Pathognomonic Imaging Signs in Abdominal Radiology

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Abdominal Radiology

https://doi.org/10.1007/s00261-019-02331-6

REVIEW

Pathognomonic imaging signs in abdominal radiology


Christopher Kloth1 · Daniel Vogele1 · Horst Brunner1 · Meinrad Beer1 · Stefan Andreas Schmidt1

© Springer Science+Business Media, LLC, part of Springer Nature 2019

Abstract
The imaging evaluation of the abdomen is of crucial importance for every radiologist. In addition to ultrasound, conventional
radiographs and contrast-enhanced computed tomography (CT) are the most common imaging procedures in the abdominal
region. Numerous pathognomonic signs should be known in this context by every radiologist. Radiographs of the abdomen
are an often used first step in radiologic imaging, while CT examinations are carried out for further differentiation, in onco-
logical settings and in time-critical emergency situations. A fast and clear assignment of these signs to a specific disease
is the basis for a correct diagnosis. This pictorial review describes the most common pathognomonic signs in abdominal
imaging. The knowledge of these pictograms is therefore essential for radiologists interested in abdominal medicine and
should also be addressed in training and further education.

Keywords  Diagnostic imaging · Abdominal radiography · Computed tomography · Magnetic resonance imaging ·
Professional education

Introduction Abdominal gas formations

The imaging evaluation of the abdomen is of crucial impor- Cupola sign


tance for every radiologist. In addition to ultrasound, con-
ventional radiographs and contrast-enhanced computed Free intraperitoneal air within the median subphrenic space
tomography (CT) are the most common imaging procedures is described as the “cupola sign” [1, 2]. Typically, the sign is
in the abdominal region. Radiographic images of the abdo- visible on radiographs at the thoraco-abdominal region when
men are an often used first step in radiologic imaging, while the patient was examined in supine position (Fig. 1). The air
CT examinations are carried out for further differentia- cupola projects onto the median, central diaphragm parts
tion, in oncological settings and in time-critical emergency overlaying with the lower thoracic spine. Because of the
situations. In all radiologic modalities, there are numerous sharp, defined, dome-like boarders between the air and the
pathognomonic signs which should be known by the radiolo- epigastric tissues, it has its name from the cupola. Intraperi-
gist. A fast and clear assignment of these signs to a specific toneal air accumulates particularly under the diaphragm, but
disease is the basis for a correct diagnosis. This pictorial also beneath the central tendon of the diaphragm and within
review describes the most common pathognomonic signs the median subphrenic space [1, 2]. This space is defined by
in abdominal imaging. the ventral stomach and the gastrohepatic ligament [1]. Due
to the manifold potential life-threatening causes of pneu-
moperitoneum, the sign is important to know. Computed
tomography is essential for further differentiation regarding
the underlying reason.

Coffee bean sign


* Christopher Kloth
christopher.kloth@uniklinik‑ulm.de The coffee bean sign (Fig. 2) is a pathognomonic radio-
1
Department of Diagnostic and Interventional Radiology,
graphic sign of dilated bowel that resembles the fissure of a
Ulm University Medical Center, Albert‑Einstein‑Allee 23, coffee bean [3, 4]. The reasons can be a sigmoid volvulus or
89081 Ulm, Germany

13
Vol.:(0123456789)
Abdominal Radiology

Fig. 1  Cupola Sign. Radiograph of thoracic-abdominal transition of cupola sign. Besides the pneumoperitoneum in the median sub-
a 62-year-old male patient 3  days after surgery of the bladder with phrenic space, free air is also demarked directly under the diaphragm
demarked air in the median subphrenic space (arrows), known as on the right and on the left side (asterix)

Fig. 2  Coffee bean sign. Abdominal radiograph examination (a) and enhanced CT images each in coronal view in soft tissue (b) and lung (c)
window with dilated bowel reminiscent of the fissure of a coffee bean

a large bowel obstruction. Volvulus represents an abdominal


emergency and is one of the most common causes of large
bowel obstruction in adults [5]. It is typically seen in elderly
men over 50 years of age [5]. A potential colonoscopic
detorsion is the treatment of choice for stable patients with-
out ischemia. The coffee bean sign can be seen as a pathog-
nomonic sign on radiographs, which is diagnostic in 57–90%
of patients [5]. Additional contrast-enhanced abdominal CTs
are often performed due to unspecific clinical symptoms or
to exclude ischemia or perforation.

Rigler and pseudo Rigler sign

Rigler sign

The Rigler sign was described first by the American radi-


ologist Leo George Rigler in 1941 [6, 7]. It is defined by
the visibility of the bowel wall and serosal surface on both
sides through free air in the abdominal cavity (pneumoperi-
toneum, Fig. 3). Because of this it is also called the double
wall sign [8]. The sign shows the presence of free air along Fig. 3  Rigler sign. Abdominal radiograph examination of a 2-week-
old male child with demarked bowel wall in the middle upper abdo-
the luminal bowel wall on supine abdominal radiographs
men. The examination was 1 week after volvulus operation, a perito-
and reflects always an emergency situation because an intes- neal tube line is still in the lower abdomen. The Rigler sign (arrows)
tinal perforation must be excluded [9]. Early diagnosis of demarked the free air around the bowel wall beside the feeding tube
intestinal perforation or other potential threatening reasons in the upper abdomen

13
Abdominal Radiology

(infection, trauma, and medication) is obligatory. It can be


also caused by surgical manipulation or diagnostic interven-
tions so that a precise anamnesis is necessary.
Sensitivity for the detection of pneumoperitoneum by
abdominal radiography varies in the literature between 60
and 90% depending on the amount of free air. CT scan has
greater sensitivity and specificity than conventional radio-
graphic technique [10].

Pseudo Rigler sign

In addition to the Rigler sign, a similar popular and wide-


spread sign is the Pseudo Rigler sign (Fig.  4) which is
defined by a lack of visualization of both sides of the intes-
tinal wall when two dilated bowel loops have contact during
X-ray [11]. Various diagnoses or pathomechanisms are pos-
sible for this imaging sign: In addition to free fluid between
the contacting bowel walls, an inflammatory wall thickening
is also conceivable. Fig. 5  Football Sign. Radiograph in supine position of a 3-day-old
male premature infant showing massive pneumoperitoneum caused
by perforation of the stomach (arrows). Free abdominal gas leads to
Abdominal gas formations in pediatrics the football sign showing an oval radiolucency and the median umbil-
ical ligament and the falciform ligament as the sutures reminiscent of
Football sign an American football

After spontaneous or iatrogenic perforation of a hollow bowel obstruction with secondary perforation or iatrogenic
viscus with massive pneumoperitoneum, the football sign perforation [14].
can be seen on supine abdominal radiographs commonly in
infants [12]. In these cases, the abdominal cavity is outlined Double bubble sign
by gas as an oval radiolucency, and the median umbilical
ligament and the falciform ligament are representing the Duodenal atresia is a rare congenital disease and can be seen
sutures of an American football (Fig. 5). The football sign in 1 in 5000 to 10,000 live births [15]. If duodenal atresia is
was first described in 1960 by Miller et al. [13]. It is often not diagnosed prenatally, the diagnosis can be made radio-
seen in children with advanced necrotizing enterocolitis, and graphically with plain abdominal radiographs after birth.

Fig. 4  Pseudo Rigler Sign. Abdominal radiograph examination a in computed tomography was performed after the radiograph examina-
supine position of a 62-year-old male patient with myelodysplastic tion (b, c). In coronal and axial series, free fluid is identified (arrows),
syndrome after allogeneic stem cell transplantation. After an epi- analog to the abdominal x-ray. Typical Pseudo Rigler sign can be
sode of diarrhea, the patient had bloated abdomen and reduced bowel seen here, which is defined by a lack of visualization of both sides of
movement. Due to suspected ileus and differential diagnosis of a the intestinal wall when two dilated bowel loops have contact during
graft-vs-host disease of the intestine, an additional contrast-enhanced X-ray

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Abdominal Radiology

Typically, the Double bubble sign (Fig. 6) is presenting as with duodenal atresia are presenting with vomiting typically
a large air-filled stomach in the left upper abdomen and a following the first oral feeding. Treatment involves nasogas-
smaller, more distal bubble to the right of midline which rep- tric suction to decompress the stomach followed by surgery
resents a dilated proximal duodenum [16]. Duodenal atresia to correct the obstruction [18].
is often associated with other anomalies, including trisomy
21 and cardiac malformations [17]. The obstruction is usu-
ally located distal to the ampulla of Vater, commonly caused Imaging signs in sectional imaging (CT/MRI)
by an error of duodenal re-canalization during the eighth
to the tenth week of embryological development. Neonates Vascular signs

Coral reef aorta

Extensive squamous calcifications of the aortic lumen with


ulcerations and an almost occlusive stenosis of the vessel
lumen due to the regular width of aortic diameter is the phe-
nomenon of a coral reef aorta (Fig. 7). The phenomenon
was named in 1984 by Qvarfordt et al. who reported about
nine patients with suprarenal obstructive calcifications of
the aorta [19, 20]. The frequency reported in the literature
ranges from 0.6% to 1.8% [20, 21]. Consecutive possible
symptoms are hypertension, intermittent claudication, and
visceral ischemia [20]. In most cases, the diagnosis is not
made until imaging is performed due to vascular compli-
cations [22]. Necessity of surgery is given in most cases,
depending on the exact localization and overall situation of
the patient. High operative mortality is associated with sur-
geries such as thromboendarterectomy, open surgery, bypass
surgery, or replacement of an aortic segment [19].

Teardrop sign of superior mesenteric vein

Surgical resection of pancreatic carcinomas is crucial for


Fig. 6  Double bubble sign. Abdominal radiograph examination in survival. Tumor infiltration into adjacent vessels, especially
supine position of a 4-day-old female infant with a duodenal atresia
the superior mesenteric artery and vein, remains the most
showing two air-filled bubbles representing the stomach and the duo-
denum (arrows). Additionally, a feeding tube and an umbilical venous important limitation for pancreaticoduodenectomy (Whip-
catheter is shown ple operation). Various imaging signs were evaluated with

Fig. 7  Coral reef aorta. Unenhanced transversal CT images of a 60-year-old female patient are presented in soft tissue (a) and lung (b) window.
Multiplanar reconstructions with sagittal view on the aorta in bone (c) and lung window (d) are given

13
Abdominal Radiology

regard to irresectability around the superior mesenteric vein, mesenteric artery (SMA) [28]. Typically, the small-bowel
including the “teardrop sign” (Fig. 8). This narrowing of and SMV were rotated clockwise around the SMA. Sen-
the superior mesenteric vein by carcinomas of the pancre- sitivity of up to 90% for ultrasound is described in the
atic head next to the confluence of the portal vein was first literature; however, CT is modality of choice.
described by Hough et al. [23]. The pathological change of
vessel configuration indicates an involvement in tumor or
venous obliteration. In addition to this tumor involvement of Sandwich sign
more than half of the vessel circumference and wall irregu-
larities of the superior mesenteric artery and vein, this indi- In mesenteric lymphoma, the sandwich sign can be seen
cates tumor infiltration and irresectability of the tumor [24]. in the transverse images of contrast-enhanced computed
With modern surgery techniques, pancreaticoduodenectomy tomography or ultrasound (Fig. 10). The mesenteric ves-
is also possible in borderline situations, however, the “tear- sels and the perivascular fat are reminiscent of sandwich
drop sign” is still associated with the necessity of extensive filling. In this context, the surrounding confluent lymph
vascular reconstruction and poor prognosis [25]. nodes represent the two halves of the sandwich bun [29,
30]. Mesenteric lymphadenopathy can occur in different
Whirl‑pool sign diseases such as lymphoma, carcinoma, abdominal tuber-
culosis, or inflammatory bowel disease, however, they
In CT examinations, the combined “whirl-pool-sign” do not produce large bulky adenopathy. The sandwich
(Fig. 9) is often registered together with the coffee bean sign is specific for mesenteric lymphoma causing large
sign [26]. The sign reflects the twisted mesenteric ves- bulky lymphomas enveloping fat, bowel, and vessels [31].
sels feeding the volvulus part of the colon. The “whirl The most common cause producing this sign is a non-
sign” was first described in 1981 by Fisher [27], who noted Hodgkin´s lymphoma. As a rare condition, it can also be
that the superior mesenteric artery was twisted and sur- seen in patients with post-transplant lymphoproliferative
rounded by bowel loops. Typically, with sonography it is disorder [32]. The sandwich sign is not specific, but it can
possible to identify the rotation of the small-bowel loops help in suggesting the diagnosis of lymphoma in order to
and superior mesenteric vein (SMV) around the superior initiate an early and appropriate treatment.

Fig. 8  Teardrop sign. Enhanced


transversal CT images of
a 60-year-old male patient
with adenocarcinoma of the
pancreatic head in portalvenous
phase (arrows). Embedding of
the upper mesenteric vein with
teardrop configuration is shown

Fig. 9  Whirl sign. Enhanced


transversal (a) and coronal
(b) CT images of a 54-year-
old male patient showing the
typically twisted mesenteric
vessels (arrows) with associated
volvulus without any previous
operations or any underlying
disease. The diagnosis was
confirmed at surgery

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Abdominal Radiology

Fig. 10  Sandwich sign.
Enhanced coronal (a) and
transversal (b) CT images of a
55-year-old male patient with
mesenteric lymphoma. The
mesenteric vessel, the perivas-
cular fat, and the surrounding
lymph nodes (arrows) represent
the sandwich sign

to moderately differentiated HCC as proof of multistep hepa-


tocarcinogenesis [37].

Sister Mary Joseph sign

A well-known imaging sign in internal medicine, gyne-


cology, and radiology is the “Sister Mary Joseph sign”,
defined as a metastasis in the umbilicus [38, 39]. The sign
was named by Hamilton Bailey in 1960 after Sister Mary
Joseph who was the first person to detect it [40, 41]. Sister
Mary Joseph (1856–1939) worked as a surgical assistant,
first recognizing an umbilical nodule in a surgery as a veri-
fied sign of intraabdominal metastasis and then Hamilton
Bailey shaped the term “Sister Mary-Joseph nodule” [41].
Fig. 11  Nodule-in-Nodule sign. Contrast-enhanced CT of a 68-year-
It represents intraabdominal malignancy with ascites and
old male patient with histologically proved HCC lesion (BCLC sta- peritoneal carcinosis that extends into the umbilicus, often
dium B). In the venous phase, hypervascular nodule (arrows) in the with herniation (Fig. 12). In the literature, the most common
central part of the HCC lesion is demarked form is a spread per continuitatem from the peritoneum;
however, genesis by iatrogenic spread after laparoscopy is
also possible [41]. Classically, it is detected in the physi-
Signs in oncology cal examination as a first hint of underlying cancer disease;
however, it can often be misinterpreted as simple hernia or
Nodule‑in‑nodule sign it is overseen . Various malignancies can be underlying, in
most cases adenocarcinomas of the gastrointestinal tract or
Several imaging signs are associated with hepatocellular ovarian cancer [41]. It can be observed both in men and
carcinoma (HCC) in different imaging techniques. A well- women, however, a poor prognosis is always associated [41].
known sign is the “nodule-in-nodule” appearance of the
relatively small HCC in MRI (Fig. 11) as well as in other Omental cake
imaging modalities [33, 34]. This imaging mark reflects
an area of increased arterial contrast enhancement in liver The abnormally thickened greater omentum is called omen-
parenchyma, in contrast to non-enhancing peripheral parts of tal cake (Fig. 13). The underlying pathology of this diffuse
HCC lesion [35]. An often discussed theory is that “nodule- peritoneal infiltration is most commonly the spread of an
in-nodule” sign within an HCC lesion may mark a part of intraperitoneal tumor which causes a diffuse or located
the tumor that has a different histological grading or differ- thickening of the omentum [42, 43]. Peritoneal metastases
entiation than the major part of the lesion. In particular, it is most often originate from tumors from the ovary, stomach,
suggested in early stage HCCs and degenerating dysplastic colon, pancreas, uterus, or bladder. Malignant melanoma
nodules [36]. Moreover, in verified HCC lesions this could and breast and lung carcinoma can cause hematogenous
show a dedifferentiation process from a well-differentiated peritoneal metastases [44]. Less common conditions causing

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Abdominal Radiology

Fig. 12  Sister Mary Joseph


sign. Contrast-enhanced com-
puted tomography of the abdo-
men of a 76-year-old female
patient with ovarian cancer and
advanced peritoneal carcinosis.
Sagittal (a) and axial (b) images
of the abdomen with ascites and
streaky peritoneal carcinosis.
Sister Mary Joseph nodule is
demarked (arrows) as a solid
peritoneal carcinosis nodule at
the umbilicus

Fig. 13  Omental cake. Enhanced transversal (a) and coronal (b) CT images of a 50-year-old male patient with tumor of the gastro-esophageal
junction. Ascites and a secondary peritoneal carcinosis is shown in the form of omental cake

an omental cake include primary tumors or inflammatory parenchyma. Hereby, the normal organ parenchyma encloses
conditions [42]. Primary tumors of the omentum include the tumor like a claw.
benign entities such as leiomyomas or lipomas and malig- The sign can be helpful in all modalities and all body
nant tumors such as mesothelioma or fibrosarcoma [45]. regions. In abdominal radiology, it is mainly used not only
Peritoneal tuberculosis is a rare inflammatory condition, for the tumors of kidneys (especially for Wilms tumor), but
which is often hard to distinguish from peritoneal carcino- also for the tumors of ovaries and uterus [47, 48]. Typical
matosis [46]. examples in which the claw symbol is useful include the dif-
ferentiation of a Wilms tumor from a neuroblastoma [49] or
Claw sign a renal angiomyolipoma from a retroperitoneal liposarcoma.

The claw sign is useful for differentiating whether a tumor Thoraco‑abdominal junction
originates from an organ or other solid structure itself or
is located adjacent and only displaces it. Its name derives Upside–down stomach
from the sharp angles on both sides of the tumor formed
by the surrounding normal organ parenchyma (Figs. 14 and Different forms of herniation of the stomach into the tho-
15). These angles can only be detected if the tumor origi- rax are possible. The rarest type of hiatal hernia is the
nates from the organ itself and displaces or thins out the upside–down stomach (Fig. 16), with a complete or nearly

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Abdominal Radiology

Figs.  14 and 15  Claw sign. Wilms tumor in a 2-year-old boy with the surrounding normal organ parenchyma. These angles can only be
typical claw sign (arrows). Contrast-enhanced T1-w MRI in axial detected if the tumor originates from the organ itself and displaces or
(a) and coronal (b) orientation. The claw sign can be seen here, thins out the parenchyma. Typically, it can help for the differentiation
derives from the sharp angles on both sides of the tumor formed by of a Wilms tumor from a neuroblastoma

Fig. 16  Upside–down stomach.
Posteroanterior (a) and lateral
(b) chest X-ray showed a large
herniation of almost the whole
stomach into the posterior
mediastinum in a 78-year-old
female patient (arrows). In the
enhanced computed tomogra-
phy in coronal (c) and axial (d)
images, the entire extent of the
gastric herniation with partial
rotation is demarked (arrows)

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Abdominal Radiology

Fig. 17  Water lily sign. Enhanced transversal CT images of a examination, floating membranes within the cyst were demarked in
25-year-old male patient with upper abdominal pain with demarked T2-w images that mimic the appearance of a water lily. The water lily
cystic lesion in the liver. The cystic lesion shows a rim enhance- sign is suggestive for Echinococcus granulosus infection which was
ment; however, no structures within. In additionally performed MRI approved in the patient by serology

complete herniation of the stomach into the posterior medi- in cases of hydatid infections (Fig. 17), e.g., Echinococ-
astinum [50, 51]. Other minor forms of migration are hiatal cus granulosus, the water lily sign can be seen typically
hernia at the gastro-esophageal junction, paraesophageal in T2-weighted MRI imaging sequences [56]. A potential
hernia of a minor part of the stomach, or a combination detachment of the endocyst membrane results in floating
of both [52, 53]. Also, an additional herniation beside the membranes within the main cyst that mimic the appear-
upside-down stomach in form of an additional paraesoph- ance of a water lily [57]. Also, calcifications of the main
ageal herniation through the hiatus or a secondary gap is capsule were possible, described in about 25 percent of
possible [54]. Various pathomechanisms or a combination all cases in the literature [58]. Free floating fragments in
of them are possible for an upside-down stomach: besides a cyst were specific for echinococcosis. The cystic lesions
increased intraabdominal pressure, trauma including widen- can be singular or multilocular. Even in CT examinations,
ing of the diaphragmatic hiatus or shortening of the esopha- the cyst wall can be detected as a well-defined thin line
gus are potential reasons [51, 54]. The stomach is usually [58]. In T1-w sequences after contrast agent application
fixed in the upper abdomen without the possibility of migra- the membranes can be masked. Overall the water-lily sign
tion or volvulus. The most rigid point is the first part of is seen in transitional stage between active and inactive
the duodenum in the retroperitoneum. This point regularly disease [59, 60].
limits a potential volvulus. Furthermore, a fixation between
gastrocolic and gastrosplenic ligaments is given [54]. Hence,
the stomach rather tends to organoaxial and mesenteroaxial
torsion than herniation into the posterior mediastinum [54]. Conclusion
Symptoms may include reflux and mechanically impaired
gastric emptying [55]. There is also an increased risk of In abdominal radiology, there are numerous memorable
incarceration and volvulus development [55]. Imaging diag- imaging signs. These are often pathognomonic for a specific
nosis is usually possible by fluoroscopy, computed tomog- disease and can therefore significantly simplify diagnosis.
raphy, or radiographs. The knowledge of these pictograms is therefore essential for
radiologists interested in abdominal imaging and should also
be addressed in training and further education.
Infection

Water lily sign describes the floating endocyst membrane Compliance with ethical standards 
within a bigger fluid filed cyst mimicking the appearance
of a water lily. Typically it can be seen in hydatid infec- Conflict of interest  The authors declares that they have no conflicts
of interest.
tions on plain radiographs or on CT and MRI. In this con-
text it can divided into the outermost layer (is called peri- Ethical approval  All procedures performed in studies involving human
cyst), the middle laminated membrane (which is acellular) participants were in accordance with the ethical standards of the insti-
and third the inner membrane. The water lily sign is clas- tutional and/or national research committee and with the 1964 Helsinki
declaration and its later amendments or comparable ethical standards.
sically described on radiographics of the chest, however,

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Abdominal Radiology

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