[Secondary surgeries in craniosynostosis and faciocraniosynostosis]. / Chirurgie secondaire des craniosténoses et faciocraniosténoses.

Secondary surgeries for single craniosynostosis surgeries are mainly esthetic refinements rather than functional indications. However, cranioplasties for bone defects correction or insufficient corrections may be undertaken. Management of syndromic craniosynostoses usually requires multiple surgical interventions, the sequence of which might vary per the genetic mutation. It is commonplace to start with posterior vault expansion before age 6 months, then treat cerebellar tonsillar herniation by the age of twelve months, and delay fronto-facial monobloc advancement until at least 18-24 months of age. Ventricular shunting is preferably avoided or delayed. Failure to respect these guidelines can significantly complicate the subsequent management. Primary fronto-orbital advancement or early facial osteotomy type Le Fort3, may compromise the subsequent fronto-facial monobloc advancement. However, this salvage secondary monobloc may be undertaken in some instances despite previous anterior osteotomies with a higher morbidity.

Beard infantile hemangioma and subglottic involvement: are median pattern and telangiectatic aspect the clue?

BACKGROUND: Identification of patient at risk of subglottic infantile hemangioma (IH) is challenging because subglottic IH can grow fast and cause airway obstruction with a fatal course. OBJECTIVE: To refine the cutaneous IH pattern at risk of subglottic IH. METHODS: Prospective and retrospective review of patients with cutaneous IH involving the beard area. IHs were classified in the bilateral pattern group (BH) or in the unilateral pattern group (UH). Infantile hemangioma topography, subtype (telangiectatic or tuberous), ear, nose and throat (ENT) manifestations and subglottic involvement were recorded. RESULTS: Thirty-one patients (21 BH and 10 UH) were included during a 20-year span. Nineteen patients (16 BH and 3 UH) had subglottic hemangioma. BH and UH group overlap on the median pattern (tongue, gum, lips, chin and neck). Median pattern, particularly the neck area and telangiectatic subtype of IH were significantly associated with subglottic involvement. CONCLUSION: Patients presenting with telangiectatic beard IH localized on the median area need early ENT exploration. They should be treated before respiratory symptoms occur.

[Craniofacial strategy for syndromic craniosynostosis]. / Stratégie craniofaciale pour les faciocraniosténoses.

The complexity of treatment of faciocraniosynostosis justifies the treatment in a reference center for rare diseases. The growth disturbances in the skull and face being variable according to the type of mutation in the FGFr (Crouzon, Pfeiffer, Apert), the strategy is adapted to the phenotype according to the following principles: posterior expansion with or without distraction around 6 months to limit the descent of the cerebellum tonsils and to prevent the turricephalic development; fronto-facial monobloc advancement with internal distraction around the age of 18 months in case of severe exorbitism or breathing impairment. The dissociated strategy (fronto-orbital advancement first, followed by facial osteotomy of Le Fort 3 type). The growing evolution dictates the sequence of subsequent surgeries according to the monitoring of intracranial pressure by fundus examination and of the respiration by polysomnography. Le Fort 3 and transversal maxillary distraction may be repeated if necessary. Orthognathic surgery is almost always compulsory after the age of 14, before the aesthetic refinements which can be undertaken ultimately (rhinoplasty, genioplasty, canthopexies, fat grafting…).

SOX10 mutations mimic isolated hearing loss.

Ninety genes have been identified to date that are involved in non-syndromic hearing loss, and more than 300 different forms of syndromic hearing impairment have been described. Mutations in SOX10, one of the genes contributing to syndromic hearing loss, induce a large range of phenotypes, including several subtypes of Waardenburg syndrome and Kallmann syndrome with deafness. In addition, rare mutations have been identified in patients with isolated signs of these diseases. We used the recent characterization of temporal bone imaging aspects in patients with SOX10 mutations to identify possible patients with isolated hearing loss due to SOX10 mutation. We selected 21 patients with isolated deafness and temporal bone morphological defects for mutational screening. We identified two SOX10 mutations and found that both resulted in a non-functional protein in vitro. Re-evaluation of the two affected patients showed that both had previously undiagnosed olfactory defects. Diagnosis of anosmia or hyposmia in young children is challenging, and particularly in the absence of magnetic resonance imaging (MRI), SOX10 mutations can mimic non-syndromic hearing impairment. MRI should complete temporal bones computed tomographic scan in the management of congenital deafness as it can detect brain anomalies, cochlear nerve defects, and olfactory bulb malformation in addition to inner ear malformations.

Antimicrobial treatment of ENT infections.

Ear, nose and throat (ENT) or upper respiratory tract infections (URTI) are the most common infections in children and the leading causes of antibiotic prescriptions. In most cases, these infections are due to (or are triggered by) viruses and even when bacterial species are implicated, recovery is usually spontaneous. The first imperative is to refrain from prescribing antibiotics in a large number of URTIs: common cold, most cases of sore throat, laryngitis, congestive otitis, and otitis media with effusion. On the contrary, a decision to treat sore throats with antibiotics is based primarily on the positivity of the Group A Streptococcus (GAS) rapid antigen diagnostic tests. For ear infections, only (a) purulent acute otitis media in children under 2 years of age and (b) complicated or symptomatic forms of purulent acute otitis media (PAOM) in older children should be treated with antibiotics. Amoxicillin is the first-line treatment in the most cases of ambulatory ENT justifying antibiotics. Severe ENT infections (mastoiditis, epiglottitis, retro- and parapharyngeal abscesses, ethmoiditis) are therapeutic emergencies necessitating hospitalization and initial intravenous antibiotic therapy.

Nasal polyposis and cystic fibrosis(CF): review of the literature.

The aim of this study was to address whether NP might be a predictive factor for severity of CF. The authors collected data from the literature on NP as a unique or associated sign in CF and reviewed the clinical and molecular aspects of CF associated with NP. CF genotypes and clinical severity in NP(+) vs. NP(-) patients were reviewed, taking into account pulmonary function, frequency of P. aeruginosa lung infection, frequency of allergy, nutritional status, and exocrine pancreatic function. The CFTR gene was also analyzed in a patient with isolated severe NP as the unique feature of CF. This review of the literature showed a `milder` phenotype in `NP+` vs. `NP-` CF patients, contrasting with a marked association between NP and `severe` CF mutations. In addition, a complex genotype was identified, associating four heterozygous variants, namely p.Q493X (a severe mutation) on the paternal allele, and p.V562I, p.A1006E, and (TG)11(T)5 (IVS8-5T) on the maternal allele, in a case of CF presenting as isolated NP. The authors speculate that genetic/environmental factors associated with NP might attenuate the functional impact of `severe` CF mutations. The overrepresentation of CF carriers among patients with isolated NP also advocates the need for CFTR molecular screening in such populations for genetic counselling purposes.

Lateral endoscopic parathyroidectomy in children.

Endoscopic thyroid and parathyroid surgery was first described by Gagner in 1996, and Henry subsequently proposed a lateral endoscopic approach in 1999. Technical progress in the fields of optics, endoscopy, digital imaging and laparoscopy has gradually enhanced the feasibility and clinical utility of this technique for the treatment of benign and malignant lesions. To date, published paediatric cases have only concerned thyroid surgery. In the light of two clinical cases, this article describes our lateral endoscopic approach applied to paediatric parathyroid surgery.

Nasal cavity shape in unilateral choanal atresia and the role of fetal ventilation in facial growth.

OBJECTIVE: The respiratory movements of fetal amniotic fluid areconsidered by certains cleft surgery teams to contribute to the growth of the nasal cavities (NC). To assess this functional hypothesis, we considered a group of patients with unilateral choanal atresia (CA) as a model of unilateral absence of amniotic fluid flux in the NC, and compared their NCs shape to age-matched controls. MATERIAL AND METHODS: Three-dimensional reconstructions of NC were performed using Avizo 9.7 (Thermo Fisher Scientific, MA, USA), based on CT-scans of 32 patients with unilateral CA and 96 age- and gender-matched controls. Landmarks were placed on anatomical structures of NC. Procrustes superimpositions and principal component analysis were performed. Anatomically relevant Euclidean distances were computed using the coordinates of selected landmarks - maxillary length, piriform orifice width, choanal width - and tested using multivariate analysis. Growth rates between patients and controls for these distances were screened for correlations. RESULTS: The atretic NC was significantly deformed when compared to the control cases: Procrustes distance was 0.28 (P<0.0001). The maxillary length and width of the atretic choana were significantly decreased compared to controls (-2.95mm and -1.35mm respectively, P<0.001). There were no differences in growth rates between CA and controls, except for the choanal width on the atretic side. CONCLUSION: NCs in CA were significantly different from controls. More precisely, the maxillary length was significantly reduced in the CA group. There was no other major shape difference between the NC in CA and controls. NC seems to develop despite abnormal fetal ventilation.

Myringoplasty without tympanomeatal flap elevation in children: A systematic review.

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 18years 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.

Outcome for bilateral cochlear implantation in patients with congenital Cytomegalovirus infection.

OBJECTIVES: To analyze the impact of bilateral cochlear implantation (CI) on perceptual and linguistic development in hearing-impaired children with congenital Cytomegalovirus (CMV) infection. PATIENTS AND METHOD: A retrospective study was performed for the period 1991-2016 in a pediatric CI reference center. Closed Set Word (CSW) recognition scores, Categories of Auditory Performance (CAP) and linguistic level on the MT Lenormand scale (MTL) were compared between bilateral (Bi) and unilateral (Uni) groups 12, 24 and 36 months after first CI (CI-1). RESULTS: 84 patients with congenital CMV infection who underwent CI were included, in 2 groups: sequential or simultaneous bilateral CI (Bi) (N=20), and unilateral CI (Uni) (N=64). Twelve, 24 and 36 months after CI-1, CSW scores were 35.56%, 64.52% and 82.93% in Uni and 60.3%, 85% (P=0.0084*), and 100% (P=0.00085*) in Bi. CAP scores 12, 24 and 36 months after CI-1 were 2.57, 3.85 and 4.3 in Uni and 3.91 (P=0.0068*), 5.00 (p=0.029*) and 5.50 (P=0.051*) in Bi. MTL linguistic level scores at 12, 24 and 36 months were 0.72, 1.25 and 1.65 in Uni, and 1.72, 3 (P=0.033) and 3.11 (P=0.045) in Bi. These significantly better scores in Bi at 24 and 36 months after CI-1 were also found on analysis of subgroups with no associated neurologic disorder (P=0.046* and P=0.032*), no associated psychiatric pathology (P=0.0055* and P=0.0073*), and no other associated disorder (P=0.0018* and P=0.035*), and for all subgroups together (P=0.0036 and P=0.037). CONCLUSION: Bilateral CI is a faster way than unilateral CI for patients with congenital CMV infection to achieve structured fluent oral language. 50% of the series showed cerebral abnormalities on MRI, without difference between groups. This was not in itself predictive of poor progression of oral communication, unless associated with major neurologic disorder. Some children made little or no use of their CI in the medium term.

Keys for analysis of diagnostic and serologic tests for CoV-2.

Based on a review of the medical literature, the authors document the key points regarding the tests available in France to screen for and diagnose of CoV-2 infestation.

Role of the ENT specialist in the diagnosis of pediatric obstructive sleep apnea-hypopnea syndrome (POSAHS). Part 3: sleep recordings.

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.

COVID-19 and ENT Surgery.

In Otorhinolaryngology - Head and Neck Surgery, clinical examination and invasive procedures on the respiratory tract and on airway-connected cavities, such as paranasal sinuses and the middle ear, expose people to direct transmission of SARS-CoV-2 by inhalation or ocular projection of contaminated droplets, and to indirect transmission by contact with contaminated hands, objects or surfaces. Estimating an R0 of COVID-19 at around 3 justified postponing non-urgent face-to-face consultations and expanding the use of teleconsultation in order to limit the risks of SARS-CoV-2 infection of patients or health workers and comply with the lockdown. The health authority recommends cancellation of all medical or surgical activities, which are not urgent as long as this does not involve a loss of chance for the patient. The purpose of this cancellation is to significantly increase critical care capacity, prioritise the reception of patients with COVID-19, prioritise the allocation of staff and provision of the equipment necessary for their medical or surgical management, and contribute to the smooth running of downstream critical care within their establishment. Another goal is to reduce the risks of patient contamination within healthcare facilities. This document provides guidance on how to proceed with and adapt ENT surgery in the current pandemic context, as well as on the management of postponed operations. This best practice advice must of course be adapted in each region according to the development of the epidemic and pre-existing arrangements. Their local application can only be decided within the framework of collaboration between the ENT teams, the operational hygiene units and all the other specialties concerned.

Best practice recommendations: ENT consultations during the COVID-19 pandemic.

These best practice recommendations for ENT consultations during the COVID-19 pandemic have been drawn up because ENT examinations and treatments are at risk of contamination by the SARS-Cov-2 virus in certain instances. Thus, ENT specialists are among the professionals who are most exposed to this infection. During the pandemic, insofar as an asymptomatic patient may be infected and contagious, the same precautions must be employed whether the patient is ill with, suspected of having, or without any clinical evidence of COVID-19 infection. According to the scientific data available, the examinations and procedures potentially exposing to projections/aerosolizations of organic material of human origin are considered to be at risk of staff contamination. For ENT examinations and procedures without exposure to such projections/aerosolizations, the professional is advised to a long sleeve clean outfit, a surgical mask and gloves in case of contact with the patient's mucosa. ENT examinations and procedures with exposure to these projections/aerosolizations require the so-called "airborne", "contact", and "droplets" additional precautions: FFP2/N95 respiratory protection device, eye protection, disposable headwear and long sleeve overgown.

Treatment of sleep apnea by ENT specialists during the COVID-19 pandemic.

The treatment of sleep disorders has been strongly impacted by the COVID-19 pandemic. When the lockdown is over, resumption of usual patient care will require precautions to limit the risk of contamination for patients and caregivers. In this document, the French Association of Otorhinolaryngology and Sleep disorders (AFSORL) and the French Society of Otorhinolaryngology (SFORL) put forward a summary of the measures for continuing the treatment of sleep apnoea syndrome in these new practice conditions. Emphasis is placed on teleconsultation, methods of nocturnal sleep studies, the conditions for treatment by continuous positive airway pressure (CPAP) ventilation, and the postponement of more invasive treatments.

Unusual presentation of a first Branchial cleft cyst associated with an abnormal bony canal -a case report.

BACKGROUND: First branchial cleft anomalies are rare, accounting for only 10% of all branchial cleft anomalies. We report an even more rare and unique case of a branchial cleft cyst with features of both first and second arch derivatives. CASE PRESENTATION: A 6-year-old boy presented to us with a left conductive hearing loss associated with pre-tympanic keratin debris and an ipsilateral painful cervical mass. He had a past medical history of left ear surgery for presumed cholesteatoma 2 years prior and left neck abscess drainage 6 months prior. CT and MRI revealed a lesion originating in the external auditory canal and extending cervically through a bony canal located medial to the facial nerve and terminating as a parapharyngeal cyst. The complete removal was accomplished in one surgical stage consisting of three distinct steps: robotic assisted transoral resection of the pharyngeal cyst, an endaural approach and a parotidectomy approach. CONCLUSION: We believe that our detailed description of this rare first branchial cleft cyst with pharyngeal extension, possibly a hybrid case between a first and second branchial cyst, can serve as a valuable tool to Otolaryngologists - Head and Neck Surgeons who come across a similar unusual presentations.

Consensus statement. Corticosteroid therapy in ENT in the context of the COVID-19 pandemic.

This consensus statement about the indications and modalities of corticosteroid treatment in the context of the COVID-19 pandemic was jointly written by experts from the French Association of Otology and Oto-Neurology (AFON) and from the French Society of Otorhinolaryngology, Head and Neck Surgery (SFORL). There is currently not enough data in favour of danger or benefit from corticosteroids in COVID-19, so until this matter is resolved it is advisable to limit their indications to the most serious clinical pictures for which it is well established that this type of treatment has a positive impact on the progression of symptoms. In Grade V and VI Bell's palsy according to the House-Brackmann grading system, a week's course of oral corticosteroids is recommended. Corticosteroid therapy is also recommended in cases of sudden hearing loss of more than 60dB, either in the form of intratympanic injections or a week's course of oral medication. In rhinology, there is no indication for systemic corticosteroid therapy in the current situation. However, patients are advised to continue with their local corticosteroid therapy in the form of a nasal spray or by inhalation. Treatments with corticosteroid nasal sprays can still be prescribed if there is no alternative. Finally, systemic or local corticosteroid therapy is not indicated for bacterial ENT infections.

French consensus regarding precautions during tracheostomy and post-tracheostomy care in the context of COVID-19 pandemic.

Tracheostomy post-tracheostomy care are regarded as at high risk for contamination of health care professionals with the new coronavirus (SARS-CoV-2). Considering the rapid spread of the infection, all patients in France must be considered as potentially infected by the virus. Nevertheless, patients without clinical or radiological (CT scan) markers of COVID-19, and with negative nasopharyngeal sample within 24h of surgery, are at low risk of being infected. Instructions for personal protection include specific wound dressings and decontamination of all material used. The operating room should be ventilated after each tracheostomy and the pressure of the room should be neutral or negative. Percutaneous tracheostomy is to be preferred over surgical cervicotomy in order to reduce aerosolization and to avoid moving patients from the intensive care unit to the operating room. Ventilation must be optimized during the procedure, to limit patient oxygen desaturation. Drug assisted neuromuscular blockage is advised to reduce coughing during tracheostomy tube insertion. An experienced team is mandatory to secure and accelerate the procedure as well as to reduce risk of contamination.

French consensus on management of head and neck cancer surgery during COVID-19 pandemic.

In the context of the current pandemic, there is a need for specific advice concerning treatment of patients with Head and Neck cancers. The rule is to limit as much as possible the number of patients in order to reduce the risks of contamination by the SARS-Cov-2 virus for both patients and the caregivers, who are particularly exposed in ENT. The aim is to minimize the risk of loss of opportunity for patients and to anticipate the increased number of cancer patients to be treated at the end of the pandemic, taking into account the degree of urgency, the difficulty of the surgery, the risk of contaminating the caregivers (tracheotomy) and the local situation (whether or not the hospital and intensive care departments are overstretched).

COVID-19 and rhinology, from the consultation room to the operating theatre.

The purpose of this article is to give rhinologists advice on how to adapt their standard practice during the COVID-19 pandemic. The main goal of these recommendations is to protect healthcare workers against COVID-19 while continuing to provide emergency care so as to prevent loss of chance for patients. We reviewed our recommendations concerning consultations, medical prescriptions and surgical activity in rhinology.