Abstract
The spondylometaphyseal dysplasias constitute a very complex group of disorders. In addition to the Kozlowski type, three subgroups can be distinguished by the appearance of the femoral neck. In the first group (A) the changes are severe with absent ossification of the femoral neck and coxa vara. In the second group (B) the changes of the femoral neck are moderate and in the third (C) mild metaphyseal irregularities are only visible. This classification is not definitive but tries to put order in this confusing section of constitutional bone diseases.
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Borochowitz Z, Berant M, Kristal H (1988) Spondylometaphyseal dyslasia: further heterogeneity. Skeletal Radiol 17: 181
Horton WA, Langer LO, Collins DL, Dwyer C (1983) Brachyolmia, recissive type (Hobaek): a clinical, radiographic, and histochemical study. Am J Med Genet 16: 201
Kozlowski K, Maroteaux P, Spranger J (1967) La dysostose spondylo-métaphysaire. Presse Méd 54: 2769
Kozlowski K, Barylak A, Middleton RW, Rybakowa M, Thomas P, Walecki J (1976) Spondylo-metaphyseal dysplasias (report of a case of common type and three pairs of siblings of new varieties). Aust Radiol 20: 154
Kozlowski K, Prokop BE, Scougall JS, Silink M, Vines RH (1979) Spondylo-metaphyseal dysplasia. Fortschr Röntgenstr 130: 222
Kozlowski K, Bacha L, Massen R, Ayati M, Sator S, Brahimi L (1988) A new type of spondylometaphyseal dysplasia—Algerian type. Report of five cases. Pediatr Radiol 18: 221
Langer LO Jr, Brill PW, Ozonoff MB, Pauli RM, Wilson WG, Alford BA, Pavlov H, Drake DG (1990) Spondylometaphyseal dysplasia, corner fracture type: a heritable condition associated with coxa vara. Radiology 175: 761
Maroteaux P, Wiedemann HR, Spranger J, Kozlowski K, Lenz L (1969) Essai de classifiaction des dysplasies spondyloépiphysaires. SIMEP, Lyon, p 35
Schmidt BJ, Beçak W, Beçak ML, Soibelman I, Da Silva Queiroz A, Lorga AP, Secaf F, Antonio CF, de Andrade Carvalho A (1963) Metaphyseal dysostosis. J Pediatr 63: 106
Sutcliffe J (1965) Metaphyseal dysostosis. Ann Radiol 9: 215
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Maroteaux, P., Spranger, J. The spondylometaphyseal dysplasias. A tentative classification. Pediatr Radiol 21, 293–297 (1991). https://doi.org/10.1007/BF02018629
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DOI: https://doi.org/10.1007/BF02018629