he typical cushingoid body habitus is caused by adipose tissue deposition that characteristically results in moon acies, a builo hump, and truncal obesiy. Fatigability and weakness, hypertension, hirsutism, and amenorrhea are each encountered in 75 to 85 percent of nonpregnant patients (Hatipoglu, 2012). Personality changes, easy bruisability, and cutaneous striae are common. Up to 60 percent may have impaired glucose tolerance. Diagnosis can be diicult and is suggested by elevated plasma cortisol levels that cannot be suppressed by dexamethasone or by elevated 24-hour urine free cortisol excretion (Arit, 2015; Loriaux, 2017). Neither test is totally accurate, and each is more diicult to interpret in obese patients. Serum corticotropin levels and CT and MR imaging are used to localize pituitary and adrenal tumors or hyperplasia.
Because most women have corticotropin-dependent Cushing syndrome, associated androgen excess may cause anovulation,
TABLE 58-7. Maternal and Perinatal Complications
Data from Lindsay, 2005.
and pregnancy is rare. In their review, Lekarev and New (2011) identiied fewer than 140 reported cases of Cushing syndrome in pregnancy. These difer compared with nonpregnant women in that half are caused by corticotropin-independent adrenal adenomas (Kamoun, 2014; Lacroix, 2015). Approximately 30 percent of cases are from a pituitary adenoma, and 10 percent from adrenal carcinomas. All reports stress diiculties in diagnosis because of pregnancy-induced increases in plasma cortisol, corticotropin, and corticotropin-releasing factor levels. Measurement of24-hour urinary free cortisol excretion is recommended, with consideration for the normal elevation seen in pregnancy.
Pregnancy outcomes in women with Cushing syndrome are listed in Table 58-7. Heart failure is common during pregnancy and is a major cause of maternal mortality (Buescher, 1992). Hypercortisolism in pregnancy may also cause poor wound healing, osteoporotic fracture, and psychiatric complications (Kamoun, 2014).
Long-term medical therapy for Cushing syndrome usually is inefective, and deinitive therapy is resection of the pituitary or adrenal adenoma or bilateral adrenalectomy for hyperplasia (Lacroix, 2015; Motivala, 2011). During pregnancy, management of hypertension in mild cases may suice until delivery. In their review, Lindsay and associates (2005) described primary medical therapy in 20 women with Cushing syndrome. Most were successfully treated with meyrapone as an interim treatment until deinitive surgery after delivery. A few cases were treated with oral ketoconazole. However, because this drug also blocks testicular steroidogenesis, treatment during pregnancy with a male fetus is worrisome. Miepristone, the norethindrone derivative used for abortion and labor induction, has shown promise for treating Cushing disease but should not be used in pregnancy for obvious reasons. If necessary, pituitary adenomas can be treated by transsphenoidal resection (Boscaro, 2001; Lindsay, 2005). Unilateral adrenalectomy has been safely performed in the early third trimester and can also be curative (Abdelmannan, 2011).