Endocrine Abstracts

Introduction: Cushing’s disease (CD) frequently leads to hypogonadotropic hypogonadism by hypercortisolism and hyperandrogenemia. In the literature, there are totally 96 cases of CD and pregnancy. Only four of 11 cases, operated transsphenoidally, were cured. We aimed to present a case of CD developing pregnancy in which hypercortisolism was successfully controlled by transsphenoidal surgery (TSS) in 2nd trimester.

Case report: 28 years old female was admitted with complaints of not able to lose weight after delivery and hirsutism. She had diagnosis of ALL 9 years ago and two operations because of avascular femoral necrosis. Physical examination revealed supraclavicular fat deposition, purple stria on abdomen, ecchymotic lesions on skin,diffuse hyerpigmentation on back and hirsutism. BMI was 33 kg/m². Laboratory investigations were compatible with ACTH dependent hypercortisolism. Sella MRI showed pituitary adenoma 12 mm in diameter in left side. She was diagnosed as CD, referred to neurosurgery. While waiting for operation, pregnancy (11 weeks) was determined. Her pregnancy had been developed after diagnosis of CD. Urinary free cortisol(UFC) excretion was 992 μg/24 hr (N:36–192 μg/24 hr). In the 2nd trimester, TSS was performed at 18th week of gestation and ACTH secreting pituitary adenoma with Ki67 2% was confirmed. As her postoperative 3rd day serum cortisol was 23 μg/dl, no need for steroid replacement was found. Postoperative UFC on first month was 152 μg/24 hr. At 6th week postoperatively, she was admitted with hypotension and nausea. Basal serum cortisol was 6.34 μg/dl. But enough increment was determined at 250 μg ACTH stimulation test (peak 33 μg/dl). Due to her low basal serum cortisol inspite of pregnancy, symptoms compatible with adrenal failure and only 6 weeks past from TSS, steroid replacement therapy was started for secondary adrenal failure with hydrocortisone 15 mg/day. Her complaints regressed after therapy. At 31st week of gestation, she gave birth to a baby by C/S. There were no complications for mother in peripartum period. The infant was treated for congenital pneumonia and jaundice in neonatal intensive care unit. Steroid replacement was stopped as hypothalamo-pituitary-adrenal axis was found to be normal with 250 μg ACTH stimulation test (peak cortisol: 23.31 μg/dl). On postpartum 2nd month, she was totally fine with BMI 26,17 kg/m², basal serum cortisol: 6.63 μg/dl, 1 mg DST: 0.56 μg/dl, UFC: 60.8 μg/24 hr.

Discussion: Pregnancy is rarely encountered during CD. Hypercortisolism is associated with increased maternal and fetal morbidity and mortality. UFC is the preferred test for diagnosis and follow-up during pregnancy. Management of patients should be performed at experienced centers and TSS should be considered as priority.