title: 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.
authors.affiliation: Department of Orthopaedic Surgery, University of Michigan, Ann Arbor, MI, USA.
authors.forename: Joshua M
authors.initials: JM
authors.lastname: Murphy
authors.forename: Kelly L
authors.initials: KL
authors.lastname: Vanderhave
authors.forename: Andrew G
authors.initials: AG
authors.lastname: Urquhart
mesh_terms: D000293:Adolescent; D001177:Arthropathy, Neurogenic / Q000150:complications*; D019644:Arthroplasty, Replacement, Hip; D060905:Coxa Vara / Q000150:complications*; D005260:Female; D006228:Hand Deformities, Congenital / Q000150:complications*; D006621:Hip Joint / Q000601:surgery*; D006801:Humans; D007592:Joint Diseases / Q000209:etiology* / Q000601:surgery*; D008297:Male; D012720:Severity of Illness Index; D013585:Synovitis / Q000150:complications*