Horizontal gaze palsy with progressive scoliosis: Further expanding the ROBO3 spectrum.

OBJECTIVE: Horizontal gaze palsy with progressive scoliosis (HGPPS) is a rare, autosomal recessive disorder resulting from axonal midline crossing defect due to variants in ROBO3. METHODS: We retrospectively evaluated demographics, clinical phenotype, course of spinal deformities, and neuroimaging findings of six Turkish patients with HGPPS. We performed targeted gene testing by next-generation sequencing. RESULTS: The median age at symptom onset and diagnosis was 1.5 years (0.5-4), and 11 years (2-16), respectively. Oculomotor signs were the most common presenting symptom (n = 4), followed by scoliosis (n = 2). The course of scoliosis was progressive and accompanied by kyphosis, showed intrafamilial variability, and was corrected surgically in three of the patients. Intellectual disability (n = 4), hypergonadotropic hypogonadism (n = 2), hearing loss (n = 2), and tranisent movement disorders (n = 1) were additional features. Targeted gene sequencing revealed five distinct homozygous variants. Of the four novel variants, two of them were located in the acceptor site of the noncoding region of the gene, remaining two were missense and frameshift variants, located in immunoglobulin-like domain-2, and cytoplasmic signaling motif 2, respectively. Structural magnetic resonance imaging (MRI) and diffusion tensor imaging (DTI) showed the absence of decussation of superior cerebellar peduncle and dorsal transverse pontine fibers. INTERPRETATION: Spectrum of HGPPS is further expanded with novel variants in the ROBO3 with clinical and radiological fingerprints. Spinal deformities require close orthopedic screening and individualized approach. Intellectual disability and hearing loss emerge as additional features. Hypogonadism and transient subtle movement disorders require further attention and confirmation from other series.

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.

Parent-patient Discrepancies in the Quality of Life Assessment of Early-onset Scoliosis: A Comparison Between 2 Questionnaires Completed on 2 Different Time Points - A Preliminary Report.

BACKGROUND: EOSQ-24 is a parent proxy questionnaire designed to assess the health-related quality of life (HRQoL) of early-onset scoliosis (EOS) patients during their childhood years. EOSQ-SELF, a novel self-reported questionnaire, assesses HRQoL in older children (>8 y) and adolescents. So far, the same group of EOS patients has not been evaluated with both EOSQ-24 and EOSQ-SELF. The aim of this study was to evaluate how the same pathology was reflected in the parent and patient at different time points by comparing the answers to the common questions between EOSQ-24 and EOSQ-SELF. METHODS: A group of otherwise healthy EOS patients whose parents filled out EOSQ-24 at the early phase of growth-friendly treatment was re-tested by the EOSQ-SELF questionnaire at the end of treatment. Both EOSQ-24 and EOSQ-SELF are validated in Turkish. Inclusion criteria were patients with EOS, independent ambulation, age of 8 years or older at EOSQ-SELF enrollment, literacy in Turkish, no apparent intellectual impairment, and a minimum of 24 months after graduation. The common questions between the 2 surveys with nearly identical phrasings were extracted. Common items from the 2 tests were compared with a Wilcoxon signed rank test. RESULTS: Twenty-one patients (15 females, 6 males) who previously filled out EOSQ-24 met the inclusion criteria. The mean age of the group was 10 (5 to 16) years at EOSQ-24 participation and 18 (13 to 24) at the final analysis. Fourteen questions were found common in 10 domains. The scores were significantly different in 5 questions of 4 domains. EOSQ-SELF had significantly less favorable scores in the pain/discomfort, pulmonary function, and fatigue/energy level domains. Scores in the parental burden/relationships domain were significantly higher (P<0.05). CONCLUSIONS: The self-reported group had a general trend of worse results. Parents and caregivers may not accurately perceive the problems of EOS patients. Our findings indicate a disconnect between caregivers and the patients, as both parties underreported the other side in some domains. These findings suggest the challenges faced by EOS patients are not adequately reflected on proxy questionnaires that assess the HRQoL of children. LEVEL OF EVIDENCE: Diagnostic Level I.

Safety and efficacy of growth-friendly instrumentation for early-onset scoliosis in patients with spinal muscular atrophy type 1 in the disease-modifying treatment era.

Purpose: To evaluate the safety of growth-friendly instrumentation for early-onset scoliosis (EOS) in patients with spinal muscular atrophy (SMA) type 1 who received disease-modifying treatment (DMT) and analyze short-term efficacy. Methods: Retrospective search was conducted between 2017 and 2023. Patients with genetically confirmed SMA type 1 who were surgically treated for spinal deformity and receiving DMTs (nusinersen, risdiplam, or onasemnogene abeparvovec) were included. SMA types 2 and 3 and patients who do not receive DMTs were excluded. Clinical and radiographic data were collected at preoperative, postoperative, and latest follow-up visits. Results: Twenty-eight patients (mean follow-up: 16 months (range 2-41)) were included. The mean age at surgery was 60 months (range 29-96). Fifteen were treated with dual magnetically controlled growing rods (MCGR), four with unilateral MCGR and a contralateral guided growth system, three with Vertical Expandable Prosthetic Titanium Rib (VEPTR®) implants, five with self-distracting systems, and one with traditional dual growing rods. The mean amount of correction was 57% (44°± 17) for scoliosis and 83% (13°± 11) for pelvic obliquity. The mean T1-12 height gain during surgery was 31 mm (±16 mm), while the mean T1 S1 height gain was 51 mm (±24 mm), and instrumented growth was observed during follow-up. Five patients (18%) developed six serious adverse events: three surgical site infections, two anchor failures, and one rod fracture, and all required unplanned reoperations. No neurologic complication, difficulty during nusinersen injections, or respiratory decline was recorded. Conclusion: We report that spinal deformity in this population can be safely treated with growth-friendly instrumentation, with similar complication rates when compared with SMA type 2.

An Eastern Europe and Middle East multinational expert Delphi consensus study on the prevention, diagnosis, and treatment of developmental dysplasia of the hip before walking age.

PURPOSE: The incidence of developmental dysplasia of the hip (DDH) is higher in Eastern Europeans and Middle Easterners. This study aimed to establish consensus among experts in this geographical area on the management of DDH before walking age. METHODS: Fourteen experienced orthopedic surgeons agreed to participate in a four-round online consensus panel by the Delphi method. The questionnaire included 31 statements concerning the prevention, diagnosis, and treatment of DDH before walking age. RESULTS: Consensus was established for 26 (84%) of 31 statements. Hip ultrasonography is the proper diagnostic tool under six months in DDH; universal newborn hip screening between three and six weeks is necessary; positive family history, breech presentation, female gender, and postnatal swaddling are the most important risk factors; Ortolani, Barlow tests, and limitation of abduction are the most important clinical findings; Pavlik harness is the first bracing preference; some Graf type IIa hips and all Graf type IIb and worse hips need abduction bracing treatment; the uppermost age limit for closed and open reductions is 12 months and 12-24 months, respectively; anatomic reduction is essential in closed and open reductions, postoperative MRI or CT is not always indicated; anterior approach open reduction is better than medial approach open reduction; forceful reduction and extreme positioning of the hips (> 60° hip abduction) are the two significant risk factors for osteonecrosis of the femoral head. CONCLUSION: The findings of the present study may be useful for clinicians because a practical reference, based on the opinions of the multinational expert panel, but may not be applicable to all settings is provided.

Management of low back pain accompanying sagittal plane pathologies in children: Spondylolysis/spondylolisthesis and Scheuermann's disease.

Background: Low back pain in childhood was underappreciated for a long time, but recent studies report higher prevalences, up to 70%. Two of the common causes are spondylolyis/spondylolisthesis and Scheuermann's disease. These disorders are relevant in a way they both cause significant back pain, and may disrupt the sagittal spinal balance. Purpose: To present the current evidence on the diagnosis, natural history and treatment of these disorders with a special focus on sagittal spinal alignment. Methods: This study is conducted as a literature review. Results and Conclusions: Spondylolysis and low-grade spondylolisthesis have a benign course and are typically treated conservatively. When pars repair is indicated, pedicle screw-based techniques achieve more than 90% fusion with acceptable complication rates. High-grade spondylolisthesis, however, is frequently progressive. Surgical treatment involves fusion, which can be done in situ or after reduction. Reduction is useful for "unbalanced" patients to acquire sagittal spinopelvic balance, and it is important to distinguish these patients. Despite lowering the risk for pseudoarthrosis, reduction brings a risk for neurologic complications. With re-operation rates as high as 40%, these patients definitely require careful preoperative planning. Scheuermann's disease generally causes back pain in addition to cosmetic discomfort during adolescence. If the kyphosis is lower than 60°, symptoms typically resolve into adulthood with conservative measures only. However, it must be kept in mind that these patients may experience problems with physical performance and have a lower quality of life even when the problem seems to have "resolved". Severe kyphosis and intractable back pain are the most frequently referred surgical indications, and surgery typically involves fusion. Proper utilization of osteotomies and proper selection of the upper and lower fusion levels are of utmost importance to prevent complications in these patients.

Does the Sleeper Plate Application for Temporary Epiphysiodesis Make Life Easier or Complicated? Increased Risk of Tethering.

BACKGROUND: The present study aims to investigate the frequency of recurrence and tethering effect after only metaphyseal screw removal (sleeper plate technique) compared with the conventional complete plate removal in the treatment of lower extremity deformities with guided growth surgery. METHODS: Seventy-two patients (107 limbs) treated by an 8-plate hemiepiphysiodesis technique around the knee joint were evaluated. After the desired correction, only metaphyseal screw was removed (sleeper plate group) in 35 limbs (25 patients), whereas both screws and plate were removed (plate removal group) in 72 limbs (47 patients). An increase of 5 degrees or more in joint orientation angles in the direction of the initial deformity was considered as recurrence. The rate of rebound, tethering, and maintenance of correction in groups was analyzed at the latest follow-up (mean of 49 mo). RESULTS: The mean age of the patients was 97 months (range: 80 to 129 mo) at the time of index surgery. After a mean of 49 months (range: 16 to 86), 17 (48.5%) limbs maintained the desired stable correction in the sleeper plate group compared with 59 stable limbs (72.2%) in the plate removal group ( P <0.001). There was no statistically significant difference regarding recurrence between the sleeper plate group and the plate removal group (34.3% vs. 27.8%, respectively) ( P =0.216). Reinsertion of the metaphyseal screw was possible 8/12 limbs, and the remaining 4 limbs underwent further surgeries. There were 6 limbs (17.3%) of tethering in the sleeper plate group, and 4/6 limbs required further corrective surgeries. The remaining 2 limbs with slight tethering did not require further surgeries. CONCLUSIONS: Removing only metaphyseal screw increases the risk of tethering. In addition, reinsertion of the screw may not be possible in all cases due to bony growth, and further corrective surgeries may be necessary. Close follow-up is required if the sleeper plate technique is to be applied. LEVEL OF EVIDENCE: Level III.

A Comprehensive Assessment of Psychosocial Well-being Among Growing Rod Graduates: A Preliminary Investigation.

BACKGROUND: Traditional growing rods (TGR) encompass a long process, in which patients experience physical and psychosocial difficulties. However, the effect of repeating surgeries on the overall psychological functioning of graduated patients has not been thoroughly investigated in the literature. The aim of this study is to evaluate the psychological well-being of graduated idiopathic early-onset scoliosis patients in terms of psychopathology, neurocognition, and psychosocial functioning, and determine the accuracy of scoliosis outcome questionnaires in these regards. METHODS: TGR graduates with idiopathic early-onset scoliosis without known intellectual disabilities or neuromuscular impairments were included. Patients were thoroughly evaluated using psychological instruments [Wechsler Adult Intelligence Scale, Auditory Consonant Trigram Test, Verbal Fluency Test, Beck Depression Inventory, Beck Anxiety Inventory, Rosenberg Self-Esteem Scale, Symptom Checklist-90, Post-Traumatic Growth Inventory, Strengths and Difficulties Questionnaire, Spinal Appearance Questionnaire, Scoliosis Research Society 22-item questionnaire (SRS-22)]. Results were compared with normative data when available. Spearman correlations were performed between the results of these tests, the total treatment duration, and the number of spinal surgeries. RESULTS: Of the 15 patients included in the study, 9 were females, and the mean age was 18.73 (16 to 23). The mean age at index surgery was 6.38 (3 to 10) whereas that of graduation was 14.00 (12 to 16). The average number of spinal surgeries was 14.28 (7 to 20). Two patients performed below the range of adult intellectual functioning. Auditory Consonant Trigram Test showed normal verbal working memory and attention control. Six patients had abnormal Verbal Fluency Test performance. Eight patients had abnormal ratings on at least one of the assessment scales of psychopathology (Symptom Checklist-90, Beck Depression Inventory, and Beck Anxiety Inventory). Eight patients had low-to-moderate self-esteem (Rosenberg self-esteem scale). The median spinal appearance questionnaire and SRS-22 scores were 34 and 4.18, respectively. Pain and function subdomains of SRS-22 scored higher than self-image and mental health. No correlation was found between the treatment duration and number of surgeries and test scores. SRS-22 showed correlations with multiple psychological tests. CONCLUSION: Completed TGR treatment yields acceptable correction of deformities and surgical outcomes, however, may fail to improve psychological well-being. This is the first study to find various psychosocial abnormalities in two-thirds of cases. LEVEL OF EVIDENCE: Level IV; cross-sectional study.

Reliability and validity of the Turkish version of the Kyphosis specific spinal appearance questionnaire in adolescents with moderate hyperkyphosis.

BACKGROUND: Hyperkyphosis cause poor posture, self-image, clinical or cosmetic deformity and well-being. Therefore, it is important to evaluate patients' perception of appearance in the rehabilitation process of individuals with hyperkyphosis. Recently Kyphosis Specific Spinal Appearance Questionnaire (KSAQ) has been developed for the assessment of appearance in hyperkyphosis patients. OBJECTIVE: To test validity and reliability of the Turkish version of the KSAQ. METHODS: Sixty-two patients with hyperkyphosis (curve above 50º), ranging in age between 12 and 22 years, was included in this study. Turkish translation/back-translation of the KSAQ was done by an expert committee. Internal consistency was analyzed using Cronbach's alpha. Validity was assessed by correlating the KSAQ with the Scoliosis Research Society-22 (SRS-22) Questionnaire. Its reliability was assessed using the test-retest method with two-week interval (Pearson's correlation coefficient) on 44 of these patients. RESULTS: Mean kyphosis angle was 60º ± 8.9º. KSAQ total scores showed excellent internal consistency (Cronbach's α = 0.944) and test-retest reliability (ICC = 0.890). Moderate to strong associations were found between KSAQ total score, each item scores and self-image domain of SRS-22 (r -0.299 to -0.730, p < 0.05). KSAQ total score had moderate correlation with SRS-22 total score (r 0.423, p 0.001). Overall, the KSAQ scale showed good validity. CONCLUSION: The Turkish version of the KSAQ is a reliable and valid patient reported outcome measure of kyphosis-specific aspects of appearance in patients with moderate hyperkyphosis.

The Effect of Apical Vertebra Position on Growing Rod Treatment: A Clinical and Finite Element Study.

BACKGROUND: Growing rods (GRs) is a commonly utilized technique for the management of early-onset scoliosis. The significance of the position of the apical vertebrae relative to the rods is not known. The purpose of this study is to analyze the potential effects of the position of the apical vertebrae in relation to the GRs on deformity control through plain radiographs and finite element analysis (FEA) modeling. METHODS: We identified 140 patients treated with GR between 2000 and 2018. Patients who had a congenital vertebral anomaly or <2-year follow-up were excluded. Curve magnitude, traction radiograph under general anesthesia (TRUGA) flexibility, apical rotation, the lengths of T1-12, T1-S1, and the instrumented segments were recorded. Patients were divided into 3 groups according to the apical position on the postoperative radiographs: group 1 (both pedicles are between the rods), group 2 (convex rod is between the apical vertebra pedicles), group 3 (both pedicles are lateral to the convex rod). FEA models were created simulating the 3 groups. Both radiographic and FEA data were analyzed to compare the deformity control and growth in each group. RESULTS: Fifty-eight patients were included in the final analyses (mean age 84 mo; range: 38 to 148). Ten patients (17%) were in group 1, 34 (59%) in group 2, and 14 (24%) in group 3. Difference between TRUGA flexibilities was statistically insignificant. Group 3 was the least successful in terms of both height gain and rotational control. FEA showed a decrease in rotation and displacement for every group, however, the residual rotation and displacement was highest in group 3. CONCLUSIONS: Bringing the apex in line with the GR increases the capacity of growth preservation as it results in largest height gain and better deformity control. FEA model demonstrated that distraction alone is inadequate for controlling rotation, and with increasing apical translation, residual rotation after distraction also increases. LEVEL OF EVIDENCE: Level III.

The reliability of the AOSpine Thoracolumbar Spine Injury Classification System in children: an international validation study.

PURPOSE: To evaluate the AOSpine Thoracolumbar Spine Injury Classification System and if it is reliable and reproducible when applied to the paediatric population globally. METHODS: A total of 12 paediatric orthopaedic surgeons were asked to review MRI and CT imaging of 25 paediatric patients with thoracolumbar spine traumatic injuries, in order to determine the classification of the lesions observed. The evaluators classified injuries into primary categories: A, B and C. Interobserver reliability was assessed for the initial reading by Fleiss's kappa coefficient (kF) along with 95% confidence intervals (CI). For A and B type injuries, sub-classification was conducted including A0-A4 and B1-B2 subtypes. Interobserver reliability across subclasses was assessed using Krippendorff's alpha (αk) along with bootstrapped 95% CIs. A second round of classification was performed one-month later. Intraobserver reproducibility was assessed for the primary classifications using Fleiss's kappa and sub-classification reproducibility was assessed by Krippendorff's alpha (αk) along with 95% CIs. RESULTS: In total, 25 cases were read for a total of 300 initial and 300 repeated evaluations. Adjusted interobserver reliability was almost perfect (kF = 0.74; 95% CI 0.71 to 0.78) across all observers. Sub-classification reliability was substantial (αk= 0.67; 95% CI 0.51 to 0.81), Adjusted intraobserver reproducibility was almost perfect (kF = 0.91; 95% CI 0.83 to 0.99) for both primary classifications and for sub-classifications (αk = 0.88; 95% CI 0.83 to 0.93). CONCLUSION: The inter- and intraobserver reliability for the AOSpine Thoracolumbar Spine Injury Classification System was high amongst paediatric orthopaedic surgeons. The AOSpine Thoracolumbar Spine Injury Classification System is a promising option as a uniform fracture classification in children. LEVEL OF EVIDENCE: III.

Magnetically Controlled Growing Rods Graduation: Deformity Control with High Complication Rate.

STUDY DESIGN: A multicenter retrospective review of consecutive series of patients. OBJECTIVE: Long-term experience with using the magnetically controlled growing rods (MCGR) to treat patients with deformity in the growing spine to the conclusion of treatment with posterior spine fusion. SUMMARY OF BACKGROUND DATA: MCGR treatment for growing spine gained popularity with paucity of long-term follow up data. We hypothesized that final fusion might be more effective in bringing additional correction of the spine deformity after treatment with MCGR than that reported after traditional growing rods (TGR) due to less scarring and auto-fusion. METHODS: Retrospective review of 47 patients with varied etiology, treated between 2011 and 2017 which graduated treatment were followed in five academic medical centers for average of 50 months (range, 10-88). RESULTS: The initial mean coronal deformity of 69.6° (95% CI 65-74) was corrected to 40° (95% CI 36-40) immediately after the MCGR implantation but progressed to 52.8° (95% CI 46-59) prior to the final surgery (P < 0.01). Nevertheless, thoracic spine growth (T1-T12 height) improved from 187.3 mm (95% CI 179-195) following index surgery to 208.9 mm (95% CI 199-218) prior to final fusion (P < 0.01). Significant correction and spinal length were obtained at final fusion, but metallosis was a frequent observation (47%, 22/47). The average growth rate was 0.5 mm/month (95% CI 0.3-0.6). The overall complication rate within our cohort was 66% (31/47) with 45% (21/47) of unplanned returns to the operating theater. 32% (15/47) of the patients had an implant related complication. Unplanned surgery was highly correlated with thoracic kyphosis greater than 40° (OR 5.42 95% CI 1.3-23). CONCLUSION: Treatment of growing spine deformities with MCGR provides adequate control of spine deformity it is comparable to previously published data about TGR. The overall high complications rate over time and specifically implant related complications.Level of Evidence: 4.

Clinical Outcomes of 3-column Osteotomy at Growing Rod Graduation.

BACKGROUND: Several different strategies were described on how to graduate a patient when skeletal maturity is reached after growing rod treatment. A final instrumentation and fusion is commonly performed, but the stiffness of the spine after numerous lenghthenings can be a major problem. When correction is needed in a severe and rigid curve, 3-column osteotomies (3CO) can be used as a method of bringing the spine back to a well-balanced position. This study aims to evaluate the clinical outcomes of 3CO osteotomies at growing rod graduation. METHODS: A retrospective search of the database was performed between 1996 and 2018. Inclusion criteria were: diagnosis of early-onset scoliosis, underwent 3CO at the final fusion procedure, and ≥1 year of follow-up. Indications for this osteotomy were: significant sagittal and/or coronal off-balance or severe crankshaft deformity or significant distal and/or proximal adding-on. Clinical, radiographic, and operative data were analyzed. Complications were classified according to the modified Clavien-Dindo-Sink system. RESULTS: Eight patients were included in the study with a mean age of 13.5 at graduation (male/female: 2/6). The mean scoliosis angle significantly reduced from 65.6 to 39 degrees (P=0.012). Mean T1-T12 height increased from 19 to 20.5 (P=0.084) and T1-S1 from 29.6 to 31.6 (P=0.144). Five patients had a staged surgery. Average operative time was 338 minutes and blood loss was 1321 mL. Four grade 1, 1 grade 2, and 1 grade 3 complications occurred. CONCLUSIONS: This is the first case-series in the literature reporting on the use of 3CO at growing rod graduation. These procedures can be safely and effectively used to overcome significant multiplanar deformity and/or off-balance in the graduation of patients with early-onset scoliosis and significant correction can be achieved on these severe and rigid curves. LEVEL OF EVIDENCE: Level IV-case series.

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.

Aponeurotic release of semimembranosus: A technical note to increase correction gained with hamstring lengthening surgery in cerebral palsy.

OBJECTIVE: The aim of this study was to determine the intraoperative corrective effect of the aponeurotic release of semimembranosus (SM) as a single procedure or an adjunct procedure to distal myotendinous release of semitendinosus (ST) and myofascial release of SM lengthening in the correction of knee flexion deformity in cerebral palsy (CP). METHODS: In this prospective study, 46 knees of 23 consecutive ambulatory patients (15 boys and 8 girls; mean age=8.33 years; age range=5-12 years) with spastic diplegic CP with a gross motor function classification system level (GMFCS) II or III were included. The patients were then divided into 2 groups. In group I, there were 10 patients (4 boys, 6 girls; mean age=8.6±2), and combined release of ST in the myotendinous junction and SM in the myofascial junction, followed by aponeurotic release of SM were carried out. In group II, there were 13 patients (2 girls, 11 boys; mean age=8±2.35), and aponeurotic release of SM was done first and followed by the combined release of ST in the distal myotendinous junction and the myofascial release of SM. Intraoperative popliteal angle (PA) measurements were recorded in each group. RESULTS: PA was reduced from 58.1°±7.6° (range=46°-75°) to 41.2°±8.8° (range=20°-54°) in group 1 and from 59.1°±11.3° (range=40°-87°) to 42.7°±10.8° (range=24°-64°) in group 2. No significant difference was observed between the groups in terms of reduction in PA (p=0.867). In group 1, adding the aponeurotic release of SM further reduced the PA to 31.7°± 8.5° (range=14°-47°) (p=0.002). In group 2, adding the myotendinous release of ST and myofascial release of SM further reduced the PA to 32.9°±7.2° (range=16°-44°) (p=0.004). There was no significant difference between the final PA values in the 2 groups (p=0.662). There was no difference in terms of early complications. CONCLUSION: Aponeurotic release of SM is equally effective to reduce the intraoperative PA with combined myotendinous release of ST and myofascial release of SM. Combining all the 3 procedures provides a better correction without forceful manipulation or lengthening of the lateral hamstrings during the correction of knee flexion deformity in CP.

Improving Safety and Efficacy in the Surgical Management of Low-tone Neuromuscular Scoliosis: Integrated Approach With a 2-attending Surgeon Operative Team and Modified Anesthesia Protocol.

BACKGROUND: Substantially increased operative time and amount of bleeding may complicate the course of surgical treatment in neuromuscular scoliosis. A well-organized team approach is required to reduce morbidity. The aim of this study is to review our early, short-term surgical outcomes with our new integrated approach that includes a 2-attending surgeon team and modifications in the anesthesia protocol in low-tone neuromuscular scoliosis and compare with a matched cohort of our historic patients. METHODS: We retrospectively reviewed our patients with (1) neuromuscular scoliosis with collapsing spine deformity, (2) low-tone neuromuscular etiology, (3) multilevel posterior column osteotomies with posterior all pedicle screw spinal fusion, and (4) more than 1-year follow-up. Patients were grouped into 2: group 1 consisted of patients managed with the integrated surgical team approach, group 2 included the matched historic patients. RESULTS: There were 16 patients in group 1 and 17 patients in group 2. There was no significant difference between the groups regarding age, sex, body mass index, number of levels fused, major coronal deformity magnitude, pelvic obliquity, number of posterior column osteotomies, or amount of deformity correction. However, significantly shorter operative time (241 vs. 297 min, P=0.006), less intraoperative bleeding (1082 vs. 1852 mL, P=0.001), less intraoperative blood transfusion (2.1 vs. 3.1 U, P=0.028), less postoperative intensive care unit admission (23% vs. 100%, P=0.001), and shorter hospital stay (4.7 vs. 5.9 d, P=0.013) were observed in group 1. CONCLUSIONS: Our results indicate that spinal deformity surgery in patients with underlying low-tone neuromuscular disease may not be as intimidating as previously thought. Our surgical team approach integrating a 2-attending surgeon operative team, a new anesthetic protocol that includes a modification of perioperative blood management is effective in reducing operative times, blood loss, transfusion rates, intensive care unit admission, and length of hospital stay. LEVELS OF EVIDENCE: Level III-retrospective comparative study.

Sagittal spinal alignment after total hip arthroplasty for neglected high hip dysplasia: does changing the distorted mechanics of the hip normalize spinal alignment?

PURPOSE: The false acetabulum lies more laterally and posteriorly compared with the true acetabulum. Spatial orientation of the pelvis is significantly altered in patients with neglected high hip dysplasia. There has been no study to investigate how pelvic or sagittal spinal alignment change after true acetabulum gains function with hip arthroplasty. The aim of this study was to investigate the effect of total hip arthroplasty with femoral shortening on spinopelvic parameters in patients with neglected high hip dysplasia. METHODS: Twenty patients with Crowe type 3 or 4 hip dysplasia, who underwent total hip arthroplasty with femoral shortening in our institution were evaluated preoperatively after completion of rehabilitation and return to their normal daily life. Sagittal alignment (sacral slope, pelvic incidence, global tilt, segmental lordosis, segmental kyphosis, GAP score) and coronal alignment angles (coronal tilt, Cobb angle) of patients were measured by two independent observers. RESULTS: Twenty patients underwent hip arthroplasty with femoral shortening followed up for a minimum of 12 months. We found higher preoperative global lordosis (68.7 ± 9.7) and sacral slope (52.1 ± 8.8) angles, but the pelvic incidences (57.9 ± 10.1) were in the normal range. No statistically significant difference in any sagittal spinopelvic parameters between pre- and postoperative measurements was detected. GAP scores also did not change significantly (p = 0.231). Coronal plane parameters (Cobb angle, coronal pelvic tilt) were the only parameters in which a statistical change was observed (p = 0.02, p = 0.05, respectively). CONCLUSION: Lumbar lordosis and sacral slope values are outside standard ranges in patients with neglected dysplasia of the hip. The reconstruction of the distorted mechanics of the hip joint does not normalize sagittal pelvic and spine anatomy however improvements in coronal alignment were observed. Disease specific values of sagittal spinal alignment should be used in the treatment of lumbar degenerative problems in patients with neglected high hip dysplasia. LEVEL OF EVIDENCE: IV.

Early Results of a Management Algorithm for Collapsing Spine Deformity in Young Children (Below 10-Year Old) With Spinal Muscular Atrophy Type II.

BACKGROUND: Progressive C-shaped scoliosis with marked pelvic obliquity is common to spinal muscular atrophy (SMA). Reducing the number of procedures with effective deformity control is critical to minimize the risk of pulmonary complications. This study reports the preliminary results of magnetically controlled growing rods (MCGR) in SMA-related collapsing spine deformity. METHODS: Inclusion criteria for this retrospective review were: (1) SMA type 2 patients, (2) early onset scoliosis (below 10 y), (3) collapsing spine deformity with pelvic obliquity, (4) growth-friendly scoliosis treatment with MCGR, (5) in between 2014 and 2017. Extracted data included demographic and clinical information, radiologic parameters, surgical details, and final status of the patients. RESULTS: A total of 11 patients (7 boys, 4 girls) were included. The average age at index surgery was 8.2 (6 to 10) years. Dual MCGR was implanted in 8 patients. In 3 patients, because of curve rigidity and inability of apex to be brought into the stable zone, apical fusion with gliding connectors (convexity) and a single MCGR (concavity) was preferred. Instrumentation included the pelvis in 9 and stopped at the lumbar spine (L3) in 2 patients at the index procedure. Average preoperative deformity of 81.8 degrees (66 to 115) decreased to 29 degrees (11 to 57) postoperatively and was 26 degrees at average 35 months (16 to 59). Pelvic obliquity of 20.9 degrees (11 to 30) decreased to 4.9 degrees (2 to 8) after index surgery and was 6.5 degrees (2 to 16) at the last follow-up. T1-S1 height of 329 mm (280 to 376) after index surgery increased to 356 mm (312 to 390) after 9.2 (4 to 20) outpatient lengthening. No neurologic, infectious, or implant-related complication was recorded. Distal adding-on deformity occurred in 2 patients without initial pelvic fixation.One patient deceased secondary to pneumonia at 16 months after surgery. CONCLUSIONS: Short-term results indicate that MCGR may be a good option in SMA-associated collapsing spine deformity to reduce the burden of repetitive lengthening procedures. The authors recommend apical deformity control in the convex side in case of curve rigidity. In addition, including the pelvis in the instrumentation at index surgery is critical to prevent distal adding-on. LEVEL OF EVIDENCE: Level IV-retrospective case series.

Evaluation of upper extremity function and its relation to curve pattern in female adolescents with idiopathic scoliosis: a pilot study.

STUDY DESIGN: Cross-sectional and clinical measurement. OBJECTIVE: To evaluate upper extremity function and its relation to the curve pattern in idiopathic scoliosis. Postural alterations and trunk distortions-caused by three-dimensional deformity itself in idiopathic scoliosis-may lead to functional changes in the upper extremity of subjects. METHODS: Handgrip, pulp and lateral pinch strengths, hand dexterity, hand reaction time, coordination of upper extremity, upper extremity performance, throwing accuracy, and self-reported upper extremity disability were evaluated in 96 subjects. These subjects were divided into 3 groups: 47 with main thoracic curve pattern scoliosis (Lenke type 1), 31 with thoracolumbar/lumbar curve pattern scoliosis (Lenke type 5), and 18 unaffected (healthy control). Comparisons were performed between these three groups. RESULTS: The thoracic scoliosis group showed a significant decrease in concave lateral pinch strength, concave hand dexterity of turning, coordination of the upper extremities, and concave hand reaction time than the thoracolumbar/lumbar scoliosis group (p < 0.05). Bilateral handgrip strengths decreased in thoracic scoliosis group when compared to healthy controls. Healthy individuals demonstrated greater throwing accuracy than individuals with scoliosis. CONCLUSIONS: Upper extremity function was found to be affected based on the curve pattern. Individuals with main thoracic curves are likely to have deteriorated upper extremity function, especially for hand-specific motor skills, on the concave side, when compared to lumbar curves and healthy controls. LEVEL OF EVIDENCE: Level III.