HEPATOMEGALY by nikeborome

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    Katarzyna Pawelec

• A liver 1-2 cm below the right costal margin is
  considered normal up to the age of 2-3 years
• Hepatomegaly- palpable, large mass under right
  costal margin extending to midline
• Liver span should be determined as liver may
  extend below costal margin secondary to
  inspiration or to a low-lying diaphgram (normal
  liver span 6-12 cm in midclavicular line in
  children 6-12 years)
          Palpating the liver
• Approach from RIF .
• Lay one’s fingers gently on the abdomen
  and allow the child’s respiratory movement
  to bring the liver to meet your fingers
• Measure liver breadth in cm, not in fingers
• Measure the liver span
Expected Liver Span ot Intants and Children*
           MALES                                                                                         FEMELES

             MEAN ESTIMATED                                                                               MEAN ESTIMATED

AGE (yr)        LIVER SPAN                    SEM                        AGE (yr)                           LIVER SPAN     SEM
  6 mo              2.4                          2.5                          6 mo                             2.8          2.6
    1               2.8                          2.0                               1                           3.1          2.1
    2               3.5                          1.6                               2                           3.6          17
    3               4.0                          1.6                               3                           4.0          1.7
    4               4.4                          1.6                               4                           4.3          1.6
    5               4.8                          1.5                               5                           4.5          1.6
    6               5.1                          1.5                               6                           4.8          1.6
    8               5.6                          1.5                               8                           5.1          1.6
   10               6.1                          1.6                             10                            5.4          1.7
   12               6.5                          1.8                             12                            5.6          1.8
   14               6.8                          2.0                             14                            5.8          2.1
   16               7.1                          2.2                             16                            6.0          2.3
   18               7.4                          2.5                             18                            6.1          2.6
   20               7.7                          2.8                             20                            6.3          2.9
                              "From Lawson EE, Grand RJ, Neff RK, et al: Am J Dis Child 1978; 132:475.
        Differential diagnosis of
   - Viral: Hepatotrophic (A,B,C,D,E) and
     other viruses (herpes, CMV, EBV,
     Varicella, HIV, rubella, adeno, entero,
   - Protozoal: malaria, amoebic,
        Differential diagnosis of
• Bacterial:
  sepsis, tuberculosis, brucellosis, syphilis
• Helminths:
  hydatoid, visceral larva migrans
• Fungal:
       Differential diagnosis of
• Malignancies:
  leukemia, histiocytic syndromes,
  myeloproliferative syndromes, lymphomas,
  neuroblastoma, Wilms tumor
• primary tumors: hepatoblastoma,
  hepatocellular carcinoma, hemangioma,
       Differential diagnosis of

• Immunological:
  Chronic granulomatous,
• Autoimmune:
        Differential diagnosis of

• Intrahepatic obstruction to hepatic vein outflow:
  - veno-oclusive disease
  - hepatic vein thrombosis (Budd-Chiari syndrome)
• Suprahepatic obstruction to hepatic vein outflow:
  - congestive heart failure
  - pericardial disease
  - tamponade
  - constrictive pericarditis
       Differential diagnosis of

• Haemopoetic:
   - haemolytic:
     haemolytic disease of newborn,
     sickle cell anemia
      Differential diagnosis of
  hepatomegaly- storage disorders
• FAT: Reye syndrome, malnutrition, obesity,
  TPN, cystic fibrosis, diabetes mellitus
• Specific lipid storage diseases: Gaucher,
  Niemann-Pick, Walman syndrome
• Glycogen: glycogen storage diseases, TPN,
  Beckwith syndrome
• Miscellaneous: alpha- antitripsin deficiency,
  Wilson disease, hypervitaminosis A,
   Clinical Disease States and Age ot
Presentation ot Hepatomegaly- Newborn

• Intrauterine and intrapartum acquired
  infection (TORCH,t syphilis, other)
• Erythroblastosis fetalis
• Neonatal hepatitis, <X1-antitrypsin,
• Biliary atresia
• Congestive heart failure
• Sepsis
        Hepatomegaly- infant
• Cystic fibrosis
• Metabolic disease: glycogen storage <X1-
  antitrypsin deficiency, galactosemia,
  tyrosinemia, hereditary fructose intolerance,
• Neonatal hepatitis, hepatitis B
• Histiocytosis
• Malnutrition
• Tumors (intrinsic, metastatic)
• Cholelithiasis
• Choledochal cyst
     Hepatomegaly- 1-6 year old

•   Viral hepatitis
•   Orug-toxic hepatitis
•   Parasitic
•   Tumors
•   Leukemia,
•   Lymphoma
    Hepatomegaly- older child and
• Viral hepatitis Orug-toxic hepatitis Wilson disease
• Chronic active hepatitis Congenital hepatic fibrosis
• Focal nodular hyperplasia, ad en oma <X1-Antitrypsin
• Reye's syndrome
• Sickle celi anemia
• Cholelithiasis
• Juvenile rheumatoid arthritis, lupus erythematosus,
• sarcoidosis
• Leukemia, Iymphoma Gonococcal perihepatitis Cystic
• Important for separating acute from
  chronic processes
• Exposure to jaundiced or ill individuals,
  carriers of hep B or animal vectors of
  hepatotropic inf
• Previous transfusions
• Intravenous drug use
• Travel history
• Hepatotoxic drugs and toxins
• Disease of acute onset is more likely to be
  infectious, toxic or congestive
• Disease of chronic onset- infiltrative,
  obstructive, metabolic (except of the acute
  presentation of galactosemia and fructose
  intolerance in infancy or Wilson disease in
  later childhood)
• Impotance of evaluating the prodromal
    - acute hepatitis associated with EBV-
  fever, pharyngitis,
    - hepatitis A- nausea, vomiting, anorexia,
    - hepatitis B- malaise, arthralgia,
  arthritis, urticarial or maculopapular rash,
  hematuria and proteinuria
• Family history
• Past medical history
• Review of systems
         Physical examination
•   Size
•   Shape (symetry or asymetry)-
•   Consistency (soft, firm, rock-hard)
•   Presence or absence of tenderness
•   Surface contour (smooth, irregular,
        Physical examination
• The presence of asymmetry suggests a
  space occupying lesion- cyst, hydroptic
• Firm symmetric hepatomegaly with
  rounded edge is consistent with infiltration,
  fibrosis or congestion and can notes a
  more chronic process
        Physical examination
• Rock-hard hepatomegaly with a sharp
  nontender edge favors cirrosis or malignancy
• Diffuse liver tenderness implies generalized
  capsular distention from acute parenchymal
  inflammation or congestion
• Rubs and bruits should be ausculated to rule out
  infectious or traumatic inflammation of the liver
  capsule or intrahepatic vascular tumors
          Physical examination
• Spider angiomas, clubbing, gynecomastia- chronicity
• Rickets or pathologic fractures, multiple ecchymoses,
  decreased reflexes, glossitis gingival inflammation-
  malabsorption and malnutrition
• Conjugated hyperbilirubinemia and jaundice-
  extrahepatic obstruction and inflammatory hepatitis, later
  stages of many chronic processes (Wilson disease,
  cystic fibrosis)
• Jaundice is unusual in infiltrative processes
  (malignancy), storage diseases or chronic congestive
        Physical examination
• Splenomegaly associated with hepatomegaly is
  an important sign in differential dgn
  -/+ fever, malaise, pharyngitis, adenopathy
  splenomegaly- suggest EBV, CMV
  - in the absence of systemic symptoms, a large
  spleen connotes chronic liver disease with portal
  - massive enlargenent of spleen and liver-
  Gaucher, Niemann-Pick, histiocytosis,
  hematologic malignancies
       Physical examination
• Ascites
  - manifestation of chronic liver disease
  secondary to increased portal venous
  pressure and low serum albumin levels
  - hepatic venous obstruction- Budd-Chiari
         Physical examination
• Mental status- assessing severity of liver
  - inversion in the sleep-wake cycle
  - hypersomnia or insomnia
  - impaired computation
  - shortened attention span
  -euphoria or depression
  - irritability, impaired handwrinting
  - muscular incoordination
  - hyperventilation
• Chemistry panel- SGOT, SGPT, AP,
• Albumin, prothrombin time, bilirubin total and conjugated
  (direct): reflects hepatic synthetic dysfunction
• Alpha1 antitrypsin
• Blood amonia
• Abdominal ultrasound: helpful in determining portal
  hypertension, splenic anatomy, portal/splenic vein flow
• Abdominal CT scan
• Percutaneous liver biopsy: tissue for culture and
• Splenomegaly- a palpable spleen more
  than 1 cm below the left costal margin
  constitues splenomegaly
• Spleen is palpable in 30% of newborns
  and in 15% of infants younger than 6 mo
  of age
• The enlarged spleen moves on respiration
• Dull on percussion
• The spleen may enlarge medially towards
  the umbilicus or downwards the left iliac
• Splenic size should be recorded in cm
  below the costal margin
       Functions of the spleen
• Hematopoiesis-
  - active blood formation in fetal live
  - hematopoiesis persists or is resumed
  postnatally in hemolytic disease of newborn,
• Remove damaged or abnormal blood cells from
  the circulation-
  - red cells and platelets coated by antibodies are
  selectively sequestered and destroyed by the
  - destroying spherocytes
      Functions of the spleen
• „Pitting”- the spleen has the ability to
  remove or pit intracytoplasmic inclusions
  such as Howell-Jolly bodies from within
  the red cell without destroying the cell
  - the peripheral blood of a person with no
  spleen contains relatively large numbers of
  these inclusions
      Functions of the spleen
• Destruction of senescent red cells
• Filttering and immunologic functions:
  - primary defense agains bacteria which
  gain access to the circulation
  -primary site of clearance of organisms
  such as pneumococci in the absence of
  specific antibody
 - role in antibody formation in response to
  circulating antigens
Causes of splenomegaly in children
• Hematologic diseases:
     -congenital and acquired hemolytic anemia,
      hemoglobinopathies and thalasemia
• Infections:
     -bacterial : septicemias, typhoid,
     -viral –EBV, CMV
     - protozoal: malaria, toxoplasmosis
Causes of splenomegaly in children
• Congestive splenomegaly
  -secondary to portal or splenic vein
  -secondary to intrahepatic disease-cirrosis
  -chronic congestive heart failure
• Infiltrations:
  -Lipidoses- Niemann-Pick, Gaucher disease
Causes of splenomegaly in children
• Cysts:
  -congenital-epidermoid cysts
  -acquired- pseudocysts
• Neoplasms
  -leukemia and lymphoma
  -Hodgkin disease
  -hemangioma and lymphangioma
Causes of splenomegaly in children
• Rheumatoid arthritis
• Depression of 1 or more of the cellular
  elements of the blood
• Active formation of that element in the bone
• An enlarged spleen, due to number of
• Correction of the hematologic abnormalities
  by splenectomy
  - may indicate leukemia (blasts or
  depression of cell lines)
  - infection (atypical lymphocytes)
  - hemolytic anemia, hemoglobinopathy
  - leukopenia and thrombocytopenia
  secondary to splenomegaly
• Chemistry panel- SGOT, SGPT, AP,
• Albumin, prothrombin time, bilirubin total and conjugated
  (direct): reflects hepatic synthetic dysfunction
• Alpha1 antitrypsin
• Blood amonia
• Abdominal ultrasound: helpful in determining portal
  hypertension, splenic anatomy, portal/splenic vein flow
• Abdominal CT scan
• Percutaneous liver biopsy: tissue for culture and

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