Hepatomegaly

Hepatomegaly
Paula J. Woodward, MD
DIFFERENTIAL DIAGNOSIS
Common
  • Hydrops
    • Immune Hydrops
    • Nonimmune Hydrops
  • Infection
Less Common
  • Beckwith-Wiedemann Syndrome
  • Trisomy 21
Rare but Important
  • Liver Tumors
    • Hemangioendothelioma
    • Mesenchymal Hamartoma
    • Hepatoblastoma
    • Leukemia
    • Neuroblastoma Metastases
  • Glycogen Storage Disorder
ESSENTIAL INFORMATION
Key Differential Diagnosis Issues
  • Is the liver diffusely enlarged or is there a focal mass(es)?
    • Liver occupies a significant portion of the abdomen so an enlarged abdominal circumference may be first sign of diffuse enlargement
    • If focal mass, consider tumors (except leukemia)
      • Cystic mass more likely mesenchymal hamartoma
      • Marked vascularity more likely hemangioendothelioma
  • Are there calcifications (in the liver and elsewhere) that would suggest infection?
  • Is there organ overgrowth or other obvious anomalies?
  • Look for signs of hydrops
    • Hepatomegaly may be one of first signs before florid hydrops develops
    • Skin/subcutaneous edema
    • Ascites
    • Pleural effusions
    • Pericardial effusion
    • Other findings
      • Placentomegaly (placental thickness > 40 mm)
      • Polyhydramnios
Helpful Clues for Common Diagnoses
  • Hydrops
    • Vascular congestion may cause hepatomegaly
      • May occur before other more obvious signs
    • Important to recognize and institute treatment (if possible) before onset of hydrops, which often portends a poor outcome
    • Immune Hydrops
      • Maternal antibodies cross placenta and cause lysis of fetal red blood cells, leading to fetal anemia
      • Spleen often also involved (hepatosplenomegaly) but may be difficult to differentiate from liver
      • Hepatosplenomegaly not only from high cardiac output, but also may be secondary to extramedullary hematopoiesis in prolonged anemia
      • Anemia causes an elevated middle cerebral artery (MCA) peak systolic velocity (PSV)
    • Nonimmune Hydrops
      • Multiple causes, most of which have obvious other findings
      • Cardiac: Structural or arrhythmia
      • Fetal masses: Either vascular masses with arteriovenous shunting or masses which obstruct venous return
      • Placental chorioangioma
      • Aneuploidy
      • Twin-twin transfusion syndrome (TTTS)
  • Infection
    • Cytomegalovirus (most common in utero infection), toxoplasmosis, parvovirus, varicella, HIV
    • Look for punctate, non-shadowing calcification within liver
      • May see calcifications elsewhere: Brain (periventricular, cortical), diaphragm, pericardium
    • Usually other findings also present
      • Ventriculomegaly, echogenic bowel, cardiomegaly, growth restriction, polyhydramnios, hydrops
Helpful Clues for Less Common Diagnoses
  • Beckwith-Wiedemann Syndrome
    • Organomegaly, primarily hepatosplenomegaly and nephromegaly
    • Macroglossia
    • Hemihypertrophy
    • Omphalocele rarely
  • Trisomy 21
    • Hepatomegaly may be from nonimmune hydrops or myeloproliferative disorder
      • Transient myeloproliferative disorder: Variable spectrum of severity
      • Congenital leukemia: Extensive organ involvement
    • Other markers of trisomy 21 more obvious
Helpful Clues for Rare Diagnoses
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Aug 10, 2016 | Posted by in OBSTETRICS | Comments Off on Hepatomegaly

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