69 LARYNGEAL AND TRACHEAL
AIRWAY DISORDERS
Alessandro de Alarcon, MD, Robin T. Cotton, MD,
and Michael J. Rutter, MD
Advances in the management of critically ill infants with
extreme prematurity, complex congenital anomalies, air-
way trauma, and infectious diseases concomitantly created
a new subset of patients with complex airway problems.
The overall management of these patients often includes
surgical airway intervention. Evaluation requires a global
perspective, with assessments of both the airway and the
child's overall health status. This broad-based approach
provides information that is crucial to the success of air-
way reconstruction.
We have found that minimizing the risk of opera-
tive failure can best be achieved through the collabora-
tive efforts of a well-coordinated interdisciplinary team.
Thorough clinical and operative examinations should
be performed, with involved health professionals being
aware of conditions and risk factors that can significantly
impact clinical outcomes.
This chapter presents an overview of the critical aspects
of otolaryngologic management of this complex patient
population in the context of the collaborative model used
at our institution. We briefly discuss the initial assessment,
mitigating factors that can affect airway reconstruction, and
perioperative management of specific airway pathology.
OPERATIVE ASSESSMENT
The operative evaluation is performed by the interdis-
ciplinary team, with input from each physician being
crucial. This evaluation comprises three endoscopic pro-
cedures performed consecutively with the patient under
a single anesthesia: (1) flexible bronchoscopy with bron-
choalveolar lavage (BAL); (2) microlaryngoscopy and
rigid bronchoscopy; and (3) esophagoduodenoscopy
(EGD) with biopsy. For patients in whom gastroesopha-
geal reflux (GER) is suspected or in whom GER would
have negative consequences on subsequent management,
objective evaluation of GER is recommended. Each com-
ponent of this endoscopic evaluation is aimed at identify-
ing possible pathology and risk factors that can affect the
success of airway reconstruction.
Flexible Bronchoscopy
Flexible bronchoscopy offers several advantages over
rigid bronchoscopy. It can identify particular areas that
can cause airway obstruction and that may be under-
appreciated or missed with a rigid bronchoscope. More
specifically, flexible bronchoscopy provides better assess-
ment of disorders such as glossoptosis, laryngomalacia,
tracheomalacia, and bronchomalacia. Evaluation of the
distal airway may provide additional information on
vascular compression, bronchiectasis, and assessment of
aspiration by BAL. (See Chapter 66 for a more detailed
discussion.)
Microlaryngoscopy and Rigid
Bronchoscopy
Microlaryngoscopy and rigid bronchoscopy are performed
with the primary goal of identifying anatomic levels
of airway obstruction from the larynx to the carina. The
supraglottis is evaluated with attention given to the possi-
bility of supraglottic obstruction such as laryngomalacia
and supraglottic stenosis. The vocal fold level is then eval-
uated for posterior glottic stenosis, anterior glottic web,
and laryngeal cleft. If vocal fold immobility is suspected
or seen on the fiberoptic endoscopic evaluation of swal-
lowing (FEES) or on voice evaluation, the cricoarytenoid
joints should be palpated to determine if there is any fixa-
tion of the joint.
Rigid bronchoscopy is performed using a combina-
tion of Hopkins rod telescopes and rigid bronchoscopes.
The subglottis is evaluated initially. If subglottic stenosis
is present, it is classified by the Cotton-Myer scale1 and
sized using appropriate endotracheal tubes (Table 69-1).
Additionally, the length of stenosis and the proximity to
the vocal folds is assessed and documented. If a trache-
otomy is in place, attention is paid to the evaluation of
the suprastomal area, considering the possibility of supra-
stomal collapse, granuloma, intratracheal skin tract, and
high tracheotomy. The trachea is evaluated to the level
of the carina, looking for additional pathology, including
tracheal stenosis, complete tracheal rings, tracheoesopha-
geal fistula (TEF), TEF pouches, vascular compression,
and tracheomalacia.
Esophagoduodenoscopy
Evaluation of the upper gastrointestinal tract can provide
information that is crucial in decision making as to future
surgery. Inflammation in the laryngotracheal complex
can be caused by conditions of the upper gastrointestinal
tract, resulting in an “active” (i.e., inflamed) larynx. Poor
wound healing and scarring is more likely to occur in this
setting. The two gastrointestinal conditions associated
with laryngeal inflammation are eosinophilic esophagitis
(EE) and gastroesophageal reflux disease (GERD). The
diagnosis of EE is made by esophageal biopsy. Laryngeal
inflammation may resolve with appropriate treatment of
the underlying condition, permitting surgical reconstruc-
tion with a lower risk of complication.
969
970 Aerodigestive Disease

TABLE 69-1  COTTON-MYER GRADING SCALE

Section XI

PERCENTAGE OF OBSTRUCTION BY ACTUAL ENDOTRACHEAL TUBE SIZE

Normal Normal
Patient age ID OD Percentage of obstruction with actual endotracheal tube size
(mm) (mm) ID  2.0 ID  2.5 ID  3.0 ID  3.5 ID  4.0 ID  4.5 ID  5.0 ID  5.5 ID  6.0
Premature 2.0 2.8 0%
2.5 3.6 40% 0%
3.0 4.3 58% 30% 0%
0–3 mo 3.5 5.0 68% 48% 26% 0%
3–9 mo 4.0 5.6 75% 59% 41% 22% 0%
9 mo to 2y 4.5 6.2 80% 67% 53% 38% 20% 0%
2y 5.0 7.0 84% 74% 62% 50% 35% 19% 0%
4y 5.5 7.6 86% 78% 68% 57% 45% 32% 17% 0%
6y 6.0 8.2 89% 81% 73% 64% 54% 43% 30% 16% 0%
ID – Inside diameter, OD – Outside diameter

Grade I Grade II Grade III Grade IV

No detectable lumen

No 50% 51% 70% 71% 99%

obstruction obstruction obstruction obstruction obstruction obstruction

Swallowing and Voice Evaluations

Preoperative swallowing and voice evaluations can be
crucial, as both assessments may alter an otherwise sound
surgical plan.
If there is a suspicion of ongoing aspiration, if surgery
will involve the glottis, or if surgery will repair a steno-
sis that may be preventing aspiration, then a swallowing
evaluation should be pursued. The two most commonly
used evaluations of swallowing are videofluoroscopic
swallow study (VSS) and FEES. These complementary
evaluations can assess ongoing aspiration with swal-
lowing as well as the likelihood of future aspiration.2,3
The advantages of FEES are that the laryngeal protective
mechanisms can be visualized by the surgeon, vocal fold
motion can be documented, and the potential risk of aspi-
ration can be assessed prior to airway surgery.
Another evaluation that may be useful in children who
have a tracheotomy is dye testing. Through the use of
green food coloring, aspiration in general as well as spe-
cific causes of aspiration can be assessed as follows: dye
can be placed on the tongue to evaluate aspiration of
saliva or secretions; a particular consistency of food can
be dyed to assess for consistency-specific aspiration; and
gastrostomy tube feeds can be dyed to assess aspiration
of refluxed feeds. Aspiration is suspected if stained secre-
tions or feeds are noted from the tracheotomy during
feeding or at any time after feeding. Dye testing provides
no value in the evaluation of aspiration in children with a
grade IV (complete) stenosis. Information obtained from
a swallowing evaluation becomes crucial in planning the
operative procedure and in counseling the family about
the potential risk of aspiration.
Because many airway reconstructive procedures
involve a compromise between voice quality and airway
improvement, preoperative voice evaluation has become
increasingly important.4–6 This evaluation provides key
information regarding the impact of the initial surgery on
voice, the potential impact of further surgery on voice, and
the status of vocal cord mobility. In some cases, operative
planning can be modified to offer a better balance between
long-term voice and airway concerns. When vocal fold
immobility is noted on the voice evaluation, the surgeon
should search for the specific etiology of the immobility, as
various conditions may appear similar on voice evaluation
but may be treated differently (e.g., posterior glottic steno-
sis, vocal cord paralysis, and fixation of the cricoarytenoid
joints). This assessment is best done during microlaryn-
goscopy and bronchoscopy. Counseling families about the
impact of airway surgery on future voice quality and the
options of voice therapy is also an important part of the
overall process.
MITIGATING FACTORS
Bacterial Colonization
Because of the prevalence of colonization by methicillin-
resistant Staphylococcus aureus (MRSA) and Pseudomonas
aeruginosa in our patients with complex aerodigestive
problems (approximately 30%), we recommend screen-
ing for these bacteria by culture of the nares and tracheal
aspirate. Both MRSA and Pseudomonas aeruginosa have
a predilection for infecting cartilage and can lead to oper-
ative failure. Patients who are found to be positive are
treated with perioperative and postoperative antimicro-
bial therapy (Table 69-2). In our experience, this protocol
decreases the risk of infection-related morbidity (authors’
unpublished data).
Eosinophilic Esophagitis
EE is an uncommon disorder that, if left untreated,
may have a significant effect on the aerodigestive tract.
Many patients who have EE also have esophageal,
laryngotracheal, and sinonasal complaints; however,
some patients are asymptomatic.7 The diagnosis of EE is

TABLE 69-2  PERIOPERATIVE AND OPERATIVE ANTIBIOTIC PROTOCOL FOR BACTERIAL COLONIZATION
ORGANISM PERIOPERATIVE INTRAOPERATIVE
Methicillin-resistant Bactrim DS: Vancomycin: 15 mg/kg 1 h before
Staphylococcus aureus 6-12 mg/kg/day, 72 h incision followed by q6h × 2 doses
before; Bactroban (maximum)
intranasal 72 h before
Pseudomonas aeruginosa Ciprodex: Piperacillin/Tazobactam: 100 mg/kg
intratracheal 72 h before 1 h before incision followed by q6h
dosing
made by histologic examination of biopsies taken from
the esophagus at the time of esophageal endoscopy. In
patients who have active EE, the laryngotracheal com-
plex is often inflamed. Surgery in the presence of active
EE often elicits a brisk inflammatory response that can
lead to graft failure or re-stenosis. If EE is present, the
authors recommend medical management followed by
repeat endoscopy with biopsies. Once biopsies dem-
onstrate no active EE, surgery may be performed. (See
Chapter 68 for a more detailed discussion on EE and its
treatment.)
Gastroesophageal Reflux Disease
The evaluation for GERD can include esophagoscopy
with biopsies, esophagram, impedance probe, and dual
pH probe testing. Because of the potential impact of this
condition on postoperative healing, the authors routinely
administer prophylactic preoperative and postoperative
therapy to patients undergoing airway reconstruction.
Most patients are managed with a daily proton pump
inhibitor and nighttime H2 blocker therapy. Patients
continue the antireflux regimen for up to 1 year fol-
lowing successful reconstruction. Nonacidic reflux also
can play a role. The authors believe that in some cases
non-acidic reflux can cause damage in the reconstructed
airway and potentially lead to operative failure. When
medical treatment fails or nonacidic reflux is suspected,
a Nissen fundoplication should be considered before air-
way reconstruction.8–10
Obstructive Sleep Apnea
Obstructive sleep apnea (OSA) can be difficult to diag-
nose and treat, and it can cause failure in an otherwise
well-executed operative plan. In children who have multi-
level airway obstruction, including a known fixed airway
lesion above a tracheotomy, OSA may be difficult to iden-
tify because the tracheotomy cannot be capped during a
sleep study. In these cases, a two-stage airway reconstruc-
tion is often performed, leaving the tracheotomy in place,
which allows for later assessment of OSA.
Pulmonary Disease
Unrecognized or untreated pulmonary disease can
increase the risk of operative failure. This broad clas-
sification of pathology encompasses numerous diseases
that affect the upper and lower respiratory systems,
Laryngeal and Tracheal Airway Disorders 971
Chapter 69
POSTOPERATIVE
Bactrim DS:
6-12 mg/kg/day for 2 weeks;
Vancomycin 15mg/kg/24 hr × 48 h
Ciprodex: Begin 1 week after surgery
through tracheal tube;
Piperacillin/Tazobactam 100 mg/kg q6h
for 1 week after surgery
including unrecognized but significant aspiration, bron-
chopulmonary dysplasia, tracheomalacia, bronchoma-
lacia, cystic fibrosis, and reactive airway disease. Failure
to identify any of these disease processes, even when the
commonly found subglottic stenosis is identified and
treated, can result in significant airway obstruction and
operative failure. Collaboration with a pediatric pulm-
onologist is important, not only in the diagnosis, but
also in both the short- and long-term management of
these patients. When significant pulmonary disease is
identified, it is crucial that surgery be delayed until this
pathology is treated.
Inappropriate Patient Selection
Patient selection can significantly affect overall clinical
outcome. Although the goal of creating an anatomi-
cally normal airway at the site of reconstruction may be
achieved from a technical perspective, if a child remains
dependent on a tracheotomy because of oxygen or venti-
lation requirements, or suffers from chronic aspiration,
then in a more global sense the operation has failed.
OPTIMIZATION
Optimizing patient status before surgery plays a cen-
tral role in airway management and is crucial to the suc-
cess of surgery. This point cannot be overemphasized.
Many of our patients have multiple comorbidities that
complicate treatment. Inadequate management of the
­aforementioned mitigating factors can have a negative
impact on an otherwise well-conceived and well-executed
surgical plan.
MANAGEMENT OF AIRWAY
PATHOLOGY
Laryngomalacia
Laryngomalacia is the most common cause of stridor in
newborns.11 Symptoms are usually observed at birth or
within the first few days of life. Stridor is generally mild,
but it typically worsens with feeding, crying, and lying
in a supine position. In 50% of patients, stridor wors-
ens during the first 6 months of life. A subset of children
with severe laryngomalacia (5%) may present with a
spectrum of symptoms, including apnea, cyanosis, severe
retractions, and failure to thrive. Also, many patients suf-
fer from clinically significant reflux. In extremely severe
972 Aerodigestive Disease
cases, cor pulmonale is seen. Although laryngomalacia
Section XI
usually resolves spontaneously by 1 year of age, severe
disease necessitates surgical intervention.
Diagnosis is confirmed by flexible transnasal fiberoptic
laryngoscopy. Characteristic findings include short ary-
epiglottic folds, with prolapse of the cuneiform cartilages.
In some patients, a tightly curled (Ω shaped) epiglottis
is observed. Because of the Bernoulli effect, characteris-
tic collapse of the supraglottic structures is seen on inspi-
ration. Inflammation indicative of reflux laryngitis may
also be seen.
Determining whether or not to intervene surgically
is based more on the severity of symptoms than on the
endoscopic appearance of the larynx. Patients with laryn-
gomalacia rarely present with acute airway compromise.
In the 5% who require surgical intervention, this may be
planned within 1 to 2 weeks of presentation. Preoperative
management of GER is recommended.
Supraglottoplasty (also referred to as epiglottoplasty)
is currently the operative procedure of choice. This pro-
cedure is quick and effective and can be adapted to the
infant's specific laryngeal pathology. Both aryepiglot-
tic folds are divided, and one or both cuneiform carti-
lages may also be removed. If the aryepiglottic folds
alone are divided, postoperative intubation is generally
not required. However, if more extensive surgery is per-
formed, overnight intubation is prudent.
Following supraglottoplasty, patients should be
observed overnight in the intensive care unit. In some
children, obstruction persists postoperatively. Repeat
fiberoptic laryngoscopy at the bedside is valuable in
determining whether this can be attributed to laryn-
geal edema or persistent laryngomalacia that necessi-
tates further surgery. Reflux management is helpful in
minimizing laryngeal edema. Occasionally, although
the postoperative appearance of the larynx is ade-
quate, obstructive symptoms are ongoing. Such cases
may have an underlying neurologic component, which
becomes more evident with time. Supraglottoplasty in
these children often fails, thus requiring tracheotomy
placement.
Vocal Cord Paralysis
Vocal cord paralysis is the second most common cause
of stridor in newborns, and it may be either congenital
or acquired.12 Congenital vocal cord paralysis generally
manifests bilaterally. Although it is usually idiopathic, it
is sometimes seen in children with central nervous system
pathology (e.g., hydrocephalus and Arnold-Chiari mal-
formation of the brainstem). Most children with bilateral
paralysis present with significant airway compromise,
though with an excellent voice. They usually do not aspi-
rate. Acquired disease is generally, though not always,
a unilateral condition arising from iatrogenic injury to
the recurrent laryngeal nerve. Because of the length and
course of the left recurrent nerve, this is far more likely
to be damaged than the right recurrent laryngeal nerve.
As such, acquired disease usually affects the left vocal
cord. Risk factors for acquired paralysis include patent
ductus arteriosus repair, the Norwood cardiac repair,
and esophageal surgery, particularly TEF repair. In older
children, thyroid surgery is an additional risk factor.
Unlike children with bilateral vocal cord paralysis, most
children with unilateral disease have an acceptable air-
way, but a breathy voice. These children are at a slightly
higher risk of aspiration.
The diagnosis of vocal cord paralysis is established
with awake flexible transnasal fiberoptic laryngoscopy
or stroboscopy. Once paralysis has been confirmed,
management depends on a number of factors. Children
with acquired vocal cord paralysis (whether unilateral or
bilateral) may experience spontaneous recovery several
months after nerve injury; however, this occurs only if
the nerve is stretched or crushed but is otherwise intact.
Children with unilateral paralysis can be initially
managed with observation, temporary injection medial-
ization, or speech and voice therapy. Determining the
appropriate option is based on a discussion with the
patient's family, taking into account the need for resto-
ration of normal voice and improvement of aspiration.
Regardless of which option is chosen, these children
should be observed for at least 1 year prior to any per-
manent intervention. If paralysis persists after this period
of time and there is a functional deficit, long-term inter-
ventions such as ansa-cervicalis re-innervation, perma-
nent medialization laryngoplasty, or long-term injection
medialization (fat or Radiesse) are considered. These
options are discussed with the family and are often
influenced by the age of the child and the presence of
comorbidities. Medialization laryngoplasty is best per-
formed after puberty.
For patients with bilateral paralysis associated with
an underlying disease process, successful treatment of
that disease may reverse the paralysis; however, up to
90% of these infants ultimately require tracheotomy
placement. Given that up to 50% of children with con-
genital idiopathic bilateral vocal cord paralysis have
spontaneous resolution of their paralysis by 1 year of
age, surgical intervention to achieve decannulation is
almost always delayed until patients are older than 1
year of age.
Several surgical options have been used for patients
with bilateral paralysis, and no particular option offers
a universally acceptable outcome. The aim of surgery is
twofold: (1) to achieve an adequate decannulated air-
way while maintaining voice and (2) to prevent aspira-
tion. Surgical options include laser cordotomy, partial or
complete arytenoidectomy (endoscopic or open), vocal
process lateralization (open or endoscopically guided),
and posterior cricoid cartilage grafting. In a child with
a tracheotomy, it is often prudent to maintain the tra-
cheotomy to ensure an adequate airway prior to decan-
nulation. In a nontracheotomized child, a single-stage
surgical procedure can be carried out. Acquired bilat-
eral vocal cord paralysis that does not resolve spon-
taneously is usually less responsive to treatment than
idiopathic vocal cord paralysis. In these cases, more
than one operative intervention may be required to
achieve decannulation. In patients who have undergone
such interventions, postextubation stridor may respond
to continuous positive airway pressure (CPAP) or high-
flow nasal cannula. The postoperative risk of aspiration
should be evaluated by a VSS before the child returns to

a normal diet. During the initial postoperative weeks,
some children have an increased risk of aspirating with
certain textures, especially thin fluids.
Laryngeal Webs
Laryngeal webs result from a failure of recanalization of
the glottic airway in the early weeks of embryogenesis.
In severe cases, as recanalization commences posteriorly
and progresses anteriorly, complete laryngeal atresia may
occur. In less severe cases, a thin anterior glottic web may
be the only remnant of the recanalization process. The
web is typically thickened anteriorly and thins out toward
the posterior edge.
Although some anterior glottic webs are gossamer thin,
most are thick and generally associated with a subglot-
tic “sail” that compromises the subglottic lumen. Patients
have varying degrees of glottic airway compromise, which
usually manifests in an abnormal cry or respiratory dis-
tress. Thin webs may elude detection, as neonatal intuba-
tion for airway distress may lyse the web.
Thick webs require open reconstruction with either
reconstruction of the anterior commissure or placement
of a laryngeal keel.13 The presence of thick membranous
webs requires placement of a tracheotomy in approxi-
mately 40% of patients.
Subglottic Stenosis
Subglottic stenosis (SGS) can be either congenital or
acquired. Congenital SGS in the neonate is defined as a
lumen 4.0 mm in diameter or less at the level of the cri-
coid cartilage. SGS is thought to result from a failure of
the laryngeal lumen to recanalize, and it is one of a con-
tinuum of embryologic failures that include laryngeal
atresia, stenosis, and webs. Congenital SGS is often asso-
ciated with other congenital head and neck lesions and
syndromes (e.g., a small larynx in a patient with Down
syndrome). Acquired SGS is far more common and is typ-
ically a sequel of prolonged neonatal intubation, often
with an inappropriately large endotracheal tube. Other
cofactors for the development of acquired SGS include
reflux and EE.
Levels of SGS severity are graded according to the
Myer-Cotton grading system (see Table 69-1). In its
mildest form (no obstruction to 50% obstruction),
congenital SGS appears as a normal cricoid cartilage
with a smaller than average diameter, usually ­elliptical
in shape. Mild SGS may manifest in recurrent upper
respiratory infections (often diagnosed as croup) in
which minimal subglottic swelling precipitates airway
obstruction. In a young child, the greatest obstruction
is usually 2 to 3 mm below the true vocal folds. More
severe cases may present with acute airway compromise
at delivery. If endotracheal intubation is ­successful, the
patient may require intervention before extubation.
When intubation cannot be achieved, t­racheotomy
placement at the time of delivery may be life-saving.
It is important to note that infants typically have sur-
prisingly few symptoms. Even those with grade III SGS
(71% to 99% obstruction) may not be symptomatic for
weeks or months.
Laryngeal and Tracheal Airway Disorders 973
Children with mild acquired SGS may be asymptom-
Chapter 69
atic or minimally symptomatic. Observation rather than
intervention may therefore be appropriate. This is often
the case for children with grades I or II SGS. Those with
more severe SGS (grades III and IV), however, are often
symptomatic, with either tracheal dependency or stridor
and exercise intolerance.
Radiologic evaluation of an airway that is not intu-
bated may provide the clinician clues about the site and
length of the stenosis. Useful imaging modalities include
inspiratory and expiratory lateral soft-tissue neck films,
fluoroscopy to demonstrate the dynamics of the trachea
and larynx, and chest radiograph. However, the single
most important investigation is high-kilovoltage airway
films. These films are taken not only to identify the clas-
sic “steepling” observed in patients with SGS, but also to
identify possible tracheal stenosis. The latter condition is
generally caused by complete tracheal rings, which may
predispose the patient to a life-threatening situation dur-
ing rigid endoscopy.
Whether SGS is congenital or acquired, evaluation
requires endoscopic assessment, which is considered the
gold standard. Endoscopy is necessary for the diagnosis
of laryngeal stenosis. Precise evaluation of the endolarynx
should be carried out, including grading of the subglot-
tic stenosis. Stenosis caused by scarring, granulation tis-
sue, submucosal thickening, or a congenitally abnormal
cricoid cartilage can be differentiated from SGS with a
normal cricoid, but endoscopic measurement with endo-
tracheal tubes or bronchoscopes is required for an accu-
rate evaluation.
In a patient with congenital SGS, the larynx will
grow as the patient grows. As such, after initial man-
agement of SGS, the patient may not require further
surgical intervention. However, if initial management
requires intubation, the risk of developing an acquired
SGS in addition to the underlying congenital SGS is
considerable.
Unlike congenital SGS, acquired SGS is unlikely to
resolve spontaneously and thus requires intervention.
Reconstruction of the subglottic airway is a challenging
procedure, and the patient's condition should be opti-
mized before undergoing surgery. In children with mild
symptoms and a minor degree of SGS, endoscopic inter-
vention may be effective. Endoscopic options include
radial incisions (cold steel or laser) through the steno-
sis, laryngeal dilatation, the application of topical or
injected steroids, and topical mitomycin. More severe
forms of SGS are better managed with open airway
reconstruction. Laryngotracheal reconstruction using
costal cartilage grafts placed through the split lamina
of the cricoid cartilage is reliable and has withstood the
test of time.14,15 Costal cartilage grafts may be placed
through the anterior lamina of the cricoid cartilage, the
posterior lamina of the cricoid cartilage, or both. These
procedures may be performed as a two-stage procedure,
maintaining the tracheal tube and temporarily placing
a suprastomal laryngeal stent above the tracheal tube.
Alternatively, in selective cases, a single-stage procedure
may be performed, with removal of the tracheal tube
on the day of surgery and with the child requiring intu-
bation for 1 to 14 days.16,17 Higher decannulation rates
974 Aerodigestive Disease
have reportedly been achieved with cricotracheal resec-
Section XI
tion than with laryngotracheal reconstruction in the
management of severe SGS.18,19 Cricotracheal resection
is, however, a technically demanding procedure that car-
ries a significant risk of complications.
Vascular Compression
Although vascular compression of the airway is not
uncommon, most affected children are either asymptom-
atic or only minimally symptomatic. Forms of vascular
compression affecting the trachea include innominate
artery compression (most common), double aortic arch,
and pulmonary artery sling. While symptomatic vascu-
lar compression of the trachea or bronchi is rare, it is
associated with marked symptoms, including biphasic
stridor, retractions, a brassy cough, and “dying spells.”
Symptoms tend to exacerbate when the child is distressed.
Vascular rings that result from a retroesophageal subcla-
vian artery and a ligamentum arteriosum are less likely to
be associated with airway compromise. Bronchial com-
pression by either the pulmonary arteries or aorta may be
significant, but in the absence of associated major cardiac
anomalies, it is typically a unilateral problem.
The diagnosis of airway compression is best established
with rigid and/or flexible bronchoscopy. Thoracic imaging
is then useful in assessing the intrathoracic vasculature.
Imaging modalities generally include high-resolution
computed tomography (HRCT) with contrast enhance-
ment and 3-D reconstruction, magnetic resonance imag-
ing (MRI), magnetic resonance angiography (MRA), and
echocardiography. In some cases, angiography is required.
In a neonate with acute airway compromise, intubation
may be required to stabilize the airway prior to defini-
tive treatment. In some cases, CPAP offers a degree of
temporary improvement, as segmental tracheomalacia
may be present in the region of the vascular compres-
sion. Prolonged intubation should be avoided because of
the risk of forming an arterial fistula from erosion of an
endotracheal tube into the area of compression. Similarly,
while tracheotomy will establish an unobstructed airway,
there is also an increased risk of an arterial fistula into
the airway.
The surgical management of symptomatic vascular
compression varies, depending on individual pathology.
Strategies for managing innominate artery compression
include thymectomy and aortopexy; however, if little thy-
mus is present, an alternative procedure is re-implanta-
tion of the innominate artery more proximally on the
aortic arch. A double aortic arch requires ligation of the
smaller of the two arches, which is usually the left. A pul-
monary artery sling is transected at its origin, dissected
free, and re-implanted into the pulmonary trunk anterior
to the trachea. There is a high incidence of complete tra-
cheal rings in children with a pulmonary artery sling, and
these should be repaired at the time of vascular repair.
Although alleviating vascular compression improves
the airway, it takes time for the airway to completely
normalize. This is a consequence of long-standing vas-
cular compression having adversely affected the normal
cartilaginous development of the compressed segment of
trachea, with resultant cartilaginous malacia or stenosis.
Until the airway normalizes, children who are persistently
symptomatic may require stabilization with a tracheot-
omy. Tracheal stabilization with the use of intratracheal
stents is alluring, but the incidence of complications
under such circumstances is nevertheless high. Placement
of a temporary tracheotomy is thus a more desirable
alternative.20
Posterior Laryngeal Clefts
Posterior laryngeal clefts result from a failure of the
laryngotracheal groove to fuse during embryogenesis. In
a widely used anatomic classification system, these clefts
are divided into four subtypes associated with varying
levels of severity; type 1 cleft is the least severe and type
4 cleft is the most severe.21 Other associated anomalies
are common and may be divided into those that affect
the airway and those that do not. Associated airway
anomalies include tracheomalacia (>80%) and TEF for-
mation (20%). Non-airway associations include anogen-
ital anomalies and GER. The most common syndrome
associated with posterior laryngeal clefting is Opitz-Frias
syndrome, which is characterized by hypertelorism, ano-
genital anomalies, and posterior laryngeal clefting.
Although aspiration is the hallmark clinical feature of
this disorder, signs and symptoms may be nonspecific,
making the diagnosis elusive. Symptoms may also include
apnea, recurrent pneumonia, feeding difficulties, and air-
way obstruction.
VSS and FEES may suggest the risk of aspiration for
children with clefts, however a definitive diagnosis
requires rigid laryngoscopy and bronchoscopy, with the
interarytenoid area being specifically probed to determine
if a posterior laryngeal cleft is present.
Initial management decisions should consider whether
the infant requires tracheotomy placement, gastrostomy
tube placement, or Nissen fundoplication. Although none
of these procedures is essential, each increases the likeli-
hood of successful cleft repair. Protection against aspi-
ration is also crucial, and nasojejunal feeding may be a
useful way of stabilizing an infant. Surgical repair may be
performed endoscopically for most type 1 and some type
II clefts; however, longer clefts that extend into the cervical
or thoracic trachea require open repair. The transtracheal
approach is advocated in that it provides unparalleled
exposure of the cleft while protecting the recurrent laryn-
geal nerves. A two-layer closure is recommended, with the
option of performing an interposition graft if warranted;
a useful interposition graft is a free transfer of clavicular
or tibial periosteum, or costal cartilage. Because all clefts
are prone to anastomotic breakdown, repeat endoscopy
and postoperative swallow studies should be performed
to evaluate persistent aspiration and confirm a successful
functional repair.22
Tracheomalacia
Tracheomalacia is the most common congenital tracheal
anomaly. Most children are either asymptomatic or min-
imally symptomatic, and most cases involve posterior
malacia of the trachealis, with associated broad tra-
cheal rings. Commonly associated abnormalities include

laryngeal clefts, TEF, and bronchomalacia. Presenting
symptoms include a brassy cough, wheezing, respira-
tory distress when agitated, and dying spells. Diagnosis
is established with rigid or flexible bronchoscopy while
maintaining spontaneous respiration. The key elements
of diagnosis include: (1) ascertaining the severity of the
malacia; (2) ascertaining the location of the malacia,
particularly the possible presence of associated broncho-
malacia; and (3) determining whether positive ­pressure
support improves the malacia.
Although mild tracheomalacia is watched expec-
tantly and anticipated to improve with time, more severe
symptoms warrant intervention.23 The most common
intervention is tracheotomy placement, with the tip of
the tracheotomy tube bypassing the malacic segment.
Positive pressure support delivered through the trache-
otomy tube assists with the management of associated
bronchomalacia. While there is currently no definitive
surgical approach to repair tracheomalacia, this is an area
of active research.
Complete Tracheal Rings
Complete tracheal rings are a rare, though life-threatening
anomaly that presents with progressive worsening of
respiratory function over the first few months of life, stri-
dor, retractions, and marked exacerbation of symptoms
during intercurrent upper respiratory tract infections.
Children with distal tracheal stenosis usually have a char-
acteristic biphasic wet-sounding breathing pattern that
transiently clears with coughing; this pattern is referred
to as “washing machine breathing.” The risk of respira-
tory failure increases with age.
An initial high-kilovolt airway radiograph may indicate
tracheal narrowing; however, the diagnosis is established
with rigid bronchoscopy. This should be performed with
Laryngeal and Tracheal Airway Disorders 975
utmost caution, using the smallest possible telescopes,
Chapter 69
as any airway edema in the region of the stenosis may
turn a narrow airway into an extremely critical airway.
If the stenosis is severe, the stenotic airway should not
be instrumented, even with the smallest telescope. The
initial bronchoscopic view is often sufficient to establish
the diagnosis, thereby avoiding the risk of airway edema.
Because 50% of children have a tracheal inner diame-
ter of approximately 2 mm at the time of diagnosis, the
standard interventions for managing a compromised air-
way are not applicable. More specifically, the smallest
endotracheal tube has an outer diameter of 2.9 mm, and
the smallest tracheotomy tube has an outer diameter of
3.9 mm; hence, the stenotic segment cannot be intubated.
This may leave extracorporeal membrane oxygenation
(ECMO) as the only viable alternative for stabilizing the
child. This situation is best avoided by performing bron-
choscopy with the highest level of care. Over 80% of chil-
dren with complete tracheal rings have other congenital
anomalies, which are generally cardiovascular in origin.
As such, investigation should include a contrast-enhanced
chest HRCT scan and an echocardiogram. Specifically, a
pulmonary artery sling should be excluded, as this is a
common association, and if present, should be repaired
concurrent with the tracheal repair. Most children with
complete tracheal rings require tracheal reconstruc-
tion.24 The recommended surgical technique is the slide
tracheoplasty.25 This approach yields significantly better
results than any other form of tracheal reconstruction
and is applicable to all anatomic variants of complete
tracheal rings.
References
The complete reference list is available online at www.
expertconsult.com