Sándor Kiss

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.

Open adductor tenotomy in the prevention of hip subluxation in cerebral palsy

We reviewed radiographs of 76 hips in 41 patients with cerebral palsy treated with open adductor tenotomy because of hip subluxation. The majority of patients suffered from spastic diplegia. The aim of our study was to evaluate the effect of adductor release on hip subluxation. We measured the centre-edge angle and migration percentage pre-operatively and 1 and 3 years post-operatively. We considered the results according to patients’ age at time of surgery (younger or older than 4 years of age) and ability to walk. Children younger than 4 years of age had better results than children older than 4 years of age. In children without walking ability, more than half of the hips had further radiological subluxation. In children with walking ability, we observed stabilisation or improvement of femoral-head subluxation in more than three quarters of the cases.RésuméNous avons examiné les radiographies de 76 hanches de 41 malades avec une paralysie cérébrale traitée par ténotomie à ciel ouvert des adducteurs en raison d’une subluxation de la hanche. La majorité des malades souffrait de diplégie spasmodique. Le but de notre étude était d’évaluer l’effet de la suppression des adducteurs sur la subluxation de la hanche. Nous avons mesuré l’angle de couverture externe et le pourcentage de migration avant l’intervention et à un an et trois ans post-opératoires. Nous avons considéré les résultats en fonction de l’âge des malades à la chirurgie (en dessous ou au-dessus de quatre années d’âge) et de la capacité de marche. Les enfants de moins de quatre ans avaient des meilleurs résultats que les enfants plus agés. Chez les enfants sans capacité de marche, plus de la moitié des hanches avait une plus grande subluxation radiologique. Chez les enfants avec capacité de marche, nous avons observé une stabilisation ou amélioration de la subluxation de la tête fémorale dans plus de trois quarts des cas.

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.

Osteochondritis (Calvé’s disease) of a vertebral body—a rare form of vertebra plana

In 1925, Calvé described vertebra plana as an aseptic necrosis of bone involving a single vertebral body of the spinal column. This theory was set aside in 1954 by Compere, who concluded that vertebra plana is caused by eosinophilic granuloma and not by osteochondritis as suggested by Calvé. It has been well documented in literature that many factors other than eosinophilic granuloma can cause vertebra plana-like destruction of the vertebral body. However, the definition of the terms was not clear, and there was no consensus on whether to call these cases vertebra plana or not. Some authors did, some did not. Anyhow, no publication so far has reported on osteochondritis as a rare cause of vertebra plana. The case of a 12-year-old girl, presented here by the authors, suggests this explanation. Some important conclusions can be drawn from this regarding the nomenclature, the diagnosis, and the therapy.

Common Bone Dysplasias and Malformations

Multiple epiphyseal dysplasia (MED) is characterized by the disturbance of enchondral ossification involving numerous epiphyses. MED is usually transmitted in an autosomal dominant manner, although autosomal recessive transmission has also been reported. Different levels of deformities may be present in one patient. Usually lower extremity joint pain with decreased range of motions and limping are the main complaints. Dominantly hips, knees, and ankles are affected. Irregular, fragmented epiphyses and flat articular surfaces with normal metaphyses and mild shortening of the tubular bones can be observed. Upper extremity involvement may differ from minimal to severe with significant deformities (Figs. 1.1–1.8).