Post-Operative Management for Pediatric Adrenal Adenoma
The correct answer is B: Stress dosage then taper steroid. After adenectomy for adrenal adenoma causing Cushing syndrome in a child, the patient requires stress-dose glucocorticoid coverage followed by gradual tapering, NOT lifelong replacement, because the HPA axis suppression is temporary and reversible in adrenal Cushing syndrome 1.
Pathophysiology Justifying This Approach
The autonomous cortisol production from the adrenal adenoma chronically suppresses the contralateral adrenal gland and the entire HPA axis 1. After tumor removal, the patient develops tertiary adrenal insufficiency, which is temporary and reversible—unlike the permanent adrenal insufficiency following bilateral adrenalectomy 1, 2.
Immediate Perioperative Management
Intraoperative Coverage
- Administer hydrocortisone 2 mg/kg at anesthesia induction 1
- Follow with continuous IV infusion based on weight:
- Up to 10 kg: 25 mg/24 hours
- 11-20 kg: 50 mg/24 hours
- Prepubertal >20 kg: 100 mg/24 hours
- Pubertal >20 kg: 150 mg/24 hours 1
Early Post-Operative Period (First 48 Hours)
- Once stable, provide double the usual oral hydrocortisone replacement doses for 48 hours 1
- Then reduce to normal replacement doses over up to one week 1
Tapering Strategy and Timeline
Expected Recovery Duration by Etiology
The time to HPA axis recovery differs significantly by Cushing syndrome subtype 2:
- Adrenal adenoma (this case): median 2.5 years (IQR 1.6-5.4 years), with only 38% recovering within 5 years 2
- Cushing's disease: median 1.4 years 2
- Ectopic ACTH: median 0.6 years 2
Monitoring During Taper
- Measure serial morning cortisol levels regularly to guide steroid tapering and assess HPA axis recovery 1
- Monitor for clinical signs of adrenal insufficiency during dose reductions 1
Critical Pitfalls to Avoid
Do Not Taper Too Rapidly
Abrupt steroid withdrawal can precipitate adrenal crisis—the taper must be gradual and guided by biochemical monitoring 1. Most patients develop some degree of adrenal insufficiency during glucocorticoid withdrawal, which is typically managed by resuming the prior oral dose 3.
Do Not Forget Stress-Dose Coverage
Until HPA axis recovery is confirmed, the child remains at risk for adrenal insufficiency during physiologic stress (illness, surgery, trauma) and requires stress-dose glucocorticoid coverage 1. This is a common oversight that can lead to life-threatening complications.
Do Not Assume Lifelong Replacement is Needed
Unlike bilateral adrenalectomy or Cushing's disease (which requires lifelong surveillance for recurrence up to 15 years later 1, 4), adrenal adenoma does NOT require lifelong glucocorticoid therapy 1. The HPA axis will recover, though it may take months to years 2.
Long-Term Follow-Up Requirements
Regular endocrine follow-up during the recovery phase is essential to 1:
- Monitor for signs of adrenal insufficiency
- Assess growth velocity and bone density recovery (critical in pediatric patients)
- Guide steroid dose adjustments and eventual discontinuation
- Confirm HPA axis recovery before stopping replacement therapy
When to Discontinue Replacement
Glucocorticoid replacement can be discontinued once morning cortisol levels demonstrate adequate HPA axis recovery, typically after several months (mean 6.7 ± 1.2 months for adrenal adenomas) 5, though complete recovery may take up to 2.5 years in many cases 2.