Rare. Acute hepatocellular failure occurring during the 3rd trimester of pregnancy in 1/6,000 to 1/13,000 parturients (50 % of primiparous women). Mortality: 0-20 % with neonatal mortality ranging from 15 to 23 %.

Etiology: hepatic accumulation of metabolites of the 3-hydroxyacyl pregnancy following a deficiency in the β-oxidation of long chain fatty acids. Another hypothesis is that the fetus could be carrying the homozygous form and the mother the heterozygous form of a 3-hydroxyl-acyl-CoA dehydrogenase deficiency that is involved in the mitochondrial β-oxydation of long-chain fatty acids. Pathology: centrilobular  microvesicular steatosis without necrosis.

Clinical signs:

-         nausea and vomiting (80 %)

-         epigastric pain (70 %)

-         jaundice delayed for 3-5 days (60 %)

-         rapid evolution to coma with hyperammoniemic encephalopathy  and hypoglycemia

Complications: coagulopathy with thrombocytopenia, acute renal failure, sepsis, ARDS.

Treatment: emergency delivery; symptomatic treatment while the liver function recovers.

Differential diagnosis: pre-eclampsia, HELLP syndrome

Anesthetic implications: 

cesarean section in a patient with liver (and often renal) acute failure; coagulopathy; intracranial hypertension.

References : 

-        Dedecker F, Graesslin O, Palot M, Fortier D, Quéreux C, Gabriel R. 
Stéatose hépatique aiguë de la grossesse : une pathologie rare du troisième trimestre de grossesse. 
Gyn Obstétr Fertilité 2006 ; 34 : 131-3.

-         Holzman RS, Riley LE, Aron E, Fetherston J. 
Perioperative care of a patient with acute fatty liver of pregnancy. 
Anesth Analg 2001; 92: 1268-70.

-         Naidu RK, Richebe P. 
Probable local anesthetic systemic toxicity in a postpartum patient with acute fatty liver of pregnancy after transversus abdominis plane block. 
A&A Case Reports 2013; 1: 72-4

Updated: December 2019