The association between vitamin-D-resistant rickets and linear sebaceous naevus syndrome is extremely rare. Only eight cases have been described in the English literature and in none were the skeletal aspects addressed. We present three new cases and describe the musculoskeletal features. The details and outcome of surgery for correction of the deformities are discussed. The disturbances of
We investigated whether, in the management of
stable paediatric fractures of the forearm, flexible casts that
can be removed at home are as clinically effective Cite this article:
Perthes’ disease is an osteonecrosis of the juvenile
hip, the aetiology of which is unknown. A number of comorbid associations
have been suggested that may offer insights into aetiology, yet
the strength and validity of these are unclear. This study explored
such associations through a case control study using the United
Kingdom General Practice Research database. Associations investigated
were those previously suggested within the literature. Perthes’ disease has a significant association with congenital
genitourinary and inguinal anomalies, suggesting that intra-uterine
factors may be critical to causation. Other comorbid associations
may offer insight to support or refute theories of pathogenesis.
We reviewed the medical records of 115 patients with 130 hips with developmental dysplasia with complete dislocation in the absence of a neuromuscular disorder, spontaneous reduction with a Pavlik harness, and a minimum of 14 years’ follow-up. The mean age at the time of harness application was 4.8 months (1 to 12) and the mean time spent in the harness was 6.1 months (3 to 12). A total of 108 hips (83.1%) were treated with the harness alone and supplementary surgery for residual acetabular dysplasia, as defined by an acetabular index >
30°, was performed in 22 hips (16.9%). An overall satisfactory outcome (Severin grade I or II) was achieved in 119 hips (91.5%) at a mean follow-up of 16 years (14 to 32) with a follow-up rate of 75%. Avascular necrosis of the femoral head was noted in 16 hips (12.3%), seven of which (44%) underwent supplementary surgery and nine (56%) of which were classified as satisfactory. The acetabular index was the most reliable predictor of residual acetabular dysplasia.
The bicompartmental acetabulum is one of the morphological changes which may be seen in children with Legg-Calvé-Perthes’ disease. Three-dimensional CT and MRI were used to analyse the detailed morphology of the acetabulum with special reference to its inner surface, in 16 patients with Perthes’ disease and a bicompartmental acetabulum. The bicompartmental appearance was seen on the coronal plane image through the acetabular fossa. The lunate surface was seen to grow laterally resulting in an increased mediolateral thickness of the triradiate cartilage. On the horizontal plane images, the acetabular fossa had deepened and had a distinct prominence at its posterior border. The combination of these morphological changes resulted in a bicompartmental appearance on plain radiography. Acetabular bicompartmentalisation appears to be the result of an imbalance of growth between the cartilage-covered lunate surface and the cartilage-devoid acetabular fossa.