Total hip replacement in identical twins with Scheie Mucopolysaccharidosis

Abstract

Scheie Syndrome is an autosomal recessive metabolic storage disease with distinct skeletal findings known as “dysostosis multiplex”. This paper reports on identical twins with Scheie Syndrome who required total hip arthroplasty for early osteoarthritis of the hip. The surgical approach and exposure was particularly demanding due to the stiffness imposed by the soft tissues arising from infiltration of glycosaminoglycans. The small femoral head and acetabulum limited the diameter of the acetabular component and subsequently the prosthetic head diameter that could be safely utilized without compromising the thickness of the polyethylene liner. Twenty-two millimeter heads with a standard polyethylene insert were therefore utilized in the two arthroplasties performed in 1998 in the first twin, and a 28 mm head with a highly cross linked polyethylene liner, in the arthroplasty performed on the second twin in 2003. This is a relevant issue considering that these patients required an arthroplasty at a young age. Hip arthroplasty relieved pain, improved ambulation and provided a better range of motion in our two patients. There were no peri-operative complications. Long-term survivorship of total hip replacement in this population is not yet known.