Special Aspects Of Cushing'S Syndrome: Childhood

Cushing's syndrome (CS) is a rare pediatric problem on which few pediatric endocrinologists have extensive experience. Hence, close collaboration with an adult endocrinologist colleague is strongly recommended. This chapter reviews the main types of CS occurring in the pediatric age range. Results are presented from the authors' own experience of management of over 50 cases of pediatric CS during the last 25 years. These include McCune Albright syndrome in infancy, adrenocortical tumors, primary nodular adrenal hyperplasia, ectopic ACTH syndrome, and Cushing's disease. The major clinical features are described, including linear growth and puberty, and diagnostic techniques are discussed. From a personal database of 38 cases of Cushing's disease (CD), the relative benefits of pituitary MR imaging versus bilateral inferior petrosal sinus sampling for ACTH are appraised. Therapeutic strategy is discussed for primary adrenal CS and pituitary-dependent CD. The results of transsphenoidal surgery and pituitary radiotherapy are described together with the challenge of optimizing postcure linear growth and body composition.