Smith-McCort dysplasia (SMC) is a rare autosomal recessive osteochondrodysplasia characterized by short limbs and trunk with barrel-shaped chest. The radiographic phenotype includes platyspondyly, generalized abnormalities of the epiphyses and metaphyses, and a distinctive lacy appearance of the iliac crest, features identical to those of Dyggve-Melchior-Clausen disease (DMC). Spinal cord compression due to atlantoaxial instability occurs in both SMC and DMC. [From OMIM]
Smith-McCort dysplasia 1 is associated with mutations in DYM gene.