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

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

Prognostic Factors and Management of Patients with Choanal Atresia.

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

Rhabdomyosarcoma of Stensen's duct in children.

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

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

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

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

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

Multicenter evaluation of the digisonic SP cochlear implant fixation system with titanium screws in 156 patients.

OBJECTIVES: We describe and evaluate the process of fixation of the Digisonic SP cochlear implant with two titanium screws. METHODS: The characteristics of this implant allow cochlear implantation using a minimal incision, a subperiosteal pocket, and fixation with two titanium screws, without drilling a custom-fitted seat or creating suture-retaining holes in the skull. The fixation system relies on two tailfins for use of osseo-integratable screws, incorporated into the cochlear implant housing. The first version of this fixation system was modified after a case of device migration: the size of the titanium insert inside the silicone tailfin was increased. Data on 156 patients (8 months to 86 years of age) from a 4-year period in 6 cochlear implantation centers were retrospectively evaluated. Ten patients have undergone bilateral implantation. RESULTS: Of 166 implantations, 4 postoperative infections and 1 device failure after head trauma were reported. No cerebrospinal fluid leaks or epidural hematomas were reported. One device migration was observed in the first series; no device migrations occurred in the second series. CONCLUSIONS: The fixation system with screws embedded in the Digisonic SP involves a fast and simple surgical technique that seems to efficiently prevent implant migration.

Numerical simulation of nasal airflows and thermal air modification in newborns.

Warming, filtering, and humidification of inspired air are major functions of the upper airway, which can be negatively altered by local disorders or surgical interventions. These functions have not been described in neonates because of ethical and technical problems difficult to solve. Numerical simulations can get around these limitations. The objective of this study was to analyze physiological nasal airflow and thermal distribution using computational fluid dynamics (CFD) techniques in neonates. CT imaging of neonates was collected from the Pediatric Radiology Department of our center. CFD has been used to simulate nasal airflow numerically, with ambient air set at 19 °C, following the recommendations for a neonate's bedroom. Thermal distribution within the nasal cavity was analyzed and coupled with airflow patterns over complete respiratory cycles. Sixteen patients have been included in the study. During inspiration, important air warming is noticed in the first centimeter of the nasal cavity (+ 8 °C at the anterior end of the inferior turbinate). During the expiration phase, the temperature decreases slightly (- 3 °C) between the pharynx and the nostrils. A model with asymmetric nasal fossae showed that nasal obstruction leads to decreased airflow and abnormally high temperatures in the obstructed side (+ 2 °C at the nasal valve, + 4 °C at the choana). According to our results, the nasal valve area is of crucial importance in air warming in neonates, when ambient air is 19 °C, since about 70% of air warming is performed in this area. When needed, surgical interventions should respect the anatomy of this zone and restore normal airflows and warming. Graphical abstract .

International Pediatric Otolaryngology Group (IPOG) Consensus Recommendations: Congenital Cholesteatoma.

OBJECTIVE: To provide recommendations to otolaryngologists and allied physicians for the comprehensive management of children who present with signs and symptoms of congenital cholesteatoma. METHODS: A two-iterative Delphi method questionnaire was used to establish expert recommendations by the members of the International Pediatric Otolaryngology Group, on the preoperative work-up, the perioperative considerations, and follow-up. RESULTS: Twenty-two members completed the survey, in 14 tertiary-care center departments representing 5 countries. The main consensual recommendations were: a precise otoscopic description of the quadrants involved, extensive audiological workup (bilateral tonal, vocal audiometry, and BERA), and a CT scan are required. Facial nerve monitoring and a combination of microscope and telescope are recommended for surgical removal. Clinical and audiological follow-up should be pursued yearly for at least 5 years. First MRI follow-up should be done at 18 months postoperatively if the removal violated the matrix. MRI follow-up duration depends on the initial extent of the cholesteatoma. CONCLUSION: The goal of preoperative and follow-up consensus from International Pediatric Otolaryngology Group participants is to help manage infants and children with congenital cholesteatoma. The operative techniques may vary, and experienced surgeons must perform these procedures.

International Pediatric Otolaryngology Group (IPOG): Juvenile-onset recurrent respiratory papillomatosis consensus recommendations.

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.

Laser cordotomy for the treatment of bilateral vocal cord paralysis in infants.

OBJECTIVE: Vocal cord paralysis is the second cause of neonatal stridor. Several surgical treatments are proposed in order to avoid tracheotomy or to decanulate patients. Laser posterior partial cordotomy is supposed to be a minimal invasive procedure. The purpose of the study is to share our experience in management of laryngeal paralysis with this technique in infants and appreciate its role in avoiding tracheotomy in infants. DESIGN: Retrospective study. PATIENTS: The charts of 0-2-year-old patients treated for vocal cord paralysis from 1996 to 2007 are reviewed. Eleven infants with bilateral laryngeal paralysis in adduction presented severe dyspnoea. Tracheotomy was performed in four out of them, proposed in five others. One infant out of 11 underwent long-term intubation, and one presented with progressive dyspnoea. RESULTS: The laser posterior partial cordotomy allowed the decanulation after one session (n=2) or avoided tracheotomy (n=5), one patient had significant improvement of his respiratory function. Two patients needed a second session of laser cordotomy and were decanulated. The functional results for the voice and swallowing qualities were subjectively satisfactory. One patient had pejorative evolution. CONCLUSION: Posterior partial cordotomy is an effective, minimal invasive technique which can be proposed to avoid tracheotomy in infants with bilateral adduction vocal cord paralysis. No functional sequelae were observed.

Fourth branchial anomalies: Predictive factors of therapeutic success.

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.

International Consensus Recommendations on Microtia, Aural Atresia and Functional Ear Reconstruction.

The aim of this report is to provide international recommendations for functional ear reconstruction in patients with microtia and aural atresia. All patients with microtia and external auditory atresia should be seen in the setting of a multidisciplinary team and agreed treatment outcomes should be measured, so that techniques, approaches, and results can be compared. The methods are expert opinion from the members of the International Microtia and Atresia Workgroup (IMAW). The consensus recommendations reported herein take into account the variability in practice patterns present among experts in the field; the degree of consensus was quantified by presenting the percentage of above authors who agree or partially agree with each statement. Recommendations include the definition and classification of microtia/atresia, treatment of microtia, treatment of congenital aural atresia, flowchart of functional ear reconstruction, and future research directions. Patients with microtia and aural atresia can be guided by the consensus recommendations provided herein.

Surgical management of children presenting with surgical-needed tracheal stenosis.

OBJECTIVES: The purpose of this work was to assess epidemiological aspects, surgical approach, morbidity and mortality rates of patients presenting with tracheal stenosis requiring surgery, and the evolution of surgical techniques over the last years. METHODS: We performed a retrospective observational study from 1990 to 2017 in a pediatric tertiary-care center with needing surgery for tracheal stenosis. We analyzed clinical patients' characteristics, type of stenosis, type of surgery and follow-up. RESULTS: Twenty-eight children presented with tracheal stenosis, half of them with congenital stenosis (complete tracheal rings) and the other half with acquired stenosis (neoplasic or post intubation injury). 39.3% of these stenoses were associated with a vascular ring (61.5% in case of congenital stenosis). Depending on the extent of the stenosis and its origin, the surgery could be performed endoscopically or by an external approach. Enlargement tracheoplasty with an autograft (14.3%) was replaced by slide tracheoplasty with Cardio Pulmonary By-Pass (CPBP, 28.6%) with improved results for the treatment of long segment tracheal stenosis, involving more than 30% of the tracheal length (all were congenital in our study). Slide tracheoplasty has been performed since the late 90's in our institution. 25% of children have had a resection and anastomosis of the trachea because they had a stenosis involving less than 30% of tracheal length. Endoscopic surgery was performed for membranous stenoses, which were often seen after intubation or tracheotomy (32.1% of patients). CONCLUSION: Effective treatment of surgical tracheal stenosis was performed in 28 children between 1990 and 2015. Surgical techniques have evolved over time, leading to a better management of this rare and serious disease.

Solitary parathyroid adenoma: a rare cause of primary hyperparathyroidism in children.

Solitary parathyroid adenoma is a rare cause of primary hyperparathyroidism in children. We report the cases of four children, aged 7 to 14 years old, who presented with a variety of symptoms (growth retardation, glomerulonephritis, pathological fracture, mood swings). Diagnosis was based on clinical examination, imaging, and biochemical analysis. Treatment consisted of surgical excision of the adenoma. As diagnosis was delayed in all four patients, we recommend systematically checking serum calcium levels in children with certain nonspecific symptoms. Adenoma resection usually restores normal serum calcium levels and, hence, avoids further complications.

Objective tinnitus associated with essential palatal myoclonus: report in a child.

Palatal myoclonus is an uncommon, rhythmic, "shock-like" involuntary movement of the muscles of the soft palate, throat, and other structures derived from the branchial arcs. Objective tinnitus is frequently neglected in review articles about childhood tinnitus. Our aim was to present the case of a 7-year-old girl with bilateral objective tinnitus due to palatal myoclonus without hearing impairment (normal hearing thresholds between 250 Hz and 8 kHz) but with otherwise normal hearing thresholds (250 Hz-8 kHz) and no evidence of intracerebral or systemic disorders. No treatment was useful.

[Acute laryngitis and epiglottitis in children]. / Laryngite aiguë et épiglottite de l'enfant.

The anatomical characteristic of the pediatric larynx allows physicians to better understand the incidence of symptomatic and severe presentations of acute laryngitis, which are frequent pediatric emergencies. Subglottis laryngitis and epiglottitis must be distinguished from each other. These two diseases are absolutely different: the first one is essentially viral and usually moderate, even though acute respiratory distress can occur. The other (epiglottitis) is bacterial, essentially caused by Haemophilus influenza B (Hi-B), and can be life threatening. The anti Hi-B vaccine leads to a decrease of frequency but does not make them disappear. Moreover, even if a child has a history of the Hi-B vaccine, diagnosis of epiglottitis can not to be ruled out. Lastly, in case of acute laryngeal dyspnea in a child, one must think about a foreign body.

International Pediatric Otolaryngology Group: Consensus guidelines on the diagnosis and management of type I laryngeal clefts.

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.

Congenital stapes ankylosis: study of 28 cases and surgical results.

OBJECTIVE: The objective of this study was to analyze functional results after stapes surgery in patients with congenital nonprogressive conductive deafness resulting from an isolated fixation of the stapes according to age and surgical procedure. STUDY DESIGN: The authors conducted a retrospective case series from March 1993 to December 2003 in patients from two tertiary referral centers. METHODS: Twenty-eight patients were operated on by stapedotomy or partial stapedectomy using Teflon stapes prostheses. The median age at surgery was 14.2 years (range, 8.3-29.1 years). Main outcome measures were clinical and audiometric evaluation before and after surgery. Mean air conduction (MAC) and bone conduction (MBC) thresholds were recorded at 0.5, 1, 2, and 4 kHz. The evaluation of functional outcome was based on the MAC gain, the MBC comparison, and the mean postoperative and residual air-bone gaps. RESULTS: The median preoperative MAC was 50 dB (range, 19.0-65.0 dB) with a 35.0 dB median dB air-bone gap. With a mean follow up of 19 months, postoperative hearing improvement was statistically significant: median gain of 32.5 dB (P<.001) and median residual air-bone gap of 3.5 dB. The MBC was also statistically improved with median pre- and postoperative MBC of 11.5 and 6.5 dB, respectively (P<.001). Results were not dependent on the age group or type of surgery (stapedotomy or partial stapedectomy). No perceptive hearing loss was observed despite one gusher case. CONCLUSION: Surgical treatment of isolated congenital stapes ankylosis allows good functional results regardless of age or type of surgery.

Surgical repair of laryngotracheoesophageal clefts by tracheoesophagoplasty with two overlapping flaps.

We report a technique for surgical repair of laryngotracheoesophageal clefts from type II to IV through an anterior approach that involves performing a tracheoesophagoplasty with two overlapping flaps (TEP-TOF). We offer a technical description, a retrospective study, and a review of the literature. Ten children were operated on for laryngotracheoesophageal clefts of types II, III, or IV of the Benjamin and Inglis classification by the TEP-TOF procedure. After a median follow-up of 58 months (2 months to 12 years), neither complications nor recurrences were observed. All surgical details of the TEP-TOF procedure, including the approach, the kind of sutures, the method of creating the flaps, and the preoperative and postoperative management, are discussed and compared with the data found in the literature.

Auditory training improves auditory performance in cochlear implanted children.

UNLABELLED: While the positive benefits of pediatric cochlear implantation on language perception skills are now proven, the heterogeneity of outcomes remains high. The understanding of this heterogeneity and possible strategies to minimize it is of utmost importance. Our scope here is to test the effects of an auditory training strategy, "sound in Hands", using playful tasks grounded on the theoretical and empirical findings of cognitive sciences. Indeed, several basic auditory operations, such as auditory scene analysis (ASA) are not trained in the usual therapeutic interventions in deaf children. However, as they constitute a fundamental basis in auditory cognition, their development should imply general benefit in auditory processing and in turn enhance speech perception. The purpose of the present study was to determine whether cochlear implanted children could improve auditory performances in trained tasks and whether they could develop a transfer of learning to a phonetic discrimination test. MATERIAL AND METHODS: Nineteen prelingually unilateral cochlear implanted children without additional handicap (4-10 year-olds) were recruited. The four main auditory cognitive processing (identification, discrimination, ASA and auditory memory) were stimulated and trained in the Experimental Group (EG) using Sound in Hands. The EG followed 20 training weekly sessions of 30 min and the untrained group was the control group (CG). Two measures were taken for both groups: before training (T1) and after training (T2). RESULTS: EG showed a significant improvement in the identification, discrimination and auditory memory tasks. The improvement in the ASA task did not reach significance. CG did not show any significant improvement in any of the tasks assessed. Most importantly, improvement was visible in the phonetic discrimination test for EG only. Moreover, younger children benefited more from the auditory training program to develop their phonetic abilities compared to older children, supporting the idea that rehabilitative care is most efficient when it takes place early on during childhood. These results are important to pinpoint the auditory deficits in CI children, to gather a better understanding of the links between basic auditory skills and speech perception which will in turn allow more efficient rehabilitative programs.