Total Hip Arthroplasty in Dyggve-Melchior-Clausen Syndrome: Literature Review and Case Report

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.

Keywords: Case report; Dyggve-Melchior-Clausen; Femoral osteotomy; Total hip arthroplasty.

Figure 1

Bilateral lower extremity preoperative radiograph before left total hip arthroplasty. Full-length standing radiograph displays bilateral hip dysplasia with valgus knees, left worse than right, as well as bilateral degenerative changes of the knees. Right distal femur had cementing from prior surgery.

Figure 2

Bilateral hip preoperative radiograph with a demonstration of right femoral screw. Imaging of the pelvis redemonstrates bilateral hip dysplasia and lacy pelvis.

Figure 3

PA of left femur shows a necrotic, degenerative femoral head.

Figure 4

Postoperative left total hip arthroplasty that demonstrates intact hardware. Nonhardware artifacts (zipper and coin) are seen in the center.

Figure 5

Advanced degenerative changes of the femoral head and acetabulum. Osteonecrotic right femoral head secondary to prior femoral osteotomy, femoral head deformation with subluxation and collapse. Significant superiolateral bone loss in the right acetabulum.

Figure 6

AP imaging of hips demonstrating intact bilateral total hip arthroplasty without hardware failure. AP, anterior-posterior.