Last updated:
9/11/2024
Years published: 1994, 2003, 2008, 2024
NORD gratefully acknowledges Gioconda Alyea, MD (FMG), MS, National Organization for Rare Disorders, for assistance in the preparation of this report.
Wandering spleen is a rare condition characterized by the spleen’s abnormal mobility, where it shifts from its normal position in the upper left abdomen to other areas within the abdomen or pelvis. This occurs due to the absence or abnormal laxity of the ligaments that typically hold the spleen in place. These ligaments may be congenitally weak, or their weakness can be acquired later in life due to factors such as trauma, pregnancy, or connective tissue disorders.
Wandering spleen can sometimes be asymptomatic and is often discovered accidentally during imaging done for unrelated reasons. However, it can also cause symptoms if the spleen’s vascular pedicle twists (torsion), leading to infarction (tissue death) or if the spleen compresses nearby organs in its new position.
Once diagnosed, surgical treatment is typically required, either through splenopexy, which anchors the spleen in its proper place, or splenectomy, which involves removing the spleen, depending on its mobility and condition.
The signs and symptoms of wandering spleen vary widely. Many patients are asymptomatic, with the condition discovered incidentally during imaging for other reasons.
When symptoms do occur, they often involve abdominal pain, which may be acute, subacute, or chronic.
The acute presentation may present as an acute abdomen. Acute abdomen refers to sudden, severe abdominal pain, and, many times, it’s a sign of a medical emergency that requires immediate surgery. Most commonly, it manifests as intermittent acute or subacute, non-specific abdominal pain. This pain is due to the torsion and spontaneous detorsion of the loose splenic pedicle. Chronic torsion can lead to congestion and splenomegaly, resulting in a palpable, mobile mass, which is often a typical presentation.
This pain is frequently associated with the torsion (twisting) of the spleen’s vascular pedicle, leading to compromised blood flow, which can result in infarction or necrosis (tissue death) of the spleen.
Other symptoms due to the spleen’s abnormal position exerting pressure on adjacent organs may include:
Affected people might also have the following signs and symptoms:
The most severe complication is splenic infarction, where the spleen becomes enlarged (splenomegaly) and potentially necrotic (with death tissue) due to the loss of blood supply, leading to additional symptoms such as fatigue, anemia and thrombocytopenia (low platelet count). Platelets or thrombocytes are cells that are found in the blood and spleen. They help form blood clots to slow or stop bleeding and to help wounds heal.
The condition can be present from birth (congenital) resulting from developmental defects during the formation of the embryo inside the womb (embryogenesis) where a structure called the embryonic septum transversum doesn’t fuse correctly with the back wall of the abdomen. This can lead to weak or absent ligaments that normally hold the spleen in place in its normal position in the upper left abdomen (spleen ligaments). The disorder is not genetic in origin. Instead of ligaments, the spleen is attached by a stalk-like tissue supplied with blood vessels (vascular pedicle). If the pedicle is twisted in the course of the movement of the spleen, the blood supply may be interrupted or blocked (ischemia) to the point of severe damage to the blood vessels (infarction). Because there is little or nothing to hold in place the spleen “wanders” in the lower abdomen or pelvis where it may be mistaken for an unidentified abdominal mass.
Alternatively, it can be acquired due to factors like multiple pregnancies, connective tissue disorders, or trauma, which weaken the ligaments over time.
The spleen is a small organ located in the upper left portion of the abdomen. The spleen removes or filters out unnecessary or foreign material, breaks down and eliminates worn out blood cells, and produces white blood cells, which aid the body in fighting infection. Symptoms of wandering spleen are typically those associated with an abnormally large size of the spleen (splenomegaly) or the unusual position of the spleen in the abdomen. Enlargement is most often the result of twisting (torsion) of the splenic arteries and veins or, in some cases, the formation of a blood clot (infarct) in the spleen.
Wandering spleen, whether it is a condition with which a baby is born (congenital form) or is the result of multiple births in women or some sort of accident that may affect men and women (acquired form), is an extremely rare disorder. The first case of wandering spleen was reported in 1667. So far less than 600 cases have been reported worldwide.
The incidence of wandering spleen is unknown and, because the condition may be underdiagnosed, is difficult to determine. It usually reported between the ages of 20 and 40 years with sex ratios of 7 females to 1 male. Most women are of reproductive age at the time of presentation. Children make up about a third of all cases, with 30 percent under 10 years of age. Among such children, the male-female ratio is 1:1.
As noted, acquired wandering spleen is acquired usually during adulthood, and it affects females many times more frequently than males. This is probably due to the relaxation (laxity) of the splenic ligaments during the childbearing years. Pregnancy is thought to contribute to the laxity, which increases the frequency of acquired wandering spleen among women who have had children. It is mostly seen in women who have many children.
Diagnosis of wandering spleen is often incidental but can be suspected when an abdominal mass is noted that can be moved towards the spleen’s normal location.
Diagnosing a wandering spleen often happens by chance, especially if it’s discovered during tests or surgeries for other reasons. However, doctors may suspect it if they find a movable mass in the abdomen that can be shifted towards the spleen’s usual spot in the upper left abdomen.
To confirm the diagnosis, an ultrasound (USG) is usually the first step. This test uses sound waves to create images of the inside of your body. It can show if the spleen is missing from its normal position and whether it has moved to another area of the abdomen or pelvis. If the spleen’s blood supply is cut off due to twisting (called infarction), the ultrasound might show it as a mass with a different texture and a Doppler ultrasound can reveal a lack of blood flow to the spleen.
A CT scan, which takes detailed images of the organs, is especially useful for confirming a wandering spleen. It can show exactly where the spleen is located and how much damage has occurred if it’s twisted. Doctors often look for a “whorled” pattern in the images which indicates the spleen’s blood vessels are twisted.
An MRI is another imaging test that provides similar information. It can show where the spleen has moved and how healthy it is. Sometimes, a special contrast dye called gadolinium is used during the MRI to get an even clearer picture of the spleen’s condition.
The treatment of wandering spleen depends on the severity of symptoms and a thorough evaluation to determine the size, location and functional status of the spleen. Since the spleen helps to maintain the proper function of the blood and immune system, most treatments are aimed at conserving the spleen and maximizing its function. However, since a person can live reasonably well without spleen, surgical removal is considered.
The most conservative approach to the treatment of wandering spleen includes watchful waiting while observing splenic function and/or enlargement. Prevention of injury by avoiding contact sports or other activities that might threaten the spleen is also a part of the conservative approach.
However, treatment for wandering spleen is primarily surgical, and the approach depends on whether the spleen is still viable:
Following a splenectomy, patients need to be vaccinated against certain infections to prevent overwhelming post-splenectomy infection (OPSI), a serious complication due to the loss of the spleen’s immune functions. Vaccinations typically include those for Haemophilus influenzae type B, Streptococcus pneumoniae, and Neisseria meningitidis. Close monitoring for signs of infection is very important, especially in children.
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JOURNAL ARTICLES
Shibiru YA, Wondimu S, Almaw W. Wandering spleen presenting in the form of right sided pelvic mass and pain in a patient with AD-PCKD: a case report and review of the literature. J Med Case Rep. 2024 May 25;18(1):259. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11127297/
Kaur R, Dua A, Dalal A. Wandering spleen: A rare entity and a diagnostic dilemma. Indian J Radiol Imaging. 2020 Jul-Sep;30(3):389-391. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7694723/
Upadhyaya P, St. Peter SD, Holcomb III GW. Laparoscopic splenoplexy and cystectomy for an enlarged wandering spleen and splenic cyst. J Pediatr Surg. 2007;42:E23-E27.
Schaarschmidt K, Lempe M, Kolberg-Schwerdt A, et al. The technique of laparoscopic retroperitoneal splenoplexy for symptomatic wandering spleen in childhood. J Pediatr Surg. 2005;40:575-7.
Brown CVR, Virgilio GR, Vazquez WD. Wandering spleen and its complications in children: a series and review of the literature. J Pediatr Surg. 2003;38:1676-79.
Kim SC, Kim DY, Kim IK. Avulsion of wandering spleen after traumatic torsion. J Pediatr Surg. 2003;38:622-23.
Steinberg R, Karmazyn B, Dlugy E, et al. Clinical presentation of wandering spleen. J Pediatr Surg. 2002;37:E30.
Andley M, Basu S, Chibber P, Internal herniation of wandering spleen/a rare cause of recurrent abdominal pain. Int Surg. 2000;85:322-24.
Peitgen K, Majetschak M, Walz MK. Laparoscopic splenopexy by peritoneal and omental pouch construction for intermittent splenic torsion (wandering spleen). Surg Endosc. 2001;15:413.
Nomura H, Haji S, Kuroda D, et al. Laparoscopic splenopexy for adult wandering spleen: sandwich method with two sheets of absorbable knitted mesh. Surg Laparosc Endosc Percutan Tech. 2000;10:332-34.
Danaci M, Belet U, Yalin T, et al. Power Doppler sonographic diagnosis of torsion in a wandering spleen. J Clin Ultrasound. 2000;28:246-48.
Vural M, Kacar S, Kosar U, et al. Symptomatic wandering accessory spleen in the pelvis: sonographic findings. J Clin Ultrasound. 1999;27:534-36.
Haj M, Bickel A, Weiss M, et al. Laparoscopic splenopexy of a wandering spleen. J Laparoendosc Adv Surg Tech A. 1999;9:357-60.
Kanthan R, Radhi JM. The ‘true’ splenic wanderer. Can J Gastroenterol. 1999;13:169-71.
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