During the past 25 years, botulinum toxin type A (BoNT-A) has become the most widely used medical intervention in children with cerebral palsy. In this review we consider the gaps in our knowledge in the use of BoNT-A and reasons why muscle morphology and function in children with cerebral palsy are impaired. We review limitations in our knowledge regarding the mechanisms underlying the development of contractures and the difficulty in preventing them. It is clear from this review that injection of BoNT-A in the large muscles of both the upper and lower limbs of children with cerebral palsy will result in a predictable decrease in muscle activity, which is usually reported as a reduction in spasticity, for between 3 and 6 months. These changes are noted by the use of clinical tools such as the Modified Ashworth Scale and the Modified Tardieu Scale. Decreased muscle over-activity usually results in improved range of motion in distal joints. Injection of the gastrocnemius muscle for toe-walking in a child with hemiplegia or diplegia usually has the effect of increasing the passive range of dorsiflexion at the ankle. In our review, we found that this may result in a measurable improvement in gait by the use of observational gait scales or gait analysis, in some children. However, improvements in gait function are not always achieved and are small in magnitude and short lived. We found that some of the differences in outcomes in clinical trials may relate to the use of adjunctive interventions such as serial casting, orthoses, night splints and intensive therapy. We note that the majority of clinical trials of the use of BoNT-A in children with cerebral palsy have focussed on a single injection cycle and this is insufficient to understand the balance between benefit and harm. Most outcomes were reported in terms of changes in muscle tone and there were fewer studies with robust methodology that reported improvements in function. Changes in the domains of activities and participation have rarely been reported in studies to date. There were no clinical reviews to date that consider the findings of studies in human volunteers and in experimental animals and their relevance to clinical protocols. In this review we found that studies in human volunteers and in experimental animals show muscle atrophy after an injection of BoNT-A for at least 12 months. Muscle atrophy was accompanied by loss of contractile elements in muscle and replacement with fat and connective tissue. It is not currently known if these changes, mediated at a molecular level, are reversible. We conclude that there is a need to revise clinical protocols by using BoNT-A more thoughtfully, less frequently and with greatly enhanced monitoring of the effects on injected muscle for both short-term and long-term benefits and harms.
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Purpose This systematic review aims to elucidate a non-operative rehabilitation program that optimizes recovery based on published approaches and outcomes. Methods Searches of four databases from inception to 1 January 2020 were performed to identify clinical studies addressing the non-operative management of simple elbow dislocations. Results Of 2435 studies that were eligible for title screen, 15 studies satisfied inclusion criteria. Three randomized control studies demonstrated that early mobilization expedited the return of range of motion, function and return to work or activities, however, resulted in increased pain within the six-week rehabilitation period compared to Plaster of Paris casting for 21 days. Patients returned to work sooner after early mobilization (10 vs. 18 days; p = 0.02) compared to Plaster of Paris casting. In all studies, early mobilization resulted in similar re-dislocation rates of 1.3% (3/237) versus 2.2% (12/549) in those with Plaster of Paris casting as well as lower incidence of heterotopic ossification (36% vs. 54%). No significant differences between rehabilitation protocols were determined; however, the large majority of recent papers utilized rehabilitation protocols. Conclusion Early mobilization of simple elbow dislocations results in early return of Range-of-Motion, function and return to work with no increase in complication rates; however, increased pain during the rehabilitation period.
The surgical management of benign and benign aggressive bone tumors typically involves intralesional curettage and reconstruction of the resulting defect with cement or bone graft material. At the authors' institution, an injectable synthetic calcium sulfate–calcium phosphate composite is now the standard graft material for these cases. This study reports the long-term follow-up, specifically the stability of bone regeneration, for the use of the synthetic graft material for oncologic reconstruction. Fourteen patients who underwent intralesional curettage of a primary bone tumor followed by cavitary reconstruction with synthetic graft material who had at least 4-year follow-up were identified from an institutional orthopedic oncology database. Clinical outcome data, focusing on long-term clinical and radiographic features of the reconstruction, were extracted from electronic and paper medical records. Seven females and 7 males were included (mean age at surgery, 28.1 years; range, 13–64 years). Follow-up ranged from 50 to 105 months (mean, 68 months). Most surgical reconstructions were done for the lower limb (n=11), and giant cell tumor of bone was the most common tumor treated. The mean amount of synthetic graft material used was 18.6 cm 3 . Complete radiographic resorption and new bone incorporation was observed within the first year, and bone remodeling was complete in all patients. Bone remodeling remained stable throughout the longer-term follow-up (ie, up to 9 years). The use of an injectable synthetic calcium sulfate–calcium phosphate composite is a viable option in the reconstruction of cavitary bone defects following intralesional curettage of primary benign bone tumors. This reconstruction technique was safe, with no long-term complications, and led to complete radiographic resorption and new bone incorporation with long-lasting stability. [ Orthopedics . 2018; 41(6):e868–e875.]
With solid tumor cancer survivorship increasing, the number of patients requiring post-treatment surveillance also continues to increase. This highlights the need for evidence-based cancer surveillance guidelines. Ideally, these guidelines would be based on combined high-quality data from randomized controlled trials (RCTs). We present a systematic review of published cancer surveillance RCTs in which we sought to determine the feasibility of data pooling for guideline development. We carried out a systematic search of medical databases for RCTs in which adult patients with solid tumors that had undergone surgical resection with curative intent and had no metastatic disease at presentation, were randomized to different surveillance regimens that assessed effectiveness on overall survival (OS). We extracted study characteristics and primary and secondary outcomes, and assessed risk of bias and validity of evidence with standardized checklist tools. Our search yielded 32,216 articles for review and 18 distinct RCTs were included in the systematic review. The 18 trials resulted in 23 comparisons of surveillance regimens. There was a highlevel of variation between RCTs, including the study populations evaluated, interventions assessed and follow-up periods for the primary outcome. Most studies evaluated colorectal cancer patients (11/18, [61%]). The risk of bias and validity of evidence were variable and inconsistent across studies. This review demonstrated that there is tremendous heterogeneity among RCTs that evaluate effectiveness of different postoperative surveillance regimens in cancer patients, rendering the consolidation of data to inform high-quality cancer surveillance guidelines unfeasible. Future RCTs in the field should focus on consistent methodology and primary outcome definition.
Three cases of extraskeletal myxoid chondrosarcoma (EMC) in patients who presented with pulmonary metastases and were managed with long-term close observation without systemic intervention are presented. Follow-up imaging showed slow progression of their disease over several years, and the patients remained asymptomatic from their pulmonary metastases. This clinical experience provides insight into the natural history of the disease and suggests that some patients may experience long-term survival and remain asymptomatic even without systemic intervention, thereby improving their quality of life by avoiding potentially debilitating treatments.
Introduction. Mazabraud’s Syndrome is a rare condition that is defined by the presence of fibrous dysplasia lesions in the bone and intramuscular myxomas in the soft tissue. Malignant transformation, in the setting of Mazabraud’s Syndrome, of the fibrous dysplasia lesions into a sarcomatous neoplasm occurs in less than 1% of cases—with only six previously reported cases. Case Presentation. Here, we present a 62-year-old Caucasian female who developed an osteosarcoma in a fibrous dysplasia lesion of the proximal femur in the background of Mazabraud’s Syndrome. The patient was treated with wide excision and endoprosthetic reconstruction. She declined adjuvant chemotherapy. She is alive without evidence of disease one-year postoperatively. Conclusion. Patients with Mazabraud’s Syndrome remain at low risk for malignant transformation. However, close monitoring of asymptomatic patients with this condition for radiographic changes in their lesions and/or clinical symptoms is recommended.
Background: Pancytopenia is a Clinico-hematological entity, characterized by a triad of Anemia, leucopenia, and thrombocytopenia. The criterion for defining Pancytopenia is HGB < 9 g/dl: ANC < 1.5 x 109/L and platelet count <100 x 109/L. The main objective of this study is to categorize pediatric pancytopenia’s on etiological basis and to study the age wise incidence. Methodology and Results: This is a 5-year study (2016-2021) conducted in tertiary care pediatric referral hospital. All data were expressed as Mean +/ - SD and p value <0.05 is taken as statistically significant. A total of 500 bone marrow smears of pancytopenic patients were studied, of which 60% were boys & 40% girls, 64% constituted Malignant diseases and 36% were benign. Incidence wise, ALL is the most common and pancytopenia being 2nd and together comprises 64% of total cases. Some rare cases like AML –M6 in 2 months old, Chediak higashi syndrome and Familial Hemophagocytic syndrome were noted. Flow cytometry and Molecular genetics were done were ever required. Conclusion: Bone Marrow Aspiration is a reliable and sensitive test which can be used in investigation of Pediatric pancytopenia’s and if used meticulously with other routine hematological tests can give a high diagnostic accuracy. ALL is most common in our study (other studies showed Aplastic anemia), AML-M6 in 2 months, LD bodies in bone marrow, HPS were amongst the rare cases
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