Pedi Airway and Esophagus Flashcards
(126 cards)
1
Q
What is the most common benign pediatric
laryngeal neoplasm?
A
Recurrent respiratory papillomatosis (RRP)
2
Q
What are the two most common age groups affected
by recurrent respiratory papillomatosis (RRP)?
A
● < 5 years = juvenile-onset recurrent respiratory papillo-
matosis (JORRP)
● > 40 years = adult onset recurrent respiratory papilloma-
tosis (AORRP)
3
Q
What are the three most common risk factors for
development of JORRP?
A
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
4
Q
What is the strain of human papillomavirus (HPV)
most commonly responsible for JORRP, and what is
the most common anatomical area infected?
A
HPV 6 or 11. Larynx.
5
Q
What is the most frequent route of infection in
JORRP?
A
Vertical transmission during vaginal birth or less commonly
transplacental infection. In older children, infection can
occur via accidental inoculation or sexual abuse.
6
Q
How does the age of JORRP onset relate to disease
severity?
A
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.
7
Q
What symptoms are associated with JORRP?
A
Hoarseness, dysphonia, cough, dysphagia, inspiratory stridor, and potentially respiratory distress from airway obstruction
8
Q
The key to management of JORRP is surgical debulking procedures. Which techniques are com-
monly used?
A
Laser resection/ablation and microdebridement
9
Q
What is the most common antiviral agent used to
assist in treatment of JORRP?
A
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.
10
Q
Why is tracheostomy reserved only for severe cases
of JORRP with impending airway compromise?
A
There is a risk of spreading disease to the distal
tracheobronchial tree.
11
Q
Why should a biopsy be taken during surgical
debulking of RRP?
A
Document benign disease, document human papillomavi-
rus (HPV) infection, attain polymerase chain reaction (PCR)
for HPV serotype (prognostic), rule out carcinoma.
12
Q
What is the risk of malignant transformation in
JORRP?
A
< 1% but increased in patients with prolonged, extensive
disease and distal spread. HPV 11 is higher risk than is HPV
6.
13
Q
The Gardasil vaccine offers immunity against which
serotypes of HPV?
A
HPV 6, 11, 16, and 18
14
Q
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?
A
Laryngotracheobronchitis (croup)
15
Q
What is the most common cause of
laryngotracheobronchitis (croup)?
A
Parainfluenza virus (up to 75%). The most common subtype is parainfluenza type 1.
16
Q
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?
A
Subglottis
17
Q
How can the Westley croup scale be used to
differentiate mild, moderate, and severe croup?
A
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)
18
Q
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?
A
Anterior-posterior chest radiograph; “steeple sign” or sub-
glottic narrowing
19
Q
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?
A
● 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
20
Q
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?
A
Acute spasmodic laryngitis (false croup)
21
Q
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?
A
Acute epiglottitis
22
Q
What is the cause of epiglottitis?
A
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.
23
Q
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?
A
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.
24
Q
True or false: Without intervention, children with
epiglottitis are at higher risk for airway obstruction
and death than those with croup.
A
True
25
How is epiglottitis diagnosed in children?
● 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.
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?
Empiric antibiotics (third-generation cephalosporin and an
antistaphylococcal agent active against methicillin-resistant
S. aureus [MRSA]) and possibly corticosteroids (although
controversial).
27
What are common criteria for extubation in the
| setting of acute epiglottitis?
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
28
What might predispose a patient to membranous
| laryngotracheobronchitis (bacterial tracheitis)?
Previous trauma, viral infection, or anything that alters the
local immunity, thus increasing the risk of a bacterial
superinfection
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?
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.
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?
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.
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?
S. aureus
32
True or false: Obtaining IV access in children with
| bacterial tracheitis is not necessary.
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.
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?
```
Corynebacterium diphtheriae (gram-positive bacillus). Diag-
nosis = culture and positive toxin assay
```
34
How can diphtheria lead to myocarditis, nephritis,
| and central nervous system (CNS) complications?
Systemic absorption of toxin
35
How is diphtheria (1) prevented, and (2) treated?
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
36
What results if, during the 10th week of gestation,
the epithelium that normally temporarily obliterates
the laryngeal lumen fails to recanalize?
Congenital laryngeal web. Most commonly noted in the
| anterior commissure.
37
What is the most common chromosomal anomaly
| associated with laryngeal webs?
Chromosome 22q11.2 deletion
38
What congenital syndromes are related to laryngeal
| webs?
22q11.2 deletion syndromes (e.g., velocardiofacial syndro-
me, DiGeorge syndrome, conotruncal anomaly face syn-
drome
39
Cohen's classification of glottic webs can be helpful
| to describe these rare lesions. Describe this system.
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)
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?
Posterior laryngeal web
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?
EXIT (ex utero intrapartum treatment) and tracheostomy.
With early detection, patients may undergo fetal broncho-
scopy with attempted wire tracheoplasty as an adjunct
procedure.
42
How are laryngeal webs managed?
● 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
43
In a term infant, what measurement indicates
| subglottic stenosis?
Subglottic, or cricoid, diameter < 3.5 mm
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?
Congenital subglottic stenosis
45
What are the possible causes of congenital
| subglottic stenosis?
Elliptical cricoid cartilage, laryngeal cleft, cricoid flattening
(possibly from a trapped first tracheal ring), a large anterior
lamella, generalized mucosal thickening
46
How is subglottic stenosis graded?
```
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
```
47
Why is the management of congenital subglottic
stenosis different from that of acquired subglottic
stenosis?
Most congenital stenoses are cartilaginous and therefore do
| not respond to dilation or laser ablation of soft tissue.
48
How is congenital subglottic stenosis treated?
● 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
49
How can life-threatening subcutaneous emphysema
| be avoided in laryngotracheal reconstruction (LTR)?
Leave a small drain (Penrose or rubber band) to allow egress
| of air.
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?
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.
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?
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.
52
When performing a laryngofissure, should the
| anterior commissure be divided?
No
53
What percentage of the posterior lamina of the
cricoid cartilage should be divided during a
posterior cricoid split?
100%. It may extend to the interarytenoid space and into
| the posterior tracheal wall.
54
In a single-staged laryngotracheal reconstruction
| (LTR), is the tracheostomy tube left in place?
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.
55
True or false: Single-staged laryngotracheal recon-
struction can be done only for stenosis requiring
an anterior graft.
False. Single-staged LTR may include anterior grafts,
| posterior grafts, or both.
56
What is the relationship between the tracheostoma
and the planned horizontal neck incision for open
LTR?
The incision should incorporate the superior margin of the
| tracheostoma.
57
In patients with severe grade III or grade IV
subglottic stenosis, what surgical approach may
be considered instead of LTR?
```
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.
58
In a patient with a long segment of tracheal
stenosis or complete tracheal rings, what procedure
is often recommended?
Slide tracheoplasty
59
What are the basic steps involved in pediatric
| tracheostomy?
● 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.
60
Why are stay sutures so important during pediatric
| tracheostomy?
Sutures allow tracheal traction into the field for emergent
reinsertion of the tracheostomy tube after accidental
decannulation.
61
What is the primary goal of open laryngeal surgery
| for pediatric patients?
Decannulation
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?
Anterior cricoid split. Anterior cricoid split without grafting
is rarely performed today.
63
What are the most common complications of
| pediatric tracheostomy?
● 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
64
What is the most common benign laryngeal and
| upper tracheal neoplasm in the newborn or infant?
Hemangioma
65
What is the natural progression of infantile
| subglottic hemangiomas?
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
66
What congenital syndrome can be associated with
| subglottic hemangiomas?
PHACE (posterior fossa abnormalities and other brain
anomalies; hemangioma(s) of the cervicofacial region;
arterial cerebrovascular malformations; cardiac defects; eye
abnormalities)
67
How are subglottic hemangiomas treated?
● 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
68
What disorder is caused by decreased laryngeal
tone, resulting in dynamic prolapse of supraglottic
tissue into the airway, inspiratory stridor, and airway
obstruction?
Laryngomalacia
69
What is the most common cause of congenital
| stridor?
Laryngomalacia (35 to 75%)
70
Without intervention, when would you expect
| laryngomalacia symptoms to resolve?
18 to 20 months (at 18 months, 75% have no stridor)
71
What complications can result from severe
| laryngomalacia?
Difficulty feeding, failure to thrive, apnea, cyanosis, cor
| pulmonale, and cardiac failure
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?
Gastroesophageal reflux disease, or GERD
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?
Up to 17.5% will have an additional, synchronous lesion.
74
What is the standard surgical treatment for
| laryngomalacia?
Supraglottoplasty (division of aryepiglottic folds, debulking
of prolapsing arytenoid tissue, epiglottoplasty): cold steel
microlaryngeal instruments, CO2 laser, and microdebrider
have been reported.
75
What are the indications for supraglottoplasty?
Laryngomalacia with failure to thrive and/or respiratory
distress (apneas, cyanosis, hypoxia).
76
What complications are associated with
| supraglottoplasty?
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%)
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)?
Tracheomalacia
78
What is primary tracheomalacia?
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.
79
What is secondary tracheomalacia?
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).
80
What is the normal ratio of cartilage to muscle
| within the trachea and in a child with tracheomalacia?
● 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.
81
How is tracheomalacia classified based on
| histologic, endoscopic, and clinical signs?
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.
82
How is tracheomalacia managed?
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.
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?
Reflex apnea
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
```
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
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?
● 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
86
What are the most common initial symptoms
| associated with vascular rings?
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.
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
```
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
88
What are the two most common forms of vascular
| rings?
● 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)
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?
Pulmonary artery sling
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?
Anomalous innominate artery
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?
Anomalous right subclavian artery
92
What term is used to describe dysphagia caused
by extrinsic compression of an anomalous right
subclavian artery?
Dysphagia lusoria
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.
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.
94
If a patient has no respiratory symptoms, what
conservative therapy can be tried to manage dys-
phagia lusoria?
Dietary modification
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?
Cri-du-chat syndrome (5p deletion syndrome)
96
What are the laryngeal findings in a patient with
| cri-du-chat syndrome?
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.
97
Failed fusion of the posterior cricoid lamina and
incomplete development of the tracheoesophageal
septum result in what pathologies?
Posterior laryngeal clefts and laryngotracheoesophageal
| clefts
98
What symptoms might suggest laryngeal and
| laryngotracheoesophageal clefts?
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.
99
What is the “gold standard” for diagnosis of
| laryngeal clefts?
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).
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?
Tracheoesophageal fistula
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?
Gastroesophageal reflux and aspiration
102
Describe the Benjamin-Inglis classification of
| posterior laryngeal and laryngoesophageal clefts.
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.
103
Describe the Meyer-Cotton classification of
| laryngeal and laryngoesophageal clefts.
```
Meyer-Cotton classification
● LI: Interarytenoid cleft
● LII: Partial cricoid cleft
● LIII: Complete cricoid cleft
● LTEI: Into cervical esophagus
● LTEII: Into thoracic esophagus
```
104
A significant number of patients with either
laryngeal or tracheoesophageal clefts have
associated comorbidities. Which are the most
common?
```
● Tracheoesophageal fistula
● Esophageal atresia
● Congenital heart disease
● Cleft lip
● Cleft palate
● Micrognathia
● Glossoptosis
● Laryngomalacia
● Opitz-Frias syndrome
```
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?
Opitz-Frias (G syndrome)
106
Patients with which congenital syndrome can
develop posterior laryngotracheal cleft, polydactyly,
bifid epiglottis, imperforate anus, renal abnormalities,
pituitary and hypothalamic abnormalities, and
hamarblastomas?
Pallister-Hall (congenital hypothalamic hamarblastomas;
| mutation in GLI3)
107
True or false: Patients with newly diagnosed
laryngeal or laryngoesophageal clefts should undergo a trial of observation before surgical inter-
vention is considered.
False. Early surgical intervention has been recommended to
decrease the risk of irreversible pulmonary complications
associated with aspiration.
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?
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.
109
What comorbid condition can significantly
compromise the success of surgical repair of a
laryngoesophageal cleft?
Gastroesophageal reflux
110
You are evaluating an infant with inspiratory
stridor and suspect a vocal-fold paralysis. What are
the most common causes in newborns?
Traumatic birth, neurologic pathology, iatrogenic, or
| idiopathic (most common)
111
What are the most common initial clinical signs in
infants and children with unilateral vocal-fold
paralysis?
Breathiness, hoarseness, straining, muscle tension, and soft
| voice
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?
Arnold-Chiari malformation (or other brainstem compres-
| sive pathologies)
113
In infants with unilateral vocal-fold paralysis,
what is the likelihood of spontaneous recovery
after birth trauma or after neurologic or idiopathic
paralysis?
~ 70%
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?
~ 50%
115
What is the treatment for congenital unilateral
| vocal-fold paralysis?
Depends on severity, age of the patient, and the cause
Conservative management, tracheostomy, injection of filler
material, or thyroplasty
116
Surgical intervention for congenital bilateral vocal-
| fold paralysis attempts to ________ the true vocal folds to improve the airway.
Lateralize
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?
Mutational falsetto or puberphonia resulting from muscular
incoordination, hyperfunction of the cricothyroid muscle, or
psychological dysfunction
118
In patients with puberphonia or mutational
| falsetto, what is the first line of treatment?
Voice therapy and/or psychotherapy. For recalcitrant dis-
| ease, a type 3 thyroplasty can be considered.
119
What are common nonpulmonary indications for
| pediatric tracheostomy?
Acquired subglottic stenosis (31.4%)
Bilateral vocal-cord paralysis (22.2%)
Congenital airway malformations (22.2%)
Tumors (11.1%)
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?
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
121
Describe the embryology of isolated esophageal
| atresia.
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.
122
What percentage of patients with congenital
anomalies of the aerodigestive tract has isolated
esophageal atresia?
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
123
What are the various types of esophageal atresia
(EA) with or without tracheoesophageal
fistula (TEF)?
```
● EA with distal TEF (most common)
● Isolated EA
● Isolated TEF
● EA with proximal TEF
● EA with double TEF
```
124
What are prenatal signs of esophageal atresia?
● Polyhydramnios in the mother
● Inability to identify the fetal stomach bubble on a
prenatal ultrasonogram
125
What congenital anomalies are commonly
| associated with esophageal atresia?
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
126
What association is commonly diagnosed with
| esophageal atresia with or without TEF?
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)
