Post-Operative Management After Adrenalectomy for Cushing Syndrome Due to Adrenal Adenoma
The correct answer is C: Give stress dose steroid, then gradually taper down. This patient requires immediate stress-dose glucocorticoid coverage followed by gradual tapering because the contralateral adrenal gland has been chronically suppressed by autonomous cortisol production from the adenoma and cannot mount an adequate stress response immediately after surgery 1.
Immediate Perioperative Management
Stress-dose hydrocortisone is mandatory at surgery and immediately postoperatively because removal of a cortisol-secreting adrenal adenoma results in tertiary adrenal insufficiency—the hypothalamic-pituitary-adrenal (HPA) axis has been suppressed by chronic hypercortisolism 1, 2.
- For pediatric patients, hydrocortisone 2 mg/kg should be given at induction followed by continuous IV infusion based on weight (25-150 mg/24 hours depending on weight and pubertal status) 1.
- All patients with Cushing syndrome due to adrenal adenoma develop hypocortisolemia requiring glucocorticoid therapy for several months after adrenalectomy 3, 4.
Post-Operative Tapering Strategy
Once stable, implement a gradual taper over weeks to months:
- Double the usual oral hydrocortisone doses for 48 hours, then reduce to normal replacement doses over up to a week 1.
- Continue physiologic replacement doses (typically 15-25 mg hydrocortisone daily in divided doses) until HPA axis recovery is documented 1, 5.
- Serial morning cortisol monitoring is essential to guide steroid tapering and assess HPA axis recovery 1.
Duration of Replacement Therapy
The median time to HPA axis recovery in adrenal Cushing syndrome is 2.5 years (interquartile range 1.6-5.4 years) 5. This is significantly longer than Cushing's disease (1.4 years) or ectopic Cushing syndrome (0.6 years) 5.
- Only 38% of patients with adrenal Cushing syndrome recover adrenal function within 5 years, compared to 58% with Cushing's disease 5.
- Younger patients tend to recover faster, though the correlation with age is less pronounced in adrenal Cushing syndrome than in Cushing's disease 5.
Critical Pitfalls to Avoid
Do NOT discontinue steroids immediately (Option B is dangerous):
- Abrupt withdrawal can precipitate life-threatening adrenal crisis 1.
- No patients undergoing adrenalectomy for Cushing syndrome can safely avoid steroid replacement 4.
Do NOT commit to lifelong steroids (Option A is incorrect):
- Unlike bilateral adrenalectomy, unilateral adrenalectomy for adrenal adenoma allows eventual HPA axis recovery in most patients 1, 5.
- Lifelong surveillance is NOT required for adrenal adenoma 1.
Fludrocortisone (Option D) is NOT routinely needed:
- Mineralocorticoid replacement is only necessary after bilateral adrenalectomy 2.
- Unilateral adrenalectomy preserves the contralateral adrenal's mineralocorticoid function.
Stress-Dose Coverage During Recovery
Until HPA axis recovery is confirmed, the patient remains at risk for adrenal insufficiency during physiologic stress:
- Stress-dose coverage is required during intercurrent illness, trauma, or subsequent surgery 1.
- Patients should be educated on "sick day rules" (doubling steroid doses during illness) and carry emergency hydrocortisone 6.
- Volume-resistant hypotension is a late sign of adrenal crisis; earlier signs include malaise, orthostatic hypotension, and persistent pyrexia 6.
Monitoring Strategy
Regular endocrine follow-up during the recovery phase should assess:
- Signs of adrenal insufficiency requiring dose adjustment 1.
- Morning cortisol levels to guide tapering and eventual discontinuation 1.
- Growth velocity and bone density recovery in pediatric patients 1.
- Cardiovascular and metabolic improvements from resolution of hypercortisolism 5.
Steroid withdrawal syndrome may occur despite acceptable cortisol levels (100-750 nmol/L), presenting with symptoms mimicking adrenal insufficiency due to glucocorticoid tolerance 7. This self-limiting condition responds to temporary dose increases and typically resolves within 6-10 months 7.