Search

Purpose To report our experience with endoscopic Thulium LASER for treatment of recurrent TEF after EA surgery, and for H-Type fistulas. Methods A retrospective chart review of consecutive patients undergoing standardized endoscopic closure as first line therapy of recurrent tracheoesophageal fistula (RTEF) and H-type fistula using Thulium LASER, from 2013 to 2019, in a pediatric tertiary care center. Control endoscopic procedure was systematically performed. If persistence of the TEF was noted an external approach was performed. Patient demographics, medical history, symptoms, TEF type, treatment modalities, complications and outcomes were collected. Results Eleven patients with tracheoesophageal fistula were included: six RTEF after primary repair of esophageal atresia and five H-type fistulas. The average age at endoscopic treatment was 19 months (SD 23 months, range 13 days–63 months). Closure of the fistula after single endoscopic procedure with Thulium LASER was obtained in 3 RTEF (50%) and 1 H-type fistula (20%). Six patients with failure of endoscopic treatment were cured after a single external procedure without any complications. One child, treated for H-type fistula, presented a severe complication of Thulium LASER treatment. Median follow-up after last repair was 24 months (range: 14–72 months). All fistulas were successfully treated. Conclusions In H-Type fistula, success rate of Thulium LASER is only 20% and thus should not be used. In contrast, in RTEF, success rate of 50% is achieved, avoiding as many open procedures, and Thulium LASER could be considered as first line treatment. In any case, open surgery is safe and efficient and can be considered as a first-line treatment for H-type fistulas, and as a salvage treatment for endoscopic treatment failures. Levels of Evidence Level IV.

Objectives To report an unusual complication of pediatric acute maxillary sinusitis: premaxillary abscess. To describe clinical, radiological and biological presentation, treatment strategy and progression. Material and methods A retrospective study included all pediatric patients treated for premaxillary abscess complicating acute maxillary sinusitis in two ENT reference centers between 1999 and 2017. Disease history, clinical presentation, biological and radiological findings, treatment modalities and progression were studied. Results Ten patients were included, with a mean age of 10 ± 4.2 years. All presented with fever, rhinorrhea and premaxillary edema. Contrast-enhanced CT scan systematically found complete opacity of the maxillary sinus, without bone lysis, and extensive effusion along the intersinonasal wall up to the premaxillary region, extending in 3 cases back toward the parapharyngeal space. Bacteriology isolated Streptococcus anginosus most frequently (n = 4; 40%). Treatment comprised intravenous wide-spectrum antibiotics, with surgical drainage of the abscess if > 10 mm (n = 9; 90%). Seven of these 9 patients (78%) had recurrent abscess requiring surgical revision and 3 (33%) required a third drainage. All patients were cured without sequelae at 1 month. Conclusion In case of acute maxillary sinusitis with premaxillary edema, premaxillary abscess should be suspected. The high recurrence rate argues for maximalist surgery associated to close clinical monitoring with radiological examination.

Purpose The purpose of this work was to determine the epidemiology and the predictive factors of success of the surgical management of fourth branchial anomalies. Methods This is a multicentric retrospective review from 1998 to 2016 of patients who presented with an endoscopically-confirmed fourth branchial pouch anomaly. Data were analyzed according to sex, age, clinical features, number of recurrences, treatment modalities (endoscopic and/or cervicotomy), post-operative complications and follow-up. Results Fifty-two children have been included. The average age at diagnosis was 4.5 years. Among them, 73.1% were female, 11.4% were neonatal forms; 94.2% of lesions were left-sided; 75% of patients presented a cervical abscess as first symptom, and 7.7% of children presented with dyspnea. Average time between first symptoms and management was 9.5 months. Management was endoscopic in 73.1% of patients (laser in 84.2%, coagulation in 15.8%) with about a third of recurrence after one procedure. Overall success of endoscopic procedures reached 84.2%. A cervical open surgery was performed in 26.9% as first line treatment. Overall success of cervicotomy reached 85.7%. No complications of endoscopic surgery have been identified. There were 35.7% complications of cervicotomy (2 recurrent nerve palsy, 2 keloid scars, 1 pharyngostoma). An association was proved between recurrences and initial abscess (OR = 2.44), and with age between 3 and 5 (OR = 4). Conclusion Endoscopic treatments appear to be effective in first line approach in the management of fourth branchial anomalies, offering an excellent efficiency with rare complications. We identified two risk factors of recurrence: age between 3 and 5 years old and history of cervical abscesses. Level of evidence IV.

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 describe malformations associated with pediatric congenital cholesteatomas of the middle ear. STUDY DESIGN Retrospective study. SETTING Tertiary referral center. PATIENTS One hundred and seventy-three cases of middle ear congenital cholesteatoma (CC) in 171 children operated between 2007 and 2017. INTERVENTIONS Demographic, clinical, and surgical data were collected from operative reports. MAIN OUTCOME MEASURES We first described the type and rate of malformations associated with CC. Secondly, we compared cholesteatoma features in two subgroups: anterior superior (AS) versus posterior superior (PS) starting point. Third, we compared demographic, clinical, and surgical data between patients with and without malformation. RESULTS CC was associated with malformations in 17 cases (17/173; 9.8%). The main malformation was preauricular fistula (8/173; 4.6%). Other malformations were: one first branchial cleft, two labio palatine cleft, one nasal cyst, two preauricular fibrochondroma, and five other malformations. PS congenital cholesteatomas were diagnosed in older children (4.6 versus 8.6 years, p

Objectives Systematic review of the literature on myringoplasty techniques without tympanomeatal flap elevation in children. Material and methods A systematic review following PRISMA guidelines reported papers on patients under 18 years of age undergoing myringoplasty for chronic tympanic perforation on a transcanal approach without tympanomeatal flap elevation. Tympanic closure rates and audiometric results were analyzed. Results Twenty studies were included. Nine reported the butterfly technique, using a microscope or endoscope, with closure rate of 82.3% (246/299), for perforations of various sizes. Ten reported the fat-plug technique, with closure rate of 86.8% (869/1001), mostly for perforations of less than one-third of the tympanum. Both techniques improved audiometric results. Morbidity was very low. The absence of chronic otitis or co-morbidities (contralateral otitis media with effusion, craniofacial malformations, Down's syndrome) implies that patient selection technique may be necessary to obtain the best results. Conclusion Fat-plug myringoplasty, for small perforations, and butterfly cartilage myringoplasty seem to be reliable procedures in selected patients, with low morbidity in children.

Objective Joint guidelines of the French Pediatric Otolaryngology Society (AFOP) and of the French Society of otorhinolaryngology–head and neck surgery (SFORL) on the management of paediatric otolaryngology patients in the context of the COVID-19 pandemic. Methods A nation-wide workgroup drew guidelines based on clinical experience, national and local recommendations and scientific literature. Proposals may have to be updated on a day-to-day basis. Results In children, incidence of symptomatic COVID-19 (1–5%) is low and of good prognosis. The indications for nasal flexible endoscopy should be drastically limited. If undertaken, full Personal Protective Equipment (PPE) including FFP2 masks are required, as well as use of a sheath. Saline nose wash done by caregivers other than parents at home should require PPE. Unless foreign body tracheobronchial aspiration is clinically obvious, CT-scan should be performed to confirm indication of endoscopy. Surgical indications should be limited to emergencies and to cases that cannot be delayed beyond 2 months (especially endonasal, endopharyngeal laryngo-tracheobronchial procedures). Postponement should ideally be a group decision and recorded as such in the medical file. Surgical techniques should be adapted to limit the risk of viral dissemination in the air, avoiding the use of drills, microdebriders, monopolar cautery or lasers. Continuous suction should be placed near the operating field. In case of confirmed Covid-19 cases, or suspected cases (or in some centres systematically), PPE with FFP2 mask should be worn by all staff members present in the operating room.

Objectives The authors present the clinical practice guidelines of the French Society of Oto-Rhino-Laryngology and Head and Neck Surgery (SFORL) concerning the role of the ENT specialist in the management of pediatric obstructive sleep apnea hypopnea syndrome (POSAHS). Part 3 is dedicated to the place of sleep recordings in the diagnosis of POSAHS. Methods A multidisciplinary work group was commissioned to carry out a review of the scientific literature on the above topic. Based on the articles retrieved and the group members’ individual experience, guidelines were drafted and graded as A, B or C or Expert Opinion by decreasing level of evidence, then reviewed by an editorial group independent of the work group. Results Sleep recordings are presented according to the American Sleep Disorders Association's classification as type 1, 2, 3 or 4. Their modalities, interpretation, indications, advantages and limitations are detailed.

OBJECTIVES 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. METHODS 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. RESULTS 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. CONCLUSION 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. LEVEL OF EVIDENCE 5 Laryngoscope, 129:244-255, 2019.

International audience; The Société française d'ORL et de chirurgie cervico-faciale clinical practice guidelines concern the management of otitis media with effusion (OME) in children under the age of 12 years. They are based on extensive review of MEDLINE and Cochrane Library publications in English or French from 1996 to 2016 concerning the methods of diagnosis and assessment of otitis media with effusion, as well as the efficacy of tympanostomy tubes and medical and surgical treatments of OME.

Objectives To study recent cases of esophageal injury due to button-battery ingestion in children presenting in pediatric ENT emergency departments of the Paris area of France (Ile-de-France region), in order to propose appropriate preventive measures. Material and method A retrospective descriptive single-center study included all children under 15 years of age, presenting in pediatric ENT emergency departments between January 2008 and April 2014 for button-battery ingestion with esophageal impaction requiring emergency removal. Results Twenty-two boys and 4 girls, with a median age of 25 months, were included. Twenty-five of the 26 batteries had diameters of 20 mm or more. Median esophageal impaction time was 7 hours 30 minutes (range, 2 to 72 hours). The complications rate was 23%. Mean hospital stay cost was €38,751 (range, €5130–119,737). The origin of the battery was known in 23 of the 26 cases: remote control without screw-secured compartment (42.3%), open battery pack (15.4%), children's toy (15.3%), camera (7.7%), watch (1 case) and hearing aid without screw-secured compartment (1 case). Conclusion Esophageal lesions due to ingestion of button-batteries in children are almost always due to batteries larger than 20 mm in diameter, mostly from devices with a poorly protected compartment, or batteries that are not individually packaged. These lesions cause serious complications in a quarter of cases and their management entails high health costs. Legislation requiring screw-secured compartments and individual blisters for batteries could have prevented 69.2% of the ingestions.

Introduction The diagnosis and management of type I laryngeal clefts can be controversial and varies across centers and surgeons. Using existing peer-reviewed literature to develop an expert-based consensus will help guide physicians in the treatment of these patients as well as develop research hypotheses to further study this condition. Objective To provide recommendations for the diagnosis and management of type I laryngeal clefts. Methods Determination of current expert- and literature-based recommendations, via a survey of the International Pediatric Otolaryngology Group, using a modified Delphi method. Setting Multinational, multi-institutional, tertiary pediatric hospitals. Results Consensus recommendations include diagnostic workup, medical management, pre-operative, intra-operative and post-operative considerations for type I laryngeal clefts. Conclusions This guide on the diagnosis and management of patients with type I laryngeal clefts is aimed at improving patient care and promoting future hypothesis generation and research to validate the recommendations made here.

Introduction Velopharyngeal insufficiency (VPI) is usually managed, besides speech therapy, by performing a velopharyngoplasty. An alternative approach is autologous fat grafting (AFG) of the posterior pharyngeal wall. About 5% of the population has internal carotid arteries (ICA) with an aberrant course. This anatomic variation can be responsible for surgical difficulties while when performing a velopharyngoplasty, and therefore lead surgeons to only consider a speech reeducation of VPI. However, AFG is does not bear such surgical morbidity. Objective The aim of this study is to retrospectively determine AFG efficiency on VPI in patients with aberrant ICA's courses who cannot benefit from a velopharyngoplasty, by comparing pre- and postoperative Borel Maisonny score (BMS) and intelligibility (Intell). Methods We conducted a retrospective study in 2 centers, including children with VPI and aberrant ICA's courses who underwent an AFG of the posterior pharyngeal wall from 2004 to 2015, in addition to speech therapy. Results Nine patients (4–11 years old) underwent the surgical procedure, 8 of them presenting a 22q11 microdeletion. All improved their VPI by AFG of the pharyngeal wall according to BMS and Intelligibility after a 10 months follow-up. The effect was stable after 3 years of follow-up. No severe complication (apnea, vascular injury) occured. Conclusion AFG of the pharyngeal wall, associated with speech therapy, seems to be a safe procedure for patients with VIP and aberrant ICA's courses. Multiple procedures are possible if needed.

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.

Autologous Complete Tracheal Replacement Management of tracheal stenosis in a child is complex. This letter describes the 4-year follow-up of a child who underwent an autologous complete tracheal replacement.

Objectives To evaluate the use of P-values and the terms “significant”, “non-significant” and “suggestive” in Abstracts in the European Annals of Otorhinolaryngology, Head & Neck Diseases. Materials and methods Consecutive articles accepted for publication during the period January 2016 – February 2019 were systematically reviewed. Main goal: descriptive analysis of the citation of P-values and use of the terms “significant”, “non-significant” and “suggestive” in Abstracts. Secondary goal: analytic study of: (i) correlations between citation of a P-value and the main characteristics of authors and topics; and (ii) misuse of the terms “significant”, “non-significant” and “suggestive” with respect to cited P-values, and correlations with author and topic characteristics. Results In all, 91 articles were included. P-values and the terms “significant”, “non-significant” and “suggestive” were cited in 35.1%, 41.7%, 10.9% and 0% of Abstracts, respectively. Citing a P-value did not significantly correlate with author or topic characteristics. There were discrepancies between the terms “non-significant”, “significant” and “suggestive” and P-values given in the body of the article in 57.1% of Abstracts, with 30.7% overestimation and 25.2% underestimation of results, without significant correlation with author or topic characteristics. Conclusion Authors, editors and reviewers must pay particular attention to the spin resulting from inappropriate use of the terms “significant”, “non-significant” and “suggestive” in Abstracts of articles submitted to the European Annals of Otorhinolaryngology, Head & Neck Diseases, to improve the rigor, quality and value of the scientific message delivered to the reader.

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 Desmoid-type fibromatosis (DF) is a rare benign lesion known for its local aggressiveness. The tumor management still remains under debate. Primary head and neck (HN), represents the second most prevalently affected sitein children with DF. This study aims to analyze the specificity of HN-DF in children, focusing on long-term effects of the tumor and therapies. Methods This retrospective multicenter study analyzed children treated for a HN-DF between 1993 and 2013. All medical files were reviewed and their outcomes analyzed according to the initial therapies provided. Results Sixteen children were selected. Mandibular and submandibular areas were the main locations (11 cases). Eight children underwent chemotherapy as first-line therapy with tumor control in 3 cases and 5 cases needing additional treatment. Six children underwent primary surgery: isolated in 3 cases and with additional treatment after tumor progression in 3 cases. A wait-and-see attitude was adopted for 2 children without any additional treatment in 1 case, and followed by additional chemotherapy in the other case. Total burden of treatment to control the disease was a biopsy (1 case), surgery (3 unique cases, 1 multiple case), surgery with chemotherapy (6 cases), and exclusive medical therapies (5 cases). Surgical postoperative sequelae were facial palsy (cases of parotid gland affection), XIth cranial nerve sacrifice or sensory impairment. Conclusion HN-DF is a local and extensive disease that is difficult to control with surgery alone. Sequelae are frequent due to the initial tumor location or therapies. Initial conservative strategies need to be discussed in a multidisciplinary way in order to try to control the disease with the minimal morbidity.

Objectives To present the 2017 Clinical Practice Guidelines of the French Society of Otorhinolaryngology concerning the role of the ENT specialist in the diagnosis of pediatric obstructive sleep apnea-hypopnea syndrome. This manuscript specifically focuses on diagnostic investigations apart from sleep studies. Methods A multidisciplinary work-group drew up a first version of the guidelines, graded according to level of evidence following the GRADE grading system. The final version was obtained by including the suggestions and comments from the editorial group. Results At the end of the process, guidelines were established and graded regarding diagnostic investigations apart from sleep studies, in particular respiratory functional tests, biological markers, and morphologic assessment under induced sleep (drug-induced sleep endoscopy (DISE) and cine-MRI).

Objectives To present the 2017 Clinical Practice Guidelines of the French Society of Otorhinolaryngology concerning the role of the ENT specialist in the diagnosis of pediatric obstructive sleep apnea-hypopnea syndrome. This article focuses specifically on medical history and physical examination. Methods A multidisciplinary work-group drew up a first version of the guidelines, graded according to level of evidence following the GRADE grading system. The final version was obtained by including the suggestions and comments from the editorial group. Results At the end of the process, guidelines were established and graded regarding the following points: interview and analysis of the various interview scores recommended in the literature; clinical examination with awake upper-airway endoscopy; and indications for referral to non-ENT specialists.

International audience; Introduction: acoustic rhinometry (AR) is a non-invasive method measuring the nasal volume (NV) and the nasal minimal cross-sectional area (MCA), reflecting nasal obstruction. The first objective of this study was to measure and compare NV and MCA between 3 groups of children: "achondroplasia", "Down syndrome" and "control". The control group corresponded to children with suspicion of sleep disorder disease and without cranio-facial malformation. The second objective was to correlate AR measurements with the obstructive apnea-hypopnea index (OAHI).Methods: prospective study between February and July 2017, in a tertiary care center. The following data were collected: demographic characteristics, medical and surgical history, NV, MCA, and OAHI.Results: 83 children were included. The mean NV was lower in achondroplasia group compared to control group: 2.75 cm3 vs 3.60 cm3 (p = 0.02, 95% CI [0.0694, 0.7456]). Negative correlation was found between the NV and the OAHI for children with achondroplasia (T = -0.37; p = 0.02).Conclusions: AR is an effective tool for assessing nasal obstruction in children. Nasal obstruction was correlated to OAHI in achondroplasia. AR could become a routine tool in the management of nasal obstruction of children with cranio-facial malformations.

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.

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.

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.

Pierre Robin sequence (PRS) may lead to life-threatening respiratory and feeding disorders. With the aim to analyse the association of the severities of retrognathia and glossoptosis with those of respiratory and feeding disorders, we retrospectively studied a series of 50 infants with retrognathia, glossoptosis, cleft palate, and airway obstruction. The patients were managed from birth to at least 6 years of age by a single pediatric team at the Armand Trousseau Hospital in Paris within a 12 years period (2000–2012). Retrognathia and glossoptosis were graded in the neonatal period according to a specific clinical examination. Ventilation assistance was required for 32/50 (64%) patients, and enteral feeding for 41/50 (82%). The grades of retrognathia and glossoptosis and the severity of respiratory disorders did not differ between patients with isolated PRS and syndromic PRS. Severe respiratory disorders were more common and long-lasting feeding (>12 months) was more frequently required in patients with syndromic PRS compared with isolated PRS (42 vs. 13%, p = 0.04 and 42 vs. 4%, p < 0.01 respectively). Using univariate analysis, neurological impairments and laryngomalacia were associated with severe respiratory disorders [Odds ratio (OR) 5.0, 95% CI 1.3–19.6; and OR 14.6, 95% CI 1.3–161.4; p < 0.05] as well as with long-lasting feeding (>12 months) disorders (OR 18.6, 95% CI 3.9–89.2 and OR 20.4, 95% CI 3,4–122.8; p < 10−2). Syndromic SPR status was also associated with severe respiratory disorders (OR 4.9, 95% CI 1–32.5; p < 0.05). Using multivariate analysis, only syndromic PRS status was predictive for severe respiratory disorders (adjusted OR 8, 95% CI 1.47–44.57; p < 0.05); and only neurological impairments remained a significant risk for long lasting feeding disorders (>12 months) (adjusted OR 21.72, 95% CI 3.4–138.63; p < 10−2). The grades of retrognathia and glossoptosis were not predictive factors for the severity of respiratory and feeding disorders.Conclusion: In children with PRS, the severity of clinical conditions may not correlate with anatomic variables but rather with laryngeal abnormalities, neurological impairement and syndromic PRS status.

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 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.

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.

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.

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.