Farley, David R.

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.

AbstractAlthough palpable axillary lymphadenopathy is most often related to benign disorders, it may be a harbinger of an underlying advanced malignancy. Fewer than 1% of patients diagnosed with breast cancer initially present with axillary metastases as their only clinical manifestation. This study represents the Mayo Clinic experience with women with occult breast cancer who presented with axillary metastases. Among a group of 44 women undergoing axillary biopsy for a palpable mass, 35 had histologic evidence of a metastatic carcinoma of primary breast cancer origin. Our retrospective analysis focused on the workup, identification, treatment, and outcome of these 35 women. All 35 women had a palpable axillary nodule, no dominant breast mass, and normal mammograms and chest radiographs. Histologic analysis of the axillary node revealed a probable breast cancer in all patients. Long‐term follow‐up was available for 33 patients (mean 71.5 months, range 4–252 months). Mastectomy was performed as part of the primary treatment in 18 patients (51%), and among them a primary breast tumor was found in 6 (33%). Of the women who underwent mastectomy (n = 18) and for whom follow‐up data were available (n = 17), six developed tumor recurrence (35%), and four died of their disease (24%). Among the patients who did not undergo mastectomy (n = 17) as their primary treatment, follow‐up revealed that 12 (of 16) patients developed recurrent disease (75%), and 11 patients died of breast carcinoma (69%). Despite extensive clinical and radiologic evaluation, a primary tumor was not initially located in any of the 35 women with axillary metastases from a presumed occult breast carcinoma. As a consequence of aggressive surgical intervention, six primary breast cancers were located in mastectomy specimens, and those 17 patients followed after mastectomy fared significantly better than the 16 patients without mastectomy (p = 0.047).

Abstract.Duodenal leiomyomas are rare neoplasms and when present are usually asymptomatic. There are, however, isolated case reports of such leiomyomas giving rise to complications including gastrointestinal hemorrhage and obstruction. The purpose of this study was to review the surgical experience with duodenal leiomyomas at a large tertiary referral center. A retrospective review was performed of all patients with histologically proven duodenal leiomyomas encountered from 1975 to 1995. Twelve patients with surgically treated duodenal leiomyomas were identified. There were eight men and four women with a mean age of 57 years (range 20–73 years) at diagnosis. Anemia was the presenting sign in 10 patients (83%). The mean hemoglobin level at presentation was 10.8 ± 2.67 g/dl. Other presenting signs included melena (n= 5) and epigastric tenderness (n= 4). No patient had obstructive symptoms. Upper endoscopy visualized the lesion in 9 of 10 patients, but endoscopic biopsy provided an accurate histologic diagnosis in only two of six patients biopsied. All resected specimens underwent frozen section and permanent histopathologic analysis. Local excision was performed in eight patients, as benignancy was anticipated at surgical exploration and frozen section findings. Two patients underwent segmental duodenal resection with primary end‐to‐end anastomosis, and two patients underwent a Whipple procedure. There was no perioperative mortality. Morbidity was minimal. There was no tumor recurrence during a median follow‐up of 8.4 years. Gastrointestinal blood loss is the most common complication of duodenal leiomyomas requiring surgical intervention. Small symptomatic leiomyomas with benign features may be safely treated with local excision.

Abstract Background Papillary thyroid cancer (PTC) has a generally favourable prognosis, but elderly patients with distant metastases or extracapsular invasion fair poorly. In small studies with short follow-up, young patients presenting with such extracapsular invasion and pulmonary metastases have faired well. This retrospective study was undertaken to clarify the long-term prognosis of such patients with advanced PTC. Methods Twenty-one children and young adults (median age 14 (range 6–20) years) presenting with PTC and pulmonary metastases were treated at a single institution between 1947 and 1998. Mean maximal tumour diameter was 4·65 cm. Initial surgical treatment consisted of total thyroidectomy (n = 16), subtotal thyroidectomy (n = 4) and isthmectomy (n = 1), coupled with a variety of lymph node dissections (n = 20). After operation, 19 patients were treated with ablative and incremental doses of iodine-131 until disease free. All patients were placed on suppressive thyroid hormone after operation. Mean length of follow-up was 21 years (range 3 months to 47 years). Follow-up was less than 3 years in four patients. All patients have undergone post-treatment radionucleotide and radiological evaluation. Results Nine of the 21 patients developed recurrent disease. The risk of recurrence at 5 years was 39 (95 per cent confidence interval 14–57) per cent. Eight had cervical lymph node recurrence and no patient developed recurrent pulmonary disease. All patients with identifiable recurrent disease underwent selective lymph node resection, which involved multiple resections in four. At follow-up, 18 patients remain completely free of disease, one patient has recurrent cervical node disease and two patients have died. The disease-free survival at 5 years was 95 (95 per cent confidence interval 86–100) per cent. Cause-specific death occurred in a single patient who died from extensive local disease at age 29 years after 12 years of multiple cervical lymph node recurrences. Conclusion A stepwise treatment approach including total thyroidectomy, high-dose iodine-131 treatment and early surgical reintervention for suspected local recurrent disease allows long-term survival and frequent ‘cure’ for young patients with PTC and concomitant pulmonary metastases.

Abstract Background Calciphylaxis is a rare, painful, life-threatening problem of cutaneous necrosis and refractory healing seen in patients with uraemia and secondary hyperparathyroidism. The pathogenesis involves abnormalities in calcium and phosphorus metabolism, and acute deposition of calcium in tissues. Methods The clinical course of 16 patients diagnosed with calciphylaxis at this institution from 1994 to 1998 was reviewed. Results Fourteen women and two men, with a mean age of 56 (range 39–70) years, presented with chronic renal disease of various causes, hyperparathyroidism and characteristic skin lesions. All patients underwent intensive medical therapy, including haemodialysis (n = 16), parathyroidectomy (PTX) (n = 7) and skin debridement of cutaneous lesions (n = 8). Mean preoperative serum values in surgical (PTX) versus non-surgical patients were: calcium 9·9 and 9·3 mg dl−1 (P = 0·25); phosphorus 5·8 and 4·9 mg dl−1 (P = 0·04); calcium–phosphorus product 61·6 and 45·2 (P = 0·03); and parathyroid hormone (PTH) 56·8 and 5·9 pmol l−1 (P = 0·0001) respectively. Mean postoperative values for calcium (8·7 mg dl−1), phosphorus (4·2 mg dl−1), calcium–phosphorus product (30·5) and PTH (4·0 pmol l−1) in surgical patients changed significantly (P < 0·05). Median overall survival was 9·4 months with 15 patients now deceased. Median survival was 14·8 and 6·3 months for PTX versus no PTX (P = 0·22), 14·1 and 6·1 months for skin debridement versus no debridement (P = 0·08), 12·4 and 6·6 months for proximal versus distal skin lesions (P = 0·60), and 6·5 and 13·9 months for diabetic versus non-diabetic patients was (P = 0·11). Conclusion Calciphylaxis appears to have a female preponderance with a very dismal prognosis. A multidisciplinary approach using frequent haemodialysis to normalize serum calcium and phosphorus levels, and local debridement of skin lesions seems prudent. PTX cannot be recommended routinely in all such patients unless severe secondary hyperparathyroidism with poorly controlled metabolic disturbance mandates surgery. The exact indications for PTX for calciphylaxis remain unclear.

Abstract Spigelian hernia is a rare partial abdominal wall defect. The frequent lack of physical findings along with vague associated abdominal complaints makes the diagnosis elusive. A retrospective review of Mayo Clinic patients was performed to find all patients who had undergone surgical repair of a Spigelian hernia from 1976 to 1997. Patients were scrutinized for presentation, work‐up, therapy, and outcome. The goal of this study was to obtain long‐term outcome. The study was set in a tertiary referral center. There were 76 patients in whom 81 Spigelian hernias were repaired. Symptoms most commonly included an intermittent mass (n = 29), pain (n = 20), pain with a mass (n = 22), and bowel obstruction (n = 5). Five patients were asymptomatic. Preoperative imaging was performed in 21 patients and correctly diagnosed the hernia in 15. Spigelian hernias were repaired by primary suture closure (n = 75), mesh (n = 5), and laparoscopic (n = 1) techniques. Eight patients (10%) required emergent operations. Thirteen hernias (17%) were found to be incarcerated at the time of the operation. Overall mean follow‐up for the 76 patients was 8 years, with three hernia recurrences identified. Spigelian hernia is rare and requires a high index of suspicion given the lack of consistent symptoms and signs. An astute physician may couple a proper history and physical examination with preoperative imaging to secure the diagnosis. Mesh and laparoscopic repairs are viable alternatives to the durable results of standard primary closure. Given the high rate of incarceration/strangulation, the diagnosis of Spigelian hernia is an indication for surgical repair.

The trend toward limited exploration for primary hyperparathyroidism (1° HPT) has stemmed from advances in sestamibi scanning, gamma probe technology, and intraoperative parathyroid hormone monitoring (iPTH). Prior to widespread application, directed parathyroidectomy must be shown to meet the high standards of conventional cervical exploration. In this prospective, nonrandomized trial, results of parathyroidectomy utilizing the gamma probe, iPTH, or neither technique were evaluated. Altogether, 150 patients underwent parathyroidectomy utilizing the gamma probe (n = 50), iPTH (n = 50), or neither technique (n = 50). Each group was evaluated for operating time, length of hospitalization, cure rate, morbidity, mortality, and cost. A telephone survey was conducted with 25 patients in each group (n = 75) to address patient satisfaction. The gamma probe localized the abnormal gland in 66% of patients and confirmed cure intraoperatively in 84%. In the iPTH group, a more than 50% decrease from baseline occurred in 98%. The mean operating times were 76, 84, and 90 minutes, respectively (p = 0.16); and the mean length of hospitalization was 1 day. The biochemical cure rates were 98%, 100%, and 96%, respectively (p = 0.17). Total costs were $4476, $3918, and $3905, respectively. A total of 96% of patients in all three groups were "very satisfied" with their surgical procedure. Directed parathyroidectomy utilizing the gamma probe or iPTH assay does not significantly alter the operating time, length of hospitalization, cure rate, morbidity, mortality, or patient satisfaction when compared to conventional exploration in our practice. The probe is more expensive and was not a consistently reliable tool for localizing parathyroid pathology. PTH monitoring reliably predicts cure intraoperatively.

AbstractWe currently recommend excision of adrenal incidentalomas ≥ 4 cm in size and all hormonally active tumors. The optimal management and follow‐up of smaller nonfunctioning tumors are controversial. The aim of this study was to determine the clinical outcome of a well defined population of patients with incidentalomas followed without operative intervention. The study group comprised 231 patients, identified from the records of abdominal or thoracic computed tomographic (CT) scans performed between 1985 and 1989. The primary outcome variable analyzed was survival. Follow‐up was obtained by office records, telephone contact, or letter. There were 101 male and 130 female patients with a mean age at diagnosis of 64 years (range 5–86 years). Most adrenal tumors were unilateral (right 113; left 98); 20 were bilateral. Mean tumor size was 2 cm (range 1–6 cm). In nine (4%) patients the tumor was ≥ 4 cm. Follow‐up [mean 7 years; range 1 month (patient died) to 11.7 years] was complete in 224 (97%) patients. Ninety‐one (39%) patients had one or more additional CT scans performed during the follow‐up period, with only four patients demonstrating a > 1 cm increase in the size of the adrenal mass. Surgical excision of these four lesions identified benign pathology. Eighty‐one (35%) patients died of conditions unrelated to adrenal pathology. No patient developed subsequent adrenal hyperfunction or adrenal malignancy. Within the context of our guidelines, conservative management of adrenal incidentalomas considered benign or nonfunctioning at diagnosis is appropriate. Additional information provided by repeat CT scanning appears to confer limited benefit. This study does not support laparoscopic removal of small, nonfunctional adrenal tumors, as has been suggested.