Ilizarov technique in an adolescent patient with progressive pseudorheumatoid dysplasia: A case report. Issue 31 (August 2018)

• Record Type: Journal Article
• Authors: Xiao, Ke; Li, Tao; Jiang, Yaping; Li, Zheng; Zhu, Qiankun; Wu, Zhihong; Weng, Xisheng
• Other Names: NA. section editor.
• Is Part Of: Medicine. Volume 97:Issue 31(2018)
• Publication Date: 2018-08
• Subjects: Ilizarov technique -- joint deformity -- progressive pseudorheumatoid dysplasia; Medicine -- Periodicals; Medicine -- Periodicals; Médecine -- Périodiques; Geneeskunde; Medicine; Periodicals; Periodicals; 610.5
• DOI: 10.1097/MD.0000000000011375
• Languages: English
• ISSNs: 0025-7974

Abstract

Abstract: Rationale: Progressive pseudorheumatoid dysplasia (PPD) is a rare autosomal recessive inherited disease that causes severe systemic joint deformity and articular dysfunction in young patients. Patient concerns: Ilizarov technique treatment in PPD patients has never been reported before. Diagnoses: 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. Interventions: Taking his young age into consideration, the Ilizarov external fixation technique was adopted for the treatment of the deformity in knees. Outcomes: One year after the operation, the improvement of joint deformity was satisfactory. Lessons: 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.