Wandering spleen

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Wandering spleen
Other namesPelvic spleen, displaced spleen, drifting spleen, splenoptosis, floating spleen, splenic ptosis
Wanderingspleen.PNG
A picture of an enlarged spleen taken using medical ultrasonography.
Specialty Angiology   OOjs UI icon edit-ltr-progressive.svg
Causes Connective tissue disease, complications of pregnancy

Wandering spleen (or pelvic spleen) is a rare medical disease caused by the loss or weakening of the ligaments that help to hold the spleen stationary. [1]

Contents

Symptoms and signs

Although symptoms include an enlargement in the size of the spleen, [2] or a change from the spleen's original position to another location, usually in either other parts of the abdomen or into the pelvis. This ability to move to other locations is commonly attributed to the spleen's pedicle being abnormally long. [3]

Physical factors may cause ischuria, constipation, as well as numerous spleen-related diseases such as hypersplenism, thrombocytopenia, and lymphoma. [4] Blocking of the arteries and torsion (twisting that interrupts the blood supply to that organ) in the spleen can also result in abdominal pain or swelling. [5] However, lack of visible symptoms — except in incidents of abdominal pain — makes the disease difficult for doctors to diagnose, [6] though medical imaging techniques such as medical ultrasonography, magnetic resonance imaging, or computed tomography can be used to confirm its occurrence. Characteristics of the disorder include the loss, weakening, or malformation of the ligaments [2] that help to keep the spleen located in the upper left part of the abdomen.[ citation needed ]

Cause

Though not a genetic disease, wandering spleen is often found at birth. It can occur in adults as the result of injuries and other similar conditions that cause the ligaments to weaken, such as connective tissue disease or pregnancy. [2] Wandering spleen (splenoptosis) predisposes the spleen to complications such as torsion, splenic infarction, pancreatic necrosis and rarely pseudocyst formation. [7]

Diagnosis

Treatment

The usual treatment is splenopexy, fixation of the spleen, but if there is no blood flow after unwinding the spleen through detorsion then splenectomy must be performed. [6] Although there have been few reported cases of treatment through laparoscopic surgery due to the rarity of the disease, it has been proven to be an effective surgical technique. [8]

Prevalence

Wandering spleen is most commonly diagnosed in young children [3] as well as women between the ages of 20 and 40. [6] Even so, the disease is very rare and fewer than 500 occurrences of the disease have been reported as of 2005, [3] of which around 148 (including both children and adult cases) were documented to have been from between 1960 and 1992. [4] Less than 0.5% of all splenectomies, surgical removal of the spleen, are performed due to having this disorder. In 1992, the youngest case of the literature of torsion of wandering spleen at two days of birth was reported in Lebanon, by Dr Edouard Sayad. [9]

Media

Susan Mayer, in season 2 of the television show Desperate Housewives , had an operation to fix her wandering spleen.

Related Research Articles

Spleen Internal organ in most vertebrate animals

The spleen is an organ found in all vertebrates. Similar in structure to a large lymph node, it acts primarily as a blood filter. The word spleen comes from Ancient Greek σπλήν (splḗn).

Splenectomy

A splenectomy is the surgical procedure that partially or completely removes the spleen.

Asplenia refers to the absence of normal spleen function and is associated with some serious infection risks. Hyposplenism is used to describe reduced ('hypo-') splenic functioning, but not as severely affected as with asplenism.

Splenomegaly Medical condition

Splenomegaly is an enlargement of the spleen. The spleen usually lies in the left upper quadrant (LUQ) of the human abdomen. Splenomegaly is one of the four cardinal signs of hypersplenism which include: some reduction in number of circulating blood cells affecting granulocytes, erythrocytes or platelets in any combination; a compensatory proliferative response in the bone marrow; and the potential for correction of these abnormalities by splenectomy. Splenomegaly is usually associated with increased workload, which suggests that it is a response to hyperfunction. It is therefore not surprising that splenomegaly is associated with any disease process that involves abnormal red blood cells being destroyed in the spleen. Other common causes include congestion due to portal hypertension and infiltration by leukemias and lymphomas. Thus, the finding of an enlarged spleen, along with caput medusae, is an important sign of portal hypertension.

An autosplenectomy is a negative outcome of disease and occurs when a disease damages the spleen to such an extent that it becomes shrunken and non-functional. The spleen is an important immunological organ that acts as a filter for red blood cells, triggers phagocytosis of invaders, and mounts an immunological response when necessary. Lack of a spleen, called asplenia, can occur by autosplenectomy or the surgical counterpart, splenectomy. Asplenia can increase susceptibility to infection. Autosplenectomy can occur in cases of sickle-cell disease where the misshapen cells block blood flow to the spleen, causing scarring and eventual atrophy of the organ. Autosplenectomy is a rare condition that is linked to certain diseases but is not a common occurrence. It is also seen in systemic lupus erythematosus (SLE).

Volvulus Twisting of part of the intestine, causing a bowel obstruction

A volvulus is when a loop of intestine twists around itself and the mesentery that supports it, resulting in a bowel obstruction. Symptoms include abdominal pain, abdominal bloating, vomiting, constipation, and bloody stool. Onset of symptoms may be rapid or more gradual. The mesentery may become so tightly twisted that blood flow to part of the intestine is cut off, resulting in ischemic bowel. In this situation there may be fever or significant pain when the abdomen is touched.

Splenic artery

The splenic artery or lienal artery is the blood vessel that supplies oxygenated blood to the spleen. It branches from the celiac artery, and follows a course superior to the pancreas. It is known for its tortuous path to the spleen.

Abdominal pregnancy Medical condition

An abdominal pregnancy can be regarded as a form of an ectopic pregnancy where the embryo or fetus is growing and developing outside the womb in the abdomen, but not in the Fallopian tube, ovary or broad ligament.

Pseudocyst Medical condition

Pseudocysts are like cysts, but lack epithelial or endothelial cells. Initial management consists of general supportive care. Symptoms and complications caused by pseudocysts require surgery. Computed tomography (CT) scans are used for initial imaging of cysts, and endoscopic ultrasounds are used in differentiating between cysts and pseudocysts. Endoscopic drainage is a popular and effective method of treating pseudocysts.

Splenic injury Medical condition

A splenic injury, which includes a ruptured spleen, is any injury to the spleen. The rupture of a normal spleen can be caused by trauma, such as a traffic collision.

Spigelian hernia Surgical condition

A Spigelian is the type of ventral hernia where aponeurotic fascia pushes through a hole in the junction of the Linea Semilunaris and the arcuate line creating a bulge. It appears in the abdomen lower quadrant between an area of dense fibrous tissue and abdominal wall muscles causing a

Splenic infarction Medical condition

Splenic infarction is a condition in which blood flow supply to the spleen is compromised, leading to partial or complete infarction in the organ. Splenic infarction occurs when the splenic artery or one of its branches are occluded, for example by a blood clot.

Hemoperitoneum is the presence of blood in the peritoneal cavity. The blood accumulates in the space between the inner lining of the abdominal wall and the internal abdominal organs. Hemoperitoneum is generally classified as a surgical emergency; in most cases, urgent laparotomy is needed to identify and control the source of the bleeding. In selected cases, careful observation may be permissible. The abdominal cavity is highly distensible and may easily hold greater than five liters of blood, or more than the entire circulating blood volume for an average-sized individual. Therefore, large-scale or rapid blood loss into the abdomen will reliably induce hemorrhagic shock and, if untreated, may rapidly lead to death.

Phrenicocolic ligament

A fold of peritoneum, the phrenicocolic ligament is continued from the left colic flexure to the thoracic diaphragm opposite the tenth and eleventh ribs; it passes below and serves to support the spleen, and therefore has received the name of sustentaculum lienis.

Accessory spleen Small nodule found apart from the main body of the spleen

An accessory spleen is a small nodule of splenic tissue found apart from the main body of the spleen. Accessory spleens are found in approximately 10 percent of the population and are typically around 1 centimetre in diameter. They may resemble a lymph node or a small spleen. They form either by the result of developmental anomalies or trauma. They are medically significant in that they may result in interpretation errors in diagnostic imaging or continued symptoms after therapeutic splenectomy. Polysplenia is the presence of multiple accessory spleens rather than one normal spleen.

Blunt splenic trauma

Blunt splenic trauma occurs when a significant impact to the spleen from some outside source damages or ruptures the spleen. Treatment varies depending on severity, but often consists of embolism or splenectomy.

Ovarian torsion Medical condition

Ovarian torsion (OT) is when an ovary twists on its attachment to other structures, such that blood flow is decreased. Symptoms typically include pelvic pain on one side. While classically the pain is sudden in onset, this is not always the case. Other symptoms may include nausea. Complications may include infection, bleeding, or infertility.

An exploratory laparotomy is a general surgical operation where the abdomen is opened and the abdominal organs are examined for injury or disease. It is the standard of care in various blunt and penetrating trauma situations in which there may be life-threatening internal injuries. It is also used in certain diagnostic situations, in which the operation is undertaken in search of a unifying cause for multiple signs and symptoms of disease, and in the staging of some cancers.

Splenosis is the result of spleen tissue breaking off the main organ and implanting at another site inside the body. This is called heterotopic autotransplantation of the spleen. It most commonly occurs as a result of traumatic splenic rupture or abdominal surgery. Depending on the location of the spleen, the new piece usually implants in another part of the abdominal cavity. Single case reports also describe splenosis in the thoracic cavity, in subcutaneous tissue, in the liver or in the cranial cavity. Splenosis must be distinguished from the presence of additional spleens, which are innate and are the result of differences in embryological development. Additionally, splenosis must be differentiated from malignant tumors.

Spleen pain

Spleen pain is a pain felt from the left upper quadrant of the abdomen or epigastrium where the human spleen is located or neighboring.

References

  1. "Wandering spleen". Genetic and Rare Diseases Information Center (GARD) – an NCATS Program. rarediseases.info.nih.gov. Retrieved 2018-04-17.
  2. 1 2 3 "Wandering Spleen". NORD. Retrieved 2007-02-25.
  3. 1 2 3 Hasan Alawi, Malak; Ahmad Khalifa; Sami Hassan Bana (October–December 2005). "Wandering Spleen: A Challenging Diagnosis" (PDF). Pakistan Journal of Medical Sciences. Open Publishing. Retrieved 2007-02-25.
  4. 1 2 Satyadas T, Nasir N, Bradpiece HA (April 27, 2002). "Wandering spleen: case report and literature review". J. R. Coll. Edinb. 47 (2): 512–4. PMID   12018698.
  5. "Wandering spleen". Archived from the original on 2012-02-05. Retrieved 2007-02-26.
  6. 1 2 3 Safioleas MC, Stamatakos MC, Diab AI, Safioleas PM (January 2007). "Wandering spleen with torsion of the pedicle". Saudi Med J. 28 (1): 135–6. PMID   17206307.
  7. M, Noushif (Jul 2011). "Splenic pseudocyst: a rare association with splenoptosis and vertebral segmentation anomalies". Singapore Med J. 52 (7): e141-2. PMID   21808945.
  8. Castellón-Pavón CJ, Valderrábano-González S, Anchústegui-Melgarejo P, et al. (December 2006). "[Laparoscopic splenectomy due to torsion of a wandering spleen]". Cir Esp (in Spanish). 80 (6): 406–8. doi:10.1016/s0009-739x(06)70996-4. PMID   17192227.
  9. Sayad E, Bouchi J, Abou Haidar A (1992). "[Volvulus of a wandering spleen on the 2nd day after birth]". Le Journal Médical Libanais. The Lebanese Medical Journal (in French). 40 (3): 163–5. PMID   1339897.
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