Clinical efficacy of screw placement assisted with the 3D printing pre-installed screw channel model in the single complete segmented congenital hemivertebrectomy / 中华实用儿科临床杂志

Objective:To explore the clinical effect of the 3D printing pre-installed screw channel model in assisting screw placement of single complete segmented congenital hemivertebrectomy.Methods:Clinical data of 13 children treated with single complete segmented congenital hemivertebrectomy in the Department of Spine and Spinal Surgery of Henan Provincial People′s Hospital from August 2016 to January 2019 were retrospectively analyzed.Among them, there were 5 males and 8 females with the mean age of 9.9 (5-14) years.Categorized by the lesion location, 3 cases were located at T 9, 2 cases at T 10, 5 cases at T 11, 1 case at T 12, and 2 cases at L 1.During the operation, the 3D printing pre-installed screw channel model was used to assist the placement of pedicle screws.The accuracy of screw placement was assessed by the postoperative CT.All children were routinely examined by full-length anterior and lateral X-ray of spine in the standing position before and after surgery to measure the Cobb angles at the coronal and sagittal view.Furthermore, the correction rate of scoliosis and kyphosis after surgery and during follow-up was also calculated.The One-Way repeated measures ANOVA was used to compare the Cobb angle of scoliosis and kyphosis before surgery, after surgery and during follow-up. Results:A total of 85 pedicle screws were placed in 13 children, with the accuracy rate of screw placement of 95.3%.The mean surgery time and intraoperative blood loss were (216.9±28.3) min, and (478.5±132.6) mL, respectively.Scoliosis Cobb was corrected from (57.1±12.7)° to (12.7±4.7)° with a correction rate of (78.4±5.9)%, which was (14.2±7.0)° at the last follow-up.Kyphosis angle was corrected from (46.2±8.4)° to (13.2±4.4)° with a correction rate of (72.6±7.0)%, which was (14.0±3.4)° at the last follow-up.None of the children had serious complications like vascular and nerve damage.The mean postoperative follow-up was 12.3 (6-18) months.No significant loss of angle was detected during the follow-up period.There were significant differences in the lateral and kyphotic angles after surgery and during follow-up compared with preoperative ones (all P<0.05). No significant difference was detected between the postoperative lateral and kyphotic angles and those at the last follow-up (all P>0.05). Conclusion:The 3D printing pre-installed screw channel model used to assist screw placement of single complete segmented congenital hemivertebrectomy can improve the precision of screw placement and the orthopedic effect on lateral kyphosis.

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)

A small supernumerary marker chromosome resulting in mosaic partial tetrasomy 4q26-q31.21 in a foetus with multiple congenital malformations

A parental diagnosis was performed for an unborn foetus of a healthy couple, who was due for ultrasound detection of multiple malformations and abnormal amniotic fluid karyotypes. For an accurate diagnosis, routine G-banding analysis and nextgeneration sequencing (NGS)were carried out. Finally, conventional cytogenetic analysis suggested that the foetus had a karyotype of47,XX,+mar[52]/46,XN,meanwhileNGSalso revealed a partial tetrasomy of 27.84Mbfrom4q26-q31.21 (117,385,735–145,225,759), and G-banding analysis excluded the couple to have carried the 4q26-q31.21 duplication. We have identified a de novo mosaic small supernumerary marker chromosomes (sSMC) derived from 4q26-q31.21 in a foetus with hemivertebra, polydactyly, abnormal ears, and heart and ventricular septal defect.

Resolución quirúrgica de escoliosis congénita: reporte de caso / Surgical resolution of congenital scoliosis: case report

Las deformidades congénitas de la columna vertebral, constituyen uno de los trastornos musculoesqueléticos no traumáticos de difícil manejo clínico quirúrgico, por el alto grado de deformidad que producen y porque su incidencia es menor que las idiopáticas. Se presenta el caso de una paciente de 14 años de edad, quien fue valorada en una consulta externa del Hospital Carlos Andrade Marín de la ciudad de Quito, Ecuador, con una deformidad severa toracolumbar, presente desde el nacimiento, que ha ido progresando hasta causar dolor a la movilidad y limitando sus actividades diarias, impidiendo un desarrollo social adecuado, por lo que, se decide su resolución quirúrgica mediante artrodesis posterior instrumentada, osteotomías correctivas y resección de hemivértebra y barra espinal, posterior a lo cual se realizó fisioterapia y un seguimiento por consulta externa de un año. Su evolución fue favorable, mejorando su sintomatología y movilidad.

Congenital deformities of the spine constitute one of the non-traumatic musculoskeletal disorders of difficult clinical surgical management, due to the high degree of deformity they produce and their incidence is lower than idiopathic ones. We present the case of a 14-year-old patient who was evaluated in the outpatient clinic of the Carlos Andrade Marín Hospital in the city of Quito, Ecuador, with a severe thoracolumbar deformity, present from birth, which has progressed to cause pain to mobility, limiting daily activities, preventing an adequate social development, for which, surgical resolution was decided through instrumented posterior arthrodesis, corrective osteotomies, and resection of hemivertebra and spinal rod, after which physiotherapy was performed and follow-up by external consultation for 1 year. Its evolution was favorable, improving its symptomatology and mobility.

Proximal junctional kyphosis after posterior hemivertebra resection and instrumented fusion in young children with congenital scoliosis / 中华外科杂志

Objective@#To investigate the risk factors of proximal junctional kyphosis(PJK) in young children who underwent posterior hemivertebra resection and instrumented fusion.@*Methods@#This study reviewed the charts and radiographs of 136 consecutive young children with congenital scoliosis who underwent posterior hemivertebra resection and instrumented fusion in Department of Orthopaedics, Nanjing Drum Tower Hospital, the Affiliated Hospital of Nanjing University Medical School from January 2009 to June 2014, including 75 males and 61 females, with an average age of (5.0±1.7)years(3-10 years). Proximal junctional angle(PJA), spino-pelvic parameters and segmental kyphosis (SK) were recorded pre- and post-operation and at last follow-up.The changes of PJA and radiographic features of proximal junction were also observed.χ2 test and t-test were used to analyzed enumeration data and measurement data, respectively.@*Results@#The average follow-up period was (32.8±10.3)months (ranging from 24 to 73 months) by June 2016. Among these patients, PJK occurred in 19 cases. Fifteen patients developed PJK during the first 3 months after surgery.The most common type of PJK was ligamentous failure.Compared with the non-PJK group (22.2%, 37.6%, 13.7%), the PJK group showed higher rate of preoperative TK>40°(9/19), fusion levels >4 (13/19) and greater SK change > 30°(9/19)(χ2=7.259, 6.375, 12.368; all P<0.05), while there were no difference between the two groups in terms of preoperative PJA, lumbar lordosis, SVA and upper instrument vertebra location(all P>0.05). The average PJA increased from 7.5°±2.9° to 21.3°±4.3° at 3 months after surgery to 20.6°±3.7° at the final follow-up visit in the PJK group.At the time of the final follow-up visit, ten patients received brace treatment, with no significant progression of PJA.@*Conclusions@#PJK might mainly occurs within 3 months postoperatively.Its prognostic factors include preoperative hyperkyphosis, over correction of kyphosis and ligamentous failure.

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 Tethered Cord with Hemivertebra: A Case Report and Review of Literature

Spinal dysraphisms are defined as open and closed dysraphisms. A hemivertebra is a congenital condition seen in 61% of patients with congenital anomalies. The first report of the excision of a hemivertebra was by Royle in 1928. A sixteen-year-old girl was admitted to our clinic with a congenital stain on the waist and a normal neurological examination. No new cases have been reported in recent literature. Our case, which is also rare, is associated with a tethered cord only and no other congenital abnormalities.

Early diagnosis and treatment of congenital hemivertebra malformation / 中华实用儿科临床杂志

Hemivertebra (HV) is a congenital spinal abnormality due to a defection of formation of one side vertebral.It is divided into 3 types according to whether it fusion with adjacent vertebral : fully segmented, semi-segmented and incarcerated.HV deformity is diagnosed depending on clinical symptoms, X rays, CT and other examine.Preoperative magnetic resonance imaging of the spine, genitourinary and cardiac ultrasound should be performed at the same time.Congenital scoliosis due to HV especially fully segmented is nonresponsive to bracing,operative treatment is the mainstay of care.The 3 basic operations are fusion in situ, convex growth arrest (epiphysiodesis), and HV resection.The single posterior approach or combined anterior and posterior approach of HV resection with instrumentation is the main method now.