Ewing's sarcoma family of tumors in Finland during 1990-2009: a population-based study.

BACKGROUND: Ewing's sarcoma family of tumors (ESFTs) are rare bone and soft tissue tumors characterized by specific genetic alterations. Our aim was to carry out a nationwide analysis of ESFT, to survey the treatments used and to report the five-year disease specific and event-free survival rates (EFS and DSS). MATERIAL AND METHODS: The study data was gathered from the Finnish National Cancer Registry and all five University Hospitals and consisted of 76 bone and soft tissue ESFT patients diagnosed during 1990-2009. Their medical records were reviewed and data on their disease, treatments, complications and outcome were analyzed. RESULTS: The five-year EFS and DSS of patients with localized disease at diagnosis (n = 57) were 70% and 60%, respectively. Factors contributing to DSS and EFS were the axial vs. peripheral site of primary tumor and adequate surgical resection of the primary tumor. DSS was also affected by patient's age at diagnosis and the treatment employed. The five-year DSS of patients with metastatic disease at diagnosis (n = 19) was 33% and both preoperative and high dose chemotherapy were associated with improved survival. CONCLUSION: Population-based studies including both bone and soft tissue ESFTs are few. In this nationwide, population-based study on Finnish bone and soft tissue ESFT patients, we find their treatment successful and results comparable to those previously published. Absence of metastases, young age at diagnosis and a peripheral primary tumor site were associated with a better prognosis. It seems that surgical resection of the primary tumor should be performed whenever adequate resection margins can be achieved. The role of high dose chemotherapy merits further studies in this setting.

Less-Invasive Approach to Early-Onset Scoliosis-Surgical Technique for Magnetically Controlled Growing Rod (MCGR) Based on Treatment of 2-Year-Old Child with Severe Scoliosis.

BACKGROUND: Spinal deformities in children can be caused by various etiologies, such as congenital, syndromic, neuromuscular, or idiopathic. Early-onset scoliosis (EOS) is diagnosed before the age of ten years, and when the curvature continues to progress and exceeds a Cobb angle of 60-65 degrees, surgical treatment should be considered. Initial minimally invasive surgery and the implantation of magnetically controlled growing rods (MCGRs) allows for the noninvasive distraction of the spine, growing, and avoids multiple operations associated with the classic distractions of standard growing rods. CASE PRESENTATION: A 2-year-old girl was admitted to our clinic with rapidly progressive thoracic scoliosis. The major curve of the thoracic spine Cobb angle was 122° at 30 months. No congenital deformities were detected. The surgical technique was the less-invasive percutaneous and subfascial implantation of MCGRs, without long incisions on the back and the non-invasive ambulatory lengthening of her spine over the next 4 years. CONCLUSIONS: MCGR is a safe procedure for EOS patients. It is extremely effective at correcting spinal deformity; controlling the growth and curvature of the spine as the child develops during growth; reducing the number of hospitalizations and anesthesia; and minimizing the physical and mental burden of young patients, parents, and their families.

A Rare Complication of Thoracic Spine Surgery: Pediatric Horner's Syndrome after Posterior Vertebral Column Resection-A Case Report.

BACKGROUND: Horner's syndrome (HS) classically consists of the symptom triad of miosis, ptosis, and anhidrosis. It is caused by impairment of a certain pathway in the sympathetic nervous system. It may also appear as part of the clinical signs of other diseases and syndromes, including Pancoast tumors, intradural and/or epidural tumors, thoracic outlet syndrome, syringomyelia, brachial plexus injury, and aortic dissection. Here, we report a very rare complication of vertebral column resection in children, and we present the clinical findings of a case of Horner's syndrome with a current literature review. CASE PRESENTATION: A five-year-old child with severe congenital kyphoscoliosis qualified for surgical treatment of the spinal deformity via a posterior approach, with three-column osteotomy and fusion. RESULTS: After successful surgery, the patient presented with HS due to distraction of the sympathetic nerve trunk and, thus, innervation to the left eye. At the 4-year follow-up, the child had fully recovered. CONCLUSIONS: Pediatric HS after posterior instrumented scoliosis correction surgery with posterior vertebral column resection of the thoracic spine is very rare. This is the first reported case of HS after posterior vertebral column resection and spinal fusion for congenital kyphoscoliosis without the use of epidural analgesia. Symptom resolution may be variable and, in some cases, delayed.

The Efficacy of a Posterior Approach to Surgical Correction for Neglected Idiopathic Scoliosis: A Comparative Analysis According to Health-Related Quality of Life, Pulmonary Function, Back Pain and Sexual Function.

BACKGROUND: This study aimed to evaluate the treatment outcomes of severe idiopathic scoliosis (IS) and hypothesized that surgical treatment would have a superior impact on the health-related quality of life (HRQoL), pulmonary function (PF), back pain, and sexual function. METHODS: We retrospectively reviewed 195 consecutive patients with IS classified into severe (SG) and moderate groups (MG) with a minimum follow-up of two years. RESULTS: The mean preoperative curve was 131° and 60° in the SG and MG, respectively. The mean preoperative flexibility in the bending films averaged between 22% in the SG and 41% in the MG. After definitive surgery, the main curve was corrected to 61° and 18° in the SG and MG, respectively. The mean preoperative thoracic kyphosis was 83° in the SG and 25° in the MG, which was corrected to 35° in the SG and 25° in the MG. At baseline, the percentage of predicted lung volume (FVC) was significantly lower in the SG than that in the MG (51.2% vs. 83%). The baseline percentage of the predicted FEV1 values was also significantly lower in the SG than in the MG (60.8% vs. 77%). During the two-year follow-up, the percentage of predicted FVC showed significant improvement in the SG (69.9%) (p < 0.001), and the percentage of predicted FEV1 values during the follow-up improved significantly in the SG (76.9%) (p < 0.001) compared with the MG (81%), with no statistical difference observed during the two-year follow-up. The SRS-22r showed a clinically and statistically significant improvement in the preoperative results to those of the final follow-up (p < 0.001). CONCLUSIONS: Surgical treatment of severe scoliosis can be safe. It provided a mean correction of the deformity for 59% of patients and significantly improved respiratory function, with the percentage of predicted forced expiratory volume in 1 s improving by 60% and the forced vital capacity improving by 50%, resulting in clinically and statistically significant improvements in the SRS-22r, HRQoL outcome scores, and back pain (reduced from 36% to 8%), as well as improved sexual function. The planned surgical treatment can achieve a very significant deformity correction with a minimal risk of complications. The surgical treatment has a superior impact on the quality of life patients with severe spinal deformities and significantly improves function in every sphere of life.

No Benefits in Using Magnetically Controlled Growing Rod as Temporary Internal Distraction Device in Staged Surgical Procedure for Management of Severe and Neglected Scoliosis in Adolescents.

BACKGROUND: Severe spinal curvatures (SSCs) in children and adolescents have long been treated with preoperative Halo traction, in its various variations. There are also several radical techniques available for the management of neglected SSCs, such as osteotomies; however, these can be risky. Comparing the treatment outcomes when using preoperative Halo Gravity Traction (HGT) against the use of a Magnetically Controlled Growing Rod (MCGR) as a temporary internal distraction (TID) device, we evaluated the differences in surgical and radiological outcomes. METHODS: We conducted a retrospective study of 30 patients with SSCs, treated with HGT followed by posterior spinal fusion (PSF; Group 1, n = 18) or treated using a temporary MCGR as a TID followed by PSF (Group 2, n = 12). All patients underwent surgical treatment between 2016 and 2022. The inclusion criteria were SSC > 90°, flexibility < 30%, and the use of preoperative HGT followed by PSF or the two-stage surgical procedure with initial TID rod placement (Stage 1) followed by PSF (Stage 2). The evaluated parameters were as follows: rib hump, trunk height, and radiographic outcomes. All parameters were collected preoperatively, after the initial surgery, after final correction and fusion, and during the final follow-up. RESULTS: In Group 1, we evaluated 18 patients with a mean age of 15.5 years; in Group 2, we evaluated 12 patients with a mean age of 14.2 years. The interval between the staged procedures averaged 32.7 days. The mean preoperative main curves (MC) were 118° and 112° in Group 1 and Group 2, respectively. After definitive surgery, the MC was corrected to 42° and 44° in G1 and G2, respectively. The mean percentage correction of the MC was similar in both groups (65% vs. 61% in G1 and G2, respectively). The mean preoperative thoracic kyphosis was 92.5° in G1 and 98° in G2, corrected to 43.8° in G1 and 38.8° in G2. Trunk height increased by 9 cm on average. CONCLUSIONS: There are no benefits in using a MCGR as a temporary internal distraction device in the management of neglected scoliosis in adolescents. Surgical treatment of severe scoliosis may be safe, with a reduced risk of potential complications, when using preoperative HGT. A specific intraoperative complication when using a MCGR as a temporary internal distraction device was a 50% risk of transient neuromonitoring changes, due to significant force applied to the spine and radical distraction of the spine. We achieved similar clinical, radiographic, and pulmonary function outcomes for both techniques. The use of HGT causes less blood loss with a shorter overall time under anesthesia. Partial correction significantly aids the subsequent operation by facilitating a gradual reduction in the curvature, thereby reducing the difficulty of surgical treatment and the risk of neurological deficits.

Influences of Increasing Pedicle Screw Diameter on Widening Vertebral Pedicle Size during Surgery in Spinal Deformities in Children and Adolescents without Higher Risk of Pedicle and Vertebral Breaches.

BACKGROUND: A very common technique for treating spinal deformities in children and adolescents is the use of segmental screws. In order to obtain proper stability and the best possible correction, the screws must first be precisely inserted. Additional factors influencing the quality and success of the operation are the size and quality of the bone, the skills of the surgeon, and biomechanical factors, i.e., the width and length of the screws used during surgery. Our study was focused on evaluating the effect of increasing the diameter of the instrumented pedicles by pedicle screws and assessing the safety of expanding these pedicles with screws of various sizes in children with spinal deformities during the growth period, using preoperative magnetic resonance imaging and postoperative computed tomography (CT) to assess and compare preoperative size measurements from MRI to postoperative CT measurements. METHODS: We obtained data for evaluation from the available medical records and treatment histories of patients aged 2 to 18 who underwent surgical treatment of spinal deformities in the years 2016-2023. In 230 patients (28 male and 202 female), 7954 vertebral bodies were scanned by preoperative MRI, and 5080 pedicle screws were inserted during surgery, which were then assessed by postoperative CT scan. For the most accurate assessment, patients were classified into three age groups: 2-5 years (Group 1), 6-10 years (Group 2), and 11-18 years (Group 3). In addition, we studied implant subgroups: vertebral bodies with inserted pedicles of screw sizes 5.0 mm and 5.5 mm (Group S), and pedicles of screw sizes 6.0 mm, 6.5 mm, and 7.0 mm (Group L). RESULTS: The morphology of pedicles (Lenke classification) analyzed before surgery using MRI was 55.2% type A, 33.8% type B, 4.7% type C, and 6.3% type D. The postoperative lateral and medial breaches were noted, and these did not cause any complications requiring revision surgery. The mean pedicle diameter before surgery for T1-L5 vertebral pedicles was between 3.79 (1.44) mm and 5.68 (1.64) mm. The mean expanding diameter of pedicles after surgery for T1-L5 vertebral pedicles ranged from 1.90 (0.39) mm to 2.92 (0.28) mm, which corresponds to the extension of the pedicle diameter in the mean range of 47% (4.1)-71% (3.0). We noted that the mean vertebral pedicle expansion was 49% in Group 1, 52% in Group 2, and 62% in Group 3 (N.S.), and the mean expansion for 7.0 mm screw pedicles was 78%. CONCLUSIONS: Our study confirms that there is a wide range of expansion of the vertebral pedicle during screw insertion (up to 78%) with a low risk of lateral or medial breaches and without an increased risk of complications. The larger the diameter of the screw inserted into the pedicle, the more the pedicle expands. Pedicle measurements by preoperative MRI may be helpful for sufficient reliability in preoperative planning.

The Child's Age and the Size of the Curvature Do Not Affect the Accuracy of Screw Placement with the Free-Hand Technique in Spinal Deformities in Children and Adolescents.

BACKGROUND: The current method of treatment of spinal deformities would be almost impossible without pedicle screws (PS) placement. There are only a few studies evaluating the safety of PS placement and possible complications in children during growth. The present study was carried out to evaluate the safety and accuracy of PS placement in children with spinal deformities at any age using postoperative computed tomography (CT) scans. METHODS: 318 patients (34 males and 284 females) who underwent 6358 PS fixations for pediatric spinal deformities were enrolled in this multi-center study. The patients were divided into three age groups: less than 10 years old, 11-13 years old, and 14-18 years old. These patients underwent postoperative CT scans and were analyzed for pedicle screw malposition (anterior, superior, inferior, medial, and lateral breaches). RESULTS: The breach rate was 5.92% for all pedicles. There were 1.47% lateral and 3.12% medial breaches for all pedicles with tapping canals, and 2.66% lateral and 3.84% medial breaches for all pedicles without a tapping canal for the screw. Of the 6358 screws placed in the thoracic, lumbar, and sacral spine, 98% of the screws were accurately placed (grade 0, 1, and juxta pedicular). A total of 56 screws (0.88%) breached more than 4 mm (grade 3), and 17 (0.26%) screws were replaced. No new and permanent neurological, vascular, or visceral complications were encountered. CONCLUSIONS: The free-hand technique for pedicle screw placement in the acceptable and safety zone in pedicles and vertebral bodies was 98%. No complications associated with screw insertion in growth were noted. The free-hand technique for pedicle screw placement can be safely used in patients at any age. The screw accuracy does not depend on the child's age nor the size of the deformity curve. Segmental instrumentation with posterior fixation in children with spinal deformities can be performed with a very low complication rate. Navigation of the robot is only an auxiliary tool in the hands of the surgeons, and the result of the work ultimately depends on the surgeons.

Beam-Like rods do not Provide Additional Improvement to Thoracic Kyphosis Restoration when Compared to Sagittal Reinforced rods in Adolescents Undergoing Spinal Fusion with Pedicle Screw Instrumentation for Idiopathic Scoliosis.

OBJECTIVE: Operative treatment of adolescent idiopathic scoliosis (AIS) with posterior spinal fusion aims for three-dimensional correction of coronal curve and thoracic kyphosis. Our aim was to compare two different designs of asymmetrical rods in adolescents who underwent a posterior spinal fusion with pedicle screw instrumentation for AIS with an emphasis on thoracic kyphosis restoration. METHODS: This study was made with 76 consecutive adolescents (mean age 15.6 years, SD 2.0). Thirty-nine patients were operated with sagittal reinforced rods and 37 patients were operated with beam-like rods. The clinical and radiological results were assessed preoperatively, postoperatively, and during the follow-up visits at the outpatient clinic 6 months and 2 years after the surgery. RESULTS: At the last follow-up, the mean (SD) major thoracic curves were 13° (6.2°) and 13° (6.0°) (P = 0.717). Correction percentages were 75% in the sagittal reinforced group and 73% in the beam-like rod group (P = 0.517). The mean (SD) thoracic kyphosis was 24° (11°) and 22° (7.8°) at the two year follow-up in the sagittal reinforced rod group and beamlike rod group (P = 0.517). There was a slight negative correlation between the major curve correction and thoracic kyphosis change in both groups, although this was not statistically significant (R = -0.19, P = 0.094 in the sagittal reinforced rod group, R=-0.16, P = 0.180 in the beam like rod group). CONCLUSIONS: There are no significant differences in the coronal or sagittal deformity restoration in adolescent patients who underwent a posterior spinal fusion with sagittal reinforced rods and beam-like rods for adolescent idiopathic scoliosis.

Matched Comparison of Magnetically Controlled Growing Rods with Traditional Growing Rods in Severe Early-Onset Scoliosis of ≥90°: An Interim Report on Outcomes 2 Years After Treatment.

BACKGROUND: Severe early-onset scoliosis (EOS) is managed surgically but represents a challenge due to limited implant fixation points, large curve size, and fragile patients with comorbidities. Magnetically controlled growing rods (MCGRs) have the advantage of avoiding surgical intervention for routine lengthening, but their ability to address severe EOS has not been studied, to our knowledge. METHODS: A retrospective review of a prospectively collected international database identified 44 children with severe (≥90°) EOS treated with MCGRs who met our study criteria. Etiology, age, and sex-matched patients treated with traditional growing rods (TGRs) were identified from the same database. Patients were evaluated at a 2-year follow-up. No patients with vertically expandable prosthetic titanium ribs (VEPTRs) were included. The health-related quality of life was evaluated with the 24-Item Early Onset Scoliosis Questionnaire (EOSQ-24). RESULTS: The mean preoperative major coronal curve was 104° in the MCGR group and 104° in the TGR group. At the 2-year follow-up, the mean major coronal curves were 52° and 66° (p = 0.001), respectively. The mean T1-T12 heights were 155 mm and 152 mm preoperatively and 202 mm and 192 mm at the 2-year follow-up (p = 0.088). According to Kaplan-Meier analysis, the 2-year unplanned-revision-free survival was 91% in the MCGR group and 71% in the TGR group (p < 0.005). The 2-year score in the EOSQ-24 pulmonary function domain was better in the MCGR group. There were no other significant differences in the EOSQ-24 scores between the groups. CONCLUSIONS: MCGRs for severe EOS provided significantly better major curve correction with significantly fewer unplanned revisions than TGRs at a 2-year follow-up. LEVEL OF EVIDENCE: Therapeutic Level III. See Instructions for Authors for a complete description of levels of evidence.

Range of motion and strength after surgery for brachial plexus birth palsy.

BACKGROUND: There is little information about the range of motion (ROM) and strength of the affected upper limbs of patients with permanent brachial plexus birth palsy. PATIENTS AND METHODS: 107 patients who had brachial plexus surgery in Finland between 1971 and 1998 were investigated in this population-based, cross-sectional, 12-year follow-up study. During the follow-up, 59 patients underwent secondary procedures. ROM and isometric strength of the shoulders, elbows, wrists, and thumbs were measured. Ratios for ROM and strength between the affected and unaffected sides were calculated. RESULTS: 61 patients (57%) had no active shoulder external rotation (median 0° (-75-90)). Median active abduction was 90° (1-170). Shoulder external rotation strength of the affected side was diminished (median ratio 28% (0-83)). Active elbow extension deficiency was recorded in 82 patients (median 25° (5-80)). Elbow flexion strength of the affected side was uniformly impaired (median ratio 43% (0-79)). Median active extension of the wrist was 55° (-70-90). The median ratio of grip strength for the affected side vs. the unaffected side was 68% (0-121). Patients with total injury had poorer ROM and strength than those with C5-6 injury. Incongruity of the radiohumeral joint and avulsion were associated with poor strength values. INTERPRETATION: ROM and strength of affected upper limbs of patients with surgically treated brachial plexus birth palsy were reduced. Patients with avulsion injuries and/or consequent joint deformities fared worst.

Outcomes of early hemivertebrectomy in children with congenital scoliosis: A prospective follow-up study.

BACKGROUND AND OBJECTIVE: Posterolateral hemivertebrectomy with monosegmental instrumentation at an early age is an established method to correct congenital scoliosis but is associated with a relatively high risk of instrumentation failure and health-related quality of life outcomes are not available. We aimed to investigate the effects hemivertebrectomy with postoperative hip spica cast on complications and health-related quality of life in small children with congenital scoliosis. METHODS: A follow-up study of 30 children (at mean age = 3.4 years, range = 1.0-8.5 years) undergoing posterolateral hemivertebrectomy and short pedicle instrumentation. Children were immobilized postoperative with hip spica cast for 6 weeks and immobilization for 4 months using a rigid thoracolumbosacral orthosis. The Caregivers filled out Scoliosis Research Society 24 outcome questionnaire preoperatively, at 6 months, and at final follow-up visit on behalf of their child. Standing radiographs were obtained preoperatively, postoperatively in the cast, and standing at final follow-up. RESULTS: Mean major curve was 41° (range = 26°-87°) preoperatively and was corrected to 14° (4.0°-35°) at final follow-up. Eight (27%) children had postoperative complications, including three (10%) deep surgical site infections. The Scoliosis Research Society 24 back domain showed an improvement from a mean of 3.8 preoperatively to 4.4 at final follow-up (p < 0.001). Function from back condition domain showed a significant deterioration from 4.2 preoperatively to 3.7 at 6 months (p = 0.020) but improved back to baseline at final follow-up (4.2, p = 0.0022 6 months vs final follow-up). CONCLUSIONS: Hemivertebrectomy with short instrumentation resulted into 64% correction of scoliosis and improved health-related quality of life in back pain and function domains.

Primary malignant bone tumours of spine and pelvis in children.

PURPOSE: Axial malignant bone tumours are rare in children and adolescents, and their prognosis is still relatively poor due to non-specific symptoms, such as back or groin pain, which may result in late hospital presentation. Therefore, it is very important to raise awareness regarding this pathology. METHODS: We performed a narrative review, including scientific publications published in English. We searched Medline and Google Scholar databases for information on the incidence and prognosis of axial malignant bone tumours in children and adolescents (< 18 years). Outcomes of different surgical management strategies and reconstruction options were assessed. RESULTS: The incidence of primary malignant bone tumours before the age of 18 years is approximately five per one million population; around 25% of these tumours are located in the axial skeleton. With a five-year survival rate of 50%, tumours in an axial location (chest cage, spine, pelvis) are associated with a poorer prognosis than tumours in more peripheral locations. En bloc excision with clear margins has been shown to improve local control and overall survival, even though obtaining adequate surgical margins is difficult due to the close location of large neurovascular structures and other major organs. Spinal reconstruction options include instrumented fusion with allograft or expandable cage. Pelvic reconstruction is needed in internal hemipelvectomy, and the options include biological, endoprosthetic reconstructions, hip transposition, arthrodesis or creation of pseudoarthrosis and lumbopelvic instrumentation. CONCLUSION: Early diagnosis, a timely adequate multidisciplinary management, appropriate en bloc excision, and reconstruction improve survival and quality of life in these patients. LEVEL OF EVIDENCE: V.

Results of Conservative and Surgical Management in Children with Idiopathic and Nonidiopathic Os Odontoideum.

OBJECTIVE: The outcomes of conservative and operative treatment of os odontoideum in children remain unclear. Our objective was to study the outcomes of conservative and surgical treatment of idiopathic os odontoideum in children and compare these outcomes in age- and treatment-matched nonidiopathic children with os odontoideum. METHODS: A retrospective multicenter review identified 102 children with os odontoideum, of whom 44 were idiopathic with minimum 2-year follow-up. Ten patients were treated conservatively, and 34 underwent spinal arthrodesis. Both groups were matched with nonidiopathic patients by age and type of treatment. Cervical arthrodesis was recommended for patients with increased atlantoaxial distance or reduced space available for the cord in flexion-extension radiographs. RESULTS: All 20 children undergoing conservative treatment remained asymptomatic during follow-up, but 1 nonidiopathic patient developed cervical instability. The idiopathic group had significantly less severe radiographic cervical instability and less neurologic complications than the nonidiopathic group (P < 0.05 for all comparisons). Thirty-three (97%) patients in the idiopathic group and 32 (94%) patients in the nonidiopathic group (94%) had spinal fusion at final follow-up (P = 0.55). The risk of complications (15% vs. 41%; odds ratio 0.234, 95% confidence interval 0.072-0.757, P = 0.015) and nonunion (6% vs. 24%; odds ratio 0.203, 95% confidence interval 0.040-0.99, P = 0.040) were significantly lower in the idiopathic than in the nonidiopathic group. Idiopathic children undergoing rigid fixation achieved spinal fusion. CONCLUSIONS: Idiopathic patients with stable atlantoaxial joint at presentation remained asymptomatic and intact during conservative treatment. Idiopathic children with os odontoideum undergoing spinal arthrodesis had significantly fewer complications and nonunion than nonidiopathic children. LEVEL OF EVIDENCE: III.

Standard and magnetically controlled growing rods for the treatment of early onset scoliosis.

Distraction based spinal instrumentation represents the most common and standard surgical technique to correct early onset scoliosis (EOS), i.e., scoliosis which has been diagnosed before the age of 10 years. Surgical treatment of EOS aims at controlling spinal deformity while maintaining spinal growth which is mandatory for the development of normal lung capacity. To achieve these goals the spinal instrumentation needs to be distracted to facilitate spinal growth during treatment. Distraction can be obtained by repeated surgical lengthenings (traditional growing rods, TGRs) or using magnetically controlled growing rods (MCGRs), which can be lengthened using external remote controller on an outpatient basis. The outcomes of TGR instrumentation for EOS are well described with follow-up until skeletal maturity: normal spinal growth can be maintained, 40-50% of the scoliosis can be corrected, but there is an over 50% risk of complications including deep wound infection, rod failure, and instrumentation pull-out. MCGR instrumentation may reduce the risk of wound related complications, provides similar deformity correction, but may not provide as much spinal growth. Metallosis around the instrumentation necessitates MCGR removal and definitive final instrumented fusion at the end of growth friendly management. Even severe EOS can be treated using distraction based spinal instrumentation.

Clinical and Radiological Outcomes of Less Invasive Temporary Internal Distraction Followed by Staged Pedicle Screw Instrumentation in Adolescents with Severe Idiopathic Scoliosis at 2-Year Minimum Follow-Up.

OBJECTIVES: Temporary placement of an internal distraction rod is one of the surgical options in the treatment of severe scoliosis to avoid preoperative halo traction or vertebral column resection. This technique can be applied in a single session or staged. The aim of our study was to report the outcomes of less-invasive temporary internal distraction (LI-TID) in the surgical treatment of adolescents with severe idiopathic scoliosis (IS). METHODS: We performed a single-center, retrospective study of 22 adolescents (19 girls; mean age, 14.8 ± 2.0 years) with severe IS (major curve, ≥90°) who had undergone LI-TID, followed by staged pedicle screw instrumentation, with a minimum of 2 years of follow-up available. The demographic data, radiographic outcomes, pulmonary function test results, perioperative data, revised 22-item Scoliosis Research Society Outcomes questionnaire results, complications, and neuromonitoring data were collected. RESULTS: The average major curve was 120° (range, 90°-160°) preoperatively and 59° (range, 29°-69°) at the final follow-up examination. Thoracic kyphosis (T5-T12) had improved from an average of 80° preoperatively to 33° postoperatively (range, 22°-69°), and the spinal height (T1-S1) had increased from 332 mm (range, 198-432 mm) preoperatively to 405 mm (range, 258-495 mm) at the final follow-up visit. Of the 22 patients, 5 (22.7%) had experienced an intraoperative neuromonitoring change without postoperative neurologic deficits, and 2 had developed superior mesenteric artery syndrome, with resolution after conservative treatment. The mean percentage of the predicted forced vital capacity had improved from 44.5% ± 11.3% to 66.5 ± 10.8 at final follow-up (P < 0.05). The total questionnaire score had improved significantly from 2.9 ± 0.61 to 4.1 ± 0.44 (P < 0.05). CONCLUSION: The results from the present study have shown that staged LI-TID, followed by pedicle screw instrumentation, is safe and effective in adolescents with severe IS, with improvements in spinal deformity, pulmonary function, and health-related quality of life.

Cerebral palsy with dislocated hip and scoliosis: what to deal with first?

PURPOSE: Hip dislocation and scoliosis are common in children with cerebral palsy (CP). Hip dislocation develops in 15% and 20% of children with CP, mainly between three and six years of age and especially in the spastic and dyskinetic subtypes. The risk of scoliosis increases with age and increasing disability as expressed by the Gross Motor Function Score. METHODS: A hip surveillance programme and early surgical treatment have been shown to reduce the hip dislocation, but it remains unclear if a similar programme could reduce the need for neuromuscular scoliosis. When hip dislocation and neuromuscular scoliosis are co-existent, there appears to be no clear guidelines as to which of these deformities should be addressed first: hip or spine. RESULTS: Hip dislocation or windswept deformity may cause pelvic obliquity and initiate scoliosis, while neuromuscular scoliosis itself leads to pelvic obliquity and may increase the risk of hip dislocation especially on the high side. It remains unclear if treating imminent hip dislocation can prevent development of scoliosis and vice versa, but they may present at the same time for surgery. Current expert opinion suggests that when hip dislocation and scoliosis present at the same time, scoliosis associated pelvic obliquity should be corrected before hip reconstruction. If the patient is not presenting with pelvic obliquity the more symptomatic condition should be addressed first. CONCLUSION: Early identification of hip displacement and neuromuscular scoliosis appears to be important for better surgical outcomes.

Preemptive Pregabalin in Children and Adolescents Undergoing Posterior Instrumented Spinal Fusion: A Double-Blinded, Placebo-Controlled, Randomized Clinical Trial.

BACKGROUND: Pregabalin as part of a multimodal pain-management regimen has been shown to reduce opioid consumption after spinal surgery in adults but it is unclear whether this is also true in adolescents. Pregabalin has been found to have neuroprotective effects and therefore could have a positive impact on pain after spinal deformity surgery. We conducted a randomized, double-blinded, placebo-controlled clinical trial of adolescent patients undergoing spinal fusion to evaluate the short-term effects of pregabalin on postoperative pain and opioid consumption. METHODS: Adolescents with adolescent idiopathic scoliosis, Scheuermann kyphosis, or spondylolisthesis who were scheduled for posterior spinal fusion with all-pedicle-screw instrumentation were randomized to receive either pregabalin (2 mg/kg twice daily) or placebo preoperatively and for 5 days after surgery. The patients ranged from 10 to 21 years of age. The primary outcome was total opioid consumption as measured with use of patient-controlled analgesia. Postoperative pain scores and opioid-related adverse effects were evaluated. RESULTS: Sixty-three of 77 eligible patients were included and analyzed. Cumulative oxycodone consumption per kilogram did not differ between the study groups during the first 48 hours postoperatively, with a median of 1.44 mg/kg (95% confidence interval [CI],1.32 to 1.67 mg/kg) in the pregabalin group and 1.50 mg/kg (95% CI, 1.39 to 1.79 mg/kg) in the placebo group (p = 0.433). A subgroup analysis of 51 patients with adolescent idiopathic scoliosis showed the same result, with a mean of 1.45 mg/kg (95% CI, 1.24 to 1.65 mg/kg) in the pregabalin group and 1.59 mg/kg (95% CI, 1.37 to 1.82 mg/kg) in the placebo group (p = 0.289). Total oxycodone consumption per hour (mg/kg/hr) was not different between the groups over the time points (p = 0.752). The postoperative pain scores did not differ significantly between the groups (p = 0.196). CONCLUSIONS: The use of perioperative pregabalin does not reduce the postoperative opioid consumption or pain scores in adolescents after posterior spinal fusion surgery. LEVEL OF EVIDENCE: Therapeutic Level I. See Instructions for Authors for a complete description of levels of evidence.

Surgical and Health-related Quality-of-Life Outcomes of Growing Rod "Graduates" With Severe versus Moderate Early-onset Scoliosis.

STUDY DESIGN: A retrospective review of a prospective, multicenter database. OBJECTIVE: The aim of this study was to compare surgical and quality-of-life outcomes at the end of growing rod treatment in patients with severe versus moderate early-onset scoliosis (EOS). SUMMARY OF BACKGROUND DATA: Knowledge of the outcomes of severe EOS after growth-friendly treatment is limited because this condition is uncommon. METHODS: We identified 40 children with severe EOS (major curve ≥90°) treated with growing rods before age 10 with minimum 2-year follow-up after last lengthening or final fusion. From the same registry, we matched 40 patients with moderate EOS (major curve < 90°). Twenty-seven patients in the severe group and 12 in the moderate group underwent final fusion (P < 0.001). RESULTS: Mean preoperative curves were 102° (range, 90°-139°) in the severe group and 63° (range, 33°-88°) in the moderate group (P < 0.001). At final follow-up, mean curves were 56° (range, 10°-91°) and 36° (range, 12°-89°), respectively (P < 0.001). Fourteen (35%) children in the severe group and 32 (80%) in the moderate group had scoliosis of < 45° at final follow-up [risk ratio (RR), 0.44; 95% confidence interval (95% CI), 0.20-0.57]. At final follow-up, 30 (75%) children in the severe group and 35 (88%) in the moderate group had achieved T1-T12 length of ≥18 cm (RR, 0.86; 95% CI, 0.70-1.09). Thirty-five children in the severe group and 26 in the moderate group had at least one complication (RR, 1.35; 95% CI, 1.05-1.73). Mean 24-Item Early-Onset Scoliosis Questionnaire scores were similar between groups at final follow-up. CONCLUSION: Delaying surgery until the major curve has progressed beyond 90° is associated with larger residual deformity and more complications than treating at a lesser curve magnitude. Quality-of-life outcomes were similar between those with severe and moderate EOS. LEVEL OF EVIDENCE: 3.

Os Odontoideum in Children: Treatment Outcomes and Neurological Risk Factors.

BACKGROUND: Treatment outcomes and risk factors for neurological deficits in pediatric patients with an os odontoideum are unclear. METHODS: We reviewed the data for 102 children with os odontoideum who were managed at 11 centers between 2000 and 2016 and had a minimum duration of follow-up of 2 years. Thirty-one children had nonoperative treatment, and 71 underwent instrumented posterior cervical spinal arthrodesis for the treatment of C1-C2 instability. Nonoperative treatment consisted of observation (n = 29) or immobilization with a cervical collar (n = 1) or halo body jacket (n = 1). Surgical treatment consisted of atlantoaxial (n = 50) or occipitocervical (n = 21) arthrodesis. One patient also underwent transoral odontoidectomy. RESULTS: Thirty children (29%) presented with neurological deficits, 28 of whom had radiographic atlantoaxial instability (atlantoaxial distance >5 mm) or limited space (≤13 mm) available for the spinal cord (risk ratio, 7.8 [95% confidence interval, 2.0 to 31] compared with children with no radiographic risk factors). The 27 children without neurological deficits or atlantoaxial instability at presentation underwent nonoperative treatment and remained asymptomatic. Of the initial nonoperative cohort, one child developed atlantoaxial instability, and another had a persistent neurological deficit; both children underwent spinal arthrodesis during the study period. One child with cervical instability declined surgery and remained asymptomatic. Spinal fusion occurred in 68 patients in the surgical group by the end of the study period (mean, 3.7 years; range, 2.0 to 11.8 years). Surgical complications occurred in 21 children, including nonunion in 12, new neurological deficits in 4, cerebrospinal fluid leak in 2, symptomatic instrumentation requiring removal 2, and vertebral artery injury in 1. Nine children underwent revision surgery. In the surgical group, Japanese Orthopaedic Association neurological function scores improved significantly from preoperatively to the latest follow-up for the upper extremities (p = 0.026) and lower extremities (p = 0.007). CONCLUSIONS: The risk of developing a neurological deficit was strongly associated with atlantoaxial instability and limited space available for the spinal cord in children with os odontoideum. Nonoperative treatment was safe for asymptomatic patients without atlantoaxial instability. Spinal arthrodesis resolved the neurological deficits of children with symptomatic os odontoideum. LEVEL OF EVIDENCE: Therapeutic Level III. See Instructions for Authors for a complete description of levels of evidence.

Results of growth-friendly management of early-onset scoliosis in children with and without skeletal dysplasias: a matched comparison.

AIMS: The aim of this study was to compare the surgical and quality-of-life outcomes of children with skeletal dysplasia to those in children with idiopathic early-onset scoliosis (EOS) undergoing growth-friendly management. PATIENTS AND METHODS: A retrospective review of two prospective multicentre EOS databases identified 33 children with skeletal dysplasia and EOS (major curve ≥ 30°) who were treated with growth-friendly instrumentation at younger than ten years of age, had a minimum two years of postoperative follow-up, and had undergone three or more lengthening procedures. From the same registries, 33 matched controls with idiopathic EOS were identified. A total of 20 children in both groups were treated with growing rods and 13 children were treated with vertical expandable prosthetic titanium rib (VEPTR) instrumentation. RESULTS: Mean preoperative major curves were 76° (34° to 115°) in the skeletal dysplasia group and 75° (51° to 113°) in the idiopathic group (p = 0.55), which were corrected at final follow-up to 49° (13° to 113°) and 46° (12° to 112°; p = 0.68), respectively. T1-S1 height increased by a mean of 36 mm (0 to 105) in the skeletal dysplasia group and 38 mm (7 to 104) in the idiopathic group at the index surgery (p = 0.40), and by 21 mm (1 to 68) and 46 mm (7 to 157), respectively, during the distraction period (p = 0.0085). The skeletal dysplasia group had significantly worse scores in the physical function, daily living, financial impact, and parent satisfaction preoperatively, as well as on financial impact and child satisfaction at final follow-up, than the idiopathic group (all p < 0.05). The domains of the 24-Item Early-Onset Scoliosis Questionnaire (EOSQ24) remained at the same level from preoperative to final follow-up in the skeletal dysplasia group (all p > 0.10). CONCLUSION: Children with skeletal dysplasia gained significantly less spinal growth during growth-friendly management of their EOS and their health-related quality of life was significantly lower both preoperatively and at final follow-up than in children with idiopathic EOS. Cite this article: Bone Joint J 2019;101-B:1563-1569.