7907 - Cushing's syndrome

DBQ: Link to Index of DBQ/Exams by Disability for DC 7907 

Definition

Cushing's syndrome is a set of symptoms occurring together resulting from hypersecretion of adrenocorticotropin (ACTH) from the cortex of the adrenal gland in which there is excessive production of glucocorticoids. This syndrome pertains to a wide range of abnormalities caused by excess corticosteroids, especially glucocorticoids.

Etiology

Causes of the condition include: tumor of the adrenal gland, prolonged administration of high doses of corticosteroids, excess secretion of the pituitary hormone, adrenocorticotropin (ACTH) from carcinoma of the lung, and excessive function of the anterior pituitary.

Signs & Symptoms

General symptoms of Cushing's syndrome include: rounded, reddish face (moon face), obesity of the body trunk, a buffalo hump, and hypertension. Other specific deviations related to the syndrome include:

  • Skin manifested by increased pigmentation, easy bruising, acne, edema, increased body and facial hair, reddish purple lines on the abdomen (striae), and poor wound healing.

  • Musculoskeletal manifested by osteoporosis and muscle weakness.

  • Neuropsychiatric manifested by depression, irritability and psychosis.

  • Metabolic manifested by low potassium level, increased calcium level with kidney stone development, and glucose intolerance or diabetes mellitus.

  • Other manifested by cessation of menstruation in women.

Tests

Normally, the body has high blood levels of cortisol in the morning, which lower by nighttime. If the lowering does not occur, Cushing's syndrome may be considered as a possible cause. Tests that may be done to determine the underlying causes of Cushing's syndrome are overnight dexamethasone suppression test (DST), serum ACTH levels, and cortisol level. If the overnight DST is abnormal, then it is followed by a high dose 2-day DST test. In addition, imaging of the pituitary, venous sampling, and computed tomography (CT) scan of the abdomen may be indicated.

Treatment

The goal is to correct the malfunction of either the pituitary or the adrenal cortex. If the pituitary is the cause, then surgical removal is indicated. If no tumor can be found, then radiation is used. Tumors of the adrenal cortex require total adrenalectomy. If the tumor cannot be excised, then medications that inhibit production of adrenal hormones are indicated. In addition, treatment by irradiation has been done and, in some cases, bilateral adrenalectomy.

Residuals

When bilateral adrenalectomy is done due to continued high cortisol levels, there is an associated morbidity and mortality rate of 5%. A possible aftermath of this surgery is the development of Nelson's syndrome where the pituitary gland continues to expand. If this occurs, it then becomes necessary to remove the pituitary gland (hypophysectomy) and administer radiation. Also, a permanent Addisonian state (caused by decreased adrenocortical hormone) results. (See Diagnostic Code: 7911 Addison's disease.)

Special Considerations

  • Consider service connection on a presumptive basis as a chronic condition (38 CFR §3.307§3.309 (a)).  

Notes

  • The evaluations specifically indicated under this diagnostic code shall continue for six months following initial diagnosis. After six months, rate on residuals under the appropriate diagnostic code(s) within the appropriate body system(s).