Splenomegaly
Matthew J. Ryan, MD
Splenomegaly - BASICS
Splenomegaly - 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 10 years of age.
- Normal spleens are no >6 cm at 3 months, 7 cm at 12 months, 9.5 cm at 6 years, 11.5 cm at 12 years, and no >13 cm for adolescents.
- The clinical significance of splenomegaly found on radiologic study, but not palpable on physical exam, requires other laboratory or clinical data to establish its importance.
- Normal spleens are soft at the midclavicular line 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 abnormal.
Splenomegaly - pathophysiology
- The spleen is an 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.
Splenomegaly - DIAGNOSIS
General goal is to determine the etiology of the large spleen:
- Phase 1: Establish the presence of enlarged spleen, not a palpable spleen that is pushed down by inflated lungs.
- Phase 2: Rule out common causes such as a viral infection, bacterial infection, or anemia.
- Phase 3: Rule out malignancy or storage disease or other rare causes of large spleen.
Splenomegaly - signs & symptoms
Splenomegaly - history
- History of acute illness: Suggests infection
- History of GI bleeding with splenomegaly: Suggests portal hypertension
- Familial history of hematologic or immune disease: Suggests genetic etiology
- An enlarged liver, developmental delay, or neurologic findings: May suggest a storage disease or metabolic disorder
Splenomegaly - 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.
- Auscultate for rub or bruit.
- Look for signs of storage disease (retinal exam, coarse facies).
- Complete evaluation of lymph nodes: Enlargement suggests infection or neoplasia.
- Look for ascites or hepatomegaly: Suggests underlying hepatic disease
- Prominent abdominal veins or hemorrhoids: Suggest increased portal venous pressure
- Pain/tenderness: Suggests capsular distention secondary to perisplenitis or trauma; also raises the question of splenic infarct
- Asthmatic patients may have palpable spleen secondary to overinflation of lungs and depressed diaphragm.
Splenomegaly - tests
- CBC with manual differential and smear for sickle cell disease, hemolytic anemia, leukemia
- Decreased WBC count and platelets often seen with splenic sequestration or portal hypertension
- Reticulocyte count for hemolytic anemia
- Hepatic function panel (liver enzymes, albumin, bilirubin) and PT/PTT for cirrhosis, hepatic obstruction
Splenomegaly - lab
Discriminating laboratory tests: If no hemolytic disease, with signs of infection:
- Blood culture
- Thick smear of blood for malaria
- Viral testing
Splenomegaly - imaging
- If no hemolytic disease, no sign of infection, no sign of congestion:
- Ultrasound with Doppler
- Liver spleen scan
- Biopsy of lymph node, liver, or other tissue, depending on findings
- If no hemolytic disease, no sign of infection, but sign of congestion:
- Ultrasound with Doppler
- MRI; consider MRA/MRV
- CT scan
Splenomegaly - differencial diagnosis
- Infection:
- Bacteria:
- Bacteremia
- Pneumonia
- Sepsis
- Subacute bacterial endocarditis
- Salmonella
- 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
- Hereditary hemochromatosis
- Congenital hepatic fibrosi
- Autoimmune hepatiti
- 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 reticulocytosis
- Miscellaneous:
- Serum sickness
- Connective-tissue disorders
- Juvenile rheumatoid arthritis
- Systemic lupus erythematosus
- Sarcoidosis
- Splenic hamartoma
- Splenic cysts: Congenital and posttraumatic
- Nonsplenic upper left quadrant abdominal masses:
- Large kidney
- Retroperitoneal tumor
- Adrenal neoplasm
- Ovarian cyst
- Pancreatic cyst
- Mesenteric cyst
- 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.
Splenomegaly - FOLLOW UP
Splenomegaly - disposition
Splenomegaly - issues for referral
- Disproportionate size of spleen
- Increasing size over serial examinations
- Unexplained lymphadenopathy
- Liver dysfunction
- Ascites
- Signs of storage or metabolic disease
- Howell–Jolly bodies on peripheral smear, suggesting splenic dysfunction
Splenomegaly - bibliography
- Donnelly LF, Foss JN, Frush DP, et al. Heterogeneous splenic enhancement patterns on spiral CT images in children: Minimizing misinterpretation. Radiology. 1999;210:493–497.
- Imrie J, Wraith JE. Isolated splenomegaly as the presenting feature of Niemann-Pick disease type C. Arch Dis Child. 2001;84:427–429.
- McCormick PA, Murphy KM. Splenomegaly, hypersplenism and coagulation abnormalities in liver disease. Best Pract Res Clin Gastroenterol. 2000;14:1009–1031.
- Rosenberg HK, Markowitz RI, Kolberg H, et al. Normal splenic size in infants and children: Sonographic measurements. Am J Roentgenol. 1991;19:1465.
- Sheth S, Ruzal-Shapiro C, Piomelli S, et al. CT imaging of splenic sequestration in sickle cell disease. Pediatr Radiol. 2000;30:830–833.
- Zimmerman SA, Ware RE. Palpable splenomegaly in children with haemoglobin SC disease: Haematological and clinical manifestations. Clin Lab Haematol. 2000;22:145–150.
Splenomegaly - CODES
Splenomegaly - icd9
789.2 Splenomegaly, enlargement of spleen
Splenomegaly - FAQ
- Q: How long will the enlarged spleen secondary to a viral infection be present?
- A: The enlarged spleen may persist for several months.
Book Source Details
- Book Title: The 5-Minute Pediatric Consult
- Author(s): M. William Schwartz MD; et al.
- Year of Publication: 2008
- Copyright Details: The 5-Minute Pediatric Consult, Copyright © 2008 Lippincott Williams & Wilkins.
Other Book Chapters Related to Swollen spleen
Read excerpts from these other book chapters related to Swollen spleen:
Medical Books Excerpts
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- "Professional Guide to Signs & Symptoms (Fifth Edition)" (2006)
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- "Professional Guide to Signs & Symptoms (Fifth Edition)" (2006)
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- Hepatomegaly
- "The 10-Minute Diagnosis Manual: Symptoms and Signs in the Time-Limited Encounter" (2000)
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- Splenomegaly
- "The 10-Minute Diagnosis Manual: Symptoms and Signs in the Time-Limited Encounter" (2000)
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- Splenomegaly
- "Alarming Signs and Symptoms: Lippincott Manual of Nursing Practice Series" (2007)
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- Hepatomegaly
- "The Diagnostic Approach to Symptoms and Signs in Pediatrics" (2006)
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- Splenomegaly
- "The Diagnostic Approach to Symptoms and Signs in Pediatrics" (2006)
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Copyright Details: The 5-Minute Pediatric Consult, Copyright © 2008 Williams & Wilkins.
More About Causes of Swollen spleen
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More About This Book:
Title: The 5-Minute Pediatric Consult
Authors: M. William Schwartz MD; et al.
Publisher: Lippincott Williams & Wilkins
Copyright: 2008
ISBN: 0-7817-7577-9
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