Partial Glossectomy Combined With Radiofrequency Ablation for Macroglossia in Beckwith-Wiedemann Syndrome.

OBJECTIVE: As the most common feature of Beckwith-Wiedemann syndrome (BWS), macroglossia may influence the quality of life, maxillofacial growth, and speech development of children. The retrospective study aimed to investigate the therapeutic effect of partial glossectomy combined with radiofrequency ablation (RFA) for macroglossia patients in BWS. METHODS: A retrospective study was conducted in BWS-derived macroglossia patients who underwent partial glossectomy combined with RFA from May 2019 to January 2021. In total, 35 patients consisting of 17 males and 18 females met the inclusion criteria and underwent surgery by the same plastic surgeon. Demographic characteristics, BWS features, operation details, preoperative and postoperative outcomes, satisfaction evaluations, and subgroup analysis were collected and assessed. RESULTS: Of the 35 patients involved, the average age at the time of surgery was 14.05±8.08 months, and the average surgery duration was 48.17±6.72 minutes. Only 1 patient suffered ventral tongue wound dehiscence, and the rest of the patients did not develop any other complications. The severity and frequency of tongue protrusion, drooling, snoring, and feeding difficulty were significantly ameliorated. The patient's parents showed satisfaction towards the overall surgery, tongue's appearance, and tongue's motor function. Tongue's height decreased from 32.09±1.16 mm before the operation to 29.29±1.33 mm after the operation. CONCLUSION: The partial glossectomy combined RFA exerts a safe, effective and viable technique to treat BWS-derived macroglossia.

Upper airway flow characteristics of childhood obstructive sleep apnea-hypopnea syndrome.

Revealing the structural morphology and inner flow field of the upper airway is important for understanding obstructive sleep apnea-hypopnea syndrome (OSAHS) incidence phenomena and pathological diagnosis in children. However, prior work on this topic has been focused on adults and the findings cannot be directly extrapolated to children because of different inducing factors. Therefore, this paper employs a simulation method to investigate upper airway flow characteristics of childhood OSAHS. It is found that the Reynold number changes highly throughout the whole upper airway, and the laminar assumption is no longer suitable for low Reynold number flow, which is much unlike classic fluid mechanics. Turbulent models of Standard k-ω and Spalart-Allmaras were developed prior to suggestion. The simulation is validated by experiments with an error of approximately 20%. Additionally, carried out in this analysis is the influence of adenoidal hypertrophy with different narrow levels. The cross-sectional area, flow velocity, pressure drop and volume rate will change greatly when the narrow level is above 64% of the upper airway, which can be a quantitative explanation for medical intervention if adenoid hypertrophy blocks 2/3 of the upper airway in the common clinical judgment of otorhinolaryngology. It is expected that this paper can be a meaningful instruction on OSAHS surgery plan making as well as recovery evaluation postoperatively.

[Clinical analysis of treatment and postoperative efficacy in neonatal congenital pyriform sinus fistula].

Objective:To discuss the diagnosis and treatment of congenital pyriform sinus fistula（CPSF） in newborn. Methods:Clinical data of 5 patients with CPSF innewborn were reviewed and the clinical symptoms, auxiliary examinations, surgical methods were analyzed after the operation, patients were followed up closely at different stages. Results:All the 5 neonates successfully completed the surgery without pharyngeal fistula, dysphagia, perifistula and distal fistula infection. Follow-up survey ranged from 3 months to 2 years and no one recurred. Conclusion:Neonatal CPSF is a rare disease with a short course of disease and rapid progression. In severe cases, it may threaten life and should be treated in time.

[Analysis of upper airway flow field between obstructive sleep apnea and normal children based on computational fluid dynamics].

Objective:In this study, the characteristics of the upper airway flow field were analyzed by using computational fluid dynamics（CFD）. The study analyze the differences in the upper airway flow field between normal children and children with obstructive sleep apnea（OSA）, and the pathological characteristics of children with OSA were elaborated from the perspective of airway fluid dynamics. Methods:The upper airway models of a normal child and a child with OSA were constructed. The differences in the same inspiration pressure, such as airflow velocity, airflow pattern, ventilation volume, and pressure, were analyzed. To verify CFD results, rhinomanometry was carried out and an experimental bench based 3D technology was also built. Results:The CFD results are consistent with the in vitro 3D model experiments and clinical measurement results. The adenoid area of nasopharynx is only 11.274 mm²of the child with OSA, about 1/6 of that of normal children. At the area of nasopharyngeal in OSA children, the flow velocity increased but the pressure dropped sharply, which was 69.197% of the total pressure drop, and the resistance value was 6.59 times of that of normal children. Streamline of nasopharyngeal is more disorder. Normal children's inspiratory flow was 116.139 mL/s, while OSA children's inspiratory flow was 47.055 mL/s, with a difference rate as high as 59.48%. Conclusion:The airflow of OSA children in nasopharynx is significantly different from that of normal children. The airflow characteristics of upper airway were discussed in detail with the use of CFD, which can help clinicians intuitively understand the abnormal flow behavior of children with OSA.

[Clinical efficacy of low-temperature radiofrequency ablaion assisted by endoscopy for the treatment of congenital pyriform sinus fistula in infants].

Objective:To discuss the clinical efficacy of low-temperature radiofrequency ablaion assisted by endoscopy for the treatment of congenital pyriform sinus fistula in infants. Method:Clinical data of 15 patients with CPSF received the low-temperature radiofrequency ablaion assisted by endoscopy under laryngoscope were reviewed. After the operation, patients were followed up closely at different stages. All patients underwent neck color doppler ultrasound and electronic laryngoscopy, and the results were analyzed. Result:The operation of 15 children was successfully completed without pharyngeal fistula, dysphagia, perifistula and distal fistula infection. Followup survey ranged from 3 months to 3 years and no one recurred. Conclusion:Low-temperature radiofrequency ablaion assisted by endoscopy is a promising method for the CPSF with less trauma, less haemorrhage and good vision. It is safe and effective for the treatment of CPSF in infants and children, and is worthy of clinical promotion.

Association Between Helicobacter pylori Infection and Otitis Media With Effusion Risk in Children: A Systematic Review and Meta-analysis.

OBJECTIVE: To investigate the potential correlation between Helicobacter pylori infection and otitis media with effusion (OME) risk in children. DATA SOURCES: Electronic databases were searched, including Cochrane Library, PubMed, Embase, China Biology Medicine disc, China Science and Technology Journal Database, Wanfang Database, and China National Knowledge Infrastructure Database. REVIEW METHODS: A systematic review and meta-analysis were conducted with Revman 5.3 software. Combined odds ratios (ORs) and 95% confidence intervals (CIs) were calculated to estimate infection-disease association. RESULTS: In total, 11 studies from 9 articles regarding H pylori infection and OME risk were enrolled in this meta-analysis. A significant association between H pylori infection and OME was detected for both adenoid samples from the case group (OR, 2.75; 95% CI, 1.43-5.30; P = .002) and middle ear fluid samples from the case group (OR, 4.45; 95% CI, 2.52-7.88; P < .00001). Subgroup analyses suggested a stronger correlation in African and Asian populations. CONCLUSION: This study indicated the correlation between H pylori infection and increased risk of OME in children, especially in African and Asian populations. Further well-designed studies regarding the white population are strongly recommended in the future.

A prospective observational study to investigate the correlation analysis between neonatal hyperbilirubinemia and deafness gene: Study protocol clinical trial (SPIRIT compliant).

INTRODUCTION: There are many studies on the relationship between serum levels of hyperbilirubinemia and hearing impairment. However, the mechanism of hyperbilirubinemia on auditory impairment is not clear. METHODS AND ANALYSIS: A total of 1000 children with hyperbilirubinemia who are mainly indirectly elevated bilirubin in the full-term neonatal ward of Xiamen Children's Hospital from March 2020 to September 2020 will be enrolled. Using second-generation high-throughput sequencing technology, 127 deaf-related genes were sequenced from the collected samples. At the same time, physical audiometry was performed on the selected persons and audiometry data were recorded. DISCUSSION: In this study, we will combine gene sequencing with clinical indications of hyperbilirubinemia to find the loci suitable for high-frequency pathogenic deafness related to hyperbilirubinemia, so as to provide early guidance for deafness gene screening in children with hyperbilirubinemia. TRIAL REGISTRATION: Chinese Clinical trial registry: ChiCTR2000030075.

Identification of causative variants in patients with non-syndromic hearing loss in the Minnan region, China by targeted next-generation sequencing.

BACKGROUND: Due to extreme genetic heterogeneity, targeted next-generation sequencing (NGS) can be an efficient tool for rapid genetic diagnosis of hereditary non-syndromic hearing loss (NSHL). AIMS/OBJECTIVES: This study was aiming to identify causative variants in NSHL patients from the Minnan region through targeted NGS. MATERIAL AND METHODS: Genomic DNA samples from 90 NSHL subjects were extracted and subjected to SureSelect target enrichment system to capture the entire coding exons and flanking intronic regions of gene GJB2, SLC26A4, and MT-RNR1. The captured DNA was then sequenced by Illumina HiSeq2500. The sequence data was processed and quality-checked using Burrows-Wheeler Alignment, Picard, and GATK, and annotated by SnpEff, SIFT, and GERP++. RESULTS: Twenty-six candidate variants in GJB2, SLC26A4, and MT-RNR1 were detected in 57 of 90 NSHL patients. GJB2 c.109G > A was the most frequent variant, followed by GJB2 c.608T > C and c.235delC. Genetic diagnosis was available for 22 NSHL patients, including 19 patients associated with autosomal recessive NSHL, one patients with autosomal dominant NSHL, and two individuals with mitochondrial disorders. CONCLUSIONS AND SIGNIFICANCE: Our targeted NGS analysis added supports for the application of NGS in routine diagnosis and provided an overview of genetic variants with allele frequencies in the deaf population from the Minnan region.

Ubiquitin E3 ligase TNFIAP3 mediates endosome/lysosome fusion in nasal epithelial cells.

The nasal epithelial barrier dysfunction is associated with the pathogenesis of nasal allergy; the causative factors are to be further elucidated. Ubiquitin E3 ligase TNFIAP3 (TNFIAP3, in short) plays a role in the maintenance of the homeostasis in the body. This study aims to elucidate the role of TNFIAP3 in the degradation of endocytic substances in nasal epithelial cells. The nasal epithelial cell line, RPMI 2650 cells (RPC), was cultured into monolayers in transwells. The endocytosis of staphylococcal enterotoxin B (SEB) by RPC monolayers was assessed by enzyme-linked immunoassay. The endocytosis of SEB-triggered endosome/lysosome fusion was observed by immunocytochemistry. The results showed that RPC monolayers expressed TNFIAP3 upon the endocytosis of SEB. Deficiency of TNFIAP3 resulted in abundant SEBs being transported to the basal chambers of transwells via the intracellular pathway. In the TNFIAP3-sufficient RPC, SEB-carrying endosomes fused with lysosomes were observed. The TNFIAP3-deficient RPC showed few SEB-carrying endosomes fused with lysosomes. In summary, TNFIAP3 plays an important role in tethering endosomes to lysosomes in RPC.

Free jejunal graft for reconstruction of defects in the hypopharynx and cervical esophagus following the cancer resections.

OBJECTIVES: The reconstruction of esophagus defects after hypopharyngeal and cervical esophageal carcinoma resection is an ongoing problem. The objective of this article was to investigate the techniques of the free jejunal graft for the reconstruction of hypopharyngeal and cervical esophagus and discuss the outcome related to the procedures. SUBJECTS AND METHODS: From July of 2005 to December 2007, seven patients with hypopharyngeal and cervical esophageal cancer underwent free jejunal graft reconstruction of the hypopharyngeal and cervical esophagus. Their clinical data were retrospectively analyzed. All patients received postoperative radiotherapy and were followed up for 7-24 months. RESULTS: Despite the multistep and time-consuming procedure, free jejunal graft survival was 100%. Operation-induced complications did not occur in six patients. One patient developed pharyngeal fistula. CONCLUSION: The present experience supports the use of free jejunal grafts in reconstruction of the hypopharyngeal and cervical esophagus defects after exenteration of the central compartment of the neck. A high successful rate with low incidence of complications in reconstruction of the hypopharyngeal and cervical esophagus was obtained in this study.

[To study the clinical valuation of different operation methods in treatment primary trigeminal neuralgia by retrosigmoidal approach].

OBJECTIVE: To study the clinical valuation of different operation methods in treatment primary trigeminal neuralgia by retrosigmoidal approach. METHOD: The clinical data of 165 cases with primary trigeminal neuralgia were studied, all head MRI(B-FFE) examination was made before surgery. According to patients' face sensation and cerebellopontine angle conditions, different operation methods were choose. RESULT: Among of 165 cases, 7 cases with face numbness and sensory loss symptom before surgery, were treated with partial rhizotomy of trigeminal sensory root and nerve combing and all cured, followed-up study 3.5 to 5.0 years and no relapse and face sequelae is same as before; 145 cases with blood vessel compression and no face sequelae were treated with microvascular decompression and nerve combing of trigeminal nerve sensory root, 144 cases were cured and 1 case relieve, 15 cases with herpes simplex were cured after 1-2 weeks, 4 cases with oral lips or tongue numbness were cured after 1-3 months. Followed-up study 3 to 5 years,3 cases relapse and after second same operation methods cured. Among of 13 cases without face sequelae, 5 cases with artery compression were not suit to microvascular decompression and 8 cases without blood vessel compression. All these cases were treated with surface coagulation and nerve combing of trigeminal nerve sensory root, 2 cases with herpes simplex were cured after one week. Followed-up studying of 3.2 to 5.0 years, one case relapse, All 13 cases were cured and no neuralgia relapse and no severe sequelae. Among the 165 cases, two cases died of hemorrhage cephalon in operation or postoperation. CONCLUSION: It is a safe and effective way to choose different methods combine to treat primary trigeminal neuralgia according to patients' face sensation and cerebellopontine angle conditions, but the risk of operation should not be ignored.

[Surgical treatment of trigeminal neuralgia with microvascular decompression sensory root of trigeminal nerve combing].

OBJECTIVE: To evaluate the short-term and long-term curative effect of microvascular decompression sensory root of trigeminal nerve combing (MVD + SRTNC) by post-sigmoid approach as well as the complications in these operations. METHODS: The data from 138 idiopathic trigeminal neuralgia (TN) patients treated with MVD + STRNC that followed up 33 months to 58 months was analyzed. RESULTS: Immediate relief from pain occurred in 136 patients (98.6%). On Kaplan-Meier analysis, cumulative proportion effectiveness at the end of 25 months decreased to 91.4%, and 85.8% from 49 months to 58 months. There were cases of 63.83% and 19.15% with slight and midrange hypoesthesia after surgery but majority recovered at the end of 3-58 months (P < 0.01). No serious hypoesthesia occurred. The complications including leakage of cerebrospinal fluid, herpes and headache caused by high intracranial pressure, were observed in 4 cases (2.90%), 36 cases (26.09%) and 3 cases (2.17%), respectively. Two cases (1.43%) died of cerebral hemorrhage. CONCLUSIONS: The proposed surgical strategy of standard MVD plus sensory root of trigeminal nerve combing was a good strategy option for TN, but It was important for surgeons to think highly of the risks of the surgery.