Management of hip instability in trisomy 21.

ABSTRACT

The unstable hip in Trisomy 21 presents with a spectrum of hip instability with different problems at different ages. What links this multiphase problem, in many patients, is the final common pathway of untreated instability, that of a stiff, dislocated, and often-painful hip, leading to significant functional disability. Historically, the results of treating hip instability in Trisomy 21 were variable with a notable frequency of poor results. With an improved understanding of the Trisomy 21 hip in terms of its pathoanatomy and a more contemporary surgical approach to hip reconstruction, much improved results can be expected and indeed have recently been shown. The mainstay of treatment for the habitual dislocation group presenting before 8 years of age is the femoral varus derotation osteotomy. The older group presenting with painful subluxation often show signs of secondary acetabular dysplasia and thus are best treated with redirectional acetabular osteotomy with or without the use of femoral varus derotation osteotomy. The presence of radiographic features of degenerative arthritis in the fixed dislocation group precludes the use of joint-preserving techniques for hip reconstruction, and these patients can achieve excellent results with total joint arthroplasty. The natural history, historical results, assessment, treatment, and management of complications of hip instability in Trisomy 21 are addressed in this paper.