Background The effect of bracing over natural history of stable dysplastic hips is not well known. This multicenter randomized trial aimed at objectifying the effect of abduction treatment versus active surveillance in infants of 3 to 4 months of age. Methods Patients were randomized to either Pavlik harness or active surveillance group. Ultrasound was repeated at 6 and 12 weeks post randomization. the primary outcome was the degree of dysplasia using the Graf α-angle at 6 months of age. The measurement of the acetabular index (Ai) on plain pelvis X-rays was used to identify persistent dysplasia after 9 months and walking age (after 18 months). Findings The Pavlik harness group (n = 55) and active surveillance group (n = 49) were comparable for predictors of outcome. At 12 weeks follow-up the mean α-angle was 60.5° ± 3.8° in the Pavlik harness group and 60.0° ± 5.6° in the active surveillance group. (p = 0.30). Analysis of secondary outcomes (standard of care) showed no treatment differences for acetabular index at age 10 months (p = 0.82) and walking age (p = 0.35). Interpretation Pavlik harness treatment of stable but sonographic dysplastic hips has no effect on acetabular development. Eighty percent of the patients will have a normal development of the hip after twelve weeks. therefore, we recommend observation rather than treatment for stable dysplastic hips.
The natural history of sonographic developmental dysplasia of the hip was determined in a population-based study in which 5170 infants were screened by ultrasound using Graf's method. Of the normal hips at the age of 1 month, 99.6% were still normal at the age of 3 months. Of the immature type IIa/IIa+ and type IIa- hips, if untreated, 95.3 and 84.4% had become normal, respectively. Of the infants with type IIc, D and III/IV hips at the age of 1 month 70, 58.3 and 90.9% were treated, respectively. This study shows that normal hips remain normal in nearly 100% of cases, but development to abnormality is possible. It shows also that most of the immature hips at the age of 1 month become normal without treatment. Although treatment seems to be indicated in the majority of sonographically abnormal hips, the occurrence of overtreatment could not be excluded in our study.
In this study, we followed-up the family with bilateral hereditary micro-epiphyseal dysplasia (BHMED) originally described by Elsbach [1959: J Bone Joint Surg [Br] 41-B:514-523]. Clinical re-examination of all available family members resulted in further delineation of the clinical and radiological phenotype, which is distinct from common multiple epiphyseal dysplasia (MED). Linkage analysis excluded EDM1, EDM2, and EDM3 as candidate genes. Linkage and mutation analysis of matrilin-3 (MATN-3) revealed a new pathogenic mutation confirming that BHMED is indeed a distinct disease entity among MED and MED-like disorders.
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