Observation Versus Cast Treatment of Toddler's Fractures.

BACKGROUND: Nondisplaced metaphyseal fractures of the distal tibia, or toddler's fractures, are one of the most common pediatric injuries. Healing typically occurs quickly without sequelae. Treatment ranges from long leg cast immobilization to observation. This study compares short-term clinical and radiographic outcomes of toddler's fractures treated with long leg casting versus observation. METHODS: Patients with toddler's fractures were offered enrollment and randomization at diagnosis. Because many families opposed randomization, a preference arm was added after one year. All subjects were analyzed as a prospective cohort. Radiographs were obtained at diagnosis and 4 weeks. A modified Oxford Ankle Foot Questionnaire for Children (OAFQ-C) and family satisfaction survey were collected at diagnosis, 4 and 8 weeks. Scores were analyzed using mixed effect models. Family satisfaction surveys were compared using a Wilcoxon rank sum test. RESULTS: Forty-four subjects participated in the study, 34 (77%) in the preference arm and 10 (23%) in the randomized cohort. The median patient age was comparable between the cast and the observation groups, 2.0 versus 1.8 years, respectively. Significant improvement in OAFQ-C scores was observed in both groups over 8 weeks (P<0.01). Patients in the observation group had a higher initial play score than the cast group (P=0.03). The observation group trended toward higher physical scores at all time points (P=0.11). There was no significant difference in emotional scores between groups (P=0.77). No displacement was observed in any patient. Casted patients had significantly more minor complications with 4 patients requiring cast change or removal compared with 0 in the observed group (P=0.01). At 8 weeks, 80% of parents in the cast group were likely or very likely to choose the same treatment compared with 95.6% in the observation group. Family satisfaction scores did not differ between groups (P=0.18). They demonstrated differences in perceived normal walking at 4 weeks, with 50% of casted patients walking normally compared with 92% of observed patients. Over 90% of patients in both groups were reportedly walking normally at week 8. CONCLUSION: Observation of toddler's fractures results in equivalent clinical and radiographic outcomes, high family satisfaction and fewer complications compared with treatment with a long leg cast. LEVEL OF EVIDENCE: Level II.

Abbreviated Outpatient Upper Extremity Fracture Care to Avoid Clinic and Hospital Environmental Encounters During the COVID-19 Pandemic: A New Approach to Fracture Care?

BACKGROUND: To minimize in-person visits during the COVID-19 pandemic, a new fracture care protocol for children with complete and stable, nondisplaced or minimally displaced upper extremity (UE) fractures has been implemented. This protocol involves immobilization with a bivalved cast, which allows for home cast removal during a telemedicine visit, and no follow-up radiographs, thus eliminating the requirement for a return to clinic. The purpose of this study is to evaluate the outcomes and parent satisfaction of this new abbreviated fracture care protocol. METHODS: Between May 2020 and April 2021, during the COVID-19 pandemic, children with complete and stable, nondisplaced or minimally displaced UE fractures were treated with a bivalved cast and 1 follow-up telemedicine visit for home cast removal. A prospective longitudinal study of these patients was performed. The PROMIS Upper Extremity questionnaire was administered at enrollment and 3 months follow-up. Parents completed a satisfaction survey after home cast removal. Demographic data and information regarding complications were collected. A historical cohort of controls treated with standard cast in 2019 was used for comparison. RESULTS: A total of 56 patients with a mean age of 8±3 years (range 2 to 15) were prospectively enrolled in this study. Parent-reported PROMIS Upper Extremity scores showed a significant increase from 24.9 (95% confidence interval=20.8-29.1) at enrollment to 51.6 (95% confidence interval=50.8-52.5) at 3 months follow-up (P<0.001). Results of the satisfaction survey (n=39) showed all parents were either very satisfied (85%) or satisfied (15%). In addition, 10% of parents would have initially preferred to come into clinic for cast removal and 90% of parents would prefer this new treatment plan in the future. Patients in the abbreviated care cohort returned to clinic for a median 1 in-person visits, compared with 2 for historical controls (n=183, P<0.001). Abbreviated care patients received fewer (1.0) radiographs than controls (2.0, P<0.001). Complication rate did not differ between the groups (P=0.77). CONCLUSIONS: Complete and stable, nonminimally or minimally displaced UE fractures can be cared for safely and effectively in a single in-person visit, with a telemedicine cast removal visit. Parents are satisfied with this abbreviated protocol and prefer it to additional in-person visits. LEVEL OF EVIDENCE: Level III.

Low Preoperative Lung Functions in Children With Early Onset Scoliosis Predict Postoperative Length of Stay.

INTRODUCTION: Children with early onset scoliosis (EOS) undergoing spine surgery often have significant respiratory disease. Preoperative risk assessments that predict an increased length of hospital stay (LOS) for this group have not been previously evaluated. METHODS: A voluntary protocol using preoperative lung function studies began among participants of a multicenter registry in 2016. Preoperative assessments were standardized to include spirometry, blood hemoglobin levels, serum bicarbonate, albumin and prealbumin; radiographic parameters of the spine, C-EOS classification and need for preoperative pulmonary assistance before initial growth friendly device insertion or "definitive" spine fusion. Primary outcome was LOS postoperatively. Data, including age, diagnosis, and type of surgery, was collected prospectively. Secondary outcomes measured included intensive care unit LOS, requirement for new pulmonary assistance on discharge, and pulmonary complications. Groups were compared using the Fisher exact tests. RESULTS: Of 525 children enrolled, 101 (20%) had preoperative spirometry. Median age was 8.9 years [interquartile range (IQR): 4.27]. Etiologies for EOS included 29 neuromuscular (28%), 33 idiopathic (32%), 19 syndromic (19%), and 22 congenital (21%) scoliosis. Eighty (78%) had growing rod (GR) insertions; 23 (22%) had spine fusion SF. Eighteen subjects (17%) were hospitalized ≥7 days (median=9 d); 83 had a LOS <7 days (median=3 d). Percentage of forced vital capacity (FVC%) predicted was inversely associated with LOS ≥7 days with a median of 75.3% (IQR: 41.7) for LOS <7 days and 51.7% (IQR: 41.6) (P=0.02). There were no detectable differences in LOS for other preoperative values. CONCLUSION: FVC predicted ≤50% preoperatively in children undergoing initial growth friendly rod insertion or definitive fusion after growth friendly treatment is associated with an increased risk of postoperative hospital stays ≥7 days. As demonstrated in previous studies, severe restrictive lung disease (FVC% predicted at or below 50%) is associated with increased risk of poorer outcomes for EOS patients.

Seasonal Variation in Parental Satisfaction With Pediatric Orthopaedics.

BACKGROUND: The degree of parental satisfaction with health care is determined by the family's characteristics and expectations. Many aspects of human physiology and behavior have seasonal rhythms. The purpose of the present study was to determine whether parental satisfaction scores vary across the year in a pediatric orthopaedic outpatient population. METHODS: We retrospectively reviewed a total of 22,951 parental satisfaction scores related to outpatient pediatric orthopaedic encounters between October 2015 and April 2019. Parental satisfaction was measured using the provider subdomain of the shortened version of the Hospital Consumer Assessment of Healthcare Providers and Systems (HCAHPS). Families were stratified according to various pediatric orthopaedic subspecialty services. RESULTS: The satisfaction scores of all pediatric orthopaedic encounters combined did not vary significantly across the year (P=0.8745). When the families were stratified into groups by pediatric orthopaedic subspecialty clinic, variation in seasons was not associated with statistically significant variation in satisfaction scores for the elective surgery, sports medicine, trauma/fracture care, and hand/upper extremity services. However, satisfaction with the spine service was significantly lower in the winter compared with the summer and fall (73.9±3.8 vs. 83.5±5.3 and 82.6±3.6, respectively; P=0.0147). CONCLUSIONS: The provision of pediatric spine care in a region with 4 distinct seasons received lower parental satisfaction scores during the winter than during the summer and fall. This seasonal variation is an additional source of bias in the measurement of satisfaction with health care. LEVEL OF EVIDENCE: Level II-retrospective study.

The Utility of the Early Postoperative Follow-up and Radiographs After Operative Treatment of Supracondylar Humerus Fractures in Children.

BACKGROUND: Supracondylar humerus (SCH) fractures are common elbow injuries in pediatric patients. The American Academy of Orthopedic Surgeons published guidelines for the standard of care in the treatment of displaced SCH fractures, however, no recommendations for follow-up care were made. With the recent push to eliminate unnecessary radiographs and decrease health care costs, many are questioning postoperative protocols. The purpose of our study was to evaluate the utility of the 1-week follow-up appointment after closed reduction and percutaneous pinning (CRPP) of displaced SCH fractures. METHODS: A retrospective review performed at a single institution from 2014 to 2016 included patients under 14 years of age with a closed, displaced SCH fracture treated with CRPP. Exclusion criteria included patients without complete clinical or radiographic follow-up. Variables examined included time to initial follow-up, change in treatment plan after 1-week x-rays, complications, demographics, fracture type, pin number and configuration, reduction parameters, immobilization, time to pin removal, duration of casting, and clinical outcome. RESULTS: A total of 412 patients were divided into 2 groups based on time to initial follow-up. Overall, 368 had an initial follow-up at 7 to 10 days (group 1) and 44 at 21 to 28 days (group 2). There was no difference in age, sex, fracture type, pin configuration, or a number of pins between groups. Statistically significant findings included time to initial follow-up and days to pin removal (group 1 at 26.2 d vs. group 2 at 23.8 d), type of immobilization (group 1 with 5% circumferential casts and group 2 with 70%), and time to surgery (26.2 vs. 62.9 h, respectively). There was no significant difference in complication rates and only a 0.5% rate of change in management in group 1. CONCLUSIONS: Early postoperative follow-up and radiographs did not change the patient outcome and might be eliminated in children with displaced SCH fractures treated with CRPP. Given the current focus of on efficiency and cost-effective care, eliminating the 1-week postoperative appointment would improve appointment availability and decrease medical cost. LEVEL OF EVIDENCE: Level III-Therapeutic.

Persistence and Progression of Airway Obstruction in Children With Early Onset Scoliosis.

BACKGROUND: Obstructive lung disease occurs in 30% of children with early onset scoliosis (EOS); changes in degree of airway obstruction over time have not been reported. METHODS: Longitudinal patterns of incidental, persistent, and progressive airway obstruction were retrospectively analyzed in a cohort of children with EOS with at least 1 forced expiratory volume in 1 second (FEV1)/forced vital capacity (FVC) value <85% on serial spirometric assessments over a ≥3-year observation period. The prevalence of clinical features and the severity of coronal and sagittal spine deformities for each group at the beginning and end of the study period were compared. RESULTS: Airway obstruction was incidental in 12 (24%) and persistent in 37 (76%) of 49 children with EOS. Twenty of 37 (54%) of those with persistent obstruction developed progressive airway obstruction. The decline in FEV1/FVC over 6±2 years was insignificant in the incidental group (4%±2%) and the persistent nonprogressive group (7%±4%) but significant in the progressive group (13%±4%, t test; P=0.002). In total, 29% of the 49 children at the onset and 57% at the end of the study had airway obstruction. The incidental, persistent nonprogressive, and progressive groups did not differ with regard to age, diagnosis distribution, or sex. The initial coronal curve size, apex, direction of the curve, and degree of kyphosis were statistically similar among the 3 groups. Coronal curve magnitude inversely correlated with FEV1/FVC at the end but not the beginning of the study (r=-0.19, P=0.002). Six of 19 responded to bronchodilator treatment, suggesting concurrent asthma. Airway obstruction did not relate to restrictive pulmonary abnormalities measured by FVC at first or last timepoints [slope=-0.076 (95% confidence interval, -0.99 to 0.038; P=0.19)]. Changes in degrees of airway obstruction and restrictive lung disease over time did not correlate [slope=-0.125 (95% confidence interval, -0.294 to 0.044; P=0.14)]. CONCLUSIONS: Children with EOS and progressive airway obstruction represent an important subgroup which may require new surgical and nonsurgical treatment strategies to prevent loss of lung function over time.

Best practice guidelines regarding diagnosis and management of patients with type II collagen disorders.

PURPOSE: Skeletal dysplasias comprise a heterogeneous group of inherited disorders of development, growth, and maintenance of the human skeleton. Because of their relative rarity and wide phenotypic variability, patients should be accurately identified, uniformly assessed, and managed by clinicians who are aware of their potential complications and possess the knowledge and resources to treat them effectively. This study presents expert guidelines developed to improve the diagnosis and management of patients with type II collagen skeletal disorders to optimize clinical outcomes. METHODS: A panel of 11 multidisciplinary international experts in the field of skeletal dysplasia participated in a Delphi process, which comprised analysis of a thorough literature review with subsequent generation of 26 diagnosis and care recommendations, followed by two rounds of anonymous voting with an intervening face-to-face meeting. Those recommendations with more than 80% agreement were considered as consensual. RESULTS: After the first voting round, consensus was reached to support 12 of 26 (46%) statements. After the panel discussion, the group reached consensus on 22 of 24 revised statements (92%). CONCLUSIONS: Consensus-based, expert best practice guidelines developed as a standard of care to assist accurate diagnosis, minimize associated health risks, and improve clinical outcomes for patients with type II collagen skeletal dysplasias.

The Impact of Transphyseal Anterior Cruciate Ligament Reconstruction on Lower Extremity Growth and Alignment.

PURPOSE: To evaluate the effect of transphyseal anterior cruciate ligament (ACL) reconstruction on lower extremity radiographic growth and alignment. METHODS: We retrospectively reviewed patients who underwent transphyseal ACL reconstruction and were followed to skeletal maturity or at least 2 years, with the nonoperative limb used as an internal control. Changes in coronal plane alignments and tibial slope of the operative limb were compared with a Wilcoxon test. Associations among sex, tunnel, and graft characteristics and failure; changes in coronal plane measures and tunnel size; and tunnel angles and the development of deformity were examined by χ-square and correlation coefficients. RESULTS: Fifty-nine patients (41 boys and 18 girls) underwent surgery at a mean age of 12.5 years (range, 6.8-16.0 years). There were differences in changes in the mechanical lateral distal femoral angle comparing operative and nonoperative limbs (decreased 1.1° in girls and 1.9° in boys ≤13 years of age, P = .0008 and .025, respectively) and in changes in tibial slope of the operative limb (decreased 2.1° in male patients >13 years, P = .012). No patient developed a new limb length difference >1 cm. Two boys were treated for deformities. Eight additional patients developed >5° difference in alignment for a rate of radiologic deformity of 10 of 59 or 17%. Neither graft failure nor the presence of deformity was associated with sex, tunnel size, mode of femoral tunnel positioning, inclination of tunnels, or the use of allograft. CONCLUSIONS: Radiographically evident limb deformities following transphyseal ACL reconstruction occurred at a rate of 17%, although these deformities were clinically evident in only 5% of patients. Tunnels intersecting physes near cortical margins may increase the risk of developing deformity. Regular follow-up should include alignment radiographs to detect deformities despite the clinical appearance of neutral limb alignment. LEVEL OF EVIDENCE: Level III, case-control study.

Concomitant Anterior Cruciate Ligament Reconstruction and Temporary Hemiepiphysiodesis in the Skeletally Immature: A Combined Technique.

BACKGROUND: Young athletes with an anterior cruciate ligament (ACL) disruption and limb malalignment pose a treatment dilemma. Little has been published regarding limb malalignment in this population. Our aim is to review the results of combined treatment of an ACL deficient knee and genu valgum in skeletally immature patients. METHODS: A retrospective review of skeletally immature patients who underwent transphyseal ACL reconstruction and concomitant hemiepiphysiodesis between 2004 and 2015 by 1 surgeon at a single institution was performed. Included patients had at least a year of growth remaining and were followed to skeletal maturity. Patients with a diagnosis of a connective tissue disorder were excluded. Knee stability, rate of retear, the rate of mechanical axis correction, and time to full correction were determined. RESULTS: Ninety skeletally immature patients underwent transphyseal ACL reconstruction, 8 of which met inclusion criteria. Mean time to correction of the valgus deformity was 13 months (0.4 degree/mo). No patient required additional surgeries for malalignment. All patients had improvement in knee stability. One patient had a retear of their ACL reconstruction, for a failure rate of 13%. Preoperative mechanical lateral distal femoral angle and mechanical axis deviation corrected to near-neutral alignment for all treated limbs and were significantly different (P=0.001) than those measured preoperatively. CONCLUSIONS: Promising results were seen for simultaneous correction of genu valgum and transphyseal ACL reconstruction. Treatment of both pathologies in a concomitant surgery can be considered in the appropriate population, with expected results comparable to each procedure in isolation. LEVEL OF EVIDENCE: Level IV-case series.

Best practice guidelines regarding prenatal evaluation and delivery of patients with skeletal dysplasia.

BACKGROUND: Skeletal dysplasia comprises a heterogeneous and collectively common group of inherited disorders of development, growth, and maintenance of the human skeleton. There is potential for increased perinatal morbidity and mortality in pregnant women who themselves have skeletal dysplasia, and for affected fetuses where skeletal dysplasia is suspected in utero. OBJECTIVE: We sought to establish guidelines for perinatal health care professionals who should be aware of these risks, to optimize maternal and child health pregnancy outcomes through best prenatal and delivery management practices. STUDY DESIGN: A panel of 13 multidisciplinary international experts participated in a Delphi process, which comprised consideration of thorough literature review and a list of 54 possible care recommendations subject to 2 rounds of anonymous voting and a face-to-face meeting. Those recommendations with >80% agreement were considered as consensual. RESULTS: During the first round, consensus was reached to support 30 out of the 54 statements. After the panel discussion, the group reached consensus on 40 statements. These statements include guidelines for the evaluation and treatment of pregnant women with skeletal dysplasia and for the unborn child with or suspected to have skeletal dysplasia. CONCLUSION: Consensus-based best practice guidelines are provided as a minimum of standard care to minimize associated health risks, and improve clinical outcomes for patients with skeletal dysplasia.

Forearm Fixation is Not Necessary in the Treatment of Pediatric Floating Elbow.

BACKGROUND: Ipsilateral supracondylar humerus and forearm fractures in the pediatric population are an uncommon injury associated with high-energy trauma. Current literature suggests a high rate of compartmental syndrome with this fracture pattern and recommends surgical stabilization of both injuries. We investigate whether surgical treatment of the supracondylar fracture with closed reduction of the forearm fracture and placement into a noncircumferential cast may be an appropriate treatment. METHODS: Retrospective clinical and radiographic review of 47 patients (22 male, 25 female; mean age 6 y) with modified Gartland type 2 or type 3 supracondylar humerus fracture requiring surgical stabilization and an ipsilateral forearm fracture from a single institution over 78 months. RESULTS: Forty-seven pediatric "floating elbow" cases that had operative management of the supracondylar fracture were identified. A total of 21/47 (45%) had displaced forearm fractures that required closed manipulation. Of these, 17/21 (81%) underwent closed reduction of the displaced forearm fracture(s) and were placed into a noncircumferential cast or splint. No patients lost reduction or required remanipulation of either fracture. No patients developed signs of elevated compartment pressures. All patients went on to radiographic union without secondary procedures. CONCLUSIONS: We demonstrate that a supracondylar humerus fracture with an ipsilateral forearm fracture can be safely managed with operative stabilization of the supracondylar humerus fracture alone. Simultaneous closed reduction of the ipsilateral displaced forearm fracture and use of noncircumferential immobilization postoperatively is safe and was not associated with the development of elevated compartment pressures or need for remanipulation. Previous studies that relate a high rate of compartment syndrome with this injury pattern may be misguided, as method of postoperative immobilization may be a more significant factor in the development of elevated compartment pressures than the injury pattern. LEVEL OF EVIDENCE: Level IV.

A comparison of the functional health of children with Costello syndrome in 1999 and in 2015.

Costello Syndrome is a rare congenital condition characterized by failure-to-thrive, cardiac abnormalities, distinctive facial features, predisposition to malignant tumors, and developmental delay. In 1999, we analyzed the functional health in a cohort of 18 patients. Since then, a mutation in the HRAS gene has been found to be causative, medical management has been refined, and the level of awareness has increased. The purpose of this study is to compare the functional health outcomes from the 1999 cohort with data prospectively collected from a comparable cohort in 2015. The Pediatric Outcome Data Collection Instrument (PODCI) was administered to parents of children with Costello syndrome during the 2015 International Costello Syndrome Conference. The same instrument and setting were used in the 1999 study. We compared functional health scores from the two groups. A total of 21 participants were included in the 2015 cohort; 15 females (71%) and 6 males (29%). Average age was 5.8 years (range 2-16). When comparing functional health outcomes, we found that the 2015 cohort scored slightly higher in Upper Extremity and Physical Function (57 vs. 54) and Comfort scales (86 vs. 82). However, there was no significant difference in any of the PODCI scales between the two groups. When compared with normative scores, both groups scored significantly lower in every scale except for happiness (p = 0.2952). Despite recent advancements, functional health outcomes in 2015 were similar to those measured in a different cohort in 1999.

Best practices in peri-operative management of patients with skeletal dysplasias.

Patients with skeletal dysplasia frequently require surgery. This patient population has an increased risk for peri-operative complications related to the anatomy of their upper airway, abnormalities of tracheal-bronchial morphology and function; deformity of their chest wall; abnormal mobility of their upper cervical spine; and associated issues with general health and body habitus. Utilizing evidence analysis and expert opinion, this study aims to describe best practices regarding the peri-operative management of patients with skeletal dysplasia. A panel of 13 multidisciplinary international experts participated in a Delphi process that included a thorough literature review; a list of 22 possible care recommendations; two rounds of anonymous voting; and a face to face meeting. Those recommendations with more than 80% agreement were considered as consensual. Consensus was reached to support 19 recommendations for best pre-operative management of patients with skeletal dysplasia. These recommendations include pre-operative pulmonary, polysomnography; cardiac, and neurological evaluations; imaging of the cervical spine; and anesthetic management of patients with a difficult airway for intubation and extubation. The goals of this consensus based best practice guideline are to provide a minimum of standardized care, reduce perioperative complications, and improve clinical outcomes for patients with skeletal dysplasia.

Is there a correlation between sleep disordered breathing and foramen magnum stenosis in children with achondroplasia?

Children with achondroplasia have midface hypoplasia, frontal bossing, spinal stenosis, rhizomelia, and a small foramen magnum. Central sleep apnea, with potential resultant sudden death, is thought to be related to compression of the spinal cord at the cervicomedullary junction in these patients. Screening polysomnography and/or cervical spine MRI are often performed for infants with achondroplasia. Decompressive suboccipital craniectomy has been performed in selected cases. We aim to better delineate the relationship between polysomnography, cervical spine MRI, and indications for surgical decompression in achondroplasia.We retrospectively review electronic medical records of all children with achondroplasia in our IRB-approved skeletal dysplasia registry who had received screening polysomnography and cervical spine MRI examination was performed. We explored correlations of polysomnography, MRI parameters, and need for decompressive surgery. Seventeen patients with both polysomnography and MRI of the cervical spine met inclusion criteria. The average age at time of the sleep study was 2.4 ± 3.6 years. An abnormal apnea-hypopnea index was found in all patients, with central sleep apnea found in 6/17. Five patients (29%) required foramen magnum decompression. We found no statistically significant correlation between central sleep apnea and abnormal MRI findings suggestive of foramen magnum stenosis. Screening polysomnography is an important tool but does not appear to correlate with MRI findings of foramen magnum stenosis. Cord compression, with either associated T2 cord signal abnormality or clinical findings of clonus, was most predictive of subsequent surgical decompression.

Best practices in the evaluation and treatment of foramen magnum stenosis in achondroplasia during infancy.

Achondroplasia is the most common inherited disorder of bone growth (skeletal dysplasia). Despite this fact, consistent and evidence-based management approaches to recognized, life-threatening complications, such as foramen magnum stenosis, are lacking. This study aims to outline best practice, based on evidence and expert consensus, regarding the diagnosis, assessment, and management of foramen magnum stenosis in achondroplasia during infancy. A panel of 11 multidisciplinary international experts on skeletal dysplasia was invited to participate in a Delphi process. They were: 1) presented with a list of 26 indications and a thorough literature review, 2) given the opportunity to anonymously rate the indications and discuss in face to face discussion; 3) edit the list and rate it in a second round. Those indications with more than 80% agreement were considered as consensual. After two rounds of rating and a face-to-face meeting, consensus was reached to support 22 recommendations for the evaluation and treatment of foramen magnum stenosis in infants with achondroplasia. These recommendations include indications for surgical decompression, ventriculomegaly, and hydrocephalus, sleep-disordered breathing, physical exams and the use of polysomnography and imaging in this condition. We present a consensus-based best practice guidelines consisting of 22 recommendations. It is hoped that these guidelines will lead to more uniform and structured evaluation, standardizing care pathways, and improving mortality and morbidity outcomes for this cohort.

The effect of surgery with radiation on pelvic Ewing sarcoma survival.

BACKGROUND: Pelvic Ewing sarcoma (ES) has poorer outcomes than extremity-based lesions and the method of local control is controversial. METHODS: A retrospective review was performed of 40 primary pelvic or sacral ES treated by a single surgeon. All received modern chemotherapy and those that received radiation were treated with modern dosages. RESULTS: Fifty-five percent were disease-free at latest follow-up (median, 83.1 mos). Sixty-one percent had ≥ 99% necrosis, which was associated with 65% disease-free survival. Larger size (P = 0.016) and the absence of metastatic disease (P = 0.005) was predictive of survival. Eighty-three percent of relapsed patients were DOD. Half of patients who received surgery alone or RT alone have NED while 57% of those who received S/RT have NED. Complication rates were 69% (S/RT), 75% (surgery alone), 10% (RT alone). Functional outcomes were similar. CONCLUSION: Primary pelvic ES is localized at presentation in 50% and the absence of metastases is the strongest predictor for survival. Chemotherapy is key, but excellent histologic response is neither a guarantee nor a necessity for survival. More than one-third die despite an excellent histologic response and at least one-third with lung metastases survive. With chemotherapy, radiation, and surgery, reasonable control of disease can be achieved. LEVEL OF EVIDENCE III: Case-control or retrospective cohort study.

Vertical expandable prosthetic titanium rib (VEPTR): a review of indications, normal radiographic appearance and complications.

Vertical expandable prosthetic titanium rib (VEPTR) is increasingly used in the treatment of thoracic insufficiency, idiopathic and neuromuscular scoliosis and chest wall defects in children. In contrast to spinal fusion surgery, the VEPTR allows for growth while stabilizing the deformity. We illustrate the common indications and normal radiographic appearance of the three common configurations of VEPTR (cradle-to-cradle assembly, cradle with lumbar extension assembly, cradle-to-ala hook assembly). There is a relatively high rate of reported complications with VEPTR in the literature. We discuss the potential complications of VEPTR, including infection, rib fracture, dislodged hardware and neurological injury, with an emphasis on imaging diagnosis.

High satisfaction yet decreased activity 4 years after transphyseal ACL reconstruction.

BACKGROUND: ACL injuries in preteens and teens are common occurrences. Reconstruction is believed to be optimum treatment for those wishing to return to running, cutting, and jumping sports. Rates of reoperation, satisfaction, and long-term return to and maintenance of preinjury activity after ACL reconstruction in young athletes are important information for physicians, patients, and parents. QUESTIONS/PURPOSES: The purposes of this study were to address the following questions in this skeletally immature patient population undergoing ACL reconstruction: (1) What is the reinjury rate and the need for subsequent surgeries? (2) How do patient satisfaction and function as assessed by patient and physician correlate with return to sport? (3) What factors contribute to failure to return to preinjury activity levels? METHODS: This is a retrospective review of 29 patients who underwent transphyseal ACL reconstruction using soft tissue grafts passed through open physes and followed to skeletal maturity, and at least 2 years from their index surgery, who were invited and returned for a study interview and examination. Pre- and postinjury activity levels were assessed via the Tegner activity score, satisfaction was determined using a 10-point Likert scale, function was assessed via the Lysholm score and IKDC grade, and an open-ended questionnaire was used for explanations of changes in activity levels. Reoperations were classified as major or minor, determined from a review of the medical records conducted after interview and examination. RESULTS: At a minimum followup of 2 years (mean, 4 years; range, 2-8 years), four revision reconstructions and seven minor operations were performed for a reoperation rate of 11 of 29 (38%). Eight of 29 patients (28%) sustained contralateral ACL ruptures. The mean satisfaction score was 9 (range, 4-10) and mean Lysholm score was 91 (range, 61-100). Only 12 of 29 (41%) patients returned to and maintained their preinjury level of sport. High satisfaction correlated with return to prior level of sports, although there was no relationship between function and activity level. Reoperation on the index knee or contralateral ACL tear did not correlate with a change in activity level; rather, most patients who were less active indicated a change in interest with advancing age. CONCLUSIONS: Despite high satisfaction and function, less than 50% of patients maintained their preinjury level of play 4 years after ACL reconstruction. Satisfaction correlated significantly with knee function; highly satisfied patients were more likely to return to and maintain their prior level of participation in sports. Contributing factors to decreased activity include changes in lifestyle with increasing age. Reoperation did not correlate with lower activity scores or failure to return to sports. LEVEL OF EVIDENCE: Level IV, therapeutic study. See the Instructions for Authors for a complete description of levels of evidence.

Musculoskeletal functional outcomes in children with osteogenesis imperfecta: associations with disease severity and pamidronate therapy.

BACKGROUND: Intravenous pamidronate has been used off-label in the treatment of severe osteogenesis imperfecta (OI) for almost 20 years. Previous studies have found correlations between function and bone density in patients with OI, but have not studied the functional outcomes of these patients after bisphosphonate therapy with a validated outcome measure. The goal of this investigation is to describe the functionality and comfort of children with OI. We hypothesize that function is impaired in children with severe OI as measured using the Pediatric Outcomes Data Collection Instrument (PODCI) and that improvements in the function of children with severe OI may be observed in association with intravenous bisphosphonate therapy. METHODS: A total of 25 patients with OI were evaluated, of those, 15 received pamidronate therapy. Children with >2 long bone fractures per year were classified as having severe OI and were eligible for pamidronate therapy. Functional evaluation was performed using the PODCI for children who qualified for pamidronate therapy (severe OI) and those who did not qualify for pamidronate therapy (mild OI). PODCI evaluation was also performed after treatment with pamidronate in the group that qualified for pamidronate therapy. RESULTS: There was a statistically significant difference at baseline between patients with "mild" and "severe" OI in the sports/physical functioning scale (P=0.0032). Among the children who received bisphosphonate therapy, PODCI scores in the sports/physical functioning domain were significantly improved after pamidronate therapy (P=0.0364). CONCLUSIONS: This study indicates that children with mild forms of OI can be differentiated from their more severe counterparts by their ability to participate in high-level play activities. Furthermore, patients with "severe" OI show a significant improvement in their ability to participate in high-level play after 1 year of pamidronate. LEVEL OF EVIDENCE: Level IV.

Musculoskeletal function in patients with mucopolysaccharidosis using the pediatric outcomes data collection instrument.

BACKGROUND: Musculoskeletal function is often the limiting factor in quality of life in patients with mucopolysaccharidosis (MPS). The Pediatric Outcomes Data Collection Instrument (PODCI) is a validated tool to be used in children aged 2 to 18 years with chronic musculoskeletal disorders and is freely available to any investigator. The goal of this study is to establish normative data for patients with MPS. METHODS: Of our 25 patients with MPS, 16 had PODCI's performed at initial evaluation. This included 4 patients with MPS IH (Hurler), 7 patients with MPS II (Hunter), 4 patients with MPS IV (Morquio), and 1 patient with MPS VI (Maroteaux-Lamy). Differences in PODCI scores among children with MPS IH (Hurler), II (Hunter), and IV were estimated using a Kruskal-Wallis test. RESULTS: With the exception of upper extremity and physical functioning function in MPS IV, all domains for our MPS patients measured below average for typically developing children. We found a statistically significant difference among the groups in the upper extremity and physical function scale [H (2) = 8.16, P = 0.02]. The single MPS VI patient had the highest scores overall. MPS IV patients scored better than the MPS IH patients, and the MPS II patients scored lowest. CONCLUSIONS: This study demonstrates the limited musculoskeletal function in patients with MPS and the differences among different MPS diagnoses. CLINICAL RELEVANCE: The data establishes a basic understanding of musculoskeletal function in patients with MPS and should provide comparative data for future studies in which musculoskeletal function is measured as an objective outcome. It can also serve as a better objective measure for interventions, as previous models have only served as proxies to musculoskeletal function.