Bone substitutes in adolescent idiopathic scoliosis surgery using sublaminar bands: is it useful? A case-control study.

INTRODUCTION: In order to avoid pseudarthrosis in adolescent idiopathic (AIS) patients, it is recommended to bring additional bone graft or substitute. Modern rigid instrumentations have been shown to provide less pseudarthroses even without bone substitutes. The aim of our study was to determine the impact of using bones substitutes on fusion rates in adolescent idiopathic scoliosis patients undergoing PSF with sublaminar bands. METHOD: AIS patients scheduled to undergo PSF with sublaminar bands were prospectively enrolled into this study and not given any bone substitutes (no-substitute group). Data were collected and analyzed in patients with at least two years of follow-up. Pseudarthrosis was diagnosed if at least one of the following was present: persistent back pain, hardware failure, loss of correction greater than 10°. The results were compared to a control group who received bone substitutes for the same surgical procedure. RESULTS: Eighty-eight patients were included. For the whole cohort, the mean age was 14.8 years old and the mean follow-up was 30.9 months. For the 'no-substitute' group (n = 44), the mean Cobb angle was 56° pre-operatively, 20.1° post-operatively, and 22° at final follow-up. The fusion rate was not statistically different between the two groups (97.7% vs 95.5%, p = 0.56). At last follow-up, one pseudarthrosis occurred in the 'no substitute' group and two in the control group. DISCUSSION: This is the first study to determine the impact of bone substitutes in AIS fusion using sublaminar bands. In our study, the use of local autologous bone graft alone resulted in a fusion rate of 97.7% despite the use of more flexible instrumentation. The high rate of fusion in AIS patients is more probably due to the healing potential of these young patients rather than to the type of instrumentation. CONCLUSION: The use of additional bone graft or bone substitutes may not be mandatory when managing AIS. LEVEL OF EVIDENCE: 4.

Intraoperative spinal cord monitoring in children under 4 years old.

PURPOSE: The purpose was to study intraoperative spinal cord monitoring (IOM) particularities and performances in children under 4 years old with congenital spinal deformities. IOM is characterized by specific difficulties due to the immaturity of sensory-motor pathways in such young patients. To the best of our knowledge, this study is the largest dedicated to IOM in this challenging population. METHODS: This study was retrospective and monocentric. 78 IOM in children under 4 years old were analyzed. Somatosensory evoked potentials (SSEP), neurogenic mixed evoked potentials (NMEP) and D waves were performed. Patients were classified into one of four categories with respect to IOM data and clinical outcome: true positive, false positive, true negative and false negative. Sensitivity and specificity were then calculated accordingly. RESULTS: There were 6 true positive, 3 false positive, 67 true negative and 1 false negative outcomes. One patient was characterized by unreliable baseline IOM data. IOM sensitivity was 86 %. IOM specificity was 96 %. The false negative case was monitored using SSEP alone. IOM was difficult in 10/77 cases, being unilateral because of a planned fibular graft harvest. NMEP were characterized by higher signal-to-noise ratio than SSEP. CONCLUSIONS: Immaturity of sensory-motor pathways predominates in the central rather than the peripheral nervous system in young children. MEP and D waves could thus be difficult to obtain. Although non-specific to motor pathways, it could be useful to "by-pass" the brain, using NMEP. In regard to maturational particularities, IOM in children under 4 years old should be performed by experienced neurophysiologists, avoiding in these cases surgeon-guided devices.

Circumferential fusion using a custom-made screw in the management of high-grade spondylolisthesis.

PURPOSE: Management of high-grade spondylolisthesis is challenging and to date no therapeutic consensus is available. Various surgical procedures have been described using unique or double approach. The aim of the study is to describe an original technique using a posterior-only approach to achieve a circumferential lumbosacral fusion with a custom-made screw. METHODS: In our experience, eight patients (mean age 15 years old) were treated for grade III or IV slipping without instrumental reduction. Surgical technique included a laminectomy from L5 to S2, then after mobilization of neural elements a guide wire was inserted from the posterior side of S2 to the antero-superior corner of L5. A specific drill was used and the 10-mm screw was then inserted under fluoroscopic guidance. Further steps included a discectomy, lumbosacral interbody graft and posterolateral graft to obtain a circumferential fusion. Clinical and radiological evaluations were obtained. RESULTS: On the whole series, solid fusion was achieved after 6 months on average. Sagittal realignment was observed with a regression of pelvic retroversion and hip flessum, a lumbarization of the lumbar lordosis and an improvement of the thoracic kyphosis. For one patient, a postoperative S1 deficit was observed. CONCLUSION: This technique provides satisfactory results in the management of high-grade spondylolisthesis. This concept is based on a double spine instability considering viscoelastic properties of the disc and postoperative sagittal reciprocal changes as prerequisite. This posterior-only approach represents a valuable alternative to other procedures.

Idiopathic scoliosis and balance organisation in seated position on a seesaw.

The aim was to determine the biomechanical processes involved in postural regulation when self-imposed disturbances occur in the seated position in the antero-posterior direction. Twelve female adolescents with right thoracic scoliosis (SG) (Cobb = 30.4 degrees +/- 9.7) and 15 control adolescents (CG) were included in this study. The ground reaction forces (GRF) were studied whilst the subjects maintained their balance in the sitting position on a seesaw. Six conditions were tested: eyes open and closed; with an additional load placed on the subject's right or left shoulder; and with an additional load on the subject's right or left pelvis. The SG showed significantly higher force amplitudes and variability and fewer oscillations than the CG in all the conditions. In the SG, the time analysis showed that the duration of the GRF was significantly higher in forward and left directions. Whatever the condition tested, the intra-group differences were not significant. The scoliotic patients in seated position were characterised by larger changes of the GRF, especially with a postural control in the forward and left directions, corresponding to that on the concave side of their spinal curvature. No significant differences were found to exist between the various conditions (load and unload, eyes open and eyes closed). Clinical tests and rehabilitation methods should include assessments of seated patients' spatio-temporal adaptation to GRF.

Dynamical asymmetries in idiopathic scoliosis during forward and lateral initiation step.

Adolescent idiopathic scoliosis (AIS) is characterized by morphological trunk modifications acting on body mass distribution. Some specific biomechanical strategies during postural regulation have been reported. Given that spinal deformity is three-dimensional, some strategy analysis resulting from different stepping directions should lead to a better understanding of the dynamic adaptation of behaviour. The aim of this study is to identify dynamic strategies of AIS patients stepping in lateral and forward directions. Ten AIS patients with a right thoracic curve and 15 controlled volunteers have been tested. Ground reaction forces (GRF) have been recorded for right-limb stepping and for left-limb stepping associated to forward and lateral directions. Force amplitudes, corresponding occurrences, impulses of stepping phases and an asymmetry index have been computed. Asymmetry and variability increased in the AIS group, compared to the control group, whatever the stepping direction is. Asymmetry for AIS patients systematically provides an increased left initiation GRF compared to a right initiation. Nevertheless, for both groups, lateral initiation shows the largest asymmetry index reported for a forward initiation. More precisely, adaptive dynamic strategies for the AIS group have been characterized by an asymmetry between right and left limbs for lateral and forward initiation. These results can be explained by the influence of scoliosis pathology on dynamic movements due to spinal deformity. A right thoracic curve leads to an extra weight on the limb, which needs to be moved; consequently, stepping initiation with the right limb was more challenging for patients than stepping with the left limb. For the AIS group, the observed variability can also depend on the ontogenesis of adaptive strategies. Lateral step initiation has to be considered as the most relevant paradigm to study scoliosis and may also serve as a clinical basis for treatment to analyse the dynamic postural control and asymmetry strategies of the scoliosis patient.

Genu valgum in Legg-Calvé-Perthes disease treated with femoral varus osteotomy.

UNLABELLED: Femoral varus osteotomy is one of the most common treatments for patients with symptomatic Legg-Calvé-Perthes disease with more severe deformities. We hypothesized knee valgus alignment at maturity in patients with Legg-Calvé-Perthes disease is an effect of the disease and not an effect of femoral varus osteotomy. We retrospectively compared matched groups of 28 patients with and without femoral varus osteotomy. The two groups were similar with respect to age at onset and classification of Herring et al. The distribution of valgus alignment among patients who had surgery and patients who did not have surgery was assessed at maturity and was similar between the operative and nonoperative groups. The data suggest valgus malalignment (genu valgum) is not a side effect of femoral varus osteotomy in patients with Legg-Calvé-Perthes disease, but rather an effect of the disease. LEVEL OF EVIDENCE: Level III, prognostic study. See the Guidelines for Authors for a complete description of levels of evidence.

Congenital dislocated spine: implications for orthopaedic management.

BACKGROUND: Congenital dislocation of the spine (CDS) is a rare malformation due to a developmental failure of the spine and the spinal cord at a single spinal level. New embryological and physiopathological findings define CDS as an autonomous entity compared with multilevel pathologies. The severity of CDS neurological outcome requires its treatment by experienced pediatric spine surgeons in a pediatric specialty hospital. This report aims to propose a comprehensive orthopaedic management strategy and operative technique of CDS in 6 new patients. METHODS: The records of patients treated at our institution for congenital anomalies of the spine were reviewed in a retrospective study. Inclusion criteria were extracted from the actual context of new embryologic theories: single level involvement; sudden mainly sagittal vertebral displacement with anterior translation of the entire cranial vertebral column on the caudal vertebrae ("step-off sign"); underlying spinal malformation at a clearly distinct level; spinal cord intact both cranial and caudal to the malformation; possibility of malformed aspect of the 2 involved vertebrae. Demographic data, family, and clinical history were collected. Complete set of plain radiographs and modern imaging computed tomography and magnetic resonance imaging were analyzed. RESULTS: Six children treated between 1993 and 2007 have been classified as CDS. The mean follow-up to date of the 4 patients alive after the last corrective surgery is 9.8 years (range, 1-14.6 years). All patients alive have at follow-up solid stable fusion and no progression of spinal deformity. Two of the patients are independent walkers. CONCLUSIONS: The neurological involvement of CDS if present initially is the consequence of an associated spinal cord malformation without mechanical factor. Adaptation of the therapeutic strategy may avoid secondary neurological damage. Parents should be counseled as soon as the diagnosis is made, the obstetrical and postnatal orthopaedic management has to be adapted. Stabilization of the spine including very early cast immobilization and an early instrumented decompression-stabilization with circumferential fusion in 1 stage is required.

Symptomatic os trigonum in children.

BACKGROUND: Symptomatic os trigonum is a rare condition well described in adults responsible for chronic ankle pain. To date there is no published case of successfully managed symptomatic os trigonum in children. METHODS: We retrospectively reviewed 4 paediatric patients (11-17) successfully operated on for a symptomatic os trigonum through a postero medial approach. One case was bilateral. RESULTS: Pain relief was postoperatively obtained in all cases. All of the patients were able to return to physical activities after 3 months. The mean follow up was 12 months. CONCLUSION: Symptomatic os trigonum may be responsible for chronic ankle pain in children and adolescents as well as in adults. The surgical treatment is effective in children.

The influence of adolescent idiopathic scoliosis on the dynamic adaptive behaviour.

The idiopathic scoliosis is characterized by a three-dimensional spinal deformity involving new dynamical strategies to regulate the posture. The aim is to analyze the centre of pressure (CP) behaviour in forward stepping (FS) and lateral stepping (LS) to determine the dynamical consequences of scoliosis. Ten adolescents suffering from right thoracic scoliosis (Cobb>or=18 degrees ) and 15 healthy adolescents participated in this study. Two forceplates recorded the CP evolution in medio-lateral and antero-posterior axes resulting from FS and, LS with the dominant (D) and with the non-dominant (nD) limbs. Our results showed between groups and within groups differences respect to axis of motion. The comparison between groups in the LS showed the increase of the CP total displacement only when stepping with nD limb. Conversely no major evidence emerges from the FS analysis. Whatever the axis was, the CP total displacement of the D limb did not differ between groups. The comparison between lower limbs for healthy subjects was always different for FS whereas this comparison became non-significant for LS. For patients the same analysis showed results less systematically different. The correlation analysis, only when LS is initiated with nD limb, revealed opposite CP dynamical strategies between groups. These results may be explained by the influence of the spinal deformation on internal mass distribution and the asymmetrical neurophysiological factors previously described. Therefore, to perform LS the patients develop an asymmetry between both limbs to guarantee the balance despite scoliosis. Thus LS reveals the differences between groups and between initiation limbs.

Rotational profile of the lower limb in 1319 healthy children.

Lower limb rotational profile in children may cause great concern to parents and relatives. In order to give parents clear information, there is a need for referential studies giving normative data of lower limb rotational profile and its normal changes expected over growth. Our aim was to collect a large clinical series of healthy children, out of a clinic, selected from a non-consulting population and to analyse Tibial Torsion and Femoral Anteversion according to age and gender. One thousand three hundred and nineteen healthy children underwent a clinical evaluation. Tibial Torsion was assessed using the method described by Staheli and Engel, whereas Femoral Anteversion was assessed using the method described by Netter. Our results showed that there was a significant difference between males and females in Femoral Anteversion, whereas there was no significant difference between the right side and the left side. Femoral Anteversion was higher in females, and was markedly correlated with age in both genders. There was no significant difference between males and females in Tibial Torsion, nor significant difference between the right side and the left side. Tibial Torsion was slightly correlated with age in both genders. Normative data were statistically defined in this work using the +/-2S.D. range. To our knowledge, there is no large and comprehensive series in the English speaking literature that gives normative data of Femoral Anteversion. Concerning Tibial Torsion, our results compared to those published in the literature.

Salter-Harris Type III and IV medial malleolar fractures: growth arrest: is it a fate? A retrospective study of 48 cases with open reduction.

Salter-Harris type III and IV medial malleolar fractures (MacFarland fracture) is a joint fracture of the ankle in children. The fracture line passes through the medial part of the lower epiphyseal disk of the tibia. Prognosis is dominated by later risk of misalignment and osteoarthritis. The aim of this study was to evaluate the functional and radiological outcome of these fractures. We retrospectively analyzed the cases of 48 children with MacFarland fractures (31 boys and 17 girls), mean age at the time of trauma 11 years 6 months (range, 8-15 years). The fractures were classed into two groups according to the Salter and Harris classification for epiphyseal detachment: Salter III (30 cases) and Salter IV (18 cases). Surgical treatment was given in all cases (46 screw fixations, 2 pin fixations). Three outcome categories were used: good (no pain, no stiffness, no limp, no misalignment, no surgical complication, no healing problem), fair (pain and/or stiffness and/or limp and/or healing problem without misalignment, no surgical complication), and poor (misalignment or surgical complication). Mean follow-up was 3 years and 3 months (24-94 months). Twenty-eight children were skeletally mature at the longest follow-up. The three-month postoperative assessment showed 35 patients with good results and 13 children with fair results. Ankle stiffness was noted in 6 cases, ankle pain in 4 cases, wound healing complications in 4 cases, limp in 1 case, and snapping in 1 case. The long-term outcome was considered good for 45 patients, fair for 2 patients (1 wound adherence and 1 hypertrophic scar tissue), and poor for 1 patient (6-degree varus deformity). We did not note leg-length discrepancy or malunion at the longest follow-up. Our results show that growth arrest after MacFarland fracture is no fate. We used surgery more than is generally reported by other teams, opting for surgery as soon as the displacement was >or=1 mm. Surgical treatment was arthrotomy in all cases to achieve anatomical reduction under direct view, followed by osteosynthesis. We believe that it is difficult to evaluate if the reduction is perfect under the control of the intensifier screen alone. Arthrotomy did not lead to ankle stiffness, in any of our patients at longest follow-up.

Ezrin and alpha-smooth muscle actin are immunohistochemical prognostic markers in conventional osteosarcomas.

Ezrin is a cytoskeleton linker protein that is actively involved in the metastatic process of cancer cells. We have searched for a prognostic value of ezrin and some of its partners: alpha-smooth muscle actin and CD44H in 37 patients with an osteosarcoma. Automate immunohistochemistry (IHC) with anti-ezrin, alpha-smooth muscle actin and CD44H antibodies was performed in 66 specimens: 37 biopsies before chemotherapy, 16 resected tumours of "poor" responders and 13 metastases. The messenger RNA (mRNA) levels of ezrin of 13 frozen biopsies and 4 metastases were evaluated by real-time quantitative reverse transcriptase polymerase chain reaction (RT-PCR). All results were correlated to the following clinical data. Ezrin expression by IHC was found in 62% of 37 biopsies in the different histological subtypes. A good correlation was found between positive or negative samples by IHC and mRNA levels. Ezrin expression was recorded in 84.5% of metastastic samples. The mean expression of ezrin was higher in metastases than biopsies (p = 0.024). In multivariate analysis, ezrin was an independent prognostic marker for event-free survival and overall survival (OS) with p < 0.001 and p = 0.003, respectively, and alpha-smooth muscle actin for OS only (p = 0.024). Our findings suggest that ezrin and alpha-smooth muscle actin are predictive IHC prognostic markers for patients with an osteosarcoma.

Assessment of acute ankle sprain with os subfibularein children.

Children commonly present with acute inversion injuries of the ankle. Radiographs occasionally show small fragments under the tip of the lateral malleolus. The purpose of our study was to clarify their origin. A prospective standardized clinical examination and a magnetic resonance imaging were carried out. Among the 116 included patients, seven of them showed an os subfibulare. They presented more pains on the lateral ankle area than the other children. We found also more injuries on the anterior talofibular ligament. The os subfibulare could be secondary to an accessory ossification but the lateral area of the ankle would be likely to be injured.

Neurological classification in myelomeningocele as a spine deformity predictor.

In myelomeningocele, spinal deformities are responsible for major disability. Our aim was to check the predictive power for future spine deformity of a neurological classification applied at 5 years of age. We classified patients into four groups according to their neurological examination made at the age of 5 years. Groups were defined as follows: group I, L5 or below (meaning that all the patients in this group have a paralysis that at least leaves the L5 segment intact); group II, L3-L4; group III, L1-L2; group IV, T12 and above (meaning that all the patients in this group have a paralysis that reaches T11 or above). One hundred and sixty-three patients were included. The results showed that group I is a predictor for the absence of spinal deformity. Group III or IV is a predictor for spinal deformity. Group IV is a predictor of kyphosis. It was previously known that the higher the neurological level, the higher the rate of spinal deformity at maturity, but no work had given physicians a guideline to assess the spinal prognosis in myelomeningocele patients. Our work showed, on the basis of this classification made at the age of 5 years, that future spinal disorders may be expected in some patients, while no spinal deformity may be expected in some others. Thus, an appropriate therapeutic strategy and follow-up can be planned.

The lateral wedge augmentation trochleoplasty in a pediatric population: a 5-year follow-up study.

Patellofemoral instability in children requires surgery when recurrent dislocation occurs. The aim of this study is to report the 5-year clinical and radiographic outcomes following lateral wedge augmentation trochleoplasty of the femoral trochlea. This is a review of 23 (mean age: 12.5 years) patients who underwent surgery for recurrent patellar dislocations related to a flat trochlea. In every case, a lateral wedge augmentation trochleoplasty was performed with preservation of the growth plate. This surgery was consistently associated with a soft tissue procedure. All patients were reviewed for a clinical and radiographic evaluation [computed tomography (CT)-scan]. No case of recurrent patellar dislocation was reported and all patients were satisfied with the procedure; three patients reported anterior knee pain after physical activities. CT scan analysis revealed improvement of the trochlear shape measurements in all cases, but osteoarthritis lesions (narrowing of the lateral patellofemoral compartment) were visible on four CT scans. This trochleoplasty technique augmented by a soft tissue procedure on the extensor apparatus led to acceptable clinical mid-term results in terms of stability for patients with a flat trochlea (grade B according to Dejour classification). However, at the 5-year follow-up, four patients showed signs of osteoarthritis on CT scan evaluation. This warrants serious concern in this young population, and alternative methods should be considered whenever possible.

Lumbar hemivertebra resection.

BACKGROUND: A single lumbar hemivertebra can be expected to cause progressive scoliosis. The aim of this study was to evaluate the results of a lumbar hemivertebra resection and short-segment fusion through a combined posterior and anterior approach. METHODS: From 1987 to 2002, a consecutive series of twenty-one patients with congenital scoliosis or kyphoscoliosis due to a lumbar hemivertebra were managed by resection of the hemivertebra through a combined posterior and anterior approach and with the use of a short anterior and posterior convex-side fusion. RESULTS: The mean age at the time of surgery was 3.3 years (range, twelve months to 10.2 years). The mean followup period was 8.6 years. There was a mean improvement of 71.4% in the segmental scoliosis curve from a mean angle of 32.9 degrees before surgery to 9.4 degrees at the time of the latest follow-up assessment, and a mean improvement of 63.9% in the global scoliosis curve from 34.1 degrees to 12.3 degrees. The mean final lordosis was within normal values. CONCLUSIONS: Excision of a lumbar hemivertebra is safe and provides stable correction when combined with a short-segment fusion. Surgery should be performed as early as possible to avert the development of severe local deformities and prevent secondary structural deformities that would require a more extensive fusion later.

Polyarthritis and familial pulmonary fibrosis in a child.

A 7-year-old girl presented with seropositive polyarthritis, autoimmune thyroiditis, and pulmonary fibrosis. Several family members had complex autoimmune disorders and pulmonary fibrosis, and the pedigree was consistent with autosomal dominant inheritance. The possible links between polyarthritis and familial pulmonary fibrosis are discussed, as well as the therapeutic challenges raised by this extraordinarily rare combination.

Lipoblastoma and lipoblastomatosis.

We report two cases of lipoblastoma of the buttock in a 10-month-old boy and a 20-month-old girl, the first with rearrangement of chromosome 8 and the second without cytogenetic abnormality, and one case of lipoblastomatosis of a leg in a 6-month-old boy with a normal karyotype but with a rearrangement of the PLAG1 gene. Lipoblastoma and lipoblastomatosis are two different presentation of the same rare benign soft tissue mesenchymal tumour arising from fetal white fat and occurring almost exclusively in young children under 3 years. These neoplasms have no malignant potential but may recur in cases of incomplete resection. Histological diagnosis sometimes used to be difficult because of the close resemblance of the lesion with myxoïd liposarcoma. Nowadays, cytogenetic analysis may contribute to the diagnosis by showing abnormalities of the long arm of chromosome 8, leading to rearrangement of the PLAG1 gene. Actual advances in cytogenetic molecular analysis may aid in accurate diagnosis.

[Scoliosis in children]. / Scoliose de l'enfant.

The diagnosis of scoliosis is based on the association of kyphosis, vertebral rotation, and lateral deviation of the spine. The clinical examination has to be rigorous in order to rule out a potential etiology for this tridimensional deformity. The assessment of the growth, and of the progressive nature of the scoliosis, is essential before any treatment. For minor deformities, a simple clinical monitoring will be necessary. On the other hand, for moderate deformities, a non-surgical treatment with brace will be recommended. Finally, for severe deformities, the treatment will be a surgical treatment. Surgery will consist of a spinal arthrodesis.

Experience in Perioperative Management of Patients Undergoing Posterior Spine Fusion for Neuromuscular Scoliosis.

The objective of this investigation was to determine the outcome of spine fusion for neuromuscular (NM) scoliosis, using Unit Rod technique, with emphasis on complications related to preoperative general health. Between 1997 and 2007, 96 consecutive patients with neuromuscular scoliosis operated on with Unit Rod instrumentation were retrospectively reviewed. The inclusion criteria were diagnosis of NM scoliosis due to cerebral palsy (CP) and muscular dystrophy (DMD). Patient's preoperative general health, weight, and nutrition were collected. Different radiographic and clinical parameters were evaluated. There were 66 CP patients (59 nonwalking) and 30 DMD patients (24 nonwalking). Mean age at surgery was 16.5 years and 13.9 years, respectively. All radiographic measurements improved significantly. Wound infection rate was 16.7% (11% of reoperation rate in CP; 10% in DMD; 3 hardware removal cases). No pelvic fracture due to rod irritation was observed. Unit Rod technique provides good radiographic and clinical outcomes even if this surgery is associated with a high complication rate. It is a quick, simple, and reliable technique. Perioperative management strategy should decrease postoperative complications and increases outcome. A standardized preoperative patient evaluation and preparation including respiratory capacity and nutritional, digestive, and musculoskeletal status are mandatory prior to surgery.