We report about a male patient, who underwent bilateral total adrenalectomy due to ACTH-dependent Cushing's syndrome without pituitary adenoma in his 11th year of life. Symptoms of Cushing's disease were absent during the follow-up period. Height and body weight followed the percentiles 25 and 50 respectively. An endogenous cortisol production was not detected and steroid substitution became necessary. Five years after the surgery, he showed normal cortisol levels, but there was no cortisol increase under ACTH-loading. Nine and 12 years (1993) after surgery, we found normal, physiological levels of cortisol suggestive of normal functioning adrenal glands (day profile of cortisol, Liddle-Test, ACTH-Test, CRH-Test, cortisol excretion in 24-h-urine). Moreover, baseline ACTH and its rise under CRH stimulation were normal. Scintigraphy revealed normal-sized adrenal tissue in orthotopic position on both sides. Steroid supplementation was discontinued. Presently the patient is healthy, active and under no steroid therapy. No evidence of residual or relapsing Cushing's disease or Nelson's syndrome has been found up to this point.
