Journal of Pediatric Surgery (2009) 44, E15–E18
www.elsevier.com/locate/jpedsurg
Accessory spleen torsion: rare cause of acute abdomen
in children and review of literature
Pietro Impellizzeri, Angela S. Montalto, Francesca A. Borruto, Pietro Antonuccio,
Gianfranco Scalfari, Francesco Arena, Carmelo Romeo ⁎
Unit of Pediatric Surgery - University of Messina, 98124 Messina, Italy
Received 17 November 2008; revised 10 June 2009; accepted 12 June 2009
Key words:
Accessory spleen;
Torsion;
Acute abdomen
Abstract Torsion of an accessory spleen is an extremely rare condition. We describe an unusual case of
acute abdomen caused by torsion of an accessory spleen in a 12-year-old boy. The patient underwent a
laparotomy with splenectomy; the course was favorable. We discuss the clinical findings and values of
preoperative instrumental diagnosis. The literature is also reviewed. This is the 11th case reported in the
English literature. Torsion of an accessory spleen should be considered in the differential diagnosis of
acute abdomen or subacute abdominal pain.
© 2009 Elsevier Inc. All rights reserved.
Accessory spleen is a congenital anomaly characterized
by ectopic splenic tissue separated from the main body of the
spleen. It is a relatively common condition that appears in
10% to 30% of autopsy findings and is usually asymptomatic
[1]. Torsion is a possible complication and occurs exceptionally. Its clinical presentation is characterized by a
nonspecific acute onset or recurrent abdominal pain. The
diagnosis is difficult, even with the modern imaging
techniques [2,3]. We report a case of acute torsion of an
accessory spleen in a 12-year-old boy presenting with acute
abdominal pain. The preoperative diagnosis with abdominal
ultrasonography (US) and computerized tomography (CT)
was not specific. The literature is also reviewed.
⁎ Corresponding author. Pediatric Surgery Unit, Policlinico G. Martino
Viale Gazzi, 98124 Messina, Italy. Tel.: +39 0902213025; fax: +39
0902213025.
E-mail address: [email protected] (C. Romeo).
0022-3468/$ – see front matter © 2009 Elsevier Inc. All rights reserved.
doi:10.1016/j.jpedsurg.2009.06.029
1. Case report
A 12-year-old boy was admitted to our institution after 1
day of increasing intense abdominal pain with vomiting,
diarrhea, and no urinary symptoms. The pain was constant
and exacerbated by movements. At the time of admission,
there was mild pyrexia (37.5°C). Physical examination
revealed tenderness to palpation in the mesogastrium and
right iliac fossa with moderate resistance. Rectal tenderness
was also present. Laboratory findings disclosed only a white
blood cell count of 13 600 mmc with 84% neutrophils and creactive protein of 12.70 mg/dL. An ultrasound examination
was performed and revealed a well-defined, homogeneous
hypoecohogenic oval mass, measuring 8.7 cm in diameter, in
mesogastrium, anterior to the aorta and iliac bifurcation
(Fig. 1). Kidneys, liver, pancreas, and spleen appeared of
normal size and without any morphological alterations. No
evidence of ascites was documented. At Doppler US, no flow
within the mass could be recorded. At CT scan, a
E16
P. Impellizzeri et al.
Fig. 1
The US shows a well-defined, homogeneous hypoecohogenic oval mass, in mesogastrium.
homogeneous mass of 8.5 cm in the midportion of the
abdomen, within the mesentery, displacing adjacent loops of
bowel and in contact with anterior abdominal wall was
evident. The mass after intravenous injection remained
hypodense with a clear cleavage plane with adjacent
structures (Fig. 2). With the suspected diagnosis of congenital
malformations of the gut or complicated mesenteric cyst, the
patient was operated on. At laparotomy, a rounded violet
mass measuring 8,5 cm in diameter with a 760° torsion
around a long vascular pedicle along the left side of the
abdomen was found and resected (Fig. 3). The pedicle was
attached to the root of mesentery. During abdominal revision,
the appendix appeared inflamed and then resected. The
patient's postoperative course was uneventful, and he was
discharged in good condition on the sixth postoperative day.
Histological examination of the mass was consistent with
hemorrhagic and necrotic splenic tissue.
2. Discussion
The spleen derives, around the fifth week of pregnancy,
from mesenchymal tissue in the dorsal mesogastrium
between the pancreas and stomach. An accessory spleen
most likely originates from incomplete fusion of the
mesenchymal buds. The accessory spleen may be leaded
by splenic ligaments to ectopic locations. Accessory spleens
are always situated on the left side of the abdomen because of
the rotation of the spleen, during embryogenesis, to the left
side. The most common site of an accessory spleen is the
splenic hilum (75%), pancreatic tail (20%), splenic artery,
gastrosplenic and splenocolic ligament, and gastrocolic
ligament. Other rare locations are mesenterium, as in our
case, splenorenal ligament, greater omentum, jejunal wall,
presacral area, adnexal region, scrotum, and mediastinum
[1,2,4-6]. Accessory spleens can be either solitary or multiple
and receive their vascular supply from branches of the
splenic artery.
Usually, accessory spleen is asymptomatic; torsion and
infarction, rupture with bleeding, and infection with abscess
are a very rare complications [2,7,8]. In these cases, the
clinical presentation may be as acute abdomen or with a
history of intermittent abdominal pain of uncertain origin.
Intermittent torsion-detorsion may produce recurrent bouts
of abdominal pain caused by short-lasting ischemia of the
accessory spleen or from direct mechanical irritation of
surrounding organs [5]. Torsion of the accessory spleen with
Fig. 2 The CT images scan from the upper abdomen show the spleen in the usual location (S); scan shows at a lower level the homogeneous
hypodense mass (M).
Accessory spleen torsion
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accessory spleen presenting as acute abdomen (Table 1). A
case has been recently reported in a neonate [10].
Diagnostic imagings (US, CT) can provide many but
aspecific informations; however, the radiological findings are
mandatory for preoperative diagnosis despite not being
always available in an emergency situation. The US findings
can include different heterogenous features [1,2,11,12]. In our
case, US was characterized by well-defined, homogeneous
hypoechogenic oval mass in the mesogastrium. The absence
of flow within the mass documented at Doppler US did not
permit to distinguish between cystic or corpuscular nature.
The CT findings of a twisted infarcted accessory spleen
do not differ from that of an orthotopic splenic infarct [1-3].
In our case, the CT revealed only a cystic mass.
Consequently, the Doppler US and CT findings were not
suggestive of a twisted mass, and the exact organ involved
was not determined preoperatively. Moreover, the large size
of the mass was atypical for an accessory spleen, as well as
the lack of proximity to the spleen made the exact diagnosis
uncertain. In all the previous reported cases with torsion of
an accessory spleen, as our case, the diagnosis was made in
the operating room [1-4,7,13-17].
In conclusion, torsion of an accessory spleen is extremely
rare and is still a diagnostic dilemma. In presence of a patient
with acute or intermittent abdominal pain and an avascular
intraperitoneal mass, torsion of an accessory spleen should
also be considered in the differential diagnosis of acute
abdomen in children. This condition is very likely if the
Fig. 3 Intraoperative image highlights the mass within the
mesenterium with a 760° torsion of its long vascular pedicle.
resultant infarction and necrosis may cause an acute
abdomen secondary to long vascular pedicle twisting. This
entity is slightly different from splenic torsion in cases of
wandering spleen [9].
In our case, we documented an accessory spleen along the
mesenteric side presenting as acute abdomen. The torted
mass was very large, measuring 8.7 × 8.2 cm. The diagnosis
was made in the operating room and confirmed by
histological examination. The review of the literature
revealed only 13 cases in the pediatric age of torsion of
Table 1
Review of literature
Author
Cases
Age
Sex
Location
Diagnostic procedures
Settle, 1940 [15]
2
1
2
Nutman et al, 1983 [14]
Muller, 1988 [13]
Seo et al, 1994 [2]
1
1
1
Male
Female
Female
Male
Female
Female
Female
Male
Gastrosplenic ligament
Gastrocolic ligament
Not stated
Jejunal wall
Not stated
Great omentum
Great omentum
Great omentum
–
Terence et al, 1974 [16]
Onuigbo et al, 1978 [4]
4y
8y
5y
9y
5y
3.5 y
15 mo
10 y
Chateil et al, 1996 [12]
1
15 y
Female
Not stated
Valls et al, 1998 [1]
1
13 y
Female
Pancreatic tail
Pèrez Fontàn et al, 2001 [3]
1
13 y
Male
Great omentum
Gardikis et al, 2005 [10]
1
14 d
Female
Great omentum
Mendi et al, 2006 [17]
Present case
1
1
12 y
12 y
Female
Male
Splenic hilus
Mesenterium
MR indicates magnetic resonance.
–
–
–
US
US
CT
MR
US
CT
US
CT
X-ray
US
CT
MR
X-ray
US
CT
US
CT
E18
spleen is in normal position. However, preoperative
diagnosis is only hypothetical, and prompt surgical intervention is necessary for definitive diagnosis and treatment.
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