Chapter 125: Surgical Diseases of the Newborn: Diseases of the Airway, Tracheobronchial Tree, and Lungs

I. OVERVIEW OF AIRWAY PROBLEMS IN THE NEWBORN

Any newborn experiencing feeding problems, repeated aspiration, and respiratory distress should have a complete evaluation of the airway. Temporary or permanent feeding difficulty may be caused by congenital infections, maternal drug use, hypoxia, or some type of birth trauma. A variety of surgical diseases (intrinsic airway abnormalities such as vocal cord paralysis or laryngomalacia), choanal atresia, laryngotracheal esophageal cleft, tracheoesophageal fistula, cricopharyngeal spasm, syndromic anomalies, and others should be included in the differential diagnosis of these patients. Respiratory distress in the absence of feeding can also be caused by intrinsic and extrinsic pulmonary issues.

II. INTRINSIC ABNORMALITIES OF THE AIRWAY

1. There are 4 common intrinsic upper airway diseases in the newborn.

   1. Laryngomalacia is due to delayed development of 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 is a vascular malformation that can occur below the glottis, engorge, and obstruct with agitation.

2. Clinical presentation. Varies from mild respiratory stridor to complete airway obstruction.

3. Diagnosis. Airway endoscopy with careful visual inspection.

4. Management

   1. Individualized based on pathology and endoscopic findings.

   2. Some problems (laryngomalacia) may be outgrown and require supportive care only.

   3. Other lesions (subglottic webs and hemangiomas) may require endoscopic resection or laser therapy.

III. CHOANAL ATRESIA

1. Definition

   1. Choanal atresia is congenital blockage of posterior nares caused by persistence of the bony septum (90%) or soft tissue membrane (10%). It can be associated with Treacher Collins, Tessier, and CHARGE (coloboma, central nervous system abnormalities, heart defects, atresia of the choanae, restricted growth and/or development, genital abnormalities, and ear anomalies) syndromes.

2. Pathophysiology

   1. Neonates are obligate nose breathers.

   2. True choanal atresia is complete and bilateral and causes immediate respiratory distress in the newborn.

   3. Unilateral defects may be well tolerated and often go unnoticed.

3. Clinical presentation

   1. Respiratory distress resulting from partial or complete upper airway obstruction.

   2. Cyanosis in an infant while breast feeding is another presentation.

4. Diagnosis. Inability to pass catheter into nasopharynx via either nostril.

5. Management

   1. Stimulating baby to cry will initiate mouth breathing and temporarily improve respiratory status.

   2. Insertion of oral airway.

   3. Definitive management requires resection of soft tissue or bony septum.

IV. PIERRE ROBIN SYNDROME/SEQUENCE

1. Definition

   1. Pierre Robin syndrome/sequence is a set of anomalies including mandibular hypoplasia (micrognathia) and a tongue that is placed further back than normal (glossoptosis) in association with cleft palate.

   2. Other associated syndromes include Stickler, velocardiofacial, craniofacial microsomia, and Treacher Collins syndromes.

2. Pathophysiology. Airway obstruction produced by posterior displacement of the tongue associated with small mandible. Feeding difficulty is also usually present.

3. Clinical presentation. Severity of symptoms varies, but most neonates manifest high-grade partial upper airway obstruction.

4. Management

   1. Infants with mild involvement can be placed in prone position and fed using ...