To report a case of ACTH-independent Cushing’s syndrome due to isolated micronodular adrenal disease in a very young child.
Approach:
Imaging Studies: Adrenal MRI showed morphologically normal adrenal glands; adrenal scintigraphy indicated bilateral adrenal uptake, slightly more prominent on the right.
Post-Operative Outcomes: Post-surgery, significant improvement in blood pressure, metabolic abnormalities, cutaneous manifestations, and motor function was observed. Steroid supplementation was required for only 3 days post-operatively, with no evidence of persistent adrenal insufficiency.
Key Findings:
Isolated micronodular adrenal disease can cause ACTH-independent Cushing’s syndrome in very young children.
Normal adrenal MRI does not exclude the diagnosis of isolated micronodular adrenal disease.
Unilateral adrenalectomy can lead to durable remission without persistent adrenal insufficiency.
Interpretation:
The case highlights the need to consider isolated micronodular adrenal disease in young children with ACTH-independent Cushing’s syndrome, even when imaging results are normal.
Limitations:
The study is based on a single case report, limiting generalizability.
Long-term outcomes beyond 3 years are not addressed.
Conclusion:
This case underscores the importance of functional adrenal imaging and the potential for successful surgical intervention in young patients with ACTH-independent Cushing’s syndrome.
