Pregnancy during the course of Cushing’s syndrome: a case report and literature review

Abstract

Summary Cushing’s syndrome is an endocrine disorder that causes anovulatory infertility secondary to hypercortisolism; therefore, pregnancy rarely occurs during its course. We present the case of a 24-year-old, 16-week pregnant female with a 10-month history of unintentional weight gain, dorsal gibbus, nonpruritic comedones, hirsutism and hair loss. Initial biochemical, hormonal and ultrasound investigations revealed hypokalemia, increased nocturnal cortisolemia and a right adrenal mass. The patient had persistent high blood pressure, hyperglycemia and hypercortisolemia. She was initially treated with antihypertensive medications and insulin therapy. Endogenous Cushing’s syndrome was confirmed by an abdominal MRI that demonstrated a right adrenal adenoma. The patient underwent right laparoscopic adrenalectomy and anatomopathological examination revealed an adrenal adenoma with areas of oncocytic changes. Finally, antihypertensive medication was progressively reduced and glycemic control and hypokalemia reversal were achieved. Long-term therapy consisted of low-dose daily prednisone. During follow-up, despite favorable outcomes regarding the patient’s Cushing’s syndrome, stillbirth was confirmed at 28 weeks of pregnancy. We discuss the importance of early diagnosis and treatment of Cushing’s syndrome to prevent severe maternal and fetal complications. Learning points: Pregnancy can occur, though rarely, during the course of Cushing’s syndrome. Pregnancy is a transient physiological state of hypercortisolism and it must be differentiated from Cushing’s syndrome based on clinical manifestations and laboratory tests. The diagnosis of Cushing’s syndrome during pregnancy may be challenging, particularly in the second and third trimesters because of the changes in the maternal hypothalamic-pituitary-adrenal axis. Pregnancy during the course of Cushing’s syndrome is associated with severe maternal and fetal complications; therefore, its early diagnosis and treatment is critical.

Three Successful Pregnancies in a Patient with Recurrent Cushing’s Disease

“…The few studies regarding the safety of cabergoline in pregnancy showed no higher rate of maternal-fetal complications and it is currently an FDA pregnancy category B drug [ 18 ]. The stage of pregnancy, etiology, severity of hypercortisolism, and potential benefit of treatment, all contribute to treatment decision-making [ 2 ]. Pregnant women with mild hypercortisolism, as our patient, and/or diagnosed late in gestation may be managed expectantly, and causal treatment deferred until after delivery.…”

“…Cushing's disease (CD) is a severe endocrinopathy caused by a pituitary corticotroph adenoma that primarily affects females of childbearing age [ 1 ]. However, pregnancy rarely occurs during the course of CD due to the influence of hypercortisolism on the reproductive axis [ 2 ]. The best approach to women with active CD who become pregnant is still controversial [ 3 ].…”

Diagnosis and Treatment of High-risk Pregnant Women With Cushing Syndrome Caused by Adrenal Tumor

“…There is a very low occurrence of pregnancy complicated by CS, and only about 300 cases have been reported worldwide in the literature 3 , 4 . About 85% of women with CS had ovulatory dysfunction, leading to infertility because of feedback inhibition of the hypothalamus-pituitary-ovary axis due to secretion of large amounts of cortisol hormone 5 .…”

“…Cushing Syndrome (CS) in women patients often leads to infertility due to ovulatory dysfunction 1 , and once they get pregnant, abnormal hormonal secretion during pregnancy would in turn promote the progression of CS, causing hypertensive crisis, heart failure, respiratory failure, and other severe complications that seriously endanger the life of the patients. Misdiagnosis and delayed treatment are likely to occur because it is sometimes difficult to differentiate increased body weight and stretchmarks during pregnancy from the symptoms of CS, which poses a huge threat to the life of both mother and fetus 2 , 3 , and therefore should arouse high vigilance in clinical practice. In this article, we report two high-risk pregnant women complicated with CS who were admitted and treated in our hospital during the past four years.…”

The diagnosis and management of Cushing's syndrome in pregnancy

“…Both unilateral and bilateral adrenalectomy have been performed during pregnancy, most with reasonably good outcomes. 9,39,78,[102][103][104] Adrenal surgery is ideally recommended in the second trimester, but some advocate its use even in the third trimester. 105,106 Perinatal morbidity and mortality are improved, although premature delivery and intrauterine growth restriction (IUGR) do not appear to be influenced by unilateral adrenalectomy.…”