july 2002 to august 2007,there were 15 congenital kyphoscoliotic patients,which included 8 males and 7 females with an average age of 11.6 years.The average Cobb’s angle of scoliosis was 78?(35?~156?),the average Cobb’s angle of kyphosis was 25?(15?~38?).For the 9 kyphoscolsis patients with hemivertebra,hemivertebra resection and intervertebral fusion and fixation with pedicle screw instrument system were used,Luque’s systems was used in 2 cases,for the 3 cases accompanied with Chiari malformation and/or syringomyelia and 1 case with neurofibroma,posterior correction and long segmental pedicle screw fixation were performed and the compensative curve was included in the instrumentation level.[Results]The operation time was 3.5~7.5 h (average,5.5 h ),the blood loss was 300~2 100 ml (mean,850 ml);the average follow-up was 43 months (10~82 months),and the radiographs taken from standing posteroanterior position and lateral side showed that the mean Cobb’s angle of scoliosis was corrected from preoperative 78? to postoperative 28?(11?~38?),with a correction rate of 64%.Postoperatively ,the angle of kyphosis was improved from the preoperative 25? to 16?( 7?~33?),with a correction rate of 36%.Solid fusion was achieved in all 15 cases of patients. But significant blood loss occurred in the operative procedure of neurofibroma. And long term follow-up showed 3 cases of rod fracture,1 case of crankshaft phenomenon.[Conclusion]The congenital kyphoscolosis deformity is a kind of complex syndrome concerning skeleton-muscle-nerve system,aim to different cause,strategy of treatment should be individualized.The posterior hemivertebral resection and correction is a safe and effective method for the prepubertal children with the congenital hemivertebrae deformity.

Surgical treatment of scoliosis of thoracic hemivertebra and diastematomyelia of adolescent / 中国矫形外科杂志

[Objective] To discuss the operation and clinical results about surgical treatment of scoliosis of thoracic hemivertebra and diastematomyelia of adolescent.[Methods]From Jan 2001 to June 2007,15 patients were treated with one-stage posterior hemivertebrae and osseous divide resection combined with transpedicular instrumentation and bone graft.There were 6 males and 9 females with an average age of 21.2 years(range 16 to 24 years).All cases were segmented hemivertebrae.Hemivertebrae were located at T11(n=3)and T12(n=12).The status of the spinal fusion,correction rate and instrumentation were evaluated after surgery.[Results]All cases were followed up for 19 to 45 months with an average of 34 months.Cobb's angles of the main curve were 52.3??3.8?before surgery,10.2??1.4? after surgery.At the final follow-up there was 1.6? loss of correction.The mean height was increased by 3.76 cm.The district of bone graft showed good bone fusion.The time of vertebra fusion was 3 to 5 months(mean 3.6 months).No instrumentation,spinal fusion failure or other severe complications were noted.[Conclusion]One-stage posterior hemivertebrae and osseous divide resection combined with transpedicular instrumentation and bone graft can achieve a satisfactory result for the treatment of scoliosis of thoracic hemivertebra and diastematomyelia of adolescent.It can be recommended in clinical practice because of a good stability of fixation and fine spinal fusion.

Posterior hemivertebra resection and instrumentation correction in the treatment of congenital scoliosis / 中国矫形外科杂志

[Objective]To evaluate the clinical results of posterior hemivertebra resection and correction with transpedicular instrumentation for congenital scoliosis.[Method]From July 2002 to December 2006,thirty-nine consecutive cases,including 22 males and 17 females,underwent posterior hemivertebra resection and correction using transpedicular instrumentation.The average age at surgery was 8.3 years ranging from 3 to 15 years.There were 30 cases with fully segmented hemivertebra and 9 cases with partial segmented hemivertebra.Instrument included 16 cases of ISOLA,10 cases of CD-Horizon,10 cases of Moss-Miami and 3 cases of TSRH.The pre-and post-operative standing radiographic and at final follow-up were measured,and the Cobb's angles in the coronal and sagittal plane were analyzed,respectively.[Result]The average follow-up was 2.8 years ranging from 1 to 4.5 years.The Cobb's angle in coronal plane was corrected from 48? to 13?postoperatively and 13.2? at final follow-up,representing 70.2% correction rate.The kyphosis was corrected from 39? to 12.1? postoperatively and 12.7? at final follow-up,representing 68.7% correction rate.All cases had bony fusion,there was no neurological complication,no implant failure or infection.One case had crankshaft phenomenon.[Conclusion]Posterior hemiveterbra resection with instrumentation is a safe and effective treatment option for congenital scoliosis.Congenital scoliosis should be treated in early age.

Posterior segmental instrumentation for thoracolumbar hemivertebra:the case of loosening of instrumentation and reversion / 中国矫形外科杂志

[Objective]To evaluate the factors causing loosening of instrumentation and the progressive deformities after treating thoracolumbar bemivertebra with segmental instrumentation,and analyze the outcomes and strategies of reversion.[Method]A retrospective study was made in 7 cases with loosening of instrumentation and the progressive deformities secondary to operation on thoracolumbar hemivertebra in our hospital.There were 3 males and 4 females,with age range of 1.8~13 years.Preoperative mean Cobb's angles of scoliosis was 34.1?,and kyphosis was 40.2?.All cases were treated with posterior reversion.[Result]The failure factors of primary surgery were resulted from:failure of single hemivertebra resection(4 cases),shortage of segments fixation and bone graft(5 cases),unsatisfactory selection of instrumentation(2 cases),failure of manipulation in young children(2 cases),segment fixation without fusion(1 case).Postoperative mean Cobb's angle of scoliosis was 12.6?,with correction rate of 63.0%,and kyphosis was 17.3?,with correction rate of 56.9%.The revision cases were followed up for 18 to 28 months and showed that all patients got satisfactory cosmetic correction and fine fusion.No neurological complication or infection occurred,no implant failure were verified at the final radiographic evaluation.[Conclusion]The reasons for postoperative loosening of instrumentation and the progressive deformities includs:free from hemivertebrectomy,inappropriate segments fixation and bone graft,unsatisfactory selection of instrumentation,impropermanipulation in young children,fixation without fusion.If the above-mentioned five reasons of reversion strategies have been considered and avoided,the clinical results will be satisfactory.

Types and Associated Anomalies of Congenital Scoliosis / 대한척추외과학회지

STUDY DESIGN: Analysis was based on radiographic appearance of 57 cases of congenital scoliosis and associated anomaly PURPOSE: The aim of the present study was to assess the incidence, morphology and the associated anomalies of the congenital spinal scoliosis. SUMMARY OF LITERATURE REVIEW: Hemivertebra is the most common type of congenital scoliosis and urogenital, musculoskeletal and cardiac anomalies are strongly associated. MATERIALS AND METHODS: The authors analysed the morphology and the associated anomalies of 57 cases of congenital scoliosis from 1994 to 2000. RESULTS: It was more common in male(32 males and 25 females). The bony anomalies were classified as failure of formation(40cases, 70.2%), failure of segmentation(11cases, 19.3%) and mixed type(6cases, 10.5%). Of the failure of formation, there were 36 cases(63.2%) of hemivertebra, 2 cases of posterior quadrant vertebra and 2 cases of wedge vertebra. We found associated anomalies in 26 patients(45.6%). Associated cardiac anomalies were 2 dextrocardia, ventricular septal defect, atrial septal defect and patent ductus arteriosus. Associated musculoskeletal anomalies were 5 rib fusion, 2 developmental dysplastic hip, 3 Klippel-Feil syndrome, Achondroplasia, Arnold-Chiari malformation, spinal dysraphism with sacral hair patch, cleft palate with congenital anklyloglossia. Associated neurogenic anomalies were 2 cases of syringomyelia and 3 mental retardation. There were unilateral renal agenesis and undescended testicle in urogenital anomalies. CONCLUSIONS: Common type of the congenital scoliosis was hemivertebra(63.2%) caused by the failure of formation(70.2%). Associated anomalies were found in 26 patients(45.6%).