Respiratory distress

syndrome
Dr Yuranga
Weerakkody◉ and Rishi
Agrawal et al.
Respiratory distress
syndrome (RDS) is a
relatively common condition
resulting from insufficient
production of surfactant that
occurs in preterm neonates.
On imaging, the condition
generally presents as
bilateral and relatively
symmetric diffuse ground
glass lungs with low volumes
and a bell-shaped thorax.
Terminology
RDS is also known
as hyaline membrane
disease (not favoured as
reflects non-specific
histological
findings), neonatal respirat
ory distress
syndrome, lung disease of
prematurity (both non-
specific terms), or as some
authors prefer surfactant-
2
deficiency disorder .
Epidemiology
The incidence is estimated at
6 per 1000 births 2.
Clinical presentation
Respiratory distress presents
in the first few hours of life in
a premature baby.
Symptoms include
tachypnoea, expiratory
grunting, nasal flaring. The
infant may or may not be
cyanosed. Substernal and
intercostal retractions may
be evident.
Risk factors include maternal
diabetes, greater
prematurity, prenatal
asphyxia and multiple
gestations.
Associated abnormalities are
those that can occur in
prematurity: germinal matrix
haemorrhage,necrotising
enterocolitis, patent ductus
arteriosus, delayed
developmental milestones,
hypothermia and
hypoglycaemia.
Pathology
Immature type II
pneumocytes cannot
produce surfactant. The lack
of surfactant increases the
surface tension in alveoli
causing collapse. Patients
have a decreased lecithin:
sphingomyelin
ratio. Damaged cells,
necrotic cells, and mucus
line the alveoli.
Radiographic features
Plain radiograph
 typically gives diffuse
ground glass lungs with
low volumes and a bell-
shaped thorax
 often tends to be bilateral
and symmetrical

 air bronchograms may

be evident

 lung whiteout in severe

cases

 hyperinflation (in a non-

ventilated patient)
excludes the diagnosis
  radiographs may show
hyperinflation if the
patient is intubated

RDS can be safely excluded

if the neonate has a normal
chest radiograph at six hours
after birth.
If treated with surfactant
therapy there may be a
symmetric improvement.
Treatment and prognosis
Exogenous surfactant
administration. Supportive
oxygen therapy.
Complications
Acute
 persistent patent ductus
arteriosus (PDA) due to
reduced oxygen stimulus

 pulmonary interstitial
emphysema (from
treatment)
  oxygen toxicity (from
treatment)

 pulmonary
haemorrhage (can also
be included in the
differential diagnosis)
Chronic
 bronchopulmonary
dysplasia

 recurrent pulmonary
infection
  subglottic stenosis (from
intubation)

Symptoms & Causes

In-Depth
What causes HMD?
HMD occurs when there is
not enough of a substance in
the lungs called surfactant.
Surfactant is made by the
cells in the airways and
consists of phospholipids
and protein. It begins to be
produced in the fetus at
about 24 to 28 weeks of
pregnancy, and is found in
amniotic fluid between 28
and 32 weeks. By about 35
weeks gestation, most
babies have developed
adequate amounts of
surfactant.

Neonatal Respiratory
Distress Syndrome -
NRDS (Hyaline membrane
disease) is characterized by
collapsed alveoli alternating
with hyperaerated alveoli,
vascular congestion
and hyaline
membranes (resulted from
fibrin, cellular debris, red
blood cells). Hyaline
membranes appear like an
eosinophilic, amorphous
material, lining or filling the
alveolar spaces and blocking
the gases exchange. (H&E,
ob. x10)

the first 48 to 72 hours, then

improves with treatment.

How is RDS diagnosed?

RDS is usually diagnosed by

a combination of

assessments, including the

following:
 Appearance, color, and

breathing efforts (indicate a

baby's need for oxygen).

 Chest X-rays of lungs--

often show a unique

"ground glass" appearance

called a reticulogranular

pattern. X-rays are

 electromagnetic energy

used to produce images of

bones and internal organs

onto film.

 Blood gases (tests for

oxygen, carbon dioxide and

acid in arterial blood)--often

show lowered amounts of

 oxygen and increased

carbon dioxide.

 Echocardiography (EKG)--

sometimes used to rule out

heart problems that might

cause symptoms similar to

RDS. An electrocardiogram

is a test that records the

electrical activity of the

heart, shows abnormal

rhythms (arrhythmias or

dysrhythmias), and detects

heart muscle damage.

Prevention of RDS

Preventing a preterm birth is

the primary means of

preventing RDS. When a

preterm birth cannot be

prevented, giving the mother

medications called
corticosteroids before

delivery has been shown to

dramatically lower the risk

and severity of RDS in the

baby. These steroids are

often given to women

between 24 and 34 weeks

gestation who are at risk of

early delivery.
Respiratory distress
syndrome (RDS) is one of
the most common causes of
morbidity in preterm
neonates,
although lack of a precise
definition in infants with very
low birth weight necessitates
cautious interpretation of
statistics regarding
incidence, mortality, and
outcome [1].
There are many causes of
acute RDS in the preterm
newborn; the most common
cause, however, is RDS,
also
known as hyaline membrane
disease (HMD) [2].
Histologically, hyaline
membranes occur lining the
terminal airways. This gives
the condition its alternative
name – HMD – which should
only be used in the presence
of histological confirmation.
Other clinical scenarios such
as infection
(pneumonia)/inflammation or
aspiration syndromes
may cause an acute
respiratory distress, with or
without hyaline membranes
formation [2].
We have analyzed the lung
necropsy findings and
placenta histology of
deceased newborns whose
diagnosis
of HMD was made on a
combination of clinical and
radiographic features, to
assess the extent of
agreement
between clinics and
pathological findings.
diagnosed with a HMD
on a combination of clinical
and radiographic features
according to the criteria of
RDS of the Vermont Oxford
Network (VON), once our
unit is a collaborative
member.
The VON criteria of RDS are:
(1) PaO2550 mmHg in
room air, central cyanosis in
room air, a requirement for
supplemental oxygen to
maintain PaO2450 mmHg, or
a
requirement for
supplemental oxygen to
maintain a
pulse oximeter saturation
over 85% within the first 24 h
of life and; (2) a chest
radiograph consistent with
RDS
(reticulogranular appearance
to lung fields with or without
low lung volumes and air
bronchograms) within the
first
24 h of life. We use, at our
unit, for practical purposes,
the classification from I to III
(I – light; II – moderate;
III – severe), according to the
X-ray appearance ranging
from a light reticulogranular
with air bronchograms to
white lungs, adapted from
the classification of
Couchard
et al. [3]. HMD is diagnosed
in the presence of
eosinophilic
membranes (hyaline
membranes) lining the visible
airways,
that normally constitute
terminal bronchioles and
alveolar
ducts [2]. Meconium is a
granular eosinophilic
material
with nonnucleated scales. In
this study, meconium was
diagnosed by the presence
of pigment alone, no special
stains were used.
Pneumonia was diagnosed
in the basis of
an inflammatory reaction
with polymorphonuclear
leukocytes
in the in airspaces and lung
interstitial tissue [2].
Bacteria were infrequently
seen in this study. Cultures
for
bacteria were negative,
probably because antibiotics
had
been administered mothers
and newborns. RDSis an
acute illness, usually of
preterm infants, and the
classic clinical presentation
is characterized by a
respiratory
rate over 60/min, dyspnoea
(intercostals, subcostal
indrawing,
sternal retraction, nasal
flaring, cyanosis) with a
predominantly diaphragmatic
breathing pattern and a
characteristic expiratory
grunt or moan, all presenting
within
4–6 h of delivery [8]. Oxygen
administration is required to
prevent cyanosis, and there
is a reticulogranular chest X-
ray
appearance as a result of
widespread atelectasis. The
condition is characterized by
noncompliant (stiff) lungs,
which contain less surfactant
than normal and become
atelectatic at end-expiration.
The characteristic picture is
modified in many infants as a
result of the early
administration
of exogenous surfactant and
immediate assisted
ventilation [9]. The diagnosis
can be established
pathologically
or by biochemical
documentation of surfactant
deficiency, nonetheless,
most series refer only to a
combination
of clinical and radiographic
features [9].
The lungs of infants who
succumb from RDS have a
characteristic uniformly
ruddy and airless
appearance,
macroscopically resembling
hepatic tissue.
Microscopically,
atelectactic alveoli are poorly
developed. When the infant
dies early in
the course of the disease,
necrotic cellular debris is
present
in the terminal bronchioles
and alveolar ducts. Later the
necrotic material becomes
incorporated within
eosinophilic
hyaline membranes lining
the respiratory bronchioles,
alveolar ducts, and random
alveoli, mostly the proximal
alveoli. The membranes are
largely made up of fibrinogen
and fibrin admixed with cell
debris derived chiefly from
necrotic type II
pneumocytes. There is a
remarkable
paucity of neutrophilic
inflammatory reaction
associated
with these membranes. The
lesions of HMD are never
seen
in stillborn infants or in
newborns who die within a
few
hours of birth. The recovery
phase is characterized by
regeneration of alveolar
cells, including type II cells,
with a
resultant increase in
surfactant activity [2].
In this study, we reviewed
the necropsy studies of the
deceased neonates with the
diagnosis of HMD, whose
parents consented to
autopsy. We focused our
attention
mainly in lung tissue findings
and it was surprising that
only 40% of the clinical
HMDs were confirmed at the
necropsy study. The other
60% of diagnosed HMD
were
associated to, or were
congenital pneumonias,
meconium
aspiration, or pulmonary
hemorrhage.