Residents Section Pat tern of the Month

Wasilewska et al.
Unilateral Hyperlucent Lung in Children

Residents Section
Pattern of the Month
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Residents

Unilateral Hyperlucent
inRadiology Lung in Children
Ewa Wasilewska1

I
maging evaluation plays an important role in making an accurate diagnosis
Edward Y. Lee1 among the various causes of unilateral hyperlucent lung in pediatric patients
Ronald L. Eisenberg2 based on their characteristic imaging appearances. The causes of unilateral hy-
perlucent lung can be categorized into five general groups (Table 1) on the basis
Wasilewska E, Lee EY, Eisenberg RL of whether the abnormality primarily involves the chest wall, lung parenchyma, pulmonary
vessels, central airways, or mediastinum. Using this systematic diagnostic approach can lead
to an early and accurate diagnosis that permits optimal patient management.

Chest Wall Abnormality

Keywords: children, lung, pediatric
DOI:10.2214/AJR.11.7028
Received April 4, 2011; accepted after revision
September 5, 2011.
Poland Syndrome
Poland syndrome is a nonhereditary condition characterized by congenital unilateral aplasia
of the pectoralis muscles. Other anomalies associated with Poland syndrome, which occur on the
ipsilateral side and have variable expression, include absence of the costosternal portion of the
pectoralis major muscle, absence of the pectoralis minor muscle, hypoplasia of the latissimus
dorsi and deltoid muscles, dextrocardia (in
left-sided Poland syndrome), aplasia or defor- TABLE 1: Unilateral Hyperlucent
mity of the costal cartilages of the second Lung in Children
through fifth ribs, hypoplasia of the subcutane-
Chest wall abnormality
ous tissues, hypoplasia or absence of the breast
and nipple, alopecia of the axillary and mam- Poland syndrome
mary region, and brachysyndactyly. Lung parenchymal abnormality
The precise cause of Poland syndrome is Bronchial atresia
uncertain, but the most widely accepted the-
Congenital lobar emphysema
ory relates to disruption of the blood supply
Tin the subclavian artery distribution during Congenital pulmonary airway malformation
chest wall development in utero. Poland syn- Swyer-James syndrome
drome may be sporadic or familial. The Atelectasis and lung collapse
more frequent sporadic type shows male and
Pneumothorax
right-side predominance. The majority of pe-
diatric patients with Poland syndrome are Postpneumonectomy

1
Department of Radiology, Childrens Hospital Boston,
Harvard Medical School, Boston, MA.
2
Department of Radiology, Beth Israel Deaconess
Medical Center, Harvard Medical School, 330 Brookline
Ave, Boston, MA 02215. Address correspondence to
R.L.Eisenberg (rleisenb@bidmc.harvard.edu).
WEB
This is a Web exclusive article.
AJR 2012; 198:W400W414
0361803X/12/1985W400
American Roentgen Ray Society
asymptomatic. However, affected patients Pulmonary vasculature abnormality
may present with respiratory distress, chest Pulmonary agenesis
pain, or limited range of shoulder movement
Proximal interruption of the pulmonary artery
depending on the degree of the underlying
cardiopulmonary impairment or functional Pulmonary artery sling
deficiency of the shoulder. Scimitar syndrome
The characteristic radiographic findings of Central airway abnormality
Poland syndrome include unilateral hyperlu-
Foreign body aspiration
cency of the lung related to the lack of soft
tissues of the chest wall (Fig. 1). Cross-sec- Endobronchial tumor
tional imaging studies, such as CT or MRI, Mediastinal abnormality
can confirm the diagnosis of Poland syn- Foregut malformation
drome by showing unilateral aplasia of the
Lymphadenopathy
pectoralis muscles. On CT, the pulmonary

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Unilateral Hyperlucent Lung in Children

Fig. 1Poland syndrome. Frontal chest radiograph

obtained for evaluation of pneumonia in 13-year-old
boy with cough and fever shows hyperlucent right
hemithorax due to aplasia of pectoralis muscles.
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parenchyma is normal unless there is lung herniation related to the chest wall deformity and
rib defects. The current imaging modality of choice in pediatric patients with Poland syndrome
is MRI because of the lack of associated ionizing radiation exposure.

Lung Parenchymal Abnormality

Bronchial Atresia
Bronchial atresia is a congenital anomaly that results from focal obliteration of a proximal
segmental or subsegmental bronchus without a communication with the central airways. The
development of the airways distal to the atretic segment is normal. However, the loss of com-
munication with the central airway and preserved ventilation via collateral pathways (i.e.,
Kohn pores in alveolar septa or anastomosing bronchioles) lead to air trapping and hyperin-
flation. The bronchus distal to the area of atresia becomes filled with secretions and forms a
bronchocele. The upper lobe bronchi are most frequently affected. Most pediatric cases of
bronchial atresia are congenital, but the condition may be acquired secondary to traumatic or
infectious injury to the bronchus. Approximately 50% of affected patients are asymptomatic,
with the diagnosis usually made incidentally on imaging studies (chest radiography, CT)
performed for other reasons. The remaining patients usually present with nonspecific symp-
toms, such as dyspnea or pneumonia.
The characteristic radiographic appearance of bronchial atresia is a round or oval branching
opacity radiating from the hilum (representing a bronchocele) with an associated area of hyper-
lucency in the adjacent lung parenchyma (Fig. 2A). The bronchial atresia may contain air-fluid
levels within the dilated bronchus. CT is the current imaging modality of choice for confirming
bronchial atresia suspected on chest radiographs because it allows excellent visualization of the

A B
Fig. 2Bronchial atresia in 2-year-old boy with possible abnormal chest radiograph at outside hospital.
A, Frontal chest radiograph shows right upper lung lucency (arrows) with masslike opacity (asterisk) in right
paratracheal region.
B, Coronal CT image shows masslike oval opacity (curved arrow) with surrounding hyperinflation (straight
arrows) of right upper lobe. Pulmonary vessels and airways are attenuated in this region.

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mucoid impaction within the dilated bronchus, associated segmental hypoattenuation, and de-
creased vascularity. On CT, a bronchial atresia appears as an oval or round branching structure
that radiates from the atretic bronchus in the hilum and extends peripherally (Fig. 2B). The lung
surrounding the bronchial atresia often shows air trapping, which is best visualized on expira-
tory phase CT images. CT can also help to exclude a hilar mass as the underlying cause of post-
obstructive changes. MRI is less frequently used to evaluate bronchial atresia in pediatric pa-
tients after birth because it is less available than CT and cannot depict the associated regional
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air trapping. However, the underlying MRI appearance of bronchial atresia is beneficial because
bronchial atresia may be encountered on prenatal MRI. On MRI, bronchial atresia typically
shows high signal intensity on both T1- and T2-weighted images.

Congenital Lobar Emphysema

Congenital lobar emphysema (CLE), also known as infantile lobar emphysema or congenital
lobar hyperinflation, is a congenital lung anomaly that is characterized by progressive hyperin-
flation of a lobe. Although the precise cause is uncertain, the most likely explanation is a check-
valve mechanism within a bronchus that can result from areas of malacia or stenosis of the
bronchial cartilage and lead to overdistention of the lung from diminished flow of air on expira-
tion. There is no destruction of the alveolar wall. Most patients with CLE present with respira-
tory distress and are diagnosed before age 6 months. CLE occurs in classic and polyalveolar
forms. The classic form has a normal or decreased number of overdistended alveoli. In contrast,
the number of alveoli is greater than expected in the recently described polyalveolar form. CLE
most commonly involves the left upper (42%) and right middle (35%) lobes. Right upper lobe
involvement occurs in 21%, and the lower lobes are infrequently affected (< 1% each).
The characteristic radiographic appearance of CLE is asymmetric hyperlucency of the
lobe, which often develops after initial lobar opacity that is related to retention of fetal lung
fluid just after birth (Fig. 3A). The presence of bronchovascular markings in CLE helps dis-
tinguish it from pneumothorax. The overinflated lobe acts as a space-occupying lesion that
produces a mass effect on adjacent structures. CT is an excellent imaging modality to exclude
an underlying hilar mass or intraluminal bronchial process. Additionally, CT can precisely
delineate and localize the lesion, which is particularly helpful for preoperative evaluation. CT
typically shows both the hyperinflated lobe and the attenuated bronchovascular bundle, which
runs at the periphery of the expanded alveoli (Fig. 3B).

Congenital Pulmonary Airway Malformation

Congenital pulmonary airway malformation is a developmental anomaly characterized by
disorganized hamartomatous and adenomatoid proliferation of primary bronchioles that com-
municate with the bronchial tree. The lesion involves only one lobe in approximately 95% of

A B
Fig. 3Congenital lobar emphysema in 2-day-old girl with respiratory distress.
A, Frontal chest radiograph shows hyperinflated and lucent left upper lobe with atelectatic lingula and left
lower lobe (asterisk).
B, Axial CT image shows hyperinflated and lucent left upper lobe with attenuated bronchovascular bundle.
There is mediastinal shift to right due to hyperinflation of left lung.

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A B
Fig. 4Congenital pulmonary airway malformation in 5-day-old boy with respiratory distress.
A, Frontal chest radiograph shows hyperinflated and lucent right hemithorax. Note multiple cystic septations.
B, Axial CT image shows multiple cystic masses (arrows) in right lower lobe.

cases, without any lobar predilection. Congenital pulmonary airway malformation has tradition-
ally been classified into three main categories (Tom Stoker classification). In type 1 (most com-
mon), there is at least one dominant cyst (> 2 cm in diameter). Type 2 lesions are composed of
multiple smaller cysts (< 2 cm in diameter), whereas a type 3 lesion has a solid appearance but is
composed of microcysts that are discernible only on microscopic examination. The recent reclas-
sification of congenital pulmonary airway malformation includes two additional categories: type 0
and type 4. Type 0 develops from acinar dysgenesis or dysplasia of the large airway, such as trachea
or bronchus, involving all lobes. Affected patients with type 0 typically die in utero; therefore, the
imaging appearance of type 0 congenital pulmonary airway malformation is not known. Type 4 is
caused by an abnormality of the distal acinus or alveolar saccular-alveolus region. The imaging
appearance of type 4 is characterized by large air-filled cysts located in a peripheral portion of the
lungs and, often, associated mediastinal shift to the contralateral side. About 85% of patients with
congenital pulmonary airway malformation become symptomatic in the neonatal period, leading to
respiratory compromise or recurrent pulmonary infections. Rarely, congenital pulmonary airway
malformation can be incidentally detected in an asymptomatic older pediatric patient.
The radiographic appearance of congenital pulmonary airway malformation depends on the
number and size of the cystic spaces forming the lesion and whether there is superimposed in-
fection. The most common imaging finding, except in the type 3 lesion, is an area of hyperlu-
cency that is usually confined to one lobe (Fig. 4A). CT can aid in characterizing congenital
pulmonary airway malformation and showing the cystic spaces that differentiate it from other
causes of abnormal hyperlucency of the lungs (Fig. 4B). Type 1 congenital pulmonary airway
malformation typically appears as one or more large air-filled cystic structures. In type 2, there
is an air-filled multicystic mass or focal area of consolidation, whereas a type 3 lesion appears
on imaging as a solid mass because the underlying microscopic cysts (< 2 mm) can be identified
only at histologic evaluation. Internal air-fluid levels, enhancing thick wall, and surrounding
inflammatory changes suggest an infected congenital pulmonary airway malformation.

Swyer-James Syndrome
Swyer-James syndrome is a condition characterized by unilateral hyperlucent lung that usu-
ally develops during childhood as a sequela of postinfectious bronchiolitis obliterans. Swyer-
James syndrome can result from various lower respiratory tract infections, including adenovi-
rus, measles, pertussis, tuberculosis, or mycoplasma. The infection leads to inflammation and
fibrosis of the respiratory bronchiolar walls and the interalveolar septa. Narrowing of bronchio-
lar lumens reduces ventilation and leads to compensatory hypoperfusion of the affected lung.
This process also results in hyperexpansion of the peripheral airway, which contributes to the
hyperlucency seen on imaging studies. Most cases of the postinfectious constrictive bronchiol-
itis become symptomatic in childhood or early adulthood. Affected patients frequently have a
history of recurrent pulmonary infection and present with nonspecific respiratory symptoms,
such as dyspnea on exertion, productive cough, and shortness of breath.
The characteristic radiographic findings of Swyer-James syndrome consist of asymmetric hy-
perlucency of the lung or lobe, small hilar shadow with small pulmonary vessels, bronchial wall
thickening, and bronchiectasis (Fig. 5A). The hyperlucent lung can have preserved or diminished

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A B
Fig. 5Swyer-James syndrome in 11-year-old girl with recurrent pneumonia.
A, Frontal chest radiograph shows hyperlucent left lung and small left hilar shadow with small pulmonary vessels.
B, Axial CT image obtained at end-expiration shows substantial air trapping (arrows) in left lung. Several small
areas of air trapping are also seen in right lung.

size. Air trapping can cause mediastinal shift toward the affected side on inspiration and the con-
tralateral side on expiration. Characteristic CT appearances include lobar hyperlucency due to
underlying air trapping, with decreased size of the pulmonary artery and reduction in the number
of its branches (Fig. 5B). Although involvement of more than one lobe or of both lungs in Swyer-
James syndrome is frequently seen on CT, the lungs are typically asymmetrically affected, result-
ing in more prominent hyperlucency of one lobe or one lung. At times, an inhomogeneous distribu-
tion of findings can produce a mosaic attenuation pattern or involvement that is not confined to one
lung. Radionuclide lung scanning can assist in reaching the correct diagnosis by showing a
matched ventilation-perfusion defect in the lobar distribution.

Atelectasis and Lung Collapse

Atelectasis and lung collapse create a potential space within the thorax. This must be com-
pensated for by a combination of decrease in thorax size, displacement of the mediastinal
structures, elevation of the hemidiaphragm, or compensatory overexpansion of the ventilated
portion of the pulmonary parenchyma. Compensatory overexpansion of the lung can lead to
unilateral lung hyperlucency.
The radiographic appearance of atelectasis or lung collapse depends on which part of the lung
is affected. Right upper lobe atelectasis or collapse causes an upward shift of the minor fissure
and hemidiaphragm. In this condition, the right middle lobe and lower lobes are hyperinflated
and appear hyperlucent. In left upper lobe collapse, a paraaortic crescentic lucency (luftsichel
sign) classically extends from the apex of the left hemithorax to the left superior pulmonary vein.
This represents the hyperinflated superior segment of the left lower lobe interposed between the

Fig. 6Left lung collapse in newborn girl with

worsening respiratory distress after intubation.
Frontal chest radiograph shows hyperlucent right
lung and completely opacified left lung. Endotracheal
tube tip (arrow) is located in right mainstem bronchus,
resulting in preferential hyperventation of right lung
and left lung collapse.

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Fig. 7Right lung collapse in 15-year-old girl with

acute respiratory distress, decreased oxygen
saturation, and no breath sound in right hemithorax
on physical examination. Frontal chest radiograph
shows complete collapse of right lung (arrow) with
markedly hyperlucent right hemithorax due to large
right pneumothorax.
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collapsed left upper lobe and the aortic arch. Associated radiographic findings often related to the
volume loss include elevation of the left hilum and a near horizontal course of the left mainstem
bronchus. An atelectatic right middle lobe causes increased density obliterating the right heart
border, with the apex directed toward the hilum. The collapse of the right middle lobe leads to el-
evation of the ipsilateral major fissure and depression of the minor fissure. There may be associated
hyperinflation of the upper lobe or the contralateral lung and often elevation of the right hemidia-
phragm. Lower lobe atelectasis or collapse produces triangular paraspinal opacity with compensa-
tory overinflation of the upper lung. There is often inferior displacement of the major fissure and
ipsilateral hilum. When the entire right or left lung is atelectatic or collapsed, the contralateral lung
may appear hyperlucent (Fig. 6) and there is mediastinal shift to the ipsilateral side.

Pneumothorax
Pneumothorax occurs when air is introduced into the pleural cavity. In pediatric patients,
cause of pneumothorax is more frequently spontaneous or develops after trauma to the chest.
The majority of pneumothorax presents in neonates, which results from the transthoracic pres-
sure differential occurring with initial lung expansion with air. Affected patients typically pres-
ent with acute chest pain and shortness of breath. Although the diagnosis of pneumothorax may
be made by physical examination in severe cases, it usually requires an imaging study such as a
chest radiograph. Knowledge of the radiographic appearance of pneumothorax is critical, be-
cause it may be fatal unless recognized correctly and treated urgently.
The key to diagnosis is detecting the presence of a pleural line with no pulmonary markings
extending beyond it. When the entire lung is collapsed, a pneumothorax appears as a globular
opacity located near the hilum with associated hyperlucency of the ipsilateral hemithorax (Fig.
7). However, in neonates or infants particularly on supine position, lucency representing anteri-
orly placed pneumothorax typically presents along the mediastinum. A progressive deterioration
and worsening of a simple pneumothorax can result in a life-threatening tension pneumothorax,
which produces mediastinal shift to the contralateral side, widened rib spaces, and a flattened
hemidiaphragm. The treatment of pneumothorax depends on the size of pneumothorax and de-
gree of cardiorespiratory compromise. A small pneumothorax in asymptomatic pediatric patients
may be managed conservatively with serial follow-up chest radiographs for documentation of
interval resolution and may not need aggressive treatment such as needle decompression or chest
tube placement. However, large pneumothorax in symptomatic pediatric patients with tension
component will require immediate needle decompression. Needle decompression of pneumotho-
rax can be performed by inserting an 18- or 20-gauge over-the-needle catheter over the top of the
childs third rib in the midclavicular line. Nonresolving pneumothorax can be managed with a
chest tube placement. The chest tube is typically inserted in an area under the axilla delineated
by a horizontal line at the level of the nipple and two muscles of the chest wall including latis-
simus dorsi and pectoralis major.

Pneumonectomy and Lobectomy

Pneumonectomy or lobectomy often leads to substantial intrathoracic changes requiring
reorientation of the anatomic structures. There is volume loss with mediastinal shift toward

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Fig. 8Right middle lobectomy in 18-month-old

girl after surgical resection of right middle lobe
for bronchial atresia. Frontal chest radiograph
shows hyperlucent right lower lobe (arrows) due to
compensatory hyperinflation of right lower lobe.
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the affected side, displacement of the interlobar fissures, and rotation of the heart. Over time,
the space typically fills with fluid and fibrothorax. Compensatory hyperinflation of the re-
sidual lung may also fill the postpneumonectomy or postlobectomy space, resulting in radio-
graphically apparent unilateral lung hyperlucency. The changes related to volume loss associ-
ated with pneumonectomy or lobectomy can be similar to the findings seen in the lung or
lobar collapse, but evidence of postthoracotomy rib changes and postsurgical suture material
should suggest the correct diagnosis (Fig. 8).

Pulmonary Vasculature Abnormality

Pulmonary Agenesis
Pulmonary agenesis is a congenital underdevelopment of the lung that occurs on either side
with almost equal frequency. It represents a spectrum of lung underdevelopment including
lung agenesis (total absence of the lung, bronchus, and pulmonary artery); lung aplasia (total
absence of the lung and pulmonary artery with a rudimentary main bronchus); and lung hy-
poplasia (hypoplastic bronchus and pulmonary artery and an associated variable amount of
lung tissue). Abnormal blood flow in the dorsal aortic arch during embryonic phase is the
suspected, but unproven, underlying pathogenesis of this anomaly.
Bilateral pulmonary agenesis is incompatible with life. Patients with unilateral lung under-
development typically present in the neonatal period with tachypnea, stridor, or feeding dif-
ficulties. This condition has a high association with other congenital anomalies involving the
skeletal (limb and rib anomalies, vertebral segmentation anomalies), cardiovascular (patent
ductus arteriosus, patent foramen ovale), gastrointestinal (tracheoesophageal fistula, imper-
forate anus), or genitourinary systems. Prognosis and mortality of affected patients usually
depend on the type and degree of coexisting congenital anomalies rather than on the lung
underdevelopment itself. In asymptomatic patients, pulmonary agenesis may be discovered as
an incidental finding during childhood or even in adults.
Asymmetric aeration of the lungs with complete opacification of the affected hemithorax is
the characteristic imaging finding of pulmonary agenesis on a frontal chest radiograph (Fig.
9A). Associated volume loss produces ipsilateral mediastinal shift, elevation of the hemidia-
phragm, and small intercostal spaces. The hyperinflated contralateral lung can extend anteri-
orly into the affected hemithorax and appear as an area of hyperinflation located anterior to the
heart on a lateral chest radiograph (Fig. 9B). However, this finding may be also seen in other
conditions, such as postobstructive lung collapse. Therefore, further evaluation with cross-sec-
tional imaging is often necessary to confirm the diagnosis and further characterize the ana-
tomic structures. CT provides a definitive diagnosis of pulmonary agenesis by showing absence
of the lung parenchyma, bronchus, and pulmonary artery on the involved side or of pulmonary
aplasia by showing the presence of a short blind-ending bronchus with some rudimentary lung
parenchyma (Fig. 9C). The osseous structures may be hypoplastic on the affected side.

Proximal Interruption of the Pulmonary Artery

Proximal interruption of the pulmonary artery is a rare congenital anomaly. Because most
patients are asymptomatic, the condition may not be diagnosed until adulthood. The typical
radiographic appearance of proximal interruption of the pulmonary artery includes a small

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A B
Fig. 9Pulmonary agenesis in 8-year-old boy with
known left lung agenesis.
A, Frontal chest radiograph shows asymmetric
aeration of lungs with complete opacification of left
hemithorax. Right lung is relatively hyperinflated and
lucent compared with left lung.
B, Lateral chest radiograph shows hyperinflated
right lung (arrows) herniating anterior to mediastinal
structures.
C, Three-dimensional volume-rendered image of
central airways and lung shows no left mainstem
bronchus and left lung.

A B
Fig. 10Proximal interruption of pulmonary artery in 6-year-old boy with respiratory distress.
A, Frontal chest radiograph shows small right hemithorax with volume loss indicated by elevation of right
hemidiaphragm and mediastinal shift toward right. Left lung appears hyperlucent in comparison with right lung.
B, Axial contrast-enhanced CT image shows no right proximal pulmonary artery. MP = main pulmonary artery,
LP = left proximal pulmonary artery.

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ipsilateral hemithorax with volume loss, which is indicated by elevation of the hemidia-
phragm, mediastinal shift toward the involved side, and narrowed intercostal spaces (Fig.
10A). In contrast, the contralateral side typically shows compensatory hyperinflation with
frequent anterior lobar herniation across the midline, creating a unilateral hyperlucent lung.
Angiography (CT or traditional) can confirm the diagnosis by showing absence of the main
branch of the pulmonary artery (Fig. 10B).
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Pulmonary Artery Sling

Pulmonary artery sling is the most frequent type of congenital mediastinal vascular anom-
aly that results in unilateral hyperlucent lung due to obstruction of the mainstem bronchus.
Also known as anomalous origin of the left pulmonary artery, it is formed when the left pul-
monary artery arises from the proximal right pulmonary artery and crosses the mediastinum
between the trachea and the esophagus toward the left hilum, resulting in a sling around the
distal trachea. This aberrant course of the left pulmonary artery may cause impingement of
the right main bronchus that can lead to right-lung air trapping and hyperinflation. Alterna-
tively, it can result in right-lung atelectasis that mimics left-lung hyperlucency (Fig. 11).
There are two main types of pulmonary artery slings. In type 1, the carina is situated in a
normal position (T4T5 level) and there is usually compression of the posterior aspect of the
trachea, right mainstem bronchus, and anterior aspect of the esophagus. In type 2, there is a
low-lying (T6 level) T-shaped carina formed by a horizontal course of the main bronchi. This
condition is associated with congenital long-segment tracheal stenosis; bridging bronchus; and
several other congenital anomalies, such as hypoplasia or agenesis of right lung, horseshoe lung,
and congenital heart diseases. Patients with pulmonary artery sling typically present during the
neonatal period with wheezing, stridor, choking episodes, or aspiration pneumonia.
The imaging appearance of a pulmonary artery sling primarily depends on the type and
the presence of other congenital anomalies. In type 1, the anomalous left pulmonary artery
causes characteristic posterior compression of the trachea and anterior compression of the
esophagus (Fig. 11). There may be hyperinflation or atelectasis of the right lung related to
compression of the right mainstem bronchus. Type 2 pulmonary artery sling often shows
tracheal stenosis, trachea bifurcation at the T6 level, and a horizontal course of the right and
left mainstem bronchi that forms a T-shaped carina.

Scimitar Syndrome
Scimitar syndrome, also known as venolobar syndrome or hypogenetic lung syndrome, is
a congenital malformation of the right lung. It represents a spectrum of abnormalities that
include right lung anomalies, dextroversion of the heart, ipsilateral partial anomalous pulmo-
nary venous return, various degree of hypoplasia of the pulmonary artery, and anomalous

A B
Fig. 11Pulmonary artery sling in newborn boy who presented with severe respiratory distress.
A, Frontal chest radiograph shows opacified right lung and hyperinflated left lung. Left lung appears
hyperlucent compared with right lung.
B, Axial contrast-enhanced CT image shows anomalous left pulmonary artery (arrow) arising from main
pulmonary artery (MP). Right mainstem bronchus is compressed by anomalous left main pulmonary artery.

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A B
Fig. 12Scimitar syndrome in 6-year-old girl with
known anomaly.
A, Frontal chest radiograph shows hypoplasia of right
lung, which gives appearance of hyperlucency of left
lung. Curvilinear opacity (arrow) parallel to right heart
border represents scimitar vein.
B, Coronal MR image shows scimitar vein (arrow).
C, Three-dimensional volume-rendered image shows
entire course of scimitar vein (arrows), which drains
into inferior vena cava (IVC).

systemic collaterals providing blood supply to the right lung. The involved lung is hypoplas-
tic, and there may be bronchial stenosis or atresia or variability of lung lobation. The anomalous
pulmonary venous return of the right lung produces a left-to-right shunt. The vein usually drains
into the subdiaphragmatic inferior vena cava but may also extend to the portal or hepatic veins,
right ventricle, or coronary sinus. Collaterals arising from the aorta (thoracic or abdominal) and
bronchial arteries may provide the compensatory blood supply to the right lung.
The scimitar syndrome has a high association with other congenital anomalies (approxi-
mately 25%) involving the cardiovascular (atrial and ventricular septal defects, patent ductus
arteriosus, tetralogy of Fallot), skeletal, and genitourinary systems as well as abnormalities of
the right hemidiaphragm (defects or accessory diaphragms). Children with this condition are
asymptomatic unless there is an associated congenital heart defect or significant left-to-right
shunt that leads to the right heart volume overload and failure. Over time, affected individuals
may develop fatigue, dyspnea, or frequent right basilar pulmonary infections. Those with
advanced underlying pulmonary hypertension may become cyanotic.
On chest radiography, the characteristic appearance is asymmetric lung aeration related to
the hypoplasia of the right lung, which often gives an appearance of hyperlucency of the contra-
lateral lung (Fig. 12A). Therefore, it is critical to recognize ancillary findings that indicate the
correct side of the abnormality in patients with scimitar syndrome. Volume loss of the right lung
causes mediastinal shift and dextroversion of the heart. The right hilum can be small secondary
to hypoplasia or aplasia of the right pulmonary artery. The pathognomonic imaging finding is
the scimitar sign, a broad curvilinear opacity formed by the anomalous imaging vein that runs
parallel to the right-heart border and courses from the middle of the lung toward the cardiophren-
ic angle (Fig. 12B). This venous structure usually increases in size as it courses caudally. Cross-

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sectional imaging studies (CT or MRI with 3D reconstruction images) better delineate the size
and course of the anomalous pulmonary venous return as well as hypoplasia of the pulmonary
artery and associated systemic collateral vessels (Fig. 12C). Anomalous lung lobation and ab-
normal bronchial branching pattern can also be seen on CT.

Central Airway Abnormality

Foreign Body Aspiration
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Foreign body aspiration is the most frequent cause of intraluminal airway abnormality in
children 6 months to 3 years of age. Most aspirated foreign bodies are nonradiopaque food ma-
terials, such as nuts, beans, or seeds. The diagnosis of foreign body aspiration can be challenging
solely on the basis of clinical history and physical examination findings; only 60% of cases in
children are successfully recognized within 24 hours. The typical clinical presentation is acute
choking with cough and wheezing. Foreign body aspiration should also be suspected in a child
with no history of asthma who presents with unilateral wheezing and recurrent lobar pneumonia.

A B
Fig. 13Radiopaque foreign body aspiration in 8-month-old boy with respiratory distress. Bronchoscopy
showed beach pebble lodged in right mainstem bronchus.
A, Frontal chest radiograph shows hyperlucent right lung with oval opacity (arrow) in right mainstem bronchus.
B, Lateral chest radiograph shows oval foreign body (arrow) in proximal right mainstem bronchus.

A B
Fig. 14Radiolucent foreign body aspiration in 5-month-old boy with respiratory distress and wheezing.
Bronchoscopy showed piece of plastic toy lodged in left mainstem bronchus.
A, Frontal chest radiograph shows hyperlucent left lung.
B, Left-side-down decubitus radiograph shows persistently hyperlucent left lung, consistent with air trapping
in left lung.

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The radiographic appearance of foreign body aspiration depends on the size, location, duration,
and nature of the aspirated material (Fig. 13). Radiopaque foreign bodies can be easily identified
on radiographs. With a nonradiopaque foreign body, the most common findings include obstruc-
tive lobar or segmental hyperinflation of the lung or volume loss with atelectasis and consolidation
(Fig. 14). Inspiratory and expiratory views or supine decubitus views or airway fluoroscopy in
smaller children aid in making the diagnosis by showing air trapping due to the ball-valve effect
of the endobronchial foreign body (Fig. 14). However, several studies have reported that 2434%
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of patients with normal radiographic findings were proven to have a foreign body at bronchoscopy,
which should be considered in equivocal cases if there is a high clinical suspicion. If bronchoscopy
is not readily available, CT is a reasonable diagnostic alternative. This imaging modality may di-
rectly identify a foreign body lodged in the tracheobronchial tree as well as show such secondary
changes as retained secretions, hyperaeration, atelectasis, and consolidation.

Endobronchial Tumors
Endobronchial tumors are rare in the pediatric population. However, they should be consid-
ered in the differential diagnosis when there is persistent unilateral air trapping causing hyper-
lucency on chest radiographs but no other radiographically evident abnormality. The endobron-
chial tumors that occur in pediatric patients include carcinoid tumor, papilloma, adenoid cystic
carcinoma, mucoepidermoid carcinoma, myofibroblastic tumor, and metastasis.

A B

C D
Fig. 15Endobronchial tumor in 15-year-old boy who presented with cough.
A, Frontal chest radiograph shows triangular opacity (asterisk) in medial right lower lobe. Area of hyperlucency
(arrows) is noted in right lower lung zone.
B, Axial CT image shows heterogeneously enhancing endobronchial mass (arrow), which has resulted in right
lower lobe collapse and bronchiectatic change (curved arrow).
C, Coronal minimum intensity projection image shows area of hyperlucency (asterisk) in right lower lung zone
due to hyperinflation of right middle lobe.
D, Three-dimensional volume-rendered image of central airways and lungs shows obstructed right lower lobe
bronchus (straight arrow). Note postobstructive bronchiectatic change (curved arrow).

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Carcinoid tumors represent about 80% of endobronchial neoplasms in children. These tu-
mors most frequently occur in the central bronchi (85%), often presenting as a hilar mass.
There may be intraluminal, mural, or extrabronchial components. The remaining 15% of
carcinoid tumors arise peripherally and appear as circumscribed pulmonary nodules or mass-
es. About one third of carcinoid tumors have speckled calcifications, and most show avid
contrast enhancement. Octreotide radionuclide imaging is also helpful because it can detect
carcinoid tumor with 86% sensitivity by showing avid tracer uptake within the tumor. Chil-
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dren with endobronchial carcinoid tumors typically present with obstructive symptoms, in-
cluding dyspnea, wheezing, and cough in addition to hemoptysis.
There is substantial overlap in imaging findings among the different types of endobron-
chial tumors. Thus, the definitive diagnosis is usually based on histopathologic analysis. In
general, benign endobronchial tumors are small (< 2 cm in diameter) and well defined, where-
as malignant neoplasms are typically large and show heterogeneous contrast enhancement as
well as local invasion. Air trapping from the ball-valve effect of an endobronchial mass often
results in a unilateral hyperlucent lung (Fig. 15A). Associated lobar collapse or consolidation
can be also seen. CT is more accurate than radiography in correctly indentifying endobron-
chial lesions and the secondary postobstructive changes (Figs. 15B15D).

Mediastinal Abnormality
Foregut Malformation
Foregut malformation develops from abnormal budding of the primitive foregut during the
sixth to ninth weeks of gestation, which leads to a focal cystic duplication of the tracheo-
bronchial tree. Although there are three distinct types of foregut malformation (broncho-
genic cyst, enteric cyst, and neuroenteric cyst), these lesions have such a substantial overlap
in their imaging appearances that the definitive diagnosis is usually based on tissue diagnosis
by identifying the type of epithelium lining the cyst. The most frequent type of foregut mal-
formation is a bronchogenic cyst, which can occur anywhere along the respiratory tract in the
mediastinum (two thirds of cases) or lung parenchyma. Mediastinal bronchogenic cysts are
typically located in the paratracheal, carina (most common) or hilar region. Intraparenchy-
mal bronchogenic cysts usually occur in the medial third of a lower lobe. Most patients with
bronchogenic cysts are asymptomatic, with the lesion found incidentally on imaging per-
formed for other reasons. Symptoms are frequently related to the size and location of a medi-
astinal cyst causing an extrinsic mass effect on the adjacent trachea or bronchi. They typi-
cally include cough, wheezing, stridor, dyspnea, cyanotic spells, and pneumonia.
A large bronchogenic cyst located in the hilar or subcarinal region can exert a mass effect on the
adjacent bronchus and result in the appearance on chest radiographs of a round or oval opacity
(representing a bronchogenic cyst) in the middle mediastinal compartment that is associated with
ipsilateral hyperlucent lung (Fig. 16A). On CT or MRI, a bronchogenic cyst appears as a well-de-
fined round or oval cystic lesion (Fig. 16B). The intraluminal attenuation value varies with the
contents of mucous material and the presence of calcium. A finding of no contrast enhancement is

A B
Fig. 16Bronchogenic cyst in 5-month-old boy with respiratory distress.
A, Frontal chest radiograph shows hyperlucent left lung with opacity (asterisk) in subcarinal region.
B, Coronal CT image shows large cystic mass (asterisk) in subcarinal region that obstructs left mainstem
bronchus (arrow) and results in hyperinflated lucent left lung.

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A B

C D
Fig. 17Tuberculosis in 14-month-old boy who presented with fever, cough, and weight loss.
A, Frontal chest radiograph shows hyperlucent left lung along with right hilar (arrows) and subcarinal (asterisk)
opacities.
B, Axial soft-tissue window CT image shows large subcarinal lymphadenopathy (asterisk) and partially
obstructed left mainstem bronchus (arrow).
C, Axial lung window CT image shows hyperinflated left lung and partially obstructed left mainstem bronchus
(arrow).
D, Three-dimensional volume-rendered image shows hyperinflated left lung with obstruction of left mainstem
bronchus (arrow).

important because it can differentiate a bronchogenic cyst from a solid enhancing mass. On MRI,
the signal intensity of a bronchogenic cyst typically varies from low to high on T1-weighted im-
ages, but it always has high signal on T2-weighted images.

Mediastinal Lymphadenopathy
Although numerous conditions can produce mediastinal lymphadenopathy in children, the
two most frequent causes are infectious and neoplastic processes. Among infectious causes,
mycobacterial and fungal lymphadenopathy are most likely to compromise the bronchial air-
way and result in a unilateral hyperlucent lung (Fig. 17). Among neoplasms in pediatric pa-
tients, lymphoma is the most frequent primary lesion to cause symptomatic bronchial narrow-
ing and associated unilateral hyperlucent lung (Fig. 18). However, this can also be due to ex-
trinsic mass effect from other mediastinal masses (germ cell and neurogenic tumors) located
near the hilum. Other neoplasms that can cause bronchial narrowing and unilateral hyperlu-
cent lung in children are metastatic mediastinal lymphadenopathy from neoplasms of solid
abdominal organs and rhabdomyosarcoma.
On chest radiography, lymphadenopathy produces a mediastinal contour abnormality
(Figs. 17 and 18). If the enlarged lymph nodes cause a partial obstruction or distortion of the
airway, there may be uneven aeration, resulting in a unilateral hyperlucent lung due to under-
lying air trapping. CT and MRI can provide a more definitive diagnosis with better assess-
ment of the degree of airway compromise (Figs. 17 and 18). CT is superior for evaluating
underlying air trapping and can also show abnormal lung findings indicating an infectious
cause of mediastinal lymphadenopathy, such as tuberculosis.

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A B
Fig. 18Hodgkin lymphoma in 10-year-old boy with
weight loss and malaise for past 6 months.
A, Frontal chest radiograph shows hyperlucent right
lung with large mediastinal mass.
B, Axial contrast-enhanced CT image shows
narrowing of left main stem bronchus (straight
arrow) due to large anterior mediastinal mass. Note
atelectatic left lung (curved arrow).
C, Coronal contrast-enhanced CT image shows large
mediastinal mass (asterisk). Majority of left lung is
atelectatic, and right lung is hyperinflated.

C
Conclusion
A variety of abnormalities of the chest wall, lung parenchyma, pulmonary vessels, central
airways, and mediastinum can result in unilateral hyperlucent lung in children. Understand-
ing the spectrum of these conditions and their characteristic imaging findings can permit
early and accurate diagnosis, which in turn can result in optimal patient management.

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