EAONO/JOS, STAMCO, ChOLE & Potsic Staging of 271 Pediatric Cholesteatoma: Evidence-Based Mod-Pot Staging System.

OBJECTIVE: The objective was to assess Potsic, EAONO/JOS, STAMCO, and ChOLE cholesteatoma staging systems in a large homogenous pediatric cohort with long-term follow-up and propose an evidence-based improved version. STUDY DESIGN: Cohort study. SETTING: Retrospective study in a tertiary referral center. METHODS: Children with congenital or acquired cholesteatoma who underwent surgery between 2008 and 2015 and had a minimum of five years follow-up were included. ROC areas under curve (AUCs) were performed for the residual disease proportion, total number of surgeries, hearing, and C-index for recurrence proportion at the last follow-up. RESULTS: Data from 271 ears with cholesteatoma were collected. Mean age at diagnosis was 7.9 years and 77 (28%) were congenital. Almost all patients (99%) had a canal wall up approach. The mean follow-up was 97.8 months. The Potsic classification, initially designed for congenital cholesteatoma, had the highest AUCs for residual disease rate and for number of surgeries outcomes (respectively 0.73 [0.67-0.78] and 0.71 [0.66-0.77]). For recurrence rate, all the classifications' C-index were low (<0.7). For postoperative hearing, STAMCO ossicular subscore had the highest AUC (0.73 [0.67-0.79]). The Mod-Pot staging system (modified Potsic), including stapes superstructure status and anterior epitympanum or supratubal involvement, improved the Potsic performance especially for postoperative hearing with AUC 0.73 [0.67-0.80], p < 0.001. CONCLUSION: The Potsic, ChOLE, EAONO/JOS, and STAMCO cholesteatoma classifications may be used in children, but in this specific population, their contribution remains limited to predict outcome. The Mod-Pot classification improved the performance of pediatric cholesteatoma classification while remaining simple and intuitive. LEVEL OF EVIDENCE: 3 Laryngoscope, 2024.

Early detection and treatment of obstructive sleep apnoea in infants with Down syndrome: a prospective, non-randomised, controlled, interventional study.

Background: Infants with Down syndrome (DS) are at high risk of obstructive sleep apnoea (OSA) which is associated with neurocognitive dysfunction and behaviour problems. The aim of our study was to evaluate the effect of early OSA treatment in infants with DS on neurocognitive development and behaviour. Methods: In this prospective, interventional, non-randomised study, 40 infants with DS underwent polysomnography (PSG) every 6 months in room air between 6 and 36 months of age (Screened Group) and were compared to a control group of 40 infants with DS receiving standard of care and a single, systematic PSG in room air at 36 months of age (Standard Care Group). When present, OSA was treated. The primary endpoint was the total score of the Griffiths Scales of Child Development, Third Edition (Griffiths III) and its subscores at 36 months. Secondary endpoints included a battery of neurocognitive and behaviour questionnaires, and PSG outcomes. Findings: On the Griffiths III, the total score was significantly higher in the Screened Group compared to the Standard Care Group (difference: 4.1; 95%CI: 1.3; 7.6; p = 0.009). Results in Griffiths III subscores and secondary endpoints were in support of better neurocognitive outcomes in the Screened Group compared with the Standard Care Group. At 36 months, median (Q1; Q3) apnoea-hypopnea index was higher in the Standard Care Group (4.0 [1.5; 9.0] events/hour) compared to the Screened Group (1.0 [1.0; 3.0] events/hour, p = 0.006). Moderate and severe OSA were more frequent in the Standard Care Group as compared to the Screened Group (18.9% versus 3.7% for moderate OSA and 27.0% versus 7.4% for severe OSA). Interpretation: Early diagnosis and treatment of OSA in infants with DS may contribute to a significantly better neurocognitive outcome and behaviour at the age of 36 months. Funding: The study was funded by the Jérôme Lejeune Foundation.

Lingual Tonsillectomy as Part of a DISE-Directed Multilevel Upper Airway Surgery to Treat Complex Pediatric OSA: A Safe and Appropriate Procedure.

OBJECTIVE: To study the efficiency of lingual tonsillectomy (LT) as part of multilevel surgery in children with complex obstructive sleep apnea (OSA). To evaluate the safety and the outcomes of LT. STUDY DESIGN: Retrospective case series. SETTING: Pediatric tertiary care academic center. METHODS: We included all children operated for LT to treat complex OSA, from January 2018 to June 2022. All patients underwent a protocolized drug-induced sleep endoscopy (DISE) followed by a coblation LT, associated with the treatment of all other obstructive sites. Patient demographics, medical history, surgery, and outcomes were reviewed. The efficiency of LT was analyzed exclusively in patients with a preoperative and postoperative sleep study. RESULTS: One hundred twenty-three patients were included. Median age was 8 years (interquartile range, IQR [3-12]). Sixty-five (53%) patients had Down syndrome, 22 (18%) had a craniofacial malformation, and 8 (7%) were obese. LT was associated with adenoidectomy (n = 78, 63%), partial tonsillectomy (n = 70, 57%), inferior turbinoplasty/turbinectomy (n = 59, 48%), epiglottoplasty (n = 92, 75%), and/or expansion pharyngoplasty (n = 2, 2%). Eighty-nine patients underwent a sleep study before and after surgery. The median apnea-hypopnea index (AHI) decreased from 18 events/h (IQR [9-36]) before surgery to 3 events/h (IQR [1-5]) after surgery (P < .001) (patients with a postoperative AHI <1.5 events/h, n = 31, 35%, and an AHI <5 events/h, n = 32, 36%). Seventeen out of 30 (57%) patients could be weaned from continuous positive airway pressure after surgery. Two patients had a postoperative hemorrhage and 2 patients required a transient postoperative reintubation. CONCLUSION: In children with complex OSA, LT as part of a DISE-directed multilevel upper airway surgery, was a very efficient and safe procedure.

Development and validation of a smartphone-based deep-learning-enabled system to detect middle-ear conditions in otoscopic images.

Middle-ear conditions are common causes of primary care visits, hearing impairment, and inappropriate antibiotic use. Deep learning (DL) may assist clinicians in interpreting otoscopic images. This study included patients over 5 years old from an ambulatory ENT practice in Strasbourg, France, between 2013 and 2020. Digital otoscopic images were obtained using a smartphone-attached otoscope (Smart Scope, Karl Storz, Germany) and labeled by a senior ENT specialist across 11 diagnostic classes (reference standard). An Inception-v2 DL model was trained using 41,664 otoscopic images, and its diagnostic accuracy was evaluated by calculating class-specific estimates of sensitivity and specificity. The model was then incorporated into a smartphone app called i-Nside. The DL model was evaluated on a validation set of 3,962 images and a held-out test set comprising 326 images. On the validation set, all class-specific estimates of sensitivity and specificity exceeded 98%. On the test set, the DL model achieved a sensitivity of 99.0% (95% confidence interval: 94.5-100) and a specificity of 95.2% (91.5-97.6) for the binary classification of normal vs. abnormal images; wax plugs were detected with a sensitivity of 100% (94.6-100) and specificity of 97.7% (95.0-99.1); other class-specific estimates of sensitivity and specificity ranged from 33.3% to 92.3% and 96.0% to 100%, respectively. We present an end-to-end DL-enabled system able to achieve expert-level diagnostic accuracy for identifying normal tympanic aspects and wax plugs within digital otoscopic images. However, the system's performance varied for other middle-ear conditions. Further prospective validation is necessary before wider clinical deployment.

Pediatric Cholesteatoma Follow-Up: Residual and Recurrence in 239 Cases with Over 5-Year Hindsight.

INTRODUCTION: Pediatric cholesteatoma is an aggressive disease which requires long-term evaluation to assess management strategies. The objective was to determine optimal follow-up duration in pediatric cholesteatoma to detect residual and recurrent diseases. METHODS: This cohort study was set in a tertiary referral center. All consecutive patients with a minimum 5-year follow-up were included. Medical history, initial extension, surgical procedures, and follow-up were collected. The main outcome measure were Kaplan-Meier survival curves of residual and recurrence cumulative incidence. RESULTS: Totally 239 ears with the first tympanoplasty between 2008 and 2014 were studied including 25% congenital. At first surgery (S1), mean age was 8.4 years and mean follow-up time 7.9 years. Mastoidectomy was performed in 69% and stapes was absent in 38% of cases. Notably, 83% (199 ears) had a second procedure (S2) of which 186 were planned. After S1, maximum cumulated incidence of residual was 45% [95%CI, 38%; 52%] at 74 months, with probability of residual of 39% at 3 years. After S2 (n = 199), maximum cumulated residual incidence was 21% [95%CI, 12%; 32%] at 62 months and 16% at 3 years. Concerning recurrence, maximum cumulated incidence after S1 (n = 239) was reached at 98 months with 21% [95%CI, 12%; 32%], 13% at 3 years and 16% at 5 years. Congenital disease had significantly less residuals after S1 (p = 0.02), but similar recurrence rate (p = 0.66) compared with acquired. CONCLUSIONS AND RELEVANCE: We recommend MRI follow-up of at least 5 years after the last surgery for residual disease and clinical follow-up of at least 10 years to detect recurrence. LEVEL OF EVIDENCE: 4 Laryngoscope, 134:4789-4798, 2024.

Management of sleep-disordered breathing in patients with syndromic hemifacial macrosomia.

PURPOSE: Patients with syndromic hemifacial microsomia (SHFM) are at risk of obstructive sleep apnea (OSA). The aim of the study was to describe the prevalence of OSA and its management, especially in patients with Goldenhar syndrome (GS). METHODS: The respiratory polygraphies and clinical management of 15 patients, aged 2 to 23 years, evaluated at a national reference center, were analyzed. RESULTS: Four (27%) patients had no OSA, 4 (27%) had mild OSA, and 7 (46%), of whom 5 were ≤ 2 years old, had severe OSA. None of the patients had central apneas. Only one patient had alveolar hypoventilation, and another one had nocturnal hypoxemia. Two patients had severe OSA despite prior adenoidectomy or mandibular distraction osteogenesis. Median duration of follow-up was 3.5 years (range 0.5-9 years). None of the patients without OSA or with mild OSA at baseline respiratory polygraphy developed OSA during the follow up. Among the 7 patients with severe OSA, 3 required continuous positive airway pressure or noninvasive ventilation, and one patient required a tracheostomy. CONCLUSION: In conclusion, patients with SHFM are at high risk of severe OSA at any age, underlining the importance of systematic sleep studies to diagnose and evaluate the severity of OSA. Individualized treatment should be privileged, based on a careful examination of the entire upper airway, taking in account potential associated risk factors. All patients with SHFM should be managed by a pediatric expert multidisciplinary medical/surgical team until the end of post pubertal growth.

The Prognostic Value of Olfactory Dysfunction in Patients with COVID-19: The COVIDORA Study.

BACKGROUND: Among all studies describing COVID-19 clinical features during the first wave of the pandemic, only a few retrospective studies have assessed the correlation between olfac-tory dysfunction (OD) and the evolution of disease severity. The main aim was to assess whether OD is a predictive factor of COVID-19 severity based on the patient's medical management (outpa-tient care, standard hospital admission, and ICU admission). METHODS: A national, prospective, mul-ticenter cohort study was conducted in 20 public hospitals and a public center for COVID-19 screen-ing. During the first wave of the pandemic, from 6 April to 11 May 2020, all patients tested positive for COVID-19 confirmed by RT-PCR underwent two follow-up ENT consultations within 10 days of symptom onset. The main outcome measures were the evolution of medical management (out-patient care, standard hospital admission, and ICU admission) at diagnosis and along the clinical course of COVID-19 disease. RESULTS: Among 481 patients included, the prevalence of OD was 60.7%, and it affected mostly female patients (74.3%) under 65 years old (92.5%), with fewer comor-bidities than patients with normal olfactory function. Here, 99.3% (290/292) of patients with OD presented with non-severe COVID-19 disease. Patients reporting OD were significantly less hospi-talized than the ones managed as outpatients, in either a standard medical unit or an ICU. Conclu-sions: As regards the clinical course of COVID-19 disease, OD could predict a decreased risk of hospitalization during the first wave of the pandemic.

Dangers and therapeutic difficulties of intracranial hemangioma in infants: A CARE case report.

INTRODUCTION: Description of neurological complications induced by intracranial hemangioma in infants and by the initiation of beta-blocker treatment (propranolol). OBSERVATION: A 2-month-old infant was referred for grade 5 non-congenital unilateral peripheral facial palsy. Work-up revealed ipsilateral profound hearing loss and two intracranial hemangiomas: one in the ipsilateral internal auditory canal (IAC), the other in the cerebellum opposite the nodule of vermis. Initial treatment with a beta-blocker (propranolol 1mg/kg/day for 1month, then 3mg/kg/day) resulted in disappearance of symptoms and regression of lesions within 8weeks. At 20months after introduction of maintenance therapy (propranolol 3mg/kg/day), two asthma attacks occurred, leading to initiation of fluticasone and continuation of the beta-blocker. Thirty months after discontinuation of treatment, no further progression was noted. DISCUSSION: Unilateral facial palsy in an infant suggests a number of diagnoses. MRI revealed IAC hemangioma. The choice of dosage and duration of treatment was based on a review of the literature and a strategy defined in multidisciplinary consultation.

Performance of Non-EPI DW MRI for Pediatric Cholesteatoma Follow-Up.

OBJECTIVE: To evaluate the accuracy, sensitivity, and specificity of nonecho planar (non-EPI) diffusion-weighted (DW) magnetic resonance imaging (MRI) to detect residual cholesteatoma in children. STUDY DESIGN: Retrospective study. SETTING: Tertiary comprehensive hospital. METHODS: Children operated on for a first-stage cholesteatoma procedure from 2010 to 2019 were included. MRIs were performed with non-EPI DW sequences. Initial reports were collected, indicating the presence or absence of hyperintensity suggestive of cholesteatoma. Three hundred twenty-three MRIs were correlated with the subsequent surgery (66%) or year-later MRI (21%), or were considered accurate if performed 5 years or more after the last surgery (13%). The sensitivity, specificity, positive predictive value (PPV), and negative predictive value (NPV) of each imaging procedure for the detection of cholesteatoma were calculated. RESULTS: Two hundred twenty-four children with mean age of 9 ± 4 years old presented with cholesteatoma. MRIs were performed 27 ± 24 months after surgery. Residual cholesteatoma was diagnosed in 35%. The sensitivity, specificity, PPV, and NPV of MRI were 62%, 86%, 74%, and 78%, respectively. Accuracy, sensitivity, and specificity increased significantly over time (multivariate analysis). The mean delay after last surgery was of 30 ± 2.0 months for accurate MRI (true positive or negative) versus 17 ± 2.0 months for nonaccurate (false positive or negative) MRIs (p < .001). CONCLUSION: However, long the delay after the last surgery, the sensitivity of non-EPI diffusion sequence MRI in children has limitations for the detection of residual cholesteatoma. Surveillance for residual cholesteatoma should incorporate findings at primary surgery, surgeon experience, a low threshold for second-look procedures, and routine imaging.

Latissimus dorsi free flap reconstruction of a temporal defect following pediatric oncologic surgery: Case report + literature review.

INTRODUCTION: Rhabdomyosarcoma (RMS) is the most common soft-tissue sarcoma in children and adolescents. Around 35 % of pediatric sarcomas occur in the head and neck region. Consequently, RMS is considered the most common type of childhood malignancy diagnosed in this region. OBSERVATION: We report the clinical case of a 6 years old patient, who presented a large temporal hollowing following oncological excision surgery for temporal rhabdomyosarcoma. He underwent surgical reconstruction to fill the right temporalis fossa using a latissimus dorsi muscle free flap micro-anastomosed to the lingual vessels. DISCUSSION: This clinical case highlights the value of plastic surgery in oncological reconstruction, which, combined with a multidisciplinary and collective approach, enables a holistic approach and facilitates socio-psychological integration after oncological surgery in the pediatric population.

International Pediatric Otolaryngology Group (IPOG) consensus on scoring of pediatric Drug Induced Sleep Endoscopy (DISE).

OBJECTIVES: To develop consensus statements for the scoring of pediatric drug induced sleep endoscopy in the diagnosis and management of pediatric obstructive sleep apnea. METHODS: The leadership group identified experts based on defined criteria and invited 18 panelists to participate in the consensus statement development group. A modified Delphi process was used to formally quantify consensus from opinion. A modified Delphi priori process was established, which included a literature review, submission of statements by panelists, and an iterative process of voting to determine consensus. Voting was based on a 9-point Likert scale. Statements achieving a mean score greater than 7 with one or fewer outliers were defined as reaching consensus. Statements achieving a mean score greater than 6.5 with two or fewer outliers were defined as near consensus. Statements with lower scores or more outliers were defined as no consensus. RESULTS: A total of 78 consensus statements were evaluated by the panelists at the first survey - 49 achieved consensus, 18 achieved near consensus, and 11 did not achieve consensus. In the second survey, 16 statements reached consensus and 5 reached near consensus. Regarding scoring, consensus was achieved on the utilization of a 3-point Likert scale for each anatomic site for maximal observed obstructions of <50% (Score 0, no-obstruction), ≥ 50% but <90% (Score 2, partial obstruction), and ≥ 90% (Score 3, complete obstruction). Anatomic sites to be scored during DISE that reached consensus or near-consensus were the nasal passages, adenoid pad, velum, lateral pharyngeal walls, tonsils (if present), tongue base, epiglottis, and arytenoids. CONCLUSION: This study developed consensus statements on the scoring of DISE in pediatric otolaryngology using a modified Delphi process. The use of a priori process, literature review, and iterative voting method allowed for the formal quantification of consensus from expert opinion. The results of this study may provide guidance for standardizing scoring of DISE in pediatric patients.

Diagnostic Accuracy of Group A Streptococcus Rapid Antigen Detection Test on Middle Ear Fluid in Children With Acute Otitis Media With Spontaneous Perforation: A Prospective Multicenter Evaluation.

Group A Streptococcus is one of the leading causes of otorrhea. The performance of rapid antigen tests in 256 children with otorrhea showed excellent sensitivity, 97.3% (95% confidence interval: 90.7%-99.7%), and specificity, 100% (95% confidence interval: 98.0%-100%). In a period of increasing invasive and noninvasive group A Streptococcus infections, an early diagnosis could be useful.

Early Magnetic Resonance Imaging to Diagnose Residual Cholesteatoma in Children and Benefit of Radiological Rereview.

OBJECTIVE: Non-echo-planar diffusion-weighted (DW) magnetic resonance imaging (non-EPI MRI) is the appropriate sequence to detect residual cholesteatoma. In the child, MRI may be clinically useful to determine the timing of the second-look procedure. The aim of this paper was to retrospectively evaluate the performance of early MRI (before the 18th postoperative month) in detecting residual cholesteatoma in children after review by experienced specialized neuroradiologists. STUDY DESIGN: Retrospective study. SETTING: One university center comparative cohort. METHODS: All patients who had a 2-staged procedure for cholesteatoma with an MRI before the second stage from 2010 to 2020 were included and analyzed. Three pediatric neuroradiologists reviewed all the images blinded to the surgical result. RESULTS: N = 141 cholesteatoma events (140 children) were included with a mean age at MRI of 10 (±4) years old. Non-EPI MRIs were performed 10.7 (±3.8) months after the first-stage surgery and 2.2 (±2.6) months before the second-stage procedure. Non-EPI MRI had a 0.57 sensitivity (SE) and 0.83 specificity (SP). MRI was reviewed in 112 cases. The diagnosis was corrected in 17 cases (15.1%) (3 true positives, 7 false negatives, and 7 false positives). SE = 0.63 (p = 0.1) and SP = 0.92 (p = 0.08) after rereading. CONCLUSION: Early MRI's SE is poor but SP is excellent after rereading. Evidence does not support the use of early non-EPI MRI to modify the surgical strategy or to postpone the second look. If performed, early non-EPI MRI should be read by specialized experienced radiologists with all 3 sequences (T1, T2, and non-EPI DW) and apparent diffusion coefficient calculation, especially in cases of otitis media with effusion.

Craniofacial growth and function in achondroplasia: a multimodal 3D study on 15 patients.

BACKGROUND: Achondroplasia is the most frequent FGFR3-related chondrodysplasia, leading to rhizomelic dwarfism, craniofacial anomalies, stenosis of the foramen magnum, and sleep apnea. Craniofacial growth and its correlation with obstructive sleep apnea syndrome has not been assessed in achondroplasia. In this study, we provide a multimodal analysis of craniofacial growth and anatomo-functional correlations between craniofacial features and the severity of obstructive sleep apnea syndrome. METHODS: A multimodal study was performed based on a paediatric cohort of 15 achondroplasia patients (mean age, 7.8 ± 3.3 years), including clinical and sleep study data, 2D cephalometrics, and 3D geometric morphometry analyses, based on CT-scans (mean age at CT-scan: patients, 4.9 ± 4.9 years; controls, 3.7 ± 4.2 years). RESULTS: Craniofacial phenotype was characterized by maxillo-zygomatic retrusion, deep nasal root, and prominent forehead. 2D cephalometric studies showed constant maxillo-mandibular retrusion, with excessive vertical dimensions of the lower third of the face, and modifications of cranial base angles. All patients with available CT-scan had premature fusion of skull base synchondroses. 3D morphometric analyses showed more severe craniofacial phenotypes associated with increasing patient age, predominantly regarding the midface-with increased maxillary retrusion in older patients-and the skull base-with closure of the spheno-occipital angle. At the mandibular level, both the corpus and ramus showed shape modifications with age, with shortened anteroposterior mandibular length, as well as ramus and condylar region lengths. We report a significant correlation between the severity of maxillo-mandibular retrusion and obstructive sleep apnea syndrome (p < 0.01). CONCLUSIONS: Our study shows more severe craniofacial phenotypes at older ages, with increased maxillomandibular retrusion, and demonstrates a significant anatomo-functional correlation between the severity of midface and mandible craniofacial features and obstructive sleep apnea syndrome.

Adverse Events and Morbidity in a Multidisciplinary Pediatric Robotic Surgery Program. A prospective, Observational Study.

OBJECTIVE: To report one-year morbidity of robotic-assisted laparoscopic surgery (RALS) in a dedicated, multidisciplinary, pediatric robotic surgery program. Summary Background Data. RALS in pediatric surgery is expanding, but data on morbidity in children is limited. METHODS: All children who underwent RALS (Da Vinci Xi, Intuitive Surgical, USA) were prospectively included (October 2016 to May 2020; follow-up ≥1 year). Analyzed data: patient characteristics, surgical indication/procedure, intraoperative adverse events (ClassIntra classification), blood transfusion, hospital stay, postoperative complications (Clavien-Dindo). RESULTS: Three hundred consecutive surgeries were included: urology/gynecology (n=105), digestive surgery (n=83), oncology (n=66), ENT surgery (n=28), thoracic surgery (n=18). Median age and weight at surgery were 9.5 [interquartile range (IQR)=8.8] years and 31 [IQR=29.3] kg, respectively. Over one year, 65 (22%) children presented with ≥1 complication, with Clavien-Dindo ≥III in 14/300 (5%) children at ≤30 days, 7/300 (2%) at 30-90 days, and 12/300 (4%) at >90 days. Perioperative transfusion was necessary in 15 (5%) children, mostly oncological (n=8). Eight (3%) robotic malfunctions were noted, one leading to conversion (laparotomy). Overall conversion rate was 4%. ASA ≥3, weight ≤15 kg, and surgical oncology did not significantly increase the conversion rate, complications, or intraoperative adverse events (ClassIntra ≥2). ASA score was significantly higher in children with complications (Clavien-Dindo ≥III) than without (p=0.01). Median hospital stay was 2 [IQR=3] days. Three children died after a median follow-up of 20 [IQR=16] months. CONCLUSIONS: RALS is safe, even in the most vulnerable children with a wide scope of indications, age, and weight. Robot-specific complications or malfunctions are scarce.

Endoscopic endonasal repair of bilateral choanal atresia in a 1200 g preterm infant: Is it feasible? (With video).

INTRODUCTION: Bilateral choanal atresia (CA) is a rare congenital anomaly, that causes neonatal respiratory distress. No consensus exists regarding the most appropriate surgical approach in low-birth weight preterm infants. CASE REPORT: We present (with video) the case of a male born at 29 weeks' gestation, 1200 g, affected by bilateral CA. He was successfully treated with an endoscopic transnasal approach performed on day 4 of life which allowed a very early extubation. Otologic instruments and 2.7 mm wide endoscope were used to be able to operate in very narrow nasal cavities. Thulium LASER® was used to limit bleeding. At the follow-up visit, four months after surgery, the neochoana was widely patent. DISCUSSION: In low-birth weight preterm infants, endoscopic transnasal surgery (ETS) may be considered technically not feasible or with a high risk of early restenosis. With early ETS, we were able to avoid the morbidity of a prolonged intubation and sedation. CONCLUSION: Bilateral CA is a life threatening and challenging clinical entity, particularly difficult to treat in low-birth preterm infants. Early surgery to reduce intubation and sedation is preferable, but requires experienced teams with a surgical technique and instruments tailored to the narrowness of the nasal cavity.

French adaptation and validation of parents' evaluation of aural/oral performance of children (PEACH) scale in children.

OBJECTIVE: Hearing loss can seriously impact children's quality of life. Disease-specific questionnaires are required to optimise medical care. This study aims to translate, adapt and validate the French version of the PEACH score for the auditory performance of children. DESIGN: This is a controlled, prospective study, conducted between April and October 2020. The translation was conducted using a forward-backward technique, and statistical validation was conducted with a test and re-test, on a patient population and a control population. STUDY SAMPLE: Patients were included if they were 1-11 years old, and had at least 30 dB hearing loss in one ear. The mean age was 6 years for the 39 patients and 3.9 years for the 34 controls. RESULTS: Reproducibility, measured by Spearman's coefficient between global scores of the test and re-test was 0.78 (p < 0.001). The test was internally consistent (Cronbach's alpha was 0.89) and item per item construct validity was satisfactory. The ROC curve showed a moderate area under the curve (0.74 p < 0.001) with 67% sensitivity and 73% specificity. CONCLUSIONS: The French PEACH had good statistical properties, although a brief 13-item questionnaire, and can be used for evaluation of the disease-specific quality of life for young children with hearing loss.

French adaptation and validation of the Speech, Spatial and Qualities of Hearing scale for Parents (SSQ-P) and for Children (SSQ-Ch).

OBJECTIVES: Hearing loss can seriously impact children's daily life. This study aims to translate and validate the French versions of the hearing performance questionnaires, SSQ-Parent (for 5-18 years old children), and SSQ-Children (for 11-18 years old children). DESIGN: This controlled prospective trial was conducted between April and October 2020. The forward-backward translation method was used, and a test-retest procedure was carried out on a case and a control population. Cases had at least 30 dBHL hearing loss. STUDY SAMPLE: 54 cases (mean age 10.4 years old) and 32 controls (mean age 12.5 years old) answered the SSQ-Parent. 35 cases (mean age 13.1 years old) and 35 controls (mean age 14.3 years old) answered the SSQ-Children. RESULTS: Spearman's correlation coefficients between global scores of the test and re-test were 0.91 (p < 0.001) for SSQ-Parent, and 0.89 (p < 0.001) for SSQ-Children. Both tests were discriminant (respectively, global score 57.8 vs 92 p < 0.001, 61.2 vs 92.6 p < 0.001), and internally consistent (Cronbach's alpha 0.94 and 0.97). Items-global score correlation was satisfactory. ROC curves showed high area under curve for the SSQ-Children (0.990), and SSQ-Parent (0.988). CONCLUSION: The SSQ-Parent and SSQ-Children revealed excellent statistical properties, and can be used for the evaluation of hearing performance of children.

Cerebral sinovenous thrombosis associated with head/neck infection in children: Clues for improved management.

AIM: To compare paediatric patients with cerebral sinovenous thrombosis (CSVT) with and without head/neck infection to improve management of the condition. METHOD: We conducted a bicentric retrospective study of consecutive children (neonates excluded) with radiologically confirmed CSVT, comparing children with a concurrent head/neck infection and children with other causes. RESULTS: A total of 84 consecutive patients (46 males and 38 females) with a median age of 4 years 6 months (range 3 months-17 years 5 months) were included. Associated head/neck infection was identified in 65.4% of cases and represented the main identified CSVT aetiology. Children in the head/neck infection group displayed a milder clinical presentation and less extensive CSVT. Median time to complete recanalization was significantly shorter in this group (89 days [interquartile range 35-101] vs 112.5 days [interquartile range 83-177], p = 0.005). These findings were even more pronounced in the subgroup of patients with otogenic infection and no neurological sign. INTERPRETATION: As CSVT in the setting of an otogenic infection and no neurological sign seems to represent a milder condition with a shorter course, these results suggest adapting current recommendations: consider earlier control imaging in paediatric otogenic CSVT, and shorter anticoagulant treatment if recanalization is obtained. WHAT THIS PAPER ADDS: Children with cerebral sinovenous thrombosis related to head/neck infections have a milder clinical presentation. They also have a shorter recanalization time, especially if there is otogenic infection without neurological symptoms.

Thyroid cancer in children: A multicenter international study highlighting clinical features and surgical outcomes of primary and secondary tumors.

Background: Thyroid gland malignancies are rare in pediatric patients (0.7% of tumors); only 1.8% are observed in patients aged <20 years, with a higher prevalence recorded in women and adolescents. Risk factors include genetic syndromes, MEN disorders, autoimmune diseases, and exposure to ionizing radiation. Radiotherapy is also associated with an increased risk of secondary thyroid cancer. This study describes the clinical features and surgical outcomes of primary and secondary thyroid tumors in pediatric patients. Methods: Institutional data were collected from eight international surgical oncology centers for pediatric patients with thyroid cancer between 2000 and 2020. Statistical analyses were performed using the GraphPad Prism software. Results: Among 255 total cases of thyroid cancer, only 13 (5.1%) were secondary tumors. Primary thyroid malignancies were more likely to be multifocal in origin (odds ratio [OR] 1.993, 95% confidence interval [CI].7466-5.132, p = 0.2323), have bilateral glandular location (OR 2.847, 95% CI.6835-12.68, p = 0.2648), and be metastatic at first diagnosis (OR 1.259, 95% CI.3267-5.696, p > 0.999). Secondary tumors showed a higher incidence of disease relapse (OR 1.556, 95% CI.4579-5.57, p = 0.4525) and surgical complications (OR 2.042, 95% CI 0.7917-5.221, p = 0.1614), including hypoparathyroidism and recurrent laryngeal nerve injury. The overall survival (OS) was 99% at 1 year and 97% after 10 years. No EFS differences were evident between the primary and secondary tumors (chi-square 0.7307, p = 0.39026). Conclusions: This multicenter study demonstrated excellent survival in pediatric thyroid malignancies. Secondary tumors exhibited greater disease relapse (15.8 vs. 10.5%) and a higher incidence of surgical complications (36.8 vs. 22.2%).