Anesthesia management for a child with the Koolen-de Vries syndrome: a case report.

BACKGROUND: The Koolen-de Vries syndrome (KdVS) is a relatively new rare disease caused by micro-deletion of 17q21.31 which was first reported by Koolen in 2006. Typical phenotypes for KdVS include hypotonia, developmental delay, moderate intellectual disability, and characteristic facial dysmorphism. Up to now, there was only one case report about anesthesia management of patient diagnosed KdVS. It was a 2-year-old girl who experienced an MRI exam under anesthesia. CASE PRESENTATION: We described a 21-month-old boy who planned to undergo an orchidopexy under general anesthesia diagnosed with KdVS. He had an intellectual disability, characteristic facial dysmorphism, tracheo/laryngomalacia, patent foramen ovale, and cryptorchidism related to KdVS. Due to the complex condition especially the presence of tracheo/laryngomalacia, we took some special measures, including reducing the amount of long-acting opioid, keeping the spontaneous breath, performing a caudal block, and applying the laryngeal mask. But the laryngeal mask was changed to an endotracheal tube because it failed to provide adequate ventilation. The boy experienced mild laryngeal spasm and hypoxia after extubation, but lateral position and etomidate eased his breathing problem and re-intubation was avoided. It is indicated that anesthesia management for patients with orphan disease is a real challenge for all anesthesia providers. CONCLUSIONS: The Koolen-de Vries syndrome is a relatively new orphan disease involving multiple systems. Keeping spontaneous breath, evaluating airway potency to anesthetics, applying endotracheal tube, and post-extubation lateral or prone position may be helpful for airway management for patient with hypotonia and tracheo/laryngomalacia. KdVS patient needs prolonged post-anesthesia monitoring and/or medication for airway complications.

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.

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.

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.

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.

Evaluation of vitamin D levels and biochemical markers in infants diagnosed with laryngomalacia.

Background/aim: The pathology of laryngomalacia is still not clear. The aim of this study was to investigate the relationship between vitamin D levels and laryngomalacia, and to evaluate vitamin D levels according to the classification of laryngomalacia. Materials and methods: This retrospective study was conducted in the Kahramanmaras Sütçü Imam University Medicine Faculty's Otorhinolaryngology Clinic between June 2014 and January 2021. Laryngomalacia was classified. Laboratory tests for all patients included calcium (Ca), phosphorus (P), parathormone (PTH), blood urea nitrogen (BUN), creatinine (Cre), alanine transaminase (ALT), and 25-hydroxy vitamin D (25-OH-D). Results: Evaluations were performed for 64 infants with laryngomalacia, including 41 male and 23 female infants with a mean age of 4.6 ± 3.0 months, and a control group of 64 healthy infants with a mean age of 4.5 ± 2.8 months. A statistically significant difference was determined between the laryngomalacia group and the control group with respect to 25-OH-D and PTH levels (p < 0.001). When data were examined according to laryngomalacia types, a statistically significant difference was determined between the groups for 25-OH-D, Ca, P, PTH, and ALT values. The 25-OH-D level was statistically significantly lower in the severe laryngomalacia group than in the mild and control groups (p < 0.001). A statistically significant difference was determined between the moderate and severe laryngomalacia groups and the control group regarding PTH levels (p < 0.001). Conclusion: Vitamin D deficiency may have a role in the etiology of laryngomalacia, and this view is supported by the finding that there was a decrease in vitamin D levels associated with laryngomalacia classification. In addition, the reduction in PTH levels in infants with laryngomalacia may be explained by the change in Ca metabolism. It would be appropriate for further studies to investigate the response to vitamin D replacement therapy in patients with moderate and severe laryngomalacia.

Initiation of acid suppression therapy for laryngomalacia.

OBJECTIVE: Evidence supporting the use of acid suppression therapy (AST) for laryngomalacia (LM) is limited. The objective of this study was to determine if outpatient-initiated AST for LM was associated with symptom improvement, weight gain, and/or avoidance of surgery. METHODS: A retrospective cohort was reviewed at a tertiary-care children's hospital. Patients were included if they were diagnosed with LM at ≤6 months of age, seen in an outpatient otolaryngology clinic between 2012 and 2018, and started on AST. Primary outcomes were improvement of airway and dysphagia symptoms, weight gain, and need for surgery. Severity was assessed by symptom severity. RESULTS: Of 2693 patients reviewed, 199 met inclusion criteria. Median age of diagnosis was 4 weeks (range: 0-29 weeks). LM was classified as mild/moderate (71.4%) and severe (28.6%) based on symptom severity. Severity on flexible fiberoptic laryngoscopy (FFL) was not associated with clinical severity. Weight percentile, airway symptoms, and dysphagia symptoms improved within the cohort. In total, 26.1% underwent supraglottoplasty (SGP). In multivariate analysis, only severe LM on FFL was predictive of SGP (OR: 7.28, 95%CI: 1.91-27.67, p = .004). CONCLUSION: Clinical symptom severity did not predict response to AST raising the question of utility of AST in LM. Severity of LM based on FFL, not clinical severity, was associated with decision to pursue SGP. Prospective randomized trials are needed to better understand the role of AST in LM. LEVEL OF EVIDENCE: Level 3.

Laryngeal surgery for congenital laryngomalacia: NSQIP-P analysis of complications.

OBJECTIVE: To evaluate predictors of complications in children with congenital laryngomalacia who underwent laryngeal surgery. METHODS: Multi-institutional retrospective analysis using the American College of Surgeons-NSQIP-P database (2014-2019). CPT code 31541 and ICD-10 code Q35.1 (congenital laryngomalacia) were used to select patients <18 years. Variable predictors included demographics and medical co-morbidities. Main outcomes assessed included total length of hospital stay (LOS), reintubation, reoperation and readmission. RESULTS: 1092 children were identified, 450 (41.1%) females and 642 (58.6%) males, with a mean age of 1.1 years (95% CI 1.0-1.2). Mean LOS was 3.9 days (95% CI 3.3-4.6). Sixteen (1.5%) were reintubated, 30 (2.7%) were readmitted, and 18 (1.6%) were reoperated within 30 days. Thirty-one (2.8%) were still in hospital at 30 days. Regression analysis revealed an increase in LOS for several groups; the largest effect was seen for patients with preoperative ventilator dependence, admission within the first 28 days of life, and those who were discharged to other healthcare facilities (p < .001). Preoperative co-morbidities significantly associated with a higher frequency of reintubation included ventilator dependence (p = .003), history of prematurity (p = .016) and chronic lung disease (p = .041). Children undergoing surgery in the first 28 days of life were 10.16 times as likely to return to the OR for a related reason within 30 days than older children (p = .038). CONCLUSION: Complications were rare after laryngeal surgery with a postop diagnosis of laryngomalacia. Recognizing pertinent clinical factors can help with risk assessment and management.

Coblation supraglottoplasty: a ten-year experience in a tertiary referral hospital.

PURPOSE: The present study aims to review the outcomes of coblation supraglottoplasty performed for children with different types of laryngomalacia, and we discuss the factors affecting these outcomes. METHODS: We retrospectively reviewed the medical records of laryngomalacia patients admitted to the Otorhinolaryngology Department, Mansoura University, from 2010 to 2020. We examined the patient's demography, symptoms, comorbidities, type of laryngomalacia, oxygen saturation, and final outcomes. RESULTS: Our study included 235 patients; 122 patients responded to medical therapy, while 113 underwent surgical management. There is a significant relation between the types and therapy they underwent (p ≤ 0.001). Larger percentage within type I underwent medical therapy. There is a statistically significant difference between the studied groups regarding age at surgery. On pairwise comparison, patients with type II had the lowest age significantly at the surgery when compared with each other individual group (p ≤ 0.001). On multivariate regression analysis, the presence of comorbid congenital heart disease, neurological comorbidities significantly increased the risk of failure of surgical intervention by 17.32 and 5.803 folds, respectively. CONCLUSIONS: Coblation supraglottoplasty is effective and safe to treat severe laryngomalacia. Different morphological types of laryngomalacia require slight surgical variations of coblation supraglottoplasty. The presence of comorbid congenital heart disease, neurological comorbidities significantly increased the risk of failure of surgical intervention.

Inspiratory stridor of newborns and infants admitted to a paediatric ENT outpatient clinic: diagnostic approach and therapeutic outcome.

The main objective was to analyse the use of rigid laryngotracheoscopy under general anaesthesia (GA) and endoscopic surgery in the management of inspiratory stridor in patients referred to a paediatric ENT outpatient clinic. The secondary objective was to analyse the aetiological diagnoses made and their therapeutic management. This is a prospective study including all newborns and infants, corresponding to 190 patients, presenting for the first time in consultation for inspiratory stridor from January 2015 to December 2017. A consultation form was filled out after each consultation and added to a database; a management algorithm was used to determine which patients required a rigid laryngotracheoscopy. A 17.9% (n = 34) of the patients required rigid laryngotracheoscopy, of whom 12.6% (n = 24) underwent concomitant endoscopic surgery. A 65.8% (n = 125) of the patients were diagnosed with laryngomalacia, 21.1% (n = 40) with isolated posterior excess of mucosa, 9.5% (n = 18) with another diagnosis and 3.7% (n = 7) with a normal examination. The presence of comorbidity was associated (p < 0.001) with the use of rigid laryngotracheoscopy and endoscopic surgery.Conclusion: Rigid laryngotracheoscopy under GA was required in one in five to six patients. Conservative management with strict follow-up may be appropriate in a large number of patients, especially those with laryngomalacia. What is Known: • Previous research has established that laryngomalacia is the main aetiology of stridor. • Comorbidities are linked with a poor tolerance of stridor. What is new: • About one in five to six patients seen in consultation for stridor will require a trip to the operative room (and one in eight will require endoscopic surgery). • Laryngomalacia and isolated posterior excess of mucosa account for 85-90% of the patients seen in consultation for stridor.

Pharyngomalacia diagnosed by laryngo-tracheo-bronchoscopy in the neonatal intensive care unit.

BACKGROUND: Flexible fiber-optic laryngo-tracheo-bronchoscopy has become widely performed in infants and neonates since the introduction of thin flexible fiberscopes. Laryngomalacia is the most common airway disease in infants causing stridor. Pharyngomalacia, termed pharyngeal occlusion during inspiration, was the second most common airway disease found in our hospital in patients that underwent laryngo-tracheo-bronchoscopy, but the incidence, patient characteristics, and natural course have not been reported in large numbers in Japan. METHODS: A retrospective review was performed of medical records on patients admitted to our neonatal intensive care unit during the neonatal period diagnosed with pharyngomalacia between April 2009 and November 2018. Patient characteristics, concurrent airway diseases, comorbidities, and treatment were reviewed. RESULTS: Forty-eight patients were diagnosed with pharyngomalacia. The median gestational age was 37.1 weeks, and the median birthweight was 2,552 g. Patients were diagnosed at a median age of 29 days, and cure was achieved at a median age of 4 months. Twenty-eight patients had concurrent airway diseases, laryngomalacia being the most common. Continuous positive airway pressure or high flow nasal cannula was used in 34 patients. CONCLUSIONS: In patients with pharyngomalacia, half were born preterm, and more than half had concurrent airway diseases. The onset and diagnosis were made within the first month of life in more than half of the patients, and resolution was seen mostly within the first 6 months of life. Whenever a patient is suspected of having an airway disease, the pharyngeal space should be carefully observed to diagnose pharyngomalacia.

Investigation of the serum vitamin D level in infants followed up with the diagnosis of laryngomalacia: a case-control study.

OBJECTIVE: The possible etiological relationship of the vitamin D with laryngomalacia is unclear. The aim of the study was to demonstrate the relationship between laryngomalacia and vitamin D levels. METHODS: Twenty-three non-syndromic babies under the age of 1 year who were diagnosed with laryngomalacia were included in the study group. Forty healthy babies were included in the control group. The detailed anamnesis was obtained and a complete systemic physical examination, a flexible endoscopic laryngeal examination, and laboratory tests [calcium (Ca), phosphorus (P), alkaline phosphatase (ALP), parathormone (PTH), alanine transaminase (ALT), aspartate transaminase (AST), blood urea nitrogen (BUN), creatinine (Cre), and 25-hydroxy vitamin D (25-OH D)] were performed in all patients. All laboratory tests of the groups were compared. RESULTS: It was observed that there was no significant difference in the parameters that could affect vitamin D levels, namely type of feeding, vitamin D supplement intake, and the season when the serum sample was taken (p > 0.05). The vitamin D level was significantly lower (p = 0.003, p < 0.05) and the P and ALP levels were significantly higher (p = 0.016 and p = 0.001, respectively; p < 0.05) in the laryngomalacia group. Although the correlation between vitamin D and PTH was not statistically significant according to the Pearson correlation analysis, it was lower in the laryngomalacia group compared to the control group (p = 0.381, p > 0.05). CONCLUSION: In this study, it was observed that the vitamin D levels were lower in infants with laryngomalacia compared to the control group. We consider that vitamin D deficiency may be a factor in the etiology of laryngomalacia with a yet-to-be-clarified etiology.

Customized Pre-Epiglottic Baton Plate-A Practical Guide for Successful, Patient-Specific, Noninvasive Treatment of Neonates With Robin Sequence.

OBJECTIVE: Despite its efficiency and benefits in treating patients with Robin sequence (RS), the pre-epiglottic baton plate (PEBP) is not widely used. However, its acceptance might improve with specific defined parameters for indication and proper design of the velar extension. We present our 13-year, single-center experience in treating infants with RS using PEBP, focusing on the description and insertion of an endoscopically guided PEBP design along with its complications and limitations. DESIGN AND INNOVATION: We recommend PEBP as primary treatment for RS, suggesting a new approach of design adjustment based on endoscopic findings of multilevel upper airway obstruction. SETTING: Department of cleft lip and palate. PATIENTS: Infants with isolated or syndromic RS, period 2010 to 2019. INTERVENTIONS: Pre-epiglottic baton plate treatment, intravelar veloplasty, and hard palate closure after initial PEBP treatment. RESULTS: We treated 132 infants (isolated RS, 111; syndromic RS, 21) with PEBP. All infants with isolated RS were discharged within an average of 8 days of PEBP therapy. For them, no tracheotomy or tongue-lip adhesion procedures were needed. Only 4 of the 20 infants discharged with a nasogastric tube needed it for >2 weeks. Intravelar veloplasty and palate closure were performed after 3 and 6 months of initiating PEBP treatment, respectively. CONCLUSIONS: Application of an orthodontic device in RS therapy has not been accepted worldwide. We hope that our learning curve and recommendations about PEBP will help the implementation of this highly effective and nonsurgical treatment option.

Differential diagnosis and management of adult-onset laryngomalacia.

OBJECTIVE: Adult-onset laryngomalacia is a rare clinical entity that has been infrequently reported. This study aims to evaluate the clinical presentation, diagnosis, and management of adult-onset laryngomalacia through literature review and report of a case. METHODS: PubMed and Google Scholar databases were queried for articles published from 1960 to 2019 including only patients aged 18 years and older. Included keywords were: 'laryngomalacia', 'adult laryngomalacia', 'acquired laryngomalacia', 'idiopathic laryngomalacia', 'laryngeal obstruction', 'floppy epiglottis', 'floppy epiglottis', and 'epiglottis prolapse'. Data extracted from literature included clinical presentation, diagnostic workup, surgical management, and follow-up care. SOURCES: PubMed and Google Scholar. RESULTS: A total of 21 articles reported 41 cases of adult-onset laryngomalacia. Within these cases, 5 etiologies were identified: neurologic (n = 14), exercise-induced (n = 9), post-operative (n = 7), idiopathic (n = 7), and age-related (n = 4) laryngomalacia. Anterior prolapse of arytenoids and aryepiglottic folds was the most common laryngoscopic finding (n = 21), followed by posterior epiglottic prolapse (n = 20). Management included supraglottoplasty (n = 14), epiglottidectomy (n = 8) or epiglottopexy (n = 2). Neurologic etiology required tracheotomy more often than the other etiologies (n = 5, 36% vs. 15%). Three patients were managed expectantly without surgical intervention and reported symptom resolution. CONCLUSION: Adult laryngomalacia is a rare diagnosis comprising a spectrum of disease. This diagnosis may be overlooked, but association with neurologic injury or trauma should encourage consideration. In comparison to pediatric laryngomalacia, patients often require surgical intervention. Surgical decision is based on the direction of supraglottic collapse, where supraglottoplasty and partial epiglottidectomy are effective interventions. LEVEL OF EVIDENCE: N/A.

Minimally invasive procedure for diagnosis and treatment of vallecular cysts in children: review of 156 cases.

PURPOSE: Vallecular cyst is an uncommon but potentially life-threatening disease. The aim of this study was to review the presentation, evaluation, and treatment of vallecular cysts in children. METHODS: Medical records of 156 patients treated for vallecular cysts between 2013 and 2016 were retrospectively reviewed. The patients were divided into four age groups for comparison of clinical data: A, < 1 month; B, 1-6 months; C, 7-12 months; and D, > 1 year. RESULTS: The median age of all patients (98 males and 58 females) was 12.1 months (range 1 day-11 years), including 21, 86, 21, and 28 patients in group A, B, C, and D, respectively. A diagnosis of vallecular cysts was made for 135 patients using a combination of flexible laryngoscopy and ultrasound, and ten patients (all in group A) required pre-surgery ventilation support. The most common symptoms were wheezing (59.6%) and stridor (36.5%). Ten patients experienced difficulty with intubation. Endoscopic-assisted transoral coblation marsupialization was performed for all patients, combined with supraglottoplasty for 41 out of 68 patients with concurrent laryngomalacia. Patients in group D had a longer operation time and higher incidence of intraoperative bleeding, two of whom experienced post-operation recurrence, and symptoms resolved after a second operation in both cases. CONCLUSIONS: Flexible laryngoscopy and ultrasound are recommended for a diagnosis in suspected cases of vallecular cysts. Coblation marsupialization has advantages of minor damage, low recurrence rate, and suitability for all age groups.

Prematurity and associated future paediatric airway pathology: experience from a single tertiary paediatric ENT centre.

PURPOSE: Enhanced management of the pre-term patient has resulted in improved survival rates in increasingly premature patients. Although prematurity predisposes to congenital airway pathology, there is also increased risk of endotracheal intubation, and therefore acquired subglottic pathology. We sought to evaluate airway pathology in children outside the neonatal period with a history of prematurity to explore the relationship between prematurity and upper airway pathologies. METHODS: Data for patients undergoing elective microlaryngobronchoscopy (MLB) at our centre were collected prospectively over a 5-year period. Patients identified as premature were sub-classified by the grade of prematurity. RESULTS: 339 patients over 1 month of age underwent MLB, of which 56 (16.5%) were born prematurely. Of those with identified airway pathology, 49 (23.4%) were born prematurely, accounting for 32.6% of subglottic stenosis (n = 30), 24% of laryngomalacia (n = 13) and 19% of laryngeal cleft diagnoses (n = 16). 49 premature patients (87.5%) had one or more airway pathologies diagnosed. Multi-level airway pathology was seen in twelve premature infants (21.4%), demonstrating a statistically significant association (odds ratio 3.396; 95% CI 1.697-6.842; p value < 0.0016). Incidence of airway pathology, the severity of airway disease and multi-level airway pathology were not related to the grade of prematurity. CONCLUSIONS: Premature patients account for a significant proportion of the workload within our tertiary centre due to improving neonatal care and survival in pre-term infants. We suggest early paediatric ENT evaluation for ex-premature patients with symptoms of airway pathology, with a low threshold for MLB. Improving neonatal survival rates in ever-increasing prematurity will require the further provision of specialist paediatric ENT services to manage their ongoing care.

Validity of laryngomalacia classification systems: A multi-institutional agreement study.

OBJECTIVES: To determine the agreement of paediatric otolaryngologists on classifying laryngomalacia (LM). DESIGN: Intra- and interobserver agreement study of two classification systems. SETTING: Three tertiary referral paediatric centres. PARTICIPANTS: Three paediatric otolaryngologists, who were blinded to any clinical details, interpreted the videos of children diagnosed with LM using the Holinger and Olney classifications independently. They rated the videos twice with a washout period of at least 2 weeks. THE MAIN OUTCOME MEASURES: Inter- and intra-observer agreement measured by overall Fleiss kappa and unweighted Cohen's kappa coefficients. The secondary outcome measures were inter- and intra-observer agreement on the individual anatomical subunits of the supraglottis affected by LM, characterised by the subcategories of the classifications. RESULTS: Video records of infants and children <18 years who had an endoscopic diagnosis of LM from 2012 to 2017 were retrospectively chosen for inclusion (n = 73). The overall Fleiss kappa coefficient was 0.25 (95% CI 0.18-0.32) amongst the raters using the Holinger classification and 0.31 (95% CI 0.21-0.42) for the Olney classification. Intra-observer agreement using the Holinger classification was 0.30 (95% CI 0.18-0.42), 0.62 (95% CI 0.23-0.85) and 0.84 (95% CI 0.75-0.94], whilst the Olney classification yielded values of 0.41 (95% CI 0.26-0.56), 0.51 (95% CI 0.29-0.63) and 0.63 (95% CI 0.48-0.78). CONCLUSIONS: The agreement on types of LM between expert observers is modest using the Holinger and Olney classifications. This has significant implications for accurately diagnosing LM and exposes potential obstacles against credible pooling of data and extrapolation of information.

Magnetic resonance imaging of obstructive sleep apnea in children.

Sleep-disordered breathing has a spectrum of severity that spans from snoring and partial airway collapse with increased upper airway resistance, to complete upper airway obstruction with obstructive sleep apnea during sleeping. While snoring occurs in up to 20% of children, obstructive sleep apnea affects approximately 1-5% of children. The obstruction that occurs in obstructive sleep apnea is the result of the airway collapsing during sleep, which causes arousal and impairs restful sleep. Adenotonsillectomy is the first-line treatment of obstructive sleep apnea and is usually effective in otherwise healthy nonsyndromic children. However, there are subgroups in which this surgery is less effective. These subgroups include children with obesity, severe obstructive sleep apnea preoperatively, Down syndrome, craniofacial anomalies and polycystic ovarian disease. Continuous positive airway pressure (CPAP) is the first-line therapy for persistent obstructive sleep apnea despite previous adenotonsillectomy, but it is often poorly tolerated by children. When CPAP is not tolerated or preferred by the family, surgical options beyond adenotonsillectomy are discussed with the parent and child. Dynamic MRI of the airway provides a means to identify and localize the site or sites of obstruction for these children. In this review the authors address clinical indications for imaging, ideal team members to involve in an effective multidisciplinary program, basic anesthesia requirements, MRI protocol techniques and interpretation of the findings on MRI that help guide surgery.

Supraglottoplasty in children with laryngomalacia: A review and parents' appraisal.

PURPOSE: To assess the parents' perspective concerning the children's clinical picture before and after supraglottoplasty for the treatment of laryngomalacia (LM). MATERIALS AND METHODS: Retrospective study in 110 children diagnosed with LM followed at the Pediatric Otorhinolaryngology outpatient clinics of S. João Hospital Center, between 2008 and 2016. Children who underwent supraglottoplasty were reviewed in terms of demographics, symptoms, comorbidities, treatment and follow-up. Parents were interviewed and filled out a structured questionnaire designed to evaluate their perception of the child's clinical picture and their degree of comfort before and after surgery. RESULTS: Thirty-one children (28,2%) underwent supraglottoplasty at a median age of 6 months-old. Twelve patients had one or more medical comorbidities. Stridor was present in all children on the pre-operative period and resolved in 92,3% of the cases after supraglottoplasty; shortness of breath persisted in 3,8% in contrast to the previous 57,7%; and feeding difficulties remained in 15,4% children against the 65,4% before the procedure. Failure in thriving was also a pre-operative complaint, that recovered as reported by parents in all children after supraglottoplasty. No surgical complications were reported, and the median hospital stay was two days. In a 0 to 10 points scale, the median level of the parents' comfort with their child's clinical picture before supraglottoplasty was one point which was significantly worse than the mean level of ten points after surgery (p < 0.001). CONCLUSIONS: In severe cases, LM can have a strong negative impact on family dynamics and functioning. In selected cases, supraglottoplasty can be a safe and effective treatment option which is associated with a high degree of parental satisfaction.