Cortisol excess syndrome, also known as Cushing's syndrome, is the most common type of adrenal cortical hyperfunction. This condition results from excessive secretion of cortisol by the adrenal cortex, often accompanied by the overproduction of other corticosteroids. Pregnancy complicated by cortisol excess syndrome is extremely rare, with most cases ending in late abortion, premature labor, or stillbirth.

bubble_chart Etiology

50-70% of hypercortisolism cases are caused by adrenal cortical hyperplasia, 20-30% by adrenal adenomas or carcinomas, and 10-20% by pituitary adenomas. The condition is three times more common in women than in men, with onset typically occurring between ages 15 and 35. Adrenal cortical hyperfunction leads to amenorrhea and anovulation; approximately 30% of patients experience amenorrhea in the early stages of the disease, increasing to 50-70% as the condition progresses. This is followed by reversible ovarian atrophy, with reduced follicles at all stages leading to infertility.

bubble_chart Clinical Manifestations

The clinical manifestations of hypercortisolism are caused by excessive cortisol in the body, leading to metabolic disturbances of carbohydrates, proteins, fats, and electrolytes, as well as dysfunction of multiple organs. The main clinical features include moon face, central obesity, purple striae, acne, a tendency toward diabetes, hypertension, osteoporosis, amenorrhea, and weakness.

bubble_chart Diagnosis

During pregnancy, changes in the metabolism of glucocorticoids and mineralocorticoids occur, leading to increased production of cortisol and aldosterone as the pregnancy progresses. Therefore, diagnosing Cushing's syndrome during pregnancy can be challenging. Patients with pregnancy-associated Cushing's syndrome typically present with a moon face, purple striae on the abdomen and inner limbs, acne, weight gain, and elevated blood sugar levels. Laboratory tests may show significantly elevated 24-hour urinary 17-hydroxycorticosteroids (17-OHCS) and free cortisol levels. Adrenal ultrasound and perirenal pneumography can aid in diagnosis.

① Pregnancy complicated with hypercortisolism is prone to late abortion, dead fetus, stillbirth, and premature labor. Therefore, intensive monitoring during pregnancy and early hospitalization are necessary. ② Pregnant women often develop pregnancy-induced hypertension (PIH) in the intermediate stage [second stage] of pregnancy. Additionally, since corticosteroids have an anti-insulin effect that raises blood sugar, active control of PIH and treatment of diabetes are essential. With aggressive treatment, the pregnancy can be prolonged, increasing the chances of a live birth. ③ Metyrapone is a cortisol synthesis inhibitor, taken at 1–2 g per day in divided doses, which can be increased to 4–6 g per day. Metyrapone has few adverse effects and can reduce blood cortisol levels, alleviating symptoms. ④ Surgical treatment. For cases of adrenal hyperfunction caused by adrenal cortical tumors during pregnancy, surgery may be considered after confirmation. Bevan believes that surgery during pregnancy is safe and can significantly reduce the incidence of dead fetus and premature labor.