Nelson's syndrome after bilateral adrenalectomy for Cushing's disease.

BACKGROUND: Presentation and outcome of Nelson's syndrome after bilateral adrenalectomy is variable. METHODS: Clinical records of 39 patients who underwent bilateral adrenalectomy for primary or recurrent Cushing's disease during a 15-year period were analyzed for frequency and evolution of Nelson's syndrome. RESULTS: The study included 32 females and 7 males with a mean age of 31 years; 20 patients had a hypophysectomy as the initial procedure, and 19 had an adrenalectomy. Of the group, 17 patients received prophylactic radiation therapy to the pituitary gland. A total of 11 patients, none of whom had received prophylactic radiation therapy, developed Nelson's syndrome (determined by skin hyperpigmentation, elevated serum ACTH levels, and enlargement of a previous pituitary tumor or development of a new tumor in patients with no previous pituitary abnormality) over a mean follow-up period of 53 months. Treatment for Nelson's syndrome included valproic acid, radiation therapy, and hypophysectomy as monotherapy or combined therapy. Of the remaining 28 patients, 10 (7 without prophylactic radio therapy) developed skin hyperpigmentation and increased ACTH levels without a tumor. CONCLUSIONS: Nelson's syndrome is a frequent complication after bilateral adrenalectomy in the absence of prophylactic radiotherapy (28%). The syndrome can be successfully controlled by medical treatment and or radiotherapy; patients rarely require hypophysectomy.

Is intraoperative parathyroid hormone assay mandatory for the success of targeted parathyroidectomy?

BACKGROUND: Minimally invasive parathyroidectomy has become the first surgical option for patients with primary hyperparathyroidism (HPT) in many places. Preoperative localization studies are mandatory, and the use of a quick parathyroid hormone (PTH) assay is highly recommended. The aim of this study was to analyze our initial series of targeted parathyroidectomies. STUDY DESIGN: In a 2-year period, 50 patients underwent unilateral neck exploration for HPT under local anesthesia and light sedation. After biochemical diagnosis, a technetium 99m sestamibi scan was performed on all patients, and cervical ultrasonography was obtained in some patients. Frozen section analysis was used to confirm parathyroid tissue in all patients. There was no biochemical intraoperative evaluation of PTH. Demographics, surgical details, results, and complications were analyzed. RESULTS: There were 35 women and 15 men, with a mean age of 56 years (range 23 to 85 years). Mean preoperative calcium was 11.4 mg/dL (range 10.0 to 14.8 mg/dL), and PTH was 342 pg/mL (range 105 to 2,231 pg/mL). Mean surgical time was 52 minutes (range 30 to 100 minutes), and mean hospital stay was 2 days (range 1 to 7 days). Mean parathyroid weight was 1,000 mg (range 117 to 17,000 mg). Sestamibi scan correctly localized the abnormal gland in 47 patients (94%). There was one postoperative complication (bleeding); two patients required contralateral exploration, and persistent hypercalcemia developed in one that required surgical reintervention. After a mean followup of 12 months (range 3 to 25 months), all patients were normocalcemic. CONCLUSIONS: Targeted parathyroidectomy is safe and effective. Despite the fact that quick intraoperative PTH assay was not used, the cure rate was 98%.