Should a patient with Cushing's disease (CD), left ventricular hypertrophy (LVH), and cirrhosis, with a partially empty sella on MRI, undergo adrenalectomy?

Management of Cushing's Disease with Partially Empty Sella, LVH, and Cirrhosis

For a patient with Cushing's disease, left ventricular hypertrophy (LVH), and cirrhosis with only a partially empty sella on MRI, bilateral adrenalectomy should be considered as a treatment option, especially if the patient has severe, refractory disease with life-threatening complications.

Diagnostic Considerations

• Partially empty sella is found in approximately 22% of patients with Cushing's disease, making it a relatively common finding that doesn't rule out the presence of a pituitary adenoma 1
• The association between Cushing's disease and empty sella is likely coincidental, as the frequency of empty sella in the general population (approximately 20%) is similar to that found in Cushing's disease patients 2
• Even with a partially empty sella, Cushing's disease is still likely ACTH-dependent, requiring confirmation through appropriate testing including ACTH levels and possibly bilateral inferior petrosal sinus sampling (BSIPSS) 3, 4

Treatment Decision Algorithm

Step 1: Assess Disease Severity and Comorbidities

• The presence of LVH and cirrhosis indicates significant end-organ damage from hypercortisolism, suggesting a need for rapid cortisol normalization 3, 5
• These complications significantly increase morbidity and mortality risk, making prompt intervention crucial 4

Step 2: Consider Primary Treatment Options

• First-line treatment for Cushing's disease is typically transsphenoidal surgery to remove the pituitary adenoma 4
• However, when no clear adenoma is visible on MRI (as with partially empty sella), surgical success rates may be lower 2

Step 3: Evaluate Risk-Benefit for This Specific Case

• For this specific patient with both LVH and cirrhosis:
  • Bilateral adrenalectomy should be strongly considered due to:
    • The presence of two severe complications (LVH and cirrhosis) 3
    • The need for rapid control of hypercortisolism to prevent further cardiac and hepatic deterioration 4
    • The absence of a clearly visible adenoma on MRI, which may reduce the likelihood of successful transsphenoidal surgery 2

Treatment Recommendations

Bilateral Adrenalectomy

• Bilateral adrenalectomy is recommended for severe, refractory Cushing's disease or for life-threatening emergencies 3
• This approach provides immediate and definitive control of hypercortisolism, which is critical given the patient's LVH and cirrhosis 3, 4
• The procedure can be performed laparoscopically in most cases 3

Important Considerations with Adrenalectomy

• Lifelong glucocorticoid and mineralocorticoid replacement will be necessary following bilateral adrenalectomy 3
• There is risk of Nelson syndrome (continued growth of the pituitary tumor after adrenalectomy), which appears more common in younger patients 3
• Regular MRI monitoring of the pituitary will be required post-adrenalectomy 3

Alternative Approaches

• Medical management with ketoconazole (400-1200 mg/day) could be considered as a temporizing measure before surgery or if surgery is contraindicated 3, 6
• Some patients with empty sella and Cushing's disease have been successfully managed long-term with ketoconazole, though this is not ideal given the severity of complications in this case 6

Post-Treatment Monitoring

• Close monitoring for adrenal insufficiency is essential following adrenalectomy 3
• Regular assessment of cardiac function to monitor LVH regression 5
• Liver function monitoring to assess cirrhosis progression 3
• Regular pituitary imaging to monitor for Nelson syndrome 3

Conclusion

Given the severity of the patient's condition with both LVH and cirrhosis, and the partially empty sella finding on MRI, bilateral adrenalectomy represents a definitive treatment option that would provide immediate control of hypercortisolism and potentially reverse some of the life-threatening complications 3, 4.