Pain and fatigue in adult patients with multiple osteochondromas: The Netherlands.

BACKGROUND: Multiple Osteochondromas (MO) is a rare genetic disorder characterised by the presence of numerous benign bone tumours, known as osteochondromas. Within the spectrum of debilitating symptoms associated with MO, pain is recognized as a major problem. Interestingly, our clinical observations suggest that fatigue is also a significant concern but has merely been touched upon in MO literature. This study aims to (1) assess the level of pain and fatigue in adult patients with MO; (2) compare fatigue in MO to healthy subjects and patients with Rheumatoid Arthritis (RA); (3) identify associated variables for pain and fatigue in patients with MO. METHODS: In this cross-sectional study, 353 adult MO patients completed a survey with validated questionnaires on pain, fatigue and psychosocial factors. Pain and fatigue were assessed with the Numeric Rating Scale (NRS), and fatigue was also measured with the Checklist Individual Strength (CIS). Fatigue (CIS) was compared with reference scores of healthy subjects and patients with RA, using a one-sample t-test. Multiple linear regression models for pain and fatigue were developed using a-priori selected independent variables based on a theoretical framework (ICF-model). RESULTS: Pain was reported by 87.8% (NRS = 3.19±2.6) and fatigue by 90.4% (NRS = 4.1±2.6) of patients with MO. Fatigue scores for MO (CIS = 84.1±15.3) were significantly higher (p<0.001) compared to reference scores of healthy subjects and patients with RA. The multivariable analysis for pain provided a final regression model with six variables (R2 = 0.445, p<0.001) of which fear avoidance beliefs and fatigue had the strongest association. For the fatigue models NRS (R2 = 0.455, p<0.001) and CIS (R2 = 0.233, p<0.001), the strongest associations were found with anxiety and depression respectively. CONCLUSIONS: Pain and fatigue are highly prevalent in patients with MO. Fatigue is significantly higher compared to healthy subjects and patients with RA. Several variables associated with pain and fatigue have been identified that could help improve multidisciplinary treatment plans.

Concurrent intraosseous cartilaginous lesions in patients with multiple osteochondromas identified on total-body MR imaging.

PURPOSE: To assess the prevalence of intraosseous cartilaginous lesions in patients with multiple osteochondromas based on total-body (TB) MRI examinations, used for screening purposes. SUBJECTS AND METHODS: Between 2013 and 2020, TB-MRI examinations were performed in 366 patients with proven multiple osteochondromas syndrome, to rule out malignant progression. For this study, presence, or absence of intraosseous central or eccentrical chondroid lesions, defined as lobulated lesions with low signal intensity on T1-weighted images, replacing bone marrow and high signal intensity equal to fluid on T2-weighted images in the bone marrow of the meta-diaphysis of (one of) the long bones, were recorded in the long bones as part of a TB-MRI protocol. RESULTS: In 62 patients out of the 366 MO patients (17%), one or more intraosseous chondroid lesions (either enchondroma or atypical cartilaginous tumor) were detected. The age of the patients at time of diagnosis ranged from 17 to 61 years (mean, 36). Size of the lesions varied from 4 to 69 mm (mean, 16.3 mm). The most common location was the proximal femur (n = 29), followed by the distal femur and proximal humerus (n = 18 and n = 10, respectively). In nine of the patients with an intraosseous chondroid lesion, a second and/or third TB-MRI were available during the period of evaluation (mean interval, 2.7 years between the exams). In none of these patients increase of these intraosseous lesions was noticed. CONCLUSION: Intraosseous chondroid lesions (enchondroma and ACT) appear to occur more frequently in MO patients than in the general population. TB-MRI allows to detect these, besides the identification of OC with suspicious features.

Transitional care of adolescents with Multiple Osteochondromas: a convergent mixed-method study 'Patients', parents' and healthcare providers' perspectives on the transfer process'.

OBJECTIVES: Multiple osteochondromas (MO) is a rare hereditary disease characterised by numerous benign bone tumours. Its chronic aspect requires a well-organised transition from paediatric care to adult care; however, little is known on organising this care specific for patients with MO. This study aims to gain insight on this topic. DESIGN: Convergent mixed-method study. SETTING: This study was conducted at the orthopaedic and paediatrics department of an MO-expertise centre in the Netherlands. PARTICIPANTS: 12 patients, 10 parents and 10 healthcare professionals were interviewed. An additional survey was completed by 2 young adults. PRIMARY AND SECONDARY OUTCOMES: The (1) themes on transition, identified through template analysis and (2) transfer experience and satisfaction assessed by an 18-item On Your Own Feet-Transfer Experience Scale (OYOF-TES, range: 18-90) and by Numeric Rating Scale (NRS, range: 1-10). RESULTS: The following three key themes were identified in the qualitative analysis: (1) patient and parent in the lead can be encouraged by self-management tools, (2) successful transfers need interprofessional collaboration and communication and (3) how can we prepare patients for the transitional process? Stakeholders' insights to improve transition were listed and divided into these three themes.Several important aspects were underlined, particularly within the first theme; speaking-up was difficult for patients especially when parents were not directly involved. Moreover, the high psychological impact of the disease requires coaching of self-management and psychological counselling to facilitate stakeholders in their changing roles.Twenty patients completed the quantitative survey. Mean satisfaction score with the transfer process was poor, which was assessed with the NRS (mean=5.7±2.1; range: 1-9) and the OYOF-TES (mean=56.3±14.2; range: 32-85). The OYOF-TES only showed a negative correlation (R2=0.25; p=0.026) with the number of surgical interventions in the past. CONCLUSION: Overall, the transfer process was found unsatisfactory. Improvement can be achieved by supporting and guiding the patients to be in the lead of their care. Moreover, preparation for transfer and a multidisciplinary approach may enhance successful transition.

Comparative Analysis of Impairment Ratings From the 5th to 6th Editions of the AMA Guides.

OBJECTIVE: The association of American Medical Association's (AMA) Guides edition with impairment ratings is uncertain. METHODS: We used data from a consecutive sample of 249 injured workers referred for an independent evaluation 10 months before and after assessors switched from the 5th to the 6th edition of the AMA Guides. RESULTS: The median whole person impairment rating was 7.0% (interquartile range [IQR]: 4 to 14) for 131 claimants assessed with the 5th edition of the Guides, and 4.0% (IQR: 2 to 8) for 118 claimants assessed with the 6th edition (P-value for difference: 0.002). Multivariable analysis showed a 36.4% relative reduction (95% confidence interval [CI] 17.2% to 57.3%) in impairment rating with the 6th edition of the Guides versus the 5th edition. CONCLUSIONS: The 6th edition of the AMA Guides provides systematically lower impairment ratings for injured workers than the 5th edition.

Classification systems for distal radius fractures.

Background and purpose - The reliability of conventional radiography when classifying distal radius fractures (DRF) is fair to moderate. We investigated whether reliability increases when additional computed tomography scans (CT) are used. Patients and methods - In this prospective study, we performed pre- and postreduction posterior-anterior and lateral radiographs of 51 patients presenting with a displaced DRF. The case was included when there was a (questionable) indication for surgical treatment and an additional CT was conducted within 5 days. 4 observers assessed the cases using the Frykman, Fernández, Universal, and AO classification systems. The first 2 assessments were performed using conventional radiography alone; the following 2 assessments were performed with an additional CT. We used the intraclass correlation coefficient (ICC) to evaluate reliability. The CT was used as a reference standard to determine the accuracy. Results - The intraobserver ICC for conventional radiography alone versus radiography and an additional CT was: Frykman 0.57 vs. 0.51; Fernández 0.53 vs. 0.66; Universal 0.57 vs. 0.64; AO 0.59 vs. 0.71. The interobserver ICC was: Frykman: 0.45 vs. 0.28; Fernández: 0.38 vs. 0.44; Universal: 0.32 vs. 0.43; AO: 0.46 vs. 0.40. Interpretation - The intraobserver reliability of the classification systems was fair but improved when an additional CT was used, except for the Frykman classification. The interobserver reliability ranged from poor to fair and did not improve when using an additional CT. Additional CT scanning has implications for the accuracy of scoring the fracture types, especially for simple fracture types.

Skeletal maturity of children with multiple osteochondromas: is diminished stature due to a systemic influence?

BACKGROUND: Multiple ostechondromas (MO) is an autosomal dominant inherited disease caused by mutated exostosin genes. It mostly affects the long bones and can lead to growth disturbances, especially disproportionate short stature. Both the local effect on growth plates and the systemic influence of the gene disorder on growth mechanisms might explain the diminished stature. PURPOSE: The hypothesis of this study is that the diminished stature in adults with MO is due to a systemic influence, leading to early skeletal maturation and early closure of the growth plate. Therefore, in these patients the skeletal age in adolescence is hypothesized to be higher than the calendar age. METHODS: Radiographs of the left hand were collected from 50 MO-affected children. The skeletal age was calculated using these radiographs according to the Greulich-Pyle bone scale and was compared to the calendar age at the time of radiography. RESULTS: Children aged 3-12 years had a significantly lower skeletal age compared to their calendar age (p = 0.030). Children aged 12-17 years had a significantly higher skeletal age (p = 0.019), especially boys. Skeletal maturation in children with MO therefore differs from their peers. CONCLUSION: In this study, the skeletal age in younger children with MO is lower than their calendar age. For adolescents, particularly boys, this is reversed, suggesting an earlier or faster closure of the growth plates. These findings support a systemic influence of the gene defect on growth rate.

Long-term follow-up of Sprengel's deformity treated with the Woodward procedure.

BACKGROUND: Sprengel's deformity is a congenital anomaly of the shoulder with superior displacement and rotation of the hypoplastic scapula. The functional impairment and misshapen appearance of the shoulder can be minimized with different surgical techniques. The aim of this study was to evaluate the long-term results after correction of Sprengel's deformity using the Woodward procedure. METHODS: In this retrospective study, 8 shoulders were examined at 3 different periods in time. The average follow-up was 14.7 years (range, 8-26). Range of motion, Constant score, DASH (Disabilities of the Arm, Shoulder and Hand) score, and simple shoulder test (SST) were obtained to evaluate shoulder function. Scapula placement and degenerative disease were assessed by radiographic examination and the Rigault's classification. Cavendish grading was used to evaluate cosmetic appearance. RESULTS: Mean age at the time of surgery had been 8 years and 9 months. Mean follow-up was 13.5 years. Abduction improved by 41° in the first year after surgery, with final improvement of 56° at long-term follow-up. At the latest follow-up evaluation, the mean Constant score was 85 points, the DASH score 14.59 points, and the SST 9.5 points. Radiographs showed superior displacement of the involved scapula in all cases, with no signs of degenerative disease of the shoulder. Cavendish grade improved from grade 3 preoperatively to grade 1 or 2 at the latest follow-up examination. No long-term complications had occurred. CONCLUSION: The Woodward procedure shows to be an effective surgical procedure to improve shoulder function as well as cosmetic appearance in patients with Sprengel's deformity.

Nonoperative treatment for acute scaphoid fractures: a systematic review and meta-analysis of randomized controlled trials.

BACKGROUND: Recommendations for cast immobilization of acute scaphoid fractures vary substantially. We reviewed data from randomized controlled trials comparing nonoperative treatment methods for acute scaphoid fractures to determine the best available evidence. METHODS: A systematic search of the medical literature from 1966 to 2010 was performed. Two authors independently screened titles and abstracts, reviewed articles, assessed methodological quality according to the Grading of Recommendations Assessment Development and Evaluation system, and extracted data. The primary outcome parameter was nonunion. Data were pooled using random-effects models with standard mean differences for continuous and risk ratios for dichotomous variables, respectively. Heterogeneity across studies was assessed with calculation of the I statistic. RESULTS: The search resulted in five potentially eligible trials of which four met our inclusion criteria. In total, 523 patients were included in four trials including two evaluating below-elbow casting versus above-elbow casting; one trial comparing below-elbow casting including the thumb versus excluding the thumb; and one trial comparing fractures with a below-elbow cast with the wrist in 20-degrees flexion to 20-degrees extension, with both types excluding the thumb. There were no significant differences in union rate, pain, grip strength, time to union, or osteonecrosis for the various nonoperative treatment methods. CONCLUSIONS: There is no evidence from randomized controlled trials on physician-based or patient-based outcome to favor any nonoperative treatment method for acute scaphoid fractures.

A hinged external fixator for complex elbow dislocations: a multicenter prospective cohort study.

BACKGROUND: Elbow dislocations can be classified as simple or complex. Simple dislocations are characterized by the absence of fractures, while complex dislocations are associated with fractures of the radial head, olecranon, or coronoid process. The majority of patients with these complex dislocations are treated with open reduction and internal fixation (ORIF), or arthroplasty in case of a non-reconstructable radial head fracture. If the elbow joint remains unstable after fracture fixation, a hinged elbow fixator can be applied. The fixator provides stability to the elbow joint, and allows for early mobilization. The latter may be important for preventing stiffness of the joint. The aim of this study is to determine the effect of early mobilization with a hinged external elbow fixator on clinical outcome in patients with complex elbow dislocations with residual instability following fracture fixation. METHODS/DESIGN: The design of the study will be a multicenter prospective cohort study of 30 patients who have sustained a complex elbow dislocation and are treated with a hinged elbow fixator following fracture fixation because of residual instability. Early active motion exercises within the limits of pain will be started immediately after surgery under supervision of a physical therapist. Outcome will be evaluated at regular intervals over the subsequent 12 months. The primary outcome is the Quick Disabilities of the Arm, Shoulder, and Hand score. The secondary outcome measures are the Mayo Elbow Performance Index, Oxford Elbow Score, pain level at both sides, range of motion of the elbow joint at both sides, radiographic healing of the fractures and formation of periarticular ossifications, rate of secondary interventions and complications, and health-related quality of life (Short-Form 36). DISCUSSION: The outcome of this study will yield quantitative data on the functional outcome in patients with a complex elbow dislocation and who are treated with ORIF and additional stabilization with a hinged elbow fixator. TRIAL REGISTRATION: The trial is registered at the Netherlands Trial Register (NTR1996).

Gait and function in patients with a femoral endoprosthesis after tumor resection: 18 patients evaluated 12 years after surgery.

We performed gait analysis in 18 patients with a femoral endoprosthesis: 12 distal, 3 proximal and 3 total. Follow-up after surgery was mean 12 (0.6-19) years. The gait parameters measured were walking velocity, step length, duration of stance phase and swing phase. Goniometry of the hip, knee and ankle in both legs was determined during free-paced walking. The functional outcome score of the Musculoskeletal Tumor Society (MSTS) and the Ambulation score were also assessed in all patients. The mean free-paced walking velocity was 88% of normal. The step length of the uninvolved leg was longer than that of the involved one. The swing phase of the involved leg was longer than that of the uninvolved leg, and the stance phase of the involved leg was shorter than that of the uninvolved leg. Goniometry showed three abnormal patterns in the involved leg: a stiff knee gait in 10 patients, a flexed knee gait in 6, and an abnormal flexion-extension pattern in the hip in 9. Goniometry of the uninvolved leg was normal. The mean MSTS score was 22 points (72%). This showed a significant positive correlation to the Ambulation score, but no correlation to any of the temporal variables. Our findings indicate that the time of load on the involved leg, whether conscious or not, is reduced. Follow-up studies are needed to evaluate the effects of the asymmetrical gait pattern observed and the abnormal goniometric results on the development of endoprosthesis-related complications.