What is the immediate treatment for bilateral adrenal hyperplasia?

Immediate Treatment for Bilateral Adrenal Hyperplasia

Medical management with mineralocorticoid receptor antagonists such as spironolactone or eplerenone is the immediate treatment of choice for bilateral adrenal hyperplasia, particularly when it causes hyperaldosteronism. 1 This approach addresses the hormonal imbalances while avoiding the risks associated with bilateral adrenalectomy.

Diagnostic Confirmation

Before initiating treatment, it's crucial to confirm the type of hormonal excess:

1. For hyperaldosteronism:

   • Measure plasma aldosterone and renin activity (aldosterone-to-renin ratio >30 suggests primary hyperaldosteronism)
   • Check electrolytes (look for hypokalemia and sodium retention)
   • Confirm with saline suppression test or salt loading test 1
   • Perform adrenal vein sampling to distinguish bilateral hyperplasia from adenoma 1

2. For hypercortisolism (Cushing syndrome):

   • Perform 1mg dexamethasone suppression test
   • Measure 24-hour urinary cortisol
   • Check ACTH levels (low/undetectable ACTH suggests adrenal source) 1
   • Consider screening for congenital adrenal hyperplasia with 17-hydroxyprogesterone in bilateral cases 1

Treatment Algorithm

For Hyperaldosteronism due to Bilateral Adrenal Hyperplasia:

1. First-line treatment: Medical management with mineralocorticoid receptor antagonists

   • Spironolactone 25-100 mg daily, titrated based on response 1, 2
   • Eplerenone as an alternative if spironolactone is not tolerated 1

2. Monitoring:

   • Blood pressure
   • Serum potassium levels
   • Renal function

For Hypercortisolism (Cushing Syndrome) due to Bilateral Adrenal Hyperplasia:

1. If ACTH-independent with symmetric cortisol production:

   • Medical management with adrenostatic agents 1
   • Ketoconazole (400-1200 mg/day) is commonly used due to relatively tolerable toxicity profile
   • Mitotane can be effective for controlling cortisol excess 3

2. If ACTH-independent with asymmetric cortisol production:

   • Consider unilateral adrenalectomy of the most active side 1
   • Postoperative corticosteroid supplementation until HPA axis recovery

3. If refractory to medical management:

   • Consider bilateral adrenalectomy (laparoscopic approach when feasible) 1, 4
   • For non-surgical candidates, CT-guided percutaneous microwave ablation may be considered 5

Special Considerations

• Avoid bilateral adrenalectomy for asymptomatic cortisol-secreting adenomas due to risk of adrenal insufficiency 1
• Postoperative corticosteroid supplementation is required after adrenalectomy until HPA axis recovery 1
• Annual hormonal testing is recommended for 4-5 years, particularly for tumors >3 cm 1

Common Pitfalls

1. Misdiagnosis: Bilateral adrenal hyperplasia can be confused with bilateral adenomas. Adrenal vein sampling is crucial for correct diagnosis, especially in hyperaldosteronism 1, 6

2. Inadequate follow-up: Even with medical management, patients require ongoing monitoring for:

   • Hormonal control
   • Medication side effects
   • Development of comorbidities (hypertension, diabetes, osteoporosis)

3. Surgical risks: Bilateral adrenalectomy leads to permanent adrenal insufficiency requiring lifelong steroid replacement. This should be reserved for cases refractory to medical management 1

4. Incomplete evaluation: Always rule out ectopic sources of ACTH when evaluating bilateral adrenal hyperplasia, as these may require different treatment approaches 1

By following this structured approach, the immediate treatment can address the hormonal excess while minimizing risks and complications associated with more invasive interventions.