Spontaneous induced bone fusion in minimally invasive fusionless bipolar fixation in neuromuscular scoliosis: a computed tomography analysis.

PURPOSE: Posterior spinal fusion (PSF) at skeletal maturity is still the gold standard in children with neuromuscular scoliosis (NMS) who underwent fusionless surgery. The aim of this computed tomography (CT) study was to quantify the spontaneous bone fusion at the end of a lengthening program by minimally invasive fusionless bipolar fixation (MIFBF), that could avoid PSF. METHODS: NMS operated on with MIFBF from T1 to the pelvis and at final lengthening program were included. CT was performed at least five years postoperatively. The autofusion was classified as completely or not fused at the facets joint (on both coronal and sagittal plane, right and left side, from T1 to L5), and around the rods (axial plane, right and left side, from T5 to L5). Vertebral body heights were assessed. RESULTS: Ten patients were included (10.7y ± 2 at initial surgery). Mean Cobb angle was 82 ± 20 preoperatively and 37 ± 13 at last follow-up. CT were performed on average 6.7y ± 1.7 after initial surgery. Mean preoperative and last follow-up thoracic vertebrae height were respectively 13.5 mm ± 1.7 and 17.4 mm ± 1.7 (p < 0.001). 93% facets joints were fused (out of 320 analyzed joints), corresponding to 15/16 vertebral levels. Ossification around the rods was observed in 6.5±2.4 levels out of 13 in the convex side, and 4.2 ± 2.2 in the concave side (p = 0.04). CONCLUSIONS: This first computed quantitative study showed MIFBF in NMS preserved spinal growth, while it induced 93% of facet joints fusion. This could be is an additional argument when questionning the real need for PSF at skeletal maturity.

Primary aneurysmal bone cysts in children: percutaneous sclerotherapy with absolute alcohol and proposal of a vascular classification.

BACKGROUND: Percutaneous sclerotherapy is an effective treatment for aneurysmal bone cysts (ABCs). OBJECTIVE: The purpose of this study was to demonstrate the safety and efficacy of sclerotherapy with absolute alcohol and to propose a vascular classification of ABCs based on a retrospective review. MATERIALS AND METHODS: This was a review of children treated with absolute alcohol sclerotherapy for ABC at a single institution from January 1995 until November 2009. Treatment response was evaluated radiographically and clinically. Cyst fluid was classified as clear, partially bloody, or bloody. Presence of any venous drainage of the cyst was assessed by injection of contrast medium into the cyst cavity. RESULTS: Twenty-nine children with ages ranging from 2 to 16 years were included. Treatment response was good in 17 (59%), partial in 9 (31%), and poor in 3 (10%) children. Venous drainage was absent in six out of seven clear-fluid cysts, which we classified as lymphatic. Drainage was present in all seven bloody-fluid cysts, which we classified as venous. In seven partially bloody-fluid cysts, venous drainage was seen in three. CONCLUSION: Sclerotherapy with absolute alcohol is a safe and effective treatment of ABC. We propose classifying ABC as lymphatic or venous and suggest considering ABC intraosseous slow-flow vascular malformations.

Peripheral nerve injuries of the upper extremity in a pediatric population: Outcomes and prognostic factors.

Peripheral nerve injuries of the upper limb are rare in children and poorly documented. The aim of this retrospective study was to analyze long-term sensory and motor results, and to determine predictive factors for recovery after surgery. Eleven children, with a mean age at injury of 9.7 years (5-15), operated on between 2006 and 2018, were included. Sensory perception was measured on monofilament test and static 2-point discrimination test. Grip strength was measured with a dynamometer and motor strength was assessed on the Medical Research Council scale. Quality of life was assessed on QuickDASH. The injury involved the radial (n = 1), median (n = 9), or combined median and ulnar (n = 1) nerves and was repaired by primary direct suture (n = 11). The mechanism involved glass laceration (n = 10) or a road accident (n = 1). The dominant limb was involved in 7 cases. At a mean 7.7 years' follow-up, touch sensitivity was normal or slightly deficient on monofilament test. Discrimination test was normal or adequate. Strength was complete in 10 patients. Mean QuickDASH score was 5.99 (range, 0-18.18). There was no significant difference in sensory or motor recovery according to partial or complete lesion or to injury location. There was better sensory recovery in children <12 years (p < 0.05). Sensory prognosis was also better in the absence of associated lesions (p < 0.05). Sensory, motor and functional results after surgical treatment of peripheral nerve injuries of the upper limb in children were globally satisfactory. Sensory recovery was better at an early age and in the absence of associated lesions. LEVEL OF EVIDENCE: IV.

Efficacy of fascia iliaca nerve block in daily routine for children with femoral fractures in a pediatric emergency department.

OBJECTIVES: We aimed to evaluate the efficacy of fascia iliaca nerve block (FINB), routinely used for children with femoral fractures, in a pediatric emergency department (PED). METHODS: This retrospective, single-center, observational study examined FINB using ropivacaine and a 1% lidocaine hydrochloride solution, in all patients under 18 years of age admitted with a femoral fracture from January 2012 to December 2016. Pain was assessed using two validated pediatric pain scales: EVENDOL or a visual analog scale. A level of ≥ 4 on either scale indicates the need for an analgesic. The primary outcome was the percentage of patients who were pain free after the FINB procedure defined by a pain score of < 4. Secondary outcomes were the time spent between PED admission and FINB, the need of additional analgesics, side effects, and the success rate of FINB. RESULTS: Of 161 patients screened, 144 were included. The median age was 3.2 years (range 2 months to 16 years) and 74% were boys. The number of children determined to be pain free (pain score < 4) increased from 36 (25%) before the FINB to 123 (85%) after the FINB (absolute risk difference 60%, 95% CI: 51%-70%). Overall, 21 children (15%) required a second analgesic after the FINB. CONCLUSION: The routine use of FINB with ropivacaine and lidocaine by pediatric ED physicians provided effective pain relief for children admitted for a femoral fracture in the emergency department. Our data support the efficiency and feasibility of FINB for the antalgic management of children with femoral fracture.

Radial osteotomy for the correction of forearm deformities in hereditary multiple osteochondroma.

Forearm deformities are often observed in patients with hereditary multiple osteochondroma, resulting in functional disability and cosmetic impairment. The aim of this study was to assess clinical and radiological outcomes after corrective osteotomy of the radius (COR). We performed a retrospective analysis of clinical and radiologic data from patients with forearm deformities who underwent COR combined with osteochondroma resection between 1978 and 2015. Seventeen patients (17 forearms) were included. The mean (range) age at surgery was 11.8 years (3.2-14.4), and the mean interval between surgery and last follow-up was 8.2 years (2-34.2). Range of motion was moderately increased and postoperative radiological assessments found significant improvements in ulnar variance, radial articular angle, bowing of the radius, and carpal slip. At last follow-up, a loss of ulnar variance correction was noted in 11 cases (mean loss: 4mm). The mean score on the Quick Disabilities of the Arm, Shoulder and Hand self-administered questionnaire was 13.9. Our results show that a forearm deformity in a patient with hereditary multiple osteochondroma is an appropriate indication for COR combined with osteochondroma resection and should be performed at the end of growth. This simple, safe technique corrects bowing of the radius and radius-ulna length discrepancy and could limit the risk of radial head dislocation. LEVEL OF EVIDENCE: IV.

Soft tissue sarcoma in children, adolescents and young adults: Outcomes according to compliance with international initial care guidelines.

BACKGROUND: Outcomes for adults with soft tissue sarcoma are better when managed at referral centers. Care guidelines advise for 5 main criteria: 1-Imaging before biopsy; 2-Tumor biopsy before surgery; 3-Multidiscipinary team discussion (MTD) before biopsy; 4-Biopsy in "expert centers"; 5-Somatic molecular biology feasible. The aim is to describe and assess the prognostic impact of initial management of STS according to the type of referring centers and the number of optimal criteria. METHODS: Monocentric retrospective analysis of the management of 127 youths (0-25 years) with localized STS treated from 2006 to 2015. RESULTS: Median age at diagnosis was 9.6 years (range: 025). Overall, only 41% patients had 5/5, 28% 3-4, 31% ≤2. No adequate imaging was performed before surgery/biopsy for 18% patients, no biopsy before treatment for 29%. Patients referred by "expert centers" had higher compliance to guidelines (P = 0.025). Upfront surgery was performed in 59/127 patients. Immediate re-operation was inversely related to the number of criteria (0% when 5 criteria vs. 14% for 3-4, 46% if ≤ 2; P < 0.001). For malignant tumors, outcome was better when 5 criteria were reached: 5 year EFS 90.8% (81.4-100.0%) vs. 71.6 for (60.4-84.9%; ≤4 criteria; p = 0.033), OS 93.6% (85.5-100%) vs. 79.5% (68.9-91.8%; p = 0.11), and LRFFS 90.6% (81.0-100.0) vs. 73.1% (62.0-86.3%; p = 0.047). CONCLUSION: Less than half of the youths with STS are initially managed according to international guidelines, highlighting the need for better information about optimal management. These results plead for immediate management in reference centers to reduce initial burden of therapy.

Nonbacterial purpura fulminans and severe autoimmune acquired protein S deficiency associated with human herpesvirus-6 active replication.

Nonbacterial purpura fulminans (PF) is rare, usually follows viral infection in young children, and is characterized by specific coagulation disorders, requiring specific therapy. Following a transient rash, a 2-year-old previously healthy girl developed PF without haemodynamic impairment. Laboratory data revealed disseminated intravascular coagulation and a severe transient protein S deficiency. Antiprotein S autoantibodies and active human herpesvirus-6 (HHV6) replication were demonstrated. Purpuric skin lesions spread very rapidly despite broad-spectrum antibiotics and right leg amputation. Plasmapheresis and intravenous immunoglobulins gave complete clinical recovery and normalization of protein S level within 10 days, with progressive clearance of antiprotein S autoantibodies. Transient severe protein S deficiencies have previously been reported in patients with nonbacterial PF, usually after varicella infection. This is the first documented case of PF after HHV6 infection.

[Pigmented villonodular synovitis in children: review of six cases]. / La synovite villonodulaire de l'enfant : une revue de six cas.

PURPOSE OF THE STUDY: Pigmented villonodular synovitis is an exceptional condition in children. The clinical, biological and imaging presentation is not specific and a histology sample is required for certain diagnosis. Because of this lack of specificity, together with the rare occurrence of the disease, late diagnosis is not uncommon, making correct management an even greater challenge in the growing child. MATERIAL AND METHOD: Between 1995 and 2001, six children were treated for pigmentary villonodular synovitis, four girls and two boys, mean age 11.5 years. The knee was involved in five cases (three diffuse forms and two localized forms). One diffuse form involved the ankle. The diagnosis was suggested by the MRI findings in all patients and confirmed at the histological examination of a biopsy sample. Surgery was used in five cases and medical treatment in one patient with a diffuse form affecting the knee. A synoviorthesis (Hexatrione) was used systematically in three diffuse forms affecting the knee joint. RESULTS: Mean follow-up was 58 months. Recurrence was noted in two diffuse forms, one involving the knee at 10 months from arthroscopic synovectomy and one involving the ankle 24 months after surgical synovectomy. Systematic use of the synoviorthesis did not prevent recurrence in one case. The synoviorthesis was also used in the two cases of recurrence as a complement to surgical treatment but with no effect. Growth was not affected in any of the children. DISCUSSION: The etiopathogenic mechanism underpinning pigmented villonodular synovitis remains unclear. Genetic factors are suspected in childhood cases. MRI is the complementary examination of choice for diagnosis and follow-up. In children, treatment of pigmented villonodular synovitis depends on marginal excision of the lesion for localized forms and total synovectomy for the diffuse forms. The efficacy of the triamcinolone hexacetonide synoviorthesis remains open to debate and would require a larger series with longer follow-up for evaluation.

[Fibrous dysplasia of the proximal femur in children and teenagers: surgical results in 22 cases]. / La dysplasie fibreuse de l'extrémité proximale du fémur chez l'enfant et l'adolescent: résultats du traitement chirurgical dans 22 cas.

PURPOSE OF THE STUDY: Fibrous dysplasia is a rare benign bone tumor which occurs preferentially in the proximal femur. In children, there is a risk of repeated fractures and coxa vara deformity, particularly in the polyostotic form. The most common orthopedic problem is fibrous dysplasia of the proximal femur which generally requires surgical treatment. The purpose of this study was to analyze clinical and radiological outcome after surgical treatment. MATERIAL AND METHODS: All children who underwent surgical treatment for fibrous dysplasia of the proximal femur between 1979 and 2001 were reviewed retrospectively. The study cohort included 22 children (11 boys and 11 girls). Eight patients had a monostotic form and 14 a polyostotic form of the disease. For the monostotic forms, the type of treatment depended on the size of the tumor and its localization but curettage was used in all cases. For the polyostotic forms, treatment consisted in valgus osteotomy with "humeralization" in the event of associated coxa vara in combination with internal fixation, generally with a centromedullary nail. RESULTS: In the monostotic forms, the clinical outcome was considered good in all cases. Nearly total involution of the tumor was noted in 75% of patients. In the polyostotic forms, osteotomy with "humeralization" and centromedullary nailing provided stable correction of the deformation. Outcome was less satisfactory because of fractures and deformities. DISCUSSION: In light of our results and those reported in the literature, the prognosis of the monostotic form can be considered good after surgical treatment. For the polyostotic form, preventive fixation is necessary. Osteotomy with "humeralization" appears to correct the deformity and prevent coxa vara in certain cases. For the more severe forms, medical treatment with biphosphonates may be a useful complement to the surgical treatment.

Vascularised fibula or induced membrane to treat congenital pseudarthrosis of the Tibia: A multicentre study of 18 patients with a mean 9.5-year follow-up.

BACKGROUND: The objective of this study was to compare outcomes of two surgical techniques used to treat congenital pseudarthrosis of the tibia (CPT), the induced membrane technique (IM) and the transfer of the contralateral vascularised fibula (VF). HYPOTHESIS: The IM technique produces similar outcomes to those of VF grafting in terms of healing and function, while being simpler and having a lower complication rate. MATERIAL AND METHOD: This retrospective multicentre study included 18 patients with a mean age of 2.8 years at surgery. Among them, 11 had neurofibromatosis type 1 (NF1). The IM technique was used in 10 patients and VF grafting in 8 patients. Mean follow-up was 9.5 years (range: 5-15 years). RESULTS: The two groups showed no significant differences for healing or the occurrence of complications such as limb length discrepancy and residual malalignment. Two patients required amputation, one in each group. The mean number of surgical procedures per patient was 4.7 in the IM group and 5 in the VF group. DISCUSSION: Outcomes are similar with the two techniques. Although VF grafting theoretically involves a single stage, the mean number of surgical procedures was not lower than after the IM technique. The IM technique was associated with lower risks of complications and residual donor site abnormalities. Regardless of the reconstruction technique, the quality of the initial bone resection and internal fixation, particularly regarding alignment, is of the utmost importance. LEVEL OF EVIDENCE: IV, comparative retrospective study.

Metacarpal lengthening in children: comparison of three different techniques in 15 consecutive cases.

Metacarpal lengthening is a useful procedure to address hand deficiencies in children. In this study, we aimed to compare the results of three different techniques from one consecutive clinical series of hand deficiencies. A total of 15 metacarpal lengthenings have been performed in 12 children aged from 9 to 14 years. The callotasis technique was used in seven cases, the two-stage distraction-graft technique in four cases and the single-stage lengthening in four cases. All the metacarpals healed with bone. The lengthening obtained was a mean of 13 mm (range 8-21 mm), a mean of 22 mm (range 13-32 mm) and a mean of 12 mm (range 9-15 mm), respectively, in the three different techniques. The healing index was longer for callotasis (81 days/cm) compared with the other techniques (41 days/cm and 46 days/cm, respectively). We observed one case of fracture after callotasis and one after distraction-graft. One patient underwent tenolysis of the extensor mechanism after single-stage lengthening. In conclusion, distraction graft and single-stage lengthening may be valuable alternatives to callotasis. LEVEL OF EVIDENCE: IV; therapeutic study; multi-case series.

Malignant tumours of the foot and ankle.

Most of tumours of the foot are tumour-like (synovial cyst, foreign body reactions and epidermal inclusion cyst) or benign conditions (tenosynovial giant cells tumours, planta fibromatosis). Malignant tumours of the soft-tissue and skeleton are very rare in the foot and their diagnosis is often delayed with referral to specialised teams after initial inappropriate procedures or unplanned excisions. The adverse effect of these misdiagnosed tumours is the increasing rate of amputation or local recurrences in the involved patients. In every lump, imaging should be discussed before any local treatment. Every lesion which is not an obvious synovial cyst or plantar fibromatosis should have a biopsy performed.After the age of 40 years, chondrosarcoma is the most usual malignant tumour of the foot. In young patients bone tumours such as osteosarcoma or Ewing's sarcoma, are very unusually located in the foot. Synovial sarcoma is the most frequent histological diagnosis in soft tissues. Epithelioid sarcoma or clear cell sarcoma, involve more frequently the foot and ankle than other sites. The classic local treatment of malignant conditions of the foot and ankle was below-knee amputation at different levels. Nowadays, with the development of adjuvant therapies, some patients may benefit from conservative surgery or partial amputation after multidisciplinary team discussions.The prognosis of foot malignancy is not different from that at other locations, except perhaps in chondrosarcoma, which seems to be less aggressive in the foot. The anatomy of the foot is very complex with many bony and soft tissue structures in a relatively small space making large resections and conservative treatments difficult to achieve. Cite this article: EFORT Open Rev 2017;2. DOI: 10.1302/2058-5241.2.160078. Originally published online at www.efortopenreviews.org.

[Tillaux fractures in teenagers: a review of nineteen cases]. / Fractures de Tillaux de l'adolescent: etude d'une série de 19 cas.

PURPOSE OF THE STUDY: Tillaux fractures in adolescents correspond to Salter and Harris type III fractures involving the anterolateral portion of the tibial epiphysis. These are intra-articular fractures. The objective was to determine the circumstances of these fractures, the radiological signs, and the therapeutic modalities as well as the long-term clinical and radiological outcome. MATERIAL AND METHODS: We reviewed 19 Tillaux fractures. Ten patients underwent surgical treatment and nine orthopedic treatment following importance of displacement. RESULTS: At mean follow-up of 33.8 months, results were rated good in 17 on 19 cases. DISCUSSION: This fracture is often observed in teenagers victims of trauma with external rotation of the foot. Closure of distal growth cartilage of the tibia occurs medially to laterally, the anterolateral portion remaining open longer. Forced external rotation of the anterior tibiofibular ligament pulls off an anterolateral fragment of the distal tibial epiphysis. Surgical treatment is indicated for fractures with a displacement of more than 2 mm or a vertical displacement to achieve open reduction and screw fixation. Orthopedic treatment is used for non-displaced fractures. CONCLUSION: The prognosis of Tillaux fractures is good as was observed in our series and in series reported in the literature.

[Acute osteomyelitis and septic arthritis in children: one year experience]. / Infections bactériennes ostéoarticulaires du nourrisson et de l'enfant: expérience sur un an.

To describe bacteriologic epidemiology of bone and joint infections, a total of 52 osteomyelitis, 52 arthritis and 20 osteoarthritis of children aged one month to 15 years during a one-year period (2001) were included in a retrospective unicentric review. The mean age was 3,9 +/-3,6 years. Fever and pain were the most common clinical symptoms. The site of infection was single in 95%, involving lower extremities in 80%. Bone scintigraphy was abnormal in 71% of osteomyelitis. Positive cultures was obtained in 29% of all cases (blood cultures: 20%, aspiration cultures: 29%), but in 42% of cases which have both blood and aspiration cultures. Thirty-six bacteria were identified: 19 Staphylococcus (14 aureus), ten Streptococcus (four pneumoniae), three Salmonella, three Kingella kingae, one Moraxella. All the isolates were susceptible to the empiric antibiotic therapy. Outcome was good in 100% of osteomyelitis and in 96% of arthritis.

Bartonella henselae osteoarthritis of the upper cervical spine in a 14-year-old boy.

We report a case of Bartonella henselae, an agent of cat scratch disease, C1-C2 osteoarthritis with osteolysis of the lateral mass of C2 in a 14-year-old boy. Oral antibiotics did not successfully treat the infection and surgery was necessary to treat the septic arthritis. The case opens discussion about bacterial osteoarthritis of the cervical spine and bone involvement in disseminated bartonellosis.

[Pseudotumoral soft tissue masses in children and adolescents]. / Masses des tissus mous d'allure tumorale de l'enfant et de l'adolescent.

INTRODUCTION: Pseudotumoral soft tissue masses in children and adolescents are a frequent reason for consultation and a diagnostic dilemma. Soft tissue malignancies are relatively uncommon, unlike the large number of benign lesions that may be seen in the superficial tissue and that can be diagnosed with clinical characteristics. MATERIALS AND METHODS: This retrospective study concerns 161 children and adolescents less than 20 years old, referred for a soft tissue mass between 2007 and 2011. It describes their epidemiology, clinical characteristics, and course of care to validate a diagnostic strategy for such masses. RESULTS: Final diagnoses were malignant tumors (44%), benign tumors (32%), and pseudotumoral lesions (24%). Clinical features were similar between these three groups except for age and tumor location, with more benign thoracic masses in younger children. Clinical and radiological association led to an accurate diagnosis for 50% of benign masses and with cytological analysis contribution in 79% of benign tumors and 86% of pseudotumoral lesions. Malignant tumors were suspected in only 39% of cases with radiological exams and in 89% after fine-needle aspiration, an essential additional diagnostic tool. Final diagnoses were formally established through simple standard clinical and radiological evaluation in 19 patients (11.8%; benign tumors, seven patients; malformations, eight patients; post-traumatic lesions, two patients; infection and inflammation, one patient each); ultrasound exam in five patients (3.1%; hemangioendotheliomas, two patients, fascial dehiscence, hemangioma, and vascular malformation, one patient each); MRI in four patients (2.5%; three vascular malformations and one lipoma); CT in two cases (1.2%; vascular malformation and myositis ossificans), and radiological examinations associated with cell aspiration in 15 cases (9.3%; ten benign tumors and five malignant tumors). CONCLUSIONS: A multidisciplinary approach should be requested from oncological, radiological, and pathologic experts to optimize soft tissue mass management as soon as initial investigations start. The authors advise a diagnostic strategy for children with pseudotumoral soft tissue masses.

[Late syringomyelic syndromes in paraplegics]. / Syndromes syringomyéliques tardifs chez les paraplégiques.

The authors have reviewed 35 cases in the literature and reported on 12 cases seen personally of late onset syringomyelia in paraplegics. Initial pain was found to be present in only slightly more than half the cases and an insidious onset particularly with sensory disturbance led sometimes to delayed recognition. In the fully developed case, it can closely simulate idiopathic syringomyelia with arthropathy but impairment of touch and proprioception sometimes occurs in addition. Neurosurgical treatment to the fluid-filled cavity, does not seem to modify obviously the spontaneous evolution of the condition. In 5 cases, the paraplegia was not of traumatic origin. Possible mechanisms are discussed.

[The affected medullary segment in paraplegics. Relation to sexual function in men (author's transl)]. / Le segment médullaire lésionnel chez le paraplégique. Applications à la fonction génito-sexuelle chez l'homme.

The authors made a detailed clinical analysis of 119 stabilized total paraplegics in order to define, as precisely as possible, the limits of the medullary segment which, for each patient, had lost all its functional value. The relationships between the affected segments and the sexual capabilities of these patients show that the segment must: --exclude the sacral spinal cord if reflex erections are to occur; --underly Th. IX at least, or better still Th. XI, for psychogenic turgescence to appear; the efferent pathways seem therefore, to emerge from the cord quite close to the level which receives testicular afferent pathways; --underly or overly Th. XII, L I, and L II for at least the greater part, for emission of sperm to occur; a true abrupt ejaculation probably requires the complementary integrity of the sacral spinal cord.

[Paraplegia and bilharziasis. A propos of 4 cases (including 2 confirmed cases of bilharzial myelitis]. / Paraplégie et bilharziose. A propos de quatre observations (dont deux de myélite bilharzienne certaine).

Four observations of paraplegia of bilharzians are presented. In two of them the anatomical proof of medullar localization of parasitosis was obtained, once in vivo, only once after a post-mortem. Medical study enables us to spot 55 cases of bilharzian paraplegia, of which 25 sufficiently explicit on the clinical point of view, indicated medullar parasitosis. A parasitosis diagnosis must especially therefore be sought out with the greatest care so that the specific and remarkably active treatment may be started as soon as possible.