Abstract
Dyggve-Melchior-Clausen (DMC) disease is a rare spondyloepiphyseal autosomal recessive disorder characterized by skeletal dysplasia and intellectual disability. Hip arthritis, often secondary to hip dysplasia, presents at an early age. Current literature suggests that osteotomies do not benefit DMC syndrome-associated hip disease but reports of total hip arthroplasty in these patients are lacking. We present a case of bilateral hip replacement in a 31-year-old patient with DMC syndrome. After planning the operation with the use of computed tomography, we chose to use a small-dimension porous cup along with an appropriately sized version control stem in order to address the unique acetabular and femoral deformities. In conclusion, we consider total hip replacement in DMC syndrome to be safe and effective in addressing a challenging hip pathology.