PEDIATRIC CHEST
Congenital Cystic Adenomatoid Malformation
(CCAM)
Background: CCAM is a developmental hamartomatous
abnormality of the lung with adenomatoid proliferation of cysts
resembling bronchioles. CCAM represents approximately 25%
of all congenital lung lesions.
Pathophysiology:
CAM is believed to result from focal arrest in fetal lung
development before the seventh week of gestation secondary to
a variety of pulmonary insults. Depending on the time and type
of insult, 4-26% of cases can be associated with other
congenital abnormalities. However, arrest of pulmonary
development with distortion of architectural differentiation
may take place at any stage of embryonic development.
CAM differs from normal lung tissue because of a combination
of increased cell proliferation and decreased apoptosis. A well-
defined intrapulmonary bronchial system is lacking, and
normally formed bronchi supplying the mass are absent.
RADIOGRAPH
• Usually, the radiographic pattern appears as an
expansile soft-tissue mass containing multiple air-filled
cystic masses of varying size and shifting of the
mediastinum.
• Initially and early in life, a homogeneous fluid-opacity
pulmonary mass may present and evolve to demonstrate
an air-filled cystic radiographic appearance over time.
The initial dense appearance is a result of delayed
emptying of alveolar fluid either via the bronchi or
lymphatic and circulatory systems.
• In patients with CAM, the pattern in the lung
demonstrates multiple radiolucent areas that vary
greatly in size and shape.
• Cysts are separated from each other by strands of
opaque pulmonary tissue.
• The involved lung may appear honeycombed or spongy,
but occasionally, 1 large cyst may overshadow the
others.
• Airtrapping within cystic spaces can cause rapid
enlargement of the CAM and subsequent respiratory
embarrassment.
• Findings are usually apparent in a symptomatic
individual, but they may not be as apparent in an
asymptomatic child.
Congenital Diaphragmatic Hernia (CDH)
Background:
(CDH) constitutes a major surgical emergency in the newborn,
and the key to survival lies in prompt diagnosis and treatment.
Symptoms depend on the degree of herniation; small hernias
may initially pass unnoticed, whereas larger ones produce
immediate and severe respiratory distress.
Classification of CDH
The left-sided Bochdalek hernia is seen in approximately 90%
of cases. The major problem in a Bochdalek hernia is the
posterolateral defect of the diaphragm, which results in either
the failure of the pleuroperitoneal folds to develop or the
improper or absent migration of the diaphragmatic
musculature. Bilateral Bochdalek hernias are rare.
The Morgagni hernia is a less-common CDH, occurring in only
5-10% of cases of CDH. This hernia occurs in the anterior
midline through the sternocostal hiatus of the diaphragm, with
90% of cases occurring on the right side.
A congenital hiatus hernia is very rare in neonates. In this form,
hernia of stomach occurs through the esophageal hiatus
Radiologic features
The classic radiographic appearance is one which the
left hemithorax is filled with cystlike structures (loops of
bowel), the mediastinum is shifted to the right, and the
abdomen is relatively devoid of gas (Swischuk, 1997). In some
cases, a few loops of intestine can be seen in the abdomen, but
more often, only the stomach remains visible within the
abdomen.
Hyaline Membrane Disease
Background:Respiratory distress syndrome (RDS), also
known as hyaline membrane disease (HMD), is an acute lung
disease of the newborn caused by surfactant deficiency. It is
seen primarily in neonates younger than 36-38 weeks'
gestational age and weighing less than 2500 g. In comparison,
HMD tends to occur in neonates younger than 32 weeks'
gestational age and weighing less than 1200 g.
The incidence and severity of RDS is inversely related to
gestational age. RDS is the most common cause of respiratory
failure during the first days after birth. In addition to
prematurity, other factors contributing to the development of
RDS are maternal diabetes, cesarean delivery without
preceding labor, fetal asphyxia, and being the second born of
twins.
Pathophysiology: RDS is the result of anatomic pulmonary
immaturity and a deficiency of surfactant. Pulmonary
surfactant synthesis, in type II pneumocytes, begins at 24-28
weeks of gestation, and gradually increases until full gestation.
Pulmonary surfactant decreases surface tension in the alveolus
during expiration, allowing the alveolus to remain partly
expanded, thereby maintaining a functional residual capacity.
In premature infants, an absence of surfactant results in poor
pulmonary compliance, atelectasis, decreased gas exchange,
and severe hypoxia and acidosis. Premature infants must
expend a great deal of effort to expand their lungs with each
breath, and respiratory failure ensues.
RADIOGRAPH
Classic findings
In RDS, the classic chest radiographic findings consist of
pronounced hypoaeration, bilateral diffuse reticulogranular
opacities in the pulmonary parenchyma, and peripherally
extending air bronchograms. The reticulogranularity is due to
superimposition of multiple acinar nodules caused by
atelectatic alveoli. The development of air bronchograms
depends on the coalescence of areas of acinar atelectasis
around aerated bronchi and bronchioles. In nonintubated
infants, cephalic doming of the diaphragms and hypoexpansion
are observed.
Bronchopulmonary Dysplasia
Background: Bronchopulmonary dysplasia (BPD) is a chronic
pulmonary disorder that results from the use of high positive-
pressure mechanical ventilation and high concentration oxygen
in neonates with respiratory distress syndrome (RDS). It is
defined as oxygen dependence at 28 days. BPD is
pathologically characterized by inflammation, mucosal
necrosis, fibrosis, and smooth muscle hypertrophy of the
airways. With advances in medical management, BPD has
become the most common cause of chronic lung disease (CLD)
in children.
The following factors play a role in the development of BPD:
• Preterm delivery (immature lungs): The disease is
common in children delivered before 32 weeks'
gestation and in those weighing less than 1000 g.
• High oxygen concentration (free radical–induced lung
damage worsened by deficient antioxidants): A high
oxygen concentration is an etiologic factor in patients
with immature lungs, and any concentration greater
than 60% is associated with a high incidence of the
disease.
• Mechanical ventilation (large tidal volume and reduced
lung compliance)
• RDS that requires mechanical ventilation: Sustained
positive-pressure ventilation in preterm infants with
RDS results in dilatation of the terminal bronchioles,
which causes ischemic necrosis of the distal airways.
Resultant pulmonary interstitial emphysema (PIE) and
pneumothorax produce chronic lung damage. Although
mechanical ventilation in RDS may be the original
cause, it also occurs in patients with diaphragmatic
hernia, persistent pulmonary hypertension of the
newborn, meconium aspiration, and other diseases that
require prolonged mechanical ventilation. RDS is not
an absolute requirement for the development of BPD
because the disease can occur in those receiving
mechanical ventilation to manage other diseases.
 • Infectious agents (eg, Ureaplasma urealyticum): U
urealyticum is the most common infectious agent
responsible for BPD, producing early and severe
changes of BPD within 3 weeks. Other bacterial and
fungal agents are also implicated.
Findings: Radiography is the mainstay imaging test for the
diagnosis of BPD.
Stages of radiographic changes
Four stages of radiographic changes of BPD have been
described: stage I, which is RDS seen in the first week; stage
II, which includes generalized haziness and plethora in the
second week; stage III, which involves cystic changes and
stranding in the third week; and stage IV, which is
characterized by hyperinflation, extensive stranding, and an
enlarged heart in the fourth week.
Pulmonary Interstitial Emphysema
Background: Pulmonary interstitial emphysema (PIE) is an
iatrogenic pulmonary condition of the premature infant with
immature lungs. PIE occurs almost exclusively with
mechanical ventilation. The ventilatory pressure used to keep
the alveolar ducts open also may cause rupture of the alveolar
duct (usually at the junction of the bronchiole and alveolar
duct) and consequent escape of air into the pulmonary
interstitium, lymphatics, and venous circulation.
Pathophysiology: Immature lungs are underdeveloped and lack
adequate surfactant to keep the alveolar ducts and early alveoli
open on inspiration and expiration. The resulting diminished
surface for transfer of gas and widespread atelectasis leads to
inadequate transfer of carbon dioxide and oxygen across the
epithelial surfaces to the pulmonary microvascular system.
Methods to improve oxygen saturation include administering
high concentrations of oxygen and expanding and maintaining
that expansion of gas-exchanging surfaces of the lung using
high levels of inhaled oxygen and positive pressure ventilation.
Excessive intra-airway pressure may lead to leak from the
alveolar ducts (primordial acini) into the lung interstitium.
Once in the interstitium, the gas is picked up in the rich
lymphatic network of the neonate and carried toward the
pleural lymphatics and central bronchopleural lymphatics. PIE
usually occurs early during ventilation, and most infants
present in the first 72 hours with this abnormality.
It is observed less frequently now because of treatment of
immature babies with exogenous surfactant, which improves
compliance of the lung (thus less ventilatory pressure is
needed) and keeps the alveolar ducts open during both
inspiration and expiration. It also assists in the recruitment of
alveolar ducts to prevent areas of overinflation and
underinflation. Currently, PIE is seen more often in infants on
long-term ventilator therapy with uneven aeration and
bronchopulmonary dysplasia (BPD) leading to air trapping and
potential airspace rupture.
Findings: Radiography indicates linear, oval, and occasional
spherical cystic air-containing spaces throughout the lung
parenchyma. The interstitial changes are initially linear but
may become more cystic as the air in the interstitium
congregates locally. Subpleural cysts also develop and may
rupture to produce a pneumothorax. The heart tends to get
smaller as intrathoracic pressure increases and results in
diminished venous return into the chest. Overall lung volume is
increased; however, the lungs are less compliant because they
are splinted at a large volume by the air within the interstitium.
Gas exchange is reduced by the increase in distance between
the pulmonary vascular bed and the airspaces.
Transient Tachypnea of the Newborn
Background: Transient tachypnea of the newborn appears
soon after birth. It may be accompanied by chest retractions, by
expiratory grunting, or by cyanosis. (This last manifestation
can be relieved with minimal oxygen.) Recovery usually is
complete within 3 days.
Radiologically, this syndrome frequently is termed wet lung
disease. In the medical literature, discussions concerning
transient tachypnea of the newborn also can be found under the
following names: retained fetal lung liquid, retention of fetal
lung fluid, respiratory distress syndrome type II, transient
respiratory distress of the newborn, and neonatal retained fluid
syndrome.
Pathophysiology: During fetal life, the lungs are expanded
with an ultrafiltrate of the fetal serum. In the course of neonatal
transition, this ultrafiltrate must be removed and replaced with
air. The classic explanation for how this occurs was that
passage through the birth canal would, by squeezing the thorax,
help eliminate the liquid in the lungs, with the remaining fluid
being removed by pulmonary capillaries and the lymphatics.
Currently, however, the bulk of this clearance is thought to be
mediated by transepithelial sodium reabsorption through
sodium channels in the alveolar epithelial cells, with only a
limited contribution from mechanical factors and Starling
forces. Changes in the hormonal milieu of the fetus and its
mother, brought about mainly by the onset of spontaneous
labor, prepare the fetus for the neonatal transition to air
breathing.
Transient tachypnea of the newborn occurs when the liquid in
the lung is removed slowly or incompletely; this phenomenon
correlates with a decreased thoracic birth squeeze or
diminished respiratory effort in the newborn. Transient
tachypnea has been identified as occurring with cesarean birth
and infant sedation. Longer labor intervals, macrosomia of the
fetus, and maternal asthma also have been associated with a
higher frequency of transient tachypnea of the newborn.
Findings: Findings on chest radiographs may include mild,
symmetrical lung overaeration; prominent perihilar interstitial
markings; and small pleural effusions. Occasionally, the right
side may appear more opacified than the left.
Radiographic appearance at times can mimic the diffuse,
granular appearance of hyaline membrane disease but without
pulmonary underaeration. Neonates with transient tachypnea
usually are at term. Radiographic lung changes also may
resemble the coarse, interstitial pattern of other causes of
pulmonary edema or the irregular pattern of lung opacification
seen in meconium aspiration syndrome.
Meconium Aspiration
Background: The term meconium is derived from ancient
Greek word meconium-arion, or opium-like, from the Greek
word mekoni meaning poppy juice. In the time of Aristotle, the
term was used because it was believed that the substance
induced fetal sleep.
Pathophysiology: Passage of meconium into amniotic fluid
most often represents a normal maturational event. However, in
many instances, it may occur in response to fetal hypoxia or
acidosis. Meconium passage likely requires neural stimulation
of a mature gastrointestinal tract, without which there is no
peristalsis and relaxation of the rectal sphincter. This may
explain why meconium is rarely found in the amniotic fluid
before 34 weeks' gestation. Meconium aspiration syndrome
chiefly affects infants at term and afterward. The amniotic fluid
and meconium mix to form a greenish-black fluid of variable
thickness, or viscosity.
Meconium aspiration syndrome occurs when the newborn
aspirates the meconium-containing amniotic fluid. In addition
to obstruction of the airway, the aspiration leads to an
inflammatory response in the lung parenchyma (chemical
pneumonitis). It is this inflammation, not the meconium itself,
that results in the patchy infiltrates seen on chest radiography.
It is not clear which component(or components) of meconium
triggers the inflammatory response. However, bile and liver
enzymes have been suggested as the causative agents.
Chest radiography typically shows hyperinflation with patchy
opacities. These findings represent areas of atelectasis mixed
with areas of air trapping. As mentioned above, air leaks are
common, leading to pneumothorax, pneumomediastinum,
pneumopericardium, and/or pulmonary interstitial emphysema.
Pleural effusions may be present.
Epiglottitis
Background: Epiglottitis is the inflammation of the supraglottic
structures. In most cases, the condition is caused by a rapidly
developing bacterial infection of the epiglottis, which has the
potential to cause abrupt airway obstruction.
Traditionally, this infection was most commonly caused by
Haemophilus influenzae type b (HIB), though Streptococcus
pneumoniae, Staphylococcus aureus, and group A beta-
hemolytic streptococci were occasionally found.
Sex: Epiglottitis is more common in males than females, with a
male-to-female ratio of about 3:1.
Age: Before widespread use of the HIB vaccine, epiglottitis
occurred mainly in young children, peaking between 2 and 7
years of age. The disease is now rarely seen among children.
RADIOGRAPH
Findings: On plain radiographs, the normal epiglottis is a thin,
curved, flap of soft tissue opacity that is separated from the
base of the tongue by air in the valleculae
In epiglottitis, images show diffuse soft-tissue swelling with
enlargement of the epiglottis and also of the normally thin
aryepiglottic folds. One should look for an enlarged epiglottis
(thumbprint sign), thickened aryepiglottic folds, and ballooning
of the hypopharynx, usually with normal subglottic structures,
although a rare case of supraglottitis may cause infraglottic
swelling
Croup
Background: Croup is a generic term encompassing a
heterogeneous group of relatively acute conditions (mostly
infectious) characterized by a syndrome of distinctive brassy
coughs. These may be accompanied by inspiratory stridor,
hoarseness, and signs of respiratory distress resulting from
laryngeal obstruction. The word croup derives from an old
Scottish term roup, which means "to cry out in a shrill voice."
The most common form of croup is acute
laryngotracheobronchitis or viral croup, an infection of both
the upper and lower respiratory tracts. A reactive inflammatory
response causes subglottic edema.
Age: Viral croup is most common in patients aged 6 months to
5 years, with a peak incidence in the second year of life. Croup
is rare in the first 6 months of life. Stridor presenting in the first
6 months of life should initiate a search for other causes of
stridor. Congenital anomalies and subglottic hemangiomas are
other conditions to consider that narrow the airway and cause
stridor in infants. The youngest reported child with croup was
aged 3 months.
RADIOGRAPH
• Frontal neck radiograph: The lateral walls of the
subglottic larynx normally are convex or shouldered.
Wall edema in croup narrows this space with loss of
lateral convexity, creating a steeple shape below the
vocal cords. The narrowing may extend for 5-10 mm
below the vocal cords.
• Lateral neck radiograph: The hypopharynx is
overdistended during inspiration, and the subglottic
region is hazy as a result of narrowing of the airway by
mucosal edema. The larynx airway is indistinct. The
undersurface of the vocal cords normally identified
during phonation is not well identified. The epiglottis,
aryepiglottic folds, and prevertebral spaces appear
normal.