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O R IGINA L PA PE 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 ofpatients with vascular rings and evaluate the effective-ness of investigations. Surgical outcomes and respiratorysequelae were also examined. The design was a retro-

spective case note study over a 13-year period set in atertiary childrens hospital. Children below the age of 16years presenting with a vascular ring to the RoyalHospital for Sick Children, Glasgow were studied.Demographic data at presentation, including symptoms,were recorded. The ability of diagnostic investigations toidentify the presence of a vascular ring was evaluated.Surgical outcomes were determined by measuring sur-gical complications and mortality. Respiratory sequelaewere recorded by the presence of persistent symptoms orthe need for tracheostomy or long-term ventilation fol-lowing surgery. A total of 24 patients were identifiedwith a median age at presentation of 4.5 months. Stridor

was the commonest presenting symptom (14/24). Angi-ography, chest CT scanning and MRI were the mostaccurate imaging modalities (accurate in 100% of casesused). Chest X-ray films and echocardiography had thelowest detection rates. Surgical complications (4/24) andmortality (1/24) were low. A substantial number ofpatients available to follow-up (7/20) were still experi-encing stridor 3 months post-operatively. Conclusion:Vascular rings are rare, however, often present withcommon symptoms. Most children present in earlyinfancy, but a minority presents much later. The inves-tigation of choice is a barium swallow followed byhigh-resolution computed tomography. Surgery is safe

although a number of patients will have persistingsymptoms.

Keywords Congenital heart disease Double aorticarch Echocardiography Neonatal respiratorydistress Vascular rings

Introduction

Symptoms such as cough, stridor and vomiting feeds arecommon symptoms in early infancy. The aetiology isusually benign, although in a small number of childrenthese symptoms are caused by the presence of a completeor partial vascular ring compressing either the trachea oroesophagus. Distinguishing these patients from the muchlarger group of children with non-serious pathologies isdifficult and requires a high index of suspicion andknowledge of the appropriate investigative tools avail-able. Once a diagnosis has been made, it is important to be

aware of the outcomes and long-term complications ofsurgical correction so that appropriate counselling andfollow-up can be arranged. We therefore decided toexamine our hospitals experience of vascular rings todetermine (1) the clinical presentation of infants withvascular rings, (2) the use of investigations to investigatethe possibility of a vascular ring and (3) the consequencesof surgery and any long-term respiratory or gastrointes-tinal sequelae.

Subjects and methods

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

A. Turner (&) G. Gavel J. CouttsDepartment of Paediatrics, Royal Hospital for Sick Children,Yorkhill, Glasgow, UKE-mail: [email protected].: +44-141-201-0000

Eur J Pediatr (2005) 164: 266270DOI 10.1007/s00431-004-1607-6


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positively diagnose vascular rings noted (Table 3). Thedefinition of a positive test was adapted from the findingsof Bakker et al. [1] (Table3). The results of surgery wereevaluated by examining the length of post-operativeventilation, duration of in-patient stay, surgical compli-cations and mortality. The long-term consequences ofvascular rings were determined by the finding of persistentpost-operative symptoms and the need for tracheostomyor home ventilation (Table4).

Results

Subjects

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

diagnosis was 4.5 months (range birth8.7 years). Forcomparison, normal mediastinal anatomy is shown inFig.1. Double aortic arch (Fig. 2) was the commonesttype of vascular ring (9/24) followed by right aortic archwith persistent left ligament (8/24). Pulmonary arterysling (3/24) (Fig.3), aberrant right subclavian artery(3/24) (Fig.4) and right aortic arch with aberrant leftsubclavian artery (1/24) were less common. Age atdiagnosis was earlier in patients with pulmonary arterysling and double aortic arch (1 month and 3 monthsrespectively) than right aortic arch and persistent leftligament (45 months) (Table1. Of all patients, 50% (12/24) had associated co-morbid features. These includedtrisomy 21 (5/24), del22q11 (1/24), anal atresia (1/24),tracheal stenosis (1/24) and congenital lobar emphysema(1/24). Congenital heart disease was identified in 10/24patients 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 andfeeding difficulties (12/24). Cough and recurrent chestinfections were less prominent (both 5/24) symptoms.Two patients presented as failure to wean from positivepressure ventilation following unrelated surgery(Table 2).

Table 2 Variation in symptoms according to type of vascular ring

Type of vascular ring N Stridor Cough Wheeze Recurrentchest infection

Vomiting/feedingdifficulties

Other

Right aortic arch with persistent

left ligament

8 2 2 3 3 5 -

Double aortic arch 9 7 1 6 2 4 1 a

Pulmonary artery sling 3 2 1 3 - 2 -Aberrant right subclavian artery 3 3 1 1 - 1 -Right aortic arch with aberrant

left subclavian artery1 - - - - - 1a

Total 24 14 5 13 5 12 2

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

Table 1 Types of vascular ring and median age at diagnosis

Type of vascular ring N Median age atdiagnosis (months)

Right aortic arch withpersistent left ligament

8 45 (3105 months)

Double aortic arch 9 3 (birth to 17 months)Pulmonary artery sling 3 1 (birth to 3 months)Aberrant right subclavian artery 3 4 (4 to 5 months)Right aortic arch with aberrant

left subclavian artery1 0.5

Total 24 4.5

Table 3 Imaging modalities of vascular rings

Type of vascular ring N Chest X-ray film Barium study Echocardiogram Bronchoscopy Angiography CT scan MRI scanTracheal

narrowing

Oesophageal

indentation

Vascular

abnormality

Pulsatile

compression

Vascular

abnormality

Vascular

abnormality

Vascular

abnormality

Right aortic archwith persistentleft ligament

8 0/8 7/7 1/3 3/4 3/3 2/2 1/1

Double aortic arch 9 1/9 7/7 3/6 4/5 4/4 2/2 2/2Pulmonary artery sling 3 0/3 1/2 3/3 2/3 2/2 1/1 -Aberrant right

subclavian artery3 0/3 3/3 0/2 2/3 2/2 - -

Right aortic archwith aberrantleft subclavian artery

1 0/1 - 0/1 1/1 1/1 - -

Total 24 1/24 18/19 7/15 12/16 12/12 5/5 3/3

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Imaging

All patients had chest X-rays films taken. In only onewas 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 in7/15 of patients. Bronchoscopy revealed a pulsatile

Table 4 Outcome depending on type of vascular ring

Type of vascular ring N Number availableto follow-up

Stridorat 3 months

Stridorat 18 months

Tracheostomy Homeventilation

Death

Right aortic arch withpersistent left ligament

8 6 1 0 1 0 0

Double aortic arch 9 8 2 0 2 1 1 a

Pulmonary artery sling 3 1 1 0 0 0 0Aberrant right subclavian artery 3 3 3 1 0 0 0Right aortic arch with aberrant

left subclavian artery1 1 0 0 0 0 0

Total 24 20 7 1 3 1 1

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

Fig. 1 Normal mediastinal anatomy

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

Fig. 3 Pulmonary artery sling (LPA left pulmonary artery, MPAmiddle pulmonary artery, RPA right pulmonary artery)

Fig. 4 Aberrant right subclavian artery

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compression of the trachea in 12/16 of cases studied.Angiography, chest CT scan and MRI all demonstratedvascular abnormalities in 100% of cases used althoughthe number of patients exposed to these investigationswas limited (twelve, five and three patients respectively)(Table 3).

Outcome

All 24 patients underwent surgery for repair of vascularring (Table4). Four patients experienced post-operativecomplications. These included two chylothoraces, one

pneumothorax and one cardiac arrest from which thepatient was successfully resuscitated. The patient whoexperienced cardiac arrest had also undergone repair ofan anomalous coronary artery and ventricular septaldefect at the time of surgery. No patient died in theimmediate post-operative period. The median durationof post-operative ventilation was 1 day; however, therewas a wide range (


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operative morbidity and mortality is low. Of moreconcern, however, is the presence of persistent symp-tomatic tracheomalacia that may contribute to pro-longed post-operative ventilation in the ICU and onoccasion require the use of tracheostomy or home ven-tilation in a minority of patients. This group of patients,whilst small, represents a challenge and other interven-tions such as the use of ward-based or home ventilationshould be considered early.

References

1. Bakker DAH, Berger RMF, Witsenburg M, Bogers AJJC.(1999) Vascular rings: a rare cause of common respiratorysymptoms. Acta Paediatr 88: 947952

2. Chun K, Colombani PM, Dudgeon DL, Haller JA Jr (1992)Diagnosis and management of congenital vascular rings: a 22-year experience. Ann Thoraci Surg 53: 597602

3. Klinkhamer AC (1969) Esophagography in anomalies of theaortic arch system. Williams and Wilkins, Baltimore

4. Lillehei CW, Colan S (1992) Echocardiography in the preoper-ative evaluation of vascular rings. J Pediatr Surg 27: 11181121

5. Lowe GM, Donaldson JS, Backer CL (1991) Vascular rings: 10-year review of imaging. Radiographics 11: 637646

6. Stewart JR, Kincaid OW, Edwards JE (1964) An atlas of vas-cular rings and related malformations of the aortic system.

Charles C Thomas, Springfield, p 1117. Valleta EA, Pregarz M, Bergamo-Andreis IA, Boner AL (1997)

Tracheoesophageal compression due to congenital vascularanomalies (vascular rings). Pediatr Pulmonol 24: 93105

8. Van Son JA, Julsrud PR, Hagler DJ (1993) Surgical treatment ofvascular rings: the Mayo Clinic experience. Mayo Clinic Proc 68:11311133

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