Basics
Description
- A palpable spleen is found in most premature infants and in 30% of term infants. A spleen tip is still palpable in 10% of infants at 1 year of age and in 1% of children at 10 years of age.
- Normal spleens are not greater than 6 cm at 3 months, 7 cm at 12 months, 9.5 cm at 6 years, 11.5 cm at 12 years, and not greater than 13 cm for adolescents.
- Splenomegaly can also be a spleen width >4 cm or diameter >7 cm.
- Normal spleen size varies but is typically less than 250 g.
- The clinical significance of splenomegaly found on radiologic study, but not palpable on physical exam, is unclear in the absence of other laboratory or clinical data.
- Normal spleens are soft at the midclavicular line, nontender, and often palpable only on deep inspiration.
- Dullness on percussion beyond the 11th intercostal space suggests splenomegaly.
- A spleen edge palpated >2 cm below the costal margin is always an abnormal finding.
- Splenic tenderness is always abnormal.
Pathophysiology
- The spleen is a hematopoietic organ with 2 main parts:
- White pulp is the lymphoid tissue.
- Red pulp is the red cell mass.
- Splenic sinusoids are lined with macrophages that destroy abnormal red cells.
- The spleen also serves as a reservoir for platelets. A normal-sized spleen can hold 1/3 of the circulating platelets; an enlarged spleen can hold up to 90% of the circulating platelet mass.
- Normal splenic volume by CT scan is 214.6 cm3 (range, 107.2 cm3 " 314.5 cm3).
- Splenic size does correlate with height.
Diagnosis
History
- Question: History of acute illness?
- Significance: Suggests infection
- Question: History of GI bleeding with splenomegaly?
- Significance: Suggests portal hypertension
- Question: Familial history of hematologic or immune disease?
- Significance: Suggests genetic etiology
- Question: An enlarged liver, developmental delay, or neurologic findings?
- Significance: May suggest a storage disease or metabolic disorder
Physical Exam
Begin the abdominal examination in the lower left quadrant because an enlarged spleen may be missed in the upper quadrant exam. Stand to the right of the patient; use the right hand to palpate and the left hand to support the patient 's left lower rib cage. Flexing the legs at the knees may help to relax the abdominal musculature.
- Finding: Auscultate for rub or bruit?
- Significance: Vascular malformation
- Finding: Look for signs of storage disease?
- Significance: Retinal exam, coarse facies
- Finding: Complete evaluation of lymph nodes?
- Significance: Enlargement suggests infection or neoplasia.
- Finding: Palpate for ascites or hepatomegaly?
- Significance: Suggests underlying hepatic disease
- Finding: Prominent abdominal veins or hemorrhoids?
- Significance: Suggest increased portal venous pressure
- Finding: Pain/tenderness?
- Significance: Suggests capsular distention secondary to perisplenitis or trauma; also raises the question of splenic infarct
- Finding: Asthmatic patients may have palpable spleen?
- Significance: Secondary to overinflation of lungs with a depressed diaphragm
Diagnostic Tests & Interpretation
Lab
- Test: Blood culture, thick smear of blood for malaria, viral testing
- Significance: evaluate for infection
- Test: CBC with manual differential and smear
- Significance: For sickle cell disease, hemolytic anemia, leukemia
- Test: Decreased WBC count and platelets
- Significance: Often seen with splenic sequestration or portal hypertension
- Test: Reticulocyte count
- Significance: For hemolytic anemia
- Test: Hepatic function panel (liver enzymes, albumin, bilirubin) and prothrombin time (PT)/international normalized ratio (INR), partial thromboplastin time (PTT)
- Significance: For cirrhosis, hepatic obstruction
- Test: Serum lactate dehydrogenase (LDH)
- Significance: For hemolysis or tumor screen
Imaging
- If no hemolytic disease, no sign of infection, no sign of congestion:
- Ultrasound with Doppler
- Liver spleen scan
- If no hemolytic disease, no sign of infection but signs of congestion:
- Ultrasound with Doppler
- MRI; consider MRA/MRV
Diagnostic Procedures/Other
Biopsy of lymph node, liver, or other tissue, depending on findings
Differential Diagnosis
- Infectious
- Bacterial
- Bacteremia
- Pneumonia
- Sepsis
- Subacute bacterial endocarditis
- Salmonellosis
- Tuberculosis
- Brucellosis
- Staphylococcal shunt infections
- Tularemia
- Syphilis
- Leptospirosis
- Viral
- Epstein-Barr virus (mononucleosis)
- Cytomegalovirus
- HIV
- Rubella
- Herpes
- Hepatitis A, B, C
- Rickettsial/protozoan
- Rocky Mountain spotted fever
- Malaria
- Toxoplasmosis
- Trypanosomiasis
- Babesiosis
- Schistosomiasis
- Visceral larval migrans
- Kala azar
- Fungal
- Histoplasmosis
- Coccidioidomycosis
- Hematologic disorders
- Hereditary spherocytosis
- Sickle cell disease in early childhood or during splenic sequestration crisis
- Hemoglobin C disease
- Thalassemia major
- Autoimmune hemolytic anemia
- Glucose-6-phosphate dehydrogenase deficiency
- Isoimmunization disorders
- Infantile pyknocytosis
- Iron deficiency anemia (rare)
- Thrombocytopenic purpura
- Vascular disorders
- Cavernous transformation of the portal vein
- Budd-Chiari syndrome
- Splenic vein thrombosis
- Congenital portal vein stenosis or atresia
- Splenic hematoma
- Splenic hemangioma
- Liver disease/cirrhosis (examples include, but are not limited to)
- Biliary atresia
- Wilson disease
- Cystic fibrosis
- α-1-Antitrypsin deficiency
- Hereditary hemochromatosis
- Congenital hepatic fibrosis
- Autoimmune hepatitis
- Primary sclerosing cholangitis
- Metabolic diseases (storage)
- Gangliosidoses
- Mucolipidoses
- Metachromatic leukodystrophy
- Wolman disease
- Gaucher disease
- Niemann-Pick disease
- Amyloidosis
- Neoplastic diseases
- Leukemia
- Lymphoma
- Lymphosarcoma
- Neuroblastoma
- Histiocytosis X
- Familial hemophagocytic lymphohistiocytosis
- Miscellaneous
- Serum sickness
- Connective tissue disorders
- Juvenile rheumatoid arthritis
- Systemic lupus erythematosus
- Sarcoidosis
- Splenic hamartoma
- Splenic cysts: congenital and posttraumatic
- Trauma: subcapsular hematoma
- Nonsplenic upper left quadrant abdominal masses
- Large kidney
- Retroperitoneal tumor
- Adrenal neoplasm
- Ovarian cyst
- Pancreatic cyst
- Mesenteric cyst
- Rib anomaly
Alert
- Life-threatening causes: sepsis, severe hemolytic anemia, trauma, splenic sequestration
- A large-bore IV access route should be rapidly placed when a life-threatening cause is suspected.
Treatment
General Measures
- Treatment depends on underlying etiology.
- Spleen guards can be used to protect from traumatic splenic rupture.
Issues for Referral
- Increasing size over serial examinations (hepatology, hematology/oncology)
- Unexplained lymphadenopathy (oncology)
- Liver dysfunction and or ascites (hepatology)
- Signs of storage or metabolic disease (metabolism, GI)
- Howell-Jolly bodies on peripheral smear, suggesting splenic dysfunction (hematology)
Surgery/Other Procedures
Splenectomy indicated in certain situations including symptomatic hematologic disorders, abscess, and neoplasms.
Ongoing Care
General goal is to determine the etiology of the large spleen.
- Establish the presence of enlarged spleen, not a palpable spleen that is pushed down by inflated lungs.
- Rule out common causes such as a viral infection, bacterial infection, or anemia.
- Rule out malignancy or storage disease or other rare causes of large spleen.
- Ensure proper immunizations or antibiotic prophylaxis.
- Ongoing care depends on the underlying etiology of the splenomegaly.
Additional Reading
- Benter T, Kl Όhs L, Teichgr €ber U. Sonography of the spleen. J Ultrasound Med. 2011;30(9):1281 " 1293. [View Abstract]
- Donnelly LF, Foss JN, Frush DP, et al. Heterogeneous splenic enhancement patterns on spiral CT images in children: minimizing misinterpretation. Radiology. 1999;210(2):493 " 497. [View Abstract]
- McCormick PA, Murphy KM. Splenomegaly, hypersplenism and coagulation abnormalities in liver disease. Best Pract Res Clin Gastroenterol. 2000;14(6):1009 " 1031. [View Abstract]
- Pozo AL, Godfrey EM, Bowles KM. Splenomegaly: investigation, diagnosis and management. Blood Rev. 2009;23(3):105 " 111. [View Abstract]
- Prassopoulos P, Daskalogiannaki M, Raissaki M, et al. Determination of normal splenic volume on computed tomography in relation to age, gender and body habitus. Eur Radiol. 1997;7(2):246 " 248. [View Abstract]
- Rosenberg HK, Markowitz RI, Kolberg H, et al. Normal splenic size in infants and children: sonographic measurements. AJR Am J Roentgenol. 1991;157(1):119 " 121. [View Abstract]
Codes
ICD09
- 789.2 Splenomegaly
- 865.01 Injury to spleen without mention of open wound into cavity, hematoma without rupture of capsule
- 228.04 Hemangioma of intra-abdominal structures
- 759.0 Anomalies of spleen
ICD10
- R16.1 Splenomegaly, not elsewhere classified
- D73.5 Infarction of spleen
- D18.03 Hemangioma of intra-abdominal structures
- Q89.09 Congenital malformations of spleen
SNOMED
- 16294009 Splenomegaly (disorder)
- 262818004 Splenic hematoma (disorder)
- 93472004 Hemangioma of spleen (disorder)
- 36752001 Congenital splenomegaly (disorder)
FAQ
- Q: How long will the enlarged spleen secondary to a viral infection be present?
- A: The enlarged spleen may persist for several months.
- Q: Should patients with decreased splenic function due to splenomegaly be immunized?
- A: Immunization with a pneumococcal conjugate and/or polysaccharide vaccine should be carried out in all patients with compromised splenic function. In those patients who will be undergoing a scheduled splenectomy, the Streptococcus pneumoniae, meningococcus, and Haemophilus influenzae type B vaccines should be given at least 14 days prior to the operation.
- Q: Should a child with an enlarged spleen refrain from sports?
- A: Contact sports should be avoided for a child with an enlarged spleen. An enlarged spleen is engorged with blood, and a splenic rupture would be a catastrophic event. Children with persistent splenomegaly should be considered for a spleen guard.