Purpose of the study: Data are scarce on the long-term outcome after anterior spinal fusion for idiopathic scoliosis in adolescents. The purpose of this study was to assess the clinical and radiological results obtained in a monocentric series using a single-phase fusion with pre-formed plates for the treatment of lumbar, thoracolum-bar and thoracic idiopathic scoliosis in adolescents.

Material and method: This procedure was performed in 111 patients from 1975 to 1993. Complete clinical and radiological evaluations were available for 35 patients, 7 male and 28 female. The SRS-30 questionnaire (translated into French by the authors) and the Oswestry score were used to assess functional outcome. Radiographic data were collected by an independent observer who read the pre-, post- and last follow-up images. Full spine images were available to assess correction of the curvature, frontal and sagittal balance, pelvic parameters, and degenerative changes in the subjacent discs. Functional outcome was assessed in relation to the radiographic findings.

Results: Mean follow-up was 22 years (range 16–31). Mean age at surgery was 14.5 years. At last follow-up, none of the patients were unemployed because of a spinal problem and 21 women had had one or more successful pregnancies. The mean SRS-30 score was 3.8/5 and correlated with the Oswestry score (13.8%). One patient underwent revision for disc herniation and one for posterolateral lumbosacral fusion because of persistent frontal imbalance. There as a fracture of the proximal screw in six patients with no sign of nonunion. At last follow-up, the mean Cobb angle was 14 (0–42). Frontal imbalance was noted in 18 patients, mean 12 mm. In the sagittal plane, three patients presented anterior imbalance. The pelvic parameters were within the range of the general population. Signs of L5-S1 disc degeneration were noted in ten patients, with no correlation with functional outcome.

Conclusion: Anterior fusion gives good long-term functional results in the treatment of idiopathic scoliosis in adolescents. Patients can pursue a normal occupational and familial life. This technique has provided satisfactory correction in the frontal and sagittal planes.

Purpose of the study: We noted that four postoperative compartment syndromes observed in our department occurred in short stature patients with micromely secondary to chondrodysplasia. This complication occurred in young subjects with significant muscular development (two with intensive sports practice) whose muscle hypertrophy appeared exaggerated by the short length of the limbs.

Material and methods: The first case concerned a young man with pycnodysostosis who developed a tibial compartment syndrome after prolonged ventral decubitus subsequent to spinal surgery. No surgical procedure had been performed on the lower limb. The complication was probably triggered by simple compression of the proximal portion of the limb leading to elevated venous pressure and rapid self-aggravating muscular atrophy in a small aponeurotic compartment. The second case was a female patient with metaphyseal dysplasia who during adolescence had undergone corrective bilateral tibial osteotomy to align the lower limbs. Preventive aponeurotomy had been performed which left on one side a disgraceful muscle herniation. Aponeurotic plasty had been performed in adulthood to reconstitute the tibial compartment. A compartment syndrome developed in the immediate postoperative period. The third case was a young adult with hypochondroplasia who developed bilateral crural compartment syndrome 24 hours after femoral osteotomy with implantation of an external fixator for lower limb lengthening.

Results: Sequelae were observed in only one of these four cases of compartment syndrome (patient n° 1). Extensive necrosis of the tibialis anterior was observed due to late diagnosis, leading to legal implications and a court suit. Emergency treatment was given in the three other cases and no sequelae were observed.

Discussion: These patients with chondrodysplasia and micromely appeared to be predisposed to postoperative compartment syndrome because of the relative muscle hypertrophy and the narrow muscle compartments. The risk of this complication in small stature patients warrants the use of preventive aponeurotomy during orthopedic procedures involving the limbs and implies rigorous postoperative surveillance for early detection. Systematic aponeurotomies should be extended over the entire length of the muscle compartment involved in order to achieve effective decompression and avoid disgraceful localized muscle herniation.

Purpose of the study: Appropriate surgical treatment for severe lumbosacral spondylolisthesis remains a subject of controversy. Correction of the anterior displacement of L5 and the lumbosacral kyphosis is considered dangerous because of the risk of neurological complications. We present a consecutive series of 40 patients who were treated with the Padovani double plate method for high-grade spondylolisthesis. The long-term outcome was assessed. The reasons for abandoning this method were also discussed.

Material and methods The files of 40 patients (15 boys and 25 girls, aged 8–20 years) underwent surgery in our center from 1979 to 1996. All presented anterior displacement of L5 greater than 50%. After release of the L5 and S1 roots via a posterior approach and insertion posteriorly to anteriorly of two anchors in the S1 body, correction of the L5 displacement was achieved via a transperitoneal anterior approach using a plate applied to the anterior surface of L5 and progressively bolted to the S1 anchors. Clinical and radiological outcome was assessed. Spineview was used for angle measurements. The Beaujon and Japanese Ortopaedic Association scores were used for the clinical assessment.

Results: Lumbosacral fusion was achieved in all patients with complete correction of the L5 displacement in 38. A postoperative radicular deficit was noted in twelve patients and resolved completely in ten. Six patients experienced progressive destabilization of the L4–L5 level. Deep infections in contact with implanted material were noted in five patients. At 18 years mean follow-up, 35 patients are symptom free.

Discussion: This technique enables excellent correction of the L5 displacement and an excellent rate of fusion. The method is particularly challenging technically and the rate of complications is high. Reduction of the displacement appears to be associated with numerous radicular deficits, even after prior release. The plate which was rather large for the youngest patients led to lesions of the L4–L5 disc and destabilization of the suprajacent levels.

Conclusion: Although this technique enables optimal and definitive correction of the lumbosacral deformation, the high rate of complications has led us to change our strategy for the treatment of high-grade lumbosacral spondylolisthesis.

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 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.