Spleen ProblemsView a laproscopic splenectomy procedure.
The spleen was long believed to be an unnecessary organ that could be removed without causing any problems. Important functions of the spleen have now been discovered, however, including the removal of bacteria and waste from the blood, the making of antibodies, and the making of red blood cells.
In addition, infants and children who either never had a spleen or who had theirs removed are known to be at risk for life-threatening infections. This has completely changed the treatment of children with splenic injuries and diseases.
The blood flow inside the spleen is perfectly made so that there is a long time for blood cells and other particles to come in contact with other cells inside the spleen that remove bacteria.
Although the liver has more of these cells than the spleen and is very good in removing bacteria coated with antibodies, removal of bacteria without antibodies occurs mostly in the spleen. This is especially true of certain types of bacteria that have a capsule around them; the capsule helps protect the bacteria; examples are pneumococci, hemophilus, and meningococcus.
Some defense against bacteria is given to the newborn baby by the mother through her placenta. This type of antibody protection disappears within a few months.
The child’s own defenses against infection develop later in childhood. This is called active immunity, and it is the making of antibodies matched to each bacteria.
Until then, protection against bacteria in the blood depends mostly on the spleen cells that remove bacteria. During their travel through the spleen, immature red blood cells are changed to normal mature red blood cells. Old red blood cells are destroyed. Abnormal red blood cells are also removed.
Abnormal grooves on the surface of the spleen are common and usually cause no problems; however, completely separate or extra (accessory) spleens are important. Approximately 16% of children who have their spleen removed have these extra spleens.
Extra “accessory” spleens are very much smaller than the main spleen and are usually dark blue, but they may be difficult to identify because they look like big lymph glands. They usually are found near the tail of the pancreas, along the upper and lower borders of the pancreas, and the lower curve of the stomach.
Within a few months after removal of the spleen, these tiny extra spleens may grow and do the job of the main spleen which was removed. Failure to remove all of these tiny extra spleens may cause a return of certain spleen problems.
Absence of the spleen from birth (asplenia) is usually part of a syndrome associated with other serious birth defects, primarily heart defects. These children have an increased risk of infection.
Polysplenia patients have a normally working spleen with many pieces. The most common other problems in these patients are heart defects. The diagnosis often is made while the patient is having an operation on their belly for some other problem.
REMOVAL OF THE SPLEEN (SPLENECTOMY)
For many years, the most frequent reasons for children to undergo an operation to remove their spleen were injuries (i.e. car accidents, bicycle accidents, falls, etc.) and blood diseases.
During the 1980s and 1990s, it became clear that the injured spleen should not be removed, if possible, so that the child would still have their defense against some bacteria. Removal of the entire spleen for injuries caused by accidents is rarely done now.
Removal of a spleen because it is too big is done much less frequently than in the past. Currently, the most common reasons for removing a child’s spleen are hereditary spherocytosis, idiopathic thrombocytopenic purpura (ITP) that is not being adequately treated with medication, and injuries.
When trapping and later destruction of red blood cells within the spleen make up the major cause of anemia (also known as a low blood count), removal of the spleen often results in a partial or complete solution to the problem. This destruction of red blood cells in the spleen occurs because either the red blood cells are abnormal, or the spleen may be overactive.
In this blood problem, the red blood cells have an abnormal spherical shape like a ball instead of the normal round but almost flat shape. With this shape like a ball, they become trapped and destroyed in the spleen at a faster rate than normal. Although the basic defect of the red blood cell in these patients is unknown, it is thought to be caused by a defect on the outside of the cell. The unusual red blood cell shape makes it at risk for destruction in the slow blood flow in the spleen. Exactly how the trapped red blood cells are destroyed is not known.
These patients have a long-standing low blood hemoglobin count, anemia, that is usually mild but with occasional “crises” of rapidly developing severe drops in the blood hemoglobin count. During these crises, patients usually have infections. These crises are caused by temporary failure of the bone marrow to produce enough red blood cells to keep up with the high demand.
Although these crises are not a reason for spleen removal right away, the operation, if necessary, should be done when the bone marrow is working well and red blood cell destruction is low. Jaundice (yellowing of the skin and eyes) is usually mild or absent, and changes as the blood count changes. Rarely a newborn baby may develop a serious low blood hemoglobin count caused by destruction of red blood cells; the baby may become so yellow that spleen removal may be necessary to prevent brain damage.
The development of stones in the gallbladder and bile ducts (tubes which carry bile from the liver to the intestine) in patients with spherocytosis increases as the child grows. Although not common in infancy and early childhood, the chances may reach 50% in adolescents and 75% in adults.
Ultrasound of the belly should be done in patients with spherocytosis who have not had removal of their spleen before their teen years. Removal of the gallbladder for stones frequently is done at the time of spleen removal. Removal of gallstones without removing the gallbladder is not usually recommended because new stones can develop.
Children with spherocytosis usually have a small-to-medium increase in the size of their spleen. Their skin is usually pale and yellow. Children with long-standing severe symptoms may show some growth failure. A blood smear is a helpful test because it shows spherical red blood cells and a high number of immature red blood cells. Another helpful test is to look at the bone marrow which will show increased red blood cell formation.
Symptoms are the most common reason for non-emergent removal of the spleen in children with this problem. The removal of the spleen is usually delayed until after age 5 years because of the increased risk for serious infection in this age group.
Despite the fact that the red blood cells remain abnormal after removal of the spleen, red blood cell survival is increased, and the anemia almost always is fixed. If symptoms come back, a missed extra spleen is likely.
Idiopathic Thrombocytopenic Purpura (ITP)
Children with ITP repeatedly have low platelet counts in the blood. Platelets are items in the blood that help in clotting. The low platelet count results from the destruction of platelets by the patient’s own defenses.
In this disease, antibodies in the bloodstream find and stick to objects they match with on the platelets; this leads to the destruction of the platelets before they should normally die; this happens mainly in the spleen and liver. Children with this disease usually start getting bruises all of a sudden or less often they have nosebleeds, bleeding of the gums, or rectal bleeding.
Bleeding into the brain is seen in approximately 1% of patients. Nearly 80% of patients have had a flu-like illness within the past 3 weeks. Examination of the patient is usually normal except for bruises or small reddish-to-purple spots (purpura) on the skin.
The blood counts are normal also except for a decreased platelet count. The platelet count may be 5000/mm 3 or lower. More diagnostic blood or bone marrow tests are not required for evaluating children who just started having symptoms (acute) ITP. Patients who have vague symptoms, big lymph glands, or a big liver and/or spleen and children with long-standing symptoms (chronic) ITP should undergo bone marrow tests and a complete look at their immune defenses to make sure they do not have other diseases.
Acute Idiopathic Thrombocytopenic Purpura
In nearly 80% of children with acute ITP, the platelet count returns to normal within 6 months of diagnosis. Only 10% to 20% go on to chronic ITP, which happens when the low platelet count lasts more than 1 year. Treatment of acute ITP includes mostly limitation of physical activities and avoidance of medications that interfere with platelet function (i.e. aspirin, non-steroidal anti-inflammatory agents like ibuprofen.
Medicines to treat acute ITP are more controversial because the condition usually resolves on its own. The most commonly used medications in the treatment of acute ITP includes the use of corticosteroids, intravenous gamma globulin (IVGG), and anti-D immune globulin (WinRho). These treatments have not been shown to decrease the chances that the patient will go on to have chronic ITP, however.
There are many harmful emotional, psychological, physical, and financial problems with these medications. Corticosteroids cause mood swings, weight loss, and an altered body shape including obesity and increased facial hair growth; whereas IVGG is very expensive.
Therefore, drug treatment is reserved for patients at higher risk for bleeding, including patients older than 10 years of age (because of a higher risk of bleeding into the brain), patients with massive skin, gum or nasal bleeding, and patients with evidence of internal bleeding.
Many studies have shown that corticosteroids work when given to treat patients with acute ITP. After the patients take these meds, they have a rise in their platelet count after a few days or weeks. The typical prescription is prednisone taken by mouth, 2 mg/kg/day in divided doses for 1 to 3 weeks. Steroids should be stopped after this period of time because the side effects are worse than the chance of any more benefit from taking them.
IVGG, usually given for 5 straight days, leads to a quick rise in the platelet count in children with acute or chronic ITP. This rise is usually faster and reaches a higher peak than that seen with steroids. The rise in platelet count usually starts in 1 week and lasts several weeks.
Despite the fact that it works well, the very high cost of IVGG makes it better for the treatment of patients in whom steroids fail, patients with active bleeding, or for bleeding prevention in patients undergoing other necessary surgical procedures.
Emergency splenectomy rarely is required in acute ITP. It is saved for patients with bleeding into the brain or massive gum, nasal or internal bleeding. These patients probably should receive IVGG, steroids and platelet transfusions before and during surgery.
Chronic Idiopathic Thrombocytopenic Purpura
Children with chronic ITP have a low platelet count in the blood lasting more than 1 year. The symptoms start off more slowly than in the acute form. These patients should undergo testing for other diseases like lupus. Most children with chronic ITP do not require special treatment, but contact sports should be avoided. Treatment of chronic ITP depends on: the frequency and severity of bleeding, the age of the child (especially children >10 years old or children with heavy menses), the activity level of the child, the severity of the decrease in the platelet count, the need for non-emergent surgery, and the presence of neurologic symptoms, such as headache, sleepiness, and weakness. Corticosteroids and IVGG are the main treatment for chronic ITP. The harmful side effects of corticosteroid therapy make them a poor choice for long-term use, however. Long-term use of IVGG can delay (sometimes forever) the need for spleen removal. Newer treatments, such as WinRho, can lead to a rise in platelet count. Other drugs include vincristine, vinblastine, and colchicines. Immunosuppressive drugs, such as azathioprine, cyclophosphamide, and cyclosporine, have been given to adults and the results are good sometimes. Danazol, an artificial male hormone, increased platelet count. The drug usually works within 2 to 6 months. Interferon alfa also has been used with some success in adults. Filtering of the blood, plasmapheresis, has been offered in adults as additional treatment instead of high-dose IVGG alone; filtering has been used in children also.
Patients with chronic ITP usually have mild disease. Spleen removal for chronic ITP is saved for patients with symptoms not relieved with corticosteroid and IVGG therapy. Approximately 18% of patients with chronic ITP followed 3 to 37 years required spleen removal for uncontrolled symptoms. Long-term success is achieved in 80% of patients undergoing spleen removal. Although they occasionally produce a long-lasting solution in adults with acute or chronic ITP, the immunosuppressive drugs probably should be saved for patients who do not do well after having their spleen removed; this is because few children have taken these drugs for a long time, and doctors are not entirely sure of the long-term side effects.
Hereditary Elliptocytosis (Ovalocytosis)
Ovalocytosis is hereditary (runs in families) and, similar to spherocytosis. It is mostly a defect in red blood cell structure. How many red blood cells have the defect is usually the same in all children with the disease; however, the disease is more severe in some children than others. Destruction of red blood cells does not usually happen fast enough to cause anemia (low blood count); spleen removal is rarely indicated. Like spherocytosis, the abnormal red blood cells remain and may increase in the bloodsteam after spleen removal because only the speed of abnormal cell destruction is changed by removing the spleen.
Thalassemia (Mediterranean Anemia), Sickle Cell Disease, and Hereditary Nonspherocytic Hemolytic Anemia
In thalassemia (Mediterranean anemia), sickle cell disease, and hereditary nonspherocytic hemolytic anemia, there is an abnormality in the red blood cells, which causes a low blood hemoglobin count (anemia). Although most children who have these diseases do not do better by having their spleen removed, in some patients, a big spleen may trap many red blood cells. When trapping inside the spleen causes transfused red blood cells to die sooner than they should, spleen removal may be of benefit.
One form of hereditary nonspherocytic anemia has less of an enzyme, glucose-6-phosphate dehydrogenase, in red blood cells. Jaundice in newborn babies plus episodes of blood in the urine are seen in most of these patients. Although removal of the spleen fails to cure these patients, it may reduce the need for blood transfusion and minimize the risk of having blood in the urine.
Hypersplenism is caused by a group of blood problems. Patients with these problems have a low blood hemoglobin count (anemia), decreased white blood cells (leukopenia), or decreased platelets in the blood (thrombocytopenia) caused by removal of too many of them by a big spleen. There also are an increased number of immature cells in the bone marrow. Hypersplenism may be from a blood disease (primary hypersplenism) or it may be caused by some other disease (secondary hypersplenism) that results in a big spleen.
Most pediatric cases of primary hypersplenism have blood diseases, such as spherocytosis and ITP. Before removal of the spleen is performed for hypersplenism, the blood disease must be found. How well the spleen removes damaged blood cells should be figured out if possible. Sometimes, the spleen may produce red blood cells, and its removal may decrease the total body production of red blood cells. Nuclear medicine studies with radioactive red blood cells can show this.
In secondary hypersplenism, some other type of disease causes the spleen to become big, and the big spleen becomes overactive by trapping or destroying one or more normal types of blood cells. This form of hypersplenism is observed most commonly in patients who have portal hypertension. Spleen removal is rarely necessary. Tying off the blood supply to the spleen has been shown to reduce the symptoms without increasing the risk of severe infection that often follows spleen removal. More recently, clotting off the blood supply to the spleen (embolization) has produced similar results in a few children, although symptoms may come back as new blood vessels grow.
Secondary hypersplenism also occurs in patients with Hodgkin’s disease, Gaucher’s disease, sarcoidosis, reticuloendotheliosis, certain granulomas, and leukemia. Some children with very big spleens, especially children with Gaucher’s disease, may benefit from near-total (95%) spleen removal. Although partial spleen removal works in the short-term, enzyme treatment for the Gaucher’s disease has become the tried-and-true treatment in these patients.
Reduced spleen function may result from surgical removal of the spleen, its absence from birth, diseases that decrease the blood flow inside the spleen, such as sickle cell anemia, or diseases that involve the tissue of the spleen, such as Gaucher’s disease. After spleen removal, there may be a sharp increase in both the platelet and white blood cell count. Several diseases can produce decreased spleen function, including sickle cell anemia, sarcoidosis, chronic ulcerative colitis (rare), celiac disease (rare), and radiation therapy. Children with these diseases are at increased risk for severe life-threatening infections just like patients who have had their spleen removed.
Technique of Splenectomy (Spleen Removal)
Laparoscopic spleen removal uses small incisions and telescopic equipment; this approach has replaced the open (older style of surgery using bigger incisions) operation as the approach of choice for patients requiring non-emergent removal of the spleen except when the spleen is very large or stuck to other structures inside the abdomen. The patient is prepared for the laparoscopic operation in the same way as for the open procedure. Vaccinations are given at least 3 weeks before the operation, and the platelet count is increased to more than 20,000/mm 3, if possible, by giving either IVGG or WinRho or whatever drug works best for the patient. The patient is placed on the operating table, usually lying slightly on their right side with a roll under the left flank. The patient is secured to the operating room table because the operating room table may need to be rotated. A tube is placed through the patient’s mouth into the stomach.
Figure 2: Different ways surgeons do a laparoscopic splenectomy. On the left, three small incisions (arrows) are made in the midline. The two incisions closest to the patient’s head are small incisions through which 2.5-mm instruments are placed directly through the skin. The lowest midline incision is 5 mm and is the site through which the telescope is placed. The largest incision is 15 mm and is placed in the belly-button. It is through this incision that the spleen is brought out. On the right, two upper belly incisions are created (arrows), each measuring 5 mm, and instruments are placed through these incisions. A 10-mm incision is made in the belly-button, and the largest incision, which is 15 mm, is placed under the left rib cage. The spleen is removed through this site.
Placement of the incisions depend on the surgeon’s choice. Many surgeons choose to place 3- and 5-mm incisions in the midline of the upper belly with a 15-mm incision placed through the belly-button (Figure 1). Through this 15-mm incision, the stapler is used to staple and divide the blood supply to and drainage from the spleen. Then a bag is placed through this incision. With the bag inside of the body, the spleen is placed inside the bag and the end of the bag is removed through the belly-button incision. The spleen is cut into small pieces inside of the bag; This lets what was once a large spleen to be removed through a small incision. The surgeon takes care not to spill any of the spleen tissue inside of the body. Other surgeons may place two small incisions in the midline and one in the belly-button, but the 15-mm incision is placed in either the left mid area of the belly or the left lower area of the belly (see Figure 2). Here the stapler can be put inside the body at a 90-degree angle to the blood supply to the spleen and placed more easily across these vessels.
Regardless of the incisions, the necessary parts of the operation are as follows: A search through the telescope is performed to identify and remove any extra spleens, especially in the patient with ITP (Figure 3). Some attachments to the spleen are divided first, followed by division of the blood supply from the stomach to the spleen. The tail of the pancreas is separated from the spleen. Other attachments are then divided. When the spleen has been freed completely and is left attached only by its main blood supply, the stapler is placed across these vessels, the stapler is fired, and the vessels are divided with three rows of staples on each side (Figure 4). The main blood supply to the spleen also can be clipped and cut.
Figure 3: An extra tiny spleen (arrow) is seen near the lower part of the spleen in a child having a laparoscopic splenectomy for ITP which could not be controlled with medication.
Some surgeons prefer initial clipping of the main artery to the spleen with the advantage of reducing the size of the spleen by allowing blood to flow from the spleen back towards the heart. If this technique is desired, the main artery is clipped after the blood supply from the stomach to the spleen has been divided. One or two clips can be placed across the main artery to the spleen along the upper border of the pancreas.
When the spleen has been freed completely inside the body, a 15-mm bag is placed through the 15-mm site, and the spleen is placed into the bag. The neck of the bag is brought out through the 15-mm site. The spleen is cut into small pieces within the bag and removed. Depending on the size of the spleen, chopping up the spleen into tiny pieces and removing it can take 10 to 30 minutes. The bag is then completely removed, and a repeat look through the telescope is performed to make sure that there is no bleeding and that there is no evidence of injury to nearby organs, like the stomach and pancreas. The equipment is removed, the incisions are closed, and the patient is allowed to wake up. Most patients are ready for discharge either the first or second day after the operation.
For children in whom severe rupture of the spleen is suspected or in whom the spleen is massively enlarged, open spleen removal through a long incision underneath the left rib cage or in the midline of the belly may be needed. When the spleen is actively bleeding after injury, the main blood supply to the spleen is tied first to minimize blood loss. During this, caution is needed to avoid injury to the tail of the pancreas. A thorough search should be made for extra spleens, which should be removed in all patients who undergo spleen removal for blood diseases.
When partial removal of the spleen is performed non-emergently, the lower 35% to 50% of the spleen is removed after the blood supply to and drainage from this area are tied off and divided. The cut edge of the spleen is sewn together. Some fatty tissue inside the belly is sewn loosely to the cut surface of the spleen. In patients whose spleens are injured, the part of the spleen that is bleeding is removed.
SEVERE LIFE-THREATENING INFECTIONS FOLLOWING REMOVAL OF THE SPLEEN AND OTHER COMPLICATIONS
Major complications after surgery are usually due to the patient’s disease that caused them to need to have their spleen removed. Bleeding and infection are rare complications after spleen removal in children. Drains do not need to be placed at the time of surgery, unless there has been damage to the tail of the pancreas.
Many doctors have shown that spleen removal increases the risk of severe infections. The usual infections after spleen removal happen quickly, become severe, and are fatal in almost 50% of cases. Most cases occur within 2 years after spleen removal, but this complication can be seen many years later. The risk is greatest in children younger than 5 years of age. The risk is low but remains throughout the teen years and adult life. The risk of severe life-threatening infection is lower for children who undergo spleen removal for spherocytosis, ITP, or a bleeding injured spleen. In disorders such as histiocytosis, Wiskott-Aldrich syndrome, and leukemia, the risk of life-threatening infection is much higher. The risk is highest in thalassemia patients. In the past, nearly 8% of children undergoing spleen removal as part of an exploratory operation for Hodgkin’s disease were found to develop later severe life-threatening infections after surgery. The chance of death is greater than 40% in those patients with Hodgkin’s disease who get this life threatening infection. The risk of infection is as high in teenagers as in younger children who have undergone spleen removal for Hodgkin’s disease. In a thorough review, Dr. Singer reported an overall chance of severe infection of 4.25% and a chance of death of 2.52% in a collection of 2796 patients. The chance of a deadly infection in children without a spleen was 200 times that in normal children.
Pneumococcus is the bug responsible for approximately 50% of the severe life-threatening infections. Meningococcus, Escherichia coli, Haemophilus influenzae, Staphylococcus, and Streptococcus have been found less often. Preventive measures to reduce the chances of a severe infection have included active immunization against pneumococcus, Meningococcus, and H. influenzae; preventive penicillin therapy; and prompt, aggressive treatment for infections. The polyvalent pneumococcal vaccine (Pneumovax) is estimated to provide protection against approximately 85% of all pneumococcal infections. The vaccine should be given before spleen removal whenever possible or shortly thereafter. The same applies for the other vaccines. Children receiving corticosteroids for treatment of their disease may require a second dose of Pneumovax after surgery after the steroids have been stopped. It is estimated that antibody levels stay at satisfactory levels for at least 3.5 years. Because there is an increased risk of reaction to second vaccine injections, booster doses are rarely recommended.
Although some doctors disagree, it generally is recommended that all children receive preventive penicillin therapy after spleen removal until they become adults. Many doctors recommend penicillin only after the child develops the first symptoms of an infection of any type. Patients with advanced Hodgkin’s disease and other serious illnesses who have undergone spleen removal should receive long-term penicillin, whatever age they may be. Doctors are not sure about the effectiveness of preventive penicillin therapy after surgery, because of the difficulty in making sure the patients took their medication. Education of the parents (and the child after they grow up) about the risk of infection after spleen removal and the need for early and aggressive treatment is very important. Parents are advised to bring the child to the hospital on an emergency basis whenever a fever of up to 102∞F develops. The child is evaluated carefully; given a strong intravenous antibiotic, such as ceftriaxone; and observed for several hours. If the child shows evidence of aggressive infection, he is admitted to the hospital right away for treatment.
An increase in the platelet count above normal after spleen removal is not a great worry. All blood cell counts increase for a short time after spleen removal. The platelet count may increase to 1 million/mm 3 or more, and the white blood cell count may reach 30,000/mm 3. The highest counts usually are found approximately 1.5 weeks after spleen removal. Clotting complications are extremely rare in children. It is rarely necessary to give a blood thinner after spleen removal because of a high platelet count.
Al-Salem AH, Naserullah Z, Qaisaruddin A, et al: Splenic complications of the sickling syndromes and the role of splenectomy. J Pediatr Hematol Oncol 21:401-406, 1999.
The authors discuss their management of 113 patients with sickling disorders who underwent splenectomy over a 10-year period. The authors believe that splenectomy was beneficial in these patients in reducing the need for transfusion and its associated risks, in eliminating the discomfort from an enlarged spleen, and in avoiding the risk of acute splenic sequestration crisis.
Hemmila MR, Foley DS, Castle VP, et al: The response to splenectomy in pediatric patients with idiopathic thrombocytopenic purpura who fail high-dose intravenous immune globulin. J Pediatr Surg 35:967-971, 2000.
The authors discuss their experience with patients who were treated with intravenous immune globulin (IgG) before splenectomy. In their experience, a good or excellent response to initial treatment with IgG was associated with a significant probability of a good or excellent response to splenectomy.
Jugenberg M, Haddock G, Freedman MH, et al: The morbidity of pediatric splenectomy: Does prophylaxis make a difference? J Pediatr Surg 34:1064-1067, 1999.
The authors analyzed the incidence of postsplenectomy sepsis, morbidity, and mortality after prophylaxis in children undergoing splenectomy and compared these results with a historical period in which children did not receive prophylaxis. In their experience, the incidence of infection and mortality decreased by 47% and 88% with prophylaxis.
Reddy VS, Phan HH, O’Neill JA, et al: Laparoscopic versus open splenectomy in the pediatric population: A contemporary single-center experience, Am Surg 67:859-863, 2001.
Results are compared in patients undergoing open and laparoscopic splenectomy between 1994 and 1999. In the study, laparoscopic splenectomy seemed to result in longer operative times, but shorter lengths of stay, earlier first oral intake, and significantly fewer requirements for intravenous narcotics.
Rescorla FJ: Laparoscopic splenectomy. Semin Pediatr Surg 11:226-232, 2002.
In this review article, the author describes his experience and operative technique for laparoscopic splenectomy in 127 children. The literature regarding this operation also is reviewed.
Tarantino MD: Treatment options for chronic immune (idiopathic) thrombocytopenic purpura in children, Semin Hematol 37(Suppl 1):35-41, 2000.
This review article discusses salient issues in the treatment of ITP in children.
Article and graphics adapted from O'Neill: Principles of Pediatric Surgery. © 2003, Elsevier.