22q11.2 Deletion Syndrome as a Human Model for Idiopathic Scoliosis.

To better understand the etiology of idiopathic scoliosis, prospective research into the pre-scoliotic state is required, but this research is practically impossible to carry out in the general population. The use of 'models', such as idiopathic-like scoliosis established in genetically modified animals, may elucidate certain elements, but their translatability to the human situation is questionable. The 22q11.2 deletion syndrome (22q11.2DS), with a 20-fold increased risk of developing scoliosis, may be a valuable and more relevant alternative and serve as a human 'model' for idiopathic scoliosis. This multicenter study investigates the morphology, dynamic behavior, and presence of intraspinal anomalies in patients with 22q11.2DS and scoliosis compared to idiopathic scoliosis. Scoliosis patients with 22q11.2DS and spinal radiography (n = 185) or MRI (n = 38) were included (mean age 11.6 ± 4.2; median Cobb angle 16°) and compared to idiopathic scoliosis patients from recent literature. Radiographic analysis revealed that 98.4% of 22q11.2DS patients with scoliosis had a curve morphology following predefined criteria for idiopathic curves: eight or fewer vertebrae, an S-shape and no inclusion of the lowest lumbar vertebrae. Furthermore, curve progression was present in 54.2%, with a mean progression rate of 2.5°/year, similar to reports on idiopathic scoliosis with 49% and 2.2-9.6°/year. The prevalence of intraspinal anomalies on MRI was 10.5% in 22q11.2DS, which is also comparable to 11.4% reported for idiopathic scoliosis. This indicates that 22q11.2DS may be a good model for prospective studies to better understand the etiology of idiopathic scoliosis.

The 22q11.2 deletion syndrome as a model for idiopathic scoliosis - A hypothesis.

Adolescent idiopathic scoliosis (AIS), defined as a lateral deviation of the spine of at least ten degrees, is a classic enigma in orthopaedics and affects 1-4% of the general population. Despite (over) a century of intensive research, the etiology is still largely unknown. One of the major problems in all existing AIS research is the fact that most patients come to medical attention after onset of the curve. Therefore, it is impossible to know whether current investigated parameters are causative, or an effect of the scoliosis. Moreover, up until now there is no known animal model that captures the core features of AIS. In order to identify causal pathways leading to AIS we propose another approach, which has been of great value in other medical disciplines: To use a subset of the population, with a higher risk for a certain disease as a "model" for the general population. Such a "model" may allow the identification of causative mechanisms that might be applicable to the general population. The 22q11.2 deletion syndrome (22q11.2DS) is the most common microdeletion syndrome and occurs in ∼1:3000-6000 children and 1:1000 pregnancies. Nearly half of the population of patients with 22q11.2DS develop a scoliosis that in most cases resembles AIS as far as age at onset and curve pattern. We postulate that within 22q11.2DS certain causal pathways leading to scoliosis can be identified and that these are applicable to the general population.

Scoliosis in association with the 22q11.2 deletion syndrome: an observational study.

OBJECTIVE: The 22q11.2 deletion syndrome (22q11.2DS) is the most common microdeletion syndrome in humans. It is characterised by wide phenotypic variability, including congenital heart disease (CHD), immunodeficiency and scoliosis. However, little is known regarding the prevalence and characteristics of scoliosis in patients with 22q11.2DS. The objective of this study is to assess the prevalence of scoliosis, its characteristics and the association with CHD in patients with 22q11.2DS. DESIGN: This prevalence study is based on physical examination and questionnaires of the world's largest 22q11.2DS longitudinal collected database (n=1393, Children's Hospital of Philadelphia) and was augmented with the scoliosis prevalence based on radiography in a smaller cohort (cross-sectional, University Medical Center Utrecht). PATIENTS: Patients with a laboratory-confirmed 22q11.2 deletion who visited the specialised outpatient clinics were considered for inclusion. MAIN OUTCOME MEASURES: (1) The prevalence of scoliosis, (2) its association with CHD, and (3) the similarity between 22q11.2DS curve patterns and adolescent idiopathic scoliosis (AIS) curve patterns. RESULTS: Within the Philadelphia cohort, the prevalence of scoliosis in patients older than 16 years (n=317) was 48% (n=152). A similar prevalence (49%) was shown for the younger Utrecht cohort (n=97). The occurrence of scoliosis was not associated with the presence of CHD. Sixty-three per cent of patients with scoliosis had a scoliotic curve pattern that resembled AIS. CONCLUSIONS: Clinicians should be aware that scoliosis is highly prevalent (48%-49%) in association with 22q11.2DS, irrespective of other clinical features (eg, the presence of CHD). Furthermore, 22q11.2DS may provide insights into the causes of AIS.

Club foot in association with the 22q11.2 deletion syndrome: An observational study.

The 22q11.2 Deletion Syndrome (22q11.2DS) occurs in ~1:3,000-6,000 individuals. Features less typically associated with 22q11.2DS, such as orthopedic manifestations, may be overlooked or may not lead to appropriate diagnostic testing. Club foot has a general population prevalence of ~1:1,000 and has been occasionally described in association with 22q11.2DS. Our hypothesis is that the prevalence of club foot is higher in patients with 22q11.2DS. We performed a retrospective review in two specialized 22q11.2DS centers to determine the prevalence of club foot. "True club foot" requires treatment (either conservative or surgical), therefore we only included those patients with proof of treatment. We investigated whether congenital heart disease (CHD) and/or cleft palate were associated with the presence of club foot within 22q11.2DS. The records of 1,466 patients were reviewed. Of these, 48 (3.3%) had confirmation of club foot (95% Confidence Interval: 2.4-4.3): 22 (46%) had a bilateral, 12 (25%) left, and 14 (29%) right club foot. Within our study, neither a CHD and/or a cleft palate were associated with a club foot. The prevalence of club foot in 22q11.2DS is 30 times higher than that observed in the general population. This suggests the diagnosis of club foot, especially in the face of other typically associated abnormalities of 22q11.2DS, should provoke consideration of 22q11.2DS as an underlying diagnosis, particularly in the neonatal setting.

What is new with 22q? An update from the 22q and You Center at the Children's Hospital of Philadelphia.

22q11.2 deletion syndrome (22q11.2DS) is a disorder caused by recurrent, chromosome-specific, low copy repeat (LCR)-mediated copy-number losses of chromosome 22q11. The Children's Hospital of Philadelphia has been involved in the clinical care of individuals with what is now known as 22q11.2DS since our initial report of the association with DiGeorge syndrome in 1982. We reviewed the medical records on our continuously growing longitudinal cohort of 1,421 patients with molecularly confirmed 22q11.2DS from 1992 to 2018. Most individuals are Caucasian and older than 8 years. The mean age at diagnosis was 3.9 years. The majority of patients (85%) had typical LCR22A-LCR22D deletions, and only 7% of these typical deletions were inherited from a parent harboring the deletion constitutionally. However, 6% of individuals harbored other nested deletions that would not be identified by traditional 22q11.2 FISH, thus requiring an orthogonal technology to diagnose. Major medical problems included immune dysfunction or allergies (77%), palatal abnormalities (67%), congenital heart disease (64%), gastrointestinal difficulties (65%), endocrine dysfunction (>50%), scoliosis (50%), renal anomalies (16%), and airway abnormalities. Median full-scale intelligence quotient was 76, with no significant difference between individuals with and without congenital heart disease or hypocalcemia. Characteristic dysmorphic facial features were present in most individuals, but dermatoglyphic patterns of our cohort are similar to normal controls. This is the largest longitudinal study of patients with 22q11.2DS, helping to further describe the condition and aid in diagnosis and management. Further surveillance will likely elucidate additional clinically relevant findings as they age.

Orthopaedic manifestations within the 22q11.2 Deletion syndrome: A systematic review.

The 22q11.2 Deletion Syndrome (22q11.2DS) is the most common microdeletion syndrome with an estimated prevalence of 1:4,000 live births. 22q11.2DS is known to have wide phenotypic variability, including orthopaedic manifestations. The purpose of this systematic review is to increase the awareness of orthopaedic manifestations associated with 22q11.2DS. This systematic review was performed according to the PRISMA Guidelines. Original epidemiological studies on the prevalence of orthopaedic manifestations within 22q11.2DS were systematically searched for in PubMed and EMBASE. The included articles were scored according to a risk-of-bias tool, a best-evidence synthesis was performed and the prevalence data was extracted. Sixty-nine published manuscripts described 58 orthopaedic manifestations in a total of 6,055 patients. The prevalence of at least one cervical or occipital anomaly is 90.5-100% (strong evidence). Fourteen studies (n = 2,264) revealed moderate evidence for a wide scoliosis prevalence of 0.6-60%. Two studies demonstrated that 5-6.4% of all 22q11.2DS patients required surgical scoliosis correction. Fifteen studies (n = 2,115) reported a 1.1-13.3% prevalence of clubfoot with moderate evidence. Other reported orthopaedic manifestations are patellar dislocation (10-20%), juvenile rheumatic arthritis (3.75%), impaired growth and skeletal anomalies like polydactyly (1.0-3.7%), syndactyly (11-11.8%), butterfly vertebrae (11.1%) and 13 ribs (2-19%). Orthopaedic findings are important manifestations of the 22q11.2DS, both in bringing patients to diagnostic attention and in requiring surveillance and appropriate intervention. Data on these manifestations are scattered and incomprehensive. Routinely screening for cervical anomalies, scoliosis, and upper and lower limb malformations is recommended in this vulnerable group of patients.

Does a Weekly Didactic Conference Improve Resident Performance on the Pediatric Domain of the Orthopaedic In-Training Examination?

BACKGROUND: Performance on the Orthopaedic In-training Examination (OITE) has been correlated with performance on the written portion of the American Board of Orthopaedic Surgery examination. Herein we sought to discover whether adding a regular pediatric didactic lecture improved residents' performance on the OITE's pediatric domain. METHODS: In 2012, a didactic lecture series was started in the University of Pittsburgh Medical Center (UPMC) Hamot Orthopaedic Residency Program (Hamot). This includes all topics in pediatric orthopaedic surgery and has teaching faculty present, and occurs weekly with all residents attending. A neighboring program [UMPC Pittsburgh (Pitt)] shares in these conferences, but only during their pediatric rotation. We sought to determine the effectiveness of the conference by comparing the historic scores from each program on the pediatric domain of the OITE examination to scores after the institution of the conference, and by comparing the 2 programs' scores. RESULTS: Both programs demonstrated improvement in OITE scores. In 2008, the mean examination score was 19.6±4.3 (11.0 to 30.0), and the mean percentile was 57.7±12.6 (32.0 to 88.0); in 2014, the mean examination score was 23.5±4.2 (14.0 to 33.0) and the mean percentile was 67.1±12.1 (40.0 to 94.0). OITE scores and percentiles improved with post graduate year (P<0.0001). Compared with the preconference years, Hamot residents answered 3.99 more questions correctly (P<0.0001) and Pitt residents answered 2.93 more questions correctly (P<0.0001). Before the conference, site was not a predictor of OITE score (P=0.06) or percentile (P=0.08); there was no significant difference found between the mean scores per program. However, in the postconference years, site did predict OITE scores. Controlling for year in training, Hamot residents scored higher on the OITE (2.3 points higher, P=0.003) and had higher percentiles (0.07 higher, P=0.004) than Pitt residents during the postconference years. CONCLUSIONS: This study suggests that adding a didactic pediatric lecture improved residents' scores on the OITE and indirectly suggests that more frequent attendance is associated with better scores. LEVEL OF EVIDENCE: Level III-retrospective case-control study.

Pulmonary and Radiographic Outcomes of VEPTR (Vertical Expandable Prosthetic Titanium Rib) Treatment in Early-Onset Scoliosis.

BACKGROUND: VEPTR (vertical expandable prosthetic titanium rib) expansion thoracoplasty is used to manage thoracic insufficiency syndrome in early-onset scoliosis. Literature regarding the effects of this technique on pulmonary function is scarce. The aim of this study was to report the intermediate-term results of VEPTR expansion thoracoplasty. METHODS: Twenty-one children with thoracic insufficiency syndrome underwent VEPTR expansion thoracoplasty from 2002 to 2012 and had complete chart data, preoperative and follow-up radiographs, and pulmonary function tests performed at the index implantation, first expansion, and last expansion. Pulmonary function tests with forced and passive deflation techniques developed for children under general anesthesia were performed prior to the index implantation and each expansion surgery under the same anesthetic conditions. Pulmonary and radiographic parameters were analyzed longitudinally. RESULTS: Mean follow-up was six years, and mean age at implantation was 4.8 years. The mean number of expansion procedures per patient was eleven, and the mean number of pulmonary function tests was ten. The mean interval between surgical procedures was 6.4 months. Mean forced vital capacity (FVC) increased from 0.65 to 0.96 L (p < 0.0001). However, the percentage of the predicted FVC decreased from 77% to 58%. Respiratory system compliance normalized on the basis of body weight, Crs/kg, decreased by 39%, from 1.4 to 0.86 mL/cm H2O/kg. The mean Cobb angle before treatment was 80°, and the mean maximum thoracic kyphosis angle was 57° (range, 7° to 107°). The initial coronal correction was maintained at the time of final follow-up (67°); however, there was a trend toward a decrease in the maximum thoracic kyphosis angle (to 66°, p = 0.08). Clinically apparent proximal thoracic kyphosis occurred in four patients, and spinal imbalance occurred in seven. The mean gain in T1-T12 height during the treatment period was 18 mm (2.9 mm/year). CONCLUSIONS: FVC improved over time; however, this increase in lung volume did not keep up with the growth of the child, as the percentage of the predicted FVC decreased, and the chest wall stiffness increased. Coronal correction was maintained, but the increase in proximal thoracic kyphosis is concerning. LEVEL OF EVIDENCE: Therapeutic Level IV. See Instructions for Authors for a complete description of levels of evidence.

Spinal Deformity in Bethlem Myopathy.

STUDY DESIGN: Retrospective review. OBJECTIVES: To report the characteristics of spinal deformity in a series of 3 patients with Bethlem myopathy. SUMMARY OF BACKGROUND DATA: Bethlem myopathy presents with mild muscular weakness and typically has a benign course. Severe scoliosis in patients affected with Bethlem myopathy has not been previously reported. METHODS: Clinical records of 3 brothers with Bethlem myopathy were reviewed. Clinical and radiographic features of the spinal deformity are presented. RESULTS: All 3 patients had progressive scoliosis with coronal and sagittal imbalance. At a minimum of 26 months of follow-up after posterior instrumented fusion, there were no complications and deformity correction was maintained. Posterior instrumentation and fusion did not negatively affect the pulmonary function in this group of patients with Bethlem myopathy. CONCLUSIONS: Bethlem myopathy may present with severe scoliosis along with proximal muscle weakness. This condition should be included in the differential diagnosis of adolescent patients with progressive spinal deformity.

Somatosensory-evoked potential monitoring during instrumented scoliosis corrective procedures: validity revisited.

BACKGROUND: Intraoperative monitoring (IOM) using somatosensory-evoked potentials (SSEPs) plays an important role in reducing iatrogenic neurologic deficits during corrective pediatric idiopathic procedures for scoliosis. However, for unknown reasons, recent reports have cited that the sensitivity of SSEPs to detect neurologic deficits has decreased, in some to be less than 50%. This current trend, which is coincident with the addition of transcranial motor-evoked potentials, is surprising given that SSEPs are robust, reproducible responses that were previously shown to have sensitivity and specificity of >90%. PURPOSE: Our primary aim was to assess whether SSEPs alone can detect impending neurologic deficits with similar sensitivity and specificity as originally reported. Our secondary aim was to estimate the potential predictive value of adding transcranial motor-evoked potentials to SSEP monitoring in idiopathic scoliosis procedures. DESIGN: This was a retrospective review to analyze the efficacy of SSEP monitoring in the group of pediatric instrumented scoliosis fusion cases. PATIENT SAMPLE: We retrospectively reviewed all consecutive cases of patients who underwent idiopathic scoliosis surgery between 1999 and 2009 at Children's Hospital of Pittsburgh. We identified 477 patients who had the surgery with SSEP monitoring alone. Exclusion criteria included any patients with neuromuscular disorders or unreliable SSEP monitoring. Patients who had incomplete neurophysiology data or incomplete postoperative records were also excluded. OUTCOME MEASURES: Major outcomes measured were clinically significant postoperative sensory or motor deficits, as well as significant intraoperative SSEP changes. METHODS: Continuous interleaved upper- and lower-extremity SSEPs were obtained throughout the duration of all procedures. We considered a persistent 50% reduction in primary somatosensory cortical amplitude or a prolongation of response latency by >10% from baseline to be significant. Persistent changes represent significant deviation in SSEP amplitude or latency in more than two consecutive averaged trials. Patients were classified into one of four categories with respect to SSEP monitoring: true positive, false positive, true negative, and false negative. The sensitivity, specificity, positive predictive value, and negative predictive value were then calculated accordingly. RESULTS: Our review of 477 idiopathic scoliosis surgeries monitored using SSEPs alone revealed a new deficit rate of 0.63% with no cases of permanent injury. Sensitivity = 95.0%, specificity = 99.8%, positive predictive value = 95%, negative predictive value = 99.8%. Using evidence-based epidemiologic measures, we calculated that the number needed to treat was 1,587 patients for one intervention to be performed that would have been missed by SSEP monitoring alone. In addition, the number needed to harm, which represents the increase in false positives with the addition of transcranial electrical motor-evoked potentials, was 200. CONCLUSION: SSEP monitoring alone during idiopathic scoliosis continues to be a highly reliable method for the detection and prevention of iatrogenic injury. Our results confirm the high sensitivity and specificity of SSEP monitoring alone published in earlier literature. As such, we suggest the continued use of SSEP alone in idiopathic scoliosis surgeries. At this time we do not believe there are sufficient data to support the addition of MEP monitoring, although more studies and revised criteria for the use of MEP may provide added value for its use in the future.

VEPTR to treat nonsyndromic congenital scoliosis: a multicenter, mid-term follow-up study.

BACKGROUND: Traditional surgical management of multiple congenital vertebral anomalies in young children, including fusion in situ and hemiepiphyseodesis, do not promote spinal growth nor address the associated thoracic insufficiency syndrome. We hypothesize that vertical expandable prosthetic titanium rib (VEPTR) with expansion thoracoplasty may control spinal deformity, allow spinal growth, and address thoracic insufficiency syndrome in children with nonsyndromic complex congenital spinal deformities. METHODS: Eight pediatric spine centers prospectively entered clinical and radiographic data into a database on every congenital spinal deformity treated with VEPTR as part of an Food and Drug Administration study. We retrospectively reviewed these data and excluded patients with spina bifida, Jarcho-Levin, or other syndromes. Data analysis focused on surgical technique and expansion frequency, change in Cobb angle and thoracic heights, and adverse events for a consecutive series of patients with at least 2 years of follow-up. RESULTS: Twenty-four children with an average age at surgery of 3.3 years (range, 1.0 to 12.5 y) were treated with VEPTR insertion and expansion thoracostomy and were followed for an average of 40.7 months (range, 25 to 78 mo). Twenty-three (95.8%) had associated rib fusions. All patients had subsequent expansion surgery; 50% had 5 or more expansions. Twenty patients (83.3%) had an improvement in Cobb angle during treatment with an average improvement of 8.9 degrees. All had an increase in thoracic height, with a mean increase of 3.41 cm. The most common adverse events were device migration in 7 patients and infection or skin problems in 6 patients. CONCLUSIONS: VEPTR insertion with expansion thoracoplasty represents a successful treatment paradigm for nonsyndromic congenital spinal deformities. We report multicenter data with midterm follow-up of children without syndromic diagnoses, in which the vast majority had an improvement in Cobb angle and thoracic height over the treatment period. Challenges include the demands of multiple procedures, skin problems, and device migration. LEVEL OF EVIDENCE: Level IV-prognostic study.

Acellular dermal matrix in the treatment and prevention of exposed vertical expandable prosthetic titanium ribs.

STUDY DESIGN: Case series. OBJECTIVE: To illustrate the use of acellular dermal matrix (ADM) in treatment and prevention of exposed vertical expandable prosthetic titanium rib (VEPTR) implants. SUMMARY OF BACKGROUND DATA: In the pediatric population with severe kyphoscoliosis, VEPTR is an effective tool during growth for the correction of ribcage deformity. Prolonged VEPTR therapy can result in wound breakdown, implant exposure, and infection. Treatment includes the use of prolonged antibiotics, muscle flaps, and, when salvage fails, removal of the VEPTR. The use of ADM in the treatment and prevention of VEPTR exposure has not been previously described. METHODS: Between January 2002 and January 2010, eight patients who underwent placement of ADM for the treatment and prevention of exposed VEPTR devices were identified. Their records were reviewed for diagnosis, sex, age of patient at initial VEPTR placement, position of VEPTR placement, number of VEPTR expansions, wound complications, ADM use, adjunct procedures, and length of wound follow-up. RESULTS: ADM was used in eight patients. In five patients ADM was used for compromised soft tissue overlying the VEPTR and threatened exposure of the hardware. In these cases, subsequent expansions occurred without incident and the wound remained stable with an average follow-up of 7.6 months. In three patients, ADM was used for exposed VEPTR hardware secondary to wound breakdown. Average follow-up was 3.3 months. In two of the three cases of exposed and contaminated hardware, stable soft tissue coverage was achieved and continued VEPTR therapy was achieved. One of the three cases of exposure involved infected and prominent hardware with purulence. This patient failed to clear the infection and required complete device removal. CONCLUSION: ADM can treat and prevent exposed VEPTR, allowing subsequent VEPTR expansions and minimizing the need for muscle flap coverage and/or implant removal and replacement.

Thoracic malformation with early-onset scoliosis: effect of serial VEPTR expansion thoracoplasty on lung growth and function in children.

The effect on pulmonary function of serial VEPTR expansion thoracoplasty was studied longitudinally in anesthetized children with spondylothoracic dysplasia using a special mobile unit. The median age of 24 children at the start of surgery was 4.6 years (1.8-10.8) and most exhibited a moderate-to-severe restrictive lung defect. After a median of 3.2 years (1.0-6.5), their forced vital capacity (FVC) was found to have increased by an average of 11.1%/year. The rate of increase was greater in children who were younger than 6 years at the start of the study than in older children (14.5% versus 6.5%, p<0.01). The average specific respiratory system compliance (C(rs)) was mildly-to-moderately decreased at the start, and over the study it decreased on average to 56% of the initial value in spite of clinically successful expansion thoracoplasty and lung growth, indicating increasing stiffness of the thorax with growth.

Effects on lung function of multiple expansion thoracoplasty in children with thoracic insufficiency syndrome: a longitudinal study.

STUDY DESIGN: Longitudinal study of intraoperative pulmonary function in young children with thoracic hypoplasia and scoliosis undergoing multiple expansion thoracoplasty using the vertical expandable prosthetic titanium ribs (VEPTRs). OBJECTIVE: To test the long-term efficacy of VEPTR multiple expansion thoracoplasty. SUMMARY OF BACKGROUND DATA: To our knowledge, no direct measurements of pulmonary function have been reported with VEPTR expansion thoracoplasty. METHODS: There were 10 children with thoracic insufficiency syndrome, secondary to thoracic hypoplasia with progressive scoliosis, studied. A mobile pulmonary function laboratory unit was used to study forced vital capacity (FVC), maximum expiratory flow volume curves, and respiratory system compliance (Crs) with the patient under general anesthesia immediately before and after expansion thoracoplasty. Studies were repeated every 6 months at each subsequent operation for expansion thoracoplasty for the duration up to 33 months. RESULTS: At the baseline studies, FVC showed a moderate-to-severe decrease (69% of predicted values), indicating the presence of significant restrictive lung defect. Only 1 of 10 children had severe airway obstruction. The baseline Crs was markedly decreased in part because of the presence of significant atelectasis. Crs increased with an average of 42% after repeated hyperinflation (deep sighs). There was no change in lung volume or function immediately before versus after completion of expansion thoracoplasty. FVC increased significantly over time, with an average rate of 26.8% per year, the rate of increase similar to that of healthy children of comparative ages. In terms of percent-predicted values, FVC did not change significantly between the baseline and last test, indicating that in most children studied, lung growth kept up with body growth. CONCLUSION: Although it is difficult to assess the extent of the efficacy without a proper or historical control group for comparison, the present study indicates that in children with severe thoracic insufficiency syndrome, the insertion of VEPTRs with multiple expansion thoracoplasties is beneficial over time, by allowing the lungs to expand with body growth without further deterioration in lung function.

Spica cast application in the emergency room for select pediatric femur fractures.

OBJECTIVES: This study was designed to evaluate the efficacy and safety of immediate spica casting in the emergency room (ER) and evaluate the effect of discharge from the emergency room on short- term complications. DESIGN: Retrospective review of patients treated with immediate spica casting in the ER between June 1, 1993 and July 30, 2001. SETTING: Major, pediatric, orthopaedic trauma and referral center. PATIENTS: A total of 145 pediatric femur fractures in children, younger than age 7 years, treated with immediate spica casting in the ER were reviewed to determine radiographic outcome and short-term complication rates. INTERVENTION: All patients underwent immediate spica cast placement in the ER under conscious sedation. Patients meeting specific criteria were discharged immediately from the ER. MAIN OUTCOME MEASUREMENTS: Radiographic acceptability of alignment at fracture union (angulation, shortening), loss of reduction, number of return visits to the emergency room, and clinical outcome at final follow-up. RESULTS: Average follow-up was 20 +/- 16 weeks (range, 1-9 months). Forty-eight patients (33%) were discharged from the ER. No clinical complications were noted at last follow-up. All children younger than age 2 years, and 86.5% of children ages 2 to 5 years, met acceptable malalignment parameters on final radiographs. There were 16 ER visits (11%) for cast problems. Re-reduction in the operating room was needed in 11 patients (8.9%); 6.9% of patients had a cast problem noted during follow-up visits. Only 9% of patients developed a major complication. Initial shortening was the only independent risk factor found to be associated with loss of reduction. Admission status had no significant effect on the number of ER visits or development of a complication. CONCLUSIONS: If there are no associated factors requiring admission (ie, child abuse or polytrauma), spica casting in the ER for pediatric femur fractures followed by immediate discharge can be safely performed with a low complication rate in children younger than age 6 years, nearly eliminating the need for general anesthesia.