Post-Operative Management After Adrenalectomy for Adrenal Adenoma in Pediatric Cushing Syndrome
The correct answer is B: Stress dosage then taper steroid. After unilateral adrenalectomy for an adrenal adenoma causing Cushing syndrome, the child will require stress-dose glucocorticoid coverage perioperatively followed by a gradual taper, NOT lifelong replacement, as the contralateral adrenal gland will eventually recover function.
Immediate Perioperative Steroid Coverage
Major surgery in children requires hydrocortisone 2 mg/kg at induction followed by continuous IV infusion based on weight: 1
- Up to 10 kg: 25 mg/24 hours
- 11-20 kg: 50 mg/24 hours
- Over 20 kg prepubertal: 100 mg/24 hours
- Over 20 kg pubertal: 150 mg/24 hours
This stress-dose coverage is critical because the contralateral adrenal gland has been chronically suppressed by the autonomous cortisol production from the adenoma and cannot mount an adequate stress response immediately after surgery. 1
Post-Operative Steroid Replacement Strategy
Once stable, the child should receive double the usual oral hydrocortisone doses for 48 hours, then reduce to normal replacement doses over up to a week. 1
The key physiologic principle is that removal of a cortisol-secreting adrenal adenoma results in tertiary adrenal insufficiency because the hypothalamic-pituitary-adrenal (HPA) axis has been suppressed by chronic hypercortisolism. 2 However, this is temporary and reversible in adrenal Cushing syndrome, distinguishing it from bilateral adrenalectomy which requires lifelong replacement. 3
Expected Timeline for HPA Axis Recovery
Recovery of adrenal function after adrenalectomy for adrenal adenoma is etiology-dependent and typically prolonged: 2
- Median time to recovery in adrenal Cushing syndrome: 2.5 years (interquartile range 1.6-5.4 years) 2
- Probability of recovery within 5 years: 38% in one cohort, though many eventually recover with longer follow-up 2
- Some patients require glucocorticoid replacement for 1-4 years post-adrenalectomy 4
This is significantly longer than recovery after pituitary surgery for Cushing disease (median 1.4 years) or ectopic ACTH (median 0.6 years), likely due to more profound and prolonged HPA axis suppression from the autonomous adrenal cortisol production. 2
Monitoring and Dose Adjustment
Serial cortisol monitoring is essential to guide steroid tapering and assess HPA axis recovery: 5
- Measure morning cortisol levels regularly during follow-up
- Perform ACTH stimulation testing to assess adrenal reserve when considering discontinuation of replacement
- Postoperative cortisol levels <3 μg/dL (83 nmol/L) within 6-8 hours confirm the need for replacement therapy 5
The steroid replacement dose should be gradually tapered based on clinical status and biochemical recovery, with the goal of eventual discontinuation once the contralateral adrenal recovers. 6, 2
Critical Pitfalls to Avoid
Do not assume lifelong replacement is needed - this is the management for bilateral adrenalectomy, not unilateral adenoma removal. The contralateral adrenal will recover, though it may take months to years. 4, 2
Do not discontinue steroids too rapidly - abrupt withdrawal can precipitate adrenal crisis. The taper must be gradual and guided by biochemical monitoring. 1
Do not forget stress-dose coverage during intercurrent illness - until HPA axis recovery is confirmed, the child remains at risk for adrenal insufficiency during physiologic stress and requires stress-dose glucocorticoid coverage. 1
Long-Term Follow-Up
Lifelong surveillance is NOT required for adrenal adenoma (unlike Cushing disease where recurrence can occur up to 15 years later). 1 However, regular endocrine follow-up is needed during the recovery phase to: