The medical management of Cushing's syndrome during pregnancy

Abstract

Cushing's syndrome during pregnancy is a rare metabolic condition that is associated with high maternal and foetal morbidity. Clinical symptoms may mimic those of normal pregnancy. A diagnosis is best made based on clinical presentation, laboratory and imaging findings as well as a high index of suspicion. Medical management with anti-steroidogenic agents such as metyrapone has been shown to be effective, but surgery is usually the recommended treatment option. Its main limitation is optimal timing of the procedure in late first trimester or early second trimester to prevent spontaneous termination of pregnancy. We describe our experience and management of a 39-year-old patient with uncontrolled hypertension at 25 weeks gestation which was later diagnosed as ACTH independent Cushing's syndrome and had a favourable pregnancy outcome. The role of medical therapy and its challenges, as well as its impact on pregnancy outcomes, were explored by a literature search conducted through Pubmed and Medline databases. A total of 12 patients with Cushing's syndrome during pregnancy were reported to have been managed with metyrapone, with ketoconazole being studied to a significant degree in three cases. Of these women, 53% delivered close to term and 20% developed pre-eclampsia. Despite two neonatal deaths and one stillborn reported, medical management appeared effective in controlling hypercortisolemia during pregnancy with strict monitoring of blood pressure and foetal surveillance. It remains the only active management in the setting of pregnancy-induced Cushing's syndrome, and has shown to be a viable option in controlling serum cortisol levels especially as an adjunct to surgery as reflected in four cases. A multidisciplinary approach towards an individualised management process is warranted with medical management to ensure a safe maternal and foetal outcome.