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PubMed1996

Carotid surgery in octogenarians: is it worthwhile?

Controversy surrounds the role of carotid endarterectomy in octogenarians. Although the prognosis of severe degree carotid stenosis is more ominous in the elderly, operative risk seems more important in the aged. To evaluate the presumed detrimental effect of advanced age on the mortality-morbidity of carotid endarterectomy, the authors reviewed their common experience with carotid surgery in patients aged 80 years or more. From 1980 to 1994, 129 octogenarians were operated on for occlusive carotid artery disease in two university hospitals. The data for these patients, 80 years of age and older (group 1) are compared to these for a large middle age group (less than 80 years) (group 2) operated by the same surgeons during that period. The baseline characteristics of both groups were similar, except for smoking, diabetes and previous myocardial revascularization, more prevalent in the younger age group. In the elderly group prophylactic surgery for asymptomatic stenosis was done in 36%, versus 40% of the middle aged patients, and for stroke in evolution in 8% versus 4% (p < 0.05). The perioperative stroke rate was 0.8% in the group older than 80 years, compared to 1.2% for non octogenarians (NS). The operative mortality was similar for both age groups (2.3 and 1.5 respectively). The long-term results gave a similar outlook for both age groups. The 5-year stroke-free rate reached 89% for group 1 versus 91% for group 2. The 5-year survival rate, however, was less for the elderly patients (47% versus 77%, mean follow-up periods of 30 and 49 months). These results suggest that carotid endarterectomy can be safely done in elderly patients, with a similar risk/benefit ratio as for the younger patients. Advanced age, by itself, is not to be considered as contraindication to carotid surgery.

Adult↗

PubMed2004

Attitude toward genetic testing for cancer risk in Istanbul.

To identify attitudes toward genetic testing, and the effects of this information on decisions regarding issues such as pregnancy, abortion, and prophylactic surgery, several subsets of the Turkish population were surveyed in hospital settings. Individuals (n = 179) chosen arbitrarily from four different subsets of a Turkish population were asked to participate in a confidential 23-question survey. Survey participants were familiar with the concept of cancer being a familial disease (85.5%), and 84.7% of them expressed interest in genetic testing to determine cancer risk, 83.9% would have their fetuses tested for such cancer risk, 65.1% would terminate their pregnancies, 92.2% would have their children tested if they were determined to have an increased cancer risk, 71.9% would agree to undergo prophylactic oophorectomy or orchiectomy and 67.6% would have mastectomy/prostatectomy should there be an increased cancer risk to these organs. It appears that at least the sampled segment of a Turkish population is willing to undergo genetic testing to determine if they are at increased risk for cancer. The feasibility and acceptance of genetic testing and the influence of education and genetic counseling in the Turkish people should further be evaluated with a larger stratified sample of the population.

Adult↗

PubMed2006

ASCO/SSO review of current role of risk-reducing surgery in common hereditary cancer syndromes.

BACKGROUND: A significant portion of cancers are accounted for by a heritable component, which has increasingly been linked to mutations in specific genes. Clinical interventions have been formulated for mutation carriers within affected families. The primary interventions for mutation carriers of highly penetrant syndromes are surgical. METHODS: The American Society of Clinical Oncology and the Society of Surgical Oncology formed a task force charged with presenting an educational symposium on surgical management of hereditary cancer syndromes at annual society meetings, and this resulted in a position paper on this topic. The content of both the symposium and the position paper was developed as a consensus statement. RESULTS: This article addresses hereditary breast, colorectal, ovarian/endometrial, and multiple endocrine neoplasias. A brief introduction on the genetics and natural history of each disease is provided, followed by detailed descriptions of modern surgical approaches, clinical and genetic indications, timing of prophylactic surgery, and the efficacy of surgery (when known). Although several recent reviews have addressed the role of genetic testing for cancer susceptibility, this article focuses on the issues surrounding surgical technique, timing, and indications for surgical prophylaxis. CONCLUSIONS: Risk-reducing surgical treatment of hereditary cancer is a complex undertaking. It requires a clear understanding of the natural history of the disease, realistic appreciation of the potential benefits and risks of these procedures in potentially otherwise healthy individuals, and the long-term sequelae of such interventions, as well as the individual patient's and family's perceptions of surgical risk and anticipated benefit.

Female↗

PubMed2006

Risk-reducing surgery, screening and chemoprevention practices of BRCA1 and BRCA2 mutation carriers: a prospective cohort study.

This study prospectively evaluated the utilization of cancer risk management strategies in a multi-institutional cohort of BRCA1 and BRCA2 mutation carriers using a self-report questionnaire. Of 142 unaffected female mutation carriers, 70 (49%) had elected to receive their mutation result. Of those who knew their mutation result, 11% underwent bilateral mastectomy (BM), 29% had bilateral oophorectomy (BO), 78% performed regular breast self-examination (BSE), and 80%, 89%, 67%, and 0% had at least annual clinical breast examination (CBE), mammography, transvaginal ultrasound (TVU), and CA125, respectively. A further 20%, 7%, 0%, 21%, and 75%, respectively, reported never having had these tests. For women who elected not to receive their mutation result, 0% underwent BM, 6% underwent BO, and 77%, 42%, 56%, 7%, and 0% had regular BSE, CBE, mammography, TVU, and CA125, respectively. Only one woman used chemoprevention outside a clinical trial. Uptake of prophylactic surgery and screening was associated with knowing one's mutation status (for all behaviors except BSE), age (for BO and CBE) and residence (for mammography). In this cohort, the minority of mutation carriers utilized risk-reducing surgery or chemoprevention and a substantial minority were not undergoing regular cancer-screening tests.

Adult↗

PubMed1997

Surgical treatment supposed natural history of the tethered cord with occult spinal dysraphism.

We retrospectively evaluated the pre- and postoperative course of 34 tethered cord patients with occult spinal dysraphism in an attempt to infer the natural history of this disorder and to determine the effectiveness of the surgical treatment. There were 32 cases with lumbosacral lipoma and 2 with tight filum terminale. The age at surgery ranged from 1 month to 47 years old. Eight patients, aged 1 month to 4 years old, were asymptomatic; 26 had neurogenic bladder (26 cases) or motor problems affecting the legs (8 cases). None of the patients older than 5 years of age were asymptomatic. Untethering of the spinal cord was performed in all cases. The postoperative follow-up period ranged from 5 months to 11 years. During these periods, 7 (88%) of the 8 asymptomatic patients remained neurologically intact, 6 (23%) of the 26 symptomatic patients showed improved symptoms, and 15 patients (58%) remained unchanged. These results indicate that the neurological symptoms will appear progressively in the tethered cord patients, and that prophylactic surgery should be considered as early as possible.

Adolescent↗

PubMed1999

Results after surgery for lumbosacral lipoma: the significance of early and late worsening.

We retrospectively reviewed 32 patients operated on for lipoma of the conus and lipomyeloschisis, the two main anatomical subtypes of congenital lumbosacral lipomas associated with tethered cord syndrome. Surgery was proposed to patients when they were symptomatic, and in most cases for progressive symptoms. The evolution of the different symptoms was studied separately. In most patients, symptoms improved or stabilized after surgery; in some, however, postoperative worsening, at least of some of the symptoms, was seen. This postoperative worsening became apparent either early or late after the operation, and was not associated with surgical trauma or postoperative complications. We suggest it was caused mainly by the natural course of the disease (especially in the case of the orthopedic deformities), and in some cases by retethering. Our series is not large enough to detect statistical significance for the different symptoms or for the anatomical subgroups. Importantly, according to our analysis by the different symptoms, the operation did not seem to protect the patients from later development of new deficits. This can be interpreted as an argument against prophylactic surgery in asymptomatic patients.

Adolescent↗

PubMed2002

Perspectives on the management of the complications of senile retinoschisis.

PURPOSE: To evaluate various published views regarding the management of complications of senile retinoschisis. METHODS: To analyze and compare various reports of studies of natural history and prognosis, and comparative risks of non-intervention vs prophylactic surgery. RESULTS: Although a number of recent techniques for treatment or prevention of complications of senile retinoschisis have been reported with generally satisfactory results, the numbers of cases have been small and no consensus can be said to have been achieved as to superiority of method. When compared to the extremely low likelihood of the natural occurrence of complications such as posterior progression to involve the macula, or of the development of progressive clinical retinal detachment, as well as the occurrence of risks of treatment, it appears preferable to postpone treatment until these complications actually make their appearance. CONCLUSIONS: It is recommended that in cases of retinoschisis either without or with breaks, or retinoschisis with localized 'schisis-detachment', that prophylactic treatment be withheld except in a very few exceptional cases. In all cases of progressive, symptomatic rhegmatogenous retinal detachment however, prompt surgical treatment should be carried out.

Adult↗

PubMed1998

Current policies for surveillance and management in women at risk of breast and ovarian cancer: a survey among 16 European family cancer clinics. European Familial Breast Cancer Collaborative Group.

The recent isolation of breast cancer predisposing genes (BRCA1 and BRCA2) allows the identification of carriers within affected families. These carriers have a 50-85% risk of developing breast or ovarian cancer and need careful follow-up. The purpose of this study was to evaluate the management and screening protocols implemented in high risk families at various family cancer clinics in Europe. A questionnaire was mailed to the members of the European Familial Breast Cancer Collaborative Group (n = 30) requesting information on the following issues: indication for surveillance of breasts and ovaries, the recommended protocol, coordination of the screening examination, prophylactic surgery, the specific management of breast cancer in a mutation carrier and the use of oestrogen. 16 centres from nine countries responded. Most centres recommend surveillance of the breasts if the lifetime risk exceeds 15-20%. The surveillance protocol that is generally advised comprises monthly self breast examination, examination by a specialist every 6 months and annual mammography, all starting from an age between 25 and 35 years. Surveillance of the ovaries is recommended in BRCA1 and BRCA2-mutation carriers, in members from breast/ovarian cancer families and in some centres in 'breast cancer only' families with an early onset of breast cancer. The recommended protocol includes gynaecological examination, sonography and estimation of CA-125 at yearly intervals starting from the age 30-35 years. Prophylactic mastectomy is considered for proven mutation carriers in some centres. Most centres consider prophylactic oophorectomy in mutation carriers and some centres also consider it for members of breast/ovarian cancer families. This survey provides insight into the guidelines for surveillance and management of familial breast cancer used at various family cancer clinics in Europe; this insight may contribute to the appropriate management of these high risk women. It should be emphasised that most recommendations are based on experts' opinion rather than on any specific studies.

Adult↗

PubMed1992

The multidisciplinary management of a family with epithelial ovarian cancer.

OBJECTIVE: To describe the management of a family with an inherited predisposition to ovarian and breast cancer. Particular attention is paid to the problems of contraception, screening, prophylactic surgery and hormone replacement therapy. SETTING: The multidisciplinary Grampian Familial Epithelial Ovarian Cancer Study Group. SUBJECTS: 162 members of a family extending over five generations. In the third generation, five of the 10 women died with epithelial ovarian cancer. Three women in generation IV have developed pre-menopausal breast cancer. There are now 78 family members in the fifth generation aged between 2 and 22 years. INTERVENTIONS: Counselling of female family members is started at the age of 18 years. The combined oral contraceptive pill is advocated to suppress ovulation. Gynaecological follow-up after the age of 28 includes yearly pelvic examination, transvaginal ultrasonography and serum CA125 estimation. Laparoscopy with peritoneal cytology is indicated if any part of this yearly assessment is abnormal. Prophylactic oophorectomy is advised between the ages of 35 and 40 years after the family is complete. In generation IV, 20 of the 29 women have undergone prophylactic oophorectomy. Oestrogen hormone replacement therapy with a cyclical progestogen is recommended after prophylactic oophorectomy. Breast cancer screening starts at the age of 25 and involves annual clinical breast examination augmented by mammography and breast ultrasound. CONCLUSIONS: Only by the careful questioning and recording of family history, including at least third degree relatives (cousins), will similar groups with familial ovarian/breast cancer be identified. When predisposing genes are characterized it will be possible to identify carriers within the family and concentrate clinical effort on them while offering appropriate reassurance to those with decreased risk.

Adult↗

PubMed1995

Venous aneurysms: surgical indications and review of the literature.

BACKGROUND: During the last 20 years we diagnosed five cases of venous aneurysm of the jugular (n = 4) and basilic (n = 1) veins. The purpose of this report was to determine the natural history and indications for surgery of venous aneurysms. METHODS: Our five cases were included in an English-language literature review performed through August 1993. RESULTS: In our series two aneurysms (one external jugular vein, one basilic vein) were excised for cosmetic reasons. Three internal jugular vein aneurysms were followed up for up to 4 years without complications with serial color duplex ultrasonography. Of 32 patients with abdominal venous aneurysms (18 portal, seven inferior vena cava, four superior mesenteric, two splenic, one internal iliac), 13 (41%) had major complications including five deaths. Of 31 patients with deep venous aneurysms of the extremity (29 popliteal, two common femoral), 22 (71%) had deep vein thrombosis or pulmonary embolism and in 17 recurrent deep vein thrombosis or pulmonary embolism developed when patients were treated with anticoagulation alone. CONCLUSIONS: Prophylactic surgery is cautiously recommended for low-risk patients with venous aneurysms of the abdomen and strongly recommended for most patients with lower extremity deep venous aneurysms. Other venous aneurysms should be excised only if they are symptomatic, enlarging, or disfiguring.

Abdomen↗

PubMed2005

Age and manifestation related symptoms in familial adenomatous polyposis.

BACKGROUND: To identify early symptoms of familial adenomatous polyposis with a view to improve early diagnosis and treatment. Diagnosis on the basis of genetic testing is usually limited to where there is a known family history, so FAP is more usually diagnosed on clinical grounds. Except for those identified via FAP registers, the majority of patients are symptomatic at the time of diagnosis. METHODS: We undertook a retrospective study of 143 FAP patients treated at the Department of Surgery, University of Erlangen between 1971 and 2000. We identified patterns of symptoms, endoscopic findings and extracolonic manifestations in three age groups. RESULTS: FAP was diagnosed clinically on the basis of symptoms in 84% (120/143) of these patients. Most presented with intestinal symptoms such as colonic bleeding (68%) and diarrhea (42%). All but one of the patients between 20 and 40 years old had rectal polyps (98.7%, 75/76), whereas in those over 40 years old the prevalence was 76% (35/46). Non-specific symptoms such as abdominal pain, fatigue and bloating were less frequent and were mainly reported by patients older than 40. CONCLUSION: The commonest presenting features of FAP are alteration of bowel habit and rectal bleeding, but both are found in many other conditions. Patients with these findings need immediate endoscopy to allow prompt diagnosis and prophylactic surgery.

Abdominal Pain↗

PubMed2000

[Surgical therapy of colonic diverticulitis--how reliable is primary anastomosis?].

INTRODUCTION: The aim of the study was to evaluate the modern principles of surgery in diverticulitis, e.g. early elective resection and primary anastomosis. METHODS: The data of 445 consecutive patients were retrospectively analysed after classifying all cases in four subgroups according to a modified Hinchey classification (stages 0-III). RESULTS: Within our study group the morbidity was 26.5% (n = 118) and the mortality was 1.6% (n = 7). In 96% (n = 425) of all cases and in 64% (21/33) of patients with perforated diverticulitis and peritonitis (stage III), a primary anastomosis was performed. Four patients of the study group showed insufficient anastomosis (0.9%). No leakage was observed from any of the anastomoses performed in stage III diverticulitis. Stage of inflammation and age of patient correlate with morbidity and mortality. Some 53% (94/177) of the patients in stage II and 67% (22/33) of the patients in stage III had never showed symptoms of diverticulitis before. CONCLUSION: Prophylactic surgery to avoid life-threatening situations, including abscess formation or perforation, is not possible in many cases. However, especially patients at risk (age, coexisting illness) should undergo early surgery. Primary anastomosis can be performed safely even at an advanced stage.

Adult↗

PubMed2001

Screening for extensor tendon rupture in rheumatoid arthritis.

OBJECTIVE: Surgery can prevent extensor tendon rupture in the rheumatoid wrist but it is difficult to identify patients at risk. Extensor digiti minimi (EDM) usually ruptures first, but rupture may pass unnoticed because extensor digitorum communis (EDC) extends all four fingers simultaneously. We assessed the value of screening for EDM rupture by examining for absent independent extension of the little finger in a hospital rheumatoid arthritis population. METHODS: The EDM test was performed in 550 previously unoperated wrists. Disease activity, joint damage, wrist swelling, tenderness and crepitus were recorded. RESULTS: Unsuspected EDM loss was found in nine of the 550 wrists (1.6%); dorsal synovitis was absent or minimal in eight and ulnar tenderness was absent in six. EDM loss was not associated with activity, severity or duration of disease. CONCLUSIONS: The EDM test is simple and cheap. It may identify patients at risk and permit prophylactic surgery before hand function is lost.

Arthritis, Rheumatoid↗

PubMed2006

Mutation analysis of the RET proto-oncogene and early thyroidectomy: results of a Portuguese cancer centre.

BACKGROUND: Evidence that germline mutations in the RET proto-oncogene are the underlying cause of the familial form of medullary thyroid carcinoma (MTC) made it possible to identify gene carriers with a very high degree of accuracy. Aiming to define the mutational profile observed in our patients and to assess gene carriers' compliance with an early surgery, we reviewed results of molecular analysis of RET performed at our institution since 1994. METHODS: One hundred fifty-eight individuals were screened for germline mutations of the RET proto-oncogene. Seventy-seven patients had apparently sporadic MTC; 8 patients had both MTC and pheochromocytoma or MTC and clinical features of multiple endocrine neoplasia type 2B despite a negative family history; 8 patients were known to belong to affected kindreds; and 65 individuals were at-risk individuals to develop MTC. RESULTS: A germline mutation in RET was identified in 4% of apparently sporadic MTC patients, in 100% of patients with MTC and pheochromocytoma or MTC and clinical features of multiple endocrine neoplasia type 2B, and in 100% of probands of clinically established kindreds. The most affected codon was 634 (58%) followed by codon 804 (16%). Among at-risk individuals, 49% were identified as gene carriers. Seven individuals were submitted to prophylactic thyroidectomy (mean age, 17.7 +/- 12.5 years; range: 3-42 years), and all but 1 had MTC. CONCLUSIONS: RET mutational spectrum observed in the present population disclosed a higher frequency of codon 804 mutations than expected. Compliance with an early prophylactic surgery seemed to be influenced not only by medical advice and cultural factors but also by the aggressiveness of disease in gene carriers' families.

Adolescent↗

PubMed2003

Malignant progression from C-cell hyperplasia to medullary thyroid carcinoma in 167 carriers of RET germline mutations.

BACKGROUND: Hereditary medullary thyroid carcinoma (MTC) is the most common and potentially life-shortening phenotypic manifestation of RET (rearranged during transfection) germline mutations. If a distinct time lag between the successive stages of malignant progression were identifiable, the information could be used to individualize prophylactic surgery. The study objective was to investigate the impact of RET genotype on disease progression from C-cell hyperplasia (CCH) to MTC. METHODS: An institutional series of 167 (67 index, 100 nonindex) consecutive carriers of RET gene point mutations in exons 10, 11, 13, 14, or 16 who underwent total thyroidectomy between November 1994 and November 2002. RESULTS: Regarding codons 618, 620, 634, 768, 790, and 804, patient age at diagnosis differed significantly depending on the type of pathology encountered (CCH, MTC without and with nodal metastasis). The variability in age, which may reflect the number of necessary somatic mutations, explained the pathological strata in 38% (codon 634) to 77% (codon 768) of patients. Conversely, 62% (codon 634) to 23% (codon 768) of variability in age at different pathological strata may have been determined by the RET genotype. CONCLUSIONS: The pace of malignant progression of the RET genotype should be taken into account when considering prophylactic thyroidectomy in RET gene carriers.

Adolescent↗

PubMed1992

[Value of screening of familial adenomatous polyposis for the prevention of colorectal cancer].

Familial adenomatous polyposis coli is a hereditary autosomal dominant disease which spontaneously and inevitably leads to degeneration of colorectal adenomas and requires preventive surgical treatment. The aim of this study was to evaluate the age of colorectal degeneration and the need for a screening technique in family members. Between 1983 and 1989, 141 patients were treated for familial adenomatous polyposis in our surgical center. Mean age at surgery was 32 years and 64 patients (45.4 percent) had a colorectal carcinoma. Thirty had an in situ tumor (mean age: 30 years) and 34 had an invasive adenocarcinoma (mean age: 45 years), 7 of whom died of their cancer. No colonic cancer was found in patients younger than 20. Thirty-eight percent of the patients under 40 years of age, 73 percent of the patients older than 40 years and 81 percent of those older than 50 had an adenocarcinoma. Fifty percent of the patients with carcinoma were younger than 40 years and 7 percent were less than 25 years old. Seventy-one patients were symptomatic at the time of operation (mean age: 40 years), 32 (45 percent) had a colonic cancer. In 70 patients, familial adenomatous polyposis was detected by screening (mean age: 24) and 2.8 percent had a colonic carcinoma. We conclude that the age-related risk of developing colonic carcinoma requires prophylactic surgery in asymptomatic patients before 20 years of age, and that routine familial screening would be of some benefit.

Adenocarcinoma↗

PubMed1999

Presymptomatic testing for BRCA1 and BRCA2: how distressing are the pre-test weeks? Rotterdam/Leiden Genetics Working Group.

Presymptomatic DNA testing for autosomal dominant hereditary breast/ovarian cancer (HBOC) became an option after the identification of the BRCA1 and BRCA2 genes in 1994-1995. Healthy female mutation carriers have a high lifetime risk for breast cancer (56-87%) or ovarian cancer (10-60%) and may opt for intensive breast and ovary surveillance or prophylactic surgery (mastectomy/oophorectomy). We studied general and cancer related distress in 85 healthy women with a 25% or 50% risk of being carrier of a BRCA1/BRCA2 gene mutation and 66 partners in the six to eight week period between genetic counselling/blood sampling and disclosure of the test result. Questionnaire and interview data are analysed. Associations are explored between levels of distress and (1) expected consequences of being identified as a mutation carrier, (2) personality traits, (3) sociodemographic variables, and (4) experiences related to HBOC. Mean pre-test anxiety and depression levels in women at risk of being a carrier and partners were similar to those of a normal Dutch population. In about 25% of those at risk of being a carrier and 10% of the partners, increased to high levels of general and cancer related distress were found. Increased levels of distress were reported by women who (1) anticipated an increase in problems after an unfavourable test outcome, (2) considered prophylactic mastectomy if found to be mutation carrier, (3) had an unoptimistic personality, (4) tended to suppress their emotions, (5) were younger than 40 years, and (6) were more familiar with the serious consequences of HBOC. Recently obtained awareness of the genetic nature of cancer in the family was not predictive of distress.The majority of the women and their partners experienced a relatively calm period before the disclosure of the test result and seemed to postpone distressing thoughts until the week of disclosure of the result. The low distress levels may partly be explained by the use of strategies to minimise the emotional impact of a possibly unfavourable test outcome. However, a minority reported feeling very distressed. Several factors were found to be predictive for increased distress levels.

Adult↗

PubMed1997

[Influence of carotid atheroma on the neurologic status after myocardial revascularization].

Surgical management of the carotid disease remains controversial in patients affected with coronary artery atheromatous disease. We report the Montreal Heart Institute experience on the influence of carotid disease on postoperative neurologic events of 501 consecutive patients operated on for coronary revascularization during the period from January 1994 to December 1994. There were 381 men and 114 women averaging 62 +/- 9 years old. Major risk factors were high blood pressure (35%), and smoking habit (48%). Fifty-nine patients presented clinical signs of carotid atheromatosis and among them 21 had significant carotid stenosis (> 80% decrease of cross sectional area). During surgery, the mean duration of extracorporeal circulation (ECC) was 76 +/- 31 minutes and the mean perfusion pressure (MPP) was 70 +/- 11 mmHg. The use of inotropic drugs was mandatory in 26% of the cases and the mean arterial lactate (AL) dosage during ECG was 3.07 +/- 1.35 mM/L. During the perioperative period, 13 (2.5%) patients sustained neurologic disturbances of which 5 (1%) were lateralized. Among them, 8 completely recovered whereas 3 of the 5 with permanent damage died. None of the patients with preoperative stigmata of carotid disease experienced lateralized neurologic deficit. Multivariate regression analysis identified the use of vasopressor drugs and perioperative increase of AL as predictive factors. We conclude that in our series, the incidence of neurologic complications was low. The presence of carotid atheromatosis did not increase the postsurgical risk of cerebrovascular accident, however, the increased incidence of neurologic events associated with inotropic drugs and increased AL suggests a direct link with a systemic oxygen debt. Consequently, we do no recommend concurrent prophylactic surgery during coronary artery revascularization.

Adult↗