New surgical frontiers for 4K 3D-exoscope in paediatric head and neck surgery.

PURPOSE: To define the interest, advantages, and disadvantages of the use of a 3D-exoscope in paediatric ENT surgery. METHODS: Four surgeons with experience in paediatric surgery completed a questionnaire following each surgery performed under 3D-exoscope to evaluate the contribution of the tool compared to the usual practice (microscope or magnifying loupes). Surgeries were separated into three groups: otology, transoral and cleft palate surgery, and open head and neck surgery. RESULTS: Between June 2021 and June 2022, 151 paediatric surgeries were included in this study. Among them, 93 (62%) otologic surgeries, 35 (23%) transoral surgeries, and 23 (15%) head and neck surgeries were performed. The median age at surgery was 68 months (interquartile range 19-135 months). For otologic surgeries, the mean scores (/100) for the contribution of the exoscope compared to the microscope were 68.4(± 23.2). For transoral and cleft palate surgery, the mean score (/100) for the contribution of the use of the exoscope compared to the magnifying loupes was 92.9 (± 8.6), whereas for open head and neck surgeries, the mean score (/100) was 89.5 (± 7.2). CONCLUSION: 3D-exoscopy appears to be a relevant tool for paediatric head and neck surgery, applicable in otologic, transoral, and cervical fields. It presents educational and ergonomic advantages and improves surgical team communication.

Hearing loss in children with sickle cell disease: A prospective French cohort study.

BACKGROUND: Sickle cell disease (SCD) is the most common genetic disease in France. In developing countries, it is associated with a high incidence of hearing loss. The aim of this study was to determine the prevalence of hearing loss in French children with SCD in order to determine if they need a close audiological follow-up. METHODS: We performed a single-center prospective cross-sectional study of children with SCD. The children, without specific hearing symptom, underwent an ear, nose and throat examination with a hearing assessment between 2015 and 2016. RESULTS: Eighty-nine children were included, aged from 5 to 19 years, with 73% of SS or Sß0 genotype and 27% of SC or Sß+ genotype. Ten children (11.2%) had hearing thresholds higher than 20 dB in at least one ear: one child with subnormal hearing, six otitis media with effusion (OME), and three sensorineural hearing loss. Late age at diagnosis of SCD, a high platelet count and a low hematocrit level were significantly associated with OME; moreover, children with OME had more severe clinical and biological characteristics than children with normal hearing. Furthermore, 12.4% of the children complained of tinnitus. The rate of sudden hearing loss was 2.2%. Finally, 7.1% of patients with normal hearing showed a speech discrimination disorder. CONCLUSIONS: Several causes were identified for hearing loss in children with SCD. They therefore need a close audiological follow-up in order to avoid complications due to curable phenomena and to enable appropriate management for progressive complications.

Turner syndrome: French National Diagnosis and Care Protocol (NDCP; National Diagnosis and Care Protocol).

Turner syndrome (TS; ORPHA 881) is a rare condition in which all or part of one X chromosome is absent from some or all cells. It affects approximately one in every 1/2500 liveborn girls. The most frequently observed karyotypes are 45,X (40-50%) and the 45,X/46,XX mosaic karyotype (15-25%). Karyotypes with an X isochromosome (45,X/46,isoXq or 45,X/46,isoXp), a Y chromosome, X ring chromosome or deletions of the X chromosome are less frequent. The objective of the French National Diagnosis and Care Protocol (PNDS; Protocole National de Diagnostic et de Soins) is to provide health professionals with information about the optimal management and care for patients, based on a critical literature review and multidisciplinary expert consensus. The PNDS, written by members of the French National Reference Center for Rare Growth and Developmental Endocrine disorders, is available from the French Health Authority website. Turner Syndrome is associated with several phenotypic conditions and a higher risk of comorbidity. The most frequently reported features are growth retardation with short adult stature and gonadal dysgenesis. TS may be associated with various congenital (heart and kidney) or acquired diseases (autoimmune thyroid disease, celiac disease, hearing loss, overweight/obesity, glucose intolerance/type 2 diabetes, dyslipidemia, cardiovascular complications and liver dysfunction). Most of the clinical traits of TS are due to the haploinsufficiency of various genes on the X chromosome, particularly those in the pseudoautosomal regions (PAR 1 and PAR 2), which normally escape the physiological process of X inactivation, although other regions may also be implicated. The management of patients with TS requires collaboration between several healthcare providers. The attending physician, in collaboration with the national care network, will ensure that the patient receives optimal care through regular follow-up and screening. The various elements of this PNDS are designed to provide such support.

Surgical Treatment for Cerebrospinal Fluid Leaks in Patients With Inner Ear Malformations.

OBJECTIVE: The aim of this study is to present our technique of surgical closure in a series of patients suffering from cerebrospinal fluid (CSF) leak due to inner ear malformations. STUDY DESIGN AND SETTING: We conducted a retrospective study in our tertiary care academic department of pediatric otolaryngology. PATIENTS: We did include all patients who presented a CSF leak or bacterial meningitis (one episode or recurrent) related to a malformation of the inner ear. INTERVENTION(S): Through a retro-auricular or endaural approach we performed a filling of the vestibule cavity with multiple fragments of cartilage with perichondrium introduced through the oval window, after stapedectomy until a near-complete sealing was obtained. After the surgery, all patients received a treatment with acetazolamide during 15 days. MAIN OUTCOME MEASURE(S): We did evaluate our technics with the recurrence of CSF leak. RESULTS: Thirteen patients, from 1 to 14, were operated with our technics. With a follow-up of 4.4 ±â€Š4.7 years, only one patient needed a second intervention. None had a novel episode of meningitis. We observed no complication. The leak was observed in the oval fossa in 11 cases. CONCLUSIONS: Our "minimally invasive" technique of vestibular obliteration with cartilage inserted through the oval window after stapedectomy did demonstrate its safety and reliability.

Evaluation of Tonsillotomy Effects on Pharyngeal Volume and Compliance in Children.

Our objective was to assess whether adenotonsillotomy improved pharyngeal compliance, which is a risk factor for sleep-disordered breathing. Otherwise healthy children underwent Obstructive Sleep Apnea (OSA)-18 questionnaire and a pre- and postoperative acoustic pharyngometry in both sitting and supine positions, allowing the measurement of the volume of the palatine tonsil region and pharyngeal compliance. Thirty-five children (median age 5.3 years) were enrolled; they were reevaluated at a median of 18 days (25th-75th percentiles, 15-25) after surgery. Participants were compared according to a normal (n = 18) or an increased (n = 17) preoperative pharyngeal compliance. Surgery was associated with a significant decrease in OSA-18 and Brodsky scores, with a median increase in palatine volume of 0.13 cm3 (25th-75th percentiles, 0.00-0.73). A decrease in pharyngeal compliance was observed in children with increased preoperative compliance. The variation of palatine volume after surgery was positively related to the variation of pharyngeal compliance, suggesting that obstruction relief was associated with muscle relaxation in children with normal preoperative compliance.

In Utero Administration of Drugs Targeting Microglia Improves the Neurodevelopmental Outcome Following Cytomegalovirus Infection of the Rat Fetal Brain.

Congenital cytomegalovirus (CMV) infections represent one leading cause of neurodevelopmental disorders. Recently, we reported on a rat model of CMV infection of the developing brain in utero, characterized by early and prominent infection and alteration of microglia-the brain-resident mononuclear phagocytes. Besides their canonical function against pathogens, microglia are also pivotal to brain development. Here we show that CMV infection of the rat fetal brain recapitulated key postnatal phenotypes of human congenital CMV including increased mortality, sensorimotor impairment reminiscent of cerebral palsy, hearing defects, and epileptic seizures. The possible influence of early microglia alteration on those phenotypes was then questioned by pharmacological targeting of microglia during pregnancy. One single administration of clodronate liposomes in the embryonic brains at the time of CMV injection to deplete microglia, and maternal feeding with doxycyxline throughout pregnancy to modify microglia in the litters' brains, were both associated with dramatic improvements of survival, body weight gain, sensorimotor development and with decreased risk of epileptic seizures. Improvement of microglia activation status did not persist postnatally after doxycycline discontinuation; also, active brain infection remained unchanged by doxycycline. Altogether our data indicate that early microglia alteration, rather than brain CMV load per se, is instrumental in influencing survival and the neurological outcomes of CMV-infected rats, and suggest that microglia might participate in the neurological outcome of congenital CMV in humans. Furthermore this study represents a first proof-of-principle for the design of microglia-targeted preventive strategies in the context of congenital CMV infection of the brain.

Velopharyngeal insufficiency managed by autologous fat grafting in patients with aberrant courses of internal carotid arteries.

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