G. Szöke

Dysplasia epiphysealis hemimelica (Trevor's disease) : 7 of our own cases and a review of the literature

Background and purpose Dysplasia epiphysealis hemimelica is characterized by irregular overgrowth of cartilage in the epiphysis, usually affecting the knee and ankle. We treated 7 children by surgery between 1980 and 2005. After reporting one child case, we summarize our cases and the cases described in the literature. Method We discuss the diagnosis of this dysplasia, especially the role of radiography. We describe the suggested treatment, which could be surgical or non-surgical depending on the location and the symptoms. Results After reviewing 57 cases, we found that this dysplasia occurs twice as often in males as in females. The medial side of the epiphysis is affected twice as often as the lateral side. In two-thirds of the cases, more than one epiphysis was affected. If the location of the exostosis suggests that it might lead to joint deformity, early surgical excision is recommended. Interpretation Since there is often involvement of more than one epiphysis, we emphasize the importance of a skeletal survey once this dysplasia is diagnosed.

Osteoarticular manifestations of congenital insensitivity to pain with anhydrosis

Summary. We report the case history of a boy who suffered from congenital insensitivity to pain with anhydrosis. We discuss the orthopaedic disorders occurring in 21 cases reported in the literature.

Response of the tendon during limb lengthening

Little is known about the increase in length of tendons in postnatal life or of their response to limb lengthening procedures. A study was carried out in ten young and nine adult rabbits in which the tibia was lengthened by 20% at two rates 0.8 mm/day and 1.6 mm/day. The tendon of the flexor digitorum longus (FDL) muscle showed a significant increase in length in response to lengthening of the tibia. The young rabbits exhibited a significantly higher increase in length in the FDL tendon compared with the adults. There was no difference in the amount of lengthening of the FDL tendon at the different rates. Of the increase in length which occurred, 77% was in the proximal half of the tendon. This investigation demonstrated that tendons have the ability to lengthen during limb distraction. This occurred to a greater extent in the young who showed a higher proliferative response, suggesting that there may be less need for formal tendon lengthening in young children.

Relative ability of young and mature muscles to respond to limb lengthening

The response of the muscle is critical in determining the functional outcome of limb lengthening. We hypothesised that muscle response would vary with age and therefore studied the response of the muscles during tibial lengthening in ten young and ten mature rabbits. A bromodeoxyuridine technique was used to identify the dividing cells. The young rabbits demonstrated a significantly greater proliferative response to the distraction stimulus than the mature ones. This was particularly pronounced at the myotendinous junction, but was also evident within the muscle belly. Younger muscle adapted better to lengthening, suggesting that in patients in whom a large degree of muscle lengthening is required it may be beneficial to carry out this procedure when they are young, in order to achieve the optimal functional result.

The incidence of avascular necrosis of the healthy, contralateral femoral head at the end of the use of Pavlik harness in unilateral hip dysplasia.

We examined the incidence of avascular necrosis (AVN) of the healthy femoral head in unilateral hip dysplasia at the end of the use of the Pavlik harness. The evaluation of AVN was done with the help of standardised roentgenograms. Between 1974 and 1982, 1,064 dysplastic hips (869 children) were treated with the Pavlik harness at the Orthopaedic Department of Semmelweis University. Of these, 674 children who had unilateral hip dysplasia were chosen for this study. In the period of our investigation, ultrasonography was not yet used routinely, so in some cases the hip was mistakenly diagnosed as dysplastic. The average patient age was 3.2 months, and the average length of treatment was 4.9 months. We found no correlation between the appearance of AVN in the healthy hips at the end of treatment with the Pavlik harness and the age of the child at the start of treatment. On the other hand, there was a strong significant correlation between the appearance of AVN and the length of treatment.