Total hip arthroplasty in adolescents with severe hip arthropathy and dysplasia associated with camptodactyly-arthropathy-coxa vara-pericarditis syndrome

Abstract

Camptodactyly-arthropathy-coxa vara-pericarditis (CACP) syndrome is a rare disorder. Patients with this syndrome experience early symptomatic arthropathy of the hips. We report a case of adolescent siblings with bilateral arthropathy associated with CACP syndrome in which total hip arthroplasty was performed as treatment of severe associated disability. Postoperative Harris Hip Scores for patient 1 were 86 for the right at 18 months and 96 for the left at 12 months. Postoperative Harris Hip Score at 6 months for patient 2 was 53; however, he had good range of motion and lacked deformity. Based on our limited experience and the limited available clinical data, we feel that total hip arthroplasty is a reasonable treatment option for adolescents with debilitating hip arthropathy associated with CACP syndrome.