Treatment Option for Velopharyngeal Insufficiency: A Speech Aid.

Defects such as cleft palate often co-occur with velopharyngeal (VP) incompetence, leading to dysfunction and a characteristic nasal snort in patients. With the evolution of treatment concepts, treatment of cleft palate patients has been introduced where obturators have helped improve the speech of individuals with partial or total velar defects, that is, cleft of the soft palate. The soft palate separates the oral and nasal cavities. Treatment of VP function, especially VP incompetence, by palatal lift appliances, has become a part of the current trend for the rehabilitation of VP dysfunction. This case report describes the rehabilitation of a dentulous patient with a congenital soft palate defect using a functional impression technique in which a palatal plate with a solid one-piece pharyngeal obturator prosthesis has been used. How to cite this article: Singh R, Goswami R, Saxena D, et al. Treatment Option for Velopharyngeal Insufficiency: A Speech Aid. Int J Clin Pediatr Dent 2024;17(5):601-604.

Construction of virtual airway model to assist surgical correction of velopharyngeal insufficiency with posterior pharyngeal flap.

OBJECTIVE: Posterior pharyngeal flap (PPF) is one of the most common surgical technique to correct velopharyngeal insufficiency(VPI), during which controlling the sizes of the lateral pharyngeal ports(LPP) is the key to outcomes. One innovative procedure was developed to well control the size of LPP. MATERIALS AND METHODS: 40 patients with repaired cleft palate were collected from June 2022 to August 2023. All patients were diagnosed with VPI, and treated with modified PPF surgery. For each patient, upper airway model was reconstructed, and the virtual airway model of PPF was designed. The nasal valve area was measured as it was considered to be the narrowest part of the upper airway. The upper airway resistances under different sizes of LPP was predicted through computational fluid dynamics analysis. The minimum size of each lateral pharyngeal port without obviously increase of upper airway resistance was calculated through effect of lateral pharyngeal ports' size on upper airway resistance. Postoperative follow-up was 6-18 months, including speech outcome and respiration outcome. Resting soft palate length (RVL), effective working length of soft palate (EWL) and angle of soft palate elevation (AVL) were measured and compared according to the lateral cephalometric radiograph. RESULTS: There was a linear relationship between the threshold value and nasal valve (R=0.62). Among the forty patients, the average size of nasal valve was 47.81mm2, the average size of the threshold value of LPP was 31.63mm2. The rate of velopharyngeal closure competence after surgery was 95%. Compared with the preoperative measurements, there were significantly increase of RVL, EWL and AVL (P<0.05). There were significantly difference in the nasal obstruction symptom evaluation score in long-term follow-up compared to short-term follow-up (P<0.05), and no one needed flap revision. There was no significant difference in nasal respiration and nasal resistance before and after surgery (P>0.05). CONCLUSION: With the help of computer fluid dynamics analysis, it is possible to predict the threshold size of lateral pharyngeal port without obviously increasing upper airway resistance and reduce the risk of suffering from airway obstruction for patients undergoing PPF surgery.

Phenotypes of Velopharyngeal Tube Law in Obstructive Sleep Apnea.

OBJECTIVE: The biomechanics of upper airway collapse in obstructive sleep apnea (OSA) remains poorly understood. The goal of this study is to compare the area-pressure relationship (tube law) of the velopharynx at peak inspiration and peak expiration. STUDY DESIGN: Cross-sectional. SETTING: Academic tertiary medical center. METHODS: The velopharyngeal tube law was quantified in a convenience sample of 20 OSA patients via step reductions in nasal mask pressure during drug induced sleep endoscopy (DISE). The velopharyngeal airspace cross-sectional area was estimated from endoscopy while luminal pressure was recorded with a catheter. The tube law was quantified for nasal mask pressures from 14 to 0 cmH2O at peak inspiration and at peak expiration in all patients. The tube law was also quantified during the breathing cycle at a constant nasal mask pressure of 4 cmH2O in 3 patients representing different phenotypes. RESULTS: Velopharyngeal compliance (the slope of the tube law) was not statistically different in the peak inspiration versus peak expiration tube laws. Three phenotypes were observed, namely inspiratory collapse (phenotype 1), expiratory collapse (phenotype 2 = palatal prolapse), and a mostly stable airway during inspiration and expiration that collapsed as CPAP was reduced (phenotype 3). CONCLUSION: Velopharyngeal compliance is not significantly different at peak inspiration and peak expiration, which suggests that muscle tone is low when luminal pressure is above the closing pressure. Additional studies are needed to investigate how different phenotypes of velopharyngeal collapse may affect therapeutic outcomes.

Assessment of pharyngeal airway space with MRI In oral submucous fibrosis: A cross-sectional observational study.

Introduction: Oral submucous fibrosis (OSMF) alters the pharynx, which may affect airway size. MRI will be useful for diagnosing pharyngeal abnormalities. MRI is used to evaluate pharyngeal airway and soft palate changes in OSMF patients. Materials and methods: This study is a cross-sectional observational study that included a sample size of 42 patients. Group A consisted of 21 patients with OSMF, while Group B consisted of 21 volunteers without OSMF, who served as the control group. The patients with OSMF were classified into Stages I, II, and III according to the categorization established by Pindborg JJ in 1989, Stop-Bang questionnaire was employed to assess obstructive sleep apnoea. Magnetic Resonance Imaging (MRI) was utilized to acquire evaluations of the pharyngeal airway, encompassing measurements in the midsagittal, cross-sectional width, length planes, and cross-sectional area with volume, for all participants. The Shapiro-Wilk test determines distribution normality. We utilized one-way ANOVA to compare the means between groups. Results: The average age of OSMF patients was 45.9 ± 8.16, while the control group was 39.19 ± 4.21. Stage I of OSMF had the highest mean Stop Bang questionnaire score (2.75), followed by stage III (2.22), and stage II (1.75). Statistically significant differences (p < 0.001) were seen in volume, linear midsagittal planes, cross-sectional width and length planes, cross-sectional area, and soft palate breadth and length between OSMF and control groups. Conclusion: MRI can effectively examine early changes in the pharyngeal airway of patients with OSMF thereby serving as a constructive diagnostic and motivational tool.

Novel Variation in the External Carotid Artery: Implications for Clinical and Surgical Practice.

The external carotid artery (ECA) is a major branched artery that supplies head and neck structures. An undocumented variation of the ECA was discovered during cadaveric dissection of the anterolateral cervical region, in which a common origin for the ascending pharyngeal, facial, and lingual arteries was identified. In addition, bilateral, duplicate ascending pharyngeal arteries (APAs) were identified at the aforementioned common trunk and the bifurcation of the external and internal carotid arteries. Anatomical knowledge regarding the location of the APA is essential to physicians, as this vessel is a primary supply source for many skull base tumors and vascular lesions. Furthermore, such anatomical knowledge is essential to physicians, as there have been cases of misdiagnosis regarding APA anomalies as an internal carotid artery (ICA) dissection. In this cadaver, both ECAs exhibited typical branching into the superior thyroid artery (STA), occipital artery, posterior auricular artery, maxillary artery, and superficial temporal artery.

National epidemiological survey on pharyngeal, laryngeal, and tracheal stenosis in Japan: A National survey on airway stenosis.

OBJECTIVE: Airway stenosis impairs the quality of life of patients. However, the epidemiology and pathophysiology of airway stenosis remain underexplored owing to its rarity. Airway stenosis may go undetected for a long period without accurate diagnosis or treatment owing to the lack of established treatment guidelines. Thus, clinical information must be accumulated and analyzed to generate evidence-based treatment strategies for this rare entity. METHODS: A retrospective nationwide epidemiological survey was conducted targeting patients with pharyngeal, laryngeal, or tracheal stenosis in Japan. The initial survey was conducted across 1393 facilities between 2013 and 2017 to evaluate the treatment of airway stenosis. The clinical information of the patients was collected via a secondary survey. RESULTS: The primary survey revealed that airway stenosis was treated at only 43 % of the facilities over the 5-year period. The secondary survey revealed that 284 cases were registered across 57 facilities. The number of patients with acquired stenosis exceeded that of those with congenital stenosis. The larynx or cervical trachea was the most common site of stenosis, and intubation or tracheostomy was the most common cause of stenosis. Approximately 76 % of patients underwent surgical treatment, and tracheostomy was the most common procedure. Stenosis persisted in > 70 % of patients at the last visit. CONCLUSIONS: This study clarified the clinical background of patients with pharyngeal, laryngeal, and tracheal stenosis in Japan and the surgical treatment received. The findings of this study confirmed the rarity of airway stenosis and the difficulty in treating this entity.

Anatomical Relationships of Internal Carotid Artery with Posterior Pharyngeal Wall and Upper Cervical Spine: Analysis of 238 Computed Tomography Angiograms.

OBJECTIVE: To evaluate internal carotid artery's (ICA) anatomical relationships with atlantoaxial joints and posterior pharyngeal wall and to illustrate ICA injury risk during transoral procedures to upper cervical spine. METHODS: Cervical spine computed tomography angiography (CTA) of 238 patients were retrospectively evaluated. Each ICA was classified into one of three zones: areas medial (Zone 1), anterior (Zone 2), or lateral (Zone 3) to the atlantoaxial joint. For an ICA in Zone 1, the shortest distances to the posterior pharyngeal wall and midsagittal plane were measured. For an ICA in Zone 2, the closest distances to the midsagittal plane and anterior cortex of the C1-2 complex were measured. RESULTS: Fifteen ICAs in Zone 1 were found in 12 (5%) patients, with three female patients having bilateral ICAs medial to the atlantoaxial joint. The incidence of ICA in Zone 1 was higher in females than in males. In cases of ICAs in Zone 2, the ICAs were close to the anterior cortex of C1-2 complex, with the shortest distance being 2.6±1.5 mm. A total of 39.9% of patients had bilateral ICAs in Zone 3. CONCLUSION: Transoral surgeries in the upper cervical spine carry potential ICA injury risk. They should be carefully deliberated in patients whose ICAs are in Zone 1. In cases of ICAs in Zone 2, meticulous subperiosteal stripping and gentle traction should be performed on the posterior pharyngeal wall. Preoperative identification of the course of ICAs is mandatory in patients undergoing transoral surgeries in the upper cervical spine.

Pharyngeal Pumping Assay for Quantifying Feeding Behavior in Caenorhabditis elegans.

C. elegans is a well-established nematode model organism, with 83% of its genes conserved in humans with translation potential. C. elegans is translucent, with clearly defined cellular organization, and robustly identifiable under a microscope, being an excellent model for studying feeding behavior. Its neuromuscular pharyngeal pump undergoes a pumping motion that can be quantified to study feeding behavior at specific treatment conditions and in genetically modified worms. Understanding the evolutionarily conserved feeding behaviors and regulatory signals is vital, as unhealthy eating habits increase the risk of associated diseases. The current protocol was developed to identify and study evolutionary conserved signals regulating feeding behavior. The protocol described here is very robust in calculating the pumping rate (pumping per minute) as it directly counts the pharyngeal pumping for 30 s. This protocol uses basic laboratory instrumentation, such as a stereomicroscope with an attached camera and a computer with a video program that can be used to count manually. The advantages of studying C. elegans feeding include understanding the genetic basis of feeding regulation, dysregulation of feeding behavior in a disease model, the influence of toxic or environmental substances in feeding behavior, and modulation of feeding behavior by pharmacological agents. Key features • Quantifies pharyngeal pumping, which can be used to study up/downstream signaling in feeding regulation. • Uses a phenotype (pharyngeal pumping) that is easy to score. • Requires only a stereomicroscope with a camera to record the pharyngeal pumping, which can be counted manually.

Gα13 controls pharyngeal endoderm convergence by regulating E-cadherin expression and RhoA activation.

Pharyngeal endoderm cells undergo convergence and extension (C&E), which is essential for endoderm pouch formation and craniofacial development. Our previous work implicates Gα13/RhoA-mediated signaling in regulating this process, but the underlying mechanisms remain unclear. Here, we have used endoderm-specific transgenic and Gα13 mutant zebrafish to demonstrate that Gα13 plays a crucial role in pharyngeal endoderm C&E by regulating RhoA activation and E-cadherin expression. We showed that during C&E, endodermal cells gradually establish stable cell-cell contacts, acquire apical-basal polarity and undergo actomyosin-driven apical constriction, which are processes that require Gα13. Additionally, we found that Gα13-deficient embryos exhibit reduced E-cadherin expression, partially contributing to endoderm C&E defects. Notably, interfering with RhoA function disrupts spatial actomyosin activation without affecting E-cadherin expression. Collectively, our findings identify crucial cellular processes for pharyngeal endoderm C&E and reveal that Gα13 controls this through two independent pathways - modulating RhoA activation and regulating E-cadherin expression - thus unveiling intricate mechanisms governing pharyngeal endoderm morphogenesis.

Efficacy of Posterior Pharyngeal Wall Injection for Velopharyngeal Dysfunction in Adults.

OBJECTIVE(S): Posterior pharyngeal wall (PPW) injection is often employed to treat velopharyngeal deficiency (VPD). We sought to analyze the impact of PPW injection on severity of dysphagia and dysphonia. METHODS: Retrospective chart review was conducted of patients undergoing PPW injection from 2018 to 2023 at a tertiary laryngology center. Effects on Voice Handicap Index-10 (VHI-10), Eating Assessment Tool-10 (EAT-10), soft palate closure on modified barium swallow, and auditory-perceptual measures of hypernasality and audible nasal air emission were analyzed. RESULTS: 67 PPW injections were performed in 29 patients (11 female). Mean age was 59.4 ± 17.0 years. Etiologies were head and neck cancer (n = 23) and neurologic conditions (n = 6). 30 PPW injections were performed concurrent with intervention on the upper esophageal sphincter (25 dilations, 3 myotomies, 2 botulinum toxin injections), and 8 with a glottic procedure (6 vocal fold injections, 2 thyroplasties). Change scores were 3.87 (-6.85 to -0.89, p = .012) for VHI-10 and -3.00 (-4.75 to -1.25, p = 0.001) for EAT-10. These were statistically different from 0 for the whole cohort but not in the subset of patients undergoing concurrent voice and/or swallow surgery. Soft palate closure scores tended to be better (but not statistically significant) on MBS after PPW injection. Hypernasality and audible nasal air emission both improved after injection. CONCLUSION: PPW injection appears to have a therapeutic effect on dysphagia/dysphonia in patients with VPD; however, many patients have multifactorial impairment. Additional study is needed to determine benefit of PPW when performed in conjunction with other procedures in this complex patient population. LEVEL OF EVIDENCE: Level 4 (Case series) Laryngoscope, 2024.

Motor control of the palatoglossus and genioglossus during changes in breathing route.

High activity of upper airway dilator muscles is thought to be critical in preventing sleep-related upper airway collapse. To date, most of the research regarding upper airway dilator muscles has focused on the genioglossus muscle, which protrudes the tongue and opens the retroglossal airway. However, collapse commonly occurs in the retropalatal region. We therefore aimed to examine the motor control of the palatoglossus muscle as well as investigate breathing route-related changes in genioglossus and palatoglossus motor units. Single motor unit recordings of the genioglossus and palatoglossus were made simultaneously in healthy individuals during wakefulness while breathing through the nose with the mouth closed (NMC), nose with mouth open (NMO) or orally (OMO). The palatoglossus was found to have all 5 motor unit firing patterns that have been observed in other upper airway dilator muscles, but during nasal breathing had a higher proportion of tonically active but inspiratory modulated motor units as compared to the genioglossus (67% vs 30%). When still breathing nasally but with the mouth open, the units with an expiratory firing pattern in genioglossus, and all firing patterns in palatoglossal, increased their firing rates compared to nasal breathing with the mouth closed (GG: 17.8±4.9 vs 23.1±4.8 Hz, PG: 17.0±4.0 vs 19.3±4.0 Hz). Finally, oral breathing resulted in dramatic reductions in the number of palatoglossal motor units that were firing (35 units vs 92 during nasal breathing). Palatoglossus activity may contribute importantly to airway collapsibility and may provide an alternate pathway for preventing sleep-related airway collapse.

Signatures of convergence in Neotropical cichlid fish.

Convergent evolution of similar phenotypes suggests some predictability in the evolutionary trajectories of organisms, due to strong and repeated selective pressures, and/or developmental constraints. In adaptive radiations, particularly in cichlid fish radiations, convergent phenotypes are commonly found within and across geographical settings. Cichlids show major repeated axes of morphological diversification. Recurrent changes in body patterns reveal adaption to alternative habitats, and modifications of the trophic apparatus respond to the exploitation of different food resources. Here we compare morphologically and genetically two Neotropical cichlid assemblages, the Mexican desert cichlid and the Nicaraguan Midas cichlid, with similar polymorphic body and trophic adaptations despite their independent evolution. We found a common morphological axis of differentiation in trophic structures in both cichlid radiations, but two different axes of differentiation in body shape, defining two alternative limnetic body patterns. Adaptation to limnetic habitats implied regulation of immune functions in the Midas cichlid, while morphogenesis and metabolic functions in the desert cichlid. Convergent phenotypic adaptions could be associated to divergent gene regulation.

Obstructive sleep apnea and obesity: A review of epidemiology, pathophysiology and the effect of weight-loss treatments.

Despite the commonly-accepted paradigm that patients with obstructive sleep apnea (OSA) also invariably have obesity, OSA prevalence extends beyond obesity. This necessitates a reevaluation of screening strategies, biomarkers of increased OSA risk, and heightened awareness among healthcare providers about the array of OSA treatments for diverse adult populations. While obesity contributes importantly to OSA pathogenesis, there is substantial evidence that non-anatomical factors also play a crucial role, especially in patients who do not have obesity. In recent years, notwithstanding the recognition of diverse contributors to OSA pathogenesis, research has frequently focused on weight reduction to address OSA. Insights from past experiences with bariatric surgery in OSA serve as a lens to anticipate potential outcomes of emerging anti-obesity pharmacotherapies. Pharmacological alternatives, particularly incretin agonists, exhibit promise in weight reduction and OSA improvement, but encounter obstacles such as potential side effects and high costs. With this comprehensive narrative review, we delve into the complex epidemiological and pathophysiological connections between OSA and obesity. Additionally, we emphasize the importance of a multifaceted approach to OSA treatment, recognizing that while weight management is crucial, there is a need for comprehensive strategies that go beyond traditional weight-centric perspectives.

Discovery of novel 20S proteasome subunit ß5 PROTAC degraders as potential therapeutics for pharyngeal carcinoma and Bortezomib-resistant multiple myeloma.

Resistance to proteasome inhibitors like Bortezomib is a major challenge in the treatment of multiple myeloma (MM). Proteolysis targeting chimeras (PROTACs), an emerging therapeutic approach that induces selective degradation of target proteins, offer a promising solution to overcome drug resistance. In this study, we designed and synthesized novel small-molecule PROTACs that induce 20S proteasome subunit ß5 degradation as a strategy to overcome Bortezomib resistance. These 20S proteasome subunit ß5 PROTACs demonstrated considerable binding affinity to 20S proteasome subunit ß5 and cereblon (CRBN), effectively induced 20S proteasome subunit ß5 degradation, and exhibited potent antiproliferative activity against a panel of cancer cell lines. Notably, PROTACs 12f and 14 displayed robust antitumor effects against both the pharyngeal carcinoma cell line FaDu and the Bortezomib-resistant MM cell line KM3/BTZ in vitro and in vivo with excellent safety profiles. Taken together, our findings highlight the potential of PROTACs 12f and 14 as novel 20S proteasome subunit ß5-degrading agents for the treatment of pharyngeal carcinoma and overcoming Bortezomib resistance in MM.

Changes in the pharyngeal and nasal microbiota in pediatric patients with adenotonsillar hypertrophy.

The present study aimed to investigate the pharyngeal and nasal microbiota composition in children with adenotonsillar hypertrophy (AH) and assess longitudinal alterations in both microbiota after a probiotic oral spray treatment. A cohort of 57 AH patients were enrolled and randomly assigned to the probiotic and placebo groups for a 5-month treatment course. Pharyngeal and nasal swabs were collected before and after treatment and analyzed by 16S rRNA-based metataxonomics and axenic cultures for pathobiont identification. 16S rRNA sequences from pharyngeal and nasal swabs of 65 healthy children (HC) were used as microbiota reference profiles. We found that the pharyngeal and nasal microbiota of AH children were similar. When compared to HC, we observed an increase of the genera Rothia, Granulicatella, Streptococcus, Neisseria, and Haemophilus, as well as a reduction of Corynebacterium, Pseudomonas, Acinetobacter, and Moraxella in both microbiota of AH patients. After probiotic treatment, we confirmed the absence of adverse effects and a reduction of upper respiratory tract infections (URTI). Moreover, the composition of pharyngeal microbiota was positively influenced by the reduction of potential pathobionts, like Haemophilus spp., with an increase of beneficial microbial metabolic pathways. Finally, the probiotic reduced the abundance of the pathobionts Streptococcus mitis and Gemella haemolysans in relation to AH severity. In conclusion, our results highlight the alterations of the pharyngeal and nasal microbiota associated with AH. Moreover, probiotic administration conferred protection against URTI and reduced the presence of potential pathobionts in patients with AH. IMPORTANCE: Adenotonsillar hypertrophy (AH) is considered the main cause of breathing disorders during sleep in children. AH patients, after significant morbidity and often multiple courses of antibiotics, often proceed to tonsillectomy and/or adenoidectomy. Given the potential risks associated with these procedures, there is a growing interest in the use of nonsurgical adjuvant therapies, such as probiotics, that could potentially reduce their need for surgical intervention. In this study, we investigated the pharyngeal and nasal microbiota in patients with AH compared with healthy children. Furthermore, we tested the effects of probiotic spray administration on both disease symptoms and microbiota profiles, to evaluate the possible use of this microbial therapy as an adjuvant for AH patients.

Pharyngeal Airway Space Changes after Single Mandibular and Two-Jaw Surgery in Patients with Skeletal Class II Malocclusion.

Objective: To retrospectively evaluate the effects of single mandibular advancement (MA) and two-jaw surgery (2J-S) on the pharyngeal airway space (PAS) and hyoid position for the correction of skeletal Class II malocclusion. Methods: Eleven adult patients who underwent only MA surgery and twelve adult patients who underwent Le Fort I maxillary impaction-MA surgery (2-JS) were included in the retrospective study. A total of 46 cephalometric recordings obtained before (T1) and after treatment (T2) were examined. Craniofacial changes, area, and linear measurements of the pharyngeal airway and hyoid bone position were obtained in both groups. The Wilcoxon signed-rank test was used to evaluate time-dependent changes within groups. The Mann-Whitney U test was used to compare differences between groups. Results: Hyoid-Vert values increased significantly in both groups (MA, p<0.01; 2J-S, p<0.05); however, Hyoid-Hor values decreased significantly only in the 2J-S group (p<0.01). The anteroposterior dimensions of the airway increased in both groups, except for the PNS-P and PPS groups (p<0.01). Although a significant increase was observed in the nasopharyngeal area (A1) in the MA group (p<0.05), the decrease was found to be statistically significant in 2JG (p<0.01). Significant increases were found in the oropharyngeal (A2) and hypopharyngeal areas (A3) in both groups (p<0.01, p<0.05). Conclusion: Both surgical procedures for the correction of Class II malocclusion resulted in increased hypopharyngeal, oropharyngeal, and total airway measurements.

Fucoxanthin Inhibits the Proliferation and Metastasis of Human Pharyngeal Squamous Cell Carcinoma by Regulating the PI3K/Akt/mTOR Signaling Pathway.

Human pharyngeal squamous cell carcinoma (HPSCC) is the most common malignancy in the head and neck region, characterized by high mortality and a propensity for metastasis. Fucoxanthin, a carotenoid isolated from brown algae, exhibits pharmacological properties associated with the suppression of tumor proliferation and metastasis. Nevertheless, its potential to inhibit HPSCC proliferation and metastasis has not been fully elucidated. This study represents the first exploration of the inhibitory effects of fucoxanthin on two human pharyngeal squamous carcinoma cell lines (FaDu and Detroit 562), as well as the mechanisms underlying those effects. The results showed dose-dependent decreases in the proliferation, migration, and invasion of HPSCC cells after fucoxanthin treatment. Further studies indicated that fucoxanthin caused a significant reduction in the expression levels of proteins in the phosphoinositide 3-kinase (PI3K)/protein kinase B (AKT)/mechanistic target of rapamycin (mTOR) pathway, as well as the downstream proteins matrix metalloproteinase (MMP)-2 and MMP-9. Specific activators of PI3K/AKT reversed the effects of fucoxanthin on these proteins, as well as on cell proliferation and metastasis, in FaDu and Detroit 562 cells. Molecular docking assays confirmed that fucoxanthin strongly interacted with PI3K, AKT, mTOR, MMP-2, and MMP-9. Overall, fucoxanthin, a functional food component, is a potential therapeutic agent for HPSCC.

A 3-month-old male infant with Goldenhar syndrome: A clinical case report from Woldia, Northeast Ethiopia.

Goldenhar syndrome is a multifactorial congenital anomaly that involves structures that develop from the first and second pharyngeal arches. In this report, we present a clinical case of a 3-month-old male infant diagnosed with Goldenhar syndrome, born to a known retro-viral infected mother who was receiving antiretroviral therapy. The baby was brought to the hospital with complaints related to upper respiratory system. On examination, he had typical signs and symptoms of Goldenhar syndrome: an asymmetrical face with small left facial bones, a low-set ear, left anophthalmia, an atretic left ear with only small ear appendages, and a complete cleft lip and palate. His family had no history of birth defects or exposure to the known causes of birth defects. The baby was treated for severe community-acquired pneumonia, the diagnosis for his current presentation to our hospital, and he is now on multidisciplinary follow-up for possible medical and surgical management of the Goldenhar syndrome.

Survey of pharyngeal foreign bodies in Japan: An ecological study using the nationwide claims data.

OBJECTIVE: Pharyngeal foreign bodies (PFBs) are a prevalent disease affected by food culture and dietary habits, with fish bones as the leading cause. Most studies were limited to specific regions, and a nationwide survey was not conducted in Japan. In this ecological study, we aimed to conduct a nationwide analysis of outpatient PFB cases in Japan over three years, focusing on seasonal trends, sex- and age-stratified cases, and regional differences. METHODS: We used the National Database of Health Insurance Claims and Specific Health Checkups of Japan open data from April 2019 to March 2022. The case data were analyzed by month, age, sex, and prefecture. Additionally, we calculated the standardized claim ratios (SCRs) for each prefecture and investigated the association between dietary habits, food culture, and SCR of PFBs using a two-level linear regression model. RESULTS: We analyzed a total of 164,337 outpatient PFB cases in Japan, revealing an average incidence rate of 45.6 per 100,000 persons. The seasonal trend revealed a peak in July each year from 2019 to 2021, confirming seasonality in PFB incidents. Children reported a higher incidence rate. Living west of Japan and expenditure on fish and shellfish had a strongly positive association with the SCR of PFBs. CONCLUSION: Our nationwide survey reveals that, even within Japan, there were regional variations influenced by food culture and dietary habits. The data showed that PFB incidence was higher among children, highlighting the need for preventive education. LEVEL OF EVIDENCE: Level 3.