Internal Medicine

0521779407-C04 CUNY1086/Karliner 0 521 77940 7 June 14, 2007 20:37

438 Cushing’s Syndrome

management

General Measures

■Control diabetes, hypertension, hypokalemia, increased cardiovas-

cular risk

■Patients immunosuppressed; vigilance for opportunistic, unusual

infections

What to Do First

■Confirm the diagnosis of CS

■Distinguish between ACTH-dependent and independent

■Localize source of ACTH or cortisol excess biochemically, then by

imaging

specific therapy

Surgical Treatment

■Pituitary: transsphenoidal adenomectomy or hemihypophysectomy

■Adrenal adenoma/carcinoma: abdominal, flank, or laparoscopic

surgery

■Ectopic: surgical or chemotherapy as indicated by primary disease

■Bilateral adrenalectomy always curative; reserved for ACTH-

dependent CS that failed primary surgical and medical therapies;

lifelong glucocorticoid and mineralocorticoid replacement

■Radiotherapy

➣Pituitary irradiation for nonresectable adenoma or after bilateral

adrenalectomy in CD

■Medical Therapy

➣Enzymatic inhibitors of cortisol synthesis (ketoconazole,

metyrapone, aminoglutethimide)

➣Hydrocortisone replacement if steroidogenic blockade complete

➣Mineralocorticoid excess: mineralocorticoid receptor blockers

(spironolactone)

➣Adrenal carcinoma: adrenolytic therapy (mitotane)

follow-up

■Patients feel worse after cure of CS due to adrenal insufficiency or

corticosteroid withdrawal

■Early Postoperative:

➣Early drop in serum cortisol predictive of successful cure; use-

ful if exogenous hydrocortisone is withheld in hemodynamically

stable patient post-operative

➣If patient is hemodynamically unstable, give high-dose hydro-

cortisone immediately postoperative, then taper