Cushing syndrome in pregnancy secondary to adrenal adenoma
A 38-year-old multigravida (G3P0) woman was referred to our institution at 27 week 1 day weeks of pregnancy for evaluation of borderline blood pressure (range, 135/70-158/80 mm Hg) and generalized edema which developed 1 week prior. At her first visit to our institution, she complained excessive weight gain of over 5 kg in the last week and irregular uterine contractions. Her blood pressure and body mass index were 133/75 mm Hg and 31.2 kg/m2, respectively. A urinary dipstick test revealed 1+ proteinuria. In her past medical history, conization was performed for cervical carcinoma in situ 5 years ago. In her prenatal exams, the result of amniocentesis showed 46, XX and other obstetrical laboratory tests were normal except 50 g oral glucose screening test (145 mg/dL) which was confirmed to be normal by 100 g oral glucose tolerance test. Ultrasonography showed a single fetus with 1,107 g of estimated weight in cephalic presentation, decreased amniotic fluid volume with amniotic fluid index (AFI) 5 cm and shortened cervical length of 1.95 cm. The fetus showed reactive fetal heart rate pattern on non-stress test. She was admitted for evaluation under the suspicion of preeclampsia and preterm labor with short cervical length.\nAfter hospitalization, a diagnostic workup for preeclampsia was performed and the results were as follows. Complete blood cell count was normal and other laboratory tests including serum electrolyte, renal function test, liver enzyme and coagulation were all within normal range except potassium level (2.7 mmol/L). Initial 24-hour urine protein collection was 189.1 mg/day and repeated urine collection revealed 207.55 mg/day. Namely, the results were not consistent with preeclampsia. In addition, she had Cushingoid characteristics-moon face, central obesity, buffalo hump, abdominal and lower limb striae and hypokalemia. On the basis of physical findings and hypokalemia, we performed diagnostic tests for CS including serum cortisol, 24-hour urinary cortisol excretion, plasma adrenocorticotropic hormone (ACTH), and an overnight 1mg dexamethasone suppression test. Serum basal cortisol level was 27.3 µg/dL (normal in third trimester 1.8-26.0 µg/dL) and 24-hour urinary free cortisol excretion level was 1,900 µg/24 hr (normal in third trimester < 20.16 µg/dL []), and plasma ACTH levels were 3.8 pg/mL (normal range: 0.0-46 pg/mL). As expected, the serum cortisol (23.5 µg/dL) was not suppressed after 1 mg overnight dexamethasone suppression.\nAlthough, there was no definite criterion for interpreting a hormonal study for CS in pregnancy, the diagnostic evaluation revealed remarkable increase of urinary free cortisol. Therefore, magnetic resonance image (MRI) of adrenal gland and sellar were performed at 28 week 4 day. Adrenal gland MRI revealed a 2.5 cm × 2.0 cm × 2.5 cm tumor in the right adrenal gland (). Cranial MRI scanning showed no abnormal finding.\nConsidering the gestational age (29 week 1 day, the risk of prematurity) of the fetus and the opinion of the endocrine surgeon, immediate adrenalectomy might be helpful to the patient rather than the delay of operation; we decided to perform direct surgical treatment instead of medical treatment with metyrapone. At 29 week 1 day, unilateral adrenalectomy was done under laparotomy using laparoscopic devices such as Harmonic scalpel and graspers to minimize uterine manipulation. Histopathologic examination confirmed a benign cortical adenoma (). After surgery, there were no significant uterine contractions and tocolytic agent was not used. Glucocortioid was administered for 7 days to avoid adrenal crisis as follow. At the day of surgery and the day after surgery, 100 mg of hydrocortisone was administered intravenously three times in a day. Thereafter same dose of hydrocortisone was injected intravenously twice in a day for two days and the dose of hydrocortisone was reduced to 50 mg twice in a day. After that, we stopped intravenous steroid injection and administered 10 mg of oral prednisolone to her twice in a day. Her blood pressure started to decrease after surgery and she was discharged on seventh day after operation. She maintained her medication for seven days more after discharge.\nTwo weeks following surgery, her blood pressure had returned to normal and amniotic fluid volume also returned to normal AFI 15 cm on ultrasonography. The 24 hour urinary cortisol level decreased from 1,900 µg to 113 µg. ACTH stimulation test evaluating her remnant adrenal function suggested mild adrenal gland dysfunction; therefore, oral prednisolone therapy (2.5 mg in a day) was maintained. At 38 week 4 day weeks of gestation, premature rupture of membranes developed and she vaginally delivered a 2,450 g female infant with Apgar scores of 9 at 1 minute and 9 at 5 minutes. The level of cortisol in cord blood at birth was normal.