Keywords wandering spleen - torsion - volvulus - CT - ultrasound
Introduction
Wandering spleen, also called as floating spleen, ectopic, or ptotic spleen, is a
rare clinical entity which is found in < 0.2% of splenectomies.[1 ] It results from congenital or acquired hyperlaxity of peritoneal splenic ligaments
allowing the spleen to essentially drop to the lower abdomen by the force of gravity
attached only to its abnormally elongated vascular pedicle.[2 ] Rarely, there might be involvement of adjacent organs in the torsed splenic pedicle.
Most cases are asymptomatic initially and present to the emergency department only
after development of complications. Hence, proper understanding of a wandering spleen
and its complications is necessary to choose whether to perform a splenopexy (in case
of a viable spleen) or splenectomy (in case of an infarcted spleen).
Herein, we present a case of 26-year-old female patient with torsion of wandering
spleen, splenomegaly, splenenculi, and left-sided portal hypertension due to isolated
splenic vein thrombosis secondary to volvulus of tail of pancreas and splenic flexure.
Case Report
A 26-year-old multiparous female presented to the emergency department with complaints
of lower abdominal pain. There was no history of fever, prior surgeries, or trauma.
She had a similar episode of abdominal pain 9 months back which relieved on medication.
Patient had undergone elective lower segment caesarean section (LSCS) 1 year ago,
in view of previous LSCS and severe oligohydramnios. Family history was unremarkable.
Physical examination revealed that she was afebrile and vitals were stable. On palpation,
a large palpable mobile lump in mid-abdomen and suprapubic area with no significant
tenderness around the mass and with no rebound tenderness and guarding was observed.
Laboratory findings revealed leukocytosis of 19,400/mm3 , anemia (hemoglobin = 10.2), and normal platelet count.
Ultrasonography of the abdomen and pelvis revealed empty splenic fossa, massively
enlarged hypoechoic spleen measuring 25 cm in the lower abdomen and pelvis, and mild
ascites. Color Doppler study showed absence of color flow in splenic vein suggesting
splenic vein thrombosis. Splenic artery showed reduced diastolic flow on Doppler.
Contrast-enhanced computed tomography (CT) scan confirmed the ectopic location of
spleen in pelvis. The elongated pedicle had twisted around itself several times giving
a whirled appearance diagnostic of torsion. The tail of pancreas and splenic flexure
had also twisted along the pedicle suggestive of volvulus. However, no signs of pancreatitis
or bowel obstruction were seen. Hyperdense nonenhancing thrombus was noted along the
splenic vein. Left gastric and gastroepiploic venous collaterals were also identified
suggestive of gastric varices. Mild ascites was also seen. Two splenenculi of size
12 × 9 and 26 × 18mm were seen in splenic fossa.
Diagnosis of torsion of wandering spleen with involvement of pancreatic tail and splenic
flexure and chronic splenic vein thrombosis with asymptomatic isolated left-sided
portal hypertension was made.
The patient underwent laparotomy and an enlarged spleen with areas of hemorrhagic
infarcts was seen in the pelvis and lower abdomen. All the radiological findings described
above were confirmed. No reperfusion was demonstrated after detorsion of the spleen
and hence splenectomy was done.
The histopathology report suggested that the enlarged spleen was nonviable, had thrombi
in blood vessels with extensive areas of hemorrhagic necrosis in the parenchyma ([Figs. 1 ]
[2 ]
[3 ]
[4 ]
[5 ]
[6 ]
[7 ]
[8 ]
[9 ]
[10 ]).
Fig. 1 Ultrasonographic image shows abnormal location of spleen (star) in pelvis, in close
proximation to urinary bladder (arrow).
Fig. 2 Triplex Doppler image showing hypoechoic spleen with absence of color flow on color
Doppler and no spectral waveform.
Fig. 3 Noncontrast computed tomography (CT) image shows ectopic location of spleen in pelvis
and hyperdense thrombus in splenic vein (arrow) in a torsed splenic pedicle.
Fig. 4 Curved multiplanar reconstruction (MPR) in venous phase of contrast-enhanced computed
tomography (CT) shows abnormal position of enlarged spleen with elongated pedicle,
hyperdense nonenhancing thrombus in splenic vein, and splenenculus in splenic fossa.
The elongated pedicle is twisted upon itself with hypodense areas of intervening fat.
Fig. 5 Curved multiplanar reconstruction (MPR) in arterial phase of contrast-enhanced computed
tomography (CT) showing enlarged spleen extending from periumbilical region up to
pelvis with elongated splenic pedicle in a case of torsion of wandering spleen. Involvement
of splenic flexure and pancreatic tail in the torsed pedicle is also seen.
Fig. 6 Intraoperative image showing torsion of the elongated splenic pedicle in a case of
wandering spleen.
Fig. 7 Intraoperative image showing involvement of splenic flexure of colon in the torsed
pedicle (white arrow). Small bowel loop with mesentery is seen on the left of the
pedicle.
Fig. 8 Gross specimen of spleen showing an enlarged spleen with multiple areas of hemorrhagic
infarcts.
Fig. 9 Photomicrograph on high power magnification (40 × ) showing thrombosed splenic vein
(black arrow), hematoxylin and eosin (H&E).
Fig. 10 Photomicrograph on high power magnification (40 × ) showing loss of normal splenic
architecture with areas of infarction and necrosis, hematoxylin and eosin (H&E).
Discussion
Wandering spleen is a rare condition wherein spleen is found in an abnormal location
within the abdominal or pelvic cavity due to hyperlaxity, underdevelopment, or absence
of splenic suspensory ligaments.[3 ] It has a bimodal distribution pattern with the first peak in children less than
10 years and the second peak in women of child-bearing age group. Anomalous development
of dorsal mesogastrium resulting in failure of its fusion to posterior peritoneum
is the hypothesis in congenital cases that present before 10 years. It leads to absence
or abnormal development of one or more of gastrosplenic, splenorenal, or phrenicocolic
ligaments which hold the spleen in its normal position attached to the surrounding
viscera. The absence of the splenorenal ligament makes the pancreas not completely
retroperitoneal, with its tail localized within the splenic hilum.[4 ] Acquired cases are most likely due to multiparity, hormonal changes during pregnancy,
connective tissue disorders, splenomegaly (due to lymphoma, malaria, chronic myeloid
leukemia), trauma, and abdominal wall weakness.[5 ] Thus, an elongated pedicle predisposes to torsion. In a systematic review, splenic
torsion was diagnosed in 56% of pediatric patients with wandering spleen.[6 ] Torsion usually occurs counterclockwise, leads to chronic stasis in splenic vein,
increased backpressure in splenic vein, parenchymal congestion, splenomegaly, and
hypersplenism. Impaired venous return results in retrograde filling of short gastric
and left gastroepiploic veins.[7 ] Decompression of splenic venous outflow occurs through the short gastric veins,
coronary vein, and left gastroepiploic veins, producing gastric varices.[7 ] Thus, wandering spleen is an extremely rare cause of left-sided portal hypertension
and gastric variceal bleeding.[8 ]
Imaging plays a key role in establishing the diagnosis. Plain abdominal radiographs
may show absence of splenic silhouette and presence of small bowel loops in the left
upper quadrant; however, in most cases the findings are not conclusive. Ultrasonography
can help demonstrate an empty splenic fossa, localize the position of the wandered
spleen, and demonstrate splenomegaly if present. Echotexture of the spleen gives a
clue in regards to the extent of complications, for example, a hypoechoic spleen with
dilated hyperechoic thrombus in splenic vein suggests splenic vein thrombosis with
infarction due to torsion. Color Doppler study helps to evaluate the blood flow in
the parenchyma and in the splenic vessels. Tomographic examinations such as contrast-enhanced
CT or magnetic resonance imaging (MRI) help us identify involvement of adjacent viscera
and correctly identify accessory splenic tissue, if present. CT confirms the abnormally
positioned spleen, most commonly in the pelvis due to the effect of gravity. The “whirl
sign” of the splenic pedicle is highly specific and characteristic for splenic torsion.
It has been described in cases with involvement of pancreatic tail and part of descending
colon.[9 ] Careful evaluation of signs of pancreatitis and/or bowel obstruction is essential.
Poor enhancement of splenic parenchyma, hyperattenuating pedicle on unenhanced CT
due to acute thrombosis, or peripheral enhancement of splenic parenchyma (“pseudocapsule
sign”) are the features suggesting vascular compromise and splenic. Contrast-enhanced
MRI is helpful to assess viability of splenic tissue.[10 ]
Depending on the organ's viability, surgical treatment options like open or laparoscopic
splenopexy can be done if the spleen shows proper reperfusion after detorsion.[11 ] However, partial subtotal resection or splenectomy is considered when the spleen
is substantially infarcted. Vaccination against capsulated pathogens like pneumococcus,
Haemophilus influenzae , and meningococcus is highly recommended postsplenectomy.[12 ]
A comprehensive review of published cases of wandering spleen with pancreatic tail
involvement has been shown in [Table 1 ]. Previously, only one case with involvement of descending colon has been documented
by Seif Amir Hosseini et al in a 9-year-old male child. We present the first case
showing involvement of splenic flexure in an adult.
Table 1
Literature review of cases of wandering spleen with pancreatic volvulus
No
Author
Year
Age
Sex
Parity
Clinical features
Additional radiology findings
Management
1
Sheflin et al[13 ]
1984
33
F
_
Acute abdomen
_
Laparotomy
2
Parker et al[14 ]
1984
28
F
_
Nausea, vomiting, abdominal pain, palpable mass
Splenectomy, distal pancreatectomy
3
Moll et al[15 ]
1996
30
F
Nulliparous
Acute abdomen, thrombocytopenia
Right adnexal mass
Splenectomy
4
Ugolini et al[16 ]
2000
40
F
_
Acute abdomen, nausea, vomiting
_
Exploratory laparotomy, splenectomy
5
Karaosmanoglu et al[17 ]
2015
22
F
N/S
Acute abdominal pain, distension, nausea
_
Selenography
6
Gilman and Thomas[18 ]
2003
24
F
Multiparous 36 weeks ANC
Acute pancreatitis, acute abdominal pain, nausea, and vomiting with p/h/o operated
diaphragmatic hernia
_
Splenectomy
7
Lacreuse et al[19 ]
2007
5
Fch
_
Intermittent abdominal pain with bilious vomiting
_
Laparoscopic splenopexy
8
Feroci et al[20 ]
2008
15
M
_
Acute abdomen, distension,
_
Splenectomy
9
Magno et al[21 ]
2011
3
Mch
_
Persistent vomiting, upper abdominal pain, and pancreatitis
_
Laparoscopic splenopexy
No
Author
Year
Age
Sex
Parity
Clinical features
Additional radiology findings
Management
10
Gorsi et al[4 ]
2014
16
M
_
Acute abdomen
Gastric volvulus (mesentricoaxial)
Open laparotomy, splenectomy, splenopexy, gastrojejunostomy
11
Flores-Ríos et al[10 ]
2015
22
F
N/S
Acute abdomen and vomiting
Mesentericoaxial gastric volvulus, right-sided descending colon, and sigmoid colon
Upper GI endoscopy
12
Torri et al[22 ]
2015
13
F
_
Abdominal pain, nausea, fever
_
Laparoscopic splenectomy
13
Aswani et al[23 ]
2015
14
F
_
Acute abdomen with bilious vomiting
Diaphragmatic hernia with an intrathoracic gastric volvulus
Herniorrhaphy, gastropexy, and splenopexy
14
Ahmadi and Tehrani[24 ]
2016
14
F
_
Periumbilical pain
Sigmoid volvulus
Exploratory laparotomy, splenectomy
15
Seif Amir Hosseini et al[9 ]
2018
9
Mch
_
Acute abdomen
Distal MPD dilatation, descending colon involvement
Exploratory laparotomy, splenectomy
16
Taydas et al[25 ]
2018
27
F
N/S
Progressive abdominal pain and distension
Multiple cysts in malrotated pancreas
N/S
17
Colombo et al[26 ]
2019
18
F
Nulliparous
Recurrent abdominal pain
_
Laparoscopic splenectomy
18
Ng et al[27 ]
2019
35
F
N/S
Left upper quadrant pain, Nausea, vomiting, constipation
_
Splenectomy, distal pancreatectomy
19
Asafu Adjaye Frimpong et al[28 ]
2019
14
F
Acute abdomen
Organoaxial gastric volvulus, and cholestasis
Emergency laparotomy
20
Saldívar-Martínez et al[29 ]
2021
43
F
N/S
Abdominal pain and past history of trauma
Lumbar hernia (Grynfeltt–Lesshaft)
Exploratory laparotomy, splenectomy, distal pancreatectomy
21
Shen et al[30 ]
2021
37
F
_
Acute abdomen
Gastric volvulus
Exploratory laparotomy, splenectomy
22
Our case
2022
26
F
Multiparous
Recurrent abdominal pain
Splenic flexure involvement
Exploratory laparotomy, splenectomy
Abbreviations: Fch, female child; F, female; M, male; Mch, male child; MPD, main pancreatic
duct; Multiparous 36 weeks ANC, multiparous pregnant female patient of 36 weeks gestation.
Conclusion
Splenic torsion with involvement of neighboring anatomical structures and congestive
splenomegaly with splenic vein thrombosis is a very rare condition. Accurate preoperative
imaging is mandatory. Ultrasonography should be the first choice of investigation,
followed by contrast-enhanced CT scan to look for viability of the splenic tissue
and complications of torsion.
