Incidence of Nelson's syndrome after adrenalectomy for Cushing's disease in children: results of a nationwide survey.
To establish the incidence of Nelson's syndrome in children treated with total bilateral adrenalectomy (TBA) for Cushing's disease, a survey was made of members of The Lawson Wilkins Pediatric Endocrine Society. Thirty-one patients aged 10 months to 16 years had been treated with TBA for Cushing's disease; one had been treated with ortho para prime isomer of dichlorodiphenyldichloroethane alone. Postadrenalectomy hyperpigmentation was reported in 18 patients. Sella enlargement was detected in eight patients (25%) after 1 to 5.5 years (mean, three years) post-TBA. Five of these patients have had documented pituitary adenomas to date. This incidence is higher than the adult figure of 10% to 16%.
D000293:Adolescent; D000315:Adrenalectomy; D002648:Child; D003480:Cushing Syndrome / Q000601:surgery*; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D008297:Male; D009347:Nelson Syndrome / Q000175:diagnosis / Q000453:epidemiology*; D010911:Pituitary Neoplasms / Q000453:epidemiology*; D011183:Postoperative Complications / Q000175:diagnosis / Q000453:epidemiology*; D014481:United States