|55 Kenosia Avenue
Danbury, CT 06810
Toll Free: 1.800.999.6673
The National Organization for Rare Disorders (NORD) web site, its databases, and the contents thereof are copyrighted by NORD. No part of the NORD web site, databases, or the contents may be copied in any way, including but not limited to the following: electronically downloading, storing in a retrieval system, or redistributing for any commercial purposes without the express written permission of NORD. Permission is hereby granted to print one hard copy of the information on an individual disease for your personal use, provided that such content is in no way modified, and the credit for the source (NORD) and NORD’s copyright notice are included on the printed copy. Any other electronic reproduction or other printed versions is strictly prohibited.
The information in NORD’s Rare Disease Database is for educational purposes only. It should never be used for diagnostic or treatment purposes. If you have questions regarding a medical condition, always seek the advice of your physician or other qualified health professional. NORD’s reports provide a brief overview of rare diseases. For more specific information, we encourage you to contact your personal physician or the agencies listed as “Resources” on this report.
Copyright 1994, 2003, 2008
Congenital wandering spleen is a very rare, randomly distributed birth defect characterized by the absence or weakness of one or more of the ligaments that hold the spleen in its normal position in the upper left abdomen. 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 it in place the spleen "wanders" in the lower abdomen or pelvis where it may be mistaken for an unidentified abdominal mass.
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.
"Acquired" wandering spleen may occur during adulthood due to injuries or other underlying conditions that may weaken the ligaments that hold the spleen in its normal position (e.g., connective tissue disease or pregnancy).
Some children with wandering spleen may have no symptoms (asymptomatic), while others may experience acute or chronic abdominal pain. In most cases, episodes of pain may be related to the spontaneous twisting and untwisting of the mobile spleen (torsion and detorsion) or of the blood vessels serving the spleen. Infants with wandering spleen may attempt to relieve pain by stretching. Other symptoms may include a bulging abdominal mass, constipation, bloating, nausea, vomiting, frequent difficult urination, and/or menstrual problems in women.
In some cases, the spleen may lack proper blood supply due to the twisting of the splenic arteries. In these cases, symptoms may include abdominal pain, abnormal enlargement of the spleen (splenomegaly), bleeding into the abdomen (infarct), the accumulation of fibrous tissue in the spleen (fibrosis), and/or decay of splenic tissue (necrosis). In severe cases, blood flow into the spleen is diminished and the spleen may become greatly enlarged, as it accumulates (sequesters) blood elements such as platelets and red blood cells. Resulting symptoms may include fatigue, weakness, blood in the stools, anemia, bloody vomit (hematemesis), and/or an abnormally low level of blood platelets (thrombocytopenia).
In adulthood, wandering spleen most often causes abdominal pain or present as an abdominal mass that does not cause symptoms (asymptomatic).
The exact cause of wandering spleen is not known. Researchers suspect that multiple factors play a role in the development of the disorder (multifactorial).
Babies may be born with a wandering spleen that may be the result of a defect in a certain area of the developing embryo (mesogastrium dorsum). This is the area of the embryo that gives rise to the ligaments that normally hold the spleen in the upper left abdomen. Affected children may be missing one or all of these ligaments, or, if present, the ligaments are not positioned properly. Symptoms usually develop due to the abnormal position of the spleen in the lower abdomen or because of the abnormal enlargement of the spleen (splenomegaly).
Wandering spleen may occur during adulthood because of accident or injury, another underlying disorder (e.g., connective tissue disease), or the abnormal relaxation (laxity) of the ligaments caused by pregnancy.
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. Fewer than 500 cases of wandering spleen have been reported in the medical literature.
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.
Symptoms of the following disorders can be similar to those of wandering spleen. Comparisons may be useful for a differential diagnosis:
Peritonitis is a common disease characterized by the inflammation of the membrane that lines the abdominal wall (peritoneum). It may be caused by bacteria or other infectious organisms that enter the abdomen through a wound or hole (perforation) in one of the abdominal organs (e.g., ruptured appendix). Symptoms may include abdominal pain and rigidity, enlargement of the abdomen, vomiting, decreased bowel function, nausea, and/or an abnormally rapid heartbeat (tachycardia). If not treated, late symptoms may include chills, fever, rapid breathing, and/or shock.
Appendicitis is a common disease characterized by the acute inflammation of the appendix. If left untreated, the appendix may burst and cause peritonitis. The most common symptoms of appendicitis include pain in the lower right abdomen, vomiting, fever, and/or abdominal tenderness and rigidity. Treatment involves the prompt surgical removal of the appendix.
Diverticulitis is a common digestive disorder characterized by inflammation of one or more of the sacs (diverticula) that can form due to protrusion of the inner lining of the colon through its intestinal wall. The major symptom of diverticulitis is pain near the groin in the lower part of the abdomen. Other symptoms may include pain when urinating, constipation, diarrhea or other changes in bowel movements, fever, and/or rectal bleeding. (For more information on this disorder, choose "Diverticulitis" as your search term in the Rare Disease Database.)
Intestinal obstruction is a common condition characterized by the blockage of the intestines and a lack of intestinal motility. This results in the failure of waste (feces) to pass through intestines and be eliminated. The most common causes of intestinal obstruction are adhesions from previous surgery, impacted stools, a narrowing of the bowel because of an inflammatory bowel disease, and/or the presence of a tumor. Symptoms may include a swollen abdomen, severe abdominal pain, nausea and vomiting, and/or constipation.
Cholecystitis is a common disease characterized by inflammation of the gall bladder. This disease, which is usually caused by the presence of gallstones, can be acute or chronic. Symptoms may include ongoing or episodic severe abdominal pain, chills, nausea and vomiting, indigestion, heartburn, gassiness, fever, and/or pain in the chest, shoulder, and back. There may also be some discomfort after eating, an intolerance to fatty foods, and/or a yellowish discoloration of the skin. (For more information on this disorder, choose "Cholecystitis" as your search term in the Rare Disease Database.)
Other common diseases may also have symptoms that are similar to those of wandering spleen. These include pyelonephritis, hiatal hernia, hepatitis, gastric ulcer, gastroenteritis, and/or pancreatitis.
The following disorders may be associated with Wandering Spleen as secondary characteristics. Comparisons are not necessary for a differential diagnosis:
Thrombocytopenia is a condition characterized by abnormally low levels of platelets in the circulating blood. When the spleen becomes enlarged, platelets or other blood elements may be "captured" (sequestered) in the spleen. Symptoms of thrombocytopenia may include excessive bleeding, the tendency to bruise easily, nosebleeds, and/or abnormally heavy menstrual flow in women. If an enlarged spleen (splenomegaly)is not detected in the abdomen, then some people with wandering spleen may be misdiagnosed with other blood disorders that involve low circulating platelets (i.e., autoimmune thrombocytopenia purpura).
Prune belly syndrome is a rare congenital disorder characterized by underdevelopment of the abdominal muscles. The syndrome is associated with a number of intestinal and urogenital abnormalities. Often, the attachments of the muscles to the bones are present, but the muscles are small in size and thickness. Children with prune belly syndrome typically have abnormally large abdomens and the skin may appear loose or lax. The chest may also have a horizontal depression (Harrison groove) or may be very narrow (pigeon breast). Some children with pune belly syndrome may have wandering spleen because of the underdevelopment of the ligaments that normally anchor the spleen in the upper left abdomen. (For more information on this disorder, choose "Prune Belly" as your search term in the Rare Disease Database.)
Other conditions that have been associated with wandering spleen include the absence or abnormal enlargement of a kidney, infectious mononucleosis, malaria, sickle cell anemia, and Hodgkin's disease. (For more information on these disorders, choose "Malaria," "Sickle Cell," and "Hodgkin" as your search terms in the Rare Disease Database or use "splenomegaly" as your search term to find other diseases that include an enlarged spleen.)
The diagnosis of wandering spleen is suspected when the pain associated with an abdominal mass can be relieved by moving it toward the upper left quadrant of the abdomen, the normal position of the spleen. Wandering spleen may be confirmed by specialized examinations such as ultrasonography and CT scan that enable the physician to view the structure, size, and placement of the spleen within the abdomen or pelvis. Specialized ultrasound tests (i.e., Doppler studies) may show impaired blood flow in and out of the spleen. Radioisotopic scanning (technetium 99 sulfur colloid scan), another imaging test, allows the physician to determine how well the liver and spleen are functioning. Low spleen function (functional asplenia) may suggest that the organ is damaged as a result of arterial obstruction (infarct).
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 a 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.
For children with congenital wandering spleen who are experiencing episodes of torsion and acute pain, the treatment of choice may be surgery that anchors the spleen back in the proper position in the upper left abdomen (splenopexy). In many cases, the spleen can be preserved and the risk of torsion and infarct is reduced. Preservation of the spleen is preferred to removal through surgery because absence of the spleen can make a person vulnerable to certain infections.
If wandering spleen causes chronic abdominal pain, abnormal enlargement of the spleen, and/or deficiencies of one or more necessary blood elements (i.e., thrombocytopenic hypersplenism), the treatment of choice is usually surgery to remove the spleen (splenectomy). Acute abdominal pain associated with wandering spleen is considered a surgical emergency and may require immediate splenectomy.
The potential complications of complete removal of the spleen (splenectomy) may include postsplenectomy infection syndrome, which includes life-threatening bacterial infections (sepsis). People who have had a splenectomy are at higher lifetime risk for serious infections than the general population. Immunizations to boost immunity against haemophilus influenzae B, streptococcus pneumoniae, seisseria meningitis, and other contagious diseases are usually administered before the splenectomy is performed. All people who have had a splenectomy must be observed carefully in case of fever or other symptoms of infection. Antibiotics may be prescribed to help prevent infectious disease (prophylaxis), especially in children under the age of 2 years.
Information on current clinical trials is posted on the Internet at www.clinicaltrials.gov. All studies receiving U.S. government funding, and some supported by private industry, are posted on this government website.
For information about clinical trials being conducted at the National Institutes of Health (NIH) Clinical Center in Bethesda, MD, contact the NIH Patient Recruitment Office:
Tollfree: (800) 411-1222
TTY: (866) 411-1010
For information about clinical trials sponsored by private sources, contact:
PO Box 8126
Gaithersburg, MD 20898-8126
Phone #: 301-251-4925
800 #: 888-205-2311
Home page: http://rarediseases.info.nih.gov/GARD/
1275 Mamaroneck Avenue
White Plains, NY 10605
Phone #: 914-997-4488
800 #: 888-663-4637
Home page: http://www.marchofdimes.com
P.O. Box 30105
Bethesda, MD 20892-0105
Phone #: 301-592-8573
800 #: --
Home page: http://www.nhlbi.nih.gov/
Benevento A, Boni L, Dionigi G, et al. Emergency laparoscopic splenectomy for wandering pelvic spleen: case report and review of the literature on laparoscopic approach to splenic diseases. Surg Endosc. 2002;16:1364-65.
Satyadas T, Nasir N, Bradpiece HA. Wandering spleen: case report and literature review. J R Coll Surg Edinb. 2002;47:512-14.
Gayer G, Zissin R, Apter S, et al. CT findings in congenital anomalies of the spleen. Br J Radiol. 2001;74:767-72.
Desai DC, Hebra A, Davidoff AM, et al. Wandering spleen: a challenging diagnosis. Douth Med J. 1997;90:439-43.
Horwitz JR, Black CT. Traumatic rupture of a wandering spleen in a child: case report and literature review. J Trauma. 1996;41:348-50.
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.
Report last updated: 2008/02/11 00:00:00 GMT+0