Ilizarov technique in an adolescent patient with progressive pseudorheumatoid dysplasia: A case report.

ABSTRACT: Progressive pseudorheumatoid dysplasia (PPD) is a rare autosomal recessive inherited disease that causes severe systemic joint deformity and articular dysfunction in young patients.Ilizarov technique treatment in PPD patients has never been reported before.A 17-year-old male patient presented with a 10-year history of polyarthritis and 4-year history of progressive hip and knee pain and stiffness. Genetic testing for the WISP3 gene was done and showed compound heterozygous mutations: NM_198239.1 (WISP3):c.1064_1065dupGT (p.Gln356ValfsTer33) and NM_198239.1 (WISP3):c.643+2T?>?C.Taking his young age into consideration, the Ilizarov external fixation technique was adopted for the treatment of the deformity in knees.One year after the operation, the improvement of joint deformity was satisfactory.The Ilizarov technique is economical and less invasive, and most importantly, it can delay the possible arthroplasty. It gives young PPD patients with arthropathy an alternative treatment.

SUBMITTER: Xiao K 

PROVIDER: S-EPMC6081158 | biostudies-literature | 2018 Aug

REPOSITORIES: biostudies-literature