Splenectomy

Definition
Purpose
Demographics
Description
Diagnosis/Preparation
Aftercare
Risks
Normal results
Morbidity and mortality rates
Alternatives

Definition

A splenectomy is the total or partial surgical removal of the spleen, an organ that is part of the lymphatic system.

Purpose

The human spleen is a dark purple, bean-shaped organ located in the upper left side of the abdomen just behind the bottom of the rib cage. In adults, the spleen is about 4.8 X 2.8 X 1.6 in (12 X 7 X 4 cm) in size, and weighs about 4-5 oz (113-141 g). The spleen plays a role in the immune system of the body. It also filtersforeign substances from the blood and removes worn-out blood cells. The spleen regulates blood flow to the liver and sometimes stores blood cells—a function known as sequestration. In healthy adults, about 30% of blood platelets are sequestered in the spleen.

Splenectomies are performed for a variety of different reasons and with different degrees of urgency. Most splenectomies are done after a patient has been diagnosed with hypersplenism. Hypersplenism is not a specific disease but a syndrome (group or cluster of symptoms) that may be associated with different disorders. Hypersplenism is characterized by enlargement of the spleen (splenomegaly), defects in the blood cells, and an abnormally high turnover of blood cells. It is almost always associated with such specific disorders as cirrhosis of the liver or certain cancers. The decision to perform a splenectomy depends on the severity and prognosis of the disease that is causing the hypersplenism.

Splenectomy always required

There are two diseases for which a splenectomy is the only treatment—primary cancers of the spleen and a blood disorder called hereditary spherocytosis (HS). In HS, the absence of a specific protein in the red blood cell membrane leads to the formation of relatively fragile cells that are easily damaged when they pass through the spleen. The cell destruction does not occur elsewhere in the body and ends when the spleen is removed. HS can appear at any age, even in newborns, although doctors prefer to put off removing the spleen until the child is five to six years old.

Splenectomy usually required

There are some disorders for which a splenectomy is usually recommended. They include:

• Immune (idiopathic) thrombocytopenic purpura (ITP). ITP is a disease in which platelets are destroyed by antibodies in the body’s immune system. A splenectomy is the definitive treatment for this disease and is effective in about 70% of cases of chronic ITP.
• Trauma. The spleen can be ruptured by blunt as well as penetrating injuries to the chest or abdomen. Car accidents are the most common cause of blunt traumatic injury to the spleen.
• Abscesses. Abscesses of the spleen are relatively uncommon but have a high mortality rate.
• Rupture of the splenic artery. This artery sometimes ruptures as a complication of pregnancy.
• Hereditary elliptocytosis. This is a relatively rare disorder. It is similar to HS in that it is characterized by red blood cells with defective membranes that are destroyed by the spleen.

Splenectomy sometimes required

Other disorders may or may not necessitate a splenectomy. These include:

• Hodgkin’s disease, a serious form of cancer that causes the lymph nodes to enlarge. A splenectomy is often performed in order to find out how far the disease has progressed.
• Autoimmune hemolytic disorders. These disorders may appear in patients of any age but are most common in adults over 50. The red blood cells are destroyed by antibodies produced by the patient’s own body (autoantibodies).
• Myelofibrosis. Myelofibrosis is a disorder in which bone marrow is replaced by fibrous tissue. It produces severe and painful splenomegaly. A splenectomy does not cure myelofibrosis but may be performed to relieve pain caused by the swelling of the spleen.
• Thalassemia. Thalassemia is a hereditary form of anemia that is most common in people of Mediterranean origin. A splenectomy is sometimes performed if the patient’s spleen has become painfully enlarged.

Demographics

In the United States, splenomegaly affects as many as 30% of full-term newborns and about 10% of healthy children. Approximately 3% of healthy first-year college students also have spleens that are large enough to be felt when a doctor palpates the abdomen. Some specific causes of splenomegaly are more common in certain racial or ethnic groups. For example, splenomegaly is a common complication of sickle cell disease in patients of African or Mediterranean ancestry. In other parts of the world, splenomegaly is frequently caused by malaria, schistosomiasis, and other infections in areas where these diseases are endemic.

Hereditary spherocytosis (HS) is a disorder is most common in people of northern European descent but has been found in all races. A family history of HS increases the risk of developing this disorder.

Immune thrombocytopenic purpura (ITP) is much more common in children, with male and female children being equally afflicted. Female predominance

KEY TERMS

Computed tomography (CT) scan— An imaging technique that creates a series of pictures of areas inside the body, taken from different angles. The pictures are created by a computer linked to an x-ray machine.

Embolization— A treatment in which foam, silicone, or other substance is injected into a blood vessel in order to close it off.

Endemic— Present in a specific population or geographical area at all times. Some diseases that may affect the spleen are endemic to certain parts of Africa or Asia.

Hereditary spherocytosis— A hereditary disorder that leads to a chronic form of anemia (too few red blood cells) due to an abnormality in the red blood cell membrane.

Idiopathic thrombocytopenia purpura (ITP)— A rare autoimmune disorder characterised by an acute shortage of platelets with resultant bruising and spontaneous bleeding.

Laparoscopy— A procedure in which a laparoscope (a thin, lighted tube) is inserted through an incision in the abdominal wall to evaluate the presence or spread of disease. Tissue samples may be removed for biopsy.

Lymphatic system— The tissues and organs that produce and store cells that fight infection, together with the network of vessels that carry lymph. The organs and tissues in the lymphatic system include the bone marrow, spleen, thymus gland, and lymph nodes.

Palpate— To examine by means of touch.

Platelet— A disk-shaped structure found in blood that binds to fibrinogen at the site of a wound to begin the clotting process.

Sequestration— A process in which the spleen withdraws blood cells from the circulation and stores them.

Spleen— An organ that produces lymphocytes, filters the blood, stores blood cells and destroys those that are aging. It is located on the left side of the abdomen near the stomach.

Splenomegaly— Enlargement of the spleen.

begins at puberty and continues in adult patients. Overall, 70% of patients with ITP are female; 72% of women diagnosed with ITP are over 40 years old.

Description

Complete splenectomy

REMOVAL OF ENLARGED SPLEEN. A splenectomy is performed under general anesthesia. The most common technique is used to remove greatly enlarged spleens. After the surgeon makes a cut (incision) in the abdomen, the artery to the spleen is tied to prevent blood loss and reduce the size of the spleen. Tying the splenic artery also keeps the spleen from further sequestration of blood cells. The surgeon detaches the ligaments holding the spleen in place and removes the organ. In many cases, tissue samples will be sent to a laboratory for analysis.

REMOVAL OF RUPTURED SPLEEN. When the spleen has been ruptured by trauma, the surgeon approaches the organ from its underside and ties the splenic artery before removing the ruptured organ.

Partial splenectomy

In some cases, the surgeon removes only part of the spleen. This procedure is considered by some to be a useful compromise that reduces pain caused by an enlarged spleen while leaving the patient less vulnerable to infection.

Laparoscopic splenectomy

Laparoscopic splenectomy, or removal of the spleen through several small incisions, has been performed more frequently in recent years. Laparoscopic surgery, which is sometimes called keyhole surgery, is done with smaller surgical instruments inserted through very short incisions, with the assistance of a tiny camera and video monitor. Laparoscopic procedures reduce the length of hospital stay, the level of postoperative pain, and the risk of infection. They also leave smaller scars.

As of 2003, however, a laparoscopic procedure is contraindicated if the patient’s spleen is greatly enlarged. Most surgeons will not remove a spleen longer than 20 cm (as measured by a CT scan) by this method.

Diagnosis/Preparation

The most important part of a medical assessment in disorders of the spleen is the measurement of sple-nomegaly. The normal spleen cannot be felt when the doctor palpates the patient’s abdomen. A spleen that is large enough to be felt indicates splenomegaly. In some cases, the doctor will hear a dull sound when he or she thumps (percusses) the patient’s abdomen near the ribs on the left side. Imaging studies that can be used to confirm splenomegaly include ultrasound tests, technetium-99m sulfur colloid imaging, and CT scans . The rate of platelet or red blood cell destruction by the spleen can also be measured by tagging blood cells with radioactive chromium or platelets with radioactive indium.

Preoperative preparation for a splenectomy procedure usually includes:

• Correction of abnormalities of blood clotting and the number of red blood cells.
• Treatment of any infections.
• Control of immune reactions. Patients are usually given protective vaccinations about a month before surgery. The most common vaccines used are Pneumovax or Pnu-Imune 23 (against pneumococcal infections) and Menomune-A/C/Y/W-135 (against meningococcal infections).

Aftercare

Immediately following surgery, patients are given instructions for incision care and medications intended to prevent infection. Blood transfusions may be indicated for some patients to replace defective blood cells. The most important part of aftercare, however, is long-term caution regarding vulnerability to infection. Patients are asked to see their doctor at once if they have a fever or any other sign of infection, and to avoid travel to areas where exposure to malaria or similar diseases is likely. Children with splenectomies may be kept on antibiotic therapy until they are 16 years old. All patients can be given a booster dose of pneumococcal vaccine five to 10 years after undergoing a splenectomy.

Risks

The main risk of a splenectomy procedure is overwhelming bacterial infection, or postsplenectomy sepsis. This condition results from the body’s decreased ability to clear bacteria from the blood, and lowered levels of a protein in blood plasma that helps to fight viruses (immunoglobulin M). The risk of dying from infection after undergoing a splenectomy is highest in children, especially in the first two years after surgery. The risk of postsplenectomy sepsis can be reduced by vaccinations before the operation. Some doctors also recommend a two-year course of penicillin following splenectomy, or long-term treatment with ampicillin.

WHO PERFORMS THE PROCEDURE AND WHERE IS IT PERFORMED?

A splenectomy is performed by a surgeon trained in gastroenterology, the branch of medicine that deals with the diseases of the digestive tract. An anesthesiologist is responsible for administering anesthesia and the operation is performed in a hospital setting.

Other risks associated with the procedure include inflammation of the pancreas and collapse of the lungs. In some cases, a splenectomy does not address the underlying causes of splenomegaly or other conditions. Excessive bleeding after the operation is an additional possible complication, particularly for patients with ITP. Infection of the incision immediately following surgery may also occur.

Normal results

Results depend on the reason for the operation. In blood disorders, the splenectomy will remove the cause of the blood cell destruction. Normal results for patients with an enlarged spleen are relief of pain and the complications of splenomegaly. It is not always possible, however, to predict which patients will respond well or to what degree.

Recovery from the operation itself is fairly rapid. Hospitalization is usually less than a week (one to two days for laparoscopic splenectomy), and complete healing usually occurs within four to six weeks. Patients are encouraged to return to such normal activities as showering, driving, climbing stairs, light lifting and work as soon as they feel comfortable. Some patients may return to work in a few days while others prefer to rest at home a little longer.

Morbidity and mortality rates

The outcome of the procedure varies with the underlying disease or the extent of other injuries. Rates of complete recovery from the surgery itself are excellent, in the absence of other severe injuries or medical problems.

Splenectomy for HS patients is usually delayed in children until the age of five to prevent unnecessary infections; reported outcomes are very good.

Studies of patients with ITP show that 80%-90% of children achieve spontaneous and complete remission

QUESTIONS TO ASK THE DOCTOR

• What happens on the day of surgery?
• What type of anesthesia will be used?
• How long will it take to recover from the surgery?
• When can I expect to return to work and resume normal activities?
• What are the risks associated with a splenectomy?
• How many splenectomies do you perform in a year?
• Will I have a large scar?

in two to eight weeks. A small percentage develop chronic or persistent ITP, but 61% show complete remission by 15 years. No deaths in patients older than 15 have been attributed to ITP.

Alternatives

As of 2003 there are no medical alternatives to removing the spleen.

Splenic embolization is a surgical alternative to splenectomy that is used in some patients who are poor candidates for surgery. Embolization involves plugging or blocking the splenic artery with synthetic substances to shrink the size of the spleen. The substances that are injected during this procedure include polyvinyl alcohol foam, polystyrene, and silicone.

Resources

BOOKS

Hiatt, J. R., Phillips, E. H., and L. Morgenstern, eds. Surgical Diseases of the Spleen. New York: Springer Verlag, 1997.

Wilkins, B. S., and D. H. Wright. Illustrated Pathology of the Spleen. Cambridge, UK: Cambridge University Press, 2000.

PERIODICALS

Al-Salem A. H., and Z. Nasserulla. “Splenectomy for Children with Thalassemia.” Internal Surgery 87 (October-December 2002): 269–273.

Duperier, T., J. Felsherm, and F. Brody. “Laparoscopic Splenectomy for Evans Syndrome.” Surgical Laparoscopy, Endoscopy & Percutaneous Techniques 13 (February 2003): 45–47.

Schwartz, J., M. D. Leber, S. Gillis, et al. “Long-Term Follow-Up After Splenectomy Performed for Immune Thrombocytopenic Purpura (ITP).” American Journal of Hematology 72 (February 2003): 94–98.

Svarch, E., I. Nordet, J. Valdes, et al. “Partial Splenectomy in Children with Sickle Cell Disease.” Haematologica 88 (February 2003): 281–287.

ORGANIZATIONS

American College of Gastroenterology. 4900 B South 31st St., Arlington, VA 22206. (703) 820-7400. www.acg.gi.org

American Gastroenterological Association (AGA). 4930 Del Ray Avenue, Bethesda, MD 20814. (301) 654-2055. www.gastro.org

National Cancer Institute (NCI). NCI Public Inquiries Office, Suite 3036A, 6116 Executive Boulevard, MSC8322 Bethesda, MD 20892-8322. (800) 422-6237. www.cancer.gov

OTHER

The Body Homepage. Spleen Cancer.www.thebody.com/Forums/AIDS/Cancer/Archive/othertypes/Q141422.html.

Your surgery.com. Splenectomy.www.yoursurgery.com/ProcedureDetails.cfm?BR=l&Proc=46.

Teresa Norris, RN

Monique Laberge, Ph. D.