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Eur J Pediatr (2005) 164: 266270

DOI 10.1007/s00431-004-1607-6

O R I GI N A L P A P E R

Alastair Turner Gil Gavel Jonathan Coutts

Vascular ringspresentation, investigation and outcome

Received: 1 March 2004 / Accepted: 25 October 2004 / Published online: 22 January 2005
Springer-Verlag 2005

Abstract Our aim was to determine the presentation of


patients with vascular rings and evaluate the eectiveness of investigations. Surgical outcomes and respiratory
sequelae were also examined. The design was a retrospective case note study over a 13-year period set in a
tertiary childrens hospital. Children below the age of 16
years presenting with a vascular ring to the Royal
Hospital for Sick Children, Glasgow were studied.
Demographic data at presentation, including symptoms,
were recorded. The ability of diagnostic investigations to
identify the presence of a vascular ring was evaluated.
Surgical outcomes were determined by measuring surgical complications and mortality. Respiratory sequelae
were recorded by the presence of persistent symptoms or
the need for tracheostomy or long-term ventilation following surgery. A total of 24 patients were identied
with a median age at presentation of 4.5 months. Stridor
was the commonest presenting symptom (14/24). Angiography, chest CT scanning and MRI were the most
accurate imaging modalities (accurate in 100% of cases
used). Chest X-ray lms and echocardiography had the
lowest detection rates. Surgical complications (4/24) and
mortality (1/24) were low. A substantial number of
patients available to follow-up (7/20) were still experiencing stridor 3 months post-operatively. Conclusion:
Vascular rings are rare, however, often present with
common symptoms. Most children present in early
infancy, but a minority presents much later. The investigation of choice is a barium swallow followed by
high-resolution computed tomography. Surgery is safe
although a number of patients will have persisting
symptoms.

A. Turner (&) G. Gavel J. Coutts


Department of Paediatrics, Royal Hospital for Sick Children,
Yorkhill, Glasgow, UK
E-mail: alastairturner@yahoo.co.uk
Tel.: +44-141-201-0000

Keywords Congenital heart disease Double aortic


arch Echocardiography Neonatal respiratory
distress Vascular rings

Introduction
Symptoms such as cough, stridor and vomiting feeds are
common symptoms in early infancy. The aetiology is
usually benign, although in a small number of children
these symptoms are caused by the presence of a complete
or partial vascular ring compressing either the trachea or
oesophagus. Distinguishing these patients from the much
larger group of children with non-serious pathologies is
dicult and requires a high index of suspicion and
knowledge of the appropriate investigative tools available. Once a diagnosis has been made, it is important to be
aware of the outcomes and long-term complications of
surgical correction so that appropriate counselling and
follow-up can be arranged. We therefore decided to
examine our hospitals experience of vascular rings to
determine (1) the clinical presentation of infants with
vascular rings, (2) the use of investigations to investigate
the possibility of a vascular ring and (3) the consequences
of surgery and any long-term respiratory or gastrointestinal sequelae.

Subjects and methods


A retrospective case note study was performed utilising
the computerised coding system for all cases of vascular
rings attending the Royal Hospital for Sick Children,
Glasgow. Records from 1989 to 2002 were examined. The
authors reviewed the medical les of all cases. Basic
demographic data was retrieved (Table 1). The type of
vascular ring was classied according to Klinkhamer [3]
and Stewart et al. [6] as used in previous studies of vascular
rings [1,8]. Presenting symptoms were also noted
(Table 2). Investigations undertaken to establish the
diagnosis were also documented and their ability to

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Table 1 Types of vascular ring and median age at diagnosis
Type of vascular ring

Right aortic arch with


persistent left ligament
Double aortic arch
Pulmonary artery sling
Aberrant right subclavian artery
Right aortic arch with aberrant
left subclavian artery
Total

Median age at
diagnosis (months)

45 (3105 months)

9
3
3
1

3 (birth to 17 months)
1 (birth to 3 months)
4 (4 to 5 months)
0.5

24

4.5

positively diagnose vascular rings noted (Table 3). The


denition of a positive test was adapted from the ndings
of Bakker et al. [1] (Table 3). The results of surgery were
evaluated by examining the length of post-operative
ventilation, duration of in-patient stay, surgical complications and mortality. The long-term consequences of
vascular rings were determined by the nding of persistent
post-operative symptoms and the need for tracheostomy
or home ventilation (Table 4).

Results
Subjects
A total of 24 patients presenting with vascular rings were
identied (11 boys and 13 girls). The median age at

diagnosis was 4.5 months (range birth8.7 years). For


comparison, normal mediastinal anatomy is shown in
Fig. 1. Double aortic arch (Fig. 2) was the commonest
type of vascular ring (9/24) followed by right aortic arch
with persistent left ligament (8/24). Pulmonary artery
sling (3/24) (Fig. 3), aberrant right subclavian artery
(3/24) (Fig. 4) and right aortic arch with aberrant left
subclavian artery (1/24) were less common. Age at
diagnosis was earlier in patients with pulmonary artery
sling and double aortic arch (1 month and 3 months
respectively) than right aortic arch and persistent left
ligament (45 months) (Table 1. Of all patients, 50% (12/
24) had associated co-morbid features. These included
trisomy 21 (5/24), del22q11 (1/24), anal atresia (1/24),
tracheal stenosis (1/24) and congenital lobar emphysema
(1/24). Congenital heart disease was identied in 10/24
patients including 4/5 of the patients with trisomy 21.

Symptoms
The commonest symptoms at diagnosis were stridor
(14/24) and wheeze (13/24) followed by vomiting and
feeding diculties (12/24). Cough and recurrent chest
infections were less prominent (both 5/24) symptoms.
Two patients presented as failure to wean from positive
pressure ventilation following unrelated surgery
(Table 2).

Table 2 Variation in symptoms according to type of vascular ring


Type of vascular ring
Right aortic arch with persistent
left ligament
Double aortic arch
Pulmonary artery sling
Aberrant right subclavian artery
Right aortic arch with aberrant
left subclavian artery
Total
a

Stridor

Cough

Wheeze

Recurrent
chest infection

Vomiting/feeding
diculties

Other

9
3
3
1

7
2
3
-

1
1
1
-

6
3
1
-

2
-

4
2
1
-

1a
1a

24

14

13

12

Both presented as failure to wean from intermittent positive pressure ventilation following unrelated surgery

Table 3 Imaging modalities of vascular rings


Type of vascular ring

Right aortic arch


with persistent
left ligament
Double aortic arch
Pulmonary artery sling
Aberrant right
subclavian artery
Right aortic arch
with aberrant
left subclavian artery
Total

N Chest X-ray lm Barium study Echocardiogram Bronchoscopy Angiography CT scan


MRI scan
Tracheal
Oesophageal Vascular
Pulsatile
Vascular
Vascular
Vascular
narrowing
indentation
abnormality
compression abnormality abnormality abnormality
8

0/8

7/7

1/3

3/4

3/3

2/2

1/1

9
3
3

1/9
0/3
0/3

7/7
1/2
3/3

3/6
3/3
0/2

4/5
2/3
2/3

4/4
2/2
2/2

2/2
1/1
-

2/2
-

0/1

0/1

1/1

1/1

18/19

7/15

12/16

12/12

5/5

3/3

24 1/24

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Table 4 Outcome depending on type of vascular ring
Type of vascular ring
Right aortic arch with
persistent left ligament
Double aortic arch
Pulmonary artery sling
Aberrant right subclavian artery
Right aortic arch with aberrant
left subclavian artery
Total
a

Number available
to follow-up

Stridor
at 3 months

Stridor
at 18 months

Tracheostomy

Home
ventilation

Death

9
3
3
1

8
1
3
1

2
1
3
0

0
0
1
0

2
0
0
0

1
0
0
0

1
0
0
0

24

20

Died at the age of 10 months, 9 months following surgery, from bronchiolitis. Patient required tracheostomy post-operatively

Fig. 1 Normal mediastinal anatomy

Imaging

Fig. 3 Pulmonary artery sling (LPA left pulmonary artery, MPA


middle pulmonary artery, RPA right pulmonary artery)

All patients had chest X-rays lms taken. In only one


was characteristic tracheal narrowing noted (1/24).
Barium swallows were performed in 19 patients, of

whom 18 showed oesophageal indentation (18/19).


Echocardiography determined vascular abnormalities in
7/15 of patients. Bronchoscopy revealed a pulsatile

Fig. 2 Double aortic arch (AO aorta, LCA left carotid artery,
LSCA left subclavian artery, PA pulmonary artery, RCA right
carotid artery, RSCA right subclavian artery)

Fig. 4 Aberrant right subclavian artery

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Fig. 5 Right aortic arch with aberrant left subclavian artery and
persistent left ligament

compression of the trachea in 12/16 of cases studied.


Angiography, chest CT scan and MRI all demonstrated
vascular abnormalities in 100% of cases used although
the number of patients exposed to these investigations
was limited (twelve, ve and three patients respectively)
(Table 3).
Outcome
All 24 patients underwent surgery for repair of vascular
ring (Table 4). Four patients experienced post-operative
complications. These included two chylothoraces, one
pneumothorax and one cardiac arrest from which the
patient was successfully resuscitated. The patient who
experienced cardiac arrest had also undergone repair of
an anomalous coronary artery and ventricular septal
defect at the time of surgery. No patient died in the
immediate post-operative period. The median duration
of post-operative ventilation was 1 day; however, there
was a wide range (<1 to 200 days). Three patients required tracheostomy post-operatively for persistent tracheomalacia. The median duration of in-patient stay was
9 days (range 3276 days). Some 20 patients were
available to long-term follow-up, the remaining four
patients having moved address. Of these 20 patients, 7
had persistent stridor 3 months post-surgery although
this dropped to 1/20 at 18 months (Table 3). Of the three
patients who required tracheostomy, one required longterm home ventilation and one died 9 months postoperatively due to bronchiolitis.

Discussion
This report documents 24 cases of vascular rings
occurring in children. The majority of cases present in

early infancy. A high proportion of patients have comorbid features, particularly congenital heart disease.
Symptoms at diagnosis are typically common respiratory and gastrointestinal complaints such as stridor,
wheeze and vomiting feeds although occasionally vascular rings may present as failure to wean from
mechanical ventilation following unrelated surgery.
These ndings are in keeping with our initial impression
that the presentation of vascular rings is frequently nonspecic and that a high index of suspicion must be
maintained to allow diagnosis. Of interest is the high
proportion of patients with co-morbid features. Similar
ndings have been reported by other groups [1]. This
suggests that the possibility of a vascular ring should be
considered early in this group of patients when presenting with apparently straightforward symptoms.
When contemplating the possibility of a vascular
ring, the question arises as to the best means of imaging.
Our data, in keeping with those of others [1,5], show that
a barium swallow remains an excellent means of demonstrating the presence of a vascular ring. Chest X-ray
lms have a low detection rate. They are, however,
important in excluding other causes of common respiratory symptoms. Echocardiography, in this series of
patients, was disappointing in its ability to detect vascular rings. Other groups have had more success using
this technique [4]. This illustrates the inter-operator
variability of echocardiography. Given the high rate of
congenital heart disease in this group, it does, however,
have an important role once the diagnosis of vascular
ring has been established. Bronchoscopy was useful in
revealing the presence of a pulsatile compression of the
trachea in the majority of cases used and has the added
benet of being able to examine the upper airway for
other causes of respiratory symptoms such as tracheomalacia [7]. Unfortunately, in this age group it requires a
general anaesthetic and cannot provide information
about the anatomy of the vascular ring. Angiography
was shown to be useful in giving detailed and highly
specic information on anatomy; however, carries with
it the risks of an interventional procedure and general
anaesthesia. As such we would recommend its use only if
there was diagnostic uncertainty despite other imaging
modalities. Perhaps the most attractive imaging modality available is the use of CT or MRI. Both are noninvasive and in this series of patients, although the
numbers are low, were accurate in all cases used. MRI
and CT allow accurate imaging that may help in the
planning of surgery [2]. With the development of highspeed, high-resolution CT scanning, general anaesthesia
is usually unnecessary although may still be required for
MRI. On the basis of our ndings, we would conclude
that the rst examination should be a barium swallow
followed by a high-resolution chest CT scan.
The presence of a symptomatic vascular ring necessitates the use of thoracic surgery in a population that is
by its nature high risk (usually infants and frequently
with co-morbid conditions). As such it is pleasing to
report, in common with other studies [2], that the

270

operative morbidity and mortality is low. Of more


concern, however, is the presence of persistent symptomatic tracheomalacia that may contribute to prolonged post-operative ventilation in the ICU and on
occasion require the use of tracheostomy or home ventilation in a minority of patients. This group of patients,
whilst small, represents a challenge and other interventions such as the use of ward-based or home ventilation
should be considered early.

References
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(1999) Vascular rings: a rare cause of common respiratory
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