Death due to acute fatty liver in pregnancy

Introduction: Acute fatty liver of pregnancy (AFLP) results due to disordered metabolism of fatty acids by mitochondria in the mother, caused by deficiency ofdehydrogenase enzyme. This is a rare instance of a foetus causing metabolic disease in the mother. The ‘working diagnosis’ of the clinicians was HELLP syndrome. The exact cause of death was made clear only after the postmortem examination.

Case report : 24 year old primi with 34 weeks of gestation admitted for blood pressure monitoring. She developed jaundice, high blood pressure with proteinurea, marginally elevated liver enzymes, low platelets which warranted termination of pregnancy. Despite the termination of pregnancy, she developed renal failure and hepatic encephalopathy and died on the eleventh day after delivery. Post mortem histopathology of liver revealed that the architecture was not altered and micro-vesicular fat infiltrations in centrilobular and mid zones. Hepatocytes were swollen but inflammation was not evident. Other tissues did not show any fat infiltration. HELLP syndrome was excluded due to absence of macro-vesicular fat infiltration in modest quantities throughout the liver lobules. There were no lobular disarray with hepatocyte dropout, reticulin collapse and portal tract inflammation to suggest viral hepatitis. There were no evidence of drug induced, toxic or viral induced micro-vesicular steatosis. Reye’s syndrome is characterized by uniform micro-vesicular fatty change in the liver and other tissues such as skeletal muscles, kidney and heart.

Conclusion: Having excluded all other possibilities, the cause of death was ascertained as AFLP and this emphasizes the value of correct histopathological diagnosis.

Medico-Legal Journal of Sri Lanka 2013; 1(2) : 42-45