Definition. Abnormalities of, or within, the airway that cause partial obstruction fall into this category. Examples include laryngomalacia, paralyzed vocal cord, subglottic web, and hemangioma.
Pathophysiology. These lesions result in partial obstruction of the airway and cause stridor and respiratory distress of varying severity.
Laryngomalacia. Delayed development of the supraglottic pharynx.
Congenital paralyzed vocal cords. Can be congenital or acquired (birth trauma, patent ductus arteriosus [PDA] ligation), rarely bilateral.
Subglottic web. Congenital web that may be partial or complete.
Hemangioma. Can occur below glottis, engorge, and obstruct with agitation.
Clinical presentation. Can vary from mild respiratory stridor to complete airway obstruction depending on the exact variant.
Diagnosis. The diagnosis is established by endoscopy of the airway with careful visualization of the entire airway to establish diagnosis.
Management is individualized. Some problems, such as laryngomalacia, will be outgrown if the child can be supported through the period of acute symptoms. Other lesions, such as subglottic webs and hemangiomas, may be amenable to endoscopic resection or laser therapy.
Definition. Choanal atresia is a congenital blockage of the posterior nares caused by a persistence of a bony septum (90%) or a soft tissue membrane (10%).
Pathophysiology. Unilateral or bilateral obstruction at the posterior nares may be secondary to soft tissue or bone. True choanal atresia is complete and bilateral, and it is one cause of respiratory distress immediately after delivery. The effects of upper airway obstruction are compounded because neonates are obligate nose breathers and do not "think" to breathe through the mouth. Unilateral defects may be well tolerated and go unnoticed.
Clinical presentation. Respiratory distress resulting from partial or total upper airway obstruction is the mode of presentation.
Diagnosis is based on an inability to pass a catheter into the nasopharynx via either side of the nose.
Management. Simply making the infant cry and thereby breathe through the mouth temporarily improves breathing. Insertion of an oral airway maintains the ability to breathe until the atresia is surgically corrected. Definitive management requires resection of the soft tissue or bony septum in the nasopharynx.
Definition. This anomaly consists of mandibular hypoplasia (micrognathia) in association with cleft palate.
Pathophysiology. Airway obstruction is produced by posterior displacement of the tongue associated with the small size of the mandible.
Clinical presentation. Severity of symptoms varies, but most infants manifest a high degree of partial upper airway obstruction.
Infants with mild involvement can be cared for in the prone position and fed through a special Breck nipple. Adjustment to the airway compromise occurs over weeks to months as the mandible grows relatively faster and the degree of obstruction lessens with growth.
More severe cases require nasopharyngeal tubes or surgical procedures to hold the tongue in an anterior position. Tracheostomy is generally a last resort.
Definition. Laryngotracheal esophageal cleft is a rare congenital anomaly in which there is an incomplete separation of the larynx (and sometimes the trachea) from the esophagus, resulting in a common channel ...
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