Long-term outcome in children and adolescents after transsphenoidal surgery for Cushing's disease.

摘要: Cushing's disease refers specifically to an ACTH-producing pituitary adenoma that stimulates excess cortisol production. Transsphenoidal surgery is the treatment of choice in children and adolescents, but disparate cure rates have been reported, ranging from 50-98%. The discrepancies rate are due primarily technical success length method follow-up. We studied 42 consecutive adolescents (age, < or = 18 yr) who underwent transsphenoidal exploration for primary at University California-San Francisco 1974-1993. Only 7 patients had persistent disease, defined as evidence within 6 months surgery, yielding initial remission 83%. comprehensively evaluated 26 35 experienced remission, including testing ACTH-adrenocortical axis. mean duration follow-up 7.2 yr (range, 1.5-13.6 yr). Seven a relapse net 73%. Relapses occurred average 4.2 postoperatively 0.75-6.2 Five 5 whereas 2 relapsed more than postoperatively. Repeat was performed 8 with recurrent these remain remission. Low serum urinary measurements first post-operative week predicted were not necessarily predictive long-term cure. Hypercortisolism significant effects on bone metabolism, reflected by both diminished density majority examined decreased growth rate. Both parameters improved after surgical care, although they did fully normalize. conclude safe effective pediatric surveillance necessary detect possible recurrences.