The estimated prevalence is  at 1/26,000.

This term includes all the clinical manifestations produced by chronic exposure to excess endogenous glucocorticoids. 

The causes are grouped into 2 categories:

• ACTH dependent Cushing syndrome (about 85 % of cases): adrenals are stimulated by an excessive and inappropriate secretion of ACTH. In about 80-85 % of cases, ACTH is secreted by a benign tumor developed from hypophyseal corticotropic cells è Cushing's disease. In the remaining 15 % of cases, the ACTH comes from an ectopic origin and  is thus produced by a non-pituitary endocrine tumor ( è paraneoplastic Cushing's syndrome). An ectopic secretion of corticoliberine (CRH) is very rarely associated. 
• ACTH independent Cushing syndrome (about 15 % of cases): adrenal secretion is autonomous, independent of ACTH. In 2/3 of cases, it is a benign unilateral adrenal tumor (corticoadrenal adenoma). In about 1/3 of cases, it is a malignant tumor (corticoadrenal cancer). In about 1 % of cases, it is a primitive bilateral involvement in connection with a macronodular hyperplasia (AIMAH, ACTH Independent Macronodular Adrenal Hyperplasia) or a pigmented micronodular adrenal dysplasia   (PPNAD, Primary Pigmented Nodular Adrenal Disease) (see Carney complex).

Symptoms:

Mostly: truncal obesity, high blood pressure, glycoregulation disorders, mood disorders. 

Suggestive signs: 

-        skin: fragility, bruises, stretch marks

-        muscle: proximal atrophy

-        bone: osteoporosis

-        growth retardation in childhood, especially if it is associated with paradoxical overweight

It can happen that a primary corticoadrenal tumor is incidentally discovered  (adrenal incidentaloma) in which case further search for potential biological stigmata is warranted.

Anesthetic implications: 

overweight, difficult peripheral venous access (small fragile veins), very fragile skin, osteoporosis

References : 

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Updated: April 2018