Introduction
Laryngomalacia stands out as the most common cause of stridor in infants, making prompt and accurate Laryngomalacia Diagnosis in early infancy crucial. This condition can significantly impact various facets of an infant’s growth and overall development. Stridor, characterized by noisy breathing, serves as a key symptom that should alert clinicians to potential respiratory compromise. A comprehensive evaluation of the airway is indispensable for children suspected of having laryngomalacia. This thorough assessment is vital not only for confirming the diagnosis but also for implementing appropriate treatment strategies and addressing any co-existing health issues. Effective management of laryngomalacia often necessitates a multidisciplinary approach, involving collaboration across various medical specialties. This article offers a detailed overview of the current methods in laryngomalacia diagnosis and management, emphasizing the essential roles of an interprofessional team in delivering optimal patient care.
This discussion will highlight the spectrum of laryngomalacia severity, providing clarity on when a conservative approach of watchful waiting is adequate for milder cases, and when surgical intervention becomes necessary for more severe presentations. Clinicians will gain valuable insights into contemporary diagnostic tools and management protocols, underscoring the importance of a collaborative interprofessional team in achieving successful outcomes for infants diagnosed with laryngomalacia.
Objectives:
- Define the underlying causes and etiology of laryngomalacia.
- Distinguish between mild and severe laryngomalacia based on symptom severity and impact on respiratory function to refine laryngomalacia diagnosis.
- Apply evidence-based treatment protocols for laryngomalacia, adapting interventions to the specific severity of each case.
- Enhance coordination of care among the multiple specialties involved in laryngomalacia management, promoting an interprofessional framework to optimize patient outcomes in laryngomalacia diagnosis and treatment.
Etiology of Laryngomalacia
Several theories attempt to explain the causes of laryngomalacia, but they all converge on the same outcome: the soft, underdeveloped cartilage of an infant’s larynx collapses during inspiration, leading to inspiratory stridor, the hallmark noisy breathing. Neurological dysfunction is a leading etiological theory, proposing that laryngomalacia arises from abnormal neural control of laryngeal tone due to impaired integration within the laryngeal nerves.[1] This theory is bolstered by pathological studies demonstrating increased supraglottic nerve diameter in severe laryngomalacia cases.[2] Other theories, such as the imbalance between respiratory demand and airway capacity during infant inhalation, require further research.[3] While gastroesophageal reflux (GERD) is not considered a direct cause of laryngomalacia, it is a common comorbidity, affecting approximately 60% of infants with laryngomalacia.[4] GERD is believed to exacerbate airway obstruction by causing irritation and edema in the upper airway. Symptomatic laryngomalacia is also more prevalent in infants with neuromuscular disorders, where generalized hypotonia affects airway muscles, diminishing inspiratory strength.[5]
Epidemiology of Laryngomalacia
The exact incidence of laryngomalacia within the general population remains uncertain, with estimates ranging from 1 in 2000 to 1 in 3000 live births.[6][7] It’s possible that this is an underestimation, as milder cases are often managed clinically by pediatricians without endoscopic confirmation. While earlier data suggested a higher prevalence in males, recent studies indicate that laryngomalacia affects both sexes equally. Infants of Black and Hispanic ethnicity may face a higher risk compared to White infants.[7] Factors such as low birth weight, prematurity, and admission to the neonatal intensive care unit (NICU) after birth have also been identified as correlating factors.[7]
Pathophysiology of Laryngomalacia
Laryngomalacia is recognized as a common congenital condition that affects the structural integrity of the infant larynx. The primary pathophysiology behind laryngomalacia is an inherent weakness in the laryngeal tissues, particularly in the supraglottic region. This weakness causes the normally firm laryngeal structures to fold inwards during inspiration. This dynamic collapse leads to airway obstruction and the characteristic stridor associated with laryngomalacia (see Image. Laryngomalacia). The specific anatomical features contributing to this collapse include increased flexibility or redundancy of the epiglottis, aryepiglottic folds, and surrounding tissues. This structural vulnerability can result in intermittent airway obstruction, especially during periods of increased respiratory effort. Although laryngomalacia is often self-resolving as the infant’s laryngeal structures mature, severe cases may require medical intervention to alleviate symptoms and ensure adequate respiratory function.
History and Physical Examination in Laryngomalacia Diagnosis
A detailed birth history is crucial for laryngomalacia diagnosis, including any surgical interventions or intubations the infant has undergone and any periods of NICU stay. Parents should provide detailed information about breathing difficulties observed at home, specifically noting noisy breathing or episodes of apnea. Noisy breathing that worsens during feeding or when the infant is supine is particularly indicative of laryngomalacia. Clinicians should inquire about feeding and sleeping patterns, and note any weight loss, gastroesophageal reflux, recurrent pneumonia, or failure to thrive, as these can be indicators of the severity of laryngomalacia.
A thorough physical examination of the infant is essential, focusing particularly on the oral cavity, nose, and neck. It is important to confirm choanal patency and rule out piriform aperture stenosis. A comprehensive examination of the oral cavity should assess for cleft lip or palate, glossoptosis, Pierre-Robin sequence, or micrognathia, as these conditions may exacerbate breathing and feeding difficulties.[8] Observing the infant during feeding, both in sitting and supine positions, can provide valuable insights.
Examination of the neck should assess for retractions during breathing and the presence of any masses or vascular lesions. Chest auscultation and inspection for subcostal retractions are also necessary. Special attention should be paid to hemangiomas in a beard-like distribution, as these are associated with potential airway hemangiomas.
For proper laryngomalacia diagnosis in a patient with suspected laryngomalacia, a supraglottic airway assessment using flexible laryngoscopy in the awake infant is necessary. In cases presenting with severe symptoms, the infant should undergo diagnostic laryngoscopy and bronchoscopy in the operating room, with the possibility of immediate endoscopic intervention.[9]
Evaluation and Diagnostic Modalities for Laryngomalacia
Flexible fiberoptic laryngoscopy is the cornerstone in the diagnostic process for infant stridor and laryngomalacia diagnosis. This procedure allows for direct visualization of the upper aerodigestive tract during respiration. A complete examination offers the practitioner a clear view of the oropharynx, supraglottis, glottis, subglottis, and hypopharynx. Infants with laryngomalacia often present with characteristic findings such as shortened aryepiglottic folds that draw the epiglottis posteriorly, typically described as an omega-shaped epiglottis, and/or redundant arytenoid tissue that can prolapse over the glottis (see Image. Laryngoscopic View of Laryngomalacia). Flexible fiberoptic laryngoscopy is currently considered the gold standard for laryngomalacia diagnosis due to its convenience and ability to directly observe the dynamic collapse of the supraglottic airway in an awake, breathing infant. It is important to note that approximately 5% of infants with laryngomalacia may also have concurrent structural lower airway pathology, which flexible laryngoscopy alone may not reliably detect.[10]
Direct laryngoscopy and diagnostic bronchoscopy, performed in the operating room, provide a comprehensive evaluation of the entire upper aerodigestive tract, extending down to the level of the bronchi (mainstem bronchi with rigid bronchoscopy, and segmental bronchi and beyond with flexible bronchoscopy). This procedure is essential for patients with severe symptoms or when synchronous airway lesions are suspected. Direct laryngoscopy also allows for immediate surgical intervention if warranted.
Radiological swallow studies, ideally with a speech therapist, may be indicated if there are concerns about aspiration or swallowing difficulties in conjunction with laryngomalacia. A modified barium swallow study is preferred in infants with laryngomalacia, as aspiration can be silent and not clinically apparent.[11]
Polysomnography is a valuable tool for quantifying obstructive sleep apnea in patients with laryngomalacia, particularly in older children, sometimes referred to as sleep-exclusive laryngomalacia, with an incidence of about 4%.[12] Diagnosis in these cases is confirmed by drug-induced sleep endoscopy. Supraglottoplasty may be beneficial in these patients to improve their apnea-hypopnea index (AHI).[12]
Airway fluoroscopy is not recommended for evaluating infant stridor in laryngomalacia diagnosis due to its low sensitivity and the increased exposure to ionizing radiation.[13]
Treatment and Management Strategies for Laryngomalacia
Conservative management is the primary approach for the majority of laryngomalacia cases. For mild to moderate stridor without feeding difficulties, observation following laryngomalacia diagnosis is typically recommended. Ongoing monitoring for appropriate weight gain and the development of severe symptoms is crucial.[14] Positional feeding techniques and thickened feeds can be helpful for infants experiencing feeding difficulties.[14] Symptoms typically resolve in most patients between 12 to 18 months of age without surgical intervention.
However, approximately 10% to 20% of infants with laryngomalacia will present with severe symptoms requiring surgical intervention.[15] Supraglottoplasty has become the initial surgical treatment of choice for these severe cases. Several techniques for supraglottoplasty exist, including laser, cold steel instruments, laryngeal microdebrider, or coblation. The most common indications for surgical intervention are worsening airway symptoms and failure to thrive.[16] The supraglottoplasty procedure should be tailored to the individual patient’s anatomy but may include dividing shortened aryepiglottic folds, removing redundant arytenoid mucosa, performing an epiglottopexy, or a combination of these techniques. Care must be taken to avoid the interarytenoid mucosa to prevent glottic stenosis, a potentially severe complication resulting from scarring in this area.
Supraglottoplasty has demonstrated significant effectiveness in reducing the duration of laryngomalacia symptoms.[17] Patients generally tolerate the procedure well and are monitored postoperatively in the hospital. Steroid administration, both during and after surgery, helps to reduce airway inflammation and is a standard component of pharmaceutical therapy.[16]
Differential Diagnosis in Laryngomalacia
The differential diagnosis for laryngomalacia should encompass other causes of infant stridor, including:
- Unilateral or bilateral vocal fold paralysis
- Laryngeal papillomatosis
- Subglottic hemangioma
- Subglottic stenosis
- Tracheomalacia or bronchomalacia
- Vascular ring
- Foreign body aspiration
Unilateral vocal fold paralysis often arises after surgical procedures in the thoracic cavity or neck, although it can also be congenital. A hoarse cry is common, and these infants may have feeding difficulties. Bilateral vocal fold paralysis typically presents with biphasic stridor and may necessitate tracheostomy in cases of significant respiratory distress. Flexible fiberoptic laryngoscopy aids in diagnosing these conditions.
Laryngeal papillomatosis can cause a hoarse cry with upper airway obstruction, potentially appearing early in infancy. Diagnosis is made through flexible fiberoptic laryngoscopy or direct laryngoscopy with lesion biopsy.
Subglottic hemangiomas, a rare cause of stridor, typically present with expiratory stridor. Hemangiomas in a beard-like distribution are clinically suggestive of subglottic involvement. Direct laryngoscopy and bronchoscopy confirm the diagnosis.
Subglottic stenosis is usually congenital in this age group but can also result from subglottic scarring following prolonged intubation. Stridor may be present but is not typically position-dependent.
Tracheomalacia and bronchomalacia may coexist with laryngomalacia, often presenting with expiratory airway sounds. Bronchoscopy is used for diagnosis.
Vascular rings, a rare cause of airway obstruction, can present with feeding difficulties and stridor. Diagnosis is confirmed with contrasted computed tomography (CT) of the chest. This condition should be considered when tracheomalacia is seen on bronchoscopy or esophageal compression is noted on esophagram.
Foreign body aspiration is a possibility when an infant presents with respiratory distress, particularly after being left unattended or after a choking or coughing episode related to food ingestion. Chest x-ray findings and unilateral decreased breath sounds may suggest this diagnosis. Bronchoscopy is necessary for diagnosis and foreign body removal.
Prognosis of Laryngomalacia
While traditional understanding suggests that laryngomalacia resolves by 12 to 18 months of age, recent evidence suggests a broader range for spontaneous resolution.[18] Conservative management, including upright feeding, antireflux therapy, and close monitoring of respiratory symptoms, is generally sufficient for most infants.
For the subset of patients requiring supraglottoplasty, outcomes are generally favorable, with success rates reported as high as 95% in some studies.[19] Revision surgery may be needed for persistent symptoms, more commonly in patients younger than 2 months at initial surgery, those with neurological comorbidities like hypotonia, seizures, cerebral palsy, or cardiac comorbidities such as septal defects, aortic or pulmonary stenosis, or pulmonary hypertension. Neurological comorbidities are associated with the highest revision surgery rate, with approximately 60% of these patients eventually requiring tracheostomy due to persistent airway obstruction.[20]
Complications Associated with Laryngomalacia
Aspiration following supraglottoplasty is uncommon and not significantly linked to the surgical technique used. Factors associated with post-surgical aspiration include neurological comorbidities, revision surgery, and surgery performed before 18 months of age.[21][22]
Nonsurgical management of laryngomalacia often includes treating concurrent reflux disease. Proton pump inhibitors (PPIs) are frequently recommended. Reflux has been correlated with more severe forms of laryngomalacia, although PPI therapy has not consistently shown to improve laryngomalacia symptoms directly.[4]
Consultations for Laryngomalacia
Depending on initial findings, infants with laryngomalacia may require evaluations by specialists in neurology, gastroenterology, cardiology, developmental pediatrics, genetics, and speech pathology to manage comorbidities and optimize overall care.
Deterrence and Patient Education for Laryngomalacia
Educating parents about laryngomalacia symptoms is paramount, especially regarding the often inconsistent correlation between noisy breathing and failure to thrive. Parents should understand that noisy breathing does not always indicate severe problems, and that it may persist even after surgical intervention.[23] Caregivers should be informed that treatment is primarily conservative, but close follow-up is essential to monitor for symptom worsening. Adherence to prescribed medications and compliance with thickened and positional feedings should be strongly encouraged.
Pearls and Key Considerations in Laryngomalacia Management
Key points to remember regarding laryngomalacia:
- Most infants with laryngomalacia will naturally outgrow the condition without needing surgery.
- Laryngomalacia diagnosis is best achieved with awake fiberoptic flexible laryngoscopy.
- Severe laryngomalacia symptoms, such as recurrent cyanosis or respiratory distress, apnea, and failure to thrive, necessitate further evaluation with direct laryngoscopy and bronchoscopy to rule out other airway abnormalities.
- Surgery is generally indicated only for severe laryngomalacia cases with significant breathing difficulties or failure to thrive.
- Supraglottoplasty is the preferred surgical approach and is generally well-tolerated.
- Infants with neurological or cardiac comorbidities, and those under 2 months of age, are at higher risk for needing revision surgery for persistent airway obstruction.
Enhancing Healthcare Team Outcomes in Laryngomalacia Care
Effective, patient-centered care for laryngomalacia depends on a collaborative, interprofessional approach. Physicians must refine their diagnostic skills and surgical techniques. Advanced care practitioners contribute by implementing evidence-based strategies and delivering comprehensive care. Nurses are vital in patient monitoring and education. Pharmacists ensure medication management and patient safety.
A thorough history and physical exam are essential for initial assessment, and referral to otolaryngology for flexible fiberoptic laryngoscopy is a key step in laryngomalacia diagnosis. Continuous communication between parents, primary care clinicians, and specialists is crucial, as each provides valuable subjective and objective information. Given that most patients are initially managed with observation, input from multiple sources provides a clearer picture of the clinical course. For patients with worsening symptoms, prompt airway evaluation can lead to timely treatment. By maintaining open communication and recognizing worsening clinical trends, a collaborative approach can improve outcomes and facilitate earlier intervention for severe airway issues.
Nurses in the NICU and parents at home are often the first to notice symptoms suggestive of laryngomalacia. Nurses play a critical role in reporting these symptoms promptly. Specialists can collaborate with neonatal nursing staff and primary clinicians. Nurses are also often the first point of contact for parents during postnatal appointments, making their role in initial assessment and history gathering crucial for alerting the team to potential abnormalities and initiating diagnostic procedures when necessary.
Interprofessional communication is paramount, fostering a shared understanding and coordinated care plan. Each professional’s commitment to staying informed and engaged is essential for optimal outcomes. Collaborative care coordination ensures a smooth patient journey, enhancing team performance and the overall quality of care for infants with laryngomalacia.
Review Questions
Figure
Image Alt Text: Illustration depicting laryngomalacia, a condition causing airway obstruction in infants, with visible collapse of supraglottic structures during inhalation.
Figure
Image Alt Text: Laryngoscopic view of laryngomalacia showing the characteristic omega-shaped epiglottis and shortened aryepiglottic folds, key indicators for laryngomalacia diagnosis.
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Disclosures: Michael Klinginsmith, Ryan Winters, and Julie Goldman declare no relevant financial relationships with ineligible companies.
