Further Outpatient Care
Follow up to monitor adequacy and adverse effects of adrenal replacement therapy.
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Glucocorticoid adverse effects include iatrogenic Cushing syndrome, linear growth retardation, and hypothalamic-pituitary axis suppression.
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Mineralocorticoid adverse effects include edema, hypertension, hypokalemia, and alkalosis.
Follow-up evaluation with the short cosyntropin (Cortrosyn) stimulation test is used to assess recovery of adrenal function. This test can be performed 24 hours after the last dose of hydrocortisone or prednisone replacement.
Further Inpatient Care
If acute adrenal hemorrhage is considered likely, admit the patient to the medical or surgical intensive care unit (MICU or SICU) as indicated for treatment, including glucocorticoid replacement, supportive therapy, and treatment of the underlying disease(s).
Admit patients for elective adrenalectomy for suspected primary adrenal tumor.
Inpatient & Outpatient Medications
Treatments using medications include glucocorticoid and mineralocorticoid replacement therapies. Glucocorticoid therapy should be provided routinely in the acute stage of extensive, bilateral adrenal hemorrhage without awaiting biochemical confirmation of adrenal insufficiency.
In the acute setting, supportive therapy with mechanical ventilation, intravenous fluid administration, and pressor therapy may be necessary to provide vital function support.
Specific therapy for any underlying disease(s) must be provided acutely, including antibiotics for sepsis. The use of heparin and fresh frozen plasma has been advocated in DIC cases but remains controversial.
Patients with chronic adrenal insufficiency must temporarily increase their glucocorticoid replacement dose (2-3 times above baseline for as long as 3-5 d) in case of minor acute illness or injury. This is known as using sick-day rules.
At the time of acute illness or major surgery, patients with chronic adrenal insufficiency should receive hydrocortisone (50-100 mg IV tid) with rapid tapering to maintenance, as the general condition permits.
In the outpatient setting, the need for continued glucocorticoid and mineralocorticoid replacement should be reassessed periodically by adrenal function testing, as previously outlined.
In women with adrenal insufficiency, androgen replacement therapy, including dehydroepiandrosterone (DHEA; 25-50 mg PO qd), improves libido. [28] Although available in over-the-counter preparations, DHEA has not, at the time of this writing, been approved by the Food and Drug Administration for use in women with adrenal insufficiency.
Transfer
Transfer may be indicated for further diagnostic testing or surgery, although it is not practical in unstable patients.
Deterrence/Prevention
Avoid volume depletion and salt restriction in the presence of chronic adrenal insufficiency, unless these are required for the treatment of coexisting conditions, such as congestive heart failure.
Complications
Acute adrenal insufficiency (adrenal crisis) may occur only in cases of extensive, bilateral adrenal hemorrhage. The proportion of patients with bilateral adrenal hemorrhage who develop acute adrenal insufficiency is unknown and remains controversial.
Extensive retroperitoneal hemorrhage secondary to adrenal hemorrhage is very uncommon, although it has been reported.
Chronic adrenal insufficiency may occur and previously was thought to be permanent. Reports of recovery of adrenal function in patients with documented adrenal insufficiency associated with an episode of extensive, bilateral adrenal hemorrhage suggest that periodic adrenal function testing of these patients is indicated.
Prognosis
The acute case fatality rate associated with extensive, bilateral adrenal hemorrhage is approximately 15% and varies according to the severity of underlying illness.
In Waterhouse-Friderichsen syndrome, the case fatality rate is 55-60%, particularly when the diagnosis is delayed. Death occurs from sepsis, despite appropriate antibiotic, glucocorticoid, and supportive treatment.
Recovery of adrenal function in patients with chronic adrenal insufficiency associated with adrenal hemorrhage is possible, although it appears to occur infrequently. [29, 16]
A retrospective study by Monticone et al found that in cases of adrenal hemorrhage resulting from adrenal vein sampling, used to determine whether primary aldosteronism is unilateral or bilateral, patients tended to have good outcomes, with little or no effect on adrenal function. The study included 24 patients. [30]
Patient Education
Patients with chronic adrenal insufficiency must wear an appropriate identification tag or bracelet.
These patients must increase (double or triple) their dose of glucocorticoid replacement with minor illness or injury, according to sick-day rules.
If patients with chronic adrenal insufficiency are unable to keep liquids and medications down, or if they suffer a major illness or injury, they must self-inject with hydrocortisone 100 mg intramuscularly and promptly seek medical attention.
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Computed tomographic (CT) scans of the abdomen show normal adrenal glands several months before the onset of hemorrhage (upper panel) and enlarged adrenals 2 weeks after an acute episode of bilateral adrenal hemorrhage (lower panel). The attenuation of the adrenal glands, indicated by arrows, is increased after the acute event. Reproduced from Rao RH, Vagnucci AH, Amico JA: Bilateral massive adrenal hemorrhage: early recognition and treatment. Ann Intern Med. Feb 1 1989;110(3):227-35 with permission from the journal.
