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.

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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



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

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