Impact of Spinal Surgery on Intrathecal Nusinersen Injections in Pediatric Spinal Muscular Atrophy.

BACKGROUND: Complex spinal deformities necessitate surgical interventions that may intervene with intrathecal injections in patients with spinal muscular atrophy (SMA). This study aimed to determine the effect of spinal deformity correction surgery on nusinersen administration. METHODS: Pediatric patients with SMA, operated by a single surgeon, either via magnetically controlled growing rod (MCGR) or definitive fusion (DF) with skip instrumentation, were evaluated retrospectively in terms of safety and feasibility of intrathecal injections. Patients' and their parents' perspectives were evaluated through a questionnaire regarding any shift in the setting of injections. RESULTS: Fourteen patients with 15 spinal surgeries (10 MCGR and 5 DF) were included. Eleven patients received intrathecal treatment both before and after the surgery. Preoperative (n=3) and postoperative (n=9) fluoroscopic guidance was required leading to a shift in the application settings in 6 patients. Of 106 preoperative injections, 15% required fluoroscopy and 2% required anesthesia. Postoperatively, of 88 injections, 73% required fluoroscopy and 26% required anesthesia. No patients discontinued intrathecal injections due to technical difficulties associated with the spinal surgery. CONCLUSIONS: This study demonstrates that spinal surgery does not prevent safe and successful intrathecal nusinersen injections. In the DF group, the skip instrumentation technique provided access to interlaminal space for intrathecal injections. In either surgical group, no further auxillary approach was required. Modifications in the injection technique require an institutional approach, and concerns of patients and their families should be addressed accordingly. LEVEL OF EVIDENCE: IV.

A rare skeletal dysplasia in the etiology of severe scoliosis: Diaphanospondylodysostosis.

Diaphanospondylodysostosis is a rare genetic skeletal disorder caused by biallelic variants in the BMPER gene. The term, diaphanospondylodysostosis, includes ischiospinal dysotosis, which was previously known as a distinct entity with milder clinical features. The clinical phenotype of diaphanospondylodysostosis is quite variable with mortality in early postnatal life in some patients. Main clinical and radiographic features are narrow thorax, vertebral segmentation defects, rib anomalies, ossification defects of vertebrae, ischium and sacrum, and renal cysts. In this study, we report on a 14-year-old girl patient with diaphanospondylodysostosis harbouring a novel BMPER mutation. The patient presented with severe scoliosis and severely hypoplastic/aplastic distal phalanges of the fingers and toes, findings yet hitherto not described in this syndrome.

Transit dosimetry of stereotactic body radiotherapy treatments with electronic portal dosimetry device in patient with spinal implant.

In recent years, the use of the Electronic Portal Imaging Device (EPID) as an in vivo dosimeter has become widespread. However, reports of EPID for stereotactic body radiotherapy (SBRT) applications is scarce. There is no data on this topic especially when there are high-density materials in the radiation field. In this study, we aimed to investigate the dose distributions of SBRT treatment plans in patients with spinal implants by transit EPID dosimetry. Implants were inserted in phantoms that mimic the vertebrae, and VMAT plans were created on the phantoms to deliver 16 Gy radiation doses to the target in 1 fraction. Transit EPID measurements were performed for each irradiation. The results were compared with the treatment planning system using the gamma analysis method. According to the gamma analysis results, while the non-implant model met the acceptance criteria with a rate of 95.4%, the implanted models did not pass the test with results between the rates of 70% to 73%. In addition, while the dose difference in the isocenter was 1.3% for the non-implanted model, this difference was observed to be between 7 and 8% in the implanted models. Our study revealed that EPID can be used as transit dosimetry for the VMAT-SBRT applications. However, unacceptable dose differences were obtained by transit EPID dosimetry in the VMAT-SBRT applications of patients with an implant. In the treatment of such patients, alternative treatment methods should be preferred in which the interaction of the implants with radiation can be prevented.

Posterior Instrumentation and Fusion in Multiple-segment Adolescent Congenital Scoliosis: When Are Posterior Column and Concave Rib Osteotomies Needed?

BACKGROUND: No clear guidelines exist for appropriate surgical treatment of congenital scoliosis. The spectrum varies from isolated posterior instrumentation and fusion (PIF-only) to vertebral column resections. Multiple posterior column osteotomies (PCOs) with and without concave rib osteotomies have previously been suggested as an alternative to invasive 3-column osteotomies. The aim of the study is to analyze outcomes of spinal surgery in congenital scoliosis patients for a new treatment algorithm based on a traction radiograph under general anesthesia (TruGA). METHODS: Surgical intervention was determined according to flexibility with TruGA: patients with >40% correction underwent PIF-only; patients with <40% of correction underwent PCO. Patients, who had <30% of correction in TruGA and/or more than 5 vertebral levels with failure of segmentation, underwent additional concave rib osteotomies. The radiologic and clinical results were compared. RESULTS: Forty-three patients met inclusion criteria (14M). The mean age was 13.8 (10 to 17) years, average follow-up 67 (24 to 107) months. Fourteen patients were in the PIF-only group, 29 in the PCO group. PCO patients were significantly older (14.5 vs. 12.3, P=0.001). Mean operative time and blood loss of PCO group were significantly greater than those of PIF-only group. Nine patients required concave rib osteotomies. While the curve magnitude of patients in the PIF-only and PCO groups did not differ significantly (54.6 vs. 63 degrees, P=0.067), curve rigidity was significantly higher in the patients of PCO group (51.2% vs. 32%, P=0.001). Despite this, postoperative correction rates for 2 groups were similar (51.1% vs. 47.8%, P=0.545). CONCLUSIONS: The number of anomalous segments and the curve flexibility on TruGA play important roles in the decision-making process in congenital scoliosis and <40% flexibility of the major curve could be an important indicator of the need for PCO. Curves with more than 5 anomalous vertebral segments might be more likely to need additional concave rib osteotomies for adequate correction. LEVEL OF EVIDENCE: Level III.

Coupling of cell fate selection model enhances DNA damage response and may underlie BE phenomenon.

Double-strand break-induced (DSB) cells send signal that induces DSBs in neighbour cells, resulting in the interaction among cells sharing the same medium. Since p53 network gives oscillatory response to DSBs, such interaction among cells could be modelled as an excitatory coupling of p53 network oscillators. This study proposes a plausible coupling model of three-mode two-dimensional oscillators, which models the p53-mediated cell fate selection in globally coupled DSB-induced cells. The coupled model consists of ATM and Wip1 proteins as variables. The coupling mechanism is realised through ATM variable via a mean-field modelling the bystander signal in the intercellular medium. Investigation of the model reveals that the coupling generates more sensitive DNA damage response by affecting cell fate selection. Additionally, the authors search for the cause-effect relationship between coupled p53 network oscillators and bystander effect (BE) endpoints. For this, they search for the possible values of uncertain parameters that may replicate BE experiments' results. At certain parametric regions, there is a correlation between the outcomes of cell fate and endpoints of BE, suggesting that the intercellular coupling of p53 network may manifest itself as the form of observed BEs.

Does Decreased Surgical Stress Really Improve the Psychosocial Health of Early-onset Scoliosis Patients?: A Comparison of Traditional Growing Rods and Magnetically-controlled Growing Rods Patients Reveals Disappointing Results.

STUDY DESIGN: Cross-sectional case-control study. OBJECTIVE: Compare psychosocial profile of magnetically-controlled growing rod (MCGR) patients to traditional-growing rod (TGR) with an array of psychiatric tools, expecting improvement in MCGR due to decreased number of surgical procedures. SUMMARY OF BACKGROUND DATA: TGR treatment has had positive clinical and radiographic results; however, upward of 10 surgical sessions and high complication rates have called into question the quality of life of these children. Improvement with the introduction of the MCGR is expected. METHODS: GR patients with minimum of 2-years follow-up were recruited. None had neurological conditions. All underwent testing with the Wechsler Intelligence Scale for Children-Revised, and only those in the normal range were included. Patients filled out questionnaires with mental health professionals to measure psychosocial status. MCGR patients' results were compared to TGR patients. RESULTS: Twenty-seven patients met criteria (10 MCGR, 17 TGR): average age at enrollment 11.8 years (range 5.9-17). MCGR group was significantly younger (9.1 vs. 13.3 yr) and had significantly shorter follow-up (45.6 vs. 82.8 mo) (P < 0.05). TGR patients underwent an average of 16 surgical procedures, MCGR an average of 1.5 (including complications, P < 0.05). Age at index surgery (6 yr), preoperative and postoperative major curve magnitudes (60°, 40° respectively) were statistically similar.There was no difference in current psychiatric diagnoses between the groups. MCGR patients scored worse than TGR patients in general functionality domains. TGR patients showed increased functionality and prosocial scores with increased number of procedures. This effect was not observed in MCGR. CONCLUSIONS: The expected improvement in psychosocial status with the MCGR was not observed at a 31.6-month-follow-up. It appears that provided the patient spends enough time in the treatment process to notice benefit and does not experience major complications, noninvasiveness of lengthening procedures does not show up as an advantage with the psychosocial tools utilized in this study. LEVEL OF EVIDENCE: 3.

Revealing determinants of two-phase dynamics of P53 network under gamma irradiation based on a reduced 2D relaxation oscillator model.

This study proposes a two-dimensional (2D) oscillator model of p53 network, which is derived via reducing the multidimensional two-phase dynamics model into a model of ataxia telangiectasia mutated (ATM) and Wip1 variables, and studies the impact of p53-regulators on cell fate decision. First, the authors identify a 6D core oscillator module, then reduce this module into a 2D oscillator model while preserving the qualitative behaviours. The introduced 2D model is shown to be an excitable relaxation oscillator. This oscillator provides a mechanism that leads diverse modes underpinning cell fate, each corresponding to a cell state. To investigate the effects of p53 inhibitors and the intrinsic time delay of Wip1 on the characteristics of oscillations, they introduce also a delay differential equation version of the 2D oscillator. They observe that the suppression of p53 inhibitors decreases the amplitudes of p53 oscillation, though the suppression increases the sustained level of p53. They identify Wip1 and P53DINP1 as possible targets for cancer therapies considering their impact on the oscillator, supported by biological findings. They model some mutations as critical changes of the phase space characteristics. Possible cancer therapeutic strategies are then proposed for preventing these mutations' effects using the phase space approach.

Health-Related Quality of Life in Early-Onset Scoliosis Patients Treated Surgically: EOSQ Scores in Traditional Growing Rod Versus Magnetically Controlled Growing Rods.

STUDY DESIGN: Cross-sectional study. OBJECTIVE: To compare quality of life and caregiver burden in traditional growing rod (TGR) and magnetic controlled growing rods (MCGR) patients. SUMMARY OF BACKGROUND DATA: MCGR decrease surgical sessions associated with treatment of early onset scoliosis (EOS), hoping to minimize the burdens seen with repetitive invasive surgeries in TGR treatment. Although the clinical indications for these treatments have largely been agreed upon, there is a lack of understanding of their impact on patients' and families' quality of life. METHODS: Inclusion criteria: ≤10 years of age at index procedure, major curve ≥30°, no previous spine surgery, minimum 1-year postoperative follow-up. The previously validated 24-item early onset scoliosis questionnaire (EOSQ-24) was utilized to assess quality of life. Statistic methods were applied to compare domain scores between TGR and MCGR patients. RESULTS: Forty-four children with EOS were enrolled; 25 TGR and 19 MCGR. Groups were similar in sex and age at index surgery. Age at time of questionnaire and mean length of follow-up were significantly different; patients were older (14.0 vs. 8.8 yr) and had longer follow-up (101.3 vs. 34.3 mo) in TGR (P < 0.01). Deformity correction and complication rates were similar between groups. At the time of questionnaire, scores of economic burden and overall satisfaction in MCGR were significantly superior to those in TGR by univariate analysis. When controlled for duration of follow-up, some domain scores trended towards statistical significance, some remained stable, and others regressed to non-significance. CONCLUSION: Health related quality of life data reveal superior outcomes in overall satisfaction and financial burden domains in the MCGR group. However, the positive effects of MCGR decrease when controlled for length of follow up, indicating that the MCGR is not yet a magic fix-all, and that the TGR remains an option in the treatment of EOS. LEVEL OF EVIDENCE: 3.

Congenital unilateral absence of the upper extremity may give rise to a specific kind of thoracolumbar curve.

There is an increased incidence of scoliosis in patients with congenital malformations of the upper extremity even in the absence of overt vertebral abnormalities. In this case series, we summarize the curve characteristics of four patients presenting to two spine surgery clinics with unilateral amelia or phocomelia and a progressive scoliotic curve with the apex on the side of deficiency. All patients required orthopedic intervention for their curves. Amelia and phocomelia are severe congenital malformations of the upper limb affecting trunk balance and, conceivably, causing scoliosis with the absence of counterbalancing weight on the affected side. The combination of upper limb absence and same-sided scoliosis in these patients may provide a clue of the mechanical factors causing scoliosis in other disorders. In this article, we attempt to define this exceptional deformity, theorize on its etiology, and draw attention to this particular combination of problems. LEVEL OF EVIDENCE: Case series; Level IV.

Psychological Profile of Children Who Require Repetitive Surgical Procedures for Early Onset Scoliosis: Is a Poorer Quality of Life the Cost of a Straighter Spine?

STUDY DESIGN: Cross-sectional study. OBJECTIVE: Assess the psychosocial status of children with early-onset scoliosis (EOS) undergoing multiple procedures and evaluate associations with other variables. SUMMARY OF BACKGROUND DATA: EOS may require repetitive surgical procedures to control deformity and preserve growth. These procedures impact patients' psychosocial status because of the repetitive surgeries. METHODS: EOS patients 6-18 years, undergoing traditional growing rod treatment with more than 5 surgical procedures, and neurologically/mentally intact were included. Patients were screened for psychiatric disorders before inclusion. The Quality of Life Scale for Children (PedsQL), Strengths and Difficulties Questionnaire (SDQ) self-report form, Beck Depression Inventory, Children Depression Inventory (CDI), Beck Anxiety Inventory (BAI), and the Self-Report for Childhood Anxiety Related Disorders (SCARED) were completed by the children. PedsQL Parental Form and SDQ Parent Form were completed by their parents. RESULTS: Twenty-one patients (9 male, 12 female) met the inclusion criteria. Average age was 6.4 years (4-10.5) at index surgery, and 13.5 years (8-17) at final follow-up. The mean number of procedures was 13 (6-18). Mean follow-up was 83.9 months (36-122). Depression was observed in 23.8% of patients, and generalized anxiety disorder in 42.8%. Patients in the study group were more likely than the general population to have a psychiatric diagnosis. Number of procedures undergone was found to correlate negatively with BAI, SCARED, and the behavioral difficulties domain of SDQ parent form score and positively with emotional functioning, psychosocial health summary score, PedsQL total score, and increased social and physical functioning. Nonidiopathic etiology was found to be related to increased behavioral difficulties and lower functioning. CONCLUSION: A higher prevalence of depressive and anxiety symptoms was observed in patients with EOS along with dysfunctional areas of daily life. Other comorbidities may also contribute to dysfunction and difficulties. Determination of the aspects of EOS treatment that have a negative impact on psychosocial functioning may allow for more competent help for these patients.

Anterior and Posterior Vertebral Column Resection Versus Posterior-only Technique: A Comparison of Clinical Outcomes and Complications in Congenital Kyphoscoliosis.

STUDY DESIGN: Retrospective cohort. OBJECTIVE: The purpose of this study is to compare the anterior-posterior surgery to posterior-only vertebra resection for congenital kyphoscoliosis in pediatric patients. SUMMARY OF BACKGROUND DATA: Vertebral column resection is a very powerful correction technique mainly used in the treatment of severe and rigid spinal deformities. The technique can be applied with combined anterior and posterior vertebral column resection (APVCR) or posterior-only vertebral column resection (PVCR) approaches. PVCR has gained popularity recently due to several apparent advantages, but APVCR is still believed by some to be a viable alternative, despite the use of an anterior exposure. MATERIALS AND METHODS: A retrospective chart and radiographic review was performed including the patients with congenital kyphoscoliosis up to 16 years of age who were operated on by a single senior surgeon in our department after 2005. The data included surgical time, estimated blood loss, duration of hospital and postoperative intensive care unit stay, intraoperative and postoperative complications. Preoperative, postoperative, and follow-up radiographs were evaluated for scoliosis, kyphosis, and spinal balance. RESULTS: Twenty-six patients (20 girls, 6 boys) met the inclusion criteria. APVCR was performed on 17 (median age, 13.2 y) and PVCR on 9 (median age, 10.7 y). Scoliosis correction at the time of last follow-up was 54.3% and 52.6% for APVCR and PVCR, respectively, whereas the average kyphosis correction was 25.4 degrees in APVCR and 30.1 degrees in PVCR group. Surgical time, hospital stay, and estimated blood loss were all significantly higher in the APVCR group, whereas the complication rates were similar. No neurological or vascular complications were encountered. CONCLUSIONS: This study showed that although the operative time and surgical blood loss were higher with APVCR, there were no major complications and the radiologic outcomes were similar between APVCR and PVCR. APVCR should be considered as an acceptable technique especially in deformities where PVCR would be technically difficult or at the beginning of the spine surgeon's learning curve. LEVEL OF EVIDENCE: Level III.

Unplanned Reoperations in Magnetically Controlled Growing Rod Surgery for Early Onset Scoliosis With a Minimum of Two-Year Follow-Up.

STUDY DESIGN: A retrospective review of prospectively collected clinical and radiologic data of patients with magnetically controlled growing rods (MCGRs) from a multi-centered study with a minimum of 2-year follow-up. OBJECTIVE: The aim of this study was to describe the incidence and causes of unplanned reoperations and to report the outcomes of patients treated with MCGR for early-onset scoliosis (EOS). SUMMARY OF BACKGROUND DATA: Published clinical studies have demonstrated that MCGR is safe and effective for curvature control of EOS, and can avoid repeated surgeries for distractions. However, there have been no reports on the unplanned reoperations and complications of MCGR for EOS with a large series of patients. METHODS: Between 2009 and 2012, 30 patients with EOS underwent MCGR implantation in six institutions. A retrospective review of prospectively collected clinical and radiologic data with a minimum of 2-year follow-up was conducted. Demographic data, radiologic measurements, unplanned reoperations, and other complications were noted. Risk factors for unplanned reoperations were analyzed. RESULTS: Patients underwent MCGR implantation at the mean age of 7.2 years. The mean follow-up period was 37 months. Fourteen patients (46.7%) underwent an unplanned reoperation within the follow-up period, with a mean time to reoperation of 23 months after initial surgery (range, 5-48 months). Causes of unplanned reoperation were failure of rod distractions, proximal foundation failure, rod breakage, and infection. More frequent distractions (between 1 week and 2 months) were associated with a higher rate of reoperation than distraction frequencies between 3 and 6 months (71% vs. 25%). CONCLUSION: This is the largest series with the longest follow-up to date that examines the need for additional unplanned surgery after the initial procedure. It highlights that MCGR surgery can be associated with unplanned reoperations, and more frequent distractions may be a risk factor. Long-term comparative studies with traditional growing rod are required to evaluate the effectiveness of this implant. LEVEL OF EVIDENCE: 4.

Implant Complications After Magnetically Controlled Growing Rods for Early Onset Scoliosis: A Multicenter Retrospective Review.

BACKGROUND: Traditional growing rods have a reported wound and implant complication rate as high as 58%. It is unclear whether the use of magnetically controlled growing rods (MCGR) will affect this rate. This study was performed to characterize surgical complications following MCGR in early onset scoliosis. METHODS: A multicenter retrospective review of MCGR cases was performed. Inclusion criteria were: (1) diagnosis of early onset scoliosis of any etiology; (2) 10 years and younger at time of index surgery; (3) preoperative major curve size >30 degrees; (4) preoperative thoracic spine height <22 cm. Complications were categorized as wound related and instrumentation related. Complications were also classified as early (<6 mo from index surgery) versus late (>6 mo). Distraction technique and interval of distraction was surgeon preference without standardization across sites. RESULTS: Fifty-four MCGR patients met inclusion criteria. There were 30 primary and 24 conversion procedures. Mean age at initial surgery was 7.3 years (range, 2.4 to 11 y), and mean duration of follow-up 19.4 months. Twenty-one (38.8%) of 54 patients had at least 1 complication. Fifteen (27.8%) had at least 1 revision surgery. Six (11.1%) had broken rods (2 to 4.5 and 4 to 5.5 mm rods); two 5.5 mm rods failed early (4 mo) and 4 late (mean=14.5 mo). Six (11.1%) patients experienced 1 episode of lack or loss of lengthening, of which 4 patients subsequently lengthened. Seven patients (13.0%) had either proximal or distal fixation-related complication at average of 8.4 months. Two patients (3.7%) had infections requiring incision and drainage; 1 early (2 wk) with wound drainage and 1 late (8 mo). The late case required removal of one of the dual rods. CONCLUSIONS: This study shows that compared with traditional growing rods, MCGR has a lower infection rate (3.7% vs. 11.1%). MCGR does not appear to prevent common implant-related complications such as rod or foundation failure. The long-term implication remains to be determined. LEVEL OF EVIDENCE: Level IV.

The dosimetric impact of implants on the spinal cord dose during stereotactic body radiotherapy.

BACKGROUND: The effects of spinal implants on dose distribution have been studied for conformal treatment plans. However, the dosimetric impact of spinal implants in stereotactic body radiotherapy (SBRT) treatments has not been studied in spatial orientation. In this study we evaluated the effect of spinal implants placed in sawbone vertebra models implanted as in vivo instrumentations. METHODS: Four different spinal implant reconstruction techniques were performed using the standard sawbone lumbar vertebrae model; 1. L2-L4 posterior instrumentation without anterior column reconstruction (PI); 2. L2-L4 anterior instrumentation, L3 corpectomy, and anterior column reconstruction with a titanium cage (AIAC); 3. L2-L4 posterior instrumentation, L3 corpectomy, and anterior column reconstruction with a titanium cage (PIAC); 4. L2-L4 anterior instrumentation, L3 corpectomy, and anterior column reconstruction with chest tubes filled with bone cement (AIABc). The target was defined as the spinous process and lamina of the lumbar (L) 3 vertebra. A thermoluminescent dosimeter (TLD, LiF:Mg,Ti) was located on the measurement point anterior to the spinal cord. The prescription dose was 8 Gy and the treatment was administered in a single fraction using a CyberKnife® (Accuray Inc., Sunnyvale, CA, USA). We performed two different treatment plans. In Plan A beam interaction with the rod was not limited. In plan B the rod was considered a structure of avoidance, and interaction between the rod and beam was prevented. TLD measurements were compared with the point dose calculated by the treatment planning system (TPS). RESULTS AND DISCUSSION: In plan A, the difference between TLD measurement and the dose calculated by the TPS was 1.7 %, 2.8 %, and 2.7 % for the sawbone with no implant, PI, and PIAC models, respectively. For the AIAC model the TLD dose was 13.8 % higher than the TPS dose; the difference was 18.6 % for the AIABc model. In plan B for the AIAC and AIABc models, TLD measurement was 2.5 % and 0.9 % higher than the dose calculated by the TPS, respectively. CONCLUSIONS: Spinal implants may be present in the treatment field in patients scheduled to undergo SBRT. For the types of implants studied herein anterior rod instrumentation resulted in an increase in the spinal cord dose, whereas use of a titanium cage had a minimal effect on dose distribution. While planning SBRT in patients with spinal reconstructions, avoidance of the rod and preventing interaction between the rod and beam might be the optimal solution for preventing unexpectedly high spinal cord doses.

Adult Spinal Deformity Correction with Multi-level Anterior Column Releases: Description of a New Surgical Technique and Literature Review.

STUDY DESIGN: Case series. OBJECTIVE: To evaluate radiographic and clinical outcomes of adults with spinal deformity treated with multilevel anterior column releases (ACR). SUMMARY OF BACKGROUND DATA: Pedicle subtraction osteotomy can be used effectively to correct spinal deformity; however, it is not without complications. ACR is an attractive alternative minimally invasive technique for spinal deformity correction, although few clinical reports on its clinical effectiveness exist. METHODS: Adults with spinal deformity who underwent multilevel ACRs (≥2) followed by open posterior instrumentation with a minimum 1-year follow-up were retrospectively reviewed. Deformity radiographic data and clinical outcomes, including the Oswestry Disability Index (ODI) and the EuroQol-5D were analyzed. RESULTS: Eight patients [7 female, 1 male; mean age 65 y (49-79 y)] met inclusion criteria. The mean follow-up was 18.4 months (12-28 mo). The average number of levels treated with an ACR per patient was 2.4 (2-3). There were no anterior approach-related complications. The average number of levels instrumented posteriorly was 8.1 (3-15). Six patients underwent Schwab type 1 posterior osteotomies (partial facetectomies). After the first anterior stage, there was a significant increase in the lumbar lordosis and significant decreases in the sagittal vertical axis, pelvic tilt, and lumbopelvic mismatch (P<0.05). After the second stage there was no significant change in the sagittal vertical axis, lumbar lordosis, pelvic tilt, or lumbopelvic mismatch relative to the values obtained after ACR. There was significantly less disability postoperatively [ODI: 15 (0-30)] compared with preoperatively [ODI: 46 (16-80)] (P<0.01). There was significant improvement in general health after operation, as assessed by the EuroQol-5D utility scores [preop: 0.44 (0.21-0.82) vs. postop: 0.71 (0.60-0.80)] (P=0.01). Back and leg visual analog scale pain scores improved significantly postoperatively. CONCLUSIONS: A staged approach using multilevel ACRs with open posterior instrumentation has an acceptable complication profile and provides excellent restoration of sagittal and coronal balance and pelvic parameters in adults with spinal deformity.

Magnetically controlled Growing Rods for Early-onset Scoliosis: A Multicenter Study of 23 Cases With Minimum 2 years Follow-up.

STUDY DESIGN: Retrospective study. OBJECTIVE: To report 2-year clinical and radiographic results of patients treated with magnetically controlled growing rods (MCGR). SUMMARY OF BACKGROUND DATA: MCGR for early-onset scoliosis has been reported to provide adequate spinal growth and curve correction by eliminating surgical lengthening procedures. This study was designed to report the results of MCGR patients with 2-year follow-up. METHODS: A retrospective study of MCGR patients with the following inclusion criteria: (i) major curve size ≥30°, (ii) T1-T12 height <22 cm, (iii) <11-years old; all at the time of index surgery was performed. Of 54 patients enrolled, 23 had 2-year follow-up. Both primary and conversion patients were evaluated at baseline 6, 12, and 24 months. RESULTS: Mean preoperative age in the primary group was 6.6 ±â€Š2.6 years versus 8.3 ±â€Š2.2 years for the conversion group. A total of 41 adverse events occurred in 11 patients, of which 14 events were implant related. Major coronal curve magnitude improved from 61.3° to 34.3° from baseline to postoperation in primary cases and from 49.4° to 43.8° in conversion cases. Curve correction was maintained for 2 years in both groups. T1-S1 height improved from 252.7 to 288.9 mm in primary cases and was maintained for 2 years. However, conversion cases had some decline in T1- S1 height (270.3 at baseline to 294.4 mm post-MCGR and 290.2 mm at 2-year follow-up; mean loss of 4.2 mm (1.5%) from postoperation to 2 years, P > 0.05). CONCLUSION: This study showed satisfactory curve correction and growth is achieved among primary cases. T1-S1 height in conversion cases had a slight decline in 2 years. However, this decline was not statistically significant. LEVEL OF EVIDENCE: 3.

Convex Instrumented Hemiepiphysiodesis With Concave Distraction: A Treatment Option for Long Sweeping Congenital Curves.

BACKGROUND: Convex hemiepiphysiodesis has been reported to have mixed results in the treatment of congenital spine deformities. Multiple modifications of the original technique were suggested to improve the results. The purpose of this study is to report the results and complications of an instrumented convex growth arrest procedure modified with concave distraction. METHODS: The records of 11 patients with long sweeping congenital curves (involving multiple anomalous vertebrae) who underwent convex instrumented hemiepiphysiodesis with concave distraction were evaluated. Mean age at index operation was 58 months (range, 29 to 101 mo). Lengthening of the concave distraction rod was done every 6 months. The magnitude of coronal/sagittal deformity and T1-T12 height were measured on the preoperative, postoperative, and latest follow-up radiographs. Average follow-up was 44.9 months (range, 24 to 89 mo). RESULTS: In the coronal plane, the convex hemiepiphysiodesis segment was corrected from an average of 60.5 to 40.4 degrees postoperatively and further improved to 35.5 degrees at the latest follow-up. The distracted segment was corrected from 33.4 to 15.2 degrees postoperatively and to 12.7 degrees at the latest follow-up. Sagittal plane alignment was minimally affected. The average T1-T12 height was 157.1 mm in the early postoperative period and 181.1 mm at last follow-up. During follow-up, we identified partial pull-out of screws on the distraction side in 5 of the 11 patients and rod breakages in 3 patients. These were revised during planned lengthenings. There were no unplanned surgeries, deep wound infections, nor neurological complications. CONCLUSIONS: Convex instrumented hemiepiphysiodesis with concave distraction resulted in good curve correction while maintaining the growth of thorax. The correction of the anomalous segment improved over time, proving the effectiveness of the hemiepiphysiodesis. Addition of a concave distraction construct appears to enhance spinal growth, thereby augmenting the hemiepiphysiodesis effect.

Utilizing the "Stable-to-be Vertebra" Saves Motion Segments in Growing Rods Treatment for Early-Onset Scoliosis.

BACKGROUND: There is no consensus on the selection of distal instrumentation levels in growing rod surgery. Many surgeons utilize the stable zone of Harrington, but there is not overwhelming evidence to support this preference. The aim of this study was to determine the value of bending/traction radiographs in selection of distal instrumentation levels of a growing rod construct in children with idiopathic or idiopathic-like early-onset scoliosis (EOS). METHODS: Twenty-three consecutive patients with idiopathic or idiopathic-like EOS who underwent growing rod surgery at 2 separate institutions between 2006 and 2011 were included. Lengthening procedures were performed periodically at 6-month intervals. Analyses were performed retrospectively for age at index surgery, follow-up period, and radiographic measurements. Lower instrumented levels, neutral vertebra, stable vertebrae (SV), and stable-to-be vertebrae (StbV) were identified on the preoperative radiographs. Coronal Cobb angles, tilt of lower instrumented vertebra (LIV) and LIV+1, and disk wedging under the LIV and LIV+1 were measured on the early postoperative and latest follow-up radiographs. RESULTS: Average age at index surgery was 83.6 months. Mean follow-up period was 68.1 months. Initial analysis showed that the relationship of LIV to the StbV was a better predictive of LIV+1 tilt than the SV at the final follow-up. Therefore, the patients were grouped according to the relationship of the LIV to the StbV. LIV was the StbV in 9 patients, proximal to the StbV in 8 patients, and distal to the StbV in 6 patients. At the latest follow-up, tilt of LIV+1 exceeded 10 degrees in 7 of the 8 patients where LIV was proximal to the StbV, whereas only in 1 of 9 patients where LIV was StbV, and in none of the 6 patients where LIV was distal to the StbV. The data indicate that selection of the StbV as the LIV could spare an average of 1.8 vertebral segments when compared with the SV, as StbV is never distal but almost always proximal to the SV. CONCLUSIONS: Choosing the StbV as the LIV saves motion segments and prevents distal adding on, while providing satisfactory deformity correction in idiopathic and idiopathic-like EOS. LEVEL OF EVIDENCE: Level III-retrospective comparative study.

The Evolution of Sagittal Spinal Alignment in Sitting Position During Childhood.

STUDY DESIGN: This is a cross-sectional descriptive study. OBJECTIVE: The purposes of this study are to describe normative data of the sagittal plane in the sitting position within the pediatric population and document the evolution of sagittal alignment during the growth. SUMMARY OF BACKGROUND DATA: Surgical procedures addressing the deformity aim to make the maximal correction on the coronal and transverse planes and to restore the physiological curves on the sagittal plane. Prerequisite for sagittal plane reconstruction is to know the physiological values. METHODS: Children between 3 and 17 years of age, followed by pediatrics unit for nonskeleton disease with lateral radiographs of the entire spine and pelvis on sitting positions, were included to the study. Children with history of surgery or disease that may affect spine development were excluded. Children were evaluated in 4 age groups (3-6, 7-9, 10-12, and 13-17 yr) in terms of spinal sagittal alignment on sitting position. RESULTS: Of the screened, 124 children (49 girls, 75 boys) were included. Descriptive statistics of all possible segmental angles were summarized. Thoracic kyphosis and lumbar lordosis values were lower on sitting position than on standing position. Thoracic segmental angulations steadily increased from T1-T2 to midthoracic segments and then decreased in caudal direction. Moreover, lumbar segmental angulations steadily increased in cephalocaudal direction. Sacral slope, L4-S1 angulation, and T1-T12 and T1-S1 distance tend to increase as the age increases. CONCLUSION: Sagittal spinal alignment in the sitting position is different than that in the standing position and it changes as the child grows. There is a statistically significant difference between different age groups, especially at the cervicothoracic, thoracolumbar, and lumbosacral junctions. These findings should be taken into consideration for young nonambulatory patients who require spinal instrumentation and/or fusion. LEVEL OF EVIDENCE: 2.

Safety and Efficacy of Apical Resection Following Growth-friendly Instrumentation in Myelomeningocele Patients With Gibbus: Growing Rod Versus Luque Trolley.

INTRODUCTION: Thoracolumbar/lumbar kyphosis in myelomeningocele patients is a common and severely debilitating condition, amenable only to surgical correction. Several surgical techniques have been proposed. Growth-friendly techniques should be preferred in this patient population due to an already compromised trunk height. The growing rod (GR) and Luque trolley (LT) with Galveston instrumentation are well-known growth-friendly techniques. We compared results and complications in 2 groups of patients who have undergone kyphectomy and fixation, either with the GR (group 1) or the LT with Galveston pelvic fixation (group 2). METHODS: Ten patients undergoing GR fixation and 5 patients undergoing LT with Fackler fixation following kyphectomy (vertebral column resection or multiple eggshell) were included. GRs were lengthened every 6 months. Unplanned surgery in group 1 was defined as an unscheduled operation due to complication; all subsequent operations in group 2 were considered unplanned. Thoracic and local kyphosis and T1-S1 and T1-12 heights were measured preoperatively, postoperatively, and at final follow-up. RESULTS: Mean age at initial surgery was 6 years and 6.5 years for groups 1 and 2, respectively. Mean age at the last follow-up was 12.5 years for group 1 and 13.1 years for group 2. Mean follow-up was 72.7 months for group 1 and 68.6 months for group 2. Preoperative, postoperative, and final follow-up kyphosis angles in that order for group 1 were 72.3 degrees (10 to 110 degrees), 16.9 degrees (-50 to +55 degrees), and 21.6 degrees (-41 to +97 degrees), and for group 2 106.6 degrees (81 to 132 degrees), 15.6 degrees (-37 to +50 degrees), and 19.2 degrees (-42 to +38 degrees), respectively. Postoperative and final follow-up in that order for mean T1-T12 and T1-S1 heights for group 1 were 14 (11.2 to 18.7) cm, 20.4 (19.3 to 25.7) cm and 21 (17.2 to 23.2) cm, 31.6 (23.6 to 41.5) cm. Postoperative and final follow-up in that order for mean T1-T12 and T1-S1 heights for group 2 were 15.9 (14.3 to 19.7) cm, 20.1 (15.5 to 24.6) cm and 24.4 (17.7 to 27.8) cm, 29.5 (25.3 to 31.3) cm. Growth per year was 1.05 and 0.84 cm for groups 1 and 2, respectively (P=0.297). Fourteen versus 4 unplanned surgeries were performed in groups 1 and 2, respectively, and an additional 4 implant revisions were performed in group 1 during planned lengthenings. CONCLUSIONS: Both the LT and the GR system are reasonable alternatives of fixation postkyphectomy, both of which preserve growth to differing degrees. In this patient population with an already severely stunted trunk height, the surgeon must choose whether the amount of extra growth achieved by the GR is worth the risk of an increased number of surgeries.