Splenomegaly



Splenomegaly


Matthew J. Ryan



INTRODUCTION

Splenomegaly may be evident in benign viral infections, serious systemic infections, hematologic diseases, neoplastic conditions, and metabolic disorders. The terms “splenomegaly” and “hypersplenism” are not interchangeable. Splenomegaly refers to an enlarged spleen, whereas hypersplenism refers to a hyperfunctioning spleen that results in a reduction in the number of circulating blood cells. Hypersplenism is usually associated with splenomegaly, but not always.

A palpable spleen tip is not necessarily pathologic. Approximately 3% of older adolescents and adults have palpable spleens. A palpable spleen is detected in most premature infants and up to 30% of term infants. By adulthood, the normal spleen, which is located beneath the 9th and 11th ribs, reaches dimensions of 12 cm long, 7 cm wide, and 3 cm thick. Normal spleens are soft, located at the mid-clavicular line, nontender and often only palpable on deep inspiration. Splenic tenderness or a spleen edge >2 cm below the costal margin is always abnormal. A normal-size spleen being pushed down by hyperinflated lungs may cause apparent splenomegaly.



image HINT: Percussion can be used to evaluate splenic size. To delineate the lower “tip,” the examiner starts in the area of tympany in the left anterior axillary line of the mid-abdomen and percusses upward toward the splenic dullness. The upper border is defined by starting in the left midaxillary line of the midthorax and percussing downward toward the splenic dullness. Proper abdominal exam on a relaxed abdomen is critical to palpate an enlarged spleen. Begin in the left lower quadrant (an enlarged spleen may be missed by beginning in the left upper quadrant). Stand on the patient’s right side and use the right hand to palpate while supporting the rib cage with the left hand.


DIFFERENTIAL DIAGNOSIS LIST


Infectious Causes


Viral Infection



  • Adenovirus


  • Mononucleosis (Epstein-Barr virus [EBV] infection)


  • Cytomegalovirus (CMV) infection


  • Coxsackievirus


  • HIV


  • Rubella


  • Herpes


  • Hepatitis A, B, C



Bacterial Infection



  • Pneumonia


  • Sepsis


  • Endocarditis


  • Brucellosis


  • Tularemia


  • Splenic abscess


  • Cat-scratch disease—Bartonella sp.


  • Tuberculosis


  • Syphilis


  • Leptospirosis


  • Rocky Mountain spotted fever (RMSF)—Rickettsia rickettsii


  • Salmonella infection


  • Streptococcal infection


  • Ehrlichiosis


Other Infections



  • Malaria


  • Toxoplasmosis


  • Babesiosis


  • Histoplasmosis


  • Coccidioidomycosis


  • Schistosomiasis


  • Trypanosomiasis


Neoplastic Causes



  • Leukemia


  • Lymphoma


  • Lymphosarcoma


  • Hamartoma


  • Metastatic disease (neuroblastoma)


Traumatic Causes



  • Laceration


  • Hematoma


  • Traumatic cyst


Metabolic or Genetic Causes


Lipid Metabolism Defects



  • Gaucher disease


  • Niemann-Pick disease


  • Gangliosidoses


  • Mucolipidoses


  • Metachromatic leukodystrophy


  • Wolman disease


Mucopolysaccharidoses



  • Hurler syndrome


  • Hunter syndrome


Hematologic Causes


Red Blood Cell (RBC) Membrane Defects



  • Hereditary spherocytosis


  • Hereditary elliptocytosis


  • Hereditary stomatocytosis


RBC Enzyme Defects



  • Glucose-6-phosphate dehydrogenase (G6PD) deficiency


  • Pyruvate kinase deficiency


Hemoglobin Defects



  • Sickle cell disease (SCD)


  • Thalassemia


Extrinsic Hemolytic Anemias



  • Autoimmune hemolytic anemia


  • Erythroblastosis fetalis


Congestive Causes



  • Congestive heart failure


  • Constrictive pericarditis


  • Chronic liver disease with portal hypertension


  • Perisplenic anatomic obstructions


  • Splenic vein thrombosis


  • Splenic artery aneurysm


  • Cavernous transformation of the portal vein


Miscellaneous Causes



  • Serum sickness


  • Splenic hemangioma


  • Chronic granulomatous disease


  • Juvenile rheumatoid arthritis (JRA)


  • Systemic lupus erythematosus (SLE)


  • Autoimmune hepatitis


Histiocytic Disorders



  • Langerhans cell histiocytosis


  • Hemophagocytic lymphohistiocytosis



  • Malignant histiocytic disorders


  • Beckwith-Wiedemann syndrome


  • Amyloidosis


  • Sarcoidosis


  • Congenital splenic cyst



image HINT: Not all left upper quadrant masses are related to the spleen. Other conditions such as an enlarged kidney, a retroperitoneal tumor, an adrenal neoplasm, an ovarian, pancreatic, or mesenteric cyst may mimic splenomegaly on examination. Imaging such as ultrasound, computed tomography (CT), or MRI may be useful in the evaluation of a left upper quadrant mass if the history and laboratory studies are not consistent with the physical examination findings of splenomegaly.


DIFFERENTIAL DIAGNOSIS DISCUSSION


Benign Infection

Many benign infections, especially upper respiratory tract infections, can be associated with mild, transient splenomegaly. Adenovirus, coxsackievirus, and Streptococcus are commonly involved organisms.


Epstein-Barr Virus Infection (Infectious Mononucleosis)

Infectious mononucleosis is discussed in Chapter 50, “Lymphadenopathy.” Of note, splenomegaly may persist for several months after resolution of mononucleosis. The splenomegaly should be resolved before the athlete returns to contact sports.


Cytomegalovirus Infection


Epidemiology

CMV is ubiquitous, and most people are infected with the virus by adulthood. The source is body fluids, including blood, urine, breast milk, saliva, and feces. Transmission of CMV is both horizontal (person to person) and vertical (mother to child). CMV is the most common congenital viral infection.

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Sep 14, 2016 | Posted by in PEDIATRICS | Comments Off on Splenomegaly

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