THERAPEUTIC RESULTS IN CUSHINGS-DISEASE I N CHILDREN AND ADOLESCENTS

Background. - Trans-sphenoidal surgery is currently the treatment of c hoice for Cushing's disease in children. Patients and methods. - The r esults obtained in 20 consecutive patients referred to the Pediatric E ndocrinology Department of hopital Saint-Vincent-de-Paul are reported. Results. - A remission of Cushing's disease was observed in 12/16 (75 %) patients in whom surgery was the first treatment. Among these 12 pa tients, three relapsed (25%) 21 to 80 months after surgery. Four patie nts were initially treated with steroid synthesis inhibitors: three of those patients were subsequently operated on and their disease remitt ed. Among the seven patients in whom surgery failed (primary failure o r relapse), two were reoperated and also remitted. Taken together, 21 operations were performed and resulted in four immediate failures (19% ), three relapses (14%) and 14 long-term remissions (67%, follow-up 40 +/- 35 months). None of the biological, radiological or operative cri teria were predictive of the therapeutic results. Conclusion. - Our re sults illustrate the efficacy and limits of trans-sphenoidal surgery f or Cushing's disease of children and emphasize the need for a very lon g follow-up of these patients. Treatment of patients in whom surgery h as failed (initially or secondarily) is particularly difficult and req uires a multidisciplinary approach.