Pedi Airway and Esophagus

Question 1

What is the most common benign pediatric

laryngeal neoplasm?

Answer 1

Recurrent respiratory papillomatosis (RRP)

Question 2

What are the two most common age groups affected

by recurrent respiratory papillomatosis (RRP)?

Answer 2

● < 5 years = juvenile-onset recurrent respiratory papillo-
matosis (JORRP)

● > 40 years = adult onset recurrent respiratory papilloma-
tosis (AORRP)

Question 3

What are the three most common risk factors for

development of JORRP?

Answer 3

Clinical triad
Firstborn (longer labor) Mother is < 20 years of age (more likely a lower socio-economic status and recent infection)

Vaginal birth in a mother with genital chondylomata

Question 4

What is the strain of human papillomavirus (HPV)
most commonly responsible for JORRP, and what is
the most common anatomical area infected?

Answer 4

HPV 6 or 11. Larynx.

Question 5

What is the most frequent route of infection in

JORRP?

Answer 5

Vertical transmission during vaginal birth or less commonly
transplacental infection. In older children, infection can
occur via accidental inoculation or sexual abuse.

Question 6

How does the age of JORRP onset relate to disease

severity?

Answer 6

Children < 3 years of age require more frequent operations

(> four per year) and have disease involving more anatom-
ical subsites; 19% of children with a more aggressive course

will require > 40 surgical procedures in their lifetime.

Question 7

What symptoms are associated with JORRP?

Answer 7

Hoarseness, dysphonia, cough, dysphagia, inspiratory stridor, and potentially respiratory distress from airway obstruction

Question 8

The key to management of JORRP is surgical debulking procedures. Which techniques are com-
monly used?

Answer 8

Laser resection/ablation and microdebridement

Question 9

What is the most common antiviral agent used to

assist in treatment of JORRP?

Answer 9

Injection of cidofovir into the base of the lesion after
resection. In addition, interferon-α, indol-3-carbinol, HspE7,
and the mumps vaccine may be considered.

Question 10

Why is tracheostomy reserved only for severe cases

of JORRP with impending airway compromise?

Answer 10

There is a risk of spreading disease to the distal

tracheobronchial tree.

Question 11

Why should a biopsy be taken during surgical

debulking of RRP?

Answer 11

Document benign disease, document human papillomavi-
rus (HPV) infection, attain polymerase chain reaction (PCR)

for HPV serotype (prognostic), rule out carcinoma.

Question 12

What is the risk of malignant transformation in

JORRP?

Answer 12

< 1% but increased in patients with prolonged, extensive
disease and distal spread. HPV 11 is higher risk than is HPV
6.

Question 13

The Gardasil vaccine offers immunity against which

serotypes of HPV?

Answer 13

HPV 6, 11, 16, and 18

Question 14

Which common pediatric pathology is considered
the most common cause of acute-onset (often at
night) inspiratory stridor, barky cough, hoarseness,
and upper airway obstruction that can lead to
respiratory compromise?

Answer 14

Laryngotracheobronchitis (croup)

Question 15

What is the most common cause of

laryngotracheobronchitis (croup)?

Answer 15

Parainfluenza virus (up to 75%). The most common subtype
is parainfluenza type 1.

Question 16

Croup is caused by viral invasion of the laryngeal
mucosa that results in inflammation and edema.
Which region of the airway is predominantly
affected and narrowed?

Answer 16

Subglottis

Question 17

How can the Westley croup scale be used to

differentiate mild, moderate, and severe croup?

Answer 17

Westley croup scale
● Level of consciousness: Normal (including sleep) = 0,
altered = 5
● Cyanosis: None = 0; with agitation = 4; at rest = 5
● Stridor: None = 0; when agitated = 1; at rest = 2

● Air entry: Normal = 0; decreased = 1; markedly de-
creased = 2

● Intercostal retractions: None = 0; mild = 1; moderate = 2;
severe = 3
Severity
● Mild croup: ≤ 2 (e.g., barky cough, hoarse cry, no stridor
at rest)
● Moderate croup: 3 to 7 (e.g., stridor at rest, mild
retractions, little to no agitation)
● Severe croup: ≥ 8, (e.g., significant stridor at rest, severe
retractions, anxious/agitated/lethargic)

Question 18

Although clinical history and physical examination
are generally adequate for diagnosis of croup, what
imaging technique can be used when the diagnosis
is in question? What is the characteristic finding?

Answer 18

Anterior-posterior chest radiograph; “steeple sign” or sub-

glottic narrowing

Question 19

Although symptoms of croup often resolve within
48 hours, children can progress to respiratory
failure. Management generally rests on medical
intervention; the need for intubation or
tracheostomy is rare. What medical management
has been shown to improve symptoms in children
with mild, moderate, and severe croup?

Answer 19

● All children with respiratory distress may benefit from
supplemental oxygen.
● Mild: Single dose of oral dexamethasone
● Moderate: Dexamethasone, nebulized epinephrine, and/
or nebulized budesonide
● Severe: Dexamethasone, nebulized epinephrine

Question 20

If a child complains of isolated nocturnal stridor
but has an otherwise normal head and neck
examination with no evidence of infectious cause,
what is the likely diagnosis?

Answer 20

Acute spasmodic laryngitis (false croup)

Question 21

What pediatric pathology is associated with
cellulitis, edema, and inflammation of the
epiglottis, aryepiglottic folds, and arytenoid tissue
and is limited in its inferior extent by the tightly
bound epithelium of the true vocal folds?

Answer 21

Acute epiglottitis

Question 22

What is the cause of epiglottitis?

Answer 22

Most common cause: Haemophilus influenzae type b (Hib)
despite immunization (lack or failure of immunization).
Other common causes include Streptococcus pneumoniae,
Staphylococcus aureus, and β-hemolytic streptococcus.
Noninfectious causes include thermal or chemical injuries,

trauma, angioedema, hemophagocytic lymphohistiocyto-
sis, and some acute leukemias.

Question 23

Both epiglottitis and croup can manifest with fever,
cough, and noisy or effortful breathing. What
symptoms are more likely to be present only in
epiglottitis and may help in differentiating the two?

Answer 23

Drooling is reliably associated with epiglottitis (3 Ds of
epiglottitis are drooling, distress, and dysphagia). Less
reliable hallmarks include preference for sitting or sniffing
position, refusal to eat or drink, inability to swallow,
odynophagia, a higher grade temperature, and vomiting.

Question 24

True or false: Without intervention, children with
epiglottitis are at higher risk for airway obstruction
and death than those with croup.

Answer 24

True

Question 25

How is epiglottitis diagnosed in children?

Answer 25

● Mild distress (other diagnoses are more likely): Visualize

the epiglottis (using tongue depressor or flexible endos-
copy). The child should be kept in a calm environment

where an airway can be secured immediately. Antero-
posterior/lateral radiograph: “Thumbprinting” of the

epiglottis or supraglottic edema
● Moderate to severe distress: Do not attempt to visualize the
airway or otherwise disturb the child. IV, blood draw, rectal

temperature, etc., should be performed. Remember, bag-
valve-mask ventilation is feasible in almost all cases of

acute epiglottitis. An experienced provider should evaluate
the airway after intubation. After securing the airway,
blood work and airway cultures should be obtained.

Question 26

In a child diagnosed with epiglottitis, once the
airway is secured or deemed safe for observation
(in the intensive care unit, or ICU), what additional
medical management is indicated?

Answer 26

Empiric antibiotics (third-generation cephalosporin and an
antistaphylococcal agent active against methicillin-resistant
S. aureus [MRSA]) and possibly corticosteroids (although
controversial).

Question 27

What are common criteria for extubation in the

setting of acute epiglottitis?

Answer 27

Resolution of the inflammation, edema, and erythema of
the supraglottic structures on interval airway examination
(generally 2 to 3 days) and/or the presence of an air leak in
addition to clinical improvement

Question 28

What might predispose a patient to membranous

laryngotracheobronchitis (bacterial tracheitis)?

Answer 28

Previous trauma, viral infection, or anything that alters the
local immunity, thus increasing the risk of a bacterial
superinfection

Question 29

Bacterial superinfection of the larynx and tracheo-
bronchial tree mucosa result in a diffuse inflamma-
tory reaction associated with thick secretions and possible sloughing of fibrinous, mucopurulent, epi-
thelial lining material into the airway. Why is this more problematic in the pediatric population?

Answer 29

The smallest diameter in the pediatric airway is at the cricoid cartilage. Any edema or narrowing of this can significantly compromise a child’s respiratory status. Ac-
cording to Poiseuille’s law, airway resistance is inversely proportional to the radius of the airway to the fourth power.
So in a 4-mm infant airway, if there is 1 mm of edema, the
diameter is reduced by 50%, the cross-sectional area is
reduced by 75%, and resistance increases 16-fold. By
contrast, in an adult airway, 1 mm of edema only causes a
25% decrease in diameter, 44% decrease in area, and 3-fold
increase in resistance. More than 90% of children diagnosed
with bacterial tracheitis require intubation.

Question 30

Children with bacterial tracheitis often have fever,
dyspnea, retractions, a nonpainful cough, and
inspiratory stridor. What is the most common
cause of mortality in these children?

Answer 30

Airway obstruction resulting from sloughing of fibrinous/mucopurulent debris or membrane. Mortality rates (his-
torically high) have been decreasing as a result of early recognition, aggressive pulmonary toilet, early antibiotics,
and airway protection via intubation when necessary.

Question 31

What is the most common organism cultured from
the trachea (tracheal cultures are important for
diagnosis as blood cultures are often negative)
during an acute episode of bacterial tracheitis?

Answer 31

S. aureus

Question 32

True or false: Obtaining IV access in children with

bacterial tracheitis is not necessary.

Answer 32

False. Initiation of broad-spectrum empiric antibiotics is
imperative. However, IV access should not be attempted in
a child demonstrating respiratory distress, as agitation may
precipitate acute airway collapse. Once the airway is stable
or secured, obtain IV access.

Question 33

A 10-year-old girl has hoarseness, cough,
odynophagia, general malaise, and low-grade fever.
On examination, she has bilateral lymphadenopathy
and coalescing pseudomembranous plaques involving
her pharynx and larynx. She is a recent immigrant and
has no vaccination records. What is the likely
causative agent?

Answer 33

Corynebacterium diphtheriae (gram-positive bacillus). Diag-
nosis = culture and positive toxin assay

Question 34

How can diphtheria lead to myocarditis, nephritis,

and central nervous system (CNS) complications?

Answer 34

Systemic absorption of toxin

Question 35

How is diphtheria (1) prevented, and (2) treated?

Answer 35

1. Vaccination: Immune individuals can be asymptomatic

carriers.
2. Careful airway management (extreme caution with

intubation, early consideration for tracheostomy), diphthe-
ria antitoxin, erythromycin or penicillin, serial electro-
cardiograms and cardiac enzymes, serial neurologic checks,

symptomatic care, consideration for prophylactic treatment
of close contacts

Question 36

What results if, during the 10th week of gestation,
the epithelium that normally temporarily obliterates
the laryngeal lumen fails to recanalize?

Answer 36

Congenital laryngeal web. Most commonly noted in the

anterior commissure.

Question 37

What is the most common chromosomal anomaly

associated with laryngeal webs?

Answer 37

Chromosome 22q11.2 deletion

Question 38

What congenital syndromes are related to laryngeal

webs?

Answer 38

22q11.2 deletion syndromes (e.g., velocardiofacial syndro-
me, DiGeorge syndrome, conotruncal anomaly face syn-
drome

Question 39

Cohen’s classification of glottic webs can be helpful

to describe these rare lesions. Describe this system.

Answer 39

Cohen’s classification of glottic webs:
● Type I: Thin anterior web, < 35% glottic involvement, mild
hoarseness
● Type II: Thin to moderately thick web, 35 to 50% glottic
involvement, weak cry, mild airway symptoms
● Type III: Thick web, possible anterior cartilaginous
subglottic extension, 50 to 75% glottic involvement, weak
voice, moderate airway symptoms
● Type IV: Thick web, 75 to 90% glottic involvement,
cartilaginous subglottic extension, no cry, severe airway
distress (tracheostomy)

Question 40

You are performing a direct laryngoscopy on a newborn suffering from cyanosis, apnea, and stridor. Flexible fiberoptic laryngoscopy was suggestive of bilateral vocal-fold paralysis with no obvious webbing anteriorly. On palpation of the interarytenoid space, you note a thick band that is fixing the arytenoids and preventing adequate abduction.
What is your diagnosis?

Answer 40

Posterior laryngeal web

Question 41

You are consulted on a patient by the high-risk
maternal fetal medicine team to evaluate a fetus diagnosed radiographically with congenital high air-
way obstruction syndrome (CHAOS) resulting from nearly complete laryngeal atresia. What procedure(s)
offer a chance for survival and potential long-term
survival?

Answer 41

EXIT (ex utero intrapartum treatment) and tracheostomy.

With early detection, patients may undergo fetal broncho-
scopy with attempted wire tracheoplasty as an adjunct

procedure.

Question 42

How are laryngeal webs managed?

Answer 42

● Mild webs: Endoscopic division can be attempted but is

often unsuccessful.

● Posterior webs: Tracheostomy with delayed decannula-
tion, laryngotracheal reconstruction with posterior cri-
coidotomy, and grafting

● Anterior webs: Laryngotracheal reconstruction or lar-
yngofissure with Silastic keel placement, with or without

tracheostomy

Question 43

In a term infant, what measurement indicates

subglottic stenosis?

Answer 43

Subglottic, or cricoid, diameter < 3.5 mm

Question 44

In a term infant with recurrent prolonged episodes
of croup, no history of prior airway manipulation
(surgical or intubation), no history of trauma, and
neck films that suggest subglottic narrowing, what
underlying pathology might be found in the
evaluation?

Answer 44

Congenital subglottic stenosis

Question 45

What are the possible causes of congenital

subglottic stenosis?

Answer 45

Elliptical cricoid cartilage, laryngeal cleft, cricoid flattening
(possibly from a trapped first tracheal ring), a large anterior
lamella, generalized mucosal thickening

Question 46

How is subglottic stenosis graded?

Answer 46

Cotton-Myer grading system
● Grade I: < 50% obstruction
● Grade II: 51 to 70% obstruction
● Grade III: > 70% obstruction with a detectable lumen
● Grade IV: No detectable lumen

Question 47

Why is the management of congenital subglottic
stenosis different from that of acquired subglottic
stenosis?

Answer 47

Most congenital stenoses are cartilaginous and therefore do

not respond to dilation or laser ablation of soft tissue.

Question 48

How is congenital subglottic stenosis treated?

Answer 48

● Grade I: Generally conservative management

● Grade II and III: Tracheostomy or other surgical interven-
tion*

● Grade IV: Tracheostomy or other surgical intervention**
*Dilatation for soft stenosis, laryngotracheal reconstruction
with anterior and/or posterior grafts, anterior cricoid split
(rarely performed today), cricotracheal resection
**Cricotracheal resecton

Question 49

How can life-threatening subcutaneous emphysema

be avoided in laryngotracheal reconstruction (LTR)?

Answer 49

Leave a small drain (Penrose or rubber band) to allow egress

of air.

Question 50

When creating an anterior costal cartilage graft,
what is the most common shape that the cartilage
is carved into, and what is done with the
perichondrium?

Answer 50

The shape is a modified “boat” if there is no tracheostomy

site to close (i.e., no tracheostomy tube in place or double-
stage procedure). If performing a single-stage procedure

and closing tracheostoma, a teardrop shape is used.
Perichondrium is left intact facing toward the lumen.

Question 51

In a patient with subglottic stenosis and a posterior
glottic stenosis (grade II/III), what open procedure
is indicated for widening the patient’s airway?

Answer 51

Posterior cricoid split is done via laryngofissure or anterior
cricoid split and placement of posterior costal cartilage
graft, which may require suprastomal stenting for a period
postoperatively, but often an endotracheal tube in the
postoperative period will be an adequate stent.

Question 52

When performing a laryngofissure, should the

anterior commissure be divided?

Answer 52

No

Question 53

What percentage of the posterior lamina of the
cricoid cartilage should be divided during a
posterior cricoid split?

Answer 53

100%. It may extend to the interarytenoid space and into

the posterior tracheal wall.

Question 54

In a single-staged laryngotracheal reconstruction

(LTR), is the tracheostomy tube left in place?

Answer 54

No. This is the key difference between single- and double-
staged LTR procedures. Patients are generally nasotra-
cheally intubated at the end of the procedure and kept

intubated for 2 to 7 days, depending on the extent of the
surgical intervention.

Question 55

True or false: Single-staged laryngotracheal recon-
struction can be done only for stenosis requiring

an anterior graft.

Answer 55

False. Single-staged LTR may include anterior grafts,

posterior grafts, or both.

Question 56

What is the relationship between the tracheostoma
and the planned horizontal neck incision for open
LTR?

Answer 56

The incision should incorporate the superior margin of the

tracheostoma.

Question 57

In patients with severe grade III or grade IV
subglottic stenosis, what surgical approach may
be considered instead of LTR?

Answer 57

Cricotracheal resection (CTR) (cricoid resection, thyrotra-
cheal anastomosis) is the only option for grade IV subglottic

stenosis. Laryngotracheal reconstruction with anterior and
posterior grafts or CTR can be considered for high-grade III
subglottic stenosis.

Question 58

In a patient with a long segment of tracheal
stenosis or complete tracheal rings, what procedure
is often recommended?

Answer 58

Slide tracheoplasty

Question 59

What are the basic steps involved in pediatric

tracheostomy?

Answer 59

● The procedure can be done with the patient intubated with an endotracheal tube (ETT) or ventilating broncho-
scope.
● Horizontal incision halfway between the cricoid and
sternal notch; remove the fat (lipectomy).
● Dissect down to the trachea; this can be difficult to
palpate because the lung apices extend further superiorly
into the neck in infants and children and it can divide
isthmus or retract superiorly).
● Place right and left vertical polypropylene (e.g., 4–0
Prolene) stay sutures through the tracheal rings lateral to
planned tracheal incision through the second and third
rings.
● You may need to mature the stoma (suture stoma skin
edges to trachea), but this is usually not performed if
lipectomy is adequate and the wound is not excessively
deep.
● Make a vertical incision through two or three rings (the
ETT is placed somewhere between tracheal rings 2 and
4); no Bjork flap, no trachea removed, taking care not to
injure the cricoid.
● Withdraw the ETT or ventilating bronchoscope, and place
the tracheostomy tube.
● Using a flexible fiberoptic scope, check the tube position.
● Secure the tracheostomy tube.

Question 60

Why are stay sutures so important during pediatric

tracheostomy?

Answer 60

Sutures allow tracheal traction into the field for emergent
reinsertion of the tracheostomy tube after accidental
decannulation.

Question 61

What is the primary goal of open laryngeal surgery

for pediatric patients?

Answer 61

Decannulation

Question 62

In what procedure are the first and second tracheal
rings, cricoid, and inferior thyroid cartilage cut in
the midline to widen the diameter of the subglottis?

Answer 62

Anterior cricoid split. Anterior cricoid split without grafting
is rarely performed today.

Question 63

What are the most common complications of

pediatric tracheostomy?

Answer 63

● Early: Pneumothorax. hemorrhage, accidental decannu-
lation, tube obstruction, subcutaneous emphysema,

death
● Intermediate or late: Infection, accidental decannulation,
subglottic stenosis, granulation tissue, suprastomal
stenosis or collapse, difficult decannulation, death

Question 64

What is the most common benign laryngeal and

upper tracheal neoplasm in the newborn or infant?

Answer 64

Hemangioma

Question 65

What is the natural progression of infantile

subglottic hemangiomas?

Answer 65

Rapid growth for the first 6 months of life, relative stability
for about a year, slow involution with resolution when the
child is around 3 years of age

Question 66

What congenital syndrome can be associated with

subglottic hemangiomas?

Answer 66

PHACE (posterior fossa abnormalities and other brain
anomalies; hemangioma(s) of the cervicofacial region;
arterial cerebrovascular malformations; cardiac defects; eye
abnormalities)

Question 67

How are subglottic hemangiomas treated?

Answer 67

● Medical: Propranolol is first-line therapy; use steroids for

propranolol failure
● Surgical: Tracheostomy until resolution for obstructive
lesions; external surgical approaches (e.g., submucous

resection and laryngotracheal reconstruciton); can con-
sider laser ablation

Question 68

What disorder is caused by decreased laryngeal
tone, resulting in dynamic prolapse of supraglottic
tissue into the airway, inspiratory stridor, and airway
obstruction?

Answer 68

Laryngomalacia

Question 69

What is the most common cause of congenital

stridor?

Answer 69

Laryngomalacia (35 to 75%)

Question 70

Without intervention, when would you expect

laryngomalacia symptoms to resolve?

Answer 70

18 to 20 months (at 18 months, 75% have no stridor)

Question 71

What complications can result from severe

laryngomalacia?

Answer 71

Difficulty feeding, failure to thrive, apnea, cyanosis, cor

pulmonale, and cardiac failure

Question 72

Whereas neurologic, genetic, and cardiac diseases are
more common in infants with laryngomalacia, which
comorbidity is highly associated with laryngomalacia
and may need to be managed concomitantly with the
airway disease?

Answer 72

Gastroesophageal reflux disease, or GERD

Question 73

Why do some authorities recommend that, in
addition to laryngoscopy, a full evaluation of the
tracheobronchial tree be performed during the
evaluation of laryngomalacia?

Answer 73

Up to 17.5% will have an additional, synchronous lesion.

Question 74

What is the standard surgical treatment for

laryngomalacia?

Answer 74

Supraglottoplasty (division of aryepiglottic folds, debulking
of prolapsing arytenoid tissue, epiglottoplasty): cold steel
microlaryngeal instruments, CO2 laser, and microdebrider
have been reported.

Question 75

What are the indications for supraglottoplasty?

Answer 75

Laryngomalacia with failure to thrive and/or respiratory

distress (apneas, cyanosis, hypoxia).

Question 76

What complications are associated with

supraglottoplasty?

Answer 76

Transient dysphagia and aspiration (10 to 15%), failure or
partial improvement (8.8%; more common in children with
additional congenital anomalies), and supraglottic stenosis
(4%)

Question 77

What condition is caused by reduction and/or
atrophy of the longitudinal elastic fibers of the pars
membranacea of the trachea or impaired cartilage
integrity resulting in a soft and collapsible airway
that is worse with increased intrathoracic pressure
(Valsalva)?

Answer 77

Tracheomalacia

Question 78

What is primary tracheomalacia?

Answer 78

Tracheomalacia is the most common congenital anomaly of
the trachea; it is more often seen in premature infants and
is thought to be due to tracheobronchial cartilage
immaturity or irregularity. It can include true immaturity or
diseases resulting in the malformation of the cartilage
matrix such as polychondritis, chondromalacia, or other
congenital anomalies affecting the cartilage. It can also be
due to anatomical anomalies leading to insufficient
cartilaginous support such as tracheoesophageal fistula.

Question 79

What is secondary tracheomalacia?

Answer 79

Secondary (acquired) tracheomalacia can result from
degeneration of normal cartilaginous support and is more
common than primary tracheomalacia. It can be due to

prolonged intubation, tracheostomy, recurrent tracheo-
bronchitis, external tracheal compression (cardiovascular

abnormalities, skeletal anomalies, and space-occupying
lesions).

Question 80

What is the normal ratio of cartilage to muscle

within the trachea and in a child with tracheomalacia?

Answer 80

● Normal: Ratio of cartilage to muscle is 4.5:1.
● Tracheomalacia: The amount of cartilage decreases, thus
decreasing the ratio of cartilage to muscle. Some

authorities recommend reserving the diagnosis of tra-
cheomalacia for patients presenting with a ratio of 2:1.

Question 81

How is tracheomalacia classified based on

histologic, endoscopic, and clinical signs?

Answer 81

The major airway collapse (MAC) classification system (Mair
and Parsons, 1992, PMID: 1562133):
● Type 1: Congenital or intrinsic tracheal collapse without
airway compression. Patients may have prematurity,

esophageal atresia, or tracheoesophageal fistula, muco-
polysaccharidoses, and Larsen syndrome.

● Type 2: Extrinsic tracheal compression resulting in airway
collapse. Patients may have cardiovascular anomalies,
skeletal anomalies, or space-occupying lesions and may
be primary or secondary.
● Type 3: Secondary (acquired) tracheomalacia results from
prolonged intubation, tracheotomy, or severe/recurrent
tracheobronchitis.

Question 82

How is tracheomalacia managed?

Answer 82

Although most infants outgrow the symptoms of trache-
omalacia by 18 to 24 months of age, surgical intervention

(correction of extrinsic compressive lesion, tracheostomy,
aortopexy, stenting or possible tracheal grafts) may be
required when conservative methods fail or the child
develops life-threatening symptoms such as reflex apnea.

Question 83

What normal reflex mechanism can also be
amplified in children with tracheomalacia and result
in “death attacks,” “dying spells,” or cardiorespiratory
arrest when the trachea is stimulated by secretions, a
bolus of food in the esophagus, or pressure from an

esophagoscope or bronchoscope during examina-
tion?

Answer 83

Reflex apnea

Question 84

Describe the adult vascular structure that forms from
the following embryologic branchial arches:
● First arch
● Second arch
● Third arch
● Fourth arch
● Fifth arch
● Sixth arch
● Intersegmental arteries

Answer 84

1st None: Involutes
2nd None: Involutes
3rd Carotid system
4th Aortic arch
5th Atretic or never fully develops
6th Pulmonary artery from ventral portion; dorsal portion of
right arch disappears while the left dorsal arch becomes
the ductus arteriosus
Intersegmental arteries–> Subclavian arteries

Question 85

Any vascular anomaly that causes compression of
the trachea and/or esophagus may be considered a
vascular ring. How are complete and incomplete
rings distinguished?

Answer 85

● Complete: Arterial derivatives of the branchial arch system
that encircle the trachea and esophagus
● Incomplete: Arterial derivatives that encircle the trachea
and esophagus with and without ligaments and fibrous
bands

Question 86

What are the most common initial symptoms

associated with vascular rings?

Answer 86

The symptoms depend on the degree of compression.
Biphasic or inspiratory stridor, recurrent upper respiratory
infections, cough, and dysphagia. Severe symptoms include
“death spells” or acute apnea and cyanotic spells, often
requiring cardiopulmonary resuscitation; these can occur
with tracheal secretions, a bolus of food in the esophagus,
or pressure on the trachea during esophagoscopy or
bronchoscopy or they may be completely asymptomatic.

Question 87

What are the most common findings on barium
esophagram and bronchoscopy for the following
vascular anomalies?
● Double aortic arch
● Right aortic arch anomaly
● Anomalous innominate artery
● Pulmonary artery sling
● Aberrant right subclavian artery

Answer 87

Barium swallow
double aortic arch● Posterior and bilateral compression
right aortic arch anomaly● Right posterior and lateral compression
Anomalous innominate artery● None
Pulmonary artery sling ● Anterior compression
Aberrant right subclavian artery ● Posterior compression
Bronchoscopy
● Anterior and bilateral compression

Question 88

What are the two most common forms of vascular

rings?

Answer 88

● Double aortic arch (ascending aortic arch wraps around
the trachea and esophagus, creating a complete ring;
most common)
● Persistent right aortic arch with a left ligamentum
arteriosum and retroesophageal left subclavian artery
(incomplete ring)

Question 89

What vascular anomaly produces severe early tracheal compression, has a common site of esophageal and tracheal compression, is thought to arise from
the left pulmonary artery originating from the right pulmonary artery, and is commonly associated with complete tracheal rings and distal bronchial
hypoplasia?

Answer 89

Pulmonary artery sling

Question 90

If the innominate artery arises from the aorta to
the left of the trachea, it may result in symptomatic
compression of the trachea that can be seen
bronchoscopically as a triangular compression, which
if compressed with an endoscope will result in
dampening of the right radial pulse. What is the
vascular anomaly?

Answer 90

Anomalous innominate artery

Question 91

What is the likely diagnosis for a patient with solid
food dysphagia and a barium esophagram that
shows left to right posterior compression from
inferior to superior?

Answer 91

Anomalous right subclavian artery

Question 92

What term is used to describe dysphagia caused
by extrinsic compression of an anomalous right
subclavian artery?

Answer 92

Dysphagia lusoria

Question 93

True or false: Surgical intervention in the form of
pexy, reimplantation, or ligation, depending on the
clinical scenario, should be recommended for all
symptomatic patients with a vascular ring or anomaly.

Answer 93

True. Delay can increase the risk of sudden death, as well as
tracheal and bronchial sequelae. Outcomes are excellent
unless comorbid conditions (such as cardiac pathology) are
present.

Question 94

If a patient has no respiratory symptoms, what

conservative therapy can be tried to manage dys-
phagia lusoria?

Answer 94

Dietary modification

Question 95

A child with severe mental retardation, hypertelorism, hypotonia, microcephaly, downward slanting palpebral fissures, strabismus, low-set ears, beaklike profile, failure to thrive, and a history of a high-pitched catlike
cry in infancy most likely suffers from what congenital
anomaly?

Answer 95

Cri-du-chat syndrome (5p deletion syndrome)

Question 96

What are the laryngeal findings in a patient with

cri-du-chat syndrome?

Answer 96

Findings range from a normal examination (suggesting central reason to cry) to a characteristic narrow, diamond-
shaped larynx during inspiration, posterior commissure airleak, and flaccid epiglottis.

Question 97

Failed fusion of the posterior cricoid lamina and
incomplete development of the tracheoesophageal
septum result in what pathologies?

Answer 97

Posterior laryngeal clefts and laryngotracheoesophageal

clefts

Question 98

What symptoms might suggest laryngeal and

laryngotracheoesophageal clefts?

Answer 98

Symptoms may include feeding difficulty, failure to thrive,
aspiration, chronic cough, stridor, recurrent pneumonia,
airway obstruction, wheezing, stridor, noisy breathing, and
hoarseness. Significant defects can cause severe aspiration
and respiratory distress.

Question 99

What is the “gold standard” for diagnosis of

laryngeal clefts?

Answer 99

Microlaryngoscopy and bronchoscopy are performed under
general anesthesia with spontaneous ventilation, including
palpation of the interarytenoid space and evaluation of the
interarytenoid notch, which is normally about 3 mm (a
deeper notch may indicate a more incompetent larynx).

Question 100

What anatomical anomaly is frequently associated with posterior laryngeal and laryngotracheoesopha-
geal clefts and may have a significant negative impact on surgical repair if not adequately addressed?

Answer 100

Tracheoesophageal fistula

Question 101

While evaluating a patient with a type II laryngeal
cleft, you perform esophagoscopy and biopsy, as
well as bronchoscopy, bronchoalveolar lavage, and
analysis of lipid-laden macrophages. What are you
looking for?

Answer 101

Gastroesophageal reflux and aspiration

Question 102

Describe the Benjamin-Inglis classification of

posterior laryngeal and laryngoesophageal clefts.

Answer 102

Benjamin-Inglis classification
Occult cleft: Appreciated only by palpation or measurement
of posterior arytenoid height.
● Type 1: Limited to supraglottic interarytenoid area
● Type 2: Partial clefting of the posterior cricoid cartilage
● Type 3: Cleft of the entire cricoid cartilage and cervical
portion of the tracheoesophageal membrane, stopping
above the thoracic inlet
● Type 4: Cleft involves a significant portion of the
intrathoracic tracheoesophageal wall and may extend to the carina.

Question 103

Describe the Meyer-Cotton classification of

laryngeal and laryngoesophageal clefts.

Answer 103

Meyer-Cotton classification
● LI: Interarytenoid cleft
● LII: Partial cricoid cleft
● LIII: Complete cricoid cleft
● LTEI: Into cervical esophagus
● LTEII: Into thoracic esophagus

Question 104

A significant number of patients with either
laryngeal or tracheoesophageal clefts have
associated comorbidities. Which are the most
common?

Answer 104

● Tracheoesophageal fistula
● Esophageal atresia
● Congenital heart disease
● Cleft lip
● Cleft palate
● Micrognathia
● Glossoptosis
● Laryngomalacia
● Opitz-Frias syndrome

Question 105

A patient with hypospadias, hypertelorism, dyspha-
gia, a posterior laryngeal cleft, cleft lip/palate, and bifid scrotum, uvula, and tongue is most likely to
have what syndrome?

Answer 105

Opitz-Frias (G syndrome)

Question 106

Patients with which congenital syndrome can
develop posterior laryngotracheal cleft, polydactyly,
bifid epiglottis, imperforate anus, renal abnormalities,
pituitary and hypothalamic abnormalities, and
hamarblastomas?

Answer 106

Pallister-Hall (congenital hypothalamic hamarblastomas;

mutation in GLI3)

Question 107

True or false: Patients with newly diagnosed
laryngeal or laryngoesophageal clefts should undergo a trial of observation before surgical inter-
vention is considered.

Answer 107

False. Early surgical intervention has been recommended to
decrease the risk of irreversible pulmonary complications
associated with aspiration.

Question 108

For most clefts that involve the cricoid cartilage
and all clefts that extend beyond the cricoid
cartilage, would you recommend an endoscopic or
open approach?

Answer 108

Open surgical approach. Anterior laryngofissure, two-layer
approach, interposition grafting (sternocleidomastoid

muscle, inferiorly based strap musculature, tibial perios-
teum, auricular cartilage, temporalis fascia), or tracheoe-
sophagoplasty have all been used.

Question 109

What comorbid condition can significantly
compromise the success of surgical repair of a
laryngoesophageal cleft?

Answer 109

Gastroesophageal reflux

Question 110

You are evaluating an infant with inspiratory
stridor and suspect a vocal-fold paralysis. What are
the most common causes in newborns?

Answer 110

Traumatic birth, neurologic pathology, iatrogenic, or

idiopathic (most common)

Question 111

What are the most common initial clinical signs in
infants and children with unilateral vocal-fold
paralysis?

Answer 111

Breathiness, hoarseness, straining, muscle tension, and soft

voice

Question 112

In an infant diagnosed with idiopathic vocal-fold
paralysis on awake flexible fiberoptic examination,
what important possible cause should be
investigated with a brain MRI?

Answer 112

Arnold-Chiari malformation (or other brainstem compres-

sive pathologies)

Question 113

In infants with unilateral vocal-fold paralysis,
what is the likelihood of spontaneous recovery
after birth trauma or after neurologic or idiopathic
paralysis?

Answer 113

~ 70%

Question 114

In a newborn with bilateral vocal-fold paralysis,
what is the likelihood of spontaneous recovery if
the cause is a neurologic disorder or idiopathic?

Answer 114

~ 50%

Question 115

What is the treatment for congenital unilateral

vocal-fold paralysis?

Answer 115

Depends on severity, age of the patient, and the cause
Conservative management, tracheostomy, injection of filler
material, or thyroplasty

Question 116

Surgical intervention for congenital bilateral vocal-

fold paralysis attempts to ________ the true vocal folds to improve the airway.

Answer 116

Lateralize

Question 117

What diagnosis is defined as failure of the voice
to drop to a normal pitch at puberty and can
persist well beyond the normal age of puberty?

Answer 117

Mutational falsetto or puberphonia resulting from muscular
incoordination, hyperfunction of the cricothyroid muscle, or
psychological dysfunction

Question 118

In patients with puberphonia or mutational

falsetto, what is the first line of treatment?

Answer 118

Voice therapy and/or psychotherapy. For recalcitrant dis-

ease, a type 3 thyroplasty can be considered.

Question 119

What are common nonpulmonary indications for

pediatric tracheostomy?

Answer 119

Acquired subglottic stenosis (31.4%)
Bilateral vocal-cord paralysis (22.2%)
Congenital airway malformations (22.2%)
Tumors (11.1%)

Question 120

Although tracheostomy tube diameter and length
must be chosen carefully on an individual basis,
what helpful formula(s) can assist in predicting the
correct inner and outer diameter?

Answer 120

Age
● Inner diameter (mm) = age (years)/3 + 3.5
● Outer diameter (mm) = age (years)/3 + 5.5
Weight
● Inner diameter (mm) = [weight (kg) x 0.08] + 3.1
● Outer diameter (mm) = [weight (kg) x 0.1] + 4.7

Question 121

Describe the embryology of isolated esophageal

atresia.

Answer 121

Esophageal atresia is defined as an incomplete formation of
the esophagus. Isolated esophageal atresia is due to failure
of the recanalization of the esophagus during the eighth
week of development.

Question 122

What percentage of patients with congenital
anomalies of the aerodigestive tract has isolated
esophageal atresia?

Answer 122

About 85% of patients with esophageal atresia have a distal
tracheoesophageal fistula. Ten percent manifest with
isolated esophageal atresia and about 5% with isolated
tracheoesophageal fistula

Question 123

What are the various types of esophageal atresia
(EA) with or without tracheoesophageal
fistula (TEF)?

Answer 123

● EA with distal TEF (most common)
● Isolated EA
● Isolated TEF
● EA with proximal TEF
● EA with double TEF

Question 124

What are prenatal signs of esophageal atresia?

Answer 124

● Polyhydramnios in the mother

● Inability to identify the fetal stomach bubble on a
prenatal ultrasonogram

Question 125

What congenital anomalies are commonly

associated with esophageal atresia?

Answer 125

Found in approximately 50% of patients:

● Musculoskeletal: Hemivertebrae, radial dysplasia or ame-
lia, polydactyly, syndactyly, rib malformations, scoliosis,

lower limb defects
● Gastrointestinal: Imperforate anus, duodenal atresia,

malrotation, intestinal malformations, Meckel diverticu-
lum, annular pancreas

● Cardiac (most commonly associated): Ventricular septal
defect, patent ductus arteriosus, tetralogy of Fallot, atrial
septal defect, single umbilical artery, right-sided aortic arch
● Genitourinary: Renal agenesis or dysplasia, horseshoe

kidney, polycystic kidney, ureteral and urethral malfor-
mations, hypospadias

Question 126

What association is commonly diagnosed with

esophageal atresia with or without TEF?

Answer 126

VACTERL (10%): (V) vertebral defects, (A) anal atresia, (C)
cardiac malformations, (TE) tracheoesophageal fistula with
esophageal atresia, (R) renal dysplasia and (L) limb
anomalies (most commonly radial anomalies)