Adrenalectomy in Cushing's Disease

Abstract

SUMMARY Forty-four patients with Cushing's disease underwent bilateral adrenalectomy and 39 survivors were followed for, on an average, eight years and a half. There were three postoperative deaths and two other patients died within one year after operation. Thirty patients are now working but six remain invalidized. In four subjects cortisol secretion persisted in spite of "total" adrenalectomy but a clinical remission ultimately occurred. Five patients, who presented with an atypical clinical picture, failed to show any obvious changes in the distribution of fat or in the complexion. Body weight did not decrease after surgery in several patients and in many others body weight increased again after an initial loss of weight. Menstruations reappeared in all premenopausal women except two. Manifest diabetes, which was found in seven subjects, disappeared and in several other cases glucose tolerance improved. The blood pressure, which was high in most patients, normalized or decreased in all hypertensive patients but the blood pressure still remains elevated in 22 subjects. ECG-abnormalities disappeared in 11 of 17 subjects, who presented with ST-T changes and/or high amplitude R waves. The size of the heart decreased in most patients with cardiomegaly. A remarkable improvement in the radiological appearance of the vertebrae occurred in a 16-year-old boy but in the other patients there were no such changes. Mental disturbances, which were a main cause of preoperative disability, disappeared in most cases. Two patients, however, continue to be invalidized from mental disease, which developed during the course of the hypercorticism. One patient presented with a grossly evident pituitary tumour and in four other subjects a large pituitary tumour developed after adrenalectomy together with hyperpigmentation. Pituitary apoplexy occurred in one of these patients and another was operated upon because of visual field losses. It is concluded that the remission obtained after adrenalectomy for Cushing's disease increases the survival rate and makes a normal life possible in most patients. However, persistent hypertension and cardiovascular disease as well as the possibility of the development of a pituitary tumour make the late prognosis somewhat uncertain.

The Nelson's syndrome... revisited

“…The complications related to the Nelson's syndrome are essentially due to the tumoral volume: chiasmatic compression with visual field loss, either transitory or definitive is the most frequent complication described, with a prevalence ranging from 1/10 to 4/9 [24,30,32,35,37,38,45,47,[49][50][51][52]. Occulomotor nerve palsy has also been described [49], as well as tumor necrosis with sudden intracranial hypertension [35,51].…”

“…Occulomotor nerve palsy has also been described [49], as well as tumor necrosis with sudden intracranial hypertension [35,51]. Cases of diabetes insipidus [5] and hypopituitarism are exceptional.…”

Trans-sphenoidal microsurgical treatment of Nelson's syndrome

“…Pre-.vious literature defines Nelson's syndrome somewhat differently. Some investigators regard hyperpigmentation as important for diagnosis [3,23], whereas others require both hyperpigmentation and sellar enlargement for definite diagnosis [8,29,41]. If one follows the original description of the disease by Nelson et al [35], the diagnostic criteria of Nelson's syndrome will include a demonstrable ACTHhypersecreting pituitary adenoma with sellar enlargement, visual disturbances and increased cutaneous pigmentation that developed after bilateral adrenalectomy for Cushing's disease.…”

“…Although it is estimated by the previous literature [8,10,13,15,16,20,28,32,37,39,46,54] that 5 to 20% of adrenalectomized Cushing patients will develop pituitary tumours, the true prevalence has been unclear. The relatively low incidence of detecting Nelson's adenoma in the past is solely due to the lack of precise neurodiagnostic techniques, taking only symptomatic macroadenomas.…”

Cushing's Disease and Pregnancy

“…Postoperative replacement therapy, appropriately increased during labor or caesarean section [39], allowed gestation and delivery to proceed uneventfully [25,27]. In this context it is worth recalling the considerable number of women cured from Cushing's disease by bilateral adrenalectomy, as was the custom in the past [44], in whom pregnancy was carried to term without mishap.…”