Bethlem Myopathy (Collagen VI-Related Dystrophies): A Retrospective Cohort Study on Musculoskeletal Pathologies and Clinical Course.

BACKGROUND: Collagen VI-related myopathies with pathologic COL6A1, COL6A2, and COL6A3 variants manifest as a phenotypic continuum of rare disorders, including Bethlem myopathy (BM), characterized by early onset muscle weakness, proximal joint contractures, and distal joint laxity. Herein we discuss the concomitant orthopedic manifestations of BM, potential management strategies, and patient outcomes. METHODS: An IRB-approved retrospective cohort study (n=23) from 2 pediatric institutions with a confirmed diagnosis of BM. Charts were reviewed for demographic data, age of disease presentation and diagnosis, COL6 genotype, diagnosis method, ambulation status, need for assistance, musculoskeletal abnormalities, other systemic comorbidities, advanced imaging and screening diagnostics, previous surgical interventions, and progression of the disease. RESULTS: The mean age was 11.65 years (range 3 to 19 y). Mean age at initial presentation with symptoms was 4.18 years old, whereas diagnosis was delayed until 8.22 years old on average. Muscle weakness was the most common presenting symptom (65.2%), and 73.9% of patients required some use of assistive or mobility devices. Overall, 30.4% of patients were diagnosed with scoliosis; 57.1% required operative intervention for their scoliosis; 43.5% of patients had acetabular dysplasia; 10% required open reduction of a dislocated hip; 10% required closed reduction with hip spica application; 10% required bilateral periacetabular osteotomies for instability; 91.3% of patients developed foot and ankle deformities; 33.3% of patients underwent posteromedial-lateral equinovarus releases; 28.6% required an Achilles tendon lengthening, and 86.9% of patients had muscle tendon contractures, the most common locations being the ankle (55%) and elbow (40%). CONCLUSION: Although often less severe than other more common neuropathies and myopathies like Charcot-Marie-Tooth disease and Duchenne muscular dystrophy, BM does lead to progressive musculoskeletal deformity and disability. Its relative rarity makes it less familiar to providers and likely contributes to delays in diagnosis. Scoliosis, hip dysplasia, and equinus and varus ankle deformities are the most common musculoskeletal deformities. Physicians and surgeons should appropriately counsel patients and families about the clinical course of this disorder and the potential need for mobility assistance or surgical procedures. LEVEL OF EVIDENCE: III, Prognostic. study.

Pie-Crusting Capsulotomy Provides Similar Visualization With Increased Repair Stiffness Compared With a T-Capsulotomy: A Biomechanical Study.

PURPOSE: To compare the area of visualization, capsular stiffness, and strength between the pie-crusting capsulotomy technique and the T-capsulotomy technique following repair. METHODS: Eight matched pairs of fresh-frozen cadaveric hips (n = 16) were divided to either T-capsulotomy or pie-crusting capsulotomy followed by subsequent repair. The area of visualization was measured for all capsulotomy states using a digitizing probe. Hips were then distracted along the iliofemoral ligament in the intact, extended capsulotomy, and repair states. Afterwards, specimens were externally rotated to failure. RESULTS: An average force of 250.1 ± 16.1 N was required to distract intact hips to 6 mm. Both extended capsulotomy techniques reduced the force required to distract the hip 6 mm with no statistical difference between the two (T-capsulotomy [T-cap] = 114.3 ± 63.4 N vs pie-capsulotomy [Pie-cap] = 170.1 ± 38.8 N), P = .07. Subsequent repair of the extended capsulotomies demonstrated the pie-crust capsulotomy required significantly greater force to reach 6 mm of distraction than those with a repaired T-capsulotomy (T-cap = 165.04 ± 40.43N vs Pie-cap = 204.43 ± 10.13N), P = .03. There was no significant difference in ultimate torque to failure between the 2 techniques (T-cap = 22.0 ± 7.41 N·m vs Pie-cap = 27.01 ± 11.13 N·m), P = .28. Visualization significantly increased with each extended capsulotomy, with an average increase of 62% (P < .001) and 48% (P < .001) for the pie- and T-capsulotomies, respectively. CONCLUSIONS: The pie-crusting technique maintained similar strength and increased stiffness to the T-capsulotomy following repair while using less suture. Both techniques provided similar visualization. Clinically, the pie-crusting technique provides an alternative to the T-capsulotomy with similar biomechanical and visual outcomes. CLINICAL RELEVANCE: Visualization during hip arthroscopy can be difficult with large cam morphology. Techniques to improve visualization while restoring the native biomechanics of the hip as best as possible are important.

Validating an Algorithm to Predict Adjacent Musculoskeletal Infections in Pediatric Patients With Septic Arthritis.

BACKGROUND: Septic arthritis is frequently associated with adjacent infections including osteomyelitis and subperiosteal and intramuscular abscesses. While often clinically indiscernible from isolated septic arthritis, the diagnosis of adjacent infections is important in determining the need for additional surgical intervention. MRI has been used as the diagnostic gold standard for assessing adjacent infection. Routine MRI, however, can be resource-intensive and delay surgical treatment. In this context, there is need for additional diagnostic tools to assist clinicians in determining when to obtain preoperative MRI in children with septic arthritis. In a previous investigation by Rosenfeld et al., an algorithm, based on presenting laboratory values and symptoms, was derived to predict adjacent infections in septic arthritis. The clinical applicability of the algorithm was limited, however, in that it was built from and applied on the same population. The current study was done to address this criticism by evaluating the predictive power of the algorithm on a new patient population. QUESTIONS/PURPOSES: (1) Can a previously created algorithm used for predicting adjacent infection in septic arthritis among pediatric patients be validated in a separate population? METHODS: Records for all pediatric patients (1-18 years old) surgically treated for suspected septic arthritis during a 3-year period were retrospectively reviewed (109 patients). Of these patients, only those with a diagnosis of septic arthritis confirmed by synovial fluid analysis were included in the study population. Patients without confirmation of septic arthritis via synovial fluid analysis, Gram stain, or culture were excluded (34 patients). Patients with absence of MRI, younger than 1 year, insufficient laboratory tests, or confounding concurrent illnesses also were excluded (18 patients), resulting in a total of 57 patients in the study population. Five variables which previously were shown to be associated with risk of adjacent infection were collected: patient age (older than 4 years), duration of symptoms (> 3 days), C-reactive protein (> 8.9 mg/L), platelet count (< 310 x 10 cells/µL), and absolute neutrophil count (> 7.2 x 10 cells/µL). Adjacent infections were determined exclusively by preoperative MRI, with all patients in this study undergoing preoperative MRI. MR images were read by pediatric musculoskeletal radiologists and reviewed by the senior author. According to the algorithm we considered the presence of three or more threshold-level variables as a "positive" result, meaning the patient was predicted to have an adjacent infection. Comparing against the gold standard of MRI, the algorithm's accuracy was evaluated in terms of sensitivity, specificity, positive predictive value, and negative predictive value. RESULTS: In the new population, the sensitivity and specificity of the algorithm were 86% (95% CI, 0.70-0.95) and 85% (95% CI, 0.64-0.97), respectively. The positive predictive value was determined to be 91% (95% CI, 0.78-0.97), with a negative predictive value of 77% (95% CI, 0.61-0.89). All patients meeting four or more algorithm criteria were found to have septic arthritis with adjacent infection on MRI. CONCLUSIONS: Critical to the clinical applicability of the above-mentioned algorithm was its validation on a separate population different from the one from which it was built. In this study, the algorithm showed reproducible predictive power when tested on a new population. This model potentially can serve as a useful tool to guide patient risk stratification when determining the likelihood of adjacent infection and need of MRI. This better-informed clinical judgement regarding the need for MRI may yield improvements in patient outcomes, resource allocation, and cost. LEVEL OF EVIDENCE: Level II, diagnostic study.

Racial and Ethnic Disparities in Pediatric Musculoskeletal Care.

PURPOSE OF REVIEW: This article provides a review of recent published research studying racial, ethnic, and socioeconomic disparities in pediatric musculoskeletal care. RECENT FINDINGS: Disparities in pediatric musculoskeletal care are demonstrated in two general realms: access to care and health outcomes. Though initiatives have been proposed or enacted to address disparities, underrepresented minorities and patients from lower socioeconomic statuses continue to face barriers across the spectrum of orthopedic care and poorer ultimate outcomes after both non-operative and operative management. Minority pediatric patients and those from lower socioeconomic statuses experience delays across the spectrum of orthopedic care for both urgent and non-urgent conditions. They wait longer between injury date and initial orthopedic evaluation, longer to receipt of diagnostic imaging, and longer to ultimate treatment than their counterparts. When finally able to obtain musculoskeletal care and treatment, they are at higher risk of poor in-hospital outcomes and inpatient complications, worse patient reported outcomes, and suboptimal pain management. In the outpatient setting, they receive less physical therapy and follow-up clinic visits, resulting in greater stiffness and strength deficits, and are ultimately less likely to meet return to sport criteria.

Racial and Sex Disparities in Resident Attrition Among Surgical Subspecialties.

Importance: Racial and sex disparities are prevalent in surgical trainees. Although retrospective studies on resident attrition have been conducted for individual specialties, this study analyzes racial and sex differences in resident attrition among all surgical subspecialties over an 18-year period. Objective: To evaluate the racial and sex differences in resident attrition among surgical specialties over an 18-year period. Design, Setting, and Participants: This was a large, cross-sectional, database study that analyzed program-reported resident censuses (program information, resident demographics, and attrition status) obtained by the Association of American Medical Colleges from 2001 to 2018 for trainees in surgical residency programs. Data were analyzed from March 20, 2021, to June 8, 2022. Main Outcomes and Measures: Demographic trends (including race and ethnicity and sex) for all surgical subspecialty training programs over an 18-year period. Resident attrition includes all-cause withdrawals, dismissals, and transfers to another specialty. Unintended attrition encompasses all withdrawals, dismissals, and transfers except for changing career plans. Results: This study included 407 461 program-reported resident years collected from 112 205 individual surgical residents (67 351 male individuals [60.0%]). The mean percentage of female trainees was 40.0% (44 835) and increased over the study period. Sex disparity remained greatest in orthopedic surgery. Residents who were underrepresented in medicine (URiM) comprised 14.9% (16 695) of all surgical trainees but demonstrated a 2.1% decrease over the study period. Overall attrition rate among all specialties was 6.9% (7759), with an unintended attrition rate of 2.3% (2556). Female residents had a significantly higher relative risk (RR) of attrition (RR, 1.16; 95% CI, 1.11-1.22; P < .001) and unintended attrition (RR, 1.17; 95% CI, 1.08-1.26; P < .001) compared with their male counterparts. URiM residents were at significantly higher RR for attrition (RR, 1.40; 95% CI, 1.32-1.48; P < .001) and unintended attrition (RR, 1.92; 95% CI, 1.75-2.11; P < .001) compared with non-URiM residents. The highest attrition (10.6% [746 of 7043]) and unintended attrition (5.2% [367 of 7043]) rates were in Black/African American residents. The lowest attrition and unintended attrition rates were seen in White residents at 6.2% (4300 of 69 323) and 1.8% (1234 of 69 323), respectively. Black/African American residents were at disproportionate risk for attrition (RR, 1.66; 95% CI, 1.53-1.80; P < .001) and unintended attrition (RR, 2.59; 95% CI, 2.31-2.90; P < .001) compared with all other residents. Orthopedic surgery had the highest attrition (RR, 3.80; 95% CI, 2.84-5.09; P < .001) and unintended attrition (RR, 7.20; 95% CI, 4.84-10.71; P < .001) for Black/African American residents. Conclusions and Relevance: Results of this cross-sectional study suggest that the percentage of female residents in surgical specialties has improved over the last 18 years, and the percentage of URiM residents has remained relatively unchanged. Risk for attrition and unintended attrition was significantly elevated for female and URiM residents, specifically Black/African Americans. These results highlight current racial and sex disparities in resident attrition and demonstrate the importance of developing strategies to recruit, retain, and support residents.

Racial and Sex Disparities in Resident Attrition in Orthopaedic Surgery.

Studies have suggested that female individuals and individuals from backgrounds under-represented in medicine (URiM) are at increased risk of attrition during residency. This likely exacerbates the lack of diversity in our field. The aims of this study were to (1) characterize demographic composition in orthopaedic residency from 2001 to 2018 and (2) determine the race/ethnicity and identify any disparities. Methods: Demographic and attrition data from 2001 to 2018 were obtained from the Association of American Medical Colleges. Attrition data comprised the following categories: withdrawals, dismissals, and transfers to another specialty. Analysis compared demographic composition and determined attrition rates with subgroup analysis by race/ethnicity and sex. Results: From 2001 to 2018, female orthopaedic residents increased from 8.77% to 15.54% and URiM residents from 9.49% to 11.32%. The overall and unintended attrition rates in orthopaedic surgery were 3.20% and 1.15%, respectively. Among female residents, the overall and unintended attrition rates were 5.96% and 2.09% compared with 2.79% and 1.01%, respectively, in male residents. URiM residents had overall and unintended attrition rates of 6.16% and 3.11% compared with 2.71% and 0.83%, respectively, for their White counterparts. Black/African American residents had an attrition rate of nearly 10%. Female residents averaged 12.9% of all residents but 24% of those leaving orthopaedics. URiM residents were 10.14% of all residents but 19.51% of those experiencing attrition. In logistic regression models, female residents had a relative risk (RR) of 2.20 (p < 0.001) for experiencing all-cause attrition and 2.09 (p < 0.001) for unintended attrition compared with male residents. Compared with their White male counterparts, URiM residents had a RR for overall and unintended attrition of 2.36 and 3.84 (p < 0.001), respectively; Black/African American residents had a RR for the same of 3.80 and 7.20 (p < 0.001), respectively. Conclusion: Although female resident percentage has increased, orthopaedics continues to train fewer female surgeons than all other fields. Female and URiM residents in orthopaedic surgery are disproportionately affected by attrition. While recruitment has been the primary focus of diversity, equity, and inclusion efforts, this study suggests that resident retention through appropriately supporting residents during training is equally critical.

Preoperative Transcutaneous Oxygen Perfusion Measurements in Predicting Atraumatic Wound Healing After Major Lower Extremity Amputation.

This study examined the correlation between preoperative transcutaneous oxygen perfusion (TcPO2) measurement and the success of wound healing after major lower extremity amputation. There is no validated consensus on how to accurately determine appropriate amputation levels. A TcPO2 greater than 30 to 40 mm Hg is widely cited as a positive predictor of postoperative wound healing, but its validity has not been well defined. A retrospective chart review was performed for patients who underwent above-knee amputation (AKA), through-knee amputation, or below-knee amputation (BKA) at a single institution from 2012 to 2018 with preoperative TcPO2 values and a minimum 30-day postoperative clinical follow-up. This review yielded 141 total amputations: 93 BKAs, 6 through-knee amputations, and 42 AKAs. Fifty-five amputations were unsuccessful, defined by postoperative wound dehiscence or infection. Of these, 37 were BKAs, 4 were through-knee amputations, and 14 were AKAs. There was a significant difference in preoperative TcPO2 between the successful and unsuccessful amputations, at 46.2 and 38.3 mm Hg, respectively (P=.02). A TcPO2 of 30 to 40 mm Hg showed a 68.8% success rate, and a TcPO2 of less than 20 mm Hg showed an 18.2% success rate. A receiver operating characteristic curve for TcPO2 predicting amputation success had an area under the curve of 0.53 for AKAs and 0.61 for BKAs; the diagnostic ability is far from prognostic. There is no linear association between TcPO2 and success rate. A TcPO2 of less than 20 mm Hg has a high positive predictive value for failure, but higher levels are not 100% predictive of wound healing success after amputation, as previously reported. [Orthopedics. 2022;45(3):174-180.].

Spica MRI predictors for epiphyseal osteonecrosis after closed reduction treatment of dysplasia of the hip.

Spica MRI with intravenous gadolinium contrast after closed reduction for developmental dysplasia of the hip (DDH) helps to determine successful reduction and attempts to identify patients at risk for epiphyseal osteonecrosis. The objective of our study was to evaluate spica MRI predictors for epiphyseal osteonecrosis after closed reduction. This was a retrospective study of all patients undergoing closed reduction for DDH followed by gadolinium-enhanced spica MRI between July 2011 and November 2014. Patient demographics and clinical follow-up through 2017, including the development of epiphyseal osteonecrosis and need for reintervention after the initial reduction, were recorded. MRI data included hip abduction angles and quantifying the percentage of femoral head enhancement. Twenty-five hips in 21 patients (16 girls, five boys, mean age: 0.99 years, range: 0.4-3.1 years) were included in our study. The mean follow-up period was 3 ± 1.5 years (range: 0.65-6.1 years). Eight (32%) of 25 hips went on to develop osteonecrosis. Epiphyseal osteonecrosis was more likely with less than 80% enhancement (sensitivity 87.5%, specificity 88.25%, positive predictive value 78%, negative predictive value 94%). The mean contrast enhancement for patients developing osteonecrosis compared with those who did not was 37.5 and 86.5%, respectively; P = 0.001. Immediate postspica MRI with gadolinium is a useful prognostic tool for determining future risk for epiphyseal osteonecrosis in children treated for DDH. Our data complement existing literature and suggest that even in cases with partial epiphyseal enhancement, osteonecrosis may still develop. When the epiphyseal enhancement is less than 80%, it is recommended that spica cast revision is considered.

Patient Factors Associated With Methicillin-Resistant Staphylococcus aureus Septic Arthritis in Children.

Staphylococcus aureus is the most common causative organism in pediatric septic arthritis, with methicillin-resistant Staphylococcus aureus (MRSA) being responsible for a significant portion of these infections. Early identification and initiation of proper treatment may improve outcomes by minimizing potential morbidity. The purpose of this study was to identify variables obtained on initial patient presentation associated with MRSA septic arthritis. Sixteen factors were retrospectively evaluated in 109 consecutive pediatric patients diagnosed with culture-confirmed septic arthritis. Graphical and logistical regression analyses were employed to determine factors independently predictive of MRSA septic arthritis. Twenty-seven (25%) patients had MRSA and 82 (75%) had non-MRSA septic arthritis. C-reactive protein of 13.7 mg/L or greater, duration of symptoms of 4 days or more, heart rate of 126 beats per minute or greater, and absolute neutrophil count of 8.72×103 cells/µL or greater were associated with MRSA septic arthritis. Ultimately, 98% of patients with 1 or no risk factors had non-MRSA and 96% of patients with MRSA septic arthritis had 2 or more positive risk factors. Elevated C-reactive protein, duration of symptoms, heart rate, and absolute neutrophil count are predictive of MRSA infection in the setting of pediatric septic arthritis and can be obtained on initial evaluation. In patients for whom there is concern for MRSA infection, this may guide more expedient treatment, such as early initiation of contact precautions and appropriate antibiotic therapy before culture results become available. [Orthopedics. 2018; 41(2):e277-e282.].

Chronic Osteomyelitis Caused by Haemophilus parainfluenzae: A Case Report.

CASE: A 14-year-old boy presented with a pathologic fracture of the distal aspect of the tibia and a remote history of a dog bite near the injury site. Imaging studies, biopsy, and presentation corroborated the diagnosis of chronic osteomyelitis. Multiple diagnostic methods were negative until an open biopsy identified Haemophilus parainfluenzae, a fastidious oropharyngeal bacterium, with polymerase chain reaction analysis. The patient underwent extensive debridement, placement of external fixation, and a year-long antibiotic therapy regimen. He subsequently required a tibial-fibular osteotomy at a second site with placement of an intramedullary nail for correction of a leg-length discrepancy. CONCLUSION: This case report illustrates the complex management of chronic osteomyelitis in pediatric patients, its sequelae, and the importance of considering treatment of atypical pathogens.

Two cases of combined patellar tendon avulsion from the tibia and patella.

Avulsion fractures of the inferior pole of the patella and proximal tibial apophysis are independently rare injuries. They occur in children due to the relative weakness of the apophyseal cartilage compared to the ligaments and tendons. The combination of these two fractures, is exceedingly rare, with only a few previously described cases in the literature. Due to the infrequent presentation of this injury, careful examination and consideration of advanced imaging is important for diagnosis and preoperative planning. Here we present two cases of combined sleeve fractures of the inferior pole of the patella and tibial apophysis, with discussion of the pathophysiology, classification, identification and management of the injury.

Closed and Open Reduction of Displaced Pediatric Lateral Condyle Humeral Fractures, a Study of Short-Term Complications and Postoperative Protocols.

BACKGROUND: Displaced lateral condyle humeral fractures in children are treated operatively to maximize function and growth of the elbow. Traditionally an open approach is used for reduction of the fracture, but recent series have shown promising results with closed reduction. Percutaneous pins are typically used for fixation, no matter the reduction method. This retrospective review compares our experience with early complications after open and closed reduction of these fractures. METHODS: We retrospectively reviewed charts and radiographs of operatively treated lateral condyle fractures. The Song and Jakob classification systems were utilized to determine fracture severity. High-grade displacement was defined as Song stage 4 and 5, or Jakob type 3. Data was analyzed by fracture type (high-grade or low-grade) and by treatment method to look for differences in complication rates and treatment differences. Complications were defined as delayed union and infection. RESULTS: 172 fractures were analyzed, 141 were treated open, and 31 were treated closed. There were no statistically significant differences in pin duration, total cast time, additional procedures, or short term complications between the open and closed treatment groups, or the high and low-grade fracture groups. High-grade fractures were more likely to be treated with open reduction (p<0.0001). Pin duration prior to removal was not associated with increased incidence of infection or delayed union. CONCLUSIONS: Closed reduction and percutaneous pinning of lateral condyle fractures amenable to this treatment does not seem to require any changes in postoperative treatment or alter the incidence of early complications when compared to open procedures. LEVEL OF EVIDENCE: IV.