The Egyptian Journal of Radiology and Nuclear Medicine 48 (2017) 81–86

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The Egyptian Journal of Radiology and Nuclear Medicine

journal homepage: www.sciencedirect.com/locate/ejrnm

Review

Anatomical variant of large mediastinal veins: Incidental findings

on MDCT chest
Tamer F. Taha Ali a,⇑, Abdel-Naser Ghareep b
a
Department of Radiodiagnosis, Faculty of Medicine, Zagazig University, Egypt
b
Department of Radiodiagnosis, Faculty of Medicine, Al-Azhar University, Egypt

a r t i c l e i n f o a b s t r a c t

Article history: Thoracic veins congenital abnormalities are important uncommon developmental anomalies that have a
Received 17 October 2016 wide range of presentation from isolated incidental findings to a part of more complex anomalies. It can
Accepted 28 November 2016 be categorized into systemic venous system and pulmonary venous system. It is of important value to
Available online 4 December 2016
understand the mediastinal vascular anatomy to help to identify variable vascular anomalies.
In this study we attempt to describe some incidentally encountered mediastinal large venous anatom-
Keywords: ical variants in patients undergoing MDCT chest in order to avoid further unnecessary studies.
Chest
Ó 2016 The Egyptian Society of Radiology and Nuclear Medicine. Production and hosting by Elsevier. This
Venous
MDCT
is an open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/by-nc-nd/
Congenital 4.0/).

Contents

1. Introduction . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 81
2. The superior vena cava (SVC) . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 82
3. Azygos vein. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 82
4. Pulmonary veins. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 84
4.1. Partial anomalous pulmonary venous return (PAPVR) . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 85
4.2. Total anomalous pulmonary venous drainage (TAPVR) . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 85
5. Pulmonary varix . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 86
6. Conclusion . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 86
Conflict of interest. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 86
References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 86

1. Introduction ital anomalies can be categorized into systemic venous system

Thoracic veins congenital abnormalities are not common, how-
ever they are considered as important developmental anomalies.
Congenital anomalies of thoracic venous system has a wide range
of presentation from isolated incidental findings during lung or
cardiac imaging to a part of a more complex anomalies especially
associated with congenital cardiac diseases [1–4]. Venous congen-
Peer review under responsibility of The Egyptian Society of Radiology and Nuclear
Medicine.
⇑ Corresponding author.
E-mail address: tamerfathi2008@yahoo.com (T.F. Taha Ali).
(including SVC and the Azygos system) and pulmonary venous sys-
tem [2,4,5–8].
Systemic venous abnormalities are frequent incidental findings,
while the pulmonary venous abnormalities are usually manifested
with cyanosis due to abnormal blood flow and associated pul-
monary venous flow obstruction in some cases [1,2].
It is of important value to understand the mediastinal vascular
anatomy to help to identify variable vascular anomalies. During
study interpretation, radiologist should not only consider the var-
ious anatomic variants but also to have knowledge of their differ-
ent classifications, associated anomalies and clinical presentation.
Proper rule out of these anomalies especially when encountered

http://dx.doi.org/10.1016/j.ejrnm.2016.11.011
0378-603X/Ó 2016 The Egyptian Society of Radiology and Nuclear Medicine. Production and hosting by Elsevier.
This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/by-nc-nd/4.0/).
82 T.F. Taha Ali, A.-N. Ghareep / The Egyptian Journal of Radiology and Nuclear Medicine 48 (2017) 81–86
incidentally has a significant implication on avoiding additional
non necessary examinations especially in case of simulation of tho-
racic disease [2–4].
2. The superior vena cava (SVC)
The superior vena cava (SVC) is the largest systemic mediastinal
vein. It is formed at the right side of superior mediastinum at the
level of first costal cartilage by the union of the bilateral (right
and left brachiocephalic veins). It runs in caudal direction to end
in the right atrium (sinus venosus portion) [9,10].
During embryological life, there are right and left anterior car-
dinal as well as common cardinal veins. Normally, the SVC is
formed from the right anterior cardinal and common cardinal veins
regression of the left anterior cardinal vein occurs [9–12].
Concurrent non-regression of the left anterior cardinal vein
leads to occurrence of double SVC while if persistent left anterior
cardinal vein with regression of the right cardinal vein results in
solitary left SVC [9–12].
The left SVC has lateral relation to the aortic arch and anterior
relation to the left pulmonary hilum, it runs inferiorly to enter
the pericardium and finally drain into the coronary sinus
[2,11,13] (Fig. 1).
The accurate incidence of persistent left SVC is difficult to estab-
lish. Many previous reports showed that 0.1–0.5% is the average
prevalence of incidental finding of left SVC in the general popula-
tion while this prevalence is higher in congenital cardiac disease
patients reaching up to 2.1 and 5% [2,12–17].
The left SVC is found to be a part of a duplicated SVC in most
cases. In duplicated SVC, the right SVC is smaller than left in most
cases as well as absent left brachiocephalic vein is notable [11,13]
(Fig. 2).
Fig. 1. CT axial images (a–c) shows single LT sided SVC (L) anterior to the left hilum. CT volume rendering image (d) shows single LT sided SVC (L) draining into coronary
sinus.
A left SVC is usually asymptomatic incidental finding with no
hemodynamic significant effect [11]. However, it is of important
implications when using the left brachiocephalic the pulmonary
vasculature or right heart is accessed through the left subclavian
vein. The presence of LT side SVC can make some procedures like
Swan-Ganz catheter placement, placement of pacemaker or
implantable defibrillator difficult [3,18].
On radiographs, absence of right SVC can results in the promi-
nence of the ascending aorta which may simulate an abnormal
aorta. A left SVC can results in left side focal superior mediastinal
widening at the aortic knob [3,18,19].
3. Azygos vein
Embryologically azygos vein is formed from the right posterior
cardinal vein (inferiorly) and the right supracardinal veins (superi-
orly), while the left supracardinal vein forms the hemiazygos and
accessory hemiazygos veins [20–22].
The azygos vein is formed mostly at 12th vertebra by the union
of the ascending lumbar and right subcostal veins. It enters the
chest via the aortic hiatus or postero-lateral to the right crus. It
ascends in the posterior mediastinum before arching over the right
main bronchus at the level of T5-T6 to join SVC [21–26].
The hemiazygos vein is formed by the union of the left ascend-
ing lumbar and subcostal veins. It passes through the aortic hiatus
or the diaphragmatic crura. It ascends on the left aspect of dorsal
vertebrae then crosses the midline to join the azygos vein at the
level of 8th or 9th dorsal vertebrae [20–22].
In extremely uncommon circumstances the azygos vein is
absent and this can be a result of failure of the development of
the superior segment of the right supracardinal vein. It is usually
symptom-free as the hemiazygos and accessory hemiazygos veins
 T.F. Taha Ali, A.-N. Ghareep / The Egyptian Journal of Radiology and Nuclear Medicine 48 (2017) 81–86 83

its arterial and bronchial supplies arise from the right upper lobe
(apical or posterior segments) [22,25,26] (Fig. 3).
Failure of anastomosis of the right subcardinal vein (precursor
of pre-renal IVC) with the hepatic vein during embryogenesis will
result in interrupted IVC with azygos or hemiazygos continuation.
Blood returns to the heart from the post renal neonatal circulation
through the supracardinal veins (precursor of azygos and hemiazy-
gos veins) [22–26].
In patients with hemiazygos continuation of left sided IVC,
blood drains from hemiazygos through azygos or accessory azygos
to persistent left SVC or from hemiazygos to superior intercostal
veins into normal right sided SVC [22–26] (Fig. 4).

Fig. 2. CT axial images (a and b) Double SVC. Right SVC (R) and Left SVC (L). CT
coronal reformatted image (c) showing double SVC. Right SVC (R) draining into the
right atrium and Left SVC (L) draining into coronary sinus.

will drain the right and left intercostal veins with consequent
enlargement [23].
The azygos lobe is a normal variant. Its incidence on chest X-ray
is about 0.4–1%. It is formed by penetration of the right posterior
cardinal vein to the right lung apex instead of migration over it
with two pleural layers that invaginates into the upper portion of Fig. 3. Azygos fissure CT axial (a) and coronal reformatted images (b and c) shows
upper lobe of right lung [25,26]. It is not a true accessory lobe as azygos lobe bounded laterally by azygos arch.
84 T.F. Taha Ali, A.-N. Ghareep / The Egyptian Journal of Radiology and Nuclear Medicine 48 (2017) 81–86

Fig. 4. CT axial (a–c), coronal reformatted (d) images showing dilated hemiazygos draining into LT SVC with polysplenia (e: axial CT upper abdomen image). CT axial (a–c),
coronal reformatted (d) showing dilated hemiazygos draining into LT SVC with polysplenia (e: CT upper abdomen image).

The frequency of azygos continuation is around 0.6% [23]. It is 4. Pulmonary veins

more common in patient with polysplenia.
It is important to identify the azygos anomalies before cardiac
catheterisation and interventional procedures such as balloon
dilatation or stent and umbrella placement as well as before some
surgical interference such as procedures needing azygos ligation
[22–26].
On chest radiograph azygos continuation can result in right
paratracheal stripe focal widening just above the main stem of
right bronchus [23–25].
The superior pulmonary veins drain the upper and middle lobes
of the right lung and the upper lobe and lingua of the left lung
while the lower lung lobes drain through right and left inferior pul-
monary veins. At the hilar region, the superior pulmonary veins are
located anterior and caudal to the pulmonary arteries while infe-
rior pulmonary vein ostea are more dorsal and medial location.
Finally the four pulmonary veins drain into the left atrium [27]
(Fig. 5).
 T.F. Taha Ali, A.-N. Ghareep / The Egyptian Journal of Radiology and Nuclear Medicine 48 (2017) 81–86 85

Embrylogically, the lung buds are derived from the foregut and
drained via vascular plexus that has connection to cardinal veins
and further to systemic venous system. With further development
the common pulmonary vein is formed and become connected to
the vascular plexus while the connections with the cardinal vein
obliterates. Failure of obliteration of these connections results in
either partial or complete pulmonary venous return anomalous
[3,27–33].

4.1. Partial anomalous pulmonary venous return (PAPVR)

The overall incidence of partial anomalous pulmonary venous

return (PAPVR) is about 0.5–0.7%. In this anomaly draining of blood
from anomalous pulmonary vein into the systemic circulation
establish a right to left shunt [34–40].
The abnormal vein can drain into any of the following struc-
tures [10,34]:

 Right Upper lobe: Usually drains into the SVC, it is considered

the most common type of PAPVR and is associated with atrial
septal defect of sinus venosus type.
 Right lower lobe: usually drains into inferior vena cava (IVC),
portal or hepatic veins. This can be isolated finding or associated
with pulmonary hypoplasia (Scimitar syndrome) (Fig. 6).
 Left upper lobe: 2nd most common type of PAPVR. Drains into
the brachiocephalic vein (isolated finding).
 Left lower lobe: This is a rare entity.

An anomalous vein (scimitar vein) drains a part or total lung

into the IVC either above or below the diaphragm or can drain into
hepatic, portal or azygos vein or even to coronary sinus or right
atrium. The right lung is nearly exclusively affected. The Scimitar
syndrome is characterized also by hypoplasia of the right lung
and right pulmonary artery with abnormal systemic arterial supply Fig. 6. CT axial (a) & coronal (b): showing Scimitar vein (S) draining into IVC.
to right lung. This abnormal vein has been described as a ‘‘Scimi-
tar” as it is resemble the Middle Eastern sword. A single anomalous
vein usually has insignificant hemodynamic effect; however the
pulmonary hypoplasia with complicated pulmonary hypertension
and chest infections affects the severity of symptoms [10,35].

4.2. Total anomalous pulmonary venous drainage (TAPVR)

Total anomalous pulmonary venous drainage (TAPVR) is

categorized according to the route of venous drainage into 4 types
[10].
Type I: supracardiac (most common form), drainage to the left
brachiocephalic, right SVC or azygos vein. Type II intracardiac drai-
nage to the coronary sinus or right atrium, Type III infracardiac
drainage to the portal, ductus venosus or right atrium, or mixed
type. Patient are usually presented in the neonatal period as the
presence of an obligatory ASD or patent foramen ovale establish
a right to left shunt and cyanosis.

Fig. 5. CT volume rendering Normal pulmonary vein drain into right atrium (RA). Fig. 7. CT axial (a) & coronal reformatted (b); pulmonary varix (V).
86 T.F. Taha Ali, A.-N. Ghareep / The Egyptian Journal of Radiology and Nuclear Medicine 48 (2017) 81–86
5. Pulmonary varix
Pulmonary varix is rare condition characterized by focal
enlargement of segmental pulmonary veins. It is usually asymp-
tomatic and may be mistaken as lung nodule or mediastinal
masses at radiographs [4,41,42] (Fig. 7).
6. Conclusion
Mediastinal venous vascular anatomical variants can be inci-
dental findings on imaging studies including CT. Knowledge of
these variants is essential to avoid non required further studies
and in vascular mapping needed in any possible further
intervention.
Conflict of interest
No conflict of interest for the authors.
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