Format 2016: tachypnoea in a well baby: what to do next?

Tachypnoea in a well baby: what to do next?
Attilio Boner
University of
Verona, Italy
attilio.boner@univr.it
Introduction
Physiology of breathing
What is tachypnoea?
Is the baby unwell?
Is the examination normal?
Immediate clinic investigations:
SaO2, Chest X-ray
Further investigations
Potential causes of tachypnoea when the
diagnosis is not immediately obvious
Outcome
Summary & Conclusions

Tachypnea and cyanosis in the newborn are frequently encountered
problems in the nursery.
The incidence of respiratory distress
in newborns ranges from 2.9% to 7.6%.
4.3% of newborns may require supplemental oxygen therapy.
In the nursery
•Hjalmarson O. Epidemiology and classification of acute neonatal respiratory disorders.
Acta Paediatr Scand 1981;70:773– 83.
•Hjalmarson O. Epidemiology of neonatal disorders of respiration.
Int J Technol Assess Health Care 1991;7(Suppl):9–16.
•Kumar A, Bhat V. Epidemiology of respiratory distress of newborns. Indian J Pediatr 1996;63:93– 8.
Chest radiographs in a premature infant with
respiratory distress syndrome before and after
surfactant treatment.

Respiratory plasticity following intermittent hypoxia:
developmental interactions.
Gozal E, J Appl Physiol 2001;90(5):1995-9.
Schematic diagram showing the
potential interactions between
intermittent hypoxia and its
characteristics and the hypoxic
ventilatory response pathways
in mammals.
Intermittent hypoxia can affect
both peripheral chemoreceptor (PC)
responses as well as those
of centrally located neurons (CNS).
Such interactions will be modulated to a greater
or lesser extent by the developmental stage of
the mammals. Such global relationships will result
in phenotypic changes of ventilation (V˙ E),
metabolism (V˙ O), as well as arousal, behavior,
learning, and stress responses.
In addition, such modifications in phenotype
will depend on alterations in expression and
function of neurotransmitters and their receptors, intracellular signaling molecules, transcription factors, and genes.
++++
++++
++++ peripheral
chemoreceptor

at birth huge physiological changes
breathing begins in utero as early as 11 weeks
•this is in contrast to human adults with well
developed ventilatory control system who
demonstrate sustained hyperventilation
in response to hypoxia.
•up to 6 months of age hypoxic ventilatory response in
term infants shows considerable variability with respect
to ventilation and arousal responses with an initial period
of augmentation in ventilation followed by a sustained
reduction in ventilation similar to
or below normoxic ventilation. ventilatory
control
system
MacLean JE, Paediatr Respir Rev. 2015 Aug 14.
Physiology of breathing

Minute Ventilation in Infancy
Minute ventilation (V) = respiratory rate (RR) X tidal volume (VT)
is related to metabolic rate.
to respond to increased metabolic demand,
minute ventilation can be increased by: •increasing the RR,
•increasing the VT,
•or both.
.

Minute Ventilation in Infancy
Minute ventilation (V) = respiratory rate (RR) X tidal volume (VT)
is related to metabolic rate.
to respond to increased metabolic demand,
minute ventilation can be increased by: •increasing the RR,
•increasing the VT,
•or both.
.
In the newborn and young child, increasing the respiratory rate
(rather than tidal volume) is the most energy-efficient strategy
to cope with higher ventilatory needs.
Ross KR. Clin Chest Med. 2014;35:457.

A study of breathing pattern and ventilation in newborn
infants and adult subjects.
Al-Hathlol K, Acta Paediatr 2000;89:1420–5.
Box and Whisker plot of ventilatory variable
-minute ventilation (VE) (L/Kg/min),
-respiratory frequency (f) (n°/min),
-tidal volume (VT) (mL/Kg/min),
Instantaneous ventilation decreased from
preterm to adult subjects primarily due to
a decrease in frequency .
Coefficients of variation always decreased
with age.
Horizontal bars in the boxes reflect median
value. Differences between groups are all
significant at p < 0.05.
6-7.5 mL/kg

Breath duration and its components .
Duration of the average breath increased with
postnatal age and this was related to increases
both in:
Ti (inspiratory time in seconds) and
Te (espiratory time in seconds) .
Note the high coefficient of variation for Te in
preterm infants. The high variability in Te early
in life represents an effort to maintain lung
volume through increased post-inspiratory
diaphragmatic activity and increased
upper airway resistance in an attempt
to avoid collapse due to poor chest wall recoil.

The “effective” respiratory
timing (Ti/Ttot) is low in
preterm and increases towards
adulthood.
The changes in respiratory
frequency with age related
to adjustments in Ti and Te,
Ti increasing almost threefold
from preterm to adulthood and
Te increasing only twofold.
In the preterm infant Ti
occupied only 34% of the total
breath duration and in adults
it represented 45%
Diagram of the shape of a breath
45%34%
1:2
1:1.5
1:1
Ti:Te

Frequency of obstructive and mixed sleep apneas in
1,023 infants. Kato I, Sleep. 2000;23(4):487-92.
To collect normative data
on the frequency of
obstructive and mixed sleep
apneas in healthy infants
born at term.
1023 infants (2-27 weeks)
recorded during one night in
a sleep laboratory
Apnea defined as ≥ 3 sec.
50 –
40 –
30 –
20 –
10 –
0
% of infants with
Obstructive
Apneas (OAs)
Central Apneas
(CAs)
40.0%
22.3%
*
15.8% had both
Oas and CAs

Frequency of obstructive and mixed sleep apneas in
1,023 infants. Kato I, Sleep. 2000;23(4):487-92.
To collect normative data
on the frequency of
obstructive and mixed sleep
apneas in healthy infants
born at term.
1023 infants (2-27 weeks)
recorded during one night in
a sleep laboratory
Apnea defined as ≥ 3 sec.
50 –
40 –
30 –
20 –
10 –
0
% of infants with
Obstructive
Apneas (OAs)
Central Apneas
(CAs)
40.0%
22.3%
*
15.8% had both
Oas and CAs
Obstructive apneas
lasting > 5-10
seconds were more
frequent in the
2-7 week age group
than in the later ages
(p<0.001 for both)

Physiology of breathing
ventilatory
control
system
3-6 sec 3-6 sec 3-6 sec
≈ 20 sec ≈ 20 sec
Periodic breathing is a pattern of breathing
characterized by recurrent central apnoea
(< 6sec, usually 3 sec) that occur in groups of
3 or more, interrupted by breathing efforts
(for 20 sec) which may be seen in both preterm
and term infants after 48 hours of age.
Periodic breathing is more common
during sleep, and is usually seen from
1 week to 2 months of age; it tends to disappear
by 6 months and is of no consequence and is
thought to reflect a developmental instability
in the ventilatory control system.

Longitudinal assessment of hemoglobin oxygen saturation
in healthy infants during the first 6 months of age.
Collaborative Home Infant Monitoring Evaluation (CHIME) Study Group.
Hunt CE, J Pediatr 1999;135:580–6.
64 infants
from 2 to 25 weeks
Median baseline
SaO2 for each
postnatal week at
2 to 25 weeks
of age.
Variations in
SpO2 occurring
with increasing age
are not significant.
59% of infants had at least one epoch with a baseline <90%
saturation median was 97.9% with the 10th percentile at 95.2%

Longitudinal assessment of hemoglobin oxygen saturation
in healthy infants during the first 6 months of age.
Collaborative Home Infant Monitoring Evaluation (CHIME) Study Group.
Hunt CE, J Pediatr 1999;135:580–6.
Number of seconds
with SaO2 <90%
during acute
decrease,
expressed per hour
by
presence of
periodic breathing
or not
and by week
of age.
more frequent during active or REM sleep
number of seconds per hour with saturation < 90%
Rapid eye movement sleep characterized by
quick, random movements of the eyes and
low muscle tone

Respiratory rate in 1st year of life, awake (solid line)
and asleep (dashed line) showing 5th, 50th and 95th centiles.
Rusconi F, Pediatrics 1994;94:350–5.
The normal
respiratory
rate
gradually
decreases
over the 1st
year
There is no
gender
difference
and
the rate
is higher
in awake
babies

Respiratory rate in 1st year of life, awake (solid line)
and asleep (dashed line) showing 5th, 50th and 95th centiles.
Rusconi F, Pediatrics 1994;94:350–5.
The normal
respiratory
rate
gradually
decreases
over the 1st
year
There is no
gender
difference
and
the rate
is higher
in awake
babies
In the first
months of life
tachypnea is defined as
a respiratory rate
> 60 breaths
per minute.

Measuring the respiratory rate is not always easy, especially
if the baby is moving or crying; both these change the rate,
and the state of the baby (especially awake vs asleep)
should be recorded alongside the rate.
It is easier to use a stethoscope to listen for breaths
rather than simply watching the baby’s chest.
Auscultation usually gives a higher rate, presumably due
to missed breaths from observation alone.
The rate needs to be measured over a full minute
(or the sum of two sets of 30 s), and will need to be
repeated a few times if it is variable.
IS THE BABY TACHYPNOEIC?
Not x2
30s
+
30s

 The respiratory rate
when awake
did not correlate with
the severity of
a baby's illness or the
presence of serious lower
respiratory tract infections.
Respiratory rate and severity of illness in babies
under 6 months old. Morley CJ, Arch Dis Child. 1990;65:834-7.
RR No k
with illness
 Respiratory rate in babies
under 6 months of age.
 Counted by 2 observers
in 1007 babies of whom 709
were seen when presenting
to hospital for assessment
of an acute illness, and 298
on a random basis at home.

60 61 59 58
Respiratory rate of awake babies compared with
assessor's impression of the severity of illness
Respiratory rate and severity of illness in babies
under 6 months old. Morley CJ, Arch Dis Child. 1990;65:834-7.

 The respiratory rate (breaths per minute) thresholds are set at:
- 60 for infants < 2 months of age,
- 50 for infants from 2 to 12 months,
- 40 for children aged 1 to 5 years.
 WHO recommends a repeat respiratory rate count for infants
under 2 months of age when the initial count is 60 or higher.
Respiratory rate and pneumonia in infancy
Berman S, Arch Dis Child. 1991;66:81-4

 The respiratory rate (breaths per minute) thresholds are set at:
- 60 for infants < 2 months of age,
- 50 for infants from 2 to 12 months,
- 40 for children aged 1 to 5 years.
 WHO recommends a repeat respiratory rate count for infants
under 2 months of age when the initial count is 60 or higher.
Respiratory rate and pneumonia in infancy
Berman S, Arch Dis Child. 1991;66:81-4
a repeat count 45 to 60 minutes later will be less than the threshold in more
than two thirds of the patients with an upper respiratory infection
in contrast to patients with lower respiratory infection
who will usually maintain a raised respiratory rate.
=

Although respiratory rate is a useful predictor of lower respiratory
infection in young infants, it does not correlate well with hypoxia.
The presence of subcostal retractions
is a more useful predictor of hypoxia
expecially if associated with nasal flaring,
refusal to eat, and abdominal distention.
Berman S, Pediatr Emerg Care. 1990;6(3):179-82.
Berman S, Arch Dis Child 1991;66:81–4.
Meaning of Respiratory Rate
RR ≠ SaO2
≈ SaO2

IS THE BABY UNWELL?
Certain symptoms elicited from the history at this stage are
red flags for concern of a significant underlying condition:
▸ Persistent cough
▸ Apnoeic episodes
▸ Noisy breathing
▸ Poor feeding
▸ Vomiting
▸ Choking when drinking
Apnea in infants is most commonly
defined as a pause in breathing for
at least 20 seconds,
or a shorter pause that is
associated with bradycardia
or oxygen desaturation.
Uliel S, Chest 2004;125:872–8.
X X X X X X X X X X

IS THE EXAMINATION NORMAL?
The child’s weight, height and
head circumference will give an indication
of faltering growth which may be secondary
to tachypnoea or an underlying condition.
Underlying conditions: Weight Lenght
(Height)
Head Circumference
Malnutrition,
loss of calories, inability to
use calories peripheraly
+++ Reduced Not reduced Normal
Short stature,
heart or renal diseases,
scheletal dysplasias
+ Not much
reduced
+++ Reduced Normal/enlarged
CNS abnormalities,
chromosomal defects,
in utero/perinatal insuts
+++ Reduced +++ Reduced +++ Reduced

▸ Respiratory distress
▸ Cyanosis, paleness
▸ Cardiac murmur
▸ Abnormal femoral pulses
▸ Hepatomegaly
▸ Hypotonia
Clinical examination is important, with some red flag warning signs:
intercostal and subcostal recession,
tracheal tug,
use of accessory muscles,
grunting,
nasal flaring.
Some well babies with tachypnoea
have a degree of recession as the
only other clinical sign.
Balfour-Lynn IM, Arch Dis Child. 2015;100(8):722-7.

Obviously the presence of crackles, wheeze
and stridor is important for the diagnosis.
Paradoxical breathing, an inward movement
of the chest wall during inspiration,
often with a seesaw thoracoabdominal,
motion must be looked for
(neuromuscular disease,
diaphragmatic abnormality,
upper airway obstruction).
normal

The shape of the thorax is checked to ensure
normal dimensions:
very small in asphyxiating thoracic dystrophy,
bellshaped in pulmonary hypoplasia.

The shape of the thorax is checked to ensure
normal dimensions:
very small in asphyxiating thoracic dystrophy,
bellshaped in pulmonary hypoplasia.
Barrel-shaped chest is
frequently seen in post-term
infants who have
meconium aspiration syndrome

The presence of
persistent rhinitis in infancy
should make one consider
primary ciliary dyskinesia.
The patency of the nose must be examined to
exclude choanal stenosis or unilateral or bilateral
atresia holding the stethoscope over the nostrils
(it may be necessary to
pass a nasogastric tube down both sides).
choanal atresia

Cardiac apex may be visibly displaced
(> 5° intercostal space) if there is cardiomegaly.
Precordial palpation may reveal a
right-sided impulse in Scimitar syndrome,
or complex forms of congenital heart
disease associated with atrial isomerism
(visceral heterotaxy or a mirror image
arrangement in primary ciliary dyskinesia).
Examination focused specifically on the
cardiovascular system is also important
and often neglected.
Scimitar syndrome,
characterized by
anomalous venous
return from
the right lung
(to the systemic
venous drainage,
rather than directly
to the left atrium
1
2
3
4
5

A prominent precordial impulse is an
important sign of cardiovascular disease
with pressure or volume overload of the
heart (valvular diseases, septal defects…).
Femoral pulses which are weak or
difficult to feel (coarctation of the aorta), or
generally weak pulses (myocarditis, dilated
cardiomyopathy, severe aortic stenosis or
other forms of left heart obstruction)
can be important signs.
Liver enlargement is usually a manifestation
of heart failure, but can be palpable
if it is displaced by overinflated lungs.

These include:
•holosystolic murmur (odds ratio [OR] of pathologic murmur = 54),
•grade 3 (heard easily) or higher (OR = 4.8),
•harsh quality (OR = 2.4),
•an abnormal S2 (OR = 4.1),
•maximal intensity at the upper left sternal border (OR = 4.2),
•systolic click (OR = 8.3),
•diastolic murmur, or increased murmur intensity with standing.
•decrease or lack of change in the murmur intensity with passive leg
elevation (likelihood ratio [LR] = 8.0) or when the child moves
from standing to squatting (LR = 4.5) increases
the likelihood of hypertrophic cardiomyopathy
Certain characteristics of the murmur may be considered
red flags, prompting stronger consideration
for structural heart disease
Frommelt MA. Differential diagnosis and approach to a heart murmur in term infants.
Pediatr Clin North Am. 2004;51(4):1023–1032
pulmonary
valve

Cyanosis would be visible if the deoxygenated hemoglobin content is
> 3 g% (3 g per 100 mL).
If cyanosis is present throughout the body,
including the mucous membranes and tongue,
it is called central cyanosis.
If cyanosis is limited to the extremities,
it is called peripheral cyanosis, also known as
acrocyanosis.
1) exposure to cold,
2) polycythemia.

in conditions in which the infant is exposed to a cold environment,
it could also be the presenting sign of serious conditions such as:
1) sepsis
2) hypoglycemia
3) hypoplastic left-heart syndrome
(decreased peripheral perfusion)
peripheral cyanosis is seen:
Peripheral cyanosis should not be ignored
unless other conditions have been ruled out.
hence diffuse mottled, bluish-gray
appearance of this infant's skin
suggestive of systemic poor
perfusion

Differential Cyanosis: another form of cyanosis
1) the upper part of the
body remains pink and
lower part of the body
remains cyanotic.
right-to left shunt from the
pulmonary artery to the
descending aorta through the
patent ductus arteriosus (PDA)
such as in hypoplastic left
heart syndrome or severe
pulmonary hypertension
body remains cyanotic
while the lower part of
the body remains pink.
transposition of the great vessels
with pulmonary hypertension and
shunt through PDA,
total anomalous pulmonary
venous drainage above the
diaphragm with shunt through
PDA (higher oxygen content
in the right ventricular blood).

Differential Cyanosis: another form of cyanosis
body remains pink and
lower part of the body
remains cyanotic.
right-to left shunt from the
pulmonary artery to the
descending aorta through the
patent ductus arteriosus (PDA)
such as in hypoplastic left
heart syndrome or severe
pulmonary hypertension
body remains cyanotic
while the lower part of
the body remains pink.
transposition of the great vessels
with pulmonary hypertension and
shunt through PDA,
total anomalous pulmonary
venous drainage above the
diaphragm with shunt through
PDA (higher oxygen content
in the right ventricular blood).
Thus whenever tachypnoea has a cardiovascular
cause, even without a heart murmur there is usually
a clinical pointer to a cardiac anomaly.

Conditions that can give rise to arterial oxygen desaturation and
cyanosis:
1) Hypoventilation (tachipnea alone is not a good marker of hypoxia)
2) Significant right-to-left intracardiac or intrapulmonary shunting
3) Ventilation perfusion unevenness
4) Diffusion impairment (very rare)
5) Inadequate transport of oxygen by the hemoglobin
(mutant hemoglobins).
Sasidharan P. Pediatr Clin North Am. 2004;51(4):999-1021

Conditions that can give rise to arterial oxygen desaturation and
cyanosis:
1) Hypoventilation (tachipnea alone is not a good marker of hypoxia)
2) Significant right-to-left intracardiac or intrapulmonary shunting
3) Ventilation perfusion unevenness
4) Diffusion impairment (very rare)
5) Inadequate transport of oxygen by the hemoglobin
(mutant hemoglobins).
Sasidharan P. Pediatr Clin North Am. 2004;51(4):999-1021
The net result of an inadequate oxygen delivery
to the tissues leading to hypoxia is
increased production of hydrogen ions and lactic acid,
giving rise to metabolic acidosis.
The detection of metabolic acidosis
(pH <7.35, PaCO2 <35, HCO3- < 22 mEq/L)
along with cyanosis in an infant
usually indicates a compromised state
and requires immediate attention.

Abdominal examination is done to exclude an
enlarged liver or spleen, or an abdominal mass.
A basic neurological examination is done,
particularly for hypotonia which may indicate a
neuromuscular disorder.
Always listen over the skull and particularly the
occiput for the murmur of a cerebral arteriovenous
fistula, whose only clinical manifestation might be
tachypnoea. Iizuka Y, Neuroradiol J. 2011;24(5):772-8

Algorithm for
management
of a baby with
tachypnoea.
CXR, chest radiograph;
SpO2, oxygen saturation.
Balfour-Lynn IM, Arch Dis Child. 2015;100:722-7.
SaO2 < 95%
it is possible to wait
for 4 weeks

Oxygen saturation (SpO2) reference values
 Healthy infants aged < 1 year
The median baseline saturation in healthy term infants during the
first year of life is 97–98%.
In only 5% of healthy infants is the SpO2 < 90% for > 4% of the time.
 Healthy children aged >1 year
The median baseline SpO2 in healthy children >1 year old is 98%
with a 5th centile of 96–97%.
A healthy child aged 5–11 years spends no more than 5% of the
time below a SpO2 of 94% while asleep.
Balfour-Lynn IM, Thorax 2009;64(Suppl 2):ii1–26.

Newborn pulse oximetry screening is not just
for heart defects.
Meberg A. Acta Paediatr. 2015 Sep;104(9):856-7.
A Norwegian population-based
(n=50.008) prospective multicenter
study of postductal (foot) arterial
oxygen study on the first day of
life reported a SpO2 < 95% in
0.65% of the infants and 41% of
these were, in fact, potentially
severe extra-cardiac disorders,
such as:
•systemic infections – including
group B streptococcal septicaemia –
•amniotic fluid aspiration,
•pulmonary hypertension and
•pneumothorax.
Meberg A, J Pediatr 2008;152:761–5.
(healthy infants)
(severe polycythemia)

Oxygen saturation (SpO2) is the most important
initial investigation.
If the SpO2 is significantly low (<85%) both in room air
and 100% oxygen, one should consider a hyperoxia test.
Sasidharan P. Pediatr Clin North Am 2004;51:999–1021
a formal test is carried out with arterial blood gases (PaO2) or
transcutaneous oxygen monitoring (TcPO2) from the right arm
(preductal) after administration of 100% oxygen for 5–10 min.
if supplemental oxygen does not improve the
partial pressure of oxygen in arterial blood
(PaO2), cyanotic congenital heart disease or
some form of right to left shunting must be
considered with an immediate
cardiology assessment.

Oxygen saturation (SpO2) is the most important
initial investigation.
A useful guide to evaluate the response to hyperoxia test:
▸ PaO2> 33 kPa (247.5 mm3)—excludes cyanotic heart disease
(neuromuscolar-neurologic disease more likely)
▸ PaO2> 26 kPa (195 mm3)—cyanotic heart disease very unlikely
(pulmonology disease more likely)
▸ PaO2< 13 kPa (97.5 mm3)—cyanotic heart disease extremely likely
(1 kPa = 7.500617 mmHg; 1 mmHg = 0.133322 kPa)
Balfour-Lynn IM, Arch Dis Child. 2015;100(8):722-7.

Chest Radiography
The expansion of lungs
(lung volume) on both sides
should be checked.
Normal inspiratory films should
have 8 intercostal spaces of
lung fields on both sides.
1
2
3
4
5
6
7
8
1
2
3
4
5
6
7
8

Chest Radiography
The locations of stomach, liver, and heart
should be determined to rule out
dextrocardia and situs inversus.
stomach

Chest Radiography
Increased pulmonary vascular markings
and pulmonary congestion are indicative
of a left-to-right shunt.
Decreased pulmonary vascular markings
(oligemic lung fields) are generally indicative of
pulmonary stenosis or pulmonary atresia with
inadequate ductal shunting and occasionally
persistent pulmonary hypertension
of the newborn (PPHN).
Ventricular septal defect

Chest Radiography
Red arrow points to end of orogastric tube
which is blocked from entering the distal
esophagus by the patient's esophageal
atresia.
Note the lack of gas in the abdomen
indicating a fistulous tract does not connect
the trachea to the distal esophagus
With esophageal atresia and no fistula, no air
enters GI tract and abdomen is airless
Stomach should have air in it
15 minutes after birth normally.

Chest Radiography
Heart size, shape and position may indicate
cardiac disease.
The integrity of the diaphragm is checked
(eventration, hernia, paralysis).
Chest shape and size are assessed
(eg, bell-shaped in pulmonary hypoplasia),
as well as the ribs themselves,
which may have the classic abnormalities
of severe neonatal rickets, or be very
thin (gracile) in neuromuscular disease.
The cardiothoracic ratio aids in the
detection of cardiomegaly, is measured on a
PA chest x-ray, and is the ratio of:
maximal horizontal cardiac diameter
maximal horizontal thoracic diameter
(inner edge of ribs / edge of pleura)
A normal measurement should
be less than 0.5
normal
enlarged
<0.5

Chest Radiography
cardiac disease.
thin (gracile) in neuromuscular disease.
eventration
hernia
paralysis

Chest Radiography
cardiac disease.
thin in neuromuscular disease.
pulmonary hypoplasia
rachitic rosary

Chest Radiography
cardiac disease.
pulmonary hypoplasia
neuromuscular
disease

Chest Radiography
cardiac disease.
Additionally, as with any
unexplained
symptom in children,
non-accidental injury
must be considered
and the
ribs checked for fractures.

Oxygen saturation & Chest Radiography
At this stage, a well baby with tachypnoea
(with or without some recession),
who has a normal SpO2 and chest radiograph,
can leave clinic and go home
with follow-up in 4 weeks,
or sooner if the clinical picture changes.
normal

Generally the babies remain well and gradually the degree of
tachypnoea reduces with time.
However if there is no improvement, and
especially if the degree of respiratory distress
increases, further investigations become necessary.
FURTHER INVESTIGATIONS
Red flag initial investigations:
▸ Hypoxia (oxygen saturation (SpO2) ≤ 94%)
▸ Abnormal chest radiograph

cardiac failure,
sepsis,
asphyxia,
metabolic disorders.
closure of the ductus arteriosus in an infant who has
ductal-dependent cardiac lesion will lead to shock and
severe metabolic acidosis, with cyanosis and respiratory distress.
Arterial blood gases help in
determining the oxygenation,
ventilation, and acid-base status.
if significant
metabolic acidosis
pH < 7.35
HCO3- (mEq/L) < 22

EKG is an important test, but of limited
value except in certain specific conditions.
Normally, there is right-ventricular
predominance in the newborn, and many
cases of cyanotic congenital heart disease
(CHD) will have
similar findings.
Echocardiogram is the gold standard in the
diagnosis of congenital cardiac lesions and
pulmonary hypertension.

the second wave of investigations may well include the following:
Dual probe 24-h pH study (salivary pepsin?).
 Swallowing assessment —clinical evaluation and
videofluoroscopic swallowing study.
 Ultrasound or fluoroscopic screening of the diaphragms.
 CT chest scan —this can be done without the need
for sedation in most infants, using the ‘feed and wrap’ technique.
Sury MR, Clin Radiol 2005;60:731–41.
There is no need for a contrast-enhanced scan at this stage.
submucous
clefting

'Feed and wrap' or sedate and immobilise for
neonatal brain MRI?
Ibrahim T, Arch Dis Child Fetal Neonatal Ed. 2015;100(5):F465-6.
A sedated infant still asleep post MRI
within the Med-Vac immobiliser.
chloral hydrate
sedation
(50 mg/kg single dose)
combined with
Med-Vac
immobilization
has safely achieved a
100% success rate for
completed MRI with
good-quality clinical
and research imaging,
and has proved far
superior to the
‘feed and wrap’ method.

Tachypnoea in a well baby: what to do next?
Attilio Boner
University of
Verona, Italy
attilio.boner@univr.it
Introduction
Physiology of breathing
What is tachypnoea?
Is the baby unwell?
Is the examination normal?
Immediate clinic investigations:
SaO2, Chest X-ray
Further investigations
Potential causes of tachypnoea when
the diagnosis is not immediately obvious
Outcome
Summary & Conclusions

POTENTIAL CAUSES OF TACHYPNOEA
WHEN THE DIAGNOSIS IS NOT IMMEDIATELY OBVIOUS
•small thorax of Jeune asphyxiating thoracic dystrophy,
•bell-shaped chest from pulmonary hypoplasia,
•hypotonia associated with neurological or neuromuscular disease,
•stridor due to upper airway obstruction
•severe neonatal rickets.
Often the tachypnoea is
just one of several signs
in a clearly unwell child
•a metabolic acidosis
•bacterial sepsis; or
•significant pain,
for example, from a volvulus.
In some children,
the cause becomes
more clear
from the history
•narcotic withdrawal
•postbirth asphyxia.
In some, it may be
determined
on first examination

POTENTIAL CAUSES OF TACHYPNOEA
WHEN THE DIAGNOSIS IS NOT IMMEDIATELY OBVIOUS
Respiratory and upper airway
Infection
Cystic fibrosis
Interstitial lung disease
Congenital thoracic malformations
Pulmonary hypoplasia
Congenital diaphragmatic hernia
Diaphragmatic weakness
H-type tracheo-oesophageal fistula
Nasal obstruction

Pulmonary tuberculosis has been described in children
under 1 year, with tachypnoea the predominant sign.
Arikan-Ayyildiz Z, Turk J Pediatr 2011;53:250–4.
Infection.
It is likely the child would
have other symptoms and signs
fever
cough
shallow breathing
nevertheless,
low grade
or chronic
infection
must be
considered.
a nasopharyngeal aspirate for respiratory viruses
a cough swab for bacterial culture,
a white cell count, C reactive protein, procalcitonin
may be helpful
Chest radiograph shows cavity within
consolidation in the right lobe.

Most babies are symptom-free from the respiratory perspective,
the majority have suboptimal weight gain, often with abnormal stools.
Infants can present with tachypnoea, recurrent cough and sometimes
wheeze, but our experience is that it is most unusual for tachypnoea
to be the sole symptom in a baby with CF.
A sweat test must be considered.
Cystic fibrosis
newborn screening babies are diagnosed with cystic fibrosis
(CF) at around 3–4 weeks of age
however screening is not infallible

Interstitial lung disease
often associated with:
•recession,
•crackles
•and sometime hypoxia.
a heterogeneous
group of rare diseases
crackles,
recession,
hypoxaemia,
eventualy
failure to thrive.
most often
accompanied by:
Kuo CS, Curr Opin Pediatr 2014;26:320
in some infants , tachypnoea is the only
obvious symptom, and the severity of
symptoms and signs varies.
Neuroendocrine cell hyperplasia,
previously known as
persistent tachypnoea of infancy
Deterding RR, Pediatr Pulmonol 2005;40:157–65

European protocols for the diagnosis and initial treatment
of interstitial lung disease in children.
Bush A, Thorax. 2015;70(11):1078-84.
THE STARTING POINT: WHEN TO SUSPECT
CHILD—TYPICAL PRESENTATIONS
Shortly after birth
The earliest presentation of chILD is shortly after birth,
with unexplained respiratory distress in a term baby.
This frequently and rapidly proceeds to intubation and ventilation,
with relentlessly progressive respiratory failure leading to death or
lung transplantation, if the latter is available.
First 2 years of life
Later presentations of chILD are very non-specific and symptoms in
descending order of frequency are: fast breathing, failure to thrive,
typically dry cough, and wheeze in 25% of cases
in the absence of respiratory tract infection. XX X X X

Staging the severity of childhood interstitial lung disease
(chILD)
Pediatric interstitial lung disease revisited.
Fan LL, Pediatr Pulmonol 2004;38:369–78.
(awake)

Bush A, Thorax. 2015;70(11):1078-84.
Proposed flow chart for investigating interstitial lung disease in children (chILD)
If the child
is well
and stable,
progress
to the more
invasive
investigations
may not
be indicated.

Bush A, Thorax. 2015;70(11):1078-84.
SURGICAL LUNG BIOPSY
procedure must only be undertaken by an experienced surgeon, who is
confident of obtaining adequate biopsies (at least 10×10×10 mm);
a very superficial biopsy, which does not contain distal airways, may result in
diagnostic error.

40-month-old girl with typical appearance of neuroendocrine cell hyperplasia of infancy
Brody AS, AJR Am J Roentgenol. 2010;194:238-44
Inspiratory high-resolution CT scans show sharply defined areas of ground-glass opacification along
mediastinal borders, peripherally, and most prominently in right middle lobe and lingula (asterisks, C)
Expiratory high-resolution CT scans show only mild increase in attenuation in all areas of lung, consistent
with air trapping involving both areas with ground-glass opacification and areas with normal attenuation
* *

Surfactant protein B deficiency is lethal in neonates
so is unlikely to present with just tachypnoea.
Inherited surfactant protein C deficiency,
•Tachypnoea and other signs of respiratory distress
are likely to be present.
Thouvenin G, Arch Dis Child 2010;95:449–54.
•A chest CT scan can be highly suggestive,
although diagnostic certainty
may require a lung biopsy,
and gene mutation studies.
Interstitial lung disease
chest radiograph and computed tomography scan
of a patient at 18 months showing
diffuse ground glass pattern and
interstitial thickening.

Congenital Thoracic Malformations
Cystic congenital
thoracic malformations
usually asymptomatic
diagnosed antenatally during
fetal ultrasound scanning•Congenital cystic adenomatoid malformation (CCAM)
is usually observed in neonates because of
respiratory distress and may occasionally be
observed in older children or adults with recurrent
infection.
•This malformation consists of adenomatoid
proliferation of bronchioles that form cysts
instead of normal alveoli:
type 1 CPAM are one or more
large air-filled cystic lesions
Type 2 CPAM usually consists of an
air-filled multicystic mass or focal
area of consolidations
a) Chest radiograph shows an area of hyperlucency (arrow) in the right lower lobe.
b) CT scan demonstrates the presence of several small cysts (arrow) in the right lower lobe.
CPAM = Congenital pulmonary airway malformation

Congenital Thoracic Malformations
in some cases tachypnoea does not develop for 1–4 months and
although usually obvious on a plain chest radiograph, the lesion may be
only apparent on a CT scan. Rusakow LS, Pediatr Pulmonol 2001;32:246–9.
Congenital Lobar Emphysema
neonatal respiratory distress present
at birth or within the first 48 h,
a developmental anomaly of the lower respiratory
tract that is characterized by hyperinflation
of one or more of the pulmonary lobes

Radiographically occult congenital lobar emphysema
presenting as unexplained neonatal tachypnea
Rusakow LS, Pediatr Pulmonol 2001;32:246-9
A neonate with tachypnea of unclear etiology who was diagnosed with
congenital lobar emphysema (CLE) by chest computed tomography scan,
despite a plain chest radiograph that did not suggest the diagnosis.
Chest CT scan image demonstrating
hyperinflated (hyperlucent) left upper lobe
anterior to normal-appearing superior segment
of left lower lobe; right lung also appears normal
Chest radiograph of 8-day-old
boy demonstrating hyperinflation
without focal abnormalities

 Congenital Lobar Emphysema usually presents in infancy with
dyspnea, wheezing, cough, tachypnea, cyanosis during feeding and
crying, and intercostal retractions.
Rusakow LS, Pediatr Pulmonol 2001;32:246-9
 Classically, 3 clinical patterns have been described:
1) findings develop within the first 2 days of life;
2) findings or signs develop between 1-4 months of life;
3) a rare, fulminant form, associated with severe cardiorespiratory
involvement, which may lead to death within hours.
Leape LL, Pediatrics 1964;34:246-255
Congenital Lobar Emphysema: Patterns of Presentation

The etiologies for CLE, are thought to be multiple, and may involve:
1) partial bronchial obstruction due to extrinsic compression,
2) intraluminal obstruction,
3) intrinsic bronchial abnormality such as bronchomalacia,
4) a primary alveolar abnormality.
Leape LL, Pediatrics 1964;34:246-255
all leading to a
“check-valve” effect
Congenital Lobar Emphysema: Etiologies
Histopathology of
congenital lobar
emphysema with marked
overdistention of all alveoli.

Pulmonary Hypoplasia
Severe oligohydramnios
Congenital Diaphragmatic Hernia,
Ebstein’s malformation
of the tricuspid valve
(reduced blood flow to he lungs)
It is rarely an isolated phenomenon (primary).
Aiton NR, BMJ 1996;312:1149–50.
more commonly is
secondary and
associated with
disorders of
lung growth

Patients may present in
early infancy, and the
severity depends on the
degree of hypoplasia;
persistent tachypnoea and
breathlessness with
activity/exercise
when the hypoplasia
is not too severe
when
unilateral
the thorax is asymmetrical,
reduced air entry and
underexpansion on that side.
diffuse haziness (nebulosità) of
the right hemithorax (arrow),
slight deviation of the trachea
and heart to the right, and poor
differentiation of the right heart
border.

activity/exercise
when the hypoplasia
is not too severe
when
unilateral
border.
Confirmation can usually be made with
a chest radiograph or a chest CT scan.
With normal radiology, infant lung function testing may
be necessary which would indicate reduced lung volumes
and reduced functional
residual capacity.
Reference values for residual volume, functional residual capacity and
total lung capacity. ATS Workshop on Lung Volume Measurements.
Official Statement of The European Respiratory Society.
Stocks J, Quanjer PH. Eur Respir J. 1995 ;8(3):492-506.

activity/exercise
when the hypoplasia
is not too severe
when
unilateral
border.
Primary pulmonary hypoplasia, however, is thought
to be very rare and published reports are few.
Swischuk LE. JPediatr 1979;95:573-8.
Boylan P. Irishj Med Sci 1977;146:179-80.
There is a large discrepancy, however, with the incidence
reported in one post mortem series:
pulmonary hypoplasia was the commonest single
abnormality present in 15-20%
of early neonatal deaths.
Wrigglesworth JS. Lancet 1982;i:264-7.

Right Sided Pulmonary Hypoplasia: Scimitar syndrome
partial or complete anomalous pulmonary
venous drainage into the inferior vena cava,
commonly associated with:
persistent left superior vena cava,
dextrocardia,
pulmonary sequestration,
right pulmonary hypoplasia
If pulmonary hypoplasia
is right sided
pulmonary vasculature
must be visualised
because of the
association with
Scimitar syndrome

If pulmonary hypoplasia
is right sided
pulmonary vasculature
must be visualised
because of the
association with
Scimitar syndrome
tachypnoea, recurrent pneumonia, failure to thrive and heart failure.
the infantile form of
Scimitar tends to present
in the first 2 months with:
The triad of:
1) respiratory distress,
2) right lung hypoplasia, and
3) dextroposition of the heart
should alert the clinician to
think of scimitar syndrome.
Right Sided Pulmonary Hypoplasia: Scimitar syndrome

Congenital Diaphragmatic Hernia
Often diagnosed antenatally or
within hours or days of birth.
However late-presenting
(>1 month) is well recognised,
Tachypnoea
Vomiting
most common symptoms

Diaphragmatic Weakness
the chest wall is so compliant,
the mediastinum is more mobile
the intercostal muscles are weaker
the diaphragm is critical
to respiration in infancy
due to the fact
Even unilateral diaphragmatic weakness
can cause a degree
of respiratory distress.

due to the fact
can cause a degree
at this age, phrenic nerve injury
is the most common cause.

due to the fact
can cause a degree
at this age, phrenic nerve injury
is the most common cause.
When due to a difficult delivery, for
example, following shoulder dystocia, the
majority of infants with phrenic nerve
palsy also have a brachial plexus injury
(Erb’s palsy). (a paralysis of the arm
caused by injury to the upper group of
the arm's main nerves)

Diaphragmatic paralysis associated with neonatal brachial
plexus palsy. Bowerson M, Pediatr Neurol 2010;42:234–6.
newborns with brachial
plexus palsy evaluated
during 2005-2009 (n = 166)
at University of Michigan
Medical School
3 –
2 –
1 –
0 -
of patients with brachial plexus
palsy % with clinically significant
diaphragmatic palsy
2.4% (4/166)
of these, a majority (75%; n = 3)
manifested respiratory complications
sufficient to warrant diaphragmatic
plication

congenital neuromuscular
disorders can cause severe
diaphragmatic weakness.
spinal muscular atrophy with
respiratory distress (SMARD1)
unlike more typical
spinal muscular atrophies,
presents with respiratory distress
before distal muscular weakness is obvious

The respiratory difficulty is due to diaphragmatic weakness
followed by paralysis (usually on the right side initially),
Usually accompanied by a weak cry and stridor.
A chest X-ray may indicate an abnormal position of a hemidiaphragm.
An ultrasound or fluoroscopic examination of the diaphragms can
demonstrate abnormal movements, especially if paradoxical or
weak/paralysed.
congenital neuromuscular
disorders can cause severe
diaphragmatic weakness.
spinal muscular atrophy with
respiratory distress (SMARD1)
unlike more typical
spinal muscular atrophies,
presents with respiratory distress
before distal muscular weakness is obvious

H-type tracheo-oesophageal fistula.
85% <1%
2% 8% 4%
as well as tachypnoea, the
child would have marked
symptoms on feeding,
usually:
1) coughing,
2) choking,
3) turning blue and
4) potentially recurrent
lung infections.
A tube oesophagram
(video oesophagography)
and/or bronchoscopy may be
necessary to make the diagnosis,

Upper Airway Narrowing
It may be obvious due to the presence of significant or persisting
stridor, especially if the narrowing is more than mild.
Possible causes:
1) laryngotracheomalacia,
2)bronchomalacia,
3)laryngeal or tracheal web,
4)subglottic stenosis.
subglottic
stenosis
tracheomalacia

Upper Airway Narrowing
It may be obvious due to the presence of significant or persisting
stridor, especially if the narrowing is more than mild.
Possible causes:
1) laryngotracheomalacia,
2)bronchomalacia,
3)laryngeal or tracheal web,
4)subglottic stenosis.
subglottic
stenosis
tracheomalacia
Tachypnoea
accompanied
by noisy
breathing is
an indication for
bronchoscopy

Nasal obstruction in newborns.
Gnagi SH, Pediatr Clin North Am. 2013;60(4):903-22.
Newborn infants are obligate nasal breathers for the first several
months of life, with more than 50% of infants desaturating if nasally
obstructed.
Anatomically, their entire tongue length
is in contact with the hard and soft palate,
and the epiglottis is superior to the
soft palate, causing difficulty
with oral breathing.
This allows for concomitant respiration with
oral intake while the infant is learning to
mouth breathe in the first 4 to 6 weeks after birth.
epiglottis position for
breathing swallowing

Newborn infants are obligate nasal breathers for the first several
months of life, with more than 50% of infants desaturating if nasally
obstructed.
Anatomically, their entire tongue length
is in contact with the hard and soft palate,
and the epiglottis is superior to the
soft palate, causing difficulty
with oral breathing.
This allows for concomitant respiration with
oral intake while the infant is learning to
mouth breathe in the first 4 to 6 weeks after birth.
epiglottis position for
breathing swallowing
nasal obstruction may lead to serious
consequences in the neonate, including
respiratory distress or failure to thrive.

Nasal endoscopy revealing
membranous occlusion of the
posterior nasal cavity
consistent with choanal atresia.
Axial CT showing atretic
choanae.
Circle encompasses
atresia at posterior
nasal vault.
Note the
champagne flute
appearance of the nasal
vaults characteristic for
choanal atresia.
coloboma bilaterale dell'iride
fessura a forma di buco della serratura

SYNDROMIC ASSOCIATIONS
Several etiologies of nasal obstruction may present with concurrent
abnormalities that may require further assessment, including genetic
evaluation:
Crouzon,
CHARGE,
Pfeiffer,
Apert,
Treacher-Collins,
Down,
Fetal alcohol syndromes
nasal obstruction
+
plagiocephaly

CTscan of a child with Congenital
nasal pyriform aperture stenosis.
Note the inward bowing of the nasal
processes of the maxillary bone with
the pyriform aperture (the anterior nasal
aperture) measuring 7 mm (arrows).
Child with Congenital nasal pyriform aperture
stenosis and evidence of
a single central megaincisor.

Sagittal magnetic resonance image of
a basal meningocele protruding into
the nasopharynx.
Neonatal iatrogenic nasal
obstruction
Naso-endoscopy revealing synechia on
the right side from CPAP catheters.

Nasal obstruction
Unilateral obstruction may present later
(typically at 5–24 months), and with milder symptoms;
Unilateral atresia is more common than bilateral and accounts
for up to 75% cases of atresia.
Symptoms will be milder if it is a stenosis rather than complete
atresia.
Techniques for diagnosis
1) placing a cold mirror/metal spoon under
the nostril to look for condensation,
2) passing a small nasogastric tube,
3) putting a few drops of saline in the
nostril and looking for bubbles

Cardiac
Cardiovascular disease may present
initially during infancy with just
mild tachypnoea.
With increasing symptoms of
tachypnoea and recurrent chest infections,
cardiac disorders may masquerade as
respiratory disease.
Many causes are ‘silent’ meaning there is no
murmur, so the diagnosis is not always
obvious without assessment by a
cardiologist.
In many conditions mild arterial oxygen
desaturation is not clinically obvious
After careful examination by
the cardiologist, inevitably
children will have an
ECG and
echocardogram
performed
to exclude a number of
potential cardiac diagnoses.

Gastrointestinal
A pH study should be diagnostic, and in an infant a
dual probe study is preferable in order to take
account of any buffering of stomach acid by
frequent milk feeds.
Gastro-oesophageal
reflux
oesophagitis
tachypnoea
but it would need to be quite severe to cause
persistent symptoms;
the child would not necessarily be vomiting.

A key discriminator: GOR or GORD.
GOR: The passage of gastric contents into the
oesophagus, with or without regurgitation/
vomiting; it is considered physiological in
infants when symptoms are absent or not
troublesome,
GORD: The presence of troublesome symptoms
(ie, those that adversely affect the well-being of
the child) and/or complications
(eg, tissue damage or inflammation, as in
oesophagitis,
reactive airways disease
or pulmonary aspiration).

Gastrointestinal
often idiopathic,
sometimes associated with a bulbar palsy,
occasionally anatomical (eg, laryngeal cleft)
velopharyngeal insufficiency (submucous clefting).
Recurrent aspiration of fluid
(eg, milk, saliva)
when drinking the baby
does not necessarily:
cough,
splutter
choke.
can sometimes be silent
DiGeorge Syndrome ?

Gastrointestinal
(eg, milk, saliva)
cough,
splutter
choke.
DiGeorge Syndrome
lipid laden
macrophages.
Nasal regurgitation of
milk can be an
unmasking sign !
the opening of upper
esophageal sphincter
significantly decreased in
the cases of nasal backflow. X

Gastrointestinal
(eg, milk, saliva)
cough,
splutter
choke.
DiGeorge Syndrome
Diagnosis
is made by a
speech therapist’s
clinical assessment,
sometimes followed by
a videofluoroscopic
swallowing study.
lipid laden
macrophages.

congenital heart disease,
infection,
upper airway anomalies
(narrow trachea,
laryngomalacia,
tracheomalacia and
bronchomalacia),
aspiration due to
gastro-oesophageal reflux or
swallowing abnormalities.
Trisomy 21
in babies with Down’s syndrome

OUTCOME
tachypnoea usually resolves,
but may take as long as 6–12 months.
in the absence of
an underlying condition
this means that the
cause in most babies
is never determined.
the improvement may be due to:
1) maturation of automatic control of
breathing (based in the brainstem),
2) response to chemoreceptors, or
3) a change in the mechanical properties
(compliance and volumes) of the lungs
and chest wall.

OUTCOME
tachypnoea usually resolves,
but may take as long as 6–12 months.
in the absence of
an underlying condition
this means that the
cause in most babies
is never determined.
the improvement may be due to:
1) maturation of automatic control of
breathing (based in the brainstem),
2) response to chemoreceptors, or
3) a change in the mechanical properties
(compliance and volumes) of the lungs
and chest wall.
In those with a specific
diagnosis, the outcome
will depend on the
actual diagnosis.

Summary & Conclusions
Respiratory rate has to be counted with the stethoscope
(for 30 sec. twice than add) while the child is asleep.
Tachypnoea (> 60 breaths/min in the first months of life) may be an
self-solving problem but can also be a sign of a severe conditions
expecially if associated with hypoxia, failure to thrive, and cardiac
signs and symptoms.
Persistent cough, Apnoeic episodes, Noisy breathing,
Poor feeding, Vomiting, Choking when drinking are signs for concern.

Summary & Conclusions
If the clinical examination is not completely negative imediate
investigations are:
SaO2 and Chest X-ray, followed by ECG & Echo, and blood gases.
When the diagnosis is not immediate obvious referal is necessary
for: CT scan, bronchoscopy & biopsy, and genetics.
When your are faced with a tachypnoeic infant
it will be wise to maintain a certain degree of
uncertainty and to look for all the possibilities.
If you only trust your memory and experience
I am afraid you may do some mistakes.

19° FORMAT Verona 12-13/05/2017

Grazie per la vostra
attenzione alla storia che
vi ha raccontato il mio nonno.
Ciao a tutti.
Mia Charlize Powell

Format 2016: tachypnoea in a well baby: what to do next?

Recomendados

Recomendados

Más contenido relacionado

La actualidad más candente

La actualidad más candente (20)

Destacado

Destacado (8)

Similar a Format 2016: tachypnoea in a well baby: what to do next?

Similar a Format 2016: tachypnoea in a well baby: what to do next? (20)

Más de Envicon Medical Srl

Más de Envicon Medical Srl (20)

Último

Último (20)

Format 2016: tachypnoea in a well baby: what to do next?