Approach Considerations
Because splenomegaly is usually the result of a systemic disease, the primary goal is treatment of the underlying process.
Splenectomy
If the patient meets the appropriate criteria, splenectomy may be offered. Careful evaluation and planning are required prior to surgical removal of a child’s spleen.
Hemolytic conditions and space-occupying lesions are the most common diagnoses behind splenectomy in children.
Splenectomy for hemolytic conditions
Some hemolytic conditions with severe manifestations will require splenectomy. Appropriate evaluation of the patient’s history and laboratory findings is key.
In general, patients should be offered splenectomy only if there is severe transfusion-dependent anemia or symptomatic splenomegaly. Consult a hematologist to determine disease-specific guidelines for splenectomy. Stomatocytosis is a definitive contraindication for splenectomy due to the risk of thromboembolic complications. [11]
Sickle hemoglobinopathies may result in splenectomy if the patient meets specific criteria such as repeated or severe sequestration episodes. Conservative management is sometimes effective, however, so the decision to remove the spleen should be based on the individual child’s risk. Some children with hematologic conditions that are being treated with splenectomy should also undergo cholecystectomy at the same time as spleen removal. [12, 13]
Splenectomy for splenic lesions
Because the definition and grading of splenomegaly in children is not standardized, surgical management is not standardized either. Accurate evaluation of the spleen size and configuration is key to planning the surgical procedure. Furthermore, the benefit-to-risk ratio must be carefully determined if splenectomy is considered. Partial spleen removal may be an option. [14, 15, 16, 17] Minimally invasive techniques are preferred in splenectomy in children but require pediatric-specific instruments and protocols. [4, 18]
Splenic lesions such as cysts, abscesses, and hematomas are a common indication for splenectomy. [19]
Postsurgical complications of splenectomy
Splenectomy has been found to result in increased risk for several conditions, including stroke, pulmonary hypertension, arrhythmia, and myocardial infarction. [20, 21]
The most closely correlated adverse effect of pediatric splenectomy is infection, with all persons without normal splenic function being at increased risk for infections and children being at higher risk than adults. Infection risk is greatest during the first few years after spleen removal. [22, 23]
The risk of infection from the gram-negative organisms Capnocytophagacanimorsus and Bordetellaholmesii is particularly increased. Asplenic patients also have greater susceptibility to infection from intra-erythrocytic parasites and encapsulated bacteria.
Overwhelming post-splenectomy infection (OPSI) syndrome is rare but may be fatal. A syndrome of fulminant sepsis occurring in asplenic or hyposplenic individuals, the condition is associated with high mortality and morbidity. Poorly opsonized bacteria such as encapsulated bacteria are the most common infectious organisms causing OPSI. Early signs of OPSI may be mild and nonspecific.
Because patients without functional spleens are immunocompromised, appropriately timed vaccination must be performed to reduce the risk of post-splenectomy infection.
Asplenic patients must also undergo urgent clinical evaluation whenever they have any signs or symptoms that are suggestive of infection. The nature and duration of any antimicrobial prophylaxis should be individually determined for each post-splenectomy patient based on diagnosis, age, exposures, and other factors that modify risk. [24, 22]
Medical Care
Because splenomegaly is usually the result of a systemic disease, the primary goal is treatment of the underlying process. In certain circumstances, splenectomy may be the therapy of choice for symptoms or complications caused by the enlarged organ. However, because of the risk of overwhelming sepsis in children who are asplenic, the risks and benefits must be carefully weighed when splenectomy is being considered. [65, 66]
-
The new PCV7 conjugated pneumococcal vaccine and the older PPV23 polyvalent pneumococcal vaccines, as well as the Haemophilus influenzae vaccine, should be administered to all children who are asplenic and to those about to undergo splenectomy. The polyvalent pneumococcal vaccine is effective only if the patient is aged 2 years or older. Meningococcal vaccine, MCV4, is often administered to these patients in this age group as well. To maximize antibody formation, patients should be vaccinated at least 14 days before elective splenectomy. [36]
-
Daily penicillin is recommended to prevent pneumococcal septicemia in asplenic children younger than 5 years. Antibiotic prophylaxis is often administered for several years after splenectomy in patients older than 5 years, but the role of prophylaxis in these children is less well documented than it is in others.
-
In patients with homozygous sickle cell anemia or sickle beta-zero thalassemia, oral penicillin prophylaxis should be started when the diagnosis is established. This therapy should be administered until at least age 5 years. The role of penicillin prophylaxis in patients with hemoglobin sickle cell disease is controversial. Patients with sickle beta+ thalassemia do not appear to need penicillin prophylaxis.
-
Febrile illnesses in asplenic children should be approached as potentially life-threatening events and evaluated thoroughly, with a low threshold for treatment with intravenous antibiotics that cover Streptococcus pneumoniae and H influenzae. The increasing prevalence of drug-resistant S pneumoniae and the decreasing incidence of H influenzae infections are complicating factors in determining the optimal choice of antibiotics.
Formal vaccine recommendations in anatomic or functional asplenia from the US Centers for Disease Control and Prevention (CDC)
An increased infection risk from encapsulated bacteria, particularly S pneumoniae (pneumococcus), Neisseria meningitidis (meningococcus), and H influenzae type b (Hib), exists in persons with anatomic asplenia (that is, those whose spleen has been surgically removed or is congenitally absent) or functional asplenia (such as develops in cases of sickle cell disease). Thus, an age-appropriate series of PCV13 inoculations should be administered to children. This includes two doses of PCV13 to unvaccinated children aged 2-5 years, and a dose of PCV13 to children aged 6 years or older who have not previously been inoculated with the vaccine. Two doses of PPSV23 should be administered to patients aged 2 years or older, with each dose given 5 years apart and starting at least 8 weeks after all recommended doses of PCV13 have been completed. When both PCV13 and PPSV23 administration are indicated, inoculation should begin with doses of PCV13, with PPSV23 given 8 weeks after the last PCV13 dose. [67]
It is also recommended that persons with anatomic or functional asplenia receive meningococcal conjugate (MenACWY) and serogroup B (MenB) vaccines, while it is routinely advised that all children through age 59 months be inoculated with Hib vaccine. One dose of Hib vaccine should be administered to unimmunized asplenic patients older than 59 months. [67]
Surgical Care
Embolization and microwave ablation
Partial splenic embolization (PSE), the treatment of choice for symptomatic splenomegaly, is safe and effective. However, it is often complicated post procedure by post-embolization syndrome, a painful condition that has somewhat limited PSE’s use in children. [10]
Percutaneous microwave ablation, a well-established therapy for secondary splenomegaly in adults, is emerging as a treatment for symptomatic splenomegaly with thrombocytopenia in pediatric patients with cirrhosis. [10]
Splenectomy
Splenic trauma is the most common indication for splenectomy, although attempts at splenic preservation are important. Nonsurgical management for splenic trauma has success rates of 52-98%, with failure usually occurring in the first 96 hours. Splenic cysts, tumors, and vascular lesions may also require surgical removal. Whenever possible, splenic tissue is preserved to decrease the risk of septicemia, but total splenectomy is occasionally necessary. [68, 69]
Splenectomy can cure hypersplenism but is not usually indicated because the secondary cytopenias rarely cause serious problems. However, in patients with portal hypertension, vascular shunts may be necessary to prevent esophageal variceal bleeding.
Splenectomy may be helpful in improving cytopenias in several medical conditions, including congenital anemias (eg, hereditary spherocytosis, [70] elliptocytosis) and autoimmune disorders (eg, immune thrombocytopenic purpura, autoimmune hemolytic anemia, [24] hypersplenism). In thalassemia major, splenectomy may initially decrease the transfusion requirements caused by hypersplenism. However, the benefit must be carefully weighed against the risk of sepsis.
In Gaucher disease, splenectomy may be necessary when the mechanical strain of the enlarged spleen requires intervention.
Splenectomy may be indicated in children with sickle cell anemia and a history of splenic sequestration crisis in order to prevent recurrences of the crisis.
As part of exploratory laparotomy, splenectomy was once an important component of staging of Hodgkin disease. This procedure is rarely used because of improvements in imaging modalities, the high risk of postsplenectomy sepsis, and the increased use of chemotherapy in patients, which allows treatment decisions to be made on the basis of radiologic evaluation alone. Furthermore, data suggest that splenectomy increases the risk of second malignancy in patients treated for Hodgkin disease.
Li et al described the successful use of suture suspension in the performance of single-incision laparoscopic splenectomy. In the study, which involved nine children with splenomegaly due to hereditary spherocytosis, suture suspension reportedly improved splenic hilum exposure. [71]
For elective surgery, laparoscopic splenectomy is preferable to open splenectomy, except in patients with massive splenomegaly. [72] Even so, a retrospective study by Hassan and Al Ali on 32 children with massive splenomegaly suggested that laparoscopic splenectomy for this condition is safe and effective, with patients experiencing reductions in pain and blood loss, improved recovery, and a shorter hospital stay; operative time, though, was significantly longer than with open splenectomy. [73]
A review of the American College of Surgeons National Surgical Quality Improvement Program Pediatric (NSQIP-P) data set showed that out of 673 patients who underwent elective splenectomy from 2012-2016, 613 (91%) did so laparoscopically with minimal morbidity, suggesting this has become the new standard as surgeons become more familiar with the technique. [74]
A prospective study by Van Der Veken et al indicated that laparoscopic splenectomy in children, for either simple or massive splenomegaly, can be more safely performed if patients undergo perioperative spleen embolization, as carried out by an interventional radiologist in the operating room. [75]
Pelizzo et al advised careful evaluation of spleen size, suggesting that laparoscopic splenectomy is unsafe with a splenic long axis of 12-15 cm, and completely unfeasible in preadolescents with a splenic long axis of more than 15 cm. [4]
Consultations
A pediatric hematologist and/or oncologist may assist in the assessment of the spleen and in guiding the evaluation process when the cause of splenomegaly is not obvious or when a primary hematologic or oncologic disorder is suspected.
Activity
According to the American Academy of Pediatrics and the Council of Sports Medicine and Fitness, children with acutely enlarged spleens should avoid contact, collision, or limited-contact sports. [36] Viral-related splenomegaly rarely lasts longer than 2 months.
The duration for which contact restriction should persist after acute infectious mononucleosis remains uncertain. In the absence of trauma, the incidence of splenic rupture is approximately 1:1000, usually occurring in the first 3 weeks of illness. No evidence-based guidelines are available. More conservative suggestions recommend that children with infectious mononucleosis and resolution of splenomegaly noted on examination have 3 weeks of rest, with a 4-week graded return to activity. Recommendations should be individualized for each patient.
Even with large spleens, most disorders associated with chronic splenomegaly are characterized by reduced splenic friability with minimal risk of rupture. In children with chronically enlarged spleens, decision to restrict activity should be made on a case-by-case basis. [37]
Diet
Rarely, the condition underlying splenomegaly may require dietary modifications. There are no dietary changes that must be recommended due to splenic enlargement itself.
