Comparison of the efficacy of the convex side short fusion combined with concave side single growing rod technique and the traditional bilateral growing rod technique in the treatment of early onset scoliosis.

OBJECTIVES: The application of a growing rod technique can retain the growth and development potential of the spine and thorax while controlling the progression of scoliosis deformity. Theoretically, convex side short fusion combined with a concave side single growing rod technique can significantly reduce the asymmetric growth of the spine in the vertex region in most patients. However, the final clinical outcome of various techniques is yet to be clearly determined and compared between studies. Therefore, we compared the efficacy of these two growing rod techniques in treating early onset scoliosis. METHODS: In a retrospective study of 152 EOS patients seen between 2013.1 and 2019.12, 36 cases of EOS patients were selected for inclusion. Among the 36 cases, 11 cases were treated with convex side short fusion combined with a concave side single growing rod technique, group (A) The remaining 25 cases were treated with traditional bilateral growing rod technique, group (B) Age, gender, etiology, follow-up time, Cobb angle of main curve, T1-S1 height, coronal trunk shift, sagittal vertical axis (SVA), Cobb angle of thoracic kyphosis at last follow-up, and Cobb angle at proximal junction kyphosis of the first and last post-operation follow-up were recorded. In addition, internal fixation related complications, infection, nervous system complications were recorded as well. RESULTS: There was no statistically significant difference between group A and group B in preoperative age, Cobb angle of main curve, coronal trunk shift, T1-S1 height, SVA, Cobb angle of thoracic kyphosis (p > 0.05). However, at the last follow-up (Group A, mean 4.4 ± 1.01 years; Group B, mean 3.6 ± 0.01 years) the Cobb angle of the main curve was less and T1-S1 height greater in group A compared with group B (p < 0.05). There was no statistically significant difference between group A and group B in the correction rate of the Cobb angle of the main curve or the growth rate of T1-S1 height (p > 0.05). There was no statistically significant difference in the coronal imbalance ratio, thoracic kyphosis abnormality ratio, or the occurrence PJK ratio between group A and group B at the last follow-up (p > 0.05), but the sagittal imbalance ratio and internal fixation abnormality ratio were higher in group A than in the group B (p < 0.05). CONCLUSIONS: During the treatment of EOS, both the convex side short fusion combined with concave side single growing rod technique and traditional bilateral growing rod technique can correct the Cobb angle of main curve with no significant hindering of the spinal growth observed. The traditional bilateral growing rod technique has advantages in control of the sagittal balance of the spine, and the complications associated with internal fixation were lower.

Is it necessary to extend fusion to L4 when correcting pediatric L5-S1 spondylolisthesis?

OBJECTIVE: The objective of this study was to investigate whether extending fusion to L4 is imperative in the surgical treatment of pediatric L5-S1 spondylolisthesis. METHODS: This retrospective analysis encompassed 68 pediatric cases of dysplastic L5-S1 spondylolisthesis who underwent posterior lumbar interbody fusion surgery at two hospitals. Patients were categorized into two groups based on the upper instrumented vertebra (group L4 and group L5). Data were collected from medical records and radiological images obtained preoperatively and at last follow-up. Radiographic parameters including slip percentage (SP), lumbar lordosis (LL), sagittal vertical axis (SVA), pelvic incidence (PI), Spinal Deformity Study Group dysplastic lumbosacral angle (SDSG-LSA), pelvic tilt (PT), Dubousset's lumbosacral angle (Dub-LSA), sacral slope (SS), and severity index (SI) were measured. Surgery-related data and complication data were also collected. The incidence rates of complications were compared, including those of neurological deficit, adjacent-segment instability (ASI), and other complications. ASI was defined as progression of slippage > 3 mm or posterior opening > 5° in the adjacent segment. Clinical outcomes were assessed with the numeric rating scale (NRS) and the Oswestry Disability Index (ODI) scores. The follow-up period for all patients lasted a minimum of 2 years. RESULTS: Among all 68 patients, group L4 consisted of 15 patients and group L5 comprised 53 patients. The patients included in both groups had comparable baseline demographic characteristics and radiographic parameters. Postoperative SP and SDSG-LSA were significantly lower in group L5 (p < 0.05). No other postoperative radiographic differences were observed between groups. One patient in group L4 and 3 patients in group L5 experienced transient neurological deficits (p > 0.05). There were 13 cases of ASI in group L5 compared with none in group L4 (24.5% vs 0%, p > 0.05). Of the 13 patients with ASI, 4 underwent revision surgery due to L4-5 level instability and clinical symptoms. The remaining individuals exhibited no symptoms, and regular annual follow-up assessments are being conducted for all patients. The NRS and ODI scores at final follow-up did not exhibit any significant differences between the two groups. CONCLUSIONS: Fusion to L5 could achieve comparable satisfactory results to fixation to L4, albeit with increased likelihood of ASI. Extension of fusion to L4 may not be necessary for most patients with pediatric L5-S1 spondylolisthesis.

Robot-assisted Temporary Hemiepiphysiodesis With Eight-plates for Lower Extremity Deformities in Children.

PURPOSE: This study was performed to compare the radiographic results of robot-assisted and traditional methods of treating lower extremity deformities (LEDs). METHODS: From January 2019 to February 2022, 55 patients with LEDs were treated by temporary hemiepiphysiodesis with eight-plates. They were divided into a robot group and a freehand group. The fluoroscopy time and operation time were recorded. The accuracy of screw placement was measured after the operation using the following parameters: coronal entering point (CEP), sagittal entering point (SEP), and angle between the screw and epiphyseal plate (ASEP). The limb length discrepancy (LLD) and femorotibial angle (FTA) were measured before the operation, after the operation, and at the last follow-up. Patients were followed up for 12 to 24 months, and the radiographic results of the 2 groups were compared. RESULTS: Among the 55 patients with LEDs, 36 had LLD and 19 had angular deformities. Seventy-six screws were placed in the robot group and 85 in the freehand group. There was no difference in the CEP between the 2 groups ( P >0.05). The robot group had a better SEP (2.96±1.60 vs. 6.47±2.80 mm) and ASEP (3.46°±1.58° vs. 6.92°±3.92°) than the freehand group ( P <0.001). At the last follow-up, there was no difference in the LLD or FTA improvement between the two groups ( P >0.05). The incidence of complications was significantly lower in the robot group than in the freehand group (0/27 vs. 5/28, P <0.05). CONCLUSION: Robot-assisted temporary hemiepiphysiodesis with eight-plates is a safe and effective method for treating LEDs in children. Robotic placement of screws is superior to freehand placement with respect to the entering position and direction. Although the correction effect for LLD and angular deformity is similar, screw dislocation is less common when using robot assistance. LEVELS OF EVIDENCE: Level-III. Retrospective comparative study.

Safety and effectiveness of halo gravity traction combined with traditional growing rods in severe early-onset scoliosis with neurofibromatosis type 1.

Neurofibromatosis type 1 (NF-1) scoliosis can be difficult to treat without early detection. Correcting deformities while considering long-term growth in early-onset scoliosis (EOS) treatment is important. This study was performed to establish the safety and effectiveness of halo gravity traction (HGT) with traditional growing rods (TGRs) in NF-1 EOS. We retrospectively reviewed a cohort of 15 children (7 boys and 8 girls; mean age, 5.61 years) diagnosed with NF-1 EOS from October 2016 to March 2021. All patients underwent HGT before growing rod implantation. The growing rods were lengthened every 9-12 months, with a follow-up of 2-7 years. Cobb angle, thoracic kyphosis (TK), trunk shift (TS), sagittal vertebral axis and T1-S1 height were measured before operation, after traction, after operation and at last follow-up. Complications were also recorded. Fifteen patients with NF-1 EOS were treated with an average traction weight of 10.00 kg. After 29.20 days of HGT, the Cobb angle improved from 99.10° to 62.60°, TK from 79.33° to 55.04°, TS from 31.05 to 17.71 mm, sagittal vertebral axis from 42.07 to 25.63 mm and T1-S1 height from 27.50 to 29.70 cm (P < 0.05 for all). Postoperatively, compared with post-traction, the Cobb angle was 52.40° (P = 0.002) and TK was 44.54° (P = 0.004). No complications occurred during traction. Growing rod dislocation occurred in one patient and growing rod breakage in one patient. HGT combined with TGRs was well-tolerated and effective for treating severe NF-1 EOS. It significantly corrected the Cobb angle and TK, restored trunk balance, and increased spinal height with few complications.

Analysis of poor prognostic factors for septic arthritis of the hip in children: a case series of 76 patients.

The clinical characteristics, treatment and outcomes of children with septic arthritis of the hip in our hospital were analyzed to identify the risk factors for a poor prognosis. The clinical data of 76 children with septic arthritis of the hip who were treated at our hospital from January 2010 to December 2020 were retrospectively analyzed. According to the most recent follow-up data, the patients were classified as good prognosis or poor prognosis. The differences between the two groups were analyzed. From January 2010 to December 2020, a total of 76 children with septic arthritis of the hip were admitted to our hospital, comprising 52 (68.4%) with a good prognosis and 24 (31.6%) with a poor prognosis. The risk of a poor prognosis was significantly higher in the group with time from onset to surgery >22 days than in the group with time from onset to surgery <11 days. The risk of poor prognosis in the group with C-reactive protein (CRP) > 100 mg/L was significantly higher than that in the group with CRP < 20 mg/L. Time from onset to surgery >14 days and CRP > 93 mg/L were the cutoff values for a poor prognosis. Significant elevation of CRP and prolonged time from onset to surgery in children with septic arthritis of the hip are risk factors for a poor prognosis. Early diagnosis and effective treatment are very important because delays in these factors can lead to a poor prognosis. Level of Evidence: Level II, retrospective study.

Identification of novel homozygous nonsense SLC10A7 variant causing short stature, amelogenesis imperfecta, and skeletal dysplasia with scoliosis and surgical management of spine.

BACKGROUND: Short stature, amelogenesis imperfecta, and skeletal dysplasia with scoliosis is a rare, autosomal recessive, skeletal disorder first described in 2018. This syndrome starts with pre- and postnatal developmental delay, and gradually presents with variable facial dysmorphisms, a short stature, amelogenesis imperfecta, and progressive skeletal dysplasia affecting the limbs, joints, hands, feet, and spine. CASE PRESENTATION: We identified a homozygous novel nonsense mutation in exon 1 of SLC10A7 (NM_001300842.2: c.100G > T / p.Gly34*) segregating with the typical disease phenotype in a Han Chinese family. We reviewed the 12-year surgical treatment history with seven interventions on spine. CONCLUSION: To date, only 12 cases of the SLC10A7 mutation have been reported, mainly from consanguineous families. Our patient showed a relatively severe and broad clinical phenotype compared with previously reported cases. In this patient, annual check-ups and timely surgeries led to a good outcome.

Scoliosis risk factors and outcomes in children with dysplastic spondylolisthesis undergoing surgical reduction and fixation.

Purpose: To explore scoliosis risk factors and outcomes in children with dysplastic spondylolisthesis undergoing surgical reduction and internal fixation. Methods: We retrospectively analyzed 56 children with dysplastic spondylolisthesis who underwent surgical reduction and internal fixation. Patients were grouped according to presence of scoliosis before surgery. Radiographic parameters measured before surgery included pelvic incidence, pelvic tilt, sacral slope, coronal Cobb angle, slip percentage, Dubousset's lumbosacral angle, lumbar lordosis, sagittal vertical axis, and Spinal Deformity Study Group index. Groups were compared using logistic regression. Receiver operating characteristic analysis was performed to determine the optimal Spinal Deformity Study Group index cut-off value. All patients were followed up for at least 2 years. Results: The scoliosis group comprises 36 patients (mean age: 9.6 ± 2.7 years), while the no scoliosis group comprises 20 (mean age: 9.1 ± 2.4 years). Slip percentage and Spinal Deformity Study Group index were significantly higher in the scoliosis group (p < 0.01). Sacral slope and pelvic incidence were lower in the scoliosis group (p < 0.05). Univariate logistic regression analysis showed that slip percentage, Spinal Deformity Study Group index, pelvic incidence, and sacral slope were significantly associated with scoliosis. In the multivariate logistic regression analysis, only Spinal Deformity Study Group index was an independent risk factor for scoliosis. The optimal cut-off value for Spinal Deformity Study Group index was 0.288. Mean Cobb angle decreased from 20.3° ± 8.8° before surgery to 8.5° ± 8.9° at last follow-up; the mean scoliosis correction rate was 59.3%. Conclusion: Severe S1 dysplasia and high slip percentage may be risk factors for developing scoliosis in patients with dysplastic spondylolisthesis. Scoliosis resolved spontaneously after spondylolisthesis reduction and fixation in most patients. Level of evidence: 3.

Clinical characteristics of and risk factors for poor outcomes in children with bacterial culture-negative septic arthritis of the hip.

BACKGROUND: Septic arthritis of the hip is a serious infection in children. However, blood and joint fluid cultures are often negative, which makes the diagnosis and treatment challenging. We analyzed the clinical features of children with septic arthritis of the hip with negative bacterial cultures and identified the risk factors for poor outcomes. METHODS: The clinical data of children with septic arthritis of the hip with negative bacterial cultures who were treated at our hospital from January 2010 to December 2020 were retrospectively analyzed. The clinical characteristics and outcomes of the culture-negative cohort were compared with those of children with positive bacterial cultures treated during the same period. Culture-negative patients were divided into a group with good outcomes and a group with poor outcomes. The differences between the two groups were compared. RESULTS: Thirty-nine children with culture-negative septic arthritis of the hip were compared with 37 children with culture-positive sepsis. Compared with the culture-positive group, the culture-negative group had a significantly younger mean age and a significantly lower mean serum C-reactive protein concentration. Logistic regression analysis of culture-negative patients with good versus poor outcomes revealed that the independent risk factors for poor outcomes were an increased serum C-reactive protein concentration and prolonged time from onset to surgery. The cut-off values for predicting a poor outcome in the culture-negative group were a time from onset to surgery of greater than 21 days and a C-reactive protein concentration of greater than 23 mg/L. CONCLUSIONS: Culture-negative septic arthritis of the hip has similar clinical features to culture-positive septic arthritis of the hip and can result in sequelae of varying severity. Therefore, active anti-infective and hip drainage therapy should be performed when children present with clinical symptoms, inflammatory marker concentrations, and imaging findings that are clinically diagnostic for septic arthritis of the hip. LEVEL OF EVIDENCE: Level II, retrospective study.

Tinnitus assessment: Chinese version of the Tinnitus Primary Function Questionnaire.

Objective: The aim of this study is to evaluate the Chinese Version of the Tinnitus Primary Function Questionnaire (TPFQ). Methods: One hundred and sixteen patients who had been suffering from tinnitus for over 3 months were included in this study. Those tinnitus patients were administered the TPFQ, the Tinnitus Handicap Inventory (THI), the Beck Anxiety Inventory (BAI), the Beck Depression Inventory (BDI), and the Pittsburgh Sleep Quality Index (PSQI). Additionally, the magnitude estimate of tinnitus loudness, pure tone audiogram, and tinnitus matching was obtained. The factor structure was measured using the Kaiser-Meyer-Olkin test. The internal consistency was examined using Cronbach's α coefficient. The relationships between the TPFQ scores and other measurements were compared using Spearman's rank correlation coefficient. Results: The Cronbach's α of the 20-item version of TPFQ was 0.94, and that of the 12-item version of TPFQ was 0.92. Both the 20- and 12-item versions of TPFQ were significantly correlated with magnitude estimation of tinnitus loudness, THI, PSQI, BDI, and BAI. The average pure tone hearing threshold was significantly correlated with the hearing subscale. Conclusion: The 20- and 12-item Chinese versions of TPFQ are reliable and valid measures of tinnitus. The TPFQ can be applied to the assessment and management of tinnitus among the Chinese-speaking population.

Surgical Treatment of Severe Sprengel's Deformity: A Case Report.

CASE: An adolescent girl who presented with obviously impaired shoulder abduction due to untreated severe Sprengel's deformity underwent deformity correction surgery. Intraoperative neuromonitoring was used to warn of potential brachial plexus injury during a modified Woodward procedure. At 3-month follow-up, range of shoulder abduction had improved significantly. CONCLUSION: Sprengel's deformity is a rare congenital shoulder deformity, and the Woodward procedure could cause nerve injury in patients with severe Sprengel's deformity. Neuromonitoring can be performed intraoperatively to avoid brachial plexus injury.

Outcomes and Safety of Traditional Growing Rod Technique in the Treatment of Early-onset Dystrophic Scoliosis Secondary to Type 1 Neurofibromatosis With Intraspinal Rib Head Dislocation in Children.

PURPOSE: The purpose of this study was to investigate the outcomes and safety of traditional growing rod (TGR) in the treatment of early-onset dystrophic scoliosis secondary to type 1 neurofibromatosis (NF1-EOS) with intraspinal rib head dislocation (IRH) in children. METHODS: From September 2006 to June 2020, 21 patients with NF1-EOS were treated with TGR. The patients comprised 13 boys and 8 girls with a mean age of 7.1±1.5 years. Two patients had IRH-induced nerve injury [American Spinal Injury Association (ASIA) grade D]. No neurological symptoms were found in the other patients. The intraspinal rib proportion, apical vertebral rotation, apical vertebral translation, coronal main thoracic curve, trunk shift, thoracic kyphosis, lumbar lordosis, sagittal balance, and T1-S1 height were measured before and after TGR implantation and at the last follow-up. Complications were also evaluated. RESULTS: The mean follow-up time was 3.4±2.0 years. An average of 3.1 times (range: 1 to 8 times) lengthening procedures were performed in each patient. The intraspinal rib proportion was significantly lower postoperatively than preoperatively (22±11% vs. 33±18%, respectively; P <0.001), and no significant correction loss was found at the last follow-up (24±12%, P= 0.364). Compared with the measurements before TGR implantation, the major coronal curve and T1-S1 height after TGR implantation and at the last follow-up were significantly different ( P <0.05). Significant correction of apical vertebral translation, thoracic kyphosis, lumbar lordosis, and sagittal balance were noted after TGR implantation, and no significant correction loss was found at the last follow-up ( P >0.05). Ten complications occurred in 7 patients. Two patients with nerve injury recovered after the operation. No neurological complications were found during the follow-up. CONCLUSIONS: TGR is a safe and effective therapy for NF1-EOS with IRH where there was no direct compression of the spinal cord, which was confirmed by preoperative magnetic resonance imaging. Through this procedure, IRH could be partly removed from the spinal canal. LEVEL OF EVIDENCE: Level III.

Benefits of fixing 3 proximal vertebral bodies vs. 2 in the treatment of early-onset scoliosis with growing rods.

Additional proximal fixation for growing rods in early-onset scoliosis (EOS) may offer a more effective and safer option for severe scoliosis patients with hyper-kyphosis. Here, we compared the outcomes of EOS patients treated with growing rods in which 6 proximal anchor points on 3 vertebrae were used vs. 4 proximal anchor points on 2 vertebrae. The records of patients with EOS treated surgically from January 2016 to December 2017 were retrospectively reviewed. In the Proximal 4 group, 2 vertebral bodies were anchored proximally with 4 anchor points; in the Proximal 6 group, 3 vertebral bodies were anchored proximally with 6 anchor points. Forty-two patients (mean age 5.11 ± 1.93 years) were included; 22 Proximal 4 group, 20 Proximal 6 group. Mean follow-up was 40.86 ± 13.49 months. The decrease in main curve Cobb angle postoperatively was significantly greater in the Proximal 6 group (33.22° vs. 19.08°) ( P < 0.05). Cobb thoracic kyphosis (TK) was significantly decreased postoperatively in the Proximal 6 group (mean 20.70°); no significant decrease occurred in the Proximal 4 group. The main curve Cobb angle decrease at last follow-up was significantly greater in the Proximal 6 group (37.84° vs. 24.23°) ( P < 0.05). Cobb TK was significantly decreased at last follow-up in the Proximal 6 group (mean 25.17°, P < 0.05); no significant decrease occurred in the Proximal 4 group. Instrument complications were lower in the Proximal 6 group (15.00% vs. 45.45%) ( P < 0.05). No proximal junctional kyphosis was noted. Fixing 3 proximal vertebral bodies with 6 anchors improves radiographic outcomes of EOS treated with growing rods, and has a lower rate of screw pull-out.

Medium-term and Long-term Follow-up Surgical Outcomes of the 1-stage Posterior-only Lumbosacral Hemivertebra Resection With Short-segment Fusion in Children.

BACKGROUND: The objective of this study was to evaluate the medium-term and long-term surgical outcomes of the 1-stage posterior-only lumbosacral hemivertebra resection with short-segment fusion in children. METHODS: This retrospective chart review included 21 children with congenital scoliosis due to lumbosacral hemivertebra who received 1-stage posterior-only hemivertebra resection with short-segment fusion from 2012 to 2016 with at least 5 years of follow-up. Standing anteroposterior and lateral radiographs of the spine were compared preoperatively, postoperatively, and at last follow-up. Radiographic evaluation included measured changes in segmental scoliosis and lordosis, compensatory scoliosis, thoracic kyphosis, lumbar lordosis, trunk shift, and sagittal spinopelvic alignment. RESULTS: There were 12 boys and 9 girls with a mean age of 6.5±3.2 years. The mean follow-up period was 6.7±1.3 years. The mean fusion level was 2.7±0.9 segments. The mean segmental scoliosis was 29±6 degrees preoperatively, 9±3 degrees (correction rate of 71%) postoperatively ( P <0.05), and 7±3 degrees (correction rate of 76%) at the latest follow-up. The compensatory curve of 26±12 degrees was spontaneously corrected to 14±8 degrees (correction rate of 47%) at last follow-up ( P <0.05). Trunk shift was significantly improved on both coronal (53%) and sagittal (56%) planes after surgery ( P 0.05) and stable at follow-up. The sagittal spinopelvic alignment was balanced in all cases. There were no neurological or infectious complications. CONCLUSIONS: It is safe and effective to perform 1-stage posterior-only lumbosacral hemivertebra resection with short-segment fusion, which can significantly correct the segmental scoliosis, prevent the compensatory curve progress and improve the trunk shift. This strategy also can save motion segments and avoid long lumbar fusion. Medium-term and long-term follow-up outcomes are satisfactory. LEVEL OF EVIDENCE: Level III.

Clinical diagnosis and treatment of subungual exostosis in children.

Objective: To analyze and summarize the clinical characteristics and treatment effects for subungual exostosis in children. Methods: Clinical data for children with subungual exostosis treated in our department from January 2008 to September 2022 were evaluated. Results: Forty children with subungual exostosis were evaluated, comprising 31 boys (77.5%) and 9 girls (22.5%) with a median age of 9 years (4-17 years). The median disease course was 6 months (1-48 months). Seven patients (17.5%) had definite trauma history and 5 (12.5%) had infection. The toe or finger nail appearance was abnormal in 36 patients and normal in 4 patients. Twenty-seven patients (67.5%) had pain when wearing shoes and walking, and 25 (62.5%) had toenail tenderness. The lesions were located in the distal phalanxes of the toes in 37 patients (92.5%), with 14 patients affected on the left side and 23 on the right side. Twenty-two patients had lesions in the great toe, 6 in the second toe, 6 in the third toe, and 3 in the fourth toe. The lesions in the other 3 patients (7.5%) were located in the distal phalanxes of the fingers, with 2 patients affected in the second finger and 1 in the third finger. Regarding the relationship between lesion location and nail bed, 4 patients were type I, 21 were type II, and 15 were type III. All 40 patients received surgical treatment, with nail removal in 15. The median maximum lesion diameter was 1.0 cm (0.8-2 cm), median operation time was 25 min (20-45 min), median blood loss was 1 ml (1-2 ml), and median postoperative hospital stay was 2 days (1-4 days). All cases were histopathologically confirmed as subungual exostosis. The median follow-up time was 24 months (3-60 months), with normal appearance of the toe or finger nail. There were no complications in 38 patients (95.0%). Two patients (5.0%) relapsed at 3 months postoperatively and underwent a secondary operation, with no subsequent recurrence during 12 months of follow-up. Conclusion: Subungual exostosis in children is a rare benign disease that often occurs in the toes. Selection of the appropriate incision and nail bed treatment based on the relationship between lesion location and nail bed is helpful for improving the treatment effect.

Intraoperative hypotension during surgical treatment for Marfan syndrome scoliosis in children.

Purpose: To explore the occurrence and risk factors of intraoperative hypotension during children's Marfan syndrome scoliosis surgery and summarize the associated hemodynamic features and handle measures. Methods: Twenty-two Marfan syndrome scoliosis patients who underwent spinal surgery at Beijing Children's Hospital were retrospectively reviewed between January 2001 and January 2020. Intraoperative hypotension is defined as the minimum mean arterial pressure ≤ 60 mm Hg. The patients were divided into the hypotension group and the control group. Clinical, radiographic, and operative data were compared between the two groups. The risk factors, hemodynamic features, and handle measures for intraoperative hypotension in Marfan syndrome scoliosis surgery were analyzed and summarized. Results: Twenty-two patients were included in the study, with a mean age of 11.4 years at initial surgery. The follow-up period ranged from 24 to 152 months. Intraoperative hypotension occurred in 14 cases, with an incidence of 63.6%. The proportion of pulmonary dysfunction in the hypotension group was higher than in the control group (100.0% vs 50.0%, p < 0.05). The spinal flexibility was significantly lower in the hypotension group (28.3% ± 14.2% vs 46.5% ± 11.5%, p < 0.05). Fourteen patients with intraoperative hypotension had decreased intraoperative systolic blood pressure 21.0%-50.0% compared with baseline. One patient had a transient decrease in the muscle strength of the lower limbs. No complications were observed during the follow-up. Conclusion: The incidence of intraoperative hypotension in Marfan syndrome scoliosis children who underwent surgery was 63.6%. The risk factors included preoperative pulmonary dysfunction and poor spinal flexibility. Comprehensive preoperative evaluation and effective hemodynamic handling measures should be undertaken to prevent further complications in children with Marfan syndrome scoliosis.

Genotypes and clinical intervention of patients with neurofibromatosis type 1 associated dystrophic scoliosis.

Objective: To analyze the genotypic characteristics of patients with neurofibromatosis type 1 (NF1) associated dystrophic scoliosis and to summarize the outcomes of the surgical treatment of these patients. Methods: Exome sequencing (ES) combined with multiplex ligation-dependent probe amplification (MLPA) was used for genotypic identification. All patients underwent surgical treatments for spinal deformities, and the outcomes of the surgery was summarized by analyzing the clinical and imaging parameters before and after the surgery. Results: Fourteen patients (six males and eight females) were clinically diagnosed as NF1 associated dystrophic scoliosis with common symptoms including café-au-lait spots, paravertebral tumors, and dystrophic scoliosis. NF1 mutations were detected in 12 (85.7%) patients, including four nonsense mutations, three splicing mutations, three frameshift mutations, and two exon deletions. The first surgical procedure included growing-rod surgery in 10 patients and posterior spinal fusion in four patients. The follow-up duration was 2.3 years (1.0-10.3 years), and the Cobb angle of the main curve improved from 61.5° (30°-125°) pre-operatively to 14.5° (0°-42°) at the last follow-up, with an average correction rate of 74.0% (44-100%). Instrumentation-related complications occurred in four patients during the follow-up period. Conclusions: In patients with dystrophic scoliosis who met the clinical diagnostic criteria for NF1, the mutation detection rate of ES combined with MLPA was 85.7%. There was no mutation hotspot in NF1 gene, molecular diagnosis could offer information about genetic counseling, prenatal diagnosis and eugenics. Surgical treatment according to patient's age and severity could effectively correct the spinal deformities.

Scoliosis Secondary to Neurofibromatosis Type I Combined With Moyamoya Syndrome: A Case Report.

CASE: A 13-year-old girl presenting with scoliosis accompanied by type I neurofibromatosis (NF1) underwent deformity correction surgery. On the second postoperative day, she had sudden-onset acute cerebral infarctions and was diagnosed with moyamoya syndrome. After neurological conservative treatment, at the sixth month after surgery, her symptoms and signs were significantly improved and the daily life was not affected by herself. CONCLUSION: NF1 scoliosis with moyamoya syndrome is an uncommon inherited disorder. Intracranial vasculopathy is hardly detected from routine preoperative examinations. The specific intracranial vascular examination should be performed for patients with NF1 scoliosis, especially those who have a positive history of cerebral ischemic or hemorrhagic symptoms.

Molecular identification of T-box transcription factor 6 and prognostic assessment in patients with congenital scoliosis: A single-center study.

Background: Congenital scoliosis (CS) is characterized by vertebral malformations. The precise etiology of CS is not fully defined. A compound inheritance of TBX6 was identified in 10% of patients with CS in Han Chinese and formed a distinguishable subtype named TBX6-associated congenital scoliosis (TACS). Methods: To investigate the variants and risk haplotype of TBX6, we recruited 121 patients with CS at Beijing Children's Hospital. We collected the clinical characteristics and surgical treatment options and followed their postoperative prognoses. Results: Eight patients (6.6%) were molecularly diagnosed with TACS and carried the previously defined pathogenic TBX6 compound heterozygous variants. All the eight patients with TACS had the typical TACS clinical feature of hemivertebrae in the lower part of the spine. These patients received posterior hemivertebra resection combined with segmental fusion. Follow-ups revealed satisfactory correction without postoperative complications. Conclusion: We observed a 6.6% prevalence of TACS in our CS cohort. Follow-ups further highlighted that surgical treatment of hemivertebra resection combined with segmental fusion performed well with prognosis for patients with TACS. This could provide valuable information for CS individuals with compound heterozygosity in TBX6.

Utilization of intraoperative neuromonitoring during the Woodward procedure for treatment of Sprengel deformity.

BACKGROUND: Brachial plexus injury (BPI) leading to palsy of the upper extremities is the most serious complication of the Woodward procedure for treatment of Sprengel deformity. Intraoperative neuromonitoring (IONM) is widely used for detecting emerging spinal cord or peripheral nerve injury during spinal and shoulder surgery. However, to date, its utilization in pediatric patients with Sprengel deformity is limited. Furthermore, it remains unclear whether IONM can help prevent BPI during surgery. The purpose of the current study was to assess the feasibility and effectiveness of IONM for early identification and prevention of nerve injury during the Woodward procedure. METHODS: We retrospectively reviewed the records of patients who underwent the Woodward procedure for Sprengel deformity at our institution between January 2017 and January 2020. IONM, including somatosensory evoked potentials (SEP) and motor evoked potentials (MEPs), was performed in all patients. Detailed IONM data were collected and analyzed. Preoperative and postoperative cosmetic appearance (according to the Cavendish classification), shoulder joint abduction function, and radiologic evaluation of the scapula were reviewed. Surgical complications were recorded. RESULTS: Forty-six patients (19 girls, 27 boys) were included (mean age, 5.1 ± 2.1 years). Both SEP and MEP (amplitude of the abductor pollicis) were successfully performed (100%). MEP alerts occurred in 3 patients (6.5%). After scapula position adjustment, signals recovered in 2 patients and remained unchanged in 1 patient-this patient exhibited postoperative motor deficits that resolved completely by 4 months recovery. The SEP amplitudes decreased in all 3 patients but did not reach the warning criteria. Forty patients were classified as grade III and 6 as grade IV in the Cavendish classification, whereas 35 patients were classified as grade II and 11 as grade III in the Rigault scale. The preoperative Cavendish grade was III (III, IV) and the postoperative Cavendish grade was I (I, II) (χ2 = 88.098, P < .001). The preoperative Rigault grade was II (II, III) and the postoperative Rigault grade was I (I, II) (χ2 = 62.133, P < .001). The mean arc of shoulder joint abduction improved from 99° ± 8° to 167° ± 7° (t = -45.871, P < .001) after surgery. Except for temporary motor deficits detected in 1 patient, no other postoperative complications were observed through the time of final follow-up. CONCLUSION: IONM during the Woodward procedure for Sprengel deformity is feasible and effective in detecting intraoperative neurologic changes and may be effective in preventing BPI associated with surgery.

Surgical Treatment of the Intraspinal Rib Head Dislocation in Children With Dystrophic Scoliosis Secondary to Type 1 Neurofibromatosis.

BACKGROUND: The purpose of this study was to explore the surgical treatment of intraspinal rib head dislocation (IRH) in children with dystrophic scoliosis secondary to type 1 neurofibromatosis (NF1-DS). METHODS: From 2006 to 2019, 32 of 128 patients with NF1-DS were found to have IRH and enrolled in this study. There were 19 boys and 13 girls with an average age of 8.8±2.6 years. Patients were divided into 2 groups: group A (n=25) without IRH resection and group B (n=7) with IRH resection. The intraspinal rib proportion (IRP), apical vertebra rotation, apical vertebral translation, main thoracic curve Cobb angle, trunk shift and thoracic kyphosis, lumbar lordosis, and sagittal vertebral axis were measured before and after the operation. Spinal injury was graded based on the American Spinal Injury Association (ASIA) Impairment Scale. RESULTS: The study group had a total of 42 IRH. The mean follow-up duration was 46.1±28.7 months. The preoperative IRP in both groups was similar (35.5±14.3% vs. 31.2±15.3%, P=0.522). The postoperative IRP was lower in group B (18.5±11.2% vs. 0%, P=0.002). The IRP in group A decreased from preoperative (31.2±15.3%) to postoperative (18.5±11.2%) (P<0.05). There was no significant difference in the apical vertebra rotation, apical vertebral translation, main thoracic curve Cobb angle, trunk shift, thoracic kyphosis, lumbar lordosis, and sagittal vertebral axis between the 2 groups before surgery and after surgery. Four patients with nerve injury caused by the IRH had full neurological recovery postoperatively. All patients were ASIA grade E at the last follow-up. CONCLUSIONS: The surgical treatment of IRH in children with NF1-DS should be determined on the basis of the presence of preoperative neurological symptoms. This study supports the practice of correcting spinal deformities only in patients with mild or no spinal cord injury. If there are obvious neurological symptoms, IRH resection is necessary to relieve spinal cord compression to recover nerve function. LEVEL OF EVIDENCE: Level III.