The diagnosis and transsphenoidal microsurgery in children and adolescents with cushing's disease

Objective To discuss the diagnosis and surgical treatment of Cushing's disease in children and adolescents(under 20 years of age).Methods 11 patients were males and 23 were females.The mean ages were 15 2 yrs.Mean history was 2 1 years.The presenting signs and symptoms were concentric hypertrophy in 91 2% cases.The high dose dexamethasone(HdDXM)test showed unequivocal suppression and low dose DXM test no suppression of urine free cortisol in 70 6%.Pituitary adenomas were diagnosed by CT in 57 1%, by MRI in 70%.Transsphenoidal surgery were performed on all the patients.Pituitary microadenomas were diagnosed by pathology in 28 cases, hyperplasia in 4 cases and negative in 2 cases.Results All the patients were followed up from 3 months to 10 years.The cure rate of pituitary ACTH adenomas was 89 3%.Conclusions The transsphenoidal microsurgery is optimal treatment in children and adolescents with Cushing's disease.If endocrinological data is unequivocal for Cushing's disease but MRI scans show no significant evidance of pituitary adenoma, or if endocrinological data do not supported the diagnosis of Cushing's disease but MRI scans show pituitary adnoma in Cushing syndrome, we recommend to explore the pituitary gland by transsphenoidal surgery.