Maintaining a safe airway is one of the primary concerns when managing patients with otolaryngology problems. This includes not only ensuring adequate size and patency of the airway but also protecting against aspiration of blood, food, and secretions into the airway.

Although loss of a secure airway is one of the most feared complications in otolaryngology patients, other disorders of the head and neck can have significant morbidity if not appropriately addressed. The head and neck have an abundant vascular supply and blood loss can be brisk and substantial. Seemingly minor trauma can lead to significant cosmetic deformity if not promptly and appropriately treated. Localized infections can spread to the bloodstream or track along fascial planes to become life-threatening. The close proximity of head and neck structures to the brain facilitates easy intracranial spread of otolaryngologic disease or infection, often with devastating results. The majority of conditions that require otolaryngology referral can be safely and effectively managed in the outpatient setting. However, when significant risk of complication exists, admission for inpatient management is warranted. It is also common for patients that are already hospitalized to develop or have coexisting head and neck conditions.

INFANT WITH NOISY BREATHING

A rapid preliminary assessment of the infant who presents with noisy breathing is essential to determine the degree of respiratory distress. The presence of retractions, increased work of breathing, vital sign instability, and oxygen desaturations provide a more accurate indicator of the patient’s respiratory status than the extent of the noisy breathing. A patient that is tiring will often be less noisy as obstruction worsens and airflow decreases. In such cases, the airway must be urgently secured in a controlled fashion. However, the majority of infants can be safely evaluated without intubation.^1,2

The history and physical examination should guide the initial management and workup of an infant with noisy breathing. The history alone may suggest the diagnosis in more than 80% of cases. A few components of the history are particularly important: onset of noisy breathing (e.g. immediately following delivery, 6 to 8 weeks of age, later in infancy); quality of noisy breathing (e.g. stertor, inspiratory stridor, expiratory stridor, biphasic stridor, wheezing); quality of the cry (e.g. normal, weak, absent); and aggravating or alleviating factors (e.g. supine positioning, crying, feeding).² A thorough physical examination is then undertaken paying particular attention to the general state of the child and auscultation of the neck and chest to determine in which phase of respiration the noisy breathing is occurring. Flexible fiberoptic laryngoscopy can often confirm the diagnosis by allowing examination of the entire upper airway as well as visualization of the larynx.²

In a stable patient, the next step in the evaluation should include radiographic examination of the airway with a chest radiograph and high-kilovolt anteroposterior and lateral neck films. These imaging studies may assist with identification of the area of obstruction (e.g. adenotonsillar hypertrophy, subglottic stenosis) or identify the presence of a foreign body or pulmonary abnormalities.^2,3 Additional diagnostic information may be provided by airway fluoroscopy, esophogram, computed tomography (CT), or magnetic resonance imaging (MRI).² A pH probe study may be useful in identifying gastroesophageal reflux (GERD), which can contribute significantly to airway symptoms.⁴

Any child admitted with noisy breathing who does not require intubation should receive humidified oxygen or air and have continuous pulse oximetry monitoring. Systemic steroids and racemic epinephrine provide effective relief of acute airway obstruction.⁵ In cases where infectious etiology is suspected, appropriate antibiotic coverage should be initiated.¹ In some instances, treatment of GERD will improve airway symptoms over time.⁶

The differential diagnosis of noisy breathing in the infant is extensive and includes pathology at all levels of the upper and lower airways (see Table 158-1). Three of the more common causes are discussed below.

Laryngomalacia

Laryngomalacia is the most common cause of stridor in an infant. It typically presents around 6 weeks of life with high-pitched inspiratory stridor that is worse with supine position, feeding, and agitation. The vast majority of cases will resolve spontaneously by 12 to 18 months of age.⁷ The diagnosis is confirmed via flexible laryngoscopy which typically demonstrates short aryepiglottic folds, an omega-shaped epiglottis, and arytenoid prolapse (see Figure 158-1). Patients with significant airway obstruction or failure to thrive require supraglottoplasty (division of the aryepiglottic folds and/or excision of the redundant arytenoid mucosa).⁷

Vocal Cord Paralysis

Vocal cord paralysis (VCP) is the second most common cause of stridor in infancy accounting for approximately 10% of all congenital laryngeal lesions.⁸ Unilateral VCP is most commonly acquired due to trauma (difficult delivery, intubation injury) or due to surgery to correct congenital cardiac abnormalities.^8,9 Bilateral VCP is usually neurogenic or idiopathic. MRI is essential in patients with bilateral VCP to rule out neurologic abnormalities (e.g. Arnold-Chiari malformation, hydrocephalus, spinal cord abnormalities).¹⁰ Infants with unilateral VCP typically have a weak, hoarse cry whereas infants with bilateral VCP commonly have airway obstruction that necessitates tracheostomy. Definitive treatment is often deferred to allow for the possibility of spontaneous recovery. One of the biggest challenges in the surgical management of bilateral VCP paralysis is achieving an adequate airway without significantly compromising the quality of the voice or causing aspiration.^8,11

Subglottic Stenosis

Subglottic stenosis is another common cause of stridor in infancy. Subglottic stenosis can be congenital due to deformity of the cricoid cartilage or it can be acquired as a result of prolonged intubation.⁸ Severe cases present with stridor immediately after birth or failure to extubate. Milder cases can present as recurrent croup. Management is determined by the severity of the stenosis and the extent of respiratory distress and ranges from intermittent courses of steroids to laryngotracheal reconstruction or tracheostomy.

Two additional airway conditions warrant discussion.

RECURRENT RESPIRATORY PAPILLOMATOSIS

Recurrent respiratory papillomatosis (RRP) is the most common neoplasm involving the pediatric larynx and is the second most common cause of hoarseness in children. RRP is a disease in which benign non-keratinizing squamous papillomas grow in the upper aerodigestive tract with a particular predilection for the larynx.¹² RRP is most commonly caused by human papillomavirus (HPV) types 6 and 11.¹³ RRP is considered a benign neoplasm with rare malignant transformation.¹³

Children with RRP typically present with dysphonia and/or stridor between the ages of 2 and 5 years. Rarely, they present with acute airway obstruction. The classic history is a first-born male child delivered vaginally by a young mother. Flexible laryngoscopy is often sufficient to make the diagnosis. Thorough bronchoscopic evaluation is mandatory both to determine the extent of disease and to excise obstructing lesions.¹² Repeated surgical excision using a variety of techniques is the mainstay of treatment, the goal of which is to relieve airway obstruction while preventing complications of treatment.^13,14 Numerous adjuvant therapies exist (photodynamic therapy, systemic interferon, intralesional cidofovir, and intralesional bevacizumab) but these have met with varying degrees of success.^13,15 Tracheostomy is avoided unless absolutely necessary as it can cause distal spread of the disease.¹⁶

SUBGLOTTIC HEMANGIOMA