Characterizing Pain Among Adolescents and Young Adults With Arthrogryposis Multiplex Congenita.

PURPOSE: Primary study objectives were to ( a ) characterize pain and explore differences between adolescents and adults with arthrogryposis multiplex congenita (AMC) and ( b ) evaluate associations between pain-related outcomes and mobility. METHODS: People who can walk and with AMC completed pain-related questionnaires. RESULTS: Sixty-three participants (28 adolescents and 35 young adults) were recruited. Pain was reported in the past week by 81% of participants; intensity ratings were similar between age groups. Per the McGill Pain Questionnaire, pain severity was significantly lower among adolescents. Adults had a greater number of painful regions compared with adolescents. Greater 7-day average pain intensity, McGill Pain Questionnaire scores, and number of painful regions were associated with reduced functional mobility. CONCLUSIONS: As most adolescents and young adults with AMC have at least mild pain, and pain is associated with mobility, future longitudinal investigations of pain and its functional consequences are warranted.

Test-retest reliability for performance-based outcome measures among individuals with arthrogryposis multiplex congenita.

BACKGROUND: Most individuals with arthrogryposis multiplex congenita, a rare condition characterized by joint contractures in ≥ 2 body regions, have foot and ankle involvement leading to compromised gait and balance. The purpose of this study was to establish between-days, test-retest reliability for performance-based outcome measures evaluating gait and balance, i.e., the 10-m Walk Test, Figure-of-8 Walk Test, 360-degree Turn Test, and modified Four Square Step Test, among adolescents and adults with arthrogryposis multiplex congenita. METHODS: This reliability study included ambulatory participants, aged 10 to 50 years, with a medical diagnosis of arthrogryposis multiplex congenita. Participants completed performance-based measures, in a randomized order, on two separate occasions. Intraclass correlation coefficients with 95% confidence intervals and minimal detectable changes at the 90% and 95% confidence level were calculated. RESULTS: Participants included 38 community-ambulators with a median of 13 out of 14 upper and lower joint regions affected. Intraclass correlation coefficient point estimates and 95% confidence intervals ranged from .85-.97 and .70-.98, respectively. Minimal detectable changes were 10 to 39% of sample means and were largest for the modified Four Square Step Test. CONCLUSIONS: Among individuals with arthrogryposis, gait speed per the 10-m Walk Test, as well as non-linear walking and dynamic balance assessment per the Figure-of-8 Walk and 360 Degree Turn Tests, have adequate test-retest reliability enabling evaluation of individual patient changes. Changes in groups of ambulatory individuals with arthrogryposis multiplex congenita may be reliably evaluated with all of the studied outcome measures.

Applications and Error Ratios of Calibration Techniques in EOS, Orthoroentgenogram, and Teleoroentgenogram for Length Measurement: A Comparative Study.

BACKGROUND: Accurate length measurements of extremity bones are essential in treating limb deformities and length discrepancies in children. OBJECTIVE: This study aimed to determine errors in common techniques used to measure lower limb lengths in children. METHODS: Precision and instrument errors in length measurements were studied utilizing electro-optical system (EOS), orthoroentgenogram, and teleoroentgenogram The goal was to measure a 70-cm metallic rod phantom (average length of the lower extremity of a 10-year-old boy in the 50th percentile) in 3 phases. In Phase 1, the length measurements were performed in an EOS unit with internal calibrations, a magball/magstrip in various scan positions, and measurement with TraumaCAD software. In Phase 2, the measurements were repeated utilizing a single radiation "shot" teleoroentgenogram. In Phase 3, an orthoroentgenogram was utilized with a radiopaque ruler reference. The reliability and validity of measurements were calibrated by 4 physicians (a radiologist, senior orthopaedic attending, and 2 orthopaedic fellows). RESULTS: EOS measurements utilizing internal references had excellent accuracy (for a 700-mm real length, magnification error (ME)] of 0.09%. Teleoroentgenogram with a magball reference and measurements performed with automatic calibration by TraumaCAD program results in ME of 1.83% with insignificant intraobserver/interobserver difference. Teleoroentgenogram with a magball or magstrip reference measured manually showed that the magball has higher intraobserver/interobserver variance than magstrip, with a 6.60 and 0.33-mm SD, respectively. The length by manual measurement utilizing the magstrip has ME of 2.21%. Orthoroentgenogram is accurate with ME of 0.26%, but does not allow anatomical analysis and is also radiation-costly. CONCLUSION: EOS and orthoroentgenogram are very accurate for length measurements. Teleoroentgenogram is less accurate in measuring length; however, addition of an external reference (magball, magstrip) placed lateral to the target improves accuracy. Automatic calibration with computer-based analysis of the external reference improves the accuracy more than manual calibration. If manual calibration is utilized, the length measurement is less accurate with the magball than the magstrip. LEVEL OF EVIDENCE: Level II-comparative in vitro study.

Falls and Associated Factors among Adolescents and Young Adults with Arthrogryposis Multiplex Congenita.

BACKGROUND: Falls research among individuals with arthrogryposis multiplex congenita (AMC), a group of congenital conditions characterized by joint contractures in two or more body regions, is sparse. The primary objectives of this study were to estimate the prevalence of single, multiple, and injurious falls among adolescents and adults with AMC and identify factors associated with multiple and injurious falls. METHODS: Individuals, aged 10-50 years, with a diagnosis of AMC completed questionnaires obtaining demographic and AMC-specific information, falls history (e.g., number, injurious/non-injurious), markers of bone health, orthopedic surgical history, and mobility aid use, as well as the Gillette Functional Assessment Questionnaire and the Saltin-Grimby Physical Activity Level Scale. Falls were defined as "any body part above the ankle coming to rest on the ground, floor, or a lower level". Falling was defined as ≥ 1 fall, while multiple falls were defined as ≥ 2 falls in the past year.Differences in falling, multiple falls, and injurious falls rates between adolescents (10-17 years) and adults (aged 18-50 years) were evaluated. Using univariate binary logistic regression models, associations between participant characteristics and multiple falls, as well as injurious falls, were evaluated, while considering age as a covariate (p ≤ 0.050); odds ratios (ORs) and 95% confidence intervals (CIs) were calculated. RESULTS: Adolescents (N = 28; median age = 14 years) and adults (N = 40; median age = 32 years) with AMC had similar falling, i.e., 89.3% versus 70.0%, and injurious fall rates, i.e., 32.1% versus 27.5%, respectively (p > 0.050).Adolescents with AMC, however, were more likely to report multiple falls in the past year, i.e., 89.3%, when compared to adults with AMC, i.e., 57.5% (p = 0.005). Beyond age, multiple falls were associated with a greater number of lower-limb surgeries [p = 0.036, OR (95%CI): 1.18 (1.01-1.39)], ability to transfer floor-to-stand with support [p = 0.002, OR (95%CI): 8.98 (2.30-35.06)], and increased mobility per the Gillette Functional Assessment Questionnaire [p = 0.004, OR (95%CI): 1.48 (1.13-1.92)]. Factors associated with a reduced odds of multiple falls were spinal involvement [p = 0.025, OR (95%CI): 0.23 (0.07-0.84)], history of spinal surgery [p = 0.018, OR (95%CI: 0.18 (0.04-0.74)], greater upper extremity involvement [OR (95%CI): 0.65 (0.44-0.95)], home assistive device use [p = 0.010, OR (95%CI): 0.15 (0.03-0.63)], and community wheelchair use [p = 0.006, OR (95%CI): 0.16 (0.04-0.59)]. None of the explored characteristics were associated with injurious falls in the past year (p > 0.050). CONCLUSION: Falls are exceedingly common among adolescents and adults with AMC; potential risk and protective factors for multiple falls are identified for future prospective falls research.

Percutaneous Osteotomies in Pediatric Deformity Correction.

Lower extremity deformities in children rely on osteotomies for correction. Percutaneous osteotomies offer a method for cutting bone that is advantageous for soft tissue healing. These low-energy osteotomies preserve the blood supply to bone, which maximizes the ability of bone to heal. Some of these techniques are technically demanding and should be performed first with an experienced operator. The key to maintaining safety in these osteotomies is to remain in a subperiosteal location. The categories of percutaneous osteotomy include multiple drill hole osteotomy, corticotomy, and Gigli saw osteotomy. This article discusses the advantages and indications for each type of osteotomy.

A Metaphyseal Corner Fracture that Wasn't: A Case Report of Osteitis from Congenital Syphilis.

CASE: Congenital syphilis (CS) is an infrequently seen condition in the United States; however, rates of CS have been on the rise. We present a case of an infant with a lesion of the radius that was initially diagnosed as a metaphyseal corner fracture and treated as such until maternal syphilis testing was noted to be positive. Ultimately, the child was diagnosed with CS. She is now undergoing treatment with penicillin and recovering well. CONCLUSION: Although CS is rare, the incidence is on the rise and should remain on the differential of lytic lesions of bone in young children.