International journal of pediatric otorhinolaryngology最新文献

Purpose: Cystic fibrosis (CF) is the most common autosomal recessive disorder in the Caucasian population. Otolaryngological manifestations pose a significant impact on the quality of life of children with CF. The primary aim of this review is to provide a state of the art update of current literature on the otolaryngological manifestations of CF in children.

Methods: We systematically reviewed the PubMed, Cochrane Library, Embase and Web of Science databases, for prospective studies including pediatric patients with cystic fibrosis, reporting on otolaryngological manifestations. After assessment of the risk of bias and quality of the included studies, data were extracted.

Results: The search retrieved 6745 unique items after duplicate removal. After three selection rounds and quality assessment, 38 articles were ultimately retained for data extraction. The total number of participants in the studies was 1981. Most studies were prospective cohort studies (n = 28). The articles were divided into six groups (ear/speech disorders - aminoglycoside ototoxicity (n = 10); otolaryngology-related quality of life (n = 5); nasal and paranasal sinuses (n = 11); sleep disorders (n = 3); paranasal sinuses imaging (n = 5); other (n = 4)).

Conclusion: The most common otolaryngological manifestation of children with CF is chronic rhinosinusitis, but CF can have other otolaryngological-related pathologies such as hearing loss, middle ear problems, sleep apnea syndrome, and decreased smell and/or taste functions. We found a considerable gap in the literature if we would draw evidence-based conclusions on diagnosis and management of otolaryngological manifestations in children with CF.

Purpose: Speech-language Therapists (SLTs) are specialists in communication, feeding and swallowing as core members of the paediatric tracheostomy multidisciplinary team (MDT). Inconsistent tracheostomy care leads to staff and family frustration and delayed intervention. Little is known about international SLT tracheostomy practices.

Methods: This global survey explored training, clinical practices, perceived knowledge, skills and roles, and barriers for SLTs working with paediatric tracheostomy. Survey questions consisted of Likert scale and open-ended questions. 92 SLTs completed the survey representing 6 continents and 19 countries.

Results: Overall SLTs were confident in their knowledge of communication, and feeding but less confident in weaning, tracheostomy care skills (such as suctioning and cuff deflation) and specialist tracheostomy tube use (such as tracheostomy tubes with a subglottic suction port). Formal competency training was infrequent but most had access to supervision. Understanding of the SLT role by the MDT was perceived as good. Many felt managing one-way speaking valves was part of their role. The majority reported staffing, lack of guidelines and access to resources (one-way speaking valves and flexible endoscopic evaluation of swallowing) as barriers.

Conclusions: This expert opinion paper summarises the current evidence-base and international paediatric tracheostomy practice. SLTs were experienced and confident in tracheostomy. The SLT role varies internationally with a lack of formal written policy to guide practice and a variability in access to education.

Objective: To evaluate acute pediatric mastoiditis numbers and associated complications before, during, and after the COVID-19 pandemic.

Methods: A retrospective chart review of all children less than 18 years of age presenting to a pediatric tertiary medical center with acute mastoiditis from January 1, 2017 to October 30, 2023 was completed. Exclusion criteria included history of chronic mastoiditis, cholesteatoma and mastoidectomy. Demographic and clinical data were collected and included age, sex, race, ethnicity, duration between onset of symptoms and presentation, white blood cell count and C-reactive protein level, affected ear, intracranial and extracranial complications, procedures, readmission and reoperation, length of hospital stay, and culture results. Patients were classified in relation to the COVID pandemic into three groups: pre-COVID included patients presenting from January 1, 2017, until December 31, 2019, COVID from January 1, 2020, until December 31, 2021, and post-COVID from January 1, 2022, until October 30, 2023.

Results: A total of 89 patients, 49 males and 40 females, with a mean age of 53.8 ± 40.7 months were included. Twenty-four (27.0 %) patients presented in the pre-COVID, 14 (15.7 %) in the COVID, and 51 (57.3 %) in the post-COVID period. There was no difference in the mean duration between onset of symptoms and presentation across the groups (5.4 ± 4.4 days pre-COVID, 5.8 ± 6.8 days during COVID, and 8.5 ± 8.1 post-COVID, P = 0.16). The post-COVID mean annual number of mastoiditis cases (27.8 cases/year) was higher compared to pre-COVID (8 cases/year) and COVID (7 cases/year). Similarly, the post-COVID mean annual number of cases with complications (24 cases/year) was higher compared to pre-COVID (5 cases/year) and COVID (5.5 cases/year). Of note, the post-COVID mean annual number of cases with intracranial complications (12cases/year) was also higher compared to pre-COVID (1.3cases/year) and COVID (1.5cases/year). There was no difference in the number of cases requiring operation across cohorts (97.8 % overall, p = 0.07).

Conclusion: This study revealed a 3.5-fold increase in the mean annual number of mastoiditis cases and a 4.8-fold increase in the mean annual number of cases with complications post-COVID compared to pre-COVID.

Background: Internal and external devices may be utilized in mandibular distraction osteogenesis (MDO) for the correction of symptomatic micrognathia in infants and children.

Purpose: To compare the rate and severity of infection between internal and external MDO devices.

Study design, setting, sample: Retrospective cohort study utilizing an institutional database of patients who underwent MDO.

Independent variable: Use of internal versus external MDO hardware.

Main outcome variables: Rate of post-operative surgical site infections (minor and major).

Covariates: Patient age, device type, laterality, infection, and treatment were documented.

Analysis: Chi-Square and Fisher Exact Tests were used where appropriate for categorical variables and two-tailed T-tests were used for continuous variables. Significance was set at p < 0.05.

Results: Between 2010 and 2022, 36 infants (ages 7 days-12 months) underwent bilateral MDO. Thirteen cases utilized internal hardware (n = 26 surgical sites) and 23 cases utilized external hardware (n = 46 surgical sites). Fifteen patients developed post-operative infections (41.7 %), 11 of which were minor infections and 4 were major infections. Seven patients with internal devices (53.8 %) and 8 patients with external devices (34.8 %) developed an infection (p = 0.27). Minor infections occurred in 4 patients with internal hardware (30.8 %) and 7 patients with external hardware (30.4 %; p = 1.00). Major infections occurred in 3 patients with internal hardware (23.1 %) and 1 patient with external hardware (4.3 %; p = 0.25). There were 19 surgical site infections (26.4 %), 14 of which were minor infections and 5 of which were major infections. Ten internal devices (38.6 %) and 9 external devices (19.6 %) were complicated by infection (p = 0.08). Minor infections occurred in 6 internal devices (23.1 %) and 8 external devices (17.4 %; p = 0.56). Major infections occurred in 4 internal devices (15.4 %) and 1 external device (2.2 %; p = 0.05).

Conclusion: No significant difference was found in overall postoperative infection rate with internal and external MDO. A lower rate of major infection was observed in external devices.

Objective: To observe the efficacy of EarWell ear orthotics in treating congenital ear deformities in older children (≥7 weeks) within 98 days.

Method: A retrospective study was conducted on 58 infants (69 ears) aged 7-14 weeks after birth. Divided into three groups: A (7-9 weeks old, 26 ears), B (10-12 weeks old, 23 ears), and C (13-14 weeks old, 20 ears). Analyze the differences in treatment time, effective rate, six-month recurrence rate, and incidence of complications among three age groups.

Result: Age group A had the shortest treatment time (25.13 ± 3.28d) and the highest effective rate (84.61 %), while age group C had the longest treatment time (48.35 ± 7.64d) and the lowest effective rate (60.00 %), with statistically significant differences (p < 0.05); The six-month recurrence rate and incidence of complications in the A age group were lower than the other two groups, and the difference was statistically significant (p < 0.05); The recurrence cases in the three age groups are mainly concentrated in the cup ear and the prominent ear.

Conclusion: EarWell ear orthotics can still be chosen for morphological correction of congenital ear deformities in children aged 7-14 weeks. However, the effectiveness of treatment may be affected by the age of treatment and the type of malformation. The older the age of treatment, the lower the effectiveness of orthosis treatment, the longer the treatment time, and the higher the probability of complications. Cup ears and prominent ear over 7 weeks old have a higher recurrence rate after treatment, and it is recommended to correct them as soon as possible.

Objective: This study aims to compare the postoperative thoracic deformity and scar aesthetic outcomes of auricular reconstruction between the conventional large-incision technique and small-incision technique with perichondrium preservation for costal cartilage harvesting.

Methods: A retrospective analysis was performed on 134 patients who underwent auricular reconstruction using tissue expanders and autologous costal cartilage harvested from the right chest wall between January 2021 and September 2023. Patients were divided into two groups according to the harvesting technique: the traditional large-incision group (n = 64) and the small-incision group with perichondrium preservation (n = 70). Preoperative and postoperative chest CT scans were reviewed for thoracic deformities by measurements of the modified Haller Index (mHI) and modified correction index (mCI). Thoracic scar dimensions were measured in postoperative follow-up.

Results: In the conventional group, significant difference were observed between the left and right hemithorax in anterior-posterior diameter, maximum transverse diameter, cross-sectional area, and mHI values (p < 0.05), showing thoracic deformities. In contrast, within the small-incision group, there were no significant differences between hemithoraces. Additionally, postoperative thoracic scars were significantly smaller in the small-incision group, with shorter scar length (5.63 ± 1.33 cm vs. 11.44 ± 1.31 cm) and width (1.41 ± 0.55 cm vs. 3.75 ± 1.54 cm; p < 0.001).

Conclusion: The small-incision technique with perichondrium preservation significantly reduces postoperative thoracic deformities and results in smaller, less conspicuous scars compared to the traditional large-incision method. This approach minimizes disruption to the chest wall structure and reduces donor-site morbidity, offering improved patient outcomes.

Objectives: Define the extent to which pathogenic GJB2 (gap junction beta-2) variants are responsible for non-syndromic hearing loss (NSHL) in the Asian population.

Methods: Preferred Reporting Items for Systematic Reviews and Meta-Analysis (PRISMA) guidelines were followed. CINAHL, Embase, and PubMed's MEDLINE were accessed from 1997 to 2023 using permutations of the MeSH terms: "Asian," ''Southeast Asian,'' "South Asian," "East Asian," "Southeastern Asian," and "GJB2." Additionally, all countries within the Indian subcontinent, Far East, and Southeast Asia, were included as key terms. Exclusion criteria included non-English publications, a non-Asian study population (per US Office of Management and Budget), and literature not investigating GJB2. An allele frequency analysis of pathogenic GJB2 variants in the Asian population was performed and stratified by country of origin.

Results: One thousand one hundred and forty-one unique studies were identified, of which 420 met our inclusion criteria during the abstract screen. One hundred and ninety-five studies were included in the systematic review after full-text screen. Over 45 pathogenic variants were identified across 11 countries within the Indian subcontinent, Far East, and Southeast Asia. A total of 4,220,591 people from over 30 ethnic groups were included with ages ranging from 0 to 97 years. Of those with reported demographic information, 50 % (221,336/445,813) were female and 50 % (224,477/445,813) were male. The prevalence of pathogenic GJB2 variants varied by country, with common variants including c.235del; p.Leu79Cysfs∗3, c.109G > A; p.Val37Ile, and c.299_300del; p.His100Argfs∗14.

Conclusion: Variation in the prevalence of pathogenic GJB2 variants is likely due to the wide diversity of ancestral contributions in the Asian population. There are limited studies on the prevalence of GJB2 variants particularly for countries within the Indian subcontinent and Southeast Asia. Additional studies on the prevalence of GJB2 variants in these countries as well as ethnic sub-groups may be helpful in the development of assays for high throughput diagnosis for patients with hereditary hearing loss.

Objective: Aspirated foreign bodies (FB) are potentially life-threatening conditions which can be challenging to diagnose in children. The previous similar study from our hospital, published in 2014, created a guideline suggesting when to proceed to laryngobronchoscopy (LB) in suspected FB aspiration with over 99 % sensitivity. The grading system included the presence or absence of acute history, positive examination, and/or radiological findings, including witnessed choking, dysphonia, stridor, wheezing, or reduced air entry on examination or abnormal radiological findings. The current study reviewed a further 10 years and 5 months of data to re-evaluate the ongoing diagnostic usefulness of the three positive findings in children with suspicion of aspirated FB.

Methods: We undertook a retrospective review of all LB performed at our institution for suspected acute FB aspiration from August 2013 to January 2023. We analysed patient characteristics, clinical examination, radiological findings, and outcomes.

Results: We found a total of 100 children under the age of 16 years who underwent LB for suspected FB aspiration. The mean age of our study population was 2.6 years. The population demographics were similar to the results from the previous study from our institution, with Māori and Pasifika children having the highest probability of finding a FB on LB. Male children presenting for suspected FB aspiration are 1.39 times more likely than female children to have a foreign body at LB (p = 0.04). Overall, having two or more positive findings had a sensitivity of 100 %. History or examination alone are more sensitive (both 95.6 %) but less specific (12.5 % and 31.3 %, respectively). Abnormal radiology alone was more specific (75.0) but less sensitive (77.9 %). The most common site of FB was the right bronchial tree (45.6 %), followed by the left (39.7 %). 95 % of FB were organic, and 54 % were nuts. The negative bronchoscopy rate was 32 %.

Conclusion: Two or more positive indicators in history, examination and radiological findings continue to be highly sensitive predictors for the presence of an aspirated foreign body in our paediatric population. We recommend a future large-scale multicentre study to confirm how applicable these findings would be to a broader cohort of patients.

Objective: Although NOG variants are linked to congenital stapes fixation and conductive hearing loss (CHL), little is known about middle ear surgery outcomes and the characteristics of accompanying inner ear anomalies. We explored auditory phenotypes in patients with NOG variants, with a focus on the outcomes of middle ear surgery.

Methods: This study included 11 patients from five unrelated Korean families harboring NOG variants. Genomic investigations were conducted using whole-exome sequencing and whole-genome sequencing. The clinical phenotypes, including pre- and postoperative audiological profiles, radiological abnormalities, and other comorbidities, were analyzed.

Results: The average age at genetic testing was 8.2 years (range, 0-13 years). Two previously reported NOG variants (c.509C > T:p.Pro170Leu and c.252dup:p.Glu85ArgfsTer97) and three novel NOG variants, including the c.187G > T: p.Glu63Ter and two cryptic large deletion within the 17q22.2 region, were identified. All patients exhibited non-progressive CHL. Inner ear anomalies were documented in two patients, with variations such as cochlea and vestibular dysplasia. In this study, seven ears of four patients underwent stapedotomy, resulting in a significantly reduced air-bone gap of 10.18 ± 1.48 dB (P = 0.016), with sustained improvement. Conversely, patients carrying p.Pro170Leu variant, which is associated with poor outcomes for middle ear surgery, were excluded from surgical consideration.

Conclusion: We expanded the spectrum of genotypes and auditory phenotypes associated with NOG variants. Surgical intervention for CHL underlying NOG variants elicits favorable outcomes. However, clinicians should consider the potential for poor prognosis in certain NOG variants. Collectively, identifying NOG variants could guide the treatment strategies to improve CHL.

Objective: The hypothesis tested was the negative impact of electronic smoke exposure on the developing of respiratory infection, specifically in the upper tract. In this study, we aimed to investigate if smoke exposure increased the number of tonsillectomy surgery in children compared to the hernia repair control group.

Methods: The design of our study was retrospective case-control. We retrospectively reviewed medical records or children of up to 12 years of age that were admitted for tonsillectomy at King Abdullah University Hospital (KAUH) from January 2019 to December 2023 to determine exposure to both electronic and tobacco smoking. Records of hernia repair surgery children aged 12 and less at this facility were also retrospectively reviewed for both electronic and tobacco smoke exposure during the same period. Data was collected from medical records and phone call interviews with the patients and their families.

Results: A total of 743 children were included, 64 % were males. The participants were divided into two groups: 439 had recurrent tonsillitis and needed tonsillectomy, the other 304 underwent hernia repair surgery (control group). Overall, 28 % of children were exposed to electronic smoking, 29 % to tobacco smoking, and 5.9 % to both. Electronic and tobacco smoke exposure was significantly evident among the tonsillectomy group compared to the hernia group. Logistic regression analysis showed that exposure to electronic smoking (OR: 2.74) and tobacco smoking (OR: 2.47) were significantly associated with an increased likelihood of undergoing tonsillectomy. However, patients with exposure to both smoking types did not show a significant link, while the absence of any smoking exposure was significantly associated to lower odds of tonsillectomy.

Conclusion: Children who underwent tonsillectomy had a higher likelihood of being exposed to tobacco or electronic smoking compared to children in the hernia repair surgery group.