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Pediatric Splenomegaly Clinical Presentation

  • Author: Alexander Gozman, MD; Chief Editor: Robert J Arceci, MD, PhD  more...
Updated: Oct 05, 2015


Despite the extensive differential diagnoses of splenomegaly, careful history taking and physical examination, along with a CBC count and liver function tests, often help in narrowing the list of possible causes. The history should include attention to the following important areas, as listed below.[16]

  • Chief symptoms
    • Acute or chronic nature
    • Painful or asymptomatic manifestation
    • Ingestion of hepatotoxic agents resulting in hepatitis or portal hypertension
    • Abdominal trauma that may cause splenic hematoma
    • Acute illness such as hepatitis, mononucleosis, or malaria
    • Diarrhea (eg, salmonellosis, inflammatory bowel disease)
    • Bone pain, fever, malaise, lethargy, or bruising (eg, associated with leukemia)
    • Weight loss, fevers, night sweats (eg, associated with Hodgkin disease)
    • Jaundice
  • Medical history
    • Complicated neonatal period (eg, sepsis, hypotension)
    • Umbilical catheter thrombosis
    • Hyperbilirubinemia, anemia (eg, due to hereditary spherocytosis or hemolysis)
    • Heart disease (eg, congestive heart failure)
    • Past surgeries (eg, leading to infection, thrombosis, portal hypertension)
    • Transfusions (eg, resulting in hepatitis)
    • Hepatitis
    • Abdominal trauma (possibly resulting in splenic pseudocyst)
    • Travel (possible presence of malaria, leishmaniasis, schistosomiasis, or trypanosomiasis)
    • Sexual behavior (possible presence of hepatitis, cytomegalovirus [CMV], or human immunodeficiency virus [HIV])
    • Known blood disorder (eg, sickle cell disease, hereditary spherocytosis)
  • Family history
    • Anemia, cholecystectomy (eg, due to hemolytic anemia–associated gallstones)
    • Splenectomy (eg, due to hemolytic anemia)
    • Mediterranean ethnicity (increased incidence of thalassemia and glucose-6-phosphate dehydrogenase [G6PD] deficiency)
    • African ethnicity (increased incidence of sickle cell anemia, G6PD deficiency, and hereditary pyropoikilocytosis)
    • Ashkenazi Jewish ethnicity (increased incidence of Gaucher disease[17] and Niemann-Pick disease)
    • Northern European ethnicity (increased incidence of pyruvate kinase deficiency and hereditary spherocytosis)
    • Asian ethnicity (increased incidence of G6PD)
    • South Asian ethnicity (increased incidence of portal hypertension secondary to noncirrhotic portal fibrosis)


The patient should be examined in the supine or right lateral decubitus position. The spleen is best palpated with the clinician kneeling on the patient's right side and by palpating the left upper quadrant of the abdomen with the right hand.

Palpation should start just above the pubis and move toward the left upper quadrant to find the medial border of the spleen. The examiner usually palpates the spleen by feeling its inferolateral margins. If the enlarged tip of the spleen is below the examiner's hands, he or she often misses it. Likewise, light pressure should be used with small children, because the spleen can easily be pushed out of the way without the clinician feeling its edge. At times, the superior medial edge of the spleen is more readily palpated than the inferior margin.

Percussion over the left lateral areas of the lower ribs may reveal splenomegaly that is not evident upon palpation.[18, 19, 20] This procedure is particularly helpful in a crying child in whom splenic palpation is difficult. It is similarly helpful in children and adolescents who are obese. A normal-sized liver and spleen may also become palpable if pulmonary pathology is causing hyperinflation of the lungs (pseudosplenomegaly). The spleen is occasionally confused with the left lobe of the liver or with a tumor in the left upper quadrant (eg, Wilms tumor, neuroblastoma). The characteristic downward movement of the spleen with inspiration and its relatively flat surface and shape can help in differentiating it from other masses of the left upper quadrant. Important features on physical examination are as follows:[16]

  • General findings - Failure to thrive, ill-appearing (eg, in the presence of malignancy, chronic hemolysis, chronic infection, metabolic disease, liver disease, or inflammatory disease)
  • Dermal findings
    • Pallor (eg, due to anemia, which may indicate hemolysis, bone marrow infiltration, or hypersplenism)
    • Petechiae, purpura (eg, due to thrombocytopenia, which may indicate bone marrow failure, autoimmune disorder, or hypersplenism)
    • Jaundice (eg, due to hemolytic anemia or liver disease)
    • Itching, pruritus (eg, due to liver dysfunction, Hodgkin lymphoma)
    • Rashes (eg, due to acute and chronic infections, systemic lupus erythematosus, rheumatoid arthritis, infective endocarditis, histiocytoses, or hemangiomata)
    • Eczematous rash (eg, due to Langerhans cell histiocytosis or immunodeficiency)
  • Head, eyes, ears, nose, and throat findings
    • Icterus (eg, due to hemolytic anemia or liver dysfunction)
    • Cherry red retinal spots, cloudy corneas (eg, due to lipid storage diseases)
  • Respiratory and cardiovascular findings
    • Dyspnea, fatigue (eg, due to anemia or congestive heart failure)
    • New murmur (eg, due to infective endocarditis)
  • GI findings
    • Abdominal tenderness (eg, due to gallstones, hepatitis, trauma, or acute splenomegaly)
    • Distention, prominent abdominal veins, ascites (eg, due to liver disease)
    • Enlarged liver with a firm, knobby texture
  • Musculoskeletal findings
    • Joint pain (eg, due to systemic lupus erythematosus, rheumatoid arthritis, or autoimmune inflammatory diseases)
    • Poor bone growth (eg, due to storage diseases or osteopetrosis)
    • Bone pain (eg, due to leukemia or Gaucher disease)
  • Neurologic findings
    • Poor vision (eg, due to osteopetrosis)
    • Uveitis, iritis (eg, due to sarcoidosis or rheumatoid arthritis)
    • Loss of developmental milestones (eg, due to storage diseases, chronic infection, or immunodeficiency)


Despite the numerous causes of splenomegaly (see Differentials), the spleen is rarely the primary site of disease.

  • The most common mechanism of splenomegaly in children is hyperplasia of the MPS, which can be categorized as excessive antigenic stimulation (ie, infection), disorders of immunoregulation (ie, autoimmune disorders), or excessive destruction of abnormal blood cells (ie, hemolysis). [3, 21]
    • Excessive antigenic stimulation due to infection is the cause of most cases of splenomegaly in children. Viral infections are the most frequent culprits, and the associated splenomegaly is usually transient and only mild to moderate in severity. Although Epstein-Barr virus (EBV) and CMV are well known causes of splenomegaly, the most typical viral illnesses of childhood are the most frequent causes.
    • Other common infectious etiologies include bacterial, protozoal, and fungal infections. In endemic areas, malaria and schistosomiasis are routine causes of splenomegaly. Concomitant generalized lymphadenopathy is common in many of these infectious etiologies.
    • Inflammation due to collagen vascular diseases, such as juvenile rheumatoid arthritis, and increased destruction of blood cells from hemolytic anemias are relatively uncommon, but clinically significant, causes of splenomegaly.
    • Splenomegaly can be a presenting sign of neoplasia. One half of children with acute lymphoblastic leukemia have splenomegaly. Splenomegaly is also a frequent finding in non-Hodgkin lymphoma, Hodgkin disease, and acute or chronic myeloid leukemia. It is also a key presenting feature of a rare form of leukemia, juvenile myelomonocytic leukemia. Metastatic involvement of the spleen, which is uncommon in children, is most often caused by neuroblastoma. Histiocytes can infiltrate the spleen; Langerhans cell histiocytosis and other rare histiocytic disorders are usually the cause of this condition.
    • Obstructed venous blood flow of intrahepatic or extrahepatic etiology can cause splenomegaly. The most common causes include portal vein thrombosis, hepatic cirrhosis, and congestive heart failure. Children with extrahepatic portal venous obstruction, such as cavernous transformation, often present with splenomegaly as the primary manifestation of their disease.
    • Many storage diseases result in splenomegaly. In Gaucher or Niemann-Pick disease, splenomegaly is often the first clinical manifestation. Splenomegaly is the result of the accumulation of abnormal lipids in splenic macrophages.
    • After trauma, palpable subcapsular hematomas may develop in the spleen, which may eventually develop into clinically palpable pseudocysts. Patients with congenital splenic cysts usually present with asymptomatic splenomegaly.
    • Although normally found only during the first 6 months of life, extramedullary hematopoiesis may occur in diseases associated with intense demand on the bone marrow for cell production. Thalassemia major, osteopetrosis, and idiopathic myelofibrosis are examples of this rare cause of splenomegaly.
  • Hypersplenism is a clinical syndrome in which cytopenias result from excessive splenic function, which occurs as the spleen and its MPS tissues hypertrophy. The pathologic action of the spleen, ie, the reduction of circulating blood elements, has been attributed to 4 possible mechanisms: excessive splenic phagocytic activity, splenic production of an antibody that results in the destruction of hematopoietic cells, overactivity of splenic function, and sequestration. [22] In patients with cirrhosis, abnormalities of cytokine production may contribute to the cytopenias noted. [14]
    • As the spleen enlarges, it can sequester erythrocytes, leukocytes, and platelets, resulting in mild-to-moderate decreases in some or all of these cell lines. Severe reductions in cell counts are unusual and should prompt a search for alternative etiologies.
    • Venous obstruction is the most common cause of hypersplenism. Any increase in portal pressure is reflected in the splenic venous sinuses. This impairs blood flow out of the cords and results in the sequestration of blood cells and hypersplenism. Hypersplenism in children is most frequently caused by portal hypertension. Extrahepatic venous obstruction from portal vein thrombosis is the most common cause of increased portal pressures. In extrahepatic venous obstruction, hepatic function is normal. Intrahepatic venous obstruction is usually due to cirrhosis.
    • Portal hypertension usually increases flow through minor collateral vessels between the portal circulation and the systemic circulation. Portal hypertension can result in recognizable dilatation of the superficial abdominal veins and esophageal varices. Patients with these varices may present with sudden and catastrophic GI hemorrhage.
  • Splenic sequestration crisis is a specific form of acute hypersplenism in young children with sickle cell anemia. [22]
    • Children less than 6 years old can develop rapid splenic sequestration and splenomegaly with the consumption of large volumes of erythrocytes. They present with sudden weakness, dyspnea, and left-sided abdominal pain in addition to splenomegaly.
    • Splenic sequestration is an emergency. Rapid death from hypovolemic shock can result.
    • Treatment consists of fluids and erythrocyte transfusions. To prevent recurrences, splenectomy may be indicated. In most patients with sickle cell disease, the spleen eventually involutes, and sequestration is no longer possible.
Contributor Information and Disclosures

Alexander Gozman, MD Assistant Professor, Department of Pediatrics, Division of Hematology/Oncology, Albany Medical Center

Alexander Gozman, MD is a member of the following medical societies: American Medical Association, American Society of Hematology, American Society of Pediatric Hematology/Oncology, Children's Oncology Group, American Society of Clinical Oncology

Disclosure: Nothing to disclose.


Richard H Sills, MD Professor of Pediatrics, Upstate Medical University

Richard H Sills, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Pediatrics, American Society of Hematology, American Society of Pediatric Hematology/Oncology

Disclosure: Nothing to disclose.

Vikramjit S Kanwar, MBBS, MBA, MRCP(UK), FAAP Professor of Pediatrics, Albany Medical College; Chief, Division of Pediatric Hematology-Oncology, John and Anna Landis Endowed Chair for Pediatric Hematology-Oncology, Medical Director, Melodies Center for Childhood Cancer and Blood Disorders, Albany Medical Center

Vikramjit S Kanwar, MBBS, MBA, MRCP(UK), FAAP is a member of the following medical societies: American Academy of Pediatrics, American Society of Hematology, American Society of Pediatric Hematology/Oncology, Children's Oncology Group, International Society of Pediatric Oncology

Disclosure: Nothing to disclose.

Specialty Editor Board

Mary L Windle, PharmD Adjunct Associate Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Nothing to disclose.

James L Harper, MD Associate Professor, Department of Pediatrics, Division of Hematology/Oncology and Bone Marrow Transplantation, Associate Chairman for Education, Department of Pediatrics, University of Nebraska Medical Center; Associate Clinical Professor, Department of Pediatrics, Creighton University School of Medicine; Director, Continuing Medical Education, Children's Memorial Hospital; Pediatric Director, Nebraska Regional Hemophilia Treatment Center

James L Harper, MD is a member of the following medical societies: American Society of Pediatric Hematology/Oncology, American Federation for Clinical Research, Council on Medical Student Education in Pediatrics, Hemophilia and Thrombosis Research Society, American Academy of Pediatrics, American Association for Cancer Research, American Society of Hematology

Disclosure: Nothing to disclose.

Chief Editor

Robert J Arceci, MD, PhD Director, Children’s Center for Cancer and Blood Disorders, Department of Hematology/Oncology, Co-Director of the Ron Matricaria Institute of Molecular Medicine, Phoenix Children’s Hospital; Editor-in-Chief, Pediatric Blood and Cancer; Professor, Department of Child Health, University of Arizona College of Medicine

Robert J Arceci, MD, PhD is a member of the following medical societies: American Association for the Advancement of Science, American Association for Cancer Research, American Pediatric Society, American Society of Hematology, American Society of Pediatric Hematology/Oncology

Disclosure: Nothing to disclose.

Additional Contributors

J Martin Johnston, MD Associate Professor of Pediatrics, Mercer University School of Medicine; Director of Hematology/Oncology, The Children's Hospital at Memorial University Medical Center; Consulting Oncologist/Hematologist, St Damien's Pediatric Hospital

J Martin Johnston, MD is a member of the following medical societies: American Academy of Pediatrics, American Society of Pediatric Hematology/Oncology, International Society of Paediatric Oncology

Disclosure: Nothing to disclose.


The authors and editors of Medscape Reference gratefully acknowledge the contributions of previous authors Wayne Hioe, MD, and Mundeep K Kainth, DO, to the development and writing of this article.

  1. Arkles LB, Gill GD, Molan MP. A palpable spleen is not necessarily enlarged or pathological. Med J Aust. 1986 Jul 7. 145(1):15-7. [Medline].

  2. Brown NF, Marks DJ, Smith PJ, Bloom SL. Splenomegaly. Br J Hosp Med (Lond). 2011 Nov. 72(11):M166-9. [Medline].

  3. Sills RH. Splenic function: physiology and splenic hypofunction. Crit Rev Oncol Hematol. 1987. 7(1):1-36. [Medline].

  4. Mebius RE, Kraal G. Structure and function of the spleen. Nat Rev Immunol. 2005 Aug. 5(8):606-16. [Medline].

  5. McIntyre OR, Ebaugh FG. Palpable spleens in college freshmen. Ann Intern Med. 1967 Feb. 66(2):301-6. [Medline].

  6. Ebaugh FG, McIntyre OR. Palpable spleens: ten-year follow-up. Ann Intern Med. 1979 Jan. 90(1):130-1. [Medline].

  7. Ancliff P, Hann I. Splenomegaly. Sills RH, ed. Practical Algorithms in Pediatric Hematology and Oncology. Basel, Switzerland: Karger; 2003. 50-1.

  8. Genton B, al-Yaman F, Beck HP, et al. The epidemiology of malaria in the Wosera area, East Sepik Province, Papua New Guinea, in preparation for vaccine trials. I. Malariometric indices and immunity. Ann Trop Med Parasitol. 1995 Aug. 89(4):359-76. [Medline].

  9. Pitney WR. The tropical splenomegaly syndrome. Trans R Soc Trop Med Hyg. 1968. 62(5):717-28. [Medline].

  10. Farley DR, Zietlow SP, Bannon MP, Farnell MB. Spontaneous rupture of the spleen due to infectious mononucleosis. Mayo Clin Proc. 1992 Sep. 67(9):846-53. [Medline].

  11. Castagnola E, Fioredda F. Prevention of life-threatening infections due to encapsulated bacteria in children with hyposplenia or asplenia: a brief review of current recommendations for practical purposes. Eur J Haematol. 2003 Nov. 71(5):319-26. [Medline].

  12. Price VE, Dutta S, Blanchette VS, Butchart S, Kirby M, Langer JC, et al. The prevention and treatment of bacterial infections in children with asplenia or hyposplenia: practice considerations at the Hospital for Sick Children, Toronto. Pediatr Blood Cancer. 2006 May 1. 46(5):597-603. [Medline].

  13. Wilson DB. Acquired platelet defects. Nathan DG, Orkin SH, Ginsburg D, Look AT. Nathan and Oski's hematology of infancy and childhood. 6th ed. Philadelphia, PA: WB Saunders; 2003. Vol 2: 1599.

  14. Peck-Radosavljevic M. Hypersplenism. Eur J Gastroenterol Hepatol. 2001 Apr. 13(4):317-23. [Medline].

  15. Sarin SK, Kapoor D. Non-cirrhotic portal fibrosis: current concepts and management. J Gastroenterol Hepatol. 2002 May. 17(5):526-34. [Medline].

  16. Tunnessen WW Jr. Splenomegaly. Roberts K, Tunnessen W, eds. Signs and Symptoms in Pediatrics. 3rd ed. Philadelphia, PA: Lippincott Williams and Wilkins; 1999. 475-83.

  17. Baris HN, Cohen IJ, Mistry PK. Gaucher disease: the metabolic defect, pathophysiology, phenotypes and natural history. Pediatr Endocrinol Rev. 2014 Sep. 12 Suppl 1:72-81. [Medline].

  18. Nixon RK Jr. The detection of splenomegaly by percussion. N Engl J Med. 1954 Jan 28. 250(4):166-7. [Medline].

  19. Castell DO. The spleen percussion sign. A useful diagnostic technique. Ann Intern Med. 1967 Dec. 67(6):1265-7. [Medline].

  20. Grover SA, Barkun AN, Sackett DL. The rational clinical examination. Does this patient have splenomegaly?. JAMA. 1993 Nov 10. 270(18):2218-21. [Medline].

  21. Pochedly C, Sills RH, Schwartz AD, eds. Disorders of the Spleen: Pathophysiology and Management. New York, NY: Marcel Dekker; 1989.

  22. Kinney TR, Ware RE, Schultz WH, Filston HC. Long-term management of splenic sequestration in children with sickle cell disease. J Pediatr. 1990 Aug. 117(2 Pt 1):194-9. [Medline].

  23. Robertson F, Leander P, Ekberg O. Radiology of the spleen. Eur Radiol. 2001. 11(1):80-95. [Medline].

  24. Schlesinger AE, Hildebolt CF, Siegel MJ, Pilgrim TK. Splenic volume in children: simplified estimation at CT. Radiology. 1994 Nov. 193(2):578-80. [Medline].

  25. Ginzel AW, Kransdorf MJ, Peterson JJ, Garner HW, Murphey MD. Mass-like extramedullary hematopoiesis: imaging features. Skeletal Radiol. 2011 Nov 20. [Medline].

  26. AAP. Immunocompromised children. Pickering LK, ed. Red Book: 2003 Report of the Committee on Infectious Diseases. 26th ed. Elk Grove, IL: American Academy of Pediatrics; 2003. 69-81.

  27. Lane PA. The spleen in children. Curr Opin Pediatr. 1995 Feb. 7(1):36-41. [Medline].

  28. Rice HE, Oldham KT, Hillery CA, Skinner MA, O'Hara SM, Ware RE. Clinical and hematologic benefits of partial splenectomy for congenital hemolytic anemias in children. Ann Surg. 2003 Feb. 237(2):281-8. [Medline].

  29. Ahad S, Gonczy C, Advani V, Markwell S, Hassan I. True benefit or selection bias: an analysis of laparoscopic versus open splenectomy from the ACS-NSQIP. Surg Endosc. 2013 Jan 26. [Medline].

  30. Hassan ME, Al Ali K. Massive splenomegaly in children: laparoscopic versus open splenectomy. JSLS. 2014 Jul-Sep. 18 (3):[Medline].

  31. Li S, Li M, Xu W, Sun C, Liu L. Single-Incision Laparoscopic Splenectomy Using the Suture Suspension Technique for Splenomegaly in Children with Hereditary Spherocytosis. J Laparoendosc Adv Surg Tech A. 2015 Sep. 25 (9):770-4. [Medline].

  32. Eichner ER. Sports medicine pearls and pitfalls--defending the spleen: return to play after infectious mononucleosis. Curr Sports Med Rep. 2007 Apr. 6(2):68-9. [Medline].

  33. Rice SG; American Academy of Pediatrics Council on Sports Medicine and Fitness. Medical conditions affecting sports participation. Pediatrics. 2008 Apr. 121(4):841-8. [Medline].

  34. Goddard SL, Chesney AE, Reis MD, et al. Pathological splenic rupture: a rare complication of chronic myelomonocytic leukemia. Am J Hematol. 2007 May. 82(5):405-8. [Medline].

  35. Jandl JH, Aster RH. Increased splenic pooling and the pathogenesis of hypersplenism. Am J Med Sci. 1967 Apr. 253(4):383-98. [Medline].

  36. Goodman J, Newman MI, Chapman WC. Disorders of the spleen. Greer JP, Foerster J, Lukens J, et al, eds. Wintrobe's Clinical Hematology. 11th ed. Philadelphia, Pa: Lippincott Williams and Wilkins; 2004. 1893-909.

  37. Shurin SB. Splenomegaly. Kliegman R, Nieder M, Super D, et al, eds. Practical Strategies in Pediatric Diagnosis and Therapy. Philadelphia, PA: WB Saunders; 1996. 352-9.

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