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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.
Acquired UAO
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
71
72
Table 2
Estimation of severity of respiratory distress.
CLINICAL FINDING
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
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.
73
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,
74
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
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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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