double aortic arch* - thorax · the recognition of a double aortic arch still remained largely...

Thorax (1955), 10, 37.

DOUBLE AORTIC ARCH*BY

R. E. STEEN AND S. J. DOUGLASFrom the National Children's Hospital, Dublin

(RECEIVED FOR PUBLICATION OCTOBER 29, 1954)

"Aortic ring," " bifid aortic arch," and "splitaortic arch" are names given to a congenital mal-formation where, as a result of persistence of bothfourth branchial arches (Fig. 1), the aorta, insteadof being a single tube, divides into two, one branchgoing in front of the trachea, the other behind andalmost invariably behind the oesophagus as well,the two limbs uniting again to form one descendingaorta. The trachea and oesophagus are thereforegripped as it were in a vice between the two limbs,and it is easy to see how urgent obstructive symp-toms may easily arise from what would otherwisebe quite a trivial upper respiratory infection (Fig.2). The posterior limb of the arch, spoken of asa retro-oesophageal aorta, usually carries most ofthe blood, the anterior limb being practically in-variably the smaller of the two.

Recognition of vascular anomalies in this regiongoes back as far as 1794, when Bayford describeddysphagia lusoria from the Latin lusus naturae,meaning a "freak of nature." The dysphagia,due to this particular freak, was in this case theresult of an anomalous right subclavian artery,taking origin from the left side and passing overbehind the oesophagus to the right side of thebody. This is still the commonest anomaly accord-ing to Neuhauser, of Boston, but it is usuallysymptomless. In recent times Bedford and Parkin-son (1936) have shown that a retro-oesophagealaorta may occur in two other conditions besidesa double aortic arch. First, where in associa-tion with a right aortic arch, that is one whicharches to the right side instead of the left, thereis a ductus arteriosus originating in the normalmanner from the sixth left branchial arch. Thisdrags the descending aorta behind the oesophagusand down on the left side. Secondly, a similarstate of affairs can occur with a left subclavianartery originating from a right aortic arch andtravelling across from right to left, dragging theaorta over in the same way. Indeed, Paul (1948)

*Part of a paper read at the meeting of the Thoracic Societyin Dublin in June, 1951, the subject for discussion being

*Tracheobronchitis."

has described an even rarer form of retro-oesophageal aorta, namely one in which the aorticarch is on the left side in the normal manner, butwhere the descending aorta is dragged across fromleft to right by an aberrant right subclavian artery.(Bayford's original case and similar ones are dueto a right subclavian artery alone passing behindthe oesophagus, whereas in Paul's case the aortaitself is dragged to the right and becomes retro-oesophageal.)Helen Taussig (1947) holds that it is not possible

to differentiate a retro-oesophageal aorta from anyone of these causes from one due to a doubleaortic arch, but Gross, of Boston, would not agreewith this, and our experience accords with his thatat least in some cases it is possible to make thediagnosis with certainty.Arkin (1926) was the first to describe a double

aortic arch. Ten years later he had collectedanother five cases, all being in adults and for themost part symptomless. Apparently once the childreaches 2 or 3 years old the danger period is pastand symptoms tend to disappear. Possibly somefactor like growth causes superimposed respiratoryinfection to be less liable to cause obstruction.After this age a double aortic arch is so symptom-less that, although in an adult the retro-oesophagealaorta still remains tightly packed between the oeso-phagus and the veretebral column, one may almostguarantee its benign nature, provided the individualdoes not get an aneurysm.The recognition of a double aortic arch still

remained largely academic, however, until veryrecent times, when interest has been stimulated inthese vascular anomalies by advances in thoracicsurgery. These have culminated in the work ofGross, of Boston, who, in his Surgery of Infancyand Childhood (1953), gives an account of 21 casesof double aortic arch where from the previoushistory death would almost certainly haveoccurred, and in which, as a result of his sever-ing the vascular ring, complete relief of symptomsresulted in 16 cases.

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R. E. STEEN and S. J. DOUGLAS

E.W F. V[Couirtesy of Hamilton, Boyd, and Mossman

FIG. 1.-Abnormalities in the development of the branchial archarterial pattern. The persisting systemic arteries are repre-sented in solid black; the sections in outline representembryonic structures which have atrophied and disappeared;the pulmonary trunk, its branches, and the ductus arteriosus arestippled. (A) Normal pattern; (B) double aortic arch; (C)abnormal origin of the right subclavian artery; (D) right aorticarch; (E) absence of both common carotid arteries; (F) extremedegree of coarctation of the aorta, the descending aorta beingsupplied by the ductus arteriosus alone.

(3) attacks of severe acute obstructive laryngo-tracheitis due to a superimposed respiratory infec-tion which would otherwise usually be a compara-tively trivial illness.

DIAGNOSIS

The diagnosis of a retro-oesophageal aorta is asimple matter once the condition is suspected,since, as Taussig points out, a teaspoonful ofbarium will clearly demonstrate the forward dis-placement of the oesophagus. The confirmationof a double aortic arch is then made by demon-strating as in our case that there was also pressurefrom in front (Figs. 3, 4, 5, and 6).

PROGNOSISThis is a very uncertain matter during the first

few years of life, as death may easily occur fromasphyxia during feeding, or from an attack ofacute obstructive laryngotracheitis with its com-plications.

TREATMENTIn Gross's cases he severed the narrower anterior

limb and as a result completely relieved all symp-toms. The only alternative line of treatment is apalliative one to try to tide the child over until thespontaneous recovery occurs at 2 or 3 years old.

Outline of Trachea-

R. Common Carotid Art.

R. Subclavian Art. ,

AscendingAor-ta

PulmonaryArtery

O-esophagus8 L. Common Carotid Art.

,.Posterior portion ofArch of Aorta

-L. Subclavian Art.

-Anterior Arch ofAorta joins

Descending Aorta

-L. Pulmonary Art.

--Descending Aorta

FIo. 2.-Double aortic arch. The diagram shows the aorta dividinginto a narrower anterior branch which passes in front of thetrachea, and a larger posterior branch passing behind theoesophagus.

SYMPTOMATOLOGYThis is as follows: (1) Stridor, often accom-

panied by a brassy cough due to pressure on theleft recurrent laryngeal nerve; (2) dysphagia;

CASE REPORTNuala, aged 7 weeks, referred to me by Dr.

Courtney, of Nenagh, was admitted to the NationalChildren's Hospital, Dublin, on February 25, 1949,with a history of stridor since birth.

She was an only child, and there was no historyof rubella or other virus infection during the mother'spregnancy.At first the stridor was only present when the child

was receiving feeds, but during the fortnight beforeadmission it had been constantly present and asso-ciated with a cough. A few days before admissionthe child's condition had deteriorated. On examina-tion she was a poor colour with marked stridor, andthe extraordinary muscles of respiration were in fullaction with inspiratory recession of the ribs. Sul-phonamide, penicillin, and streptomycin were giventogether, and after a week what must have been asuperimposed bronchitis or bronchopneumonia sub-sided. The child still had stridor and marked asphyxialattacks which occurred always at feeding time andoften lasted as long as five minutes, by which timeon many occasions the child appeared dead. Thinkingthe condition might be a trachea-oesophageal fistula,in which case the giving of barium always results inaspiration into the lungs and a fatal broncho-pneumonia, 1 or 2 ml. " lipiodol " was introduced intothe oesophagus. A diagnosis of double aortic arch wasmade. The question of operation was then consideredand angiocardiography was discussed. I would have

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FIG. 3.-Postero-anterior view of the oesophagus outlined by a"lipiodol" swallow which shows the hold-up of "lipiodol" anddouble indentation of the wall of the oesophagus by aconstricting vascular ring.

FIG. 5.-Right oblique view showing the hold-up in the oesophagusand narrowing of the trachea in front following "lipiodol" in-stillation into the trachea.

SR~~~~~~~~~~~~~~~~~~

FIG. 4.-Lateral view showing the oesophagus pushed forward andthe narrowed tracheal air-column in front and slightly higher.

FIG. 6.-Lefl oblique v.ew showing the barium-filled oesophaguspushed forward by the posterior branch (retro-oesophagealaorta) and just above this the indentation from in front ot theanterior branch.



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R. E. STEEN and S. J. DOUGLAS

FIG. 7.-Anterior view showing the aorta dividing into the anterior FIG. 9.-View taken from above to show the trachea and the oeso-and posterior limbs surrounding the trachea and oesophagus; phagus as it were caught in a vice between the two limbs ofpulmonary artery with vertical slit. the aorta.

liked very much to have done an angiocardiogram, butwe did not consider it justifiable when the diagnosiswas unequivocal, since there is a definite slightmortality rate from angiocardiography even in babies,and especially if the case was not going to be operatedupon. By now the child was putting on weight atthe normal rate and the asphyxial attacks at feedingtime, which had been very numerous every day at thebeginning, had now become infrequent at fortnightlyor three-weekly intervals, although feeding had stillto be very careful. One hoped also that superimposedinfections might be controlled by sulphonamideand antibiotics, and it was finally decided to waitin the hope of a spontaneous recovery rather thanrisk an operation. We seemed to be making goodprogress when quite suddenly, towards the end ofSeptember, 1949, at the age of 9 months, and whenshe had reached a weight of 19 lb., she died fromasphyxia during feeding.

XA I _ l l X@Atnecropsy a double aortic arch was found withthe larger limb passing behind the oesophagus andthe narrower anterior limb passing in front of thetrachea (Figs. 7, 8, and 9). The heart was other-wise normal.

It is easy to be wise after the event, but I thinkthe decision to operate or not was a very close one.Mr. Henry and I discussed this case on innumer-able occasions. Of course if there were no ten-dency to spontaneous recovery there would be noquestion that an operation must be carried out,

Fc 8.-Posterior view showinz the retro-oesohaizeal aorta. but a number of cases recover in this manner.

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DOUBLE AORTIC ARCH

The two main dangers appear to be asphyxia on

the one hand, always associated with feeding, or

superimposed infection leading to acute obstruc-tive laryngotracheitis. The chance of fatalitiesfrom the latter are, I think, greatly minimized byantibiotics, and indeed I think this child would nothave died from this cause, but even with carefulfeeding the risk of asphyxia is apparently verygreat. This baby very nearly came through on con-servative treatment, and in mild cases with mini-mal symptoms this line of therapy appears to bethe best plan, as surgical treatment carries a defi-nite operative mortality. On the other hand, wheresymptoms, as in our case, were severe, and especi-ally where asphyxial attacks, as in this child, werea feature, we should on another occasion feelinclined to give the verdict in favour of operation.

SUMMARYA case of double aortic arch is described. It is

probable that this condition is more common thanwould appear from the number of recorded cases,and that its recognition and treatment would meana saving of life.

We are indebted to Dr. Helen Taussig and the Com-monwealth Fund for permission to reproduce Figs. 1and 2.

REFERENCES

Arkin, A. (1926). Wien. Arch. inn. Med., 12, 385.Bedford, D. E., and Parkinson, J. (1936). Brit. J. Radiol., 9, 776.Gross, R. (1953). The Surgery of Infancy and Childhood. Saunders,

Philadelphia.Paul, R. N. (1948). J. Pediat., 32, 19.Taussig, H. B. (1947). Congenital Malformations of the Heart.

Commonwealth Fund, New York.

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