A successfull case of pregnancy in a woman with ACTH-independent Cushing's syndrome
Agnieszka Jagodzinska, Renata Michalak & Wojciech Zieleniewski
The most typical signs of Cushings syndrome (CS) are easy bruising, hypertension and cushingoid phenotype with central deposits of fat tissue. Moreover, hypercortisolemia may affect also reproductive system leading to loss of libido and impotence in men and menstrual disturbances and infertility in women.
However, some of the patients remains fertile, although pregnancy is uncommon and is associated with fetal morbidity and mortality in about 70% of cases. Most common are premature births, stillbirths and spontaneous abortions.
Here, we present a case of 31-year old woman with easy bruising, hypertension, oligomenorrhea and muscle weekness. Hormonal results performed in January 2008 showed undetectable ACTH concentration and stiff diurnal rhythm of cortisol without suppression after 1 and 8 mg of dexamethasone what confirmed ACTH-independent CS. Moreover, elevated BhCG might suggest 35 weeks of gestation. US scan revealed a 2 cm adenoma of the left adrenal gland, confirmed by CT after parturition.
She was given metyrapone (0.5 g t.i.d.), but as this treatment was not significally efficient, ketoconazole (0.4 g twice a day) was added. The treatment was well tolerated.
She delivered a healthy boy in September 2008 by a cesarean section. A videoscopic adrenalectomy was performed later and hitopathological exam confirmed a 23 mm adenoma of the left adrenal.
Concluding, we think that medical treatment of CS in pregnant women is well-tolerated and safe both for the mother and fetus.