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Clinical Anatomy 27:757–763 (2014)

REVIEW

Pneumomediastinum and the Aortic Nipple: The


Clinical Relevance of the Left Superior
Intercostal Vein
ANDY WALTERS, LINDSEY CASSIDY, MITCHEL MUHLEMAN, ASHLEY PETERSON,
CHRISTA BLAAK, AND MARIOS LOUKAS*
Department of Anatomical Sciences, St. George’s University, School of Medicine, St. George, Grenada

This article discusses the appearance of the “aortic nipple” in chest radiogra-
phy, and reviews the embryology and anatomy of the left superior intercostal
vein which causes the appearance of an “aortic nipple.” This radiological sign
is useful in differentiating certain thoracic pathologies, such as pneumome-
diastinum, pneumopericardium, and medial pneumothorax. Pneumomediasti-
num is an encompassing term describing the presence of air in the
mediastinum, and may arise from a wide range of pathological conditions.
Despite the well-described imaging of pneumomediastinum, it is sometimes
difficult to differentiate from other conditions such as pneumopericardium and
medial pneumothorax. A separate finding, “aortic nipple” is the radiographic
term used to describe the lateral nipple-like projection from the aortic knob
present in a small number of individuals. The aortic nipple corresponds to the
end-on appearance of the left superior intercostal vein coursing around the
aortic knob, and may be mistaken radiologically for lymphadenopathy or a
neoplasm. Despite their relative independence, the aortic nipple is defined by
new contours in cases of pneumomediastinum, taking on an “inverted aortic
nipple” appearance. In this position, the inverted aortic nipple may facilitate
radiographic discrimination of pneumomediastinum from similar conditions.
This study aims to review the common clinical and radiographic features of
both pneumomediastinum and the aortic nipple. The radiologic appearance of
the aortic nipple occurring in unison with pneumomediastinum, and its poten-
tial role as a tool in the differentiation of pneumomediastinum from similarly
presenting conditions will also be described. Clin. Anat. 27:757–763,
2014. VC 2013 Wiley Periodicals, Inc.

Key words: aortic nipple; pneumomediastinum; radiography; left superior


intercostal vein; mediastinum; medial pneumothorax; pneumo-
pericarditis; lymphadenopathy

INTRODUCTION
The aortic nipple was first described by McDonald
et al. (1970) as a lateral, “nipple-like” projection from
the aortic knob. These authors further identified the
*Correspondence to: Marios Loukas, Department of Anatomical
anatomic structure of the aortic nipple to be the end- Sciences, St. George’s University, School of Medicine, Grenada.
on appearance of the left superior intercostal vein E-mail: mloukas@sgu.edu
(LSIV), traveling anteriorly around the aorta on its
way to the left brachiocephalic vein. The aortic nipple Received 5 March 2013; Accepted 20 May 2013
was subsequently classified as a normal radiographic Published online 27 June 2013 in Wiley Online Library
variant in erect anteroposterior chest radiographs, (wileyonlinelibrary.com). DOI: 10.1002/ca.22279

C
V 2013 Wiley Periodicals, Inc.
758 Walters et al.

Fig. 1. AP chest radiograph showing the classic vein. The left superior intercostal vein is bordered medi-
“aortic nipple” in a patient with pneumomediastinum. ally by the aortic arch and laterally by the mediastinal
Labels are added on the left to more clearly delineate the pleura. [Color figure can be viewed in the online issue,
nipple-like appearance of the left superior intercostal which is available at wileyonlinelibrary.com.]

found in a small number of individuals (McDonald et al., circumstances, additional radiographic features unique
1970; Friedman et al., 1978; Ball and Proto, 1982). to pneumomediastinum have important diagnostic
Aside from its presentation as a normal variant in relevance. As the aortic nipple may be mistaken for
healthy individuals, the spontaneous appearance of the lymphadenopathy or neoplasm (McDonald et al.,
aortic nipple may be the cause for concern. In several 1970), or may have underlying pathological origins
cases, the aortic nipple has been shown to precede (Friedman et al., 1978; Byerly and Schlesinger, 2010),
pathological conditions such as venous obstruction in accurate identification of an aortic nipple in cases of
the superior vena cava, inferior vena cava, or left bra- pneumomediastinum may have a significant impact
chiocephalic vein (Carter et al., 1985; Byerly and on patient morbidity.
Schlesinger, 2010). Furthermore, the aortic nipple has This article will review the anatomy, embryology,
been identified in conditions where venous flow and radiological imaging of the LSIV, which leads to
through the LSIV is increased, such as portal hyperten- the appearance of the aortic nipple on radiographs.
sion and certain congenital venous anomalies (Carter Furthermore, unique radiographic characteristics of
et al., 1985; Hatfield et al., 1987; Chen et al., 2008). the aortic nipple in cases of pneumomediastinum will
In contrast to the aortic nipple, pneumomediasti- be presented in an effort to reduce the misdiagnosis
num is an abnormal radiographic finding which of lymphadenopathy or neoplasm, and affirm the
encompasses a broad clinical condition in which air potential role of the aortic nipple in the accurate iden-
becomes trapped in the mediastinal space. First tification of pneumomediastinum.
described by Lae € nnac in 1834, pneumomediastinum
most commonly presents with central chest pain,
dyspnea, and retrosternal pleuritic pain radiating to EMBRYOLOGY
the back and shoulders (Macia et al., 2007). Following
its initial description, Hamman (1939) demonstrated The embryogenesis of the LSIV is seldom the focus
that pneumomediastinum might be accompanied by of developmental reviews, and tends to be present
auscultatory crepitations coinciding with normal heart- secondary to the reports of larger embryonic venous
beats (Hamman, 1939). structures. Correspondingly, the LSIV has been
Similar to the aortic nipple, pneumomediastinum reported to arise from one of the two major cardinal
has well-documented radiographic features (Fig. 1). veins, depending on the study cited (Gladstone, 1912;
Generally, pneumomediastinum is described on ante- Campbell and Duechar, 1954; Lane et al., 1976;
roposterior chest radiographs as the depiction of nor- Fasouliotis et al., 2002; Paval and Nayak, 2007).
mal anatomical structures highlighted by air leaving Nowhere has the embryogenesis of the LSIV been so
the mediastinum (Zylak et al., 2000). Although pneu- thoroughly described as in a case report by Campbell
momediastinum often presents a variety of radiologi- and Duechar (1950) of left-sided superior vena cava.
cal features, it is often confused with similarly The consecutive generation and involution of com-
appearing conditions such as pneumopericarditis and municating shunts characterize the development of
medial pneumothorax (Cyrlak et al., 1984; Bejvan the embryonic venous system. Under the guidance of
and Godwin, 1996; Zylak et al., 2000). Under these such mechanisms, the LSIV appears to arise from the
Clinical Relevance of LSIV 759

common intersection of the three left cardinal veins.


The cardinal veins appear in the fourth week of gesta-
tion as symmetrically paired anterior and posterior
cardinal veins connected by two short common cardi-
nal veins, also referred to as the ducts of Cuvier. The
anterior and posterior cardinal veins drain the cranial
and caudal portions of the embryo, respectively, and
communicate centrally through the ducts of Cuvier.
Traveling inward, the ducts of Cuvier converge upon
the sinus venosus as it receives maternal blood from
the developing heart (Campbell and Duechar, 1954).
As the embryonic heart descends caudally into the
thorax, the adjoining ducts of Cuvier descend from
either side of the developing heart. As the anterior
and posterior cardinal veins remain stationary
throughout this process of development, the lateral
portions of the ducts of Cuvier are stretched to lie in
the vertical planes of the embryo. By the eighth week
of gestation, the left and right anterior cardinal veins
form an oblique anastomosis, establishing a left-to-
right venous shunt, which corresponds to the adult
left brachiocephalic vein (Campbell and Duechar,
1954).
At this point in development, the vertically elon-
gated left duct of Cuvier sits immediately below the Fig. 2. Schematic representation of the tributaries of
intersection of the left anterior cardinal and brachioce- the left superior intercostal vein and its course with
phalic veins. To approach this intersection, the left respect to the aorta. After passing lateral to the aorta, the
duct of Cuvier travels laterally away from the sinus LSIV drains into the left brachiocephalic vein.[Color figure
venosus before ascending cranially. At its vertex, the can be viewed in the online issue, which is available at
left duct of Cuvier bends caudally again as the left wileyonlinelibrary.com.]
posterior cardinal vein. In this position, the anterior
cardinal vein, caudal to its juncture with the left bra-
chiocephalic vein, receives the apical bend of the left hemiazygos and left superior intercostal veins (Lane
duct of Cuvier (Campbell and Duechar, 1954). et al., 1976; Carter et al., 1985; Godwin and Chen,
The left brachiocephalic vein diverts venous blood 1986; Byerly and Schlesinger, 2010). As diverted flow
from the left posterior cardinal vein, allowing its produces venous dilation, obstruction to either vena
simultaneous degeneration with the left ascending cava has been demonstrated to manifest radiologically
duct of Cuvier. The persisting apical bend composed of as the spontaneous appearance of an aortic nipple.
the left duct of Cuvier and posterior cardinal vein After receiving intercostal and hemiazygos tributa-
remains connected to the receiving portion of the left ries, the LSIV courses ventrally to round the arch of
anterior cardinal vein, constituting the embryonic the aorta. Traveling anteriorly in the horizontal plane,
components of the adult LSIV (Campbell and Duechar, the LSIV is bordered along its lateral surface by the
1954). mediastinal pleura. This portion of the route is some-
times seen in anteroposterior chest radiographs as
the aortic nipple, and is also clearly demonstrated in
LEFT SUPERIOR INTERCOSTAL VEIN: horizontal plane computed tomography (CT) scans
(Godwin and Chen, 1986; Byerly and Schlesinger,
THE AORTIC NIPPLE 2010). Other notable structures surrounding the LSIV
The LSIV is a dorsal venous structure in the supe- include the left vagus nerve medially and the left
rior mediastinum draining the second, third, and phrenic nerve laterally (Standring, 2008). After round-
sometimes the fourth intercostal spaces (Ozdemir ing the aortic arch, the LSIV continues medially and
et al., 2002; Standring, 2008). It originates immedi- gradually ascends to the dorsum of the left brachioce-
ately posterior and medial to the dorsal aorta at the phalic vein. It is worth mentioning that along its route
level of the third and fourth thoracic vertebrae, and the LSIV may receive the left pericardiophrenic and
has been shown to communicate with the accessory bronchial veins, draining the left pericardium and left
hemiazygos vein in 75% of patients (McDonald et al., bronchioles, respectively (Godwin and Chen, 1986;
1970; Ball and Proto, 1982). This common anatomic Standring, 2008).
communication has important radiographic relevance
(Fig. 2). As the azygos vein receives the accessory
hemiazygos vein, an increase in azygos blood flow will RADIOLOGY
have collateral implications for the accessory hemia-
zygos and left superior intercostal veins. Indeed, flow The radiology of the LSIV was first described by
impedance to both the superior and the inferior vena McDonald (1970) as a lateral or superior “nipple-like”
cavae has been shown to divert flow to the accessory structure projecting from the aortic knob in erect
760 Walters et al.

Fig. 3. CT scan showing the left superior intercostal readily visible aortic nipple. (Used with permission from
vein travelling lateral to the aortic arch before draining Harry’s Chest Radiology Atlas at http://chestatlas.com).
into the brachiocephalic vein. Asterisk shows the course [Color figure can be viewed in the online issue, which is
of left superior intercostal vein. Arrow shows the drainage available at wileyonlinelibrary.com.]
point. An AP radiograph in this patient would reveal a

anteroposterior chest radiograms. In the same study, As the aortic nipple bears radiographic resemblance
McDonald estimated a 4% incidence of the aortic nip- to other pathologies of the superior mediastinum,
ple in healthy patients, with a corresponding fre- accurate identification of the aortic nipple is vital in
quency of 7% in diseased individuals (McDonald, reducing patient morbidity. Specifically, the aortic nip-
1970). Further evaluation of aortic nipple frequencies ple has been shown to imitate aortic aneurysm,
revealed similar ranges for healthy individuals, vary- lymphadenopathy, and neoplasm (Hatfield et al.,
ing from 0.9 to 9.5% depending on the study cited 1987). In such cases, it is prudent to undertake sec-
(Friedman et al., 1978; Ball and Proto, 1982; Abiru ondary radiological evaluation to confirm the presence
et al., 2005). In their review of 469 posteroanterior of an aortic nipple (Hatfield et al., 1987). To an extent,
chest radiographs, Ball and Proto (1982) observed the statistical limitations confining plain chest radiographic
aortic nipple in 9.5% of cases, with the appearance identification of the aortic nipple have been overcome
“varying from a subtle undulation along the aortic by CT scans and magnetic resonance imaging (MRI).
knob to a prominent protuberance.” The LSIV is readily appreciated on horizontal tho-
The diameter of the aortic nipple has been demon- racic CT scans at the levels of the aortic arch and left
strated to range from 1 to 4 mm (mean, 2.2 mm; SD, brachiocephalic vein (Dudiak et al., 1991) (Fig. 3). At
1.1 mm), with a maximum diameter of 4.5 mm in the level of the aortic arch, the LSIV may be seen pro-
healthy subjects (Friedman et al., 1978). It is also jecting laterally from the paravertebral space, adher-
notable that the aortic nipple has continuous radiopac- ing closely to the outer border of the aorta before
ity with the aortic knob, accentuating the polarity of its bending superiorly out of the horizontal plane. As it
lateral apex. Diameters of >4.5 mm have been shown rounds the aortic arch the LSIV is bordered laterally
to correlate with certain vascular disease (Friedman by the mediastinal pleura, which is itself bordered by
et al., 1978). Although congenital or impending venous a region of radiolucency corresponding to the superior
disease can be roughly extrapolated from aortic nipple lobe of the left lung. In the plane of the left brachioce-
diameter, this finding is complicated by the fact that phalic vein, the LSIV continues ventrally until it inter-
the aortic nipple has an increased diameter in supine sects with the dorsum of the left brachiocephalic vein
chest roentgenograms, regardless of individual health (Dudiak et al., 1991). As the LSIV adheres so closely
(Friedman et al., 1978). to the lateral border of the aortic arch, it may been
Clinical Relevance of LSIV 761

mistaken for a blood-filled double aortic lumen in heli- peritoneal pneumomediastinum originates in the GI
cal CT scans, as in a dissecting aortic aneurysm (Batra tract, which has been perforated by some mechanism,
et al., 2000). This resemblance may be potentially such as gastric ulcer (Stahl et al., 1977) or duodenal
resolved by superior axial sections of multidetector gastroscopy (Fierst et al., 1951).
row volume-rendered CT scans (Lawler et al., 2002). Intrathoracic pneumomediastinum is far more com-
MRI has also been proven effective in the visualiza- mon and is thought to be the result of alveolar rupture,
tion of the aortic nipple. On MRI, the aortic nipple has followed by the dissection of air into the mediastinum
been described to appear in coronal sections as a along vascular fascicular sheaths (Macklin, 1939). This
small tubular structure with low signal intensity particular cause of pneumomediastinum has been
(Medrea et al., 1988). In a study of 40 patients, documented to arise in cases where air is forced
Medrea et al. (1988) reported the presence of the aor- against a closed or highly resistant glottis (Cyrlak et al.,
tic nipple in six cases where conventional chest X-ray 1984; Zylak et al., 2000). This results in the creation of
found only one. Despite increased detection fre- a positive pressure gradient exceeding the maximum
quency, the aortic nipple was reported in MRIs only in alveolar capacity, followed by subsequent rupture of the
the presence of anomalous thoracic disturbances, and alveoli (Macklin, 1939). Pregnancy, asthma, emesis,
is therefore, of limited potential in its detection of nor- coughing or sneezing, and diabetes mellitus have all
mal aortic nipple variants. been documented as sources of pneumomediastinum
(Girard et al., 1971; Cyrlak et al., 1984; Zylak et al.,
2000). In hospital settings, respirator therapy has
PNEUMOMEDIASTINUM been demonstrated as an iatrogenic cause of pneumo-
€ nnac in 1819, pneumome- mediastinum owing to the high pressures needed to
First described by Lae
sustain ventilation (Cyrlak et al., 1984). Pneumome-
diastinum is the clinical term used to describe the
presence of air in the mediastinum (Lae € nnec and For- diastinum has also been reported to occur in up to 10%
bes, 1834). Pneumomediastinum is usually accompa- of thoracic blunt trauma cases (Wintermark and
nied by complaints of chest pain, dyspnea, and Schnyder, 2001).
retrosternal pain radiating to the back and neck, and Despite the plethora of known causes of pneumome-
should always be considered when the etiology of diastinum, spontaneous pneumomediastinum may yet
these symptoms is unclear (Newcomb and Clarke, occur in the absence of a known precedent cause. Such
2005). Furthermore, pneumomediastinum may some- cases account for a relatively small fraction of pneumo-
times present during physical examination as an aus- mediastinum cases, cited to occur in roughly one out of
cultatory crepitation coinciding with the heart beat, every 30,000 emergency room admissions (Newcomb
known as Hamman’s sign, which is accentuated and Clarke, 2005). Spontaneous pneumomediastinum
toward the end of exhalation (Hamman, 1939). occurs most frequently in young adult men, and has
The causes of pneumomediastinum are wide- been estimated to be under-diagnosed in as many as
ranging, and are often subclassified to facilitate specific 30% of all cases, most likely because of its asymptom-
diagnostic criteria. Generally, pneumomediastinum atic and self-limiting nature (Kaneki et al., 2000).
may be classified as either intra- or extrathoracic, with
respect to the original source of air (Zylak et al., 2000).
Extrathoracic pneumomediastinum can arise from RADIOLOGY
either above and below the mediastinum through the
retropharyngeal and peritoneal spaces, respectively The radiology of pneumomediastinum is simply
(Zylak et al., 2000). The mediastinal space may also be described as the depiction of normal anatomical struc-
accessed along the fascicular sheaths of major vessels tures surrounded by air migrating out of the mediasti-
traveling between the mediastinum and these areas nal space (Zylak et al., 2000). In this way, mediastinal
(Cyrlak et al., 1984). structures may acquire new radiographic appearance
As the retropharyngeal space may be accessed via with regard to both contour and shape. In anteropos-
perforation of multiple anatomical structures, includ- terior plain chest radiographs, pneumomediastinum
ing the nasopharynx, esophagus, trachea, and larynx, sharply delineates the major arteries and veins of the
extrathoracic pneumomediastinum has been reported mediastinum, as well as the trachea, esophagus, and
to arise in a wide range of clinical scenarios (Cyrlak thymus (Cyrlak et al., 1984; Zylak et al., 2000). In
et al., 1984). Owing to the sometimes traumatic cases where the volume of air is large enough, the
nature of the procedure, endotracheal intubation has thymus may become elevated and take on the
been demonstrated as an iatrogenic cause of extra- appearance of a sail, commonly known as the “thymic
thoracic pneumomediastinum (O’Neill and Symon, sail sign” (Moseley, 1960). Similarly, air surrounding
1979). The submandibular region also provides a both sides of the aorta functions to highlight its medial
route of entry into the mediastinum via communica- and lateral borders, giving it a tubular shape also
tion with the retropharyngeal space (Tofield, 1977), known as the “tubular aorta sign” (Zylak et al., 2000).
implicating seemingly simple procedures such as den- This latter feature is of significant relevance to the
tal extraction in the development of pneumomediasti- presence of an aortic nipple, as it may serve to accen-
num (Sandler et al., 1975). Furthermore, fractures to tuate the medial border of the LSIV traveling around
the mid-face have also been documented as a source the anterior arch of the aorta (Ball and Proto, 1982).
of pneumomediastinum (Tofield, 1977). Other common features of pneumomediastinum
To a lesser extent, the mediastinum is also include a thin, radiolucent line parallel to the left car-
accessed from air in the peritoneum. Most commonly, diac border. This line represents the negative space
762 Walters et al.

between the mediastinal pleura and the left cardiac are usually presented independently of one another.
border, created by pressurized mediastinal air (Maun- The aortic nipple may present as a normal radiographic
der et al., 1984). variant in healthy individuals, or arise as the result of
When discussing the radiologic presentation of an impending venous disease state. Commonly, the
pneumomediastinum, it is important to consider simi- aortic nipple appears as a lateral projection of aorta,
larly presenting conditions. Most notably, pneumoperi- sharing continuous radiopacity with the aortic knob. In
cardium and medial pneumothorax provide significant these cases, the aortic nipple may be mistaken for
diagnostic obstacles to the accurate identification of lymphadenopathy, neoplasm, or aortic aneurysm.
pneumomediastinum (Ball and Proto, 1982; Cyrlak Pneumomediastinum is a broad clinical condition,
et al., 1984; Zylak et al., 2000). Of particular similar- describing the presence of air in the mediastinal
ity to pneumomediastinum is medial pneumothorax in space. Although the radiographic features of pneumo-
the supine patient. Supine chest radiographs of medial mediastinum have been well-described, it is com-
pneumothorax depict a small pleural reflection imme- monly mistaken for similar conditions such as medial
diately left of the cardiac border, simulating pneumo- pneumothorax and pneumopericardium.
mediastinum (Bejvan and Godwin, 1996). In these The aortic nipple is defined by a new set of con-
cases, additional radiographic features specific to tours in cases of pneumomediastinum, thus we
pneumomediastinum are of particular importance. acknowledge its potential role in the discrimination of
pneumomediastinum from medial pneumothorax and
pneumopericardium. Physicians should be aware of
PNEUMOMEDIASTINUM AND THE the inverted aortic nipple appearance, and should be
AORTIC NIPPLE: THE INVERTED ruled out in cases where lymphadenopathy and neo-
NIPPLE plasm are suspected based on radiology alone. To this
end, the radiographic descriptions of lymphadenopa-
Although both pneumomediastinum and the aortic thy and neoplasm in cases of pneumomediastinum
nipple are common occurrences in anteroposterior are largely absent in the literature, and the exact radi-
chest radiographs, they are usually described inde- ographic contribution of lymphadenopathy or neo-
pendent of one another. As the aortic nipple is a plasm to pneumomediastinal radiography remains to
mediastinal structure, and most other mediastinal be determined.
structures acquire different contours in cases of pneu-
momediastinum, it follows that the aortic nipple
should also appear with newly defined contours in ACKNOWLEDGMENTS
cases of pneumomediastinum. Indeed, the aortic nip-
ple occurring in tandem with pneumomediastinum The authors wish to thank Sessica Holland, MS, Medi-
acquires a medial border, clearly delineating it from cal Illustrator in the Department of Anatomical Scien-
the outer wall of the aorta (Ball and Proto, 1982). ces, St. George’s University, Grenada, West Indies, for
More specifically, there is a loss in continuous the creation of her illustrations used in this publication.
radiopacity of the aortic nipple with the aortic knob
owing to the presence of air highlighting the unnatu-
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