Do Children With Medicaid Insurance Have Increased Revision Rates 5 Years After Posterior Spinal Fusions?

BACKGROUND: Socioeconomic disparities in musculoskeletal care are increasingly recognized, however, no studies to date have investigated the role of the insurance carrier on outcomes after posterior spinal fusion (PSF) with segmental spinal instrumentation for adolescent idiopathic scoliosis (AIS). METHODS: A US insurance dataset was queried using the PearlDiver Mariner software for all patients aged 10 to 18 undergoing PSF for a primary diagnosis of AIS between 2010 and 2020. Age, sex, geographic region, number of levels fused, and baseline medical comorbidities were queried. Complications occurring within 90 days of the index surgery were queried using the International Classification of Diseases, Ninth Revision (ICD-9) and International Classification of Diseases, 10th Revision (ICD-10) codes. Revision surgery was also queried up to 5 years after the index PSF. Categorical variables were compared using the Fisher χ 2 tests and continuous variables were compared using independent t tests. All-cause revision within 5 years was compared using the Kaplan-Meier analysis and a log-rank test. Significance was set at P -value <0.05. RESULTS: A total of 10,794 patients were identified with 9006 (83.4%) patients with private insurance and 1788 (16.6%) patients insured by Medicaid. The mean follow-up in the database was 5.36±3 years for patients with private insurance and 4.78±2.9 years for patients with Medicaid insurance ( P <0.001). Children with AIS and Medicaid insurance had a significantly higher prevalence of asthma, hypertension, and obesity. A larger percentage of children with Medicaid insurance (41.3%) underwent a ≥13-level PSF compared with privately insured children (34.5%) ( P <0.001). Medicaid patients did not experience higher odds of postoperative complications; in addition, revision surgeries occurred in 1.1% and 1.8% of patients with private insurance and Medicaid insurance, respectively at 5 years postoperatively ( P =0.223). CONCLUSION: Despite worse baseline comorbidities and longer fusion constructs, AIS patients insured with Medicaid did not have higher rates of complications or revisions at 5-year follow-up versus privately insured patients. LEVEL OF EVIDENCE: Level III-retrospective cohort study.

An Analysis of Scoliosis in Autism Spectrum Disorder.

BACKGROUND: Children with autism/Asperger are grouped into the diagnosis of autism spectrum disorder (ASD). It remains uncertain whether children with ASD and scoliosis have radiographic and clinical outcomes similar to idiopathic scoliosis (IS) patients. METHODS: A single-center, retrospective review of a prospective scoliosis registry evaluated patients who had a posterior spinal fusion±Anterior Spinal Fusion and an underlying diagnosis of ASD between 1990 and 2021. A 2:1 match with AIS patients by age and sex was compared using demographic, radiographic, intraoperative, and SRS-22/30 variables. RESULTS: Thirty patients with ASD (63% male, mean age at surgery 14.6±2.5 y) met inclusion criteria, with a follow-up of 2.46±1.00 years. Despite no differences in curve magnitude preoperatively, patients with ASD had a higher percent correction at 2-year follow-up (66% vs. 57%, P =0.01) and improved mean curve magnitude (20±10 degrees) at 2-year follow-up compared with IS patients (27±11 degrees, P <0.01). ASD patients had less lumbar lordosis preoperatively (40±12 vs. 53±14, P <0.01), but there were no significant differences in sagittal parameters at 2-year follow-up. There were no significant differences in the rate of complications at 2-year follow-up between ASD and AIS cohorts. CONCLUSIONS: Although patients with ASD exhibited decreased lordosis compared with IS patients preoperatively, their radiographic outcomes at 2-year follow-up were the same. In addition, ASD patients maintained greater curve correction than IS patients at 2 years follow-up. LEVEL OF EVIDENCE: Prognostic retrospective study.

Spinal Fusion in Patients With Classic Amyoplasia and General Arthrogryposis.

BACKGROUND: Arthrogryposis multiplex congenita is a group of conditions characterized by joint contractures affecting 2 or more joints. This study describes results of spinal fusion in patients with classic amyoplasia and general arthrogryposis. METHODS: IRB approved retrospective review of patients with a diagnosis of classic amyoplasia and general arthrogryposis who had a primary definitive posterior spinal fusion between 1990 and 2017 at a single pediatric institution. Patients with distal and syndromic arthrogryposis were excluded as well as patients treated with growth-sparing spinal instrumentation. The Modified Clavien-Dindo-sink (MCDS) classification system was used to describe postoperative complications. RESULTS: Over the 28-year period, 342 patients were diagnosed with amyoplasia and general arthrogryposis. Among the 342 patients, 60 (18%) had scoliosis, and 22 (6% of the cohort and 37% of those with scoliosis) were treated surgically. Six patients had growth-sparing techniques, initial fusion elsewhere, or ˂1 year of follow-up, leaving 15 patients. Of the 15, 9 (60%) had a posterior spinal fusion (PSF) and 6 (40%) had a combined anterior spinal fusion (ASF)/PSF. The ASF/PSF group was significantly younger at surgery, had a greater American Society of Anesthesiologists status, longer surgery duration, and lower implant density. The average preoperative major coronal deformity in ASF/PSF patients (108 degrees) was greater than patients treated with PSF alone (88 deg). There were 11 complications in 7 patients, with the most common being deep infection requiring reoperation (5/11, 45%). There was 1 instance (1/11, 9%) of each: prolonged intensive care unit admission (>72 h), superficial wound dehiscence, symptomatic implants requiring removal/revision, coronal plane progression requiring extension of fusion, recurrent pneumothorax requiring return to OR, and pseudoarthrosis leading to implant failure (without revision). Complications occurred in 1/9 (11%) PSF-only patients and 6/6 (100%) ASF/PSF patients with all 6 ASF/PSF patients requiring at least 1 reoperation. The average coronal correction was 48% in the PSF-only group and 28% in the ASF/PSF group. CONCLUSION: Complication rates after spinal fusion for scoliosis in arthrogryposis multiplex congenita patients are high, especially in patients undergoing ASF/PSF, deep infection is common, and major coronal plane curve correction is modest. LEVEL OF EVIDENCE: II Retrospective Study.

Clinical and Radiographic Assessment of Adult Function After Blount Disease in Childhood: An Exercise in Futility.

BACKGROUND: Few studies evaluate long-term function of patients with Blount disease. We sought to document patient radiographic and functional status 20 to 30 years after sugical treatment for infantile or adolescent Blount disease. METHODS: We reviewed the medical records and radiographs of patients operated at our institution for Blount disease between 1985 and 1995. Over a 4-year period, we recruited subjects for an IRB-approved call-back study. RESULTS: One hundred five patients were eligible for the call-back study. Thirty-one (30%) had a criminal record, 18 of sufficient gravity to preclude invitation to return. Of the remaining 87, 40 (46%) could not be contacted. Of 47 with valid contact information, 10 (21%) were reported as deceased (although this could not be objectively confirmed), 20 (43%) did not respond or failed to show for assessment, and 1 (2%) declined to participate. 16 subjects returned at average age 36, 22 to 31 years post-index surgery. Body mass index (BMI) averaged 45.8 (range 23.9 to 67.6). Physical Score correlated most strongly and inversely with BMI ( P <0.01). Satisfaction with life correlated strongly and inversely with mechanical axis deviation ( P =0.02) and radiographic osteoarthritis of the knee ( P =0.02), but not BMI. There also was no correlation between severity of radiographic osteoarthritis and mechanical axis deviation ( P =0.46) or BMI ( P =0.52). CONCLUSIONS: The small fraction of patients returning for evalutation minimize clinical conclusions that can be drawn from this study, despite an intensive 4-year effort to conduct it. Management of obesity and other socioeconomic characteristics are likely the most important aspects of treatment of patients with Blount disease. Our primary conclusion is that meaningful long-term functional studies of pediatric orthopaedic conditions will not be answered by retrospective call-back studies, and must be conducted within prospective registries and regular longitudinal follow-up. LEVEL OF EVIDENCE: III-Case-controlled study.

Pain Catastrophizing Influences Preoperative and Postoperative Patient-Reported Outcomes in Adolescent Idiopathic Scoliosis.

BACKGROUND: Patients with adolescent idiopathic scoliosis (AIS) often report chronic back pain; however, there is inadequate research on psychological factors associated with pain in this patient population. Pain catastrophizing, a psychological factor that describes a pattern of negative thoughts and feelings about pain, has been associated with poorer responses to medical treatment for pain. The purpose of this study was to report the prevalence of pain catastrophizing in the AIS population and assess its relationship with preoperative and postoperative self-reported outcomes. METHODS: In this prospective cohort study of consecutive patients undergoing posterior spinal fusion (PSF) for AIS, patients experiencing clinically relevant pain catastrophizing, defined as a Pain Catastrophizing Scale for Children (PCS) score in the 75th percentile or higher, were compared with patients with normal PCS scores. Preoperative and 2-year postoperative Scoliosis Research Society Society Questionnaire-30 (SRS-30) scores were correlated with the preoperative PCS score. RESULTS: One hundred and eighty-nine patients underwent PSF for AIS, and 20 (10.6%) were considered to be experiencing pain catastrophizing. Despite comparable demographic and radiographic variables, pain catastrophizing was associated with significantly lower preoperative scores than were found in the normal-PCS group in all SRS-30 domains, including pain (2.98 versus 3.95; p < 0.001), appearance (2.98 versus 3.48; p < 0.001), activity (3.51 versus 4.06; p < 0.001), mental health (3.12 versus 4.01; p < 0.001), and total score (3.18 versus 3.84; p < 0.001), except satisfaction (3.72 versus 3.69; p > 0.999). At 2 years, the pain catastrophizing group experienced significant improvement from their preoperative scores in most SRS-30 domains, including a large clinically relevant improvement in pain (from 2.98 preoperatively to 3.84 postoperatively; p < 0.001) and the total score (from 3.18 to 3.85; p < 0.001), but continued to have lower scores than the normal-PCS group for pain (3.84 versus 4.22; p = 0.028) and the total score (3.85 versus 4.15; p = 0.038). Receiver operating characteristic (ROC) curve analysis indicated that an SRS-30 pain score of <3.5 has good sensitivity for predicting pain catastrophizing (PCS ≥75th percentile). CONCLUSIONS: In this cohort, patients with AIS who exhibited pain catastrophizing experienced significant improvement in self-reported health 2 years after PSF. However, they did not have the same levels of self-reported health as the normal-PCS group. Pain catastrophizing may be identifiable by lower preoperative SRS-30 pain scores. LEVEL OF EVIDENCE: Prognostic Level II . See Instructions for Authors for a complete description of levels of evidence.

The Efficacy of Intra-articular Subtalar Steroid Injection for Symptomatic Talocalcaneal Coalitions: A 30-Year Single Institution Experience.

PURPOSE: While intra-articular steroid injection has been used anecdotally in patients with symptomatic talocalcaneal coalitions recalcitrant to traditional conservative modalities, the ability of this treatment to provide symptomatic relief and obviate or delay surgical intervention remains unknown. The purpose of this study is, therefore, to assess the treatment efficacy of intra-articular subtalar steroid injection in children with symptomatic talocalcaneal coalitions. METHODS: A retrospective study of all patients with isolated subtalar coalitions was performed at a single pediatric orthopaedic institution over a 30-year period. Radiographs were analyzed to identify the type of coalition (osseous or nonosseous), presence of any posterior facet involvement, and presence of a planovalgus foot deformity. Patients who underwent a subtalar joint steroid injection after failing other conservative treatments were identified and compared with those who did not receive an injection as part of their nonoperative management with regard to the need for ultimate surgical intervention and the time from presentation to surgery when applicable. RESULTS: A total of 83 patients (125 feet) met inclusion criteria, of whom 25 patients (34 feet) received a subtalar steroid injection. When compared with the 58 patients (91 feet) treated with standard nonoperative modalities, there were no differences with regard to sex, age at presentation (12.4 and 12.3 y, respectively), facet involvement, type of coalition, or the presence of a planovalgus deformity. In all, 12/34 (35%) feet in the injection group eventually elected surgical intervention compared with 36/91 (39%) feet that did not receive an injection (P=0.72). For those patients ultimately selecting surgical intervention, the average time from initial presentation to surgery was 878 days in the injection group versus 211 days in the noninjection group (P<0.001). CONCLUSIONS: While subtalar steroid injection can alleviate symptoms in some patients with a talocalcaneal coalition, this intervention does not appear to decrease the need for surgery when compared with traditional nonoperative therapies. In patients failing other forms of conservative treatment, subtalar steroid injections can delay surgical intervention by an average of nearly 2 years. LEVEL OF EVIDENCE: Level-III-therapeutic study.

Arthrogryposis multiplex congenita: a 28-year retrospective study.

AIM: To identify all patients with arthrogryposis multiplex congenita presenting to a specialized pediatric orthopedic institution over a 28-year period; classify them into three groups (general arthrogryposis not otherwise specified [NOS], amyoplasia, or distal arthrogryposis); report the frequency of various musculoskeletal features; and determine the rate of operative treatment. METHOD: Patients with arthrogryposis under the age of 18 years who presented between 1990 and 2017 were included. Patients were placed into one of three groups based on clinical features and family history when available. Age of presentation, joint involvement, and utilization of surgical treatment were recorded. RESULTS: There were 417 patients in total (184 females, 233 males); 235 patients (56.4%) had general arthrogryposis NOS, 107 (25.7%) had amyoplasia, and 75 (18.0%) had distal arthrogryposis. Patients with amyoplasia presented at a younger age (median 4mo) than those with general arthrogryposis NOS (median 1y 3mo, p=0.005), and had a lower rate of spine involvement than patients with general arthrogryposis NOS (p=0.004) and distal arthrogryposis (p=0.023). The average number of surgeries across all patients was 1.9 (SD 2.2). INTERPRETATION: Pediatric patients with amyoplasia present to orthopedic care earlier and are less likely to have spine involvement than other forms of arthrogryposis. Multiple surgeries are common among all patients with arthrogryposis.

Predictors of appointment adherence following operative treatment of pediatric supracondylar humerus fractures: which patients are not following up?

This study aims to identify characteristics associated with poor appointment adherence after surgical stabilization of supracondylar humerus fractures (SCHFX) in children. A retrospective review of 560 consecutive, surgically managed patients with SCHFX from 2010 to 2015 was performed. One missed follow-up appointment was classified as 'low adherence', whereas missing two or more appointments was classified as 'very low adherence'. Demographics, insurance status, estimated family income and distance from clinic were analyzed to identify differences in variables between adherent and low-adherent groups. Of 560, 121 (21.8%) missed one follow-up visit and 39/560 (7.1%) missed more than two visits. Age, gender, distance traveled, insurance status and primary language were nonpredictive. Estimated income <$50 000 was associated with a >200% increase in low adherence vs patients with estimated income >$50 000 (9.3 vs 3.8%; P = 0.012). African American patients had significantly lower adherence vs patients of other races (47.5 vs 19.6%; P < 0.0001). Ethnicity remained the only significant factor correlated to adherence after multivariate analysis. African Americans were three times more likely demonstrate low adherence (P = 0.0014). Ethnicity and estimated income <$50 000 were predictors of missing two or more visits. African American patients were four times more likely to miss two or more visits [odds ratio (OR), 4.17; P = 0.0026] than others; estimated income <$50 000 was associated with a two-fold increase in missing two or more visits (OR, 2.33; P = 0.035). By identifying at-risk patient populations, healthcare systems can adopt strategies to remove barriers of accessing follow-up care.

Incidence and significance of findings on spinal MRIs in a paediatric population with spinal column complaints.

PURPOSE: We sought to identify correlations between working diagnosis, surgeon indication for obtaining spinal MRI and positive MRI findings in paediatric patients presenting with spinal disorders or complaints. METHODS: Surgeons recorded their primary indication for ordering a spinal MRI in 385 consecutive patients. We compared radiologist-reported positive MRI findings with surgeon response, indication, working diagnosis and patient demographics. RESULTS: The most common surgeon-stated indications were pain (70) and coronal curve characteristics (63). Radiologists reported 137 (36%) normal and 248 (64%) abnormal MRIs. In total, 58% of abnormal reports (145) did not elicit a therapeutic or investigative response, which we characterized as 'clinically inconsequential'. In all, 42 of 268 (16%) presumed idiopathic scoliosis patients had intradural pathology noted on MRI.Younger age (10.3 years versus 12.0 years) was the only significant demographic difference between patients with or without intradural pathology. Surgeon indication 'curve magnitude at presentation' was associated with intradural abnormality identification. However, average Cobb angles between patients with or without an intradural abnormality was not significantly different (39° versus 37°, respectively). Back pain without neurological signs or symptoms was a negative predictor of intradural pathology. CONCLUSION: Radiologists reported a high frequency of abnormalities on MRI (64%), but 58% of those were deemed clinically inconsequential. Patients with MRI abnormalities were two years' younger than those with a normal or inconsequential MRI. 'Curve magnitude at presentation' in presumed idiopathic scoliosis patients was the only predictor of intrathecal pathology. 'Pain' was the only indication significantly associated with clinically inconsequential findings on MRI. LEVEL OF EVIDENCE: III.

Two AIS spine surgeries on the same day by the same surgeon: is performance and outcome the same for the second patient?

STUDY DESIGN: Retrospective case-controlled study. OBJECTIVES: To analyze the overall performance and outcome of two-a-day surgery days for adolescent idiopathic scoliosis (AIS). As a method to improve efficiency and operating room utilization, some surgeons are now performing two surgeries for AIS in a single day. METHODS: A prospectively collected series of AIS patients who underwent posterior spinal fusion on the same day as a second AIS patient by the same surgeon and surgical team were retrospectively reviewed. Patients who underwent same-day surgery (SD) were grouped according to whether they were the first (SD1) or second (SD2) case of the day and were matched (M1 and M2) by surgeon, curve magnitude, Lenke classification, and fusion levels. Comparisons were made: SD1 vs. SD2, SD1 vs. M1, and SD2 vs. M2. RESULTS: There were 56 patients, with no differences between groups in age, gender, BMI, or curve magnitude (66° vs. 62° vs. 65° vs. 63°). Surgical time was shorter for the SD1 group (17.2 min/level) compared to M1 (20.5 min/level) for a 15% operative time reduction of 44 min (p = 0.008). There were no differences between the groups in curve correction (65.8% vs. 62.8% vs. 66.1% vs. 58.5%), estimated blood loss (EBL), length of stay, or complication rate. One SD2 patient had a malpositioned screw that required revision. There were no other complications. CONCLUSIONS: When performing two AIS surgeries on the same day, surgical time was reduced by 44 min, or 15%, on the first case compared to a matched control. This may be a reflection of the team moving along more efficiently, given the full operative day scheduled. The performance measures of curve correction, EBL, complications, and length of stay did not decline in this new model, and no increased incidence of complications was seen.

Prospective Comparison of Patient-selected Operative Versus Nonoperative Treatment of Scheuermann Kyphosis.

BACKGROUND: Scheuermann kyphosis (SK) can be managed operatively or nonoperatively. Few studies compare the effect of operative versus nonoperative treatment on patient health-related quality of life. We compare 2-year radiographic and the Scoliosis Research Society-22 questionnaire (SRS-22) results of patients who self-selected either conservative or surgical treatment. METHODS: Single institution review of prospectively collected data for patients presenting with SK from 2006 to 2014. Forty-five of 55 patients returned for 2-year follow-up. Patients were divided into operative (n=27) or nonoperative (n=18) groups based upon their self-selected method of treatment. Radiographic data and SRS-22 scores were collected at initial presentation and 2-year follow-up. RESULTS: Operatively treated patients had larger initial sagittal Cobb angles and lower SRS-22 scores in the pain and appearance domains. Two years postoperatively, surgically treated patients had smaller Cobb angles and improved scores in these SRS-22 domains. Nonoperatively treated patients did not deteriorate over time. CONCLUSIONS: Patients who elect to receive operative treatment for SK have improved radiographic and SRS-22 parameters at 2-year follow-up compared with patients who elect nonoperative treatment. Not surprisingly, patients selecting surgical treatment had greater sagittal Cobb angles and greater levels of pain and dissatisfaction with their appearance. However, at 2-year follow-up, surgical patients experience greater (and significant) change on all parameters; exhibiting smaller Cobb angles, less pain, and greater satisfaction with their outcomes. Nonoperatively treated patients do not deteriorate over 2 years. LEVEL OF EVIDENCE: Level II-prognostic study.

Distal Junctional Failure Following Pediatric Spinal Fusion.

BACKGROUND: Adjacent segment pathology is a known complication after spinal fusion, but little has been reported on junctional failure. A series of adolescent patients presented with acute distal junctional failure (DJF). We sought to determine any common features of these patients to develop a prevention strategy. METHODS: A retrospective review was conducted of pediatric patients who developed DJF after instrumented spinal fusion performed at 2 institutions from 1999 to 2013. Patients with proximal junctional failure or junctional kyphosis without failure were excluded. RESULTS: Fifteen subjects were identified with mean follow-up of 38 months. Distal failure occurred a mean of 60 days after index surgery, with history of minor trauma in 4 patients. Failures included 3-column Chance fracture (11) or instrumentation failure (4). Thirteen patients presented with back pain and/or acute kyphosis, whereas 2 asymptomatic patients presented with healed fractures. Two patients also developed new onset of severe lower extremity neurological deficit after fracture, which improved but never resolved after revision. A total of 13/15 subjects required revision surgery, typically within 1 week. Complications associated with revision surgery were encountered in 8 patients (62%). Major complications that required return to the operating room included 2 deep infections, 2 instrumentation failures, and dense lower extremity paralysis that improved after medial screw revision and decompression. At final follow-up, 10 patients are asymptomatic, 2 have persistent neurological deficit, 2 have chronic pain, and 1 has altered gait with gait aid requirement. CONCLUSIONS: This study analyzes a heterogenous cohort of spinal fusion patients who developed DJF from 3-column Chance fracture or instrumentation failure. Revision surgery is typically required, but has a high complication rate and can result in severe neurological deficit, highlighting the morbidity of this complication. It is unclear whether level of the lowest instrumented vertebra contributes to DJF. Increased awareness of junctional failure in children may prompt additional studies to further characterize risk factors and preventative strategies. LEVEL OF EVIDENCE: Level IV-study-type case series.

Complications Associated With Epiphysiodesis for Management of Leg Length Discrepancy.

BACKGROUND: Epiphysiodesis is well-established surgical treatment for the management of leg length discrepancy (LLD) in children; however, a variety of complications may occur. This study evaluates the nature, rate, and potential risk factors of complications associated with epiphysiodesis in a large patient population treated in 1 institution. METHODS: We evaluated the medical and radiographic records of 863 children who had lower extremity epiphysiodesis to manage LLD between 1980 and 2011. RESULTS: Sixty patients (7.0%) incurred complications of some type. Twenty-three patients (2.7%) had perioperative complications unrelated to physeal growth; all resolved without surgical treatment. These included 2 patients with transient intraoperative complications (laryngospasm and allergic rash), 7 with transient neurological symptoms (5 cutaneous nerve dysesthesia or numbness and 2 peroneal nerve neuropathies), and 14 with postoperative knee stiffness which resolved with therapy. Thirty-seven patients (4.3%) developed physeal growth-related complications, including 6 patients who developed overcorrection of leg length inequality (3 had contralateral epiphysiodesis to prevent overcorrection, while 3 declined), and 31 patients who developed angular deformity and/or continued growth of the physis. Of these 31 patients, 15 had reexploration of the epiphysiodesis site, 6 underwent corrective osteotomy, while in 10, no treatment was undertaken. Compared with patients who did not develop angular deformity, these 31 patients had significantly greater LLD (5.6 vs. 3.7 cm, respectively, P<0.01), were younger (10.7 vs. 11.7 in girls; 12.4 vs. 13.5 in boys; P<0.01), and were more likely to have a congenital etiology for their LLD (P<0.01). CONCLUSIONS: Epiphysiodesis is a safe and effective procedure for the correction of leg length inequality. The rate of complications in this study was 7.0%, the most prevalent being the development of angular deformity. Congenital etiology, younger age, and larger limb length inequalities were risk factors for the development of angular deformity. Both surgeons and families should be aware of nature and rate of complications associated with epiphysiodesis. LEVEL OF EVIDENCE: Level IV.

Outcomes of Spinal Fusion for Cervical Kyphosis in Children with Neurofibromatosis.

BACKGROUND: Cervical kyphosis may occur with neurofibromatosis type I (NF1) and is often associated with vertebral dysplasia. Outcomes of cervical spinal fusion in patients with NF1 are not well described because of the rarity of the condition. We aimed to (1) characterize the clinical presentation of cervical kyphosis and (2) report the outcomes of posterior and anteroposterior cervical fusion for the condition in these children. METHODS: The medical records and imaging studies of 22 children with NF1 who had undergone spinal fusion for cervical kyphosis (mean, 67°) at a mean age of 11 years and who had been followed for a minimum of 2 years were reviewed. RESULTS: Thirteen children presented with neck pain; 10, with head tilt; 9, with a previous cervical laminectomy or fusion; and 5, with a neurologic deficit. Two patients had spontaneous dislocation of the mid-cervical spine without a neurologic deficit. Eleven had scoliosis, with the major curve measuring a mean of 61°. Nine patients underwent posterior and 13 underwent anteroposterior surgery. Twenty-one received spinal instrumentation, and 1 was not treated with instrumentation. Preoperative halo traction was used for 9 patients, and it reduced the mean preoperative kyphosis by 34% (p = 0.0059). At the time of final follow-up, all spinal fusion sites had healed and the cervical kyphosis averaged 21° (mean correction, 69%; p < 0.001). The cervical kyphosis correction was significantly better after the anteroposterior procedures (83%) than after the posterior-only procedures (58%) (p = 0.031). Vertebral dysplasia and erosion continued in all 17 patients who had presented with dysplasia preoperatively. Thirteen patients had complications, including 5 new neurologic deficits and 8 cases of junctional kyphosis. Nine patients required revision surgery. Junctional kyphosis was more common in children in whom ≤5 levels had been fused (p = 0.054). CONCLUSIONS: Anteroposterior surgery provided better correction of cervical kyphosis than posterior spinal fusion in children with NF1. Erosion of vertebral bodies continued during the postoperative follow-up period in all patients who had presented with dysplastic changes preoperatively. The cervical spine should be screened in all children with NF1. Fusion should include at least 6 levels to prevent junctional kyphosis. LEVEL OF EVIDENCE: Therapeutic Level III. See Instructions for Authors for a complete description of levels of evidence.

The Management of Soft Tissue and Bone Loss in Type IIIB and IIIC Pediatric Open Tibia Fractures.

PURPOSE: Type III B and C open tibia fractures in children pose a challenge to the orthopaedic surgeon. Limb salvage is the initial goal for the majority of patients, but managing soft-tissue defects and bone loss can be a challenge. The purpose of this study was to evaluate the use of circular external fixation in the management of these injuries. METHODS: In this retrospective review, we examined children with type IIIB and IIIC open tibial fractures treated with circular external fixation and soft-tissue coverage between 1990 and 2010. Chart review included: mechanism and severity of injury, degree of bone and soft-tissue loss, technique and duration of external fixation, additional procedures, clinical and radiographic outcomes, and complications. RESULTS: Eight patients were identified whose average age at the time of injury was 10.4 years (range, 3.8 to 15.3 y). There were 7 type IIIB and 1 type IIIC fractures. All patients received free or rotational soft-tissue flaps. Average bone loss was 5.4 cm (range, 0 to 12 cm). Three techniques of circular external fixation were used, including: (1) static stabilization to allow for soft-tissue coverage and fracture healing, (2) acute shortening with plan for later limb lengthening, and (3) stabilization of the extremity for soft-tissue coverage and intended bone transport. Seven of 8 limbs were salvaged. Of those 7, all were followed to skeletal maturity and ambulating without assistive devices at final follow-up. Three patients had a clinically relevant leg-length discrepancy (≥2 cm). Four of 8 patients required secondary or contralateral procedures. CONCLUSIONS: Pediatric type IIIB and IIIC tibia fractures are limb-threatening injuries that require dynamic thinking and management as the bone and soft-tissue injuries evolve. We have proposed a general algorithm to guide the treatment of these severe injuries. In our experience, circular external fixation, in conjunction with this algorithm, provides the appropriate stability and environment for managing soft tissue and bone loss and can facilitate limb salvage. LEVEL OF EVIDENCE: Level IV.

Mutations Preventing Regulated Exon Skipping in MET Cause Osteofibrous Dysplasia.

The periosteum contributes to bone repair and maintenance of cortical bone mass. In contrast to the understanding of bone development within the epiphyseal growth plate, factors that regulate periosteal osteogenesis have not been studied as intensively. Osteofibrous dysplasia (OFD) is a congenital disorder of osteogenesis and is typically sporadic and characterized by radiolucent lesions affecting the cortical bone immediately under the periosteum of the tibia and fibula. We identified germline mutations in MET, encoding a receptor tyrosine kinase, that segregate with an autosomal-dominant form of OFD in three families and a mutation in a fourth affected subject from a simplex family and with bilateral disease. Mutations identified in all families with dominant inheritance and in the one simplex subject with bilateral disease abolished the splice inclusion of exon 14 in MET transcripts, which resulted in a MET receptor (MET(Δ14)) lacking a cytoplasmic juxtamembrane domain. Splice exclusion of this domain occurs during normal embryonic development, and forced induction of this exon-exclusion event retarded osteoblastic differentiation in vitro and inhibited bone-matrix mineralization. In an additional subject with unilateral OFD, we identified a somatic MET mutation, also affecting exon 14, that substituted a tyrosine residue critical for MET receptor turnover and, as in the case of the MET(Δ14) mutations, had a stabilizing effect on the mature protein. Taken together, these data show that aberrant MET regulation via the juxtamembrane domain subverts core MET receptor functions that regulate osteogenesis within cortical diaphyseal bone.

Genomic Analyses of Patients With Unexplained Early-Onset Scoliosis.

STUDY DESIGN: To test for rare genetic mutations, a cohort of patients with unexplained early-onset scoliosis (EOS) was screened using high-density microarray genotyping. A cohort of patients with adolescent idiopathic scoliosis (AIS) was similarly screened and the results were compared. SUMMARY OF BACKGROUND DATA: Patients with scoliosis in infancy or early childhood (EOS) are at high risk for progressive deformity and associated problems including respiratory compromise. Early-onset scoliosis is frequently associated with genetic disorders but many patients present with nonspecific clinical features and without an associated diagnosis. The authors hypothesized that EOS in these patients may be caused by rare genetic mutations detectable by next-generation genomic methods. METHODS: The researchers identified 24 patients with unexplained EOS from pediatric orthopedic clinics. They genotyped them, along with 39 connecting family members, using the Illumina OmniExpress-12, version 1.0 beadchip. Resulting genotypes were analyzed for chromosomal changes, specifically copy number variation and absence of heterozygosity. They screened 482 adolescent idiopathic scoliosis (AIS) patients and 744 healthy controls, who were similarly genotyped with the same beadchip, for chromosomal changes identified in the EOS cohort. RESULTS: Copy number variation and absence of heterozygosity analyses revealed a genetic diagnosis of chromosome 15q24 microdeletion syndrome in 1 patient and maternal uniparental disomy of chromosome 14 in a second one. Prior genetic testing and clinical evaluations had been negative in both cases. A large novel chromosome 10 deletion was likely causal in a third EOS patient. These mutations identified in the EOS patients were absent in AIS patients and controls, and thus were not associated with AIS or found in asymptomatic individuals. CONCLUSIONS: These data underscore the usefulness of updated genetic evaluations including high-density microarray-based genotyping and other next-generation methods in patients with unexplained EOS, even when prior genetic studies were negative. These data also suggest the intriguing possibility that other mutations detectable by whole genome sequencing, as well as epigenetic effects, await discovery in the EOS population.

Serial casting as a delay tactic in the treatment of moderate-to-severe early-onset scoliosis.

BACKGROUND: Serial casting can cure mild infantile idiopathic scoliosis. Its use in delaying surgery in older children and those with larger curves or syndromes is poorly defined. METHODS: A review of a single center's experience with casting was performed. Patients were included if they had a syndromic, neuromuscular, or congenital scoliosis or were older than 2.5 years with an idiopathic scoliosis measuring >50 degrees. RESULTS: A retrospective review was performed on 29 patients meeting all inclusion criteria. Of these, 12 were idiopathic and 17 were nonidiopathic curves. Average age at first cast was 4.4 ± 2.1 years, and 3.0 ± 1.8 cast changes were performed over 1.4 ± 1.1 years. Patients were transitioned to a brace and followed up for 5.5 years (range, 2.2 to 11.4 y). The main thoracic Cobb angle before casting was 68.8 ± 12.3 degrees, which corrected to 39.1 ± 16.4 degrees in a cast. Cobb angle after cast removal was 60.9 ± 18.4 degrees, which increased to 76.3 ± 24.0 degrees at final follow-up. T1-T12 height increased to 1.1 ± 2.6 cm during the treatment period (P=0.05). There were 5 minor complications. Fifteen patients (51.7%) required surgical treatment for their scoliosis at most recent follow-up and an additional 7 patients (24.1%) were delayed until a definitive anterior/posterior spinal fusion could be performed. Surgery was delayed 39 ± 25 months from the first cast. Growing rods were required in 8 patients (27.6%). The patients who ultimately underwent surgical intervention (SG) were more likely to have a larger postcasting residual main thoracic Cobb angle than those who did not require surgery [NS; 69.5 ± 14.6 degrees (SG) vs. 51.6 ± 17.9 degrees (NS), P=0.007] and had a greater progression of their curves after cast removal [20.9 ± 13.5 degrees (SG) vs. 9.4 ± 11.0 degrees (NS), P=0.02]. CONCLUSIONS: Serial casting is a viable alternative to surgical growth sparing techniques in moderate-to-severe early-onset scoliosis and may help delay eventual surgical intervention. Although a cure cannot be expected, an average of 39 months of delay was achieved in this patient cohort and 72.4% have avoided growing spine surgery. LEVEL OF EVIDENCE: Level IV, case series.