SPECIAL ISSUE ARTICLE

Acute Respiratory Failure in Children

Matthew L. Friedman, MD; and Mara E. Nitu, MD

epidemiology is not well described

ABSTRACT due to inconsistent and heterogeneous
This article reviews the definition, pathophysiology, etiology, assessment, and manage- diagnostic criteria. In patients with
ment of acute respiratory failure in children. Acute respiratory failure is the inability of the respiratory failure who have underly-
respiratory system to maintain oxygenation or eliminate carbon dioxide. Acute respiratory ing pediatric acute respiratory distress
failure is a common cause for admission to a pediatric intensive care unit. Most causes of syndrome (ARDS), epidemiologic
acute respiratory failure can be grouped into one of three categories: lung parenchymal data reveal an annual incidence of
disease, airway obstruction, or neuromuscular dysfunction. Many patients with acute re- 2.3% of PICU admissions, and a mor-
spiratory failure are managed successfully with noninvasive respiratory support; however, tality rate of 24% to 34%.1,2
in severe cases, patients may require intubation and mechanical ventilation. [Pediatr Ann.
2018;47(7):e268-e273.] PHYSIOLOGY AND
PATHOPHYSIOLOGY

A
cute respiratory failure in chil- ever, it can only be accurately stated that Normal control of breathing is a
dren is the inability of the re- the PaCO2 is no higher than the PvCO2. complex interaction between the vas-
spiratory system to support Therefore, when PvCO2 is <50 mm Hg, culature, brain, lungs, and respiratory
oxygenation, ventilation, or both. Hy- acute hypercarbic respiratory failure can apparatus. Peripheral chemoreceptors,
poxic respiratory failure is defined by be ruled out but a PvCO2 of 55 mm Hg located in the aortic and carotid bod-
an arterial partial pressure of oxygen does not guarantee a diagnosis of hy- ies, are sensitive to PaO2, PaCO2, and
(PaO2) below 60 mm Hg, which typical- percarbic respiratory failure. PvCO2 is pH. A decrease in PaO2, a decrease
ly produces an arterial oxygen saturation a test that has high sensitivity but poor in pH or an increase in CO2 results
of 90%. Ventilation is the elimination specificity for diagnosing hypercarbic in signaling to increase ventilation.
of CO2 and is measured by the arterial respiratory failure. PvCO2 should be in- Central chemoreceptors in the brain
partial pressure of CO2 (PaCO2). Acute terpreted carefully based on location of are sensitive to cerebral spinal fluid
hypercarbic respiratory failure is defined sampling, manner of sampling, and car- (CSF) pH. The blood-brain barrier al-
by an acute increase in PaCO2 greater diac output. lows CO2, but not hydrogen ions, to
than 50 mm Hg. It is typically associated pass freely so the CSF pH is deter-
with a respiratory acidosis pH of <7.35. EPIDEMIOLOGY mined by PaCO2. Therefore, the cen-
Venous blood may be sampled in lieu Acute respiratory failure is a com- tral chemoreceptors can detect small
of arterial blood to obtain the venous mon reason for admission to the pedi- changes in CO2. Input from peripheral
partial pressure of CO2 (PvCO2); how- atric intensive care unit (PICU). The and central chemoreceptors is inte-
grated in the brainstem. The pons and
Matthew L. Friedman, MD, is the Medical Director of Community Hospital North; and an Assistant medulla generate periodic impulses to
Professor of Clinical Pediatrics. Mara E. Nitu, MD, is the Division Chief of Pediatric Critical Care, the Vice trigger breathing. Injury to the brain-
Chair of Clinical Affairs for Pediatrics, and a Professor of Clinical Pediatrics. Both authors are affiliated stem leads to characteristic, abnormal
with the Section of Pediatric Critical Care, Riley Hospital for Children, Indiana University School of respiratory patterns based on the level
Medicine. of injury.3 The cortex can override this
Address correspondence to Matthew L. Friedman, MD, 705 Riley Hospital Drive, Phase 2, Room 4927, automatic mechanism with voluntary
Riley Hospital for Children, Indianapolis, IN 46202; email: friedmml@iu.edu. respiratory effort.
Disclosure: The authors have no relevant financial relationships to disclose. The main muscle of inspiration is
doi:10.3928/19382359-20180625-01
the diaphragm, which is innervated

e268 Copyright © SLACK Incorporated


by the phrenic nerve that originates
from spinal nerve roots C3 to C5.
Thus, patients with spinal cord inju-
ries at or above this level are at risk
for diaphragmatic paralysis and respi-
ratory failure. Phrenic nerve stimula-
tion causes contraction and flattening
of the dome-shaped diaphragm. This
leads to an increase in intrathoracic
volume and consequently a decrease
in intrathoracic pressure. A negative
pressure gradient is generated be-
tween the alveoli and the external en-
vironment, resulting in net movement
of air to the alveoli. This negative
pressure breathing is contrasted to the
positive pressure breathing of invasive
mechanical ventilation.
Thoracic spinal nerve roots inner-
vate the external intercostal muscles to
aid in inspiration by pulling the chest
upward and anteriorly. Exhalation is a
passive process during quiet breathing
due to the elastic recoil of the lungs
and chest wall. When exercising or in
respiratory distress, exhalation can be
an active process assisted by internal
intercostal muscles pulling the rib
cage inwards and down, and abdomi-
nal wall musculature contracting and
forcing abdominal contents upward
into the thoracic cavity and increasing
intrathoracic pressure.
Compared to adults, children, par-
ticularly infants, are at higher risk of
acute respiratory failure. The small di-
ameter of children’s airway results in
a high resistance to flow. Resistance
is proportional to the inverse of the
radius of the airway to the 4th power;
thus, even small changes in the airway
radius can result in large increases in
airway resistance, leading to severely
decreased airflow. The pediatric air-
way is small and can be further nar-
rowed by secretions, edema, or bron-
choconstriction. Young children also
have underdeveloped collateral venti-
SPECIAL ISSUE ARTICLE
lation and an acute angle of the right
upper lobe bronchus, predisposing
them to atelectasis.4 The chest wall
of a child is more compliant, which
from a mechanical standpoint, is dis-
advantageous for normal breathing.
The diaphragm of children fatigues
quicker than adults due to fewer type-
1 muscle fibers. Lastly, in young in-
fants, the central control of breathing
is immature and prone to apnea and
bradypnea.5
Impairments in oxygenation or ven-
tilation leading to respiratory failure
are most often due to ventilation/per-
fusion (V/Q) mismatch. Although the
ideal 1:1 ratio of ventilation to perfu-
sion is rare, in acute lung disease the
mismatch becomes more severe. Lung
segments perfused but not ventilated
are considered dead space (V/Q ap-
proaches infinity). Examples of dead
space ventilation include anatomical
dead space (large airways), pulmo-
nary embolism, and severe pulmonary
hypertension. Clearance of CO2 is im-
paired when dead space is increased,
resulting in hypercarbia. Areas of the
lung that have perfusion but no ven-
tilation result in shunt physiology
(V/Q = 0). In shunt physiology, blood
passes from pulmonary artery to pul-
monary vein without being exposed to
an aerated alveolar membrane, result-
ing in hypoxemia. Examples of shunt
are lung collapse and pulmonary ar-
terial-venous connections. In most
lung diseases, there is heterogeneity
in V/Q mismatch from 0 to infinity
(Figure 1).
Respiratory failure may also be the
result of impaired diffusion of oxygen
across the alveolar-capillary mem-
brane. Diffusion limitation may coex-
ist with V/Q mismatch. An example
of diffusion impairment is pulmonary
fibrosis. Hypercarbia due to diffu-
sion impairment is rare because CO2
diffuses across the alveolar-capillary
membrane more rapidly than oxygen.
ETIOLOGY
Acute respiratory failure has three
major etiological categories: intrinsic
and acquired lung disease, airway dis-
orders, and neuromuscular dysfunc-
tion (Table 1). Diseases that lead to
respiratory failure from pulmonary
pathology are caused by V/Q mis-
matching, gas diffusion impairment,
or both. Airway disorders more com-
monly lead to respiratory failure in
more children than adults due to the
smaller radius of the airway. Neuro-
muscular causes of respiratory failure
can occur anywhere from the central
nervous system to the innervated mus-
cles of respiration.
EVALUATION
The initial assessment of children
with concern for impending acute re-
spiratory failure aims to determine
the degree of respiratory impairment.
Experienced clinicians can make this
determination quickly at the bedside
by astute observation. Assessment of
patient vital signs, general appear-
ance, lung examination, and mental
health status allow for a rapid de-
termination of the severity of illness
and often suggest which interventions
may be required to appropriately in-
tervene to reverse the course of illness
or to avoid respiratory arrest. Tachy-
pnea and hypoxemia are common
manifestations of acute respiratory
failure, although tachycardia is often
an underappreciated sign of impend-
ing respiratory failure. Increased work
of breathing manifests as retractions,
grunting, head bobbing, nasal flaring,
or belly breathing. Children with re-
spiratory failure due to neuromuscular
weakness or central nervous system
dysfunction may not exhibit typical
e269
 SPECIAL ISSUE ARTICLE
should be obtained to aid the specific
diagnosis.
Auscultation of the lung fields is
helpful for both diagnosis and man-
agement. Prolonged exhalation or au-
dible wheeze is suggestive of lower
airway bronchoconstriction. Local-
ized findings suggest a focal pneu-
monia or foreign body aspiration.
Absence of breath sounds can be
due to pneumothorax, pleural effu-
sion, or dense consolidation of lung.
Rales in all lung fields is commonly
Figure 1. Three alveolar capillary units depicting due to pulmonary edema or diffuse
normal (V/Q = 1), dead space (V/Q approaching
infinity), and shunt (V/Q = 0) physiologies. V/Q, interstitial edema. Stridor is gener-
ventilation/perfusion. ated by turbulent airflow secondary
to narrowing in the upper airway and
TABLE 1. may occur in croup, external airway
compression, and high foreign body
Causes of Acute
aspiration.
Respiratory Failure
Altered mental status may be a
Lung parenchyma
cause or consequence of respiratory
failure. Patients who are hypercarbic
present with somnolence, whereas
hypoxic patients are often agitated
due to the lack of oxygen delivery
due to sepsis or trauma
to the end organs including the cen-
tral nervous system. Children with
traumatic brain injury and a Glas-
cow Coma Score of 8 or less should
be promptly intubated for airway
protection. The use of the Glascow
Coma Score for nontraumatic causes
of altered mental health status is less
well established but provides a com-
Neuromuscular dysfunction
mon language for communicating
an objective measure to trend over
syndrome) time. A neurological examination,
particularly mental health status and
myasthenia gravis) strength, is important to help identify
neuromuscular causes of respiratory
(travel, infection, seizure) failure.
DIAGNOSIS
The initial evaluation of a child
signs of increased respiratory ef- in respiratory distress includes a tar-
fort, thus a higher index of suspicion geted but thorough history and physi-
is warranted; an arterial blood gas cal examination. A thorough history
e270
directed at identifying inciting signs
and symptoms may aid clinicians in
the underlying etiology of the acute
respiratory failure. Initial labora-
tory data include blood gas sampling
to assess acid/base status as well as
oxygenation and ventilation. Arte-
rial blood gas is preferred to venous
blood gas due to the ability to assess
oxygenation.
Chest radiography will frequently
identify the inciting cause of respira-
tory failure including inflammatory
or infectious conditions, radiopaque
foreign bodies, atelectasis, or effu-
sions. Chest radiograph also assesses
for pathology that needs emergent
intervention such as pneumothorax.
Neither the chest radiograph nor
the results of the blood gas analysis
should delay the emergent manage-
ment of an acutely deteriorating pa-
tient who requires intubation and me-
chanical ventilation.
Respiratory secretions can be sent
for microbiologic, cytology, and his-
tologic testing. A variety of methods
can be used to sample secretions.
The gold standard bronchoscopy
with bronchoalveolar lavage (BAL)
is the most invasive method but has
the advantages of obtaining the deep-
est lung sample and visualizing the
airways. If an infectious source of
respiratory failure is suspected, the
secretions are sent for the follow-
ing laboratory tests: gram stain, acid
fast bacillus stain, cell count, bacte-
rial culture (possibly also fungal and
mycobacterial culture), and/or viral
polymerase chain reaction. BAL can
also diagnose pulmonary hemor-
rhage, pulmonary hemosiderosis, and
aspiration pneumonitis.
MANAGEMENT
Supportive respiratory care is the
mainstay of management. Classical-
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