The anesthetic management of a child with ohtahara syndrome and severe stridor: a case report.

BACKGROUND: Ohtahara syndrome is a progressive developmental and epileptic encephalopathy that manifests in the early infantile period. This rare condition is characterized by intractable seizures, psychomotor retardation, and poor prognosis. To date, there are a handful of case reports regarding the anesthetic management of children with Ohtahara syndrome. However, limited reports exist of patients with Ohtahara syndrome who present with difficult airways. This report describes our airway findings and general anesthetic management of a pediatric patient with Ohtahara syndrome undergoing diagnostic bronchoscopy for severe inspiratory stridor. CASE PRESENTATION: A 14-month-old, 9 kg, male patient with Ohtahara syndrome presented with a year-long history of severe inspiratory stridor and was scheduled for bronchoscopy with lavage. On exam, the patient had noisy breathing, was non-verbal with developmental delay, and had poor head control with significant central hypotonia. The patient was induced with ketamine and general anesthesia was maintained with propofol. Bronchoscopic evaluation was completed uneventfully and revealed a diagnosis of laryngotracheomalacia. The patient's breathing was maintained spontaneously throughout the procedure and no seizures were noted. In the post anesthesia care unit, the patient's respiratory and cardiovascular function were stable. CONCLUSIONS: This report documents the unusual finding of severe inspiratory stridor in a 14-month-old child diagnosed with Ohtahara syndrome and our anesthetic management during their diagnostic bronchoscopy. Currently, documentation of complex airway pathology present in patients with Ohtahara syndrome is limited and should be further evaluated. This will assist pediatric anesthesiologists as these patients may require careful preoperative assessment, thoughtful airway management, and surgical alternatives on standby.

Does surgery affect the neurodevelopmental outcome of moderate laryngomalacia?

BACKGROUND: Moderate laryngomalacia lies in the grey zone where the parents and treating team might hesitate to decide the treatment plan for the child. Neurodevelopmental assessment of the child was neither assessed nor incorporated in surgical decision-making in the past. OBJECTIVES: To evaluate the neurodevelopmental outcome of moderate laryngomalacia treatment strategies (surgery versus conservative). METHODS: We conducted an observational multicentric cohort study. We compared two groups of patients according to their moderate laryngomalacia treatment strategy: surgical treatment and no treatment. The neurodevelopmental outcome was assessed by Griffiths-III developmental scales. RESULTS: A total of 150 children were diagnosed with moderate laryngomalacia of which 56 were successfully treated with surgery and 94 improved without intervention. The median Griffiths-III developmental quotients (DQs) of subscales A, B, C, E, and general development were significantly higher in the surgically treated group compared to conservatively treated ones. CONCLUSION: Untreated moderate laryngomalacia cases have worse neurodevelopmental outcomes than surgically treated cases.

Predominantly unilateral laryngomalacia in infants with unilateral vocal fold paralysis.

BACKGROUND: Neonatal unilateral vocal fold paralysis may arise iatrogenically, idiopathically, or in the context of an underlying neurologic disorder. Management is often supportive, focusing on diet modification to allow for safe oral feeding. We describe the clinical course of six infants with unilateral vocal fold paralysis who developed predominantly unilateral laryngomalacia ipsilateral to the affected vocal fold with associated severe respiratory symptoms and feeding difficulty. METHODS: Retrospective review of six infants with unilateral vocal fold paralysis and predominantly unilateral laryngomalacia. Charts were reviewed for etiology of vocal fold paralysis, presenting symptoms, operative details, postoperative course, and outcomes for breathing and swallowing. RESULTS: Etiology of vocal fold paralysis included cardiac surgery in four patients, intubation-related in one, and idiopathic in one. Presenting symptoms included increased work of breathing, stridor, feeding difficulty, respiratory failure requiring noninvasive respiratory support, and weak cry. All infants were on nasogastric tube feedings. Direct microlaryngoscopy with unilateral or predominantly unilateral (conservative contralateral aryepiglottic fold division) supraglottoplasty was performed. Stridor and work of breathing improved in all six patients within 1 week postoperatively. Oral feeding improved in three patients within 2 weeks. Three patients had persistent feeding impairment with improvement within one year. CONCLUSIONS: Predominantly unilateral laryngomalacia may arise in the context of unilateral vocal fold paralysis. Addressing the ipsilateral cuneiform collapse can improve breathing and feeding. This may be an under-described phenomenon and represents an additional reason to include the otolaryngologist early in the care of infants with suspected possible new unilateral vocal fold paralysis. Breathing and swallow can improve post-operatively, but feeding may remain limited by the vocal fold paralysis and any medical comorbidities. Ongoing follow-up and collaboration with speech-language pathology to optimize feeding are important.

Nap polysomnography in infants with laryngomalacia as a tool to predict treatment strategy.

PURPOSE: This study aimed to investigate the role of nap polysomnography (NPSG) in predicting treatment strategies for infants with moderate to severe laryngomalacia and to explore the association between obstructive sleep apnea (OSA) severity, weight gain, and laryngomalacia severity. METHODS: A retrospective analysis was conducted on infants diagnosed with moderate to severe laryngomalacia who underwent NPSG between January 2019 and June 2023. Clinical variables, NPSG parameters, and treatment decisions were collected. Weight gain rate and its correlation with NPSG indices were assessed. Logistic regression analyses were performed to predict treatment strategies based on NPSG findings. RESULTS: Of the 39 infants included (median age: 3.3 months), 77% exhibited OSA, with 69% having moderate to severe OSA [apnea-hypopnea index (AHI) > 5/h]. Weight gain rate correlated negatively with indices of OSA severity, including the hypopnea index (HI) and the AHI. In a multiple logistic regression analysis incorporating the severity of OSA (AHI), weight gain rate, and laryngomalacia severity, only AHI predicted the decision for surgical or non-invasive ventilation treatment (OR = 2.1, CI95 [1.6; 2.8], p ≤ 10-4). The weight gain rate was predicted (r2 = 0.28) by the AHI and the presence of retractions of auxiliary inspiratory muscles. CONCLUSION: This study underscores the importance of NPSG in assessing infants with moderate to severe laryngomalacia. The AHI from NPSG emerged as a potential predictor for treatment decisions and weight gain rate, emphasizing its clinical relevance. These findings advocate incorporating NPSG into the diagnostic and management process for infants with laryngomalacia.

Role of upper airway evaluation in the multidisciplinary management of obstructive sleep apnea in children below two years of age.

BACKGROUND: Diagnosis and treatment of obstructive sleep apnea (OSA) in infants and young children is challenging because of its clinical heterogeneity and lack of age-specific guidelines. AIM: We report the management and treatment outcome of OSA in children below 2 years of age. Treatment decisions were based upon the pattern of upper airway (UA) obstruction, clinical presentation and OSA severity. METHODS: Retrospective, non-randomized observational cohort study at a tertiary center. Children with OSA who underwent an UA evaluation (drug-induced sleep endoscopy or direct laryngoscopy) were included. RESULTS: We studied 100 patients, 57 boys and 43 girls, age 0.72 years (0.0-2.0) and OSA confirmed by polysomnography. Multilevel UA collapse was present in 26%, (adeno)tonsillar hypertrophy in 31% and 21% had laryngomalacia. Laryngomalacia was more common in children below 6 months of age and adenotonsillar hypertrophy was observed mainly in children >1.5 year of age. Surgical and nonsurgical treatment guided by UA findings, improved OSA severity at group level with a significant reduction (p < 0.001) in obstructive apnea/hypopnea index from 10.8/h (2.1-99.1) to 1.7/h (0.0-73.0), an improvement in mean oxygen saturation from 96.9% (88.9-98.4) to 97.4% (92.3-99.0), in minimal oxygen saturation from 85.4% (37.0-96.0) to 88.8% (51.0-95.5) and oxygen desaturation index from 5.1/h (0.2-52.0) to 1.3/h (0.0-47.8). CONCLUSION: Multidisciplinary management of young children with OSA guided by the pattern of UA obstruction and OSA severity, reduces OSA severity. The pattern of UA obstruction changes in the first 2 years of life from a dynamic collapse to structural abnormalities.

Management of Obstructive Sleep Apnea in the Infant and Newborn.

Obstructive sleep apnea in newborns and infants presents a unique challenge with distinct differences in sleep physiology, etiologies, and management compared to older children. The indications for and interpretation of polysomnography are less well defined in infants. There are also no broadly accepted clinical practice guidelines for treating sleep apnea in this age group. Etiologies include general causes of upper airway obstruction in infants such as laryngomalacia, micrognathia, and nasal obstruction in addition to adenotonsillar hypertrophy. Treatment strategies must be tailored to the specific anatomic features and comorbidities of the specific patients and often require a multidisciplinary approach.

Laryngeal Structural Dysphagia in Children.

The upper aerodigestive system is closely intertwined from an embryologic and functional perspective. Laryngotracheal anatomic abnormalities, such as laryngomalacia, stenosis, vocal cord paralysis, and laryngeal clefts, affect not only the respiratory function but also the swallow function. Laryngotracheal pathology can interfere with the suck-swallow-breathe mechanism in infants. It can also exacerbate gastroesophageal reflux. Chronic aspiration secondary to laryngotracheal anomalies can result in respiratory and pulmonary complications. Surgical treatment of laryngotracheal anomalies can also cause transient or long-term swallow dysfunction. Multidisciplinary approaches and clinical assessment of swallowing are important in patients with laryngotracheal pathology.

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.

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.

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.

Risk of dysphagia in a population of infants born pre-term: characteristic risk factors in a tertiary NICU.

OBJECTIVE: To examine the prevalence and characteristics of dysphagia and suck-swallow-breath incoordination as phenotypes of oral feeding difficulties. METHOD: A cross-sectional study with secondary data collected consecutively over 2 years from October 2020 to October 2022 to measure the prevalence of swallowing and oral feeding difficulty in preterm infants using Flexible endoscopic evaluation of swallowing examination at the tertiary Integrated Dysphagia Clinic. RESULTS: The prevalence of swallowing disorders was 25 % and the prevalence of suck-swallow-breath incoordination was 62.5 %. The significant risk factor that may show a possible correlation with oral feeding difficulty was mature post-menstrual age (p = 0.006) and longer length of stay (p = 0.004). The dominant percentage of upper airway abnormality and disorder were retropalatal collapse (40 %), laryngomalacia (42.5 %), paradoxical vocal cord movement (12.5 %), and gastroesophageal reflux disease (60 %). The dominant characteristic of oral motor examination and flexible endoscopic evaluation of swallowing examination was inadequate non-nutritive sucking (45 %), inadequate postural tone (35 %), and inadequate nutritive sucking (65 %). CONCLUSION: Dysphagia in preterm infants is mostly observed in those with mature post-menstrual age, longer length of stay, and the presence of gastroesophageal reflux disease with inadequate non-nutritive sucking and nutritive sucking abilities. Suck-swallow-breath incoordination is primarily observed in those with immature post-menstrual age, a higher prevalence of cardiopulmonary comorbidity, and a higher prevalence of upper airway pathologies (laryngomalacia, paradoxical vocal cord movement) with inadequate nutritive sucking ability.

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.

Polysomnogram outcomes in patients with laryngomalacia and obstructive sleep apnoea treated surgically versus non-surgically.

OBJECTIVE: To compare supraglottoplasty versus non-surgical treatment in children with laryngomalacia and mild, moderate and severe obstructive sleep apnoea. METHODS: Patients were classified based on their obstructive apnoea hypopnoea index on initial polysomnogram, which was compared to their post-treatment polysomnogram. RESULTS: Eighteen patients underwent supraglottoplasty, and 12 patients had non-surgical treatment. The average obstructive apnoea hypopnoea index after supraglottoplasty fell by 12.68 events per hour (p = 0.0039) in the supraglottoplasty group and 3.3 events per hour (p = 0.3) in the non-surgical treatment group. Comparison of the change in obstructive apnoea hypopnoea index in the surgical versus non-surgical groups did not meet statistical significance (p = 0.09). CONCLUSION: All patients with laryngomalacia and obstructive sleep apnoea had a statistically significant improvement in obstructive apnoea hypopnoea index after supraglottoplasty irrespective of obstructive sleep apnoea severity, whereas patients who received non-surgical treatment had more variable and unpredictable results. Direct comparison of the change between the two groups did not find supraglottoplasty to be superior to non-surgical treatment. Larger prospective studies are recommended.

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.

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.

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.

Prevalence and Management of Laryngomalacia in Patients With Pierre Robin Sequence.

OBJECTIVE: To characterize the prevalence and presentation of laryngomalacia and efficacy of supraglottoplasty (SGP) in a cohort of patients with Pierre Robin Sequence (PRS). DESIGN: Retrospective cohort study. SETTING: Tertiary-care children's hospital. PATIENTS, PARTICIPANTS: Consecutive patients with PRS born between January 2010 and June 2018. MAIN OUTCOME MEASURES: Chart review included demographics, comorbid airway obstruction including laryngomalacia, timing of surgical interventions, clinical symptoms, sleep study data, and modified barium swallow study data.126 patients with PRS were included; 54% had an associated syndrome, 64% had an overt cleft palate, and 22% had a submucous cleft palate. 64/126 were noted to have laryngomalacia (51%). Patients with concurrent PRS and laryngomalacia were significantly more likely to have submucous cleft palate (P = .005) and present with aspiration with cough (P = .01) compared to patients with PRS without laryngomalacia. Patients with concurrent laryngomalacia and PRS showed a significant decrease in apnea-hypopnea index (AHI) and obstructive AHI (OAHI) after mandibular distraction, with a median AHI and OAHI improvement of 22.3 (P = .001) and 19.8 (P = .002), respectively. Patients who underwent only SGP did not show significant improvement in these parameters (P = .112 for AHI, P = .064 for OAHI).The prevalence of laryngomalacia in our PRS cohort was 51%. Patients with PRS and laryngomalacia are more likely to present with overt aspiration compared to patients with PRS without laryngomalacia. These data support that laryngomalacia does not appear to be a contraindication to pursuing MDO.

Acquired laryngomalacia as a cause of post-extubation respiratory failure in patient with postoperative seizure and central pontine myelinolysis after craniotomy.

Background : Laryngomalacia is a congenital abnormality of the larynx that commonly occurs in children and rarely in adults. We report the first case of acquired laryngomalacia mainly due to postoperative seizure and central pontine myelinolysis after scheduled craniotomy. Case presentation : A 69-year-old man was admitted to the hospital for elective craniotomy for craniopharyngioma. After the surgery, he developed refractory seizure and required intubation and mechanical ventilation in the intensive-care unit (ICU). After treatment for the seizure, he was extubated. However, immediately after extubation, he developed stridor and respiratory retraction. We performed fiberoptic laryngoscopy and confirmed that the epiglottis had collapsed into the posterior wall of the pharynx during inspiration, which was suspected to be laryngomalacia. He received invasive mechanical ventilation for two days following re-extubation. After the second extubation, he developed stridor again due to acquired laryngomalacia. Six days later, his respiratory condition had worsened, and he received re-intubation and tracheostomy. After ICU discharge, central pontine myelinolysis was diagnosed by magnetic resonance imaging. Conclusions : Adult-onset laryngomalacia is a rare cause of upper airway obstruction but should be considered as a cause of postoperative extubation failure. We should not delay performing fiberoptic laryngoscopy to evaluate this pathology and provide optimal treatment. J. Med. Invest. 69 : 316-319, August, 2022.