Robert M. Salassa

Childhood Cushing disease: results of bilateral adrenalectomy.

In view of the published reports of the successful correction of hypercortisolism in adult patients with Cushing disease by external pituitary irradiation and transsphenoidal pituitary microsurgery, leaving the patients with intact pituitary and adrenocortical function, we have reviewed the results of adrenalectomy in childhood Cushing disease seen at the Mayo Clinic. Twenty-seven patients were treated with total (16 patients) or subtotal (11 patients) bilateral adrenalectomy before the age of 20 years. Follow-up ranged from one to 27 years. Although hypercortisolism was corrected in all but one patient, 12 (45%) patients had roentgenographic evidence of a pituitary tumor and six (22%) of these required pituitary surgery. An additional six patients (22%) were pigmented, but had no abnormality on roentgenographic studies. The remaining nine patients (33%) were well, without evidence of pituitary tumor. We conclude that alternate forms of therapy should be considered for childhood Cushing disease.

Adrenal insufficiency in a female pseudohermaphrodite.

A 6-year-old female pseudohermaphrodite developed adrenal cortical insufficiency during infancy. Evidence derived from a study of this patient suggests that congenital adrenal cortical hyperplasia was the cause of the pseudohermaphroditism. The cause of the subsequent adrenal cortical atrophy is unknown.

Lesions of the adrenal glands of surgical significance.

Hyperfunction of the adrenal cortices caused either by hyperplasia or by functioning tumor results in a variety of recognizable clinical syndromes, most of which are amenable to surgical treatment. Others may respond favorably to medical management. Paroxysmal or persistent hypertension caused by functioning tumor of the medulla of the adrenal gland can be cured by surgical intervention in most cases.