Surgical treatment of hyperparathyroidism in patients with multiple endocrine neoplasia type 1

HYPOTHESIS: Three-gland parathyroidectomy with trans-cervical thymectomy and cryopreservation is the preferred initial surgical approach for hyperparathyroidism (HPT) in patients with multiple endocrine neoplasia type 1. DESIGN: Retrospective cohort study. SETTING: Tertiary referral center. PATIENTS: Thirty-seven patients with multiple endocrine neoplasia type 1 who underwent 1 or more surgical procedures for HPT from January 1, 1973 to April 30, 2004. RESULTS: At initial parathyroid surgery, 16 (43%) of 37 patients had fewer than 3 parathyroid glands resected (group 1); 16 (43%) had at least 3 but fewer than 4 glands (group 2), and 5 (14%), 4 or more glands (group 3). Follow-up of at least 6 months after initial surgery was complete for 31 (84%) of 37 patients. CONCLUSIONS: Recurrent HPT in patients with multiple endocrine neoplasia type 1 is frequent if fewer than 3 glands are removed at initial parathyroidectomy. Optimal surgical intervention must balance the risk of recurrent hypercalcemia with the morbidity of permanent hypoparathyroidism. Three-gland parathyroidectomy, transcervical thymectomy, and parathyroid cryopreservation constitute our preferred initial surgical procedure.     

Long-term results of less than total parathyroidectomy for hyperparathyroidism in multiple endocrine neoplasia type 1

BACKGROUND: Our aim was to assess long-term results after less than total parathyroidectomy for hyperparathyroidism in multiple endocrine neoplasia type 1. METHODS: Of 1888 patients undergoing operation at our institution for primary hyperparathyroidism between 1972 and 2001, 83 (4.4%) had multiple endocrine neoplasia type 1. Outcome data were available for 79; 66 underwent subtotal parathyroidectomy, 55 (83%) of these with bilateral thymectomy. In 13 patients, only grossly enlarged glands were resected (mean 1.1 per patient) as the syndrome of multiple endocrine neoplasia type 1 was not yet evident or the initial exploration was performed elsewhere. RESULTS: Follow-up has been 48 +/- 51 months (mean + SD). Intraoperative serum PTH assay decay in 20 patients was suggestive of cure in 18 patients, none of whom required reoperation. Nine patients (11%) required reoperation (3 required reoperation twice) after a mean interval of 77 +/- 53 months. Subtotal parathyroidectomy resulted in a lesser reoperation rate than resection of grossly enlarged glands (7% vs 30%, P =.02). At the time of review, 63 patients (80%) were normocalcemic, 10 (13%) hypocalcemic (2 after unsuccessful delayed autograft), and 7% hypercalcemic (none after reoperation). By Kaplan-Meier analysis, the rate of surgical cure (patients who are nonhypercalcemic) is 60% and 51% at 10 and 15 years, respectively. CONCLUSION: Subtotal parathyroidectomy reduces the need for reoperation. Selective reoperation leads to long-lasting biochemic cure.     

Prospective study of surgery for primary hyperparathyroidism (HPT) in multiple endocrine neoplasia-type 1 and Zollinger-Ellison syndrome: long-term outcome of a more virulent form of HPT

BACKGROUND: Primary hyperparathyroidism (HPT) in multiple endocrine neoplasia type 1 (MEN1) patients with Zollinger-Ellison syndrome (ZES) is caused by parathyroid hyperplasia. Surgery for parathyroid hyperplasia is tricky and difficult. Long-term outcome in ZES/MEN1/HPT is not well known. METHODS: Eighty-four consecutive patients (49 F/35 M) with ZES/MEN1/HPT underwent initial parathyroidectomy (PTX) and were followed at 1- to 3-year intervals. RESULTS: Age at PTX was 36 +/- 2 years. Mean follow-up was 17 +/- 1 years. Before PTX, mean Ca = 2.8 mmol/L (normal level (nl <2.5), PTH i = 243 pg/mL (nl <65), and gastrin = 6950 pg/mL (nl < 100). Sixty-one percent had nephrolithiasis. Each patient had parathyroid hyperplasia. Fifty-eight percent of patients had 4 parathyroid glands identified. Nine of 84 (11%) had 4 glands removed with immediate autograft, 40/84 (47%) 3 to 3.5 glands, whereas 35/84 (42%) <3 glands removed. Persistent/recurrent HPT occurred in 42%/48% of patients with <3 glands, 12%/44% with 3 to 3.5 glands, and 0%/55% with 4 glands removed. Hypoparathyroidism occurred in 3%, 10%, and 22%, respectively. The disease-free interval after surgery was significantly longer if >3 glands were removed. After surgery to correct the HPT, each biochemical parameter of ZES was improved and 20% of patients no longer had laboratory evidence of ZES. CONCLUSIONS: HPT/MEN1/ZES is a severe form of parathyroid hyperplasia with a high rate of nephrolithiasis, persistent and recurrent HPT. Surgery to correct the hypercalcemia significantly ameliorates the ZES. Removal of less than 3.5 glands has an unacceptably high incidence of persistent HPT (42%), whereas 4-gland resection and transplant has a high rate of permanent hypoparathyroidism (22%). More than 3-gland resection has a longer disease-free interval. The surgical procedure of choice for patients with HPT/MEN1/ZES is 3.5-gland parathyroidectomy. Careful long-term follow-up is necessary as a significant proportion will develop recurrent HPT.     

Initial failure of surgical exploration in patients with primary hyperparathyroidism

To determine the causes of failures of cervical exploration for primary hyperparathyroidism, we reviewed 892 patients operated on by one surgeon from 1953 to 1990. Twenty-seven patients (3%) remained hypercalcemic or developed hypercalcemia within 6 months of surgery. Of these, five patients had one adenoma removed initially; at reoperation, three patients had a second adenoma that was successfully removed, whereas the other two patients had hyperplasia and required subtotal parathyroidectomies. No enlarged parathyroid glands were identified in 22 patients. Eventually, six patients became normocalcemic spontaneously, seven patients underwent re-exploration with a successful outcome in all but one case, two patients had ectopic hyperparathyroidism associated with carcinoma elsewhere, and seven patients refused reoperation and remain hypercalcemic. The failure rate of surgical exploration for primary hyperparathyroidism can be reduced by systematically exploring all four parathyroid glands. All abnormal parathyroids should be removed with histologic verification. When no abnormal glands are found, localization studies should be performed before re-exploration.     

Subtotal parathyroidectomy as an adequate treatment for primary hyperparathyroidism in multiple endocrine neoplasia type 1

HYPOTHESIS: The most appropriate surgical approach for hyperparathyroidism (HPT) in multiple endocrine neoplasia type 1 remains controversial. It has been advocated that reoperations for recurrent disease are easier to perform after total parathyroidectomy (TP) with autotransplantation than after subtotal parathyroidectomy (SP). In view of our large experience in patients with secondary HPT for whom TP with autotransplantation did not simplify reoperations, SP remains our preferred treatment for patients with HPT and multiple endocrine neoplasia type 1. DESIGN: Retrospective cohort study. SETTING: Tertiary referral medical center. PATIENTS: A total of 29 consecutive patients (22 women, 7 men; mean age, 42.2 years) with multiple endocrine neoplasia type 1 who underwent definitive cervical exploration for HPT. MAIN OUTCOME MEASURES: Temporary and permanent hypocalcemia, pattern of parathyroid disease, and sites and timing of recurrent HPT. Definitive primary surgery included SP in 21 patients, TP with autotransplantation in 4 patients, and less-than-subtotal parathyroidectomy in 4 selected patients. RESULTS: The mean follow-up was 88.5 months (range, 8-285 months). Four patients died during follow-up; 2 of these deaths were related to multiple endocrine neoplasia. No patients had persistent HPT. Temporary hypocalcemia occurred in 12 SP cases (57%), 4 TP with autotransplantation cases (100%), and 0 less-than-subtotal parathyroidectomy cases. Permanent hypocalcemia requiring long-term treatment occurred in 2 SP cases (10%), 1 TP with autotransplantation case (25%), and 0 less-than-subtotal parathyroidectomy cases. Four patients developed recurrent disease, including 1 with SP, 2 with TP with autotransplantation, and 1 with less-than-subtotal parathyroidectomy at 57 months, 197 and 180 months, and 164 months, respectively, representing 14% of all of the patients and 43% of patients with more than 10 years of follow-up. CONCLUSIONS: Recurrent HPT occurs many years after definitive primary surgery (median, 14.3 years). Surgical treatment should therefore aim to minimize the risk of permanent hypocalcemia and facilitate future surgery. When correctly performed, SP fulfills these objectives.     

Results of reoperation for persistent and recurrent hyperparathyroidism.

Between August 1975 and January 1981, 106 patients thought to have persistent or recurrent hyperparathyroidism underwent a total of 108 parathyroid re-explorations at the National Institutes of Health. These 106 patients had a total of 175 previous operations for hyperparathyroidism (156 cervical and 19 mediastinal). Nephrolithiasis (54% of patients) and bone disease (24% o patients) were the predominant symptoms. Arteriographic examination and selective venous sampling provided highly accurate localizing results in 33% of the patients, and were of some help in 64%. The final diagnoses after reoperation and re-evaluation were: single-gland disease in 58 patients, primary nonfamilial hyperplasia in 19 patients, familial hyperplasia in three patients, multiple endocrine neoplasia (MEN) Type I in ten patients, MEN Type II in two, parathyroid carcinoma in four patients, secondary hyperplasia in three patients, and familial hypocalciuric hypercalcemia (FHH) in two patients. The diagnosis was in doubt in five patients. In the 95 patients with unequivocal hyperparathyroidism, not due to parathyroid carcinoma, surgery eliminated hypercalcemia in 91 (96%). Two patients died after operation, one of disseminated candidiasis, and one patient, with an immunodeficiency, of sepsis. Five patients developed temporary, and one permanent, recurrent nerve damage; 41% of the patients were hypocalcemic, at the time of discharge from the hospital.     

Primary juvenile hyperparathyroidism. Report of 24 cases

AIM OF THE STUDY: Primary hyperparathyroidism usually affects elderly patients. Juvenile primary hyperparathyroidism is rare, and raises diagnostic and prognostic problems. The aim of this retrospective study on 24 patients is to establish clinical, histological, and therapeutic features of juvenile primary hyperparathyroidism. PATIENTS AND METHODS: From 1986 to 2001, 673 patients were treated for primary hyperparathyroidism in our department. Twenty four patients were younger than 30 years old (3.5%). There were 14 women and 10 men. Mean age was 23 year (14-30). Clinical manifestations, pathologics findings and postoperative results were studied. RESULTS: Sixteen patients presented a sporadic form of primary hyperparathyroidism with a single adenoma. Clinical manifestations were renal symptoms in 11 cases and acute hypercalcemia syndrome in 2 cases. Seven patients had a NEM I syndrome: parathyroid lesions were 6 hyperplasia and one adenoma. A 27 years old woman presented a recurrent familial isolated hyperparathyroidism. She was operated on 10 years before and at reoperation parathyroid carcinoma was found. Nineteen patients were cured after a post operative follow up ranging from 3 to 168 months. One patient had an asymptomatic hypercalcemia recurrence. Two patients presented permanent hypoparathyroidism treated with calcitriol and calcium. CONCLUSION: Sporadic forms represent majority of cases of juvenile hyperparathyroidism. Renal manifestations are usual. Nevertheless, multiple endocrine neoplasia type 1 has to be evocated.     

Prospective analysis of intraoperative and postoperative urinary cyclic adenosine 3',5'-monophosphate levels to predict outcome of patients undergoing reoperations for primary hyperparathyroidism

Twenty-five consecutive patients with either persistent or recurrent symptomatic primary hyperparathyroidism after 36 prior operations were prospectively studied to compare the usefulness of intraoperative measurement of urinary cyclic adenosine monophosphate (UcAMP) levels with standard surgical procedures to predict outcome during tedious parathyroid reoperations. The criterion based on UcAMP to predict successful outcome was a 50% decline in intraoperative UcAMP levels from the median baseline level after removal of abnormal parathyroid tissue. Standard surgical criteria were resection of four abnormal glands for hyperplasia and resection of one abnormal gland and biopsy examination of one normal gland for adenoma. In 15 patients (60%) surgery was terminated on the basis of UcAMP criterion. In one patient elevated UcAMP levels never changed and correctly predicted unsuccessful surgery. In nine patients surgery was terminated on the basis of surgical criteria, and each of these patients had a successful outcome. Operative UcAMP levels dropped after the completion of the procedure in six of these latter nine patients. Three patients did not show a significant decrease in UcAMP levels despite successful surgery, and one patient displayed an early false-positive decrease in UcAMP level. The intraoperative UcAMP criterion accurately predicted outcome in 21 of 25 patients (84%). Sensitivity of the UcAMP criterion was 87% and specificity was 50%. The results indicate that by either method a reliable prediction of the outcome of reoperative parathyroid surgery may be made. Intraoperative determination of UcAMP levels will allow successful termination of the reoperation in some patients before operative identification of adequate parathyroid tissue necessary to confidently establish the pathologic diagnosis.     

Surgery for parathyroid adenoma and hyperplasia: Relationship of histology to outcome

Recent histopathologic evidence challenges the teaching that enlargement of a solitary parathyroid gland is invariably caused by an adenoma, whereas multiple gland enlargement results from hyperplasia. We have re-examined the parathyroid tissue obtained from 152 consecutive patients undergoing surgery for primary hyperparathyroidism and compared it with their clinical outcome. Our approach was to excise enlarged glands and biopsy the remainder. In 124 patients (82%) at least three glands were biopsied or removed. The ratio of adenoma to hyperplasia was reversed by our histologic re-examination; adenomas were found in only 27 patients (25 single, two double), whereas hyperplasia was found in 117 patients (one gland, 87 patients; two glands, 16 patients; three glands, five patients; four glands, nine patients). Normal tissue only was reported in eight patients. During a 2-year follow-up, five patients (3%) developed hypocalcemia and none developed recurrent hypercalcemia. Our results indicate that a full neck exploration with removal of all enlarged glands is more important than the histologic diagnosis in planning a successful surgical strategy for primary hyperparathyroidism.     

Preoperative sestamibi scanning and surgical findings at bilateral, unilateral, or minimal reoperation for recurrent hyperparathyroidism after subtotal parathyroidectomy in patients with multiple endocrine neoplasia type 1

HYPOTHESES: Preoperative parathyroid radioisotope scanning is of little or no value in patients with multiple endocrine neoplasia type 1 when 4 or more hypertrophied glands are present. Scanning using technetium Tc 99m sestamibi and single photon emission computed tomography will achieve a high level of sensitivity and specificity after 3 or more glands have previously been removed, justifying limited surgical reexploration. DESIGN: In a prospective study, the preoperative documented report of the predicted site of residual parathyroid was compared with the surgical findings in 13 patients having 19 scans and 17 reoperations. SETTING: All patients belonged to one family, previously described as Tasman family 1, and were confirmed by genetic testing as having multiple endocrine neoplasia type 1. In 10 of 13 patients, reexploration was being undertaken more than 10 years after the first operation. MAIN OUTCOME MEASURES: Scanning was regarded as successful when the documented preoperative report correctly predicted the side and quadrant in which a gland was found at surgery. Surgery was regarded as successful when calcium levels decreased to or below normal levels and were maintained. RESULTS: All 13 scans before first reexploration were successful in identifying the location of a residual parathyroid. From a statistical viewpoint, this equates to 100% sensitivity and 92% specificity. However, despite accurate localization of 1 residual gland in every patient, 7 supernumerary glands in 4 patients and 1 parathyroid remnant in a fifth patient were not localized so that sensitivity in locating all glands in every patient was only 61%. Scans performed for persistent hypercalcemia 48 to 72 hours after reexploration in 2 patients were unsuccessful in demonstrating any residual parathyroid. Scans performed 3 months after surgery in the same 2 patients and a third patient were successful, with sensitivity and specificity of 100%. Apart from patient 11, who awaits reexploration, normocalcemia was eventually achieved in every patient, with 11 of 12 having an initial period of hypocalcemia. CONCLUSIONS: Three months after reexploration and trimming or resection with transplant of half a gland left at first operation, sestamibi scanning achieved sensitivity and specificity of 100% in locating supernumerary parathyroids in patients with multiple endocrine neoplasia type 1 and persistent hypercalcemia. Before first reexploration, however, scans rarely provided new information, predominantly showing only the hypertrophied half-gland remnant.     

Recurrent disease after limited parathyroidectomy for sporadic primary hyperparathyroidism

BACKGROUND: Limited parathyroidectomy guided by intraoperative parathyroid hormone (PTH) assay (QPTH) is highly successful (97% to 99%) in predicting postoperative eucalcemia, usually with less extensive dissection when compared with bilateral neck exploration. Because fewer glands are excised when resection is guided by QPTH as opposed to resection guided by gland size, a higher recurrence rate may occur. Recurrence rate after bilateral neck exploration is 0.4% to 5%, but frequency of recurrence after limited parathyroidectomy is unknown. This study reports outcomes of this operative approach in sporadic primary hyperparathyroidism. STUDY DESIGN: Four-hundred twenty-three patients with sporadic primary hyperparathyroidism undergoing limited parathyroidectomy, followed 6 months or more or considered operative failures, were studied. In most patients, calcium and PTH levels were measured immediately after operation, and then at 2 and 6 months and yearly intervals. Operative failure is defined as hypercalcemia and high PTH within 6 months after operation, and recurrent hyperparathyroidism is hypercalcemia and elevated PTH occurring after a successful parathyroidectomy. Recurrence distributions were estimated using Kaplan-Meier analysis. RESULTS: The success rate of limited parathyroidectomy is 97% (412/423). Four-hundred six patients were eucalcemic over an average of 34 months (median 27, range 6 to 118 months) of followup and recurrent hyperparathyroidism developed in 6 of 412 (1.5%). Estimated 5 years recurrence-free rate was 97% (95% confidence interval, 91% to 99%). Earliest and latest recurrences were diagnosed at 24 and 83 months, respectively. QPTH results did not predict any recurrence. Overall success rate was achieved, with multiple gland resections performed in only 3% of patients. CONCLUSIONS: Recurrence rate after limited parathyroidectomy is similar to rates reported after bilateral neck exploration. Parathyroidectomy guided by QPTH is successful not only in resolving hypercalcemia in the short term, but also in providing longterm eucalcemia.     

Reoperative parathyroid surgery for primary hyperparathyroidism caused by multiple-gland disease: total parathyroidectomy and autotransplantation with cryopreserved tissue

Between September 1975 and June 1980, 27 patients with primary hyperparathyroidism caused by multiple-gland disease underwent reoperative parathyroid surgery at the National Cancer Institute. In an effort to minimize persistence and recurrence we adopted a strategy of attempting total parathyroidectomy. Immediate autotransplantation was not performed, but rather tissue was assessed in vitro for suppressibility and was cryopreserved. Patients with prolonged postoperative hypoparathyroidism underwent autotransplantation with cryopreserved tissue. Of 26 surviving patients, 23 (88%) were cured of hypercalcemia. Although 13 had hypoparathyroidism at the time of discharge, 4 of these 13 no longer required calcium or vitamin D within 1 year. At follow-up 17 were normocalcemic without medication (14 without and 3 with autografts). Thirteen patients had four or five glands removed. Two of these remain hypercalcemic, and at follow-up nine were normocalcemic without medication (seven without and two with autografts). Patients with multiple-gland disease, even those undergoing total parathyroidectomy, remain at risk for persistent or recurrent hypercalcemia. Such patients do no inevitably acquire hypoparathyroidism, and normocalcemia in patients undergoing immediate autotransplantation need not be attributed to the autograft. Selective, deferred autotransplantation with cryopreserved tissue prevents confusion regarding the source of postoperative hypercalcemia should it occur, minimizes the risk of graft-dependent hypercalcemia by restricting autotransplantation to those truly in need of additional tissue, and can effectively manage hypoparathyroidism.     

Subtotal parathyroidectomy for primary chief cell hyperplasia of the multiple endocrine neoplasia type I syndrome.

To evaluate the efficacy of subtotal parathyroidectomy (STP) in the treatment of primary hyperparathyroidism due to multiple gland disease, 12 patients with multiple endocrine neoplasia (MEN) type I syndrome were reviewed out of 132 patients undergoing parathyroidectomy. Each patient had yearly follow-up examinations and calcium determinations for a minimum of four years except for one patient who died one year after S.T.P. Permanent hypoparathyroidism occurred in three patients. Two patients remained persistently hypercalcemic, and two patients developed recurrent hypercalcemia. One patient required oral administration of calcium and vitamin D for ten years following STP before recurrent hypercalcemia developed. Another patient was normocalcemic for three years before recurrent hypercalcemia was noted. Only five of these 12 patients remain normocalcemic without need of calcium and vitamin D therapy. In patients with MEN type I, the long-term results of STP are less than satisfactory. Not only is it difficult to gauge how viable parathyroid tissue must be left to prevent both permanent hypoparathyroidism and persistent hyperparathyroidism but there is also a long-term risk of recurrence.     

Hyperparathyroidism in multiple endocrine neoplasia syndrome.

BACKGROUND. We analyzed clinical findings and results of parathyroidectomy in 42 patients treated from 1936 to 1988 at the University of California, San Francisco (UCSF) for primary hyperparathyroidism and multiple endocrine neoplasia (MEN) syndrome to document results of parathyroidectomy and reasons for failed parathyroid operations. METHODS. Of the 42 patients (38 had MEN 1 syndrome; 4 had MEN 2A syndrome), 40 patients were treated surgically: 29 had initial parathyroidectomy at UCSF; 11 were referred to UCSF because of MEN syndrome. Eight of these 11 patients required reoperation for persistent or recurrent hyperparathyroidism. Patients with hyperplasia were treated with subtotal parathyroidectomy; the glands of those patients with solitary or double adenomas were removed with or without biopsy of the normal appearing glands. RESULTS. Overall, in seven (50%) of 14 patients with hyperplasia, three (16%) of 19 patients with solitary adenoma, and one (14%) of seven patients with double adenomas, recurrent or persistent hyperparathyroidism developed. Failure in patients with hyperplasia was due to missed supernumerary glands (13%) and missed ectopic glands (33%). Failure occurred in patients with solitary (three patients) or double (one patient) parathyroid tumors because of unrecognized hyperplasia. None of the four patient with MEN 2A syndrome had persistent or recurrent disease, but hypoparathyroidism developed in one patient; hypoparathyroidism developed in three patients with MEN 1 syndrome. CONCLUSIONS. These data suggest that although many patients with primary hyperparathyroidism and MEN syndrome have multiple abnormal parathyroid glands, two populations of patients exist; one population has solitary or double adenomas and recurrence is uncommon, whereas the other population of patients has hyperplasia and persistent or recurrent disease is common.     

Parathyroidectomy in secondary hyperparathyroidism: Is there an optimal operative management?

BACKGROUND: Subtotal parathyroidectomy (SPTX) and total PTX with autotransplantation (TPTX + AT) are both accepted operations for secondary hyperparathyroidism (2HPT). Studies have shown the 2 procedures to have similar rates of recurrent or persistent HPT (0% to 10%). The majority of these reports are small case series and despite apparently similar outcomes; the optimal operative management for 2HPT remains controversial. The purpose of this study was to determine whether there were any clinical outcome differences between these apparently comparable operations. METHODS: A meta-analysis of 53 publications on reoperative operation for 2HPT from 1983 to 2004 identified 501 patients who had undergone an operation for recurrent or persistent 2HPT. The data evaluated included the type of initial operation, the need for reoperative operation as it related to the type of initial operation, and the intraoperative findings. RESULTS: The initial operation had been a SPTX in 36% and a TPTX + AT in 64% of patients. Reoperative operation was for persistent 2HPT in 82 of 485 (17%) and for recurrent 2HPT in 403 of 485 (83%) patients. Findings at reoperation included: autograft hyperplasia (49%), supernumerary glands (20%), remnant hyperplasia (17%), a missed in situ gland (7%), and a negative exploration (5%). Supernumerary glands, missed in situ glands, and negative explorations occurred at equal rates for both operations. Reoperation determined that inadequate cervical explorations occurred in 42% of patients who had undergone a SPTX and in 34% of patients who had undergone a TPTX + AT. CONCLUSIONS: Operative failures occur because of the limitations in preoperative localization, inadequate exploration, and the natural history of hyperplastic parathyroid tissue. The initial operation should include an attempt to localize supernumerary glands both pre- and intra-operatively.     

Primary hyperparathyroidism in infancy

Primary hyperparathyroidism in the neonate is a rare and often fatal disorder. These infants typically display severe hypercalcemia, respiratory distress, muscular hypotonia, and skeletal demineralization. They are usually diagnosed within the first three months of life and have hyperplasia of the four parathyroid glands. Twenty-nine infants with primary hyperparathyroidism are reported in the literature. Mortality is 87.5% in medically managed patients and 24% in surgically managed patients. Surgical management has not been satisfactory, in that recurrent hypercalcemia has been encountered in most patients undergoing subtotal parathyroidectomy, and total parathyroidectomy has resulted in the need for lifelong calcium and vitamin D supplementation. We have recently cared for a term newborn female in whom the diagnosis of primary hyperparathyroidism was made clinically on the second day of life, and later was confirmed biochemically. The baby underwent neck exploration on the 11th day of life and was successfully treated with total parathyroidectomy and parathyroid autotransplantation. Although initially rendered eucalcemic, the infant subsequently developed recurrent hypercalcemia requiring the removal of some of the autograft. Currently, the child is more than 2 years following surgery, growing well, and off all medication. The world literature is reviewed in this report of one of the first and the youngest infants, to our knowledge, to undergo parathyroid autotransplantation. In view of its success in avoiding the complication of repeated neck exploration for recurrent hyperparathyroidism or the creation of permanent hypoparathyroidism, we recommend this surgical approach for the rare neonate with primary hyperparathyroidism.     

Operative treatment of renal autonomous hyperparathyroidism: cause of persistent or recurrent disease in 304 patients

BACKGROUND: Subtotal parathyroidectomy (SPTX) and total parathyroidectomy with autotransplantation (TPTX and AT) are standard procedures in the treatment of renal autonomous hyperparathyroidism. In contrast to primary hyperparathyroidism, the persistence/recurrence rate is reported of up to 12.0%. PATIENTS AND METHODS: Between 1986 and 2000 we operated on 304 patients with renal autonomous hyperparathyroidism including 14 patients who were admitted after a primary operation in an outside hospital. Mean observation period was 51.4+/-38.9 months. RESULTS: The overall persistence/recurrence rate in our patients was 9.0% (26/290). After SPTX, excluding patients with an incomplete operation, it was 3.7%, and after TPTX and AT it was 6.0%. Reasons for developing recurrent or persistent disease in these patients were removal of less than 3.5 glands ( n=12), hyperplastic autograft ( n=5), and supernumerary gland ( n=4). After the first reoperation 7 patients (26.9%) had persistent or recurrent disease. CONCLUSIONS: An incomplete primary operation caused by missed cervical glands was the major reason for persistent ( n=8) or recurrent ( n=4) disease after different operative strategies in renal autonomous hyperparathyroidism.     

Long-Term Surveillance of Treated Hyperparathyroidism for Multiple Endocrine Neoplasia Type 1: Recurrence or Hypoparathyroidism?

Background

Primary hyperparathyroidism (HPT) in multiple endocrine neoplasia type 1 (MEN1) is surgically treated with either a subtotal parathyroidectomy removing 3 or 3,5 glands (SPX), less than 3 glands (LSPX), or a total parathyroidectomy with autotransplantation (TPX). Previous studies with shorter follow-up have shown that LSPX and SPX are associated with recurrent HPT, and TPX with hypocalcemia and substitution therapy. We examined the situation after long-term follow-up (median 20,6 years).

Methods

Sixty-nine patients with MEN1 HPT underwent 110 operations, the first operation being 31 LSPX, 30 SPX, and 8 TPX. Thirty patients underwent reoperative surgery in median 120 months later, as completion to TPX (n = 12), completion of LSPX to SPX (n = 9), extirpation of single glands (n = 3) still resulting in LSPX, and resection of forearm grafts (n = 3). Nine patients underwent a second, and 2 a third reoperation. In 24 patients genetic testing confirmed MEN1, and in the remaining heredity and phenotype led to the diagnosis.

Results

TPX had higher risk for hypoparathyroidism necessitating substitution therapy, at latest follow-up 50 %, compared to SPX (16 % after 3–6 months; none at latest follow-up). Recurrent HPT was common after LSPX, leading to 24 reoperations in 17 patients. No need for substitution therapy after SPX indicated forthcoming recurrent disease. Not having hypocalcemia in the postoperative period and less radical surgery than TPX were significantly associated to risk for recurrence. Further, mutation in exon 3 in the MEN1 gene may eventually be linked to risk of recurrence.

Conclusion

LSPX is highly associated with recurrence and TPX with continuous hypoparathyroidism, also after long-term follow-up. SPX should be the chosen method in the majority of patients with MEN1 HPT.

     

Symptomatic and functional outcome after laparoscopic reoperation for failed antireflux surgery

BACKGROUND: The aim was to determine symptomatic and functional outcome after reoperative antireflux surgery for recurrent reflux, persistent dysphagia and severe gas bloat, using a primarily laparoscopic surgical approach. METHODS: This was a retrospective analysis of prospectively collected data from 118 patients, of whom 70 had reoperative surgery for recurrent reflux, 35 for dysphagia and 13 for gas bloat. DeMeester scores before and 1 year after surgery, functional symptoms after surgery and overall patient satisfaction were analysed. RESULTS: Reoperation was completed laparoscopically in 101 patients (85.6 per cent), in 28 after previous open hiatal surgery. The operation was converted from an initial laparoscopic approach to open surgery in 17 patients. One-year follow-up data were available for 104 patients (88.1 per cent). After reoperation for recurrent reflux, 84 per cent had a DeMeester heartburn score of zero or one, and 87 per cent had a regurgitation score of zero or one. After reoperation for dysphagia, 21 of 32 patients had a dysphagia score of zero or one, with improvement observed in 25. All patients undergoing reoperation for severe gas bloat were satisfied with the outcome 1 year after operation. CONCLUSION: Revisional surgery for recurrent reflux using a laparoscopic approach offered high rates of success and patient satisfaction. Swallowing returned to normal in two-thirds of patients after reoperation.     

Surgical findings and results of subtotal and total parathyroidectomy in hypercalcemic patients with uremic hyperparathyroidism

Surgical and histopathologic findings and results of subtotal resection and total parathyroidectomy with autotransplantation were evaluated in 82 hypercalcemic patients with uremic hyperparathyroidism. The mean preoperative serum calcium concentration was 2.89 mM, and the highest values were associated with more rapidly progressing hypercalcemia. Despite greatly increased total weight of the parathyroid tissue (mean 1,509 mg), 34 patients had one to five normal-sized parathyroid glands. The incidence of such glands decreased with total gland weight. Nodular hyperplasia was found in 74% of the patients, and was characterized by considerable intraindividual size difference, while diffuse hyperplasia was associated with more moderate and symmetric glandular enlargement. During follow-up averaging 4.5 years, the incidence of persistent or recurrent hypercalcemia was slightly higher after subtotal than after total parathyroidectomy (11 and 11%, vs. 9 and 7%). Biochemical and/or radiologic signs of parathyroid bone disease were present in 36 (44%) of the patients before neck exploration and normalized postoperatively in all but seven. The outcome of parathyroid surgery thus was favorable in uremic patients with hypercalcemic hyperparathyroidism.