Camurati-Engelmann disease (CED, MIM 131300) is an autosomal dominant, progressive diaphyseal dysplasia characterized by hyperosteosis and sclerosis of the diaphyses of long bones. We recently assigned the CED locus to an interval between D19S422 and D19S606 at chromosome 19q13.1-q13.3, which two other groups confirmed. As the human transforming growth factor-1 gene (TGFB1) is located within this interval, we considered it a candidate gene for CED.
The exact measurement of femoral head cover is essential for an assessment of reduction of congenital dislocation of the hip. We have compared standard anteroposterior radiographs with computerised tomograms and thereby classified the shape of the acetabular roof into four types. We found that the CE angle of Wiberg is a more reliable measure of head cover when the lateral point of bony condensation of the roof is chosen as the reference point rather than the edge of the bone, where these two points do not overlap. We conclude that head cover can be more accurately determined in younger children with dysplastic hips by our 'refined' CE angle, than by the original method of Wiberg.
Treatment of congenital pseudarthrosis of the tibia (CPT) remains a challenge. To clarify the current situation in treatment, a multicenter study was carried out to obtain information on the results of CPT treatment. The objective of this study was to propose appropriate treatment guidelines for CPT. Records of 73 patients with CPT who underwent surgical treatment were collected from 32 hospitals. The modality of the treatment was 26 with Ilizarov technique, 25 with free vascularized fibular graft, 7 with a combination of the two techniques, 6 with intramedullary nailing with free bone grafting, 5 plating with free bone grafting, and 4 with other treatments. Fifty-four procedures resulted in union, 7 resulted in delayed union, 7 were left un-united, 1 underwent amputation, and the results were unknown in 4. According to the results of this study, the most acceptable methods of treatment of CPT are the Ilizarov method and the vascularized fibular graft.
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 degrees , 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.
We reviewed 33 patients (35 hips) after open reduction of congenital dislocation of the hip using Ludloff's medial approach. The mean age at the time of operation was 14 months (5 to 29) and at the time of final follow-up 20.1 years (15 to 24) giving a mean duration of follow-up of 19.4 years (14 to 23). We evaluated the radiological results by the Severin classification and the extent of avascular necrosis using the criteria of Kalamchi. At the latest review 16 hips (45.7%) were in Severin groups I or II (acceptable results) and 19 (54.3%) in Severin groups III, IV or V (unacceptable results). There was evidence of avascular necrosis in 15 hips (42.9%). The radiological results began to become worse at about the age of ten years when type-II avascular changes presented. We conclude that Ludloff's medial approach for open reduction is unsatisfactory for the treatment of congenital dislocation since about half of our patients required additional operations.
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