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 for hypertension and hypokalemia. 1

Diagnosis Confirmation and Initial Assessment

Before initiating treatment, confirm the type of hyperplasia and hormone production:

• Measure plasma aldosterone and renin activity
  • Primary hyperaldosteronism shows elevated aldosterone and low renin activity
  • Aldosterone-to-renin ratio >30 suggests primary hyperaldosteronism 1
• Check electrolytes (sodium and potassium)
• Consider confirmatory testing with saline suppression or salt loading test 1
• For bilateral adrenal hyperplasia, adrenal vein sampling is recommended to confirm bilateral hormone production 1
• Screen for congenital adrenal hyperplasia with 17-hydroxyprogesterone 1

Treatment Algorithm Based on Hormone Production

For Aldosterone-Producing Bilateral Adrenal Hyperplasia:

1. First-line treatment: Mineralocorticoid receptor antagonists 1, 2

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

2. Monitor:

   • Blood pressure
   • Serum potassium levels
   • Renal function

For Cortisol-Producing Bilateral Adrenal Hyperplasia:

1. Medical management options:

   • Adrenostatic agents like ketoconazole (400-1200 mg/day) 1
   • Mitotane for cases refractory to other treatments 3

2. Surgical options (if medical management fails):

   • Bilateral laparoscopic adrenalectomy 4
   • CT-guided percutaneous microwave ablation (for non-surgical candidates) 5

3. Monitor:

   • Cortisol levels
   • Symptoms of Cushing syndrome
   • Comorbidities (hypertension, diabetes, osteoporosis)

Special Considerations

• For asymmetric hormone production confirmed by adrenal vein sampling, unilateral adrenalectomy of the most active side may be considered 1
• For symmetric hormone production, medical management is preferred 1
• Postoperative corticosteroid supplementation is required after bilateral adrenalectomy until recovery of the hypothalamus-pituitary-adrenal axis 1

Monitoring and Follow-up

• Annual hormonal testing for 4-5 years, especially for tumors >3 cm 1
• Regular monitoring of blood pressure, electrolytes, and glucose levels
• Screening for complications of hormone excess (osteoporosis, cardiovascular disease)

Pitfalls to Avoid

• Don't assume all bilateral adrenal hyperplasia is the same - differentiate between aldosterone and cortisol-producing variants
• Don't rush to surgery without confirming bilateral disease through adrenal vein sampling
• Don't overlook the need for long-term medical therapy and monitoring
• Avoid bilateral adrenalectomy for asymptomatic cortisol-secreting adenomas due to risk of adrenal insufficiency 1
• Be cautious with ketoconazole due to potential hepatotoxicity - monitor liver function

The treatment approach should prioritize control of hormone excess to reduce morbidity and mortality from complications like hypertension, hypokalemia, and metabolic abnormalities, while preserving adrenal function when possible.