{
 "topic": "Laryngomalacia",
 "slug": "laryngomalacia",
 "category_id": 15252,
 "summary": "Laryngomalacia: the omega-shaped epiglottis on laryngoscopy, typical timing and positional pattern of stridor, GERD/dysphagia associations, and when expectant management gives way to supraglottoplasty.",
 "written_by": "claude-sonnet",
 "references": [
  {
   "title": "Pediatric Otolaryngology (American Academy of Pediatrics) ( etc.)",
   "author": null,
   "pages": [
    356
   ]
  },
  {
   "title": "CURRENT Diagnosis and Treatment Pediatrics, Twenty-Fourth Edition",
   "author": "Hay, William W., Levin, Myron J., Deterding, Robin R., Abzug, Mark J.",
   "pages": [
    551
   ]
  },
  {
   "title": "Cover",
   "author": "Vitalsource Download",
   "pages": [
    814,
    7635
   ]
  },
  {
   "title": "Pediatric Pulmonology",
   "author": "American Academy of Pediatrics Section on Pediatric Pulmonology and Sleep Medicine;Michael J Light;Kristin Van Hook;",
   "pages": [
    272
   ]
  },
  {
   "title": "Gomella's Neonatology: Management, Procedures, On-Call Problems, Diseases, and Drugs, Eighth Edition",
   "author": "Tricia Lacy Gomella, Fabien G. Eyal and Fayez Bany-Mohammed",
   "pages": [
    1113
   ]
  },
  {
   "title": "Ghai Essential Pediatrics, 9e (Vinod K Paul, Arvind Bagga)",
   "author": "CamScanner",
   "pages": [
    375
   ]
  },
  {
   "title": "Kliegman R. Nelson Textbook of Pediatrics 2-Volume Set 22ed 2024",
   "author": null,
   "pages": [
    2591
   ]
  },
  {
   "title": "2021_Fleisher_&_Ludwig's_Textbook_of_Pediatric_Emergency_Medicine.epub",
   "author": null,
   "pages": []
  }
 ],
 "short": [
  {
   "title": "In short",
   "content": "- Laryngomalacia is the most common congenital laryngeal anomaly and the most common cause of stridor in infants, accounting for 45-75% of congenital laryngeal anomalies presenting with stridor (laryngotracheomalacia broadly accounts for 60-70% of neonatal/infant laryngeal problems).\n- Classic endoscopic findings: an omega-shaped epiglottis, short aryepiglottic folds, and prolapse of the arytenoid/cuneiform cartilage and mucosa into the airway during inspiration, causing supraglottic collapse from decreased laryngeal tone.\n- Onset is typically within the first 2-6 weeks of life; stridor is inspiratory, worsens with agitation, crying, feeding, supine positioning, and upper respiratory infections, and improves prone and at rest.\n- Symptoms typically worsen for up to about 6 months, then many infants show spontaneous improvement around 7-9 months, with the majority achieving complete resolution by 12-18 months (some cases persist longer).\n- Diagnosis is primarily clinical but confirmed by awake flexible fiberoptic laryngoscopy, which also helps rule out alternative or coexisting causes of stridor.\n- Gastroesophageal reflux disease (and laryngopharyngeal reflux) is commonly associated with laryngomalacia and can worsen its severity and clinical course; neurologic disease with hypotonia is another modifier of severity, and low Apgar scores at birth are associated with greater severity.\n- Laryngomalacia can cause feeding difficulty and dysphagia from decreased laryngeal sensation and poor suck-swallow-breath coordination; wet-sounding stridor, cough with feeds, or recurrent respiratory illness/pneumonia should prompt evaluation for dysphagia (contrast swallow study or FEES).\n- About 15-60% of infants with laryngomalacia have a synchronous airway anomaly, so complete bronchoscopy is performed for moderate-to-severe obstruction.\n- Most cases (roughly 78-90%, i.e., only about 10-22% require surgery) are managed expectantly with positioning and treatment of comorbid reflux; surgical supraglottoplasty (or rarely temporary tracheostomy) is reserved for significant airway obstruction, failure to thrive, or other severe/chronic signs (obstructive sleep apnea, hypoxemia, severe dyspnea).\n"
  }
 ],
 "long": [
  {
   "title": "Definition",
   "content": "Laryngomalacia is a benign congenital disorder in which the cartilaginous support of the supraglottic structures is underdeveloped, leading to collapse of these structures into the airway during inspiration. It is the most common congenital laryngeal anomaly and the most common cause of stridor in infants. The term laryngotracheomalacia has no clinical significance and should not be used, since laryngomalacia is a distinct entity from tracheomalacia, which is far less common.\n"
  },
  {
   "title": "Epidemiology",
   "content": "Laryngomalacia accounts for 45-75% of congenital laryngeal anomalies presenting with stridor in children, and laryngotracheomalacia broadly accounts for about 60-70% of laryngeal problems in neonates and infants.\n"
  },
  {
   "title": "Etiology",
   "content": "The underlying mechanism is decreased laryngeal tone leading to supraglottic collapse during inspiration, thought to relate to neurologic immaturity of the newborn. Gastroesophageal reflux disease (and laryngopharyngeal reflux) is a commonly associated condition that can worsen symptom severity and influence the clinical course, as can underlying neurologic disease with associated muscle hypotonia; lower Apgar scores at birth are associated with greater disease severity.\n"
  },
  {
   "title": "Clinical Features",
   "content": "Symptoms typically begin within the first 2-6 weeks of life (some sources cite onset as early as immediately after birth, more commonly at several weeks of age) and progress in severity for up to about 6 months before gradually improving. The hallmark is inspiratory stridor - high- to low-pitched, and classically worse with crying, agitation, feeding, supine positioning, and upper respiratory infection, improving with rest and prone positioning. Choking, cough, and regurgitation frequently occur during feeding, and laryngomalacia can contribute to feeding difficulty and dysphagia from decreased laryngeal sensation and poor suck-swallow-breath coordination. Severe cases can present with cyanosis, apnea, feeding difficulty, and failure to thrive. Most infants who do not require surgery show spontaneous improvement around 7-9 months of age, with the majority achieving complete resolution of stridor by 12-18 months, though symptoms can persist longer in some children. When stridor caused by laryngomalacia (or viral croup) fails to follow this expected improving course - worsening or persisting rather than improving with age - an alternate or coexisting laryngeal abnormality (such as a subglottic hemangioma, which can initially mimic laryngomalacia but tends to worsen as the lesion enlarges) should be considered.\n"
  },
  {
   "title": "Diagnostics",
   "content": "Diagnosis is primarily clinical but is confirmed by awake flexible fiberoptic laryngoscopy, which characteristically shows an omega-shaped epiglottis (tightly curled), short aryepiglottic folds, and prolapse of the arytenoid mucosa and cuneiform cartilage into the airway during inspiration, producing a flaccid, partially collapsing supraglottic airway. When work of breathing is moderate to severe, airway films and chest radiographs are indicated; oxygen saturation and chest radiographs are typically normal in isolated laryngomalacia despite the noisy upper-airway breathing. Cases presenting very early (under 4 weeks of age) or with severe obstruction should have early airway examination to exclude alternative causes requiring different treatment. When stridor sounds wet, is associated with cough, or there is a history of recurrent upper respiratory illness or pneumonia, dysphagia should be suspected and evaluated with a contrast swallow study and/or fiberoptic endoscopic evaluation of swallowing (FEES). Because 15-60% of infants with laryngomalacia have a synchronous airway anomaly, complete bronchoscopy is performed in patients with moderate to severe obstruction to look for these coexisting lesions.\n"
  },
  {
   "title": "Differential Diagnosis",
   "content": "Other causes of infantile stridor and airway obstruction include congenital (or acquired) vocal cord paralysis - unilateral paralysis typically causes a hoarse cry with minimal respiratory symptoms, while bilateral paralysis causes severe respiratory distress and may require tracheostomy, and should prompt consideration of a CNS anomaly such as Arnold-Chiari malformation; subglottic stenosis (congenital or acquired after airway manipulation), presenting with stridor, respiratory distress, or obstructive apnea; subglottic hemangioma, a vascular lesion that can engorge and obstruct with agitation and tends to worsen over time as it enlarges; and foreign body aspiration, generally an acute presentation rather than the gradually evolving course of laryngomalacia.\n"
  },
  {
   "title": "Treatment",
   "content": "Most cases of laryngomalacia are mild and managed expectantly with positioning and medical treatment of comorbidities such as GERD; expectant observation is appropriate for most infants since symptoms resolve spontaneously as the child and airway grow. Mildly affected patients without stridor at rest or retractions typically do not need treatment. Surgical intervention (supraglottoplasty, or rarely temporary tracheostomy) is reserved for significant airway obstruction or failure to thrive, and is required in only about 10-22% of cases depending on the series; indications include severe symptoms such as stridor with every breath, retractions, and increased work of breathing, or more chronic signs such as feeding difficulty, failure to thrive, obstructive sleep apnea, hypoxemia, or severe dyspnea.\n"
  },
  {
   "title": "Complications",
   "content": "While generally self-limited, severe laryngomalacia can cause life-threatening airway obstruction or failure to thrive if untreated. Severe airway obstruction is more likely when secondary lesions coexist, such as neuromuscular disorders, subglottic stenosis, or vascular rings.\n"
  }
 ],
 "clinical": [
  {
   "title": "Diagnosing and Triaging the Infant with Stridor",
   "content": "Suspect laryngomalacia in an infant with inspiratory stridor beginning in the first 2-6 weeks of life that worsens with crying, feeding, agitation, and supine positioning, and improves prone and at rest - this pattern, together with normal oxygen saturation and a normal chest radiograph, supports an upper-airway (supraglottic) source rather than lower respiratory disease. Confirm with awake flexible fiberoptic laryngoscopy, looking for the omega-shaped epiglottis, short aryepiglottic folds, and inspiratory prolapse of the arytenoid mucosa/cuneiform cartilage. Obtain early airway examination (rather than waiting) in infants presenting under 4 weeks of age or with severe obstruction, since these presentations warrant ruling out an alternative cause. If stridor sounds wet, is accompanied by cough with feeds, or there is a history of recurrent respiratory illness or pneumonia, evaluate for dysphagia with a contrast swallow study or FEES, since laryngomalacia can impair suck-swallow-breath coordination. For infants with moderate to severe obstruction, proceed to complete bronchoscopy, since 15-60% have a synchronous airway anomaly that would otherwise be missed.\n"
  },
  {
   "title": "Managing the Expected Course and Recognizing Red Flags",
   "content": "Reassure families that most laryngomalacia is mild, self-limited, and managed expectantly with positioning and treatment of any coexisting GERD, with symptoms typically peaking by about 6 months, improving from 7-9 months, and resolving completely by 12-18 months in the majority of infants. Screen for and treat reflux, since GERD/laryngopharyngeal reflux can worsen severity and prolong the clinical course. Escalate to surgical evaluation (supraglottoplasty) for stridor at rest with retractions and increased work of breathing, or for chronic signs such as failure to thrive, obstructive sleep apnea, hypoxemia, or severe dyspnea - these more severe presentations occur in only about 10-22% of cases. If stridor worsens or persists beyond the expected improving trajectory instead of following the typical course, reassess for an alternate or coexisting diagnosis (such as an enlarging subglottic hemangioma, vocal cord paralysis, or subglottic stenosis) rather than continuing to attribute symptoms to uncomplicated laryngomalacia.\n"
  }
 ]
}