Intraoperative navigation increases the projected lifetime cancer risk in patients undergoing surgery for adolescent idiopathic scoliosis.

BACKGROUND CONTEXT: Adolescent idiopathic scoliosis (AIS) is a common condition, often requiring surgical correction. Computed tomography (CT) based navigation technologies, which rely on ionizing radiation, are increasingly being utilized for surgical treatment. Although this population is highly vulnerable to radiation, given their age and female predominance, there is little available information elucidating modeled iatrogenic cancer risk. PURPOSE: To model lifetime cancer risk associated with the use of intraoperative CT-based navigation for surgical treatment of AIS. STUDY DESIGN/SETTING: This retrospective cross-sectional study took place in a quaternary care academic pediatric hospital in the United States. PATIENT SAMPLE: Adolescents aged 10-18 who underwent posterior spinal fusion for a diagnosis of AIS between July 2014 and December 2019. OUTCOMES MEASURES: Effective radiation dose and projected lifetime cancer risk associated with intraoperative doses of ionizing radiation. METHODS: Clinical and radiographic parameters were abstracted, including total radiation dose during surgery from flat plate radiographs, fluoroscopy, and intraoperative CT scans. Multivariable regression analysis was used to assess differences in radiation exposure between patients treated with conventional radiography versus intraoperative navigation. Radiation exposure was translated into lifetime cancer risk using well-established algorithms. RESULTS: In total, 245 patients were included, 119 of whom were treated with navigation. The cohort was 82.9% female and 14.4 years of age. The median radiation exposure (in millisieverts, mSv) for fluoroscopy, radiography, and navigation was 0.05, 4.14, and 8.19 mSv, respectively. When accounting for clinical and radiographic differences, patients treated with intraoperative navigation received 8.18 mSv more radiation (95%CI: 7.22-9.15, p<.001). This increase in radiation projects to 0.90 iatrogenic malignancies per 1,000 patients (95%CI 0.79-1.01). CONCLUSIONS: Ours is the first work to define cancer risk in the setting of radiation exposure for navigated AIS surgery. We project that intraoperative navigation will generate approximately one iatrogenic malignancy for every 1,000 patients treated. Given that spine surgery for AIS is common and occurs in the context of a multitude of other radiation sources, these data highlight the need for radiation budgeting protocols and continued development of lower radiation dose technologies. LEVEL OF EVIDENCE: Therapeutic, III.

Two-Surgeon Multidisciplinary Approach to Pediatric Cervical Spinal Fusion: A Single-Institution Series and Review of the Literature.

BACKGROUND: A collaborative 2-surgeon approach is becoming increasingly popular in surgery but is not widely used for pediatric cervical spine fusions. The goal of this study is to present a large single-institution experience with pediatric cervical spinal fusion using a multidisciplinary 2-surgeon team, including a neurosurgeon and an orthopedic surgeon. This team-based approach has not been previously reported in the pediatric cervical spine literature. METHODS: A single-institution review of pediatric cervical spine instrumentation and fusion performed by a surgical team composed of neurosurgery and orthopedics during 2002-2020 was performed. Demographics, presenting symptoms and indications, surgical characteristics, and outcomes were recorded. Particular focus was given to describe the primary surgical responsibility of the orthopedic surgeon and the neurosurgeon. RESULTS: A total of 112 patients (54% male) with an average age of 12.1 (range 2-26) years met the inclusion criteria. The most common indications for surgery were os odontoideum with instability (n=21) and trauma (n=18). Syndromes were present in 44 (39%) cases. Fifty-five (49%) patients presented with preoperative neurological deficits (26 motor, 12 sensory, and 17 combined deficits). At the time of the last clinical follow-up, 44 (80%) of these patients had stabilization or resolution of their neurological deficit. There was 1 new postoperative neural deficit (1%). The average time between surgery and successful radiologic arthrodesis was 13.2±10.6 mo. A total of 15 (13%) patients experienced complications within 90 days of surgery (2 intraoperative, 6 during admission, and 7 after discharge). CONCLUSIONS: A multidisciplinary 2-surgeon approach to pediatric cervical spine instrumentation and fusion provides a safe treatment option for complex pediatric cervical cases. It is hoped that this study could provide a model for other pediatric spine groups interested in implementing a multi-specialty 2-surgeon team to perform complex pediatric cervical spine fusions. LEVEL OF EVIDENCE: Level IV-case series.

Do Adolescent Idiopathic Scoliosis Patients With Vitamin D Deficiency Have Worse Spine Fusion Outcomes?

BACKGROUND: Prior research has shown that patients with adolescent idiopathic scoliosis (AIS) have a higher prevalence of vitamin D deficiency compared with healthy peers. In adult orthopaedic populations, vitamin D deficiency has been shown to be a risk factor for higher reported pain and lower function. We investigated whether there was an association between vitamin D levels and AIS patient-reported outcomes, as measured by the Scoliosis Research Society (SRS-30) questionnaire. METHODS: This was a single-center, cross-sectional study. Postoperative AIS patients were prospectively recruited during routine follow-up visits, 2 to 10 years after spine fusion. Vitamin D levels were measured by serum 25-hydroxyvitamin D (ng/mL). Patients were categorized based on vitamin D level: deficient (<20 ng/mL), insufficient (20 to 29 ng/mL), or sufficient (≥30 ng/mL). The correlation between vitamin D levels and SRS-30 scores was analyzed using multivariable analysis and pair-wise comparisons using Tukey method. RESULTS: Eighty-seven AIS patients (83% female) were enrolled who presented at median 3 years (interquartile range: 2 to 5 y; range: 2 to 10 y) after spine fusion. Age at time of surgery was mean 15 (SD±2) years. Major coronal curves were a mean of 57 (SD±8) degrees preoperatively and 18 (SD±7) degrees postoperatively. It was found that 30 (34%) of patients were vitamin D sufficient, 33 (38%) were insufficient, and 24 (28%) were deficient. Although there was no correlation between vitamin D level and Pain, Mental Health, or Satisfaction domains ( P >0.05), vitamin D-deficient patients were found to be younger ( P <0.001) and had lower SRS-30 function ( P =0.002), Self-image ( P <0.001), and total scores ( P =0.003). CONCLUSIONS: AIS patients with vitamin D deficiency (<20 ng/mL) are more likely to be younger age at time of surgery, and report lower Function, Self-image, and Total SRS-30 scores postoperatively. Further work is needed to determine whether vitamin D supplementation alters curve progression and patient outcomes. LEVEL OF EVIDENCE: Level II-prognostic study.

Bracing for juvenile idiopathic scoliosis: retrospective review from bracing to skeletal maturity.

BACKGROUND: Juvenile idiopathic scoliosis (JIS) outcomes with brace treatment are limited with poorly described bracing protocols. Between 49 and 100% of children with JIS will progress to surgery, however, young age, long follow-up, and varying treatment methods make studying this population difficult. The purpose of this study is to report the outcomes of bracing in JIS treated with a Boston brace™ and identify risk factors for progression and surgical intervention. METHODS: This is a single-center retrospective review of 175 patients with JIS who initiated brace treatment between the age of 4 and 9 years. A cohort of 140 children reached skeletal maturity; 91 children had surgery or at least 2 year follow-up after brace completion. Standard in-brace protocol for scoliosis 320° was a Boston brace for 18-20 h/day after MRI (n = 82). Family history, MRI abnormalities, comorbidities, curve type, curve magnitude, bracing duration, number of braces, compliance by report, and surgical interventions were recorded. RESULTS: Children were average 7.9 years old (range 4.1-9.8) at the initiation of bracing. The Boston brace™ was prescribed in 82 patients and nine used night bending brace. Mid-thoracic curves (53%) was the most frequent deformity. Maximum curve at presentation was on average 30 ± 9 degrees, in-brace curve angle was 16 ± 8 degrees, and in-brace correction was 58 ± 24 percent. Patients were braced an average of 4.6 ± 1.9 years. 61/91 (67%) went on to posterior spinal fusion at 13.3 ± 2.1 (range 9.3-20.9) years and curve magnitude of 61 ± 12 degrees. Of those that underwent surgery, 49/55 (86%) progressed > 10°, 6/55 (11%) stabilized within 10°, and 0/55 (0%) improved > 10° with brace wear. No children underwent growth-friendly posterior instrumentation. Of the 28 who did not have surgical correction, 3 (11%) progressed > 10°, 13/28 (46%) stabilized within 10°, and 12/28 (43%) improved > 10° with brace wear. CONCLUSIONS: This large series of JIS patients with bracing followed to skeletal maturity with long-term follow-up. Surgery was avoided in 33% of children with minimal to no progression, and no child underwent posterior growth-friendly constructs. Risk factors of needing surgery were noncompliance and larger curves at presentation.

Insurance types are correlated with baseline patient-reported outcome measures in patients with adult spinal deformity.

BACKGROUND: Patient-reported outcome measures (PROMs) are increasingly recognized as a key component of healthcare value, allowing comparison of therapeutic impact across different specialties. Prior literature suggests that insurance type may be associated with differing baseline PROMs among patients with degenerative conditions, including lumbar stenosis and hip arthritis. This association, however, has not been investigated for adult spinal deformity (ASD). METHODS: Baseline PROMs were reviewed from 207 patients with ASD presenting for treatment between 2015 and 2019. The Patient-Reported Outcomes Measurement Information System (PROMIS) Physical Function Short Form 10a (PF10a), PROMIS Global-Mental, PROMIS Global-Physical, and visual analogue scale (VAS) for back and leg pain were assessed. Negative binomial regression was used to determine the impact of sociodemographic factors, including insurance type, on severity of symptoms and degree of disability at baseline. RESULTS: Mean age of the study population was 62.2 +/- 15 years, with 61.8 % male prevalence. The Medicaid population had a greater proportion of Hispanic and non-English speaking patients, compared to commercially insured patients. Medicaid insured patients had significantly greater VAS low back pain scores compared with commercially insured individuals (IRR 1.535, 95 % CI 1.122-2.101, p = 0.007). CONCLUSIONS: Medicaid insured patients demonstrated worse baseline PROMs at presentation with ASD, as compared to commercially insured or Medicare patients. Stakeholders across spine care delivery should elucidate the etiology of baseline disparities in ASD patients, as they may result from health system asymmetries. In an ecosystem moving toward value-driven treatment algorithms, accounting for and addressing these differences will be necessary to provide equitable care for ASD populations.

Early outcomes of growth friendly instrumentation in children with Williams syndrome.

PURPOSE: Although scoliosis and kyphosis have been associated with Williams Syndrome (WS), no previous literature has reported on surgical treatment for early onset scoliosis (EOS) in WS. The aim of this case series is to report on the outcomes of spine deformity surgery in patients with EOS and WS and any perioperative anesthetic or cardiovascular complications. METHODS: One multicenter database was queried for all patients with WS who underwent growth-friendly (GF) treatment before age 12 between 2000 and 2017. Demographics, surgical, and growth-friendly data were queried. Radiographs were measured for curve magnitude, T1-T12 length, and T1-S1 length. RESULTS: Seven patients were analyzed (3 males, 4 females). Patients were at a median age of 2.8 years at initial surgery with median follow-up 3.6 years (range 2.0-12 years) after index surgery. The initial surgical treatments were as follows: 2 traditional growing rods (TGR), 2 magnetically controlled growing rods (MCGR), and 3 vertical expandable prosthetic titanium ribs (VEPTR). The median duration of growth-friendly treatment was 5.0 years (range, 2.6-10.4 years) with a median number of 9 device lengthenings. The median improvement in coronal curve magnitude from preoperative to most recent follow-up was 19° (range, 54°-9°). Three patients have completed GF treatment: one underwent definitive fusion, and two are under observation with apparent spontaneous fusion and retain the original GF implants. No peri-operative anesthetic or cardiovascular complications occurred. CONCLUSIONS: Few studies have reported on surgical outcomes in WS patients with EOS. In this case series, 6/7 patients experienced curve improvement with growth-friendly spine instrumentation. This study suggests that growth-friendly instrumentation for severe EOS in WS can be used for control of spinal deformity while allowing for further growth. Associated complications were typical of distraction-based EOS surgical treatment. There were 62 total procedures with general anesthesia, but no perioperative cardiac complications occurred.

Operative Treatment of Cervical Spine Injuries Sustained in Youth Sports.

BACKGROUND: Little data exists on surgical outcomes of sports-related cervical spine injuries (CSI) sustained in children and adolescent athletes. This study reviewed demographics, injury characteristics, management, and operative outcomes of severe CSI encountered in youth sports. METHODS: Children below 18 years with operative sports-related CSI at a Level 1 pediatric trauma center were reviewed (2004 to 2019). All patients underwent morden cervical spine instrumentation and fusion. Clinical, radiographic, and surgical characteristics were analyzed. RESULTS: A total of 3231 patients (mean, 11.3±4.6 y) with neck pain were evaluated for CSI. Sports/recreational activities were the most common etiology in 1358 cases (42.0%). Twenty-nine patients (2.1%) with sports-related CSI (mean age, 14.5 y; range, 6.4 to 17.8 y) required surgical intervention. Twenty-five were males (86%). Operative CSI occurred in football (n=8), wrestling (n=7), gymnastics (n=5), diving (n=4), trampoline (n=2), hockey (n=1), snowboarding (n=1), and biking (n=1). Mechanisms were 27 hyperflexion/axial loading (93%) and 2 hyperextension injuries (7%). Most were cervical fractures (79%) and subaxial injuries (79%). Seven patients (24%) sustained spinal cord injury (SCI) and 3 patients (10%) cord contusion or myelomalacia without neurological deficits. The risk of SCI increased with age (P=0.03). Postoperatively, 2 SCI patients (29%) improved 1 American Spinal Injury Association Impairment Scale Grade and 1 (14%) improved 2 American Spinal Injury Association Impairment Scale Grades. Increased complications developed in SCI than non-SCI cases (mean, 2.0 vs. 0.1 complications; P=0.02). Bony fusion occurred in 26/28 patients (93%) after a median of 7.2 months (interquartile range, 6 to 15 mo). Ten patients (34%) returned to their baseline sport and 9 (31%) to lower-level activities. CONCLUSIONS: The incidence of sports-related CSI requiring surgery is low with differences in age/sex, sport, and injury patterns. Older males with hyperflexion/axial loading injuries in contact sports were at greatest risk of SCI, complications, and permanent disability. Prevention campaigns, education on proper tackling techniques, and neck strength training are required in sports at high risk of hyperflexion/axial loading injury. LEVEL OF EVIDENCE: Level III-retrospective cohort study.

Growth-Friendly Spine Surgery in Arthrogryposis Multiplex Congenita.

BACKGROUND: Arthrogryposis multiplex congenita (AMC) is a condition that describes neonates born with ≥2 distinct congenital contractures. Despite spinal deformity in 3% to 69% of patients, inadequate data exist on growth-friendly instrumentation (GFI) in AMC. Our study objectives were to describe current GFI trends in children with AMC and early-onset scoliosis (EOS) and to compare long-term outcomes with a matched idiopathic EOS (IEOS) cohort to determine whether spinal rigidity or extremity contractures influenced outcomes. METHODS: Children with AMC and spinal deformity of ≥30° who were treated with GFI for ≥24 months were identified from a multicenter EOS database (1993 to 2017). Propensity scoring matched 35 patients with AMC to 112 patients with IEOS with regard to age, sex, construct, and curve. Multivariable linear mixed modeling compared changes in spinal deformity and the 24-item Early Onset Scoliosis Questionnaire (EOSQ-24) across cohorts. Cohort complications and reoperations were analyzed using multivariable Poisson regression. RESULTS: Preoperatively, groups did not differ with regard to age (p = 0.87), sex (p = 0.96), construct (p = 0.62), rate of nonoperative treatment (p = 0.54), and major coronal curve magnitude (p = 0.96). After the index GFI, patients with AMC had reduced percentage of coronal correction (35% compared with 44%; p = 0.01), larger residual coronal curves (49° compared with 42°; p = 0.03), and comparable percentage of kyphosis correction (17% compared with 21%; p = 0.52). In GFI graduates (n = 81), final coronal curve magnitude (55° compared with 43°; p = 0.22) and final sagittal curve magnitude (47° compared with 47°; p = 0.45) were not significantly different at the latest follow-up after definitive surgery. The patients with AMC had reduced T1-S1 length (p < 0.001), comparable T1-S1 growth velocity (0.66 compared with 0.85 mm/month; p = 0.05), and poorer EOSQ-24 scores at the time of the latest follow-up (64 compared with 83 points; p < 0.001). After adjusting for ambulatory status and GFI duration, patients with AMC developed 51% more complications (incidence rate ratio, 1.51 [95% confidence interval (CI), 1.11 to 2.04]; p = 0.009) and 0.2 more complications/year (95% CI, 0.02 to 0.33 more; p = 0.03) compared with patients with IEOS. CONCLUSIONS: Patients with AMC and EOS experienced less initial deformity correction after the index surgical procedure, but final GFI curve magnitudes and total T1-S1 growth during active treatment were statistically and clinically comparable with IEOS. Nonambulatory patients with AMC with longer GFI treatment durations developed the most complications. Multidisciplinary perioperative management is necessary to optimize GFI and to improve quality of life in this complex population. LEVEL OF EVIDENCE: Therapeutic Level III. See Instructions for Authors for a complete description of levels of evidence.

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.

Risk factors for gastrointestinal complications after spinal fusion in children with cerebral palsy.

DESIGN: Prospective cerebral palsy (CP) registry review. OBJECTIVES: (1) Evaluate the incidence/risk factors of gastrointestinal (GI) complications in CP patients after spinal fusion (SF); and (2) investigate the validity of the modified Clavien-Dindo-Sink classification. BACKGROUND: Perioperative GI complications result in increased length of stay (LOS) and patient morbidity/mortality. However, none have analyzed the outcomes of GI complications using an objective classification system. METHODS: A prospective/multicenter CP database identified 425 children (mean, 14.4 ± 2.9 years; range, 7.9-21 years) who underwent SF. GI complications were categorized using the modified Clavien-Dindo-Sink classification. Grades I-II were minor complications and grades III-V major. Patients with and without GI complications were compared. RESULTS: 87 GI complications developed in 69 patients (16.2%): 39 minor (57%) and 30 major (43%). Most common were pancreatitis (n = 45) and ileus (n = 22). Patients with preoperative G-tubes had 2.2 × odds of developing a GI complication compared to oral-only feeders (OR 2.2; 95% CI 0.98-4.78; p = 0.006). Similarly, combined G-tube/oral feeders had 6.7 × odds compared to oral-only (OR 6.7; 95% CI 3.10-14.66; p < 0.001). The likelihood of developing a GI complication was 3.4 × with normalized estimated blood loss (nEBL) ≥ 3 ml/kg/level fused (OR 3.41; 95% CI 1.95-5.95; p < 0.001). Patients with GI complications had more fundoplications (29% vs. 17%; p = 0.03) and longer G-tube fasting periods (3 days vs. 2 days; p < 0.001), oral fasting periods (5 days vs. 2 days; p < 0.001), ICU admissions (6 days vs. 3 days; p = 0.002), and LOS (15 days vs. 8 days; p < 0.001). LOS correlated with the Clavien-Dino-Sink classification. CONCLUSION: Gastrointestinal complications such as pancreatitis and ileus are not uncommon after SF in children with CP. This is the first study to investigate the validity of the modified Clavien-Dindo-Sink classification in GI complications after SF. Our results suggest a correlation between complication severity grade and LOS. The complexity of perioperative enteral nutritional supplementation requires prospective studies dedicated to enteral feeding protocols. LEVEL OF EVIDENCE: Therapeutic-level III.

Clavicular osteotomy in complicated revision of total shoulder arthroplasty: indications, surgical technique, and outcomes.

PURPOSE: Adequate exposure in revision of total shoulder arthroplasty (TSA) is important for optimal prosthesis placement and functional results. A clavicular osteotomy in difficult cases of revision TSA is a useful surgical technique that increases the superior exposure area, provides safer dissection, minimizes damage to the anterior deltoid muscle, improves glenoid access, and allows for superior dislocation of the humeral component. There is a paucity of literature analyzing the clavicular osteotomy during challenging cases of revision TSA. The aims of this study were to describe the application, surgical technique, and outcomes of revision TSA with a clavicular osteotomy. METHODS: This was a retrospective study of consecutive patients who underwent revision TSA with a clavicle osteotomy at a single institution (2004-2016). A curved longitudinal clavicular osteotomy is created parallel to the origin of the anterior deltoid muscle. This allows for lateral reflection of the osteotomy and anterior deltoid muscle to significantly increase superior exposure and reduce damage to remaining deltoid muscle fibres. Osteotomy closure is simple with four or five Nice knot osteosutures. The Constant-Murley score and osteotomy healing were assessed at every follow-up. All complications were reviewed. RESULTS: Forty patients who had a mean age of 63.8 years (range 37-87) at time of surgery and mean follow-up duration of 34 months (range 12-88) were analyzed. Pre-operative Constant-Murley scores improved significantly from 32 ± 19.0 to 58 ± 15.0 (p < 0.001) at one year and 65 ± 13.1 (p < 0.001) at two years. Primary osteotomy healing and callus formation were evident in 95% of cases by three months. Five patients developed post-operative complications (13%) related to the clavicular osteotomy: three mid-diaphyseal clavicular fractures sustained after trauma (8%), one clavicular stress fracture (3%), and case of one loosening (3%). Three patients (8%) required surgical revision of the osteotomy (two internal fixation and one revision osteosuturing). No neurovascular injuries or scapular fractures were encountered. CONCLUSION: A curved longitudinal clavicular osteotomy is beneficial in difficult revision TSA and is another tool in the arsenal of experienced shoulder surgeons who manage these challenging cases. This surgical technique increases glenoid exposure, facilitates superior dislocation of the humeral component, minimizes anterior deltoid damage, and reduces the risk of neurovascular injuries. All clavicular complications occurred within four months prior to osteotomy union, with many sustained due to trauma. However, patients who developed a complication had comparable shoulder function as those without.

Efficacy of bracing in skeletally immature patients with moderate-severe idiopathic scoliosis curves between 40° and 60°.

STUDY DESIGN: Retrospective case-series. OBJECTIVES: To evaluate the outcomes of bracing in skeletally immature patients with moderate-severe idiopathic scoliosis (IS) curves ≥ 40°. BACKGROUND: In contrast to prior beliefs, the recent studies have reported successful outcomes with brace treatment may occur in some patients with moderate-severe scoliosis ≥ 40°. Despite other encouraging case-series, non-operative treatment is rarely attempted and the efficacy of bracing large curves remains uncertain. METHODS: 100 skeletally immature children (mean 11.8 ± 2.36 years; range 6.1-16.5) with IS ≥ 40° were identified. 80 were adolescent IS (80%) and 20 juvenile IS (20%). The Risser plus score was used to evaluate skeletal maturity. 66 children were Risser 0 (66%). SRS-SOSORT outcome guidelines were used: > 5° progression, stabilization between - 5° and 5° and, > 5° improvement. RESULTS: Mean initial Cobb was 45° ± 3.9° (range 40°-59°), with in-brace and  % correction of 30° ± 8.7° (range 7°-48°) and 34 ± 17.5% (range 2-84%), respectively. 57 progressed (57%), 32 stabilized (32%), and 11 improved (11%) after a median of 1.8 years (IQR 1.2-2.9). Open triradiate cartilage at presentation (p = 0.005) and less in-brace correction (p = 0.009) were associated with progression. 58 children (58%) underwent surgery after a mean of 3.0 years (range 0.7-7.3). Surgical patients were younger (11.2 vs. 12.7 years; p = 0.003), more often Risser 0 (79% vs. 48%; p < 0.001); however, presented with similar curves (45° vs. 44°; p = 0.31). Open triradiate cartilage at presentation (OR 15.3; 95% CI 4.3-54.6; p < 0.001) and less in-brace correction (p = 0.03) increased the likelihood of surgery. All 20 JIS patients avoided temporary growth rods, with 18 (90%) eventually requiring surgery. CONCLUSION: Non-operative treatment was successful in 42% of children. Risk factors for surgery were younger age, open triradiates, and less in-brace correction. Bracing can be effective in delaying surgery until skeletal maturity in patients with curves ≥ 40°. Patients should be counseled on the risks and benefits of bracing and surgery. LEVEL OF EVIDENCE: Level IV.

Spinal Deformity in Sotos Syndrome: First Results of Growth-friendly Spine Surgery.

BACKGROUND: Sotos syndrome (SS), or cerebral gigantism, describes children with macrocephaly, craniofacial abnormalities, general overgrowth, ligamentous laxity, developmental delay, and neurological disabilities. Fewer than 500 cases have been reported since Sotos and colleagues described the condition in 1964 and no literature exists on the management of spinal deformity in children under 10 years old.The aims of this study were: (1) to characterize the presentation of spinal deformities in patients with SS; and (2) to provide preliminary results of growth-friendly instrumentation (GFI) in these children. METHODS: Thirteen children (9 boys) with SS and minimum of 2-year follow-up were identified from 2 multicenter early-onset scoliosis (EOS) databases (1997-2017). Mean age at index surgery and follow-up duration were 5.0 years (range, 1.8 to 10 y) and 7.2 years (range, 2.1 to 14.9 y), respectively. Patients underwent GFI for a mean of 5.7 years (range, 2 to 10.2 y), with an average of 9 lengthenings (range, 2 to 18). Definitive spinal fusion was performed in 4 patients (31%). Major curve magnitude, T1-T12 and T1-S1 lengths, thoracic kyphosis, and lumbar lordosis were evaluated preindex, postindex, latest GFI, and postfusion, when possible. RESULTS: Five thoracolumbar (38%), 4 double major (31%), 2 main thoracic (15%), and 2 double thoracic curves (15%) were seen that spanned a mean of 6.8 levels (5 to 9). Major curves improved 36% (range, 5% to 71%), from a mean of 71 degrees (range, 48 to 90 degrees) to 46 degrees (range, 20 to 73 degrees) postindex surgery (P<0.001). Major curves remained stable at a mean of 52 degrees (range, 20 to 87 degrees) at latest GFI (P=0.36). True T1-T12 and T1-S1 growth velocities during GFI were 0.5 mm/mo (range, 0.4 to 0.8 mm/mo) and 0.8 mm/mo (range, 0.1 to 2.1 mm/mo), respectively. Twenty-six complications occurred in 9 patients (69%) averaging 2 complications per patient (range, 0 to 7). CONCLUSIONS: This is the first study to evaluate the outcomes of GFI in children with SS and EOS. Compared with published data for outcomes of GFI in EOS, children with SS may have less major curve correction. Growth-friendly surgery remains an effective treatment method for EOS in patients with SS. LEVELS OF EVIDENCE: Level IV-retrospective case-series.

Chiari I malformations with syringomyelia: long-term results of neurosurgical decompression.

STUDY DESIGN: Retrospective case series. OBJECTIVES: The objective was to assess the long-term outcomes on scoliosis following Chiari-I (CM-I) decompression in patients with CM-I and syringomyelia (SM). A secondary objective was to identify risk factors of scoliosis progression. BACKGROUND: The association between CM-I with SM and scoliosis is recognized, but it remains unclear if CM-I decompression alters the long-term evolution of scoliosis in patients with associated syringomyelia. METHODS: A retrospective review of children with scoliosis, CM-I, and SM during 1997-2015 was performed. Congenital, syndromic, and neuromuscular scoliosis were excluded. Clinical and radiographic characteristics were recorded at presentation, pre-decompression, after 1-year, and latest follow-up. A scale to measure syringomyelia area on MRI was used to evaluate SM changes post-decompression. RESULTS: 65 children with CM-I, SM, and scoliosis and a mean age of 8.9 years (range 0.7-15.8) were identified. Mean follow-up was 6.9 years (range 2.0-20.4). Atypical curves were present in 28 (43%) children. Thirty-eight patients (58%) underwent decompression before 10 years. Syringomyelia size reduced a mean of 70% after decompression (p < 0.001). Scoliosis improved in 26 (40%), stabilized in 17 (26%), and progressed in 22 (34%) cases. Early spinal fusion was required in 7 (11%) patients after a mean of 0.5 ± 0.37 years and delayed fusion in 16 (25%) patients after 6.0 ± 3.24 years. The remaining 42 (65%) patients were followed for a median of 6.1 years (range 2.0-12.3) without spine instrumentation or fusion. Fusion patients experienced less improvement in curve magnitude 1-year post-decompression (p < 0.001) and had larger curves at presentation (43° vs. 34°; p = 0.004). CONCLUSIONS: Syringomyelia size decreased by 70% after CM-I decompression and scoliosis stabilized or improved in two-thirds of patients. Greater curve improvement within the first year post-decompression and smaller curves at presentation decreased the risk of spinal fusion. Neurosurgical decompression is recommended in children with CM-I, SM, and scoliosis with the potential to treat all three conditions. LEVEL OF EVIDENCE: Level IV.

Intraoperative Use of O-arm in Pediatric Cervical Spine Surgery.

INTRODUCTION: Traditionally, fluoroscopy and postoperative computed tomographic (CT) scans are used to evaluate screw position after pediatric cervical spine fusion. However, noncontained screws detected postoperatively can require revision surgery. Intraoperative O-arm is a 3-dimensional CT imaging technique, which allows intraoperative evaluation of screw position and potentially avoids reoperations because of implant malposition. This study's objective was to evaluate the use of intraoperative O-arm in determining the accuracy of cervical implants placed by a free-hand technique using anatomic landmarks or fluoroscopic guidance in pediatric cervical spine instrumentation. METHODS: A single-center retrospective study of consecutive examinations of children treated with cervical spine instrumentation and intraoperative O-arm from 2014 to 2018 was performed. In total, 44 cases (41 children, 44% men) with a mean age of 11.9 years (range, 2.1 to 23.5 y) were identified. Instability (n=16, 36%) and deformity (n=10, 23%) were the most frequent indications. Primary outcomes were screw revision rate, neurovascular complications caused by noncontained screws, and radiation exposure. RESULTS: A total of 272 screws were inserted (60 occipital and 212 cervical screws). All screws were evaluated on fluoroscopy as appropriately placed. Four screws (1.5%) in 4 cases (9%) were noncontained on O-arm imaging and required intraoperative revision. A mean of 7.7 levels (range, 5 to 13) were scanned. The mean CT dose index and dose-length product were 15.2±6.87 mGy and 212.3±120.48 mGy×cm. Mean effective dose was 1.57±0.818 mSv. There was no association between screw location and noncontainment (P=0.129). No vertebral artery injuries, dural injuries, or neurologic deficits were related to the 4 revised screws. CONCLUSIONS: Intraoperative non-navigated O-arm is a safe and efficient method to evaluate screw position in pediatric patients undergoing cervical spine instrumentation. Noncontained screws were detected in 9% of cases (n=4). O-arm delivers low radiation doses, allows for intraoperative screw revision, and negates the need for postoperative CT scans after confirmation of optimal implant position. LEVEL OF EVIDENCE: Level IV.

Acute Implant-related Complications in Pediatric Cervical Spine Fusion.

BACKGROUND: The use of modern rigid instrumentation in pediatric cervical fusions decreases the risk of implant-related complications, both acute and long term. However, previous studies have indicated that acute implant-related issues still occur in the adult population. Reports of pediatric acute implant complications, occurring within 3 months of surgery, are under-represented in the literature. The purpose of this study is to document the prevalence of acute implant-related complications in a pediatric cervical fusion population. METHODS: A retrospective review of instrumented cervical fusions from August 2002 to December 2018 was conducted. Acute implant-related complications were defined as malposition, fracture, or disengagement of cervical instrumentation, including screws, rods, and plates, within 90 days of surgery. RESULTS: A total of 166 cases were included (55% male individuals) with an average age at surgery of 12.5 years (SD, ±5.28). Acute implant-related complications occurred in 5 patients (3%). All 5 patients had a syndromic diagnosis: Loeys-Dietz (n=1), osteopetrosis (n=1), neurofibromatosis (n=1), trisomy 20 (n=1), and achondroplasia (n=1). One case involved asymptomatic screw protrusion, 1 case lateral mass screw pull-out, 2 more had screw-rod disengagement, and the last experienced dislodgement of the anterior plate. The median time until the presentation was 25 days (range, 1 to 79 d). All patients (n=5) required surgical revision. CONCLUSIONS: This case series suggests that the overall incidence of acute cervical implant failure is low. However, failure is more likely to occur in patients with underlying syndromes compared with patients with different etiologies. Intraoperative use of 3-dimensional computed tomography imaging is recommended to evaluate the screw position and potentially avoid later surgery.

Halo-gravity traction for the treatment of pediatric cervical spine disorders.

OBJECTIVE: Halo-gravity traction (HGT) is an effective and safe method for gradual correction of severe cervical deformities in adults. However, the literature is limited on the use of HGT for cervical spine deformities that develop in children. The objective of the present study was to evaluate the safety and efficacy of HGT for pediatric cervical spine deformities. METHODS: Twenty-eight patients (18 females) whose mean age was 11.3 ± 5.58 years (range 2-24.9 years) underwent HGT. Common indications included kyphosis (n = 12), rotatory subluxation (n = 7), and basilar invagination (n = 6). Three children (11%) received traction to treat severe occipitocervical instability. For these 3 patients, traction combined with a halo vest, with bars attached rigidly to the vest, but with the ability to slide through the connections to the halo crown, was used to guide the corrective forces and moments in a specific and controlled manner. Patients ambulated with a wheelchair or halo walker under constant traction. Imaging was done before and during traction to evaluate traction efficacy. The modified Clavien-Dindo-Sink classification was used to categorize complications. RESULTS: The mean duration of HGT was 25 days (IQR 13-29 days), and the mean traction was 29% ± 13.0% of body weight (IQR 19%-40% of body weight). The mean kyphosis improved from 91° ± 20.7° (range 64°-122°) to 56° ± 17.6° (range 32°-96°) during traction and corresponded to a mean percentage kyphosis correction of 38% ± 13.8% (range 21%-57%). Twenty-five patients (89%) underwent surgical stabilization, and 3 patients (11%) had rotatory subluxation that was adequately reduced by traction and were treated with a halo vest as their definitive treatment. The mean hospital stay was 35 days (IQR 17-43 days).Nine complications (32%) occurred: 8 grade I complications (28%), including 4 cases of superficial pin-site infection (14%) and 4 cases of transient paresthesia (14%). One grade II complication (4%) was seen in a child with Down syndrome and a preexisting neurological deficit; this patient developed flaccid paralysis that rapidly resolved with weight removal. Six cases (21%) of temporary neck discomfort occurred as a sequela of a preexisting condition and resolved without treatment within 24-48 hours. CONCLUSIONS: HGT in children is safe and effective for the gradual correction of cervical kyphosis, atlantoaxial subluxation, basilar invagination, and os odontoideum. Cervical traction is an additional tool for the pediatric spine surgeon if uncertainties exist that the spinal alignment required for internal fixation and deformity correction can be safely achieved surgically. Common complications included grade I complications such as superficial pin-site infections and transient paresthesias. Halo vest gravity traction may be warranted in patients with baseline neurological deficits and severe occipitocervical instability to reduce the chance of catastrophic movement.

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.

Perioperative acute neurological deficits in instrumented pediatric cervical spine fusions.

OBJECTIVE: Pediatric cervical deformity is a complex disorder often associated with neurological deterioration requiring cervical spine fusion. However, limited literature exists on new perioperative neurological deficits in children. This study describes new perioperative neurological deficits in pediatric cervical spine instrumentation and fusion. METHODS: A single-center review of pediatric cervical spine instrumentation and fusion during 2002-2018 was performed. Demographics, surgical characteristics, and neurological complications were recorded. Perioperative neurological deficits were defined as the deterioration of preexisting neurological function or the appearance of new neurological symptoms. RESULTS: A total of 184 cases (160 patients, 57% male) with an average age of 12.6 ± 5.30 years (range 0.2-24.9 years) were included. Deformity (n = 39) and instability (n = 36) were the most frequent indications. Syndromes were present in 39% (n = 71), with Down syndrome (n = 20) and neurofibromatosis (n = 12) the most prevalent. Eighty-seven (48%) children presented with preoperative neurological deficits (16 sensory, 16 motor, and 55 combined deficits).A total of 178 (96.7%) cases improved or remained neurologically stable. New neurological deficits occurred in 6 (3.3%) cases: 3 hemiparesis, 1 hemiplegia, 1 quadriplegia, and 1 quadriparesis. Preoperative neurological compromise was seen in 4 (67%) of these new deficits (3 myelopathy, 1 sensory deficit) and 5 had complex syndromes. Three new deficits were anticipated with intraoperative neuromonitoring changes (p = 0.025).Three (50.0%) patients with new neurological deficits recovered within 6 months and the child with quadriparesis was regaining neurological function at the latest follow-up. Hemiplegia persisted in 1 patient, and 1 child died due a complication related to the tracheostomy. No association was found between neurological deficits and indication (p = 0.96), etiology (p = 0.46), preoperative neurological symptoms (p = 0.65), age (p = 0.56), use of halo vest (p = 0.41), estimated blood loss (p = 0.09), levels fused (p = 0.09), approach (p = 0.07), or fusion location (p = 0.07). CONCLUSIONS: An improvement of the preexisting neurological deficit or stabilization of neurological function was seen in 96.7% of children after cervical spine fusion. New or progressive neurological deficits occurred in 3.3% of the patients and occurred more frequently in children with preoperative neurological symptoms. Patients with syndromic diagnoses are at higher risk to develop a deficit, probably due to the severity of deformity and the degree of cervical instability. Long-term outcomes of new neurological deficits are favorable, and 50% of patients experienced complete neurological recovery within 6 months.