[Results of systematic subtotal parathyroidectomy with thymectomy for tertiary hyperparathyroidism after renal transplantation - 70 patients]. / Résultats de la parathyroïdectomie subtotale de principe associée à une thymectomie systématique pour le traitement de l'hyperparathyroïdisme tertiaire après transplantation rénale chez 70 patients.

BACKGROUND: Due to the relatively small number of patients involved, there is currently no consensus on what operation should be performed in patients with tertiary hyperparathyroidism after renal transplantation. METHOD: Retrospective analysis of the 70 patients with tertiary hyperparathyroidism who all underwent subtotal parathyroidectomy with transcervical thymectomy in the same institution between 1978 and 2003. RESULTS: The delay between transplantation and parathyroidectomy was 4,1+/-4,3 years. Follow up was available for all patients. Mean follow-up was 5,6+/-5 years. Glomerular filtration rate (GFR) was 53+/-21 ml/min at parathyroidectomy and 42+/-29 ml/min at follow-up [<30 ml/min in 26 patients (37%), 30 - 60 ml/min in 25 patients (36%) et>60 ml/min in 19 patients (27%)]. One patient was successfully reoperated for persistent tertiary hyperparathyroidism during follow-up. No patient was hypercalcemic at follow-up. Four patients with a GFR<30 ml/min had a PTH level>fourfold normal values (6%) without signs or symptoms of hyperparathyroidism. One patient was hypocalcemic (1,5%) and two patients were normocalcemic with undetectable or infranormal PTH level (3%) under oral vitamin D and calcium medication. CONCLUSION: This approach permits not only to cure the majority of patients with tertiary hyperparathyroidism but also to avoid recurrence when the renal function declines. When medical management has failed, we recommend systematic subtotal parathyroidectomy with thymectomy for patients with tertiary hyperparathyroidism and this should usually be performed during the second year after transplantation.

Subtotal parathyroidectomy with thymectomy for autonomous hyperparathyroidism after renal transplantation.

BACKGROUND: There is currently no consensus on the operation that should be performed in patients with tertiary hyperparathyroidism (HPT) after renal transplantation. METHODS: : A retrospective analysis of 70 patients with tertiary HPT who underwent subtotal parathyroidectomy with transcervical thymectomy was performed. RESULTS: Mean (s.d.) follow-up was 5.6(5.0) years. Mean (s.d.) glomerular filtration rate (GFR) at follow-up was 42(29) ml/min and was less than 30 ml/min in 26 patients (37 per cent), 30-60 ml/min in 25 (36 per cent) and more than 60 ml/min in 19 (27 per cent). One patient had persistent disease and was cured after reoperation. No patient was hypercalcaemic. Four patients (6 per cent) with a GFR below 30 ml/min had a parathyroid hormone (PTH) level more than four times the normal value without any signs or symptoms of secondary HPT. One patient (1 per cent) was hypocalcaemic and two (3 per cent) were normocalcaemic, with undetectable or below-normal PTH levels while receiving oral vitamin D and calcium medication. CONCLUSION: Systematic subtotal parathyroidectomy associated with thymectomy is effective in treating most renal transplant recipients with tertiary HPT and also minimizes the recurrence of HPT in patients with declining renal function.

The effect of calcium channel blockers on outcome following the surgical treatment of phaeochromocytomas and paragangliomas.

The peri-operative management of patients undergoing surgery for phaeochromocytoma or paraganglioma with calcium channel blockers (CCB) and their impact on postoperative morbidity and mortality were studied. The medical records of 105 patients undergoing surgery between 1991 and 2002 were analysed retrospectively. In all patients, the calcium channel blocker nicardipine was used for the peri-operative management of haemodynamic changes. Sixty-five patients (61.9%) showed transient intra-operative hypertension. Systolic blood pressure (SBP) > 220 mmHg and SBP > 180 mmHg for > 10 consecutive minutes was observed in 14 (13%) and four patients (2.8%), respectively. SBP < 80 mmHg for > 10 consecutive minutes occurred in 13 patients (12.3%). Eleven patients (10.4%) developed postoperative complications and three patients died (2.8%). The median (range) ICU and hospital length of stay were, respectively, 1 (0-7) day and 10 (2-35) days. The sole use of calcium channel blockers for the peri-operative management of phaeochromocytoma and paraganglioma resection does not prevent all haemodynamic changes; however, its use was associated with a low morbidity and mortality.

Intraoperative decay profile of intact (1-84) parathyroid hormone in surgery for renal hyperparathyroidism--a consecutive series of 80 patients.

BACKGROUND: The utility of intraoperative parathyroid hormone (PTH) monitoring is unclear in the surgical management of renal hyperparathyroidism. Our goal was to define the normal pattern of decay during operation for renal hyperparathyroidism by using the rapid intact (1-84) parathyroid hormone (PTH) assay. METHODS: Eighty consecutive patients underwent neck exploration for renal hyperparathyroidism. Intact PTH levels were monitored with a rapid immunochemiluminometric assay. Samples were assayed at the induction of anesthesia, after dissection before resection, and 20 and 40 minutes after resection. Follow-up ranged from 3 to 24 months. RESULTS: Twenty minutes after resection, PTH levels remained many-fold supranormal. Seventy-seven patients (96%) were cured. Of these, 75 patients (94%) had PTH decay of more than 50% from the preoperative level; 74 (99%) were cured. Only 1 of 3 patients (33%) in whom the PTH level decreased less than 40% from the preoperative level was cured. Two patients had intermediate values and both were cured. CONCLUSIONS: The intraoperative decay of PTH during operation for renal hyperparathyroidism is slower than for patients with normal renal function. However, 20 minutes after resection, a decline to less than 50% of the preoperative level predicts cure, while a level greater than 60% predicts failure.

Supernumerary parathyroid glands: frequency and surgical significance in treatment of renal hyperparathyroidism.

Supernumerary parathyroid glands (SPGs) are found in 13% of random autopsies. The high incidence of SPGs could explain the persistence or trigger recurrence of renal hyperparathyroidism after surgery. The aim of this study was to assess the frequency and clinical relevance of SPG in patients operated on for renal hyperparathyroidism (HPT). In this retrospective study we reviewed the medical records of 290 patients with renal HPT who were initially treated in our department. We examined the anatomic and pathologic findings during cervical surgical exploration and the outcome of HPT during follow-up. SPGs were identified in 87 patients (30%) during the initial cervicotomy, corresponding to intrathymic parathyroid cell islets (one to four) in 70 cases and to extrathymic SPG in 17 patients. Among 260 patients available for follow-up, 11 experienced persistent HPT (4%), and 34 developed recurrent HPT (13%). A total of 25 patients were reoperated on, and SPGs were responsible for 4 of 8 cases of persistent HPT and 4 of 17 cases of recurrent HPT, representing an overall frequency of 32%. The anatomic distribution of SPGs found during reoperations included thymus, retroesophageal grove, carotid sheath, and mediastinum. SPGs are thus present in 30% of patients with renal HPT and are situated mainly in the thymus. Thymectomy should be performed routinely during the first surgical exploration to prevent recurrences arising from anterior mediastinal glands. SPGs were also responsible for 32% of persistent or recurrent HPT. In that setting, frankly ectopic SPGs are not rare, and preoperative imaging appears highly desirable prior to embarking on surgical reexploration.

Tumour size: the only predictive factor for 131I MIBG uptake in phaeochromocytoma and paraganglioma.

BACKGROUND: 131I Meta-iodobenzylguanidine (131I MIBG) scintigraphy can detect chromaffin tumours with a high specificity but its sensitivity remains limited. In this study, the influence of clinical features and tumour pathology on the results of 131I MIBG of patients with phaeochromocytomas and paragangliomas was examined. METHODS: The records of 104 patients operated on for chromaffin tumours who had pre-operative 131I MIBG were reviewed. Demographic data, clinical features, biochemical results and pathology of tumours were analysed. The size of the tumour was assessed by the three measured diameters of the specimen and its calculated volume. Univariate relationship between tumours' characteristics and the results of 131I MIBG were examined. RESULTS: Out of 119 tumours, 104 (87%) were detected by 131I MIBG. Mean +/- SD largest diameter and volume of the tumours were 6.0 +/- 2.4 cm (range 0.8-11.5 cm) and 68 +/- 74 cm3 (range 0.2-421 cm3). Results of 131I MIBG were significantly correlated with both the largest diameter of the tumour (P < 0.01) and by its volume (P < 0.001). 131I MIBG was negative in 35.5% of tumours < 20 cm3 and in 2.6% of those < 20 cm3. No other criteria were correlated with positive 131I MIBG, including aetiology, pathology or catecholamine secretion pattern. CONCLUSIONS: The result of 131I MIBG scanning in patients with chromaffin tumours is significantly correlated with the size of the tumour.

Current perspectives in the surgery of multiple endocrine neoplasias.

Multiple endocrine neoplasia is an uncommon but fascinating condition. In this review we examine the multifaceted presentations, the diagnostic challenges and different management strategies for various syndromes. This is an attempt to share our knowledge and experience towards achieving better outcomes in the management of the diseases.

Essential hypertension: first reason for persistent hypertension after unilateral adrenalectomy for primary aldosteronism?

BACKGROUND: Despite cure of primary aldosteronism by surgical resection, hypertension persists postoperatively in 30% to 50% of patients. The aim of this study was to determine factors influencing long-term outcome of blood pressure after unilateral adrenalectomy for primary aldosteronism. METHODS: Records of 100 patients who underwent unilateral adrenalectomy for primary aldosteronism from 1970 through 1997 were reviewed. Patients were distributed in 2 groups according to whether blood pressure was normal (criteria of World Health Organization). Clinical, biochemical, and pathologic data were compared. RESULTS: All patients were biochemically cured. Blood pressure was normal in 56 patients and improved in 44 (mean follow-up, 69 and 59 months). Persistent hypertension correlated with age, known duration and seriousness of preoperative hypertension, family history of hypertension, no preoperative response to spironolactone, and contralateral adrenal hypertrophy. Gender, surgical approach, and pathologic findings were not predictive factors of blood pressure outcome. The prevalence of hypertension was almost the same in these postoperative patients as the prevalence of essential hypertension in a random population of the same age. CONCLUSIONS: Early unilateral adrenalectomy allows cure or improvement of hypertension in all patients with primary aldosteronism induced by unilateral excessive source of aldosterone secretion regardless of the pathologic findings. Persistent hypertension suggests that coexisting essential hypertension is present.

2-Methoxyisobutylisonitrile probe during parathyroid surgery: tool or gadget?

The success of parathyroid surgery is determined by the identification and removal of all parathyroid tumors. Parathyroid tumors accumulate and retain 2-methoxyisobutylisonitrile (MIBI) labeled with technetium-99m. Intravenous injection of this radiopharmacon prior to parathyroid surgery allows identification of parathyroid tumors with a hand-held gamma detector. To assess the value of this technique, a case-control study was performed with 62 patient having nuclear-guided parathyroidectomy and 60 patients having conventional parathyroid explorations. The sensitivity rates of the MIBI probe in single and multiple gland disease were 84.6% and 63.0%, respectively. Rates of success, temporary and permanent hypoparathyroidism, and injury of the recurrent laryngeal nerve were similar in patients who underwent probe-guided surgery and those who had conventional surgery. In conclusion, although the MIBI probe appears to be a valuable tool in parathyroid surgery, its use has not improved the outcome of such surgery at our institution.

Effectiveness and limits of preoperative imaging studies for the localisation of pheochromocytomas and paragangliomas: a review of 282 cases. French Association of Surgery (AFC), and The French Association of Endocrine Surgeons (AFCE).

OBJECTIVE: To find out the optimal strategy for the preoperative location of pheochromocytomas and paragangliomas. DESIGN: Retrospective study. PATIENTS: 282 patients operated on for histologically confirmed pheochromocytoma in France between 1980 and 1991, the past decade. MAIN OUTCOME MEASURES: The results of imaging procedures, i.e. computed tomography (CT), (131)I meta-iodobenzylguanidine scintigraphy (MIBG) and magnetic resonance imaging (MRI) were reviewed. RESULTS: Pheochromocytomas were sporadic in 206 (73%). They were unilateral in 189 (67%), bilateral in 54 (19%) and extra-adrenal in 39 (14%). Overall sensitivity of the studies was 89% for CT, 98% for MRI, and 81% for (131)I-MIBG. In unilateral adrenal lesions sensitivity were 100% for CT and MRI, and 88% for (131)I-MIBG; in bilateral lesions 66% for CT, 100% for MRI, and 62% for (131)I-MIBG; in extra-adrenal lesions 64% for CT, 88% for MRI, and 64% for (131)I-MIBG. CONCLUSION: The accuracy with which pheochromocytomas and paragangliomas can be visualized questions nowadays the routine use of abdominal approach. In selected cases of sporadic unilateral chromaffin tumours, a posterior, lateral, or even laparoscopic approach should be considered.

Questionability of the benefits of routine laparotomy as the surgical approach for pheochromocytomas and abdominal paragangliomas.

BACKGROUND: Improvement of preoperative imaging of pheochromocytomas and abdominal paragangliomas may render routine laparotomy questionable as the surgical approach of choice for these lesions. METHODS: We studied the records of 100 patients with chromaffin tumors who underwent abdominal exploration. The disease was familial in 28 patients and was malignant in 19. Seventy-five patients had intraadrenal disease (bilateral in 13). Computed tomography (CT), metaiodobenzylguanidine (MIBG) scintigraphy, and magnetic resonance imaging (MRI) were performed since 1979, 1984, and 1987 in 97, 73, and 43 patients, respectively. False-positive and false-negative results were defined as any discrepancy between imaging results and surgical findings. RESULTS: Overall accuracy of preoperative localization was 85% with CT scan, 77% with MIBG scintigraphy, and 86% with MRI. In unilateral pheochromocytoma, accuracy was 94% with CT scan, 80% with MIBG scintigraphy, and 96% with MRI. When all three studies were performed (n = 38), overall accuracy was 97% and only one extraadrenal tumor in a patient with familial pheochromocytoma was overlooked. CONCLUSIONS: The outstanding accuracy of available imaging techniques questions the strategy of routine laparotomy for sporadic and seemingly benign pheochromocytomas, favoring more elective approaches such as the posterior approach or laparoscopy.

Parathyroid incidentalomas in normocalcemic patients during thyroid surgery.

The aim of this study was to compare the clinical, biochemical, and pathologic findings of normocalcemic patients with macroscopically enlarged parathyroid tissue identified at thyroid surgery with those of patients treated surgically for preoperatively proved primary hyperparathyroidism (PHPT). The records of 28 patients with incidental parathyroid enlargement and 533 patients with PHPT were reviewed to compare age, sex, serum calcium and phosphate, intact parathyroid hormone (iPTH), parathyroid weight, number of diseased glands, cell and histologic types, PTH content, and cure rate. Incidentally found lesions were lighter and developed in younger patients. Biochemistry and pathology found them to be less hyperfunctioning. Sex, number of diseased glands per patient, and cell type were not different. PTH content was low in the incidental lesions. Incidentally discovered enlarged parathyroid glands are mildly hyperfunctioning at the time of discovery. They may represent an early stage of lesion responsible for overt PHPT. In the absence of knowledge concerning their significance and evolution, we recommend that enlarged parathyroids found during the course of a thyroid operation be removed.

"The" pheochromocytoma: a benign, intra-adrenal, hypertensive, sporadic unilateral tumor. Does it exist?

This study aims to examine the frequency of the pheochromocytoma (pheo), defined as a "benign, intra-adrenal, hypertensive, sporadic, unilateral tumor." Three large series amounting to 310 subphrenic chromaffin tumors operated over periods of 17, 23, and 41 years, respectively, have been reviewed. Among those combined 310 pheos, 48 (15.5%) were malignant (i.e., metastatic) and 262 (84.5%) were benign; 42 (13.5%) were ectopic (35% of malignant tumors versus 9.5% of benign tumors); 230 (74.2%) were hypertensive (74% of benign tumors versus 73% of malignant tumors); 29 (9.4%) were bilateral, including 23 patients with a family history; 41 (13.2%) of patients had MEN II type A or B syndrome; and 20 (6.5%) occurred in a phacomatosis setting. Some of the patients had a non-MEN family history of pheo (n = 5), seemingly sporadic hyperparathyroidism (n = 4), or other associated neuroendocrine tumor (n = 9). Sometimes several of these features were combined. Finally 125 (40.3%) cases fitted the classic description of the tumor, 47.0% at the time of initial presentation and 40.3% at the end of follow-up. Late occurrence of metastases or metachronous diagnosis of familial disease make lifelong follow-up mandatory. Genetic studies may be indicated in pheochromocytoma patients.

Safety of the posterior approach in adrenal surgery: experience in 105 cases.

BACKGROUND: At the advent of laparoscopic adrenalectomy when it was timely to reappreciate the results of time-honored procedures, we reviewed the cases of 105 patients who underwent adrenalectomy through the posterior approach. METHODS: Between 1970 and 1992 among 331 patients, 105 underwent adrenalectomy through the posterior approach (0 of 111 pheochromocytomas, 48 of 64 Conn's disease, 37 of 57 Cushing's disease, 2 of 20 virilizing-feminizing tumors, 13 of 61 nonsecreting adrenalomas, 3 of 12 metastases, 2 of 6 cysts). Adrenalectomy was bilateral in 20 cases. Among 86 tumors, 28 (32.6%) were larger than 5 cm in diameter, none exceeding 10 cm. Posterior approach, initially performed in the prone position, was used in the lateral position for the last 40 patients with tumors. A hockey-stick incision was made on the twelfth or eleventh rib, which was resected. RESULTS: During operation no patient died; one minimal caval tear and 13 pleural tears occurred and were sutured, with two pleural drainages; six patients received blood transfusion. Average operative time was 132 minutes (range, 45 to 290 minutes). After operation one patient died of iatrogenic sepsis, average time to ambulation was 1.5 days, and average in-hospital stay was 7.6 days (range, 1 to 21), which after the fourth day was mostly justified for nonsurgical reasons. From 1990 through 1992, 37 of 38 patients were walking the day after operation and average postoperative stay dropped to 4.5 days (range, 1 to 7 days). CONCLUSIONS: Adrenalectomy through the posterior approach is safe and allows early postoperative discharge.

Multiglandular disease in seemingly sporadic primary hyperparathyroidism revisited: where are we in the early 1990s? A plea against unilateral parathyroid exploration.

BACKGROUND: Most of the available data on multiglandular disease (MGD) originate from long-term series. The spectrum of the disease has changed now because of earlier diagnosis. We decided to assess the current frequency of MGD in seemingly sporadic primary hyperparathyroidism. METHODS: MGD was defined as the finding that more than one grossly enlarged gland weighed more than 50 mg and the rim of normal parathyroid tissue was diagnostic of adenoma. Nine hundred eight consecutive neck explorations for hyperparathyroidism were performed, 624 before 1989 and 284 between 1989 and 1991, and studied retrospectively. Since 1989, oil Red O staining has been done to assess hyperfunction. RESULTS: When multiple endocrine neoplasia and non-multiple endocrine neoplasia familial cases of hyperparathyroidism are excluded, the frequency of MGD in seemingly sporadic primary hyperparathyroidism, 17.7% before 1989, is still 14% since then, including a 3% frequency of true multiple adenomas. With more restrictive criteria, which exclude the second enlarged gland if it weighed less than 100 mg, if it had no rim, and if oil Red O staining was negative, the frequency of MGD in sporadic hyperparathyroidism has remained 11% since 1989. Unilateral exploration had missed 78% of second enlarged glands, and preoperative imaging studies, when performed, showed more than one gland in only two of 22 cases. CONCLUSIONS: Routine bilateral neck exploration is recommended in primary hyperparathyroidism because of a current frequency of MGD of at least 11%.

Urodynamics in patients with pheochromocytoma: a peri-operative study of 10 cases.

Alpha receptors have been demonstrated in the bladder neck, and urinary retention may be the presenting symptom in an occasional pheochromocytoma patient. This prompted us to define the urodynamic profile in pheochromocytoma patients. Ten patients were studied. Except for 2 patients, all tumors secreted norepinephrine either alone (n = 4) or mixed (n = 4). Urodynamic studies (uroflowmetry, cystometry, profilometry, response to alpha-adrenergic agents) were performed with Urodyn 5000 chain (DANTEC) connected to a water perfused Bohler's catheter. Profilometry was done according to the Brown and Wickham technique. Normal values were those of the International Continence Society. Alpha blocker test was done by intravenous injection of thymoxamine (0.5 mg/kg) and was considered as positive if urethral closure pressure (UCP) decrease was greater than 30% after 10 minutes. Ten patients had a pre-operative study, omitting alpha-blocker test in 1 patient; 5 patients consented a postoperative study. Pre-operatively we could demonstrate: 1) Increased UCP in 8 of 10 patients, regardless of the secretory pattern; 2) Response to alpha-adrenolytic agents in 7 of 9 patients; and postoperatively: 3) Good correlation between a positive alpha-blocker test and a decrease in urethral pressure in 3 of 5 patients. Urodynamics in pheochromocytoma patients show a typical alpha-adrenergic pattern and may explain bladder dysfunction as a presenting symptom.