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Splenomegaly

Splenomegaly: Excerpt from The Diagnostic Approach to Symptoms and Signs in Pediatrics

Normal spleenmay be palpable 1–2 cm below left costal margin in infantsand children.

Pathologically enlarged spleen oftenhas abnormal surface or consistency and is generally associatedwith other findings.

Principal Causes of Splenomegaly

1. Infection
   1. Viral
   2. Bacterial
   3. Fungal
   4. Rickettsial
   5. Parasitic
2. Hemolytic anemia
3. Cardiac failure
4. Trauma
5. Neoplasia
6. Portal hypertension
7. Metabolic disorders
8. Other

Clinical Features and Diagnosis

Infection

In neonates,septicemia is most common cause of enlarged spleen. Usual pathogens aregroup B Streptococcus and E. coli.

Most common cause of enlarged spleenin infants, children, and adolescents is acute viral infection,especially with Epstein-Barr virus or cytomegalovirus.

Other causes are bacterial (septicemia,endocarditis, cat scratch disease, tularemia, brucellosis, tuberculosis,splenic abscess, leptospirosis, Lyme disease, syphilis); fungal(histoplasmosis, candidiasis); rickettsial (Rocky Mountain spottedfever); and parasitic (malaria, toxoplasmosis, visceral larva migrans,schistosomiasis).

Hemolytic Anemia

Any hemolytic anemia may give rise to splenomegaly.See Chap. 45, Pallor (Anemia).

Cardiac Failure

Splenomegaly may occur with cardiac failure.See Chap. 7, Cardiac Failure.

Trauma

Automobile,bicycle, and sled-riding accidents as well as falls are common causesof acute splenic injury. Splenic contusion or hematoma may producesplenic enlargement and tenderness.

Abdominal U/S and CT can locateand define extent of injury.

Neoplasia

Benign splenictumors include hemangioma, lymphangioma, and hamartoma. Spleen isenlarged and asymmetric. Abdominal U/S and CT define locationand extent of mass lesion.

Common malignancies involving spleenare acute lymphoblastic leukemia, acute myeloid leukemia, Hodgkindisease, and non-Hodgkin lymphoma. See Chap. 38, Lymphadenopathy.

Portal Hypertension

Any causeof portal hypertension may cause enlarged spleen. Major causes areliver disease (cirrhosis, hepatitis, extrahepatic biliary atresia);cavernous transformation of portal vessels; and portal or splenicvein thrombosis.

In many cases, abdominal U/Swith Doppler methods can define portal venous anatomy.

Metabolic Disorders

Splenomegalymay occur with a number of metabolic diseases:

Amino acid disorders (tyrosinemia)

Carbohydrate disorders (galactosemia,hereditary fructose intolerance)

Mucopolysaccharidoses (Hurler and Huntersyndromes)

Lipidoses (Gaucher disease, Niemann-Pickdisease, GM-1 gangliosidosis type I)

Glycoprotein disorders (sialidosistype II, fucosidosis)

See Chap.13, Developmental Delay, and Chap. 36, Jaundice, fordiscussion of these disorders.

Other

Splenomegalyalso may occur with

Splenic cysts

Connective tissue diseases (systemiclupus erythematosus, juvenile rheumatoid arthritis, systemic vasculitis)

Inflammatory bowel disease

Sarcoidosis

Histiocytoses

Drug hypersensitivity reactions

See other chapters for discussion ofthese disorders.

Diagnostic Approach

The findingof splenomegaly is usually made on physical exam.

Most common causes of enlarged spleenin pediatric population are viral infection, trauma, hemolytic anemia,cardiac failure, and malignancy.

History and physical exam provide cluesfor diagnosis and any subsequent investigation.

CBC providesinformation about hematologic, infectious, and inflammatory processes.

Finding of pancytopenia may indicatebone marrow dysfunction or portal hypertension with hypersplenism.

Increased sedimentation rate suggestsinfectious, inflammatory, or neoplastic process.

Bacterial, fungal, and other culturesmay be performed with suspected infection.

Bone marrow exam is useful in diagnosisof histiocytoses, lysosomal storage disorders, and some infections(e.g., disseminated histoplasmosis).

Liver function tests and abdominalU/S with Doppler methods should be performed with suspectedportal hypertension.

Abdominal U/S and CT locateand define extent of splenic masses.

References

1. Barness LA. Handbook of pediatric physicaldiagnosis. Philadelphia: Lippincott-Raven, 1998.
2. Rudolph AM, ed. Rudolph's pediatrics, 20thed. Stamford, CT: Appleton & Lange, 1996.
3. Shurin SB. Splenomegaly. In: Kliegman RM, ed. Practicalstrategies in pediatric diagnosis and therapy. Philadelphia: WBSaunders, 1996:352–359.
4. Tung J, Liacouras CA. Splenomegaly. In: AltschulerSM, Liacouras CA, eds. Clinical pediatric gastroenterology. Philadelphia:Churchill Livingstone, 1998: 89–93.
5. Tunnessen WW Jr. Signs and symptoms in pediatrics,3rd ed. Philadelphia: Lippincott Williams & Wilkins, 1999.

Book Source Details

• Book Title: The Diagnostic Approach to Symptoms and Signs in Pediatrics
• Author(s): Paul S. Bellet
• Year of Publication: 2006
• Copyright Details: The Diagnostic Approach to Symptoms and Signs in Pediatrics, Copyright © 2006 Lippincott Williams & Wilkins.

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More About This Book: Title: The Diagnostic Approach to Symptoms and Signs in Pediatrics Authors: Paul S. Bellet Publisher: Lippincott Williams & Wilkins Copyright: 2006 ISBN: 0-78172-899-1

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