Imaging features of lower limb malformations above the foot.

Lower limb malformations are generally isolated or sporadic events. However, they are sometimes associated with other anomalies of the bones and/or viscera in patients with constitutional syndromes or disorders of the skeleton. This paper reviews the main imaging features of these abnormalities, which generally exhibit a broad spectrum. This paper focuses on several different bone malformations: proximal focal femoral deficiency, congenital short femur and femoral duplication for the femur, tibial hemimelia (aplasia/hypoplasia of the tibia) and congenital bowing for the tibia, fibular hemimelia (aplasia/hypoplasia) for the fibula, and aplasia, hypoplasia and congenital dislocation for the patella.

Unpredictability of hip behavior in Dyggve-Melchior-Clausen syndrome: a mid-term assessment of siblings.

Dyggve-Melchior-Clausen syndrome is a rare spondylo-epiphyseal disease, which almost constantly leads to both bilateral hip degeneration and dislocation. Few authors have reported to date the surgical management of this orthopaedic disorder. We present two new cases affecting siblings. One brother was treated by unilateral triple pelvic osteotomy combined with varus osteotomy of the proximal femur; the other was treated by bilateral Pemberton osteotomies with varus osteotomy of the proximal femur. At a respective 5-year and 3-year follow-up delay, both cases had evolved towards progressive subluxation recurrence along with severe hip degeneration. Based on both our experience and literature review, it seems that one should avoid operating these hips unless pain renders surgery mandatory. Total hip arthroplasty seems the only reliable surgical solution at the adult age and paediatric surgeons should keep in mind that previous femoral osteotomies will make it more challenging for adult orthopaedic surgeons to implant on a remodeled anatomy.

Congenital high scapula (Sprengel's deformity): four cases.

We report a series of four cases of congenital high scapula (or Sprengel's deformity) diagnosed and followed in our establishment. The main feature of this very rare congenital malformation of the pectoral girdle is an abnormally high, more or less dysmorphic scapula. A congenitally high scapula is often discovered in young children, when consequences for aesthetics, and sometimes functional difficulties, are brought to light. When surgical treatment is envisaged, imaging is recommended to diagnose a supernumerary structure, ossified (an omovertebral bone) or not (a fibrous and/or cartilaginous connection), extending from the scapula to the cervical spine. This needs to be resected. A CT scan is essential for detecting an omovertebral bone and the vertebral bone abnormalities that are often associated with it. MRI and ultrasound are very useful for assessing any fibrous and/or cartilaginous components. An ultrasound examination has the undeniable advantage of being quick and easy in these young children.

[Clostridium difficile reactive arthritis in a 7-year-old child]. / Polyarthrite réactionnelle àClostridium difficile chez un enfant de 7ans.

Clostridium difficile reactive arthritis is a rare disease; only 5 pediatric cases have been reported in the literature. Its diagnosis is challenging. It manifests as asymmetric aseptic poly- or oligoarthritis, contemporary to infectious colitis, usually after a period of antibiotic therapy. We report a new case in a 7-year-old boy who presented with unusual polyarthritis affecting 12 joints 1 month after antibiotic therapy with amoxicillin-clavulanate. Punctures of both hip joints proved sterile but significantly improved symptoms. Diarrheic stool cultures during hospitalization provided the diagnosis. Antibiotic therapy using metronidazole completely resolved pain and joint swelling within a week. After 1 year of follow-up, there has been no recurrence. We present a review of the literature on this disease and underline the advantages of joint aspiration in this condition with the dual aim of not missing septic arthritis and effectively relieving pain.

Progressive anterior vertebral fusion: a report of three cases.

We report a series of three cases of progressive anterior vertebral fusion diagnosed and monitored in our establishment. This very rare condition was discovered in young children while exploring a spinal deformity. With X-rays of the thoracolumbar spine it was possible to make a positive diagnosis and ensure follow-up. The radiographic semeiotics are characteristic and combine anterior pinching of the disc, well-delimited erosion of the anterior vertebral corners and anterior then posterior intervertebral ankylosis. MRI is very useful for assessing the extent of the intervertebral ankylosis and the condition of the residual discs, without irradiation. The condition develops over several months or years. Conservative treatment is usually sufficient.

Complications of the Luque-Galveston scoliosis correction technique in paediatric cerebral palsy.

PURPOSE OF THE STUDY: Severe scoliosis is a very frequent condition in cerebral palsy children (CP). It is surgically managed, with unit rod instrumentation being the gold standard in English-speaking countries. The purpose of this work was to report on a small, homogeneous series of non-ambulatory, quadriplegic, spastic patients treated by the Luque-Galveston technique in Strasbourg, France. We present the radiographic outcome of the technique along with a full description of any post-operative complications encountered. MATERIALS AND METHODS: Twenty-eight children were operated on according to the Luque-Galveston technique between January 1997 and January 2006. This instrumentation, with fusion, included the whole spine from the sacrum to level T2. All procedures were performed as a one-stage posterior arthrodesis. The spinal deformities were single thoraco-lumbar curvatures, except in one patient. Both curve magnitude and pelvic obliquity were measured by X-ray pre-operatively, post-operatively and after longest follow-up (over 24 months). Our study focused on the rate of complications of this treatment. Only 16 patients out of 28 were tracked since the remaining 12 were lost to follow-up. RESULTS: Mean curve magnitude was corrected from 80 degrees to 34.8 degrees (mean correction, 56.5%), and pelvic obliquity, from 20.9 degrees to 4.2 degrees (mean correction, 79.6%). Loss of correction at average 3.46-year follow-up was 3.9 degrees of curve magnitude and 2.7 degrees of pelvic obliquity. Mean operating time was 301.5 minutes, and average blood loss was 861.9 ml. Patients were discharged from hospital after an average 19.5-day stay, including mean 8.4-day intensive care unit stay. A single major complication, monocular blindness, occurred during the procedure, probably resulting from air embolism. Post-operative complications (totaling 57.1% of our 28 patients) were: one death, three pneumothoraxes, six segmental atelectasias, seven pneumonias and one superficial wound infection. Late-onset complications (totaling 56.2% of our 16 patients at latest follow-up) were: seven broken sublaminar wires, one iliac perforation by the rod, one skin irritation from extreme malnutrition needing hardware removal, and three superficial sacral decubitus ulcers. DISCUSSION: Our correction rate in children affected by CP and manifesting severe scoliosis is similar to that reported in the literature by different surgical teams. Moreover, we did not observe any deep wound infection, haematoma, septicaemia, neurological and digestive complications. Late-onset complications mainly involved asymptomatic sublaminar wire breakage at the two uppermost levels, but no major complication was due to hardware failure, and vertebral fracture did not occur. There was no need for re-intervention because of the hardware, except for one case in which extreme malnutrition provoked skin conflict with the rod. We encountered 10 "windshield wiper" effects in the iliac bone, but we believe they cannot be considered as complications since they seemed to disappear after fusion was fully obtained. Last but not least, unit rod instrumentation is not very expensive compared to more modern techniques. CONCLUSION: Correction of scoliosis and pelvic obliquity, attributed to CP in non-ambulatory children, by the Luque-Galveston technique is both an effective and safe choice in such an indication. Moreover, it is far less expensive than most other techniques, an aspect which should be taken into consideration. LEVEL OF EVIDENCE: Level IV retrospective therapeutic study.

Meningococcal purpura fulminans in children: I. Initial orthopedic management.

BACKGROUND: Purpura fulminans is a rare and extremely severe infection, mostly due to Neisseria meningitidis frequently causing early orthopedic lesions. Few studies have reported on the initial surgical management of acute purpura fulminans. The aim of this study is to look at the predictive factors in orthopedic outcome in light of the initial surgical management in children surviving initial resuscitation. METHODS: Nineteen patients referred to our institution between 1987 and 2005 were taken care of at the very beginning of the purpura fulminans. All cases were retrospectively reviewed so as to collect information on the total skin necrosis, vascular insufficiency, gangrene, and total duration of vasopressive treatment. RESULTS: All patients had multiorgan failure; only one never developed any skin necrosis or ischemia. Eighteen patients lost tissue, leading to 22 skin grafts, including two total skin grafts. There was only one graft failure. Thirteen patients were concerned by an amputation, representing, in total, 54 fingers, 36 toes, two transmetatarsal, and ten transtibial below-knee amputations, with a mean delay of 4 weeks after onset of the disease. Necrosis seems to affect mainly the lower limbs, but there is no predictive factor that impacted on the orthopedic outcome. We did not perform any fasciotomy or compartment pressure measurement to avoid non-perfusion worsening; nonetheless, our outcome in this series is comparable to existing series in the literature. V.A.C.(®) therapy could be promising regarding the management of skin necrosis in this particular context. While suffering from general multiorgan failure, great care should be observed not to miss any additional osseous or articular infection, as some patients also develop local osteitis and osteomyelitis that are often not diagnosed. CONCLUSIONS: We do not advocate very early surgery during the acute phase of purpura fulminans, as it does not change the orthopedic outcome in these children. By performing amputations and skin coverage some time after the acute phase, we obtained similar results to those found in the literature.

Meningococcal purpura fulminans in children. II: Late orthopedic sequelae management.

BACKGROUND: Purpura fulminans is a rare and extremely severe infection, mostly due to Neisseria meningitidis. Nineteen patients were followed up immediately after the initial multivisceral failure in order to diagnose late-onset orthopedic sequelae. We report our experience with these 19 patients, in light of our medical follow-up protocol and surgical management. MATERIALS AND METHODS: Nineteen patients were referred for acute purpura fulminans between 1987 and 2005 to our institution and followed up prospectively until the present. We collected information on all diagnosed orthopedic sequelae, all surgical procedures performed, and the actual orthopedic outcome. RESULTS: Fourteen patients developed at least one orthopedic sequel after a mean of 2 years delay, with a mean of 8.65 years follow-up (range 3-22 years). The most common presentation was lower limb physeal growth plate arrest in eight patients involving 18 growth plates, leading to five limb length discrepancies and 12 significant knee and/or ankle deviations. Patients were treated by completing epiphysiodesis in addition to limb lengthening and/or reaxation osteotomies, except for two patients, in which epiphysiolysis was performed. All outcomes are, to date, satisfactory, with both knee and ankle axes within the physiological range. Among the seven patients who underwent below-knee amputation, six needed stump revision because of skin conflict (4) or prosthetics misadaptation due to upper tibial varus (2). Regarding the upper limb, three patients presented with four cicatricial scar bands, one located on a ring finger, two at the first commissure, and one at the wrist (all were successfully treated by enlargement Z-plasties). Two patients developed hip avascular necrosis. CONCLUSION: It is important for children diagnosed with meningococcal purpura fulminans to be followed up closely starting from the very beginning by a pediatric orthopedic surgeon. It ensures that late-onset orthopedic sequelae will be diagnosed early. In accordance to the literature, this study highlights the high rate of lower limb epiphysiodesis, above all other types of sequelae. This study reports a possible link between purpura fulminans and avascular necrosis of the hip.

Study of the pressures applied by a Chêneau brace for correction of adolescent idiopathic scoliosis.

We performed a study on 32 idiopathic scoliotic patients (30 females, 2 males) treated with a Chêneau brace. Eighteen patients had a single right thoracic curve and 14 had a single right thoracolumbar curve. We used the TekScan system (ClinSeat Type 5315 Sensor, TekScan, Boston MA, USA) to measure pressures at the skin-brace interface, assess the effect of strap tension and analyze the variation of these pressures with position and activity. The TekScan device enabled identification of the pressure areas corresponding to the brace's three loading points. The pressure under the main pad had a greater mean value than the pressure under the two counter-pads. Tightening the straps led to a significant increase in the pressures, whatever the position studied or the curve pattern. Compared with the standing (reference) position, we observed significantly higher pressures during maximal inspiration (p < 0.001) and lower pressures during maximal expiration, in the prone position and after having risen from a lying position, for both thoracic curves (p < 0.001) and thoracolumbar curves (p < 0.01). The pressures for thoracolumbar curves were lower than those for thoracic curves, whatever the position studied and both before and after strap adjustment. For lying positions, lying on the right side produced the greatest increase pressure. Even though the TekScan system does not provide direct information on the correction of spinal curvature, it appears to be a useful tool in the treatment of scoliotic patients. Strap adjustment clearly influences the applied pressures - particularly those on the rib cage. During activity, there is a natural tendency to decrease the pressure; this justifies efforts to maintain strap tensions in general and during day wear in particular.

Determination of the influence of the Chêneau brace on quality of life for adolescent with idiopathic scoliosis.

OBJECTIVE: The main objective is to determine the influence of treatment by Chêneau brace on the quality of life (QoL) of adolescents with idiopathic scoliosis. MATERIAL AND METHOD: We used the QoL scale of Climent et al. "the Quality of Life Profile for Spine Deformities" (QLPSD, in which a higher score means a worse QoL) and visual analogue scales (VAS) for pain and for QoL ranging from 0 to 100mm. The study includes 108 subjects divided in three groups, a control group of 32 patients without brace, a full-time treated group (wearing a Chêneau brace 23/24 hours) of 41 patients, and a part-time treated group (wearing the brace during the night only) of 35 patients. RESULTS: The QoL is significantly worse in the full-time treated group than in the group with night treatment and in the group without brace for the psychosocial functioning, the sleep disturbances, the back flexibility (P<0.001), the body image (P<0.01), as well as the overall score (P<0.001). On the other hand, there was no difference for the back pain. Among patients wearing the brace, the girls had an average QLPSD score higher than the boys for the psychosocial functioning, the body image, the overall score (P<0.05) and the back flexibility (P<0.01). Whereas there was no significant difference between the sexes for the sleep disturbances and the back pain. The QoL of patients who wear the brace was significantly correlated with degrees corrected wearing the brace and the patient's satisfaction on his or her health status. However, this correlation was very weak. On the other hand, there was no correlation between the quality of life and the age, the duration of brace treatment or the skeletal maturity (Risser sign). On VAS, the patients without brace have the highest score of QoL, followed the part-time treated patients while the full-time treated patients have the lowest score. CONCLUSION: The wearing of the Chêneau brace involves a significant reduction in the QoL whatever instruments of evaluations of QoL, QLPSD or VAS. The QoL of the full-time treated patients was the lowest followed part-time treated patients while the QoL of the patients without brace was the highest. However, the brace does not influence the back pain in idiopathic scoliosis at teenage.

[Electrophysical surveillance in limb lengthening in 25 children and adolescents]. / Intérêt de la surveillance électrophysiologique des allongements de membre par fixateur externe: à propos de 25 enfants et adolescents.

OBJECTIVE: To evaluate neurological tolerance during limb lengthening in children and adolescents. MATERIAL AND METHODS: Retrospective study of 25 children and adolescents [15 girls, 10 boys; mean age 11.3 years, range 5-18 years] undergoing limb lengthening. Limbs involved were the femur [11 cases], tibia [10 cases], radius [3 cases] and humerus [1 case], and lengthening was 1 mm/day, adjusted depending on clinically and electrophysically observed alterations during weekly surveillance. RESULTS: Lengthening in 8 cases was accompanied by electrophysiological deterioration and in 1 by clinical alteration without electrophysiological anomaly. Eight of 25 lengthenings had to be slowed and 1 discontinued. DISCUSSION: This work confirms poor neurological tolerance with limb lengthening. It shows the interest of clinical and electrophysiological monitoring in preventing severe neurological accidents with such lengthening.

[Evaluation of the Chêneau brace in adolescent idiopathic scoliosis]. / Evaluation du résultat du corset de Chêneau dans la scoliose idiopathique de l'adolescent.

OBJECTIVE: We aimed to evaluate the Chêneau brace in the orthopedic treatment of adolescent idiopathic scoliosis to better determine the indications under which it could be prescribed. MATERIALS AND METHODS: This was a retrospective study including 63 patients treated by Chêneau brace for adolescent idiopathic scoliosis between 1997 and 2006. The Cobb angles of the curves in the frontal and sagittal planes as well as rotations and rib hump were measured at the beginning of treatment, with the brace, at the end of treatment and 2 years after discontinuing the brace. A variation of+/-10 degrees in angle was selected to judge the results. RESULTS: At the end of the treatment, 25.4% of the curves were improved and 60.3% stabilized, with 14.3% aggravated; 5 patients (7.9%) required surgery. At 2 years, the reduction in angle was 1.8 degrees, on average. The best results were obtained for the lumbar and dorsolumbar curves. A significant improvement was noted for the dorsal curves; the major double curves are not as accessible to the treatment. For curves whose initial angle was less than 30 degrees , an initial reduction of higher than 50% with the brace presented the best improvement at the end of the follow-up. We note a significant reduction of the rib hump but not accompanied by a reduction of rotation. Finally, we note a deleterious effect in the sagittal curves, with a slight flatness of the spinal profile. CONCLUSION: The continuous wearing of the Chêneau brace can stabilize the evolution of adolescent idiopathic scoliosis, apart from the forms with major double curves, which are not as accessible to the treatment. However, the improvement comes at the price of a slight flatness of the curves of the spine in the sagittal plane. The loss of the correction after discontinuing the brace is minor. We emphasize, therefore, the necessity to monitor the sagittal aspect of the spine as well as the possibility of early treatment, strict surveillance and prolonged follow-up.

[Genetics and orthopedics: genetic implications of congenital limb abnormalities]. / Génétique et orthopédie: gènes impliqués dans les malformations congénitales des membres.

Limb malformations are frequent. These malformations are isolated or associated with anomalies of other developmental fields and accurate diagnostic is essential for prognosis evaluation, treatment and genetic counseling. Animal embryology and molecular biology techniques, have given us a better understanding of the processes of growth and patterning of the limb buds. The key genes that are involved in these processes have been identified and their interactions recognized. Human genetics has been able to identify, or at least localize, several genes implicated in limb development. We here review the present knowledge on these genes and their mutations responsible for limb anomalies.

[Facial burns in children. 197 patients]. / Les brûlures de la face chez l'enfant. A propos de 197 patients.

Facial burns frequently occur in child. A total of 197 burned patients with facial localisation were seen during the time period (1991-1996) covered by this retrospective study. One hundred and sixteen patients were less than three years old. A quarter of the child were issued from poor social classes with nine cases of child abuse by burning. One hundred and thirteen injuries were performed by hot water or drinks. The total burn area was less than 10% in 63% of the cases. The average facial burned area was 4%. The cheek, the front and the chin were often concerned. Low limbs and trunk were unjured in all cases. 130 patients needed hospitalisation, and stayed in average 17.31 days at hospital. Four patients died from respiratory complications, all after flame injuries. Fifty two child needed skin split grafts. Complete cicatrisation was observed after 21 days of treatment in child without grafts, and after 35.65 days in child with skin grafts. Twenty eight patients needed physiotherapy in center. The scars had a correct cosmetic appearance, without retraction and inflammatory signs in 158 child after two years. Eleven patients needed other surgical treatment. The authors analyse the results and insist on prevention.

Follow-up study of severe slipped capital femoral epiphysis treated with Dunn's osteotomy.

This study evaluated long-term clinical and radiologic results of Dunn's osteotomy carried out in 50 consecutive cases for severe slipped capital femoral epiphysis. The results of all the patients were analyzed with 4 years 6 months of follow-up. The clinical results were very good and good in 90% of the patients and were fair or poor in 10% of the patients. Seven patients had an important complication (total necrosis or chondrolysis), and the clinical evolution was studied. Dunn's procedure seems to be adequate to correct severe slips.

Comparative study of propofol and midazolam effects on somatosensory evoked potentials during surgical treatment of scoliosis.

OBJECTIVE: Studies of the effects on lower-limb cortical somatosensory evoked potentials (CSEP) during total intravenous anesthesia are sparse for propofol and are lacking for midazolam. This study was designed to compare the effects of propofol and midazolam on CSEP under total intravenous anesthesia during intraoperative monitoring for surgical treatment of scoliosis. METHODS: CSEPs were recorded in two groups of 15 patients during posterior instrumentation for treatment of idiopathic scoliosis. The anesthesia used the combination of atracurium, alfentanil, and an hypnotic agent (propofol for Group I or midazolam for Group II). The main characteristics of the CSEPs (P40 latency and N34-P40 and P40-N50 amplitudes) were recorded using ankle posterior tibial nerve stimulation. The CSEPs were recorded before induction, 10, 70, 100, 130, and 160 minutes after induction, and before the wake-up test. The statistical analysis involved analysis of variance for repeated measures. Both groups were homogeneous before induction. RESULTS: Neither CSEP deterioration during risk-associated surgical procedures nor postoperative clinical abnormalities were observed. Both propofol and midazolam induced increases in P40 latencies, with the increases being greater and more regular for the propofol-treated group. The amplitude values changed with time for both groups, decreasing mainly after induction; in the midazolam-treated group, the amplitudes were smaller but more stable. Propofol modified the morphological characteristics of the response by decreasing the late P60 component amplitude; the W-shaped CSEP morphological pattern was maintained with midazolam. CONCLUSION: This study demonstrates the appropriate use of either propofol or midazolam in scoliosis monitoring. Preoperative small-amplitude CSEPs might favor the use of propofol anesthesia.

[Neuromonitoring and anesthesia in surgery of the spine]. / Neuromonitorage et anesthésie dans la chirurgie du rachis.

The authors report the main effects of anaesthetic drugs that are used alone or in association with anaesthetic protocols on somatosensory evoked potentials (SEP) and on motor evoked potentials (MEP). In the first part of the article, the effects are analysed on SEPs and MEPs that are obtained from non-invasive methods; in the second part, the effects of anaesthesia are analysed with respect to invasive methods of EP recordings. The current increase of invasive techniques of neuromonitoring by SEPs and MEPs is in relation with the weak effect of anaesthetics on evoked responses. Total intravenous anaesthesia (TIVA) provides stable anaesthesia for non-invasive SEP neuromonitoring only if bolus is avoided. With TIVA and other anaesthetic techniques, the introduction of repetitive stimulation provides new possibilities for non-invasive MEP neuromonitoring.