vascular anomalies compressing the oesophagus andtrachea · aortic arch had an exploratory...

T7horax (1969), 24, 295.

Vascular anomalies compressing the oesophagusand trachea

J. C. R. LINCOLN, P. B. DEVERALL, J. STARK,E. ABERDEEN, AND D. J. WATERSTON

From the Hospital for Sick Children, Great Ormond Street, London W.C.I

Vascular rings formed by anomalies of major arteries can compress the trachea and oesophagusso much as to cause respiratory distress and dysphagia. Twenty-nine patients with this conditionare reviewed and discussed in five groups. The symptoms and signs are noted. Radiologicalexamination by barium swallow is the most useful diagnostic aid. Symptoms can only berelieved by operation. The trachea is often deformed at the site of the constricting ring. Onlyinfrequently is there immediate relief from the pre-operative symptoms. Two babies were

successfully treated for an aberrant left pulmonary artery.

The diagnosis and treatment of major arterialanomalies which cause compression of the oeso-phagus and trachea are now well established.Although an aberrant right subclavian artery wasdescribed in 1794 by Bayford (Fig. 1), who gavethe detailed post-mortem finding in a woman whohad died from starvation secondary to thisanomaly, and a double aortic arch was describedby von Siebold (1837), it was not until 1945 thatGross successfully divided a double aortic arch ina 4-month-old infant. Following this success thediagnosis and treatment of this and similar anom-alies became firmly established.

MATERIAL

The surgical experience will be discussed of 29patients treated consecutively during a 15-yearperiod from 1952 to 1967 at the Hospital for SickChildren, Great Ormond Street, London. Theembryology and classification of suoh anomaliesare not discussed because we have nothing to addto previous papers (Edwards, 1959; Stewart,Kincaid, and Edwards, 1964; Gross and Ware,1946).The anomalies are discussed under the follow-

ing headings:Double aortic archRight aortic arch in conjunction with a left

ligamentum arteriosum or persistent ductusarteriosusAberrant subclavian arteryAberrant left pulmonary arteryAbnormally placed innominate artery

DOUBLE AORTIC ARCH Nineteen children weretreated for some form of double aortic arch. Theirage at operation ranged from 1 week to 11 months,but the majority were treated at about the age of5 to 6 months (Fig. 2). Frequently the presentingsymptoms had been noticed since birth but forvarying reasons there was delay in making thecorrect diagnosis. Stridor, dysphagia or poor feed-ing, cyanotic attacks, and recurrent respiratorytract infections were the four main presentingsymptoms. All but two patients had respiratorystridor and in seven of these children there wasconcomitant dysphagia or poor feeding. Sixchildren had frequent respiratory tract infections.Initially, some children had bronohoscopy per-formed in the mistaken belief that they hadprimary laryngeal or tracheal disease. Plain radio-graphs of the chest were always taken and wereusually non-contributory. Barium swallow enabledthe correct diagnosis to be made in all patientsin whom this was performed (Fig. 3a,b). In onepatient with suspected heart disease angiographywas also performed.A right descending thoracic aorta was found in

13 patients, and in 11 of these the major archwas posterior, in one the major arch was anterior,and in one the arches were equal in diameter(Fig. 4). In a 9-day-old baby with a major pos-terior arch, the small anterior arch joined not thedescending aorta but the middle of a patent ductusarteriosus (Fig. 5).A left descending thoracic aorta was found in

six patients, the main arch being posterior in threeand anterior in three (Fig. 6). Eight patients had

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J. C. R. Lincoln, P. B. Deverall, J. Stark, E. Aberdeen, and D. J. Waterston

FIG. 1. An aberrant right subelavian artery. A drawing of the necropsy specimen describedby David Bayford at a meeting ofthe Medical Society ofLondon, 2 July 1787. In this patientthe aberrant artery passed between the oesophagus and the trachea.

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Vascular anomalies compressing the oesophagus and trachea

a patent ductus arteriosus and 11 a ligamentumarteriosum, al being on the left side.

Associated cardiovascular abnormalities werefound in three patients. In one there was a ven-tricular septal defect and a left superior vena cava;

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patient died when ventricular fibrillation occurredas the chest was opened.The anterior arch was divided distal to the

origin of the left subclavian artery and, as this wasfrequently the smaller of the two arches, it wasusually divided. A duct or ligament was alwayslooked for and divided, and the trachea andoesophagus were dissected out to free these struc-tures from constricting fibrous tissue.

O 1 2 3 4 5 6 7 8 9 10 11Aqe at operation (months)

FIG. 2. Age at operation of 19 patients with double aorticarch.

in another an aberrant left subclavian arteryassociated with a right arch; and in a third therewas a persistent truncus arteriosus (Figs 7 and 8).The baby with the truncus arteriosus and doubleaortic arch had an exploratory thoracotomy butthe vascular ring was very loose and was notdivided. This child died four months after theoperation in congestive cardiac failure. Another

(b)FHG. 3. Double aortic arch. Postero-anterior (a) andlateral (b) views of a barium swallow. There are bilateralconcave indentations of the oesophagus at the level of theaortic arch. In the lateral view the oesophagus is indentedfrom behind.

Post-operative follow-up showed that in onlytwo patients was there immediate complete relieffrom the preoperative signs, some degree ofstridor being nearly always present in the firstpostoperative week, but then resolving. Respira-tory signs became worse after the operation infive patients within 10 weeks, one requiring atracheostomy and the other nasotracheal intuba-tion.

RIGHT AORTIC ARCH WITH LEFT LIGAMENTUMARTERIOSUM OR PATENT DUCTUS ARTERIOSUSCompression of the oesophagus and trachea by avascular ring comprising a right aortic arch andFIG. 3a

z

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298 J. C. R. Lincoln, P. B. Deverall, J. Stark, E. Aberdeen, and D. J. Waterston

either a patent ductus arteriosus or a ligamentumarteriosum occurred in four patients. These child-ren tended to be older than those with a doubleaortic arch, the youngest being two months andthe oldest three years of age. All had stridor, andin three there was concomitant difficulty withfeeding; this was so in the 3-year-old child. Re-current respiratory tract inifection occurred in allbut one of the children. Plain radiographs of thechest were useful; evidence of a right arch wasnoted in all, and in the 3-year-old child the tracheaat the site of constriction was seen to be narrowed

FIG. 6. A double aortic arch with a left descendingthoracic aorta. The major arch is posterior. The anteriorarch is waisted between the innominate and left commoncarotid arteries.

FIG. 4. A double aortic arch with a right descendingthoracic aorta. The major arch is posterior.

IFIG. 7. A persistent truncus arteriosus with a doubleaortic arch. There is a right descending thoracic aorta.The innominate artery arises from the posterior arch, andthe left common carotid and the left subclavian arteriesarise from the anterior arch. Only the right pulmonaryartery is seen.

FIG. 5. A double aortic arch. The minor arch is anteriorand terminates by joining a patent ductus arteriosus.

in an oblique view (Fig. 9). Barium swallow washelpful in all cases; the oesophagus was indentedby the right arch of the aorta and on the leftside by the ligament or duct (Fig. lOa, b).

Associated congenital cardiac anomalies werenoted in two patients. One had a ventricular septaldefect and the other a persistent left superior venacava and an aberrant left subclavian artery, whicharose from a partly patent ligamentum arterio-sum (Fig. 11).

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FIG. 8. Persistent truncus arteriosus with double aortic arch. Angiogram showsa right pulmonary artery emerging from the main aortic trunk. The two aorticarches join to form a common right descending thoracic aorta.

FIG. 9. Right aortic arch with persistent ligamentum arteriosum. Oblique,plain radiograph of chest. The trachea is narrowed in its middle third.

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J. C. R. Lincoln, P. B. Deverall, J. Stark, E. Aberdeen, and D. J. Waterston

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FIG. 1l. Right aortic arch with part ligamentum arterio-sum and part patent ductus arteriosus. The left subelavianartery arises from the patent ductus arteriosus.

At operation a patent ductus arteriosus waspresent in two patients and a ligamentumarteriosum in the other two. All four had a rightdescending thoracic aorta (Fig. 12). The ligamentor duct was divided and the tissues surroundingthe trachea and oesophagus were dissected out aspreviously described. One patient had acute airwayobstruction at operation and had a cardiac arrest.During resuscitation procedures the aortic end ofthe patent ductus arteriosus slipped from thevascular clamp and receded into the right chest,allowing fatal haemorrhage.

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FIG. 10. Right aortic arch with persistent ligamentumarteriosum. Postero-anterior (a) and lateral (b) radiographsofa barium swallow. The main arch of the aorta is seen tothe right and indenting the oesophagus. The posterior defectseen in the lateral view is produced mainly by the ligamen-tum arteriosum and partly by the arch of the aorta.

FIG. 12. A right aortic arch with a right descendingthoracic aorta. There is a persistent ligamentum arteriosumcausing narrowing of the trachea and oesophagus.

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(a) (b)FIG. 13. Aberrant right subclavian artery. Postero-anterior (a) and lateral (b) radiographs of a barium swallow, showinga concave posterior indentation of the upper third of the oesophagus by an aberrant right subclavian artery. This vesselpasses upwards and obliquely at an angle of about 65°.

Complete relief from stridor was achieved bythe fifth post-operative day, and in three cases therewas immediate relief.

FIG. 14. An aberrant right subclavian artery, passingobliquely upwards and posterior to the oesophagus.

ABERRANT SUBCLAVIAN ARTERY Despite the fre-quency of aberrant subclavian arteries (Edwards,1953; Stewart et al., 1964) the number of patientsseen with symptoms or signs was small-fourpatients (ages 2 to 8 months). In one child onlywasthis the sole vascular anomaly, the others beingfound in association with a double aortic arch, atetralogy of Falilot, and a right aortic arch andleft patent ductus arterosus. One child, a poorfeeder, had a typical indentation of the posterioraspect of the upper third of the oesophagusdemonstrated at barium swallow (Fig. 13a, b).This baby had the aberrant right subclavian arterydivided at its origin and subsequently improved(Fig. 14). The other children had no symptoms.One infant had an aberrant left subclavian arterywhich passed posterior to the oesophagus, havingarisen from a right aortic arch (Fig. 15). This wasfound when performing a left Blalock-Taussigshunt.ANOMALOUS LEFT PULMONARY ARTERY Thisanomaly has been seen in three babies, their agesat operation being 1 month, 5 months, and 8months. These patients presented with inspiratory

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FIG. 15. An aberrant left subelavian artery arising froma right aortic arch.

and expiratory stridor, and in the 5-month-oldchild there was persistent right upper lobe col-lapse. There was no difficulty with feeding. Theradiological and clinical evidence of right upper

lobe collapse caused some confusion until abarium swallow was performed and the correctdiagnosis was made. The aberrant artery com-pressed the right upper lobe bronchus. Theclassical concave indentation of the anterior walloesophagus at the level of the carina was seen inall three patients (Fig. 16). However, the signi-ficance of this sign was missed in the 8-month-oldbaby. Angiography was performed in the 1-month-old baby but was not clinically essential(Fig. 17).The 8-month-old baby was admitted with acute

airway obstruction, a tracheostomy having beencarried out at the referring hospital-but withlittle relief. The baby was bronchoscoped onadmission and a concentric narrowing of thetrachea was seen 2-5 cm. below the tracheostomyopening; the trachea was indented from the rightside. A small polythene catheter was with diffi-culty threaded into the left main bronchus andthe airway was somewhat improved. Plain radio-graphs of the chest showed a large anterior medi-astinal shadow.With a presumptive diagnosis of a mediastinal

mass narrowing the trachea a right thoracotomy

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Trachea

..evel ()tariniaFIG. 16. Anomalous left pulmonaryartery. Lateral radiograph of a bariumswallow showing a concave indentationof the oesophagus at the level of thecarina.

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was performed and an aberrant left pulmonaryartery was found. It passed posteriorly and thento the right of the superior tracheo-bronchialangle, and then passed posterior to the tracheaand onward to the left pulmonary hilum. The air-way obstruction increased and therefore thetrachea was dissected out and found to be hypo-plastic and solid with complete cartilagenous rings.The aberrant pulmonary artery was then dissectedout and fascial bands were divided, but even whenthis artery was retracted away from the tracheathere was no relief of the airway obstruction. Anattempt was made to dilate the trachea from belowthrough a second tracheostomy. As the baby'scondition was perilous no attempt was made tore-implant the aberrant pulmonary artery and the

chest was closed. The baby died. Post-mortemexamination showed a congenital abnormality ofthe trachea with complete ring cartilages.The correct diagnosis was made pre-operatively

in the other two patients and at left thoracotomythe route of the aberrant artery was identical inboth. The artery arose from the terminal part ofan elongated main pulmonary artery, passed pos-teriorly around the right superior tracheo-bronohial angle, and then onto the posterior sur-face of the trachea, being anterior to theoseophagus, and so to the left lung (Fig. 18). Theartery was dissected out and divided from themain pulmonary artery. The artery was broughtanterior to the trachea. In both patients the leftpulmonary artery was anastomosed to the side of

L. PulmonaryArtery

Main Pulmonary-Artery ;,;:

R. PulmonaryArtery

FIG. 17. Anomalous left pulmonary artery. A pulmonary angiogram showing anelongated and deviated left pulmonary artery.

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the main pulmonary artery because when attempt-ing to re-anastomose at the site of transectionexcessive kinking occurred.

TABLETOTAL NUMBER OF ANOMALIES IN 29 PATIENTS

Total Age Group Death

Type of Anomaly no f M F (yrs.) inAnoma- Hos-lies 0- 1 1-2 > 2 pital

Double aortic arch.. 19 10 9 19 - -

Right arch + left liga-mentum arteriosumorP.D.A. 4 2 2 2 1 1Aberrant subclavianartery .. 4 2 2 4Anomalous left pul-monary artery 3 3 - 3Abnormally placedinnominate artery.. I - 1

In one infant excessive tracheo-bronchialsecretions necessitated a tracheostomy. Bothbabies made an uninterrupted recovery with rapidrelief of their symptoms.

ABNORMALLY PLACED INNOMINATE ARTERY In one

3-year-old child the innominate artery arose so far

to the left on the aortic arch that it compressedthe trachea (Fig. 19). She fed poorly from birthand inspiratory and expiratory stridor with costalrecession was marked. Plain radiographs of thechest and a barium swallow showed no abnor-mality.

Bronchoscopy showed indentation of thetrachea anteriorly and proximal to the carina. Onpressing the tip of the bronchoscope against theindentation, the right radial pulse diminished involume (Mustard, Trimble, and Trusler, 1962).At thoracotomy (through the third left inter-

costal space) the aortic arch was slung forward bysutures attacheed to the back of the sternum andthe pericardial reflection. Following this thestridor greatly improved.

DISCUSSION

SIGNS AND SYMPTOMS The clinical picture ofmajor vascular anomalies which compress thetrachea and oesophagus varies according to thetype of anomaly present. In the patients with a

FIG. 18. An anomalous left pulmonary artery, smaller in diameter than the rightpulmonary artery. This artery passes to the right of the right superior tracheo-bronchial angle and posterior to the trachea, and indents the anterior part of theoesophagus at the level of the carina.

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double aortic arch, right arch with ligamentumarteriosum or persistent ductus arteriosus, aber-rant left pulmonary artery and abnormally placedinnominate artery, the signs were well marked,whereas many aberrant subclavian arteries oftencause no signs. The main signs of a 'vascular ring'are stridor, respiratory embarrassment, dysphagiaor p3oo feeding, and recurrent respiratory tractinfection. These were present together or separ-ately. The respiratory tract infections followedspill-over of food and subsequent inhalation.

Children with a right aortic arch, in conjunc-tion with a left duct or ligament and aberrantsubclavian artery, presented later in life althoughnot conspicuously so in our experience. The otheranomalies usually presented in the first year oflife.

DIAGNOSIS Primary laryngeal and tracheal con-genital deformities, pressure on the trachea fromlymphadenopathy, and congenital oesophagealnarrowing were considered in the differential diag-nosis.

INVESTIGATIONS Accurate barium swaJiow wasthe most certain and, in many of the anomalies,the only method of making the diagnosis. Plainradiographs of the chest had only been informativein that group of patients with a right aortic archin combination with a left duct or ligament. Thisanomaly should be suspected when on postero-anterior radiographs the aortic knuckle is absentfrom the usual site and the aortic arch is to theright of the mid line (Neuhauser, 1946). Obliquefilms showed a well-marked narrowing of thetrachea in one child.

Barium swallow was the most useful radio-logical aid to the diagnosis. With the double aorticarch the oesophagus was narrowed by lateral pres-sure of the anterior and posterior aortic arches.The patients with a right arch and persistent leftligamentum arteriosum and patent ductus arteri-osus showed a well-marked indentation of the rightside of the oesophagus and some indentation of thzleft side of the oesophagus. This was most markedon screening when the right-sided indentation pul-sated. An aberrant subclavian artery was demon-strated by indentation of the posterior aspect ofthe upper one-third of the barium-filled oeso-phag,us when seen in the lateral view (onlyoccasionally does the artery go between the oeso-phagus and trachea, and we have had no experienceof this). When the left pulmonary artery wasanomalous, a concave indentation was seen in theoesophagus at the level of the carina.

It was rarely necessary to perform angiographyto confirm the diagnosis and this was carried outon two occasions when congenital heart diseasewas present. Tracheograms were not performed inany instance.Endoscopic examination of the larynx, trachea,

and oesophagus did not give sufficient positive ornegative information to warrant its frequent use.Bronchoscopy could be dangerous in a small babywith an already critically narrowed trachea.

OPERATIVE TREATMENT A full left lateral thora-cotomy was found to give the best exposure. Inthe only case in which a right thoracotomy wasperformed the anatomy could not be adequatelydefined and a left thoracotomy had to be per-formed.

FIG. 19. An innominate artery arising from the aorticarch further to the left than is usual, thus pressing on thetrachea.

A period of trial occlusion was carried out on

each arch separately prior to division of the arch.In this series the anterior arch was the arch mostfrequently divided. When the posterior arch was

the smaller of the two it was divided at its junc-tion with the descending thoracic aorta, and whenthe anterior arch was the smaller of the arches itwas usually divided distal to the origin of the leftsubclavian artery or sometimes between the leftcommon carotid artery and the left subclavianartery if a definite narrowing was present at thispoint (Fig. 6). The combination of a persistenttruncus arteriosus and a double aortic arch is un-comrmon, and only one other example was foundin the literature (Kerwin, 1936). Both arches were

equal in diameter and neither was divided as they

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306 J. C. R. Lincoln, P. B. Deverall, J. Stark, E. Aberdeen, and D. J. Waiterston

appeared to be very loose and not constrictingthe trachea or oesophagus.A patent ductus arteriosus or ligamentum

arteriosum was always divided whenever present.The experience of aberrant subclavian arteries

was small. It appears that this artery can bedivided with impunity but the possibility of pre-cipitating a subclavian steal syndrome should beremembered (Reivich, Holling, Roberts, andToole, 1961).When correcting an aberrant left pulmonary

artery, rerouting this artery so that it lies in frontof the left bronchus gave no problems, but itsreanastomosis to the site of transection proveddifficult because of kinking. It was necessary toreimplant the pulmonary artery into the side wallof the main pulmonary artery.

Dissection around the trachea and oesophaguswas made in all cases to divide all constrictingfascial bands.

Patients were nursed in maximum humidity inan oxygen tent and frequent oropharyngeal toiletwas performed. Tracheostomy was performedpostoperatively on two occasions for respiratorydifficulty, but this was (and should be) avoided innearly all cases. The tracheal rings are deformedand still partially obstruct the airway at the pre-vious site of constriction for some weeks or evenmonths after operation.

RESULTS

The ages, the variety of anomalies, and the deathsthat occurred in hospital are shown in the Table.The immediate response to surgical correctionwas variable, particularly in those patients with a

double aortic arch. In only 6 of 29 patients wasthere immediate postoperative relief from stridor,and, of these, three had relief of a right arch andleft ligamentum or duct, one an aberrant sub-clavian artery, and two were treated for doubleaortic arch. Occasionally the symptoms becameworse during the first week after operation. Fromone to 12 weeks elapsed before freedom fromstridor was noted.

We wish to thank our clinical colleagues for helpin managing these patients. We also wish to thankthe Department of Medical Illustration, Institute ofChild Health.

REFERENCESBayford, D. (1794). An account of a singular case of obstructed

deglutition. Mem. med. Soc. Lond., 2, 275.Edwards, F. R. (1959). Vascular compression of the trachea and oeso-

phagus. Thorax, 14, 187.Edwards, J. E. (1953). Malformations of the aortic arch system

manifested as "vascular rings". Lab. Invest., 2, 56.Gross, R. E. (1945). Surgical relief for tracheal obstruction from a

vascular ring. New Engl. J. Med., 233, 586.(1955). Arterial malformations which cause compression of thetrachea or esophagus. Circulation, 11, 124.

-and Ware, P. F. (1946). The surgical significance of aortic archanomalies. Surg. Gynec. Obstet., 83, 435.

Kerwin, A. J. (1936). Persistent (partial) truncus arteriosus associatedwith double aortic arch. J. techn. Meth., 15, 142.

Mustard, W. T., Trimble, A. W., and Trusler, G. A. (1962). Medias-tinal vascular anomalies causing tracheal and esophageal com-pression and obstruction in childhood. J. Canad. med. Ass., 87,1301.

Neuhauser, E. B. D. (1946). The roentgen diagnosis of double aorticarch and other anomalies of the great vessels. Amer. J. Roent-genol., 56, 1.

Reivich, M., Holling, H. E., Roberts, B., and Toole, J. F. (1961).Reversal of blood flow through vertebral artery and its effect oncerebral circulation. New Engl. J. Med., 265, 878.

von Siebold, C. T. (1837). Ringformiger Aorten-Bogen bei einemneugebornen blausuchtigen Kinde. J. Geburtsh. Frauenzimmer-Kinderkrank., 16, 294.

Stewart, J. R., Kincaid, 0. W., and Edwards, J. E. (1964). An Atlasof Vascular Rings and Related Malformations of the Aortic ArchSystem. Charles C. Thomas, Springfield, Illinois, U.S.A.

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