Multiple Epiphyseal Dysplasia Patients Research Articles

Prothèse totale de hanche hybride pour dysplasie épiphysaire multiple

BackgroundMultiple ephiphyseal dysplasia (MED) is a rare autosomal dominant skeletal dysplasia that causes deformation of the epiphysis of the involved joints. The hips are invariably affected and symptoms due to incapacitating early onset degenerative hip disease often begin between the second and fourth decade of life. Literature regarding the clinical and radiographical outcomes after total hip arthroplasty in this young population is very scarce. HypothesisIn patients with multiple epiphyseal dysplasia and early onset degenerative hip disease, hybrid total hip arthroplasty is a safe and reliable procedure. Patients and methodsWe followed 10 hybrid total hip arthroplasties in 6 patients with respect to the early and medium term complications. The average age at surgery was 32-years-old (17 to 41). All stems were cemented polished straight tapered stems; all cups were porous coated uncemented cups. The mean duration of follow-up was 10.3 (7–14, SD: 2.8) years. Clinical outcomes were measured using the Charnley modification of the Merle d’Aubigné-Postel grading system and VAS-scores. ResultsNo early complications and no revisions occurred and patients significantly improved for pain, function and mobility. The Charnley, Merle d’Aubigné and Postel hip scores significantly improved from 9.6 points pre-operatively (range, 8–11 points) to 17 points (range, 16–18 points) and the VAS-score significantly improved after surgery from respectively 7 at rest and 8.5 during activity preoperatively to 1 at rest and 1.5 during activity postoperatively. Radiographic evaluation showed no cases of radiolucency around the cemented femoral components. No migration or subsidence of the components was noted. With regard to the acetabular component, osteolysis was noted in 4 hips, but serial radiographs showed no progression or migration of the component and the patients were completely pain free. ConclusionHybrid total hip arthroplasty is a viable treatment option in multiple epiphyseal dysplasia patients, with excellent mid-term clinical and radiographical outcomes. Level of evidenceLevel IV, retrospective study.

Hybrid total hip arthroplasty for multiple epiphyseal dysplasia.

Multiple Ephiphyseal Dysplasia (MED) is a rare autosomal dominant skeletal dysplasia that causes deformation of the epiphysis of the involved joints. The hips are invariably affected and symptoms due to incapacitating early onset degenerative hip disease often begin between the second and fourth decade of life. Literature regarding the clinical and radiographical outcomes after total hip arthroplasty in this young population are very scarce. Hypothesis in patients with multiple epiphyseal dysplasia and early onset degenerative hip disease, hybrid total hip arthroplasty is a safe and reliable procedure. We followed 10 hybrid total hip arthroplasties in 6 patients with respect to the early and medium term complications. The average age at surgery was 32 years old (17 to 41). All stems were cemented polished straight tapered stems, all cups were porous coated uncemented cups. The mean duration of follow-up was 10.3 (7-14, SD2.8) years. Clinical outcomes were measured using the Charnley modification of the Merle d'Aubigné-Postel grading system and VAS-scores. No early complications and no revisions occurred and patients significantly improved for pain, function and mobility. The Charnley, Merle d'Aubigné and Postel hip scores significantly improved from 9.6 points pre-operatively (range: 8-11 points) to 17 points (range: 16-18 points) and the VAS-score significantly improved after surgery from respectively 7 at rest and 8.5 during activity preoperatively to 1 at rest and 1.5 during activity postoperatively. Radiographic evaluation showed no cases of radiolucency around the cemented femoral components. No migration or subsidence of the components was noted. With regard to the acetabular component, osteolysis was noted in 4 hips, but serial radiographs showed no progression or migration of the component and the patients were completely pain free. Hybrid total hip arthroplasty is a viable treatment option in multiple epiphyseal dysplasia patients, with excellent mid-term clinical and radiographical outcomes. Level IV, retrospective study.

The fate of hips that are conservatively treated in multiple epiphyseal dysplasia.

We reviewed the radiologic and clinical outcomes of hip joints affected by multiple epiphyseal dysplasia in 40 patients. The average patient age was 9.6 years. All patients were followed up for an average of 7.2 years. No patient underwent surgical treatment. The variances of the center-edge angle and femoral head coverage had the greatest tendency to increase with conservative treatment and follow-up (P=0.011 and 0.015, respectively). The acetabular angle and the acetabular depth index at the first visit and the latest follow-up were statistically significantly different (P=0.046 and 0.027, respectively). According to the Stulberg classification, the severity of hip deformity became less severe with age, but this was not statistically significant (P=0.090). Larger improvements in Harris hip scores were identified after conservative treatment (P=0.003). Favorable midterm outcomes were obtained for the treatment of hip deformity in multiple epiphyseal dysplasia patients by conservative treatment.

Arthrography of the hip. A clue to the pathogenesis of the epiphyseal dysplasias.

Hip arthrography was performed on a group of nine patients whose diagnoses included various types of multiple epiphyseal dysplasia and spondyloepiphyseal dysplasia. The cartilage anlage of the capital femoral epiphysis was normal in size and contour in all but one patient, suggesting faulty, incomplete, or delayed ossification of the epiphysis in both multiple epiphyseal dysplasia and spondyloepiphyseal dysplasia. The multiple epiphyseal dysplasia patients, as well as those with a dominant form of spondyloepiphyseal dysplasia, had decreased joint space volumes probably related to the degenerative arthritis which commonly develops in such cases. It is not possible to distinguish between multiple epiphyseal dysplasia and spondyloepiphyseal dysplasia at arthrography.