eMedicine Specialties > Pediatrics: General Medicine > Pulmonology

Laryngomalacia

Updated: Jul 24, 2009

Introduction

Background

Laryngomalacia is a congenital abnormality of the laryngeal cartilage. It is thought to represent a delay of maturation of the supporting structures of the larynx. Laryngomalacia is the most common cause of congenital stridor and is the most common congenital lesion of the larynx.

Laryngomalacia: The epiglottis is small and curled on itself (omega-shaped). Approximation of the posterior edges of the epiglottis contributes to the inspiratory obstruction. (From B Benjamin, Atlas of Paediatric Endoscopy, Oxford University Press, NY, 1981, with permission.)

Pathophysiology

Laryngomalacia may affect the epiglottis, the arytenoid cartilages, or both. When the epiglottis is involved, it is often elongated, and the walls fold in on themselves. The epiglottis in cross section resembles an omega, and the lesion has been referred to as an omega-shaped epiglottis. If the arytenoid cartilages are involved, they appear enlarged. In either case, the cartilage is floppy and is noted to prolapse over the larynx during inspiration. This inspiratory obstruction causes an inspiratory noise, which may be high-pitched sounds frequently heard in other causes of stridor, coarse sounds resembling nasal congestion, and low-pitched stertorous noises. More severe compromise may be associated with a lower ratio of the aryepiglottic fold length to the glottic length.

A classification system has been proposed. In type 1 laryngomalacia, the aryepiglottic folds are tightened or foreshortened. Type 2 is marked by redundant soft tissue in any area of the supraglottic region. Type 3 is associated with other disorders, such as neuromuscular disease and gastroesophageal reflux.

Laryngomalacia is the most common cause of chronic inspiratory noise in infants, no matter which type of noise is heard. Infants with laryngomalacia have a higher incidence of gastroesophageal reflux, presumably a result of the more negative intrathoracic pressures necessary to overcome the inspiratory obstruction. Conversely, children with significant reflux may have pathologic changes similar to laryngomalacia, especially enlargement and swelling of the arytenoid cartilages. Some of the swelling of the arytenoid cartilages and of the epiglottis may be secondary to reflux.

Occasional inflammatory changes are observed in the larynx, which is referred to as reflux laryngitis. When the epiglottis is involved, gravity makes the noise more prominent when the baby is supine.

The exaggerated inspiratory effort increases blood return to the pulmonary vascular bed. This could account for the increased likelihood of pulmonary artery hypertension in infants with hypoxemia.

Frequency

United States

Frequency is unknown. Often, the diagnosis is presumed.

Mortality/Morbidity

Rarely, the lesion may cause enough hypoxemia or hypoventilation to interfere with normal growth and development. In severe cases, when laryngomalacia may be associated with gastroesophageal reflux, feeding problems such as choking or gagging may occur.

Race

No known race predilection has been reported.

Sex

Laryngomalacia has no sex predilection.

Age

Although this is a congenital lesion, airway sounds typically begin at age 4-6 weeks. Until that age, inspiratory flow rates may not be high enough to generate the sounds.

Clinical

History

The usual history in patients with laryngomalacia is of inspiratory noises that begin during the first 2 months of life. Sounds typically start at age 4-6 weeks, but they may begin in the nursery or as late as age 2-3 months.
Noises are inspiratory and may sound like nasal congestion, with which they are initially confused. However, the noises persist and no nasal secretions are present. The noise may be more high pitched, crowing stridor.
Noise is often increased when the baby is supine, during crying or agitation, during upper respiratory infection episodes, and, in some cases, during and after feeding.
The baby's cry is usually normal, unless concomitant reflux laryngitis is present.
Usually, no feeding intolerance is noted, although occasional choking or coughing with feedings may be noted if the baby has reflux.
The infant is usually happy and thriving.

Physical

Upon examination, the baby is usually happy and appropriately interactive.
Mild tachypnea may be present.
Other vital signs are normal, and oxygen saturation is usually normal.
One can usually detect nasal airflow. The noise may be increased if the baby is placed supine.
The cry is normal. Hearing the baby's cry during the examination is important. An abnormal cry suggests pathology at or near the vocal cords.
The noise is purely inspiratory. The sounds may best be heard just above the sternal notch.
The rest of the examination findings are unremarkable, although as many as 20% of infants with laryngomalacia also have another airway lesion.

Causes

Laryngomalacia is a congenital abnormality of the larynx. The pathology is unknown. In cases in which redundant or tight tissue has been removed, it is histologically indistinct from normal tissue.
No genetic pattern is known.

Differential Diagnoses

Airway Foreign Body	Respiratory Papillomatosis
Congenital Stridor	Subglottic Stenosis
Croup
Gastroesophageal Reflux
Hypocalcemia

Workup

Laboratory Studies

Oxygen saturation should be monitored in patients with laryngomalacia.

Imaging Studies

Fluoroscopy
- Fluoroscopy of the airway may be performed by a pediatric radiologist.
- The cartilages may be observed collapsing on inspiration on a lateral view of the airway.
Laryngoscopy and bronchoscopy
- These studies are the best studies used to confirm the diagnosis. However, in an infant with typical inspiratory noises (worse when supine) who have a normal cry and normal growth and development, clinical diagnosis is not unreasonable.
- A pediatric pulmonologist or pediatric otorhinolaryngologist may perform flexible laryngoscopy or bronchoscopy. Bronchoscopy under anesthesia has been shown to be more sensitive and specific than bronchoscopy in infants who are awake.
- Direct visualization of the airway reveals an omega-shaped epiglottis that prolapses over the larynx during inspiration.
- Enlarged arytenoid cartilages that prolapse over the larynx during inspiration may also be present.

Treatment

Medical Care

In more than 99% of cases, the only treatment necessary is time. The lesion gradually improves, and noises disappear by age 2 years in virtually all infants. The noise steadily increases over the first 6 months, as inspiratory airflow increases with age. Following this increase, a plateau often occurs with a subsequent gradual disappearance of the noise. In some cases, the signs and symptoms dissipate, but the pathology may persist into childhood and adulthood. In those cases, symptoms or signs may recur with exercise or sometimes with viral infections.
If the baby has more noise and is uncomfortable when asleep, they may sleep prone, although one must then be careful to avoid soft bedding, pillows, and blankets. Initially, infants with laryngomalacia were exempt from the American Academy of Pediatrics' "Back to Sleep" campaign. More recently, the recommendations have been changed to indicate that these babies should sleep supine to reduce the likelihood of sudden infant death. Guidelines for preventive services for children and adults have been established.¹
If the baby has clinically significant hypoxemia (defined as a resting oxygen saturation <90%), supplemental oxygen should be administered. Recent data suggest infants with laryngomalacia and hypoxemia may more readily develop pulmonary hypertension.² Therefore, children with hypoxemia should periodically undergo evaluation for pulmonary hypertension.
If the baby has normal cry, normal weight gain, normal development, and purely inspiratory noise that developed within the first 2 months of life, then no further workup may be necessary. Parents may be told that laryngomalacia is the most likely diagnosis, and they can be assured of its natural history.
If the picture is not obvious or if the parents are not completely reassured, diagnostic procedures include fluoroscopy and flexible laryngoscopy or bronchoscopy. Flexible bronchoscopy with the child anesthetized is more specific and sensitive than flexible bronchoscopy in a child who is awake.

Surgical Care

In severe cases in which the laryngomalacia interferes with ventilation enough to impair normal eating, growth, and development, a surgical approach is possible.³
Operations include simple tracheotomy or laryngoplasty in which support structures are tightened and excess tissue on the epiglottis is removed. Laser epiglottopexy has been successful.⁴

Consultations

If the parents require another opinion or if the lesion is clinically severe, consultation with a pediatric pulmonologist or pediatric otorhinolaryngologist may help.

Diet

No diet restrictions are necessary.

Activity

No activity restrictions are necessary.

Medication

Drug therapy is not currently a component of the standard of care for laryngomalacia. In more than 99% of cases, the only treatment necessary is time. The lesion gradually improves, and noises disappear by age 2 years in virtually all cases. See Treatment.

Follow-up

Further Inpatient Care

No inpatient care is necessary in patients with laryngomalacia unless the baby has clinically significant hypoxemia or apnea.

Further Outpatient Care

Unless supplemental oxygen is required for oxygen saturation less than 90%, no home therapy is necessary.
The usual well-child visits should be performed.
Immunizations should not be delayed because of airway noise.

Inpatient & Outpatient Medications

No medications are necessary.

Deterrence/Prevention

Laryngomalacia is not a preventable lesion and does not appear to run in families.

Complications

Poor oxygenation that requires supplemental oxygen
Alveolar hypoventilation that requires surgery or positive pressure ventilation
Apnea
Increased likelihood of gastroesophageal reflux
Pulmonary hypertension

Prognosis

Prognosis is excellent. Most babies outgrow the condition by their second birthday, many by the first. In some cases, even though the signs and symptoms dissipate, the pathology persists. Such patients may have stridor with exercise later in life.
Laryngomalacia may be more common in children with Down syndrome, in whom it may persist beyond the second birthday.

Multimedia

Media file 1: Laryngomalacia: The epiglottis is small and curled on itself (omega-shaped). Approximation of the posterior edges of the epiglottis contributes to the inspiratory obstruction. (From B Benjamin, Atlas of Paediatric Endoscopy, Oxford University Press, NY, 1981, with permission.)

References

[Guideline] Institute for Clinical Systems Improvement (ICSI). Preventive services for children and adolescents. Oct 2008;[Full Text].
Unal E, Oran B, Baysal T, et al. Pulmonary arterial pressure in infants with laryngomalacia. Int J Pediatr Otorhinolaryngol. Dec 2006;70(12):2067-71. [Medline].
Richter GT, Thompson DM. The surgical management of laryngomalacia. Otolaryngol Clin North Am. Oct 2008;41(5):837-64, vii. [Medline].
Whymark AD, Clement WA, Kubba H, Geddes NK. Laser epiglottopexy for laryngomalacia: 10 years' experience in the west of Scotland. Arch Otolaryngol Head Neck Surg. Sep 2006;132(9):978-82. [Medline].
Bertrand P, Navarro H, Caussade S, et al. Airway anomalies in children with Down syndrome: endoscopic findings. Pediatr Pulmonol. Aug 2003;36(2):137-41. [Medline].
Cotton RT, Richardson MA. Congenital laryngeal anomalies. Otolaryngol Clin North Am. Feb 1981;14(1):203-18. [Medline].
Denoyelle F, Mondain M, Gresillon N, et al. Failures and complications of supraglottoplasty in children. Arch Otolaryngol Head Neck Surg. Oct 2003;129(10):1077-80; discussion 1080. [Medline].
Fauroux B, Pigeot J, Polkey MI, et al. Chronic stridor caused by laryngomalacia in children: work of breathing and effects of noninvasive ventilatory assistance. Am J Respir Crit Care Med. Nov 15 2001;164(10 Pt 1):1874-8. [Medline]. [Full Text].
Gessler EM, Simko EJ, Greinwald JH. Adult laryngomalacia: an uncommon clinical entity. Am J Otolaryngol. Nov-Dec 2002;23(6):386-9. [Medline].
Kay DJ, Goldsmith AJ. Laryngomalacia: a classification system and surgical treatment strategy. Ear Nose Throat J. May 2006;85(5):328-31, 336. [Medline].
Mancuso RF, Choi SS, Zalzal GH, Grundfast KM. Laryngomalacia. The search for the second lesion. Arch Otolaryngol Head Neck Surg. Mar 1996;122(3):302-6. [Medline].
Manning SC, Inglis AF, Mouzakes J, Carron J, Perkins JA. Laryngeal anatomic differences in pediatric patients with severe laryngomalacia. Arch Otolaryngol Head Neck Surg. Apr 2005;131(4):340-3. [Medline].
Midulla F, Guidi R, Tancredi G, et al. Microaspiration in infants with laryngomalacia. Laryngoscope. Sep 2004;114(9):1592-6. [Medline].
Sivan Y, Ben-Ari J, Soferman R, DeRowe A. Diagnosis of laryngomalacia by fiberoptic endoscopy: awake compared with anesthesia-aided technique. Chest. Nov 2006;130(5):1412-8. [Medline].
Smith JL, Sweeney DM, Smallman B, Mortelliti A. State-dependent laryngomalacia in sleeping children. Ann Otol Rhinol Laryngol. Feb 2005;114(2):111-4. [Medline].
Thompson DM. Abnormal sensorimotor integrative function of the larynx in congenital laryngomalacia: a new theory of etiology. Laryngoscope. Jun 2007;117(6 Pt 2 Suppl 114):1-33. [Medline].

Keywords

omega-shaped epiglottis, laryngeal cartilage, malformation of the laryngeal cartilage, chronic inspiratory noise, gastroesophageal reflux, reflux laryngitis, hypoxemia, hypoventilation, choking, laryngomalacia, congenital stridor, swollen arytenoid cartilages, pulmonary artery hypertension, hypoxemia, hypoventilation, feeding problems, treatment, diagnosis

Contributor Information and Disclosures

Author

Michael R Bye, MD, Professor of Clinical Pediatrics, Division of Pulmonary Medicine, Columbia University College of Physicians and Surgeons; Attending Physician, Pediatric Pulmonary Medicine, Morgan Stanley Children's Hospital of New York Presbyterian, Columbia University Medical Center
Michael R Bye, MD is a member of the following medical societies: American Academy of Pediatrics, American College of Chest Physicians, and American Thoracic Society
Disclosure: Merck Honoraria Speaking and teaching

Medical Editor

Girish D Sharma, MD, Associate Professor, Department of Pediatrics, Rush University Medical Center, Rush Children's Hospital; Director of Pediatric Pulmonary Section and Rush Cystic Fibrosis Center
Girish D Sharma, MD is a member of the following medical societies: American Academy of Pediatrics, American College of Chest Physicians, American Thoracic Society, and Royal College of Physicians of Ireland
Disclosure: Nothing to disclose.

Pharmacy Editor

Mary L Windle, PharmD, Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy, Pharmacy Editor, eMedicine
Disclosure: Pfizer Inc Stock Investment from financial planner; Avanir Pharma Stock Investment from financial planner ; WebMD Salary and stock Employment and investment from financial planner

Managing Editor

Charles Callahan, DO, Professor, Deputy Chief of Clinical Services, Walter Reed Army Medical Center
Charles Callahan, DO is a member of the following medical societies: American Academy of Pediatrics, American College of Chest Physicians, American College of Osteopathic Pediatricians, American Thoracic Society, Association of Military Surgeons of the US, and Christian Medical & Dental Society
Disclosure: Nothing to disclose.

CME Editor

Mary E Cataletto, MD, Associate Director, Division of Pediatric Pulmonology, Winthrop University Hospital; Professor of Clinical Pediatrics, State University of New York at Stony Brook; Director of Children's Sleep Services, Winthrop University Hospital
Mary E Cataletto, MD is a member of the following medical societies: American Academy of Pediatrics and American College of Chest Physicians
Disclosure: Shering Plough Pharmaceuticals Honoraria Consulting

Chief Editor

Further Reading