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Hyperadrenalism in childhood and adolescence
Annals of Surgery
|May 1, 1984
Summary
Pediatric hyperadrenalism management varies by cause. Transsphenoidal surgery is preferred for Cushing's disease, while adrenalectomy is reserved for specific cases like micronodular hyperplasia or ectopic ACTH. Lifelong monitoring for Nelson's syndrome post-adrenalectomy is crucial.
Area of Science:
- Pediatric Endocrinology
- Surgical Oncology
- Endocrine Surgery
Background:
- Hyperadrenalism in pediatric and adolescent patients presents unique diagnostic and management challenges.
- This review analyzes the experience with 18 patients aged 18 months to 18 years with various forms of hyperadrenalism, excluding congenital adrenal hyperplasia and hyperaldosteronism.
Observation:
- Nine patients had bilateral adrenal hyperplasia, eight had adrenal neoplasms (five adenomas, three carcinomas), and one had micronodular hyperplasia.
- Surgical interventions included total adrenalectomy for Cushing's disease and adrenal neoplasms, and transsphenoidal pituitary tumor removal for Cushing's disease.
Findings:
- No definitive pre-surgical criteria distinguished adrenal adenomas from carcinomas, though malignancy was suggested by tumor weight >75g.
- Adrenalectomy for Cushing's disease led to significant stunting in 5/6 patients and Nelson's syndrome in 4/6.
- Transsphenoidal surgery for Cushing's disease showed better outcomes, with one patient requiring subsequent pituitary irradiation for recurrence.
Implications:
- Transsphenoidal removal of pituitary adenomas is the primary treatment for pediatric Cushing's disease.
- Adrenalectomy is indicated for micronodular hyperplasia, ectopic ACTH, and recurrent Cushing's disease post-pituitary treatment.
- Lifelong surveillance for pituitary tumor progression (Nelson's syndrome) is essential in pediatric patients treated with adrenalectomy.