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

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

Filamin A is an actin-binding protein and, in humans, is encoded by FLNA gene in the long arm of X chromosome. Filamin A plays a role in the formation of cytoskeleton by crosslinking actin filaments in cytoplasm. FLNA mutations affect cytoskeletal regulatory processes and cellular migrating abnormalities that result in periventricular heterotopia. A 5-month-old girl was hospitalized because of breathing difficulty and was diagnosed as having periventricular heterotopia with laryngomalacia, cricopharyngeal incoordination, pulmonary hypertension, and chronic lung disease. A genetic test was performed to find the cause of periventricular heterotopia, and FLNA gene mutation (c.5998+1G>A) was confirmed for the first time in Korea. After discharge, she developed respiratory failure due to a viral infection at 8 months of her age. In spite of management with mechanical ventilation, she died of pneumothorax and pulmonary hemorrhage. Herein, we report a case of FLNA gene mutation who presented with periventricular nodular heterotopia with respiratory insufficiency.
Actin Cytoskeleton ; Arm ; Ataxia ; Cytoplasm ; Cytoskeleton ; Female ; Filamins ; Hemorrhage ; Humans ; Hypertension, Pulmonary ; Infant ; Korea ; Laryngomalacia ; Lung Diseases ; Periventricular Nodular Heterotopia ; Pneumothorax ; Respiration ; Respiration, Artificial ; Respiratory Insufficiency ; X Chromosome

Laryngomalacia is the most common congenital anomaly that causes inspiratory stridor and airway obstruction in the newborn. Symptoms begin to appear after weeks of age, become worse at 4-8 months, improve between 8-12 months, and usually heal naturally at 12-18 months. Despite these common natural processes, the symptoms of the disease can be very diverse and, in severe cases, require surgical treatment. The diagnosis can be made by suspicion of clinical symptoms and direct observation of the larynx with the spontaneous breathing of the child. Typical laryngeal features include omega-shaped epiglottis, retroflexed epiglottis, short aryepiglottic fold, poor visualization of the vocal folds, and edema of the posterior glottis, including inspiratory supra-arytenoid tissue prolapse. In this review, we discuss the classification and treatment based on symptoms and laryngoscopic findings in patients with laryngomalacia.
Airway Obstruction ; Child ; Classification* ; Comorbidity ; Diagnosis ; Edema ; Epiglottis ; Glottis ; Humans ; Infant, Newborn ; Laryngomalacia* ; Larynx ; Prolapse ; Respiration ; Respiratory Sounds ; Vocal Cords

OBJECTIVES: Diagnosis and clinical presentation of pediatric laryngopharyngeal reflux (LPR) is still controversial. The aims of this work were to study the possibility of performing 24-hour oropharyngeal pH monitoring for children in the outpatient clinic setup and to explore the results of this test in correlation to airway-related problems. METHODS: In this descriptive qualitative study, 26 children suffering from airway-related problems were included. Oropharyngeal 24-hour pH monitoring was performed for all subjects in the outpatient clinic setting. The distribution of airway diagnoses among the study group was studied versus the results of the pH monitoring. RESULTS: There were 16 males and 10 females participated in the study with a mean age of 6.88 (SD, ±5.77) years. Thirty-five percent of the patients were under the age of 3 years (range, 11 months to 3 years). Eight-five percent of the patients tolerated the pH probe insertion and completed 24-hour of pH recording. Laryngomalacia and subglottic stenosis (SGS) were more frequently reported in the positive LPR patients (77%). CONCLUSION: Oropharyngeal 24-hour pH monitoring can be conducted for children in the outpatient setup even in young age children below 3 years old. Among the positive LPR group, SGS and laryngomalacia were the most commonly reported airway findings.
Airway Obstruction ; Ambulatory Care Facilities ; Child* ; Constriction, Pathologic ; Diagnosis ; Female ; Humans ; Hydrogen-Ion Concentration* ; Laryngomalacia ; Laryngopharyngeal Reflux ; Male ; Outpatients ; Pediatrics

BACKGROUND AND OBJECTIVES: Adequate evaluation of the upper airway is critical in the management of pediatric patients with stridor or extubation failure. For this purpose, we designed an evaluation protocol of the upper airway for these patients, in collaboration with Dept. of Pediatrics, Intensive care team and Anesthesiology. Here we present the clinical results of our evaluation protocol and provide information about the etiology and management of the upper airway problems. SUBJECTS AND METHOD: Clinical data of 380 pediatric patients (M:F=231:149) having airway evaluation for their problems (stridor or extubation failure) were retrospectively analyzed. Among them, patients of age less than 3 months ranked first (30.0%). Comorbidities of pulmonary diseases (30.8%) and cardiovascular diseases (29.5%) were found. The pre and post-evaluation diagnosis, management and prognosis were evaluated and the usefulness of an airway evaluation protocol was discussed. RESULTS: Frequent pre-evaluation diagnoses were subglottic stenosis (55.2%), laryngomalacia (12.6%) and tracheal stenosis (9.2%) and these were changed to subglottic stenosis (44.5%), laryngomalacia (9.7%), tracheal stenosis (6.6%) and no abnormality (14.5%). Particularly, 50% of pre-diagnosis laryngomalacia, 25% of subglottic stenosis and 37% of tracheal stenosis were corrected to other causes by airway evaluation. The procedures were exam only (41.6%), endoscopic dilatation (20.8%) and tracheostomy (17.9%). In 190 out of 380 (50.0%), extubation was successful, but 151 patients (39.7%) had tracheostomy tube. CONCLUSION: Adequate evaluation of the upper airway in pediatric patients with stridor or extubation failure can facilitate the diagnosis and management of their problems.
Anesthesiology ; Cardiovascular Diseases ; Comorbidity ; Constriction, Pathologic ; Cooperative Behavior ; Diagnosis ; Dilatation ; Humans ; Critical Care ; Laryngomalacia ; Lung Diseases ; Pediatrics ; Prognosis ; Respiratory Sounds* ; Retrospective Studies ; Tracheal Stenosis ; Tracheostomy

Laryngomalacia is reported as the most common cause of congenital laryngeal stridor. Despite its benign and self-limited aspects, 10% of all cases require intervention. However, endolaryngeal surgery in neonates makes great demands on the anesthesiologist and the surgeon because of the narrowness of the airways. This case report will present a surgical management of severe laryngomalacia in a newborn using the combination carbon dioxide laser and cold instrument to surgically divide the aryepiglottic fold under conventional ventilation.
Humans ; Infant, Newborn ; Intubation, Intratracheal ; Laryngomalacia* ; Lasers, Gas ; Respiratory Sounds ; Ventilation*

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.
Child, Preschool ; Female ; Glottis ; Humans ; Infant ; Laryngomalacia ; congenital ; surgery ; Male ; Retrospective Studies ; Treatment Outcome

Although laryngomalacia is the most common cause of congenital stridor in infancy, it is a benign, self-limited disease which usually resolves by the age of 18 months with no long- term sequelae. Nevertheless, infants who suffered from recurrent stridor and respiratory distress should be evaluated for other causes of stridor such as cardiopulmonary anomalies. We report 3 cases of infants who had recurrent stridor and respiratory distress from their early infancy. Case 1 had a double aortic arch and a tracheomalacia, case 2 had a hypoplasia of the right lung, and case 3 had a horseshoe lung as well as scimitar syndrome. Physicians should be alert for the possibility of the congenital cardiopulmonary abnormalities in infants with recurrent stridor and/or respiratory distress.
Aorta, Thoracic ; Dyspnea ; Humans ; Infant ; Laryngomalacia ; Lung ; Respiratory Sounds ; Scimitar Syndrome ; Tracheomalacia

Joubert syndrome is a rare autosomal recessive condition in which there is complete or partial agenesis of the cerebellar vermis. The clinical features are hypotonia, ataxia, mental retardation, abnormal ocular movements, typical facial features and episodic tachypnea with alternating apnea. The abnormal respiratory pattern and hypotonia may be exacerbated by anesthetics, and especially, the apneic episodes may be prolonged by administering opioids. Joubert syndrome has been associated with various airway abnormalities such as a high and arched palate, a large or protruding tongue, laryngomalacia, a variable epiglottis and micrognathia. These abnormalities may cause difficulty with tracheal intubation. In this present case, direct laryngoscope-assisted, fiberscopic guided intubation was performed. This case is the first report of anesthetic management in a patient with Joubert syndrome and who underwent palatoplasty in Korea.
Analgesics, Opioid ; Anesthetics ; Apnea ; Ataxia ; Cerebellar Diseases ; Epiglottis ; Eye Abnormalities ; Humans ; Intellectual Disability ; Intubation ; Kidney Diseases, Cystic ; Korea ; Laryngomalacia ; Muscle Hypotonia ; Palate ; Retina ; Tachypnea ; Tongue