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To test the hypothesis that the reproducibility of radiographic measurements of the first metatarsophalangeal angle and the intermetatarsal angle I-II can be increased by exact guidelines, we calculated the intra- and interobserver reliability of both methods. 4 independent observers (2 senior residents and 2 orthopedic trainees) evaluated 50 pre- and 50 postoperative plain dorsoplantar radiographs with their method of preference and then with Mitchell et al.'s method (1958). The mean intraobserver coefficient of repeatability for the metatarsophalangeal angle improved from 5.9 degrees to 4.2 degrees and for the intermetatarsal angle I-II, from 4.4 degrees to 2.8 degrees. The interobserver coefficient of repeatability improved from 6.5 degrees to 5.0 degrees for the metatarsophalangeal angle, and from 4.9 degrees to 3.6 degrees for the intermetatarsal angle I-II. This improvement in measurement accuracy was more marked for postoperative measurements, due to deformation of the metatarsal after the osteotomy which made it more difficult to find the longitudinal axis of the metatarsal. The improvements in the accuracy of measurements were also greater in the two inexperienced observers, since their measurements differed more when they had no exact guidelines for their drawings.

Background: Roberts syndrome (Pseudothalidomide) is a rare birth defect that causes severe bone malformation complex. The bones of the arms, and in some cases other appendages, may be extremely shortened and even absent. The fingers of the hands may be fused. An extreme case results in the absence of the upper bones of both the arms and legs so that the hands and feet appear attached directly to the body. This is called tetraphocomelia.

Introduction: Expressionless face associated with multiple contractures has been encountered in an infant. There is a wide range of misconception regarding the categorization of children with multiple contractures among different pediatric disciplines. The fundamental element in categorizing children with multiple contractures is "the etiological understanding". In the absence of concomitant neuromuscular disease, however, the search for other reasons is mandatory. Our present paper signifies the necessity of proper interpretations of unusual clinical and radiographic features.

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Background:Thoracolumbar kyphosis has been considered as the first presenting deformity and is often a key diagnostic clue noted in children with mucopolysaccharidosis (MPS) type IV (Morquio's syndrome). However, we observed that the progressive irregularities of the epiphyses of the long bones were the most prominent skeletal pathology, causing effectively the development of diverse forms of lower limbs deformities with extreme variation in age of onset.Materials and Methods:Ten patients (seven children and three adults) with an average age of 15 years have been enrolled in this study. Age of diagnosis of MPS IVA has a variable age of onset and a MISLEADING rate of severity. Hip dislocations, genu valgum, protrusio acetabuli and osteoarthritis were the most common lower limbs deformities in these patients. Clinical and radiographic phenotypes were the baseline tools of documentation. Urinary screening and genotypic characterizations have been applied accordingly.Results:Combined pelvic and femoral procedures for hip dislocation, epiphysiodeses and supracondylar osteotomy for genu valgum and hip arthroplasty for protrusio acetabuli have been performed. All patients manifested insufficient activity of N-acetylgalactosamine-6-sulphate sulphatase, an enzyme that degrades keratin sulphate and chondroitin-6 sulphate.Conclusion:The extensive clinical heterogeneity contributed significantly in the delay in establishing the diagnosis particularly in adult patients with MPS IV. The epiphyseal irregularities of the long bones and the progressive flattening pathology of MPS IV A were the reason to falsely diagnose some patients as spondyloepiphyseal dysplasia congenital and/or tarda. Proximal femoral osteotomy, realignment osteotomy and total hip arthroplasty have been performed for coxa vara, genu valgum and protrusio acetabuli, respectively, in children and adult group of patients. The importance of early diagnosis on MPS IV A is to receive enzyme replacement therapy and plan for other therapeutic measures.

To test the hypothesis that the reproducibility of radiographic measurements of the first metatarsophalangeal angle and the intermetatarsal angle I-II can be increased by exact guidelines, we calculated the intra- and interobserver reliability of both methods. 4 independent observers (2 senior residents and 2 orthopedic trainees) evaluated 50 pre- and 50 postoperative plain dorsoplantar radiographs with their method of preference and then with Mitchell et al.'s method (1958). The mean intraobserver coefficient of repeatability for the metatarsophalangeal angle improved from 5.9 degrees to 4.2 degrees and for the intermetatarsal angle I-II, from 4.4 degrees to 2.8 degrees. The interobserver coefficient of repeatability improved from 6.5 degrees to 5.0 degrees for the metatarsophalangeal angle, and from 4.9 degrees to 3.6 degrees for the intermetatarsal angle I-II. This improvement in measurement accuracy was more marked for postoperative measurements, due to deformation of the metatarsal after the osteotomy which made it more difficult to find the longitudinal axis of the metatarsal. The improvements in the accuracy of measurements were also greater in the two inexperienced observers, since their measurements differed more when they had no exact guidelines for their drawings.

Our results are in accordance with previously published data of smaller series using the Zweymüller/Alloclassic( total hip. The survival rate of this cementless rectangular titanium prosthesis for the reported follow-up time compares favorably with contemporary cemented hip prostheses.