Clinical Report: Isolated Micronodular Adrenal Disease in a Young Child

Background

Cushing’s syndrome in early childhood is rare and can be difficult to diagnose, especially when imaging results are inconclusive. ACTH-independent forms of Cushing’s syndrome, such as isolated micronodular adrenal disease, are more common in younger children.

Data Highlights

The patient exhibited the following clinical features: rapid weight gain, cushingoid appearance, hypertrichosis, severe hypertension, irritability, and developmental regression. Biochemical assessments confirmed ACTH-independent hypercortisolism with suppressed ACTH and increased 24-hour urinary free cortisol.

Key Findings

• The patient presented with classic symptoms of Cushing’s syndrome, including rapid weight gain and hypertension.
• Biochemical tests confirmed ACTH-independent hypercortisolism with suppressed ACTH levels.
• Adrenal imaging showed normal morphology.
• Histopathological examination post-adrenalectomy revealed micronodular adrenocortical hyperplasia.
• Following surgery, the patient required minimal steroid supplementation.
• At 3 years of follow-up, there were no clinical signs of recurrent hypercortisolism.

Clinical Implications

Functional adrenal imaging may be beneficial in selected cases.

Conclusion

Isolated micronodular adrenal disease can present significant clinical challenges in young children.

Related Resources & Content

1. The Journal of Clinical Endocrinology & Metabolism, 2023 -- Management Strategies for Patients Experiencing Cyclical Cushing Syndrome
2. Frontiers in Endocrinology, 2023 -- Case Report: a family presenting with β-adrenergic/vasopressin-responsive bilateral macronodular adrenal disease with an ARMC5 mutation treated with metyrapone monotherapy for more than 5 years
3. Frontiers in Endocrinology, 2023 -- Case Report: Pediatric ACTH-secreting pituitary adenoma presenting with hypertension and anuria
4. Cushing Syndrome/Disease in Children and Adolescents - Endotext - NCBI Bookshelf, 2023
5. Frontiers in Endocrinology — Cushing’s syndrome and early growth hormone hypersecretion in a child with Carney complex: a case report
6. Cushing Syndrome/Disease in Children and Adolescents - Endotext - NCBI Bookshelf
7. Cushing Syndrome/Disease in Children and Adolescents - Endotext - NCBI Bookshelf
8. Bilateral Adrenocortical Nodular Disease and Cushing's Syndrome | The Journal of Clinical Endocrinology & Metabolism | Oxford Academic