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Treatment of Cushing's disease (C.D.) by transphenoidal microadenectomy (Tr.M.) in childhood and adolescence

The efficacy of Tr.M. in the treatment of C.D. in children and adolescents has not been assessed. We treated 8 patients (7-6/12-18-9/12 yrs old) with C.D. by Tr.M. (followup 8-72 months post-operatively). Growth failure and weight gain were the first signs of C.D.; pubertal delay, virilization and fatigability were variable features. Preoperative evaluation showed elevated urinary free cortisol and no diurnal variation in plasma cortisol or ACTH values; high dose dexamethasone therapy suppressed excretion of urinary free cortisol. Two of 8 patients lacked definite radiographic evidence of a pituitary adenoma. Transphenoidal exploration revealed 1-4 mm adenomas in 7 of 8 patients; no definite adenoma was noted in the 8th patient in spite of CT scan evidence of adenoma. Postoperative complications were limited to transient diabetes insipidus. The 7 treated patients had low cortisol and ACTH secretion postoperatively and required replacement glucocorticoid therapy for 6-12 months. Weight loss, growth and pubertal progression without recurrence of C.D. were noted in 6 patients with long term followup. Our experience indicates that ACTH secreting microadenomas are the principal cause of C.D. in childhood and adolescence and suggests that transphenoidal microadenectomy is the initial treatment of choice for Cushing's disease in young patients.