Surgical management of Cushing's syndrome.

Twenty-seven operations for Cushing's syndrome were reviewed. Included were five cases of adenoma and 22 of adrenal cortical hyperplasia. Preoperative laboratory data, particularly the metyrapone test, were highly accurate in distinguishing adenoma from hyperplasia. Bilateral flank incisions are preferable to the transabdominal approach, with fewer complications, less postoperative ileus, and shorter hospital stays. The inability to inspect both glands simultaneously is of little consequence because biochemical testing data are sufficiently accurate to obviate the need for gross evaluation. The use of perioperative prophylaxis seems to be of importance for preventing thromboembolism in these hypercoagulable patients. The preoperative use of adrenal cortical blocking agents has not proved to yield significant advantages.     

Surgical management of pediatric Cushing's disease

BACKGROUND: Cushing's disease may have a direct effect on growth pattern, pubertal maturation, and long-term survival in pediatric patients. METHODS: Dexamethasone suppression test was done in 10 children (median age, 15 years) and showed variable suppressibility, with microadenoma seen in 5, macroadenoma in 3, and normal gland in 2 patients. Inferior petrosal sinus sampling (IPPS) was also carried out and confirmed pituitary adenoma as source of ACTH in 2 patients. We adopted sublabial transsphenoidal (n = 9) or pterional transsylvian route (n = 1) in macroadenoma with conchal sphenoid sinus. Serum cortisol level <50 nmol/L was taken as the criteria for biochemical remission. RESULTS: Clinical remission was achieved in 7 of 10 operated patients. In 2 patients where clinical remission was achieved, postoperative BSC could not be done. Postoperative BSC was less than 50 nmol/L in 2 (25%) of 8 patients and remained elevated in 6. Remission was achieved in both patients with postoperative BSC less than 50 nmol/L and in 3 of 6 with elevated levels. Three patients had neither clinical nor biochemical remission: 2 underwent bilateral adrenalectomy and 1 received radiotherapy. Postoperative CSF leak seen even in microadenomas associated with arachnoidal prolapse. Among 7 patients who initially remitted (median follow-up of 82 months; range, 24-120 months), recurrence of disease occurred in 3 (42.8%) patients after a median interval of 5 years. CONCLUSIONS: In children with CD, endocrinal manifestations are more frequent than visual symptoms. Transsphenoidal route is the preferred approach, but a nonpneumatised sphenoid sinus may be present. Sellar arachnoidal prolapse may cause postoperative CSF leak even in microadenomas. Surgery is the first line of treatment, but constant monitoring is mandatory to pick up the relapsed cases.     

Contemporary evaluation and management of Cushing's syndrome

Cushing's syndrome, characterized by unregulated cortisol secretion, may be caused by a variety of adrenal, pituitary, or other tumors. The best biochemical test for establishing the diagnosis is determination of 24-h urinary free cortisol. The specific causes for Cushing's syndrome may be further differentiated by plasma adrenocorticotrophic hormone (ACTH). Primary adrenal cortical diseases are associated with low levels of ACTH and are considered ACTH-independent. Pituitary disease and the ectopic ACTH syndrome are associated with normal or elevated ACTH levels and are considered ACTH-dependent. Adrenal forms of Cushing's syndrome may result from either adenoma or carcinoma. The diagnostic approach to Cushing's syndrome and the clinical, biochemical, and radiographic features that distiguish adrenal adenoma and carcinoma are the subjects of this paper.     

Surgical management of postvagotomy syndrome

The purpose of the present report is to demonstrate the results of conservative surgical treatment in twelve patients with postvagotomy syndrome (PVS). The reconstructive operation performed in each case depended on the preexisting drainage procedure. Out of eight patients who had undergone vagotomy and gastrojejunostomy (GJ) four suffered from bile reflux and vomiting, four from dumping (DU) and four had diarrhea. All underwent closure of GJ and two of them duodenoplasty for coexisting duodenal stenosis. The results were very good (Visick grade I and II) in all patients except for one complete failure. Out of four patients who had undergone vagotomy and pyloroplasty, three had DU, two diarrhea and two bile reflux and vomiting. Pyloric reconstruction was performed in all subjects. Symptoms were relieved in all cases. In one patient radioisotopic control showed mild G/O and D/G reflux. Conservative surgical operations give satisfactory results in the treatment of PVS. Furthermore they have lower morbidity and mortality than radical surgery.     

Surgical management of adrenocorticotropic hormone-secreting macroadenomas: outcome and challenges in patients with Cushing's disease or Nelson's syndrome

OBJECT: Adrenocorticotropic hormone (ACTH)-secreting pituitary macroadenomas are an uncommon cause of Cushing's disease (CD) and, subsequently, Nelson's syndrome (NS). They have been associated with low postoperative remission rates. The outcome of modern surgical treatment is unclear and thus was assessed in a series of 43 patients, with the goal of improving therapeutic results in patients with ACTH-secreting macroadenomas. METHODS: Thirty-seven patients presented with CD and six with NS. They represented 15% of the patients surgically treated at the authors' institution for ACTH-secreting adenomas. The median patient age was 38 years (range 14-71 years), and the mean duration of follow up was 37 months (range 1-108 months). Remission occurred in 25 (67.6%) of 37 patients with CD, whereas the disease persisted in 12 (32.4%) of 37 patients. After an initial remission, three (12%) of 25 patients demonstrated signs and symptoms indicative of disease recurrence. Among patients with NS, only one (16.6%) displayed remission. Invasion of the dura mater by tumor was histologically demonstrated in 10 patients with CD and in two patients with NS. CONCLUSIONS: Comprehensive management of CD caused by ACTH-secreting macroadenomas through the appropriate use of combination therapy, including surgery, radiotherapy, radiosurgery, and adrenalectomy, can lead to outcomes similar to those for microadenomas. Disease recurrence and persistence rates are higher, often because of the invasiveness associated with macroadenomas. Remission of NS-associated macroadenomas is difficult to achieve. Strategies for the characterization and treatment of invasive macroadenomas are needed.     

Cushing's syndrome

Cushing's syndrome remains one of the great diagnostic challenges to internists and endocrinologists. Urologists have the primary role in the treatment of many of the forms of Cushing's syndrome, especially adrenal tumors and micronodular hyperplasia. As we continue to gain experience with Cushing's disease treated with pituitary surgery and with etopic ACTH syndrome and the other forms of Cushing's syndrome, urologists are likely to become even more involved in the therapy of all types of Cushing's syndrome.     

Surgical management of visceral heterotaxy syndrome

Surgical management of the heterotaxy syndrome including asplenia or polysplenia is still challenging, because they have not only congenital heart defects but also gastrointestinal abnormalities. In most cases, they are Fontan candidates, however, indications and procedures of surgical strategy toward Fontan operation are quite difficult because of cardiac complications or abdominal manifestations. We mentioned indications, surgical technique, peri- and post-operative managements of asplenia syndrome with our experiences and results.     

Surgical management of the preinfarction syndrome

The indications for coronary reconstruction have been extended to include those patients with the preinfarction syndrome who have failed to respond to medical therapy. Preinfarction syndrome is characterized by: (1) rapidly progressive angina; (2) exacerbation of previously stable angina; and (3) recurrent bouts of coronary insufficiency. During a three-year period 60 patients with this syndrome ranging in age between 30 and 72 years underwent urgent or emergency bypass procedures. Twenty showed electrocardiographic evidence of previous myocardial infarction. Single-vessel disease (> 75% obstruction) was demonstrated by coronary angiography in 18, double-vessel disease in 14, and triple-vessel involvement in 28. The operative technique is described. Eight sustained an early and 5 a late myocardial infarction with 1 and 3 deaths, respectively. A detailed analysis of these patients is presented including restudies and pathological findings. Of the 56 survivors, only 2 continue to have incapacitating angina. From this experience we conclude that the surgical management of a selected group of patients with the preinfarction syndrome is associated with a lower mortality and an improved functional result when compared with the natural history of the syndrome.