Real-world effectiveness of burosumab in children with X-linked hypophosphatemic rickets.

BACKGROUND: X-linked hypophosphatemic rickets (XLH) is the most common cause of inherited rickets. Historically, XLH was treated with oral phosphate and calcitriol (conventional treatment). Burosumab, a fibroblast growth factor 23 (FGF-23) monoclonal antibody, was approved by the United States Food and Drug Administration (FDA) in 2018 for XLH treatment. Nevertheless, conventional treatment of XLH continues to be recommended by some specialists due to lack of published experience with burosumab in the clinical setting. We compared laboratory and radiographic changes observed following transition from conventional therapy to burosumab in pediatric XLH patients as part of routine care. METHODS: This retrospective single-center study identified and retroactively studied twelve patients aged 1-18 years old with XLH previously treated with conventional therapy and transitioned to burosumab. Laboratory studies and radiographs were obtained routinely as standard of care during two treatment periods: (1) conventional therapy and (2) burosumab treatment. Laboratory values and radiologic rickets severity scores were compared between periods. RESULTS: All laboratory values demonstrated improvement following 1 month of burosumab treatment, findings which were sustained over the 2-year study period. Rickets severity scores and height z-scores also improved with burosumab. There were no serious adverse events with burosumab, and adverse events overall were very infrequent and mild. One patient developed an asymptomatic mild elevation of serum phosphate while taking burosumab resulting in a temporary pause in therapy. CONCLUSIONS: Safety and effectiveness of burosumab in treatment of XLH were demonstrated as burosumab yielded statistically significant improvement in laboratory and radiographic markers of rickets and height compared to conventional therapy. A higher resolution version of the Graphical abstract is available as Supplementary information.

The diagnosis and management of common childhood orthopedic disorders: An update.

Musculoskeletal illness represents a significant portion of office visits to primary and urgent care clinicians. Despite this, little emphasis is placed on learning pediatric orthopaedics during medical school or residency. The focus of this paper is to provide a systematic approach to this general musculoskeletal physical exam and to assist in the recognition of what conditions are normal development and what conditions require observation, workup and referral to an experienced pediatric orthopaedist.

KIAA1217: A novel candidate gene associated with isolated and syndromic vertebral malformations.

Vertebral malformations (VMs) are caused by alterations in somitogenesis and may occur in association with other congenital anomalies. The genetic etiology of most VMs remains unknown and their identification may facilitate the development of novel therapeutic and prevention strategies. Exome sequencing was performed on both the discovery cohort of nine unrelated probands from the USA with VMs and the replication cohort from China (Deciphering Disorders Involving Scoliosis & COmorbidities study). The discovery cohort was analyzed using the PhenoDB analysis tool. Heterozygous and homozygous, rare and functional variants were selected and evaluated for their ClinVar, HGMD, OMIM, GWAS, mouse model phenotypes, and other annotations to identify the best candidates. Genes with candidate variants in three or more probands were selected. The replication cohort was analyzed by another in-house developed pipeline. We identified rare heterozygous variants in KIAA1217 in four out of nine probands in the discovery cohort and in five out of 35 probands in the replication cohort. Collectively, we identified 11 KIAA1217 rare variants in 10 probands, three of which have not been described in gnomAD and one of which is a nonsense variant. We propose that genetic variations of KIAA1217 may contribute to the etiology of VMs.

Physical Activity Assessment and Counseling in Pediatric Clinical Settings.

Physical activity plays an important role in children's cardiovascular health, musculoskeletal health, mental and behavioral health, and physical, social, and cognitive development. Despite the importance in children's lives, pediatricians are unfamiliar with assessment and guidance regarding physical activity in children. With the release of the 2018 Physical Activity Guidelines by the US Department of Health and Human Services, pediatricians play a critical role in encouraging physical activity in children through assessing physical activity and physical literacy; providing guidance toward meeting recommendations by children and their families; advocating for opportunities for physical activity for all children in schools, communities, and hospitals; setting an example and remaining physically active personally; advocating for the use of assessment tools and insurance coverage of physical activity and physical literacy screening; and incorporating physical activity assessment and prescription in medical school curricula.

Increasing Wellness Through Physical Activity in Children With Chronic Disease and Disability.

Children with chronic medical conditions face many challenges when considering sport participation. Compared with their healthy counterparts, they are often discouraged from physical activity or sports participation because of real or perceived limitations imposed by their condition. Prescribed exercise should be based on the demands of the sport, the effect of the disease on performance, and the potential for exercise-induced acute or chronic worsening of the illness or disability. This article will focus on several examples of chronic medical conditions and the clinician's role in providing advice about sport participation.

To Cast, to Saw, and Not to Injure: Can Safety Strips Decrease Cast Saw Injuries?

BACKGROUND: Placement and removal of fiberglass casts are among the more-common interventions performed in pediatric orthopaedic surgery offices. However, cast removal is associated with abrasive injuries and burns from the oscillating cast saw, and these injuries can occur even when the cast is removed by experienced personnel. It is unknown whether an added barrier, such as a safety strip, can mitigate injuries from blade-to-skin contact during cast removal with the oscillating saw. QUESTIONS/PURPOSES: We asked: (1) Can a safety strip provide a physical barrier during cast removal, decreasing blade-to-skin contact? (2) Does the safety strip lessen heat transfer? (3) Will the use of the safety strip prevent cast pressure from being released when the cast is split? METHODS: Standard long-arm fiberglass casts were removed by experienced and inexperienced healthcare personnel (n = 35) from life-sized pediatric models. A commercially available woven cast saw safety strip, commonly incorporated in waterproof cast constructs, was chosen as the protective strip. Each participant removed a cast with and without the safety strip present. All participants were blinded to the presence or absence of the safety strip at the time of cast removal. The number of touches was compared between cast removal with and without protective strips. A separate model was designed to assess prevention of heat transfer. Temperatures were recorded, using thermocouples, for three designated temperatures. Five to six trials were conducted at each designated temperature for each of two conditions, with and without the safety strip. Finally, to assess if the safety strip would prevent cast pressure from being released, a third model was used. Thirty standard short-arm casts were applied and removed from the arm models by one of the authors. Pressure data were collected from between the padding layers, in casts with and without the safety strip present, after application, univalving and bivalving each cast. RESULTS: Use of the safety strip reduced the number of simulated skin touches compared with casts removed without the safety strip, among experienced users (mean, 9.0 [range, 1-28] versus 0.1 [range, 0-1], mean ratio, 0.0012; 95% CI, 0.002-0.063; p < 0.001) and inexperienced users (mean, 8.5 [range, 0-31] versus 0.6 [range, 0-3], mean ratio, 0.07; 95% CI, 0.03-0.15; p < 0.001). The safety strips decreased heat transfer, preventing temperatures at the cast-skin interface from reaching 50 °C. Finally, after splitting the cast, with the numbers available, there was no increase in the pressure beneath the casts in those with the safety strip present (mean without, 0.23 [SD, 0.070] versus safety strip in the padding 0.20 [SD, 0.091] and safety strip on top padding, 0.21 [SD, 0.090]; p = 0.446 and p = 0.65 respectively). CONCLUSIONS: Our study showed the effectiveness of a safety strip in reducing simulated touches with the oscillating cast saw during cast splitting. Additional studies are warranted to investigate the clinical use and utility of the safety strip in practice. CLINICAL RELEVANCE: The findings of this study suggest that using safety strips in clinical practice could decrease blade-to-skin contact and therefore minimize cast saw injuries. However, validation of these findings in the clinical setting is necessary before drawing a definitive conclusion.

Epidural Baclofen for the Management of Postoperative Pain in Children With Cerebral Palsy.

INTRODUCTION: Children with cerebral palsy undergoing soft tissue and bony procedures often experience pain and spasticity postoperatively. Differentiation of pain from spasticity complicates management, so controlling spasticity with a continuous infusion of baclofen, an antispasmodic, through an already present indwelling epidural catheter holds interest. METHODS: A retrospective chart review was performed of patients with cerebral palsy undergoing single event, multilevel lower extremity surgery at a single institution who received epidural analgesia with or without continuous baclofen infusion. Primary outcomes included need for supplemental narcotic analgesics and benzodiazepines postoperatively. Duration of hospitalization, pain scores, and complications were also evaluated. RESULTS: Forty-four patients were identified, ranging in age from 3 to 17 years, 19 of whom received epidural baclofen. No differences were found in use of supplemental narcotic analgesia, benzodiazepines, or duration of hospitalization. Differences in pain scores were not statistically significant (0.82±0.95 for baclofen vs. 1.48±0.99 for controls) (P=0.391). Mean arterial pressure was lower in patients receiving baclofen (P=0.004). No potential side effects attributable to baclofen were noted. CONCLUSIONS: Continuous epidural baclofen infusion seems unlikely to alter the pain-spasm cycle experienced by patients with cerebral palsy following orthopaedic surgery to a clinically significant degree. More effective, and cost-effective, measures at assessing and controlling pain and muscle spasm should be explored to benefit cerebral palsy patients postoperatively. LEVEL OF EVIDENCE: Level III-therapeutic study.

In Nonobese Children, Fitness and BMI are Independent Predictors of Fasting Insulin.

OBJECTIVE: Although fitness and obesity have been shown to be independent predictors of cardiometabolic disease risk in obese children, this interaction is not well defined in nonobese children. The purpose of this study was to define the relationships between peak aerobic capacity, body composition, and fasting insulin levels in nonobese middle school children. STUDY DESIGN: 148 middle school children (mean age 11.0 ± 2.1 years, 49% male) underwent determination of body mass index (BMI) z-score, fasting glucose, fasting insulin, body composition by DXA scan (lean body mass and body fat percentage), and peak oxygen uptake per kg of lean body mass (VO2peak). Univariate correlations and multivariate regression analysis were used to identify independent predictors of fasting insulin using age, sex, percent body fat, body mass index z-score, and VO2peak. RESULTS: fasting insulin was significantly related to VO2peak (r =-0.37, p < .001), percent body fat (r = .27, p < .001), and BMI z-score (r = .33, p = .002). After inclusion in the multivariate model, VO2peak (p = .018) and body mass index z-score (p = .043) remained significant predictors of fasting insulin, while age (p = .39), sex (p = .49), and percent body fat (p = .72) did not. CONCLUSIONS: Among nonobese middle school children, fasting insulin is independently related to aerobic fitness after accounting for age, sex, and body composition. Public health efforts to reduce cardiometabolic disease risk among all adolescents should include exercise programs to increase cardiovascular fitness.

Reliability of radiographic measures in infants with clubfoot treated with the Ponseti method.

PURPOSE: The aim of this study was two-fold: (1) to determine if radiographic measures can be reliably made in infants being treated with the Ponseti method and (2) to document radiographic changes before and after Achilles tenotomy. METHODS: A retrospective radiographic and chart review was performed on children with clubfoot treated by the Ponseti method at a single institution over a 10-year period. Five independent reviewers measured a series of angles from a lateral forced dorsiflexion radiograph taken prior to and following Achilles tenotomy. These measures were taken in triplicate to determine the intra- and inter-reader reliability of dorsiflexion, tibio-calcaneal, talo-calcaneal, and talo-first metatarsal angles. RESULTS: Thirty-six subjects (56 feet) were treated with the Ponseti method and met the inclusion criteria. The median (range) age of patients at the time of tenotomy was 52 (34-147) days. The intra-reader reliability [intra-rater correlation coefficient (ICC)] for each of the measured angles pre- and post-tenotomy ranged from 0.933 to 0.995 and 0.864 to 0.995, respectively. Similarly, the inter-reader reliabilities (ICC) ranged from 0.727 for the pre-tenotomy (talo-calcaneal) to 0.950 for the post-tenotomy (talo-first metatarsal) angles. The mean differences between pre- and post-tenotomy radiographs were: dorsiflexion increase of 17°, tibio-calcaneal angle increase of 19°, talo-calcaneal angle increase of 9°, and talo-first metatarsal angle increase of 10° (p-value ≤0.001 for all measurements except the talo-first metatarsal angle, with a p-value of 0.001). CONCLUSIONS: Reliable radiographic measures can be made from lateral dorsiflexion radiographs of clubfeet treated with the Ponseti method before and after Achilles tenotomy.

Can Radiographs Predict Outcome in Patients With Idiopathic Clubfeet Treated With the Ponseti Method?

BACKGROUND: The aim of this study was to determine if radiographic measurements, taken before tenotomy, can predict outcome in children with idiopathic clubfoot treated by the Ponseti method. METHODS: A retrospective chart and radiographic review was performed on children with idiopathic clubfoot treated with the Ponseti method over a 10-year period with minimum 2-year follow-up that had a forced dorsiflexion lateral foot radiograph before tenotomy. All angles were measured in duplicate on the pretenotomy radiographs, including: foot dorsiflexion (defined as the 90 minus the angle between the tibial shaft and a plastic plate used to dorsiflex the foot), tibio-calcaneal, talo-calcaneal, and talo-first metatarsal angles. Clinical review of patient records identified different patient outcomes: no additional treatment required, relapse (additional casting and/or surgery required), recurrence (any additional surgery required), or reconstruction (surgery not including repeat tenotomy). RESULTS: Forty-five patients (71 feet) were included in the study. The median age at follow-up was 4.6 years. The intrareader reliability was acceptable for all measures. Thirteen of the 71 (18%) feet required additional surgery, occurring at a median age of 3.6 years. Of the 4 radiographic measures, only pretenotomy foot dorsiflexion predicted recurrence (hazard ratio=0.96, P=0.03). Youden's method identified 16.6 degrees of dorsiflexion as the optimal cutoff. Feet with at least that amount of dorsiflexion pretenotomy (n=21) experienced no recurrences; feet with less than that amount of dorsiflexion (n=50) experienced 13 recurrences (P=0.007). CONCLUSIONS: Reduced foot dorsiflexion on lateral forced dorsiflexion pretenotomy radiograph was associated with an increased risk of recurrence. Radiographic dorsiflexion to 15 degrees past neutral before tenotomy appears to predict successful treatment via the Ponseti method.

Magnetic resonance imaging of the pediatric knee.

In pediatric patients, the high resolution and excellent soft-tissue contrast of magnetic resonance (MR) imaging allows for complete evaluation of osseous and soft-tissue structures around the knee joint, and its lack of ionizing radiation makes it a preferred modality for advanced imaging. Older children and adolescents are most commonly imaged to evaluate athletic and traumatic injuries, whereas in infants and school age children MR imaging is used to evaluate developmental conditions such as Blount disease or assess for causes of atraumatic pain such as infection or inflammatory arthritis. A thorough understanding of normal skeletal development is necessary to avoid misdiagnoses.

Cast-saw injuries: assessing blade-to-skin contact during cast removal. Does experience or education matter?

BACKGROUND: Cast-saw injuries are sustained during cast removal or splitting of a cast when a hot cast-saw blade touches the patient's skin inadvertently during cast removal. Other studies have evaluated risk factors associated with saw-blade temperature, however, none have documented the number and duration of blade-to-skin contacts during cast removal. METHODS: Using a pediatric long-arm model capable of detecting cast-saw blade contact, we tested the ability of health care providers to apply and remove casts before and after a brief education module. The total number and duration of "touches" between the saw and the model's "skin" were recorded. Correlations between user "touches," and experience and comparisons between pre- and post-education "touches" were performed. RESULTS: Of the 18 study participants, 16 touched the model surface with the cast saw; 7 of the 18 participants maintained blade contact with the skin for > 1 second 22 times during the testing process. Participants with less experience averaged 20 (± 16) touches, whereas more experienced participants averaged 24 (± 19) touches (P = 0.7). Average number of touches was similar-before 22 (± 20) and after 25 (± 22); P = 0.5-participants completed an education module. No correlation between experience or participation in the education program was found with decreased number of blade-to-skin touches. CONCLUSION: Nearly all clinicians inadvertently contacted the underlying skin with the cast-saw blade. In our limited sample size, experience and education did not prevent this; therefore, minimizing time of contact and blade temperature may be more important factors in minimizing cast-saw injuries.

Analysis of maternal risk factors associated with congenital vertebral malformations.

STUDY DESIGN: A retrospective medical record review of cases with congenital vertebral malformations (CVMs) and controls with normal spine morphology. OBJECTIVE: To determine the relative contribution of maternal environmental factors (MEFs) during pregnancy to CVM development. SUMMARY OF BACKGROUND DATA: CVMs represent defects in formation and segmentation of somites occurring with an estimated incidence of between 0.13 and 0.50 per 1000 live births. CVMs may be associated with various phenotypes and represent significant morbidity due to pain and cosmetic disfigurement. METHODS: A multicenter retrospective medical record review of 229 cases with CVM and 267 controls with normal spine morphology between the ages of 1 and 50 years was performed to obtain the odds ratio (OR) of MEF related to CVM among cases versus controls. An imputation-based analysis was performed in which subjects with no documentation of MEF history were treated as "no maternal exposure." Univariate and multivariate analyses were conducted to calculate the OR. RESULTS: Of the 229 total cases, 104 cases had single or multiple CVMs without additional congenital malformations (group 1) and 125 cases had single or multiple CVMs and additional congenital malformations (group 2). Nineteen percent of total cases had an identified MEF. The OR for MEF history for group 1 was 6.0 (95% confidence interval, 2.4-15.1; P < 0.001) in the univariate analysis. The OR for MEF history in group 2 was 9.1 (95% confidence interval, 3.8-21.6, P < 0.001) in the univariate analysis. The results were confirmed in the multivariate analysis after adjusting for age, sex, and institution. CONCLUSIONS: These results support a hypothesis for an association between these MEFs during pregnancy and CVM and have implications for development of prevention strategies. Further prospective studies are needed to quantify association between CVMs and specific MEF. LEVEL OF EVIDENCE: 4.

Variations in AP and frog-leg pelvic radiographs in a pediatric population.

INTRODUCTION: It is common for pediatric patients with a history of hip dysplasia to undergo anteroposterior (AP) and frog-leg (FL) lateral radiographs to monitor development of the hip. Because of sensitivity of pediatric tissues to ionizing radiation, we sought to document the relationship between AP and FL radiographs and to determine which image was better to assess acetabular dysplasia. METHODS: An Institutional Review Board-approved, retrospective review was conducted on 33 patients screened for hip dysplasia at a single institution. We sought to determine whether either film was more likely to be qualitatively better in position and whether there were differences in acetabular measures between the 2 radiographs. Standard measurements on AP and FL radiographs were made by 5 observers on 2 different occasions to assess interobserver and intraobserver variability. RESULTS: The mean age was 23+1 months, and 80% were female; none of the children had hip dysplasia. There was no statistical significance in the quotient of pelvic rotation (AP 1.2 + 0.1 degrees and FL 1.1 + 0.1 degrees; P = 0.84). There was a statistical difference in obturator height in mm (AP 16.0 + 0.3 and FL 17.8 + 0.2; P = 7.2E-10). The mean intraobserver variability for the acetabular index was 1.8 + 0.2 and 1.7 + 0.2 degrees for AP and FL alignments, respectively (P = 0.58), and the mean interobserver variability was 1.7 + 0.1 and 1.7 + 0.1 degrees, respectively (P = 0.75). The measurements of linear variability between the AP and FL showed no statistical significant difference with the exception of the left Hilgenreiner distance in mm (AP 1.00 + 0.08 and FL 0.81 + 0.08; P = 0.02). DISCUSSION: These results demonstrate that there is little clinical difference in variability of positioning and rotational variables between AP and FL radiographs for hip dysplasia. These results indicate that either image could be used assess to acetabular dysplasia thus decreasing risks of ionizing radiation. LEVEL OF EVIDENCE: Level III.

Pain management in patients with adolescent idiopathic scoliosis undergoing posterior spinal fusion: combined intrathecal morphine and continuous epidural versus PCA.

STUDY DESIGN: A retrospective case-comparison study. OBJECTIVE: Compare efficacy and safety of combined intrathecal morphine (ITM) and epidural analgesia (EPI) to that of conventional intravenous patient-controlled analgesia (IV-PCA) after posterior spinal fusion (PSF) for adolescent idiopathic scoliosis (AIS). SUMMARY OF BACKGROUND DATA: Pain control after PSF in AIS has been managed traditionally with IV-PCA. More recently studies have shown improvement in pain control with the use of continuous EPI or intraoperative ITM. No studies to our knowledge have compared the use of both ITM and EPI analgesia to that of IV-PCA. METHODS: An Institutional Review Board-approved retrospective case-comparison study was performed from 1989 to 2009 of all patients undergoing PSF for AIS. Patients received either IV-PCA or ITM/EPI. Daily pain scores were recorded along with total opioid and benzodiazepine use. Adverse events were recorded for all the patients. RESULTS: A total of 146 patients were initially included in the study; 95 patients received ITM/EPI and 51 received IV-PCA as a historical control. Eight patients from the ITM/EPI group were excluded from the pain comparison portion of the study. There were no statistical differences in age, sex, weight, or hospital stay between the 2 groups. The ITM/EPI group had, on average, 1 additional level of fusion (P = 0.001). Daily average pain scores were lower in the ITM/EPI group on all hospital days, and statistically lower in days 1 and 3 to 5. Total opioid requirement was significantly lower in the ITM/EPI patients, although oral opioid use was higher among this group. Total benzodiazepine use was lower among the IV-PCA group. A total of 15.7% of the IV-PCA patients had bladder hypotonia, compared with 1.1% of the ITM/EPI group (P = 0.002). The rate of illeus was 15.7% in the IV-PCA patients and 5.7% in the ITM/EPI (P = 0.071). Respiratory depression was reported in 4 ITM/EPI patients, 0 in our PCA group. Technical catheter malfunction was reported in 8.5% of the EPI group. CONCLUSIONS: The use of ITM/EPI after PSF for AIS is safe and effective, this methodology provided significantly lower pain scores and lowers total opioid use which can lead to urinary and bowel dysfunction.

Ulnar fracture with late radial head dislocation: delayed Monteggia fracture.

Monteggia fractures are rare but commonly discussed lesions, with increasing complications due to late diagnosis. This article describes a case of a Monteggia fracture with delayed dislocation of the radial head. Previous radiographs of a 2-year 8-month-old boy show complete fracture of the distal ulna, with no radial head dislocation. The radial head remained well positioned after 4 weeks. Seven years later, he sustained another arm injury. He was diagnosed with a hematoma but was later believed to have nursemaid's elbow. He presented to our institution 5 weeks after the injury, and the radial head was found to be chronically dislocated, indicating a displacement occurring sometime during the past 7 years. After failing conservative treatment, the patient underwent surgical repair. The annular ligament was reconstructed using a harvested triceps fascia band, and an ulnar osteotomy was performed. A review of the literature found few reports of delayed Monteggia fractures, which accounted the delayed dislocations to ulnar angulation. However, our patient showed minimal ulnar angular deformity. We propose that the initial fracture disrupted the annular ligament and the radial head spontaneously relocated prior to being seen, which put the radial head at risk for later dislocation. We present an alternative hypothesis of dislocation after fracture healing and report the longest known period of delay between fracture and dislocation.

Osteonecrosis of the distal tibia metaphysis after a Salter-Harris I injury: a case report.

Posttraumatic osteonecrosis has been well described as a common phenomenon seen in fractures of the femoral neck, talus, and scaphoid. In the following case, we describe posttraumatic osteonecrosis in a rare location: the distal tibia. Our report details a child who sustained a distal tibia physeal injury and subsequently developed radiographic findings consistent with aseptic necrosis. Besides a traumatic incident, the patient did not have any of the risk factors known to cause osteonecrosis. Awareness of this complication after Salter-Harris I fractures will help reduce time to diagnosis and optimize treatment.

Early treatment of scoliosis with growing rods in children with severe spinal muscular atrophy: a preliminary report.

BACKGROUND: Spinal muscle atrophy (SMA) is a progressive neuromuscular disease predominantly presenting in infancy and early childhood. Scoliosis is the most common spinal deformity in these patients and treatment in SMA patients is controversial. Treatment is usually definitive fusion. The purpose of this study is to evaluate a novel growing rod technique used to treat more involved children with SMA types I and II with scoliosis at an earlier age. METHODS: An Institutional Review Board approved retrospective medical record review was performed of children with SMA who were treated for scoliosis with the growing rod construct. Chart and radiographic data were reviewed. Eleven patients met the inclusion criteria, 4 male and 7 female patients. No patients were lost to follow-up. Mean follow-up was 43 months (range, 24 to 76 mo). The average age at time of surgery was 6 years. Five patients had a diagnosis of SMA I, 6 patients with SMA II. The 11 children underwent 45 surgical procedures, 12 growing rod implantations with 34 lengthenings. TECHNIQUE: 4.5 mm titanium rods (Medronic Memphis, TN) were implanted obtaining a stable anchor point proximally with 4 pedicle screws and a cross link, and distally with 2 iliac bolts and 2 pedicle screws. Fusion is obtained at both anchor points by decortications and the use of bone graft. Subcutaneous low profile rods span between both anchor points using axial connectors. RESULTS: The average preoperative Cobb angle measurement of the major curve was 51.5 degrees (range, 38 to 76 degrees), postoperatively, 21.6 degrees (range, 2 to 34 degrees), and follow-up 18.7 degrees (range, 5 to 34 degrees). No surgical complications were identified or unplanned return to surgery. Medical complications were seen in 2 patients for postoperative pneumonia and anemia. CONCLUSIONS: Growing rod construct is an effective option in the treatment of scoliosis in SMA patients with scoliosis.