Heerden, Jon A. van

AbstractPersistent or recurrent hypercalcemia following operation for benign hyperparathyroidism (HPT) remains a surgical challenge. From 1978 to 1984 at the Mayo Clinic, 157 patients underwent reexploration. This included 110 cervical, 38 combined cervical and mediastinal, and 9 mediastinal explorations. A total of 197 previous operations (range 1–5) had been performed, including 136 patients for sporadic disease, 14 for multiple endocrine neoplasia, and 7 for familial HPT. Preoperative localizing studies were employed in 137 patients (87%), Cervical ultrasonography had an accuracy of 76% (sensitivity, 82%; positive predictive value, 81%), while mediastinal computed tomography was accurate in 76% (sensitivity, 57%; positive predictive value, 80%). Arteriography, venography, and venous sampling were not used. Guided by high‐resolution, small parts ultrasonography, fine‐needle aspiration biopsy confirmed localization of parathyroid tissue in 3 patients. Successful resolution of hypercalcemia occurred in 139 patients (89%) with a mean follow‐up of 22 months. There were no operative deaths; 12 patients (8%) had temporary neuropraxia of the recurrent laryngeal nerve, 6 patients (4%) suffered permanent unilateral paralysis, and 20 (13%) became permanently hypoparathyroid. Two of 4 hypoparathyroid patients who were autotransplanted with cryopreserved parathyroid tissue have become eucalcemic. The results indicate that the disease site is usually in the neck, that noninvasive localizing techniques have superseded arteriography and venous sampling, and that reoperative parathyroid surgery can be performed with rare mortality and acceptable morbidity. Although these refinements have led to improved success, reoperative parathyroid surgery should not be performed by the occasional parathyroid surgeon.

AbstractEighty‐nine patients underwent adrenal exploration for Cushing's syndrome at our institution during the period 1970–1979. On the basis of clinical and histological findings related to the initial adrenal operation, patients were divided into 6 major subgroups: (a) adrenocortical adenoma; (b) adrenocortical carcinoma; (c) proven ectopic ACTH adrenocortical hyperplasia; (d) Cushing's disease; (e) uncertain diagnostic group (probably ectopic ACTH syndrome—site unknown); and (f) “non‐ACTH‐dependent” hypercortisolism due to micronodular hyperplasia.Results of pathologic examination included 25 adrenal adenomas, 15 adrenocortical carcinomas, 40 bilateral adrenocortical hyperplasias, and a single case of micronodular hyperplasia. The postoperative mortality rate was 5.6%, and significant morbidity occurred in 40% of patients. Four of the 5 patients who died within 30 days of operation were in the subgroups with adrenal carcinoma (2 patients) and Cushing's disease (2 patients). Survival in patients with adrenocortical adenomas was significantly decreased at 2 years (relative adrenocortical insufficiency?), but thereafter, it paralleled the expected survival. Patients with the ectopic ACTH syndrome and adrenocortical carcinoma had significantly decreased survival, with only one‐third of carcinoma patients alive 2 years postoperatively. The survival of patients with Cushing's disease was lower than expected at both 5 and 10 years.These results confirm that the diagnosis of patients with endogenous hypercortisolism is usually straightforward and accurate, surgical intervention in this group of patients is still accompanied by significant short‐term mortality and morbidity, and long‐term survival in all subgroups is decreased.

AbstractThe collective term “pancreatic cancer” should be replaced by more specific terminology indicating the histological subtype, e.g., pancreatic ductal adenocarcinoma, ampullary adenocarcinoma, duodenal carcinoma, pancreatic cystadenocarcinoma, cholangiocarcinoma, and malignant islet cell carcinoma. Each of these tumors has its own inherent prognostic and therapeutic characteristics‐95% of ampullary lesions being resectable in contrast to ±10% of pancreatic ductal malignancies and comparable 5‐year survival figures for these two subtypes are 40% and 5%, respectively.Since the results of adjuvant therapy (chemo‐ and/or radiotherapy) have been dismal, the only chance of cure remains resective therapy, either by the performance of a total or subtotal pancreatectomy (Whipple procedure). The operative mortality rate for these procedures at our institution has been 12% and 4%, respectively, with corresponding 5‐year survival rates of 2% and 4%. Despite earlier promise that total pancreatectomy only would be a superior procedure (elimination of the pancreatojejunal anastomosis, wider lymphadenectomy, irradication of multicentric disease), the operation has thus far not fulfilled these expectations.The Whipple procedure remains the resective procedure of choice at our institution—total pancreatectomy being performed principally in those patients in whom a safe pancreatojejunal anastomosis cannot be constructed.Value of early diagnosis in patients with ductal adenocarcinoma does not appear to imply improved prognosis. Energies should be diverted toward etiological identification of this lethal malignancy which is steadily increasing in incidence.

AbstractInsulinoma in patients with multiple endocrine neoplasia (MEN) is a rare condition that because of its usual multicentricity presents difficulties not encountered in sporadic patients. In contrast to gastrinoma, which is the most common pancreatic neoplasm associated with MEN I, malignancy and duodenal tumors are much less common for patients with insulinomas, and excellent palliative medication is not available. Accordingly, there is a much greater reliance on surgical therapy for this group of patients. Between 1970 and 1991 a total of 19 patients had surgical treatment of MEN I‐related insulinoma. Each patient had hyperinsulinemic hypoglycemia. One patient, with extensive metastases, had unresectable disease. Of the remaining 18, there were 16 (89%) multiple pancreatic tumors. Tumors were located in the neck, body, or tail in 17 cases, 10 of whom also had tumors in the head. Pancreatic resections performed were 1 total, 12 subtotal (7 also had enucleation of tumors from the pancreatic head), and 5 limited distal resections and/or enucleation (conservative resection). There was no operative mortality. One patient developed pancreatitis, fistula, and diabetes following subtotal resection and enucleation. Postoperative cure was achieved in 17 of 18 cases. Recurrent disease occurred in 2 of 5 conservative resections compared to 0 of 12 subtotal resections, with median follow‐up times of 10.4 and 10.3 years, respectively. During the follow‐up period, four patients died, possibly all due to MEN I‐related conditions. Hyperinsulinism in MEN I is associated with the occurrence of multiple, usually benign, pancreatic islet cell tumors, and surgery is an effective treatment modality. Surgical management should be guided by two principles: total removal of gross disease and safe prophylactic pancreatic resection. Acceptable morbidity, low recurrence rates, and a low risk of inducing diabetes lead us to recommend routine subtotal distal pancreatectomy. Meticulous evaluation of the pancreatic head using intraoperative ultrasonography to locate tumors and to locate the pancreatic duct, followed by precise enucleation of residual tumors, is essential to ensure cure.

AbstractAmiodarone‐associated thyrotoxicosis (AAT) is often poorly tolerated owing to underlying cardiac disease, and it is frequently refractory to conventional medical treatment. The goal of this study was to describe the patient characteristics, management, and outcomes of all the patients treated surgically for AAT at a single institution. We conducted a retrospective chart review of all patients managed surgically for AAT (April 1985 through November 2002) at the Mayo Clinic in Rochester, Minnesota. Altogether, 29 men and 5 women, ages 39 to 85 years (median 60 years), treated with amiodarone for 3 to 108 months underwent near‐total or total thyroidectomy. Frequent symptoms were worsening heart failure, fatigue, weight loss, and tremor. Altogether, 12 patients failed medical management of their AAT, and 21 received no preoperative medical therapy. One patient had been successfully managed medically but required definitive treatment. Common indications for operation were the need to remain on amiodarone, cardiac decompensation, medically refractory disease, and severe symptoms, both hyperthyroid and cardiac, necessitating prompt resolution. The median ± SD American Society of Anesthesiologists (ASA) classification (1 = healthy through 5 = moribund) was 3.00 ± 0.58. A total of 27 specimens had histology consistent with AAT. Complications included death (n = 3), rehospitalization (n = 3), symptomatic hypocalcemia (n = 2), pneumonia (n = 2), cervical hematoma (n = 1), prolonged ventilatory wean (n = 1), and stroke (n = 1); one patient developed hypotension, adult respiratory distress syndrome, and sepsis. Of the 31 surviving patients, 25 (80%) remained on amiodarone postoperatively. The median follow‐up was 29 months, at which time all surviving patients were free of hyperthyroid symptoms. Thyroidectomy is an effective treatment for AAT but has a high incidence of perioperative morbidity and mortality. The cardiovascular co‐morbidities and high operative risk in this group of patients may account for the increased complication rate.

AbstractSelected patients with primary hyperparathyroidism (pHPT) who have a positive preoperative sestamibi scan can be managed safely and successfully with a focused cervical exploration without either adjuvant intraoperative parathyroid hormone (PTH) monitoring or use of a gamma probe. This article reports a retrospective analysis of a consecutive series of patients surgically treated at a tertiary referral center. From August 1998 to August 2002, 100 patients (68 women, 32 men; mean age 63 years [range: 29–89 years]) underwent a focused cervical approach without intraoperative PTH monitoring or use of the gamma probe after perioperative sestamibi injection. The study group comprised 9% of all patients (n = 1063) undergoing cervical exploration for pHPT during the study period. Ninety patients underwent an initial exploration, and 10 others underwent repeat cervical exploration following prior parathyroid (n = 7) or thyroid (n = 3) operation. Sestamibi scanning correlated with one enlarged parathyroid gland in all patients. Other enlarged glands were, however, not demonstrated in three patients (true positive = 97%; false negative = 3%). The single enlarged glands excised in all patients had a mean weight of 795 mg (range: 90–3640) and were histologically compatible with an adenoma. Postoperatively, 97% of patients were eucalcemic. Three patients remained hypercalcemic (3%). Of the three patients with persistent hypercalcemia, one underwent successful re‐exploration with excision of a 500 mg second adenoma, whereas the other two patients (with confirmed familial HPT) remained hypercalcemic. Mean hospitalization was 0.5 days (range: 0–3 days). There was no operative mortality. No patients had permanent hypocalcemia. Postoperative morbidity occurred in three patients: two self‐limiting cervical hematomas and one permanent vocal cord paralysis. Selected patients with pHPT due to single‐gland disease and an unequivocally positive preoperative sestamibi scan can safely and successfully be managed with a focused unilateral cervical exploration without either intraoperative PTH monitoring or use of the gamma probe. Further experience with this surgical approach seems warranted to determine the overall cure rate, operative morbidity, and the sensitivity and specificity of preoperative localization studies.