Addison's disease in a monochorionic diamniotic twin pregnancy – Addison disease

Abstract

Addisons disease is combined with a deficiency of cortisol and aldosterone. In women, the autoimmune form is more common and associated in up to 40% with other autoimmune conditions. Prior to the advant of steroid therapy it was associated with a high maternal mortality. Adrenal antibodies cross the placenta but neonatal adrenal insufficiency is rarely encountered. We here report on a mother who had a spontaneous monochorionic diamniotic (MCDA) twin pregnancy and Addison's disease. Morbus Addison had been diagnosed at the age of 19 years due clinical symptoms of hypotonia, nausea, fatigue and hyperpigmentation. The patient received 25mg hydrocortison and 0,2mg 9-alpha-fluorhydrocortison per day and got pregnant of MCDA twins. Hydrocortison was increased to 50mg/day considering the increased plasam volume in multiple pregnancy and clinical symptoms of nausea. The pregnancy was uneventful until 31 weeks when she was admitted with PPROM. The patient received oxytocin-antagonists during 48 hours and betamethasone. Due to progression of labor she received peridural anaesthesia and parental application of 100mg hydrocortison/6hours. With fully dilatation and both twins in vertex position the first twin developed variable decelerations and a Cesarean section was performed. Both boys had a normal outcome and did not need steroid supplementation (normal cortisol levels) nor ventilation. The mother presented with hypotension and diuresis postnatally but recovered rapidly. When interpreting results of cortisol in pregnancy one should consider that total serum and free cortisol levels are increased and an abnormally low cortisol level may fall within the normal non-pregnant range.