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Background: Treatment of spinal muscular atrophy (SMA) scoliosis has evolved in the last decade, with the emergence of fusionless surgical techniques that allow correction of the deformity before the end of growth spurt. These techniques are expected to delay definitive spine fusion and preserve trunk growth., Purpose: The aim was to evaluate long-term clinical, radiologic, and respiratory outcomes of a minimally invasive fusionless surgery (MIFLS) in SMA scoliosis., Methods: All children affected with SMA scoliosis who underwent MIFLS in our department from 2011 to 2019 were included. The instrumentation consisted in a bilateral sliding rod construct from T1 to the sacrum, anchored proximally by double-hook claws and distally by iliosacral screws. Clinical, genetic, respiratory and radiographic data were retrospectively reviewed. A patient's satisfaction survey was performed., Results: A total of 59 children with genetic confirmation of SMA (9SMA1c, 47SMA2, and 3SMA3) underwent MIFLS at a mean age of 11±1.9 years. All of them were nonwalker at the time of surgery. Twenty-six were treated with intrathecal Nusinersen. Mean follow-up was 5.2 years (2 to 9.6 y). Mean major coronal curve improved from 79±15 to 41±16 degrees and pelvic obliquity decreased from 24±11 to 5.9±4 degrees. Mean space available for lung improved from 77% to 93%. Mechanical or infectious complications occurred in 9 patients, with removal of the implant in 1. 6 children required unplanned surgeries. Postoperative bracing was needed in 13 children. Mean gain weight 3 years after the first surgery was 6 kg. 91.5% of patients had a positive satisfaction of the surgery. There was no significant impact in respiratory function postoperatively. Only 30 children required rod lengthening procedures, with a mean interval between procedures of 1.9 years (0.5 to 3.7 y). No arthrodesis was required at last follow-up in any patient., Conclusion: Bipolar MIFLS in SMA preserves spinal and thoracic growth without interference with respiratory function. It provides a significant correction of spinal deformity and pelvic obliquity, having a reduced rate of complications. The correction of spinal deformity was maintained at long term, not requiring definitive fusion at the end of growth., Level of Evidence: Level IV., Competing Interests: L.M. has intellectual property rights with Euros company. S.Q.-R. is consultant and belongs to advisory boards for Biogen, Avexis (Novartis Gene therapies), Roche, Sanofi-Genzyme. The remaining authors declare no conflicts of interest., (Copyright © 2021 Wolters Kluwer Health, Inc. All rights reserved.)

Background: Multiple epiphyseal dysplasia (MED) and pseudoachondroplasia (PSACH) are congenital skeletal disorders characterized by irregular epiphyses, mild or severe short stature and early-onset osteoarthritis which frequently affect the hips. The current study evaluates the long-term results of the Chiari osteotomy in MED and PSACH patients., Methods: Twenty patients (14 MED and 6 PSACH) were retrospectively included. Clinical assessment used the Postel Merle d'Aubigné (PMA) score and the Hip disability and Osteoarthritis Outcome Score (HOOS). Risser index, Sharp angle, acetabular depth index, center-edge angle, Tönnis angle, and femoral head coverage were measured on the preoperative radiographs and at last follow-up. The Treble index, which identifies the hip at risk in MED patients, was also determined. Stulberg classification (grades I to V) was used to evaluate the risk of osteoarthritis in the mature hips.Statistical analyses determined differences between preoperative and postoperative data. The Kaplan Meier method was used to calculate the survival rate of the operated hips using total hip arthroplasty as the endpoint., Results: Thirty-three hips which underwent a Chiari osteotomy were reviewed. The average follow-up was 20.1 years. The PMA scores were significantly better at last follow-up than preoperatively. All radiographic parameters significantly improved. Moreover, the Sharp angle, center-edge angle, and femoral head coverage improved to a normal value at hip maturity. All of the operated hips had a Treble index of type I. At hip maturity, a majority of hip were aspherical congruent (Stulberg grades of III and IV). The survival rate of the operated hips was 80.7% at 24 years postoperative., Conclusions: The Chiari osteotomy is a satisfying solution for severe symptomatic hip lesions in MED and PSACH patients. At long-term follow-up, this procedure lessens pain and improves hip function, which delays total hip arthroplasty indication., Level of Evidence: Level IV., Competing Interests: The authors declare no conflicts of interest., (Copyright © 2020 Wolters Kluwer Health, Inc. All rights reserved.)

Objective: To present a series of pediatric cervical spine (CS) aneurysmal bone cysts (ABC), to review the literature, and to propose a treatment algorithm., Material: We present a series of 4 cases of ABC and review the literature using PubMed, EMBASE, and Google scholar., Results: Only 51 cases are documented. The mean age at diagnosis is 11.5 years, and there is a small female predominance (F:M ratio=1.6). Most of ABC occurs in the upper CS (41%), are located in the posterior component (75%), and extends in 40% of the vertebral body. A single treatment modality was used in 56.9%, whereas combination of surgery with other treatment modalities was used in the rest. Of the total number of cases, 56.8% were managed with marginal resection, and instrumentation was used in 80%. Mean follow-up was 72.5 months, with the majority of patients disease free. Pain is the most common symptom present at the latest follow-up., Conclusions: ABC of the spine is a pediatric tumor occurring rarely in the CS. Treatment options vary from simple curettage to total resection with or without instrumentation. Recurrence after surgery is highest after curettage alone. The main indications for surgery are rapid progression, despite intracystic injection, and/or the presence of neurological signs or symptoms.

Background: Two main options for treatment of congenital idiopathic clubfoot are the "French" functional method and the Ponseti method. The goal of this article was to evaluate the results of the functional treatment method, which, if necessary, is completed by a surgical release., Patients and Methods: A series of 187 feet (129 patients) underwent functional conservative treatment. At first evaluation, the feet were classified according to the classification of Dimeglio. All patients then underwent daily physiotherapy and splintage, which was progressively stopped during childhood. Among these 187 feet, 85 feet (45.5%) required soft-tissue release to correct the remaining deformity. Surgery, when required, consisted of a complete posterolateral and medial release procedure, combined with a lengthening of the tibialis anterior tendon in most cases and a bony lateral procedure in case of forefoot adduction., Results: At the latest follow-up (14.7 y; range, 7.4 to 23 y), results were "good" or "very good" in almost 98% of feet, according to the Ghanem and Seringe score. Severe feet at first consultation showed a worse result and required surgery more often than did the less severe ones. Among nonoperated feet, very good results were found in 99% of feet, and none had a fair or bad result. The average age at surgery was 2.5 years. Feet operated upon had lower results compared with the others. At last follow-up, among the operated feet, the results were excellent or good in 95% of the feet. The results were fair or bad in 4 cases; all 4 feet had been operated upon more than once. The results were not statistically dependent on age at the time of surgery, but feet operated upon before the age of 2 years had statistically more flattening of the talar dome and subtalar stiffness., Conclusions: The functional treatment of clubfoot leads to a very good result without the need for surgery in more than half of the patients. The initial severity of the feet is the main factor that influences the final result. The rate of feet not requiring surgery should be increased by recent modifications to the method, including percutaneous Achilles tenotomy., Level of Evidence: Level IV-retrospective series.

Background: To better understand anatomical and functional outcomes of coxa vara in chondrodysplasia according to the initial presenting hip morphology, disease type, and impact of surgery., Methods: Clinical and radiographic records of 19 children (35 hips) diagnosed with coxa vara and with osteochondrodysplasia were reviewed. We classified the hip radiographic findings into 2 groups: (a) group I, coxa vara with a fragmented and/or nonossified head; and (b) group II, coxa vara with a regular femoral head. Surgical indications in coxa vara included decreased range of hip motion (usually diminished abduction, extension, and internal rotation), coxa vara with progression documented on regular follow-up hip radiographs, and/or severe coxa vara with a Hilgenreiner epiphyseal angle (HEA) of 60 degrees or more. Follow-up was until the completion of growth and, for some patients, into early adulthood. Mean follow-up was 8 years., Results: Twenty-five hips were operated on in 13 patients. In 23 hips, the procedure was a valgus osteotomy fixed by pins and wire. A pelvic extension osteotomy without valgus osteotomy was performed in one patient (2 hips). Coxa vara recurred in 7 hips. In 4 of these hips, repeat surgery with hypervalgus osteotomy was indicated to stop epiphyseal slipping (3 hips) or to improve the arc of motion (1 hip). Functional outcomes were poor in coxa vara associated with poor epiphyseal development (nonossified or fragmented epiphysis) as seen in spondyloepiphyseal dysplasia congenita, spondyloepimetaphyseal dysplasia, Kniest disease, and multiple epiphyseal dysplasia. Coxa vara with physeal instability as observed in spondylometaphyseal dysplasia resulted in deformity recurrence postoperatively during growth. In contrast, outcome was better in cases of coxa vara with nonphyseal/nonepiphyseal involvement, that is, good femoral head morphology, stable physis, and good articular cartilage, as seen in cases of metaphyseal dysplasia and cleidocranial dysplasia., Conclusions: Coxa vara with physeal and epiphyseal involvement and severe impairment of the articular cartilage has a poor prognosis even after reconstructive surgery. In coxa vara with an abnormal physis, there were numerous postsurgical recurrences of the deformity during growth if the physis was not stabilized at the time of valgus osteotomy. In these cases, we should delay osteotomy until an HEA greater than 60 degrees. Coxa vara in which only the metaphysis of the femoral neck is involved, the deformity is milder and often requires no treatment. Indications for surgery in this group are increasing coxa vara, Trendelenburg gait, or an HEA greater than 60 degrees.

Juvenile idiopathic arthritis (JIA) is the most common rheumatic disease in children. The hip is usually affected later, and its involvement is the most common cause of disability in JIA. Failure of medical and preventive treatment, significant joint destruction, or multiarticular involvement may necessitate total hip arthroplasty (THA) to restore good function. Related clinical trials show initially good THA results in most of the series, but long-term outcomes are uncertain due to a significant arthroplasty loosening rate in the initial years, particularly with cemented stems. The authors report the results of 62 noncemented THAs in 34 children with JIA after an average follow-up of 6 years (range 3-13). Mean age at surgery was 18.3 years (range 11.8-31) and 14 of the 34 children had active disease. Clinical results were good for hip function but less for global function. There were no infections. Two acetabular cups early in the series failed due to poor primary fixation and had to be revised. Survivorship analysis was performed with the Kaplan-Meier method. At 13 years, the survival rate was 100% for the femoral component and 90.1% for the acetabular component.

Traumatic dislocation of the hip in childhood is uncommon and can be a consequence of minor trauma. The authors report a series of 35 dislocations in skeletally immature patients. Most were isolated posterior dislocations without acetabular lesions. In 75% of cases, reduction of the dislocation was easy. Nine children required surgery to remove interposed joint capsule and/or osteochondral fragments to achieve anatomic reduction. Outcomes were generally good, except in one patient in whom a displaced fracture of the femoral physis was followed by total head avascular necrosis. One case of partial necrosis had a satisfactory outcome. Epiphyseal necrosis, though uncommon, appeared to be inconsistent to prevent and hard to predict. Bone scan seems to be more effective than MRI for the detection of necrosis.

Fractures of the odontoid process are rare in children. We retrospectively reviewed 15 cases in children younger than 6 years, with an average follow-up of 4 years and 3 months. Eight of these patients had neurologic involvement; magnetic resonance imaging (MRI) changes were seen in the spinal cord at the cervicothoracic junction in six. This neurologic injury was thought to be due to the major anterior displacement of the upper spine, causing spinal cord stretch at the spinal apex of the cervical and thoracic spine. Eight patients secured in forward-facing car seats were injured as the result of motor vehicle accident. Conservatively treated fractures fused without problem. By contrast, the three cases operated on as the initial management of the fracture had complications postoperatively.

The purpose of this investigation was to determine whether induced micromovement during the elongation period could improve the consolidation of diaphyseal elongation obtained by callus distraction. Two series of paired rabbit hindlimbs were studied. The surgical procedure and the waiting period were identical. During elongation, one hindlimb was stimulated, and the other was the control. The consolidation period was 2 days. Reproducible tibial osteotomy and lengthening of the two tibiae was confirmed radiographically. The mineralized callus was quantified by dual-beam x-ray absorptiometry. The callus diameters were measured. Bones were axially compressed to failure. Callus volume, mineral quantity, mineral density, and resistance to failure were not different on the stimulated side compared with the unstimulated side, so micromovement applied during elongation had no effect on bone consolidation. For all tibiae, resistance to failure of the callus was significantly correlated to callus volume, to callus mineral content, and to callus mineral density.

We observed 39 feet with a "too-long" anteromedial process of the calcaneus (TLAP) in 25 children and adolescents. The abnormality was diagnosed from symptoms (ankle sprains or persistent ankle or foot pain) in 33 cases. Six cases of TLAP were noted on radiographs taken of the patient's opposite, asymptomatic foot. Initial treatment by 3 weeks of strapping failed in seven of seven cases, and 3-6 weeks of primary plaster immobilization failed in 10 of 25 feet. Primary immobilization treatment failed in 17 of 33 symptomatic feet. Resection of the TLAP provided good results in 14 of 15 feet when used as a secondary procedure after failure of immobilization treatment and in one foot in which it was used as primary treatment. We believe that this abnormality is an anatomic variant that becomes symptomatic because of inversion stress with impingement of the abnormally long process between talus and cuboid.

We studied vertebral growth after thoracic or lumbar fractures in 52 children followed up from the time of fracture until skeletal maturity. We identified pure anterior compression in 32, combined anterior and lateral compression in 11, and total vertical compression in nine. Six patients had no treatment, 42 had a conservative treatment, and four were operated on. In fractures with anterior compression < 10 degrees, no significant difference could be found between the group without treatment and the group with conservative treatment when the Risser sign was 0 or 1 at the time of injury. In such fractures with angles > 10 degrees, conservative treatment gave better results when the Risser sign at injury was < or = 2. Prolonged conservative treatment was not effective in children with a Risser sign of 3. The evolution of lateral compression seems to be related to the initial injury rather than to treatment. Vertical compression injuries were positively influenced by a conservative treatment when the Risser sign was < or = 2.

The purpose of this investigation was to determine whether induced micromovement could improve the consolidation of diaphyseal elongation by callus distraction. Two series of paired rabbit hindlimbs were studied. The surgical procedure, waiting period, and elongation period were identical. One hindlimb was then left under neutralization conditions, but the other limb was stimulated by axial micromovements. Reproducible tibial osteotomy and lengthening of the two tibiae were confirmed radiographically. The mineralized callus was quantified by dual-beam x-ray absorptiometry. The anteroposterior and lateral diameters of the callus were measured. A semiquantitative histologic study allowed the ratio between fibrous or cartilaginous callus or both and mineralized callus to be determined. Bones were axially compressed to failure. Callus volume, callus mineral content, callus mineral density, and mechanical forces required to failure were significantly superior on the stimulated side compared with the neutralized side, so micromovements applied after the end of elongation were beneficial for bone healing. Mechanical forces required to failure were significantly correlated to callus volume and callus mineral density.

The authors reviewed 70 femoral lengthenings performed for limb-length discrepancy in 66 children and adolescents using gradual incremental distraction. Nine were performed using the Judet lengthener and 61, the Orthofix external fixator. Etiology of the femoral shortening was congenital in 22, posttraumatic in 17, postinfection in 13, neurologic in 12, and miscellaneous in six. There were 83 complications, which were assessed as to their relation to the etiology of shortening, amount of lengthening, and age. The incidence of joint complications did not seem to be less than that previously encountered with rapid distraction methods of lengthening. Bony consolidation was achieved without additional surgery in 88% of cases. Delayed consolidation was most commonly encountered in children younger than 8 years old with congenitally short femora. The authors believe that good results can be obtained by incremental distraction by using uniplanar fixation by aggressive physical therapy, proper fixator application, and appropriate dynamization of the fixator.