MINI-SYMPOSIUM: IMAGING AND INTERVENTIONAL RADIOLOGY

Vascular compression of the airway in children

PAEDIATRIC RESPIRATORY REVIEWS (2008) 9, 85–94

Clare A. McLaren2,*, Martin J. Elliott1 and Derek J. Roebuck2

1Department of Cardiothoracic Surgery, The Great Ormond Street Hospital for Children NHS Trust, London, WC1N3JH, UK; 2Department of Radiology, The Great Ormond Street Hospital for Children NHS Trust, London,WC1N 3JH, UK

KEYWORDS

airway compression;

self-expanding stents;

vascular rings;

tracheomalacia

Summary Congenital heart disease (CHD) is an important clinical problem. Althoughsurvival has improved over recent decades, certain children with CHD remain difficult totreat, usually because of severe co-morbidity or uncorrectable defects. Vascular com-pression of the airway is one such co-morbidity, occurring in approximately 1–2% ofchildren with CHD. It may be caused by congenital anomalies of the configuration of thegreat vessels, enlargement of otherwise normal structures or as a result of surgery. Theanatomical patterns seen in these children may be complex, and as surgical correction isusually required to relieve the compression, the pre-operative imaging assessment shouldbe as complete as possible. Precise diagnosis and therapy are essential because chronicairway compression in childhood carries a significant morbidity and mortality. Airwaystenting is currently reserved for rare occasions when surgical correction is not possible.� 2008 Elsevier Ltd. All rights reserved.

Vascular compression of the airway in children is usuallycaused either by congenital anomalies of the configuration ofthe great vessels or enlargement of otherwise normalstructures (Table 1).1 The most common congenitalanomalies associated with airway compression are thevascular rings. The ring may be patent (as in double aorticarch) or alternatively be completed by an atretic arch orligamentum arteriosum (as in right-sided aortic arch withaberrant left subclavian artery). The aortic arch and itsbranches and the pulmonary arteries are derived from theembryonic branchial arches. Failure of part of this complexembryological process is uncommon, but is the cause ofmany of the malformations that result in vascular airwaycompression.1–3 An understanding of the embryology ofthe aortic arch and related structures is helpful for imageinterpretation.

* Corresponding author.E-mail address: mclarc@gosh.nhs.uk (C.A. McLaren).

1526-0542/$ – see front matter � 2008 Elsevier Ltd. All rights reserved.

doi:10.1016/j.prrv.2007.12.008

Other vascular mechanisms of airway compressionexist. The most important of these are seen in childrenwith absent pulmonary valve syndrome, interrupted aorticarch and dilated cardiomyopathy (see below). Less com-mon causes of vascular airway compression include pul-monary artery sling without long segment congenitaltracheal stenosis and compression of the trachea by theinnominate artery (brachiocephalic trunk).

Symptoms at presentation are variable, ranging fromdysphagia, recurrent respiratory infections and stridor toacute respiratory distress or ‘dying spells’.2–5 Symptomssuch as chest discomfort, dyspnoea, wheezing and coughare often misdiagnosed as asthma, which is much morecommon in older children.6 Affected children may requiremechanical ventilation and some may remain ventilator-dependent, even after surgery.

A combination of imaging techniques is usuallyrequired for full pre-operative assessment. Precise diag-nosis and therapy are essential because chronic airway

86 C. A. MCLAREN ET AL.

Table 1 Causes of vascular compression of the airway inchildren

� Anomalies of the aorta* Double aortic arch* Interrupted aortic arch (after surgical repair)* Right-sided aortic arch� With aberrant left subclavian artery� With mirror-image branching and right ligamentum

arteriosum* Left-sided aortic arch� With aberrant right subclavian artery and right

ligamentum arteriosum� Right-sided descending aorta with right ligamentum

arteriosum* Cervical aortic arch

� Absent pulmonary valve syndrome� Aberrant left pulmonary artery (’pulmonary artery sling’)� Acquired cardiovascular disease

* Dilated cardiomyopathy* Aneurysm� Ascending aorta* Ductus arteriosus

compression in childhood carries a significant morbidityand mortality.7,8

Interventional radiology is limited in what it has to offerthese children. Stenting is only appropriate when surgicalcorrection is not possible. In our practice the usual indica-tion is a palliative setting as it allows a child to be extubated.

IMAGING TECHNIQUES

Different imaging approaches may be appropriate fordifferent causes of vascular compression of the airway.The historical approach was to perform chest radiographyand barium swallow (upper gastrointestinal study) to eval-uate children with suspected extrinsic compression of theairway. This has now been replaced in most centres bymultidetector computed tomography (MDCT) and mag-netic resonance imaging (MRI).

The barium swallow is probably acceptably accurate fordiagnosis of a vascular ring, but does not delineate theprecise anatomy required for surgical planning3 and isusually not helpful for bronchial compression.

The diagnostic imaging pathway in our centre startswith echocardiography and flexible bronchoscopy (com-bined with bronchography when appropriate). Weusually also perform either MDCT or MRI, and occa-sionally both.7,9 These are the most useful radiologicaltechniques as they provide information about the tra-cheobronchial tree, the cardiovascular structures andtheir relationship to each other. A limitation of MDCTand MRI is that obliterated vascular segments (e.g. theligamentum arteriosum or an atretic aortic arch) cannotbe directly visualized.4

Diagnostic catheter angiography is now obsolescent inthis context and has largely been replaced by cross-sectional

imaging.3,10 Chest radiographs may show aortic arch anoma-lies and dilated pulmonary arteries; however, althoughairway compression can sometimes be seen,11 a compre-hensive assessment is not possible. Radiographs may,however, show hyperinflation, collapse or other lungpathology.

Echocardiography

This is essential for the evaluation of associated conge-nital heart disease (CHD) and usually clearly showsabnormal vascular structures. Echocardiography aidsthe surgeon in understanding complex three-dimensional(3D) relationships. Direct evaluation of airway compres-sion is limited.1

Multidetector computed tomography

The development of MDCT technology has greatlyextended the applications of chest CT in children.12 Scantimes for MDCT are much shorter than for MRI and spatialresolution is higher.7 Axial MDCT data are generally suffi-cient to diagnose the type and severity of airway compres-sion, but multiplanar reconstruction and 3D volumerendered images may provide further useful information.13

Virtual bronchoscopy images can also be generated fromMDCT data but rarely add diagnostic information12 andcannot yet be used as a substitute for bronchoscopy. Theyserve mainly as a method of conveying some informationabout the airway to clinicians who are familiar with this typeof view.

The main disadvantage of MDCT is exposure of thepatient to ionizing radiation at the age of greatest sensitivityto its carcinogenic effects. Scan parameters should bemodified from adult protocols to reduce the radiationdose. This can be achieved using weight-based protocols13

or tube current modulation techniques. When carefultechnique is used, the effective dose for a CT study ofthe chest should be <3 mSv, which is comparable to 200frontal chest radiographs.12

Magnetic resonance imaging

MRI has excellent intrinsic contrast resolution and multi-planar imaging capabilities.13 Evaluation of cardiac anatomyand physiology with MRI is usually superior to CT. How-ever, most MRI studies for vascular compression will bequite prolonged (>30 min), requiring sedation or generalanaesthesia in young children. Sedation risks for childrenwith a compromised airway are significant.1,13 The scantimes are often considered to be too long for haemody-namically unstable patients,7 especially given the relativeinaccessibility of the MRI scanner.14 In practice, the increasein speed and quality of multiplanar reconstructions pro-vided by MDCT technology means that CT is used moreoften than MRI in most centres.7,15

VASCULAR COMPRESSION OF THE AIRWAY 87

Bronchoscopy and bronchography

Cross-sectional imaging is probably much better thanbronchoscopy at determining the nature of vascular com-pression of the airway. Current MDCT and MRI techniquesdo not reliably distinguish between dynamic or staticnarrowing.14,15 This is a very important practical point asmany children with prolonged airway compression developsecondary malacia. Although improvements in scanningtechnique are likely to overcome this problem, broncho-scopy and bronchography are currently the best techniquesfor this purpose. We usually perform these examinations atthe same time, injecting isotonic non-ionic contrast (iotro-lan [Isovist-240], Schering, Burgess Hill, UK) down theworking channel of a flexible bronchoscope or througha separate catheter.16 Malacia should only be assessedwhen the patient is breathing spontaneously.

Interventional radiology

The role of metal stents in the airway of children remainscontroversial. We prefer to restrict their use to twoindications: palliation and when all other potential treat-ments have failed. Stenting with palliative intent may beused to allow a child to be extubated, so he/she can die athome. Our experience has been very favourable in thiscontext, with several children surviving much longer thanexpected with a good quality of life, e.g. those with acquiredcardiomyopathy. In other patients, however, it is sensible tobe cautious.

Correct stent selection is important for children withvascular compression of the airway.16 Balloon expandablestents, such as the Palmaz stent (Cordis Europa N.V.,Roden, The Netherlands), are rigid and this can lead toerosion through the wall of the airway. Self-expandingstents are much more flexible and less likely to lead tovascular erosion.16 They are also less likely to fracture whenthere is severe compression, e.g. in dilated cardiomyopathy.The major problems with this type of stent are that they aredifficult to remove and cannot easily be post-dilated toallow for future growth.

We usually perform stent insertion under broncho-graphic control in an angiography suite. The advantageover rigid bronchoscopy is that the whole stent can bevisualized on fluoroscopy, ensuring the it does not moveduring deployment and allowing the operator to position itas close as possible to a bifurcation without covering abronchial orifice. The procedure is performed under gen-eral anaesthesia with muscle relaxation. We usually per-form flexible bronchoscopy initially to confirm thediagnosis. Contrast is then injected either through thebronchoscope or through a small angle-tip catheter. Whena bronchographic roadmap of the compressed area hasbeen obtained, a guidewire is then passed into a peripheralbronchus. The stent delivery device is passed over the wireand the stent is deployed under fluoroscopic control. The

stent can be repositioned during the initial part of deploy-ment, if required. The final stent position can be checkedwith flexible bronchoscopy if necessary.

Covered, retrievable self-expanding stents are availablefor use in the airway. These may be easier to deploy thansilicone (Dumon) stents, but can be expected to havesimilar disadvantages such as stent migration and blockagecaused by the covering of the stent inhibiting ciliary clear-ance of secretions from the lungs. It is possible that someform of absorbable stent will be available in the future, inwhich case the indications for stent insertion may broadensignificantly.

DOUBLE AORTIC ARCH

Autopsy studies suggest that 3% of people have a con-genital malformation of the aortic arch, but most remainundiagnosed throughout life.3 Double aortic arch (DAA) isthe most common cause of vascular compression of theairway in children.3,4,9,17,18 DAA is defined by the presenceof both left- and right-sided aortic arches, which togethersurround the trachea and oesophagus (Fig. 1). The rightarch is usually larger (‘dominant’). The left arch is usuallysmall (‘hypoplastic’) or forms a fibrous cord (‘atretic’ seg-ment) beyond the origin of the left common carotid orsubclavian artery.1,19 The fibrous cord tethers the patentpart of the left-sided arch to the descending aorta, com-pleting the ring. A ductal ligament may also be relevant inthe creation of a ring at this point, since the ligament oftenconnects the distal left arch to the proximal left pulmonaryartery. Schlesinger et al. reviewed the MRI and CT findingsof DAA with an atretic left arch.19 They found that thefibrous cord could not be seen on imaging and that thediagnosis was made from the presence of an incompleteleft arch.

The descending aorta may be left- or right-sided, or mayrun in the midline anterior to the vertebral column. Whenthe descending aorta is midline, the structures of themediastinum are said to be ‘stacked’ abnormally, resultingin compression between the spinal column and the ster-num.15

Children with DAA usually present in infancy,1 withsymptoms including dysphagia, stridor, wheezing andrespiratory distress.10 Surgical correction, usually by trans-ection of the non-dominant arch, is required to relieve theairway compression.9 It is always important to diagnose thearch anatomy before surgery because this determines theoperative approach.

About 30% of children have residual symptoms despitesurgical treatment of DAA.15 Although Fleck et al. foundthat residual symptoms may be due to persistent airwaycompression, some children have severe malacia of thelower trachea (extending to the carina).15 This maydevelop as a secondary effect of prolonged severe extrinsiccompression. In our experience, in most children thisproblem is self-limiting, and eventually their airway cartilage

88 C. A. MCLAREN ET AL.

Figure 1 Persistent airway compression in a 20-month-old boy following surgery for double aortic arch. (a) 3D CT reconstruction(posterosuperior view) shows the divided left arch and surgical clips (white arrows). The right arch is indicated by a red arrow. (b)Compression is most severe where the descending aorta (arrow) crosses from right to left behind the airway. (c) Frontal bronchographyshows a right-sided impression on the trachea from the right aortic arch. (d) Lateral bronchography shows posterior compression by thedescending aorta (arrows). (e) 2.2-mm flexible bronchoscopy shows extrinsic compression at the level of the carina.

VASCULAR COMPRESSION OF THE AIRWAY 89

regains sufficient stiffness for the symptoms to resolve.Although an occasional patient requires tracheostomyduring this period, we have never had to insert an airwaystent in a child with DAA. Detailed studies of late respira-tory function are required in this group.

INTERRUPTED AORTIC ARCH

Interrupted aortic arch (IAA), in which some part of thelumen of the aortic arch is discontinuous, is found in about1% of all children with CHD. Children typically present asneonates and, if untreated, usually die by the age of 10days.20 In type A (30–40%) the arch is interrupted betweenthe origin of the left subclavian artery and the ductusarteriosus. In type B (50–55%) the interruption occursbetween the origins of the left common carotid arteryand the left subclavian artery. Type C (interruption betweenthe innominate artery and the left common carotid artery)is very uncommon. In all types, deoxygenated blood flowsto the lower part of the body through an enlarged ductusarteriosus. Over 95% of children have associated cardiacanomalies.20,21

Airway compression in IAA is a consequence of surgicalrepair rather than the malformation itself. Shortening of thearch is inherent in the procedure, in which an end-to-endanastomosis is performed between the ascending anddescending aorta, irrespective of the type of IAA. Thisresults in anterior displacement of the descending aorta,posterior displacement of the anterior aorta and conse-quent compression of the left main bronchus (LMB)between the aorta and the left pulmonary artery (Fig. 2).Surgical correction of this compression is technically chal-lenging22 and further surgery may be required as the childgrows. Tracheostomy with pressure support may fail toovercome the LMB compression, and excessive continuouspositive airway pressure may even damage the right lung. Insome children, therefore, it may be justified to use metalairway stents, despite the risks. As noted above, balloon-

Figure 2 A 4-month-old girl with compression of the left mainbronchus following surgery for interrupted aortic arch. Axial CT ofthe thorax showing compression in the midline by the descendingaorta (arrow).

expandable and self-expanding designs each have advan-tages and disadvantages, and the choice of stent will dependon individual clinical factors.16,23

RIGHT-SIDED AORTIC ARCH WITHABERRANT LEFT SUBCLAVIANARTERY

Right-sided aortic arch (RAA) with an aberrant left sub-clavian artery and/or a left ligamentum arteriosum isreported in 12–25% of children with vascular rings. Mostpatients with these anomalies are asymptomatic.24

There are two main patterns of origin of the greatarteries to the head and upper limbs in RAA. In ‘mirror-image branching’, a left brachiocephalic trunk (innominateartery) arises first, followed by the right common carotidand the right subclavian arteries. In RAA with aberrant left

Figure 3 A 5-year-old boy with a right-sided aortic arch andaberrant left subclavian artery. (a) Axial CT of the thorax showingcompression of the trachea by the aberrant artery (arrow). (b) 3Dvolume rendered image (posterior view) shows the right-sidedaortic arch (red arrow) and the aberrant left subclavian artery(white arrow) arising from the descending aorta.

90 C. A. MCLAREN ET AL.

subclavian artery (Fig. 3) the first branch is the left commoncarotid, followed by the right common carotid and rightsubclavian arteries. The aberrant left subclavian artery arisesfrom the descending aorta and passes behind the oeso-phagus. The vascular ring is completed by the left ligamen-tum arteriosum, which passes from the origin of the leftsubclavian artery to the left pulmonary artery.1 The leftsubclavian artery often originates from an outpouching ofthe descending aorta, called a Kommerell’s diverticulum.Airway compression in RAA is usually due to enlargementof the Kommerell’s diverticulum, a short (tight) ligamentumarteriosum or a midline descending aorta.24 Relief ofsymptoms is usually achieved by transection of the liga-mentum arteriosum,24 but it is very important also to excisethe diverticulum since this can result in late compression ofthe oesophagus and/or trachea.

Late diagnosis of this condition does occur, particularly inpatients with a Kommerell’s diverticulum.

ABSENT PULMONARY VALVESYNDROME

Absent pulmonary valve syndrome (APVS) is characterizedby the presence of enlarged pulmonary arteries and hypo-plastic pulmonary valve cusps. It is most often seen inassociation with ventricular septal defect (VSD) and rightventricular outflow tract obstruction (RVOTO). APVSoccurs in 3–6% of patients with tetralogy of Fallot, butcan also be seen in isolation, with an intact ventricularseptum and other congenital anomalies.25,26 There is astrong association with DiGeorge syndrome (chromosome22q11 deletion).11

The characteristic pattern of compression of the lowertrachea, LMB and right main bronchus or bronchus inter-medius (Fig. 4) is caused by enlargement of the pulmonaryarteries and left atrium.1,11,25

The prognosis in APVS depends on the age at pre-sentation and the severity of symptoms. Neonates andinfants often present with severe cardiorespiratory com-promise.26 Mortality is between 16% and 56% in this group,with an especially poor prognosis in those infants whorequire mechanical ventilation.11,25,26 Children who pre-sent later often have milder symptoms, and surgery may beperformed on an elective basis, usually simply by repairingthe Fallot component of the defect.26

In small babies, there remains some controversyabout which surgical technique is best to relieve thetracheobronchial compression. The usual approachinvolves replacement of the pulmonary artery with con-duits or reduction arterioplasty.26,27 However, the addi-tion of the Lecompte manœuvre (transecting the aorta,allowing the pulmonary arteries to lie anterior to thereconstructed aorta) has proved successful. Any asso-ciated cardiac anomalies are corrected at the sameoperation.25–27

If possible, airway stenting is avoided in APVS because ofthe risk of erosion of a pulmonary artery with fatal hae-morrhage. This risk can theoretically be minimized by theuse of self-expanding stents, but the potential to dilatethese stents to allow for future growth is very limited.Stenting is therefore restricted to palliative indications orwhen all surgical options have been exhausted. Bothballoon-expandable28,29 and self-expanding stents30 havebeen used.

OTHER VASCULAR CAUSES OFAIRWAY COMPRESSION

Pulmonary artery sling

In this condition the left pulmonary artery has an anom-alous course, arising from the posterior aspect of the rightpulmonary artery and passing between the lower tracheaand the oesophagus to enter the hilum of the left lung.There is a strong association with long segment congenitaltracheal stenosis (LSCTS) with complete tracheal carti-lage rings. The literature usually states that LSCTS ispresent in 50% of children with pulmonary artery sling(PAS), but in our experience the proportion is muchhigher than this, and a diagnosis of airway compressiondue to PAS without LSCTS should be treated withsuspicion.31 Non-invasive imaging is not adequate toevaluate for complete rings and good quality broncho-scopy is mandatory. The distinction is clinically important,because it seems that the prognosis is much better if theLSCTS is repaired at the same time as the left pulmonaryartery is re-implanted.31 Slide tracheoplasty is now thetreatment of choice for LSCTS.

Innominate artery compression

Anterior compression of the trachea by the brachioce-phalic trunk (innominate artery) is a controversial entity.First, this condition seems to have been over-diagnosedand possibly over-treated in the past. Second, in manypatients said to have innominate artery compression(IAC) the main pathophysiological mechanism appearsto be tracheomalacia rather than extrinsic compression.32

This is particularly true in children with oesophagealatresia. Third, a large proportion of normal infants haveimaging evidence of an anterior impression on the tracheaat the level where it is crossed by the innominate artery. Inone study, an anterior impression on the tracheal aircolumn was seen on lateral chest radiographs in 30% ofchildren younger than 2 years of age.33This finding is lesscommon in older children.33,34 Nevertheless, there doappear to be some children in whom arteriopexy,35 re-implantation or even transection of the innominateartery36 is beneficial.

A high (cervical) aortic arch may also compress thetrachea, but this is extremely rare.

VASCULAR COMPRESSION OF THE AIRWAY 91

Figure 4 Tetralogy of Fallot and absent pulmonary valve syndrome with airway compression in a 15-month-old boy. (a) CT volumerendered image shows compression of the left main bronchus (arrow). (b) Axial CT of the thorax shows severe compression of the airwaybetween the vertebral body and the grossly enlarged pulmonary arteries. (c) Lateral bronchogram confirms severe anterior compressionof the airway caused by the enlarged pulmonary arteries. (d) 2.2-mm flexible bronchoscopy shows canal compression.

92 C. A. MCLAREN ET AL.

Figure 5 A 12-month-old girl, ventilator-dependent, with dilated cardiomyopathy. (a) 2.2-mm flexible bronchoscopy showscompression of the left main bronchus (arrow). (b) On bronchography the left main bronchus is completely occluded with no passageof contrast into the left lung. (c) A self-expanding stent has been deployed. (d) Bronchoscopy shows good position of the stent, withpatency of the left main bronchus.

Left-sided aortic arch with aberrant rightsubclavian artery

This relatively common anomaly does not lead to airwaycompression unless there is a right-sided ligamentum arter-iosum.3

Acquired cardiovascular disease

Dilated cardiomyopathy causes airway obstruction whenthe left atrium is sufficiently dilated to compress the LMB.This may lead to chronic infection of the left lung andprogressive deterioration in the child’s clinical condition. If

VASCULAR COMPRESSION OF THE AIRWAY 93

other treatments are unsuccessful, the insertion of a self-expanding stent in the LMB may be effective (Fig. 5).

Aneurysm of the aorta or ductus arteriosus is a very rarecause of airway compression in childhood.37

CONCLUSION

Imaging plays a crucial role in the diagnosis and treatment ofvascular compression of the airway in children. In mostcentres, MDCT and MRI are increasingly used for theevaluation of children with suspected vascular compression,replacing barium swallow studies and catheter angiography.Although we agree with this change in imaging strategy,these techniques still have some limitations. Both MRI andMDCT are at present inadequate to differentiate reliablybetween dynamic airway obstruction (caused by tracheo-bronchomalacia) and fixed airway obstruction, as seen inextrinsic vascular compression. This is important as thepresence and the severity of tracheobronchomalacia arestrongly related to patient outcome. In addition, MDCT andMRI cannot yet identify complete tracheal rings. Stentinsertion is only useful in selected patients with airwaycompression, but it may be appropriate for patients who failall other forms of treatment.

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