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Paediatric Respiratory Reviews 14 (2013) 7077

Contents lists available at SciVerse ScienceDirect

Paediatric Respiratory Reviews

Mini-Symposium: Paediatric Respiratory Emergencies

Management of acute severe upper airway obstruction in children


Andreas Peger, Ernst Eber *
Respiratory and Allergic Disease Division, Department of Paediatrics and Adolescence Medicine, Medical University of Graz

EDUCATIONAL AIMS
After reading this review readers will be able to
 Describe the general principles of clinical assessment, and diagnostic and therapeutic interventions in severe upper airway
obstruction from infancy to adolescence
 Discuss in detail the diagnostic and therapeutic concepts in relevant congenital causes of severe upper airway obstruction
 Discuss both diagnosis and treatment of acquired infectious and non-infectious causes of severe upper airway obstruction from
infancy to adolescence

A R T I C L E I N F O

S U M M A R Y

Keywords:
Acquired
Assessment of severity
Children
Congenital
Emergency
Infants
Management
Upper airway obstruction

There are many causes of acute severe upper airway obstruction (UAO) in children. The timing of symptom
onset and the presence of fever will help to distinguish infectious from non-infectious conditions. Signs and
symptoms from congenital malformations often present at birth but may also develop over time. The most
common cause of UAO in children is croup. Choking on a foreign body also occurs relatively frequently.
Evaluation of the child with UAO starts with a detailed history followed by a thorough physical examination,
including an assessment of severity. Severe airway obstruction will result in respiratory failure. This
situation requires an immediate response. A child with partial airway obstruction may initially have an
adequate airway. However, this situation can deteriorate rapidly. Therefore, providing supportive care and
mobilizing resources for denitive airway management may be the most appropriate interventions.
2013 Elsevier Ltd. All rights reserved.

INTRODUCTION
Upper airway obstruction (UAO) is infrequent in the newborn
but relatively common in infants and young children. Congenital
forms can be differentiated from acquired, infectious from noninfectious and acute from chronic ones (Table 1). Numerous causes
can be distinguished in the newborn and infant, and most of them
are congenital abnormalities. In contrast, there are relatively few
causes in children, and croup (acute viral laryngotracheobronchitis) is the most frequent of them.
Compared with adults, infants and young children have small
and compliant airways and can quickly develop clinically
signicant UAO. The resulting increased work of breathing can
rapidly progress to respiratory failure because these young
patients have less respiratory reserve. Thus, acute UAO from any
cause can be a life-threatening emergency, and early and accurate
diagnosis and treatment are essential.

Evaluation of the child with UAO starts with a detailed history


followed by a thorough physical examination, including an
assessment of severity. In many cases, a correct diagnosis can
be made or at least suspected by history and physical examination
alone. Depending on the age of the patient and the suspected
aetiology, additional studies such as pulmonary function testing,
imaging studies, and especially the endoscopic evaluation of the
airway may be helpful in establishing the diagnosis. In case of
respiratory distress, however, intervention may be necessary
before aetiologic evaluation can take place.
This article in its rst part will focus on general principles of
clinical assessment and airway management and discuss specic
therapeutic issues in more detail when describing several
examples of congenital and acquired UAO.
CLINICAL ASSESSMENT/AIRWAY MANAGEMENT
History

* Corresponding author. Klinische Abteilung fur Padiatrische Pulmonologie und


Allergologie, Universitatsklinik fur Kinder- und Jugendheilkunde, Medizinische
Universitat Graz, Auenbruggerplatz 34/2, 8036 Graz, AUSTRIA.
Tel.: +43 316 385 12620; fax: +43 316 385 13276.
E-mail address: ernst.eber@medunigraz.at (E. Eber).
1526-0542/$ see front matter 2013 Elsevier Ltd. All rights reserved.
http://dx.doi.org/10.1016/j.prrv.2013.02.010

A careful history usually provides valuable guidance in the


evaluation of the child with UAO. Family, birth and neonatal
histories, as well as previous respiratory problems should be

A. Peger, E. Eber / Paediatric Respiratory Reviews 14 (2013) 7077


Table 1
Causes of upper airway obstruction (UAO).
Congenital UAO

Acquired UAO

Micrognathia and glossoptosis

Decreased muscle tone


(e.g. unconsciousness)
Epiglottitis (supraglottitis)
Croup

Choanal atresia
Congenital obstructive
lesions of the nose
Laryngomalacia
Vocal cord paralysis
Congenital subglottic stenosis
Subglottic haemangioma
Congenital laryngeal web
Congenital laryngeal cyst
Laryngeal atresia
Lymphangioma
Complex laryngotracheal
anomalies
Vascular ring/sling

Bacterial tracheitis
Retropharyngeal and
peritonsillar abscess
Infectious mononucleosis
Foreign body (airway and oesophageal)
Trauma (blunt and penetrating injury)
Burn injury
Anaphylaxis
Hereditary angiooedema
Inducible laryngeal obstruction
(vocal cord dysfunction)
Damage from endotracheal
intubation and tracheostomy
Respiratory papillomatosis

explored in detail (e.g. the reason for and the duration of


endotracheal intubation, trauma during birth, etc.). Duration of
stridor and an association with precipitating events such as crying
or feeding, or an association with the infants or childs position,
the nature of the voice, and the presence of other signs and
symptoms, such as fever, cough, aspiration or drooling may be
helpful in establishing the diagnosis. Two variables that are
particularly helpful in determining initial management priorities
are onset of symptoms and presence of fever. The sudden onset of
choking, gagging, or stridor suggests a foreign body or an allergic
reaction. In general, symptoms that have been rapidly progressing
indicate severe disease, whereas the gradual onset of hoarseness
then worsening to stridor suggests less severe involvement, as in
viral croup. A history of fever suggests an infectious aetiology.
Rapid onset of symptoms in a febrile child is more likely in a
bacterial process such as epiglottitis (supraglottitis), bacterial
tracheitis, retropharyngeal abscess, or peritonsillar abscess.
More severe symptoms are frequently observed in children
with congenital anomalies and/or previous airway surgery who
develop inammation of the upper airway due to any cause.
Similarly, children with underlying poor muscle tone or neuromuscular disease (Down syndrome, cerebral palsy, muscular
dystrophy, quadriplegia) are at increased risk of more severe
symptoms.
Physical examination
Particular notice should be taken of craniofacial morphology;
skin colour; use of accessory muscles of respiration, nasal aring,
and chest wall retractions (to assess work of breathing); and level
of consciousness. If cough is present, its character can provide
important clues to the aetiology of the problem. A barking
character of the cough (especially at night) suggests croup. Stridor,
a variably pitched respiratory sound, often is the most prominent
sign of UAO. Usually, stridor is indicative of substantial narrowing
or obstruction of the larynx or extrathoracic trachea. In minor
degrees of narrowing, breathing may be quiet at rest; with
increased activity (e.g. crying) and consequently increased velocity
of airow, however, stridor may develop. Stridor is predominantly
an inspiratory sound, although in some infants with subglottic
lesions (particularly if there is involvement of the upper trachea as
well) a soft expiratory element may be noted. Biphasic stridor
suggests severe, xed airway obstruction at the level of the glottis,
subglottis, or upper trachea. Loud stridor is usually believed to
represent a signicant narrowing of the airway; however, a sudden

71

decrease in volume can sometimes signify worsening obstruction


and decreasing air movement. The character of the voice often
provides important information. Hoarseness with or without
stridor suggests an abnormality of the vocal cords. A mufed voice,
associated with a low-pitched stridor but no hoarseness, may
indicate a supraglottic process such as epiglottitis (supraglottitis).
Another physical sign associated with severe UAO is pulsus
paradoxus. Pulsus paradoxus is quantied as the difference
between the systolic pressures measured during inspiration and
expiration. It can be detected by palpation and observed on the
plethysmographic waveform of the pulse oximeter demonstrating
a decrease in pulse pressure during inspiration when the difference
is greater than 20 mmHg. Pulsus paradoxus has been reported to
be elevated in children with croup, and its magnitude to correlate
with severity as measured by the Westley croup score.1 Intraoral
examination should include attention to signs of infection,
swelling or poor handling of secretions. The presence and size
of the tonsils, as well as any other masses, especially unilateral
enlargement as seen in retropharyngeal or tonsillar abscess, should
be noted. The posterior part of the tongue should be palpated in
infants with stridor in order to identify or rule out a cyst arising
from the tongue.2,3 The intraoral examination should of course be
deferred in a child with severe respiratory distress, as this may lead
to local trauma, agitation, and emotional distress which all have
the potential to worsen UAO. The neck should be palpated for
adenopathy and masses. A child presenting with a rapidly
progressive illness and characteristic signs and symptoms such
as toxicity, fever, sore throat, mufed voice, dyspnoea, dysphagia
and drooling is likely to suffer from epiglottitis (supraglottitis).
Such a child should be approached in a calm and reassuring
manner, and physical examination should be deferred, as
emotional upset may precipitate complete airway obstruction.
Although laryngeal foreign bodies typically produce pain, laryngeal spasm, inspiratory stridor and hoarseness, foreign bodies
lodged in the region of the cricopharyngeus muscle produce
dysphagia and drooling rather than stridor.
Assessment of severity and principles of airway management
A previously healthy infant or child will readily tolerate UAO of
moderate severity. With increasing airway obstruction, tidal
volume is maintained virtually to the point of exhaustion, at
which time hypoxaemia, hypercapnia and acidosis progress
rapidly, and cardiorespiratory arrest will follow soon unless the
obstruction is relieved. Arterial blood gas analysis, transcutaneous
measurement of carbon dioxide pressure, changes in tracheal
diameter as measured by uoroscopy, thoraco-abdominal asynchrony assessed by non-calibrated inductance plethysmography
and noninvasively measured pulsus paradoxus were suggested as
objective measures of severity; however, none of these methods
has been established in clinical practice.4 Rather, it is standard
practice to judge the severity of UAO clinically. Thus, assessment of
the severity and response to treatment of UAO relies on subjective
parameters and often may be difcult. Stridor is a poor indicator of
the severity of airway obstruction, because it may decrease with
worsening obstruction. Severity of dyspnoea, increase in tachypnoea nally followed by bradypnoea, poor air entry, tachycardia,
cyanosis, evidence of fatigue and level of consciousness and
responsivity of the child are generally used to predict the onset of
respiratory failure and the need for an articial airway. Measurable
signs (e.g. pulse rate or respiratory rate) may be of much less
clinical value than the childs general appearance to an experienced observer. The key to appropriate management in an
emergent evaluation of acute UAO is a brief history detailing the
course and associated symptoms, followed by rapid assessment of
clinical signs to determine the approximate level of airway

A. Peger, E. Eber / Paediatric Respiratory Reviews 14 (2013) 7077

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Table 2
Estimation of severity of respiratory distress.
CLINICAL FINDING

MILD RESPIRATORY DISTRESS

MODERATE RESPIRATORY DISTRESS

SEVERE RESPIRATORY DISTRESS

Color
Retractions

Normal
Absent to mild

Normal
Moderate

Air entry
Level of consciousness

Mildly decreased
Normal or restless when disturbed

Moderately decreased
Anxious, restless when undisturbed

Pale, dusky, or cyanotic


Severe and generalized with
use of accessory muscles
Severely decreased
Lethargic, depressed

With permission from Davis HW, Gartner JC, Galvis AG, Michaels RH, Mestad PH. Acute upper airway obstruction: Croup and epiglottitis. Pediatr Clin North Am 1981; 28:
859880.

involvement and the degree of respiratory distress (Tables 2 and 3).


This can be done for the most part through visual inspection,
without ever touching the patient. It is particularly important to
avoid upsetting a child with UAO who shows signs of fatigue or
cyanosis or meets any of the other criteria for severe respiratory
distress. Such disturbances can serve only to worsen distress and
may precipitate complete obstruction. Therefore when a child has
signs of moderately severe or severe obstruction, his or her parents
should be allowed to remain with him or her; any positional
preference (if manifested) should be honoured; and oral examination, venipuncture, i.v. line placement, and radiographs should be
deferred until the airway is secure. Once the initial assessment is
done the most skilled personnel available are assembled to
stabilize the airway. This procedure is best accomplished under
controlled conditions in the intensive care unit or operating room.
Bag-mask ventilation may be of particular value in stenting the
airway open in the child who has arrested from UAO. It is
important to understand the anatomic and physiologic consequences of simple manoeuvres such as chin lift or jaw thrust and to
apply these and use other non-invasive airway devices correctly to
assure efcient mask ventilation. In some circumstances (endoscopic) endotracheal intubation may have to follow.
CONGENITAL UPPER AIRWAY OBSTRUCTION
Clinical situations involving neonatal UAO can either meet the
neonatologist unexpectedly or be anticipated when an antenatal
diagnosis of (high) airway obstruction has been established.
Prenatal sonography looking for congenital anomalies should
demonstrate large obstructing masses such as cervical teratoma,
lymphatic malformations or vascular anomalies causing extrinsic
laryngotracheal obstruction and intrinsic causes related to atresia
or stenosis of the larynx or upper trachea termed congenital high
airway obstruction syndrome (CHAOS).
CHAOS
CHAOS leads to a number of pulmonary and extra-pulmonary
ndings. Pulmonary ndings include dilated airways below the
level of obstruction, hyperexpanded lungs and attened or
Table 3
Typical ndings in acute upper airway obstruction that may suggest a particular
diagnosis.
 High fever, toxic appearance, and respiratory distress in a school age child
suggest bacterial tracheitis or epiglottitis.
 Choking, gagging, and difculty swallowing suggest an upper airway
foreign body.
 Acute onset of upper airway obstruction with facial swelling and wheezing
suggests an allergic reaction.
 Bleeding, bruising, and subcutaneous air suggest blunt or penetrating trauma.
 Burns or singed hair, and hoarseness or respiratory distress suggest a burn
injury to the upper airway.
 Irritability, neck stiffness, and poor feeding in an infant or toddler
suggest retropharyngeal abscess.
 Mufed voice, trismus, and palatal cellulitis and a bulging mass on
examination of the pharynx in the older child suggest peritonsillar abscess.

inverted diaphragm. Extra-pulmonary ndings include fetal


hydrops or ascites and placentomegaly. Although the exact
aetiology is not clear, the abnormal hyperexpansion of the lungs,
which results in attening or eversion of the diaphragm, is believed
to cause impaired venous return to the heart. It has been proposed
that this impaired venous return leads to the subsequent
development of fetal hydrops and ascites. Accurate distinction
of these conditions by sonography and magnetic resonance
imaging (MRI) can help facilitate management, including the ex
utero intrapartum treatment (EXIT) procedure that can be used to
deliver affected fetuses by caesarean section under deep maternal
and fetal anaesthesia, allowing for safe airway control and
management. MRI can localize the level of obstruction better
than sonography. This helps in parental counseling and in deciding
between fetal versus neonatal intervention with the EXIT
procedure. The identication of airway obstruction is key to
establishing the diagnosis of CHAOS and to differentiating it from
bilateral lung masses such as a bilateral congenital pulmonary
airway malformation (CPAM) or other causes of extrinsic airway
obstruction such as a double aortic arch, and that is best done with
MRI.5 Polyhydramnios may occur with obstructing lesions (e.g.
cervical and oral teratomas), reecting impaired fetal swallowing.
Lymphatic malformations are complicated by hydrops, likely
caused by compression of neck vessels.6
Choanal atresia and stenosis
This condition represents one of the most common congenital
upper airway anomalies (1 in 8000 live births).7 The vast majority
of cases are due to bony occlusion of the airway. About two thirds
of patients with choanal atresia have associated congenital
anomalies (e.g. CHARGE association, consisting of coloboma, heart
anomalies, retardation of growth and development, genital and
urinary defects and ear anomalies). Thus, a multidisciplinary
approach to management is essential. Bilateral choanal atresia
causes immediate respiratory distress at birth, and the emergency
treatment consists of the insertion of an oral (oropharyngeal or
orotracheal) airway. Flexible nasal endoscopy and computed
tomography can conrm the diagnosis. A nasal airway should
be established as soon as possible. This can be achieved by a
transnasal approach using a dilating instrument and passing
airway stents through the nasal passage to ensure continued
patency for a period of several weeks. Alternatively, repeated
choanal dilatation may be performed at weekly intervals. In severe
cases, more extensive, transpalatal surgery may be required.7
Pierre-Robin sequence or Robin sequence (RS)
The triad of micrognathia, glossoptosis, and resultant airway
obstruction can necessitate intubation in severe cases, especially
when associated airway pathologies (e.g. laryngomalacia, tracheomalacia) exist.7 Intubation is often difcult owing to the
micrognathia and is usually done endoscopically. Prone positional
therapy has proved to be efcient in less severe cases. When
position alone fails, tongue-base airway obstruction may be

A. Peger, E. Eber / Paediatric Respiratory Reviews 14 (2013) 7077

relieved by placement of a nasopharyngeal airway (NPA). The


distal end of a modied endotracheal tube is placed intranasally
and positioned in the distal oropharynx, beyond the area of
glossoptosis. Placing the endoscope within the nasopharyngeal
tube allows direct visualization and position verication. Persistent airway obstruction despite positioning of an NPA is an
indication for endoscopy of the distal airways and evaluation of
central respiratory drive via polysomnography. The level of airway
obstruction or presence of multiple levels of airway narrowing
should guide further interventions. Non-invasive respiratory
support can relieve upper airway obstruction, with continuous
positive airway pressure (CPAP) ventilation in mild and moderate
cases or with non-invasive positive pressure ventilation in severe
UAO.8,9 Surgical procedures used for patients with RS include
tongue-lip adhesion, mandibular distraction osteogenesis and
tracheostomy.10 Feeding difculties can be alleviated by upright
feeding techniques, modication of the nipple for bottle feeding,
temporary use of nasogastric or orogastric feeding tubes, and the
placement of a gastrostomy.11 Palatal plates such as the preepiglottic baton plate, which have a velar extension, pull the base
of the tongue forward. This can be helpful in the relief of airway
obstruction, and it also facilitates the swallowing mechanism
during feeds and accelerates mandibular growth.12 More than half
of the infants with RS will have an associated syndrome, the most
common of which are Stickler syndrome and 22q11.2 deletion
syndrome.

73

arterial lesions, cardiac anomalies and coarctation of the aorta, eye


anomalies with or without sternal clefts) is a neurocutaneous
disorder.19 It is important to rule out PHACE syndrome in patients
with subglottic haemangioma because the knowledge of cardiac
and central nervous system arterial anomalies is important to
therapeutic decision making, as these anomalies may increase the
risk of adverse events in patients who are receiving both steroids
(which can induce hypertension) and propranolol (which can
induce hypotension).18 There are reports of children with PHACE
syndrome who have experienced ischaemic stroke, seizures,
chronic headaches, and developmental delays. If the lesion is
small and only causes mild symptoms it may be treated with
(intermittent) systemic corticosteroids. Repeated local corticosteroid injection is an alternative treatment option, and may be
used to prevent side effects caused by long-term systemic
corticosteroid therapy. In the past, interferon alpha 2a has been
used for haemangiomas not responding to corticosteroid therapy,
but this treatment has been associated with neurological
complications. Recently, case reports describing successful treatment with propranolol or acebutolol have been published, and
some authors already recommend oral beta blocker therapy as a
rst-line treatment of subglottic haemangiomas.20 Surgical treatment options include repeated endoscopic laser resection, surgical
resection via open excision for large haemangiomas, and long-term
tracheostomy.
ACQUIRED UPPER AIRWAY OBSTRUCTION

Craniofacial anomalies
UAO is a major problem for children with craniofacial
anomalies such as Crouzon, Apert, Pfeiffer, Treacher-Collins,
craniofacial microsomia and Goldenhar syndromes. Furthermore,
thirty-six syndromes with craniofacial anomalies were found to be
associated with one or more of 14 laryngotracheal malformations
(laryngeal anomalies like laryngeal atresia, laryngeal hypoplasia,
laryngomalacia, vocal cord paralysis, laryngeal stenosis, -webs, and
-clefts, tracheobronchial anomalies like congenital tracheal
stenosis, tracheomalacia and bronchomalacia).13,14
Congenital malformations of the larynx
Congenital malformations of the larynx like laryngomalacia
(the most common accounting for 60%), vocal cord paralysis (the
second most common with 1520%), congenital subglottic stenosis
(the third most common with 15%), subglottic haemangioma
(1.5%) and congenital laryngeal webs, laryngeal atresia, cysts, and
lymphangioma (these are very rare) may present with severe,
acute UAO. The initial management in acute severe cases is
endotracheal intubation followed by tracheostomy in some.
Flexible endoscopy is the diagnostic procedure of choice to
establish the diagnosis and to detect or exclude concurrent airway
anomalies.15
Airway haemangiomas
More than 50% of infants with airway haemangiomas also have
cutaneous haemangiomas.16,17 Patients with haemangiomas in the
mandibular or beard distribution are known to be at especially
high risk. Many haemangiomas show rapid growth until six to ten
months of age; involution usually only begins at about 18 months.
Infants may become symptomatic between two weeks and six
months of age. The lesion is usually located posterolaterally in the
submucosa in the subglottis, or in the upper trachea. It may be
unilateral or bilateral and circumferential. Subglottic haemangiomas may extend into the supraglottic region or oral cavity.18 The
lesion is pink-blue, sessile, and easily compressible. PHACE
syndrome (posterior fossa brain malformations, haemangiomas,

Major causes of acquired infectious and noninfectious acute


UAO are severe tonsillitis with adenoid enlargement (e.g. in
infectious mononucleosis), retropharyngeal and peritonsillar
abscess, epiglottitis (supraglottitis), croup (viral laryngotracheobronchitis), bacterial tracheitis, foreign body aspiration, anaphylaxis and angiooedema, and traumatic lesions such as vocal cord
paralysis, thermal injury, caustic ingestion, damage from endotracheal intubation and tracheostomy. Further rare causes may be
benign airway neoplasms such as respiratory papillomatosis, and
even rarer malignant airway tumours (rhabdomyosarcomas and
squamous cell carcinomas). Functional pathologies (inducible
laryngeal obstruction or vocal cord dysfunction) and loss of
consciousness may also cause acute UAO.
Croup (viral laryngotracheobronchitis)
Viral croup is the most frequent cause of acute UAO in children
six months to six years of age, with a peak incidence at around two
years. Parainuenza viruses types 1 and 3 account for the majority
of cases. The disease is characterized by the usually sudden onset
of a very typical seal-like barking cough associated with
hoarseness and varying degrees of stridor, retractions and
respiratory distress due to narrowing of the subglottic region.
These symptoms are frequently preceded by non-specic symptoms from upper respiratory tract infection for one to two days.
Most children with croup are deemed to have a mild illness but
pronounced laryngeal obstruction can lead to asynchronous chest
wall and abdominal movements, fatigue, and eventually to
hypoxia, hypercapnia, and respiratory failure. It is estimated that
less than 1% of children with croup have a severe form, and the
mortality rate is very low. Differential diagnoses are important and
include other acute obstructive illnesses such as epiglottitis (still to
be considered!), bacterial tracheitis, foreign body aspiration,
angioneurotic oedema, retropharyngeal and peritonsillar
abscesses, and diphteria. Systemic corticosteroids (dexamethasone, prednisolone) are the treatment of choice, and benet is seen
in patients with all levels of severity. Nebulised epinephrine is
effective for temporary relief of symptoms of airway obstruction

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A. Peger, E. Eber / Paediatric Respiratory Reviews 14 (2013) 7077

and may be used until corticosteroids take effect.21,22 Mist therapy


is ineffective.23,24 Endotracheal intubation may be necessary in the
most severe cases that do not respond to medical treatment.
Uncuffed endotracheal tubes which are at least 1 to 2 sizes smaller
than would normally be selected for the childs age should be used.
Flexible bronchoscopy should be considered in children presenting
with atypical, prolonged or frequently recurrent symptoms, and
children who do not respond to medical treatment, or are outside
the typical age range for croup.25

coverage against -lactamase-producing H. inuenzae type B,


penicillin-resistant S. pneumoniae, beta-haemolytic streptococci,
S. aureus (incl. methicillin-resistant strains if there is aetiologic
concern), e.g. cefotaxime or cefriaxone plus clindamycin or
vancomycin. Single agent therapy with ampicillin-sulbactam or
cefuroxime is an option if MRSA is not an aetiologic concern. The
duration of intubation is usually short (2448 hours). Decision to
extubate is usually based on clinical criteria (fever resolved,
patient can handle secretions, and presence of a leak around the
endotracheal tube) and laryngoscopic evaluation.21,22,36,37

Epiglottitis (supraglottitis)
Bacterial tracheitis
Epiglottitis is a life-threatening infection characterized by
rapidly progressive inammation and oedema of the pharynx,
epiglottis, aryepiglottic folds, and ventricular bands. In the past
most cases have been caused by Haemophilus inuenzae type B
(HIB). The peak age was one to seven years, but infants and older
children may also be affected. Since the widespread introduction
of the HIB vaccination in the early 1990s, epiglottitis has become
much less common and physicians are less likely to see this
disease. Patients suffering from epiglottitis today tend to be older
and may present with atypical symptoms. Risk factors for
epiglottitis in children include incomplete or lack of immunisation for HIB and immunodeciency; some hosts may be
genetically more susceptible to invasive HIB infection.2631
Organisms reported to cause epiglottitis include group A haemolytic Streptococcus pneumoniae, Klebsiella pneumoniae,
Staphylococcus aureus (including methicillin-resistant strains),
Haemophilus parainuenzae, Neisseria menigitidis, Candida albicans, Varicella zoster and several other viruses.32,33 Onset is
sudden, with high fever, and symptoms (quiet, gurgling stridor,
severe throat pain, dysphagia and drooling and soon thereafter
dyspnoea and anxiety) progress rapidly within hours. On
examination, the child is usually toxic, anxious, and remarkably
still, sitting bolt upright with the neck extended and head
forward with the jaw thrust out to maximise laryngeal opening
(tripod or snifng position). If the child will talk, which is
unusual, the voice is mufed (dysphonia). The term 4Ds
(drooling, dysphagia, dysphonia, and dyspnoea) has been
introduced as a mnemonic for epiglottitis. The best course of
action after assessment is airway stabilisation under controlled
conditions in the operating room or PICU via intubation.
Intubation with a tube 0.5 to 1.0 mm smaller than would
otherwise be used is commonly performed under inhalation
anaesthesia without the use of neuromuscular blockers. Nasotracheal intubation is preferred for the management of epiglottitis, but, depending on the skills of the physician, oral intubation
using a stylet may be safer to secure the airway. If an abrupt
obstruction occurs bag-valve mask ventilation with 100% oxygen
should be attempted. During laryngoscopy, pressure on the chest
by an assistant may produce bubbling and help indicate the
location of the glottis. Endoscopic nasotracheal intubation under
controlled conditions is preferred, if immediately available.3437
Paediatric epiglottitis is one of the few instances in which the
emergency physician may need to rapidly perform needle
cricothyrotomy. Needle cricothyrotomy is a temporizing method
used to treat cases of severe epiglottitis when the patient cannot
be intubated before a formal tracheostomy. This procedure
involves inserting a needle through the cricothyroid membrane
which lies inferior to the thyroid cartilage and superior to the
cricoid cartilage. The cricothyroid arteries typically cross through
the superior portion of the membrane. Tracheostomy should be
reserved for patients in which endotracheal intubation is
unsuccessful due to severe laryngeal oedema.38,39 After intubation, cultures should be obtained and the patient be started
on antibiotics. The empiric regimen generally should provide

Bacterial tracheitis is a rare airway infection that is also known


as bacterial laryngotracheobronchitis, membranous laryngotracheobronchitis, or pseudomembranous croup. In a retrospective,
descriptive case series of 107 children hospitalized between 1997
and 2006 with various upper respiratory illnesses, two had
epiglottitis and 18 had bacterial tracheitis. Of those with bacterial
tracheitis, 15 were intubated and ve had serious complications
(ARDS and multisystem organ failure). Bacterial tracheitis was
three times more likely to cause respiratory failure than viral croup
and epiglottitis combined, and therefore should be considered in
all children who present with an acute, life-threatening upper
respiratory infection.40 It affects children six months to eight years
of age (average age 46 years). Compared to children with viral
croup, children with bacterial tracheitis are generally older, more
ill appearing, and rarely respond to epinephrine.41 Compared to
children with epiglottitis, patients with bacterial tracheitis have a
signicant cough, are comfortable when lying at, and control
secretions (i.e. do not tend to drool). Causative agents are S. aureus,
H. inuenzae, S. pneumoniae, S. pyogenes and Moraxella catarrhalis.
The clinical picture with several days of fever, cough, and stridor
initially resembles that of viral croup. Children then progress to
rapid onset of high fever, difculty breathing, and an ill
appearance. Diagnosis is usually made by endoscopy demonstrating subglottic oedema with ulcerations, copious secretions and
formation of pseudomembranes. Active management, including
timely intubation, is vital to prevent complete UAO. Based on data
presented in a recent review, initial antibiotic therapy should be of
broad spectrum coverage, including an appropriate anti-staphylococcal agent with a view to optimize therapy once culture and
susceptibility results become available.34,42
Infectious mononucleosis (IM)
UAO due to massive tonsillar enlargement and mucosal oedema
is an uncommon and potentially fatal complication of IM (13.5%).
The use of corticosteroids to reduce pharyngeal oedema and
lymphoid hypertrophy is advocated for individuals with incipient
obstruction. This complication occurs especially in the younger
child and usually presents with dyspnoea, sore throat, dysphagia
and drooling a few days after the onset of IM. Passage of a
nasopharyngeal tube is an effective treatment to bypass the
pharyngeal obstruction in most cases. In rare instances, panpharyngeal and transglottic inammatory oedema may necessitate
endotracheal intubation or even tracheotomy.43 Acute tonsillectomy has been advocated by some authors in severe UAO that failed
to respond to corticosteroids.44,45 It is not, however, universally
practiced owing to the high risk of perioperative bleeding, with
rates reported as high as 13%.46,47
Retropharyngeal and peritonsillar abscess
A retropharyngeal abscess usually involves one of the
retropharyngeal lymph nodes that run in chains through the

A. Peger, E. Eber / Paediatric Respiratory Reviews 14 (2013) 7077

retropharyngeal tissues on either side of the midline. Because these


nodes tend to atrophy after 4 years of age, the disorder is primarily
seen in children younger than three or four years. Initially, patients
present with symptoms of pharyngitis (such as fever and
odynophagia) for several days. Subsequently, they develop high
fever, drooling, poor feeding, neck stiffness and swelling, and
torticollis caused by muscular irritation from the infectious tissue. A
study in 64 patients found that the most common chief complaints
were neck pain (38%), fever (17%), sore throat (17%), neck mass
(16%), and respiratory distress or stridor (5%). Almost half of the
patients had limitations of neck extension, 27% had torticollis, and
13% had limitation of neck exion. Stridor was a rare nding.
Physical examination ndings include midline fullness of the
oropharynx, although this can be difcult to appreciate. Causative
organisms include S. aureus, Streptococcus species, and anaerobes.
Protection of the airway from becoming completely blocked by
retropharyngeal swelling is rarely indicated. Further complications include aspiration pneumonia from rupture of the abscess
into the oropharynx, extension of the infection to adjacent
structures or compartments, sepsis, abscess recurrence, and
mediastinitis. Diagnostic ultrasound (and/or lateral soft tissue
neck radiograph) and CT imaging before surgical drainage may
have to follow in most but not all cases. Early administration of
intravenous antibiotics is crucial.48
Parapharyngeal abscesses in infants and children show a nearly
identical clinical picture, however these patients have torticollis,
bending toward the affected side, and examination of the neck
reveals diffuse anterolateral swelling that is exquisitely tender.
Peritonsillar infections are the most common deep neck
infections in adults and children and may rarely cause symptoms
of severe UAO. On examination, an erythematous bulging of the
soft palate above the tonsil and uvular deviation away from the
infected side are noted.21

75

completely obstruct the trachea. In this situation, the trachea may be


intubated and the endotracheal tube advanced into the right
mainstem bronchus as an attempt to relieve the tracheal obstruction
by pushing the foreign body into the right mainstem bronchus. At
this point, the endotracheal tube should be withdrawn to a position
above the carina to allow for ventilation of the left lung. The child will
now have a bronchial foreign body, but may receive adequate
ventilation and oxygenation while preparations are being made to
remove the foreign body in the operating room. There is no evidence
to support the effectiveness of this approach in the setting of
complete subglottic airway obstruction from a foreign body.
However, case reports of patients requiring one lung ventilation
for surgical procedures and studies in dogs have demonstrated
effective oxygenation and ventilation. If the airway is not completely
obstructed, what might produce stridor, hoarseness, cough, sore
throat and dyspnoea, and the patient can properly mobilize air, the
Heimlich manoeuvre or other removal attempts can unintentionally
rush events by complete blockade of the airway; in this case the
patient should be rushed to an institution with appropriate
equipment and personnel to handle the problem. The hypopharynx
should not be explored with a nger that might push the foreign
body downwards to the larynx or force it into the oesophagus where
it can compress the trachea against the sternum, causing complete
airway obstruction.5557
Anaphylaxis
Anaphylaxis and anaphylactoid reactions may be severe and
life-threatening when oedema involves the retropharynx and/or
larynx. Onset of symptoms is usually sudden, and there may be
associated signs such as urticaria and facial swelling (angiooedema). The cornerstone of pharmacological therapy is adrenalin.58
Hereditary angiooedema (HAE)

Foreign body aspiration (FBA)


Small children often choke on food or small objects and usually
clear the obstruction spontaneously with coughing and choking.
Eighty percent of paediatric FBAs occur in children younger than
three years, with the peak incidence between one and three years of
age. FBA is the fourth leading cause of accidental death in this group
and the third in infants under one year.4952 Exploration of the
environment through the mouth, lack of molars which decreases the
ability to properly chew food, lack of cognitive capacity to
distinguish between edible and inedible objects, and tendency to
distraction and to perform other activities, like playing whilst eating
are the main reasons for FBA.53 Most aspirated objects lodge in the
bronchi, although rarely, foreign bodies in the larynx and trachea (3
and 13% in a case series of 1068 children)54 can cause complete or
signicant partial airway obstruction. If a child is unable to speak or
cough, dislodgement using ve back blows followed by chest
compression in infants less than one year of age is recommended.
Abdominal thrusts are not advisable because they may cause
damage to the liver, which is relatively large and unprotected in this
age group. For children >1 year of age ve abdominal thrusts
(Heimlich manoeuvre) should be performed. Direct laryngoscopy
should be performed when complete UAO has not been relieved
with basic life support manoeuvres. In some cases, foreign material
can be removed with a Magill forceps and/or suction. The child who
begins to breathe adequately should receive supportive care,
including supplemental oxygen, and be admitted to hospital. Bagmask ventilation should be provided to patients who do not resume
breathing spontaneously. When a foreign body causing complete
UAO above the vocal cords cannot be removed, needle cricothyrotomy with percutaneous transtracheal ventilation is recommended. A foreign body that is lodged below the vocal cords may

Laryngeal oedema occurs in approximately one-half of all


patients with HAE at some point during their lifetime. Tooth
extraction and oral surgery are common triggers for laryngeal
attacks. Pathogenesis is linked to a quantitative or qualitative
deciency of C1 esterase inhibitor (C1 EI), leading to uncontrolled
activation of the complement system, coagulation factors and
various vasoactive kinins. Specic treatment consists of i.v.
substitution of C1 EI concentrate, subcutaneous administration
of icatibant (a selective competitive antagonist for the bradykinin
B2 receptor) or ecallantide (kallikrein inhibitor), or alternatively i.v.
substitution of fresh frozen plasma. As the therapeutic effect is
delayed by at least 30 to 60 min, a-sympathomimetics such as
adrenaline should be given a trial, where intubation or tracheotomy may be the ultimate way to save a patients life.5961
Thermal injuries, burn injuries, and blunt and penetrating injuries
Life-threatening UAO may develop rapidly as a result of
laryngeal burns, caustic ingestions and inhalation injury. Any
toddler with a scald injury to face, neck or both should be
suspected of having aspirated hot liquid and of developing
laryngeal swelling. When smoke is inhaled, most of the heat is
dissipated by the upper airways before the inhaled material
reaches the carina. Thermal injury therefore primarily affects the
supraglottic airway. Intrathoracic airways may be involved when
prolonged exposure to high ambient temperature or inhalation of
steam occurs. Nebulised adrenaline may have a role in certain
circumstances. Further management depends on the extension of
the trauma and the underlying cause and should include
endoscopy of the airways. Blunt or penetrating injury to various
anatomic structures of the upper airway may cause UAO.6264

76

A. Peger, E. Eber / Paediatric Respiratory Reviews 14 (2013) 7077

Traumatic lesions
Traumatic lesions leading to UAO may occur from endotracheal
intubation and tracheostomy. The interested reader is referred to
an earlier review where such lesions (including vocal cord
paralysis) are discussed in more detail.22
Functional upper airway obstruction
Episodes of unintentional paradoxical adduction of the vocal
cords (vocal cord dysfunction) or the supraglottic arytenoid
region may induce severe dyspnoea attacks. The term inducible
laryngeal obstruction (ILO) includes both glottic and supraglottic
levels of obstruction. The aetiology is complex. The essential
pathophysiology is that of a hyperfunctional laryngeal reex to
protect the lower airway as a result of any combination of postnasal drip, gastro-oesophageal reux, laryngopharyngeal reux
and/or psychological conditions. It may be induced by exercise
(e.g. in young athletes). Laryngoscopic demonstration of the
dysfunction while the patient is stridorous or wheezing is the
diagnostic gold standard.65 Excluding structural upper airway
anomalies and diseases like Myasthenia which may mimic ILO is
important. Speech therapy and treatment of reux disease or
underlying psychiatric disorders if present are crucial. In the acute
setting reassurance and supportive care until the episode
spontaneously resolves are recommended; heliox (20 to 40%
oxygen in helium) has been reported as useful adjunct by some
authors.6668
CONCLUSION
Acute severe UAO due to non-infectious causes such as foreign
body aspiration, thermal injury, trauma, anaphylaxis and angiooedema as well as infectious causes like croup, epiglottitis, bacterial
tracheitis, and due to congenital airway abnormalities can be a lifethreatening emergency. The key to management is a history
detailing the clinical course and associated symptoms, followed by
rapid assessment of clinical signs to determine the approximate
level of airway involvement and the degree of respiratory distress.
Early appropriate treatment of many causes of UAO may stabilize
the childrens respiratory function. Prompt recognition of the child
with complete airway obstruction who requires immediate
therapeutic intervention is crucial.
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