Causas de estridor congénito / Causes of congenital stridor

INTRODUCTION. Airway abnormalities are rare but potentially fatal. Stridor is a res-piratory noise with greater predominance in the inspiratory phase. OBJECTIVE. To evaluate the etiology of stridor, determine its comorbidities and mortality. MATERIALS AND METHODS. Retrospective cross-sectional study. Population of 110 and sample of 33 data from the Medical Records of neonatal or infant patients who presented stri-dor at the Carlos Andrade Marín Specialties Hospital of Quito-Ecuador, from january 2009 to december 2020. RESULTS. The 51,51% (17; 33) of cases were men. The age of the first consultation for stridor was within the first month in 18,00% (6; 33) and 40,00% (13; 33) at 3 months. The most frequent congenital laryngeal patholo-gy was: laryngomalacia 81,82% (27; 33), followed by subglottic stenosis 9,09% (3; 33), bilateral chordal paralysis 6,06% (2; 33) and tracheal stenosis 3,03% (1; 33). The 51,51% (17; 33) presented comorbidities of causes: neurological, pulmonary and genetic among the main ones. Mortality was 18,20% (6; 33) related to the severity of comorbidities, except one secondary to tracheal stenosis. CONCLUSION. Laryn-gomalacia and subglottic stenosis were the predominant pathologies with congenital stridor. The comorbidities that occurred were neurological, pulmonary, genetic and caused mortality within 90 days after diagnosis.

INTRODUCCIÓN. Las anomalías de la vía aérea son poco frecuentes, pero potencialmente mortales. El estridor es un ruido respiratorio con mayor predominio en la fase inspiratoria. OBJETIVO. Evaluar la etiología del estridor, determinar sus comorbilidades y la mortalidad. MATERIALES Y MÉTODOS. Estudio transversal retrospectivo. Población de 110 y muestra de 33 datos de Historias Clínicas de pacientes neonatos o lactantes que presentaron estridor en el Hospital de Especialidades Carlos Andrade Marín de Quito - Ecuador, de enero 2009 a diciembre 2020. RESULTADOS. El 51,51% (17; 33) de casos fueron hombres. La edad de la primera consulta por estridor fue dentro del primer mes en el 18,00% (6; 33) y del 40,00% (13; 33) a los 3 meses. La patología congénita laríngea más frecuente fue: laringomalacia 81,82% (27; 33), seguida de estenosis subglótica 9,09% (3; 33), parálisis cordal bila-teral 6,06% (2; 33) y estenosis traqueal 3,03% (1; 33). El 51,51% (17; 33) presentaron comorbilidades de causas: neurológica, pulmonar y genética entre las principales. La mortalidad fue 18,20% (6; 33) relacionada con la severidad de las comorbilidades, excepto una secundaria a estenosis traqueal. CONCLUSIÓN. La laringomalacia y la estenosis subglótica fueron las patologías que predominaron con estridor congénito. Las comorbilidades que se presentaron fueron neurológica, pulmonar, genética y causaron mortalidad dentro de los 90 días posteriores al diagnóstico.

Identification of a deep intronic POLR3A variant causing inclusion of a pseudoexon derived from an Alu element in Pol III-related leukodystrophy.

Pseudoexon inclusion caused by deep intronic variants is an important genetic cause for various disorders. Here, we present a case of a hypomyelinating leukodystrophy with developmental delay, intellectual disability, autism spectrum disorder, and hypodontia, which are consistent with autosomal recessive POLR3-related leukodystrophy. Whole-exome sequencing identified only a heterozygous missense variant (c.1451G>A) in POLR3A. To explore possible involvement of a deep intronic variant in another allele, we performed whole-genome sequencing of the patient with variant annotation by SpliceAI, a deep-learning-based splicing prediction tool. A deep intronic variant (c.645 + 312C>T) in POLR3A, which was predicted to cause inclusion of a pseudoexon derived from an Alu element, was identified and confirmed by mRNA analysis. These results clearly showed that whole-genome sequencing, in combination with deep-learning-based annotation tools such as SpliceAI, will bring us further benefits in detecting and evaluating possible pathogenic variants in deep intronic regions.

Congenital laryngomalacia: Is it an inflammatory disease? The role of vitamin D.

OBJECTIVES/HYPOTHESIS: Laryngomalacia is the most common cause of stridor in infants. The exact pathophysiology is still not well understood. Our objective was to investigate whether laryngomalacia is an inflammatory disease, focusing on the possible role of vitamin D. STUDY DESIGN: Case-control study. METHODS: Sixty Egyptian infants and 60 mothers were included in this study. They were divided into four equal groups (n = 30 for each): infants with laryngomalacia (LM-infants), control infants (C-infants), mothers of the infants with laryngomalacia (LM-mothers), and mothers of the control infants (C-mothers). Laryngoscopy was performed and serum 25-hydroxyvitamin D (25[OH]-vitamin-D) and interleukin 6 (IL-6) were estimated. RESULTS: Significant increase of serum IL-6 associated with a significant decrease in serum 25(OH)-vitamin D was observed in the LM-infants compared to the C-infants (P < .001 for both). LM-mothers had significantly lower 25(OH)-vitamin D status compared to C-mothers (P < .001). CONCLUSIONS: Deficiency of 25(OH)-vitamin D in LM-infants may result in dysregulation of the immune responses with elevation of a proinflammatory cytokine (IL-6). Laryngomalacia could be an inflammatory disease due to 25(OH)-vitamin D deficiency as evidenced by the high level of IL-6. This finding may open the door to the appropriate prevention, diagnosis, and treatment, especially for moderate to severe laryngomalacia. LEVEL OF EVIDENCE: 3b Laryngoscope, 130:448-453, 2020.

A novel technique for unilateral supraglottoplasty.

OBJECTIVES: Traditional supraglottoplasty for pediatric laryngomalacia is most commonly conducted with either CO2 laser or cold steel instruments. While the procedure enjoys high success rates, serious complications such as excessive bleeding, supraglottic stenosis and aspiration can occur. Unilateral coblation supraglottoplasty may reduce this risk, but data on respiratory and swallowing outcomes are lacking. This study reports our experiences with unilateral coblation supraglottoplasty. METHODS: Pediatric patients with severe congenital laryngomalacia who underwent unilateral supraglottoplasty at a single institution from 2013 to 2016 were retrospectively reviewed. Bipolar radiofrequency ablation (Coblation) was utilized with partial arytenoidectomy, aryepiglottoplasty, and advancement of mucosal flaps. Outcome measures included apnea-hypopnea index (AHI), weight-by-age percentile, and decannulation rate. RESULTS: Twelve patients were included with an average age of 13.1 months (range 2-28 months). In patients without tracheostomy, 88% had complete resolution of respiratory symptoms, while the remainder had significant improvement. In patients without gastrostomy tubes, there was an average increase in weight-age percentile of 6.1, 7.8, and 15.3 points at 1, 3, and 6 months postoperatively, respectively. Three patients had complete polysomnography data with a mean preoperative AHI of 19.3 and postoperative AHI of 4.0. Three of four patients with tracheostomy have been decannulated at a mean follow-up of 1.5 years. There were no early or late postoperative complications and no revision supraglottoplasty. CONCLUSION: Unilateral supraglottoplasty with bipolar radiofrequency ablation can improve respiratory symptoms and decrease OSA severity in severe congenital laryngomalacia. This technique is safe and can lead to substantial improvement in AHI in patients with OSA.

Congenital laryngomalacia is related to exercise-induced laryngeal obstruction in adolescence.

OBJECTIVES: Congenital laryngomalacia (CLM) is the major cause of stridor in infants. Most cases are expected to resolve before 2 years of age, but long-term respiratory prospects are poorly described. We aimed to investigate if CLM was associated with altered laryngeal structure or function in later life. METHODS: Twenty of 23 (87%) infants hospitalised at Haukeland University Hospital during 1990-2000 for CLM without comorbidities and matched controls were assessed at mean age 13 years. Past and current respiratory morbidity was recorded in a questionnaire, and spirometry performed according to standard quality criteria. Laryngoscopy was performed at rest and continuously throughout a maximal treadmill exercise test (continuous laryngoscopy exercise test (CLE-test)), and scored and classified in a blinded fashion according to preset criteria. RESULTS: In the CLM group, laryngeal anatomy supporting CLM in infancy was described at rest in nine (45%) adolescents. Eleven (55%) reported breathing difficulties in relation to exercise, of whom 7 had similarities to CLM at rest and 10 had supraglottic obstruction during CLE-test. Overall, 6/20 had symptoms during exercise and similarities to CLM at rest and obstruction during CLE-test. In the control group, one adolescent reported breathing difficulty during exercise and two had laryngeal obstruction during CLE-test. The two groups differed significantly from each other regarding laryngoscopy scores, obtained at rest and during exercise (p=0.001 or less). CONCLUSIONS: CLM had left footprints that increased the risk of later exercise-induced symptoms and laryngeal obstruction. The findings underline the heterogeneity of childhood respiratory disease and the importance of considering early life factors.

Supraglottoplasty in Infants: A Staged Approach.

OBJECTIVE: While generally well tolerated for the treatment of severe laryngomalacia, bilateral supraglottoplasty has potential complications including supraglottic stenosis and aspiration. We report a more conservative staged supraglottoplasty in infants with severe laryngomalacia. METHODS: A retrospective review was performed of our patients who underwent staged supraglottoplasty from June 2007 to June 2012. Fifteen infants were identified and scored based on stridor, retractions, oxygen saturation, and feeding quality. Outcomes were compared with those reported in the literature for conventional bilateral supraglottoplasty. RESULTS: Seventy-three percent had significant improvement or resolution of stridor following the first stage of surgery and 100% in those undergoing a second stage. Twelve patients (80%) had mild to no retractions following one procedure and 100% had resolution after a second surgery. All 6 patients with recurrent preoperative desaturations had resolution after the first stage of surgery. Of the 11 infants who had preoperative moderate-severe feeding problems, 9 of them (82%) had resolution after one surgery and the remaining 2 had resolution after a second surgery. There were no complications in any of the patients. CONCLUSIONS: Staged supraglottoplasty appears to be an effective, low-risk method to treat severe laryngomalacia. A second procedure was only required in 40% of patients.

Congenital laryngeal anomalies.

INTRODUCTION: It is essential for clinicians to understand issues relevant to the airway management of infants and to be cognizant of the fact that infants with congenital laryngeal anomalies are at particular risk for an unstable airway. OBJECTIVES: To familiarize clinicians with issues relevant to the airway management of infants and to present a succinct description of the diagnosis and management of an array of congenital laryngeal anomalies. METHODS: Revision article, in which the main aspects concerning airway management of infants will be analyzed. CONCLUSIONS: It is critical for clinicians to understand issues relevant to the airway management of infants.

Congenital laryngeal anomalies / Anomalias congênitas da laringe

Introduction: It is essential for clinicians to understand issues relevant to the airway management of infants and to be cognizant of the fact that infants with congenital laryngeal anomalies are at particular risk for an unstable airway. Objectives: To familiarize clinicians with issues relevant to the airway management of infants and to present a succinct description of the diagnosis and management of an array of congenital laryngeal anomalies. Methods: Revision article, in which the main aspects concerning airway management of infants will be analyzed. Conclusions: It is critical for clinicians to understand issues relevant to the airway management of infants. .

Introdução: As anomalias congênitas da laringe estão em risco de uma via aérea instável, sendo essencial que o clínico tenha uma boa compreensão dos problemas relevantes para o manuseio das vias aéreas de bebês. Objetivos: Familiarizar os clínicos com os problemas relevantes para o manuseio das vias aéreas de bebês e apresentar uma descrição sucinta do diagnóstico e tratamento de uma série de anomalias congênitas da laringe. Método: Artigo de revisão, no qual serão tratados os principais aspectos relacionados ao manuseio das vias aéreas de bebês. Conclusões: É essencial que o clínico tenha um bom conhecimento dos problemas relevantes ao manuseio das vias aéreas de bebês. .

Supraglottoplasty outcomes in neurologically affected and syndromic children.

IMPORTANCE: Supraglottoplasty (SGP) failure is frequently attributed to coexistent medical comorbidities, but studies specifically evaluating outcomes in these populations are lacking. OBJECTIVE: To assess SGP outcomes in patients with neurologic and syndromic comorbidities and severe laryngomalacia (LM). DESIGN, SETTING, AND PARTICIPANTS: Case series with retrospective review of medical records of 54 patients with neurologic and/or syndromic comorbidity and severe LM who underwent SGP between 2004 and 2012 at a tertiary care pediatric institution. INTERVENTIONS: Patients presented with severe LM that required SGP. Supraglottoplasty failure necessitated revision SGP, tracheostomy, or gastrostomy tube insertion, or LM and obstructive sleep apnea that required assisted ventilation (continuous positive airway pressure and bilevel positive airway pressure). MAIN OUTCOMES AND MEASURES: Medical records were reviewed with a focus on patient factors, surgical timing, complications, and surgical and dysphagia outcomes. Patients were grouped based on their age at the time of SGP as infants (aged ≤12 months) and children (aged >12 months). Statistical comparisons were performed with SGP outcomes of infants with LM and no comorbidities. RESULTS: Fifty-four patients met the inclusion criteria. Thirty-one (13 infants, 18 children) had a neurologic condition and 23 (15 infants, 8 children) had syndromes. The overall success rate of SGP was 67% (36 of 54) in these populations. Neurologic (P = .003) and syndromic (P < .001) comorbidities were associated with significant reduction in SGP success rates vs no comorbidities. Among SGP failures (18 of 54 [33%]), 13% (7 of 54) required tracheostomy, 9% (5 of 54) needed assisted ventilation, 7% (4 of 54) required a postoperative gastrostomy tube, and 4% (2 of 54) required revision SGP. In the neurologic comorbidities group, patients with cerebral palsy had significantly higher tracheostomy rates compared with those who had other neurologic pathologies constituting comorbidities (2 of 11 [18%] vs 0 of 20; P = .049). In infants, acute airway obstruction was the most common indication for SGP in the neurologically comorbidity and syndrome populations (success rates, 69% and 67%, respectively). In children, obstructive sleep apnea was the most common indication for SGP in the neurologic comorbidity and syndrome populations (success rates, 78% and 50%, respectively). CONCLUSIONS AND RELEVANCE: Supraglottoplasty remains useful and outcomes were better in patients with neurologic comorbidity than in patients with syndromic comorbidity.

Medical and surgical management of congenital laryngomalacia: a case-control study.

OBJECTIVE: To compare the growth of infants with moderate to severe laryngomalacia who underwent supraglottoplasty to the growth of those treated with medical therapy alone. STUDY DESIGN: Case-control study of patients treated between 2008 and 2013. SETTING: Tertiary care pediatric otolaryngology practice. SUBJECTS AND METHODS: Fifty-one infants newly diagnosed with moderate to severe congenital laryngomalacia. Seventeen infants underwent supraglottoplasty and 34 matched controls had medical management, which included acid suppression therapy, speech and swallowing therapy, and/or high-calorie formula. The primary outcome measure was weight percentile recorded at the second clinic visit and at the last available follow-up. The secondary outcomes were the need for primary or revision supraglottoplasty, tracheostomy or gastrostromy, or the development of or persistence of failure to thrive. RESULTS: There was no difference in the mean weight percentile between the surgical and nonsurgical groups at the time of last follow-up (P = .89). The mean change in weight percentile during the study period was 32% (95% CI, 15%-48%) in the supraglottoplasty group and 31% (95% CI, 22%-40%) in the medical group (P = .97). Five of 5 (100%) patients with failure to thrive managed surgically and 10 of 10 (100%) managed medically were above the fifth percentile at the end of the follow-up period. One (3%) patient in the medical management group required tracheostomy and gastrostomy tube placement. CONCLUSIONS: Medical management and close observation of infants with moderate to severe congenital laryngomalacia may be a viable alternative to supraglottoplasty in appropriately selected infants.

Prevalence and risk factors for central sleep apnea in infants with laryngomalacia.

OBJECTIVE: To identify the prevalence of and risk factors for central sleep apnea (CSA) in infants who are diagnosed with laryngomalacia. STUDY DESIGN: Case series with chart review. SETTING: Quaternary care pediatric hospital. SUBJECTS AND METHODS: We performed a chart review in infants with laryngomalacia. All infants had diagnostic polysomnography (PSG) performed from 2003 to 2012. Infants who underwent supraglottoplasty or other upper airway surgery prior to PSG were excluded. CSA was defined as central apnea index ≥ 5. Demographic data, underlying diseases, and PSG data were reviewed and analyzed. RESULTS: Fifty-four patients met the inclusion criteria. The mean age at the date PSG was performed was 3.4 ± 2.7 months. The prevalence of CSA in infants with laryngomalacia was 46.3%. Odds ratio (OR) of CSA was above 2.0 in patients with the following risk factors: underlying neurologic disease, hypotonia, or syndrome (OR = 2.5, P = .13), history of apparent life-threatening events (OR = 2.7, P = .19), premature infants (OR = 2.2, P = .33), and age less than 3 months (OR = 2.3, P = .15). However, none of the risk factors were statistically significant. Analysis of sleep architecture revealed a decrease in total sleep time (345.4 ± 70.6 minutes vs 393.5 ± 68.3 minutes, P = .02) and sleep efficiency (67.7 ± 8.9% vs 75.2 ± 9.3%, P = .004) in the CSA group. CONCLUSION: CSA is relatively common in infants with laryngomalacia. There seems to be a higher prevalence of CSA in infants with certain risk factors, but none of the risk factors are statistically significant. The presence of CSA can lead to alteration in sleep architecture. In addition to clinical evaluation, polysomnography may be warranted for the evaluation of infants with laryngomalacia and associated complex medical conditions.

Prospective quality of life assessment in congenital laryngomalacia.

PURPOSE OF STUDY: Disturbances in breathing or feeding may profoundly affect parental perceptions of a newborn's health. Previous research into quality of life for patients with laryngomalacia is limited to retrospective analysis. The purpose of this study is to prospectively evaluate the quality of life of families of infants with laryngomalacia and the impact of surgical and non-surgical treatments. DESIGN AND METHOD: Pilot prospective analysis using the laryngomalacia quality of life (QOL) survey in families of infants with newly diagnosed laryngomalacia under age one year. A 29-question survey regarding severity of symptoms related to overall health, airway, and swallowing is completed at initial and post-treatment visits. Responses are quantified over a range from 1 to 5 (1 never to 5 always). RESULTS: Twenty-six families were enrolled in the study. Eleven patients were managed medically and fifteen underwent supraglottoplasty. The overall mean QOL score for patients treated medically was 2.57 (standard error, SE 0.16) on initial visit and 1.67 (SE 0.16) post-treatment (mean 3.9 months). Patients undergoing supraglottoplasty had an overall mean QOL score of 3.59 (SE 0.14) on initial visit and 2.22 (SE 0.22) post-treatment (mean 3.5 months). Analysis of variance (ANOVA) and post hoc testing revealed significant improvement between initial and follow-up visits in both treatment groups (p < 0.01). Patients who underwent supraglottoplasty had significantly higher scores at initial visit (p < 0.01). No statistically significant difference was noted between patient groups post-treatment (p > 0.05). CONCLUSIONS: Prospective QOL assessment of children with laryngomalacia and their families reveals a significant burden of disease. Quality of life improves in all patients but may improve more significantly in patients managed surgically.

Supraglottoplasty in premature infants with laryngomalacia: does gestation age at birth influence outcomes?

PURPOSE: Prematurity is thought to be to be an independent risk factor for supraglottoplasty (SGP) failure. The purpose of this study was to compare the outcomes of supraglottoplasty in premature infants with congenital laryngomalacia (LM) with that of term infants. STUDY DESIGN: Case series with chart review. SETTING: Tertiary-care pediatric institution. METHODS: Analysis was performed on 325 consecutive patients undergoing SGP between 2004 and 2012. Patients older than 12 months age or with syndrome, neurologic, or cardiac comorbidities were excluded. Resolution of airway symptoms after SGP was considered successful while revision SGP and tracheostomy were considered failures. The rates of secondary airway lesions (SAL), dysphagia, and gastrostomy tube (GT) placement were also compared. RESULTS: A total of 176 infants (136 term, 40 preterm) were identified. SGP was successful in 92.7% term and 90% preterm infants with no significant difference (P = .5865). Incidence of revision SGP and tracheostomy was similar among the groups. The outcomes of SGP were the same in all groups stratified by age. Incidence of SAL was significantly higher in preterm (72.5%) compared to term infants (34.6%, P = .0002). Dysphagia rates in the follow-up were higher in preterm (32.5%) versus term infants (6.6%, P < .001). The rate of GT insertion was significantly greater (P = .003) in preterm (27.5%) than term infants (6.6%). The preoperative dysphagia, persistent dysphagia, and GT in follow-up was significantly higher when gestation age <32 weeks (25%, P = .0168). CONCLUSION: Supraglottoplasty outcomes in term and preterm infants were similar irrespective of gestation age. Higher rates of dysphagia in follow-up are seen when gestation age <32 weeks.

[Anesthetic management for pediatric congenital laryngomalacia].

OBJECTIVE: To explore our experience of anesthetic management for pediatric congenital laryngomalacia operation. METHODS: A total of 27 pediatric patients with congenital laryngomalacia were treated at our hospital between December 2010 and November 2012. All patients were anesthetized by intravenous anesthesia of propofol-remifentanil and spontaneous breathing. Oxygen was insufflated at a rate of 4 L/min through an endotracheal tube near glottis. Propofol was set at a constant rate of 100 µg · kg(-1) · min(-1). The initial dose of remifentanil at 0.05 µg·kg(-1)·min(-1) was adjusted in 0.05 µg·kg(-1)·min(-1) increments to titrate a 50% reduction in baseline respiratory rate. Heart rate (HR), mean arterial pressure, pulse oxygen saturation (SpO2), respiratory rate (RR), operation time, anesthesia time and remifentanil rate were recorded. Adverse events and interventions were also examined. RESULTS: Comparison with induction of anesthesia, HR and RR changed significantly intraoperatively (P < 0.05). MAP, SpO2 were no significantly change during operation (P > 0.05). The induction time was 9-12 min and the highest remifentanil rate stood at (0.18 ± 0.03) µg·kg(-1)·min(-1). Body movements occurred in 3 (11%) patients and a bolus of propofol was administered. Desaturation below 95% occurred in 2 (7%) patients in which interventions were offered by decreasing the remifentanil infusion rate. No complications such as cough, hypoxemia, laryngospasm or bronchospasm, nausea or vomiting, arrhythmia were observed. CONCLUSION: Key points of anesthetic management for pediatric congenital laryngomalacia include sufficient preoperative evaluation, spontaneous respiration anesthesia technique with total intravenous anesthesia, suitable anesthesia depth and intensive intraoperative monitoring.

Pectus excavatum in children with laryngomalacia.

BACKGROUND: Laryngomalacia is the most common congenital laryngeal anomaly and is associated with several disorders including gastric reflux, sleep apnea, hypotonia and failure to thrive. Pectus excavatum (PE) is the most common chest wall deformity affecting 1-300/1000 individuals. Though many authors presume a relationship between PE and laryngomalacia, there is no published data to establish this association. GOAL: To test the hypothesis that patients referred to our pediatric otolaryngology clinic for evaluation of laryngomalacia exhibit higher rates of PE than the general population. METHODS: Retrospective review of prospectively enrolled children who presented with laryngomalacia (January 2008-June 2012) to a tertiary care, hospital based, pediatric otolaryngology practice. Each chart was examined for a concurrent diagnosis of pectus deformity. RESULTS: Of the 137 laryngomalacia patients, 9 (6.6%) had documented PE. This represents a significantly increased rate of PE when compared to children without laryngomalacia (p = 0.001). Four of the 9 children with PE underwent supraglottoplasty for laryngomalacia, a significantly greater proportion than the 9/128 of the children with isolated laryngomalacia who underwent supraglottoplasty (p = 0.004). CONCLUSIONS: This study suggests an association between laryngomalacia and PE. Pediatric otolaryngologists should be cognizant of this relationship, though further studies are needed to elucidate the nature of this association.

[Congenital laryngeal stridor - an interdisciplinary problem]. / Wrodzony stridor krtaniowy­ problem interdyscyplinarny.

UNLABELLED: The most common causes of laryngeal stridor are laryngomalacia (60%), vocal cord paralysis (VCP) (10 %) and subglottic laryngeal stenosis. Majority of cases of VCP are idiopathic, less frequently it is the effect of abnormalities in central nervous system (Arnold - Chiari syndrome, hydrocephalus, neonatal hypoxia). Differential diagnosis should also include anomalies of aortic arch and its branches (vascular rings). The authors present two cases of neonatal congenital laryngeal stridor. In the first case the girl presented with VCP of unknown etiology. The perinatal period was normal, ultrasound of central nervous system and neurologic examination revealed no abnormalities. Due to sustained VCP in control laryngeal ultrasound examinations, tracheostomy was performed in the third month of life. In the second case, stridor was caused by laryngomalacia and subglottic laryngeal stenosis of first grade according to Meyer-Cotton scale (larynx lumen diameter <4 mm). The diagnosis was established by laryngotracheobronchoscopy. In both children angiotomography was performed and vascular ring was diagnosed (aberrant right subclavian artery). Vascular anomaly was suspected in barium X-ray. In both cases echocardiographic examination did not visualize the fourth vessel of the aortic arch. Chest X- rays were normal. Both children had no symptoms caused by vascular ring. CONCLUSION: Diagnosis of congenital laryngeal stridor is an indication for complete evaluation to establish the cause of the airway obstruction. The differential diagnosis should include laryngomalacia, vocal cord paralysis, subglottic laryngeal stenosis, congenital anomalies of large vessels and abnormalities of the central nervous system.

[Surgical management for severe congenital laryngomalacia: 16 consecutive cases].

OBJECTIVE: Laryngomalacia is the most common cause for stridor in neonate and infant. Our study aims at assessing the outcome of surgical management in patients diagnosed by fibrolaryngoscope as congenital Laryngomalacia. METHOD: Retrospective study of 16 patients undergoing surgery for severe laryngomalacia. The patients' symptoms, associated medical conditions and surgical management were recorded. RESULT: Stridor and feeding difficulty were observed in 16 patients on admission, while dyspnea was found in 11 patients. Medical co-morbidities exist in 14 cases. The mean age of surgery was 23 weeks, 15 patients had follow-up records for 24 months. Fourteen cases underwent supraglortoplasty. Tracheotomy was performed on the other 2 cases complicated with tracheomalacia. Nine cases showed full recovery of stridor 48 hours post-supraglottoplasty, and 7 were free of feeding difficulties. In the 6 months follow-up, complete or partial relief was achieved in all main symptoms and signs. CONCLUSION: Supraglottoplasty is effective in relieving stridor and feeding difficulty as well as respiratory insufficiency, which makes it a first line option for managing severe laryngomalacia.

Flexible bronchoscopy as a valuable tool in the evaluation of infants with stridor.

The aim is to determine clinical characteristics, flexible bronchoscopy (FB) findings including associated airway abnormalities and other conditions, treatment modalities and long term follow-up of children with congenital stridor. Medical records of children, who underwent FB for the evaluation of stridor between 1 January 2004 and 31 December 2009 were retrospectively reviewed. Demographic characteristics, symptoms and physical examination findings at presentation, FB findings, follow-up data including the time to resolution of symptoms and treatment modalities, presence of associated conditions were assessed. 109 children were enrolled to the study. Laryngomalacia was the most common etiology for stridor. Laryngomalacia was isolated in 37 patients and 54 patients had secondary airway lesions (SALs). Diagnoses other than laryngomalacia such as subglottic hemangioma, subglottic web, isolated tracheomalacia were found in 18 patients. In 90 % of patients, stridor resolved before 3 years of age without any surgical intervention and there was no significant difference in terms of the persistence of stridor between patients with isolated laryngomalacia and associated SALs. Duration of stridor was significantly longer in both patients with neurological abnormalities and reflux symptoms. Surgical procedure was performed in 19 of the patients. There is a high incidence of SALs in patients with laryngomalacia. FB is helpful for identifying anomalies requiring surgical treatment.

[Epidemiological aspects of congenital stridor].

The objective of the present work was to study epidemiology of congenital stridor as a leading symptom of laryngeal malformation. The continuous sampling method was employed to perform the retrospective analysis of the growth charts of the patients attending three children's polyclinics in Moscow (9.625 patients born between 2005 and 2009). In addition, the medical histories of 4.623 newborn and breast-fed babies under the age of 1 year admitted to the Department of Newborn and Neonatal Pathology, Saint Vladimir City Children's Clinical Hospital, and 347 patients of the Department of Reconstructive Laryngeal Surgery were analysed. The children with the history of tracheal intubation in the preceding period were excluded from the study. The frequency of congenital stridor annually diagnosed in the aforementioned polyclinics varied from 0.17 to 5.8% compared with 1.5% in the general population. It was 2.21 to 3.14% (mean 2.47%) among the children treated at the Clinical Hospital. In the children under the age of 1 year, congenital malformations accounted for 90.8% of all laryngeal diseases. The principal cause of stridor was shown to be laryngomalacia. This pathology was diagnosed in 91.9% of the cases included in this study. In 11.2% of the patients, this condition occurred in combination with other congenital pathologies. It is concluded that the diagnosis of congenital stridor is an indication for laryngeal endoscopy regardless of the children's age starting from the first day of life. Meeting this recommendation allows the cause of stridor to be established and the treatment strategy to be developed on an individual basis.