Abstract
The clinical and roentgenographic manifestations of six cases suffering from spondyloepiphyscal dysplasia tarda with progressive arthropathy are presented. The roentgenographic features consist of generalized platyspondyly and varying degrees of epiphyseal involvement with conspicuous enlargement of both ends of the short tubular bones of the hands. This disorder appears at an earlier age, and is more crippling than the usual form of spondyloepiphyseal dysplasia tarda. It is suggested that orthopaedic measures are essential for all patients with this disorder, as profound disability may be expected by progressive involvement of the major joints.
This is a preview of subscription content, log in via an institution to check access.
Similar content being viewed by others
References
Lachman RS, Rimoin DL, Hollister DW, Dorst JP, Siggers DC, McAlister W, Kaufman RL, Langer LO (1975) The Kniest syndrome. AJR 123: 805
Siggers DC, Rimoin DL, Dorst JP, Doty SB, Williams BR, Hollister DW, Silberberg R, Cranley RE, Kaufman RL, McKusick VA (1974) The Kniest syndrome. Birth Defects 10: 193
Spranger J, Langer LO (1974) Spondyloepiphyseal dysplasias. Birth Defects 10: 19
Spranger J, Langer LO, Wiedemann H-R (1974) Bone dysplasias: An atlas of constitutional disorders of skeletal development. Saunders, Philadelphia
Spranger J, Maroteaux P (1974) Kniest disease. Birth Defects 10: 50
Wynne-Davies R, Hall C, Ansell BM (1982) Spondyloepiphyseal dysplasia tarda with progressive arthropathy. A new disorder of autosomal recessive inheritance. J Bone Joint Surg [Br] 64: 442
Author information
Authors and Affiliations
Rights and permissions
About this article
Cite this article
Kaibara, N., Takagishi, K., Katsuki, I. et al. Spondyloepiphyseal dysplasia tarda with progressive arthropathy. Skeletal Radiol 10, 13–16 (1983). https://doi.org/10.1007/BF00355384
Issue Date:
DOI: https://doi.org/10.1007/BF00355384