Acute Fatty Liver of

Pregnancy
Jonathan Healan and Niamh O Shiel

Patient
25 year old seen in Antenatal clinic 10 weeks
gestation.
G2P1 (still birth 38w back in 2011)

Previous pregnancy normal until week 38, patient

felt nauseous with abdominal pain and was admitted
to hospital. Very unwell.
No fetal heart rate on CTG confirmed IUD
Vaginal delivery.

Patient
Liver enzymes
Leucocytes
Uric acid

PMHx: Nil
FHx: Nil
Diagnosis Fatty liver of pregnancy, liver enzymes
began to normalise following delivery with no
lasting effect on patient.

Acute Fatty Liver of

Pregnancy (AFLP)
Obstetric emergency; Maternal and fetal mortality
significantly raised, 18% and 23% respectively.
Usually occurs in third trimester. Microvesicular
infiltration of hepatocytes.
Fortunately it is a rare condition. UK study found
incidence of 5/100,000.
Gold standard of diagnosis is liver biopsy, but
Swansea classification relies on signs and symptoms.

These changes usually revert

to normal within days to
weeks following delivery
without persistent hepatic
injury.

(Chng et al.2002)

Pathophysiology
Abnormality in mitochondrial
oxidation is recognised as the
cause of this condition.
Long-chain 3-hydroxyacyl-CoA
dehydrogenase (LCHAD)
catalyses the third step in the
oxidation of fatty acids in
mitochondria.

(Joshi et al. 2010)

If fetus is deficient in LCHAD toxic metabolites can accumulate and cause

damage to the mothers liver. The long chain fatty acids are deposited in the
mothers liver giving rise to the characteristic appearance of steatosis
histologically.

(Joshi et al. 2010)

Pathophysiology
It is recessive and so it is thought
that under normal physiological
conditions the mother has normal
fatty acid oxidation
If both parents are heterozygous, the fetus can inherit both mutations and
will be unable to oxidise long-chain fatty acids
The free fatty acids remain unmetabolised and are returned to the mothers
circulation
This causes strain on the mothers liver and overwhelms any diminished
maternal hepatic enzymatic activity
(Hin Ko & Yoshida, 2004)

Pathophysiology
Progressive lipid accumulation in hepatocytes
Usually fat content of liver is 5%, can go up to 19% in
AFLP
Fat accumulation plus ammonia production leads to
coagulopathy and hypoglycemia
Liver can be small, soft and yellow

Microvesicular fat can also infiltrate

kidney, brain and bone marrow
(McNulty 2004)

Management
Get the baby out!
Early diagnosis, quick delivery and intensive care
Stablise mother airway management, IV fluids, correct
hypoglycemia, electrolyte and coagulation abnormalities,
blood products
Once stabilised, deliver baby vaginal birth if possible,
but often c-section is necessary
Postpartum plasma exchange in severe cases
(Moldenhauer, OBrien, Barton & Sibai, 2004)

Complications
Patients at high risk of bleeding due to coagulation
problems blood transfusion and fluids may be
needed
Risk of hypoglycaemia glucose infusion may be
needed
Pancreatitis can develop following renal and hepatic
dysfunction

Prognosis
Mortality from AFLP app 18% for mothers, 23% for
fetus - deaths usually secondary to sepsis, renal failure,
circulatory collapse, pancreatitis or GI bleeding
Maternal outcomes liver function tests can
continuously worsen for up to a week but then recover
Fetal outcomes there can be fetal distress in a clinically
stable mother so fetal monitoring is essential
Recurrence of AFLP can occur in further pregnancies
but is quite uncommon
(Hin Ko & Yoshida, 2004)

Patient
Autosomal recessive condition. Recurrence of
AFLP estimated around 20% in general population.
Greater risk in primiparous women.

Surveillance of pregnancy paramount, especially in

third trimester.
Deliver baby quickly if showing signs of AFLP.
Hopefully at viable gestation, as delivery is only
cure.

GUT 2002
Prospective study of liver dysfunction in pregnancy in
Southwest Wales
C L Chng, M Morgan, I Hainsworth,JGC Kingham

LFT abnormalities were investigated in 4377 deliveries during the 15

month study period.

Liver dysfunction was seen in 3% of deliveries during the 15 month

prospective study.

Of the 3% only 5 cases of AFLP. Close medical and obstetric

collaboration ensued low mortality.

References
Hin Ko, H. & Yoshida, E. (2006) Acute fatty liver of
pregnancy. Canadian Journal of Gastroenterology. 20: 25-30
McNulty J. Acute fatty liver of pregnancy. In: Foley MR,
Strong TH, Garite TJ, editors. Obstetric Intensive Care
Manual. 2. New York: The McGraw-Hill Companies Inc;
2004. pp. 20715.
Moldenhauer, J.S., OBrien, J.M., Barton, J.R., & Sibai,
B. (2004). Acute fatty liver of pregnancy associated with
pancreatitis: a life-threatening complication. American
Journal of Obstetrics and Gynaecology. 190:502-505

 Marsha F. Browning, Harvey L. Levy, Louise E. WilkinsHaug, Cecilia Larson and Vivian E. Shih. (2006) Fetal Fatty
Acid Oxidation Defects and Maternal Liver Disease in
Pregnancy, American college of obstetricians and gynaecologists.
1:107.
Chng C, Morgan M, Hainsworth I, Kingham J. (2002)
Prospective study of liver dysfunction in pregnancy in
Southwest Wales. GUT. 51:876880.

Joshi D, James A, Quaglia A, Westbrook R, Heneghan

M. (2010) Liver Disease in Pregnancy. The Lancet.
375:594-605
Willacy H. (2011) Jaundice in Pregnancy. Patient.co.uk.
Available at: http://www.patient.co.uk/doctor/Jaundicein-Pregnancy.htm#ref-6