Recurrent and persistent Cushing's syndrome after assumed total bilateral adrenalectomy.
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Abstract:
Two cases of recurrent Cushing's syndrome after assumed total bilateral adrenalectomy are presented. Both cases were due to adrenal tissue left at the adrenalectomy. In the first case the lack of preoperative localization of the adrenal remnant caused severe postoperative complications. In the other patient localization of the adrenal tissue was achieved by scanning after administration of 131I-19-iodocholesterol. The reoperation was easy and the postoperative course smooth.Keywords:
Bilateral adrenalectomy
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Bilateral total adrenalectomy failed to cure Cushing's syndrome due to adrenal hyperplasia in the case presented here. However, removal of bilateral paraovarian nodules of ectopic adrenocortical tissue resulted in abolition of Cushing's syndrome. Determination of cortisol in venous blood from various locations was useful in diagnostic evaluation.
Bilateral adrenalectomy
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Searchable abstracts of presentations at key conferences in endocrinology ISSN 1470-3947 (print) | ISSN 1479-6848 (online)
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Bilateral adrenalectomy
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OBJECTIVE The prevalence of Nelson's syndrome has varied greatly, at least in part because of the variability of the diagnostic criteria employed by different authors. We define Nelson's syndrome as the presence of an enlarging pituitary tumour associated with elevated fasting plasma ACTH levels and hyperpigmentation in patients with Cushing's disease after bilateral adrenalectomy. We have compared patients with Cushing's disease who developed Nelson's syndrome after bilateral adrenalectomy with those who did not. Our objective was to find differences between the two groups which might predict the development of Nelson's syndrome. PATIENTS AND METHODS We have reviewed the records of 30 patients with Cushing's disease after adrenalectomy, and divided them into two groups; I: 14 who developed Nelson's syndrome and II, 16 who did not. The two groups of patients were compared in their clinical, laboratory and imaging data as well as in the therapeutic procedures that preceeded the adrenalectomy. RESULTS The comparison between the two groups of patients demonstrated a highly significant difference in relation to the development of cutaneous hyperpigmentation (100% in group I and 19% in group II) and neuro‐ophthalmological symptoms (21% in group I and 0% in group II) after adrenalectomy. There were no significant differences in laboratory data before adrenalectomy. After adrenalectomy, plasma ACTH levels increased significantly in the patients of both groups, but to much higher levels in those who developed Nelson's syndrome. Plasma ACTH concentrations above 154 pmol/l occurred only in the subjects with Nelson's syndrome. Before adrenalectomy, a pituitary tumour was more frequent in the patients who developed Nelson's syndrome (55% vs . 33% at transsphenoidal pituitary exploration). Pituitary surgery and irradiation were undertaken before adrenalectomy in approximately equal numbers of patients in each group. DISCUSSION The prevalence of Nelson's syndrome was 47% in our series of 30 patients with Cushing's disease after bilateral adrenalectomy. No clinical or laboratory data before adrenalectomy predicted the development of the syndrome. The value of prophylactic pituitary irradiation could not be evaluated from our clinical material. However, after adrenalectomy, the presence of hyperpigmentation and ACTH levels above 154 pmol/l had positive predictive value for the development of Nelson's syndrome. In this situation magnetic resonance imaging (MRI) of the pituitary is mandatory and, if no tumour is detected, MRI should be repeated at intervals.
Bilateral adrenalectomy
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Oophorectomy
Bilateral adrenalectomy
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Bilateral adrenalectomy
Presentation (obstetrics)
Case presentation
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Bilateral adrenalectomy
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Two cases of recurrent Cushing's syndrome after assumed total bilateral adrenalectomy are presented. Both cases were due to adrenal tissue left at the adrenalectomy. In the first case the lack of preoperative localization of the adrenal remnant caused severe postoperative complications. In the other patient localization of the adrenal tissue was achieved by scanning after administration of 131I-19-iodocholesterol. The reoperation was easy and the postoperative course smooth.
Bilateral adrenalectomy
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Bilateral adrenalectomy
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Bilateral adrenalectomy
Cushing's disease
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Summary OBJECTIVES Bilateral adrenalectomy may be indicated in patients with Cushing's disease in whom hypercortlsolism is not resolved after pituitary microsurgery. However, Nelson's syndrome is a major long‐term complication of such therapy. We have carried out a longitudinal study on patients with Cushing's disease who underwent bilateral adrenalectomy comparing plasma /Mipotrophin ( β ‐LPH) with ACTH levels. PATIENTS AND METHODS Seven patients unsuccessfully treated by pituitary surgery for Cushing's disease underwent bilateral adrenalectomy. Blood samples were collected on days 8 and 15 and at 1, 2, 3, 6, 9,12,18 and 24 months after adrenalectomy in all patients. Five patients were followed up for the longer periods of 30,33,39,72 and 84 months respectively. Plasma ACTH and β ‐LPH were determined by RIA after extraction. Pituitary CT scan was done at 6‐8‐month intervals. RESULTS A pituitary tumour was detected in three patients at 14, 24 and 26 months after adrenalectomy respectively. The basal ACTH (range 8‐21 pmol/l) began to rise between 15 and 30 days and exhibited a sharp increase with a range of 36‐114 pmol/l at 3 months and a range of 53‐187 pmol/l at 6‐9 months after adrenalectomy. The ACTH levels in the three patients who later presented with a pituitary tumour were indistinguishable from those observed in the other patients up to 12 months after adrenalectomy. Only at 12 months or thereafter were their ACTH levels higher than in the other patients (958 ± 252 vs 205 ± 22 pmol/l). β ‐LPH concentrations changed in parallel with ACTH levels. The ACTH levels correlated positively with β ‐LPH levels ( r = 0 76). CONCLUSIONS In patients with Cushing's disease undergoing bilateral adrenalectomy, plasma ACTH and β ‐LPH concentrations cannot predict the development of pituitary tumours until 12 months after surgery.
Cushing's disease
Bilateral adrenalectomy
Basal (medicine)
Cushing Disease
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