Recurrent laryngeal nerve monitoring in children using cricothyroid membrane needle electrodes.

AIMS: Thyroid pathology is rare in children and the rate of malignancy is higher than in adults. Thyroid surgery in children is therefore particularly at risk of causing recurrent laryngeal nerve (RLN) palsies. The classical technique for monitoring the RLN is not always adapted to children due to the large size of the dedicated endotracheal tubes. MATERIAL AND METHODS: Double-needle electrodes (NIM 3.0) were placed medially or paramedially through the cricothyroid membrane and carefully kept submucosal just below the level of the vocal folds. Before identification of the RLN, the vagal nerve was dissected on the side of the concerned lobe and stimulated. The thyroid surgery was then performed with the routine identification of the RLN. The response of the RLN is periodically checked using a stimulating probe. The main outcomes were the identification and stimulation of the RLN, quality of the voice in post-operative time. OBJECTIVE: The main objective of our study is to present a simple and efficient method, available for children of all ages, in order to perform monitoring of the recurrent laryngeal nerves during thyroid surgery. RESULTS: We present the results of our retrospective series, in a tertiary-care university pediatric hospital. We included nine children, corresponding to 15 nerves. In all cases, the RLN was identified, stimulated and a positive response was obtained via monitoring. CONCLUSIONS: This technique of monitoring is safe, feasible at any age, even in neonates, and, as the electrode stays in the operation field, its position is easily controlled.

Recurrent laryngeal nerve monitoring during thyroidectomy and parathyroidectomy in children.

OBJECTIVE: Injury of the recurrent laryngeal nerve (RLN) is the most frequent complication of thyroid and parathyroid (TP) surgery. Monitoring of the RLN in children is not widely studied as this is not a common disease in the pediatric population. The aim of our study was to evaluate the reliability, feasibility and benefits of RLN monitoring during TP surgery in children. METHODS: We analyzed all children who underwent TP surgery in our department between January 2009 and March 2018. Patients were classified into three groups: without monitoring (group 1), monitoring with an endotracheal tube (group 2) and monitoring with a double-needle electrode placed through the cricothyroid ligament (group 3). RESULTS: We performed 53 surgeries (77 RLNs at risk) on 47 patients aged between 6 months and 18 years. Fourteen RLNs were in group 1, 47 in group 2 and 16 in group 3. We found one transient lesion of the RLN in group 1, three in group 2 and two in group 3. Seven patients in group 3 could not undergo monitoring with the tube because they need an orotracheal tube smaller than the smallest monitoring tube size available. The sensitivity and specificity values of the monitoring methods were 33% and 97% in group 2 and 67% and 100% in group 3, respectively. The positive and negative predictive values were 50% and 95% in group 2 and 100% and 92% in group 3, respectively. No complications arose during insertion of the electrode through the cricothyroid ligament. CONCLUSION: Monitoring of the RLN with a double-needle electrode inserted through the cricothyroid ligament is a reliable method with no associated complication, which is achievable at any age.

Otologic disorders in Turner syndrome.

INTRODUCTION: Patients with Turner syndrome (TS) have craniofacial malformations, such as Eustachian tube hypoplasia and dysfunction and velar dysfunction, which foster acute otitis media. The aim of this study was to inventory pediatric otologic disorders in patients with TS at their first ENT consultation in our center. PATIENTS AND METHODS: We reviewed the ENT consultation data of pediatric TS patients followed in our center between 2005 and 2015: otoscopy, hearing threshold, and history of acute otitis media or ENT surgery. Data were compared according to karyotype: X monosomy (45,X), mosaic (45,X/46,XX), isochromosome (46,Xi [Xq]), X ring chromosome X (XrX), with Y material, and "other". RESULTS: Ninety patients, with mean age 11.9years (±4.8years) at first ENT consultation, were included: 29% showed tympanic abnormality on otoscopy, 21% had hearing loss, 24% had history of recurrent acute otitis media; 18% had undergone adenoidectomy, 24% T-tube insertion, and 5.6% tympanoplasty. No particular karyotype was associated with higher risk of hearing loss or acute otitis media. CONCLUSION: Patients with TS showed high prevalence of pediatric otologic disorders; they therefore require close and prolonged ENT follow-up.

CNTN6 mutations are risk factors for abnormal auditory sensory perception in autism spectrum disorders.

Contactin genes CNTN5 and CNTN6 code for neuronal cell adhesion molecules that promote neurite outgrowth in sensory-motor neuronal pathways. Mutations of CNTN5 and CNTN6 have previously been reported in individuals with autism spectrum disorders (ASDs), but very little is known on their prevalence and clinical impact. In this study, we identified CNTN5 and CNTN6 deleterious variants in individuals with ASD. Among the carriers, a girl with ASD and attention-deficit/hyperactivity disorder was carrying five copies of CNTN5. For CNTN6, both deletions (6/1534 ASD vs 1/8936 controls; P=0.00006) and private coding sequence variants (18/501 ASD vs 535/33480 controls; P=0.0005) were enriched in individuals with ASD. Among the rare CNTN6 variants, two deletions were transmitted by fathers diagnosed with ASD, one stop mutation CNTN6W923X was transmitted by a mother to her two sons with ASD and one variant CNTN6P770L was found de novo in a boy with ASD. Clinical investigations of the patients carrying CNTN5 or CNTN6 variants showed that they were hypersensitive to sounds (a condition called hyperacusis) and displayed changes in wave latency within the auditory pathway. These results reinforce the hypothesis of abnormal neuronal connectivity in the pathophysiology of ASD and shed new light on the genes that increase risk for abnormal sensory perception in ASD.

Audiovestibular consequences of congenital cytomegalovirus infection.

Congenital cytomegalovirus (CMV) infection is the second most frequent cause of mental retardation and sensorineural hearing loss, after genetic factors. Recently, pediatric forensic and fetopathological studies have led to progress in understanding the pathophysiological mechanisms underlying the various neurosensory sequelae. Thanks to the identification of certain prognostic factors of hearing loss, therapeutic protocols based on antiviral molecules are now proposed for target populations. This treatment has shown efficacy in limiting hearing threshold deterioration and even, in some cases, seems to provide partial recovery of hearing in symptomatic congenitally infected CMV neonates. However, optimal treatment duration and administration modalities are not clearly defined. This article reviews recent data concerning audiovestibular sequelae and their management in children congenitally infected by CMV.

Tracheotomy in children: A series of 57 consecutive cases.

OBJECTIVES: With medical improvements in pediatrics, the role of tracheotomy has evolved. The aim of this study was to specify the indications for and complications of tracheotomy performed on children in a teaching hospital containing a level-3 maternity department and pediatric intensive care unit. MATERIAL AND METHODS: A retrospective study was conducted in pediatric tracheotomies performed from 2004 to 2014. Indications, early and late complications and the number and timing of decannulations were collated. RESULTS: Fifty-seven patients were included. Tracheotomy was motivated by upper airway obstruction in 39 children (68%) (median age, 4.9 months) or the need for prolonged ventilation in 18 children (32%) (median age, 6 months). There were 4 early complications (7%) (2 decannulations, including 1 fatal; an obstructive plug, responsible for another death; and 1 pneumothorax during an EXIT procedure), and 15 secondary complications requiring further surgery (26%). Twenty-seven patients (47%) were decannulated, with a mean tracheotomy duration of 26 months. In 9 cases (33% of decannulations), persistence of tracheocutaneous fistula required surgical repair. CONCLUSION: Tracheotomy for infection is almost a thing of the past; tracheotomy for airway obstruction is also likely to decrease, thanks to medical treatment (for hemangioma) and surgical techniques (for congenital stenosis). Tracheotomy for prolonged ventilation, on the other hand, remains. Complications of tracheotomy in children are rare but potentially serious, requiring care in a specialized center within a multidisciplinary team with defined care protocols.

[Antibiotherapy of severe ENT infections in children: peripharyngeal abscesses]. / Antibiothérapie des infections ORL sévères du nourrisson et de l'enfant : infections péripharyngées.

Neck infections in children are categorized as peritonsillar infections, latero and retroparapharyngeal infections. The clinical features and severity of these infections vary according to different pædiatric age groups, in relation to the location of the infection. In France, the antimicrobial therapy should consider meticillin sensitive S. aureus, Streptococcus pyogenes and anerobic bacteria. Empiric initial antimicrobial therapy consists in high doses of parenteral amoxicillin-clavulanate (150 mg/kg d. in 3-4 doses) during a few days then changed to oral amoxicillin-clavulanate (80 mg/kg/d). The total course of therapy should be 10 to 14 days.

Fetal airway management on placental support: limitations and ethical considerations in seven cases.

The aim of this study was to evaluate the paediatric and maternal outcome after ex utero intrapartum treatment (EXIT). A retrospective review was carried out of the medical charts (gestational age, circumstances of diagnosis, multidisciplinary prenatal decision, date of surgery, paediatric and maternal outcome) of all the fetuses eligible for/delivered via the EXIT procedure in our paediatrics and obstetrics tertiary care and teaching centre, between October 2004 and May 2011. Seven fetuses with cervical teratoma, epignathus tumour or congenital high airway obstruction syndrome (CHAOS) were included in our study. Two pregnancies were terminated and five fetuses were delivered alive. The airway was secured in all five cases (two endotracheal intubations and three tracheostomies). No maternal complications were observed. On average, babies were delivered at 32 gestational weeks, and spent 31 days in the intensive care unit. All but one baby were ventilated for 18 days. Long-term paediatric outcome was favourable. It is concluded that airway management by the EXIT procedure has become an efficient technique. A multidisciplinary prenatal assessment is essential in order to select appropriate cases.

[Audiophonological evaluation of 16 children fitted with cochlear implants for sensorineural hearing loss induced by bacterial meningitis]. / Implants cochléaires dans les surdités après méningite bactérienne: suivi audiologique de 16 enfants.

BACKGROUND: Bacterial meningitis (BM) is the primary etiology of acquired sensorineural hearing loss (SNHL) in children and may compromise language development. Since the 1990 s, cochlear implants (CIs) have become part of the management of children with profound SNHL with encouraging results. The aim of this study was to analyze the audiophonological performance of children before and after cochlear implantation for SNHL following bacterial meningitis. METHODS: Retrospective study of all children fitted with CIs for bilateral severe to profound SNHL after bacterial meningitis in the Robert-Debré pediatric ENT department between August 1990 and March 2009. Audiophonological performance was assessed using the APCEI profile. RESULTS: Of the 283 children receiving implants during that period, 16 children (6%; 6 boys, 10 girls) underwent CI implantation after bacterial meningitis (Streptococcus pneumoniae in 8 cases, Neisseria meningitidis in 2 cases, and Haemophilus influenzae in 4 cases). The mean time from meningitis to SNHL was 8.3 months (median, 1.5 months; range, 1 day to 13 years). The mean time from meningitis to cochlear implantation was 2 years and 3 months (median, 7 months; range, 1 month to 13 years 3 months). Twelve children (75%) presented partial cochlear and/or vestibular ossification on presurgical CT scan. Three children received bilateral implants. DISCUSSION: Thirteen children (81%) developed early SNHL in the first 3 months, whereas 3 children developed SNHL more than 10 months after meningitis. As for the benefits of cochlear implantation, 11 children presented near to normal intelligibility and optimal use of their cochlear implant; 5 children presented partial benefits due to neurological sequelae (1), a long delay before implantation (1), technical problems (2), or a social problem in relation to low socioeconomic status (1). CONCLUSION: After bacterial meningitis, audiological evaluation must be made carefully during the first 3 months to detect early SNHL, but SNHL may also develop several years later. In case of profound SNHL and a modified signal of the labyrinth on the MRI, cochlear implantation must be performed without delay before cochlear and/or vestibular ossification. Cochlear implantation is an effective technique with good long-term audiologic results. The coexistence of neurological lesions may compromise the results, but it should not contraindicate a cochlear implantation.

Management of laryngomalacia.

Laryngomalacia is the most common laryngeal disease of infancy. It is poorly tolerated in 10% of cases, requiring assessment and management, generally surgical. Surgery often consists of supraglottoplasty, for which a large number of technical variants have been described. This surgery, performed in an appropriate setting, relieves the symptoms in the great majority of cases with low morbidity. However, few data are available concerning the objective results: preoperative and postoperative objective assessment of these infants is therefore necessary whenever possible. Noninvasive ventilation (NIV) may be indicated in some infants with comorbid conditions or failing to respond to surgical management.

Pathophysiology and diagnostic approach to laryngomalacia in infants.

Laryngomalacia is defined as collapse of supraglottic structures during inspiration. It is the most common laryngeal disease of infancy. Laryngomalacia presents in the form of stridor, a high-pitched, musical, vibrating, multiphase inspiratory noise appearing within the first 10 days of life. Signs of severity are present in 10% of cases: poor weight gain (probably the most contributive element), dyspnoea with permanent and severe intercostal or xyphoid retraction, episodes of respiratory distress, obstructive sleep apnoea, and/or episodes of suffocation while feeding or feeding difficulties. The diagnosis is based on systematic office flexible laryngoscopy to confirm laryngomalacia and exclude other causes of supraglottic obstruction. Rigid endoscopy under general anaesthesia is only performed in the following cases: absence of laryngomalacia on flexible laryngoscopy, presence of laryngomalacia with signs of severity, search for any associated lesions prior to surgery, discrepancy between the severity of symptoms and the appearance on flexible laryngoscopy, and/or atypical symptoms (mostly aspirations). The work-up must be adapted to each child; however, guidelines recommend objective respiratory investigations in infants presenting signs of severity.

Endonasal endoscopic approach for removal of intranasal nasal glial heterotopias.

BACKGROUND: Nasal Glial Heterotopias also called Nasal Gliomas (NG) are rare congenital tumours of the midline frontonasal space arising from a normal neurectodermal tissue entrapped during the closure of the anterior neuropore. Historically, such tumours were approached using a frontal craniotomy. The study aims to evaluate a fully endonasal endoscopic approach for intranasal NG removal. METHODS: We report a retrospective study of intranasal and mixed NG treated using endonasal endoscopic techniques and computer assisted navigation system from 1997 to 2010 in two tertiary referral centres of Paediatric Otolaryngology. All tumours were investigated using two imaging modalities: craniofacial MRI and CT-scan. RESULTS: Fifteen patients were included (0 to 14 years of age). All tumours were totally removed and no recurrence was observed after a mean follow-up of 32 months. A skull base plasty was done in 13 cases to cover a bony defect or to treat a cerebrospinal leak. Nasal packing was usually removed 24 hours after surgery and all children were discharged home after 2 to 4 days. CONCLUSION: Removal of intranasal NGs using an endonasal endoscopic approach and a dedicated computer assisted navigation system is a safe and efficient procedure. Early management is recommended to treat neonatal airway obstruction.

Laryngotracheal separation in neonatal brainstem dysfunction.

INTRODUCTION: Neonatal brainstem dysfunction (NBD) associates four symptoms of variable presence and intensity: suction-swallowing dysfunction, abnormal laryngeal sensitivity and motility, gastroesophageal reflux, and cardiac vagal overactivity. MATERIALS AND METHODS: We report three cases of severe NBD with chronic aspiration which required surgical management. Successive failures and clinical deterioration led us to perform laryngotracheal separation. The surgical procedure consisted in suturing the distal segment of the trachea to the cervical skin after complete closure of the larynx. RESULTS: After surgery, these children did not present any pulmonary infection and were allowed oral nutrition. However, oral communication was no longer possible. Although it is a theoretically reversible procedure, the decision is ethically difficult in children free of mental deficiency, because of the vocal loss and the unpredictable NBD outcome. CONCLUSION: Laryngotracheal separation may be recommended after multidisciplinary decision for severe chronic aspiration in the particular case of children presenting with NBD.

Long term results of ossiculoplasties with partial and total titanium Vario Kurz prostheses in children.

OBJECTIVE: To assess and compare the efficacy of the Kurz titanium prostheses for total or partial ossicular reconstruction in children. PATIENTS AND METHODS: Retrospective chart review was performed for 71 consecutive children who underwent 74 ossicular reconstructions with Kurz prostheses between December 1999 and October 2007 (27 PORP and 47 TORP). Audiological parameters using four-frequency averages (0.5, 1, 2 and 4 kHz) were assessed pre- and postoperatively. Clinical and audiometric follow-up times were respectively 33±18.6 and 30±17.7 months (mean±SD). RESULTS: Fifty-two percent of all patients achieved an air bone gap (ABG) ≤20 dB, 53.8% in the PORP group and 51.1% in the TORP group. The improvement of the mean ABG was 13.3 dB, 9.4 dB for PORP and 14.6 dB for TORP. Overall mean pure tone averages improved 13.2±1.8 dB (mean±SEM) with 10±2.7 dB for PORP and 15±2.4 dB for TORP. The sensory neural hearing loss rate was 1.4% (one TORP) and the extrusion rate was 2.7% (n=2/74). CONCLUSION: The success rate (ABG≤20 dB) and the pure tone averages were not different between PORP and TORP. Kurz titanium ossicular prostheses offer high biocompatibility and high stability with low complication rates in pediatric ossicular reconstruction.

[Rectal pentobarbital sedation for children undergoing auditory brainstem response testing]. / Sédation au pentobarbital par voie rectale pour enregistrement des PEA chez l'enfant.

OBJECTIVES: The aim of our study was to determine if rectal sedation with pentobarbital sodium provides safe and effective sedation for children undergoing auditory brainstem response (ABR) testing. MATERIAL AND METHODS: A prospective study was conducted in the ENT pediatric department of Robert Debre's hospital (APHP, Paris). 68 children under 8 years of age were given rectal pentobarbital for ABR testing at a dosage of about 5 mg/kg. RESULTS: 61 children of 68 (89.7%) were adequately sedated with rectal pentobarbital. The mean elapsed time from drug administration to full sedation was 36,1 minutes. No adverse event was reported in 84.1% of children. CONCLUSION: Pentobarbital provides safe and effective sedation. Rectal administration is easy, painless and with brief duration of action. It's a good alternative to general anesthesia for young children undergoing ABR testing.

[The deaf child: anatomy, etiologies and management]. / Le sourd est un enfant: qu'est-ce que ça change. Anatomie, pathologie, prise en charge.

Temporal bone imaging in children shows radioanatomical aspects and diseases distinct from the imaging and pathology results found in adults. Imaging modalities such as CT and MR bring out these differences. The aim of this study is to present the CT and MR particularities of the temporal bone during postnatal growth. The mastoid air cells form mostly in the postnatal period and the course of pneumatization is directly correlated with middle ear successive inflammatory episodes. The most frequent etiologies of hearing loss in children are reviewed, emphasizing their specificities in clinical presentation, radiological aspects, and treatment. In children, conductive hearing loss with normal tympanic membrane is mostly caused by minor aplasia rather than otosclerosis. Sensorineural hearing loss, even when unilateral, is predominantly due to malformation or infection and in rare cases to posterior fossa tumor.