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INTRODUCTION: Fibrous dysplasia (FD) of bone can be present with pain, deformity and pathological fractures. Management is both medical and surgical. Little literature exists on the surgical management of both monostotic and polyostotic FD. We present our experience of limb reconstruction surgery in this pathological group of bone disease. MATERIALS AND METHODS: A retrospective cohort of children who underwent limb reconstruction surgery at a single high-volume paediatric centre was identified from a prospective database. Case notes and radiographs were reviewed. Surgical techniques, outcomes and difficulties were explored. RESULTS: Twenty-one patients were identified aged between 7 and 13 at presentation to the limb reconstruction unit. Eleven were female, nine had McCune-Albright syndrome, seven had polyostotic FD and five had monostotic. Proximal femoral varus procurvatum deformity was the most common site requiring surgical intervention. The distal femur, tibia, humerus and forearm were also treated.Methods include deformity correction with intramedullary fixation including endo-exo-endo techniques, elastic nailing, guided growth, circular fixator technique and fixator-assisted plating. Correction of deformity and leg length discrepancies was common.The osteotomies went on to heal with no nonunions or delayed healing. We encountered secondary deformity at distal end of nails as the children grew as expected. These were managed with revision nailing techniques and in some cases external fixation. There was one implant failure, which did not require revision surgery. CONCLUSION: The surgical management of pathological bone disease is challenging. Corrective osteotomies with intramedullary fixation can be very successful if appropriate limb reconstruction principles are adhered to. Deformity correction, guided growth and lengthening can all be successfully achieved in bone affected by FD. Polyostotic FD can be present with secondary deformities, and these can be difficult to manage. HOW TO CITE THIS ARTICLE: Hampton MJ, Weston-Simmons S, Giles SN, et al. Deformity Correction, Surgical Stabilisation and Limb Length Equalisation in Patients with Fibrous Dysplasia: A 20-year Experience. Strategies Trauma Limb Reconstr 2021;16(1):41-45.
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The pilot of a light aircraft that crashed after a loss of power was found to have ethanol in the vitreous and the blood, but almost none in the urine. The globes of the eyes were intact, and the body was refrigerated after recovery until the autopsy was performed the following morning. The pilot was described as a "nondrinker," and additional specialized toxicology testing results were inconsistent with ethanol ingestion. The pilot's body was extensively exposed to fuel during the prolonged extraction. Investigation determined that the aircraft had been fueled with gasoline that contained 10% ethanol. Although exposure to automotive fuel has not been previously described as a source of ethanol in postmortem specimens, it may represent a source for the ethanol detected during postmortem toxicology testing in this case, and this finding may be relevant to other cases with similar exposure.
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Acidentes Aeronáuticos , Etanol/análise , Gasolina , Absorção Cutânea , Solventes/análise , Queimaduras Químicas/patologia , Exposição Ambiental/análise , Etanol/farmacocinética , Humanos , Masculino , Traumatismo Múltiplo/patologia , Solventes/farmacocinética , Corpo Vítreo/químicaRESUMO
Undiagnosed neoplasms in childhood are rare causes of sudden and unexpected death. Deaths due to undiagnosed hematologic malignancies are limited to a small number of case reports. The following case of acute leukemia was diagnosed at forensic autopsy in a 5-year-old boy with no significant past medical history. He complained of nausea and vomiting 2 days before his death, with the subsequent development of fever. Meningitis was the initial suspected cause of death. Findings at autopsy included a 100% cellular bone marrow with greater than 95% blasts. Hemorrhages involving the cerebrum, pons, epicardium, lungs, and thymus were present. Prominent leukemic infiltrates and leukostasis were present in the brain, heart, lungs, spleen, hilar lymph nodes, liver, and kidneys. A peripheral blood smear and automated blood cell count showed a white blood cell count of 435 × 10/L with greater than 80% circulating blasts. Immunohistochemical stains confirmed the diagnosis of T lymphoblastic leukemia/lymphoma. Given these circumstances, the diagnosis of acute leukemia should be considered when an intracerebral hemorrhage and/or visceral hemorrhages are identified on internal examination for appropriate collection of tissue for smears and microscopic examination. This case also highlights the uncommon, although serious, risks associated with acute lymphoblastic leukemia and hyperleukocytosis.
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Morte Súbita/etiologia , Leucemia-Linfoma Linfoblástico de Células T Precursoras/diagnóstico , Medula Óssea/patologia , Hemorragia Cerebral/patologia , Pré-Escolar , Patologia Legal , Hemorragia/patologia , Humanos , Leucocitose/patologia , Pulmão/patologia , Masculino , Pericárdio/patologia , Ponte/patologia , Timo/patologiaRESUMO
Methodology is adequate to reduce substantially the prevalence of diarrhea and associated infant mortality in areas of high endemicity. The success of such programs depends primarily upon managerial and technical resources. In areas of moderate endemicity where Shigella persists and sanitation facilities are adequate, further reductions in diarrheal diseases probably can be achieved through improvement in personal hygienic practices. In some instances the nature of misuse of facilities must be determined to develop or improve techniques for control. Where diarrheal diseases and associated infant mortality have been reduced substantially, the precise etiologic significance of pathogens, such as the enterpathogenic strains of Escherichia coli, associated with cases of diarrhea should be determined. The epidemiology of organisms incriminated as causal agents of diarrhea should be explored to the extent necessary for development of control measures. Much additional exploratory work is needed to assess adequately the role of viruses in diarrhea of both infants and adults and to develop control measures
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Diarreia/prevenção & controle , Diarreia/etiologia , Programas de Nutrição , Estados Unidos , Shigella , Monitoramento EpidemiológicoRESUMO
Methodology is adequate to reduce substantially the prevalence of diarrhea and associated infant mortality in areas of high endemicity. The success of such programs depends primarily upon managerial and technical resources. In areas of moderate endemicity where Shigella persists and sanitation facilities are adequate, further reductions in diarrheal diseases probably can be achieved through improvement in personal hygienic practices. In some instances the nature of misuse of facilities must be determined to develop or improve techniques for control. Where diarrheal diseases and associated infant mortality have been reduced substantially, the precise etiologic significance of pathogens, such as the enterpathogenic strains of Escherichia coli, associated with cases of diarrhea should be determined. The epidemiology of organisms incriminated as causal agents of diarrhea should be explored to the extent necessary for development of control measures. Much additional exploratory work is needed to assess adequately the role of viruses in diarrhea of both infants and adults and to develop control measures
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Diarreia , Estados Unidos , Shigella , Monitoramento Epidemiológico , Programas de NutriçãoRESUMO
WHO Bull 3, 1950 (English)