Fatty Liver of Pregnancy

 Some affected mothers and infants have inherited long-chain 3-hydroxyacyl-CoA dehydrogenase (LCHAD) deficiency


image Carnitine palmitoyltransferase I deficiency also associated with fatty liver of pregnancy





Clinical Issues




• Most commonly occurs in last 10 weeks and usually in last 4 weeks of pregnancy
• Rapidly progressive acute liver failure

• Early diagnosis and delivery are critical

image Can be fatal for mother and fetus if not diagnosed and treated early

• Treatment
image Supportive care and urgent delivery of infant

• Early diagnosis and delivery associated with excellent outcome


Microscopic




• Severe microvesicular steatosis, predominantly centrilobular or diffuse
image May show small rim of periportal sparing

• Zone 3 perivenular canalicular cholestasis and hepatocellular cholestasis


Top Differential Diagnoses




• Acute hepatic failure unrelated to pregnancy

• Preeclampsia/eclampsia

image
Centrilobular Microvesicular Steatosis
Extensive microvesicular steatosis involves the centrilobular or zone 3 hepatocytes. A central vein image is present in the center of the image.


image
Microvesicular Steatosis
High-power view demonstrates the extensive microvesicular steatosis typical of fatty liver of pregnancy. Numerous small steatotic vacuoles image surround and focally indent the hepatocyte nucleus.

image
Periportal Hepatocyte Sparing
There is a background of extensive microvesicular steatosis, but there is relative sparing of a small rim of periportal hepatocytes image .

image
Acute Pancreatitis in Fatty Liver of Pregnancy
Acute pancreatitis image with necrosis image may also occur in patients with fatty liver of pregnancy.


TERMINOLOGY


Synonyms




• Acute fatty liver of pregnancy (AFLP)

• Hepatic lipidosis of pregnancy

• Sheehan syndrome


Definitions




• Acute liver failure during 2nd half of pregnancy with severe fatty infiltration of liver

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Apr 20, 2017 | Posted by in PATHOLOGY & LABORATORY MEDICINE | Comments Off on Fatty Liver of Pregnancy

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