Open AccessIlizarov technique in an adolescent patient with progressive pseudorheumatoid dysplasia
Author(s) -
Ke Xiao,
Tao Li,
Yaping Jiang,
Zheng Li,
Qiankun Zhu,
Zhihong Wu,
Xisheng Weng
Publication year - 2018
Publication title -
medicine
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.59
H-Index - 148
eISSN - 1536-5964
pISSN - 0025-7974
DOI - 10.1097/md.0000000000011375
Subject(s) - medicine , deformity , ilizarov technique , polyarthritis , surgery , arthritis , arthropathy , compound heterozygosity , kyphoscoliosis , dysplasia , external fixation , osteoarthritis , scoliosis , orthopedic surgery , prosthesis , pathology , biochemistry , chemistry , alternative medicine , gene , allele
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.