Deep cervical abscesses in children: efficacy of the cefotaxime-rifampicin combination.

The objective is to determine whether a medical treatment, the combination of cefotaxime and rifampicin, is effective in avoiding surgery for managing deep cervical abscesses in children and to determine prognostic factors in the efficacy of this medical treatment. This is a retrospective analysis of all patients under 18 presenting with para- or retro-pharyngeal abscess over the period 2010-2020 in two hospitals' pediatric otorhinolaryngology departments. One hundred six records were included. Multivariate analyses were performed to study the relationship between the prescription of the Cefotaxime-rifampicin protocol at the onset of the management and the use of surgery and to evaluate the prognostic factors of its efficacy. The 53 patients who received the cefotaxime-rifampicin protocol as first-line treatment (vs. 53 patients receiving a different protocol) required surgery less frequently: 7.5% versus 32.1%, validated by a Kaplan-Meier survival curve and a Cox model analysis adjusted for age and abscess size (Hazard Ratio = 0.21). This good outcome of the cefotaxime-rifampicin protocol was not demonstrated when it was instituted as a second-line treatment after the failure of a different protocol. An abscess larger than 32 mm at hospitalization was significantly associated with more frequent use of surgery in multivariate analysis adjusted for age and sex (Hazard Ratio = 8.5).    Conclusions: The cefotaxime-rifampicin protocol appears to be an effective first-line treatment in managing non-complicated deep cervical abscesses in children. What is Known: • Nowadays, medical treatment is preferred for managing deep neck abscesses in children. There has yet to be a consensus on the antibiotic therapy to be proposed. • Staphylococcus aureus and streptococci are the most frequent causative organisms. What is New: • The cefotaxime-rifampicin protocol introduced at first intention is effective, with only 7.5% of patients requiring drainage surgery. • The only risk factor for failure of the medical treatment is the initial size of the abscess.

Evaluation of preoperative cutaneous fistula as a risk factor for recurrence of thyroglossal duct cyst in children.

The aim of this study is to investigate the risk factors for recurrence after thyroglossal duct cyst (TGDC) surgery, differentiating between infections with and without a cutaneous fistula. This is a retrospective analysis of all paediatric TGDC in a tertiary care centre with at least 2 years of postoperative follow-up. One hundred and thirty-one patients met the inclusion criteria of the study. A multivariate analysis was performed to analyse the main risk factors for recurrence (presence of a fistula, infection, age). 116/131 patients were managed primarily in our institution; 15 patients had previously been operated on in another centre. The recurrence rate was 4.3% (5/116) when the patient was operated on in our institution at the first operation, and overall, recurrence of TGDC occurred in 20/131 (15.3%) patients. Age was not a risk factor for recurrence (p = 0.596). Two or more episodes of preoperative TGDC infection were a statistically significant risk factor in univariate analysis (p = 0.021) but not in multivariate analysis adjusted for age and the presence of a cutaneous fistula (p = 0.385). In multivariate analysis, cutaneous fistula formation was an independent risk factor for recurrence when adjusted for age and preoperative TGDC infection (Hazard ratio = 5.35; p = 0.011). CONCLUSIONS: A preoperative cutaneous fistula was a critical and independent risk factor for recurrence of operated TGDC, whereas age and TGDC infection were not identified as risk factors for recurrence after surgery. This information should be given to patients and parents before surgery. WHAT IS KNOWN: • The risk factors for recurrence after thyroglossal duct cyst surgery described in the literature are preoperative infection and young age, but this is not supported by strong evidence. • The role of cutaneous fistula formation is unclear. WHAT IS NEW: • The main risk factor for recurrence of TGDC is the presence of a preoperative cutaneous fistula, with an estimated hazard ratio of 4.95 (p = 0.016) in multivariate analysis. • The presence of two preoperative infections was also associated with a greater risk of recurrence in univariate analysis; age and gender were not associated with an increased risk of recurrence.

Submucosal thulium laser turbinoplasty in children: assessment of efficacy and comparison with partial inferior turbinectomy.

This study aimed to assess efficacy of submucosal thulium laser turbinoplasty in children and to compare thulium laser turbinoplasty with partial inferior turbinectomy. This is a retrospective study over 10 years from 1 January 2009, conducted in a Pediatric Otorhinolaryngology Department in a university tertiary care center, including all the children who underwent thulium laser inferior turbinoplasty and partial inferior turbinectomy. The main outcome measures were the improvement in airflow using rhinomanometric cumulative flow at 150 Pa and then functional improvement using "OPERAS" score (nasal Obstruction, facial Pain, Epistaxis, Rhinorrhea, Anosmia, Sneezing). Eighty-six children underwent 47 submucosal thulium laser turbinoplasties and 48 partial turbinectomies. After submucosal thulium laser turbinoplasty, the cumulative flow at 150 Pa improved from 308.2 to 454.4 cm3 s-1 (p < 0.01) and the OPERAS score decrease was 1.5 point (p < 0.01). Daily use of intra nasal corticosteroid and antihistamine both decreased significantly. No local complications of this technique have been reported. Partial turbinectomies show a higher reduction of the OPERAS score and a better improvement of rhinomanometric parameters, but also a significantly higher complication rate. Submucosal thulium laser turbinoplasty is a good compromise between safety and efficacy, with substantial functional and rhinomanometric postoperative improvement.

Inspiratory stridor of newborns and infants admitted to a paediatric ENT outpatient clinic: diagnostic approach and therapeutic outcome.

The main objective was to analyse the use of rigid laryngotracheoscopy under general anaesthesia (GA) and endoscopic surgery in the management of inspiratory stridor in patients referred to a paediatric ENT outpatient clinic. The secondary objective was to analyse the aetiological diagnoses made and their therapeutic management. This is a prospective study including all newborns and infants, corresponding to 190 patients, presenting for the first time in consultation for inspiratory stridor from January 2015 to December 2017. A consultation form was filled out after each consultation and added to a database; a management algorithm was used to determine which patients required a rigid laryngotracheoscopy. A 17.9% (n = 34) of the patients required rigid laryngotracheoscopy, of whom 12.6% (n = 24) underwent concomitant endoscopic surgery. A 65.8% (n = 125) of the patients were diagnosed with laryngomalacia, 21.1% (n = 40) with isolated posterior excess of mucosa, 9.5% (n = 18) with another diagnosis and 3.7% (n = 7) with a normal examination. The presence of comorbidity was associated (p < 0.001) with the use of rigid laryngotracheoscopy and endoscopic surgery.Conclusion: Rigid laryngotracheoscopy under GA was required in one in five to six patients. Conservative management with strict follow-up may be appropriate in a large number of patients, especially those with laryngomalacia. What is Known: • Previous research has established that laryngomalacia is the main aetiology of stridor. • Comorbidities are linked with a poor tolerance of stridor. What is new: • About one in five to six patients seen in consultation for stridor will require a trip to the operative room (and one in eight will require endoscopic surgery). • Laryngomalacia and isolated posterior excess of mucosa account for 85-90% of the patients seen in consultation for stridor.

Fusobacterium necrophorum, a major provider of sinus thrombosis in acute mastoiditis: A retrospective multicentre paediatric study.

OBJECTIVE: To evaluate in children the clinical severity and evolution of otogenic lateral sinus thrombosis (OLST) due to Fusobacterium necrophorum compared with other bacterial otogenic thrombosis and propose a specific management flowchart for Fusobacterium OLST. DESIGN: A retrospective multicentre cohort study. SETTINGS: Four French ENT paediatric departments. PARTICIPANTS: A total of 260 under 18 years old admitted for acute mastoiditis were included. Initial imaging was reviewed to focus on complicated mastoiditis and 52 OLST were identified. Children were then divided into two groups according to bacteriological results: 28 in the "OLST Fusobacterium group" and 24 in the "OLST other bacteria group". RESULTS: There was a significant association between F necrophorum and OLST (P < .001). When compared to the OLST other bacteria group, children in the OLST Fusobacterium group were significantly younger (61 months vs 23 months, P < .01) and had a more severe clinical presentation: higher CRP (113 mg/L vs 175.7 mg/L, P = .02) and larger subperiosteal abscess (14 mm vs 21 mm, P < .01). Medical management was also more intensive in the OLST Fusobacterium group than in the OLST other bacteria group: increased number of conservative surgeries (66.7% vs 92.9%, P = .03) and longer hospital stay (13.7 days vs 19.8 days, P = .02). At the end of follow-up, the clinical course was good in both groups without any neurological sequelae. CONCLUSIONS: Thrombotic complications are very frequent in case of Fusobacterium mastoiditis and clinicians should be aware of the initial severity of the clinical presentation. Under appropriate management, the clinical course of Fusobacterium OLST is as good as that of other bacterial otogenic thrombosis.

Prognostic Factors and Management of Patients with Choanal Atresia.

OBJECTIVE: To analyze prognostic factors in the management of patients with choanal atresia. STUDY DESIGN: This is a review of 114 patients operated on for choanal atresia in a tertiary care center between November 1986 and November 2016, including clinical characteristics, surgical management, and postoperative course with final nasal patency. The data were collected in a database that was updated over time. RESULTS: Among the 114 patients, 78 were female, 77 presented with unilateral choanal atresia, and 37 presented with bilateral unilateral choanal atresia, corresponding to 151 nasal fossae. Forty-seven patients had associated abnormalities (41.2%), including CHARGE, diagnosed in 20 children (17.5%). At the end of follow-up, 91 patients (79.8%) had normal choanal patency. The identified risk factors of surgical failure were age <6 months (P = .004), weight <5 kg (P = .007), and bilateral choanal atresia (P < .001). The type of atresia, presence of associated abnormalities, surgical approach, stenting, and use of mitomycin were not significantly linked with the surgical results. CONCLUSIONS: This series highlights the importance of the timing of surgery, which is guided by the clinical ability of the infant to tolerate the procedure. Endoscopic approaches are widely performed, but a transpalatal approach, necessary in some cases of bilateral choanal atresia, does not alter the results. Unilateral choanal atresia surgery should be delayed after age 6 months and/or weight >5 kg when possible.

Computational fluid dynamics (CFD), virtual rhinomanometry, and virtual surgery for neonatal congenital nasal pyriform aperture stenosis.

OBJECTIVES: Investigate the implications of Congenital Nasal Pyriform Aperture Stenosis (CNPAS) on neonatal nasal airflow through computational fluid dynamics (CFD), create a virtual rhinomanometry, and simulate the prospective outcomes post-virtual surgical intervention. METHODS: CT scanning of a neonate diagnosed with CNPAS and a control model were used to execute CFD simulations. The segmentation file of the CNPAS underwent manual modifications to simulate a virtual surgical procedure, resulting in a geometry that mirrors a post-operatively corrected patient. Virtual rhinomanometry was reconstructed, and airflow dynamics within the nasal cavity were systematically assessed. The results of the three models were compared. RESULTS: In the CNPAS model, airflow dynamics underwent discernible alterations, with the principal airflow corridor confined to the nasal cavity's upper region. There was a marked pressure drop around the nasal valve, and diminished velocities. This first model of virtual surgery has allowed us to observe that the airflow parameters trended toward the control model, reintroducing an airflow trajectory between the lower and middle turbinates. Virtual rhinomanometry presented near-complete nasal obstruction in the CNPAS model, which showed considerable improvement after the virtual surgery. CONCLUSION: CFD highlights the aerodynamic changes resulting from CNPAS. It also allows for the creation of virtual rhinomanometry and the performance of virtual surgeries. Virtual surgery confirms the therapeutic potential of pyriform aperture enlargement techniques used in clinical practice to improve nasal respiratory function. Future research will investigate additional surgical scenarios and the application of these findings to optimize surgical interventions for CNPAS.

Assessment of high-flow nasal cannula efficacy in humidification of infant airways: A computational fluid dynamics approach.

INTRODUCTION: High-flow nasal cannula therapy has garnered significant interest for managing pathologies affecting infants' airways, particularly for humidifying areas inaccessible to local treatments. This therapy promotes mucosal healing during the postoperative period. However, further data are needed to optimize the use of these devices. In vivo measurement of pediatric airway humidification presents a challenge; thus, this study aimed to investigate the airflow dynamics and humidification effects of high-flow nasal cannulas on an infant's airway using computational fluid dynamics. METHODS: Two detailed models of an infant's upper airway were reconstructed from CT scans, with high-flow nasal cannula devices inserted at the nasal inlets. The airflow was analyzed, and wall humidification was modeled using a film-fluid approach. RESULTS: Air velocities and pressure were very high at the airway inlet but decreased rapidly towards the nasopharynx. Maximum relative humidity-close to 100%-was achieved in the nasopharynx. Fluid film development along the airway was heterogeneous, with condensation primarily occurring in the nasal vestibule and larynx. CONCLUSION: This study provides comprehensive models of airway humidification, which pave the way for future studies to assess the impact of surgical interventions on humidification and drug deposition directly at operative sites, such as the nasopharynx or larynx, in infants.

Tympanic Perforations in Children: When to Propose Surgical Closure?

OBJECTIVE: This study aims to analyze the impact of age and other prognostic factors on the success of myringoplasty. STUDY DESIGN: A retrospective case series. SETTINGS: Pediatric ENT department of a tertiary academic center. PATIENTS: Two hundred forty-one children (318 ears) aged 3 to 17 years with tympanic perforation. INTERVENTION: Myringoplasty performed between 2009 and 2019. MAIN OUTCOMES MEASURES: The rate of tympanic closure, perforation recurrence, revision surgery, and audiometric gain were collected. The impact of age and anatomical and surgical factors was analyzed for each procedure. RESULTS: With a mean follow-up time of 1 year, the tympanic closure rate was 87.7%, the perforation recurrence rate was 18.6%, and 16.7% of ears required reoperation. The mean air-bone gap decreased from 21 dB preoperatively to 12 dB postoperatively ( p < 0.0001). We did not find different anatomical and audiometric results for our three groups of patients classified according to age. Audiometric results were associated with the location of the perforation, intraoperative inflammation of the middle ear mucosa, and the surgical technique performed. CONCLUSION: Myringoplasty in children is associated with excellent anatomical and functional results, even in the youngest patients. It can be proposed whatever the child's age if the patients are well selected before giving the indication.

The role of radiation therapy in pediatric mucoepidermoid carcinomas of the salivary glands.

OBJECTIVE: To investigate the role of radiation therapy in rare salivary gland pediatric mucoepidermoid carcinoma (MEC). STUDY DESIGN: A French multicenter retrospective study (level of evidence 4) of children/adolescents treated for MEC between 1980 and 2010 was conducted. RESULTS: Median age of the 38 patients was 14 years. Parotid subsite, low-grade, and early primary stage tumors were encountered in 81%, 82%, and 68% of cases, respectively. All except 1 patient were treated by tumoral surgical excision, and 53% by neck dissection (80% of high grades). Postoperative radiation therapy and chemotherapy were performed in 29% and 11% of cases. With a median 62-month follow-up, overall survival and local control rates were 95% and 84%, respectively. There was 1 nodal relapse. Lower grade and early stage tumors had better survival. Postoperative radiation therapy and chemotherapy were associated with similar local rates. Patients with or without prior cancer had similar outcomes. CONCLUSIONS: Pediatric salivary gland MEC carries a good prognosis. Low-intermediate grade, early-stage tumors should be treated with surgery alone. Neck dissection should be performed in high-grade tumors. Radiation therapy should be proposed for high grade and/or advanced primary stage MEC. For high-grade tumors without massive neck involvement, irradiation volumes may be limited to the primary area, given the risk of long-term side effects of radiation therapy in children. Pediatric MEC as second cancers retain a similar prognosis. Long-term follow-up is needed to assess late side effects and second cancers.

Rhabdomyosarcoma of Stensen's duct in children.

OBJECTIVES: Stensen's duct is a very uncommon location for rhabdomyosarcoma. The purpose of this article was to review the clinical history of 2 patients who had rhabdomyosarcoma of Stensen's duct. METHODS: We reviewed the clinical history, imaging studies, histologic analysis, and treatment of 2 patients with rhabdomyosarcoma of Stensen's duct. RESULTS: An 8-year-old boy (case 1) and a 17-year-old boy (case 2) presented with nonspecific facial swelling. In both patients, imaging studies showed a tumor at Stensen's duct, and biopsy showed embryonal rhabdomyosarcoma. Both patients were treated with preoperative chemotherapy, parotidectomy, and resection of Stensen's duct and postoperative chemotherapy and radiation therapy. Follow-up at 9 years (case 1) and 2 years (case 2) after surgery showed that the patients were free of disease. CONCLUSIONS: Stensen's duct rhabdomyosarcoma is rare and may have a better prognosis than rhabdomyosarcoma in other locations in the head and neck.

Heliox simulations for initial management of congenital laryngotracheal stenosis.

OBJECTIVES: Congenital laryngotracheal stenosis is rare, potentially severe, and difficult to manage. Heliox is a medical gas effective in obstructive airway pathologies, given its physical properties. This study aims to model the interest of Heliox in reducing the respiratory work in congenital laryngotracheal stenosis, using numerical fluid flow simulations, before considering its clinical use. DESIGN: This is a retrospective study, performing Computational Fluid Dynamics numerical simulations of the resistances to airflow and three types of Heliox, on 3D reconstructions from CT scans of children presenting with laryngotracheal stenosis. PATIENTS: Infants and children who were managed in the Pediatric ENT department of a tertiary-care center and underwent CT scanning for laryngotracheal stenosis between 2008 and 2018 were included. RESULTS: Fourteen models of congenital laryngotracheal stenosis were performed in children aged from 16 days to 5 years, and one model of the normal trachea in a 5-year-old child. Tightest stenosis obtained the highest airway resistances, ranging from 40 to 10 kPa/L/s (up to 800 times higher than in the normal case). Heliox enabled a decrease in pressure drops and airway resistances in all stenosis cases, correlated to increasing Helium concentration. CONCLUSIONS: Heliox appears to reduce pressure drops and airway resistances in 3D models of laryngotracheal stenosis. It may represent a supportive treatment for laryngotracheal stenosis, while waiting for specialized care, thanks to the reduction of respiratory work.

Drug-Induced sleep endoscopy in children: NAVOTEL scoring system development.

OBJECTIVES: Pediatric drug-induced sleep endoscopy (DISE) lacks a universal and easy-to-use scoring system. The velum, oropharynx, tongue, epiglottis (VOTE) scoring system is widely used but needs to be completed in pediatrics. The main objective of this study was to investigate the distribution of obstructive sites in DISE and to propose an appropriate pediatric scoring system. The secondary objective was to evaluate the changes in surgical management induced by the proposed scoring system. METHODS: A single-center prospective 5-year study was conducted from March 2016 to December 2021, including 99 children with a mean age of 7.2 years (±3.7), with pathological preoperative sleep recordings and undergoing DISE. The distribution of all upper airway obstructive sites was studied. RESULTS: Adenoids (A) were the most frequent obstructive site (63% of patients), and the nasal cavities (N) and the larynx (L) were other frequent obstructive sites. These sites are not explored by the VOTE scoring system, leading to the creation of the nose, adenoids, velum, oropharynx, tongue, epiglottis, larynx (NAVOTEL) scoring system. NAVOTEL was significantly correlated with the severity of obstructive sleep apnea-hypopnea syndrome (OSAS) (ρ = 0.2; p = 0.04) and highlighted obstructive sites in 6/9 patients with VOTE = 0. Of these patients, 4 had a complete obstructive site, and 3 had a multisite obstruction. VOTE indicated 8 additional surgical actions; NAVOTEL indicated 50 other actions compared to clinical examination. The NAVOTEL scoring system was exhaustive regarding surgical indications for OSAS. CONCLUSIONS: The NAVOTEL scoring system is exhaustive in pediatric DISE and correlated to OSAS severity. It should be preferred in pediatric DISE.

Obstructive Sleep-Disordered Breathing in Infants with Normal Awake Clinical Examination: Contribution of Drug-Induced Sleep Endoscopy.

OBJECTIVES: To determine the cause of obstructive sleep-disordered breathing using drug-induced sleep endoscopy (DISE) in infants without co-morbidities and with normal clinical examination. METHODS: This prospective study was conducted between December 2019 and June 2022 (31 mo). All infants referred for obstructive sleep-disordered breathing with normal clinical examination and flexible laryngoscopy were included and underwent DISE. The location of the obstructive sites was scored similarly according to the NAVOTEL scoring system, previously established and validated by authors' team. RESULTS: Thirty-two infants were included. DISE identified an obstructive site in 94% of cases. The mean age was 16.4 mo, and the median was 16.7 mo. The obstructive sites found were isolated sleep laryngomalacia (37.4%), adenotonsillar hypertrophy (21.8%), isolated adenoidal hypertrophy (6.3%), adenotonsillar hypertrophy associated with sleep laryngomalacia (6.3%), circumferential upper airway narrowing (6.3%), glossoptosis (6.3%), isolated inferior turbinate hypertrophy (3.1%), adenoidal and tongue base hypertrophy (3.1%), and adenoidal hypertrophy with sleep laryngomalacia (3.1%). No obstructive causes could be found in 2 cases (6.3%). DISE identified an obstructive site in 30/32 patients (93.8%) and guided the surgical management in 26/32 cases (81.3%) during the same general anesthesia. The infants without surgical obstacles were referred for medical treatment. CONCLUSIONS: DISE is an excellent diagnostic and pre-therapeutic tool in infants with no apparent cause at the awake examination to identify the obstructive sites and guide the treatment.

The very first cry: a multidisciplinary approach toward a model.

OBJECTIVES: In previous work, we showed that a rigid larynx-like geometry can generate a sound by itself. However, very little is known about the exact mechanisms and control of the larynx during the first cry of life. The goal of this work was to understand how the very first cry is generated. METHODS: Simultaneous high-speed imaging and sound recording on 2 excised 38-week term human fetus larynges were performed. The behaviors of the vocal folds and the false vocal folds were studied separately. The behavior of the vocal folds after resection of the supraglottic structures was also analyzed. A comparative acoustic analysis of the first cry and of the sound generated by the excised organs was performed. RESULTS: Our data showed that the vocal folds in a larynx with the pressure conditions of the first cry do not generate sound themselves, but induce aerodynamic conditions leading to vibrations of other parts of the larynx. CONCLUSIONS: The similarities between the sound generated by an excised larynx and the first cry suggest a lack of neurologic control of the larynx during production of the first cry. A model-algorithm is proposed.

International Pediatric Otolaryngology Group (IPOG) consensus recommendations: Evaluation and management of congenital tracheal stenosis.

OBJECTIVES: To outline an expert-based consensus of recommendations for the diagnosis and management of pediatric patients with congenital tracheal stenosis. METHODS: Expert opinions were sought from members of the International Pediatric Otolaryngology Group (IPOG) via completion of an 18-item survey utilizing an iterative Delphi method and review of the literature. RESULTS: Forty-three members completed the survey providing recommendations regarding the initial history, clinical evaluation, diagnostic evaluation, temporizing measures, definitive repair, and post-repair care of children with congenital tracheal stenosis. CONCLUSION: These recommendations are intended to be used to support clinical decision-making regarding the evaluation and management of children with congenital tracheal stenosis. Responses highlight the diverse management strategies and the importance of a multidisciplinary approach to care of these patients.

[Hearing disorders in 3- to 12-year-olds]. / L'audition de l'enfant de 3 à 12 ans.

The hearing disorders of the child between 3 and 12 years are dominated by the conductive hearing loss. The questioning, looking for risk factors, as well as the otoscopy are essential to detect and diagnose a hearing disorder. Screening tests help to identify the children with risk of hearing loss. Then, the child is sent for diagnosis towards an ENT specialist or a specialized structure. In spite of the frequency of the conductive hearing loss, in case of the slightest doubt, a sensorineural hearing loss must be always looked for by a puretone audiometry examination via the headphones.

International Pediatric Otolaryngology Group (IPOG) survey: Efforts to avoid complications in home tracheostomy care.

OBJECTIVE: To provide guidance for home care tracheostomy management in the pediatric population. The mission of the IPOG is to develop expertise-based recommendations for the management of pediatric otolaryngologic disorders with the goal of improving patient care. METHODS: Survey of expert opinion by the members of the International Pediatric Otolaryngology Group (IPOG). RESULTS: Survey results provide guidance for caregiver teaching, the reuse of tracheostomies and suction catheters while inpatient and following discharge, acceptable sterilization practices for tracheostomies, tracheitis workup and management, and outpatient follow-up practices. CONCLUSION: This presentation of common home tracheostomy care practices are aimed at improving patient-centered care in the pediatric population.

Locoregional Control and Survival in Children, Adolescents, and Young Adults With Localized Head and Neck Alveolar Rhabdomyosarcoma-The French Experience.

INTRODUCTION: The head and neck (HN) are the most frequent sites of pediatric rhabdomyosarcoma (RMS). Alveolar RMS (ARMS) represents ~20% of all RMS cases and frequently spread to lymph nodes (LNs). The aim was to report locoregional control, event-free survival (EFS), and overall survival (OS), according to clinical and pathological features, LN staging, and treatment modalities. METHODS: The study included all patients prospectively enrolled in EpSSG RMS 2005 study under 21 years of age with localized HN ARMS and diagnosed between 2005 and 2016 in France. Medical data including imaging, surgical report, and radiation therapy planes were analyzed. RESULTS: Forty-eight patients (median age 6 years; range 4 months-21 years), corresponding to 30 parameningeal and 18 non-parameningeal ARMS, were included. There were 33 boys (69%). Tumor locations included the following: orbit (n = 7) among which four cases had bone erosion, paranasal sinuses and nasal cavity (n = 16), deep facial spaces (n = 10), nasolabial fold (n = 8), and other non-parameningeal HN sites (n = 7). A fusion transcript of PAX3-FOXO1 or PAX7-FOXO1 was expressed in 33 of the 45 cases (73%) with molecular analysis. At diagnosis, 10 patients had primary resection of the primary tumor (PRPT) (none with microscopic complete resection) and 9 had LN staging. After induction chemotherapy, 26 patients (54%) had secondary resection of the primary tumor (SRPT) and 13 patients (27%) had cervical LN dissection. A total of 43 patients (90%) were treated with radiation therapy.With a median follow-up of 7 years (range 2-13 years), 5-year OS and EFS were 78% (95% CI, 63-88%) and 66% (95% CI, 51-78%), respectively. We observed 16 events (10 deaths): 4 local, 4 regional, 1 local and regional, and 7 metastatic. In univariate analysis, OS was only superior for patients under 10 years of age (p = 0.002), while FOXO1-negative ARMS, SRPT for parameningeal ARMS, and LN surgery were associated with significantly better EFS. CONCLUSION: Our study confirms a better outcome for fusion-negative ARMS and ARMS in children under 10 years. Moreover, LN surgery and SRPT of parameningeal tumor may improve EFS of ARMS. Larger studies are needed to confirm our findings.