HYALINE MEMBRANE
DISEASE
RESPIRATORY DISTRESS
SYNDROME
Prof. Dr. Mohammed A. Younis
Tikrit University
College of Medicine
Pediatrics Department
Objectives
1.
To understand the risk factors, pathogenesis ,
pathology,and clinical features of respiratory distress
syndrome
2.
To list the differential diagnosis of respiratory distress in
newborn baby
3.
To recognise how to to investigate and manage a
newborn baby with respiratory distress syndrome
4.
To understand the features and management of patent
ducutus arteriosus
5.
To recognise the pathogenesis and clinical features of
bronchopulmonary dysplasia and retinopathy of
prematurity
Respiratory distress in the newborn is defined
by the presence of one or more of the
following:
tachypnea, retractions, nasal flaring, grunting,
and cyanosis.
INCIDENCE
HMD occurs primarily in premature infants,
and its incidence inversely proportional to the
gestational age and birth weight.
60-80% of infants less than 28wk of gestation
15-30% of infants between 32&36 wk
in about 5% beyond 37 wk, and rarely at term
The risk of developing RDS
increases with
1. Prematurity
2. maternal diabetes
3. Multifetal pregnancy
4. cesarean section delivery
5. precipitous delivery
6. asphyxia
7. cold stress
8. history of previously affected infants.
The incidence is highest in preterm male or white
infants.
The risk of RDS is reduced in
1. pregnancies with chronic or pregnancy-
associated hypertension
2. maternal heroin use
3. prolonged rupture of membranes
4. antenatal corticosteroid prophylaxis
ETIOLOGY
&PATHOPHISOLOGY
Surfactant deficiency [decreased
production&secretion] is the primary cause
of HMD.
Surfactant Is phospholipid protein ,its major
constituents:
1-Dipalmitoyl phostidylcholine [Lecithine]
2-Phosphatidyle glycerol
3-Apoproteins
4-Cholesterol
Surfactants are synthesized and stored in
type2 alveolar cells.
Deficiency of surfactant leads
to:
-alveolar collapse
-decreased lung volume &compliance
-ventilation-perfusion abnormalities
-right to left shunt
-persistent hypoxemia[<30mm Hg]causes
metabolic acidosis
-respiratory acidosis also present because
alveolar hypoventilation
Decreased myocardial contractility, decreased
cardiac out put&arterial blood pressure
-Perfusion of kidneys,GIT,muscle,&skin is reduced
leading to edema & electrolytes disorders.
PATHOLOGY
The lungs appear deep purplish red,&liver like in
consistency.
Microscopically:
A number of alveolar ducts, alveoli,& resp.
bronchiole are lined with acidophilic homogenous,
or granular membrane.
Clinical manifestations
Signs of HMD usually appear within minutes
of birth, but may be delayed for several hours
in large premature infants.
Early clinical signs of HMD:
1-Tachypnea[>60/min]
2-Expiratory grunting
3-Sternal&intercostal recession
4-Cyanosis in room air
5-Delayed onset of respiration in very
immature babies
Late clinical signs in severe HMD
1-Decrease blood pressure
2-Fatigue
3-Cyanosis
4-Pallor increase
5-Grunting decrease or disappears
6-Apnea& irregular respiration[ominous sign]
Other signs:
-Mixed resp.& metabolic acidosis
-Edema,ileus,oliguria
In most cases symptoms&signs reach peak within 3 days
after which improvement is gradual.
Cyanosis. This critically ill infant exhibits cyanosis and poor skin perfusion.
Flaring. Reflexive widening of the nares may be seen
in infants with respiratory distress.
Retractions. The inward collapse of the lower anterior chest wall
can be seen in this premature infant with RDS.
INVESTIGATIONS
Chest x.ray
-Grade-1-fine reticular granular mottling, good lung
expansion
-Grade-2-mottling with air bronchogram
-Grade-3-diffuse mottling,heart border just
discernable,prominent air bronchogram
-Grade-4-bilateral confluent opacification of
lungs[white out]
BD gas analysis
1-Initially hypoxemia
2-Later progressive hypoxemia ,hypercapnia,
&metabolic acidosis
RDS. Note the ground-glass appearance and the presence
of air bronchograms
Differential diagnosis of RDS
1-congenital pneumonia
2-aspiration pneumonia
3-meconium aspiration syndrome
4-air leak [pneumothorax, pulmonary
interstitial emphesema, pneumomediastinum]
5-transient tachypnea of newborn
6-lobar emphesema
7-pulmonary hypoplasia
8-diaphragmatic hernia
9-heart failure
10-persistent pulmonary hypertension
11-asphyxia&increased intracranial pressure
12-metabolic acidosis
13-congenital neuromuscular disorder
14-anemia&hypovolemia
Initial Laboratory Evaluation of
Respiratory Distress
1. Chest radiograph
2. Arterial blood gas
3. Complete blood count
4.
Blood culture
5. Blood glucose
6. Echocardiogram, ECG
Prevention
1-Prevention of prematurity, including :
-avoidance of unnecessary or poorly timed c.s
-appropriate management of high risk
pregnancy& labour.
-Estimation of fetal head circumferance by
ultrasound& determination of lecithin
concentration in the amniotic fluid by
[L/S]ratio decrease likehood of delivering
premature infants
2-Adminstration of betamethasone to women
48hr before delivery of fetuses between 24-
34wk of gestation significantly reduce the
incidence&mortality&morbidity of HMD.One
course of corticosteroid required.
3-Adminstration of first dose of surfactant in
to the trachea of symptomatic premature
infants immediately after birth[prophylactic]
reduce air leak&mortality from HMD
Treatment
Maintenance of temperature:
Preterm infants should be nursed in incubator or
under radiant heat warmer [maintain core temp
36.5-37° c].
Calories &fluid:
Provided by intravenous fluid.
Excessive fluid contribute to development of
PDA,NEC&BPD.
Maintenance of normoxemia:
The aim is to keep arterial oxygen tension in
range of[55-75mm Hg].
For babies with spontaneous respiration
humidified oxygen should be given.
Too little oxygen will cause hypoxemia,
metabolic acidosis,&tissue damage.
Too much oxygen associated with
development of retinopathy of prematurity.
Assisted ventilation
1-CPAP: is distending pressure which prevent
alveolar collapse during expiration& thus improving
oxygenation.
2-Mechanical ventilation:
indication for I.P.P.V
1-failure to establish respiration at birth
2-intractable apneic attacks
3-respiratory failure [ph<7.2,paco2>66mm Hg
,pao2<53mm Hg in 90%O2
3-High frequency ventilation
Surfactant therapy:
Synthetic &natural surfactants[from calf,pig,&cow
lungs].Multidose endotracheal instillation of
surfactant
Metabolic acidosis:
in RDS may be a result from perinatal asphyxia
&hypotension.
The aim to keep pH above 7.25.
It is treated by sodium bicarbonate 1-2meq/kg
administered over 15-20min through peripheral or
umbilical vein.
Complications of HMD
1-Patent ductus arteroisus
2-Interventricular hemorrhage
3-pulmonary:
A-air leak:
pneumothorax,pneumomediastinum,
P.I.E,pneumopericardium,pneumoperitonium,air
embolism, subcutanous emphesema.
B-bronchopulmonary dysplasia.
C-pneumonia: aspiration,bacterial.
4-Complication of mechanical ventilation.
5-Long term neurological sequele.
Patent Ductus Arteriosus
The ductus arteriosus constrict after birth in normal term infants
in response to elevated PaO2 level.
The ductus in preterm infant is less responsive to
vasoconstrictive stimuli due to persistant vasodilator effect of
PGE2 in addition to hypoxemia during RDS leads to persistent
PDA that creat shunt between the pulmonary&systemic
circulation.
Clinical features:
When RDS improves&pulmonary vascular resistance declines
&flow through ductus increases in a left to right direction.
It may produce no symptoms or it may cause apnoea and
bradycardia, increased oxygen requirement and difficulty in
weaning the infant from artificial ventilation.
Pulse pressure widens,active precordial impulse.
Active&bounding peripheral pulse. The murmur of PDA may
be continous or usually systolic. Heart failure&pulmonary
edema result in rales & hepatomegally.
Chest x-ray:
cardiomegally &pulmonary edema.
Treatment:
during RDS involves an initial period of
fluid restriction& diuretics.If no improvement after 24-48 hr
indomethacin {prostaglandin synthetase inhibitor} 0.2mg/kg
I.V every 12 hr, 3 doses. If the patient not respond to repeated
courses of indomethacine & in heart failure surgical ligation
is required.
BRNCHOPULMONARY
DYSPLASIA
Oxygen concentration above 40% are toxic to the
neonatal lung.
Oxygen mediated lung injury results from generation
of super oxides, hydrogen
peroxides[H2O2],&Oxygen free radicals which
disrupt membrane lipids. Mechanical ventilation with
high peak pressure produces barotroma.
Definition: Failure of RDS to improve after 2
weeks& need for prolonged mechanical
ventilation,&oxygen therapy required at 36 weeks
post conception age.
Clinical feature:
Oxygen dependence, hypercapnia , compensatory
metabolic alkalosis,pulmonary hypertension, poor
growth,& development of right sided heart
failure.Increase air way resistance with reactive air
way constriction.
Treatment:
1-Bronchodilator
2-Fluid restriction& diuretics
3-Mechanical ventilation
4-Dexamethazone
Bronchopulmonary dysplasia. Note the alternating areas of
hyperinflation and atelectasis.
Retinopathy of prematurity
[ROP]Retrolental fibroplasia
It is caused by acute and chronic effects of oxygen
toxicity on the developing blood vessels of
premature retina.
The completely vascularized retina of term infant is
not susceptible to ROP.
ROP is a leading cause of blindness for VLBW
infant[<15oogm].
Excessive arterial oxygen tensions produce
vasoconstriction of retinal vessels this followed by
vaso obliteration,then proliferative
stages[extraretinal fibrovascular proliferation].
Severe cases leads to retinal detachment, leukokoria,
glucoma
The incidence of ROP may be reduced by careful
monitoring of arterial blood gases& to keep arterial
PaO250-70mm Hg.
Infant <1500gm, or born before 28 weeks gestation
should be screened when they are older than 7 weeks
old.
Laser therapy &less often cryotherapy may be used
for viterous hemorrhage& for severe progressive
proliferation.
Surgery indicated for retinal detachment.
Retrolental fibroplasia with temporal tugging of the disc