Long-term outcomes of ilio-sacral screws in minimally invasive bipolar fusionless technique for neuromuscular scoliosis: a retrospective study in 167 patients.

INTRODUCTION: Pelvic fixation in patients with neuromuscular scoliosis is difficult, due to their fragile general condition and poor bone quality. Many techniques have been described, associated with high rates of mechanical complications. The objective of this work was to evaluate the mechanical complications and long-term radiological results of ilio-sacral screw pelvic fixation. MATERIALS AND METHODS: 167 consecutive patients with neuromuscular scoliosis who underwent minimally invasive bipolar fixation with ilio-sacral screw pelvic fixation were retrospectively reviewed. The instrumentation consisted in a bilateral sliding rods construct extended from T1 to the sacrum, anchored proximally by double-hook claws and distally by ilio-sacral screws through a minimally invasive approach. Mechanical complications and radiographic measurements (angle of the major coronal curve, pelvic obliquity, lumbar lordosis) were evaluated preoperatively, postoperatively, and at the last follow-up. RESULTS: Mean operative age was 12 ± 3 years, and follow-up 6.4 years (3.0-10.4 years). Pelvic obliquity decreased from 20° preoperatively to 5° (77% correction) at last follow-up, Angle of the major coronal curve from 75° to 36° (52% correction), and lumbar lordosis from 28° to 38°. 16 mechanical complications in nine patients occurred: screw prominence (n = 1), connector failure (n = 4), screw malposition (n = 11). Unplanned surgery was required in seven cases, two were managed during rod lengthening, seven did not require treatment. CONCLUSION: In this series of neuromuscular patients operated by ilio-sacral screws as pelvic fixation, the results were stable with a mean follow-up of more than 6 years and the complication rate was reduced comparatively to the literature.

Perioperative complications after posterior spinal fusion versus minimally invasive fusionless surgery in neuromuscular scoliosis: a comparative study.

INTRODUCTION: Early-onset scoliosis is a common deformity in neuromuscular disease. When conservative treatment becomes ineffective, several surgical options can be proposed. The most common technique is posterior spinal fusion (PSF) consisting of performing a multiple segmental instrumentation with pedicular screws on the full spine associated with decortication and bone graft. Minimally invasive fusionless surgery (MIFS) is an alternative to correct and fix definitively the spine without graft. The objective of this study was to compare early surgical inpatient period between PSF and MIFS in neuromuscular scoliosis. MATERIAL AND METHODS: 140 NMS operated by PSF or MIFS between 2012 and 2017 was retrospectively reviewed. The following data were compared between groups: general characteristics (age, sex, etiology), preoperative preparation (halo traction, noninvasive ventilation or tracheostomy), Cobb angle and pelvic obliquity correction, use of drugs (vasopressor and/or inotropes, expansion fluids, transfusion and volumes), postoperative complications, and need of noninvasive ventilation. RESULTS: 75 patients were managed by PSF with a mean age of 14.3 ± 2.3y and 65 by MIFS with a mean age of 11.8 ± 3y. Average pelvic obliquity and major curve correction were similar postoperatively. Intraoperative blood transfusion was significantly more common in PSF group (OR, 14; 95% CI [6.3-33.0]). Vasopressors were used non-significantly more often in the PSF group and expansion fluids similar in the two groups. PSF group had more overall complications (OR, 4.6; 95% CI [2.3-9.8]), more infections (OR, 3.6; 95% CI [1.5-9.3]) and more hemodynamic complications (OR, 4.1; 95% CI [1.4-15.1]). Average intubation duration was 5 days in the PSF and 4 days in MIFS (p = 0.05). CONCLUSION: In this series of neuromuscular patients, the complication rate was reduced in MIFS comparatively to PSF, with lower blood transfusion and less infections.

Long-term outcomes of non-invasive expandable endoprostheses for primary malignant tumors around the knee in skeletally-immature patients.

INTRODUCTION: Expandable endoprostheses are used to restore limb function and compensate for the sacrifice physis involved in carcinologic resection. Long-term outcomes of the last generation of knee "non-invasive" expandable endoprostheses are required. Objectives were to report on oncologic results of bone sarcoma resection around the knee with expandable endoprosthesis reconstruction and to compare the surgical outcomes of the "non-invasive" expandable endoprostheses used in our department. MATERIALS AND METHODS: Retrospective study that included all children with bone sarcoma around the knee that underwent tumor resection reconstructed with non-invasive expandable prosthesis. Phenix-Repiphysis was used from 1994 to 2008 followed by Stanmore JTS non-invasive from 2008 to 2016. Survival and complications were recorded. Functional outcomes included Musculoskeletal Tumor Society (MSTS) score, knee range of motion, lower limb discrepancy (LLD). RESULTS: Forty children (Sex Ratio = 1) aged a mean 8.8 years (range, 5.6-13.8) at surgery were included in the study. There were 36 osteosarcoma and 4 Ewing sarcoma that involved 33 distal femur and 7 proximal tibia. Cohort (n = 40) consisted of 28 Phenix-Repiphysis and 12 Stanmore with a mean follow-up of 9.8 ± 5.8 years and 6.1 ± 3.1 years, respectively. Postoperative infection rate was 7.5% in the cohort (3 Repiphysis). Functional results were significantly better in the Stanmore group with a mean MSTS of 87.6 ± 5.4% and knee flexion of 112 ± 38°. At last follow-up, implant survival was 100% in Stanmore group, whereas all living Phenix-Repiphysis were explanted. Mechanical failure was the primary cause for revision of Phenix-Repiphysis. Limb length equality was noted in 79% patients with Phenix-Repiphysis and 84% with Stanmore at last follow-up. CONCLUSION: Chemotherapy and limb-salvage surgery yield good oncologic outcomes. Expandable endoprostheses are effective in maintaining satisfactory function and lower limb equality. With improvements made in the last generation of "non-invasive" prostheses, implants' survival has been substantially lengthened.

Can Chiari Osteotomy Favorably Influence Long-term Hip Degradation in Multiple Epiphyseal Dysplasia and Pseudoachondroplasia?

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.

Minimally Invasive Fusionless Surgery for Scoliosis in Spinal Muscular Atrophy: Long-term Follow-up Results in a Series of 59 Patients.

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.

Optimal ilio-sacral screw trajectory in paediatric patients : a computed tomography study.

Pelvic fixation during procedures performed to treat spinal deformities in paediatric patients remains challenging. No computed tomography studies in paediatric have assessed the optimal trajectory of ilio- sacral screws to prevent screw malposition. We used pelvic computed tomography from 80 children divided into four groups : females <10 and ≥10 years and males <10 and ≥10 years. A secure triangular corridor parallel to the upper S1 endplate was delineated based on three fixed landmarks. The optimal screw insertion angle was subtended by the horizontal and the line bisecting the secure corridor. Student's t test was applied to determine whether the optimal screw insertion angle and/or anatomical parameters were associated with age and/or sex. Mean optimal angle was 32.3°±3.6°, 33.8°±4.7°, 30.2°±5.0°, and 30.4°±4.7° in the younger females, younger males, older females, and older males, respectively. The mean optimal angle differed between the two age groups (p=0.004) but not between females and males (p=0.55). Optimal mean screw length was 73.4±9.9 mm. Anatomical spinal canal parameters in the transverse plane varied with age (p=0.02) and with sex in the older children (p=0.008), and those in the sagittal plane varied with sex (p=0.04). Age affected ilio-sacral screw positioning, whereas sex did not. Several anatomical spinal canal parameters varied with age and sex. These results should help to ensure safe and easy ilio-sacral screw placement within a secure corridor.

Multidisciplinary surgical strategy for dumbbell neuroblastoma: A single-center experience of 32 cases.

INTRODUCTION: Prognosis of dumbbell neuroblastoma (NBL) is mainly determined by the sequelae induced by the tumor itself and the neurosurgical approach. However, after primary chemotherapy, surgical management of the residual tumor, especially the spinal canal component, remains controversial. METHODS: We conducted a single-center retrospective cohort study over the last 15 years (2002-2017) including patients treated for NBL with spinal canal extension focusing on timing and type of surgery, complications, and functional and oncological follow-up. RESULTS: Thirty-two children (14 M, 18 F) were managed for NBL, with the majority (26) presenting with NBL stroma poor while four had ganglioneuroblastoma intermixed, one nodular, and one ganglioneuroma. All but two patients received neoadjuvant chemotherapy. Upfront laminotomy for spinal cord decompression was performed in two patients; nine patients had extraspinal surgery with a follow-up neurosurgical procedure in seven cases; eight patients had initial neurosurgery followed by an extraspinal procedure, while six patients underwent a combined multidisciplinary approach. With a median follow up of 3.6 years (0.1-14.9), 29 patients (90.6) are alive and two out of three (19, 65.5%) have functional sequelae. CONCLUSION: Patients with NBL with persistent spinal canal extension of the tumor after neoadjuvant chemotherapy treated at our center had outcomes that compare favorably with the literature. This is likely due to the multidisciplinary approach to optimal surgical strategy and continuous evaluation of the respective risks of tumor progression. Neurological disability results from initial spinal cord compression or the radicular sacrifice required for tumor resection.

Long-Term Results of the "Horseman" Procedure for Severe Idiopathic Flatfoot in Children: A Retrospective Analysis of 41 Consecutive Cases With Mean 8.9 Year Duration of Follow-Up.

The "horseman" procedure is a surgical technique used to correct the talocalcaneal joint displacement of severe idiopathic flatfoot in children while maintaining the reduction with a temporary talocalcaneal screw. While this technique has been used since the early 1960s, very little has been reported on its results. Our objectives were to estimate the correction, functional results, and postoperative complications of the "horseman" procedure. We conducted a retrospective study on 23 consecutive patients (41 cases) who underwent the "horseman" procedure for a talocalcaneal joint displacement. Mean follow-up was 8.9 (range 1 to 28) years, and 8 patients (12 feet) had reached bone maturity at last follow-up. Mean age at surgery was 6.6 (range 4 to 9.5) years. At last follow-up, all the patients were asymptomatic except 2 [8.7%] (4 [9.8%] cases). The talocalcaneal divergence on anteroposterior and lateral radiographic views was reduced by 8.9° and 11.4°, respectively, after the surgery, and the correction was maintained with loss of 0.7° and 2.9°, respectively, at final follow-up. The talonavicular coverage angle was reduced by 25° without loss of correction at last follow-up. The calcaneal pitch angle did not change after the surgery. Mean American Orthopedic Foot and Ankle Society score increased from 88.7 of 100 (63 of 100 to 93 of 100) preoperatively to 99 of 100 (97 to 100 of 100) at last follow-up. No major complication occurred. The "horseman" procedure allows an immediate and lasting correction of severe idiopathic flatfoot in children.

Long term results of the revisited Meary closing wedge tarsectomy for the treatment of the fixed cavo-varus foot in adolescent with Charcot-Marie-Tooth disease.

BACKGROUND: Various techniques have been proposed for the treatment of cavovarus feet (CVF). The aim of this study was to report outcomes of the revisited Meary's dorsal closing wedge tarsectomy for fixed CVF secondary to Charcot-Marie-Tooth (CMT) disease. METHODS: All CVF operated on between 1977 and 2011 were included. The tarsectomy design was modified from its original description and systemically combined with a plantar fascia release, a Dwyer osteotomy and a proximal extension osteotomy of the 1st metatarsal bone if required. Outcomes were assessed by 2 functional scores and radiographically. RESULTS: Among the 26 feet (20 patients), the Wicart and Seringe score was very good or good, fair and poor in respectively 58%, 23% and 19% of the feet. Hindfoot and midfoot AOFASs were of 95.5 and 75 respectively. All radiographic measures were significantly improved. CONCLUSIONS: This complete revisited procedure is an efficient and safe surgical technique for the treatment of the CMT disease CVF. LEVEL OF EVIDENCE: Level IV.

Multicentric carpotarsal osteolysis syndrome: long-term follow-up of three patients.

Multicentric carpotarsal osteolysis (MCTO) is a rare skeletal disorder characterized by progressive carpal and tarsal destruction. The upper and lower limbs may be involved, leading to deformities and joint limitation. These anatomic features may be associated with progressive renal failure. The radiographs obtained during childhood showed a carpal and tarsal osteolysis and an asymmetrical involvement. Here, we report on the long-term clinical and radiological findings of three patients with skeletal manifestations of MCTO.

Surgical Treatment of Enchondromas of the Hand During Childhood in Ollier Disease.

PURPOSE: The objective of the present study was to evaluate the outcome of surgical treatment of hand lesions in Ollier disease (OD) carried out during childhood. METHODS: A retrospective review was carried out of 10 pediatric patients with hand involvement of OD, who had undergone surgery for metacarpal or phalangeal enchondromas. The technique comprised curettage and cortical bone reconstruction with corticoplasty, to restore a near-normal phalangeal volume. The range of finger motion (pulp-to-palm distance), the shortened Disabilities of the Arm, Shoulder, and Hand (QuickDASH) questionnaire score, cosmetic improvement, radiological findings (according to Tordai's classification), and recurrence were recorded after a mean follow-up of 7.5 years (range, 4-11.3 years). RESULTS: The mean age at surgery was 10.7 years (range, 6-14 years). Curettage was performed on 35 enchondromas, and 9 cavities were filled with a bone graft. The mean pulp-to-palm distance was significantly lower after surgery (from 1.5 cm to 0.25 cm; P < .05). The mean QuickDASH score was 3.84 (range, 0-11.4). A marked cosmetic improvement was noted for 83% of the hands. Three enchondromas recurred in 1 patient, requiring a second curettage. Fifty-seven percent of the cavities were completely filled with bone (Tordai stage 1) at last follow-up. The outcome did not depend on the presence or absence of a bone graft. CONCLUSIONS: Our results suggest that early surgical treatment comprising curettage and corticoplasty leads to good clinical, cosmetic, and radiological outcomes. Early surgical treatment of well-developed and/or symptomatic enchondromas of the hand in OD should be considered. TYPE OF STUDY/LEVEL OF EVIDENCE: Therapeutic V.

Cobb-1 versus cobb-to-cobb anterior fusion for adolescent idiopathic scoliosis Lenke 5C curves: a radiological comparative study.

PURPOSE: Anterior fusion is a well-established procedure for the treatment of Lenke 5C adolescent idiopathic scoliosis (AIS). This retrospective study aimed to assess preoperative and postoperative radiographic differences between the conventional anterior fusion and anterior short fusions (ASF) in Lenke 5C AIS. METHODS: Radiographic data of 42 consecutive cases of Lenke 5C AIS, which were surgically treated through anterior segmental fusion, were analyzed retrospectively. Patients have been divided into two groups: C group (n = 19) treated by the conventional fusion from the proximal end vertebra to the distal end vertebra of the main curve and C-1 group (n = 23) treated by ASF, sparing the lowest end vertebra of the main curve. Cobb angles of main curve, apical vertebra tilt, C7 coronal tilt, lowest instrumented vertebra angle (LIV angle), LIV adjacent level disc angulation (LIVDA), and the first uninstrumented vertebra angle (FUV angle) were measured on anteroposterior radiographs of the entire spine. LIVDA, FUV wedging, and rotation were measured on stretch films. C7 sagittal tilt, lumbar lordosis, thoracic kyphosis, and proximal kyphosis junction were measured on lateral radiographs of the entire spine. Repeated-measures analysis of variance and fisher test were used for the statistical analysis. RESULTS: The preoperative main curve was 39.9 ± 9.1° in the C group vs. (versus) 42.8 ± 11.8° in the C-1 group. At a mean follow-up of 26.2 months (range 10.5-66.3 months), postoperative main curve improved of 75.8 ± 21.2 % (10.4 ± 9.2°, p < 0.001) in the C group and of 52.7 ± 18.7 % (20.1 ± 8.1°, p < 0.001) in the C-1 group. All parameters improved except the LIVDA, which was slightly impaired, especially in the C-1 group with 5.6 ± 4.2° vs. 4.4 ± 2.2° in the C group. On stretch films, FUV rotation was the only parameter to differ; it was graded at 1 ± 0.7 in the C-1 group vs. 0.6 ± 0.5 in the C group (p = 0.04). No disk obliquity just under the future instrumentation and equivalent FUV wedging were found in any of the two groups. CONCLUSIONS: The conventional anterior fusion and ASF give equivalent correction in Lenke 5C AIS, but ASF seems to induce adding-on of the disk below the LIV. FUV rotation on stretch films does not seem to be a predictive factor of postoperative radiological outcome.

Long-term results of ankle arthrodesis in children and adolescents with haemophilia.

INTRODUCTION: Ankle arthrodesis in patients with haemophilia is a joint-non-preserving treatment option often resulting in substantial pain relief and termination of haemarthrosis by osseous consolidating of the joint. However, limited data are available regarding ankle arthrodesis in young patients. AIM: The aim of this study was to evaluate the long-term clinical and radiographic results with a minimum follow-up of ten years in children with haemophilia who underwent ankle arthrodesis. METHODS: We retrospectively reviewed the results of ankle fusions performed in young haemophiliacs in our department between 1980 and 2006. The Méary procedure was used for arthrodesis, performed on patients with closed growth plates. Only one patient had no fixation, due to being only six years old. The modified American Orthopaedic Foot & Ankle Society (AOFAS) hindfoot score was calculated at last follow-up, and standard radiographic evaluations were performed. RESULTS: A total of 22 ankles were analysed from 17 patients. The mean follow-up was 19.7 ± 8 years. The mean age of the patients was 15.5 (6-23) years at the time of index surgery. There were no intra-operative or peri-operative complications related to ankle arthrodesis. The mean modified AOFAS score at last follow-up was 83 ± 10.5. Radiographic assessment demonstrated solid osseous fusion at the arthrodesis site, with no axis deformities. Two patients developed secondary subtalar arthrosis, treated by subtalar arthrodesis six years after initial ankle arthrodesis in one case. CONCLUSION: In our study, tibiotalar arthrodesis in young patients with haemophilia resulted in good long-term functional outcome with a low surgery-related complication rate.

Internal fixation with occipital hooks construct for occipito-cervical arthrodesis. Results in 14 young or small children.

PURPOSE: Evaluation of effectiveness of internal rigid fixation for occipito-cervical fusion with occipital hooks and cervical screws and/or hooks to improve fusion in young or small children with various bone dysplasia or congenital abnormality. METHODS: Fourteen children with mean age of 8.4 years and height and weight below 140 cm and 35 kg, respectively, who underwent occipito-cervical fusion for miscellaneous pathologies reading to stenosis and/or instability were reviewed. The posterior instrumentation consisted of a precontoured titanium plate rod with an occipital fixation by hooks and a vertebral fixation by screws and/or hooks. Eight patients had cervical fixation with C2 pedicle screws. The mean follow-up was 48 months (27-81 months). RESULTS: Thirteen patients (92.8 %) had a complete fusion and no failure of the fixation was observed. We had two deep wound infections not related to implants. CONCLUSION: Instrumentation was technically feasible even in a very young child. Rigid internal fixation in the little children did not increase surgical complications and significantly increased the union rate of occipito-cervical arthrodesis.

Aneurysmal Bone Cyst of the Cervical Spine in Children: A Review and a Focus on Available Treatment Options.

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.

Orthopedics management of acromicric dysplasia: follow up of nine patients.

UNLABELLED: Acromicric dysplasia (AD) is an autosomal dominant disorder characterized by short stature, short extremities, stiff joint and skeleton features including brachymetacarpia, cone-shaped epiphyses, internal notch of the femoral head, and delayed bone age. Recently, we identified fibrillin 1 (FBN1) as the disease gene of AD. The aim of our study was to further describe the long-term follow up of AD patients with an emphasis on orthopedic management. Nine patients with FBN1 mutations were included in the study ranging in age from 5.5 to 64 years. For all, detailed clinical and radiological data were available. RESULTS: Birth parameters were always normal and patients progressively developed short stature <-3 SD. Carpal tunnel syndrome was observed in four patients. We found discrepancy between the carpal bone age and the radius and ulna epiphysis bone ages, a variable severity of hip dysplasia with acetabular dysplasia, epiphyseal and metaphyseal femoral dysplasia resembling Legg-Perthes-Calvé disease and variable pelvic anteversion and hyperlordosis. Orthopedic surgery was required in two patients for hip dysplasia, in one for limb lengthening and in three for carpal tunnel syndrome. Our observations expand the AD phenotype and emphasize the importance of regular orthopedic survey.

Voluminous arteriovenous malformation of a child's forearm treated by sequential embolizations: case report.

An arteriovenous malformation (AVM) is a congenital lesion with high vascular flow resulting from direct connections between arteries and veins. Its treatment is often complex, and most authors recommend a multidisciplinary approach combining surgical and endovascular treatments. We report the case of a 6-month-old boy with a voluminous AVM of the left forearm inducing osteolysis of the radius, with bowing of its diaphysis and subsequent radial head dislocation. Surgical excision of the AVM was not possible, but 2 sequential coil embolizations achieved control of the lesion. After 3.5 years, the AVM was undetectable, and notable improvement was noted both in symptoms and radiographic findings. This case underlines how an AVM can have noteworthy influence on surrounding tissues and shows that embolization alone can achieve a satisfying midterm outcome even when surgery is not possible.

Long-term results of treatment of congenital idiopathic clubfoot in 187 feet: outcome of the functional "French" method, if necessary completed by soft-tissue release.

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.

[The reconstruction of limbs after a malignant tumor in children]. / La reconstruction des membres après une tumeur maligne chez l'enfant.

Large segmental resections of malignant bone tumours in children require a reconstruction of the segment which has been removed by oncology surgery. A number of prosthetic and biological techniques are now used. The child's capacity to repair the limb sometimes enables high quality reconstructions to be performed.