compression of the trachea or esophagus

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CLINICAL PROGRESS Editor: HERRMAN L. BLUMGART, M.D. Associate Editor: A. STONE FREEDBERG, M.D. Arterial Malformations which Cause Compression of the Trachea or Esophagus By IROBERT E. GROss, M.D. HERE are many vascular malformations in the superior mediastinum which have little or no clinical significance, but some arterial anomalies in this region assume importance because an abnormal or a displaced vessel can compress the trachea or the esopha- gus (or both) and partly obstruct these vital passages. Since surgical methods are now available for thoracic exploration of even the youngest subjects, it is important to recognize those malformations which threaten health or life; certain of these can now be brought under surgical attack, thus affording relief of the esophageal or tracheal obstruction. Arterial derangements within the chest are common, canl be complex, and can assume many forms. These facts are confirmed by a very extensive literature. A presentation of mono- graphic size would be necessary to list and describe all of the anomalies which have been encountered. It is intended here to deal only with those, which so far have been found at the operating table when attempting to relieve an existing tracheal or esophageal compression. The following sections are based on the study and surgical care of 70 babies and children with such vascular anomalies. Occasionally, one of these vascular malformations might come to light in an adult, but almost certainly the vast majority of malformations, which are serious enough to cause important troubles, will come to the attention of a clinician or a From the Surgical Service of The Children's Hos- pital and the Department of Surgery of the Harvard Medical School. 124 surgeon in the early years of life, as was the experience in the series of cases described here. The arterial malformations for which surgery has some therapeutic value include double aortic arch, right aortic arch with a left ligamen- tumrn arteriosum, anomalous innominate artery~ anomalous left common carotid artery, and aberrant right subclavian artery. DOUBLE AORTIC ARCH Pathologic Anatomy. The fundamental patho- logic change is concerned with the fact that the ascending aorta bifurcates into two branches, one passes in front of and to the left of the trachea, while the other progresses to the right of the trachea and esophagus; both limbs then join to form a descending aorta (figs. 1 and 2). In the majority of cases, the left (anterior) arch is the smaller of the two; in a minority, the right (posterior) arch is the smaller ill size. The descending aorta is generally on the left side of the spinal column, but in an occasional case it is to the right of the midline (fig. 3). The space between the two aortic arches is insuffi- cient for accommodation of a trachea and esophagus of normal size. Therefore, both of these structures become compressed inl the crowded region between the two aortic seg- ments. Clinical picture. In rare cases, the space pro- vided between the two aortic arch limbs is relatively large; the encircled trachea and esophagus have a fair amount of room and there is little or no attendant difficulty from interference with the functions of these two pathways. However, most subjects with a Circulation, Volume XI, January, 1955 by guest on April 11, 2018 http://circ.ahajournals.org/ Downloaded from

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Page 1: Compression of the Trachea or Esophagus

CLINICAL PROGRESSEditor: HERRMAN L. BLUMGART, M.D.Associate Editor: A. STONE FREEDBERG, M.D.

Arterial Malformations which CauseCompression of the Trachea

or EsophagusBy IROBERT E. GROss, M.D.

HERE are many vascular malformationsin the superior mediastinum which havelittle or no clinical significance, but

some arterial anomalies in this region assumeimportance because an abnormal or a displacedvessel can compress the trachea or the esopha-gus (or both) and partly obstruct these vitalpassages. Since surgical methods are nowavailable for thoracic exploration of even theyoungest subjects, it is important to recognizethose malformations which threaten health orlife; certain of these can now be brought undersurgical attack, thus affording relief of theesophageal or tracheal obstruction.

Arterial derangements within the chest arecommon, canl be complex, and can assume manyforms. These facts are confirmed by a veryextensive literature. A presentation of mono-graphic size would be necessary to list anddescribe all of the anomalies which have beenencountered. It is intended here to deal onlywith those, which so far have been found at theoperating table when attempting to relieve an

existing tracheal or esophageal compression.The following sections are based on the studyand surgical care of 70 babies and childrenwith such vascular anomalies. Occasionally,one of these vascular malformations mightcome to light in an adult, but almost certainlythe vast majority of malformations, which areserious enough to cause important troubles,will come to the attention of a clinician or a

From the Surgical Service of The Children's Hos-pital and the Department of Surgery of the HarvardMedical School.

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surgeon in the early years of life, as was theexperience in the series of cases described here.The arterial malformations for which surgery

has some therapeutic value include doubleaortic arch, right aortic arch with a left ligamen-tumrn arteriosum, anomalous innominate artery~anomalous left common carotid artery, andaberrant right subclavian artery.

DOUBLE AORTIC ARCH

Pathologic Anatomy. The fundamental patho-logic change is concerned with the fact that theascending aorta bifurcates into two branches,one passes in front of and to the left of thetrachea, while the other progresses to the rightof the trachea and esophagus; both limbs thenjoin to form a descending aorta (figs. 1 and 2).In the majority of cases, the left (anterior) archis the smaller of the two; in a minority, theright (posterior) arch is the smaller ill size. Thedescending aorta is generally on the left sideof the spinal column, but in an occasional caseit is to the right of the midline (fig. 3). Thespace between the two aortic arches is insuffi-cient for accommodation of a trachea andesophagus of normal size. Therefore, both ofthese structures become compressed inl thecrowded region between the two aortic seg-ments.

Clinical picture. In rare cases, the space pro-vided between the two aortic arch limbs isrelatively large; the encircled trachea andesophagus have a fair amount of room andthere is little or no attendant difficulty frominterference with the functions of these twopathways. However, most subjects with a

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FIG. 1. Double aortic arch producing compression of the trachea and esophagus. Left. Preopera-tive state. There is a small anterior arch and a large posterior limb, which join and form the descend-ing aorta. Right. Surgical therapy by dividing the ligamentum arteriosum and also the anterior arch.The left common carotid artery is tacked forward to the sternum, thus keeping it from pressing onthe trachea. These various steps give sufficient room for the trachea and the esophagus to bulge tothe patient's left and be relieved of constriction.

double aortic arch have very little room be-tween the two arterial limbs, so that theesophagus and particularly, the trachea, aregreatly compressed. While a double aorticarch is compatible with a long life and rela-tively minor symptoms, the anomaly is usuallya serious one and has often led to fatality withinthe first year or two of life; the marked trachealobstruction has led to superimposed pulmo-nary infection which has overwhelmed many ofthese babies.Most human subjects with a double aortic

arch have sufficient symptoms to come to theattention of a physician during infancy. Theremay be mild or moderate hesitation in swallow-

ing. Of greater significance are the alarmingsymptoms which come from tracheal narrowing.The respiratory rate is generally increased. Thebaby struggles to obtain an adequate exchangeof air, an effort which often requires the use ofthe accessory muscles of respiration. Duringinspiration there may be intercostal and supra-sternal retraction. A loud wheeze can be heardby stethoscopic auscultation, but usually thenoise is great enough to be heard with the un-aided ear many feet or yards away. There isapt to be a "crowing" type of breathing, witha marked inspiratory and expiratory stridor.Respiratory distress is very apt to be madeworse during or immediately after the swallow-

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FIG. 2. Double aortic arch with large anterior limb and a smaller posterior limb, producing com-pression of the trachea and esophagus. Above. The two drawings show the anterior and posteriorviews of the anomaly. Below. Treatment of the condition by division of the small posterior arch,thus returning all structures to normal.

ing of milk or food. Ill severe cases, symptomsare serious enough to demand oxygell therapy.These babies are prone to lie with their headsin hyperextension, a position which tends toattenuate the trachea and push away from itsanterior surface any structure which is im-pinging upon it. If the examiner forceablystraightens the head, or flexes it toward thesternum, the exchange of air is reduced or calleven be completely shut off, though the respira-tory movements of the thorax continue.

Roentgenographic Findings. Roentgenogramsof the chest may show some pneumonitis, ifthey happen to be taken at a time when thereis superimposed lung infection. Films takeniiduring inspiration may indicate poor or ir-regular aeration of the lungs, whereas during

expiration there is apt to be an appearance ofhyperaeration. Lateral films, if made at thecorrect exposure, can frequently outline thetrachea by the air which it contains. Thus,without the use of a contrast medium, a fairappraisal can be made of the anteroposteriordiameter of the trachea. While its upper portionis of normal caliber, the lower part has a

markedly reduced lumen and the structure isapt to be displaced forward. A swallow ofbarium shows indentation of the posterior wallof the esophagus at the level of the third orfourth thoracic vertebra, this defect beingniearly a horizontal one, and occurring at alevel close to that where the tracheal compres-sion is found. If further evidence regarding thestatus of the trachea appears to be desirable,

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FIG. 3. Double aortic arch with the aorta descending on the right, constricting the trachea andesophagus. Above. Anterior and posterior drawings of the anomaly. The left arch is the smaller ofthe two. The ligamentum arteriosum between the pulmonary artery and the aorta also forms a

part of the constricting mechanism. Below. Method of surgical therapy. The smaller left arch hasbeen divided and cut away from the descending aorta; the left subclavian artery has been divided atits origin. The left common carotid artery has been tacked forward to keep it off of the trachea. Theligamentum arteriosum has also been cut.

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this can be gained by lipiodol injection and de-lineation; for children under a year of age thiscan generally be done without anesthesia, butfor older subjects it is best carried out undersome form of general narcosis. Anteroposteriorfilms show a definite compression of both sidesof the trachea in its lower third; lateral viewsshows a constriction which is striking. Thisnarrowing is at a level approximating thatwhere the defect was found on the posteriorwall of the esophagus. The combination of a

posterior esophageal compression and an an-

terior tracheal defect (when found in theabsence of a demonstrable mediastinal mass) isalmost certain proof of some type of an en-

circling "vascular ring". The size of theposterior esophageal indentation is usually a

fair indication of whether the posterior arch isthe larger or the smaller of the two.

Surgical therapy. Surgery has much to offerthese patients, because division of the smallerarch provides more room for the esophagus andparticularly for the trachea. Without discussingthe details of operative steps, the surgicalsolution of the problem is indicated in figs.1, 2, and 3. Most commonly, it is the anteriorarch which is the smaller of the two and thusis the one which must be severed. In a minorityof cases, the posterior arch is the smaller and isthe one which must be divided. In some cases

the pulmonary artery has been held in a retro-displaced position by virtue of its attachmentto the aorta through the ligamentum arteri-osum. This ligament should be divided, toallow the pulmonary artery to fall forward.While this additional step seems to have littlevalue for some patients, it is certainly an im-portant one for others.

Results qf therapy. Of the 26 patients withdouble aortic arches who have come to oper-ation, 16 had the aorta descending on the leftand 10 on the right. Of the 16 with a leftdescending aorta, there were 11 in whom theleft (anterior) arch was the smaller of the twoand was accordingly divided, and there were

five in whom the right (posterior) limb was thesmaller one and was therefore sectioned. Of the10 patients who had double arches and a rightdescending aorta, it was always the left (pos-terior) one which was severed. These 26 sub-

jects ranged in age from one month to threeyears. There have been five deaths; two fromhemorrhage at the operating table, one fromcerebral edema the day following surgery (thechild had been dangerously ill and in an oxygentent for several months prior to surgicaltherapy), and two from pneumonia 3 and 10days after operation. The 21 surviving patientshave been followed for varying lengths of time,up to nine years, since operation. They havehad extraordinary relief of symptoms. Usually,a marked improvement in the airway has beennoted immediately after operation. In allinstances, the exchange of air has obviouslybeen more free, and the subjects have not hadto use the accessory muscles of respiration forordinary activity. Stridor has usually vanished,but in a few instances a trace of it remains.Postoperative tracheograms have usuallyshown marked improvement in size of thetracheal lumen after operation, but generallysome deformity persists in the cartilages. It isreasonable to believe that the removal of allexternal pressure will give these tracheas abetter chance to develop in future years as thechildren progress in body growth.The overall results postoperatively have

been exceedingly dramatic and have provedbeyond a doubt that an operative attack on thisvascular abnormality has great merit. Thisdoes not imply that all humans with a doubleaortic arch should necessarily be operatedupon, because there are doubtlessly a few whotolerate the condition in a reasonably satis-factory way through a long life. However, itappears that most infants and young childrenwith this abnormality are extremely apt todevelop serious or even fatal complications atsome subsequent time, and, hence, we feel thatit is generally highly desirable to undertakesurgical correction of the vascular anomalywhenever it is discovered.

RIGHT AORTIC ARCH WITH LEFTLIGAMENTUM ARTERIOSUM

Pathologic anatomy. The first part of theascending aorta lies in a normal position, but,instead of being directed upward and to theleft in front of the trachea, it ascends and passesto the right of the trachea and the esophagus,

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FIG. 4. Right aortic arch, with a left ligamentum arteriosum, producing compression of the tra-chea and esophagus. Above. Anterior and posterior views of the anomaly. Below. Surgical correc-tion, by division of the ligamentum arteriosum and the first part of the left subclavian artery. Thesesteps allow the trachea and esophagus to bulge to the left and to the rear.

and then continues as a descending aorta whichmay lie either to the left or to the right of thevertebral column. A right aortic arch in itselfcauses little or no disturbance, though at timesit has been known to press on the right mainbronchus or one of its branches and has led topathologic changes in the lung. If the ligamen-tum arteriosum, or a patent ductus, runs from

the pulmonary artery to the left of the tracheaand the posterior aspect of the esophagus tojoin the descending aorta, this completes a

constricting ring which encircles the esophagusand trachea (fig. 4). If this ring is sufficientlylarge, the functions of the esophagus and thetrachea are not altered to any important degree,but if the ligamentum arteriosum is taut, the

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space within the elncirclilng structures is sosmall that the compressed esophagus, andespecially the trachea, are greatly disturbed.

Clinical picture. With this anomaly, symp-toms can develop quite similar to those whichare produced by a double aortic arch, butgenerally are apt not to be quite so severe. Asa rule, the onset of complaints is delayed untila bit later in childhood; the patients that wehave encountered were on all average a fewyears older than those with a double aorticarch, the latter usually being seeni in infancy.There may be a crowing type of respiration,some intercostal and suprasternal retraction,possibly recurrent pulmonary infection, andoccasionally even some hesitancy in swallowing.The respiratory symptoms are generally ag-gravated during and following deglutition.

Roentgenographic findings. There may beevidence of pulmonary infection. There is a

prominent shadow (aortic arch) projectingfrom the right side of the superior mediasti-num. During inspiration there may be incom-plete aeration of the lungs, and during expira-tion there may be hyperaeration, indicative ofan obstruction somewhere above the carina.Lateral films of the chest, if appropriately ex-posed, can outline the air-filled trachea, show-ing the upper portion to be normal, whereas thelower segment is distinctly narrowed. Instil-lation of lipiodol into the trachea gives bettervisualization, shows a slight and ratherelongated indentation of the right wall of thetrachea, imposed by the adjacent aortic arch,inldentation of the anterior surface of thetrachea where the pulmonary artery is pulledagainst it, and a depression on the left side ofthe trachea from the ligamentum arteriosum.With a swallow of barium, one finds, at thesame level as the tracheal defects were ob-served, that the esophagus has a narrow butvery deep constriction in its left-lateral andposterior surfaces. Above this posterior notch-ing of the esophagus, it is sometimes possibleto identify a separate defect, passing obliquelyupward and to the left, which is caused by theleft subclavian artery which arises from theaorta and passes behind the esophagus to reachthe left apex of the chest. By fluoroscopic andfilm studies, it is usually possible to recognize

accurately this combination of vascular anoma-lies, but in some instances it is almost impos-sible to differentiate it from a double aorticarch which is combined with a right descendingaorta.

Surgical therapy. Treatment of this conditionis obviously one of alleviation and not of cure;it is impossible to shift the aortic arch from itsright-sided bed, but we can divide the ligamenl-tum arteriosum, which thus breaks the con-stricting "ring". The ligamentum arteriosumshould also be divided. When these steps areproperly performed, the pulmonary artery fallsforward into a more normal position and suffi-cient room is given for the esophagus, and par-ticularly the trachea, to drop backward and tothe left. Whenever the left subclavian artery isfound behind the esophagus, it, too, should besevered.

Results of therapy. Eighteen patients withthis anomaly have been operated upon, theyoungest two months and the oldest 12 yearsof age. There have been no deaths. The resultin all subjects has been striking improvement.There has always been complete disappearanceof any pre-existing dysphagia. There has beenmarked alleviation of respiratory distresswhich all subjects had prior to operationii. In oneof the older children, repeat tracheograms atthe end of one year showed only disappointinggrowth of the tracheal rings in the involvedarea: this case emphasizes the desirability ofcarrying out these operations at an earlier timein life so that removal of all external pressurewill give the trachea the best possible chance toenlarge during the growth period of the child.

It has long been known that a considerablenumber of humans possess a right aortic arch,but have few symptoms therefrom. Presuma-bly, these have had a ligamentum arteriosumin front of the trachea or else this structure,even though it passes to the left and posterioraspects of the trachea and esophagus, has beenvery long and lax. In such instances operativeintervention is not required. However, it is im-portant to recognize those occasional indi-viduals who do have a high degree of trachealnarrowing from one of the encirclements underdiscussion, because it is in this group thatsurgical therapy is a procedure of great value,particularly when it is performed early in life

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V

FIG. 5. Compression of the trachea by an anomalous origin of the innominate artery (or the leftcommon carotid artery) from the aortic arch. Left. Drawing showing pressure on anterior surface oftrachea by an anomalous left common carotid artery. Right. Alleviation of the tracheal compressionby grasping advential structures of the offending vessels and drawing them to the sternum.

before the tracheal wall has become perma-nently deformed.

ANOMALOUS INNOMINATE ARTERY

Pathologic anatomy. The innominate arterycan originate at a point farther along on thearch than is normal; when it does so, it mustwind around the anterior surface of the tracheaas it courses upward and to the right, to reachthe right apex of the chest. If this vessel is largeand taut, it can compress the trachea to aserious degree (fig. 5). While the pathologicanatomy is seemingly only a minor variationfrom normal and would appear to be of littleconsequence, the severity of symptoms whichcan accompany it has been emphasized byseveral patients who have been studied andfound to have this anomaly.

Clinical picture. There can be inspiratory andexpiratory crow of considerable degree, inter-costal and suprasternal retraction, markeddyspnea, and even superimposed pneumonitis.Tracheal compression call be sufficiently severeto require the use of an oxygen tent. Thehead is apt to be held in hyperextension, whichapparently improves the exchange of air for thechild. There is no hesitancy in swallowing, norare the respiratory symptoms made worseduring feeding.

Roentgenographic findings. By roentgenologicstudy, the esophagus is entirely normal. The

aortic arch is normal in size and in position.Visualization of the trachea may show little inthe anteroposterior view, but lateral pro-jections indicate a considerable narrowing inits lower third, the indentation appearingentirely on the anterior wall, whereas theposterior wall is straight and in normal align-ment. It may be exceedingly difficult, or indeedimpossible, to determine whether the base ofthe innominate artery or the base of the leftcommon carotid artery (described in the nextsection) is at fault, but in some instances theobliquity of the anterior tracheal defect sug-gests which of these vessels is the offending one.In the absence of any anterior mediastinalshadow (such as a cyst or neoplasm) a trachealdefect of this sort certainly suggests some ab-normal blood vessel in the region as a cause ofthe trouble. Any narrowing of the trachea whichhints of the presence of an abnormal bloodvessel of this sort can raise the question ofwhether the diminished lumen of the trachea isdependent upon incomplete development of itstracheal cartilages. Data on this point can begathered from direct tracheoscopy and also byserial observations made roentgenologicallythroughout the respiratory cycle. If the trachealdefect is due to some external pressure, thenarrowing remains relatively constant through-out the respiratory cycle; if the narrowing isdue to deficient cartilagenous rings, the caliber

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of the trachea will increase during inspirationand decrease during expiration.

Surgical therapy. After dissecting off and dis-carding the thymus, it is possible to view theaortic arch and the vessels in question. Thebase of the innominate artery can be seenwrapped around the anterior surface of thetrachea, causing a decided indentation. Be-cause of possible consequent brain damage, theoffending vessel should not be severed. How-ever, the first part of this artery and the ac-companying aortic arch can be drawn forwardby sutures taken through their adventitias andthe surrounding soft tissues and running thesesilk sutures forward through the substance ofthe sternum. Appropriate tension on these silksutures draws the vessels forward and thuscarries them away from the trachea, therebycompletely relieving the preexisting externalpressure (fig. 5). These maneuvers are rela-tively easy to perform.

Results of therapy. Nine patients, all underone year of age, have been operated upon;there have been no deaths. The results of theseundertakings have been very gratifying. Thefirst patient had previously been hospitalizedfor four months because of severe respiratory

distress; she was discharged eight days afteroperation, completely free of symptoms. Theother patients have likewise been dramaticallyrelieved of their respiratory complaints.ANOMALOUS LEFT COMMON CAROTID ARTERY

Pathologic anatomy. This malformation isprobably very rare. It is somewhat akin to theanomalous innominate artery described in thelast section. The vessel is of normal size anddistribution, but has an origin which permitsit to branch off of the aortic arch more to thepatient's right than is normal (fig. 6). It musttherefore wind around the anterior surface ofthe trachea as it courses upward and to theleft, to reach the left apex of the chest.

Clinical picture. The symptoms producedwill obviously depend upon the degree of pres-sure which is exerted upon the front of thetrachea. There may be moderate respiratorydistress, some crowing during respiratorymovements, dyspnea of mild to moderatedegree, and possibly bouts of pulmonary in-fection. There are no disturbances inswallowing.

Roentgenologic findings. By roentgenographicexamination, the findings are quite similar to,

FIG. 6. Aberrant right subclavian artery, producing "dysphagia lusoria" by posterior compres-sion of the esophagus. The right subclavian artery, instead of arising normally from the innominateartery, has an abnormal origin from the descending aorta, from which it courses upward and tothe patient's right to emerge from the chest. Left. Drawing of the malformation. Right. Treatmentof the dysphagia by ligation and division of the aberrant right subclavian artery.

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and often indistinguishable from those de-scribed in the last section for anomalous in-nominate artery. The esophagus is normal bybarium study. The trachea has a posterior wallwhich is sharp, distinct, and of normal contourand position, whereas the anterior surface ofthe trachea shows an indentation just at orabove the level of the aortic arch, there beingno anterior mediastinal cyst or tumor toaccount for these changes. Films taken atvarious angles may show that the trachealdefect is a grooved one, running upward andobliquely to the left, and strongly suggestingthat the indentation is due to the unusualorigin and pressure of the left common carotidartery. However, it is often difficult to see thatsuch an obliquity exists, and hence the roent-genologist may not be able to determine whichvessel is the offending one.

Surgical therapy. The surgical treatment forthis condition is similar to that described in thelast section for anomalous innominate artery.The first part of the left common carotid arteryimpinges against the trachea; this could berelieved by division of the vessel, but thechances of producing cerebral ischemia are sogreat that this maneuver should be avoided.The therapy consists of pulling the carotidartery and the adjacent portion of the aorticarch forward with mattress sutures whichpierce the substance of the sternum. Appropri-ate tension applied through these silk suturesdraws the common carotid artery and the archforward so that they no longer are in contactwith the trachea.

Results of therapy. Five patients have beensubjected to this undertaking. In each instance,there has been complete relief of the pre-existing respiratory symptoms.ABERRANT RIGHT SUBCLAVIAN ARTERY

Pathologic anatomy. As long ago as 1794,Bayford gave a fascinating description of theclinical picture and the relevant pathology in acase of an aberrant right subclavian arterywhich produced "dysphagia lusoria". Theright subclavian artery, instead of arising in anormal fashion from the innominate artery,takes off independently from the distal part ofthe aortic arch. It then ascends and passestowards the right, to reach the right apex of the

chest. It is rare for this artery to course infront of the trachea and it is uncommon for itto pass between the trachea and esophagus. Inthe majority of cases, it runs behind the esopha-gus in its upward path.

Clinical picture. An aberrant right subclavianartery is often recognized by radiologists whosee a posterior indentation of the esophagusduring barium studies of the alimentary tract.In a very high percentage of subjects there havebeen no important disturbances in the swallow-ing function. However, in a few cases, the sub-clavian artery can stretch sufficiently tautaround the esophagus so that the peristalticwave in the latter is altered and the patientexperiences hesitation in swallowing whichmay be bothersome and indeed can interferewith the nutrition because of the diminishedintake of food. In babies, delay in swallowingcan be found in the early months of life. Thesubject is hungry and is eager for food and willstart to swallow in an apparently normal way;he then has difficulty in getting the bolusstarted along the esophageal pathway. Some ofthe food or milk may pass downward, while theremainder stays in the pharynx for a consider-able period of time, or is expectorated. Some ofthese patients have no delay in the swallowingof milk or other fluids, but do encounter diffi-culty when solid or semisolid food is lateradded to the diet. At no time is there respira-tory distress, but some aspiration of materialwhile attempting to swallow is not uncommon,so that gurgling or noisy respirations appear atsuch times.

Roentgenographic findings. By roentgeno-graphic examination, the abnormality can beidentified quickly and with certainty. Theaortic arch is normal in size, position, anddirection. The trachea shows no abnormality.With a swallow of barium, the lateral viewshows a defect of rather small caliber on theposterior wall of the esophagus at the level ofthe third or fourth thoracic vertebra. Byanteroposterior view, this indentation runsobliquely upward and towards the patient'sright, the direction and position of this defectbeing in a line from a distal part of the aorticarch to the right apex of the chest. There isusually little or no ballooning of the esophagusabove this zone, but a delay in the passage of

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barium is often observed, due to all inter-ference with progression of the peristalticwave.

Surgical therapy. The surgical undertakingfor the treatment of dysphagia lusoria is arather simple one. It consists merely of ex-ploration of the posterior mediastinum, freeingthe anomalous artery from its bed, doublyligating aid dividinlg it. There are alwayssufficient collateral vessels to provide all ade-(luate supply of blood to the right arm.

Results of therapy. In our series, 12 patielltshave been operated upon; these varied in agefrom three weeks to six years. All subjectssurvived this surgery and in each instancethere was complete disappearance of the dys-phagia which had existed prior to operation.

SUMMARY

Iin recent years observations have showni thatthere are certain arterial malformations ill thesuperior mediastinum which bring about signifi-calnt compressioll of either the trachea oresophagus, or both of these structures. If thearterial derangements are of such a nature thatsymptoms are produced by obstruction of theesophagus and particularly by the impairmentof the tracheal airway, it is of prime importanceto recognize the underlying vascular malforma-tion because surgical approach to the problemhas a great deal to offer either by division of anoffending vessel or by displacement of anartery in such a manner that more room isprovided for the esophagus and trachea.

Roeniitgenographic examination of theesophagus and trachea with contrast mediausually gives a fairly clear impression of thearterial derangement and leads to accuratediagnosis. Visualization of the aortic pathwaysby angiocardiography or by retrograde aor-tography is seldom necessary.At operation, all these anomalies canl be

exposed through a left anterolateral trans-pleural approach to the superior mediastinum.Most of the thymus gland can be dissected offand discarded, allowing good visualizationl ofthe region.Emphasis must be placed upon the fact that

constrictions of the esophagus or trachea arenot caused solely by vessels or ligaments, but

that constrictionl is likewise produced by fibrousbands or sheaths which accompany thesevessels and ligaments. Hence, it is important,not only to divide or displace the appropriatevessel or ligament, butt also to cut any strandsor bands of tissue which accompany thesestructures and which form a part of a c(ol-strictilng mechanism.

In a series of cases in which surgical therapyhas been undertaken for alleviating compres-sion of the esophagus or trachea for the variousvascular anomalies unider consideration, it hasbeen shown that the risks of surgery can bekept relatively low and that the benefits whichaccrue from such treatment are real and wellworth seeking.

REFERENC(S1BAYFORD, 1).: An account of a singular case of

deglutition. Mere. M. Soc.. London, 2: 275,1794.

2 CONGDON, E. D.: Transformation of the aorticarch system dturing the dlevelopment of thehuman embryo. Contrib. Embryol., No. 14.Carnegie Institution of Washington, 1922.

3 COPLEMAN, B.: Anomalous right subclavian artery.Am. Jr. Roentgenol., 54: 270, 1945.

4 FABER, R. K., HOPE, J. W., AND ROBINSON, F. L.:Chronic stridor in early life. J. Pediat., 26:128, 1945.

5 GROSS, R. F.: Surgical relief for tracheal obstruc-tion from a vascular ring. New England J.Med., 233: 586, 1945.

6-: Surgical treatment for dyslphagia lusoria.Ann. Surg., 124: 532, 1946.

7-, AND NEUHAUSER E. B. I).: Compression ofthe trachea by an anomalous innominateartery. An operation for its relief. A. M. A.Am. J. Dis. Child., 75: 570, 1948.

8-: The Surgery of Infancy and Childhood.Philadelphia, 1953, W. B. Saunders Co.,Chap. 65.

9 HOLZAPFEL, G.: Ungewohnlicher Ursprung und(lVerlauf der arteria subclavia (textra. Anat.Hefte, 12: 369, 1899.

10 NEUHAUSER, EV. B. 1).: The roentgen dliagnosis ofdlouble aortic arch and other anomalies of thegreat vessels. Am. J. Roentgenol., 51: 1, 1946.

11 POTTS, W. J., GIBSON, S., AND ROTHWELL, R.:I)ouble aortic arch; Report of two cases. A. MN. A.Arch. Surg., 57: 227, 1948.

12 SPRAGUE, H. B., ERNLUND, C. H., AND ALBRIGHT,F.: Clinical aspects of persistent right aorticroot. New England J. Medt., 209: 679, 1933.

13 WOLMAN, I. J.: Syn(ldrome of (onstri(ting doubleaortic arch in infancy. Relport of a case. J.Pedtiat., 14: 527, 1939.

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ROBERT E. GROSSArterial Malformations which Cause Compression of the Trachea or Esophagus

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