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.

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.

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.

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.

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.

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.

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.

International Pediatric Otolaryngology Group (IPOG) consensus recommendations: Hearing loss in the pediatric patient.

OBJECTIVE: To provide recommendations for the workup of hearing loss in the pediatric patient. METHODS: Expert opinion by the members of the International Pediatric Otolaryngology Group. RESULTS: Consensus recommendations include initial screening and diagnosis as well as the workup of sensorineural, conductive and mixed hearing loss in children. The consensus statement discusses the role of genetic testing and imaging and provides algorithms to guide the workup of children with hearing loss. CONCLUSION: The workup of children with hearing loss can be guided by the recommendations provided herein.

Laryngotracheal Stenosis in Children and Infants With Neurological Disorders: Management and Outcome.

INTRODUCTION: The goal of this retrospective study is to compare the management and outcome of surgical treatment of laryngotracheal stenosis in children and infants with and without an associated neurological disorder. PATIENTS AND METHOD: In a series of children operated on for subglottic stenosis (SGS), patients with an associated neurological disorder were identified. The following criteria were compared in children with and without neurological disease: grade of stenosis, age, technique (Crico-Tracheal Resection (CTR), Laryngo-Tracheo-Plasty (LTP) in single and 2 stage, laser), analyzing duration, preoperative tracheostomy, decannulation rate, preoperative gastrostomy, and number of days in intensive care unit and in hospital. RESULTS: Two hundred twenty-three children were operated on for subglottic stenosis, of whom 68 (30.5%) had an associated neurological disorder. Some criteria were found to be statistically different between the 2 populations: mean age of 43 months in neurological population versus 13 months (P < .001). The distribution of the grades of SGS appeared similar in the 2 groups (P = .088), and the mean duration of stay in hospital and in ICU were not statistically different (respectively, P = .186 and P = .056) between the 2 groups; a 2-stage procedure was performed more frequently than 1-stage in the cases with associated neurological disorder-66.6% versus 36.5% (P = .013); the median duration of stenting was 20 days in those with neurological disease versus 12 (P = .021). Preoperative tracheotomy was noted in 75% of neurological patients versus 47.7% of the others (P < .001). The outcome was considered to be good (decannulation and no further treatment) following a single procedure in 82.4% of patients with neurological disorder, as against 86.5% of neurologically unimpaired subjects. The difference in outcome of surgery was not statistically different (P = .392) between the 2 groups. DISCUSSION AND CONCLUSION: It appears that subglottic stenosis in children with associated neurological disorder is not more severe than in neurologically normal patients. In three-quarters of the neurologically impaired cases, a preoperative tracheostomy was needed, but the rates of failure of postoperative decannulation are not statistically significant between the 2 groups. In our experience, 2-stage techniques are more often performed than single stage in this population in order to allow airway safety, for example after feeding. If properly managed, the final results are similar in the 2 populations.

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.

International Pediatric Otolaryngology Group (IPOG) consensus recommendations: Routine peri-operative pediatric tracheotomy care.

OBJECTIVES: To develop consensus recommendations for peri-operative tracheotomy care 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 pre-operative, intra-operative, and post-operative considerations, as well as sedation and nutrition management are described. These recommendations are based on the collective opinion of the IPOG members and are targeted to (i) otolaryngologists who perform tracheotomies on pediatric patients, (ii) intensivists who are involved in the shared-care of these patients, and (iii) allied health professionals. CONCLUSION: Pediatric peri-operative tracheotomy care consensus recommendations are aimed at improving patient-centered care in this patient population.

Combined endonasal and neurosurgical resection of a congenital teratoma with pharyngeal, intracranial and orbital extension: Case report, surgical technique and review of the literature.

OBJECTIVES: This study reports a patient with a large teratoma involving the oropharynx, the nasopharynx and the left orbit, with intracranial extension. This case represents one of the first reported instances of such an association. A literature review reporting head and neck teratomas with intracranial involvement is also presented. CASE REPORT: The authors describe a case of a neonate presenting with a huge teratoma causing respiratory distress due to upper airway obstruction. The child was operated on at 3 months of age with a combined neurosurgical and endonasal endoscopic-assisted approach. After more than 1-year follow-up, the child has no recurrence and no complications of surgery. DISCUSSION: This type of teratoma is very rare and surgical morbidity is common. The diagnosis may be apparent before birth, which will facilitate the planning of respiratory management. The combined neurosurgical and otolaryngologic approach for a neonatal teratoma has not previously been described. Using an endoscopic-assisted approach for intranasal tumors removal in neonates and infants is a very rare surgical challenge. CONCLUSION: Large nasopharyngeal teratomas of infancy with sinonasal and intracranial extension may be managed using a combined endoscopic-assisted endonasal and neurosurgical procedure.

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.

Prenatal diagnosis of congenital dacryocystocele.

Congenital bilateral dacryocystocele was diagnosed prenatally by ultrasonography in 3 female fetuses at 32.5 weeks gestation. After birth, first baby developed respiratory distress and was treated with endoscopic marsupialization of the cysts; the second baby had no respiratory symptoms and had spontaneous resolution of the cysts without surgery. The last one was expected to have a left dacryocystocele in US but the clinical examination after birth showed a bilateral lesion, with predominance on the right side and underwent an endoscopic marsupialization for nasal obstruction. Prenatal diagnosis with ultrasonography facilitated the education of the mothers and staff and helped minimize the risk of potential complications.

Middle ear implant in conductive and mixed congenital hearing loss in children.

PURPOSE: Active middle ear implant can be used in children and adolescents with congenital hearing loss. The authors report their experience with the semi implantable Medel Vibrant Soundbridge(®) (VSB) in the audiologic rehabilitation of such patients. METHODS: In this retrospective study, audiological and surgical data of 10 children (10.5±4 years) implanted with 12 VSB in 2 tertiary cares ENT Departments were analysed. RESULTS: Two children with bilateral external auditory canal (EAC) atresia and mixed hearing loss (mean air conduction (AC) thresholds=65dB HL) were bilaterally implanted. Eight children presented with microtia associated with EAC atresia bilaterally (n=3) and unilaterally (n=5). All of them had a conductive hearing loss in the implanted ear (mean (AC) thresholds were 58.75dB HL preoperatively). The Floating Mass Transducer was crimped on the long process of the incus (n=8) or on the suprastructure of the stapes (n=4). There were no intra- or postoperative surgical complications. All the children wore their implants after 5 weeks. Postoperative mean bone conduction (BC) thresholds were unchanged. The mean aided thresholds with VSB (four frequencies warble tones at 0.5, 1, 2 and 4 kHz) were 28dB HL (± 10). Word discrimination threshold in quiet conditions in free field with the VSB unilaterally activated was 50% at 38dB SPL (± 9). CONCLUSION: The results indicate that satisfaction of the children and their parents is very encouraging but surgeons should be cautious with this new approach in relation to the pinna reconstruction and to possible risks to inner ear and facial nerve.

A preliminary study of computer assisted evaluation of congenital tracheal stenosis: a new tool for surgical decision-making.

OBJECTIVE: Congenital tracheal stenosis is a rare but severe condition with tracheal narrowing. There is no absolute correlation between luminal diameter and prognosis, and therapeutic decisions are difficult for intermediate cases. The aim of this study was to develop a dynamic model of the ventilatory consequences of congenital tracheal stenosis using computational fluid dynamics. METHODS: In 8 children with congenital tracheal stenosis and 1 healthy child, 3-dimensional geometries of the trachea were constructed with computed tomography images and specialized software (ITK-SNAP). Airflow simulations were performed for each geometry using 2 physiologic inhalation flow rates under steady and laminar flow conditions. Flow velocity, static and total airway pressure, and pressure drop across the entire trachea were determined. RESULTS: In the patients with congenital tracheal stenosis, the pressure drop from the tracheal inlet to outlet, at flow rate 3L/min, ranged from 14 to 430Pa; the pressure drop at flow rate 7.3L/min ranged from 60 to 1825Pa. The pressure drop enabled a classification based on the severity of stenosis. The classification based on pressure drop was retrospectively consistent with the classification based on clinical data from the patients. CONCLUSIONS: Simulations with computational fluid dynamics may provide an objective method to evaluate the severity of the symptoms in patients with congenital tracheal stenosis and may help guide treatment.

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

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

Cochlear implant electrode array misplaced in Hyrtl's fissure.

Hyrtl's fissure is a cleft that is present in the developing fetal petrous temporal bone and extends from the area inferior to the round window to the meninges of the posterior fossa. Persistent Hyrtl's fissure, due to incomplete ossification, is considered a rare temporal bone malformation, and is a known cause of perilabyrinthine cerebrospinal fluid fistula. Very few cases are reported as being at risk of complication of cochlear implant surgery. Here we report the case of an 8-year-old boy with misplacement of an electrode array in Hyrtl's fissure. The diagnosis was made postoperatively, since cochlear implant failure was suspected from non-auditory responses. Computed tomography (CT) revealed the extracochlear location of the electrode array. We emphasize the role of presurgical imaging CT and magnetic resonance imaging in detecting temporal bone abnormalities, and we discuss the value of intraoperative auditory nerve response telemetry and postoperative radiological evaluation in diagnosing cochlear implant misplacement.