Characteristics and outcomes of interventions for pediatric laryngomalacia: A systematic review with meta-analysis.

OBJECTIVES: To analyze characteristics of children treated for laryngomalacia to determine predictive factors and provide an updated meta-analysis on outcomes. METHODS: A systematic review was conducted according to PRISMA guidelines from inception to May 2, 2023, using CINAHL, PubMed, and Scopus databases. Study screening, data extraction, quality rating, and risk of bias assessment were performed by 2 independent reviewers. Data were meta-analyzed using fixed-/random-effects model to derive continuous measures (mean), proportions (%), and mean difference (Δ) with 95% confidence interval (CI). RESULTS: 100 articles were identified with information on outcomes of pediatric patients with laryngomalacia (N = 18,317). The mean age was 10.6 months (range: 0 to 252, 95%CI: 9.6 to 11.6, p = 0.00) with a 1.4:1 male to female ratio. Many patients presented with stridor (87.9%, 95% CI: 69.8 to 98.4), and the most common comorbidity at time of diagnosis was gastroesophageal reflux disease (48.8%, 95%CI: 40.9 to 56.8). Based on the patient population included in our analysis, 86.1% received supraglottoplasty (95% CI: 78.7 to 92.1). A total of 73.6% (95% CI: 65.5 to 81.0) had reported complete resolution of symptoms. For patients with a concurrent diagnosis of sleep disordered breathing receiving supraglottoplasty, the apnea-hypopnea index improved with a mean difference of -10.0 (95%CI: 15.6 to -4.5) events per hour post-treatment. CONCLUSIONS: Laryngomalacia continues to be a common problem in the pediatric population. Supraglottoplasty remains an effective treatment option leading to symptomatic improvement in many cases. For those with concurrent sleep disordered breathing, supraglottoplasty lowers the apnea-hypopnea index.

Adult Laryngomalacia: A Scoping Review.

OBJECTIVE: This review sought to determine the characteristics of adults diagnosed with new onset laryngomalacia including airway symptoms, laryngoscopic findings, treatments, and outcomes. Moreover, we wanted to highlight suspected limitations in the literature. DATA SOURCES: Studies were identified through CINAHL, Cochrane Review, PubMed, and Scopus published between 1966 and 2023. REVIEW METHODS: The search was performed in accordance with the Preferred Reporting Items for Systematic Reviews and Meta-analysis Extension for Scoping Reviews checklist by 2 independent investigators. A meta-analysis of proportions and continuous measures was conducted. RESULTS: Of the 1121 abstracts identified, 33 articles pertaining to laryngomalacia in the adult population were included. The most common presenting symptoms were stridor at rest (78.3%, 65.1-88.3) and dyspnea with exertion (83.8%, 64.8-96.3). The most suspected etiology was exercise-induced (86.0%, 69.4-95.5), and the most common description of laryngomalacia on visualization was collapse of supraglottic structures during exercise (93.3%, 79.0-99.1). Nonsurgical options were attempted in 87.0% (54.0-99.1), which included oral appliances, respiratory retraining, breathing techniques, and working with a speech pathologist. Surgical options were ultimately performed in 84.2% (75.0-91.0). Complete resolution of symptoms following therapy was seen in 61.9% (48.0-74.6). CONCLUSION: Adult onset laryngomalacia is difficult to characterize. It typically presents in patients during exercise, with neurological injury, or idiopathically. Surgical management can lead to improvement or complete resolution of symptoms. The need for a universal nomenclature is highlighted in this review, as it is inconsistently classified.

Evaluating the Need for Intensive Care Admission After Supraglottoplasty for Severe Laryngomalacia.

OBJECTIVE: Postoperative airway concerns persist despite a low rate of post-supraglottoplasty complications for children with laryngomalacia. The objective of this study is to determine the factors associated with the need for intensive care unit (ICU) admission following supraglottoplasty. METHODS: A 7-year retrospective cohort analysis was conducted between 2014 and 2021. A patient requiring ICU level of care was defined as the use of respiratory support such as intubation, positive pressure ventilation, high-flow nasal cannula, or multiple doses of nebulized epinephrine. RESULTS: About 134 medical charts were reviewed; 12 patients were excluded because of concurrent surgery. Age at the time of surgery was 2.8 (4.3) months (median [interquartile range]). About 33 (27.0%) ultimately required ICU-level care. Prematurity (odds ratio [OR] 13.8), neurological condition (OR ∞), American Society of Anesthesiology class 3-4 (OR 6.5), and younger age (OR 1.8) were more likely to require ICU admission. No patient above 10 months of age needed ICU monitoring. The use of respiratory support justifying ICU was known within the first 4 h after surgery for almost all (32/33, 97%) of these patients. 4/33 (12.1%) were kept intubated and the remaining needed non-invasive ventilation. Only one patient (1/122, 0.8%) was reintubated 12 h after surgery for progressive respiratory distress. CONCLUSION: Approximately a quarter of patients required ICU-level care after supraglottoplasty. For nearly all patients without comorbidities requiring ICU, this can be safely predicted within the first 4 h after surgery. Our data suggest that selected patients undergoing supraglottoplasty may be safely monitored outside of an ICU setting after an observation period in the post-anesthesia care unit. LEVEL OF EVIDENCE: 4 Laryngoscope, 134:466-470, 2024.

Profiling the clinical characteristics and surgical efficacy of laryngomalacia in children.

OBJECTIVE: To analyze the clinical characteristics of laryngomalacia in Chinese children and explore the surgical efficacy and factors influencing severe laryngomalacia. METHODS: Children (0-18 years) diagnosed with laryngomalacia in our hospital from January 2016 to January 2022 were enrolled in this study. Clinical data of patients, including general conditions, clinical symptoms, grading and classification, medical comorbidities, surgical efficacy, and the risk factors influencing severe laryngomalacia were retrospectively analyzed. RESULTS: A total of 1810 children were enrolled (male:female; 2.02:1), among which most were infants under 1 year (77.18%). Inspiratory laryngeal stridor (69.56%) was the most common symptom. Most patients had mild laryngomalacia (79.28%), with type IV laryngomalacia being the most common classification (52.27%). Congenital heart disease (37.85%) was the most common medical comorbidity. A total of 168 severe laryngomalacia cases were treated via supraglottoplasty with an effective rate of 83.93%. Notably, preterm birth (OR = 3.868, 95% CI 1.340 ~ 11.168), low birth weight (OR = 4.517, 95% CI 1.477 ~ 13.819) and medical comorbidities (OR = 7.219, 95% CI 2.534 ~ 20.564) were independent risk factors for poor prognosis (P < 0.05). CONCLUSION: Laryngomalacia is common among infants under the age of one, and it is mostly characterized by inspiratory laryngeal stridor with various medical comorbidity. Supraglottoplasty is the first treatment choice for severe laryngomalacia cases with high success rates. However, premature delivery, low birth weight, and medical comorbidities significantly affect the efficacy of surgery.

Findings on drug-induced sleep endoscopy in infants with laryngomalacia.

INTRODUCTION: Obstructive sleep apnea (OSA) is highly prevalent in infants with laryngomalacia (LM). Previous studies have reported high rates of persistent OSA following supraglottoplasty (SGP). The objective of this study is to describe the findings on drug-induced sleep endoscopy (DISE) in infants with LM. METHODS: A retrospective review was performed including infants (12.5 months of age or less) with LM who underwent DISE along with direct laryngoscopy and bronchoscopy (DLB). Data collected included clinical presentation, swallow study results, sleep study results, past medical and surgical history, and postoperative outcomes when follow-up data was available. The findings on DISE as well as DLB were reviewed and described. RESULTS: Thirty-five infants were included in the study with a mean age of 0.42 years (range 0.04-1.04). Obstructive sleep-disordered breathing (oSDB) and/or OSA were identified in 26 patients (74.3%) in addition to 7 patients (20%) with noisy breathing during sleep. DISE demonstrated LM in 31 patients (89%). DISE identified 12 patients (34%) with partial or complete obstruction at the level of the tongue base. DLB identified 14 patients (40%) with a secondary airway abnormality including 7 patients (20%) with type 1 laryngeal cleft and 4 patients (11%) with grade 1 subglottic stenosis. Eighteen patients (51%) underwent SGP. CONCLUSION: DISE at the time of airway endoscopy for infants with LM can be helpful in identifying additional sites of obstruction including the tongue base. Glossoptosis may explain and/or predict the previously reported persistence of OSA following SGP. Additionally, DISE can complement awake flexible laryngoscopy in the assessment of severity of LM.

3D-4K Exoscope-Assisted CO2 Laser Supraglottoplasty for Severe Laryngomalacia in a Pediatric Patient.

Laryngomalacia is the most frequent congenital laryngeal anomaly. Surgery is possible by means of cold knife, carbon dioxide (CO2 ) laser, microdebrider and coblation, traditionally under microscopic view. We here describe the use of the 3D-4K exoscope assisted CO2 laser supraglottoplasty in tubeless general anesthesia in spontaneous breathing in a 5 month-old patient with severe laryngomalacia. Laryngoscope, 134:1359-1362, 2024.

Polysomnographic parameters and clinical risk factors predicting postoperative respiratory complications in children undergoing supraglottoplasty.

STUDY OBJECTIVES: Postoperative respiratory complications (PORCs) can occur following supraglottoplasty (SGP) for obstructive sleep apnea. However, there are very limited data on risk factors associated with these complications. This study aims to evaluate the occurrence of PORC in children undergoing SGP and to assess clinical factors and polysomnographic parameters predicting these complications. METHODS: A retrospective study was performed in children with laryngomalacia who underwent SGP with preoperative polysomnography. RESULTS: 400 children who underwent SGP met the criteria for entry into the analysis with a total of 416 surgeries with corresponding preoperative polysomnography. The median age (interquartile range) at the time of polysomnography was 0.4 (0.2, 1.5) years. A total of 96 (23.1%) PORCs were noted. Compared with those without complications, children with PORCs had a higher proportion of congenital heart disease (P < .05), higher median obstructive apnea-hypopnea index (obstructive AHI; median 16.0 vs 11.4 events/h; P < .01), and lower median oxygen saturation (SpO2) (P < .001). The unadjusted odd ratio indicated an increased risk of PORCs in children with congenital heart disease (odds ratio 1.66; P < .05) and those with an obstructive AHI > 10 events/h (odds ratio 2.06; P < .01). Multiple regression analysis demonstrated that an obstructive AHI > 10 events/h was the only independent risk factor for PORCs (P < .05). CONCLUSIONS: In our cohort of children with laryngomalacia undergoing SGP, those with underlying congenital heart disease, higher obstructive AHI, and lower SpO2 were more likely to develop PORCs. Only children with an obstructive AHI > 10 events/h were at significantly increased risk for PORCs following SGP. Preoperative polysomnography is useful in preoperative planning in children undergoing SGP. CITATION: Kanavitoon S, Ngamprasertwong P, Gurbani N, et al. Polysomnographic parameters and clinical risk factors predicting postoperative respiratory complications in children undergoing supraglottoplasty. J Clin Sleep Med. 2024;20(1):9-16.

Post-operative safety of pediatric supraglottoplasty: Is post-operative admission necessary?

OBJECTIVES: The purpose of this study was to determine the incidence of adverse events in the first 48 h (acute) 14 days (subacute) in post supraglottoplasty pediatric patients. A secondary aim was to determine if postoperative hospital admission after supraglottoplasty in pediatric patients is necessary. METHODS: This study was a retrospective review of pediatric patients who underwent supraglottoplasty at a tertiary care center. Data were obtained from January 2017-December 2020, totaling 107 patients. Pediatric patients who underwent supraglottoplasty were included in the study. Information regarding patients' demographics, length of postoperative hospital stay, comorbid conditions, unit of hospital admission, intraoperative and postoperative adverse events, and readmission within the first 14 days was gathered and analyzed. RESULTS: The incidence of postoperative adverse events for all subjects after supraglottoplasty was 5.7 % (N = 6). The most common postoperative complications were respiratory distress (N = 2), followed by substernal retractions, stridor, and decreased oral intake (N = 1). There was no statistically significant increased incidence in any group of patients, regardless of their unit of stay post-operatively (p = 0.39). CONCLUSIONS: Supraglottoplasty is a safe surgical option for patients with severe laryngomalacia. While each patient's care is individualized, we demonstrate that post-operative hospital admission is not necessary for healthy children undergoing supraglottoplasty. LEVEL OF EVIDENCE: III - This is a retrospective chart review.

[New progress in diagnosis and treatment of congenital laryngomalacia in infants].

Congenital laryngomalacia is the most common disease causing laryngeal stridor in infants. The pathogenesis has not yet been clearly concluded. It may be related to abnormal development of laryngeal cartilage anatomical structure, neuromuscular dysfunction, gastroesophageal and laryngeal reflux disease, etc. The typical manifestations of the disease are inspiratory laryngeal stridor and feeding difficulties, which can be divided into mild, moderate and severe according to the severity of symptoms. The diagnosis is mainly based on clinical symptoms, signs and endoscopy, among which endoscopy is an important diagnostic basis. The treatment of laryngomalacia depends on the severity of symptoms. Mild and some moderate congenital laryngomalacia children can be relieved by conservative treatment, and severe and some moderate congenital laryngomalacia children should be treated by surgery. Supraglottic plasty is the main surgical method, which can effectively improve the symptoms of laryngeal stridor, dyspnea, feeding difficulties and growth retardation in most children, and the surgical effect is good.

[The airway management and treatment of newborns with micrognathia and laryngomalacia].

Objective:To explore the perioperative airway management and treatment of newborns with micrognathia and laryngomalacia. Methods:From January to December 2022, a total of 6 newborns with micrognathia and laryngomalacia were included. Preoperative laryngoscopy revealed concomitant laryngomalacia. These micrognathia were diagnosed as Pierre Robin sequences. All patients had grade Ⅱ or higher symptoms of laryngeal obstruction and required oxygen therapy or non-invasive ventilatory support. All patients underwent simultaneous laryngomalacia surgery and mandibular distraction osteogenesis. The shortened aryepiglottic folds were ablated using a low-temperature plasma radiofrequency during the operation. Tracheal intubation was maintained for 3-5 days postoperatively. Polysomnography（PSG） and airway CT examination were performed before and 3 months after the surgery. Results:Among the 6 patients, 4 required oxygen therapy preoperatively and 2 required non-invasiveventilatory support. The mean age of patients was 40 days at surgery. The inferior alveolar nerve bundle was not damaged during the operation, and there were no signs of mandibular branch injury such as facial asymmetry after the surgery. Laryngomalacia presented as mixed type: type Ⅱ+ type Ⅲ. The maximum mandibular distraction distance was 20 mm, the minimum was 12 mm, and the mean was 16 mm. The posterior airway space increased from a preoperative average of 3.5 mm to a postoperative average of 9.5 mm. The AHI decreased from a mean of 5.65 to 0.85, and the lowest oxygen saturation increased from a mean of 78% to 95%. All patients were successfully extubated after the surgery, and symptoms of laryngeal obstruction such as hypoxia and feeding difficulties disappeared. Conclusion:Newborns with micrognathia and laryngomalacia have multi-planar airway obstruction. Simultaneous laryngomalacia surgery and mandibular distraction osteogenesis are safe and feasible, and can effectively alleviate symptoms of laryngeal obstruction such as hypoxia and feeding difficulties, while significantly improving the appearance of micrognathia.

Supraglottoplasty for severe laryngomalacia can be effective and safe also in children with high-risk comorbidities - Experience from a tertiary center.

OBJECTIVES: Supraglottoplasty (SGP) for severe laryngomalacia (LM) in children with medical comorbidities has been associated with high risk of surgical failure and increased need of postoperative pediatric intensive care unit (PICU) intervention, but evidence for this is ambiguous. The objective was to evaluate surgical outcome and risk of need for PICU-intervention following SGP for severe LM in comorbid patients. METHODS: Retrospective observational study of 116 patients treated with SGP for severe LM between 2000 and 2021 at a tertial referral pediatric airway surgery center Karolinska University Hospital. Medical records were reviewed and patient data regarding surgical timing, type of SGP procedure, PICU-intervention, complications, and outcomes were recorded. Patients were defined as non-comorbid vs high-risk comorbid (HRC) based on a coexisting comorbidity for risk of surgical failure and postoperative PICU-intervention. Surgical failure was defined as need of revision surgery, tracheostomy or assisted ventilation (continuous positive airway pressure and bilevel positive airway pressure). PICU intervention was defined as need of postoperative assisted ventilation or intubation. Statistical comparisons were performed with outcome of SGP on children with LM and no comorbidities. RESULTS: 41/116 patients included had a HRC associated with an increased risk of PICU-intervention and surgical failure. 75/116 patients were defined as non-comorbid. The overall surgical success in the study population was 89.7% (104/116), 94.7% in the non HRC group vs 80.5% in the HRC-group. 5/41 HRC patients and 1/75 non-comorbid patients needed SGP revision in which 5/6 was successful. There was no significantly increased need for postoperative PICU intervention in HRC patients. CONCLUSION: SGP for severe LM patients with high-risk comorbidities performed in a tertiary setting had an overall good result and low risk of PICU-intervention. Revision SGP was more common in HRC patients but had a good outcome. Multidisciplinary experience in perioperative care of comorbid patients may be of key importance for outcome and children with high-risk comorbidities should thus not be withheld the possible benefit of SGP without assessment at a tertiary pediatric airway center.

A Novel 13q12 Microdeletion Associated with Familial Syndromic Corneal Opacification.

Progressive corneal opacification can result from multiple etiologies, including corneal dystrophies or systemic and genetic diseases. We describe a novel syndrome featuring progressive epithelial and anterior stromal opacification in a brother and sister and their mildly affected father, with all three family members having sensorineural hearing loss and two also with tracheomalacia/laryngomalacia. All carried a 1.2 Mb deletion at chromosome 13q12.11, with no other noteworthy co-segregating variants identified on clinical exome or chromosomal microarray. RNAseq analysis from an affected corneal epithelial sample from the proband's brother revealed downregulation of XPO4, IFT88, ZDHHC20, LATS2, SAP18, and EEF1AKMT1 within the microdeletion interval, with no notable effect on the expression of nearby genes. Pathway analysis showed upregulation of collagen metabolism and extracellular matrix (ECM) formation/maintenance, with no significantly down-regulated pathways. Analysis of overlapping deletions/variants demonstrated that deleterious variants in XPO4 were found in patients with laryngomalacia and sensorineural hearing loss, with the latter phenotype also being a feature of variants in the partially overlapping DFNB1 locus, yet none of these had reported corneal phenotypes. Together, these data define a novel microdeletion-associated syndromic progressive corneal opacification and suggest that a combination of genes within the microdeletion may contribute to ECM dysregulation leading to pathogenesis.

Management and outcomes of obstructive sleep apnea in infants.

OBJECTIVE: To characterize the clinical characteristics of infants with obstructive sleep apnea (OSA), define the resolution rate of infant OSA, and identify factors associated with OSA resolution. METHODS: We identified infants diagnosed with OSA via retrospective chart review at less than one year of age at a tertiary care center. We identified patient comorbidities, flexible or rigid airway evaluations, surgical procedures, and oxygen/other respiratory support administration. We identified infants as having resolved OSA based on clinical or polysomnogram resolution. We compared the frequency of comorbid diagnoses and receipt of interventions in infants with resolved versus non-resolved OSA by χ2 analysis. RESULTS: 83 patients were included. Prematurity was found in 35/83 (42%), hypotonia-related diagnoses in 31/83 (37%), and craniofacial abnormalities in 34/83 (41%). Resolution was observed in 61/83 (74%), either clinically or by polysomnogram, during follow up. On χ2 analysis, surgical intervention was not associated with likelihood of resolution (73% versus 74% in those without surgical intervention, p = 0.98). Patients with airway abnormalities on flexible or rigid evaluation were less likely to have OSA resolution than those without (63% versus 100%, p = 0.010), as were patients with hypotonia-related diagnoses (58% versus 83%, p = 0.014). In patients with laryngomalacia, there was no association of supraglottoplasty with increased resolution (88% with supraglottoplasty versus 80% without, p = 1.00). CONCLUSIONS: We identified a group of infants with OSA with diverse comorbidities. There was a high rate of resolution. This data can assist with treatment planning and family counselling for infants with OSA. A prospective clinical trial is needed to better assess consequences of OSA in this age.

Epiglottopexy in Infants Younger Than 6 Months Old: A Case Series.

BACKGROUND: Severe laryngomalacia, characterized by apnea, hypoxia, and feeding difficulties, is an uncommon diagnosis that often requires surgical intervention with supraglottoplasty. Children who require surgery at a young age and those with additional comorbidities pose a special challenge and may require further surgical interventions. Posterior displacement of the epiglottis has been noted in some infants with congenital stridor and is commonly treated with epiglottopexy. The goal of our study is to review the outcomes of epiglottopexy combined with supraglottoplasty in our cohort of infants younger than 6 months old with severe laryngomalacia. METHODS: A retrospective chart review of infants younger than 6 months old who underwent epiglottopexy combined with supraglottoplasty for severe laryngomalacia from January 2018 to July 2021 at a tertiary care children's hospital. RESULTS: 13 patients (age 1.3 week-5.2 months) underwent supraglottoplasty and epiglottopexy for severe laryngomalacia and epiglottis retroflection. The patients were admitted to the intensive care unit and remained intubated for at least one night. All patients demonstrated subjective and objective improvement in upper airway respiratory signs and symptoms. Ten patients demonstrated aspiration immediately postoperatively, despite 4 of them having no concern for aspiration at preoperative evaluation. On follow-up, 1 patient required revision supraglottoplasty and epiglottopexy for persistent laryngomalacia, and 2 patients required tracheostomy tube placement due to cardiopulmonary comorbidities. CONCLUSION: Infants younger than 6 months old with medical comorbidities undergoing epiglottopexy with supraglottoplasty may demonstrate significant improvement in respiratory symptoms. Worsening dysphagia may complicate the postoperative period, particularly among children with medical comorbidities.

Von Mises stress peak (VMSP) and laryngomalacia severity score (LSS) are extremely useful in the selection of treatment for laryngomalacia.

OBJECTIVE: To analyze the judgment efficiency of a computer stress model and severity score in severity evaluation and treatment plan selection of laryngomalacia patients. METHODS: Twenty-two children (12 cases in the operation group and 10 cases in the follow-up group) with moderate to severe laryngomalacia were assessed by laryngomalacia severity score (LSS) which included visual analogue scale (VAS) and clinical score. A computer stress model of the laryngeal cavity was constructed for all children, with the von Mises stress peak (VMSP) of the model used as another quantitative evaluation method. The ROC curves of two quantitative evaluation methods, the LSS and the VMSP, were analyzed respectively, according to the clinical guideline which is regarded as the gold standard for judging whether surgery is needed. The diagnostic efficiency indexes such as sensitivity, specificity, and accuracy were calculated. The area under ROC curves (AUC) of the two methods were compared by a DeLong model. Spearman correlation analysis and Kappa test were used to test the correlation and consistency of the two quantitative evaluation methods. The independent sample t test was used to compare the difference of LSS and VMSP between operation group and follow-up group. RESULTS: The sensitivity, specificity, and accuracy of LSS in judging whether laryngomalacia was operated or not were 83.33%, 80.00% and 81.82%, respectively, and the area under ROC curve (AUC) was 0.825 (p < 0.05). The sensitivity, specificity, and accuracy of the computer stress model for laryngomalacia were 58.33%, 90.00% and 72.73%, respectively, and the AUC was 0.796 (p < 0.05). The spearman correlation coefficient between LSS and VMSP was 0.833, p < 0.001, which is statistically significant. LSS (t = 3.251, p = 0.004) and VMSP (t = 2.435, p = 0.024) of the two groups were statistically different. CONCLUSION: VMSP and LSS have high diagnostic efficacy in the quantitative evaluation of the severity of laryngomalacia and the selection of treatment plan. The consistency of the two quantitative evaluation methods is good, which has practical value for the evaluation of the severity of laryngomalacia and has guiding significance for surgery.

Congenital subglottic cyst: a rare cause for sudden airway compromise in a newborn.

A full-term infant developed stridor, respiratory distress and hypercarbia shortly after birth requiring urgent airway intervention. The patient underwent urgent intubation via rigid bronchoscopy. The diagnosis of congenital subglottic cyst was made. The cyst was decompressed and the patient was extubated the following day. On repeat laryngoscopy 1 month later, there was no residual disease and the patient remained symptom free. Congenital subglottic cysts are extremely rare and the diagnosis can be either missed or misdiagnosed with more common causes of stridor, such as laryngomalacia. Subglottic cysts may cause total airway obstruction and even death if they are large enough and not treated immediately.

Cochlear implantation in a patient with congenital microtia, cochlear hypoplasia, venous anomalies of the temporal bone and laryngomalacia: Challenges and surgical considerations.

RATIONALE AND PATIENT CONCERNS: Congenital hearing loss is often caused by an inner ear malformation, in such cases, the presence of other anomalies, such as microtia, and venous anomalies of the temporal bone and laryngomalacia makes it challenging to perform cochlear implantation surgery. DIAGNOSES: This study reports the case of a 28-month-old girl with congenital profound hearing loss, laryngomalacia, and malformed inner ear, who received cochlear implantation surgery. The bony structure, vessels and nerves were first assessed through magnetic resonance imaging and computed tomography before exploring the genetic basis of the condition using trio-based whole exome sequencing. Perioperative evaluation and management of the airway was then performed by experienced anesthesiologist, with the surgical challenges as well as problems encountered fully evaluated. INTERVENTIONS: Cochlear implantation was eventually performed using a trans-mastoid approach under uneventful general anesthesia. OUTCOMES: Due to the small size of the cochlea, a short electrode FLEX24 was inserted through the cochleostomy. LESSONS: Considering the high risk of facial nerve injury and limited access to the cochlea when patients present significant bony and venous anomalies, cochlear implantation in such patients require careful preoperative evaluation and thoughtful planning. In these cases, airway assessment, magnetic resonance venography, magnetic resonance arteriography, and magnetic resonance imaging and computed tomography can be useful to minimize the risks. Intraoperative facial nerve monitoring is also recommended to assist in the safe location of facial nerve.

New approach to establish a surgical planning in infantile vallecular cyst synchronous with laryngomalacia based on aerodynamic analysis.

BACKGROUND AND OBJECTIVES: A large proportion of infants with vallecular cyst (VC) have coexisting laryngomalacia (LM). Feeding difficulties, regurgitation, occasional cough, and sleep-disordered breathing are the common symptoms in moderate to severe cases. The surgical management of these cases is more challenging and remains controversial. The purpose of this study is to help surgeons select the effective surgical strategies by computer-aided design (CAD) and computational fluid dynamics (CFD) simulations of the upper airway flow characteristics. METHODS: The three dimensional (3D) geometric model of the upper airway was reconstructed based on two dimensional (2D) medical images of the patient with VC accompanied with LM. Virtual surgeries were carried out preoperatively to simulate three possible post-operative states in silico. The different outcomes of virtual surgical strategies were predicted based on computational evaluations of airway fluid dynamics including pressure, resistance, velocity, and wall shear stress (WSS). RESULTS: The CFD results of this study suggested the importance of the angle between the rim of epiglottis and arytenoid epiglottic (AE) fold. There was a small impact on the upper airway flow field while the VC was removed and the angle of epiglottis was unchanged. The partial lifting of epiglottis can further improve the flow field. With performing supraglottoplasty (SGP) and the marsupialization of VC, epiglottis was completely recovered, and the flow field was significantly improved. The clinical symptoms of this patient improved greatly after surgeries and no recurrence or growth retardation were noted during 1-year follow-up. The clinical prognosis was consistent with the prediction of the CFD results. CONCLUSIONS: The state of epiglottis needs to be carefully checked to evaluate the necessity of performing further SGP in the patients with VC accompanied with LM. CFD and CAD could be developed as a new approach to help surgeons predict the post-operative outcomes through quantification of the airflow dynamics, and make the optimal and individualized surgical approaches for patients with airway obstruction.

Symptomatic Lingual Thyroglossal Duct Cyst in Children: A Laryngomalacia Phenotype.

OBJECTIVES: Patients with lingual thyroglossal duct cyst (TGDC) can present as symptomatic with obstructive airway and feeding difficulties. METHODS: We present 3 cases of symptomatic lingual TGDC. RESULTS: All 3 patients were diagnosed with laryngomalacia and underwent further concurrent or delayed airway intervention, in addition to cyst removal. CONCLUSIONS: We reason that there is a phenotype of laryngomalacia in the symptomatic lingual thyroglossal duct cyst patients who present with symptoms due to disruption in laryngeal anatomy rather than the cyst itself causing obstructive symptoms. Distinguishing this phenotype, especially in comparison to other pathologies such as vallecular cysts, may better allow for planning of concurrent or delayed airway procedures and overall counseling of parents.