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Introduction: Non-tuberculous mycobacterial (NTM) infection commonly manifests as subacute or chronic cervicofacial lymphadenitis in immunocompetent children. The optimal management of this pathology remains controversial. Objectives: This international consensus guideline aims to understand the practice patterns for NTM cervicofacial lymphadenitis and to address the primary diagnostic and management challenges. Methods: A modified three-iterative Delphi method was used to establish expert recommendations on the diagnostic considerations, expectant or medical management, and operative considerations. The recommendations herein are derived from current expert consensus and critical review of the literature. Setting: Multinational, multi-institutional, tertiary pediatric hospitals. Results: Consensus recommendations include diagnostic work-up, goals of treatment and management options including surgery, prolonged antibiotic therapy and observation. Conclusion: The recommendations formulated in this International Pediatric Otolaryngology Group (IPOG) consensus statement on the diagnosis and management of patients with NTM lymphadenitis are aimed at improving patient care and promoting future hypothesis generation. Keywords: Atypical mycobacteria; Cervical lymphadenitis; Consensus recommendations; Non-tuberculous mycobacteria.

IntroductionSevere sialorrhoea is a common, distressing problem in children/adolescents with neurodisabilities, which has adverse health and social consequences. The SALIVA trial is designed to evaluate the efficacy and safety of a paediatric-specific oral solution of glycopyrronium along with its impact on quality-of-life (QoL), which has been lacking from previous trials of sialorrhoea treatments.Methods and analysisA double-blind, placebo-controlled, randomised phase IV trial is ongoing in several centres across France. Eighty children aged 3–17 years with severe sialorrhoea (≥6 on the modified Teachers Drooling Scale) related to chronic neurological disorders in whom non-pharmacological standard of care has already been implemented or has failed, will be recruited. Patients will be randomised 1:1 to receive a 2 mg/5 mL solution of glycopyrronium bromide (Sialanar 320 µg/mL glycopyrronium) or placebo three times daily during a 3-month blinded period. After Day 84, participants will be invited into a 6-month, open-label study extension period, where they will all receive glycopyrronium. The primary endpoint of the double-blind period will be the change from baseline to Day 84 in the Drooling Impact Scale (DIS), a validated measure to assess sialorrhoea. A series of secondary efficacy endpoints involving change in total DIS, specific DIS items and response (DIS improvement ≥13.6 points) will be analysed in a prespecified hierarchy. QoL data will be collected from parents, caregivers and patients where possible using specific DIS questions and DISABKIDS questionnaires. Safety endpoints, including adverse events, will be assessed throughout the trial periods.Ethics and disseminationIn total, 87 children have been recruited and recruitment is now complete. Final results are expected by the end of 2023. Findings will be presented at conferences and published in peer-reviewed journals.Trial registration numberEudraCT 2020-005534-15.

Objective To provide guidance for the comprehensive management of children referred for anterior drooling. The mission of the International Pediatric Otolaryngology Group (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). The recommendations are derived from current expert consensus and critical review of the literature. Results Consensus recommendations include initial care and approach recommendations for health care providers who commonly evaluate children with drooling. This includes evaluation and treatment considerations for commonly debated issues in drooling management, initial work-up of children referred for anterior drooling, treatment recommendations, indications and contra-indications for rehabilitation, medical, and surgical management, as well as pros and cons of different surgical procedures in the hands of drooling management experts. Conclusion Anterior drooling consensus recommendations are aimed at improving patient-centered care in children referred for sialorrhea.

Resume Objectifs Les auteurs exposent la recommandation pour la pratique clinique de la Societe francaise d’oto-rhino-laryngologie et de chirurgie de la face et du cou (SFORL) concernant le role de l’ORL dans la prise en charge du syndrome d’apnees-hypopnees obstructives (SAHOS) de l’enfant. Ce chapitre concerne la place des enregistrements du sommeil dans le diagnostic du SAHOS. Methodes Un groupe de travail multidisciplinaire a ete charge d’effectuer une revue de la litterature scientifique sur la thematique etudiee. A partir des articles selectionnes et de l’experience de chacun, des recommandations ont ete redigees et classees en grade A, B ou C ou Accord professionnel selon un niveau de preuve scientifique decroissant, puis relues par un groupe de lecture independant du groupe de travail. Resultats Les enregistrements du sommeil sont presentes selon la classification de l’American Sleep Disorders Association en type 1, 2, 3 ou 4. Leurs modalites de realisation, d’interpretation, indications, avantages et limites sont detailles.

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.

Resume Introduction Les anti-inflammatoires non steroidiens (AINS) sont connus pour inhiber le chimiotactisme, l’explosion oxydative et la phagocytose, la bactericidie dans les granulocytes et pour inhiber l’agregation ou la degranulation des neutrophiles et perturber la fonction des lymphocytes. D’autre part, l’ibuprofene est largement prescrit en pediatrie pour ses puissants effets analgesiques et antipyretiques. A notre connaissance, aucune publication anterieure ne decrit la relation entre l’ibuprofene et un risque accru de complications intracrâniennes et/ou orbitaires dans les sinusites fronto-ethmoidales aigues chez l’enfant. Objectif Rechercher une relation entre l’ibuprofene et l’apparition de complications intracrâniennes et/ou orbitaires de sinusite fronto-ethmoidale aigue en pediatrie. Patients et methodes Les dossiers medicaux des patients de moins de 18 ans admis dans les services ORL de 4 CHU pendant 2 annees consecutives pour sinusite fronto-ethmoidale ont ete revus retrospectivement. La prise d’ibuprofene, l’apparition de complications (orbitaires ou intracrâniennes) ainsi que les donnees demographiques habituelles ont ete notees. Une analyse statistique a ete effectuee afin de determiner s’il existe une relation entre la prise d’AINS et l’apparition d’une complication intracrânienne et/ou orbitaire. Resultats La prise d’ibuprofene semble etre un facteur de risque de complications intracrâniennes ou de complications orbitaires et surtout intracrâniennes en cas de sinusite fronto-ethmoidale aigue chez l’enfant. Ni le sexe, ni l’âge, ni l’intensite initiale de la douleur n’etaient statistiquement lies a l’apparition des complications. Conclusion Cette etude retrospective multicentrique semble suggerer que l’ibuprofene augmente le risque de complications orbitaires et/ou intracrâniennes en cas de sinusite fronto-ethmoidale aigue dans la population pediatrique. Par consequent, nous recommandons de ne pas prescrire d’ibuprofene si l’on soupconne une sinusite aigue chez un enfant ou un adolescent.

International audience; AimOesophageal atresia is frequently associated with other malformations, and our aim was to use computed tomography (CT) to explore intrathoracic malformations in patients with this condition.MethodThis was retrospective study of children aged 0-16 with oesophageal atresia who were born in 1996-2013 and followed up at the French reference centre for rare oesophageal diseases at the University of Lille. Computed tomography scans were available for 48 of the 234 patients during follow-up visits, and these were reviewed by a thoracic radiologist.ResultsMore than two-thirds of the scans were performed to explore persistent respiratory symptoms. We found that six patients had a pulmonary malformations: four lobar agenesis, one right pulmonary aplasia and one congenital cystic adenomatoid malformation. Computed tomography enabled us to diagnose unexpected thoracic malformations in 16 patients: four lobar agenesis, six arteria lusoria, five persistent left superior vena cava and one partial anomalous pulmonary venous return. It also confirmed the diagnoses of suspected malformations in five patients: one congenital cystic adenomatoid malformation, one pulmonary hypoplasia, two right-sided aortic arches and one communicating bronchopulmonary foregut malformation.ConclusionIntrathoracic anomalies were frequently associated with oesophageal atresia, and contrast-enhanced chest CT scans should be performed on patients with persistent respiratory symptoms.

Resume Objectif Les auteurs exposent les recommandations de la SFORL concernant le diagnostic des malformations lymphatiques cervicales de l’adulte et de l’enfant. Methodes A partir de la revue de la litterature scientifique et de l’experience d’un groupe de travail multidisciplinaire, des recommandations ont ete redigees, puis relues par un groupe de lecture independant, et finalisees dans une reunion de coordination. Les recommandations proposees ont ete classees en grade A, B, C ou accord professionnel selon un niveau de preuve scientifique decroissant. Resultats Il est recommande de faire un examen clinique ORL complet afin d’identifier les lesions a risque de complications ou de mauvais pronostic. Il est recommande, en cas de doute diagnostique pour les lesions latero-cervicales et du plancher buccal, de realiser une cytoponction avant une decision therapeutique. Il est recommande d’utiliser une ou plusieurs des classifications validees pour evaluer l’efficacite des traitements et suivre l’evolution de la maladie. Il est recommande, afin d’ameliorer la fiabilite du diagnostic antenatal d’associer IRM et echographie. Il est recommande dans le cadre du diagnostic antenatal d’evaluer de facon precise l’extension locoregionale d’une malformation lymphatique cervicale afin d’en estimer le pronostic, et de rechercher systematiquement des malformations associees afin de guider les propositions therapeutiques.

Objectives The authors present the guidelines of the French Society of Otorhinolaryngology (SFORL) for the diagnosis of cervical lymphatic malformation in adults and children. Methods A multidisciplinary work group was entrusted with a review of the scientific literature on the above topic. Guidelines were drawn up, based on the articles retrieved and the group members’ individual experience. They were then read over by an editorial group independent of the work group, and finalized in a coordination meeting. Guidelines were graded A, B, C or expert opinion, by decreasing level of evidence. Results The SFORL recommends that complete ENT examination should be performed to identify lesions at high risk of complication or associated with poor prognosis. In case of diagnostic doubt, especially in latero-cervical or oral floor lesions, fine-needle aspiration cytology should be performed before therapeutic decision-making. One or more validated classifications should be used to assess treatment efficacy and monitor progression. The reliability of antenatal diagnosis should be ensured by associating MRI to ultrasound. In antenatal diagnosis, the locoregional extension of the cervical lymphatic malformation should be evaluated accurately for prognosis, and associated malformations should be screened for, to guide treatment options.

Objective To study the histoanatomical structure of laryngeal atresia with a focus on the laryngeal functional components in order to evaluate the functional prognosis of laryngeal atresia repair. Methods Twenty-one consecutives cases of laryngeal atresia were diagnosed at our institution between 2009 and 2016. Morphological analysis by macroscopic exam during autopsy was performed in 19 cases. Histological study of the larynx included hematoxylin and eosin staining and protein S100 immunostaining. Our analysis focused on the vocal folds, structures of the lamina propria, cricoarytenoid joints, muscles, and innervation. For each case, associated malformations were classified into two groups: severe and moderate. Results Antenatal diagnosis was suspected because of congenital high airway obstruction syndrome in nine cases (37%). Associated malformations were present in 19 cases (90%), including severe malformations in 12 cases (57%). Atresia involved the cricoid in all cases, with a residual lumen in only one case and the glottis in 18 cases. Separation between the cricoid and arytenoid cartilages was observed in all cases. Fusion of the vocal process of the arytenoids in the midline was present in 13 cases. According to the gestational age, posterior maculae flavae (MF) were present in 17 of 19 cases, with abnormal structure and median fusion in 13 cases. Anterior MF were present in nine of 18 cases, with fusion on the midline in five cases. Intrinsic abductors and adductors muscles were identifiable in all cases, with fusion of thyroarytenoids muscles in the midline in 18 cases. Both recurrent laryngeal nerves were observed in all cases. Conclusion Laryngeal atresia is generally associated with other malformations, with a high risk of fatal outcomes. We observed that the functional structures of the glottic plane were present in most cases, with the exception of MF, which were frequently abnormal. Level of evidence 4 Laryngoscope, 130:252-256, 2020.

Resume Objectifs Les auteurs exposent la recommandation pour la pratique clinique de la Societe francaise d’oto-rhino-laryngologie et de chirurgie de la face et du cou (SFORL) concernant le role de l’ORL dans la prise en charge du syndrome d’apnees-hypopnees obstructives (SAHOS) de l’enfant. Ce chapitre concerne le protocole de suivi des enfants traites. Methodes Un groupe de travail multidisciplinaire a ete charge d’effectuer une revue de la litterature scientifique sur la thematique etudiee. A partir des articles selectionnes et de l’experience de chacun, des recommandations ont ete redigees et classees en grade A, B ou C ou accord professionnel selon un niveau de preuve scientifique decroissant, puis relues par un groupe de lecture independant du groupe de travail. Une reunion de synthese a permis d’aboutir au texte final. Resultats Un suivi clinique et paraclinique a court, moyen et long terme est necessaire compte tenu du risque de SAHOS residuel, et de recidive de SAHOS liee a une repousse adenoidienne et amygdalienne en cas d’adenoido-amygdalectomie, traitement le plus frequemment realise. Les modalites du suivi sont detaillees apres traitement chirurgical, ventilation par pression positive continue (PPC), traitement orthodontique, reeducation myofaciale, et traitement medicamenteux. Les indications de la nasofibroscopie et de l’enregistrement du sommeil dans le cadre du suivi sont precisees.

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.

Resume Objectif Rediger des conseils de bonnes pratiques de prise en charge des pathologies ORL pediatriques en contexte d’epidemie de SARS-CoV-2. Methodes Ces conseils ont ete rediges sur la base de l’experience clinique des experts sollicites pour la redaction de manuscrit, ainsi que sur les recommandations de pratique clinique et publications disponibles sur le sujet dans la litterature. Les propositions feront l’objet de mises a jour en fonction de l’evolution des connaissances sur cette epidemie. Resultats L’incidence des formes symptomatiques d’infection a SARS-CoV-2 chez l’enfant est faible (1–5 %) et le pronostic est bon. Les indications de fibroscopies en consultation doivent etre reduites aux seuls cas indispensables et ne pouvant etre differes. Leur realisation doit etre entouree de precautions maximales, avec notamment port d’un masque « Filtering FacePiece type 2 » (FFP2). Les memes precautions s’appliquent aux lavages de nez au serum sale lorsqu’ils sont pratiques en milieu hospitalier, ainsi qu’aux soins de tracheotomie. Concernant les suspicions de corps etrangers inhales et en dehors des cas cliniquement evidents, il est recommande de prescrire un scanner thoracique avant de programmer une eventuelle endoscopie sous anesthesie generale. Les indications chirurgicales doivent se limiter aux urgences et aux cas ne pouvant etre differes de plus de deux mois, particulierement si ces chirurgies concernent les voies aeriennes et notamment les fosses nasales et le cavum. Les decisions doivent idealement etre prises collegialement et notees par ecrit. Les techniques chirurgicales doivent etre adaptees aux risques de dissemination virale. Dans les cas confirmes ou suspects ou systematiquement pour certains centres, les conditions maximales de protection individuelle avec notamment port d’un masque FFP2 et de lunettes protectrices sont recommandees pour les soignants presents en salle d’operation.

Objective To provide recommendations for the comprehensive management of airway obstruction in patients with Robin Sequence. Methods Expert opinion by the members of the International Pediatric Otolaryngology Group (IPOG). Results The consensus statement provides recommendations for medical specialists who manage infants with Robin Sequence including: evaluation and treatment considerations for commonly debated issues in post-natal airway obstruction, assessment of antenatal obstruction and perinatal airway management. Conclusion Consensus recommendations are aimed at improving management of airway obstruction in patients with Robin Sequence.

Objectives To develop consensus recommendations for the evaluation and management of juvenile-onset recurrent respiratory papillomatosis (JORRP) in pediatric patients. Methods Expert opinion by the members of the International Pediatric Otolaryngology Group (IPOG). The mission of the IPOG is to develop expertise-based consensus recommendations for the management of pediatric otolaryngologic disorders with the goal of improving patient care. The consensus recommendations herein represent the first publication by the group. Results Consensus recommendations including diagnostic considerations, surgical management, systemic adjuvant therapies, postoperative management, surveillance, and voice evaluation. These recommendations are based on the collective opinion of the IPOG members and are targeted for otolaryngologists, primary care providers, pulmonologists, infectious disease specialists, and any other health care providers that manage patients with JORRP. Conclusions Pediatric JORRP consensus recommendations are aimed at improving care and outcomes in this patient population.

Resume Objectif Les auteurs exposent les recommandations de la Societe francaise d’oto-rhino-laryngologie et de chirurgie de la face et du cou (SFORL) concernant le role de l’otorhinolaryngologiste (ORL) dans le syndrome d’apnees-hypopnees obstructives (SAHOS) de l’enfant. Ce chapitre de recommandations concerne la place des differents traitements medicaux et chirurgicaux. Methode Un groupe de travail multidisciplinaire a ete charge d’effectuer une revue de la litterature scientifique sur la thematique etudiee. A partir de ces textes et de l’experience de chacun, des recommandations ont ete redigees, puis relues par un groupe de lecture independant du groupe de travail. Une reunion de synthese a permis d’aboutir au texte final. Resultats L’adenoido-amygdalectomie est le traitement de reference du SAHOS d’un enfant presentant une hypertrophie adenoido-amygdalienne. Il est recommande de mettre en place une assistance respiratoire chez un enfant presentant un SAHOS severe en l’absence d’obstacle rhino et/ou oropharynge, apres chirurgie si le SAHOS persiste, en cas de contre-indication a la chirurgie, dans les obstructions complexes liees a des pathologies pharyngolaryngees et laryngees ou des comorbidites, ou comme alternative a la realisation d’une tracheotomie. Les corticoides par voie nasale peuvent etre utilises dans le SAHOS de l’enfant en cas d’obstruction nasale associee.

OBJECTIVES: Research on long-term use of mitomycin C (MC) for recurrent esophageal stenoses is limited. We assessed the long-term efficacy and safety of local application of MC for recurrent esophageal stenoses in children. METHODS: This was a retrospective study of 39 patients (17 girls) with a median age of 19.5 months (range: 2.4-196.0) at the time of MC application. The etiologies of stenosis were esophageal atresia (n = 25), caustic ingestion (n = 9), congenital esophageal stenosis (n = 3), and other causes (n = 2). Stenosis was single in 35 (90%) patients and multiple in 4 (10%). Before MC, patients underwent multiple repeated dilations (median: 3 dilations per child [range: 2-26]) over a median period of 7 months (range: 2.6-49.3). Treatment success was defined a priori as a reduction in the number of dilations over the same period from before to after the application of MC. RESULTS: For 26 (67%) patients, the application of MC was considered a success: 102 versus 17 dilatations (P

Tongue-lip adhesion may be used to relieve obstructive sleep apnoea in infants with Pierre Robin sequence (PRS), but only a few studies have objectively evaluated its efficacy. The purpose of this study was to evaluate the results of tongue-lip adhesion by polysomnography.A single-centre retrospective study was conducted in infants with PRS treated by tongue-lip adhesion from 2004 to 2015, in whom at least laryngotracheal endoscopy and polysomnography were performed. The variables collected were the syndromic diagnosis, demographic data, respiratory management before tongue-lip adhesion, symptoms, and additional airway interventions. Obstructive sleep apnoea was classified into 3 groups according to severity. Polysomnography was performed one month after tongue-lip adhesion. Statistical analysis was performed with Wilcoxon signed-rank test with a limit of statistical significance of P0.005.Thirty-seven subjects in whom tongue-lip adhesion was performed at a mean age of 45 days (8 to 210 days) were included. Thirty-one patients had isolated PRS and 6 patients had associated anomalies. All patients had confirmed severe obstructive sleep apnoea. All patients required respiratory support prior to surgery: 8 intubated patients, 15 patients with noninvasive ventilation and 14 patients with nasopharyngeal airways. Eight patients had bradycardia before tongue-lip adhesion. All parameters were improved on postoperative polysomnography: oxygen saturation, hypercapnia, apnoea-hypopnoea index, bradycardia (P0.005). Only 8 patients had persistent severe obstructive sleep apnoea and required tracheostomy (n=5) or noninvasive ventilation (n=3). No significant correlation was observed between treatment success and any predictive variables.Tongue-lip adhesion improved airway obstruction in all infants with PRS and resolved obstructive sleep apnoea in 29 patients. However, 8 patients required additional airway interventions.

Resume Objectifs Evaluer l’efficacite de la labioglossopexie sur le SAOS par polysomnographie chez des enfants porteurs d’une sequence de Pierre Robin. Materiel et methodes Etude retrospective monocentrique de 2004 a 2015 chez des enfants ayant beneficie d’une labioglossopexie pour un syndrome d’apnee obstructif du sommeil dans le cadre d’un syndrome de Pierre Robin. Dans le cadre du bilan preoperatoire, une polysomnographie et une endoscopie laryngotracheale etaient realisees. Les donnees recueillies etaient le diagnostic syndromique, l’assistance respiratoire en pre operatoire et ses modalites. Le syndrome d’apnee obstructif du sommeil etait classe en 3 categories selon sa severite. La polysomnographie de controle etait realisee 1 mois apres l’intervention. Resultats Au total, 37 patients ayant eu une labioglossopexie ont ete inclus. L’âge median lors de l’intervention etait de 45 jours (8–210). Au total, 31 patients avaient une sequence de Pierre Robin isolee et 6 avaient un syndrome associe. Tous les patients avaient un syndrome d’apnee obstructif du sommeil severe necessitant une aide respiratoire en preoperatoire : 8 intubations orotracheale, 15 ventilations non invasive, 14 sondes nasopharyngees. La labioglossopexie ameliore significativement la saturation en oxygene, la capnie et l’index d’apnee hypopnee ( p n = 5) ou la mise en place d’une ventilation non invasive ( n = 3). Conclusion La labioglossopexie ameliore l’obstruction respiratoire chez les enfants avec un syndrome de Pierre Robin permettant d’eviter dans la plupart des cas ( n = 29) des therapeutiques plus invasives.

Esophageal atresia (EA) is the most common congenital esophageal malformation. Airway pathology, in particular, tracheomalacia and laryngotracheal anomalies is a major cause of morbidity and mortalilty in patients with EA. The aim of this study was to report the incidence and type of laryngotracheal anomalies seen in a large series of patients with EA, and to evaluate their impact on the management of children with EA. Retrospective study. Retrospective cohort including all patients referred to the EA National Reference Center from January 2002 to December 2014. Airway assessment was based on endoscopy performed before, during and/or after esophageal surgery. One-hundred and fifty-eight patients were included in the study. Endoscopy revealed tracheomalacia in 141 cases (89.2%) and other laryngotracheal anomalies in 43 patients (27.2%). Ninety-six patients (60.7%) presented with persistent respiratory symptoms, including acute life-threatening events in 21 cases, leading to death in 6 cases. A correlation was observed between degree of tracheal collapse and presence of acute life-threatening events. Laryngotracheal surgery was required in 35 cases (22%). Laryngotracheal anomalies are frequently associated with EA and represent an important etiology of morbidity and mortality that can be prevented by early and systematic diagnosis and aggressive management. An early systematic endoscopic evaluation is recommended to coordinate the airway management with the EA surgery.

Resume Objectif Les tumeurs desmoides cervico-faciales de l’enfant sont des tumeurs rares a agressivite locale potentiellement letales. L’objectif est de discuter la prise en charge de ces tumeurs a partir d’une serie de cas et de l’analyse de la litterature. Materiel et methodes L’etude retrospective reprenait les donnees medicales des enfants traites pour tumeur desmoide cervico-faciale entre 1976 et 2014. Resultats Sur les 6 enfants traites, 5 ont beneficie d’une chirurgie d’exerese large dont 2 avaient une invasion microscopie des berges (R1) avec recidive necessitant une nouvelle exerese. Un enfant presentant une lesion trop etendue a beneficie d’une chimiotherapie par methotrexate et vinorelbine hebdomadaire pendant 18 mois, permettant une regression du volume lesionnel de 93 % sans recidive. Conclusions Les tumeurs desmoides cervico-faciales de l’enfant sont plus agressives que chez l’adulte, avec une morbi-mortalite elevee et un taux eleve de recidive. Un bilan d’imagerie par scanner et IRM est preferable avant biopsie. Bien qu’efficace chez l’adulte, la radiotherapie externe doit etre evitee chez l’enfant compte tenu d’une moins bonne efficacite que chez l’adulte, et des sequelles esthetiques, fonctionnelles voire neoplasiques qu’elle comporte a long terme. Le traitement est chirurgical a chaque fois qu’une exerese radicale large est possible. En cas de risque vital ou fonctionnel trop important, une chimiotherapie sans effet indesirable a long terme (type methotrexate associe a un vinca-alcaloide) pourra etre proposee. Un suivi prolonge doit etre realise compte tenu du risque de recidives.

Background Knowledge about airway dimensions during child growth is of paramount importance for pediatric clinical practice. Decisions about airway management in children are based on relatively limited, imprecise, or incomplete data about airway size. Aims The aim of this work was to determine the anatomical development and size of airway structures from birth to adolescence using high-resolution computed tomography scans and to study the correlation between airway measurements and biometric data. Methods We conducted a retrospective study of all high-resolution computed tomography scans including the respiratory tract, performed in our tertiary pediatric center (for reasons unrelated to airway symptoms) between June 1, 2016, and October 15, 2017, on children aged from 1 day to 14 years old. On each scan, 23 measurements of the larynx, trachea, and mainstem bronchi were performed. Patients were stratified into 16 groups according to their age. We calculated median value for each measurement in each group. Statistical models were calculated to explore correlation between measurements and age or weight. Results A total of 192 scans were included (127 boys/65 girls). The mean age was 7 years. The correlations between airway measurements and age or weight were always significant. The relationship between measurements and age was found to be suitably represented by a cubic polynomial equation suggesting that the airway has a rapid growth phase in the first 3 years, followed by a slow growth phase and a second rapid growth phase during adolescence. The most relevant biometric parameter was age concerning 21 of the measurements. Conclusion This comprehensive anatomical database of upper airway dimensions provides important data in the field of pediatric airway anatomy, particularly relating to the cricoid. We demonstrated that laryngeal, tracheal, and bronchial parameters correlate better to age and have three different growth phases.

Introduction Esophageal atresia (EA) is frequently associated with other malformations although few data are available. Objective Describe tracheobronchial, pulmonary and/or vascular malformations in patients with EA using chest CT scans. Methods Monocentric retrospective study in children with EA, born between 1996 and 2013, who had a CT scan during their follow-up, reviewed by a pediatric radiologist. Results Among 234 children with EA, 48 patients underwent a CT scan available for interpretation, among which 69% were performed to explore persistent respiratory symptoms. Thirty-nine children (81%) were type III EA, 13 (27%) had a VACTERL association. Six patients (13%) had a pulmonary malformation: 4 lobar agenesis, 1 right pulmonary aplasia, and 1 congenital cystic adenomatoid malformation. All these patients presented with at least one associated malformation. Combined with the results of laryngotracheal endoscopy (n = 30), 43 patients (90%) had a tracheobronchial anomaly: tracheomalacia for 40 (83%), tracheal stenosis for 12 (25%), right tracheal bronchus for 2 (4%), communicating bronchopulmonary foregut malformation for 1 (2%). Combined with the results of echocardiography (n = 47), 7 patients (15%) had an isolated vascular anomaly, 8 (17%) had an isolated congenital heart disease and 7 (15%) had both. CT scans permitted the diagnosis of 6 pulmonary malformations (13%), 15 tracheobronchial anomalies (31%), and 2 vascular anomalies (4%). Only one patient (2%) in our study presented with an isolated EA. Conclusion Our study confirms the association of tracheobronchial, pulmonary, and vascular anomalies in patients with EA. Contrast-enhanced CT scan is complementary to echocardiography and laryngotracheal endoscopy in the exploration of persistent respiratory symptoms.

Objectives To provide recommendations to otolaryngologists, pulmonologists, and allied clinicians for tracheostomy decannulation in pediatric patients. Methods An iterative questionnaire was used to establish expert recommendations by the members of the International Pediatric Otolaryngology Group. Results Twenty-six members completed the survey. Recommendations address patient criteria for decannulation readiness, airway evaluation prior to decannulation, decannulation protocol, and follow-up after both successful and failed decannulation. Conclusion Tracheostomy decannulation recommendations are aimed at improving patient-centered care, quality and safety in children with tracheostomies.

Introduction Suprastomal Collapse (SuStCo) is a common complication of prolonged tracheostomy in children. There is a paucity of literature on this subject, especially regarding how to manage significant suprastomal collapse that prevents safe decannulation. Objective Provide a definition, classification system, and recommend management options for significant suprastomal collapse in children with tracheostomy. Methods Members of the International Pediatric Otolaryngology Group (IPOG) who are experts in pediatric airway conditions were surveyed and results were refined using a modified Delphi method. Results Consensus was defined as > 70% agreement on a subject. The experts achieved consensus: • on a standard definition of Suprastomal Collapse • that there is clinical importance between significant and insignificant collapse • that the gold standard diagnostic modality is endoscopic examination under anesthesia • that the severity of collapse is a major determinant in the surgical approach • that surgical intervention is the first line management in combined anterior and lateral Suprastomal Collapse. Conclusion This consensus statement provides recommendations for medical specialists who manage infants and children with tracheostomies with significant Suprastomal Collapse. It provides a classification system to facilitate diagnosis and treatment options for this condition.

Objective To make recommendations on the identification, routine evaluation, and management of fetuses at risk for airway compromise at delivery. Methods Recommendations are based on expert opinion by members of the International Pediatric Otolaryngology Group (IPOG). A two-iterative Delphi method questionnaire was distributed to all members of the IPOG and responses recorded. The respondents were given the opportunity to comment on the content and format of the survey, which was modified for the second round. “Consensus” was defined by >80% respondent affirmative responses, “agreement” by 51–80% affirmative responses, and “no agreement” by 50% or less affirmative responses. Results Recommendations are provided regarding etiologies of perinatal airway obstruction, imaging evaluation, adjunct evaluation, multidisciplinary team and decision factors, micrognathia management, congenital high airway obstruction syndrome management, head and neck mass management, attended delivery procedure, and delivery on placental support procedure. Conclusions Thorough evaluation and thoughtful decision making are required to optimally balance fetal and maternal risks/benefits.

Objective Vocal fold paralysis (VFP) in adducted position remains a challenge for airway surgeons. Alternatives to tracheostomies such as lateralization, cordectomy, and posterior rib grafting disrupt the laryngeal tissue or framework and carry an increased risk of aspiration. Laryngeal reinnervation using nerve-muscle pedicle (NMP), carries the distinct advantage of preserving the larynx, sparing the recurrent laryngeal nerve, and obtaining an active VF abduction. The aim of this study was to evaluate the success and complications of laryngeal reinnervation using nerve-muscle pedicle (NMP) in pediatric patients presenting with dyspnea related to VFP in adducted position. Methods In this case series performed at a tertiary care referral center, review of medical records on all pediatric patients with VFP in adduction treated with laryngeal reinnervation using NMP between 1999 and 2017. Data were collected on the preoperative flexible laryngoscopy, suspension micro-laryngoscopy, and laryngeal electromyography as well as post-operative clinical assessment of the voice and airway. All patients underwent surgery consisting of the transfer of an innervated omohyoid muscle pedicle onto the paralyzed posterior cricoarytenoid muscle. The main outcomes measured were the clinical and fiberoptic laryngoscopic airway assessment monthly for the first 6 months, then at 12 months and annually thereafter. Clinical assessment included dyspnea evaluation based on a visual analog scale and voice assessment using the GRBAS scores. Complications from the treatment were also noted. These outcomes were determined before collection of data. Results 16 cases were identified, with a mean age of 4 years. The recurrent laryngeal nerve paralysis was bilateral in 3 cases and unilateral in 13 cases. There were no peri or postoperative complications. After a mean follow-up of 7 years, vocal fold abduction was observed in 10 out of 16 cases and disappearance of paradoxical inspiratory adduction in 3 cases. Persistent dyspnea was noted in 7 cases (44%), and moderate dysphonia was present in 11 cases (69%). Finally, additional procedures were necessary in 2 patients (13%) to achieve the outcomes. Conclusions Laryngeal reinnervation using NMP may be used in pediatric patients. This procedure, is safe and allows us to spare the recurrent laryngeal nerve while obtaining an active VF abduction in the majority of cases, and an improvement in breathing in most cases. Quality of evidence 4.

In 2005, the French law on patients' rights at the end of life required that decisions to withdraw or withhold life-sustaining treatments be made and carried out by the physician in charge of the patient, after obtaining advice from an independent consulting colleague and the caregiving team. The purpose of this study was to identify theoretical and practical obstacles to this collaborative deliberation and to propose practical guidelines to organize it.

Background Adequate endotracheal tube positioning in preterm infants is complicated by the short length of the airway. Distal markers were designed to help with the insertion of endotracheal tubes at an appropriate depth below the vocal cords. However, those markers are not standardized between manufacturers, each tube size displays only one (sometimes 2) markers to provide information for infants of various gestational ages, and indicated distances are often too long for extremely preterm infants. Aims The study aims to describe vocal cords to mid-tracheal distance for different gestational ages and determine if depth markers should be adjusted accordingly. Methods Half the tracheal length added to the height of the posterior lamina of the cricoids approximates the distance between vocal cords and mid-trachea. Those dimensions were retrospectively retrieved from a database of laryngo-tracheal measurements obtained during autopsies of fetuses and newborn infants free of upper airway malformations. The equation of correlation between gestational age and distance from vocal cords to mid-trachea was used to calculate those distances for different gestational ages. Results Data were derived from 114 patients. Vocal cords to mid-trachea distance is linearly correlated with gestational age (r = .90; distance = 2.831 + 0.6208 × gestational age). We suggest depth markers at 17.7, 19.0, 20.8, 22.7, 24.6, and 26.4 mm for gestational ages of 24, 26, 29, 32, 35, and 38 weeks, respectively, indicated by contrasting colors. Conclusion The linear relationship between laryngo-tracheal size and gestational age offers the opportunity to revise endotracheal tube depth markers for the smallest patients. Trials comparing those suggested markers with those currently in use are needed before implementation.

Constitutive COL2A1 mutations are associated with a wide variety of clinical manifestations known as type II collagenopathies. Among them is Kniest dysplasia, which is phenotypically variable and includes both skeletal (short trunk and limbs, kyphoscoliosis, prominent joints, and osteoarthritis) and craniofacial characteristics. Kniest dysplasia mutations primarily arise in the triple-helicoidal region of the alpha 1 (II) chain in COL2A1 between exons 12 and 24. Somatic COL2A1 mutations have been identified in chondrosarcoma, a rare cartilage forming neoplasm, with a hypermutability of the gene reported in 37% of cases. However, to the best of our knowledge, there is no reported increase in predisposition to chondrosarcoma in human collagenopathies, and no reported clinical association between these congenital diseases and cartilaginous tumors. In the case study presented here, we report the first description of an association between these two rare diseases involving COL2A1, in a child presenting with Kniest dysplasia and a grade I sphenoethmoidal chondrosarcoma. We also describe a new constitutive mutation in COL2A1.

Oncologic management in pediatric patient may be associated with a high risk of neurosensory deficit, such as taste, olfaction, vision and hearing. These neurosensory deficits can be linked to chemotherapy toxicity or to a direct deleterious effect of local radiotherapy or surgical management in case of craniofacial cancers. Neurosensory deficit may be temporary but are usually irreversible and frequently progress after the completion of treatment. Taste and olfaction deficits expose to high risk of nutritional complications and quality of life alteration. Hyposialia, as a result of irradiation of the salivary glands, increases taste changes and the risk of dental caries. The risk of cataract is present in patients who received high dose corticosteroids and/or brain or orbital irradiation. When hearing is affected, a risk of impaired intellectual or academic performance is increased with an impact on the quality of life in absence of specific care. Finally, there are some cosmetic consequences of therapy such as alopecia and scarring that alter the image of the patient. Early detection of these problems in order to limit medical, psychological, educational and social impact is mandatory. Moreover, high risk of worsening of these deficits after completion of therapy support long-term follow-up children treated for cancer, especially with head and neck primary.

Resume La dysphonie de l’enfant est une pathologie courante a l’âge scolaire. La perception du trouble fonctionnel est subjective et le plus souvent exprimee par une personne n’appartenant pas a l’entourage proche de l’enfant. Recueillir l’anamnese et les antecedents permet souvent de s’orienter vers une lesion malformative ou acquise. L’evaluation fonctionnelle est utile pour apprecier le handicap vocal et le qualifier. Un examen physique et endoscopique realise en consultation est l’examen cle. Il est rarement impossible chez l’enfant et permet tres souvent de poser un diagnostic. Les examens complementaires sont parfois necessaires.

OBJECTIVES It has been demonstrated that both heterotopic and orthotopic transplants of epithelium-denuded cryopreserved tracheal allografts are feasible in immunosuppressant-free rabbits. Validation of these results in large animals is required before considering clinical applications. We evaluated the viability, immune tolerance and strain properties of such tracheal allografts heterotopically transplanted in a pig model. METHODS Ten tracheal segments, 5 short (5 rings) and 5 long (10 rings), were obtained from male Landrace pigs. The tracheal segments were surgically denuded of their epithelium, then cryopreserved and stored in a tissue bank for 33 to 232 days. After thawing, tracheal segments stented with a silicone tube were wrapped in the omentum in 2 groups of 5 female recipients. The animals did not receive any immunosuppressive drugs. The animals were euthanized from Day 6 to Day 90 in both groups. RESULTS An effective revascularization of allografts regardless of length was observed. Lymphocyte infiltrate was shown in the early postoperative period and became non-significant after 30 days. Allografts displayed high levels of neoangiogenesis and viable cartilage rings with islets of calcification. Biomechanical measurements demonstrated strain properties similar to those of a fresh tracheal segment from Day 58. CONCLUSIONS Our results demonstrate the acceptability and satisfactory stiffness of epithelium-denuded cryopreserved tracheal allografts implanted in the omentum, despite the absence of immunosuppressive drugs. Since the omentum has the capability to reach the tracheal region, this approach should be investigated in the setting of orthotopic transplants in a pig model before considering clinical applications.

Objectives The present clinical practice guidelines cover the entire field of management of post-tonsillectomy pain. Given the French and European regulatory restrictions on the use of codeine, an update appears necessary to clarify the management of post-tonsillectomy pain in adults. Method A work group approached the issue of pain management, following the chronological pathway from initial consultation to postoperative period. As exhaustive a study of the literature as possible assessed the pain impact of the various surgical techniques and the efficacy of the various analgesia schedules. Results Guidelines on the management of post-tonsillectomy pain in adults were drawn up and graded, based on the levels of evidence of selected articles and on work group consensus. The guidelines stress the importance of patient information and seek to harmonize practice, reduce the risk of postoperative complications and above all improve control of post-tonsillectomy pain in adults.