What is the evaluation and treatment approach for adrenal hyperplasia?

Evaluation and Treatment of Adrenal Hyperplasia

The evaluation of adrenal hyperplasia requires a systematic diagnostic approach including hormone testing, imaging, and in some cases adrenal vein sampling, with treatment determined by whether the condition is unilateral or bilateral, with laparoscopic adrenalectomy recommended for unilateral adenomas and medical management for bilateral hyperplasia. 1, 2

Diagnostic Evaluation

Initial Hormone Testing

• Measure plasma aldosterone and renin activity to assess for primary hyperaldosteronism; a plasma aldosterone-to-renin ratio >30 suggests primary hyperaldosteronism 1, 2
• Perform confirmatory testing with saline suppression or salt loading tests to rule out false positives 1
• Measure serum electrolytes, as excessive aldosterone production causes sodium retention and potassium excretion 1
• For suspected hypercortisolism, obtain 24-hour urine cortisol levels; elevated levels indicate Cushing syndrome 1
• Measure ACTH levels to determine if excessive cortisol secretion is ACTH-dependent (pituitary or ectopic source) or ACTH-independent (adrenal source) 1
• For bilateral adrenal hyperplasia, consider screening for congenital adrenal hyperplasia with 17-hydroxyprogesterone 1

Imaging Studies

• CT with contrast is the primary imaging modality for adrenal evaluation due to its reproducibility, resolution, and accessibility 3, 4
• MRI serves as a complementary role for further evaluation of indeterminate CT findings or in patients who cannot receive radiation 4
• Do not rely solely on CT imaging for distinguishing between unilateral adenoma and bilateral hyperplasia, as this could lead to unnecessary adrenalectomy in approximately 25% of patients 2

Specialized Testing

• Adrenal vein sampling is considered the gold standard for distinguishing between unilateral adenoma and bilateral hyperplasia in surgical candidates 1, 2
• May be reasonable to exclude adrenal vein sampling in patients younger than 40 years when imaging only shows one affected gland 1
• For suspected congenital adrenal hyperplasia, genetic testing may be appropriate to confirm the diagnosis 5

Treatment Approach

For Unilateral Adenoma

• Laparoscopic adrenalectomy is the recommended treatment for unilateral adrenal adenomas 1, 2
• Postoperative corticosteroid supplementation is required until recovery of the hypothalamus-pituitary-adrenal axis 1, 2
• Open adrenalectomy is recommended when malignant hyperaldosteronism is suspected (irregular morphology, lipid-poor, no washout on contrast-enhanced CT, >3 cm, or secreting multiple hormones) 1

For Bilateral Adrenal Hyperplasia

• Medical management with mineralocorticoid antagonists (spironolactone or eplerenone) is recommended for hypertension and hypokalemia 1, 2
• For hypercortisolism, adrenostatic agents such as ketoconazole (400-1200 mg/day) or mitotane may be used 1, 2
• Octreotide can be considered for ectopic Cushing syndrome if the tumor is Octreoscan-positive 1, 2

For Congenital Adrenal Hyperplasia

• Lifelong glucocorticoid replacement therapy is required, especially for classic phenotypes 6, 5
• Hydrocortisone is the drug of choice at 15-25 mg daily in split doses for adults 2
• Most patients with primary adrenal insufficiency should take 50-200 μg fludrocortisone as a single daily dose 2
• Regular monitoring of serum steroids (particularly 17-hydroxyprogesterone and androstenedione) is essential to assess treatment efficacy 7

Follow-up and Monitoring

• Patients with adrenal hyperplasia should be reviewed at least annually 2
• Assessment should include health and well-being, weight, blood pressure, and serum electrolytes 2
• For patients with congenital adrenal hyperplasia, regular monitoring of growth velocity and bone age in children is essential 7
• Monitor for the development of new autoimmune disorders, particularly hypothyroidism 2
• Assessment for complications of glucocorticoid therapy should include monitoring of bone mineral density every 3-5 years 2

Management of Adrenal Crisis

• Adrenal crisis should be treated immediately with IV or IM hydrocortisone, 100 mg followed by 100 mg every 6-8 hours until recovered 2
• Isotonic (0.9%) sodium chloride solution should be administered at an initial rate of 1 L/hour until hemodynamic improvement 2
• All patients with adrenal insufficiency should wear medical alert identification and carry a steroid alert card 2

Common Pitfalls and Caveats

• Relying solely on CT imaging for treatment decisions can lead to inappropriate management 2
• Failure to diagnose congenital adrenal hyperplasia can result in serious harm, as patients may present with shock, hyponatremia, and hyperkalemia 1
• Poor medication adherence can lead to inadequate disease control; education about the importance of consistent medication use is crucial 6
• Patients with the more severe forms of congenital adrenal hyperplasia tend to have higher adherence rates and better endocrine knowledge 6