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Feeding abnormalities, swallowing dysfunction, and gastrointestinal issues cause poor weight gain, oral motor dysfunction, and air swallowing in children with Rett syndrome (RTT). Pneumonia is the leading cause of death. Our study describes fiberoptic endoscopic swallowing findings in 11 female RTT children. Each patient was evaluated using the 8-point Penetration/Aspiration Scale (PAS). The average age was 7 years. All patients had tongue dyskinesis and prolonged oral stage. Eight girls exhibited liquid entering the airway without coughing, whereas 6 did well with pureed meal. Three girls had pneumonia. Age was not correlated with pneumonia episodes (P =.18). Pureed material was related with pneumonia (P =.006), whereas liquids were not. Pureed PAS was positively correlated with Liquid PAS (P =.008) and age (P =.004). All aspiration/penetration incidents occurred before the pharyngeal phase. No patient under 7 years experienced pneumonia episodes. Silent aspiration can occur early in infancy, although pneumonia episodes can occur later. [ABSTRACT FROM AUTHOR]

Background Spontaneous esophageal perforation traditionally mandates urgent surgical treatment. Lately, esophageal stents have been used to reduce the associated morbidity and mortality. The current systematic review aimed to assess the efficacy of stents as a primary treatment option in this scenario. Methods A systematic search was conducted in PubMed/MEDLINE, Scopus and the Cochrane Library for studies published in the English language between 2000 and 2023. We included observational studies reporting on the use of stents, alongside conservative measures and drainage procedures, in patients with spontaneous esophageal perforations. Primary outcomes were sealing rate (persistent leak occlusion) and failure rate (mortality or conversion to a major surgical operation). Secondary outcomes included patients' presentation, sepsis, drainage procedures, and reinterventions. Results for primary outcomes were presented as pooled rates with 95% confidence intervals (CIs), using a random-effects model. Methodological quality was assessed using the MINORS score. Results Eighteen studies involving 171 patients were included. Sealing rate was 86% (95%CI 77-93%) and failure rate was 14% (95%CI 7-22%). Weighted mortality rate was 6% (95%CI 2-13%), while conversion to surgical treatment was 2% (95%CI 0-9%). Late presentation was not related to a statistically significant increase in treatment failure (odds ratio 1.85, 95%CI 0.37-9.30; P=0.72). Drainage procedures were required for the majority of patients, with a high rate of surgical and endoscopic reinterventions. Conclusions Our results imply that stents may offer an effective and safe alternative treatment for patients with spontaneous esophageal perforations. Additional endoscopic and surgical drainage procedures are frequently needed. [ABSTRACT FROM AUTHOR]

Introduction: Proteus syndrome (PS) is a rare genetic disorder usually caused by mutations in AKT1 or PTEN genes, characterized by multiple, asymmetric tissue overgrowth with high clinical variability. Sinonasal neuroendocrine carcinomas (SNEC) are exceptionally rare tumors encountered in the ethmoid sinus, nasal cavity, or maxillary sinus. Case Report: We report a 35-year-old patient with PS, who underwent successful surgical removal of a welldifferentiated SNEC obstructing his nasal cavity and highlight the role of the otolaryngologist for safe airway management, minimal surgical intervention and coordination of the multidisciplinary care. Histologically, focally hyperplastic mucosal epithelium of respiratory type of the nasal chamber was noticed along with seromucinous glands and capillary congestion of the subepithelial fibrovascular tissue. The limited presence of neoplastic tissue with histomorphological and immunophenotypic features of a neuroendocrine neoplasm was focally observed. Tumor cells grow in the form of islets within a vascular stroma; these neoplastic cells are immunohistochemically positive for synaptophysin, CD56, EMA, Ki67 (low expression, cell proliferation rate: 2%), CD31, chromogranin and pancytokeratin AE1 / AE3 as well as for S-100 protein (weak intensity). Conclusions: This first description of a SNEC in a PS patient, might hint towards a common basis between the two conditions, due to the mosaic AKT1 variant and an activated AKT/PIK3CA/PTEN pathway. [ABSTRACT FROM AUTHOR]

Objectives: To evaluate clinical and microbiological findings that are correlated with abscess formation in adult acute epiglottitis (AE). Methods: We reviewed 140 cases of adult AE. Demographic, clinical, imaging, and microbiological findings are analyzed for all patients with AE in comparison to those with epiglottic abscess (EA). Results: A total of 113 patients presented with AE and 27 presented or progressed to EA (19.3%). Age, sex, seasonality, smoking, body mass index (BMI), and comorbidities were statistically insignificant between the 2 groups. Muffled voice (P <.013), respiratory distress (P <.001), and pre-existence of epiglottic cyst (P <.001) are symptoms and signs connected with abscess formation. A total of 120 patients were treated conservatively. Surgical treatment was performed on 20 patients with EA. About 72 out of 80 cultures revealed monomicrobial infection. Mixed flora was isolated in 8 patients with EA. Streptococcus was isolated in 51 out of 80 positive cultures (64%). Haemophilus Influenza (Hib) was not isolated in any sample. EA and mixed flora relates to a higher rate of airway intervention (P <.001). Conclusion: A high level of suspicion for abscess formation is required if clinical examination reveals dyspnea, muffled voice, or an epiglottic cyst in adult with AE. The existence of EA doubles the duration of hospitalization. EA is typically found on the lingual surface of the epiglottis. Supraglottic or deep neck space expansion should be treated surgically. EA is associated with a mixed flora and a higher rate of airway obstruction. Streptococcus is discovered to be the most common pathogen. [ABSTRACT FROM AUTHOR]

BACKGROUND: Τo investigate the differences in regard to the clinical, laboratory, and imaging findings as well as the treatment course between diabetic and non-diabetic, non-immunocompromised patients with malignant otitis externa. METHODS: A total of 36 hospitalized patients diagnosed with malignant otitis externa between January 2011 and December 2020 were divided into 2 groups according to their medical history, blood glucose, and glycated hemoglobin levels. RESULTS: Thirty-two patients were diabetic (group A) and 4 were non-diabetic, non-immunocompromised (group B). Otalgia was present in all patients (100%), followed by otorrhoea (67%) and edema (64%). Polyps were present in 18 patients (50%). Pseudomonas aeruginosa was isolated in 16 out of 25 positive cultures (64%). Four patients of group A and none of group B underwent surgery. Five patients of group A and none of group B had at least 1 cranial nerve involvement. The mean age was 77.22 ± 8.17 for group A and 47.25 ± 3.59 for group B (P < .001). No statistical significance was observed in regards to major symptoms, inflammatory markers (white blood cell, C-reactive protein, and erythrocyte sedimentation rate), positive imaging, and microbiological findings between the 2 groups. The average days of hospitalization were 42.41 ± 31.06 for group A and 10.25 ± 2.63 for group B (P < .049). Four diabetic patients died. CONCLUSION: Non-diabetic, non-immunocompromised adult patients with malignant otitis externa had a better response to antibiotic therapy and a shorter length of hospitalization. A high clinical suspicion for malignant otitis externa should always raise in cases of otitis externa that fail to respond in a topic and/or oral antibiotic treatment for more than a week. [ABSTRACT FROM AUTHOR]

Screen exposure has both negative and positive effects on the level of language skills a child acquires. The purpose of this review is to address current literature on the possible relationship between unsupervised screen exposure and language development in children and to provide recommendations to caregivers regarding screen exposure of children, taking into consideration the possible effects. A scoping review was conducted using the PubMed/MEDLINE (Medical Literature Analysis and Retrieval System Online) database. A total of 590 articles were retrieved and considered for inclusion. Twenty-one articles were finally included and reviewed with an emphasis on language, communication, and executive skills as well as cognitive development. The negative effects of screen exposure for children outweigh the positive effects. The largest number of studies demonstrate that unsupervised screen exposure may negatively impact a child's language usage and cognitive and executive skills, disrupt playtime, and affect the quality of sleep. On the other hand, supervised screen use is associated with improved language skills. More evidence is needed on unsupervised exposure in children to new types of screens. As technology could play a significant role in schools in the future, additional research is required to create educational media for schoolchildren with specific guidelines., Competing Interests: The authors have declared that no competing interests exist., (Copyright © 2024, Korres et al.)