Cholestasis of pregnancy (intrahepatic cholestasis of pregnancy) is thought to result from an exaggerated cholestatic effect of oestrogens. It occurs in approximately 0.2% of pregnancies (except in Scandinavia and South America, where incidences of up to 10% have been reported) and is associated with multiple pregnancy. Its presenting symptom is localised (usually to the hands and feet) or generalised pruritus, occurring particularly at night. This is caused by the deposition of bile acids in the skin. Symptoms usually occur during the third trimester and may be sufficiently distressing to necessitate induction of labour. The condition is specific to pregnancy, and patients are invariably asymptomatic within 6 weeks after delivery. The condition tends to recur in subsequent pregnancies.
Problems and special considerations
Although symptoms usually occur in the third trimester, the condition may present at any stage of pregnancy. There is a personal or family history of jaundice while using the oral contraceptive pill, or during a previous pregnancy, in about 50% of cases.
Jaundice occurs in up to 50% of cases if untreated or undelivered. Clinical examination is otherwise normal except for scratch marks because of the severe itching. Fat malabsorption occurs, and the mother may complain of steatorrhoea. Malabsorption may result in vitamin K deficiency. Symptoms tend to precede changes in liver function tests, which reveal predominantly conjugated bilirubinaemia, with markedly increased alkaline phosphatase and mildly increased transaminases. Liver ultrasound is usually normal.
While the prognosis is favourable for mothers, the condition is not as benign for babies. There is an increased risk of premature delivery (spontaneous and iatrogenic), fetal distress and intrauterine death, presumed to be secondary to reduced placental blood flow or a direct effect of bile salts. Risk of fetal morbidity and mortality increases with gestational age, and early delivery is often recommended.
Management options
Treatment with cholestyramine, antihistamines or topical preparations has variable success. Corticosteroids have also been used, but most women are now treated with ursodeoxycholic acid, which has been shown to improve symptoms and reduce serum bile acid levels. Both the mother and the neonate should receive vitamin K therapy.
The mother’s coagulation should be checked during the antenatal period and before considering insertion of an epidural or spinal needle, although coagulopathy is rare. While it is acknowledged that changes in coagulation are not rapid, guidance issued by the Association of Anaesthetists of Great Britain and Ireland (AAGBI) recommends that clotting studies be checked in the 24 hours preceding neuraxial intervention. If coagulation studies are within normal limits, the mother should be encouraged to have epidural analgesia for labour, since there is an increased incidence of caesarean section.
Key points
• Cholestasis of pregnancy is a benign and self-limiting condition for the mother but is associated with an increased incidence of preterm labour, fetal distress and caesarean delivery.
• Coagulopathy may occur secondary to vitamin K malabsorption.
• The only definitive treatment is delivery.
• Epidural analgesia should be encouraged unless contraindicated by coagulopathy.
Further reading
Joshi D, James A, Quaglia A, Westbrook RH, Heneghan MA. Liver disease in pregnancy. Lancet 2010; 375: 594-605.
Royal College of Obstetricians and Gynaecologists. Obstetric Cholestasis. Green-top Guideline 43.
London: RCOG, 2011 (updated 2014). www.rcog.org.uk/en/guidelines-research-services/guide lines/gtg43 (accessed December 2018).
Westbrook RH, Dusheiko G, Williamson C. Pregnancy and liver disease. J Hepatol 2016; 64: 933-45.