Purpose of the study: Determine the prevalence and course of spinal deformations in Willi-Prader syndrome and assess the effect of treatment with growth hormone. Analyze outcome after conservative and surgical treatments.

Material and methods: We reviewed the files of 51 patients with Willi-Prader syndrome proven genetically. Spinal deformations were classed according to the SRS system. Body mass index (BMI) was determined and correlated with age and administration of growth hormone. Statistical analysis used the coefficient of correlation and the chi-square test to search for correlations between qualitative variables.

Results: There were 37 girls and 24 boys, mean age at last follow-up 10.7±6.7 years. The prevalence of scoliosis was 52% and varied according to genotype.

The prevalence of scoliosis deformations was higher in patients aged over ten years (p< 0.01). The prevalence of scoliosis was greater in female patients. Patients with BMI< 25 had a significantly lower risk of scoliosis. Treatment with growth hormone was associated with a significant decrease in risk of scoliosis. Among scoliosis patients, ten had a main curvature < 15° and were monitored. Eleven had a curvature > 15° (31±11°) and were treated with a corset. Five had a curvature > 50° and trunk imbalance and were treated surgically. Four of these patients developed serious complications.

Discussion: Scoliosis deformation is frequent in Willi-Prader syndrome. Weight control is very important and BMI should be maintained below 25 to limit the risk of scoliosis. Treatment with growth hormone helps limit BMI and thus the risk of scoliosis. For major deformations, surgical treatment is indicated but at the risk of serious postoperative complications.

Purpose: We reviewed our pre-puberty patients whose scoliosis or kyphoscoliosis involved a hemivertebra. The purpose of our work was to evaluate the surgical technique used and evaluate spinal static as well as functional outcome at skeletal maturity.

Material and methods: This retrospective analysis included 21 patients who underwent surgery before the age of 10 years and were followed to skeletal maturity. We excluded children with a multiple malformation syndrome or multiple vertebral malformations. The type and localisation of the hemivertebra was noted. Deformation, transversal balance, and radiographic measures were recorded preoperatively. Elements contributing to the indication for surgery, the type of procedure, and complications were also recorded. Events recorded during growth were the clinical course, complementary treatments, and possible surgical revision. Functional and aesthetic outcome was assessed at last follow-up. Spinal deviation was measured and compared with the preoperative angles.

Results: Twenty-one children (13 girls, 8 boys), mean age three years ten months (range 10 months – 10 years) met the inclusion criteria. These children had a hemivertebra of the thoracic spine (n=9), the thoracolumbar junction (n=4), the lumbar spine (n=4), or the lumbosacral region (n=4). Surgery was indicated to arrest clinical and radiographic degradation. Several surgical procedures were used: fusion without resection for thoracic vertebrae and resection associated with arthrodesis or epiphysiodesis for other localisations. There were five complications: neurological (n=2), infectious (n=2) and disassembly (n=1). The clinical and radiographic course led to revision in ten children, including two who required a new operation. At mean follow-up of 14 years (9–23 years), the functional outcome was good in 19 patients, poor in one patient with a lumbosacral hemivertebra and in another with a thoracic hemivertebra. The aesthetic result was good in 16 patients. Five of the nine patients with a thoracic hemivertebra remained unsatisfied with the outcome. Mean curvature correction ranged from 26% at the thoracic level to 50% at the thoracolumbar and lumbosacral levels and 75% at the lumbar level.

Discussion: The long follow-up of this series is exceptional. Treatment of evolving spinal malformations is a difficult challenge. Early surgery does not guarantee the final outcome and 50% of patients have to be reoperated at the end of growth. The good long-term functional and aesthetic outcome is however encouraging, particularly when hemivertebrectomy can be performed.

Purpose: Tillaux fractures correspond to type III joint fractures involving the anterolateral tubercle of the inferior tibia in the Salter and Harris classification. These fractures generally occur by external rotation in children about 13 years old at the time the medial part of inferior growth cartilage of the tibial epiphysis has fused. The purpose of the present study was to analyse long-term outcome in a retrospective series of twenty children.

Material and methods: All twenty patients underwent physical examination and a standard radiographic work-up at last follow-up. Results were classed into three categories: good (free of pain, stiffness, gait impairment, joint degeneration), faire (moderate pain and/or stiffness and/or gait impairment without joint degeneration), poor (severe pain and/or severe stiffness and/or limping and/or joint degeneration).

Results: This series included eight boys and twelve girls, mean age 12.8 years (range 3.4 – 14.9). Mean follow-up was three years eleven months (range 12 months – 8 years 9 months). Eleven patients underwent surgical treatment and nine orthopaedic treatment. The lateral segment of the growth cartilage was open in all cases but the medial segment was open in two and completely closed in six. A computed tomography scan was required in one patient to assess displacement. Outcome was considered good in eighteen patients and fair in two. These two patients had persistent pain without functional impairment at about one year follow-up.

Discussion: Overall, the long-term outcome is good for Tillaux fractures if they are correctly identified and evaluated, with computed tomography if necessary, to enable appropriate surgical or orthopaedic treatment. Inappropriate management of this joint fracture can lead to joint incongruency and secondary degeneration. Most of these fractures occur in adolescents and secondary growth impairment is negligible. This type of fracture may also occur in adults. Prognosis is excellent with adapted treatment.

Purpose of the study: Congenital pseudarthrosis of the clavicle is rare, only 200 cases having been reported. Based on 25 personal cases and an overview of the literature, we try to explain the etiology of this condition and the different kinds of treatment.

Material and methods: A retrospective analysis was performed on twenty-five children (16 females, 9 males, mean age at the end of the follow up – 11.5 yrs) from three different centers. We assessed the outcome of surgical and nonsurgical procedures, based on pain, functional ability, cosmetic results, and x-ray examination.

Results: A family background was noted in three children. The lesion always involved the right side. Twenty patients presented a bump over the middle third of the clavicle, thirteen a foreshortened shoulder girdle, three complained of discomfort. In two cases, palpation of the clavicular area was painful. No neurovascular compressive syndrome was reported. None of the patients complained of a decrease in the range of motion or in the strength of the upper limb. X-rays showed a middle third defect. In five cases we found abnormal first ribs.

Seventeen patients underwent surgery, at a mean age of 6 years and 4 months. The procedure always included excision of the pseudarthrosis at both ends and internal fixation with a wire or a plate. In only eight cases a bone graft was used. Healing was achieved in fourteen patients. Three patients needed a second surgical procedure. In these 3 cases we had not used bone grafting. All patients had a normal range of shoulder motion, except a twelve year old girl who complained of discomfort of the right upper limb. The cosmetic result was good in eleven cases, one surgical wound was noted as hypertrophic, and one developed a keloid. An asymmetry of the trunk was still noted in seven cases. The x-rays showed symmetric clavicles in ten cases.

Eight patients were treated conservatively. All of them had a normal range of motion of the shoulder, six had a good cosmetic result and two cases a poor one.

Discussion

According to Alldred, the anomaly results from the failed coalescence of the two primary ossification centers of the clavicle. The overview of the literature and our findings (in one case) confirm that the cartilage which covers both ends of the bone is made of growth cartilage. However, the true mechanism of the nonunion is still unknown. The three familial cases of our work suggest a possible genetic transmission of the disease.

The diagnosis is based on the following criteria: right side lesion, found in infancy, without previous fracture, increasing size with growth, without major functional consequences, without neurofibromatosis or cleidocranial dysostosis symptom. X-rays or histologic examination will confirm the diagnosis showing the usual findings described above.

Complications of the pseudarthrosis of the clavicle are rare and late. Conservative management appears to give good results as seen with our eight patients. However surgical treatment ensures symmetrical shoulder girdles and good function with few complications. Therefore, we recommend performing an excision of the cartilaginous caps, followed by an iliac bone graft and an internal fixation with wire. Surgical management will be preferred in symptomatic patients, in the case of major or increasing deformity, or on parental request.