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CT findings in congenital anomalies of the spleen

Article  in  British Journal of Radiology · September 2001

DOI: 10.1259/bjr.74.884.740767 · Source: PubMed

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The British Journal of Radiology, 74 (2001), 767–772 E 2001 The British Institute of Radiology

Pictorial review
CT findings in congenital anomalies of the spleen
1
G GAYER, MD, 2R ZISSIN, MD, 1S APTER, MD, 1E ATAR, MD, 1O PORTNOY, MD and
1
Y ITZCHAK, MD
Departments of Diagnostic Imaging, 1The Chaim Sheba Medical Center and 2Sapir Medical Center, Affiliated
to the Sackler Faculty of Medicine, Tel Aviv University, Israel

Abstract. There is a wide range of congenital anomalies of the spleen. Some are common, such as
splenic lobulation and accessory spleen. Other less common conditions, such as wandering spleen
and polysplenia, have particular clinical significance. Radiologists need to be aware of the various
congenital variants of the spleen in order to recognize clinically important anomalies and to avoid
mistaking less significant ones for an abnormality. In this pictorial review, the embryology of
congenital anomalies of the spleen as well as their appearance on CT are described, diagnostic
pitfalls are identified and complications of the anomalies are discussed.

The spleen is included and well demonstrated on
every CT of the abdomen. It is also often included
on CT of the lower part of the chest. There is a
wide spectrum of congenital anomalies, ranging
from the common splenic lobulation and acces-
sory spleen to rare conditions such as a wandering
spleen and polysplenia. The majority of these
anatomical variants have no clinical significance,
although they need to be recognized as anatomi-
cal variants by the radiologist. On the other hand,
a wandering spleen may rotate around its pedicle
and present as an acute abdomen due to splenic
infarction. Awareness of the various splenic
congenital variants is important for the radi-
ologist to interpret the findings correctly and to
avoid mistaking them for an abnormality.
We review the embryology of congenital
anomalies of the spleen as well as their appear-
ance on CT, stress pitfalls and describe complica-
tions resulting from these anomalies.
connection, the splenorenal ligament, between
the spleen and the left kidney (Figure 1c). The
gastrosplenic ligament is the portion of dorsal
mesentery between the spleen and the stomach
[1, 2]. The fetal spleen is lobulated, but these lob-
ules normally disappear before birth [1]. Splenic
lobulations may persist along the medial part of
the spleen (Figure 2a). A persisting bulge or
lobule of splenic tissue sometimes extends medi-
ally, anterior to the upper pole of the left kidney
(Figure 2b). Less often, such a lobule lies partially
posterior to the upper pole of the left kidney and
displaces it anteriorly [3].
The notches or clefts on the superior border of
the adult spleen are remnants of the grooves that
originally separated the fetal lobules [1]. These
clefts can be sharp and are occasionally as deep as
2–3 cm (Figure 3). They may be erroneously
interpreted as splenic laceration in patients with
abdominal trauma.

Splenic clefts, notches and lobulations

The spleen begins to develop during the fifth
week of fetal life from a mass of mesenchymal
cells originating in the dorsal mesogastrium
(Figure 1a). Rotation of the stomach and
growth of the dorsal mesogastrium translocate
the spleen from the midline to the left side of the
abdominal cavity (Figure 1b). Rotation of the
dorsal mesogastrium establishes a mesenteric
Received 23 June 2000 and in revised form 20 September
2000, accepted 16 November 2000.
Address correspondence to Dr G Gayer, Department
of Diagnostic Imaging, Sheba Medical Center, Tel
Hashomer 52621, Israel.
Accessory spleen
One or more small splenic masses may develop in
one of the peritoneal folds early in fetal life. An
accessory spleen is present in about 10% of
individuals, commonly situated near the hilum of
the spleen or adjacent to the tail of the pancreas [1,
4]. However, an accessory spleen may also occur
along the splenic vessels, in the gastrosplenic or
splenorenal ligaments (Figure 4), within the
pancreatic tail, in the wall of the stomach or bowel,
in the greater omentum or the mesentery or even
in the pelvis and scrotum [5, 6]. Accessory spleens
are usually about 1 cm in diameter, but vary

The British Journal of Radiology, August 2001 767


from microscopic deposits not visible on CT to
2 cm or 3 cm in diameter [1, 3, 4].
An accessory spleen resembles a lymph node,
both on CT and macroscopically [4]. It is round
or oval and its attenuation is identical to that of
splenic tissue, both before and after administra-
tion of contrast medium. When situated in an
atypical location, an accessory spleen can mimic
an enlarged lymph node as well as a tumour in the
adrenal gland, pancreas, stomach or intestine, and
even in the testis [6]. A mass in the splenic hilum
that fails to enhance to the same degree as the
splenic parenchyma should not be considered an
accessory spleen (Figure 5).
An accessory spleen is an incidental finding of
no clinical significance in most patients.
Awareness of the presence of an accessory
spleen is important in a patient evaluated by
CT prior to splenectomy, as failure to remove it
may result in persistence of the condition that
indicated the need for splenectomy [4]. An
accessory spleen may be of clinical importance
as a source of ‘‘preservable’’ splenic tissue in cases
of a ruptured primary spleen. An accessory spleen
can be reliably identified by radionuclide imaging
with technetium sulphur colloid if it is 2 cm or
greater in diameter [5]. Complications involving
an accessory spleen are rare and include torsion of
a wandering accessory spleen [7] or bleeding
caused by spontaneous rupture [8].
Polysplenia
Polysplenia is a complex congenital syndrome
characterized by partial visceral heterotaxia (situs
ambiguous) and concomitant levoisomerism
(bilateral left-sidedness). It is associated with
multiple, highly variable cardiovascular and
visceral anomalies. The splenic mass is usually
divided into fairly equally sized masses, varying in
number from two to six and ranging from 1 cm to
6 cm in diameter, which together equal the mass
of a normal spleen (Figure 6). Less often there
may be several small spleens adjacent to either
one or two large spleens (Figure 7). The location
of the spleens is in either the left or right upper
quadrant, along the greater curvature of the
stomach [9]. A single bilobed spleen is a rare
variant.
In addition to multiple spleens, there are often
other abdominal anomalies including a right-sided
stomach (Figure 6), a midline or left-sided liver
(Figure 6), malrotation of the intestine, a short
pancreas and inferior vena cava anomalies. These
should be recognized as part of a syndrome to avoid
misinterpreting them as pathological processes [10].
768
G Gayer, R Zissin, S Apter et al
Wandering spleen
Wandering or ectopic spleen refers to migration
of the spleen from its normally fixed location in
the left upper quadrant. The spleen is anchored in
its normal position by two ligaments: the gastro-
splenic ligament, which connects the greater
curvature of the stomach to the ventral aspect
of the spleen; and the splenorenal ligament
between the left kidney and the spleen, attaching
the spleen to the posterior abdominal wall [2, 11].
Failure of development of these ligaments results
in a long splenic mesentery and an abnormally
mobile spleen. Acquired factors that may increase
splenic mobility include abdominal wall laxity, the
hormonal effects of pregnancy and splenomegaly
[11]. This anomaly is quite rare, with a reported
incidence in several large series of splenectomies
of less than 0.5%. Wandering spleens are mainly
found in children [11] and in women aged 20–40
years [5]. The wandering spleen may be incident-
ally detected as an abdominal or pelvic mass. CT
findings of a wandering spleen are absence of the
spleen in its normal position with a location
somewhere else in the abdomen or pelvis
(Figure 8).
The major complication of a wandering spleen
is acute, chronic or intermittent torsion caused by
its increased mobility. Symptoms and signs of
splenic torsion are notoriously variable: chronic
abdominal discomfort probably due to splenic
congestion or ligamentous pressure, intermittent
pain presumably due to spontaneous torsion and
detorsion, and less often severe abdominal pain
from acute torsion and infarction, which produce
marked congestion and capsular stretching [5, 11,
12]. Physical examination may demonstrate a
tender mass [11].
In cases of torsion, the wandering spleen is not
only located in an abnormal position but shows
additional CT findings: (1) a circular whorled
structure of alternating bands of radiolucency and
radiodensity, usually at the splenic hilum, repre-
senting the splenic vessels and surrounding fat of
the twisted splenic pedicle (Figure 9a); (2) hyper-
dense intraluminal filling defects of the splenic
vessels on a pre-contrast scan (Figure 9a) and no
enhancement of the vessels on a post-contrast
scan in acute thrombosis; and (3) partial or total
failure of the spleen to enhance with iv contrast
medium, indicating infarction (Figure 9b). Contrast
enhanced CT in these cases provides information
concerning the viability of the splenic parenchyma.
This information is valuable in deciding whether
splenopexy rather than splenectomy is an option,
especially in young children [11].
The British Journal of Radiology, August 2001
Pictorial review: CT findings in congenital anomalies of the spleen

(a) (b)

Figure 1. Schematic cross-sections of the upper abdo-

men at different stages of embryonic development. L,
liver; S, stomach; Sp, spleen. Modified from reference
[1]. (a) At 5 weeks, the spleen, developing within the
dorsal mesogastrium (DM), is in the midline. (b) At
8 weeks, the spleen and the stomach rotate to the
left while the liver enlarges and rotates to the right.
(c) A mature fetus. A short splenorenal ligament
resulting from fusion of the dorsal mesogastrium
with the posterior peritoneum. The gastrosplenic liga-
ment is the portion of dorsal mesentery between the
spleen and the stomach. 1, falciform ligament; 2, gastro-
hepatic ligament; 3, gastrosplenic ligament; 4, spleno-
(c) renal ligament.

(a) (b)
Figure 2. Splenic lobulations in two different patients. (a) Typical lobulation along the medial aspect of the
spleen. (b) A prominent lobule of splenic tissue (S) extends medially, anterior to the upper pole of the left
kidney. It may be mistaken for a space-occupying lesion arising from the kidney. P, posterior.

The British Journal of Radiology, August 2001 769

 G Gayer, R Zissin, S Apter et al

Figure 4. Accessory spleen in a less typical location

Figure 3. Splenic cleft. A prominent cleft (arrow)
between lobulations of the spleen. This anatomical
variant may be mistaken for a laceration in trauma-
tized patients.
as an oval mass (arrowhead) measuring 1 cm61.5 cm,
posterior to the spleen, with attenuation identical to
that of the spleen.

(a) (b)

Figure 5. Varices medial to the spleen mimicking an

accessory spleen. (a) Pre-contrast CT, showing an
oval mass (w), measuring 2 cm61.5 cm, medial to
the spleen. Its attenuation on this unenhanced scan is
similar to that of the spleen. (b) On post-contrast
CT, the mass has enhanced to a greater degree than
the splenic parenchyma and is therefore not an acces-
sory spleen. (c) 2 cm caudal to (b). Multiple round
masses of higher attenuation than the spleen and situ-
ated medial to the spleen represent typical varices (v).
(c)
Note the large spleen, secondary to liver cirrhosis.

770 The British Journal of Radiology, August 2001

Pictorial review: CT findings in congenital anomalies of the spleen

Figure 6. Abdominal heterotaxia in a 30-year-old

woman with polysplenia syndrome evaluated for fever
and abdominal pain. There are two round splenules
(S) of similar size in the right upper quadrant. The
stomach (white arrow) is also right sided. The liver is
in the midline and its two lobes are equal in size.
The intrahepatic segment of the inferior vena cava
(black arrow) is on the left of the aorta.
Figure 7. Polysplenia syndrome in a 70-year-old
man with anaemia. Three round, soft tissue masses of
different sizes (w) in the left upper quadrant are
splenules. Note absence of the hepatic segment of
the inferior vena cava (IVC). A dilated azygos vein
(arrowhead) is situated in the right retrocrural space,
reflecting azygos continuation of the interrupted IVC,
a common anomaly in polysplenia syndrome.

(a) (b)

(c) (d)
Figure 8. Wandering spleen. A 26-year-old woman with vague abdominal pain and a palpable mid abdominal
mass. (a) CT at the level of the upper abdomen shows the left kidney (K) in a high position and absence of the
spleen in its normal anatomical location. The left lobe of the liver extends into the right upper quadrant. (b) A
more caudal image shows the spleen (S) in the left mid abdomen, mimicking the appearance of a space-occupying
lesion. (c) 4 years later, an image at the level of the pelvis shows the spleen has rotated and descended into the
pelvis. (d) The spleen (S) extends into the lower pelvis and is adjacent to the bladder (B) and the uterus (U). An
intrauterine device is present in the uterine cavity.

The British Journal of Radiology, August 2001 771

 G Gayer, R Zissin, S Apter et al

(a) (b)

Figure 9. Torsion of a wandering spleen. A 29-year-old woman with severe abdominal pain for 48 h and a history
of bouts of abdominal pain since childhood. (a) On a pre-contrast image of the upper abdomen, the spleen is
absent although splenic vessels (arrow) are seen in the left upper abdomen. These vessels have a whorled appear-
ance with a hyperdense centre, compatible with a twisted splenic pedicle and fresh thrombus in the splenic vessels.
(b) On a post-contrast image the torsioned spleen (S) lies in the left mid abdomen and shows no enhancement.

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