A case of Nelson syndrome was presented. Male patient of 26 years old suffering from Cushing disease underwent an bilateral epinephrectomy, after that, Nelson syndrome developed and the patient was treated by Cobalt 60, the skin was discolored and head pain was relieved. Nelson syndrome is caused by total epinephrectomy E in Cushing disease’s patient, its clinical manifestation is typically a leader skin, head pain, high blood concentration of ACTH. The treatment of choice for this syndrome is an operation of pyfrophyse through sphenoidal bone or irradiation into hypophysal hole
Nelson Syndrome ; Cushing Syndrome ; Diseases

OBJECTIVETo discuss the etiology, diagnostic criteria and treatment of Nelson's syndrome.

METHODSTwenty-three patients with Nelson's syndrome who were treated in our department over the last 19 years were analyzed retrospectively. Removal of adenoma by the transsphenoidal approach was done in 21 patients and by transfrontal craniotomy in 2. The follow-up period ranged from six months to nine years.

RESULTSThe incidence of Nelson's syndrome was 7.7% in a series of 300 patients with Cushing's disease treated by microsurgery in the same period. Hyperpigmentation was relieved and adrenocorticotropic hormone (ACTH) levels decreased in all patients after tumor excision. Eight patients with visual disturbance improved after surgery. The curative and remission rates were 56.5% and 26.1%, respectively.

CONCLUSIONSTranssphenoidal microsurgical removal of pituitary ACTH adenoma is the first choice in the prevention and treatment of Nelson's syndrome. Regular follow-up examinations should be performed over a long time.

Adolescent ; Adult ; Female ; Follow-Up Studies ; Humans ; Incidence ; Male ; Microsurgery ; methods ; Middle Aged ; Nelson Syndrome ; epidemiology ; etiology ; surgery

OBJECTIVETo discuss the effects of adrenalectomy (ADX) on the treatment of Cushing's disease(CD).

METHODSClinical data of 15 cases of CD between January 1980 and December 2005 were analyzed to evaluate operative indications, complications and the changes of hypercortisolism and hormone levels pre- and post- adrenalectomy.

RESULTSAll the patients involved underwent transsphenoidal pituitary surgery previously. Repeated transsphenoidal surgery was performed in 4 cases. Pituitary radiotherapy was done in 4 cases. The average time from original transsphenoidal operation to ADX was 25.7 months. Pre- and post- adrenalectomy serum cortisol median level were 1156.4 nmol/L and 99.4 nmol/L, the 24 h urinary-free cortisol median level were 315.0 and 5.4 microg, respectively. Hormone replacement therapy was needed in all cases. Average follow-up period was 47 months (9-120 months). Nelson syndrome (NS) appeared in 5 cases (33.3%), while 10 cases showed no NS.

CONCLUSIONSADX is an effective and symptomatic treatment to relieve hypercortisolism caused by CD but with the risk of NS. Longtime hormone replacement therapy and follow up are needed after ADX.

Adolescent ; Adrenalectomy ; adverse effects ; methods ; Adrenocorticotropic Hormone ; blood ; Adult ; Child ; Female ; Follow-Up Studies ; Humans ; Hydrocortisone ; blood ; Male ; Middle Aged ; Nelson Syndrome ; etiology ; prevention & control ; Pituitary ACTH Hypersecretion ; blood ; surgery ; Postoperative Complications ; prevention & control ; Retrospective Studies ; Treatment Outcome