This is a case of a 29 year old female with persistent Cushing’s disease after repeat surgery, wishing to become pregnant. Cushing’s disease/syndrome in pregnancy is associated with significant maternal and/or fetal morbidity and mortality in approximately 70% of cases. For the mother there is an increased risk of hypertension, diabetes, osteoporosis with fracture, poor wound healing and preeclampsia. For the fetus there is a high rate of premature birth as well as increased risk of intrauterine growth restriction, miscarriage and still birth.
The treatment options during pregnancy are either surgical or medical. A review of published cases has shown that treatment is associated with a 13% increase in the rate of live births (76% to 89%). Metyrapone is the most commonly used and is generally well tolerated but has been associated with hypertension and preeclampsia. Ketoconazole has been used in 3 pregnancies with no adverse events. Somatostatin analogues and dopamine agonists are other possibilities but there is little experience or proof of efficacy.
Monitoring the efficacy and titrating the dosing of treatment in Cushing’s disease during pregnancy is complicated by the physiological hypercortisolaemia of pregnancy which can result in an up to three times elevation in urinary free cortisol.
Our case is of a 29 year old female with active Cushing’s disease who wishes to become pregnant. We review the available evidence in relation to the complications, treatment and monitoring of Cushing’s disease during pregnancy and discuss the options for optimizing maternal and fetal outcomes.