Surgical management of pediatric Cushing's disease: an analysis of 15 consecutive cases at a specialized neurosurgical center.
OBJECTIVE
The aim of this study was to review the results of surgery for pediatric patients with Cushing's disease who were less than 18 years old and underwent transsphenoidal surgery in a specialized center during a 25-year period.


METHODS
Retrospective study, in which the medical records, histology and pituitary imaging of 15 consecutive pediatric patients with Cushing's disease (mean age: 13 years) were evaluated by the same team of endocrinologists and a neurosurgeon from 1982 to 2006. Patients were considered cured when there was clinical adrenal insufficiency and serum cortisol levels were below 1. 8 microg/dL or 50 nmol/L after one, two, three, or seven days following surgery; they therefore required cortisone replacement therapy. Follow-up was for a median time of 11.5 years (range: 2 to 25 years).


RESULTS
Clinical and biochemical cure was achieved in 9/15 patients (60%) exclusively after transsphenoidal surgery. Hypopituitarism was observed in four patients; growth hormone deficiency, in two; permanent diabetes insipidus, in one case.


CONCLUSIONS
Cushing's disease is rare in children and adolescents. Transsphenoidal surgery is an effective and safe treatment in most of these patients. Plasma cortisol level < 1. 8 microg/dL following surgery is the treatment goal and is a good predictive factor for long-term cure of Cushing's disease.
Oliveira
Ricardo Santos de
RS
Divisão de Neurocirurgia Pediátrica, Departamento de Cirurgia e Anatomia, Faculdade de Medicina de Ribeirão Preto, Universidade de São Paulo, Ribeirão Preto, SP, Brasil. rsoliveira@hcrp.fmrp.usp.br
Castro
Margaret de
Md
Antonini
Sonir Roberto Rauber
SR
Martinelli
Carlos Eduardo
CE
Moreira
Ayrton Custódio
AC
Machado
Helio Rubens
HR
D000236:Adenoma / Q000473:pathology / Q000601:surgery*; D000293:Adolescent; D000309:Adrenal Insufficiency / Q000473:pathology; D002648:Child; D004812:Epidemiologic Methods; D005260:Female; D006801:Humans; D006854:Hydrocortisone / Q000097:blood; D007016:Hypophysectomy / Q000379:methods*; D008297:Male; D047748:Pituitary ACTH Hypersecretion / Q000601:surgery*; D010911:Pituitary Neoplasms / Q000473:pathology / Q000601:surgery*; D012016:Reference Values; D016896:Treatment Outcome
D006854:Hydrocortisone