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Aim Acetabular dysplasia is the one of main causes of hip displacement in patients with cerebral palsy (CP). Although several studies have shown a relationship between hip displacement and acetabular dysplasia, relatively few have evaluated the association between quantitative acetabular dysplasia and related factors, such as Gross Motor Function Classification System (GMFCS) level. Method We performed a morphometric analysis of the acetabulum in patients with CP using multiplanar reformation of computed tomography data. The three directional acetabular indices (anterosuperior, superolateral, and posterosuperior) were used to evaluate acetabular dysplasia. Consequently, linear mixed-effects models were used to adjust for related factors such as age, sex, GMFCS level, and migration percentage. Results A total of 176 patients (mean age 9y 5mo, range 2y 4mo–19y 6mo; 104 males, 72 females) with CP and 55 typically developing individuals (mean age 13y 6mo, range 2y 5mo–19y 10mo; 37 males, 18 females) in a comparison group were enrolled in this study. Statistical modelling showed that all three directional acetabular indices independently increased with GMFCS level (p

The pathogenic mechanism responsible for congenital pseudarthrosis of the tibia (CPT) is not well understood although the possibility of double inactivation of the neurofibromatosis type 1 (Nf1) gene has been suggested. In the present study, loss of heterozygosity was investigated in fibrous hamartoma tissues harvested from 16 patients with CPT associated with NF1 using four genetic markers that span the Nf1 gene. Based on the assumption that a single cell with double inactivation of Nf1 would undergo clonal growth and cause fibrous hamartoma, we investigated clonality in fibrous hamartoma tissues by analyzing X-chromosome inactivation patterns in 11 female patients. Loss of Nf1 heterozygosity in fibrous hamartoma tissues was observed at one or two genetic markers in 4 out of the 16 patients tested. In clonality assays, 3 of 11 patients showed a clonal growth pattern, 5 a non-clonal pattern, and 3 were non-informative. These findings support that double inactivation of the Nf1 gene and subsequent clonal growth could be a pathogenic feature of the fibrous hamartoma tissue at least in some of the CPT but might not be essential requirements of CPT development. © 2012 Orthopaedic Research Society. Published by Wiley Periodicals, Inc. J Orthop Res 30:1535–1540, 2012

Aim This study was undertaken to determine the need for concurrent prophylactic femoral varization osteotomy (FVO) of contralateral stable hips at the time of hip reconstructive surgery on unstable hips in non-ambulant individuals with cerebral palsy (Gross Motor Function Classification System levels IV and V). Method A decision analysis model was constructed that included (1) the probability of unstable hips during observation of the contralateral stable hips, (2) unstable hip rate (subluxation or dislocation rate) after prophylactic FVO or after delayed reconstructive surgery (including FVO) for unstable hips, and (3) complication rates after concurrent prophylactic FVO or after hip reconstructive surgery. The final outcome score was based on pain utility measures. The probabilities of all cases and the utility score were obtained by literature review. Results The decision model favoured concurrent prophylactic FVO for the contralateral stable hips over observation (pain utility measure scores 0.814 vs 0.781). In a one-way sensitivity analysis, the decision model favoured concurrent prophylactic FVO when the unstable hip rate of contralateral stable hips during observation was 27% or over. Concurrent prophylactic surgery also demonstrated higher utility scores than observation when the unstable hip rate after FVO was between 0% and 29%. Interpretation The decision analysis model demonstrated that concurrent prophylactic FVO for a contralateral stable hip in individuals with cerebral palsy undergoing hip reconstructive surgery was beneficial from a medical perspective, which was based on current evidence.

This scanning electron microscopic study examined the spatial and temporal features of proliferating vessels of regenerating bone tissue and blood supply during distraction osteogenesis. A rat model of tibial lengthening was used with a protocol divided into a latency period of 7 days, a distraction period that lasted 14 days with a daily distraction rate of 0.5 mm in two steps, and a consolidation period of 21 days. Vascular casting was done on the hindlimbs before osteotomy and on postoperative days 7, 14, 21, 28, and 42. Scanning electron microscopic findings were correlated with radiological and histological observations. On days 7 and 14, the proliferation of periosteal vessels was pronounced and there was distinct subperiosteal bone formation on the osteotomized surfaces. On day 21, vascular branches from the medullary canal of the host bone formed a vascular network, which gave rise to multiple axial, straight vascular branches, running parallel to the direction of distraction, toward the interzone, in accordance with the progress of mineralization. On day 28, the periosteum provided vascularization to the peripheral side of the interzone whereas the center of the interzone was still relatively avascular. On day 42, the periosteal and medullary vascular channels were completely connected at the distraction site including the interzone, which was occupied by developing and mature bone trabeculae. These results suggest that vascular proliferation occurs actively during the latency and distraction periods and then gradually decreases over time. A close temporal and spatial relationship exists between formation of regenerated bone and vascular proliferation of the periosteum and medullary canal.

Clinicopathological features in six cases of focal fibrocartilaginous dysplasia (FFCD) which involved either the tibia (n = 4) or the femur (n = 2) were reviewed. All cases presented clinical and radiological characteristic features, and histopathological findings were analyzed in five of the six cases. The subject group comprised three boys and three girls, ages ranged from 12 to 18 months. Histologically, the individual lesions showed regional variation in cellularity, amount of fibrous and cartilaginous components. Paucicellular areas were mainly composed of dense fibrous tissue while more cellular areas contained foci of fibrocartilaginous element. The chondrocytes and stellate cells around cartilaginous area were positive for S-100 protein. One case contained both hyaline and fibrocartilage, and architecturally mimicked normal tendinous insertion. One case, which involved proximal tibia, was purely composed of fibrous tissue without fibrocartilage. All cases formed undulating and irregular borders against underlying cortical bone. Histopathologically variable spectrum suggests a strong possibility of undergoing transition from initial cellular and cartilagnous to late paucicellular, fibrous phase. Although any evidence that can explain basic pathogenesis or prognostic histological parameter is lacking, we believe that the term FFCD is not relevant because the presence of fibrocartilage is not an essential feature, and it can cause confusion with other pathological processes. We propose the term 'subperiosteal fibrocartilaginous pseudotumor of long bone' for this unique clinicopathological entity with which heterologous cartilaginous element can be associated.