Incidence of Nelson's syndrome after adrenalectomy for Cushing's disease in children: results of a nationwide survey
N. J. Hopwood and F. M. Kenny
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%.
