[Cushing disease in children and in adolescents. Therapeutic results].
BACKGROUND
Trans-sphenoidal surgery is currently the treatment of choice for Cushing's disease in children.


METHODS
The results obtained in 20 consecutive patients referred to the Pediatric Endocrinology Department of hôpital 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 patients, three relapsed (25%) 21 to 80 months after surgery. Four patients were initially treated with steroid synthesis inhibitors: three of those patients were subsequently operated on and their disease remitted. Among the seven patients in whom surgery failed (primary failure or 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 criteria were predictive of the therapeutic results.


CONCLUSIONS
Our results illustrate the efficacy and limits of trans-sphenoidal surgery for Cushing's disease of children and emphasize the need for a very long follow-up of these patients. Treatment of patients in whom surgery has failed (initially or secondarily) is particularly difficult and requires a multidisciplinary approach.
Mathivon
L
L
Service d'endocrinologie pédiatrique, hôpital Saint-Vincent-de-Paul, Paris, France.
Carel
J C
JC
Coutant
R
R
Derome
P
P
Adamsbaum
C
C
Bougnères
P
P
Chaussain
J L
JL
D000237:Adenoma, Basophil / Q000601:surgery; D000293:Adolescent; D018931:Antineoplastic Agents, Hormonal / Q000627:therapeutic use; D002648:Child; D003480:Cushing Syndrome / Q000188:drug therapy* / Q000601:surgery*; D005260:Female; D006801:Humans; D008297:Male; D008939:Mitotane / Q000627:therapeutic use; D010902:Pituitary Gland / Q000601:surgery; D010911:Pituitary Neoplasms / Q000601:surgery; D011184:Postoperative Period; D012074:Remission Induction; D013100:Sphenoid Bone; D017211:Treatment Failure
D018931:Antineoplastic Agents, Hormonal; D008939:Mitotane
Maladie de Cushing de l'enfant et de l'adolescent. Résultats thérapeutiques.