CXR

CXR
Diagnosis
Diagnosis
:
:

Vascular Ring
Vascular Ring
-
-
probable double aortic arch
probable double aortic arch
Vascular Rings & Slings
Vascular Rings & Slings
Morning Report
Morning Report
December 13, 2005
December 13, 2005
Introduction
Introduction

Vascular ring
Vascular ring

refers to a variety of
refers to a variety of
congenital vascular anomalies that encircle
congenital vascular anomalies that encircle
and compress the esophagus and trachea
and compress the esophagus and trachea

Complete or
Complete or

true ring
true ring

vs. Incomplete
vs. Incomplete

Abnormalities of the aortic arch and its

Abnormalities of the aortic arch and its
branching arteries make up most of the
branching arteries make up most of the

vascular rings
vascular rings

(exception (exception- - pulmonary artery sling) pulmonary artery sling)

Embryology
Embryology
In the embryonic aortic arch, In the embryonic aortic arch,
the ventral and dorsal aorta the ventral and dorsal aorta
are connected by six primitive are connected by six primitive
aortic arches aortic arches
Regressions or persistence of Regressions or persistence of
the primitive arches cause the the primitive arches cause the
different vascular ring different vascular ring
anomalies anomalies
A A left left or or right right aortic arch is aortic arch is
defined by the defined by the mainstem mainstem
bronchus bronchus that is crossed by the that is crossed by the
aorta arch aorta arch (not to the side of the (not to the side of the
midline the aorta descends) midline the aorta descends)
www.childsdoc.org/spring98/vascular
Clinical Presentations
Clinical Presentations

Respiratory Symptoms
Respiratory Symptoms
(Most Common)
(Most Common)

Inspiratory
Inspiratory
stridor
stridor

noisy breathing
noisy breathing

or
or
wheezing
wheezing

Chronic cough
Chronic cough

Recurrent respiratory
Recurrent respiratory
infections
infections

Hoarse cry
Hoarse cry

ALTE/apnea
ALTE/apnea (infants) (infants)

Feeding Symptoms
Feeding Symptoms
(More common in older pts) (More common in older pts)

Gagging or choking w/
Gagging or choking w/
food
food

Recurrent emesis
Recurrent emesis

Dysphagia
Dysphagia (typically in older (typically in older
children/ especially with solid children/ especially with solid
foods) foods)

Failure to thrive
Failure to thrive (food (food
avoidance) avoidance)
Symptoms are often made worse w/ feeding and during intercurrent illness
Physical Exam
Physical Exam

Often Normal
Often Normal

Poor weight gain (if severe compression)

Poor weight gain (if severe compression)

Pulmonary exam may reveal wheezing,

Pulmonary exam may reveal wheezing,
stridor
stridor
,
,
dyspnea
dyspnea
, retractions
, retractions

Abnormal positioning may be observed

Abnormal positioning may be observed

Lie w/ neck extended, back arched when supine to

Lie w/ neck extended, back arched when supine to
minimize tracheal narrowing
minimize tracheal narrowing
Vascular Rings
Vascular Rings

Complete
Complete
(encircle and compress the (encircle and compress the
esophagus and trachea) esophagus and trachea)

Double aortic arch

Double aortic arch

Right aortic arch w/

Right aortic arch w/
aberrant left
aberrant left
subclavian
subclavian (and left (and left
ligamentum ligamentumarteriosum arteriosum) )

Incomplete
Incomplete
(compress but do not encircle (compress but do not encircle
the esophagus and trachea) the esophagus and trachea)

Aberrant right
Aberrant right
subclavian
subclavian
artery
artery

Anomalous
Anomalous
innominate
innominate
artery
artery

Pulmonary vascular
Pulmonary vascular
sling
sling

Right aortic arch w/

Right aortic arch w/
mirror image
mirror image
branching
branching
These two anomalies
make up >95% of all
complete vascular rings
Park: Pediatric Cardiology for Practitioners, 4th ed., Copyright 2002 Mosby, Inc
Normal Aortic Arch Anatomy
Normal Aortic Arch Anatomy
(aka innominate
artery)
www.pediatriconcall.com/fordoctor/Diseasesandcondition/Difficultydia.asp
Double Aortic Arch
Double Aortic Arch

Most common vascular
Most common vascular
ring (40%)
ring (40%)

Persistence of both right
Persistence of both right
and left
and left
branchial
branchial
arches
arches

Infants present early in
Infants present early in
life w/ respiratory distress
life w/ respiratory distress
(
(
stridor
stridor
) and feeding
) and feeding
problems
problems

Right
Right
-
-
arch dominant is
arch dominant is
most common (75%)
most common (75%)

Usually an isolated
Usually an isolated
anomaly
anomaly
www.childsdoc.org/spring98/vascular
Right Aortic Arch w/ Aberrant Left
Right Aortic Arch w/ Aberrant Left
Subclavian
Subclavian
Artery
Artery
Second most common vascular Second most common vascular
ring (30%) ring (30%)
The ring is formed by the right The ring is formed by the right
aortic arch w/ left sided aortic arch w/ left sided
ligamentum ligamentumarteriosum arteriosum
attached to an aberrant LSA attached to an aberrant LSA
Present later in life (3 Present later in life (3- -9mo) 9mo)
b/c b/c the ring is the ring is looser looser
compared to double aortic arch compared to double aortic arch
Not often associated w/ Not often associated w/
congenital heart defects congenital heart defects
(except w/ (except w/ mirror image mirror image
branching branching type) type)
www.childsdoc.org/spring98/vascular
Right Aortic Arch Anomalies
Right Aortic Arch Anomalies
www.childsdoc.org/spring98/vascular
Complete Ring Incomplete Ring
Incomplete Vascular Rings
Incomplete Vascular Rings

Anomalous
Anomalous
Innominate
Innominate
Artery
Artery
The The innominate innominate arises from the left side of the aortic arch and arises from the left side of the aortic arch and
compresses the trachea compresses the trachea
Asymptomatic or causes mild respiratory symptoms Asymptomatic or causes mild respiratory symptoms
Barium Barium Esophagram Esophagramis normal; is normal; Bronchoscopy Bronchoscopy can be diagnostic can be diagnostic
Commonly associated with other cardiac defects Commonly associated with other cardiac defects

Aberrant Right
Aberrant Right
Subclavian
Subclavian
Artery
Artery
RSA arises from the descending aorta and courses behind the RSA arises from the descending aorta and courses behind the
esophagus esophagus
Usually asymptomatic Usually asymptomatic
Mild feeding problems may occur/swallowing dysfunction Mild feeding problems may occur/swallowing dysfunction
Usually an isolated anomaly Usually an isolated anomaly
Anomalous Left Pulmonary Artery
Anomalous Left Pulmonary Artery

Pulmonary Vascular Sling

Pulmonary Vascular Sling

Left PA arises from the RPA Left PA arises from the RPA
and courses behind the and courses behind the
trachea and in front of the trachea and in front of the
esophagus to enter the esophagus to enter the hilum hilum
of the left lung of the left lung
Respiratory AND feeding Respiratory AND feeding
problems may occur problems may occur- -
symptoms may be severe symptoms may be severe
Esophagram Esophagramshows shows anterior anterior
compression only compression only
Bronchoscopy Bronchoscopy is required to is required to
r/o r/o associated tracheal associated tracheal
stenosis stenosis w/ complete tracheal w/ complete tracheal
rings rings
ring ring- -sling complex sling complex (50%) (50%)
www.childsdoc.org/spring98/vascular
Diagnosis
Diagnosis

CXR
CXR (AP and lateral) (AP and lateral)
Tracheal narrowing and/or displacement (lateral) Tracheal narrowing and/or displacement (lateral)
Aortic arch location Aortic arch location
Tracheal Position: abnormal deviation to the left Tracheal Position: abnormal deviation to the left
(due to the aorta coursing over the right (due to the aorta coursing over the right mainstem mainstembronchus) bronchus) OR OR
Midline position with double aortic arch Midline position with double aortic arch
Atelectasis Atelectasis, hyperinflation, or pneumonia may be present , hyperinflation, or pneumonia may be present
(especially in pulmonary artery slings) (especially in pulmonary artery slings)

Barium
Barium
Esophagram
Esophagram
Most important and reliable diagnostic tool Most important and reliable diagnostic tool
Except in anomalous Except in anomalous innominate innominate artery artery
Posterior Posterior (and anterior) compression of esophagus on lateral (and anterior) compression of esophagus on lateral
Except in pulmonary artery sling Except in pulmonary artery sling anterior anterior compression only compression only
CXR
CXR
www.uhrad.com/ pedsarc/peds056a.jpg
Tracheal Deviation
Tracheal Deviation
www.pediatriconcall.com/fordoctor/Diseasesandcondition/Difficultydia.asp
Barium
Barium
Esophagram
Esophagram
Other Imaging
Other Imaging

ECHO ECHO
Recommended to exclude other cardiac defects and visualizes the Recommended to exclude other cardiac defects and visualizes the
vascular ring vascular ring
Very useful in diagnosing pulmonary artery sling Very useful in diagnosing pulmonary artery sling
CT/MRI CT/MRI
Identify vascular structures and anatomy of Identify vascular structures and anatomy of tracheobronchial tracheobronchial tree tree
Angiography Angiography
Considered Considered gold standard gold standard , but rarely needed for diagnosis , but rarely needed for diagnosis
Bronchoscopy Bronchoscopy
Use as a diagnostic tool is controversial Use as a diagnostic tool is controversial
Recommended for diagnosis of a vascular sling to Recommended for diagnosis of a vascular sling to r/o r/o concomitant concomitant
tracheal rings (and useful for aberrant tracheal rings (and useful for aberrant innominate innominate artery) artery)
Management
Management

Asymptomatic patients need no surgical treatment, even
Asymptomatic patients need no surgical treatment, even
when anomalies are found incidentally
when anomalies are found incidentally

Medical management is recommended for infants with
Medical management is recommended for infants with
mild symptoms
mild symptoms
Careful feeding w/ soft foods Careful feeding w/ soft foods
Aggressive treatment of pulmonary infections Aggressive treatment of pulmonary infections

Respiratory distress,
Respiratory distress,
h/o
h/o
recurrent pulmonary infections,
recurrent pulmonary infections,
apneic
apneic
spells, FTT are indications for surgical
spells, FTT are indications for surgical
intervention
intervention
Surgical Management
Surgical Management

Double Aortic Arch
Double Aortic Arch
Division of the smaller of the two arches (usually the left arch Division of the smaller of the two arches (usually the left arch) )

Right Aortic Arch w/ Aberrant LSA
Right Aortic Arch w/ Aberrant LSA
Division of the Division of the ligamentum ligamentumarteriosum arteriosum

Anomalous
Anomalous
Innominate
Innominate
Artery
Artery
Surgical suturing of the artery to the sternum (if >75% tracheal Surgical suturing of the artery to the sternum (if >75% tracheal
narrowing) narrowing)- - rarely indicated rarely indicated

Aberrant right
Aberrant right
Subclavian
Subclavian
Artery
Artery
Surgical interruption of the artery (rarely indicated) Surgical interruption of the artery (rarely indicated)

Anomalous Left Pulmonary Artery
Anomalous Left Pulmonary Artery
(Pulmonary Vascular
(Pulmonary Vascular
Sling)
Sling)
Surgical division and Surgical division and reimplantation reimplantation of the LPA of the LPA
Complications
Complications

Surgical Mortality rate is generally low (<5%)

Surgical Mortality rate is generally low (<5%)
(higher with repair of pulmonary vascular sling)
(higher with repair of pulmonary vascular sling)

Underlying
Underlying
tracheomalacia
tracheomalacia
is often present
is often present
(secondary to the compression) (secondary to the compression)
and may cause persistent
and may cause persistent
airway obstruction for weeks to months post
airway obstruction for weeks to months post
-
-
operatively
operatively

Important to inform parents that the

Important to inform parents that the

noisy
noisy
breathing
breathing

may persist for months following surgery

may persist for months following surgery