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 midstance 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 shortterm 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.
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.
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.
IntroductionArthrogryposis 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 analysisParticipants 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 disseminationEthical 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.
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.
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