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Laryngomalacia
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Laryngomalacia-associated breathing was first described in 1851 by the French physician Rilliet in a case report of a benign inspiratory noise from an infant. This sound was subsequently termed stridor in 1853 by Rilliet and Barthez in their textbook, Of Diseases of Children . It was not until 1897 that the laryngoscopic physical findings associated with this disorder were accurately described in 16 patients by Sutherland and Lack as congenital laryngeal obstruction, while the actual term “laryngomalacia” (“malacia,” Greek malakia —softening of part of tissue) was not used until 1942 when Chevalier Jackson described this disorder.
The underlying etiology for laryngomalacia remains relatively unknown. The predominant underlying concepts to explain this supraglottic instability are excess redundant mucosa, poor cartilaginous support, and/or abnormal neurologic laryngeal tone. Kletzker observed mucosal hypertrophy in histologic normal supraglottic tissues and proposed that pulsion forces from the back and forth of mucosa on overlying cartilage resulted in hypermobility and redundancy, which then becomes obstructive. The lack of cartilaginous support (chondromalacia) theory has become controversial by histologic studies demonstrating similar cartilage in patients having laryngomalacia when compared to controls.
The predominant theory to date is that laryngomalacia may be a result of delayed neurologic function and tone in the supraglottic laryngeal structures. The work by Thompson correlated increased laryngopharyngeal sensory testing thresholds with laryngomalacia severity, while a recent supraglottic histologic study demonstrated increased neural perimeter and surface area innervation by the superior laryngeal nerve branch of vagus in patients with laryngomalacia when compared to controls. This information supports altered vagal neuronal innervation and stimulation in this patient population.
Laryngomalacia affects approximately 35% to 75% of infants presenting with stridor and is the most common cause of stridor in the pediatric age group. This disorder primarily presents with inspiratory stridor as early as 2 weeks of age, with progressive worsening up to 4 to 8 months and resolution typically by 12 to 18 months without requiring surgical intervention. Respiratory symptoms are often exacerbated with feeding, exertion, and supine positioning. Swallowing dysfunctions (penetration or aspiration) have been reported in up to 88% of patients with severe laryngomalacia. A discoordinated suck, swallow, breath sequence can result in respiratory distress and arrest, insufficient weight gain/failure to thrive, feeding complications, poor quality of life, and high parental anxiety.
Clinically, this condition is often separated into mild, moderate, and severe categories based on feeding and obstructive symptomatology. Mild disease is described as inconsequential intermittent stridor and sporadic feeding difficulties, and moderate disease presents with dyspnea and consistent feeding difficulties. Severe laryngomalacia is present in approximately 20% of infants with stridor and is characterized by recurrent cyanosis, apneic events, and difficulty with feeding, often with associated penetration/aspiration and failure to thrive.
Anatomically, laryngomalacia is described as the collapse of supraglottic tissue with associated inspiratory stridor and upper airway obstruction. This supraglottic dysfunction is primarily represented by prolapse of arytenoid cartilage or redundant tissue ( Figs. 193.1 and 193.2 ), foreshortened aryepiglottic (AE) folds ( Fig. 193.3 ; also see Fig. 193.2 ), and/or epiglottal prolapse/omega-shaped epiglottis (see Fig. 193.3 ). This prolapse of tissue causes obstruction of the glottic introitus upon inspiratory associated negative pressure, resulting in airway distress.
First described in 1922 by Iglauer, supraglottoplasty is a generalized term used to describe a surgical technique in which the supraglottic structures of the larynx are altered in a manner to prevent further collapse and subsequent obstruction of the glottic introitus. This technique did not replace the tracheostomy for relief of laryngomalacia until the 1980s when refined and modernized by Drs. Lane and Seid.
Supraglottoplasty has a reported success rate of over 90% in otherwise healthy children with this disease. Infants with underlying neurologic conditions require surgical intervention at higher rates and have higher failure rates. As high as 70% of neurologically delayed children with laryngomalacia will require revision supraglottoplasty, and up to 60% of supraglottoplasty failures in the neurologically delayed may require tracheostomy.
Today the modern supraglottoplasty is considered the standard of care in the treatment of severe laryngomalacia. This procedure is performed transorally using suspension laryngoscopy and microlaryngeal instruments. It has a high success and a low morbidity rate, making it a first-line option for the otolaryngologist.
Key Learning Points
- 1.
Laryngomalacia is the most common cause of stridor in the pediatric age group.
- 2.
Laryngomalacia is predominately self-limiting, with resolution typically by 18 months of age.
- 3.
Children with laryngomalacia can present with various aerodigestive complaints that can affect overall child health as well as child and parental quality of life.
- 4.
Supraglottoplasty is a surgical technique reserved for severe laryngomalacia and is a safe procedure.
- 5.
Supraglottoplasty is the surgical treatment of choice when conservative therapy fails.
Preoperative
History
- 1.
Complete prenatal and birth history should be obtained:
- a.
Were prenatal visits and care completed?
- b.
Maternal exposure to alcohol, tobacco, drugs, or teratogens
- c.
Birthing history (complications, cesarean section, vaginal delivery)
- d.
Full or preterm birth—number of weeks
- e.
APGAR score
- f.
Intubation history, ICU stay, and any further interventions should be documented.
- g.
Congenital anomalies and syndromes
- 1)
Estimated in 8% to 20% of patients with laryngomalacia and up to 40% in patients diagnosed with severe laryngomalacia
- 2)
Down syndrome is the most common associated genetic disorder.
- 1)
- a.
- 2.
Questions directed toward laryngomalacia and airway complaints
- a.
When did the breathing problems begin?
- b.
Does the child having any problems feeding?
- c.
Can you please describe the stridor?
- 1)
Inspiratory: Caused by obstruction above the vocal folds and is most commonly secondary to laryngomalacia
- 2)
Biphasic: Caused by obstruction or narrowing at or directly below the vocal folds and is demonstrated in vocal fold immobility, croup, or subglottic stenosis
- 3)
Expiratory: Caused by proximal and distal trachea pathology and most commonly a sign of tracheomalacia
- 1)
- d.
Is patient’s breathing or stridor worsened with head or body position?
- 1)
Laryngomalacia-associated stridor and respiratory distress are worsened with head flexion and when lying in the supine position. They are often improved with head extension and prone positioning.
- 1)
- e.
Please describe any other situations in which stridor or breathing is worsened?
- 1)
Classically, laryngomalacia-associated complaints are worse with agitation, excitement, and feeding.
- 1)
- f.
The presence of GERD and swallowing dysfunction–associated signs and symptoms as well as possible treatments should always be questioned in these patients.
- 1)
Coughing or choking with or after feeds, frequent regurgitation, presence of agitation or back arching when lying supine
- 1)
- a.
- 3.
Medications
- a.
Antireflux therapy
- 1)
The high incidence of GERD in patients having laryngomalacia suggests that antireflux medication and feeding modification are essential in these patients prior to and after both conservative and surgical therapy.
- 1)
- b.
Antiplatelet medications and herbal supplementation associated with increased bleeding should be discontinued prior to surgical intervention.
- a.
- 4.
Prior treatment
- a.
If GERD is present, it should be controlled prior to supraglottoplasty.
- 1)
Gastroenterology consult should be considered if reflux is not controlled with conventional management.
- 2)
Nissen fundoplication can be considered for most severe cases of GERD with laryngomalacia.
- 1)
- a.
- 5.
Family history
- a.
No significant link to family history of laryngomalacia
- a.
Physical Examination
- 1.
General
- a.
Patient’s general appearance and overall well-being should be examined.
- 1)
Patient size and weight for age
- 1)
- b.
Patient’s respiratory status should be evaluated.
- 1)
How audible and consistent is the stridor?
- 2)
Presence of tachypnea or dyspnea
- 3)
Cyanosis of lips and/or extremities
- 1)
- a.
- 2.
Nasal examination
- a.
Assess for nasal flaring and proper air movement.
- b.
Piriform aperture stenosis, choanal stenosis, and choanal atresia should be ruled out by nasal endoscopy or at the minimum cannulation with a 6- or 8-French nasogastric tube.
- a.
- 3.
Oral examination
- a.
Assess for micro- or retrognathia.
- 1)
Patients often will have airway obstruction secondary to retro-displacement of tongue.
- 1)
- b.
Assess for macroglossia.
- 1)
Patients can have airway obstruction secondary to enlargement of the tongue.
- 1)
- c.
Assess the size of the tonsils.
- a.
- 4.
Neck and chest examination
- a.
Assess for supra-, inter-, and/or substernal retractions.
- b.
A stethoscope can be placed over the neck, specifically over the supraglottis, glottis, subglottis, and over the thorax to localize stridor and/or wheezing.
- c.
Assess for pectus excavatum.
- 1)
Defined as concavity of sternocostal cartilage of the anterior chest wall
- 2)
Can be associated with laryngomalacia and primarily seen in moderate and severe cases
- 1)
- a.
- 5.
FFL ± fiberoptic endoscopic examination of swallowing (FEES)
- a.
Allows for complete evaluation of upper airway
- b.
Allows for examination of supraglottic structures on inspiration and associated pathology contributing to laryngomalacia
- c.
GERD changes can be demonstrated by arytenoid, postglottic, and vocal fold edema and/or erythema.
- d.
FEES can be performed to assess swallowing status.
- 1)
This can be performed with a speech pathologist.
- 2)
Aids in assessment of disease severity and effect on swallowing
- 3)
Allows for assessment and management of safe consistencies for feeding in order to prevent aspiration and penetration
- 1)
- a.
Imaging/Further Diagnostic Evaluation
-
None
-
In most situations, clinical history and physical examination provide adequate information for the nonsurgical or surgical management of laryngomalacia.
-
-
Polysomnogram—optional
-
Patients with laryngomalacia often demonstrate associated sleep apnea and hypopnea. A polysomnogram (PSG) allows the physician to document the degree of disease and can provide objective information of apnea status for poor historians—clinical histories that do not correlate with anatomic findings and vice versa. It is also useful when treating patients with associated comorbidities and/or SALs, as you can objectively follow the course of the disease and response to treatment.
-
-
Modified barium swallow (MBS) study—optional
-
MBS can be used to assess aspiration and penetration status in patients that do not tolerate FEES, have an incomplete FEES, or if the status is questionable.
-
Indications
- 1.
Severe laryngomalacia
- 2.
Direct laryngoscopy and bronchoscopy with possible supraglottoplasty should be performed on all of the following:
- a.
Infants with laryngomalacia and severe respiratory distress, failure to thrive, apnea events, or recurrent pneumonia
- b.
Infants with symptoms that do not match the degree of laryngomalacia noted on flexible fiberoptic laryngoscopy (FFL) to rule out synchronous airway lesions (SALs)
- a.
Contraindications
- 1.
Medical comorbidities with increased risk of anesthesia and inability to tolerate spontaneous ventilation
- 2.
Patients currently intubated or tracheostomy tube dependent secondary to ventilator dependence (e.g., bronchopulmonary dysplasia)
- 3.
SALs of higher contribution to airway obstruction
- a.
SALs are present in 27% of patients with laryngomalacia
- a.
Preoperative Preparation
- 1.
Respiratory and swallowing status should be stabilized to the best scenario prior to surgery (supplemental oxygen, hospital admission, thickening of feeds, and nasogastric tube feeding).
- 2.
GERD should be controlled prior to surgery.
- 3.
Discontinuation of any anticoagulants at appropriate time or reversal
- 4.
Complications and indications of the procedure should be discussed at length with responsible individuals.
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