15
Aneurysmal Dilatations of the Superior Vena Caval System * OsLE A. ABBoTrT** M.D., TED F. LEIGH,*** M.D. From the Divisions of Thoracic and Cardiovascular Surgery, Department of Surgery and the Department of Radiology, Emory University School of Medicine, Atlanta, Georgia PRIOR to the use of autogenous vein grafts to span defects produced by excision of arterial segments, the term aneurysm was seldom, if ever, applied to purely ve- nous structures. The production of signifi- cant aneurysmal dilatation in such autoge- nous vein grafts when not completely sur- rounded by parallel bundles of voluntary muscle resulted in restriction of £utogenous venous grafts for reconstruction of arterial flow in the four extremities. True or pure primary venous aneurysms were not de- scribed in textbooks of pathology or sur- gery prior to 1962.34 Our interest in this subject was aroused by the finding of a pure or primary (con- genital) aneurysm of the superior vena cava in a university student in February of 1948.2 Review of the literature revealed a single previous report of the pure venous aneurysm, that of Harris.28 He described "a congenital venous cyst of the medias- tinum" which was found at autopsy of a five-month-old infant. This sacculation had direct connections via feeder veins with the left innominate, inferior thyroid and right jugular veins. Following a visit to the University of Istanbul, Turkey, in 1962, we became aware of the presentation of a similar type case to the Turkish Medical Association in 1948.'5 This was a large soli- tary, saccular, congenital aneurysm above * Presented before the Southern Surgical Asso- ciation, Hot Springs, Virginia, December 10-12, 1963. ** Division of Thoracic and Cardiovascular Surgery. *** Department of Radiology. and behind the ear of a 14-year-old boy, which enlarged significantly with exercise or fatigue. Simple ligation of the single vein of entry and exit produced cure. In 1956, Lawrence and Burford 32 reported the suc- cessful excision of a solitary venous aneu- rysm which communicated via a 2 mm. stalk with the superior vena cava imme- diately above the entrance of the azygos major vein. The senior author (0. A. A.) has the privilege of including herein the initial presentation of two further examples of this type of primary or pure venous aneurysm studied and treated by Scannell 51 and Robertson.47 The first of these was a soft, vascular mass in the right supraclavic- ular region which communicated with the external jugular vein by one small venous channel. The pathologist chose to term this a venous hemangioma. Robertson's case oc- curred in a woman and proved to be a massive venous sacculation communicating directly into the left innominate vein. Aneu- rysm of the splenic vein has been reported by Lowenthal.35 The above six cases had no known ante- cedant trauma to the area involved, dem- onstrated no arteriovenous communications, and were not the result of an associated cardiac anomaly which could produce ab- normal pressure or blood flow within the involved structures. A solitary area of vein sacculation connected by a single channel to a major venous structure, or marked fusi- form dilatation occurring in the course of a major vein, does not properly fall under the classification of venous angiomata. We believe that such lesions might be better 858

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Page 1: Aneurysmal Dilatations of the Superior Vena Caval System *

Aneurysmal Dilatations of the Superior Vena Caval System *

OsLE A. ABBoTrT** M.D., TED F. LEIGH,*** M.D.

From the Divisions of Thoracic and Cardiovascular Surgery, Department of Surgery and the Departmentof Radiology, Emory University School of Medicine, Atlanta, Georgia

PRIOR to the use of autogenous veingrafts to span defects produced by excisionof arterial segments, the term aneurysmwas seldom, if ever, applied to purely ve-nous structures. The production of signifi-cant aneurysmal dilatation in such autoge-nous vein grafts when not completely sur-rounded by parallel bundles of voluntarymuscle resulted in restriction of £utogenousvenous grafts for reconstruction of arterialflow in the four extremities. True or pureprimary venous aneurysms were not de-scribed in textbooks of pathology or sur-gery prior to 1962.34Our interest in this subject was aroused

by the finding of a pure or primary (con-genital) aneurysm of the superior venacava in a university student in February of1948.2 Review of the literature revealed asingle previous report of the pure venousaneurysm, that of Harris.28 He described"a congenital venous cyst of the medias-tinum" which was found at autopsy of afive-month-old infant. This sacculation haddirect connections via feeder veins withthe left innominate, inferior thyroid andright jugular veins. Following a visit tothe University of Istanbul, Turkey, in 1962,we became aware of the presentation ofa similar type case to the Turkish MedicalAssociation in 1948.'5 This was a large soli-tary, saccular, congenital aneurysm above

* Presented before the Southern Surgical Asso-ciation, Hot Springs, Virginia, December 10-12,1963.

** Division of Thoracic and CardiovascularSurgery.

*** Department of Radiology.

and behind the ear of a 14-year-old boy,which enlarged significantly with exerciseor fatigue. Simple ligation of the single veinof entry and exit produced cure. In 1956,Lawrence and Burford 32 reported the suc-cessful excision of a solitary venous aneu-rysm which communicated via a 2 mm.stalk with the superior vena cava imme-diately above the entrance of the azygosmajor vein. The senior author (0. A. A.)has the privilege of including herein theinitial presentation of two further examplesof this type of primary or pure venousaneurysm studied and treated by Scannell 51

and Robertson.47 The first of these was asoft, vascular mass in the right supraclavic-ular region which communicated with theexternal jugular vein by one small venouschannel. The pathologist chose to term thisa venous hemangioma. Robertson's case oc-curred in a woman and proved to be amassive venous sacculation communicatingdirectly into the left innominate vein. Aneu-rysm of the splenic vein has been reportedby Lowenthal.35The above six cases had no known ante-

cedant trauma to the area involved, dem-onstrated no arteriovenous communications,and were not the result of an associatedcardiac anomaly which could produce ab-normal pressure or blood flow within theinvolved structures. A solitary area of veinsacculation connected by a single channelto a major venous structure, or marked fusi-form dilatation occurring in the course ofa major vein, does not properly fall underthe classification of venous angiomata. Webelieve that such lesions might be better

858

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Volume 159 ANEURYSMAL DILATATIONS OF THE SUPERIOR VENA CAVAL SYSTEM 859Number 6

classified as two basic sub-types of pure

venous aneurysms.

Consideration of these cases developedan appreciation for the need of an over-allclassification of aneurysmal and other sig-nificant dilatations of veins. Although thispresentation is confined to a description ofsuch pathology as it involves the superiorvena caval system, it is hoped that the out-line suggested would apply to the inferior

caval system as well. Pathologic dilatationof the pulmonary veins and the coronary

venous system might require a separate

classification.The basic purpose for this discussion,

therefore, will be threefold: 1) To empha-size the existence of venous aneurysms andthe need for clarification of our understand-ing of the same; 2) to present new ex-

emplary cases and review the pertinent

TABLE 1. Proposed Classification of Aneurysmal Disease Superior Vena Caval Systems

I. Congenital lesionsA. Primary or simple

1. Fusiform(I*)2. Saccular or diverticudar-(2)

B. Secondary or complex (resulting from associated anomalies)

1. Total anomalous pulmonary venous return-(9)2. Anomalous vena cavae

a. Hypoplasia or agenesis inferior vena cava-(3)b. Persistent levoatrial cavac. Complete transposition one or both cavae

C. Obstructive anomalies (intravenous diaphrams, etc)D. Miscellaneous

II. Acquired lesionsA. Primary

1. Fusiform-unsupported vein graft arterial replacementB. Secondary

1. Trauma (false type)-(1)2. Obstruction (mediastinitis, cirrhosis): varices-(2)3. Adjacent neoplasms-(I)

III. Pseudo-aneurysmsA. Transient

1. Cardiac failure (numerous)2. Cardiac failure plus Budd-Chiari Syndrome-(1)

B. Venous neoplasms (angiomata)-(2)

IV. Arteriovenous aneurysmsA. Congenital

1. Systemic vessels2. Systemic and pulmonary vessels (1)3. Cirsoid

B. Acquired1. Trauma-(6**)2. Surgical-purposeful, non-purposeful-(4)3. Associated disease (lues)

* Numbers in parentheses indicate cases of that type encountered by the authors.** Includes only one of numerous cases previously reported by Elkin.

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860literature; and 3) to analyze the clinicalsignificance of the individual types as wellas the appropriate diagnostic and thera-peutic measures recommended.A suggested classification is presented in

Table 1. This is the result of a study ofthe various lesions reported to date and issubject to considerable change inasmuch as

the present literature is rather limited.Type l.A. congenital lesions of primary

type are those in which there is no associ-ated abnormality producing increased bloodflow or elevation of venous pressure. Thefusiform lesions include those reported byourselves 1, 2 and Dorken.15 A similar type le-sion of the splenic vein has been presentedby Loewenthal and Jacob.35 The sacculartype are represented by those described byHarris,28 Lawrence and Burford 32 and thetwo new ones herein reported through thecourtesy of Scannell 51 and Robertson.47This latter group may cause some dissen-sion by pathologists who prefer to includethem among the various venous angiom-ata. The fact that they are large solitarysacculations and have a single communica-tion with the major venous trunk mightsuggest the term venous diverticula. Theyare neither cirsoid nor neoplastic and are

free of arteriovenous components. We be-lieve they deserve to be classified as truevenous aneurysms.

Type I.B. The second group includedunder Type I (congenital lesions) includesthose congenital abnormalities of the heart,pulmonary veins or vena cava which pro-

duce an inordinate flow within a venous

structure or persistently elevated venous

pressure. One might consider these as con-

genital venous aneurysms of an acquiredtype. The literature is replete with descrip-tions of this type of lesion. Excellent sum-

maries in this regard can be found in thebooks of Taussig,'58 Gould26 and Nadas.40

Total anomalous pulmonary venous drain-age can be of several types, but the typeinvolving a single collecting trunk whichempties via the left innominate vein into

Annals of SurgeryJune 1964

the superior vena cava is that one most

prone to produce huge aneurysmal changes.The lesions produced by actual vena cavalanomalies are less common, but they in-clude a hypoplasia or agenesis of theinferior vena cava; 5,17, 38 Tuchman59 de-scribed a persistent left vena cava enteringthe left atrium, while Taussig 58 has so welldepicted the situation wherein both venae

cavae empty into the left atrium alongwith the pulmonary veins. The cavae can

show marked aneurysmal dilatation underany of the above situations. Of considerableinterest here is the recent report of Fox andGoss 22 who produced transposition of theleft superior vena cava in the offspring ofrat dams injected with trypan blue solution81/% days past insemination.Type I.C. Significant local or widespread

dilatation of veins as the result of a congen-

ital obstructive process within a major veniis rare. Rafinski43 described one in the su-

perior vena cava causing death in a five-month-old infant, while Cucci13 removedan anomalous diaphragm-like valve whichwas producing obstruction of a left sub-clavian vein.Type II.A. The second grouping includes

sundry acquired lesions. The only pure,

primary type would involve those instanceswherein autogenous vein grafts have beenused to span segmental arterial defects inthe thorax or abdomen of experimental ani-mals. On rare occasions these have beenused in humans, but such use now would beconfined to unusual emergency situations,and then only until an adequate prosthesismay be obtained to replace it.Type II.B. The first subtype in this group

includes false aneurysms of veins due totrauma or caused by adjacent neoplasms.Previous reference to the same has not beenfound in the literature. However, the au-

thors present herein individual examples ofeach of these. The second subtype has

been previously reported by US33 as theresult of cirrhosis of the liver. Renbouen"described this as the result of a thrombosis

ABBOTT AND LEIGH

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Volume 159 ANEURYSMAL DILATATIONS OF THE SUPERIOR VENA CAVAL SYSTEM 861Number 6

of the superior vena cava of 50 yearsduration.Type III. In the third basic group are

found the pseudoaneurysms. Fleischner andUdis 21 stated that the normal width of theazygos shadow is rarely more than 5 mm.and may enlarge to as much as 1.3 mm.with chronic heart failure. One of our pa-tients who has been presented elsewhere 54

presented an azygos shadow 3 cm. in widthas the result of chronic heart failure super-imposed upon Budd-Chiari's syndrome ofhigh inferior vena cava thrombosis. Otherreports of azygos dilatation simulating me-diastinal tumor include those of Sayer,49Schmidt,52 Stauffer,56 and Bronte-Stewart,10and Magbitang.30 Venous angiomata andleiomyosarcomata can produce massive ve-nous dilatations and may appropriately be-long in this group.Type IV.A. A final grouping includes the

arteriovenous fistulae. The writings ofReid,45 Elkin,19 and Holman 29, 30 are repletewith instances of various types of these le-sions occurring throughout the body. Thecongenital lesions of this type are relativelyrare but such have been described whichinvolve the 1) phrenic; 20, 27 2) pericar-dial; 14 3) tonsillar;6 4) subclavian;57 and5) internal maxillary and pterygoid,24 aswell as other vessels. These have involvedsystemic arteries and veins. Still anothergroup of congenital arterio-venous aneu-rysms include those joining bronchial ar-teries to pulmonary veins,4 and finally inthis category are systemic vein to pulmo-nary artery (reported herein), and pulmo-nary vein to systemic vein (levo-atrio-cardinal vein anomaly) .18 Cirsoid aneu-rysms of the chest wall 8 11, 37, 42 and scalp 44have been described.Type IV.B. The acquired group of ar-

teriovenous fistula emphasizes the role ofsurgery as well as nonpurposeful trauma.Surgery may purposely produce one tempo-rarily to promote unilateral overgrowth ofone extremity 31 or as an attempt to increasecoronary collateral circulation.9 However,

Elkin19 emphasized that "trauma incidentto en masse ligation of vessels during stur-gical operation has become recognized asa cause of arteriovenous fistula." Some ofthe branches of the superior vena caval sys-tem which have become so involved bysurgery or by accidental trauma includethe 1 ) phrenic; 20, 27 intercostal (reportedbelow); 3) internal mammary; 25 4) thy-roid; I 5) subclavian;61 6) vertebral;53 7)cervical; 41 8) humeral; 79 ) axillary; 5' andother vessels.Under rare circumstances arteriovenous

aneurysms can be produced by specific dis-ease. Franklin and Pollock 23 have revieweda total of 120 cases of thoracic aorto-cavalaneurysms reported to date. These havebeen mostly luetic and had a life durationof from one to 526 days after onset, withaverage expectancy of 45 days.

Case Material

To date we have encountered a group of32 cases which could fall within the afore-mentioned classification. These are speci-fied in Table 1. For the sake of brevity wehave chosen to limit specific description tothe more unusual cases.

Type I.A. Congenital Lesions.A. Fusiform. H. S., a 19-year-old student-le-

sion involved superior vena cava reported in detailpreviously.2 Exemplary roentgenograms are shownin Figure 1 a-d. Lesion was wrapped with re-active cellophane July 25, 1948, and adjacent nor-mal venous structures protected by nonreactivematerial. Patient has remained well for 15 years,no detectable abnormalities of venous pressure orcirculation time. Lesion has remained stable insize on roentgenograms since surgery (Fig. 1 a-d).

Saccular or Diverticular. Two cases throughthe courtesy of colleagues. Relative to the first ofthese Scannell 51 wrote, "The patient was 11years old . . . noted a soft fluctuant mass abovethe right clavicle. This mass was particularly no-ticeable after exercise, and seemed to have in-creased during the antecedent two years . . . adefinite bruit beneath the clavicle . . . was laterpointed out to be a normal venous hum. Therewas no particular evidence of circulatory embar-rassment in the patient's right arm. . . . When anincision was made over the clavicle, a soft vascu-

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862ABBOTT AND LEIGSH Annals of Surgery862 ABBOTT AND LEIGH June 1964

FIG. 1. (H. S.) a. b.£FRATION Inspiration and expira-

tion. Note changes inSPIAATI size and shape of con-

genital primary fusiformaneurysm of superiorvena cava (type I.A.1)in right upper parame-diastinal area. c. Obliqueview of superior venacaval aneurysm. Bordersoutlined by dotted linesreveal false aneurysm(F. A.) probably pro-duced by catheter. d. An-

4r- :tA :] _ o viographic visualizationof aneurysm 2 years aftercircumferential wrappingwith reactive cellophane.

lar mass was discovered communicating with the The second case history in this category was

external jugular vein by one small venous channel. described by Robertson.47 This patient was a

It was not really an aneurysm but rather a venous young woman who noticed some dyspnea. Roent-hemagnioma; and this was confirmed by the patho- genogram revealed a large opacity occupyinglogical report." the upper two-thirds of the left hemithorax. In-

FIG. 2. (N. C.) a. Postero-anterior roentgenogram portraying "figure-of-eight" or "snowmanshadow" considered characteristic of total anomalous pulmonary venous drainage. Unusual amountof shadow seen in right upper paramediastinal area was produced by massive aneurysmal changes insuperior vena cava. b. Venous angiogram revealing huge aneurysm of superior vena cava area. "B.A."(blackout" or "washout area") demonstrates region of decreased concentration of contrast media pro-

duced by inflow of pulmonary venous blood through orifice of left innominate vein. c. Photographtaken at time of thoracotomy showing large, thin-walled aneurysm of superior vena cava (S.V.C.)compressing adjacent lung tissue.

. -I t

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Volume 159 ANEURYSMAL DILATATIONS OF THE SUPERIOR VENA CAVAL SYSTEM 863Number 6

vestigation and surgical exploration revealed thelesion to be a massive, solitary, cystic structurecontaining venous blood. This structure communi-cated with the left subclavian and innominatevein juncture by a single short communicatingstructure, so short as to suggest the lesion to bean actual diverticulum of the main venous trunk.

Type I.B. Congenital Lesions.

B. Secondary or Complex, Resulting from Asso-ciated Anomalies. Total anomalous pulmonary ve-nous return. We have encountered nine suchcases in our clinic of various types. The firstcase seen (June 1948) happened to show themost advanced aneurysmal changes of the supe-rior vena cava (Fig. 2 a-c). This 12-month-old girl suffered from progessively more severe

FIG. 3. a. Drawing ofanatomico-pathologic Sit- .. fl VC5uation present in N. C.,wherein all pulmonaryvenous drainage enterscommon collecting trunkbehind heart, thence via £.another venous trunk toleft innominate vein andfinally into aneurysmalsuperior vena cava. b.Outline drawing usedto clarify cardiovascularanomaly shown in a. ........ ...

dyspnea and tachyeardia. Standard postero-anteriorroentgenograms showed the snow-man, figure-of-eight described by Taussig.58 The opacification ofthe upper half of the right hemithorax was morcextensive than usually seen. Angiocardiograms re-vealed the aneurysm of the superior vena cavaand showed the characteristic black-out of themedial aspect of the contrast media filled areadue to significant inflow of blood from the leftinnominate vein. This patient was subjected tosurgery prior to the days of cardiopulmonary by-pass, and the procedure performed was unsuccess-ful. The massive aneurysm as it appeared duringsurgery was photographed (Fig. 2c); a dramaticturbulence of admixed venous and oxygenatedblood within the thin-walled aneurysm was aconspicuous finding. Subsequent cases of a simi-lar nature have been operated upon and the

Left :nnom :L..Vei

~~~~~~~~~~~~~~~~_ 0~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~... .... .... .... nl ~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~............ ... ,..: .......... ..............

l_;~~~~~Rt. Ventvicle

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ABBOTT AND LEIGH Annals of SurgeryJune 1964

FIG. 4. Series of angiograms obtained in study of patient with congenital pulmonic stenosisand "congenital absence of inferior vena cava." a. Cardiac catheter introduced via femoral veinenters right atrium through azygos major vein. Initial injection outlines area of azygos entry intoright atrium. b. Right atrium and early right ventricular filling. c. Post stenotic dilatation ofpulmonary artery demonstrated.

aneurysmal dilatation of the superior vena cava

diminished markedly (Fig. 3 a, b).Anomalies of the Vena Caval System. We have

seen three patients of this type who presentedclassical roentgenographic findings (Fig. 4 a-c).The first case was a 25-month-old infant withclassical symptoms of tetralogy of Fallot. The

Superiorveelac-cwa

Hltun

~~v

Inferior venco covo

FIG. 5. Schematic drawing illustrating "con-genital absence of the inferior vena cava." A di-lated azygos vein acts as collecting system for allabdominal organs other than liver. Azygos veinmay be "aneurysmally dilated" at it enters thesuperior vena cava. (Reprinted by permission fromGould's "Pathology of the Heart.')

vena caval status was defined by angiography.The presence of a right aortic arch led us toperform a Blalock operation on the left side,which proved successful. Our second patient was

an 11-year-old girl with severe cyanosis due touniversal pulmonary arteriovenous fistulae. Angi-ography proved the right paramediastinal mass tobe due to a massively dilated azygos venous sys-

tem. She died from her extensive and uncorrect-able pulmonary lesions, and postmortem confirmedthe roentgenographic findings. A third infant has

just completed angiographic studies and has theidentical pattern so well portrayed in a drawingfrom Gould 26 (Fig. 5).

Type II. Acquired Lesions

A. Primary Fusiform. Vein grafts in animals toreplace excised segments of iliac arteries, abdomi-nal or thoracic aorta have developed fusiform or

more extensive aneurysmal changes in our labora-tories as they have elsewhere.39 Autogenous veingrafts placed in extremities and surrounded byadjacent musculature have not shown such changesto date in numerous human cases.

Type II. Acquired Lesions (Secondary)

B.1. Secondary to Trauma (False Type).We have not found a report to date of such a

lesion. However, in 1949 we did see the followingunusual situation: J. F., a 29-year-old Negro man,was first seen in the Grady Memorial Hospital in1947 because of a penetrating stab wound in theleft parasternal area. An extensive hemothorax de-veloped, but the patient refused further treatmentafter an initial thoracentesis and was discharged.Screening 72 mm. roentgenograms in 1949 re-

sulted in his re-admission and exploratory tho-racotomy for suspected anterior mediastinal tumor

864

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Volume 159 ANEURYSMAL DILATATIONS OF THE SUPERIOR VENA CAVAL SYSTEM 865Number 6

Fic. 6. (.F.) PAand lateral films demon-strating a false aneurysmof left internal mammaryvein; study performed 2years following stabwound by ice pick in leftparasternal area.

(Fig. 6). No angiographic studies were performedprior to surgery. At surgery the lesion proved tobe a false aneurysm arising from the left internalmammary vein, presumably from the trauma whichoccurred two years previously. Delicate dissectionproved the lesion to be totally separate from theinternal mammary artery which was actually leftfunctional and intact within the patient. The saccontained well organized clot and had been non-pulsatile on fluoroscopic examination (Fig. 6 a, b).

B.2. Varices. Secondary to Mediastinitis, Cirrho-sis of Liver. Two patients in this category havebeen seen, in both of whom the underlying path-ology was severe hepatic cirrhosis. In one patient(Fig. 7) the basic lesion was diffuse aneurysmalenlargement of the hemiazygos system. In theother there was a remarkable degree of massivevarices of the posterior mediastinum (Fig. 8 a, b).The patients have been previously described.33

B.3. Secondary to Neoplasmns. This 32-year-old man was seen at the Atlanta VA Hospitalin 1954. Major chest injury occurred in 1945 androentgenograms at that time revealed some ab-normality of the left upper paramediastinal area.Actually a somewhat similar lesion was noted onre-examination of his induction films in 1942. Weperformed venous angiography in 1954. A distinct,saccular aneurysm of the inferior surface of theleft innominate vein was demonstrated. Importantaccessory findings were the irregular edges of thissac and its incomplete emptying of containedcontrast material throughout the later stages ofthis study. At surgery a vascular mass was foundto occupy the thymus gland which was firmlyadherent to the inferior surface of the left innomi-nate vein, and a secondary saccular or diverticlate

projection of this vein had been produced. Thy-mectomy and surgical resection of the diverticulumof the vein with reconstruction of the innominatevein wall was well tolerated. The excised specimenrevealed a diffuse malignancy of the thymus, wellencapsulated, and adherent vein aneurysm wall.

Type III. Pseudo-Aneurysms (Transient andMiscellaneous Dilatations)

III.A. Transient. The enlargement of the azygosvein with cardiac failure especially when examinedradiographically in the supine position has been

FIG. 7. (P. B.) Overexposed Buckey roent-genogram of chest revealing diffuse, fusiform dila-tations of hemiazygos venous system in a patientwith hepatic cirrhosis. Venous dilatations here aremore fusiform and less saccular than those inFigure 8.

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ABBOTT AND LEIGH Annals of SurgeryJune 1964

FIG. 8a, b. (P. B.)a. Film of chest. b. Spotfilm (angiogram) demon-strating massive aneu-rysmal dilatations (vari-ces) of hemiazygosvenous system in asso-ciation with cirrhosis ofliver. (Courtesy of Dr.W. Molner.)

seen frequently in our institution.2 An unusualcase seen in our medical school occurred in a

49-year-old white man in April 1957 and has beenpreviously described.54 The patient had sufferedan extensive thrombosis of the inferior vena cavalsystem up to the level of the right renal vein 22years previously. The superimposition of chroniccardiac failure due to hypertensive and cardiovas-cular renal disease in 1957 produced a remarkabledistention of the azygos vein (Fig. 9 a-c). Thislesion was nonpulsatile. Following extensive ther-apy a remarkable decrease in the size of thislesion occurred.

III.B. Venous Neoplasms. J. L., a 49-year-oldman, presented in 1958 with an extensive opacityin the left chest (Fig. 10 a, b). Following extensivestudy, exploratory thoracotomy was performed.The left chest wall, entire mediastinum, and dia-

phragm were covered with huge, tortuous, vein-

like structures with interspersed vascular neoplastictissue. Biopsy revealed it to be an angiosarcoma.Subsequent radiation therapy was somewhat pal-liative.

Type IV. Arteriovenous Aneurysms

IV.A. Congenital-Simple (Systemic and Pul-monary Vessels). In 1948, J. S., an 11-year-oldwhite boy, presented with classical symptoms andfindings of tetralogy of Fallot. Dr. R. B. Logueheard a localized continuous murmur in the thirdleft interspace about 8 cm. lateral to the sternum.He suspected a bronchial artery to pulmonary ar-

tery fistula. At the time of a left Blalock opera-

tion (patient had a right aortic arch), we were

surprised to find a vascular mass in the left upper

lobe. Dissection proved this to be an aneurysm

Apf- .''? +SFt81l"[ ~~~~~~~~~~~~~~~~~~~~~..,FIG. 9. (J. R. B.) Roentgenographic studies demonstrating azygos vein dilatation simulating

a mediastinal tumor. a. Pseudo-aneurysm as it appeared prior to treatment. b. and c. Markeddecrease in size of azygos phlebectasia following 20 days of medical therapy. Note larger sizeof lesion when studies are made in supine position.

866

A

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Volume 159 ANEURYSMAL DILATATIONS OF THE SUPERIOR VENA CAVAL SYSTEMNumber 6

FIG. 10. (A. S. R.) a.Antero-posterior and b.lateral angiograms dem-onstrating massively di-lated venous channelswidespread in left hemi-thorax. Exploration re-

vealed irrsectable angio-sarcoma.

which had formed at the juncture of the segmentalartery to the superior segment of the left upper

lobe and an aberrant vein connecting to the leftinnominate vein. This partially imitated the per-

sistent left cardinal vein situation wherein a per-

sistent vein drains the superior pulmonary venous

Ccivlt

supevioo ...

ea

system into the left innominate vein. We have seen

this latter situation on two occasions (one in asso-

ciation with persistent ductus arteriosus, the otherin a patient with interatrial septal defect andtransposition of right pulmonary veins). Fortu-nately, in the former instance findings were docu-

Phvenic N.

FIG. 11. Patient J. S. a. Unusual communication between apical branch left pulmonaryartery and left innominate vein. Note dilatation at venopulmonary artery junction labeled"arteriovenous fistula." Patient had tetralogy of Fallot with right aortic arch. b. Followingtransection of fistulous communication and preservation of pulmonary artery, left subclavianarising from left innominate artery was anastomosed to pulmonary artery (Blalock-Taussigprocedure).

867

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ABBOTT AND LEIGH Annals of SurgeryJune 1964

FIG. 12. Arteriovenouisaneurysm occurring aftermultiple thoracenteses.(Originally published byElkin, D. C., J.A.NM.A.,141:531, 1949.)

mented by motion pictures and was truly an

anomaly involving the left pulmonary arterial sys-tem. Pressure studies suggested that blood was

flowing from the systemic vein to pulmonary ar-

tery. The fistula was excised with reconstructionof the pulmonary arterial wall. A classical Blalockoperation was performed employing the left sub-clavian artery. Improved oxygenation resulted(Fig. 11 a, b).

IV. B1. Acquired-Trauma. Figure 12 is takenfrom a previous communication by Elkin20 con-

ceming a patient treated at Emory Hospital in1949. She had undergone numerous antecedentthoracenteses considered to be the cause for thisarteriovenous fistula of the phrenic vessels. In1960 we saw D. S., a 29-year-old white man, wholhad been treated for traumatic hemothorax else-where requiring multiple thoracenteses. Six monthsafter recovery from this episode he was noted tohave a continuous murmur over the posterior as-

pect of the left eighth rib (the site of previousthoracenteses). Local compression could occludethe murmur. Attempts to record the same byphonocardiography were only partially successful.Angiography was of limited value. Total excisionof the lesion was simply performed (Fig. 13).The vessels involved proved to be perforating ves-

sels as they came to the surface over the erectorspinae muscles. Cure resulted. We have had fourarteriovenous fistulas of the subclavian artery andvein occurring following stab wounds. This has

been satisfactorily handled by the recommendedsurgical approach with excision of the clavicle.61

IV. B.2. Acquired-Surgical (Purposeful or

Nonpurposeful). Recently we have seen this typeof lesion in a patient who had undergone correctivesurgery at our institution three years previously forcoarctation of the aorta. The continuous murmur

in this instance was heard just lateral to the apexof the heart and could be occluded by local pres-sure. The fistula is considered to involve inter-costal vessels at the site of rib resection. Further-more, we have a child under observation at theGrady Memorial Hospital who underwent closureof an interventricular septal defect at another hos-

pital. An arteriovenous fistula has appeared at thesite of ligation of the intemal mammary arteryand simulates the lesion described by Glenn andSteinberg.25 We have on four occasions attemptedto produce pleural arteriovenous fistula at the timeof surgery for pulmonary atresia. In two instancesexcellent long-term results have occurred followingstaged bilateral pleurectomy of this type. In twoinstances death ensued, in one of which a suddenembolic occlusion of a thumb-sized bronchial ar-

tery was the cause of fatality nine days after suchsurgery.

Roentgenologic Considerations

Earlier considerations in this regard were

reported by us in 1954,33 wherein discus-sion was confined to lesions of this typeappearing within the thorax. On plain pos-

tero-anterior roentgenograms the aneurysms

usually appear as a smooth, rounded struc-ture. There may be significant change in

868

..

IArtepiovmous,9aneLIYaysm \S 31

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size and shape on inspiration-expirationfilm studies. We have been somewhat im-pressed with the tendency for even largevenous aneurysms to be much less distinctin contour, or even seemingly absent, onstudies in the lateral projection. Fluoros-copy has shown interesting pulsations whichwere paradoxical in timing relative to ven-tricular contraction in the pure fusiformlesion without associated anomalies. Thisis in contrast to the lack of pulsation seenin the simple saccular or diverticular typereported. We have been impressed withthe absence of pulsations in aneurysmal di-latation of the superior vena cava due tototal anomalous pulmonary venous drain-age and in the pseudo-aneurysm due tocombined cardiac failure and high inferiorvena caval thrombosis. High, constant flowor pressure in these lesions seems to de-crease the tendency to pulsations. Venousaneurysms of the superior mediastinummay enlarge when studied in the supineposition; and such enlargement would beaccentuated by the Mueller or Valsalvamaneuvers.

Ultimately all venous aneurysms requirevenography for final diagnosis. The evi-dence at surgery of recent, walled-off per-foration by a cardiac catheter in PatientH. S.2 has led us to recommend that pa-tients suspected of superior vena cavalaneurysm should have the contrast mediainjection at a site not requiring entranceof the catheter into the lumen of the aneu-rysm. Total anomalous pulmonary venousdrainage can be well studied by injectingthrough a catheter placed in the rightatrium via the femoral vein. Our solitaryinstance of a residual opacity noted afterangiography of an aneurysm in associationwith a neoplasm suggests the possible sig-nificance of delayed emptying time. If acatheter had been placed in this area, theresidual opacity might have been inter-preted as being the result of penetration ofthe vein wall.

FiG. 13. Specimen excised from chest wall ofD. S. Lesion presented after multiple thoracentesesperformed for the management of a hemothorax,resulting from trauma to another area of chest.

Clinical CharacteristicsThe clinical features of these lesions vary

according to the size, position and asso-ciated lesions which may be present. Thepure venous aneurysms of the periauriculartissues 15 and the supraclavicular space 51became distended with fatigue and effortas well as showing progressive enlarge-ment. The pure fusiform superior venacaval aneurysm 2 was asymptomatic. Thesaccular or diverticular lesion of Law-rence 32 caused local pain. That of Robert-son47 caused dyspnea due to its size.The secondary congenital lesions asso-

ciated with causative anomalies basicallyowe their symptom complex to the under-lying cardiovascular abnormality. In a simi-lar way the acquired lesions relate theirsymptoms to the responsible disease state.The solitary false aneurysm was not symp-tomatic. Pseudo-aneurysms again relatetheir complaints according to the predis-posing cause.

Arteriovenous aneurysms are the onlylesions of this group which produce a con-tinuous murmur, bruit, or thrill. Severalexcellent studies have portrayed in com-plete detail the symptoms, findings andphysiologic effects of these lesions.'2 29 30,45, 48, 60

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870 ABBOTT AND LEIGH Annals of SurgeryJune 1964

TherapyThe treatment of venous aneurysms var-

ies markedly with their type. Whensoeverthe involved vein may be sacrificed, localexcision is preferable. Lesions involving es-sential veins will require reinforcement,reconstruction or graft replacement.50 Thefundamental principle in the managementof congenital venous aneurysms is to ob-tain exact definition of their cause. In thoseresulting from associated anomalies, correc-tion of the causative abnormality controlsthe venous aneurysm. This basic funda-mental underlies the therapy of the ac-quired lesions and the pseudo-aneurysms.Complete excision with or without graftreplacement is curative for the various ar-teriovenous fistulae.

Summary and ConclusionsThe authors have presented evidence

from the literature and from their personalcases which appears to prove that venousaneurysms are a true entity. A suggestedclassification has been presented. The placefor venous angiomata and neoplasm in sucha classification is debatable. From a reviewof the literature as well as a review of 32personal cases, we have attempted to pre-sent the radiologic criteria, clinical features,and recommended therapeutic measures.From this material we think we are justifiedin concluding that:

1. Aneurysmal disease of veins is lessrare than previously suspected.

2. A useful classification has been pre-sented whose major weakness involves thestatus of venous angiomata and neoplasms.

3. Catheterization can be a specific haz-ard in these lesions unless such studies havebeen so planned as to avoid actual insertionof the catheter with the lumen of the aneu-rysmal vein.

4. Roentgenographic and fluoroscopicstudy reveals varying findings according tothe specific type of lesion being inves-tigated. Venous aneurysms may appear

vague or absent on lateral roentgenograms.Dilatation of intrathoracic veins can imi-tate mediastinal tumors. Final diagnosisrequires venography.

5. The clinical pictures varies markedlyaccording to type, location, and size of thelesion.

6. Treatment is excisional or reparativein pure primary, false venous aneurysms,and arteriovenous fistulae. For other typesthe major effort should be directed towardscorrrection of the underlying patho-physi-ology; and, as such, the therapy may bebasically medical or surgical.

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