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

Tags:
Aug 10, 2016 | Posted by in OBSTETRICS | Comments Off on Hepatomegaly

Full access? Get Clinical Tree
Get Clinical Tree app for offline access