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ObjectiveTo develop a classification for neuromuscular disease patients in each of the three motor function domains (D1: standing and transfers; D2: axial and proximal function; D3: distal function).Materials and methodsA draft classification was developed by a study group and then improved by qualitative validation studies (according to the Delphi method) and quantitative validation studies (content validity, criterion validity and inter-rater reliability). A total of 448 patients with genetic neuromuscular diseases participated in the studies.ResultsOn average, it took 6.3minutes to rate a patient. The inter-rater agreement was good when the classification was based on patient observation or an interview with the patient (Cohen's kappa=0.770, 0.690 and 0.642 for NM-Score D1, D2 and D3 domains, respectively). Stronger correlations (according to Spearman's coefficient) with the respective “gold standard” classifications were found for NM-Score D1 (0.86 vs. the Vignos Scale and −0.88 vs. the Motor Function Measure [MFM]-D1) and NM-Score D2 (−0.7 vs. the Brooke Scale and 0.64 vs. MFM D2) than for NM-Score D3 (0.49 vs. the Brooke scale and −0.49 vs. MFM D3).Discussion/conclusionsThe NM-Score is a reliable, reproducible outcome measure with value in clinical practice and in clinical research for the description of patients and the constitution of uniform patient groups (in terms of motor function).

We report the case of a 15-year-old girl who suffered an indirect knee trauma. The standard X-rays revealed a tract of ivory-like bone partially obstructing the medullary canal of the femur and the tibia. Magnetic resonance imaging produced a hypointense signal on the T1 and T2 sequences. The radiographic diagnosis was melorheostosis which was confirmed on the biopsy specimen which ruled out other diagnoses. The MRI also revealed a tear of the anterior cruciate ligament, treated conservatively. Clinical and radiological surveillance were proposed for the melorheostosis.

In clinical practice, it is generally accepted that hamstring tightness results in incomplete knee extension when the hip is in flexion and in smaller conventional and modified popliteal angles. Similarly, a difference between the conventional popliteal angle and the modified popliteal angle (popliteal differential) would be associated with a permanent deficit in knee extension. The purpose of this study was to determine whether these two hypothesis correlate with clinical findings.The series was composed of 35 walking cerebral palsy children, 16 girls and 19 boys, mean age 11+/-3.6 years with a pathological conventional popliteal angle. These children walked using the jump knee (n=24) or the crouch knee (n=11) pattern. Permanent hip flexion and the conventional and modified popliteal angles were noted. SPSS version 10.1.3 for Window was used to search for a correlation between the popliteal differential and the presence of permanent hip flexion using several values for the popliteal differential (5, 10, 15, 20, and 30 degrees ). Data were adjusted for age and gender.The statistical analyses demonstrated a significant relationship between the presence of permanent hip flexion and a popliteal differential strictly less than 10 degrees and between the absence of permanent hip flexion and a popliteal angle greater or equal to 10 degrees . These statistically significant results, which demonstrated the opposite of what was expected, were independent of age and gender.Our findings demonstrate that examination of the knee joint is indispensable but insufficient. The conventional popliteal angle is not a reliable indicator of hamstring tightness. The normal value of the modified popliteal angle has not been established so that it is impossible to determine what a pathological angle is. We do not know whether measurement of this angle is sufficient to establish indications for surgery. In the future, the development of muscle models coupled with gait analysis should enable more reliable prediction of outcome after surgery. At the present time, we recommend repeated physical examination using a standardized protocol, taking into consideration, several parameters including spasticity, selectivity and muscle force and to perform quantified gait analysis before scheduling hamstring lengthening surgery for walking cerebral palsy children.

The appropriate treatment for unilateral slipping of the upper epiphysis of the femur is controversial. Prophylactic surgical treatment raises the risk of bilateralization. The procedure is often unnecessary as the natural history of epiphyseal slipping is often favorable when the displacement is minimal. We analyzed the complications of systematic prophylactic treatment in order to determine the optimal attitude.This was a retrospective series of 62 children who underwent surgery for unilateral slipping of the superior femoral epiphysis between 1996 and 2005. Prophylactic treatment of the healthy hip was instituted systematically. The complications were noted according to Paley. We searched for factors of risk of complications, studying the bone maturity indexes, the characteristic features of the healthy femur epiphysis and the surgical technique used.There were seven complications, six following prophylactic treatment and one after material removal. The rate of complications reached 11.3%. Five of the complications were minor: two local infections with favorable outcome and three patients who complained of pain in the hip at mobilization, which had totally resolved at one year. One moderate complication was noted: spiral fracture of the upper femur starting from the screw head and treated with plate fixation. The outcome was favorable. There was one severe complication: osteoarthritis of the hip joint with septicemia on day 3. The outcome was unfavorable with necrosis of the femoral head, resection and use of a spacer. A total hip arthroplasty was performed at one year.Complications were globally more frequent and more severe compared to series using prophylactic screwing. The major infection complication (osteoarthritis) observed in this series has not been reported elsewhere. There was probably a center effect since we recruited all of the most complex cases in our region. We were unable to identify any factor predictive of complications. There is apparently no clear choice between systematic prophylactic treatment and careful surveillance. Prophylactic treatment in selected patients might be the key to a successful preventive approach.

Compare the irradiation delivered in conventional radiography and digital radiography by image intensifier during a scoliosis workup.Our prospective randomized study included 105 patients, all of whom were identified according to sociodemographic parameters as well as criteria evaluating the quality of the full front spinal x-ray at PA incidence. The entry dose at the scapula and the exit dose in interorbital, thyroid, mammary, and hypogastric projection was measured by thermoluminescent dosimeters.The results of 71 girls and 28 boys, aged a mean 13.8 years with a mean weight of 47 kg were analyzed. At equal image quality, the entry dose was not significantly different between the two techniques; the mean exit dose reduction was 64% during digital acquisition. This reduction involved the interorbital (162%), mammary (43%), and thyroid (309%) regions. However, this system is more irradiating in the hypogastric region (34%).The dosimetric evaluation of the different imaging techniques used to explore the entirety of the spine should be part of radiologists' quality standard used to document their work and their choices.

The natural history of congenital scoliosis or kypho-scoliosis resulting from a hemivertebra is well documented. The spinal deformation generally worsens in children with a free or semi-segmented hemivertebra situated in the thoraco-lumbar, lumbar or lumbosacral region.From 1982 to 1997, fifteen pediatric patients with 15 hemivertebrae causing progressive scoliosis or kypho-scolisosis underwent hemivertebral resection via a double posterior and anterior approach associated with convex fusion. Mean age at surgery was 4.4 years. The fifteen patients were reviewed at bone maturity (Risser 4 or 5) to assess outcome.Genitourinary tract anomalies were associated in five of the 15 patients and intrathecal anomalies in two. Mean follow-up was 12.1 years. Segmental scoliosis was 30.2 degrees preoperatively, 12.3 degrees postoperatively and 8.9 degrees at last follow-up. The values for total scoliosis were 30.5 degrees, 12.3 degrees and 12.6 degrees respectively. This was a 70.5% improvement at last follow-up for segmental scoliosis and 58.7% for total scoliosis. The real trunk imbalance improved from 31% preoperatively to 9% at last follow-up. All these differences were significant.The main objective of surgical treatment for congenital scoliosis due to hemivertebra is to prevent the development of severe deformation which would necessitate a dangerous and difficult procedure to achieve correction. Resection of the hemivertebra via a double approach is the ideal method for early correction. The procedure not only corrects the spinal deformation but also prevents later deterioration. Correction should be performed as early as possible. The result is sustained over time if there is no other associated spinal anomaly. The procedure is safe and the technique well controlled in experimented hands. Patients need to wear a corset for only six months after resection of the hemivertebra.

Resection of malignant tumors of the proximal humerus often requires dissection of the rotator cuffs and the deltoid muscle. There is no consensus on the ideal method for shoulder reconstruction. We report the functional outcome in a homogeneous series of eight patients treated by arthrodesis using a vascularized free fibular flap.Eight patients were included in this study. All had an aggressive tumor of the upper humerus. Tumor resection was associated with a rotator cuff and deltoid muscle resection in all patients. All patients then underwent shoulder arthrodesis using a free vascularized fibular flap fixed with a plate. Clinical and radiological evaluation was available for six patients at mean 28 months. The Musculoskeletal Tumor Society function score was used to assess overall function of the upper limb. The cosmetic outcome and radiographic bone healing as well as hypertrophy of the fibular graft were noted.Active abduction and active flexion were 82 degrees on average. All patients could bring their hand to the mouth. Circumduction was possible but limited in amplitude. The mean function score was 26.5/30 with an excellent functional outcome in all patients. The cosmetic outcome was considered poor by all patients. Radiographically, bone healing was achieved at last follow-up in all patients but there was one case of failed fusion between the fibular graft and the scapula which required secondary iliac grafting. Mean fibular graft hypertrophy was 32.8% at last follow-up.Two reconstruction methods have been described for patients who require tumor resection of the upper humerus: reconstruction with preservation of glenohumeral joint function and shoulder arthrodesis. Many techniques have been described for each method. It is however difficult to compare the different series reported in the literature because rotator cuff and deltoid muscle resection was not systematically performed and reconstruction methods varied between patients. An analysis of the literature shows that preservation of motion of the scapular glenoid joint can give good functional results when the rotator cuff and deltoid muscle can be preserved. If they cannot, results favor shoulder arthrodesis which provides the patient with very satisfactory upper limb function. Use of a vascularized fibular flap has provided very good arthrodesis results. The patient must however be informed of the probable poor final cosmetic result.

The aim of this work was to validate the pertinence of a radiological index (P/A) used as a predictive factor for risk of kyphosis in children with spina bifida. P/A is defined as the ratio between P, the distance between the posterior vertebral wall and the remnant of the posterior arch, over A, the distance between the anterior wall and the posterior vertebral wall, measured on the lateral view of the spine in the most dysplastic vertebra before one year of age.The files of 163 children were reviewed. The value of the P/A ratio was measured on lateral views of the spine obtained before the age of one year. The bone level on the anteroposterior film was also noted. The presence or absence of kyphosis was determined from the lateral views obtained at ten years of age. Paired variable analysis was used to compare the mean differences in P/A at one year and at ten years in 31 patients with a bone level of L1 or higher. The exact Fisher test was used to determine the difference in the kyphosis distributions in patients with a bone level of L1 or higher between those with P/Aor = 1/2 and the others. In addition, the specificity, sensitivity, and positive predictive value of a positive test (bone level L1 or higher and P/Aor = 1/2) for development of kyphosis were calculated. Results There was no statistically significant difference in the P/A mean value before the age of one year and after ten years. The distribution of patients with kyphosis was different between patients with a bone level of L1 or higher and P/A/2 and the other patients (p0.01). Considering having a bone level of L1 or higher and a P/Aor = 1/2 as a prognostic test, the specificity was 100%, the sensitivity 87.56%, and the positive predictive value 100%. Many patients with spina bifida will develop kyphosis in the course of life. This spinal deformation raises many specific problems for patient management. Use of a radiological index as presented here allows early detection of patients at risk and can also predict which patients will remain free of deformation. This allows a selection of patients who can benefit most from more regular surveillance of the spinal static and avoid unnecessary radiographic examinations in others.

Hip disease can produce major pain and functional disorders in children who should benefit from total hip arthroplasty. We report our experience with total hip prostheses implanted without cement in children.We performed 17 total hip arthroplasties in 13 children who had various conditions, mainly chronic juvenile osteoarthritis and aseptic osteonecrosis secondary to sickle cell anemia. The acetabular inserts were not cemented. All the femoral stems were custom-made using computer-assisted preoperative planning based on standard x-rays and computed tomography findings. Outcome was assessed on the basis of patient satisfaction, pain, and function. Radiographically, we assessed stem implantation, stability and integration. Results were classed with the Harris score and also with the Steinbrocker classification in order to take into account the child's overall functional handicap.Mean follow-up was 36.4 months. There were three cases of superficial hematoma, one case of superficial sepsis and one acetabular loosening. All patients were satisfied. Unsatisfactory function, observed in 80% of the children preoperatively, was found in only 17% postoperatively. The Harris score improved from 23.8 preoperatively to 87.7 at last follow-up. There were no cases of stem loosening and integration was achieved in 85.3% of the cases proximally in the area with hydroxyapatite surfacing.The problem with these children is to determine when total hip arthroplasty should be proposed. We retain three important indications: uncontrollable chronic pain, normal school attendance impossible, no other possibility for conservative surgery. Several studies have reported only mediocre results with cemented stems. We opted for custom-made stems without cement for three reasons: preservation of bone stock, better adaptation to bone whose quality and morphology had been remodeled by the underlying condition and repeated osteotomies which also affect the gluteus medius, and finally, better chance of success for future revisions in these active young patients.The question on whether or not total hip arthroplasty should be performed early in these children to avoid osteotomies which could alter the longevity of a future prosthesis remains open. Custom-made stems inserted without cement have provided satisfactory results in our experience. To date, follow-up is too short to compare our results with those obtained by others using cemented stems.

The study aimed to compare two successive series of procedure using first pieces of natural madreporic coral, then coralline hydroxyapatite in traumatology. The goal of this work was to evaluate long term radiological features of absorption and influencing factors.Within six years, 65 pieces were used; only impaction articular fracture on the lateral tibial plateau (31) and calcaneum fractures (23) were included 21 cases completed inclusion criteria. As there were 3 secondary infections, 18 had therefore, an aseptic evolution and were included in this series.Absorption was evaluated in five degrees according to the volume of the remaining piece on X-rays. We also took into consideration the position of the pieces and the amount of surfaces in contact with cancellous bone.Among the 18 pieces followed up for more than 19 months (average 40), there were 4 sequestrations (1 tibia, 3 calcaneum), all were natural madreporic coral, the others presented osteointegration. Only natural madreporic coral pieces were absorbed, but never completely and the absorption was slow (3 to 7 years) and never complete. A radiolucent line was present in 7 out of 10 pieces of madreporic coral and none around coralline hydroxyapatite. Three developed favorably. Four were sequestred. Number of surfaces in contact with cancellous bone was correlated with osteointegration.The calcium carbonate which makes up the natural madreporic coral pieces is the site of destruction by osteoclasts. The radiolucent line is the specific witness of this biochemical activity. The radiolucent line proceeds sequestrations. No substitute of coralline hydroxyapatite has presented the same sign of peripheral absorption or sequestration. Coralline hydroxyapatite could be the correct coralline material to fill cancellous defect after elevation of an articular depression. In every case, the piece of coral must be fitted in contact with the cancellous bone in the depth of the bone. Conditions of osteoconduction of madreporic coral must be reconsidered. The degree and speed of peripheral absorption must be controlled before weight bearing is allowed.