DOI:10.2214/AJR.05.0672
AJR 2007; 188:W328-W330
© American Roentgen Ray Society
Wandering Spleen: An Unusual Association with Gastric Volvulus
Huai-Tzu Michael Liu1,2 and
Kenneth K. Lau1
1 Department of Radiology, Monash Medical Center, Rm. 116, 246 Clayton Rd.,
Clayton South, Victoria 3168, Australia.
2 Present address: Department of Radiology, The Canberra Hospital, Yamba Dr.,
Garran, ACT 2065, Australia.
Received April 19, 2005;
accepted after revision June 17, 2005.
Address correspondence to H.-T. M. Liu
(htliu2003{at}yahoo.com.au).
WEB This is a Web exclusive article.
Keywords: abdomen abdominal imaging anatomy pediatric radiology spleen
Introduction
Wandering spleen is a rare condition characterized by the absence or
underdevelopment of one or all of the ligaments that hold the spleen in its
normal position in the left upper quadrant of the abdomen. Wandering spleen
and gastric volvulus share a common cause, the absence of an intraperitoneal
visceral ligament. Herein we describe an unusual case of pediatric wandering
spleen in association with gastric volvulus and diaphragmatic hernia.
Case Report
A 44-month-old girl presented to the accident and emergency department with
vomiting and dehydration. She had no history of fever, diarrhea, abdominal
pain, toxic ingestion, or trauma. On physical examination, she showed signs of
10% dehydration, with dry mucocutaneous membrane and reduced skin turgor. The
physical examination also revealed a firm epigastric mass that was non-tender
with abdominal palpation.
Abdominal radiography was unremarkable, showing no dilatation of bowel
loops. A barium meal examination was performed because of the clinical
suspicion of malrotation. It showed a left-sided congenital diaphragmatic
hernia and an intrathoracic gastric volvulus
(Fig. 1A). Sonography revealed
a medial displacement of the spleen immediately inferior to the right lobe of
liver (Fig. 1B). Subsequent CT
showed the intrathoracic position of the left kidney and stomach and a gastric
volvulus in the mesoaxial direction (along the line joining the greater and
lesser curvature) (Fig. 1C). It
also confirmed the displacement of the spleen to the right side of the abdomen
(Fig. 1D).

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Fig. 1A 44-month-old girl with wandering spleen in association with gastric
volvulus and diaphragmatic hernia. Barium meal examination shows leftsided
congenital diaphragmatic hernia containing intrathoracic gastric volvulus.
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Fig. 1B 44-month-old girl with wandering spleen in association with gastric
volvulus and diaphragmatic hernia. Sonogram of upper abdomen reveals abnormal
position of spleen, which lies inferior to right lobe of liver.
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Fig. 1C 44-month-old girl with wandering spleen in association with gastric
volvulus and diaphragmatic hernia. Axial CT image of lower chest shows
congenital diaphragmatic hernia containing stomach and left kidney.
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Fig. 1D 44-month-old girl with wandering spleen in association with gastric
volvulus and diaphragmatic hernia. Axial contrast-enhanced CT image confirms
displacement of spleen to inferomedial aspect of right lobe of liver.
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The child underwent an open laparotomy. At surgery, the findings of a large
left-sided diaphragmatic hernia with intrathoracic stomach and left kidney
were confirmed. A mesoaxial volvulus was seen. The spleen was noted to be
completely free-floating because of the lack of ligamentous attachment.
Splenopexy and gastropexy were performed. The patient's recovery was
uneventful and she was discharged on the fifth postoperative day.
Discussion
Gastric volvulus is defined as an abnormal degree of rotation of one part
of the stomach around another. Gastric volvulus is rare in pediatric patients
and is frequently described in association with congenital diaphragmatic
hernia and eventration of the diaphragm
[1,
2]. Most cases are mesoaxial
and occur with a rotation along the long axis of the gastrohepatic omentum.
Diagnostic delay can lead to gastric ischemia, perforation, and death
[3,
4].
Wandering spleen is by definition a mobile spleen that is attached only by
an elongated vascular pedicle, allowing it to migrate to any part of the
abdomen or pelvis. It is a rare congenital malformation resulting from
abnormal development of the splenic peritoneal attachments
[5,
6]. Most cases in children
occur when the child is younger than 1 year; the male-to-female ratio is 6:1
[7,
8]. During fetal development,
the dorsal mesogastrium fails to fuse with the posterior peritoneum, leading
to a laxity or absence of formation of splenic supporting ligaments
[5,
6]. These ligaments include the
pancreaticocolic, splenocolic, gastrosplenic, pancreaticosplenic,
phrenicocolic, splenorenal, and phrenicosplenic ligaments. For this reason,
the splenic pedicle has an increased risk of axial torsion that can lead to
splenic congestion and ultimately to splenic infarction
[7,
8].
The condition also can occur in adults at 20-40 years because of laxity of
the ligamentous support as a result of splenomegaly or pregnancy
[9]. Fifteen percent of
children with wandering spleen are asymptomatic, whereas 55% present with
abdominal pain and 90% present with a palpable mass outside the left upper
quadrant [10]. Torsion
complicates 64% of pediatric wandering spleens
[11]. Splenic torsion is
usually clockwise and can cause vascular congestion, infarction, and even
gangrene of the spleen [12,
13].
Several imaging studies may be helpful in establishing the diagnosis of
wandering spleen, and debate still exists concerning which test is most
appropriate. Abdominal radiography may reveal the absence of a splenic
silhouette [4]. Abdominal
sonography is useful in establishing the absence or presence of a spleen in
the left upper quadrant, but it may be obscured by bowel gas
[13]. Also, a duplex study can
be performed to assess splenic blood flow and is useful in establishing
whether splenic torsion is present
[13]. CT shows an abnormal
location of the spleen or abnormality of surrounding viscera, such as gastric
malposition or volvulus [1,
2,
5]. Other findings include
necrosis and involvement of the pancreatic tail and surrounding fat due to
torsed splenic vessels
[2-4].
Splenopexy is the procedure of choice to prevent future torsion while
preserving functional splenic tissue when a viable wandering spleen is found
at surgery
[10-12].
There is a rare association between gastric volvulus and wandering spleen
[2,
5]. The two entities share a
common cause, the absence or laxity of intraperitoneal visceral ligaments.
Prophylactic gastropexy in patients with wandering spleen may therefore be
implicated [5].
Our patient also had a coincidental left diaphragmatic hernia.
In conclusion, it is important to recognize a wandering spleen because of
the configuration of its vascular pedicle, which makes it prone to splenic
torsion. Wandering spleen has a rare association with gastric volvulus. Both
splenic torsion and gastric volvulus are potentially life-threatening if not
immediately managed surgically.
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volvulus mimicking pyloric stenosis. J Pediatr Child
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