Hyaline Fibromatosis Syndrome: Early Outcomes Following Major Craniofacial Mass Excision.

Hyaline fibromatosis syndrome (HFS) is a rare congenital disorder characterized by abnormal hyaline deposition within soft tissues. Hyaline fibromatosis syndrome manifests in 2 distinct forms: (1) infantile systemic hyalinosis and (2) juvenile hyaline fibromatosis. Infantile systemic hyalinosis, the more severe form, typically emerges in early childhood with extensive systemic involvement. In contrast, juvenile HFS is less severe, allowing patients to survive into adulthood. Common clinical manifestations include thickened skin, hyperpigmented patches, gingival hypertrophy, skin nodules, and progressive severe joint contractures, leading to significant morbidity and potential mortality. This case report describes a 7-year-old child who was diagnosed with HFS and presented with a very large, ulcerated, rapidly expanding craniofacial mass. The patient underwent successful treatment involving a multidisciplinary medical team and strategic surgical intervention, achieving favorable postoperative outcomes.

Prognostic Risks for Tracheostomy in Pierre Robin Sequence: A Cohort From a Tertiary Hospital in Thailand.

BACKGROUND: Pierre Robin sequence (PRS), characterized by micrognathia, glossoptosis, cleft palate, and obstructed airways, is one of the craniofacial conditions challenging various approaches to managing compromised airways, ranging from conservative measures to invasive airway surgery. This study was conducted to identify predictive risk factors for tracheostomy in the PRS. METHODS: A retrospective chart review was conducted at a tertiary referral hospital in Thailand. Children diagnosed with PRS from January 2010 to December 2021 were enrolled. Univariate and multivariate analysis methods were used to identify the risk factors. RESULTS: Sixty-five patients with PRS were identified in electronic medical records, but 6 were excluded. Thirteen of the remaining 59 patients underwent tracheostomy. There were no significant differences in sex, preterm gestational age, cleft palate, associated syndromes, chromosome abnormalities, or cardiac or neurological involvement between patients who received tracheostomy and those who did not. However, patients with oropharyngeal dysfunction who received tracheostomy tended to use a nasogastric tube or percutaneous gastrostomy 92.3% of the time ( P = 0.043). In addition, patients with abnormal airways from endoscopy were more likely to undergo tracheostomy (odds ratio, 2.17; 95% confidence interval [CI], 1.20-3.90). Interestingly, patients with a sum of Apgar scores at 1 and 5 minutes <15 were more likely to undergo tracheostomy (adjusted odds ratio, 9.91; 95% CI, 1.32-74.52). Furthermore, patients with at least 3 identified comorbidities were more likely to undergo tracheostomy (adjusted odds ratio, 11.34; 95% CI, 1.16-111.15). CONCLUSIONS: The need for tracheostomy depends on comorbidities, Apgar scores, and abnormal airway anatomy. Feeding methods can become more complex with tracheostomy. Prognostic risk factors can help guide treatment and counseling for health care workers and caregivers.

Efficacy of Pneumococcal Vaccine on Otitis Media: A Systematic Review and Meta-Analysis.

OBJECTIVE: To assess the effect of the pneumococcal vaccine (PCV) toward the surgical management and complications of otitis media. DATA SOURCES: MEDLINE, EMBASE, PubMed, Scopus, and clinicaltrial.gov. REVIEW METHODS: A systematic search was performed using a combination of keywords and standardized terms about PCV and surgical management or complications of otitis media. Following Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines, studies were screened by 3 independent reviewers. Risk of bias assessment, followed by meta-analysis in only randomized-controlled trials was conducted. Vaccine efficacy (VE) and 95% confidence interval (CI) were reported. RESULTS: Of the 2649 abstracts reviewed, 27 studies were included in the qualitative analysis and were categorized into 6 outcomes: tympanostomy tube insertion, otitis media with effusion (OME), mastoiditis, spontaneous tympanic membrane (TM) perforation, recurrent acute otitis media (AOM), and severe AOM. Fifteen studies were included in the meta-analysis to evaluate the rate of tympanostomy tube insertion, OME, and recurrent AOM. PCV was significantly more effective in lowering the rate of tympanostomy tube insertion (VE, 22.2%; 95% CI, 14.6-29.8) and recurrent AOM (VE, 10.06%; 95% CI, 7.46-12.65) when compared with the control group, with no significant difference in reducing the incidence of OME. The qualitative analysis revealed that PCV had efficacy in preventing severe AOM and spontaneous TM perforation but the effect on mastoiditis remained unclear. CONCLUSION: The PCV was effective in reducing the rate of tympanostomy tube insertion and the incidence of recurrent AOM with a nonsignificant effect in preventing OME in children.

Pediatric respiratory tract foreign bodies in children: A systematic review.

OBJECTIVE: Pediatric respiratory emergencies of airway foreign body (FB) are a common cause of visits to the emergency department (ED) and respiratory failure is a major cause of cardiopulmonary arrest. The purpose of this study is to evaluate the literature and update our current understanding of pediatric respiratory tract FBs in children by clearly considering the aspect of the complications and related factors. METHODS: A systematic search of PubMed and Embase yielded a total of 2035 studies related to the respiratory tract FB in children. After screening the abstracts, 118 articles were included for analysis. However, 56 articles were excluded due to the published data more than 10 years. Meanwhile, 6 articles were duplicated and 3 articles were the secondary data. Thus, 53 full text articles were assessed for eligibility. Then, 46 full text articles were excluded due to irrelevant contents. Finally, there were 7 qualitative articles in this systematic review. RESULTS: Most children with FBs in the aerodigestive tract are 1-3 years of age. Most FBs are organic, especially seeds. The most commonly obstructed airway is the right primary bronchus. The most common and severe complications are pneumonia, pulmonary atelectasis, lung consolidation, pneumothorax, bronchiectasis, and death. The main device for the removal of FBs from the airways is a rigid bronchoscope. Duration of diagnosis is major factors that related with severe complication. CONCLUSION: FBs obstructive conditions in respiratory tract of children are serious and life-threatening conditions. The likelihood of death depends on the location of the obstruction, the nature of FB, time to removal, and initial resuscitation. Moreover, even after a FB has been removed, complications can lead to death. Educating parents and immediate treatment is very important. Rapid diagnosis is important factor to prevent complication.

Prevalence of high-risk for obstructive sleep apnea in attention deficit hyperactivity disorder children referred to psychiatry clinic and impact on quality of life.

Objectives: To study the prevalence of high-risk obstructive sleep apnea (OSA) in attention deficit hyperactivity disorder (ADHD) children in a child and adolescent psychiatry clinic using the Thai version of the Pediatric Obstructive Sleep Apnea Screening Tool (POSAST) questionnaire. The secondary objective was to evaluate the quality of life and identify associated factors for high-risk OSA in ADHD children. Study design: Prospective cross-sectional study. Material and method: Caregivers of pediatric patients aged 5-18 years old and diagnosed with ADHD by child and adolescent psychiatrists were surveyed about their child's sleeping habits. Results: Two hundred and seventy-four subjects were included. The patients' mean age was 10.4 ± 2.6 years, and 82.8% were males. There were 30 children (10.9%) diagnosed with obesity, 46 (16.8%) with chronic rhinitis, and 9 (3.3%) with asthma. The median duration of ADHD symptoms was 22.1 months. The prevalence of high-risk OSA was 18.2% and was associated with significantly reduced quality of life (adjusted OR = 4.46, 95% CI: 2.26-8.81, P < 0.001). A significant association between high-risk OSA and obesity also emerged (adjusted OR = 2.84, 95% CI: 1.17-6.88, P = 0.021). Conclusion: An elevated prevalence of high-risk OSA is present among Thai children with ADHD, and significantly impacts quality of life. A significant association between high-risk OSA and obesity is also detected in patients with ADHD. Therefore, screening for high-risk OSA in ADHD patients may likely facilitate early detection and treatment of OSA, and potentially prevent adverse consequences.

Adenoid measurement accuracy: A comparison of lateral skull film, flexible endoscopy, and intraoperative rigid endoscopy (gold standard).

OBJECTIVE: To compare the accuracy of lateral skull film, flexible endoscopy, and intraoperative rigid endoscopy in assessment of adenoid size. METHODS: A cross-sectional study was conducted in 43 pediatric patients undergoing ENT surgery from July 2017 to December 2018. All patients underwent preoperative lateral skull film, flexible endoscopy, and intraoperative rigid endoscopy. RESULTS: The average adenoidal-nasopharyngeal (A/N) ratio obtained from lateral skull film, flexible endoscopy, and intraoperative rigid endoscopy was 72.9, 79.5, and 81.6, respectively. There was a moderate correlation between A/N ratio from lateral skull film and intraoperative rigid endoscopy (Pearson's correlation: 0.567, p˂0.001). Whereas, the A/N ratio from flexible endoscopy compared to intraoperative rigid endoscopy showed a stronger correlation (Pearson's correlation: 0.791, p˂0.001). From linear regression analysis, the intraoperative adenoid measurement was estimated from the results of flexible endoscopy (intraoperative rigid endoscopy: 0.72 [flexible endoscopy] +24.47) and lateral skull film (intraoperative rigid endoscopy = 0.65 [lateral skull film] + 34) CONCLUSION: Flexible endoscopy yields the most accuracy in the assessment of adenoid size and nasopharynx visualization, without radiation exposure or anesthesia. Despite less accuracy, lateral skull film is more availability in every hospital. The correlation of adenoid size measurement in this study can also be applied for the actual size of adenoid.

Validity and reliability of the Thai version of the pediatric obstructive sleep apnea screening tool.

OBJECTIVE: Obstructive sleep apnea (OSA) is highly prevalent in children and requires an expensive and relatively unavailable sleep study for diagnosis. This study was undertaken to translate the previously validated pediatric OSA screening tool (POSAST) to the Thai language and assess its accuracy and test-retest reliability in at-risk symptomatic children. STUDY DESIGN: Prospective cross-sectional cohort study. METHODS: Pediatric patients clinically referred for suspected OSA who underwent overnight polysomnography (PSG) were recruited, and caregivers completed the Thai version of the POSAST. The same questionnaire was completed again after 2-4 weeks. The reliability of the questionnaire was determined by internal consistency and test-retest reliability. The validity of the questionnaire was assessed by constructing receiver operating characteristic (ROC) curves to identify the equation-derived score and total additive score cutoff points that identify high risk for moderate and severe OSA (AHI of ≥ 5 events/h). RESULTS: One hundred and ten subjects completed the study. The mean age was 8.4 ± 2.9 years. The mean apnea-hypopnea index (AHI) was 10.9 ± 11.9 events/h. Test-retest reliability (Pearson correlation coefficient = 0.96, p < .001) and internal consistency between each question (Cronbach's alpha coefficient = 0.82, p < .001) were excellent. An equation-derived score cut-off of 1.9 yielded 78.4% sensitivity, 50.0% specificity, 76.3% positive predictive value (PPV), and 52.9% negative predictive value (NPV), while a total additive score cut-off of 8 corresponded to 81.1% sensitivity, 52.8% specificity, 77.9% PPV, and 57.6% NPV for diagnosing moderate and severe OSA (AHI ≥ 5 events/h). CONCLUSION: The internal consistency and reproducibility of the Thai version of the POSAST are satisfactory, display acceptable validity, and the instrument can be used for screening symptomatic Thai children for OSA.

Association between adenoid bacteriology and clinical characteristics of adenoid-related diseases in children.

INTRODUCTION: The aim of this research is to find the association between the pathogenic bacteria obtained from the adenoid culture and clinical characteristics of adenoid-related diseases in children. METHODS: In this retrospective study, we reviewed the medical records of children who had adenoidectomy for adenoid-related diseases. Demographic data, diagnoses, indications for adenoidectomy and bacterial culture results were collected. The adenoid size was measured in the lateral skull X-ray as adenoid-nasopharyngeal ratio. Associations between the culture results and the demographic data, adenoid size, and the diagnoses were analyzed. RESULTS: There were 407 children who had adenoidectomy for obstructive sleep-disordered breathing (75.2%), otitis media with effusion (19.2%), and chronic sinusitis (5.6%). Median age was 5.9 years. Common pathogenic bacteria in the adenoid were Haemophilus influenzae (26.2%), Staphylococcus aureus (23.5%), Streptococcus pneumoniae (18.2%), and Moraxella catarrhalis (12%). The patient's age had significant association with the prevalence of pathogenic bacteria. S. pneumoniae was most prevalent in young children up to 7 years. S. aureus was more common in children over 7 years. H. influenzae had similar prevalence in all age groups. Size of the adenoid and type of adenoid-related diseases had no association with the outcome of bacterial culture. CONCLUSION: Age of the patients was the significant factor associated with the bacteriological findings of the adenoid while size and types of adenoid-related diseases were not associated with the outcome of bacterial culture.

The effect of steam inhalation on nasal obstruction in patients with allergic rhinitis.

BACKGROUND: Nasal obstruction is often impact on quality of life in allergic rhinitis (AR). The steam inhalation is one of widely used home remedies to soothe and open the nasal passages. Furthermore, steam inhalation may provide change in objective nasal airway assessment. OBJECTIVE: To compare the effect of steam inhalation on nasal obstruction between patients with AR and normal individuals, as well as the change in the cross-sectional area of the nasal cavity and in nasal airway resistance (NAR) between 2 groups. METHODS: A prospective comparative, parallel group study was conducted in AR and normal individuals. Steam with the temperature of 42-44°C was inhaled. Variables obtained before and after steam inhalation were compared. RESULTS: After steam inhalation, nasal symptom score, combined global symptoms, and Visual Analog scale (VAS) of combined global symptoms of AR patients showed statistically significant improvement. Whereas, normal individuals, there was statistically significant improvement only in sneezing and combined global symptoms. Meanwhile, the change of each measurement score, combined global symptoms, and VAS of the symptoms in those with AR were significantly higher than those of normal individuals. Total nasal airflow, NAR, volume, and mean minimal cross-sectional area of AR patients tended to better improve after steam inhalation. CONCLUSIONS: The steam inhalation significantly improved nasal obstruction in AR patients, but no statistical significant difference between both groups for any parameters. The different response between the 2 groups may be due to different nasal mucosa sensitivity to stimuli.

Watch Peripheral Arterial Tonometry in the Diagnosis of Pediatric Obstructive Sleep Apnea.

Objective To assess the accuracy and clinical reliability of watch peripheral arterial tonometry (PAT) compared with polysomnography (PSG) for the diagnosis of pediatric obstructive sleep apnea (OSA). Study Design Prospective, diagnostic test study. Setting National tertiary referral hospital. Subjects and Methods Patients aged 8 to 15 years with clinically suspected OSA were recruited. All participants underwent PSG and PAT simultaneously in the sleep laboratory. Results Thirty-six patients were included, with a mean age of 10.2 ± 1.8 years. Median (interquartile range) of the apnea hypopnea index (AHI) was 8.0 (5.5-12) and 2.9 (0.5-7.5) events/h, median oxygen desaturation index (ODI) was 2.5 (1.4-8.3) and 1.3 (0.2-3.8) events/h, mean ± standard deviation total sleep time was 398.4 ± 38.3 and 401.9 ± 36.1 minutes, and mean minimum oxygen saturation was 87.1% ± 8.1% and 89.4% ± 7.1% for PSG and PAT sleep parameter results, respectively. Agreement between methods was excellent for the AHI (intraclass correlation coefficient [ICC]: 0.89; 95% CI, 0.40-0.96; P < .001) and ODI (ICC: 0.87; 95% CI, 0.69-0.94; P < .001). Correlation between methods was very good for the ODI ( r = 0.83; 95% CI, 0.67-0.90; P < .001) and moderate for the AHI ( r = 0.64; 95% CI, 0.30-0.85; P < .001). From the receiver operating characteristic curve constructed to assess PAT diagnostic capability, AHI of PAT (W-AHI) at a cutoff of 3.5 events/h provided the highest accuracy (76.9% sensitivity, 78.3% specificity), while W-AHI at 10 events/h yielded 91.3% specificity for diagnosing severe OSA. Conclusion PAT correlated well and had good agreement with PSG. Children with W-AHI ≥10 had high specificity for the diagnosis of severe OSA. Larger studies with PAT designed for children across all age ranges and with a normal control group are still needed.

Surveillance of Otitis Media With Effusion in Thai Children With Cleft Palate: Cumulative Incidence and Outcome of the Management.

OBJECTIVE: To study the incidence and outcome of management of otitis media with effusion in Thai children with cleft palate. DESIGN: Retrospective cohort study in the tertiary care center. PATIENTS: Ninety-five children with cleft palate were referred for ear evaluation, from June 1997 to January 2015. Fifteen children (15.8%) had associated craniofacial syndromic anomalies. MAIN OUTCOME MEASURES: Cumulative incidence of otitis media with effusion, rate of ventilation tube insertion, duration of indwelling tubes, hearing outcome, and complications of ventilation tubes. RESULTS: Ear examinations were done every 8 to 12 weeks throughout the study. Cumulative incidence of otitis media with effusion was 53.7% in children within 12 months of age and 81.1% within 24 months of age. At the end of the study, all of the patients had at least 1 episode of otitis media with effusion. Eighty-eight children (92.6%) had palatoplasty, and there was no significant difference in the incidence of otitis media before and after palatoplasty. The mean hearing level at recruitment was 40.8 ±18.4 dB. Ventilation tube insertion was done in 76 patients (80%). The median time for indwelling tubes was 11.7 months. Rate of ventilation tube insertion was 0.5/year. The mean hearing level at last follow-up was 23.5 ± 14 dB. Otorrhea through tube was found in 24 cases (31.6%). CONCLUSION: Otitis media with effusion was common in Thai children with cleft palate. Surveillance of middle ear effusion and ventilation tube insertion contributed to a favorable hearing outcome.