Intraoperative Parathormone Measurement in Patients with Multiple Endocrine Neoplasia Type I Syndrome and Hyperparathyroidism

Total or subtotal parathyroidectomy is considered the treatment of choice for multiple endocrine neoplasia type I (MEN-I)-associated primary hyperparathyroidism (HPT). However, persistent or recurrent HPT is frequently observed. The development of a rapid two-site immunoradiometric assay (IRMA) method for measuring intact parathormone (PTH) has provided a valuable tool for recognizing possible surgical failures. Our experience includes 16 MEN-I patients (10 females, 6 males) of mean age 35.5 years operated on between 1990 and 1996. Total parathyroidectomy (TPTX) with autotransplantation of parathyroid tissue was the standard treatment. Blood samples for PTH measurement were drawn at the induction of anesthesia (basal value), 10 and 20 minutes after the removal of each gland, and 60 minutes after TPTX. Rapid PTH measurement, which required only 15 minutes of incubation at 37°C, showed a highly significant correlation (p < 0.0001) with the standard method. Circulating PTH levels exhibited a stepwise decrease during TPTX, reaching a mean value of 22.3% of the baseline 20 minutes after removal of the last gland. Two patients showed a prompt decrease of PTH after removal of the single enlarged gland, featuring the kinetics observed in the adenomas. One of these two patients was successfully treated with more conservative surgery. None of the patients showed persistence or recurrence of HPT. In our experience, intraoperative measurement of PTH seems to be a valuable adjunct in both the diagnosis of multiglandular involvement and the prediction of surgical treatment in patients with primary parathyroid hyperplasia.     

Management of Pancreatic Endocrine Tumors in Multiple Endocrine Neoplasia Type 1

Introduction Pancreatic endocrine tumors (PETs) occur in at least 50% of patients with multiple endocrine neoplasia type 1 (MEN1) and are the leading cause of disease-specific mortality. However, the timing and extent of surgery for MEN1-related PETs is controversial owing to the indolent tumor growth seen in most patients and the desire to avoid complications associated with insulin dependence. To help resolve this controversy, we retrospectively analyzed the clinical characteristics, surgical treatment, and clinical outcome of patients with MEN1-related PETs. Methods All patients had histologic or radiographic confirmation of a PET in the setting of MEN1. Disease progression was defined radiographically as the development of new pancreatic tumors or distant metastases. Progression-free survival (PFS) and overall survival (OS) were used as the endpoints of this analysis. Results We identified 98 patients with MEN1, 55 (56%) of whom had PETs, including 27 women and 28 men with a median age of 37 years (range 8–69 years) at the time of diagnosis. Functioning PETs were present in 35 (64%) of 55 patients, and nonfunctioning tumors were present in 20 (36%). Pancreatic surgery was performed in 38 (69%) of the 55 patients; and the first operation included enucleation (n = 4), total pancreatectomy (n = 3), Whipple procedure (n = 4), and distal pancreatectomy (n = 27). The median size of the resected tumors was 2.8 cm (range 0.6–11.0 cm). Recurrent disease developed in the residual pancreas in 7 (20%) of 35 at-risk patients a median of 7.8 years after the first operation, and distant metastases occurred in 5 (14 %) of 36 surgically treated patients without distant metastasis (2 patients had distant metastases when surgery on the primary tumor was performed) at a median of 2.7 years following surgery. At last follow-up, 16 (29%) of 55 patients with PETs had died, 12 (22%) were alive with disease, 26 (47%) were alive without evidence of disease, and 1 (2%) was lost to follow-up. The median OS was 19.5 years (range 13–26 years) and was significantly longer for patients who had functioning PETs versus those with nonfunctioning tumors (P = 0.0007), for patients who underwent surgical resection of their PETs versus those who did not (P = 0.0043), and for patients with localized versus metastatic PETs at the time of diagnosis (P < 0.0001). Multivariate analysis revealed that younger age, hormonal function, and PET resection were independently associated with longer OS. Conclusions Our data suggest that early diagnosis and surgical excision of MEN1-related PETs improves survival. However, translating these data into a surveillance strategy for the early detection of PETs is complex owing to the potential morbidity of pancreatic resection and the risk of long-term insulin dependence.     

Unilateral Clearance for Primary Hyperparathyroidism in Selected Patients with Multiple Endocrine Neoplasia Type 1

Background

Primary hyperparathyroidism is the most common manifestation of multiple endocrine neoplasia type 1 (MEN1). Guidelines advocate subtotal parathyroidectomy (STP) or total parathyroidectomy with autotransplantation due to high prevalence of multiglandular disease; however, both are associated with a significant risk of permanent hypoparathyroidism. More accurate imaging and use of intraoperative PTH levels may allow a less extensive initial parathyroidectomy (unilateral clearance, removing both parathyroids with cervical thymectomy) in selected MEN1 patients with primary hyperparathyroidism.

Methods

We performed a retrospective cohort study at a high-volume tertiary medical center including patients with MEN1 and primary hyperparathyroidism, who underwent STP or unilateral clearance as their initial surgery from 1995 to 2015. Unilateral clearance was offered to patients who had concordant sestamibi and ultrasound showing a single enlarged parathyroid gland. For both the groups, we compared rates of persistent/recurrent disease and permanent hypoparathyroidism.

Results

Eight patients had unilateral clearance and 16 had STP. Subtotal parathyroidectomy patients were younger (37 vs 52 years). One patient in each group had persistent disease. One (13 %) unilateral clearance and five (31 %) STP patients had recurrent hyperparathyroidism after a mean follow-up of 47 and 68 months (p = 0.62). No unilateral clearance patients and two of 16 SPT patients had permanent hypoparathyroidism (p = 0.54).

Conclusions

Some MEN1 patients with primary hyperparathyroidism who have concordant localizing studies may be selected for unilateral clearance as an alternative to STP. For appropriately selected MEN1 patients, unilateral clearance can achieve similar results as STP and has no risk of permanent hypoparathyroidism, and may facilitate possible future reoperations.

     

Bronchial Carcinoid and Type 1 Multiple Endocrine Neoplasia Syndrome. A Case Report

Carcinoid tumours of bronchial origin are rare in type 1 multiple endocrine neoplasia (MEN1) syndrome. The prevalence of histologically confirmed cases is approximately 5–8%, although in more recent studies it is estimated that it could be much higher and a possible relationship with the presence of hypergastrinaemia is suggested. We report a patient with a type 1 MEN syndrome, with no respiratory symptoms, with hypergastrinaemia, and in whom a 5 mm diameter nodule was detected in the wall of the left main bronchus by computed tomography. The bronchial biopsy confirmed that it was a typical bronchial carcinoid and the octreoscan showed a single focus of high uptake coinciding with this lesion. A bronchoplastic (sleeve) was performed with extirpation of 3 bronchial rings, which also demonstrated that it was a typical carcinoid.     

Prospective Evaluation of Imaging Procedures for the Detection of Pancreaticoduodenal Endocrine Tumors in Patients with Multiple Endocrine Neoplasia Type 1

Early identification of pancreaticoduodenal endocrine tumors (PETs) in multiple endocrine neoplasia type 1 (MEN-1) is mandatory, because these tumors represent the most common cause of death within the syndrome. The diagnostic value of imaging procedures has therefore been evaluated in a prospective observational study. Between December 1997 and June 2003 twenty-two MEN-1 patients with genetically confirmed disease were followed for PETs using a standardized screening program with serum hormone measurements, endoscopic ultrasonography (EUS), computed tomography (CT), and somatostatin-receptor scintigraphy (SRS). Results could be validated by surgery and histopathology in 13 patients during 18 operations. In 12 asymptomatic patients with tumors measuring 10 mm or less, who have not yet undergone operation, PETs were detected by EUS in 12/12, by CT in 1/12, and by SRS in 2/11 cases. In 13 patients who have undergone surgical exploration EUS, CT, and SRS were true positive in 12 of 16, 7 of 13, and 12 of 17 cases, respectively, although the number of tumors detected by each imaging procedure alone was lower than the number detected intraoperatively and histopathologically in almost every case. A solitary liver metastasis in one patient and a nonfunctioning PET recurrence in another were identified only by SRS. Endoscopic untrasonography is the most sensitive imaging procedure for the detection of small ( 10 mm) PETs in MEN-1, whereas SRS is the procedure of choice for the identification of metastases of MEN-1 PETs—i.e., for staging. Detection of PETs at an early stage by an aggressive screening program using EUS may lead to prompt surgical intervention and improved prognosis of MEN-1 PETs.This article was presented at the International Association of Endocrine Surgeons meeting, Uppsala, Sweden, June 14–17, 2004.     

Limited Value of Ga-68-DOTATOC-PET-CT in Routine Screening of Patients with Multiple Endocrine Neoplasia Type 1

Background

Routine screening is recommended for patients with multiple endocrine neoplasia type 1 (MEN1) to enable early detection and treatment of associated neuroendocrine neoplasms (NEN). Gallium⁶⁸-DOTATOC-Positron emission tomography combined with computed tomography (Ga-68-DOTATOC-PET-CT) is a very sensitive and specific imaging technique for the detection of sporadic neuroendocrine tumors. The present study evaluated the value of Ga-68-DOTATOC-PET-CT in routine screening of patients with MEN1.

Methods

Between January 2014 and March 2016, all MEN1 patients underwent Ga-68-DOTATOC-PET-CT in addition to conventional imaging (computed tomography of the thorax, magnetic resonance imaging of the abdomen and pituitary, endoscopic ultrasonography). The diagnostic yield of conventional imaging and Ga-68-DOTATOC-PET-CT was prospectively documented and compared, and treatment changes caused by the addition of Ga-68-DOTATOC-PET-CT were recorded.

Results

Conventional imaging detected 145 NENs, mainly pancreaticoduodenal NENs (n = 117, 81%), in 31 of 33 MEN1 patients. Ga-68-DOTATOC-PET-CT detected 55 NENs in 23 of the 33 patients (p = 0.0001). Ninety (62%) NENs detected by conventional imaging were missed by DOTATOC-PET-CT. The majority of missed lesions were pNEN (n = 68; 74%). The sensitivity of Ga-68-DOTATOC-PET-CT for NENs <5, 5–9, 10–19 and ≥20 mm was 0, 29, 81 and 100%, respectively. However, Ga-68-DOTATOC-PET-CT detected more liver and lymph node metastases in patients with known metastatic disease, which did not lead to a change of patients’ management. In one patient (3%), Ga-68-DOTATOC-PET-CT was the only imaging modality that detected a small intestine NEN and led to potentially curative surgery.

Conclusion

Ga-68-DOTATOC-PET-CT cannot be recommended for routine screening of MEN1 patients. It might provide important additional information in patients with suspected or known metastatic disease.

     

Screening of Patients with Multiple Endocrine Neoplasia Type 1 (MEN-1): A Critical Analysis of Its Value

Background  Screening of multiple endocrine neoplasia type 1 (MEN-1) patients is widely recommended because one-fifth succumb to malignant neoplasms. However, recommendations for screening modalities and intervals are based mostly on nonprospective data. Methods  Thirty-five of 48 MEN-1 patients were evaluated at least twice by an annual screening program in a single-center, prospective, nonrandomized study between 1997 and 2006. The screening program comprised anamnesis, clinical examination, imaging procedures, and extensive biochemical evaluations. Prospectively diagnosed lesions were evaluated separately from nonprospectively diagnosed lesions at first evaluation. Results  The median age of the patients was 45 years (range = 15–70) at initial assessment. They were followed for a median of 72 months (range = 24–108) by a median of 6 (range = 2–10) evaluations. The vast majority of lesions were nonprospectively diagnosed at initial evaluation: 13 of 17 patients had primary hyperparathyroidism (pHPT), 24 of 29 had pancreatic endocrine tumors (PETs), and 4 of 4 had carcinoids. Vice versa adrenal lesions were mostly prospectively detected (18/23). Malignancy was observed in 10 patients (28%) in the initial assessment and without symptoms in 5 patients (9 PETs, 3 carcinoids). Endoscopic ultrasound (EUS) of 29 patients detected 88 PETs which were followed for 157 patient years. The mean annual growing rate was 13.28 ± 28.23 mm with respect to the baseline tumor diameter of 9 mm. In 35 patients the mean incidence of newly diagnosed PETs was 0.52/year. Adrenal lesions were invariably nonfunctional. A mean change in diameter of 6.7 ± 23.44% was monitored and malignant transformation was absent. Conclusions  Most lesions are detected at initial screening, particularly malignant tumors. Computed tomography of the abdomen and chest did not identify additional lesions. The interval between screenings could be extended to 3 years based on annually calculated growth rates and the incidence of MEN-1-associated lesions. The assessment of calcium, gastrin, and prolactin is sufficient for biochemical screening in MEN-1.     

Utility of Intraoperative Parathyroid Hormone Monitoring in Patients with Multiple Endocrine Neoplasia Type 1-Associated Primary Hyperparathyroidism Undergoing Initial Parathyroidectomy

Background

Intraoperative parathyroid hormone monitoring (IOPTH) is a widely used adjunct for primary hyperparathyroidism (pHPT). However, the benefit of IOPTH in familial pHPT, such as in multiple endocrine neoplasia type I (MEN1), remains unclear.

Methods

We performed a retrospective analysis of 52 patients with MEN1-associated pHPT undergoing initial parathyroidectomy with IOPTH monitoring at our institution. Parathyroid hormone (PTH) levels were measured before skin incision and 10 min after resection of the last parathyroid gland. Variables analyzed included percent drop of PTH from baseline and the final PTH level compared to the normal reference range (RR).

Results

A total of 52 patients underwent initial subtotal parathyroidectomy with IOPTH. An IOPTH decrease cutoff of ≥75 % from baseline had the highest biochemical cure rate (87 %). In the remaining 13 % who met this cutoff, all had persistent pHPT, with ≥90 % drop of PTH from baseline. The remaining patients, who did not meet the ≥75 % cutoff, were cured. Follow-up was available for three of four patients with final IOPTH levels above the RR: one had persistent pHPT, two had hypoparathyroidism (50 %). When a postresection PTH level was within the RR, 88 % of patients were cured. While considered cured from pHPT, 7 % of patients in this group developed permanent hypoparathyroidism. When the final PTH level dropped below the RR, 28 % developed permanent hypoparathyroidism.

Conclusions

A cutoff in IOPTH decrease of ≥75 % from baseline has the highest biochemically cure rate in patients with pHPT associated with MEN1. However, a 75 % cutoff in IOPTH decrease does not exclude persistent pHPT. The absolute IOPTH value does not accurately predict postoperative hypoparathyroidism.     

Thymic Carcinoid in a Patient with Multiple Endocrine Neoplasia Type 1: Report of a Case

We report herein the rare case of a 33-year-old man found to have a multiple endocrine neoplasia type 1 (MEN1)-associated carcinoid tumor in the thymus. A chest roentgenogram demonstrated an asymptomatic anterior mediastinal mass, 7 cm in diameter, and ultrasound-guided percutaneous Tru-Cut biopsy revealed a carcinoid tumor of the thymus. An extended thymectomy was performed through a median sternotomy and pathological examination confirmed the diagnosis of a thymic carcinoid tumor, which was mainly encapsulated with locally invasive growth into the pleura. Despite the absence of a family history of MEN1, he was treated for two pancreatic islet cell tumors, hyperparathyroidism, an adrenal tumor, and a retroperitoneal lipoma. MEN1 mutations were detected both in blood samples and pancreatic tumor tissues. He is now well without any evidence of tumor recurrence 27 months after the operation for the thymic carcinoid. MEN1 mutations were screened by direct nucleotide sequencing of all protein-coding regions of exons 2–10 of the MEN1 gene. Heterozygous germline mutation was detected in the blood sample analyses. Moreover, fresh-frozen pancreatic tumor tissues showed a loss of heterozygosity in the MEN1 region. Received: January 25, 2000 / Accepted: November 20, 2000     

Primary Hyperparathyroidism in Multiple Endocrine Neoplasia Type 1: Individualized Management With Low Recurrence Rates

Background To evaluate the outcomes in different surgical modalities for primary hyperparathyroidism in multiple endocrine neoplasia type 1 (MEN1) patients, intraoperative findings from a single surgeon were studied to investigate a potentially improved modality of parathyroidectomy (PTx). Methods All 22 patients had PTx by a single surgeon in the past 21 years. Three modalities of PTx were used, depending on the operative findings, after all parathyroids and the thymus were identified. If fewer than three glands were enlarged, selective removal of the enlarged glands with or without biopsy of a normal-appearing gland was performed (selective PTx); if all glands were enlarged, either a subtotal PTx leaving a 50-mg remnant in situ or a total PTx with autotransplantation (TPTx + AT) was performed. Results There were 7 men and 15 women, aged 22 to 67 years (average, 43 years). Sixteen had familial and six had sporadic MEN1. They underwent 23 operations, including 11 selective PTx, 6 subtotal PTx, and 6 TPTx + AT. On follow-up for 1 to 19 years, only one patient (4.6%) had recurrent hyperparathyroidism 5.5 years after subtotal PTx. Others had either normocalcemia (n = 14; 63.6%) or hypocalcemia (n = 7; 31.8%). Those who had either a subtotal PTx or TPTx + AT had a significantly higher rate of postoperative hypocalcemia than those who had a selective PTx (9.9% vs. 54.5%; P = .032; Fisher’s exact test). Conclusions Primary hyperparathyroidism in our MEN1 patients was less aggressive than that reported in the literature. Selective PTx according to the intraoperative findings achieved optimal outcomes.     

Factors Indicating Surgical Excision in Classical Type of Lobular Neoplasia of the Breast

PurposeClassical type of lobular neoplasia (LN) encompassing both atypical lobular hyperplasia and classical lobular carcinoma in situ of the breast is a lesion with uncertain malignant potential and has been the topic of several studies with conflicting outcome results. The aim of our study was to clarify outcome-relevant factors and treatment options of classical LN.MethodsWe performed a pathological re-evaluation of the preoperative biopsy specimens and a retrospective clinical and radiological data analysis of 160 patients with LN from the Breast Center Zurich. Open surgery was performed in 65 patients, vacuum-assisted biopsy (VAB) in 79 patients, and surveillance after breast core needle biopsy (CNB) in 16 patients.ResultsThe upgrade rate into ductal carcinoma in situ/invasive cancer was the highest in case of imaging/histology discordance (40%). If the number of foci in the biopsy specimen was ≥3, the upgrade rate in the consecutive surgical specimens was increased (p = 0.01). The association of classical LN with histological microcalcification correlated with shortened disease-free survival (p < 0.01), whereas other factors showed no impact on follow-up.ConclusionsSurveillance or subsequent VAB after CNB of LN is sufficient in most cases. Careful consideration of individual radiological and histological factors is required to identify patients with a high risk of upgrade into malignancy. In those cases, surgical excision is indicated.     

Ultrasonography Should Not Guide the Timing of Thyroidectomy in Pediatric Patients Diagnosed with Multiple Endocrine Neoplasia Syndrome 2A through Genetic Screening

Background

American Thyroid Association (ATA) guidelines suggest that thyroidectomy can be delayed in some children with multiple endocrine neoplasia syndrome 2A (MEN2A) if serum calcitonin (Ct) and neck ultrasonography (US) are normal. We hypothesized that normal US would not exclude a final pathology diagnosis of medullary thyroid cancer (MTC).

Methods

We retrospectively queried a MEN2A database for patients aged <18 years, diagnosed through genetic screening, who underwent preoperative US and thyroidectomy at our institution, comparing preoperative US and Ct results with pathologic findings.

Results

35 eligible patients underwent surgery at median age of 6.3 (range 3.0–13.8) years. Mean MTC size was 2.9 (range 0.5–6.0) mm. The sensitivity of a US lesion ≥5 mm in predicting MTC was 13 % [95 % confidence interval (CI) 2 %, 40 %], and the specificity was 95 % [95 % CI 75 %, 100 %]. Elevated Ct predicted MTC in 13/15 patients (sensitivity 87 % [95 % CI 60 %, 98 %], specificity 35 % [95 % CI 15 %, 59 %]). The area under the receiver operating characteristic curve (AUC) for using US lesion of any size to predict MTC was 0.50 [95 % CI 0.33, 0.66], suggesting that US size has poor ability to discriminate MTC from non-MTC cases. The AUC for Ct level at 0.65 [95 % CI 0.46, 0.85] was better than that of US but not age [AUC 0.62, 95 % CI 0.42, 0.82].

Conclusions

In asymptomatic children with MEN2A diagnosed by genetic screening, preoperative thyroid US was not sensitive in identifying MTC of any size and, when determining the age for surgery, should not be used to predict microscopic MTC.     

Factors Predicting Outcome of Total Thyroidectomy in Young Patients with Multiple Endocrine Neoplasia Type 2: A Nationwide Long-Term Follow-up Study

Background  

Multiple endocrine neoplasia type 2 (MEN 2) is caused by a RET mutation in chromosome 10. All MEN 2 patients develop medullary thyroid carcinoma (MTC). The age-related risk of MTC is associated with the type of RET mutation. Our aim was to identify prognostic factors associated with recurrent MTC in MEN 2 patients.     

Surgical Strategy in a Kindred with a Rare RET Protooncogene Mutation of Variable Penetrance with Regard to Multiple Endocrine Neoplasia

Prophylactic thyroidectomy is recommended for carriers of RET protooncogene mutations owing to their nearly complete penetrance for medullary thyroid carcinoma (MTC). However, this guideline is challenged by mutations exhibiting variable penetrance of C-cell pathology. A 38-year-old woman presented with pathologic basal and pentagastrin-stimulated calcitonin levels. Genetic analysis revealed a heterozygous RET protooncogene germline mutation in codon 791 (exon 13) (TAT(Tyr)-->TTT(Phe)), followed by thyroidectomy and systematic central lymph node dissection. Histology showed C-cell hyperplasia (CCH) only. Three additional carriers were identified among family members. The 71-year-old father refused surgery despite pathologic calcitonin levels. The index patient's 37-year-old sister had normal basal and stimulated calcitonin levels, and her 6-year-old son had a 10-fold rise of calcitonin after pentagastrin stimulation. Both patients underwent the same operation as the index patient. The sister had 25 hyperplastic C-cells, but the her son had extensive CCH without MTC. The eldest uncle of the index patient had died of metastatic MTC at the age of 52 with unknown carrier status. Despite variable penetrance, each carrier of a RET protooncogene germline mutation should undergo thyroidectomy, even if basal and stimulated calcitonin levels are normal because at present no test can exclude or predict the age of development of MTC. Moreover, pathologic calcitonin levels cannot differentiate between CCH and MTC. Central lymph node dissection is recommended, as lymph node metastases occur early, significantly worsening the prognosis.     

Causes and Treatment of Recurrent Hyperparathyroidism After Subtotal Parathyroidectomy in the Presence of Multiple Endocrine Neoplasia 1

Background  

Subtotal parathyroidectomy (SPTX) is the treatment of choice for hyperparathyroidism in a patient with multiple endocrine neoplasia type 1 (HPT-MEN-1). There are scarce data on the causes, timing, and appropriate surgical treatment of patients with recurrent HPT-MEN-1. The aim of this study was to investigate the timing, causes, site of recurrence, and surgical treatment of recurrent HPT-MEN-1 in patients who underwent SPTX.     

埃布斯坦综合征合并预激综合征的外科治疗

目的　为了提高手术疗效 ,总结埃布斯坦综合征 (Ebstein syndrome)合并预激综合征的手术治疗经验。方法　 11例患者均有埃布斯坦综合征及严重三尖瓣关闭不全 ,合并 A型预激综合征 4例 ,B型 7例。术中电生理标测显示 11例中有 12条附加旁道 ,右心室游离壁 6例 ,左心室游离壁 3例 ,后间隔 1例 ,后间隔和右心室游离壁双旁道 1例。所有患者的房室附加旁道均在体外循环下被成功地手术切割 ;埃布斯坦综合征按 Danielson方法行成形术 9例 ,三尖瓣置换术 2例。　结果　全组 11例无手术后早、晚期死亡 ;术后随访 4个月～ 6年 ,平均随访 3.4± 1.3年 ,无预激综合征复发 ,2例有轻度三尖瓣关闭不全。心功能 级 9例 , 级 2例。　结论　手术治疗埃布斯坦综合征合并预激综合征 ,可同时纠正埃布斯坦综合征伴三尖瓣关闭不全和预激综合征 ,治疗效果良好。     

马方综合征并发A型主动脉夹层的手术疗效

目的观察并评价马方综合征(Marfan syndrome,MFS)并发A型主动脉夹层(type A aortic dissection,AAD)的手术疗效。方法回顾性分析2006年1月至2012年1月武汉亚洲心脏病医院收治44例MFS并发AAD患者的临床资料,其中男31例,女13例;年龄12～54(33.0±9.8)岁。23例在AAD发病后不同疾病阶段施行外科手术治疗,均行Bentall手术;21例因经济或其它原因未采取手术治疗。采用含时变协变量的COX回归分析,分别以主要终点事件、主要+次要终点事件作为结局变量对相关因素进行分析,观察手术患者术后手术疗效和生活质量,并与未手术患者的随访资料进行对比。结果术后有1例手术患者死于多器官功能衰竭,其余22例手术均成功。随访43例,随访时间1～75个月。22例手术患者术后3年生存率为95.7%,随访期间生活质量良好。未行手术治疗的21例患者生存状况及生活质量较差,13例(61.9%)死亡,主要死亡原因:急性心脏压塞、主动脉夹层破裂、急性心肌梗死并发心源性休克等;发病后3年生存率仅为31.7%。采用含时变协变量COX回归模型分析校正手术时间不一产生影响后的结果显示,手术治疗患者与未手术治疗患者相比较,其临床疗效的差异有统计学意义(T_COV_的OR值=0.088,P=0.028),手术治疗死亡风险仅为非手术治疗的8.8%(P<0.05)。结论 MFS并发AAD预后凶险,应尽早外科手术,不论是急性期还是慢性期均可获益,疗效可靠,随访结果令人满意。     

Long-Term Results of Wide Surgical Excision in 106 Patients with Hidradenitis Suppurativa

BACKGROUND: Hidradenitis suppurativa (acne inversa) is a chronic recurrent disorder characterized by abscessing inflammation, fistulating sinus tracts, and scarring. Predilection sites are the intertriginal regions. The severe course of the disease demands an early and curative treatment. OBJECTIVE: The aim of this study was to review the effect of radical surgical excision concerning cure rate and potential complications within a large group of patients. METHODS: We analyzed data for 106 patients suffering from hidradenitis suppurativa treated during the period 1980-1998. The mean duration of the disease was 7 years. In about 90% of the cases, two or more sites were affected. Inguinal (70.8%) and axillary regions (61.3%) were most commonly involved. All patients were treated by radical wide excision using intraoperative marking of sinus tracts with methylviolet solution. The method of reconstruction depended on the size and location of the defect. Median postoperative follow-up time was 36 months. RESULTS: The overall complication rate was 17.8%. Most of these were minor complications such as suture dehiscence, postoperative bleeding, and hematoma. Wound infection occurred in only 3.7% of patients. The rate of recurrence within the operated fields was 2.5%. There was no relation between the surgical method of reconstruction and the rate of recurrence. Recurrence was related to the severity of the disorder. CONCLUSION: Our results confirm early radical excision as the treatment of choice for hidradenitis suppurativa. Using intraoperative color-marking of sinus tracts, the recurrence rate is minimal. The method of reconstruction has no influence on recurrence and should be chosen with respect to the size and location of the excised area.