Recurrent Coins and Recurrent Respiratory Infections
Radiology Cases in Pediatric Emergency Medicine
Volume 6, Case 20
Daniel D. Guzman, MD
Collin S. Goto, MD
Children's Medical Center of Dallas
University of Texas Southwestern School of Medicine
This is a 15-month old male who presents to the
Emergency Department with his parents stating that
while he was standing, he became red in the face while
gagging and his head began to rock forward and
backward with his eyes closed. This episode lasted
less that 5 minutes, then he became quiet and resumed
playing. This occurred one more time and his parents
then brought him in to the Emergency Department.
His past history is remarkable for multiple upper
respiratory infections in the past several months. He
has also been seen in the past for two separate
episodes of esophageal coins requiring endoscopic
removal 5 months ago and 1 week ago. He was born
at 36 weeks gestation, but without complications. He
has no previous history of seizures and no family
history of seizures. He is on no medications. There is
no witnessed foreign body ingestion or aspiration for his
Exam: VS T 37.7, P 104, R 33, BP 111/83, oxygen
saturation 98% on room air. He is alert, active, and in
no distress. He has mild inspiratory stridor at rest. His
lung fields demonstrate good aeration. No cyanosis is
noted. He has a grade 1/6 systolic murmur at the left
sternal border, with no radiation. No diastolic murmurs,
ejection clicks or gallops are appreciated and his
peripheral pulses are brisk. His abdomen is soft,
non-tender and without organomegaly. His neurologic
exam reveals no deficits.
His previous radiographs are examined. Five
months prior, he presented with an esophageal coin
diagnosed on chest radiographs.
View CXR from 5 months ago (CXR #1).
The esophageal coin is visible on the PA and lateral
views. The coin was endoscopically removed.
View CXR from 1 week ago (CXR #2).
This chest radiograph is obtained from the
fluoroscope. The esophageal coin is visible on the PA
and lateral views. The coin was again endoscopically
A careful review of these two previous chest
radiographs reveals a narrowed tracheal air column
best visible on both lateral views. On the set of
radiographs 1 week ago (#2), the tracheal air column on
the lateral appears to be bent suggesting a mass
pushing on it.
View these CXR's again.
An esophageal mass or edema surrounding the
foreign body could result in extrinsic compression of the
trachea. Once the esophageal coin is removed, it
would not be routine to repeat the chest radiographs to
examine the caliber of the tracheal air column again.
Clinical examination following the coin removal is
generally sufficient. In this case, the stridor appears to
The presentation of a child with persistent stridor
and the two previous occurrences of a foreign body
lodged in the esophagus, suggests intrathoracic
compression of both the trachea and the esophagus.
The differential diagnosis includes vascular rings or
slings, a mediastinal mass, or an intrinsic esophageal
tumor or duplication. Another chest radiograph is
View current CXR (#3).
These chest radiographs show a normal cardiac
silhouette without mediastinal widening, but there is
some suggestion of a right-sided aortic arch with slight
narrowing of the trachea on the frontal view. There are
also perihilar opacities in the lungs likely secondary to a
poor inspiration. The tracheal air column on this set of
radiographs is not as narrow as it was on the previous
chest radiographs, but the bend in the trachea seen on
the lateral view is still present.
At this time, a vascular ring is suspected and a
barium swallow is performed.
View Barium Swallow.
The barium swallow study confirms the presence of
a right sided aortic arch. The views shown here
demonstrate an extrinsic mass compressing the
esophagus at the level of the aortic arch. Real time
fluoroscopy also revealed intermittent narrowing of the
trachea at the same level. This is consistent with a
vascular ring due to a double aortic arch encircling and
compressing both the trachea and the esophagus.
Refer to the anatomic drawing (Fig 1) in the previous
case (Case 19 of Volume 6).
An MRI was performed to further delineate the
intrathoracic anatomy. The MRI showed a double
aortic arch with the right arch being larger than the left
arch. The two arches meet to form one descending
aorta on the left. The right common carotid artery and
the right subclavian originate from the right arch and the
left common carotid and the left subclavian originate
from the left arch. The cardiac size is normal. The
trachea is narrowed at the site of the double aortic arch
and is more severely narrowed inferiorly with the
narrowing extending to the carinal region and to the
right main stem bronchus. The narrowing is due to
tracheomalacia, which is associated with the vascular
An echocardiogram revealed normal intracardiac
anatomy and function. The cardiology and
cardiothoracic surgery teams were involved and the
patient underwent operative repair with an uneventful
Vascular Ring Teaching Points
Vascular rings are congenital anomalies of the aortic
arch and its tributaries, causing varying degrees of
compression of the trachea and esophagus.
Five different variations of vascular rings exist: 1)
double aortic arch (most common), 2) right aortic arch
with left ligamentum arteriosum, 3) anomalous right
subclavian artery, 4) anomalous innominate artery, and
5) anomalous left common carotid artery.
Double aortic arch is an anomaly in which both right
and left arches are present and may be one of several
variations: 1) both arches widely patent, 2) hypoplasia
of one arch (usually the left), 3) atresia of one arch
(usually the left).
Double aortic arch represents a persistence of both
right and left embryonic fourth branchial arches joining
the aortic portion of the truncoaortic sac to their
respective dorsal aorta. The ascending aorta bifurcates
anterior to the trachea and each arch courses either to
the left or to the right of the trachea or esophagus. The
larger of the two arches usually crosses posterior to the
esophagus and joins with the other arch in the posterior
mediastinum to form the unified descending aorta.
Thus a complete vascular ring is formed.
Double aortic arch is rarely associated with
congenital heart disease, but when present, Tetralogy
of Fallot and transposition of the great vessels are
Clinical manifestations are related to the nature of
malformation and tightness of the ring. If both arches
are widely patent, the rings are tight and patients
present with stridor in the first weeks of life. When one
arch is hypoplastic or atretic, the rings are usually
looser, with presentation at 3-6 months of age. Rarely,
does double aortic arch present in adulthood.
Children with double aortic arch are often small,
poorly developed and hold their head in hyperextension.
Repeated severe respiratory infections may occur.
Physical exam may be within normal limits, but one
may see coughing, dyspnea, drooling or dysphagia.
Infants will feed poorly due respiratory distress and may
have life-threatening episodes of apnea and cyanosis.
Cardiac exam will most often be normal. Lung exam
may or may not show evidence of pneumonia.
Radiographs may show no significant abnormalities.
Tracheal deformities can sometimes be detected or at
least suspected from the shadow of the air column in
the trachea without contrast. Both arches may be seen
on either side of the trachea in the anteroposterior
projection. There may also be evidence of
hyperinflation of either or both lungs due to obstruction
of the lower trachea and main stem bronchi. An
esophagram may not only establish the diagnosis, but
also identify the structures making up the constricting
ring. Computerized imaging (CT and/or MRI) provides
information noninvasively and identifies important
spatial relationships between the vessels, trachea and
esophagus to better permit surgical planning.
Surgical division of the vascular ring is indicated in
any patient who is symptomatic with airway or
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McMillan JA, Warshaw JB (eds). Principles and
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1994, pp. 1588-1589.
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