135: Surgical Diseases of the Newborn: Diseases of the Airway, Tracheobronchial Tree, and Lungs

INTRINSIC ABNORMALITIES OF THE AIRWAY

I. DEFINITION

Intrinsic abnormalities of the airway that cause partial obstruction of the airway fall into this category. Examples include laryngomalacia, vocal cord paralysis, subglottic web, and hemangioma.

II. PATHOPHYSIOLOGY

Lesions result in partial obstruction of the airway and cause stridor and respiratory distress of varying severity.

1. Laryngomalacia is due to delayed development of the supraglottic pharynx.

2. Congenital vocal cord paralysis can be congenital or acquired (birth trauma, patent ductus arteriosus ligation) and unilateral or bilateral.

3. Subglottic web is a congenital short-segment obstruction that may be partial or complete.

4. Hemangioma can occur below the glottis, engorge, and obstruct with agitation.

III. CLINICAL PRESENTATION

This can vary from mild respiratory stridor to complete airway obstruction depending on pathology.

IV. DIAGNOSIS

The diagnosis is established by airway endoscopy with careful visual inspection.

V. MANAGEMENT

Management is individualized. Some problems, such as laryngomalacia, will be outgrown and require only supportive care. Other lesions, such as subglottic webs and hemangiomas, may be amenable to endoscopic resection or laser therapy.

CHOANAL ATRESIA

I. 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%).

II. PATHOPHYSIOLOGY

True choanal atresia is complete and bilateral, and it is one cause of respiratory distress immediately after delivery. Neonates are obligate nose breathers and do not automatically breathe through the mouth. Unilateral defects may be well tolerated and often go unnoticed.

III. CLINICAL PRESENTATION

Respiratory distress resulting from partial or total upper airway obstruction is the mode of presentation.

IV. DIAGNOSIS

Diagnosis is based on the inability to pass a catheter into the nasopharynx via either side of the nose.

V. MANAGEMENT

Making the baby cry will initiate mouth breathing and temporarily improves the respiratory status. 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.

PIERRE ROBIN SEQUENCE

I. DEFINITION

This anomaly consists of mandibular hypoplasia (micrognathia) in association with cleft palate.

II. PATHOPHYSIOLOGY

Airway obstruction is produced by posterior displacement of the tongue associated with the small size of the mandible.

III. CLINICAL PRESENTATION

Severity of symptoms varies, but most infants manifest a high degree ...