Cushing's Syndrome in Children and Adolescents -- Presentation, Diagnosis, and Therapy
Maria Alexandra Magiakou, George Mastorakos, Edward H. Oldfield, M. Teresa Gomez, John L. Doppman, Gordon B. Cutler, Lynnette K. Nieman, and George P. Chrousos
Background and Methods Cushing's syndrome is rare in childrenand adolescents. We analyzed the clinical presentation, diagnosticevaluation, and treatment of 59 patients with Cushing's syndromebetween the ages of 4 and 20 years who were admitted to theNational Institutes of Health during the period from 1982 to1992. The cause of hypercortisolism was identified by low- andhigh-dose dexamethasone suppression tests, the ovine corticotropin-releasinghormone (CRH) stimulation test, imaging studies, and bilateralsampling of the inferior petrosal sinuses combined with administrationof CRH.
Results Fifty patients had Cushing's disease, six had primaryadrenal disease, and three had ectopic corticotropin secretion.The initial signs were excessive weight gain in 90 percent ofthe patients and growth retardation in 83 percent. Most patients(81 percent) had normal bone age at the time of diagnosis. Forty-sevenpercent had hypertension, whereas only 19 percent had mentalor behavioral problems. The high-dose dexamethasone suppressiontest and the CRH stimulation test identified 68 and 80 percent,respectively, of the patients with Cushing's disease. Magneticresonance imaging of the pituitary indicated the presence oftumor in 52 percent of the patients with pituitary adenomas.The maximal central-to-peripheral ratio of plasma corticotropinduring sampling of the interior petrosal sinuses was 2.5 inall the patients with Cushing's disease and <2.5 in thosewith ectopic corticotropin secretion. Remission of hypercortisolismwas achieved in 48 of the 49 patients who underwent transsphenoidalsurgery for Cushing's disease, in all 6 of the patients whounderwent adrenalectomy for primary adrenal disease, and inthe 2 patients in whom the ectopic source of corticotropin couldbe identified.
Conclusions Weight gain and growth retardation are common clinicalcharacteristics of Cushing's syndrome in children and adolescents.Diagnostic evaluation of such patients with CRH stimulationalone and combined with inferior petrosal sinus sampling andimaging studies is accurate, and therapy is usually successful.
Source Information
From the Developmental Endocrinology Branch, National Institute of Child Health and Human Development (M.A.M., G.M., M.T.G., G.B.C., L.K.N., G.P.C.); the Surgical Neurology Branch, National Institute of Neurological Disorders and Stroke (E.H.O.); and the Diagnostic Radiology Department, National Institutes of Health (J.L.D.) -- all in Bethesda, Md.
Address reprint requests to Dr. Magiakou at NICHD/DEB Bldg. 10, Rm. 10N262, 9000 Rockville Pike, Bethesda, MD 20892.
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2.5 in all the patients with Cushing's disease and <2.5 in those with ectopic corticotropin secretion. Remission of hypercortisolism was achieved in 48 of the 49 patients who underwent transsphenoidal surgery for Cushing's disease, in all 6 of the patients who underwent adrenalectomy for primary adrenal disease, and in the 2 patients in whom the ectopic source of corticotropin could be identified. 
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