A Comparative Analysis of Revision Surgery Before or After 2 Years After Graduation From Growth-friendly Surgery for Early Onset Scoliosis.

INTRODUCTION: After discontinuation of growth-friendly (GF) surgery for early onset scoliosis, patients are termed graduates: they undergo a spinal fusion, are observed after final lengthening with GF implant maintenance, or are observed after GF implant removal. The purpose of this study was to compare the rates of and reasons for revision surgery in two cohorts of GF graduates: before or after 2 years of follow-up from graduation. METHODS: A pediatric spine registry was queried for patients who underwent GF spine surgery with a minimum of 2 years of follow-ups after graduation by clinical and/or radiographic evidence. Scoliosis etiology, graduation strategy, number of, and reasons for revision surgery were queried. RESULTS: There were 834 patients with a minimum of 2-year follow-up after graduation who were analyzed. There were 241 (29%) congenital, 271 (33%) neuromuscular, 168 (20%) syndromic, and 154 (18%) idiopathic. 803 (96%) had traditional growing rod/vertical expandable titanium rib as their GF construct and 31 (4%) had magnetically controlled growing rod. Five hundred ninety-six patients (71%) underwent spinal fusion at graduation, 208 (25%) had GF implants retained, and 30 (4%) had GF implants removed.In the entire cohort, there were 108/834 (13%) patients who underwent revision surgery. Of the revisions, 71/108 (66%) occurred as acute revisions (ARs) between 0 and 2 years from graduation (mean 0.6 y), and the most common AR indication was infection (26/71, 37%). The remaining 37/108 (34%) patients underwent delayed revision (DR) surgery >2 years (mean 3.8 y) from graduation, and the most common DR indication was implant issues (17/37, 46%).Graduation strategy affected revision rates. Of the 596 patients with spinal fusion as a graduation strategy, 98/596 (16%) underwent revision, compared with only 8/208 (4%) patients who had their GF implants retained, and 2/30 (7%) that had their GF implants removed ( P ≤ 0.001).A significantly higher percentage of the ARs had a spinal fusion as the graduation strategy (68/71, 96%) compared with 30/37 DRs, (81%, P = 0.015). In addition, the 71 patients who underwent AR undergo more revision surgeries (mean: 2, range: 1 to 7) than 37 patients who underwent DR (mean: 1, range: 1 to 2) ( P = 0.001). CONCLUSION: In this largest reported series of GF graduates to date, the overall risk of revision was 13%. Patients who undergo a revision at any time, as well as ARs in particular, are more likely to have a spinal fusion as their graduation strategy. Patients who underwent AR, on average, undergo more revision surgeries than patients who underwent DR. LEVEL OF EVIDENCE: Level III, comparative.

Definition of Tweener: Consensus Among Experts in Treating Early-onset Scoliosis.

BACKGROUND: The term "Tweener" is colloquially used to refer to early-onset scoliosis (EOS) patients whose age and development make them candidates for multiple surgical options. The purpose of this study was to establish expert consensus on a definition to formally characterize the Tweener population. METHODS: A 3-round survey of surgeons in an international EOS study group was conducted. Surgeons were provided with various patient characteristics and asked if each was part of their definition for Tweener patients. Responses were analyzed for consensus (≥70%), near-consensus (60% to 69%), and no consensus (<60%). RESULTS: Consensus was reached (89% of respondents) for including chronological age in the Tweener definition; 8 to 10 years for females and 9 to 11 years for males. Surgeons agreed for inclusion of Sanders score, particularly Sanders 2 (86.0%). Patients who have reached Sanders 4, postmenarche, or have closed triradiate cartilage should not be considered Tweeners. Bone age range of 8 years and 10 months to 10 years and 10 months for females (12 y for males) could be part of the Tweener definition. CONCLUSIONS: This study suggests that the Tweener definition could be the following: patients with open triradiate cartilage who are not postmenarche and have not reached Sanders 4, and if they have one of the following: Sanders 2 or chronological age 8 to 10 years for females (9 to 11 y for males) or bone age 8 years and 10 months to 10 years and 10 months for females (12 y for males). This definition will allow for more focused and comparative research on this population. LEVEL OF EVIDENCE: Level V-expert opinion.

Consensus-Based Best Practice Guidelines for the Management of Spinal Deformity and Associated Tumors in Pediatric Neurofibromatosis Type 1: Screening and Surveillance, Surgical Intervention, and Medical Therapy.

BACKGROUND: Spinal conditions, such as scoliosis and spinal tumors, are prevalent in neurofibromatosis type 1 (NF1). Despite the recognized importance of their early detection and treatment, there remain knowledge gaps in how to approach these manifestations. The purpose of this study was to utilize the experience of a multidisciplinary committee of experts to establish consensus-based best practice guidelines (BPGs) for spinal screening and surveillance, surgical intervention, and medical therapy in pediatric patients with NF1. METHODS: Using the results of a prior systematic review, 10 key questions that required further assessment were first identified. A committee of 20 experts across medical specialties was then chosen based on their clinical experience with spinal deformity and tumors in NF1. These were 9 orthopaedic surgeons, 4 neuro-oncologists/oncologists, 3 neurosurgeons, 2 neurologists, 1 pulmonologist, and 1 clinical geneticist. An initial online survey on current practices and opinions was conducted, followed by 2 additional surveys via a formal consensus-based modified Delphi method. The final survey involved voting on agreement or disagreement with 35 recommendations. Items reaching consensus (≥70% agreement or disagreement) were included in the final BPGs. RESULTS: Consensus was reached for 30 total recommendations on the management of spinal deformity and tumors in NF1. These were 11 recommendations on screening and surveillance, 16 on surgical intervention, and 3 on medical therapy. Five recommendations did not achieve consensus and were excluded from the BPGs. CONCLUSION: We present a set of consensus-based BPGs comprised of 30 recommendations for spinal screening and surveillance, surgical intervention, and medical therapy in pediatric NF1.

Spinal Fusion in Pediatric Patients With Low Bone Density: Defining the Value of DXA.

BACKGROUND: Children with medical complexity are at increased risk of low bone mineral density (BMD) and complications after spinal fusion compared with idiopathic scoliosis patients. Our aim was to compare treatments and outcomes of children with medical complexity undergoing spinal fusion in those who had dual-energy x-ray absorptiometry (DXA) scans versus those who did not in an effort to standardize the workup of these patients before undergoing spinal surgery. METHODS: We conducted a retrospective review of patients with low BMD who underwent spinal fusion at a tertiary care pediatric hospital between 2004 and 2016. We consulted with a pediatric endocrinologist to create standard definitions for low BMD to classify each subject. Regardless of DXA status, all patients were given a clinical diagnosis of osteoporosis [at least 2 long bone or 1 vertebral pathologic fracture(s)], osteopenia (stated on radiograph or by the physician), or clinically low bone density belonging to neither category. The last classification was used for patients whose clinicians had documented low bone density not meeting the criteria for osteoporosis or osteopenia. Fifty-nine patients met the criteria, and 314 were excluded for insufficient follow-up and/or not meeting a diagnosis definition. BMD Z -scores compare bone density ascertained by DXA to an age-matched and sex-matched average. Patients who had a DXA scan were also given a DXA diagnosis of low bone density (≤-2 SD), slightly low bone density (-1.0 to -1.9 SD), or neither (>-1.0 SD) based on the lowest BMD Z -score recorded. RESULTS: Fifty-nine patients were analyzed. Fifty-four percent had at least 1 DXA scan preoperatively. Eighty-one percent of DXA patients received some form of treatment compared with 52% of non-DXA patients ( P =0.03). CONCLUSIONS: Patients referred for DXA scans were more likely to be treated for low BMD, although there is no standardized system in place to determine which patients should get scans. Our research highlights the need to implement clinical protocols to optimize bone health preoperatively. LEVEL OF EVIDENCE: Level II-retrospective prognostic study.

The Impact of Unplanned Return to the Operating Room on Health-related Quality of Life at the End of Growth-friendly Surgical Treatment for Early-onset Scoliosis.

BACKGROUND: Limiting complications, especially unplanned return to the operating room (UPROR), is a major focus in the surgical management of early-onset scoliosis (EOS). Although UPROR remains common in this population, its effect on long-term health-related quality of life (HRQoL) remains unclear. The purpose of this study was to investigate the association between UPROR and end-of-treatment HRQoL in EOS patients treated with growth-friendly instrumentation. METHODS: Patients with EOS who underwent growth-friendly instrumentation at age less than 10 years from 1993 to 2018, and completed treatment, were identified in a multicenter EOS registry. UPROR events were recorded, and end-of-treatment (defined as skeletal maturity and/or definitive spinal fusion) HRQoL was assessed via the 24-item Early-Onset Scoliosis Questionnaire (EOSQ-24). RESULTS: A total of 825 patients were identified, and 325 patients (age at surgery: 6.4 y, follow-up: 8.1 y) had end-of-treatment HRQoL data necessary for our investigation. Overall, 129/325 (39.7%) patients experienced 264 UPROR events; the majority (54.2%) were implant-related. Aside from age and etiology, no other variables were determined to be confounders or effect modifiers. Congenital patients with UPROR had worse pain/discomfort by 10.4 points (P=0.057) and worse pulmonary function by 7.8 points (P=0.102) compared with non-UPROR patients adjusting for age. Neuromuscular patients with UPROR had worse pulmonary function by 10.1 points compared with non-UPROR patients adjusting for age (P=0.037). Idiopathic and syndromic patients with UPROR reported consistently worse domain scores than their non-UPROR counterparts, but smaller (<5-point) differences were seen. CONCLUSIONS: UPROR during growth-friendly surgical treatment for EOS is associated with worse HRQoL in all patients, but particularly in those with neuromuscular or congenital etiologies. Ongoing efforts to avoid UPROR are critical. LEVEL OF EVIDENCE: Level II. This is a multicenter retrospective cohort study investigating the effect of UPROR on HRQoL (prognostic study).

Treatment of Early-onset Scoliosis: Similar Outcomes Despite Different Etiologic Subtypes in Traditional Growing Rod Graduates.

BACKGROUND: It is unclear whether traditional growing rod (TGR) treatment outcomes vary by early-onset scoliosis (EOS) subtype. The goal of this study was to compare radiographic outcomes and complications of TGR treatment by EOS subtype. METHODS: We queried an international database of EOS patients from 20 centers to identify "graduates" who had (1) undergone primary TGR treatment from 1993 to 2014; (2) completed TGR treatment; and (3) had an uneventful clinical examination within 6 months after completion of TGR treatment with no anticipated further intervention. We included 202 patients in 4 etiologic subgroups: neuromuscular (n=65), syndromic (n=57), idiopathic (n=52), and congenital (n=28). Mean age at surgery was 7.1 years (range, 1.6 to 14.9 y); mean duration of follow-up was 8 years (range, 2 to 18.6 y). The groups did not differ by mean age, body mass index, sex, number of lengthenings, or duration of follow-up. The following preoperative differences were significant: (1) greater mean major curve in the neuromuscular versus idiopathic subgroup; (2) shorter spinal height (T1-S1) in the congenital versus idiopathic subgroup; and (3) smaller proportion of ambulatory patients in the neuromuscular subgroup versus all other subgroups. RESULTS: We found no significant differences among subgroups in mean major curve correction or changes in thoracic height (T1-T12), spinal height, or global kyphosis at any point. Rates of deep surgical site infection, implant-related complications, and neurological complications were not different among subgroups. The medical complication rate was significantly lower in the idiopathic group compared with the other groups. CONCLUSIONS: Major curve correction and spinal and thoracic height increases did not differ significantly at any point by EOS subtype. Rates of deep surgical site infection, implant-related complications, and neurological complications did not differ by subtype. Except for the lower rate of medical complications in the idiopathic group, our findings suggest that, after TGR treatment, patients can expect similar outcomes regardless of their EOS subtype. LEVEL OF EVIDENCE: Level III, therapeutic.

The Effect of Surgeon Experience on Outcomes Following Growth Friendly Instrumentation for Early Onset Scoliosis.

BACKGROUND: The purpose of this study was to utilize a multicenter, multisurgeon cohort to assess the effect of surgeon experience on outcomes of growth friendly instrumentation (GFI) in early onset scoliosis (EOS). We hypothesized that unplanned return to the operating room (UPROR), estimated blood loss (EBL), and surgical time would be greater amongst early career surgeons (ECSs) when compared with advanced career surgeons (ACSs). METHODS: An international pediatric spine database was queried for patients ages 2 to 10 years treated by posterior distraction-based GFI with at least of 2-year follow up. Two groups were created for analysis based on surgeon experience: ECSs (with ≤10 y of experience) and ACSs (with >10 y of experience). The primary outcome was UPROR. Additional outcomes included: operating room time, EBL, neurological deficits, infection rate, hardware failure, and the Early Onset Scoliosis Questionnaire (EOSQ-24). Subgroup analysis was performed for further assessment based on procedure type, superior anchor type, etiology, and curve severity. RESULTS: A total of 960 patients met inclusion criteria including 243 (25.3%) treated by ECS. Etiology, sex, superior anchor, and EOSQ-24 scores were similar between groups (P>0.05). There were no clinically significant differences in patient age or preoperative major coronal curve. UPROR (35.8% vs. 32.7%, P=0.532), infection (17.0% vs. 15.6%, P=0.698), operating room time (235 vs. 231 min, P=0.755), and EBL (151 vs. 155 mL, P=0.833) were comparable between ECS and ACS groups. The frequency of having at least 1 complication was relatively high but comparable among groups (60.7% vs. 62.6%, P=0.709). EOSQ-24 subdomain scores were similar between groups at 2-year follow-up (P>0.05). Subgroup analysis revealed that ECS had increased surgical time compared with ACS in severe curves >90 degrees (270 vs. 229 min, P=0.05). CONCLUSIONS: This study represents the first multicenter assessment of surgeon experience on outcomes in EOS. Overall, surgeon experience did not significantly influence UPROR, complication rates, EBL, or surgical time associated with GFI in this cohort of EOS patients. LEVEL OF EVIDENCE: Level III.

Mortality in Early-Onset Scoliosis During the Growth-friendly Surgery Era.

INTRODUCTION: Early-onset scoliosis (EOS) is a spinal deformity that occurs in patients 9 years of age or younger. Severe deformity may result in thoracic insufficiency, respiratory failure, and premature death. The purpose of this study is to describe the modern-day natural history of mortality in patients with EOS. METHODS: The multicenter Pediatric Spine Study Group database was queried for all patients with EOS who are deceased, without exclusion. Demographics, underlying diagnoses, EOS etiology, operative and nonoperative treatments or observation, complications, and date of death were retrieved. Descriptive statistics and survival analysis with Kaplan-Meier curves were performed. RESULTS: There were 130/8009 patients identified as deceased for a registry mortality rate of 16 per 1000 patients. The mean age at death was 10.6 years (range: 1.0 to 30.2 y) and the most common EOS etiology was neuromuscular (73/130, 56.2%; P<0.001). Deceased patients were more likely be treated operatively than nonoperatively or observed (P<0.001). The mean age of death for patients treated operatively (12.3 y) was older than those treated nonoperatively (7.0 y) or observed (6.3 y) (P<0.001) despite a larger deformity and similar index visit body mass index and ventilation requirements. Kaplan-Meier analysis confirmed an increased survival time in patients with a history of any spine operation compared with patients without a history of spine operation (P<0.0001). Operatively treated patients experienced a median of 3.0 complications from diagnosis to death. Overall, cardiopulmonary related complications were the most common (129/271, 47.6%; P<0.001), followed by implant-related (57/271, 21.0%) and wound-related (26/271, 9.6%). The primary cause of death was identified for 78/130 (60.0%) patients, of which 57/78 (73.1%) were cardiopulmonary related. CONCLUSIONS: This study represents the largest collection of EOS mortality to date, providing surgeons with a modern-day examination of the effects of surgical intervention to better council patients and families. Both fatal and nonfatal complications in children with EOS are most likely to involve the cardiopulmonary system. LEVEL OF EVIDENCE: Level IV-therapeutic.

Variability in Antibiotic Treatment of Pediatric Surgical Site Infection After Spinal Fusion at A Single Institution.

BACKGROUND: Recent focus on surgical site infections (SSIs) after posterior spine fusion (PSF) has lowered infection rates by standardizing perioperative antibiotic prophylaxis. However, efforts have neglected to detail antibiotic treatment of SSIs. Our aim was to document variability in antibiotic regimens prescribed for acute and latent SSIs following PSF in children with idiopathic, neuromuscular, and syndromic scoliosis. METHODS: This study included patients who developed a SSI after PSF for scoliosis at a pediatric tertiary care hospital between 2004 and 2019. Patients had to be 21 years or younger at surgery. Exclusion criteria included growing rods, staged surgery, and revision or removal before SSI diagnosis. Infection was classified as acute (within 90 d) or latent. Clinical resolution of SSI was measured by return to normal lab values. Each antibiotic was categorized as empiric or tailored. RESULTS: Eighty subjects were identified. The average age at fusion was 14.7 years and 40% of the cohort was male. Most diagnoses were neuromuscular (53%) or idiopathic (41%).Sixty-three percent of patients had an acute infection and 88% had a deep infection. The majority (54%) of subjects began on tailored antibiotic therapy versus empiric (46%). Patients with a neuromuscular diagnosis had 4.0 times the odds of receiving initial empiric treatment compared with patients with an idiopathic diagnosis, controlling for infection type and time (P=0.01). Ninety-two percent of patients with acute SSI retained implants at the time of infection and 76% retained them as of August 2020. In the latent cohort, 27% retained implants at infection and 17% retained them as of August 2020. CONCLUSIONS: Patients with acute infections were on antibiotics longer than patients with latent infections. Those with retained implants were on antibiotics longer than those who underwent removal. By providing averages of antibiotic duration and lab normalization, we hope to standardize regimens moving forward and develop SSI-reducing pathways encompassing low-risk patients. LEVEL OF EVIDENCE: Level III.

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.

Minimum 5-Year Follow-up on Graduates of Growing Spine Surgery for Early Onset Scoliosis.

INTRODUCTION: After discontinuation of growth friendly (GF) surgery for early onset scoliosis, patients undergo spinal fusion or continued observation. This last planned treatment is colloquially called "definitive" treatment, conferring these patients as "graduates" of a growing program. The 5-year radiographic and clinical outcomes of this cohort are unknown. METHODS: An international pediatric spine database was queried for patients from a GF program (spine or rib-based) with minimum 5-year follow-up from last planned surgery (GF or spinal fusion). Radiographs and charts were reviewed for main coronal curve angle and maximum kyphosis as well as occurrence of secondary surgery. RESULTS: Of 580 graduates, 170 (29%) had minimum 5-year follow-up (37% male). Scoliosis etiology was congenital in 41 (24%), idiopathic 36 (21%), neuromuscular 51 (30%), and syndromic 42 (25%). Index surgery consisted of spine-based growing rods in 122 (71%) and rib-based distraction in 48 (29%). Mean age at index surgery was 6.8 years, and patients underwent an average of 5.4 lengthenings over an average of 4.9 years (range, 6 mo to 11 y). Last planned treatment was at an average age of 11.8 years (range, 7 to 17 years). Last planned treatment consisted of spinal fusion in 114 patients, 47 had growing implants maintained, 9 had implants removed. Average follow-up was 7.3 years (range, 5 to 13 y).When compared from postdefinitive treatment to 2-year follow-up, there was noted progression of the coronal curve angle (46±19 to 51±21 degrees, P=0.046) and kyphosis (48±20 to 57±25 degrees, P=0.03). However, between 2 and 5 years, no further progression occurred in the coronal (51±21 to 53±21 degrees, P=0.26) or sagittal (57±25 to 54±28 degrees, P=0.93) planes. When stratified based on etiology, there was no significant coronal curve progression between 2- and 5-year follow-up. When comparing spinal fusion patients to those who had maintenance of their growing construct, there was also no significant curve progression.Thirty-seven (21%) underwent at least 1 (average, 1.7; range, 1 to 7) revision surgery following graduation, and 15 of 37 (41%) underwent 2 or more revision surgeries. Reason for revision was implant revision (either GF or spinal fusion) in 34 patients, and implant removal in 3. On an average, the first revision was 2.5 years after the definitive treatment plan (range, 0.02 to 7.4 y). In total, 15 of 37 (41%) revisions occurred over 2 years following the final decision for treatment plan, and 7 of 37 (19%) occurred 5 or more years after the definitive treatment.Patients who underwent spinal fusion as a definitive treatment strategy were more likely to undergo revision surgery (27%) than patients who had their GF implants maintained (11%) (P=0.04). CONCLUSIONS: Five years following "graduation" from growing surgery for early onset scoliosis, there is progression of curve magnitude in both the coronal and sagittal planes up to 2 years, with no further progression at 5 years. A total of 21% of patients undergo at least 1 revision surgery, and average time to revision surgery is over 2 years from last planned surgery. Risk of revision surgery was higher in patients who underwent a spinal fusion as their definitive treatment strategy. LEVEL EVIDENCE: Level III-retrospective comparative. TYPE OF EVIDENCE: Therapeutic.

Occipital Plate Fixation in the Pediatric Population.

BACKGROUND: Occipital plate fixation has been shown to improve outcomes in cervical spine fusion. There is a paucity of literature describing occipital plate fixation, especially in the pediatric population. The authors reviewed a case series of 34 patients at a pediatric hospital who underwent cervical spine fusion with occipital plate fixation between 2003 and 2016. This study describes how occipital plates aid the cervical spine union in a case series of diverse, complex pediatric patients. METHODS: Our orthopaedic database at our institution was queried for patients undergoing an instrumented cervical spine procedure between 2003 and 2016. Medical records were used to collect diagnoses, fusion levels, surgical technique, and length of hospitalization, neurophysiological monitoring, complications, and revision procedures. RESULTS: Thirty-four patients met the inclusion criteria. The mean age was 10.9 years (range, 3-21 y). Indications for surgery included cervical instability, basilar invagination, and os odontoideum. These indications were often secondary to a variety of diagnoses, including trisomy 21, Klippel-Feil syndrome, and rheumatoid arthritis. The mean length of hospitalization was 10 days (range, 2 to 80 d). There were no cases of intraoperative dural leak, venous sinus bleeding from occipital screw placement, or implant-related complications. Postoperative complications included 2 cases of nonunion. Eight patients (24%) had follow-up surgery, only 3 (9%) of which were instrumentation revisions. Both patients with nonunion had repeat occipitocervical fixation procedures and achieved union with revision. CONCLUSIONS: Occipital plate fixation was successful for pediatric cervical spine fusion in this diverse cohort. The only procedure-related complication demonstrated was delayed union or nonunion and implant loosening (4/34, 12%) and there were no plate-related complications. This novel case series shows that occipital plate fixation is safe and effective for pediatric patients with complex diagnoses. LEVEL OF EVIDENCE: Level IV-case series.

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.

Why Irrigate for the Same Contamination Rate: Wound Contamination in Pediatric Spinal Surgery Using Betadine Versus Saline.

BACKGROUND: The risk of surgical site infection in pediatric posterior spine fusion (PSF) is up to 4.3% in idiopathic populations and 24% in patients with neuromuscular disease. Twenty-three percent of pediatric PSF tissue cultures are positive before closure, with a higher rate in neuromuscular patients. Our primary aim was to evaluate the feasibility of a complete randomized controlled trial to study the efficacy of surgical site irrigation with povidone-iodine (PVP-I) compared with sterile saline (SS) to reduce the bacterial contamination rate before closure in children undergoing PSF. METHODS: One hundred seventy-five subjects undergoing PSF were enrolled in a multicenter, single-blind, pilot randomized controlled trial. We recruited patients at low-risk (LR) and high-risk (HR) for infection 3:1, respectively. Before closure, a wound culture was collected. Nonviable tissues were debrided and the wound was soaked with 0.35% PVP-I or SS for 3 minutes. The wound was then irrigated with 2 L of saline and a second sample was collected. RESULTS: One hundred fifty-three subjects completed the protocol. Seventy-seven subjects were allocated to PVP-I (18 HR, 59 LR) and 76 to SS (19 HR, 57 LR). Cultures were positive in 18% (14/77) of PVP-I samples (2 HR, 12 LR) and in 17% (13/76) of SS samples (3 HR, 10 LR) preirrigation and in 16% (12/77) of PVP-I samples (5 HR, 7 LR) and in 18% (14/76) of SS samples (4 HR, 10 LR) postirrigation. Eight percent (3/37) HR subjects (1 PVP-I, 2 SS) experienced infection at 30 days postoperative. No LR subjects experienced infection. CONCLUSIONS: Positive cultures were similar across treatment and risk groups. The bacterial contamination of wounds before closure remains high regardless of irrigation type. A complete randomized controlled trial would be challenging to adequately power given the similarity of tissue positivity across groups. LEVEL OF EVIDENCE: Level II-pilot randomized controlled trial.

Adverse Perioperative Events in Children with Complex Congenital Heart Disease Undergoing Operative Scoliosis Repair in the Contemporary Era.

Scoliosis is common in children with congenital heart disease (CHD) and may have deleterious effects on quality of life and hemodynamics. Relatively little is known about the outcomes of spinal fusion for scoliosis repair in children with complex CHD. We reviewed all cases of children with CHD undergoing first time spinal fusion excluding those with minor CHD between 1995 and 2015. Seventy-eight patients were identified and included in the study. 97.4% of patients included had undergone prior cardiac surgery and sixteen patients had single ventricle circulations. 17.9% of patients experienced a significant perioperative event defined as an aggregate of the presence of any of the following: need for early unanticipated reoperation, neurologic deficit, postoperative bleeding requiring intervention, end organ dysfunction, or death. There were no deaths in our cohort. 38.5% of patients experienced any adverse event, the majority of which were related to perioperative fluid shifts. Larger preoperative Cobb angle and longer length of spinal fusion were associated with increased risk of significant perioperative event while larger preoperative Cobb angle and longer length of spinal fusion, older age at time of surgery, single ventricle circulation, cyanosis and patients taking cardiac medications at the time of surgery were more likely to experience any adverse event. Operative repair of scoliosis in children with complex CHD has been performed without mortality over a 20-year period in a single institution, albeit with a higher rate of perioperative complication than is seen in the general pediatric population. Patients with large preoperative Cobb angles and cyanotic single ventricle circulations appear to be at the highest risk for perioperative complications.

What Are the Indications for Spinal Fusion Surgery in Scheuermann Kyphosis?

BACKGROUND: Surgical indications for Scheuermann kyphosis are variable. We sought to evaluate the characteristics of patients undergoing operative versus nonoperative treatment of Scheuermann kyphosis to better understand current practices and the factors which contribute to the decision for surgical management. METHODS: Multicenter prospective cohort study. We evaluated consecutive patients presenting with Scheuermann kyphosis. Patients underwent either surgical or nonoperative management according to surgeon and patient discretion. Preoperative patient-reported outcome measures (Scoliosis Research Society and Spinal Appearance Questionnaire scores), demographics, and radiographic characteristics were assessed. RESULTS: Overall, 150 patients with Scheuermann kyphosis were enrolled, with 77 choosing nonoperative treatment and 73 treated operatively. Compared with the nonoperative cohort, patients treated operatively were older (16.3±2.0 vs. 15.1±2.2, P=0.0004), and had higher body mass index (26.3±7.2 vs. 22.7±6.5, P=0.003), had greater T2-T12 kyphosis (71±14 degrees vs. 61±12 degrees, P<0.001), increased pelvic incidence (46 vs. 41 degrees, P=0.03) and pelvic tilt (10 vs. 3 degrees, P=0.03). There was no detected difference in maximal sagittal Cobb angle in the operative versus nonoperative patients (73±11 vs. 70±12 degrees, P=0.11). Functionally, the operative patients had worse Scoliosis Research Society pain scores (3.7±0.9 vs. 4.1±0.7, P=0.0027) and appearance scores (2.9±0.7 vs. 3.4±0.8, P <0.0001). CONCLUSIONS: Patients undergoing surgical management of Scheuermann disease were more likely to have large body mass index and worse pain scores. Other factors beyond radiographic measurement likely contribute to the decision for surgical management of Scheuermann kyphosis. LEVEL OF EVIDENCE: Level II.

Growing Rods Are an Effective Fusionless Method of Controlling Early-Onset Scoliosis Associated With Neurofibromatosis Type 1 (NF1): A Multicenter Retrospective Case Series.

BACKGROUND: Spinal deformities associated with neurofibromatosis type 1 (NF1) often have an early onset. These curves frequently develop dysplastic features. Rapid progression is common, and is often difficult to control with casting or bracing. Spinal fusion at a young age can potentially interfere with chest and trunk growth. Growing rods (GRs) have been used in early-onset scoliosis (EOS) effectively. The purpose of this study was to evaluate GR use in NF1. METHODS: Retrospective data collection was performed from a multicenter EOS database with additional patients from our own institute. Each patient had a genetic diagnosis of NF1 and was treated with GR. Results were compared with reported results of GR in EOS in the literature. RESULTS: Fourteen patients from 5 centers underwent a total of 71 procedures with an average follow-up of 54 months. Mean age at surgery was 6.8 years. Means of initial and final curves were 74 and 36 degrees, respectively (51% correction). Spine grew at an average of 39 mm (11.2 mm per year). Implant-related complications were the most common (8/14, 57%), including failure of proximal construct (5/14), rod breakage (2/14), and prominent implants (1/14). There was no significant difference between screws and hooks as proximal anchors (Fischer test). Two patients had deep infection that needed debridement. CONCLUSIONS: This retrospective pooled data study is the first report on the treatment of early-onset NF1 scoliosis with GRs. The use of GRs in these patients effectively controls the spinal deformity and facilitates growth of the spine. The complications were no greater than those seen in other conditions causing EOS. Failure of proximal anchors was found to be the most common complication. LEVEL OF EVIDENCE: Level IV-retrospective case series.

Staged Growing Rods With Preimplantation of Spinal Anchors for Complex Early Onset Scoliosis.

BACKGROUND: Treatment of early onset scoliosis (EOS) with growing rods (GR) can be challenging in patients with significant deformity, hyperkyphosis, or poor bone quality, due to risks of neurological deficit and hardware pull-out. The objective of this study is to report a series of EOS patients managed with a 2-stage GR technique used to minimize these complications. METHODS: Two-stage GR technique was performed in 8 patients at mean age of 5.4 (range, 3.4 to 7.9) years. At stage 1, proximal and distal anchors were implanted with local fusion. At stage 2, the distraction rods were inserted. There were at least 3 months between stages 1 and 2, and halo-gravity traction was used before stage 2 unless contraindicated. Demographic, clinical, and surgical data were retrospectively reviewed with mean 4.9 (range, 2.0 to 9.4) years of follow-up. Radiographic measurements including Cobb and kyphosis angles were evaluated before stage 1, after halo-gravity traction, after stage 2, and at last follow-up. Indications for staging, anchor healing time, and complications were collected and analyzed. RESULTS: Indications for 2-stage surgery were poor bone quality in 5 patients and neurological changes during initial attempt at GR placement in 3 patients. The mean time between stage 1 and 2 was 23 (15 to 45) weeks. Patients have undergone mean 7 (3 to 16) lengthenings. Three patients have been converted to magnetically controlled GR. The major coronal Cobb angle improved from mean 81 degrees (range, 61 to 97) preoperatively to 40 degrees (24 to 50) after stage 2 and remained at 40 degrees (27 to 53) at last follow-up. Kyphosis remained controlled from 45 degrees (10 to 76) preoperatively to 38 degrees (9 to 61) after stage 2 to 41 degrees (17 to 65) at last follow-up. Complications included superficial wound problems (4 patients), broken rods (2 patients), proximal migration (2 patients), and implant prominence (1 patient). At minimum 2-year follow-up, no patients had lingering neurological complications or instrumentation-bone failure of the GR construct. CONCLUSIONS: Two-stage GR technique can effectively be utilized in EOS patients with poor bone quality or in the event of intraoperative neurological changes. Satisfactory deformity correction can be obtained and routine serial lengthenings can take place with minimal complications. LEVEL OF EVIDENCE: Level IV- case series.

VEPTR Implantation After Age 3 is Associated With Similar Radiographic Outcomes With Fewer Complications.

BACKGROUND: The purpose of this study was to analyze the risks and benefits of early vertical expandable prosthetic titanium rib (VEPTR) implantation (below 3 y of age) compared with delayed intervention (3 to 6 y of age). Early intervention with milder deformity may make device implantation easier and may facilitate symmetric chest and pulmonary development. However, later intervention may decrease the number of lengthenings required, and implant anchor points and bone quality may be better in older patients. METHODS: A multicenter prospective database of patients treated with the VEPTR device was retrospectively reviewed. A total of 71 patients with minimum 5-year follow-up were included. Thirty-seven patients had VEPTR surgery below 3 years of age (group 1), and 34 patients had surgery between 3 and 6 years of age (group 2). Radiographs from the preoperative, immediate postoperative, and most recent follow-up visits were reviewed to determine coronal plane deformity and change in T1-T12 height over time. Complications were analyzed using a nonparametric mean cumulative function for the number of complications per subject over time. RESULTS: Patients in both groups had similar diagnoses, ASA scores (P=0.22), and number of lengthenings (10±4 in both groups, P=0.40). Preoperative and most recent major Cobb angles were not significantly different between the 2 groups [66±22 and 60±20 degrees (group 1) vs. 63±19 and 57±23 degrees (group 2), respectively] (P=0.38). The change in T1-T12 was significantly correlated with the number of lengthenings (P<0.001); however, there was no difference between age groups (P=0.55). There were 118 complications in 33 subjects in group 1 and there were 69 complications in 28 subjects in group 2. The number of complications per lengthening was significantly different between the 2 age groups (P<0.001). Subjects in group 2 had 41% fewer complications per lengthening compared with subjects in group 1 (P<0.001). CONCLUSIONS: VEPTR treatment resulted in similar deformity control and thoracic growth in both age groups with lower complication rates in the older population. The rate of complications was similar between the 2 groups for the first 2 years postimplantation, but after 2 years, the rate of complications increased over time faster for the younger group compared with the older group. LEVEL OF EVIDENCE: Level III-retrospective chart and radiographic review.