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Resume L’obstruction nasale chez l’enfant est un symptome tres frequent pouvant relever de nombreuses etiologies. Le diagnostic clinique facile ne doit pas faire oublier la recherche d’un retentissement general et d’une etiologie rare. Le choix des explorations complementaires sera guide par les orientations diagnostiques. Bien que l’obstruction nasale soit sans gravite dans la grande majorite des cas, elle peut mettre la vie en jeu chez le nouveau-ne et le nourrisson. Un catalogue exhaustif des etiologies d’obstruction nasale chez l’enfant est impossible et inutile. Cependant, il est pertinent d’etablir une distinction entre les obstructions nasales chez le nourrisson et celles chez l’enfant plus âge, dont les causes sont tres differentes. En dresser la liste des plus frequentes ou des plus graves permet de faciliter la pratique clinique. C’est ce que propose la presente mise au point.

Lymphatic malformations are benign malformations frequently occurring in the head and neck. Retropharyngeal location is rare, can be life threating and its management is particularly challenging. Over a three-year period, three patients presented with symptomatic (dyspnea and/or dysphagia) retropharyngeal lymphatic malformation. All were treated using a radiofrequency ablation of lymphatic malformation through a trans-oral approach. No major complications occurred following the surgery. During the follow-up, no recurrence was noted and all patients were asymptomatic. Radiofrequency ablation in the management of retropharyngeal lymphatic malformations is a simple technique with very good results and allows a fast recovery with minimum morbidity and a short hospital stay.

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 To provide recommendations to otolaryngologists and allied physicians for the comprehensive management of young infants who present with signs or symptoms of choanal atresia. Methods A two-iterative delphi method questionnaire was used to establish expert recommendations by the members of the International Otolaryngology Group (IPOG), on the diagnostic, intra-operative, post-operative and revision surgery considerations. Results Twenty-eight members completed the survey, in 22 tertiary-care center departments representing 8 countries. The main consensual recommendations were: nasal endoscopy or fiberscopy and CT imaging are recommended for diagnosis; unilateral choanal atresia repair should be delayed after at least age 6 months whenever possible; transnasal endoscopic repair is the preferred technique; long term follow-up is recommended (minimum one year) using nasal nasofiberscopy or rigid endoscopy, without systematic imaging. Conclusion Choanal atresia care consensus recommendations are aimed at improving patient-centered care in neonates, infants and children with choanal atresia.

International audience; OBJECTIVES: The authors present the French Society of Oto-Rhino-Laryngology and Head and Neck Surgery (SFORL) clinical practice guidelines concerning the role of otorhinolaryngologists in the management of paediatric obstructive sleep apnoea syndrome (OSAS). This chapter is devoted to the follow-up protocol for children treated for OSAS. METHODS: A multidisciplinary task force was commissioned to carry out a review of the scientific literature on this topic. On the basis of the articles selected and the personal experience of each member of the task force, guidelines were drafted and graded as A, B or C or expert opinion according to a decreasing level of scientific evidence, and were then reviewed by a reading committee, independently of the task force. The final guidelines were established at a consensus meeting. RESULTS: Short-term, medium-term and long-term clinical follow-up and complementary investigations are necessary in view of the risk of residual OSAS, and the risk of recurrence of OSAS related to adenoid and tonsillar regrowth following adenotonsillectomy, the treatment most commonly performed. The modalities of follow-up after surgery, continuous positive airway pressure (CPAP) ventilation, orthodontic treatment, myofascial rehabilitation, and drug therapy are described. The indications for nasal endoscopy and sleep studies as part of follow-up are specified.

Objective The authors present the guidelines of the French Society of ENT and Head & Neck Surgery (SFORL) on the role of the ENT physician in childhood obstructive sleep apnea-hypopnea syndrome (OSAHS). This section of the guidelines concerns the roles of the various medical and surgical treatment options. Method A multidisciplinary work-group was entrusted with a review of the scientific literature on the topic. Based on the retrieved articles and the group members’ own experience, guidelines were drawn up, then read over by a reading group independent of the work-group. An editorial meeting then produced the final text. Results Adenotonsillectomy is the reference treatment for childhood OSAHS with adenotonsillar hypertrophy. Respiratory assistance is recommended in children with severe OSAHS without nasal and/or oropharyngeal obstacle, after surgery in case of persistent OSAHS, in case of contraindications to surgery, in complex obstruction related to pharyngolaryngeal or laryngeal pathology or comorbidity, or as an alternative to tracheotomy. Nasal route corticosteroids may be used in childhood OSAHS in with associated nasal obstruction.

Resume Introduction Les rhabdomyosarcomes (RMS) sont les plus frequents des sarcomes des tissus mous de l’enfant et representent 5 % de toutes les tumeurs malignes pediatriques. Les principales localisations de la region cephalique sont la base du crâne, le nasopharynx, la cavite nasale et l’orbite. Le developpement d’un RMS au niveau de l’oreille externe est considere comme extremement rare. Nous presentons trois cas d’enfants, âges de 6 a 14 ans presentant un RMS du pavillon de l’oreille. Resume des cas cliniques Le premier, âge de 6 ans, a ete traite par 4 cures de chimiotherapie suivie d’une exerese chirurgicale du lit tumoral et complete par 5 cures de chimiotherapie. Le deuxieme, âge de 14 ans, a ete traite par 4 cures de chimiotherapie puis une radiotherapie externe du lit tumoral et des aires ganglionnaires suivie de 5 cures de chimiotherapie. La troisieme, âgee de 13 ans, a ete traite par 4 cures de chimiotherapie puis chirurgie et complete par 5 cures. Discussion Chez ces trois patients, les protocoles de traitement ont permis un controle complet de la maladie. Le pronostic est bon en raison de l’accessibilite chirurgicale. Le diagnostic doit etre evoque devant une tumefaction inhabituelle, evolutive de l’oreille externe.

Resume L’amygdalectomie est une intervention chirurgicale frequente et banalisee, mais non denuee de complications. L’hemorragie post-amygdalectomie (HPA) est l’une de ces complications. Elle peut etre precoce, survenant dans les 6 premieres heures postoperatoires, ou tardives, survenant principalement entre le 5 e et le 15 e jours postoperatoires. Les principaux facteurs de risque d’HPA sont l’âge, le sexe et la technique chirurgicale. L’utilisation d’anti-inflammatoires ne majore pas le risque d’HPA. Devant le diagnostic d’HPA, tardive dans la majorite des cas, la reprise chirurgicale est indiquee dans la moitie des cas. La prise en charge anesthesique de cette hemostase chirurgicale doit prendre en compte l’anemie potentielle plus ou moins hypovolemique du patient et impose une induction en sequence rapide. Cette complication est encore de nos jours susceptible de mettre en jeu le pronostic. Une optimisation des nouvelles techniques chirurgicales et une bonne cooperation anesthesiste–chirurgien permettent d’en limiter la morbi-mortalite.

Develop multidisciplinary and international consensus on patient, disease, procedural, and perioperative factors, as well as key outcome measures and complications, to be reported for pediatric airway reconstruction studies.Standard Delphi methods were applied. Participants proposed items in three categories: 1) patient/disease characteristics, 2) procedural/intraoperative/perioperative factors, and 3) outcome measures and complications. Both general and anatomic site-specific measures were elicited. Participants also suggested specific operations to be encompassed by this project. We then used iterative ranking and review to develop consensus lists via a priori Delphi consensus criteria.Thirty-three pediatric airway experts from eight countries in North and South America, Europe, and Australia participated, representing otolaryngology (including International Pediatric Otolaryngology Group members), pulmonology, general surgery, and cardiothoracic surgery. Consensus led to inclusion of 19 operations comprising open expansion, resection, and slide procedures of the larynx, trachea, and bronchi as well as three endoscopic procedures. Consensus was achieved on multiple patient/comorbidity (10), disease/stenosis (7), perioperative-/intraoperative-/procedure-related (16) factors. Consensus was reached on multiple outcome and complication measures, both general and site-specific (8 general, 13 supraglottic, 15 glottic, 17 subglottic, 8 cervical tracheal, 12 thoracic tracheal). The group was able to clarify how each outcome should be measured, with specific instruments defined where applicable.This consensus statement provides a framework to communicate results consistently and reproducibly, facilitating meta-analyses, quality improvement, transfer of information, and surgeon self-assessment. It also clarifies expert opinion on which patient, disease, procedural, and outcome measures may be important to consider in any pediatric airway reconstruction patient.5 Laryngoscope, 129:244-255, 2019.

Objectives To analyze vestibular function Q2 (VF) after cochlear implantation (CI) in children. Methods Retrospective cohort study at a tertiary pediatric referral center. 43 children that had vestibular testing (VT) after unilateral cochlear implantation, from 2001 to 2010, were included. CT scan of the temporal bone was done systematically before surgery. VT included three tests: head-impulse test, caloric tests, vestibular evoked myogenic potentials. VF was graded in: normal (type 1), partial dysfunction (type 2), severe dysfunction and areflexia (type 3). In 12 cases, VT was done before and after CI. Vestibular function was analyzed looking to side, etiology and preoperative status. Results Mean age at CI was 2.9 years. Bilateral inner ear malformation were retrieved in 16%. Before surgery, 50% of children had normal vestibular responses, 4/12 had bilateral type 2, two had asymmetrical VF. In this group, after surgery, 2 children had VF worsening, none on the CI side only. Considering all 43 patients, post operative VT showed normal response in 48.8% and type 2&3 in 16.2%. Children had asymmetrical poorer vestibular function on the side of CI in 19%. Among them, 75% had normal contralateral VF. Sensorineural hearing loss etiologies known to be associated with vestibulopathy (Usher/Meningitis/Inner Ear Malformations/CMV) were associated to abnormal vestibular function more frequently than in other causes ( p = 0.01). Conclusion Half of the children had initial vestibular dysfunction. In our study, 20% of cochlear implantation could have worsened vestibular function. As vestibular function should be part of the choice in cochlear implantation, side of implantation and survey; and vestibular tests are uneasy to achieve in pediatric population, we propose a three-steps evaluation and gradation which allow easier comparison.

Objectives/Hypothesis Vocal fold paralysis is the second most common congenital laryngeal anomaly in newborns. Bilateral paralysis is a severe condition and often remains of unknown etiology. We report our experience of congenital idiopathic bilateral vocal fold paralysis in newborns and infants, and discuss the therapeutic options. Study Design Retrospective review. Methods A retrospective review was carried out at a single tertiary referral center over a 15-year period of children presenting with congenital idiopathic bilateral laryngeal paralysis (CIBP). Results Twenty-six patients were identified and managed over period of the study (mean follow-up, 6.7 years). A tracheostomy was eventually performed in 14 patients, 12 during the neonatal period. Endoscopic treatment was performed in 16 patients and an external approach in five patients after endoscopic failure. Three patients were managed by watchful waiting. Spontaneous recovery occurred in 16 patients (median age, 14.5 months), including nine with a tracheostomy. Decannulation was achieved in 12 patients (median age, 42 months), and noninvasive positive pressure ventilation (NPPV) was applied in six patients. Conclusions Only inhomogeneous series of pediatric bilateral laryngeal palsies have been published so far, making it difficult to identify prognostic and predictive markers, as well as therapeutic guidelines. Focusing on CIBP, our data suggest that the management strategy must take into account the possibility of a delayed spontaneous recovery. NPPV is an interesting therapeutic tool in this situation. A systematic somatic assessment is mandatory to look for associated conditions. Level of Evidence 4 Laryngoscope, 125:1724–1728, 2015

OBJECTIVES To assess the anatomic and functional outcome of underlay cartilage myringoplasty in children with cleft palate, at different postoperative periods compared with a patients-matched control group STUDY DESIGN Case control study, tertiary referral center. METHODS An otologic database was used to select children with cleft palate and perforated tympanic membrane who underwent myringoplasty between 1995 and 2012. These subjects were matched with control patients, without cleft palate, using the following criteria: age, size of perforation, status of contralateral ear, and status of middle ear mucosa. Charts were reviewed for the following: patients characteristics, preoperative findings, surgical data, postoperative anatomic and functional outcomes, and reinterventions. The postoperative findings were divided into 5 different periods. RESULTS A group of 32 cleft palate patients as well as 32 control patients were included in this study, with a mean follow up of 63.6 ± 41 months. There were no differences between the groups in anatomic success as it was achieved in 84% in both groups. No differences were seen in functional outcome when compared with each different postoperative period. Using the last available audiogram, the postoperative mean air conduction and the air-bone gap were significantly worse in the cleft group compared with the control group, respectively, 26.1 ± 13.7 dB versus 18.4 ± 10.1 dB, p = 0.042; and 16.5 ± 9.4 dB and 11.3 ± 6.4 dB, p = 0.046. Additionally, the functional success was significantly worse in the cleft group; 58% versus 87% in the control group (OR, 5.5 [95% CI, 1.22-24.81], p = 0.027). CONCLUSION Children with cleft palate can benefit from cartilage underlay myringoplasty in terms of closure of tympanic membrane, although there is a worse functional outcome.

Summary The acoustic reflection method (ARM) is based on the analysis of the reflection of an acoustic wave in the airway, allowing the calculation of its longitudinal cross-sectional area profile and airway resistance. The ARM represents a simple, quick and totally non invasive tool for the investigation of the upper airways, requiring minimal cooperation, which makes this technique particularly suitable for children. Normal values of the minimal cross sectional area of the upper airways in children have been recently published. The ARM has shown its utility in the investigation of the nasal cavity in healthy and diseased children and of the upper airways in children with various diseases such as mucopolysaccharidosis and cystic fibrosis. This simple, rapid, and inexpensive technique seems promising for the non-invasive investigation of the whole upper airway during wakefulness in children, both in health and disease, and may constitute a complementary or alternative method to explore and follow children with anomalies of the upper airways.

OBJECTIVES To standardize the information for families of children having functional surgery for middle ear malformations, we describe the audiometric results of the subgroup of patients with the most favorable anatomic conditions: viable auditory canal, intact tympanic membrane, mobile stapes, and corresponding to a Jahrsdoerfer score of 8 or higher. STUDY DESIGN Case series, tertiary referral center. METHODS Charts of patients undergoing functional surgery for congenital middle ear malformations were reviewed for demographic data, preoperative Jahrsdoerfer score, ossicular chain status, type of ossiculoplasty, and audiometric data before and 6 months postsurgery. RESULTS Eighteen consecutive interventions were performed on 13 patients (average age of 9 years, 8 girls and 5 boys) between 2004 and 2011. The ossiculoplasties performed were as follows: incus repositioning (4), double-layer tragal cartilage (5), intact native chain reconstruction (3), and partial ossicular prosthesis (6). Mean air bone gap (ABG) was 40.8 ± 12.4 dB preoperatively and 20.9 ± 12.9 dB postoperatively (p < 0.0001). Preoperative and postoperatively mean air conduction PTA thresholds were 49.9 ± 9.5 and 30.0 ± 14.1 dB, respectively (p < 0.0001). All ears operated on except one had air conduction improvement. There were no complications. CONCLUSION Functional surgery for congenital middle ear malformations gives variable hearing outcomes. In this study, with the most favorable anatomic conditions, 12 ears (67%) of 18 had air conduction improvement below 30 dB.

Les pathologies obstructives des voies aériennes supérieures de l’enfant sont fréquentes et très largement dominées par l’hypertrophie des amygdales et des végétations adénoïdes et, dans une moindre mesure, par celle des cornets inférieurs. Une indication d’adéno-amygdalectomie se base sur des critères cliniques, éventuellement complétés dans certains cas par une polysomnographie; cette intervention ne doit pas être réalisée systématiquement en cas d’hypertrophie amygdalienne, même en cas de ronflement simple ou si un traitement orthodontique est envisagé. L’éventuelle participation d’une l’hypertrophie turbinale ou d’une déviation septale doit également être prise en compte.

A 4-year-old girl with bilateral vocal fold palsy was successfully decannulated from tracheotomy after seven laryngeal procedures. But an important stridor and dyspnea recurred 13 months after decannulation. Nocturnal gas exchange was normal but her daytime work of breathing was increased by fourfold, without any beneficial effect of nasal noninvasive continuous positive airway pressure ventilation (CPAP), reflecting a severe fixed airway obstruction. Endoscopic examination confirmed the work of breathing findings showing glottic and supraglottic stenosis. This upper airway obstruction was successfully treated with a recannulation. In conclusion, the major message of this case report is that measurement of the work of breathing was able to document the "fixed" nature of the airway obstruction, by showing no improvement even with highest tolerated levels of nasal CPAP. As such, the work of breathing may be proposed as a screening tool to quantify and assess the reversibility of severe upper airway obstruction in children.

Objective To report preliminary results of a new closed-skin, transcutaneous bone conduction device (BCD) in 6 children with high-grade ear atresia. Settings Tertiary care center; prospective study; we evaluated the gain with masking of the contralateral ear and the benefit of hearing rehabilitation with the transcutaneous BCD in noise: speech-in-noise tests, conducted in real life condition (with contralateral ear unmasked and fitted with a hearing device if done before implantation), with and without BCD, with determination of the speech reception threshold (SRT). Children and parent's satisfaction was assessed. Results Patients' ages ranged from 6 to 9 years. All had high-grade ear atresia with a preoperative mean pure-tone average (PTA) loss of 71.46 +/- 6.59 dB on air conduction and 14 +/- 4.98 dB on bone conduction. At M6, all children used the implant 5 to 12 hours daily (mean, 10) without pain or cutaneous complications. At M6, the mean air conduction PTA with transcutaneous BCD was 28.45 +/- 1.68 dB, the mean gain 43 +/- 6.96 dB, and the mean SRT gain 33.33 +/- 10.75 dB. Using speech-in-noise tests in real-life conditions, the mean SRT was statistically improved with the transcutaneous BCD (-8 +/- 2.83 dB, p = 0.0313). Both children and parents reported being satisfied or very satisfied. Conclusion These preliminary results show satisfactory functional gain, cutaneous tolerance, and patients' satisfaction with the new transcutaneous BCD.

International audience; The French national cohort of children with Langerhans cell histiocytosis (LCH) has included 1478 patients since it was established in 1983. LCH therapeutic strategies substantially changed in 1998, so we have divided the cohort into two 15-year periods. Starting in 1998, therapy duration increased from 6 to 12 months, repeated induction therapy was performed in cases showing a poor response to the first induction with vinblastine and steroids, and refractory disease in a risk organ (RO+) was treated with cladribine and cytarabine. A total of 483 (33%) patients were enrolled before 1998, and 995 (67%) after 1998. Five-year survival was 96·6% (95% confidence interval: 95·4–97·5%) overall, improving from 92% pre-1998 to 99% post-1998 (P \textless 0·001 adjusted to disease extent). This change was supported by an increase in 5-year survival from 60% to 92% in the RO+ group. Survival was particularly associated with cladribine and cytarabine among refractory RO+ patients. Disease reactivation was slightly less frequent after 1998, due to better enrolment of single-system patients, extended therapy duration, and more efficient second-line therapy. The crude rates of endocrine and neurological sequelae (the most frequent sequelae) appeared to improve over time, but this difference was not observed when the analysis was stratified by disease extent

Summary Objective To evaluate the psychological preparation and stress of medical students associated with their first cadaver dissection class, covering the head and neck region. Materials and methods Prospective evaluation of a group of 58 second-year medical students. Results Participants experienced this first dissection class with little stress, with no significant gender difference. Men, however, felt better prepared than the women did. The smell and sight of the cadaver's face were their main concerns. Discussion The students were evaluated with STAI questionnaires and visual analog scales just before, immediately after, and one month after their first dissection experience. This is the first time this scheme has been used. Students suggested several improvements for advance preparation, most of them inexpensive in terms of human and material resources. The low stress caused by dissection is probably influenced by the fact that student participation is voluntary. Conclusion Although they felt that they were relatively unprepared from a psychological perspective, the vast majority of students felt ready to participate in the dissections and had a very good perception of this experience.

Background Upper airway obstruction is common in children with mucopolysaccharidosis. The acoustic reflection method is a noninvasive technique that can analyze the caliber of the upper airways. The aim of the study was to evaluate the feasibility of the acoustic reflection method in children with mucopolysaccharidosis, and to compare the characteristics of the upper airways evaluated by the acoustic reflection method in patients with mucopolysaccharidosis to matched healthy counterparts. Methods Open, single center, prospective, study. Results Accurate acoustic reflection measurements could be obtained in 7 of 10 patients (mean age: 10.4 ± 3.9 years; mucopolysaccharidosis type II (n = 3); type IV (n = 2), type VI (n = 1), and fucosidosis (n = 1)). The mean minimum cross-sectional area was lower in mucopolysaccharidosis patients (1.6 ± 0.3 cm2) as compared to 14 healthy counterparts (1.8 ± 0.3 cm2; P = 0.03). The mean resistance of the airways was significantly higher in the MPS group (7.9 ± 1.8 cmH2O l−1 sec) as compared to the controls (5.5 ± 1.2 cmH2O l−1 sec; P = 0.006). Conclusion This study is the first to analyze the upper airways by the noninvasive acoustic reflection method in children with mucopolysaccharidosis. Due to a lack of cooperation, reliable measurements could only be obtained in 70% of a selected group of patients. Children with mucopolysaccharidosis have significant upper airway obstruction as assessed by the reduction of the minimal cross-sectional area of the upper airways and the increase in airway resistance. Pediatr. Pulmonol. 2011; 46:587–594. © 2011 Wiley-Liss, Inc.

Objective Preliminary assessment of the efficacy of propranolol on subglottic hemangioma in children on a nation-wide scale. Methods Multicentric, retrospective study of clinical files of 14 children; pre- and post-treatment endoscopies. Results Mean age at diagnosis was 2.3 (0.7–4) months. Mean percentage of airway obstruction was 68% (15–90) before propranolol introduction. Propranolol was started at 5.2 (0.7–16) months of age. This treatment was effective in all cases with a mean regression of the stenosis to 22% after 2 weeks and 12% after 4 weeks. Other medical treatments (steroids) could be stopped. In one patient, a side effect of propranolol motivated the switch to another β-blocker. In four patients, treatment was stopped after 5.2 (1–10) months with a relapse in 2 (50%) cases. One of these two patients developed a resistance to propranolol and required a surgical procedure by external approach. Conclusion This preliminary nation-wide survey confirms propranolol high effectiveness against airways’ localization of infantile hemangiomas. Propranolol also allows alleviation or cut-off of previous medical treatments. However, recurrences are possible after early treatment interruption.

OBJECTIVE: To show that noninvasive positive-pressure ventilation by means of a nasal mask may avoid recannulation after decannulation and facilitate early decannulation. DESIGN: Retrospective cohort study. SETTING: Ear-nose-and-throat and pulmonary department of a pediatric university hospital. PATIENTS: The data from 15 patients (age = 2-12 yrs) who needed a tracheotomy for upper airway obstruction (n = 13), congenital diaphragmatic hypoplasia (n = 1), or lung disease (n = 1) were analyzed. Four patients received also nocturnal invasive ventilatory support for associated lung disease (n = 3) or congenital diaphragmatic hypoplasia (n = 1). Decannulation was proposed in all patients because endoscopic evaluation showed sufficient upper airway patency and normal nocturnal gas exchange with a small size closed tracheal tube, but obstructive airway symptoms occurred either immediately or with delay after decannulation without noninvasive positive-pressure ventilation. INTERVENTIONS: In nine patients, noninvasive positive-pressure ventilation was started after recurrence of obstructive symptoms after a delay of 1 to 48 mos after a successful immediate decannulation. Noninvasive positive-pressure ventilation was anticipated in six patients who failed repeated decannulation trials because of poor clinical tolerance of tracheal tube removal or tube closure during sleep. MEASUREMENTS AND MAIN RESULTS: After noninvasive positive-pressure ventilation acclimatization, decannulation was performed with success in all patients. Noninvasive positive-pressure ventilation was associated with an improvement in nocturnal gas exchange and marked clinical improvement in their obstructive sleep apnea symptoms. None of the 15 patients needed tracheal recannulation. Noninvasive positive-pressure ventilation could be withdrawn in six patients after 2 yrs to 8.5 yrs. The other nine patients still receive noninvasive positive-pressure ventilation after 1 yr to 6 yrs. CONCLUSIONS: In selected patients with upper airway obstruction or lung disease, noninvasive positive-pressure ventilation may represent a valuable tool to treat the recurrence of obstructive symptoms after decannulation and may facilitate early weaning from tracheotomy in children who failed repeated decannulation trials.

To report on the outcomes of sequential bilateral cochlear implantation (CI) in children with inner ear malformation. The study design is a retrospective case study. The setting is a tertiary reference center. Two children presenting a profound bilateral congenital hearing loss with bilateral hypoplasia of the cochleovestibular nerves and hypoplasic external semicircular canal had a cochlear implant at respectively 16 months and 33 months. A second implant was proposed at respectively 17 and 20 months after the first implant. The main outcome measures are audiometry, perceptive results in closed and open set words (CSW and OSW) and oral production at follow-up. The first cochlear implant gave respectively mean thresholds at 60 dB and 70 dB. Bilateral CI showed mean threshold at respectively 40 dB and 55 dB. In case 1, perceptive assessment was 83% and 70% in respectively CSW and OSW with oral production and comprehension of sentences after 1 year follow-up. In case 2, the perceptive assessment showed no perceptive or linguistic evolution at 6 months follow-up. In cochleovestibular nerve hypoplasia, bilateral implantation could be discussed in cases of limited result after unilateral implant.

Laryngeal clefts are rare congenital anomalies, first described in 1792 by Richter, that allow communication between the tracheal and esophageal axis. The incidence is 1 in 10,000 to 20,000 births, which represents approximately 1.5% of the laryngeal pathology in children. Laryngeal clefts result from a failure of fusion of the posterior cricoid lamina and development of the tracheoesophageal septum. Recent work has further refined our understanding of this complex development.

Lymphangiomas of the mouth and tongue pose considerable therapeutic problems. Their complete exeresis is not feasible, and they can be a major functional impediment and cause face and jaw deformities. A risk of secondary growth is classically described after surgical reduction. Invasion of lingual mucosa, often papillomatous, results in accidental biting, recurrent bleeding and pain. Here, we report on 7 cases of children with haemorrhagic mucosal lingual lymphangiomas, one with noteworthy drop in the haemoglobin, treated by surface radiofrequency reduction. With a short follow-up (3-13 months), a functional improvement was observed in every case, as a complete disappearance of bleeding in 5/7 cases, and a moderate local relapse in 2/7 cases. This technique did not induce progressive regrowth. It provides a new therapeutic tool for the treatment of lingual microcystic lymphangioma.

The objective of this retrospective study is to evaluate the efficiency of hyperosmolar therapy for cerebrum spinal fluid (CSF) leakage in cochlear implant (CI) surgery in children with inner ear malformations. Between 1991 and 2006, 490 cochlear implantations were performed in Armand Trousseau Children's Hospital. Thirty-seven patients (7.5%) had inner ear malformation. They were classified as isolated enlargement of the vestibular aqueduct (EVA) (18 cases), incomplete partition (IP) (11 cases), common cavity (CC) (1 case) and variable canal and vestibular malformations (VSCC) (7 cases). A hyperosmolar protocol was applied during surgery to 13 patients after 2003 (Gp) to be compared to the 24 patients without treatment previously to this date (G0). Mean age at implant CI was 8.1 years (1-20 years), mean follow up was 3.9 years (1 month-15 years). Per operative observations were collected for all patients with an empiric method of evaluation of the leakage. A grading using five steps ranged from Grade 0 (no leak) to Grade 4 (gusher). Grading, complications and perceptive results in closed and open set word (Lafon lists) at respectively preoperatively, at 3 and 24 months were gathered and compared between the two groups. Important per operative leak was observed (Grade 4) in 24.3% cases (9/37) of Grade 4, 88.8% of them in G0 (8/9). In 66.6% cases there was a severe dysplasia (CC or IP) (6/9), to be compared to the 21.4% of cases of severe dysplasia with Grade 0.05). Grade 4 was seen in 16.6% cases of EVA (3/18); there were no Grade 4 observed in Gp (0/10), and 37.5% cases of EVA in G0 (3/8) (p=0.04). Grade 4 was observed in 100% case in CC in the G0 (1/1). Severe complications were misplacement of the electrode in one case (G0), persistent leakage in one case (G0) and meningitis in one case (Gp). Vertigo was observed in 29.7% of cases (11/37) in this population, 72.7% of them in G0 (8/11). Vertigo was associated to severe dysplasia in 75% cases in G0 (6/8), and to EVA in all cases in Gp. In G0, mean perceptive scores showed for G0, preoperatively and at 3 months, respectively, 1.3% and 50.6% in closed set word (CSW), and 65.9% in open set words at 2 years. In Gp mean perceptive scores showed preoperatively and at 3 months, respectively 6.1% and 69.8% in CSW, and 81% in open set words at 2 years. The differences between the two groups are not significant (p>0.05). Osmotherapy is known to be effective for cerebral oedema and regularly used in neurological surgery. In inner ear malformation, gusher at surgery is directly related to the intra-cerebral pressure (ICP). Corresponding to neurosurgical practice, the mainstay of our protocol rests on hyperosmolar treatment, to reduce the ICP the time of the surgery. Our results suggest that this treatment is effective for a better control of leakage at cochleostomy on EVA, and could be effective on more severe malformations. No severe complication related to surgery was seen in Gp. Its good tolerance could allow its use in most patients with inner ear malformation. Vertigo was a frequent complication. The possibility of vertigo depends on the initial vestibular status and on the course of the surgery. The protocol could protect the vestibular function, lowering the pressure and quantity of the liquid issue. The treatment does not seem to influence the perceptive results.

We report on two sporadic and two familial new cases with sensorineural hearing impairment and ovarian dysgenesis which are the cardinal signs of Perrault syndrome in females. Only one of them has a nervous system defect. We reviewed all the published cases of Perrault syndrome in order to define the clinical variability and to evaluate the frequency of the neurological anomalies in this clinical entity. Moreover we excluded GJB2, POLG, and FOXL2 as candidate genes in Perrault syndrome.

The aim of the study was to evaluate noninvasive continuous positive airway pressure as first-line treatment for upper airway obstruction in neonates with Pierre Robin sequence. Forty-four neonates were enrolled and classified in 4 groups: ventilator-dependent patients (severe upper airway obstruction group), patients with clinical upper airway obstruction, an abnormal (moderate upper airway obstruction group) or normal polygraphy (mild upper airway obstruction group), and those with no clinical upper airway obstruction (no upper airway obstruction group). Five neonates in the severe upper airway obstruction group (n = 9) were successfully managed by noninvasive continuous positive airway pressure and 4 required a tracheotomy. The moderate upper airway obstruction group (n = 4) was successfully managed by noninvasive continuous positive airway pressure and the other patients by prone positioning. Continuous positive airway pressure represents an effective treatment of upper airway obstruction in Pierre Robin sequence and may be recommended in selected patients.Therapeutic, IV.

Tracheostomy is an age-old technique that has undergone a remarkable transformation in recent years, both in its indications and in the profi le of the tracheostomized patients. Until some 40 years ago, it was considered a short-term emergency procedure, mainly to resolve acute upper-airway obstruction, mostly for infectious causes, such as diphtheria, epiglottis, or laryngotracheitis. Despite the decrease of these pathologies, the incidence of tracheostomy in children has remained stable over the past decades.

Introduction Rhabdomyosarcoma (RMS) is the most frequent soft-tissue sarcoma in children and makes up 5% of all pediatric malignant tumors. The main head and neck locations are the base of the skull, nasopharynx, nasal cavity and orbit. An outer ear location is considered extremely rare. We present 3 cases of children, aged 6 to 14 years, presenting with auricular RMS. Case reports The first child, aged 6, was managed by 4 chemotherapy cycles followed by surgical resection of the tumor bed, completed by 5 further cycles of chemotherapy. The second, aged 14, was managed by 4 chemotherapy cycles followed by external radiation therapy of the tumor bed and lymph node areas, completed by 5 further cycles of chemotherapy. The third, aged 13, was managed by 4 chemotherapy cycles followed by surgery, completed by 5 further cycles of chemotherapy. Discussion In these 3 patients, the treatment program achieved complete disease control. Prognosis was good, thanks to good surgical access. Diagnosis should be considered in case of unusual progressive swelling in the outer ear.

Aim To analyze the indications and outcomes of open neurosurgical approaches (ONA) and endoscopic transnasal approaches (ETA) in the surgical management of pediatric sinogenic subdural and epidural empyema. Material and methods Retrospective single-center study design within a tertiary care referral center setting. Children less than 18 years of age consecutively operated on between January 2012 and February 2014 for drainage of a sinogenic subdural empyema (SE) or epidural (EE) empyema were included. Main outcome measures: success of first surgical procedure, persistent symptoms and sequelae at the end of the follow-up period. Results Nine SE (53%) and 8 EE (47%) were observed. Neurological symptoms, especially seizures, were more frequent in the SE group. Perioperative pus samples were positive in 67% of the SE group and in 75% of the EE group. The most frequently isolated bacteria belonged to the Streptococcus anginosus group. CT or MR imaging showed that most empyema probably originated from the frontal sinus. However, two cases resulted from an ethmoiditis and one case from a Pott's puffy tumor, without any direct contact with the paranasal sinus. In cases of SE, the most effective surgical technique was ONA with craniotomy. Associated endoscopic sinus drainage was useful for the purpose of bacteriological diagnosis. In cases of EE, effectiveness was noted in both ONA and ETA techniques. In two cases of EE, the ETA procedure encompassed direct drainage of the empyema through the posterior wall of the frontal sinus (Draf III approach). The number of patients successfully treated after a single surgical procedure was higher in the EE group (p = 0.05). Regarding outcomes, no mortalities were observed. Persistent disorders at the end of the follow-up period, especially headaches, cognitive, concentration or schooling problems, tended to be more frequent in the SE group than in the EE group (67% vs 29%), and were more commonly observed in cases requiring several surgical procedures (75% vs 12.5%) (p = 0.05). Discussion Endoscopic sinus surgery plays a critical role in the surgical management of pediatric sinogenic SE and EE. In cases of small volume EE, the endoscopic approach associated with antibiotherapy may be sufficient to treat the infectious process.

Cherubism is a rare genetic disorder characterized by extensive growth of a bilateral granuloma of the jaws, resulting in facial disfigurement. Cherubism is caused by gain-of-function mutations in the SH3BP2 gene, leading to overactivation of nuclear factor of activated T cells, cytoplasmic 1 (NFATc1)-dependent osteoclastogenesis. Recent findings in human and mouse cherubism have suggested that calcineurin inhibitors might be drug candidates in cherubism medical treatment. A 4-year-old boy with aggressive cherubism was treated with the calcineurin inhibitor tacrolimus for 1 year, and clinical, radiological, and molecular data were obtained. Immunohistologic analysis was performed to compare preoperative and postoperative NFATc1 staining and tartrate resistant acid phosphatase (TRAP) activity. Real-time PCR was performed to analyze the relative expression levels of OPG and RANKL. After tacrolimus therapy, the patient showed significant clinical improvement, including stabilization of jaw size and intraosseous osteogenesis. Immunohistologic analyses on granuloma showed that tacrolimus caused a significant reduction in the number of TRAP-positive osteoclasts and NFATc1 nuclear staining in multinucleated giant cells. Molecular analysis showed that tacrolimus treatment also resulted in increased OPG expression. We present the first case of effective medical therapy in cherubism. Tacrolimus enhanced bone formation by stimulating osteogenesis and inhibiting osteoclastogenesis.

Juvenile recurrent respiratory papillomatosis is the most common benign neoplastic disease of the larynx in children, characterized by numerous squamous papillomas caused by Human Papilloma Virus type 6 and 11. HPV is thought to be acquired at the time of vaginal delivery from maternal genital condylomas. Juvenile recurrent respiratory papillomatosis can be protracted by surgical interventions performed to avoid airway obstruction and extend below the vocal cords as far as the main stem bronchi. Lung involvement in Juvenile recurrent respiratory papillomatosis seems to be more prevalent than non-systematic reviews have reported until now and progression to cancer occurs in a significant proportion of these cases at a younger age than previously reported. This would suggest that closer attention should be paid to these children. We report a case of malignant transformation in a 12 year-old boy followed-up since the birth for an invasive juvenile recurrent respiratory papillomatosis with pulmonary involvement. The presence of HPV 6/11 was demonstrated by PCR analysis performed on material obtained from a metastatic vertebral lesion.

Introduction Head and neck teratoma is a rare entity. Its prognosis mostly depends on the risk of neonatal respiratory distress, its extension and potential malignancy. Surgical management must be as complete as possible to avoid recurrences and malignant transformation. The authors present a retrospective analysis of 6 cervicofacial teratomas and a review of the literature. The aim of the study was to analyse prenatal, neonatal and postnatal management of teratoma. Materials and methods Charts of children presenting with a head and neck teratoma, managed by our maxillofacial and plastic surgery unit, were analysed and antenatal, clinical, biological, radiological and pathological characteristics were collected. Surgical treatment, recurrences and surgical outcomes were analysed. Results Six patients were included: 2 with a cervical teratoma, 2 with a facial teratoma and 2 with intraoral teratomas. In 2 cases, the lesions were diagnosed antenatally and both patients required neonatal resuscitation. All the patients underwent early surgery, and 3 with complete excisions. All patients with an initial incomplete excision eventually presented a recurrence and therefore second look surgery. No malignant transformation was noted. Conclusion Early prenatal diagnosis is crucial to neonatal care. Early surgery and meticulous follow-up are critical in the long-term favourable outcome.

SummaryGranulomatous diseases are common in HIV-infected patients and are usually related to opportunistic infectious or tumoral conditions. We report three cases of uncommon granulomatous disease in HIV-infected patients who had previously received silicone and for which diagnostic investigations remained negative.

Summary Introduction Fetal rhabdomyomas are very rare benign tumors with infrequent head and neck involvement. We present the first report of a case of a 3-year-old child with an infratemporal fossa location. Material and methods Case study. Discussion Given the volume of the tumor and its difficult-to-access location, medical treatment associating methotrexate and Velbe ® was proposed, but was not effective on the tumor volume after four courses. Surgical intervention via a combined intraoral and preauricular temporal approach allowed monoblock excision. There were no postoperative complications. Conclusion Medical treatment was unsuccessful. Surgical excision controlled the lesion with no recurrence since surgery. The main differential diagnosis is rhabdomyosarcoma.

Resume Introduction Les rhabdomyomes fœtaux sont des tumeurs benignes tres rares. Les atteintes cephaliques sont peu frequentes. Nous presentons le cas d’un enfant de trois ans avec une localisation a la fosse infratemporale, ce qui n’avait jamais ete decrit auparavant. Materiel et methode Etude d’un cas. Discussion Compte tenu du volume de la tumeur et de sa localisation d’acces difficile, un traitement medical associant methotrexate et Velbe ® a ete propose, sans efficacite sur le volume tumoral apres quatre cures. Une intervention par voie combinee endobuccale et temporale preauriculaire a ete pratiquee, permettant une exerese en monobloc. Les suites operatoires ont ete simples. Conclusion Le traitement medical n’a apporte aucun benefice. L’exerese chirurgicale a permis le controle de la lesion sans recidive a ce jour. Le principal diagnostic differentiel est le rhabdomyosarcome.

Objectives/Hypothesis This study aimed to report our experience in the management of congenital laryngeal stenosis and to compare our series and results to published data in the literature. Study Design Retrospective case series. Tertiary referral center for rare pediatric head and neck malformations. Methods Medical charts of patients diagnosed with congenital laryngeal stenosis in our institution were reviewed over a 15-year period, from 1996 to 2011. Surgical treatment consisted of an endoscopic procedure, open laryngeal surgery (OLS), or a combination of both. Results Sixteen patients met the inclusion criteria for the study and were divided in two groups: the endoscopic laryngoplasty (EL) group, with patients who underwent the endoscopic procedure as first-line treatment, and the OLS group, whose patients underwent open laryngoplasty with cartilage graft as first-line treatment. Each group contained eight patients with grade II to IV congenital stenosis. All patients, except one in the EL group, achieved a good result (

Introduction In children, and specifically in infants, odontogenic myxomas are extremely rare. In infants, myxoma seems to display mostly the same clinical, radiological and pathological characteristics. This paper presents a series of odontogenic myxomas in infant patients. Materials and methods Four infant patients were included in this retrospective study. The clinical, radiological and pathological presentation was characterized and the treatment analysed. Results All patients presented with a rapidly evolving paranasal swelling. CT-scan showed a maxillary homogeneous unilocular and intraosseous tumour. In all cases, pathological examination revealed a loose myxoid stroma within stellate and spindle shaped cells. All patients underwent conservative surgery through a vestibular approach. Conclusion This patient series and a review of the literature demonstrates that odontogenic myxoma is specific in infant. We propose the name of Infant Odontogenic Myxoma for this entity.

Obstructive diseases of children's upper airways are frequent, mostly caused by tonsils and adenoids hypertrophy. A nasal septum deviation or a lower turbinate hypertrophy may also cause a significant obstruction. Tonsillectomy and adenoidectomy must be performed in case of clinical obstruction, sometimes documented by a sleep study. A tonsils' hypertrophy without significant obstruction and clinical symptoms is not an indication of adenotonsillectomy, even in case of snoring or if an orthodontic treatment is planned.

International audience; INTRODUCTION: Noninvasive continuous positive airway pressure (CPAP) is recognized as an effective treatment for severe airway obstruction in young children. The aim of the present study was to compare a clinical setting with a physiological setting of noninvasive CPAP in infants with nocturnal alveolar hypoventilation due to severe upper airway obstruction (UAO) or bronchopulmonary dysplasia (BPD). METHODS: The breathing pattern and respiratory muscle output of all consecutive infants due to start CPAP in our noninvasive ventilation unit were retrospectively analysed. CPAP set on clinical noninvasive parameters (clinical CPAP) was compared to CPAP set on the normalization or the maximal reduction of the oesophageal pressure (Poes) and transdiaphragmatic pressure (Pdi) swings (physiological CPAP). Expiratory gastric pressure (Pgas) swing was measured. RESULTS: The data of 12 infants (mean age 10 ± 8 mo) with UAO (n = 7) or BPD (n = 5) were gathered. The mean clinical CPAP (8 ± 2 cmH₂O) was associated with a significant decrease in Poes and Pdi swings. Indeed, Poes swing decreased from 31 ± 15 cmH₂O during spontaneous breathing to 21 ± 10 cmH₂O during CPAP (P < 0.05). The mean physiological CPAP level was 2 ± 2 cmH2₂O higher than the mean clinical CPAP level and was associated with a significantly greater improvement in all indices of respiratory effort (Poes swing 11 ± 5 cm H₂O; P < 0.05 compared to clinical CPAP). Expiratory abdominal activity was present during the clinical CPAP and decreased during physiological CPAP. CONCLUSIONS: A physiological setting of noninvasive CPAP, based on the recording of Poes and Pgas, is superior to a clinical setting, based on clinical noninvasive parameters. Expiratory abdominal activity was present during spontaneous breathing and decreased in the physiological CPAP setting.

Adenoidectomy is a commonly performed surgery in children, even though its effectiveness is still under investigation. However, in children with risk factors such as age under 3 years old, associated comorbidities, or severe obstructive sleep apneas, a high postoperative respiratory morbidity is possible. We report the case of a 15-month-old boy with Noonan syndrome and a complex clinical history, who presented with a life-threatening obstructive sleep apnea due to hypertrophy of the adenoids which resolved completely after adenoidectomy.

Background Congenital facial paralysis is a rare disorder with an incidence of less than 1 in 1000 live births. Several surgical rehabilitation techniques have been described, such as free flaps or transpositions of the temporalis muscle. We report our experience of transposition of the temporalis muscle in this indication. Materials and methods Retrospective monocentric study between January 2005 and December 2009. Results Five children (mean age at the surgery: 7.3 years, range 6–13) presenting with a congenital facial paralysis were treated by a temporalis muscle transposition during the considered period. An etiological assessment was achieved by carrying out a petrous bone CT, a cerebral and petrous MRI, and audiometric evaluation. Four patients out of 5 (80%) showed a satisfactory and stable functional outcome over time (average follow-up: 26 months). Conclusion Temporalis muscle transposition is a single-stage procedure, easily adapted for the pediatric condition of congenital facial paralysis. It is our opinion that this procedure needs to be carried out at the end of the pre-school period.