Pediatric emergency medicine trisk 634

The neonatal larynx is less than one-third the size of an adult. Even a
small obstruction can lead to rapid deterioration of the baby’s
respiratory status. Always triage rapidly.
Respiratory distress in infants with Pierre Robin sequence,
micrognathia, or low tone affecting airway patency may be relieved by
placing prone or by placing a nasopharyngeal trumpet.
Infants with laryngomalacia often have associated gastroesophageal
reflux. Remember to ask about reflux symptoms and treat accordingly.
Laryngotracheomalacia will improve at approximately 18 months of
age.
Pierre Robin Syndrome
Pierre Robin syndrome describes a triad of micrognathia (small jaw), glossoptosis
(downward displacement and retraction of the tongue), and a U-shaped cleft of
the small palate. It occurs in 1 in 8,500 live births. During the 8th to 10th week of
embryologic development, the small jaw places the tongue posteriorly causing it
to lie between the two developing shelves of the soft palate, hindering their
fusion. This single mechanical incident produces this sequence of events; hence,
Pierre Robin syndrome is actually a sequence. It may also occur as part of other
syndromes (e.g., Stickler or Velocardiofacial syndrome). Symptoms of airway
obstruction (stridor, respiratory distress, and cyanosis) are variable depending on
the degree to which the tongue obstructs the nasopharynx posteriorly. Neonates
can also present with feeding difficulty and failure to gain weight because of the
cleft palate and poor breathing–swallowing coordination. Severe cases are
diagnosed in the nursery and remain hospitalized. Milder cases may present in the
emergency room with worsening respiratory distress or aspiration. Examination
of the face is notable for micrognathia and cleft of the soft palate ( Fig. 96.34 ).
The baby may have audible stridor or retractions that worsen when placed supine.
Initial management should include placing the baby prone to relieve airway
obstruction by allowing the tongue to fall away from the nasopharynx. Infants
that do not respond may benefit from nasopharyngeal airway placement or an
endotracheal tube placed nasally to break the seal between the posterior tongue

and pharynx that is causing the obstruction. Nasal CPAP may establish patency of
the airway. Intubation and escalation of respiratory support to mechanical
ventilation may be required if the infant is in severe respiratory distress. Infants
with Pierre Robin syndrome should be continually monitored while in the ED and
admitted to the intensive care unit for ongoing serial monitoring for


cardiorespiratory deterioration. Blood gas analysis may reveal chronic carbon
dioxide retention. Consultation with a craniofacial specialist is advised.
Otolaryngology evaluation may reveal other nasal or nasopharyngeal anomalies
contributing to the obstruction. A geneticist should also be consulted if other
syndromic features are present. Polysomnography may be helpful in determining
the degree of obstruction or apnea and rule out central causes in infants who do
not have overt cardiorespiratory events.

FIGURE 96.34 Lateral view of an infant with Pierre Robin sequence. Note the hypoplastic
mandible resulting in retrognathia. (Courtesy of Joseph Losee. In: Orebaugh SL, Bigeleisen PE,
eds. Atlas of Airway Management . 2nd ed. Philadelphia, PA: Lippincott Williams & Wilkins;
2011. With permission.)

Laryngotracheomalacia
Laryngotracheomalacia accounts for approximately 60% to 70% of laryngeal
problems in neonates and infants. It develops as a result of a low laryngeal tone
thought to be related to neurologic immaturity of the newborn and can be
compounded by the presence of gastroesophageal reflux disease (GERD).
Neonates present around 2 to 4 weeks of age with noisy breathing or coarse
inspiratory stridor that is accentuated by agitation, crying, and being in a supine
position. Stridor improves at rest and in the prone position. Severe cases may
present with cyanosis, apnea, choking, and feeding difficulties. Laryngomalacia
improves over time with complete resolution by approximately 18 months of age.

Severe airway obstruction can occur when there are secondary lesions, such as


neuromuscular disorders, subglottic stenosis, or vascular rings. Laryngomalacia
or tracheomalacia may simulate respiratory distress, with noisy respirations
originating in the upper airway, but oxygen saturation and chest radiographs are
normal. Clinicians should also look for signs and symptoms of GERD, which are
commonly associated with laryngomalacia. The reflux of gastric content causes
swelling of the vocal cords but it is also thought that laryngomalacia causes
increases in negative intrathoracic pressure in an attempt to overcome the
laryngeal obstruction, and this incites gastric contents to reflux. In moderate to
severe cases, diagnosis can be confirmed by flexible fiberoptic nasolaryngoscopy
performed at the bedside by an ear, nose, and throat (ENT) surgeon ( Fig. 96.35 ).
ENT consultation for fiberoptic laryngoscopy is also recommended for mild cases
with progressive symptoms or other associated symptoms. Occasionally, the ENT
surgeon will request a modified barium swallow or rigid bronchoscopy to rule out
secondary comorbidities in cases where stridor is out of proportion to the degree
of laryngomalacia seen on flexible laryngoscopy or in the presence of aspiration
suspicious for posterior laryngeal cleft or tracheosophageal fistula. Management
is mainly conservative (watchful waiting) as infants will outgrow the condition.
Control of reflux with positioning after feeds and acid suppression therapy may
be helpful. Surgery (supraglottoplasty) is rarely required and is only indicated for
cases with severe obstruction, hypoxic episodes, pulmonary hypertension, and
failure to thrive.


FIGURE 96.35 Laryngomalacia affecting the posterior airway with collapse of the
aryepiglottic folds and arytenoid tissue during inspiration. (Reprinted with permission from
McMillan JA, Feigin RD, DeAngelis CJ, et al. Oski’s Solution . Philadelphia, PA: Lippincott
Williams & Wilkins; 2006.)

Vocal Cord Paralysis
Vocal cord paralysis accounts for 10% to 20% of laryngeal problems in neonates
and infants. Unilateral vocal cord paralysis is more common than bilateral. Most
cases are idiopathic in nature, however, neurologic disorders (Arnold–Chiari
malformation,
hydrocephalus,
brainstem
anomalies,
encephalocele,
leukodystrophy, myasthenia gravis, spinal muscle atrophy, and other
neuromuscular disorders), cardiac disorders (tetralogy of Fallot, VSD, closure of
a patent DA, and vascular rings), genetic (autosomal dominant bilateral palsy),
and birth trauma have also been implicated in the etiology. In most unilateral
cases, neonates will present with a weak cry; additional symptoms related to
aspiration and feeding difficulties may be present. Neonates with bilateral vocal
cord palsy present with a good cry but biphasic stridor. Vocal cord paralysis
causing respiratory distress may require intubation in severe cases. The first