CT Evaluation of Con-

genital and Acquired

Abnormalities of the
Azygos System1
Christine M. Dudiak, MD
Mary C. Olson, MD
Harold V. Posniak, MD

Congenital and acquired abnormalities may alter the anatomy, size, or

position of the azygos system. Computed tomography is often able to
depict these abnormalities and in many cases indicate the cause. In
this article, the normal anatomy of the azygos system is discussed and
examples of congenital and acquired abnormalities are presented.

U INTRODUCTION
The azygos system
is a paired venous pathway of the posterior thorax that may be
affected by numerous
congenital and acquired conditions. Serving as an important
collateral route, the azygos system enlarges in cases of compromise or interrup-
lion of the superior vena cava (SVC) or inferior vena cava (LVC) (1) The azygos .

system may similarly enlarge under conditions of increased venous blood flow or
pressure. The tributaries of the azygos system may be congenitally altered or dis-
placed by adjacent abnormalities. Computed tomography (CT) with its superior ,

display of cross-sectional anatomy, is often able to depict changes in venous anato-

my, size, or position and may also indicate the cause. In this article, we discuss the
anatomy and several normal variations of the azygos system, as well as the appear-
ance of various congenital and acquired conditions on CT scans (2,3).

Abbreviations: IVC inferior vena cava, SVC superior vena cava

Index terms: Thorax, anatomy, 567.92 Thorax,

#{149} CT, 567.121 1 Veins,
#{149} abnormalities, 567. 1 594, 567. 1599
Veins, azygos, 567.139, 567.1594, 567.1599, 567.92 Veins,
#{149} CT, 67.1211

RadioGraphics 1991; 1 1:233-246

I From the Department of Radiology, Loyoia University Medical Center, 2160 5 First Aye, Maywood, IL 60153. From
the 1989 RSNA scientific assembly. Receivedjuly 23, 1990; revision requested October 12 and receivedjanuary 7,
1991; acceptedJanuary 8. Address reprint requests to C.M.D.
CRSNA, 1991

233
 ;iv

Figure 1. Drawing of the azygos system, fron-

tal view. AHAZ = accessory hemiazygos vein,
AZ = azygos vein, HAZ hemiazygos vein, LBC
left brachiocephalic vein, LSIV left superior
intercostal vein, RBC right brachiocephalic
vein, RSIV = right superior intercostal vein.

2. 3.
Figures 2, 3- (2) Normal anatomy at the level of the diaphragm. CT scan shows the azygos vein (solid an-
now) hemiazygos
, vein (open arrow) and aorta (A) (3) Normal
, . anatomy at the level of the great vessels.
CT scan depicts the right superior intercostal vein (solid arrow) and left superior intercostal vein (open
arrow).

U ANATOMY costal vein, and, occasionally, branches of

The azygos system is a paired paravertebral the right renal vein and the IVC (5,6) . After
pathway in the posterior thorax (Figs i 2).
, entering the thorax through the aortic hiatus
The azygos vein is embryologically formed or behind the lateral aspect of the right crus,
from the right posterior cardinal vein inferi- the azygos vein ascends along the right an-
only and the right supracardinal vein superi- terolateral surface of the thoracic vertebrae.
orly (4) It arises at the level of the first or
. At the level of the fourth thoracic vertebra, it
second lumbar vertebral body from the van- arches anteriorly just cephalad to the right
able union of the lumbar azygos vein, the main system bronchus to join the SVC (7).
right ascending lumbar vein, the right sub- Rarely, the azygos vein may drain into the
right subclavian vein, right brachiocephahic
vein, intrapericardial SVC, or right atrium.

234 U RadioGraphics U Dudiak et al Volume 11 Number 2

 gure 4. Normal anatomy at the level of the
- - superior
t intercostal vein. (a) CT scan re-
eals a malpositioned central venous catheter (ar-
ows) entering the right superior intercostal vein.
‘rontal (b) and lateral (c) radiographs of the
- demonstrate
,‘ that the catheter (arrows) lies
ostenlorly, along the right anterolateral thoracic
pine, In the right superior intercostal vein supe-
:#..Iv and the azygos vein infenlorly.

a.

b. c.

The hemiazygos and accessory hemiazygos to the azygos vein. The transvertebral con-
veins are embryologically derived from the nections between the azygos vein and the
heft supracardinal vein and lie to the left of hemiazygos veins are also variable, number-
the thoracic vertebral column (4) The he- . ing between one and five (5).
miazygos vein is usually formed by the union The azygos, hemiazygos, and accessory
of the left ascending lumbar vein, the left hemiazygos veins receive tributaries from
subcostah vein, and, often, a branch of the the intercostal, esophageal, mediastinal, and
left renal vein (5) The hemiazygos. vein as- pericardial veins. The right superior inter-
cends along the left anterohateral aspect of costal vein lies along the right anterolateral
the thoracic vertebrae and crosses to the surface of the thoracic vertebrae, cephahad to
right, posterior to the aorta and esophagus, the level of the azygos arch. It drains the
to join the azygos vein at the level of the right second through fourth intercostal veins
eighth or ninth thoracic vertebra. The acces- and empties into the azygos arch (Figs 3 4). ,

sory hemiazygos vein runs cephalad from The heft superior intercostal vein lies in an
this point in a left paravertebrah location and analogous position along the left anterolater-
may communicate with the azygos, hemiazy- al thoracic spine and drains the left second
gos, or heft brachiocephalic vein. There is through fourth intercostal veins. The left su-
greater variation in the size of the hemiazy-
gos and accessory hemiazygos veins relative

March 1991 Dudlak et al U Ra4ioGrapbks U 235

 5. 6.
Figures 5, 6 (5) Norma! anatomy at the level of the aortic arch. CT scan shows the left superior inter-
costa! vein (arrow) arching anteriorly, lateral to the aorta. S = SVC. (6) Norma! anatomy at the level of the
left brachiocephalic vein. CT scan demonstrates the left superior intercostal vein (long open arrow) drain-
ing into the left brachiocephalic vein (long solid arrow), accessory hemiazygos vein (short solid arrow),
and right superior intercostal vein (short open arrow).

perior intercostal vein joins

the accessory
hemiazygos vein in 75% of cases
and then
arches anterolaterally to the aorta and drains
into the left brachiocephalic vein opposite
the orifice of the left internal jugular vein
(8). Tributaries include the left bronchial
veins and, occasionally, the left pericar-
diophrenic vein (Figs 5, 6) (5,8). The arch
of the left superior intercostal vein repre-
sents the “aortic nipple,” which may be seen Figure 7. CT scan reveals an azygos lobe (al),
on frontal radiographs of the chest. bounded by the azygos arch (arrow) laterally.
Normally, CT rarely depicts the many ye-
nous tributaries of the azygos system. They
may become visible, however, in cases of
. Absence of the Azygos Vein
congenital anomaly, acquired interruption of
Congenital absence of the azygos vein is rare,
the SVC or IVC, or increased venous flow or
with only two cases reported in the radiolog-
pressure.
ic literature (9, i 0) CT may demonstrate
. ab-
sence of the azygos vein, with consequent
U CONGENITAL ANOMALIES
enlargement of the hemiazygos, accessory
Developmental variation in the azygos sys-
hemiazygos, and heft superior intercostal
tern rarely causes symptoms. These cases are
veins.
usually detected incidentally on CT scans of
the thorax.
. The Azygos Lobe
Incomplete media! migration of the right
posterior cardinal vein, precursor of the azy-
gos vein, gives rise to an azygos lobe of the
right lung in 0.4%-i% of the population
(i i i 2). This lobe is easily
, identified on CT
scans of the thorax (Fig 7) The azygos . vein
is more lateral than usual as it ascends along

236 U RadioGrapbks U Dudlak et al Volume 11 Number 2

 - ,.

Figure 8. Situs indeterminatus and polysplenia

with azygos and hemiazygos continuation of the
IVC. A = aorta. (a) The azygos arch (arrow) is en-
hanged as it drains into the SVC. (b) Enlarged azy-
gos vein (AZ) parallels the aorta. (c) Enlarged

r azygos vein (arrow)

seen in the upper abdomen
and hemiazygos
proximal
vein (H) are
to their
junction. L midline liver, S polysplenia on
I the right.

A’ #{149} cursor of the prerenal segment of the IVC,

fails to anastomose with the hepatic veins
C.
during embryogenesis. Blood returns to the
heart from the postrenal NC through the su-
the vertebral column and rises farther superi- pracardinal veins, precursors of the azygos
only before arching anteriorly to enter the and hemiazygos veins (i 3) Associated
.

SVC or right brachiocephahic vein. The later- anomalies include duplicated or left IVC,
ally positioned azygos arch allows intrusion congenital heart disease, asphenia, poly-
of lung farther into the pretracheal and retro- splenia, and abnormalities of abdominal situs
tracheal mediastinum than is found in nor- (Fig8) (i3-i6).
ma! patients. The mediastinal contour often CT examination of patients with azygos
appears deeply concave at CT. The azygos fis- continuation of the IVC reveals enlargement
sure represents four layers of pleura inter-
posed between the azygos lobe medially and
the remainder of the right hung laterally.

. Azygos and Hemiazygos

Continuation of the IVC
Congenital interruption of the IVC with con-
tinuation of the azygos or hemiazygos vein
occurs when the right subcardinah vein, pre-

March 1991 Dudlak et al U RadioGraphics U 237

 . __

Figure 9. Duplication of the NC with hemiazy-

gos continuation of the left NC. The hemiazygos
vein drains into the azygos vein in the lower tho-
rax. A = aorta. (a) The enlarged azygos arch (az)
drains into the SVC. (b) The hemiazygos vein (b)
is enlarged. (C) Duplicated IVC (I) lies on either
side of the aorta below the level of the renal hila.

of the azygos vein and azygos arch secondary C.

to increased flow. The intrahepatic portion
ofthe IVC is absent (i4,i6).
In patients with hemiazygos continuation azygos arch above it (Fig 9) (i 7).
of a left-sided WC, blood in the hemiazygos 2 Blood
. flows preferentially from the
vein may reach the right atrium by three dif- hemiazygos vein into the accessory hemiazy-
ferent routes (17): gos vein and then into a persistent left SVC
i The hemiazygos
. vein drains into the azy- and coronary sinus, all of which are dilated
gos vein at approximately the eighth or ninth (Fig iO).
thoracic vertebral level. CT demonstrates en- 3 Blood
. flows from the hemiazygos vein
largement of the hemiazygos vein below this to the accessory hemiazygos vein, to the heft
level and enlargement of the azygos vein and superior intercostal vein, and to the left bra-
chiocephalic vein, which empties into a nor-
ma! right-sided SVC. All of these veins are en-
harged and easily demonstrated with CT
(i 8) . This is the least common pathway.

238 U RadioGraphics U Dudlak et al Volume 11 Number 2

 b.
Figure 10. Complex congenital heart disease
and hemiazygos continuation of a left IVC into a
heft SVC. (a) Left SVC (arrow) lies adjacent to the
aortic arch (A). (b) Accessory hemiazygos vein
(AH) arches anteriorly to join the left SVC (an-
row) (C) At a more caudal level, the enlarged
. left
SvC (arrow) is seen distal to its junction with the
accessory hemlazygos arch. A = aorta, AH = ac-
cessory hemiazygos vein, P = enlarged pulmonary
artery

March 1991 Dudlak Ct a! U RO4iOGrapbiCS U 239

 .1,’I ‘

a. b.
Figure 11. Partial anomalous pulmonary venous
return to the azygos arch. (a) Frontal chest radio-
graph demonstrates an anomalous vessel (arrows)
draining into an enlarged azygos vein (az).
(b) CT scan reveals markedly enlarged azygos
arch (AZ) distal to its junction with the anoma-
bus vessel. The night superior intercostal vein
(arrow) is near norma! in caliber. A aorta, S =
SVC. (C) CT scans obtained with lung windows
highlight the course of the anomalous vessel (an-
rows).

. Partial Anomalous Pulmonary

Venous Return
Anomalous drainage of one or more pulmo- C-
nary veins into the azygos system is a rare oc-
currence. Embryohogically, this results when
communication persists between portions of pulmonary vascularity, and, rarely, pulmo-
the pulmonary venous plexus of the hung and nary edema may be present, since partial
the azygos system (i 9) CT may demonstrate
. anomalous pulmonary venous return func-
the abnormal vessel in the lung, as well as its lions as a heft-to-right shunt (i 9).
connection with the azygos system. Because
of an increase in blood flow, the veins of the U ACQUIRED ABNORMALITIES
azygos system distal to the junction with the Acquired abnormalities of the azygos system
anomalous vessel are enlarged (Fig i i) As- . are much more common than congenital
sociated findings of cardiomegaly, increased anomalies and may have numerous causes.

. Right Atrial Hypertension

Elevated right atriah pressure may be trans-
mitted to the SVC and azygos system, with
subsequent enlargement of the vessels. This

240 U RadioGrapbks U Dudlak et a! Volume 11 Number 2

13a. 13b.
Figures 12, 13. (12) Left brachiocephalic vein obstructed by thrombus. (a) Thrombus is present in the
left internal jugular vein (solid arrows) Note the collateral
. vessels (open arrows) near the spinous pro-
cesses and the chest wall. (b) Collateral flow drains forward into the distal SVC through the right superior
intercostal vein (straight arrow) Thrombus . extends distally into the left brachiocephalic vein (curved an-
rows). (13) Distal obstruction of the SVC by neoplasm. (a) Thrombus is present in the SVC (straight an-
now) above the site of obstruction. Note dense enhancement of the azygos vein (curved arrow) indicating
,

retrograde flow. (b) Adenocarcinoma (M) is seen invading the mediastinum, causing distal SVC obstruc-
tion.

hypertension is most commonly due to right- then flows forward into

the right atrium via
sided heart failure but may also be caused by the distal SVC (Fig i 2)
If the SVC obstruc-
.

constrictive pericarditis, large penicardial ef- tion is distal to the azygos arch, there is a ret-
fusions, and tricuspid valvular disease (20). rograde flow from the proximal SVC into the
azygos arch and vein. This is manifested on
. SVC Obstruction CT scans by dense enhancement of the azy-
Obstruction of the SVC will result in in- gos vein during bolus intravenous adminis-
creased flow in the azygos system and its tration of contrast medium (Figs i 3 14) ,

tributaries. The pattern and direction of coh- (2i-24).

lateral flow in the azygos system vary with Malignant neoplasm in the hung or medias-
the site of SVC obstruction relative to the tinum is the most common cause of obstruc-
azygos arch. If the SVC is obstructed proxi- lion of the SVC. Obstruction may be due to
mal to the azygos arch and is patent distally, compression or direct invasion (Fig i 3).
blood flows via chest wall collateral vessels
and superior intercostal veins into the acces-
sory hemiazygos and azygos veins, where it

March 1991 Dudiak et al U RadioGrapbks U 241

 15a. 15b.
Figures 14, 15. (14) Distal compression ofthe SVC by an aortic aneurysm. A = aorta. (a) CT scan shows
retrograde flow of intravenous contrast material in densely enhanced azygos arch and vein (arrows) . S =

SVC. (b) More cauda! scan demonstrates compression of the SVC (open arrow) by an aortic aneurysm
(AA) and retrograde flow in the azygos vein (solid arrow). (15) Fibrosing mediastinitis secondary to histo-
plasmosis with SVC obstruction. (a) Collateral flow occurs through an enlarged right superior intercostal
vein (curved arrow) and paraspinous vessels (arrowheads) . Note aberrant right subclavian artery (straight
arrow) There is infiltration
. of the mediastinal fat compatible with fibrosis. (b) At a more inferior level,
the SVC is nearly obliterated by thrombus (open arrow). Retrograde flow occurs in the azygos vein (solid
arrow), with dense enhancement. Note calcified and noncalcified mediastinal nodes.

Nonmalignant causes include mediastinah fi- an increase in collateral blood flow. IVC ob-
brosis, retrosternal goiter, aortic aneurysm, struction may be the result of compression,
and SVC thrombosis (Figs 1 2 i 4 i 5)
, , thrombosis, or direct invasion by neoplasm,
(21,22,25). particularly hypernephroma, adrenal carci-
noma, and hepatocehlular carcinoma (Figs
. IVC Obstruction i 6, i 7) latrogenic
. causes include surgical
Acquired obstruction of the IVC may result resection and occlusion of the IVC by caval
in enlargement of the azygos system due to filters and chips (Fig 18).

242 U RadioGrapbks U Dudlak et al Volume 11 Number 2

a. b.
Figure 16. Enlargement of the azygos and hemiazygos veins secondary to IVC invasion by a hepatoma.
(a) Enlarged azygos vein (solid arrow) and hemiazygos vein (open arrow) at the level of the diaphragm.
(b) Hepatoma (H) invading the IVC. Note enlarged hemiazygos vein (arrow). (Courtesy ofJosephJ. Por-
ada, MD, StJames Hospital, Chicago Heights, Ill.)

Figure 17. Hepatoma invading the NC, with

azygos vein enlargement. (a) Hepatoma (*) in-
volves the dome of the liver. Tumor thrombus (7)
extends into the middle hepatic vein. (b) Tumor
thrombus (7) extends into the IVC from the mid-
die hepatic vein on a more cephalic image. * =
hepatoma. (c) Tumor thrombus (7) fills the right
atrium. The azygos vein (arrow) is enlarged due
to collateral flow.

C.

March 1991 Dudiak et al U RadioGraphics U 243

 a. b.
Figure 18. SurgIcal WC interruption with azygos vein enlargement. (a) Collateral flow through an en-
hanged hemlazygos vein (arrow) (b) . The NC Is occluded by a surgical clip (arrow).

a.
Figure 19 CT scans of a patient with cirrhosis, portal hypertension, varices, and an enlarged azygos
vein. (a) The azygos vein is enlarged (solid arrow) just above the diaphragm. Note vanices (open arrows).
(b) Cirrhotic liver (L) spienomegaly
, (S) gastric varices (arrowheads)
, and numerous collateral
, vessels in
the splenic hilum (v) are seen in the upper abdomen.

. Portal Hypertension . Aortoazygos Fistula

A complex collateral pathway connects the Posttraumatic aortoazygos fistula results in
portal and systemic
venous systems. In cases enlargement of the azygos vein distal to the
of elevated portal venous pressure, blood traumatic communication. Although this en-
flows into the coronary, esophageal, and gas- largement may be detected at CT, the diagno-
tnic veins and then into the azygos and 515 of a fistula is difficult and may require an-
hemiazygos veins to the SVC. Increased flow giography (26).
through these collateral veins may result in
enlargement of the azygos and hemiazygos . Displacement of the
veins (Fig i9). Azygos System
Displacement of the azygos vein by adjacent
lesions is the most common abnormality of
the azygos system encountered at CT of the

244 U RadioGrapbks U Dudiak et al Volume 11 Number 2

 cinnati: Audiovisual Radiology of Cincin-
nat!, 1987; 471-5 14.
4 - Allan PD. Cardiovascular system: arteries
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5. Gray H; William PL, Warwick R, eds. Angi-
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7. Hollinshead WH. Textbook ofanatomy. 3rd
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and left superior intercostal vein (curved arrow) 8. BallJB, Proto AV. The variable appearance
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A aorta, N= nodes. gy 1982; 144:445-452.
9. Hatfield MK, Vyborny CJ, MacMahon H,
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1i . SpeckmanJM, Gamsu G, Webb WR. Alter-
displacement include granulomatous disease
ations in CT mediastinal anatomy produced
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The azygos system is an important accessory 13 . Chuang VP, Mena CE, Hoskins PA. Congeni-
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lateral circulation between the SVC and NC, view of embryogenesis and presentation of a
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246 U RadioGrapbks U Dudlak et al Volume 11 Number 2