Growth Charts for Children With Arthrogryposis Multiplex Congenita.

Children with arthrogryposis multiplex congenita (AMC) often demonstrate growth differences compared with typically developing (TD) children. However, growth charts have not been developed for this population. The purpose of this study was to create AMC-specific growth charts and to compare these values to those of TD children. A retrospective review of height/length and weight for 206 children with AMC was performed. Growth charts were developed and stratified over seven percentiles; these were then compared with growth charts of TD children. Children with AMC tend to be smaller in stature and weight compared with TD children, particularly in the first 36 months of life. Thereafter, weight values trend toward the 50th percentile of TD children, but height/length values persist around the 5th percentile of TD children. The development of AMC-specific growth charts provides health care providers an objective tool to evaluate growth patterns of patients with AMC.

Functional Independence of Children With Arthrogryposis.

BACKGROUND: Arthrogryposis (AMC) is a descriptive term to characterize a child born with multiple joint contractures. Treatment aims to improve functional independence, yet the literature objectively describing functional independence in this population is scarce. This study aimed to describe the functional independence of children with AMC through the lens of the Pediatric Evaluation of Disability Inventory-Computer Adaptive Test (PEDI-CAT) and observational activities of daily living (ADL) tasks. METHODS: Patients with AMC between the ages of 3 and 12 years participated in this prospective study. Parents completed the PEDI-CAT while a trained occupational therapist observed children as they completed a checklist of functional ADL tasks. Patients were grouped according to developmental age groups: "preschoolers" (3 to 5 y), "early school-age" (6 to 9 y), and "late school-age" (10 to 12 y). Patient's PEDI-CAT normative scores were described, comparing the study population to typically developing children, and differences in each domain were examined between developmental age groups. The observed ADL tasks completed were also described, and differences in scores were examined between developmental age groups. RESULTS: Forty-four patients (mean age of 7±2.86 y) were enrolled. The distribution between age groups was nearly even. Mean daily activities T -score for patients with AMC was 25.80±11.98 and the mean mobility T -score was 17.39±9.77. Late school-age children scored significantly lower than preschool-age children in both of these domains ( P <0.01). Observed ADL tasks demonstrated a high level of required assistance for patients (range: 27.3% to 61.4%), although older school-age children did show greater independence with tested activities than preschool-age children ( P =0.05). CONCLUSION: Children with AMC are significantly limited in functional independence, particularly regarding age-appropriate daily activities and mobility. Outcomes from this study provide a reference to help gauge the results of nonoperative and surgical treatment toward improving functional independence in this population. LEVEL OF EVIDENCE: Level III: prognostic study.

Is peak hamstrings muscle-tendon length criterion a sufficient indicator to recommend against surgical lengthening of hamstrings?

BACKGROUND: Excessive knee flexion during stance in children with cerebral palsy is often treated by surgical hamstrings lengthening. Pre-operative hamstrings muscle-tendon length can be estimated from kinematics and often used for decision making to rule out surgical lengthening if peak hamstrings muscle-tendon length is 'Not Short'. RESEARCH QUESTION: If peak hamstrings muscle-tendon length is within two standard deviations of typical, is that a sufficient indicator to rule out surgical hamstrings lengthening? METHODS: Three motion analysis centers retrospectively identified children with cerebral palsy, age 6-17 years, who had consecutive gait analyses with knee flexion at initial contact > 20° and popliteal angle > 35° at initial study. Three groups were considered: Medial Hamstrings Lengthening (MHL), Medial and Lateral Hamstrings Lengthening (MLHL), no surgical intervention (Control). Peak hamstrings muscle-tendon length at initial gait study was computed and categorized as 'Short' or 'Not Short'. Two outcomes variables were considered: change in peak knee extension (PKE) and change in pelvic tilt. Univariate comparisons of all variables were assessed along with a multivariate stepwise regression analysis to identify pre-operative characteristics that may predict post-operative improvement. RESULTS: 440 individuals met inclusion criteria. Percentage of individuals with improved PKE by grouping were- MHL-'Short': 60%, MHL-'Not Short': 65%, MLHL-'Short': 74%, MLHL-'Not Short': 74%, Control 'Short': 20%, Control 'Not Short': 19%. Percentage of individuals with worsened pelvic tilt were- MHL-'Short': 25%, MHL-'Not Short': 11%, MLHL-'Short': 42%, MLHL-'Not Short': 21% with significantly more individuals in MHL-'Short' subgroup compared to MHL-'Not Short'. Multivariate analysis suggested that pre-operative pelvic tilt and weak hip extensor strength have the largest effect on predicting post-operative increase in APT. Peak muscle-tendon length was not a significant predictor of post-operative knee kinematics or increase in APT. SIGNIFICANCE: This study suggests that hamstrings muscle-tendon length criteria by itself is not a sufficient indicator to recommend against hamstrings lengthening.

Hip reconstruction in children with cerebral palsy: does magnitude of surgery influence complications and outcomes?

Children with cerebral palsy (CP) and neuromuscular hip dysplasia (NMHD) frequently require hip reconstruction including femoral and pelvic osteotomies. Outcomes and complications in CP patients with NMHD who underwent varying degrees of hip reconstruction surgeries were examined. Ninety-nine patients with a diagnosis of CP who underwent surgical management of NMHD over a 15-year period (2005-2020) were reviewed. Three patient cohorts with varying degrees of surgery were analyzed including: (1) unilateral femoral osteotomy with pelvic osteotomy; (2) bilateral femoral osteotomy with unilateral pelvic osteotomy; and (3) simultaneous bilateral femoral osteotomy and bilateral pelvic osteotomy. Data points included operative (OR) time, length of stay (LOS), estimated blood loss (EBL), and transfusion requirement. Complications during the perioperative and postoperative periods were identified and graded by severity. Increased EBL, need for transfusion, OR time, and LOS were observed more frequently in patients undergoing bilateral reconstruction. Complication rates were high for all three cohorts, with 87% of cases in the bilateral pelvic osteotomy cohort experiencing ≥1 complication. However, 90% of these were considered grade I or II and required no or minimal intervention. The average postoperative migration index at final follow-up for treated hips was significantly less in bilateral pelvic osteotomy cases. Patients with CP and bilateral NMHD can be safely managed with bilateral simultaneous comprehensive reconstruction. The data presented in this study can help both surgeons and families anticipate a more accurate postoperative course. Level of evidence: III.

Does use of ankle foot orthoses affect the dynamic motor control index during walking in cerebral palsy and idiopathic toe walking populations?

BACKGROUND: The dynamic motor control (walk-DMC) index during walking is a measure of the complexity of muscle activation pattern. Ankle Foot Orthoses (AFO) are frequently used to improve the gait of children with Cerebral Palsy (CP) and Idiopathic Toe Walking (ITW). The purpose of this study was to assess the change in walk-DMC index secondary to AFO use. RESEARCH QUESTION: Does the change in walk-DMC reflect the change in walking kinematics with the use of AFO. METHODS: Individuals with diagnosis of CP or ITW with gait analysis data available for barefoot and AFO condition were retrospectively identified. For each individual, the walk-DMC index, Gait Deviation Index (GDI) and Gait Variable Scores (GVS) of knee and ankle kinematics were computed for BF and AFO conditions. Paired t-tests were used to compare key variables between BF and AFO conditions. Multi-variate stepwise regression analysis was performed to identify variables that may predict the increase in walk-DMC between BF and AFO condition. RESULTS: 253 individuals were included in the study. For CP individuals (n = 208), statistically significant but quantitatively minimal improvement was observed in walk-DMC (1 ± 9), GDI (2 ± 9) and ankle GVS (2 ± 7). For ITW individuals (n = 45), larger improvements were observed in walk-DMC (11 ± 13), GDI (9 ± 11) and ankle GVS (6 ± 7). Diagnosis of ITW, use of Solid-AFO and Posterior Leaf Spring-AFO were the significant predictor of increase in walk-DMC with AFO. Higher ankle GVS at BF condition (larger deviation from TD) led to larger increase in walk-DMC. Higher knee GVS (larger deviation from TD) led to smaller increase in walk-DMC. SIGNIFICANCE: Use of AFO can lead to improvement in walking kinematics that is reflected in increase in walk-DMC with AFO compared to BF for ITW individuals. The change in kinematics and walk-DMC with use of AFO was minimal for CP individuals.

Observation versus Cast Treatment of Toddler's Fracture: A Prospective Pilot Study.

"Toddler's fractures" are common in the pediatric population. Traditional treatment recommends casting these fractures, although with their inherent stability, may be unnecessary. This study evaluated if toddler's fractures can be treated with observation alone as opposed to casts. A prospective pilot study was performed with randomization and observational arms. Children were placed in short casts or observed without immobilization. A 21-day log was given to families to record walking. Follow up with radiographs occurred at 3 and 12 weeks. Twenty-one patients enrolled, three randomized and 18 chose their treatment. Thirteen patients were casted, and eight went without immobilization. Ambulation time was similar between groups (p = 0.260). Three without immobilization returned early but none converted to cast. All fractures healed uneventfully. There were no cast complications. Toddler's fractures treated with or without cast immobilization appears to be safe and effective but should be a shared decision between physician and parents. (Journal of Surgical Orthopaedic Advances 32(3):207-211, 2023).

Epidemiology, aetiology, interventions and genomics in children with arthrogryposis multiplex congenita: protocol for a multisite registry.

INTRODUCTION: Arthrogryposis multiplex congenita (AMC) is an umbrella term including hundreds of conditions with the common clinical manifestation of multiple congenital contractures. AMC affects 1 in 3000 live births and is caused by lack of movement in utero. To understand the long-term needs of individuals diagnosed with a rare condition, it is essential to know the prevalence, aetiology and functional outcomes in a large sample. The development and implementation of a multicentre registry is critical to gather this data. This registry aims to improve health through genetic and outcomes research, and ultimately identify new therapeutic targets and diagnostics for treating children with AMC. METHODS AND ANALYSIS: Participants for the AMC registry will be recruited from seven orthopaedic hospitals in North America. Enrollment occurs in two phases; Part 1 focuses on epidemiology, aetiology and interventions. For this part, retrospective and cross-sectional data will be collected using a combination of patient-reported outcomes and clinical measures. Part 2 focuses on core subset of the study team, including a geneticist and bioinformatician, identifying causative genes and linking the phenotype to genotype via whole genome sequencing to identify genetic variants and correlating these findings with pedigree, photographs and clinical information. Descriptive analyses on the sample of 400 participants and logistic regression models to evaluate relationships between outcomes will be conducted. ETHICS AND DISSEMINATION: Ethical approval has been granted from corresponding governing bodies in North America. Dissemination of findings will occur via traditional platforms (conferences, manuscripts) for the scientific community. Other modalities will be employed to ensure that all stakeholders, including youth, families and patient support groups, may be provided with findings derived from the registry. Ensuring the findings are circulated to a maximum amount of interested parties will ensure that the registry can continue to serve as a platform for hypothesis-driven research and further advancement for AMC.

Patient-Reported Outcome Measurement Information System (PROMIS) Scores in Pediatric Patients With Arthrogryposis.

BACKGROUND: Arthrogryposis multiplex congenita (AMC) is a clinical term that is used to describe congenital contractures that lead to childhood deformities. Treatment aims are to maximize function while minimizing pain and disability. Few studies have explored patient-reported outcomes in the pediatric arthrogrypotic population, particularly concerning mobility. The aim of this study was to report scores for the Patient-Reported Outcome Measurement Information System (PROMIS) questionnaire for pediatric patients with arthrogryposis with regards to mobility, upper extremity (UE) function, pain interference, and peer relationships. METHODS: A retrospective chart review of 76 patients with AMC aged 5 to 17 who completed the PROMIS questionnaire between January 1, 2017 to March 24, 2020 was performed. Results were collected for four domains: mobility, UE function, pain interference, and peer relationships. Outcomes were stratified by type of arthrogryposis: Amyoplasia (Am), Distal Arthrogryposis (DA), and Other Diagnoses (OD). Results of subjects with isolated upper or lower extremity involvement were compared with subjects with involvement of upper and/or lower extremities. Outcomes were correlated with history of surgical intervention. RESULTS: Children with Am and OD demonstrated moderate impairment of mobility (average: 35.2 and 35.9, respectively), while those with DA reported only mild impairment (average: 44.9). UE function was severely impaired for children with Am (average: 23.0), moderately impaired for OD (average: 33.0), and mildly impaired for DA (average: 43.4). All patient groups reported normal ranges of pain interference, as well as good peer relationships. A moderate negative correlation between number of surgical interventions and mobility scores, and a weak negative correlation between number of surgeries and pain interference scores were found. CONCLUSIONS: Children with AMC experience limited mobility and UE function, but normal levels of pain interference and good peer relationships. The average values provided in this study will serve as a baseline from which to evaluate the efficacy of both nonoperative and surgical interventions. LEVEL OF EVIDENCE: Level II-prognostic study; retrospective study.

Outcomes of Patellar Tendon Imbrication With Distal Femoral Extension Osteotomy for Treatment of Crouch Gait.

BACKGROUND: Crouch gait is a frequent gait abnormality observed in children with cerebral palsy. Distal femoral extension osteotomy (DFEO) with the tightening of the extensor mechanism is a common treatment strategy to address the pathologic knee flexion contracture and patella alta. The goal of this study was to review the results of a patellar tendon imbrication (PTI) strategy to address quadriceps insufficiency in the setting of children undergoing DFEO. METHODS: After institutional review board approval, all patients with crouch gait treated at a single institution with DFEO and PTI were identified. Clinical, radiographic, and instrumented gait analysis data were analyzed preoperatively and at 1 year following surgery. RESULTS: Twenty-eight patients (54 extremities) with a diagnosis of cerebral palsy and crouch gait were included. Significant improvements were appreciated in the degree of knee flexion contracture, quadriceps strength, knee extensor lag, and popliteal angle (P<0.01). Knee flexion at initial contact and during mid-stance improved significantly (P<0.0001), and knee moments in late stance were significantly reduced (P<0.01). The anterior pelvic tilt, however, significantly increased postoperatively (P<0.0001). Radiographic improvements were seen in the knee flexion angle and patellar station as assessed by the Koshino Sugimoto Index (P<0.0001). Four patients (14.2%) developed a recurrence of knee flexion contracture requiring further intervention. CONCLUSIONS: PTI is a simplified and safe technique to address quadriceps insufficiency when performing DFEO. The short-term results of patients who underwent DFEO with PTI demonstrated improvements in clinical, radiographic, and gait analysis variables of the knee. Investigating long-term outcomes, comparing techniques, and assessing quality of life measures are important next steps in research. LEVEL OF EVIDENCE: Level IV-case series.

Calcaneocuboid distraction arthrodesis for children with symptomatic pes planovalgus: does fixation improve outcomes?

Lateral column lengthening is a common surgical procedure for addressing symptomatic pes planovalgus foot deformity. For more severe cases, the use of a calcaneocuboid distraction arthrodesis (CCDA) can allow for more powerful correction. Previous reports have cited an increased risk of graft collapse with loss of correction when this procedure is performed without supplemental hardware fixation. The purpose of this study was to assess the outcomes of CCDA in children with and without supplemental locking fixation. A retrospective review from 2008 to 2016 of CCDA procedures with and without supplemental fixation was performed. The primary endpoint was graft collapse. Secondary objectives included evaluation of radiographic loss of correction, hardware failure, pain at 1-year follow-up, reoperations, and changes in the foot loading pattern foot per pedobarography. Twenty-nine feet in 24 patients were eligible for review. Supplemental locked fixation was used in 18 feet [hardware (HW)], with the remaining 11 feet managed without fixation [no hardware (NoHW)]. The overall failure rate on the basis of graft collapse and loss of correction was 55% (56% for the HW group, 55% for the NoHW group). Eleven patients (61%) in the HW group experienced hardware failure, with six (33%) of these requiring hardware removal. Fifty-six percent of the HW group and 45% of the NoHW group reported continued pain at 1-year follow-up. One patient from each group underwent revision arthrodesis. Supplemental locked fixation did not provide additional benefit in preventing graft collapse and loss of correction in this cohort. Alternative strategies should be considered to improve the outcomes for this procedure.

Iatrogenic Distal Radial Physeal Fracture During Insertion of Intramedullary Fixation for a Both Bone Forearm Fracture: A Case Report.

CASE: A 12-year-old boy with an isolated both bone forearm fracture was treated with closed reduction and flexible intramedullary nailing 11 days postinjury and after the loss of initial fracture alignment. On nail insertion, an intraoperative distal radial physeal fracture was encountered, thereby warranting modification in treatment. CONCLUSION: Physeal injury is a rare intraoperative complication of intramedullary fixation of forearm fractures. This report provides insight regarding the management and prevention of this rare complication.

Guided Growth for Ankle Valgus Deformity: The Challenges of Hardware Removal.

BACKGROUND: Ankle valgus deformity is associated with conditions such as clubfoot, cerebral palsy, and myelodysplasia. Guided growth strategies using a transphyseal screw provide effective correction of ankle valgus deformity. When correction occurs before skeletal maturity, screw removal is required to prevent overcorrection in the coronal plane. In this study, we reviewed the outcomes of guided growth procedures for correction of ankle valgus and related difficulty with hardware extraction. METHODS: A retrospective review of patients with ankle valgus managed with transphyseal screw placement was performed. Clinical and radiographic data, including the lateral distal tibial angle (LDTA), type of screw placed, and time to correction was recorded. At hardware removal, we reviewed elements associated with difficult extraction defined as requiring the use of specialized screw removal/extraction sets or inability to remove the entirety of the screw. RESULTS: One hundred nineteen patients (189 extremities) with a mean age of 11.7 years at time of screw placement met study inclusion criteria. Following correction of the valgus deformity, hardware removal occurred at an average of 18.4 months after placement of the screw. Preoperatively, the mean LDTA for the entire cohort was 81.3 degrees, and was corrected to a mean LDTA of 91.1 degrees. Complicated hardware removal occurred in 69 (37%) extremities. These 69 extremities had hardware in place an average of 1.8 years compared with an average of 1.4 years in extremities without difficult extraction (P<0.01). Six (9%) screws were unable to be removed in their entirety. Rebound valgus deformity occurred in 5 extremities (3%). CONCLUSIONS: Extraction of transphyseal screws in the correction of ankle valgus can be problematic. Specialized instrumentation was required in approximately one third of cases. Longevity of screw placement may be a factor that affects the ease of extraction. Additional exposure, access to specialized instrumentation, and additional operative time may be required for extraction. LEVEL OF EVIDENCE: Level IV-case series.