Genetic skeletal disorders constitute a rare and heterogeneous bone diseases often leading to poor quality of life. Several surgical options are available. The surgeon must deal with specific features (bone deformity, previous procedures, abnormal bone quality, stiffness or instability, muscle weakness). The questions concern the feasibility of the procedures and the surgical strategy. 55 patients (26 W, 29 M) were reviewed between 2016 and 2022. The mean age of the patients was 35 years (17–71). The diagnosis included 9 hereditary multiple exostoses, 8 osteogenesis imperfecta, 6 multiple epiphyseal dysplasia congenita, 6 achondroplasia, 4 osteopetrosis, 3 pycnodysostosis, 3 hypophosphatemic rickets, 3 fibrous dysplasia, 2 mucopolysaccharidosis, and 10 miscellaneous. 25 patients were referred for hip problems (40 hips). 4 patients (7 hips) requiring a THA have not been operated (4 planned). 4 patients (6 hips) had a proximal femoral fixation (2 osteotomies, 4 fracture fixations). 17 patients (27 hips) sustained a THA (25 primary, 2 revisions). All of them were operated by one operator, using a posterolateral approach and standard implants (including 7 dysplastic and 2 short stems). No customized implant has been used. As regard the 27 THAs, the mean follow up was 4.2 years (1–12). The early complications included 2 femoral cracks and 1 femur fracture. There were 2 revisions (1 cup loosening at 2 years, 1 stem loosening at 4 years). No infection nor dislocation occurred. All the patients were satisfied with their treatment and regain some autonomy. 3 THA were considered as unfeasible. Constitutional bone diseases need a multidisciplinary program of care. The indication for surgery is based on a mutual trust patient/surgeon, a careful evaluation of benefits/risks, and an accurate imaging to anticipate the difficulties. The expected results are a better function and quality of life, and a stability over time

The hip joint is commonly involved in multiple epiphyseal dysplasia and patients may require total hip replacement before the age of 30 years.

We retrospectively reviewed nine patients (16 hips) from four families. The diagnosis of multiple epiphyseal dysplasia was based on a family history, genetic counselling, clinical features and radiological findings. The mean age at surgery was 32 years (17 to 63), with a mean follow-up of 15.9 years (5.5 to 24).

Of the 16 hips, ten required revision at a mean of 12.5 years (5 to 15) consisting of complete revision of the acetabular component in three hips and isolated exchange of the liner in seven. No femoral component has loosened or required revision during the period of follow-up.

With revision for any reason, the 15-year survival was only 11.4% (95% confidence interval 1.4 to 21.4). However, when considering revision of the acetabular shell in isolation the survival at ten years was 93.7% (95% confidence interval 87.7 to 99.7), reducing to 76.7% (95% confidence interval 87.7 to 98.7) at 15 and 20 years, respectively.