The Prevalence of Scoliosis after Fontan Circulation Surgery Followed-Up to Adolescence.

Introduction: The advancement of surgical techniques and perioperative management for congenital heart disease (CHD) has increased life expectancy. The surgical creation of the Fontan circulation maintains pulmonary blood flow without relying on an effective pump from the abnormal heart, relying on peripheral vascular resistance to maintain effective flow through the lungs. Unfortunately, this delicate mechanism is compromised when scoliosis restricts ventilation, leading to Fontan failure and a poor prognosis for life. This report describes the prevalence of scoliosis with Fontan completion surgery and the role of screening and surgical correction. Methods: Ninety-six consecutive Japanese patients undergoing Fontan completion surgery for CHD between 2000 and 2017 were identified in our institutional records. The inclusion criterion was at least 7 years of follow-up after Fontan completion surgery, while the exclusion criteria were congenital, syndromic, and neuromuscular scoliosis. Radiographic and clinical parameters, including cardio-thoracic ratio (CTR) for cardiomegaly and cyanosis saturation, were compared between with and without scoliosis. Results: There were 23 and 40 patients in the scoliosis and no scoliosis groups, respectively. The mean age at the final follow-up was 18.5 and 16.7 years in the scoliosis and no scoliosis groups, respectively (p=0.02). Mean CTR was 43.7% and 39.4% in the scoliosis and no scoliosis groups (p=0.016), and the mean saturation in room air at the final follow-up was 88.8% and 93.2%, respectively (p=0.036). There were no significant differences to clarify the risk factors with multivariate logistic regression analysis. Conclusions: The prevalence of scoliosis with Fontan completion surgery was 36.5%. Screening for scoliosis is important for children with Fontan circulation surgery as part of their routine follow-up at least until they reach adolescence.Evidence Level: 4.

RASopathies and spinal deformities for screening of scoliosis.

BACKGROUND: The RASopathies (Noonan syndrome [NS] and Costello syndrome [CS]) are rare disorders. Although these have been characterized, precise delineation of the differences in the spinal deformities associated with RASopathy has not been described. This study characterized the spinal deformities found in NS and CS and describes a strategy for the screening of scoliosis. METHODS: The clinical records and spinal X-rays of 35 consecutive NS and CS patients were reviewed. Spinal X-rays were assessed to define the presence and progression of scoliosis. Clinical records were examined to identify the risk factors associated with scoliosis. In addition, we investigated the association between clinical records and scoliosis using logistic regression analysis. RESULTS: Twenty-four patients with NS and 11 with CS were included. Nine patients with NS and five with CS showed scoliosis. The mean ± SD age at diagnosis was 12.6 ± 2.4 years in NS and 11.4 ± 2.5 years in CS (p = 0.55), and mean follow-up period was 4.8 ± 2.6 years and 6.3 ± 2.4 years (p = 0.42), respectively. The coronal angular deformity at final follow-up was 27.3 ± 8.5° in NS and 19.4 ± 6.9° in CS (p = 0.030) with a mean annual progression of 2.8 ± 1.1° in NS 1.0 ± 1.0° in CS (p = 0.030). Cardiac disease was present in eight out of nine patients with NS with concomitant scoliosis in NS, and significantly more than in CS (p = 0.007). PTPN11 significantly correlated with scoliosis (odds ratio 12.4 0.035, 95% confidence interval: 1.20-128.00). CONCLUSIONS: Spinal deformity in NS is more severe than in CS. This study identified a relationship between PTPN11 and scoliosis. Therefore, PTPN11 can be used for the screening of scoliosis.

Utilization of individual components of enhanced recovery after surgery (ERAS) protocol improves post-operative outcomes in adolescent idiopathic scoliosis: a blueprint for progressive adoption of ERAS.

PURPOSE: Enhanced recovery after surgery [ERAS] is an approach for standardization of perioperative care aimed at improving patient outcomes. The primary aim of this study was to determine if length of stay (LOS) differed by protocol type (ERAS vs. non-ERAS [N-ERAS]) in patients undergoing surgery for adolescent idiopathic scoliosis (AIS). METHODS: A retrospective cohort study was conducted. Patient characteristics were collected and compared between groups. Differences in LOS were assessed using regression adjusting for age, sex, BMI, pre-surgical Cobb angle, levels fused and year of surgery. RESULTS: Fifty nine ERAS patients were compared to 81 N-ERAS patients. Patients were comparable in their baseline characteristics. Median LOS was 3 days (IQR = 3-4) for the ERAS group, compared to 5 days (IQR = 4-5) for the N-ERAS group (p < 0.001). The ERAS group had a significantly lower adjusted rate of stay (RR = 0.75; 95% CI = 0.62-0.92). The ERAS group had significantly lower average pain on post-operative days 0 (least-squares-mean [LSM] 2.66 vs. 4.41, p < 0.001), POD1 (LSM 3.12 vs. 4.48, p < 0.001) and POD5 (LSM 2.84 vs. 4.42, p = 0.035). The ERAS group had lower opioid consumption (p < 0.001). LOS was predicted by the number of protocol elements received; those receiving two (RR = 1.54 95% CI = 1.05-2.24), one (RR = 1.49; 95% CI = 1.09-2.03) or none (RR = 1.60, 95% CI = 1.21-2.13) had significantly longer rates of stay than those receiving all four. CONCLUSION: Adoption of modified ERAS-based protocol for patients undergoing PSF for AIS led to significant reduction in LOS, average pain scores, and opioid consumption.

A comparison of three- and two-rod constructs in the correction of severe pediatric scoliosis.

Purpose: Managing severe scoliosis is challenging and risky with a significant complication rate regardless of treatment strategy. In this retrospective comparative study, we report our results using a three-rod compared to two-rod construct in the surgical treatment of severe spine deformities to investigate which technique is safer, and which provides superior radiological outcomes. Methods: Forty-six consecutive patients undergoing posterior spine fusion for scoliosis between 2006 and 2017 were identified in our institutional records. Inclusion criteria were minimum coronal deformity of 90°, age < 18 years at the time of surgery and a minimum 2 years of follow-up. Radiographic and clinical parameters, as well as post-operative complications were compared between the two groups. Results: There were 21 patients in the three-rod group and 25 in the two-rod group. The mean preoperative major coronal deformity was 100°± 9 and 102°± 10 in the three-rod and two-rod, respectively (p = 0.6). The average major curve correction was 51% and 59% in three-rod and two-rod groups, respectively (p = 0.03). The post-operative thoracic kyphosis was 30°± 11 and 21°± 12 in the three-rod and the two-rod groups, respectively (p = 0.01). The surgical time was 476 ± 52 and 387 ± 84 min in three-rod and two-rod, respectively (p < 0.01). One patient in the two-rod cohort showed permanent post-operative sensory deficit. There were three unplanned returns to operating theater in the two-rod group. Conclusions: Coronal correction was better with two-rod, whereas sagittal balance was superior with three-rod. Both techniques achieved balanced spine treating severe scoliosis. The two-rod technique was associated with a higher likelihood of requiring revision surgery. Level of evidence: level 3.

Costello syndrome-associated orthopaedic manifestations focussed on kyphoscoliosis: a case series describing the natural course.

Costello syndrome (CS) is a rare genetic condition caused by a heterozygous mutation in the HRAS gene, with an estimated prevalence of 1: 300 000. Individuals with CS present with characteristic features including scoliosis, kyphosis, Chiari 1 malformation, and syringomyelia. The natural history of the spinal deformity associated with CS has been incompletely described. This case series describes the spinal deformity associated with CS and sets out a strategy for screening and treatment. The clinical records and spinal radiographs of nine consecutive CS patients encountered at a single centre were reviewed. Radiological assessments for the presence and progression of scoliosis were studied. Nine patients with confirmed CS were followed for a mean of 6.6 years. Five patients showed mild scoliosis. Two patients had lumbar kyphosis in addition to their scoliosis, and one showed scoliosis with proximal thoracic kyphosis. Three patients underwent investigation with MRI, one of which showed Chiari I malformation and a syrinx. One showed no change in the severity of their deformity over time. The remaining four patients showed a rate of increasing coronal deformity of 2.1° per year. There were no cases of rapid progression. All cases showed delayed skeletal maturity. The spinal deformity in CS appears to be slowly progressive. To identify those at risk of more rapid progression, brain and spine MRI should be carried out to exclude structural neurological abnormalities. Long follow-up is required for patients with spinal deformity in CS due to the delay in reaching skeletal maturity. Evidence level: 4.

Peroneal Nerve Palsy Caused by Proximal Fibular Solitary Osteochondroma: Case Report and Literature Review.

Osteochondroma is a relatively common benign tumor of the bone, and compressive neuropathies due to osteochondroma are comparatively rare. Here, we present a rare case of osteochondroma of the fibular head that caused peroneal nerve palsy in an 8-year-old girl. Physical examination revealed 0/5 tibialis anterior, 1/5 extensor hallucis longus, and 1/5 peroneal brevis muscle power-according to the manual muscle testing grading system, as well as numbness on the lateral side of the right leg and the back of the foot. Radiological examination and ultrasound revealed a bone tumor in the head of the right fibula. Magnetic resonance imaging ruled out spinal nerve root compression. It was discovered that the bone tumor in the fibular head had compressed and displaced the common peroneal nerve. The patient underwent surgical decompression of the right peroneal nerve. A bone region measuring 22 × 14 × 8 mm was removed. Three months postoperatively, the preoperative neurological deficits were found to be nearly resolved. The patient presented with a foot drop for 1 year, but symptoms resolved 3 months after surgery. Conventional wisdom states that surgery should be performed within 3 months, but we recommend that surgery be performed as soon as diagnosis is made even in cases with a long history, as it may improve patient symptoms and outcomes.

The Association Between Radiographic and MRI Cervical Spine Parameters in Patients With Down Syndrome.

Introduction Many patients with Down syndrome (DS) develop upper cervical spine instability that may lead to spinal cord injury. The purpose of this study was to investigate the association between the spinal cord compression in MRI and the occipto-cervical instability evident on plain radiographs in a Japanese population. Methods A retrospective analysis of cervical spine radiographs and MRI acquired from patients with DS was performed. Radiographic evaluation included measuring the atlanto-dental interval (ADI) and space available for the cord. The basion axial interval (BAI) and Weisel-Rothman (WR) measurements were taken to quantify occipto-axial (OA) and atlanto-occipital (AO) instability. These parameters were collected in patients both with (positive) and without (negative) spinal cord compression evident on MR imaging in a neutral position and the values were compared. In addition, we investigated the association between spinal cord compression and previously defined abnormal values with logistic regression analysis (abnormal values: ADI>6mm, SAC<14mm, BAI<-12mm or >5mm in neutral position). Results There were 17 patients in the positive group and 52 patients in the negative group. WR was 7.4 mm±6.0 in positive group and 8.6 mm±4.8 in negative group (p=0.31) in neutral position, 3.9 mm±5.4 and 6.3±5.0 (p=0.06) in flexion, and 7.0 mm±6.8 and 7.2 mm±4.8 (p=0.75) in extension, respectively. The difference in WR between flexion and extension was 3.1 mm ± 4.6 and 0.9 mm ± 3.8, respectively (p=0.15). All other parameters showed significant differences between the two groups excluding BAI in extension (p<0.05). In addition, abnormal values that significantly correlated with cord compression were ADI (odds ratio 42.3 p<0.01 95% CI 4.16-430.0) and SAC (odds ratio 31.90 p=0.013 95% CI 2.06-494.0). Conclusions These data suggest that OA and AA instability measured with ADI, SAC, and BAI are significantly associated with spinal cord compression in MRI; whereas instability measured with WR and DWR is not. In addition, the previously defined abnormal thresholds for the ADI and SAC can be used for screening the Japanese population.

A comparison of the reliability and vulnerability of 3D sterEOS and 2D EOS when measuring the sagittal spinal alignment of patients with adolescent idiopathic scoliosis.

PURPOSE: An essential component of making the diagnosis of adolescent idiopathic scoliosis (AIS) is standing anteroposterior and lateral radiographs. Two-dimensional (2D) radiographs inevitably fail to reflect every plane of the three-dimensional (3D) deformity in scoliosis. We have tested the hypothesis that there is no difference in the assessment of the sagittal plane deformity when measured with either 2D or 3D EOS radiography. METHODS: A retrospective radiographic analysis was performed on patients diagnosed with AIS, with subdivided into three groups according to the coronal angular deformity (mild group: 45°-69°, moderate group: 70°-89°, and severe group: 90° +). The sagittal parameters were compared between manual measurement with 2D sterEOS and those made using computer-aided 3D reconstruction. RESULTS: Fifty-two patients were included in each group. The inter-study reliability when measuring the thoracic Kyphosis (TK) and lumbar lordosis (LL) between the two study modalities was excellent in mild group (ICC: 0.90, 95% CI 0.82 ~ 0.94 and ICC: 0.84, 95% CI 0.74 ~ 0.91), excellent in TK and fair in LL in moderate group (ICC: 0.76, 95% CI 0.61 ~ 0.85 and ICC: 0.70, 95% CI 0.53 ~ 0.81), and fair in TK and LL in severe group, respectively (ICC: 0.74, 95% CI 0.57 ~ 0.84 and ICC: 0.65, 95% CI 0.46 ~ 0.84). A Bland-Altman plot showed proportional bias in TK measurements in each group and LL in moderate group, which means the measured value is underestimated in 2D method when the angle is small. CONCLUSION: 3D sterEOS is less vulnerable to the influence of coronal plane than 2D EOS in evaluating the sagittal spinal parameters of patients with a coronal deformity exceeding 70°.

Increased proximal vertebral rotation is associated with shoulder imbalance after posterior spinal fusion for severe adolescent idiopathic scoliosis.

PURPOSE: Residual shoulder imbalance is associated with suboptimal outcomes following the surgical correction of adolescent idiopathic scoliosis (AIS) including poor patient satisfaction. In this retrospective study, we evaluate the radiographic parameters and the relationship between the global and local indices of spinal alignment with shoulder balance pre- and postoperatively utilizing EOS imaging and 3D reconstruction. METHODS: A retrospective radiographic analysis was performed on patients with AIS, treated with posterior spinal fusion. Postoperative radiographs were obtained immediately following surgery, at 6 months and final follow-up over 2 years postoperatively. 3D Radiographic measurements included in the coronal plane radiographic shoulder height difference (RSHD), proximal thoracic Cobb angle (PT) and main thoracic Cobb (MT), in the sagittal plane T4-T12 kyphosis, T12-L5 lordosis, in the axial plane proximal thoracic (PT AVR) and main thoracic apical vertebral rotation (MT AVR). RESULTS: Sixty-six patients were included (63 females) with an average main thoracic curvature of 76 degrees. RSHD averaged 14 mm ± 14 preoperatively, -15 mm ± 12 postoperatively, -8.5 mm ± 11 at 6 months, and -8.3 mm ± 8.7 at final follow-up, respectively. Statistical analysis revealed a significant correlation between RSHD and proximal thoracic Cobb angle, between RSHD and proximal thoracic apical vertebral rotation (PTAVR) (r > 0.20, p < 0.05). CONCLUSION: The significant correlation presented in this study suggests that PT Cobb angle and PT AVR are involved in postoperative shoulder imbalance. THE LEVEL OF EVIDENCE: Level 4.

Implant-Related Complications Using Uniaxial Implants In Pediatric Spinal Deformity Surgery.

INTRODUCTION:  The successful surgical treatment of paediatric spinal deformity relies on robust anchors to achieve correction. Uniaxial pedicle screws are designed with articulation between the screw head and screw shaft, thus reducing the risk of anchor failure whilst permitting corrective manoeuvres. The purpose of this study was to describe the incidence, nature, and chronology of implant-related complications in pediatric spinal deformity treated with uniaxial pedicle screws. METHODS: A retrospective radiographic analysis was carried out on paediatric patients treated for spinal deformity with more than two years of follow-up. Each was treated with posterior instrumented spinal fusion (PISF) using a uniaxial pedicle screw system by a single surgeon at a single institution. Surgical records, post-operative radiographs, and follow-up documentation were scrutinised for details of the implants used, implant failure, and revision procedures. RESULTS: Three hundred and eighty-nine eligible patients with a mean follow-up of 3.3 years were identified. The mean anchor density was 1.7. Seven implant complications were observed. Early complications (<12 months) occurred in four cases and late (>12 months) in three cases. None of the early complications were associated with non-union. Two early and two late complications required revision surgery to manage implant failure and non-union. Patients who underwent fusion across the lumbosacral junction showed a higher than expected rate of implant-related complication (P=0.02). CONCLUSION:  This study shows that there is a rate of implant-related failure of 1.8% after PISF with uniaxial implants in pediatric spinal deformities. There is a distinction between early and late implant-related complications, with early failure being due to loss of construct integrity, whereas late failure is due to pseudarthrosis and construct fatigue.

The use of three rods in correcting severe scoliosis.

PURPOSE: The three-rod technique, utilising a short apical concavity rod is an option to achieve controlled correction in severe scoliosis. We describe this technique, the complications encountered, and the long-term outcomes. METHOD: All paediatric patients who had at least 2 years follow-up after undergoing corrective surgery for scoliosis ≥ 100° using 3 parallel rods were included. Radiographs were assessed to evaluate the correction and clinical records examined for any loss of correction, complications, revision procedures or neuromonitoring events. RESULTS: Twenty-five patients met the inclusion criteria. Four underwent prior anterior fusion to prevent crankshaft phenomenon. The mean angle of the deformity was 112.0° (range 100.3-137.1). Mean maximal kyphosis was 48.8° (range 11.4-78.8°) and mean curve flexibility 4.4% (range 0-37.0%). Intraoperative traction achieved an average of 70.4% (95% CI 56.6-84.1%). Nine patients (39%) showed a reduction in MEPs during definitive surgery. All returned to within 75% of baseline by the end of surgery. All patients had normal postoperative neurology. One patient underwent removal of hardware for late infection. The mean overall Cobb correction was 55.7° (95% CI 50.2-61.2°), equating to 50.2% (95% CI 44.9-55.4%) of the mean initial deformity. Thoracic kyphosis reduced by a mean of 18.2° (95% CI 12.8-23.6°). CONCLUSION: Our series suggests that three-rod constructs are able to safely and effectively achieve 50% correction of severe scoliosis.

The Association of Scoliosis and NSD1 Gene Deletion in Sotos Syndrome Patients.

STUDY DESIGN: A retrospective comparative study. OBJECTIVE: The aim of this study was to examine the NSD1 abnormalities in patients diagnosed with Sotos syndrome and its correlation with the presence, severity, and progression of associated scoliosis. SUMMARY OF BACKGROUND DATA: Scoliosis has been reported in approximately 30% of patients diagnosed with Sotos syndrome, a genetic disorder characterized by a distinctive facial appearance, learning disability, and overgrowth. Sotos syndrome is mainly attributed to NSD1 haploinsufficiency, but with ethnical differences in genetic profile: NSD1 microdeletions are frequently identified in Japanese Sotos patients whereas intragenic mutations are more frequently found in non-Japanese patients. Although possible genotype-phenotype correlations have been proposed, the genotype of Sotos syndrome patients suffering from scoliosis has not been examined. METHODS: The medical records and spinal radiographs of 63 consecutive Sotos syndrome patients at a single center were reviewed. Fluorescent in situ hybridization or microarray comparative genomic hybridization and DNA sequencing or multiplex ligation-dependent probe amplification were performed to detect 5q35 microdeletion involving the NSD1 gene and intragenic mutations of the NSD1 gene, respectively. The phenotypes of all cases and radiological assessments for the presence and progression of scoliosis were studied. RESULTS: NSD1 abnormalities were identified in 55 patients (87%): microdeletion in 34 patients (54%) and intragenic mutation in 22 patients (33%). Scoliosis was observed in 26 patients (41%), with a significantly higher ratio of microdeletions than mutations. The 10 patients with progressive scoliosis all had NSD1 microdeletions. CONCLUSION: Scoliosis was a common phenotypical trait in children with Sotos syndrome and its presence as well as progression were higher in cases with NSD1 microdeletions. Although all Sotos syndrome patients should be monitored for scoliosis, clinicians should be made aware that patients with NSD1 microdeletions have a higher probability of scoliosis development and progression that may require early intervention.Level of Evidence: 3.

Reoperation rate and risk factors of elective spinal surgery for degenerative spondylolisthesis: minimum 5-year follow-up.

BACKGROUND CONTEXT: The favorable outcome of surgical treatment for degenerative lumbar spondylolisthesis (DS) is widely recognized, but some patients require reoperation because of complications, such as pseudoarthrosis, persistent pain, infection, and progressive degenerative changes. Among these changes, adjacent segmental disease (ASD) and same segmental disease (SSD) are common reasons for reoperation. However, the relative risks of the various factors and their interactions are unclear. PURPOSE: The purpose of this study was to determine the longitudinal reoperation rate after surgery for DS and to assess the incidence and independent risk factors for ASD and SSD. STUDY DESIGN: This study is a retrospective consecutive case series of patients with DS who were surgically treated. PATIENT SAMPLE: We assessed 163 consecutive patients who were surgically treated for DS between 2003 and 2008. Individual patients were followed for at least 5 years after the initial surgery. OUTCOME MEASURES: The primary end point was any type of second lumbar surgery. Radiographic measurements and demographic data were reviewed. We compared patients who underwent reoperation with those who did not. Logistic regression analysis was used to determine the relative risk of ASD and SSD in patients surgically treated for DS. METHODS: Radiographic measurements and demographic data were reviewed. We identified the incidence and risk factors for reoperation, and we performed univariate and multivariate analyses to determine the independent risk factors for revision surgery for SSD and for ASD as the two distinct reasons for the reoperation. Age, gender, etiology, body mass index (BMI), and other radiographic data were analyzed to determine the risk factors for developing SSD and ASD. RESULTS: The average patient age was 65.8 (50-81 years; 73 women and 90 men; mean follow-up, 5.9±1.6 years). Eighty-nine patients had posterior lumbar interbody fusion and 74 had laminotomies. Twenty-two patients had L3-L4 involvement and 141 had L4-L5 involvement. The cumulative reoperation rate was 6.1% at 1 year, 8.5% at 2 years, 15.2% at 3 years, 17.7% at 5 years, and 23.3% (38/163 patients) at the final follow-up. A significantly higher reoperation rate was observed for patients undergoing laminotomy than for patients undergoing posterior lumbar interbody fusion (33.8% vs. 14.4%, p=.01). Eighteen patients (11.0%) had SSD, and 13 patients (8.9%) developed ASD. Higher BMI (obesity) and greater disc height (greater than 10 mm) predicted the occurrence of SSD in the multivariate model (BMI=odds ratio 4.11 [95% confidence interval 1.29-13.11], p=.016; disc height=3.18 [1.03-9.82], p=.044), and gender (male) and facet degeneration (Fujiwara grade greater than 3) predicted the development of ASD in the multivariate model (gender=4.74 [1.09-20.45], p=.037; facet degeneration=6.31 [1.09-36.52], p=.039). CONCLUSIONS: The incidence of reoperation in patients surgically treated for DS was 23.2% at a mean time of 5.9 years. A significantly higher incidence of reoperation was observed in patients treated with decompression alone compared with those treated with decompression and fusion. Body mass index and disc height were identified as independent risk factors for SSD, whereas male gender and facet degeneration were identified as independent risk factors for ASD. The results of this comprehensive review will guide spine surgeons in their preoperative planning and in the surgical management of patients with DS, thereby reducing the reoperation rate.