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PubMed2003

[Problems raised by the gynaecologic management of women with BRCA 1 & 2 mutations].

Breast and ovarian cancer occur more frequently in young women with BRCA 1 &2 mutations (respective cumulative risks from 37 to 85% and 10 to 20%), and raise specific gynaecologic problems as prophylactic surgery and hormonal treatments. Two medical files from 2 sisters with BRCA 1 mutation (exon 11) are presented and the authors discuss the therapeutic options chosen. BRCA 1 & 2 tumour suppressor genes seem to play a major role in the repair of cellular damages inducing by the estrogenic proliferative signal. The prophylactic mastectomy is effective for the breast cancer prevention but its acceptance is low. The oophorectomydecreases the ovarian risk (knowing the occurrence of peritoneal carcinomatosis in 1.8% of cases) and currently the breast cancer risk (RR = 0.47) by the hormonal privation: the hormone replacement therapy does not seem to increase the breast cancer risk in the small series published. The HRT is possible in women with BRCA mutation under medical supervision and if the doses of hormones are light. The first results concerning the chemoprevention by Tamoxifen are encouraging (RR = 0.38) in these patients, but more studies are needed. The oral contraception exerts an uncertain effect against ovarian cancer, but possibly enhances the breast cancer risk in this group of women (RR = 3.3). The management of women with BRCA mutation is varying according to their own priorities, which can change during their life.

Adolescent↗

PubMed2004

Late onset vitreoretinal complications of regressed retinopathy of prematurity.

AIM: To report the clinical findings, management, and outcomes in eyes undergoing surgery for regressed retinopathy of prematurity (ROP) with vitreoretinal complications. METHOD: Retrospective review of 40 eyes of 32 patients with regressed ROP who presented between 1989 and 2001 at two UK referral centres. RESULTS: Of 29 eyes presenting with rhegmatogenous retinal detachment (RRD), 15 initially underwent a scleral buckling procedure and 14 initially underwent vitrectomy with or without additional buckling. Primary surgery was anatomically successful in 11/15 eyes that underwent a non-vitrectomy retinal detachment repair and 8/14 that required vitrectomy. The final reattachment rate after reoperation was 28/29 eyes. Median visual acuity improved from 6/60 to 6/36 following retinal detachment repair. A further 11 eyes of eight patients from this series underwent prophylactic surgery, laser, or cryotherapy for predisposing vitreoretinal pathology and/or retinal breaks, all of which were stabilised. CONCLUSIONS: In eyes with RRD and signs of regressed ROP successful reattachment of the retina can be achieved using either vitrectomy or external surgery with an associated overall improvement in visual acuity. A range of external and closed microsurgical approaches is required to effectively deal with the diverse manifestations of regressed ROP.

Adolescent↗

PubMed2006

Surgery for prolactinomas.

A large proportion of prolactin secreting tumours of the pituitary gland are treatable by dopamine agonist drugs. However certain subgroups of these patients are better managed by surgical excision. The indications for surgery in prolactinomas remain controversial and this paper attempts to clarify these with illustrative cases. The factors favouring a surgical approach include poor tolerance of long term medical therapy, progression of visual deficits or recurrence while on medical treatment, cystic change in the tumour with pressure effects, CSF fistula secondary to tumour shrinkage and prophylactic surgery in tumours anticipated to cause CSF leak following medical therapy. The size and invasiveness of the tumour and the prolactin level are also important determinants of treatment planning. The indications for surgery in prolactinomas are discussed and illustrative cases are presented.

Adolescent↗

PubMed1977

Asymptomatic coronary artery disease and coronary bypass surgery.

The actuarial survival curves of "medically treated" patients whose arteriographic studies demonstrated coronary arterial lesions of various degrees-- now used widt applicable to the asymptomatic patient. No information is available regarding the course or prognosis of the asymptomatic patient with demonstrated lesions in the coronary arteries. For the reasons explained one can propose a hypothesis that the overall prognosis of this type of patient is better than average, probably better than that shown in the best data collected on symptomatic patients. The prophylactic value of aortocoronary bypass operations in preventing myocardial infarction and death has not been established. One can therefore question the justification for the wide case-finding effort of subjecting asymptomatic persons to coronary arteriography, even in light of the low risk of this procedure, unless unusual findings suggest an especially poor prognosis (one example might be past myocardial infarction in a very young patient). Although there are exceptional instances when prophylactic surgery is indicated for asymptomatic patients, further investigation of this subject is needed before the procedure becomes generally accepted.

Coronary Angiography↗

PubMed1986

Factors associated with postoperative complications in diabetics after biliary tract surgery.

We reviewed all first operations for cholelithiasis at the North Carolina Memorial Hospital from 1973 to 1982 to assess factors associated with postoperative morbidity in diabetics after biliary tract surgery. Diabetics (n = 126) had more frequent postoperative complications (24.6% vs. 12.5%, p less than 0.001) and higher mortality (7.9% vs. 3.0%, p = 0.02) than the nondiabetics (n = 855). Postoperative complications were more frequent in diabetics at both emergency surgery (57.9% vs. 39.1%) and at nonemergency surgery (18.7% vs. 10.2%). Diabetics were older, however, and had more preoperative renal, cardiovascular, and neurologic disease. We used logistic regression analysis to adjust for these differences and to determine independent predictors of postoperative complications. Urgent surgery, operation other than cholecystectomy, cardiovascular disease, and male sex were associated with an increased risk of postoperative morbidity. Diabetes was associated with an increase in risk that was not statistically significant. We conclude that diabetics have increased morbidity primarily because they are older and have other medical problems. The risk conferred by uncomplicated diabetes is modest, and recommendations for prophylactic surgery in asymptomatic diabetics with gallstones should be reexamined in this light.

Cholelithiasis↗

PubMed1987

[Cancer of the gallbladder in the Côte d'Or].

The registry of digestive tract tumours established for the department of Côte d'Or was used to study the epidemiologic characteristics and treatment of gallbladder cancer. During 8 years (1976-1983), 139 new cases were recorded. Age standardized incidence rates based on world standard population were 3.1/100,000 for females, 0.9/100,000 for males. As in all population-based studies, there was a female predominance. As compared with the data of other cancer registries the risk in the Côte d'Or is in the intermediate range. The incidence of gallbladder cancer was similar in urban and rural areas, but high risk lieus were identified by the study. The most frequent histologic type was adenocarcinoma (79.5 p. 100). Only 20.9 p. 100 of the patients underwent curative surgery. The overall 1-year survival rate was 14.4 p. 100 and the 5-year survival rate was 2.9 p. 100. The 5-year survival rate was 11.5 p. 100 after curative surgery. These results underline the fact that the prognosis of gallbladder cancer in a well defined population remains poor. The frequency of gallstones was 73.3 p. 100 in females and 56.5 p. 100 in males. Although this association is frequent few people with cholelithiasis develop gallbladder cancer. Among patients with gallstones, a subgroup at high risk of gallbladder cancer, in whom prophylactic surgery might be justified, remains to be identified.

Aged↗

PubMed1996

[Infrarenal abdominal aortic aneurysm--indications for surgery and survival].

PURPOSE: Elective operation is an established therapy in the management of infrarenal aortic aneurysms. Surgery has an acceptable low risk in the presence of experienced surgeons and anaesthetists. Indication for surgery nevertheless remains a difficult problem, because diagnostic procedures and evaluation of different criteria do not exactly predict the likelihood of rupture. Indication for surgery is moreover dependent on operative mortality and late survival. This study analyses early and late results associated with the repair of abdominal aortic aneurysms in our institution in Hamburg and comments on indication for elective surgery. METHODS: Life-Table analysis was used to determine survival rate. Calculation of expected prevalence of abdominal aortic aneurysms in the Hamburg population was based on demographic statistics and results in the literature. RESULTS: Between January 1981 and April 1995 1242 patients underwent abdominal aortic aneurysm repair, 1018 electively, 224 for ruptured aneurysm. Operative mortality was 4% and 50% respectively. The survival rate after elective operation was 64% at 5, 43% at 10 and 35.4% at 14 years, while after emergency surgery for ruptured aneurysm it was 40% at 5, 31.9% at 10 and 29.8% at 14 years. The survival rate of survivors after repair for ruptured aneurysms is similar to that after elective surgery. Over a 13 years period the rate of ruptured aneurysms did not decrease. Comparison of surgical data and calculated prevalence data show that a three times higher operative activity is requested to repair all occurring abdominal aortic aneurysms larger than 5 cm in diameter. CONCLUSIONS: In order to reduce the still high mortality associated with aneurysm rupture there is still a need for more prophylactic surgery.

Aged↗

PubMed1997

[Our experience in portal cavernomatosis in childhood: cross-sectional study and analysis of results].

A retrospective and transversal study of our patients with portal cavernomatosis is developed, with the aim of comparing the evolution and the present status of the patients that have undergone different therapeutical approaches. Between 1975 and 1995, 15 patients with portal cavernomatosis have been treated. Personal history, signs and symptoms at the moment of diagnosis, and treatment were analyzed. Now, the controls have been made by physical examination and ECO-Doppler of the espleno-portal system. In 6 cases the absence of bleeding made surgery unnecessary. In the 9 patients with digestive haemorrhage, it could be controlled pharmacologically, with a later surgical approach. Now, all non-operated and 6 operated patients show splenomegaly, without bleeding. The ECO-Doppler shows colateral flow, the splenomegaly in the non-operated and the surgical shunts and the hepatofugal circulation in the operated patients. Looking at our results, we believe that prophylactic surgery is not indicated. In those cases of digestive bleeding, surgery after the complete resolution of the acute disease shows good results at a short and long term.

Child, Preschool↗

PubMed1994

[The clinical and echocardiographic factors predictive of bioprosthetic leaflet rupture. The clinical tolerance for prosthetic malfunction].

OBJECTIVE: The objective of this research was to determine the existence of predictors of bioprosthetic valve disruption depending on the echocardiographic characteristic of the biologic prosthesis. It also determines the factors that provoke a need of emergency surgery and a bad clinical tolerance to the prosthetic dysfunction. METHODS: Clinical and echocardiographic features of two groups of 28 and 21 patients, all of them carrying bioprosthesis, were compared. The groups were homogeneous in sex, age, location and durability of the prosthesis. The first group showed leaflet disruption in a period of 6 months to 1 year after the study, not in the second group. Calcification, thickness and mobility of leaflets, prosthetic function were studied. An echocardiographic score was given between 4 and 10 points. Left ventricular function and pulmonary pressure were also studied. RESULTS: Valve thickness was statistically different in both groups (95% of the first group vs 71.3% of the second, p = 0.0281). There was no significant difference in calcification (52% vs 38.7%), nor in leaflet mobility (71.5% normal in the first group vs the 80% of the second group), nor in the prosthetic function (52.3% vs 76.6%, respectively). It was not found in the echocardiographic score either. Left ventricular function and pulmonary pressure were 91.6% and 55% normal respectively in the first group. They were 80% and 45.83% normal in the second group. Clinical onset of disruption was: 5 asymptomatic patients, 2 patients noticed a change in prosthetic click, 14 cases with progressive dyspnea, 6 patients with acute pulmonary edema and 1 patient with cardiopulmonary arrest and effective resuscitation. Symptoms were kept under control with medical treatment in 22 patients (78.55%) and 6 patients were referred to surgery. These latter suffered from pulmonary hypertension and two of them had left ventricular dysfunction. Surgical mortality was 3% (1 patient). CONCLUSIONS: No echocardiographic features were found as being predictors of imminent bioprosthetic disruption. However, leaflet thickness is the most common finding. Symptoms of disruption in patients without high surgical risk factors (left ventricular dysfunction or pulmonary hypertension) are kept under control with medical treatment. Therefore, "prophylactic" surgery is not needed in bioprosthesis with signs of degeneration and normal hemodynamic performance.

Adolescent↗

PubMedDate not supplied

Prophylaxis and screening options: recommendations for young women with BRCA mutations.

Young women who carry a BRCA mutation face difficult decisions regarding radiologic screening modalities and prophylactic surgery. Decisions regarding these choices may have short and/or long-term consequences and significant impacts on breast cancer risk. A comprehensive review of currently available data supports the following recommendations for young women with BRCA mutations: perform monthly self breast exam, obtain clinical breast exam 2-4 times per year, discuss annual imaging options with a breast specialist, complete child-bearing by 35 with subsequent prophylactic oopherectomy, avoid hormone replacement therapy, and consider prophylactic mastectomy.

Adult↗

PubMed2006

Monozygotic twins with Marfan's syndrome and ascending aortic aneurysm.

Marfan's syndrome is a hereditary connective tissue disease, in which cardiovascular abnormalities (especially aortic root dilatation) are the most important cause of morbidity and mortality. In this report, we describe two 24-year-old twins, with a history of surgery for lens subluxation and severe cardiovascular manifestations secondary to Marfan's syndrome. One of the twins suffered a type A aortic dissection, which required replacement of the ascending aorta, and the other twin had an aneurysmal dilatation of the ascending aorta (46mm) and was prescribed medical treatment with atenolol and periodic controls to detect the presence of a critical diameter (50mm) that would indicate the need for prophylactic surgery.

Adult↗

PubMed1998

Low molecular weight heparins for venous thromboembolism.

Five years ago, we concluded that in patients undergoing major orthopaedic surgery, prophylactic use of a low molecular weight heparin (LMWH) gave greater protection against deep vein thrombosis (DVT) than did conventional unfractionated heparin (UFH). The risk of bleeding appeared to be the same. It was unclear whether the clinical advantages of the LMWHs offset their greater cost. In the UK, four LMWHs ([symbol: see text]certoparin, dalteparin, enoxaparin and tinzaparin) are now licensed for prophylaxis against venous thromboembolism during or after surgery; a fifth (nadroparin) is licensed but not yet marketed in the UK. Dalteparin, enoxaparin and tinzaparin are licensed for the treatment of DVT. Additionally, tinzaparin is licensed for the treatment of pulmonary embolism (PE). Here, we review the use of LMWHs for these indications.

Anticoagulants↗

PubMed2000

Extraintestinal polyps in Peutz-Jeghers syndrome: presentation of four cases and review of the literature. Deutsche Peutz-Jeghers-Studiengruppe.

Peutz-Jeghers syndrome (PJS) is a rare hereditary disorder characterized by hamartomatous polyps in the gastrointestinal tract and typical pigment lesions. Extraintestinal polyps have rarely been reported. Possible sites include the respiratory tract, urogenital tract, and gallbladder. We here describe four cases of extraintestinal polyps in PJS patients and review the literature on the need for operative therapy of extraintestinal polyps in PJS. Three nonrelated patients were examined who had PJS and polyps in the gallbladder; the fourth patient had PJS and recurrent choanal polyps. Surgery has so far been performed only for symptomatic polyps: one laparoscopic cholecystectomy and removal of the choanal polyps for recurrent infections of the respiratory tract. The remaining two patients reported no symptoms from the extraintestinal polyps. No malignant transformation was found in these patients, nor has such been reported in the literature on PJS. The frequent observation of this manifestation in our patients raises the question of clinical management: Is prophylactic surgery indicated? Since malignant transformation of PJS polyps in the intestine is extremely rare we see no reason for operative therapy as long as the polyps are small and asymptomatic. Regular sonographic controls are recommended since the risk of malignant transformation cannot be ruled out at present.

Adult↗

PubMed2000

[The congener eye in patients with unilateral classical malignant glaucoma].

PURPOSE: To prospectively study in a follow-up period, ranging from 5 to 20 years, "the fate" of the fellow eye in patients with unilateral classical malignant glaucoma. METHODS: The fellow eye of 10 patients had been thoroughly examined immediately after the beginning of an adequate therapy of the unilateral classical malignant glaucoma. RESULTS: In 6 cases the fellow eye was apparently normal and 4 cases exhibited an intermittent primary angle-closure glaucoma. Surgery of the fellow eye was accepted immediately by two patients; in one a peripheral iridectomy was carried out and in the other one a trabeculectomy. The other 8 patients initially turned down surgery but accepted in thereafter in a period of time ranging between 1 and 8 years. A peripheral iridectomy was performed in two cases, a trabeculectomy in other two and a combined procedure (trabeculectomy with pars plana aspiration of the aqueous trapped within the vitreous in 3 cases and the same procedure plus lens extraction on one patient) in the remaining 4 cases, since they developed into a primary chronic angle-closure glaucoma. CONCLUSIONS: Prophylactic surgery should be best done when the fellow eye is apparently normal having an entirely open angle and both the ocular tension and the visual function normal. The treatment of the advanced primary chronic angle-closure glaucoma of the fellow eye consists in trabeculectomy with pars plana aspiration associated with cataract extraction if the lens is opaque.

Adult↗

PubMed1997

Mesenteric revascularization during aneurysmectomy.

The most important association between aortic aneurysm surgery and the mesenteric circulation is the potential for inadvertent compromise to the mesenteric trunks and their end organs. Preservation of visceral blood flow is a critical objective in each case, using methods that have been developed as techniques for suprarenal abdominal and thoracoabdominal aneurysmectomy have evolved. Beyond simply preserving what existed, revascularization by endarterectomy or bypass has a role in selected cases to improve the natural history of advanced mesenteric occlusive disease, although the rationale for such prophylactic surgery must remain relatively weak until more is learned about disease progression in the visceral arteries. Finally, a small number of patients with mesenteric ischemic symptoms at the time of aneurysmectomy clearly benefit from combined surgery.

Aortic Aneurysm, Abdominal↗

PubMed1979

Clinical and morphological characteristics of colitis carcinoma and colorectal carcinoma in young people.

Two series of young patients (less than 40 years of age) with colorectal carcinoma (22 idiopathic carcinomas and 25 carcinomas complicating ulcerative proctocolitis), well matched for age and sex, were compared with regard to clinical features, tumour morphology and stage, and ultimate outcome after surgery. The cure rate in both series was low. Although a failure to diagnose colitis carcinoma accurately at an early stage might have contributed to the poor results, such a delay could hardly be responsible for the bad prognosis in patients with idiopathic carcinoma. The vast majority of the patients in both groups studied had highly malignant and/or mucoid adenocarcinoma, and surgery was palliative in about 40% of the patients in both series, owing to widespread dissemination. The general impression gained from this study of factors of histologic grade of malignancy, extent of spread, and survival rate was that colorectal carcinomas in the young, irrespective of being idiopathic or complicating ulcerative colitis, run a rapid course and have a gloomy prognosis. The outloook depends largely on the biologic characteristics of the tumours concerned. The results support previous statements that prophylactic surgery is justified in patients with long-standing ulcerative colitis with total involvement of the colon, particularly in the young. Regrettably, patients with idiopathic carcinoma will not have this chance.

Adenocarcinoma, Mucinous↗

PubMedDate not supplied

[Etiopathogenic factors in colorectal cancer. Genetic and clinical features (first of 2 parts)].

INTRODUCTION: Colorectal Cancer (CRC) is the second cause of death in Spain, reaching the first place in non-smoking population. It is also the tumour with the greatest one-year incidence. The last few years some reduction in its incidence along with a greater 5-year average survival has been observed. PATHOGENESIS: Most CRCs develop from benign polyps. Vogelstein's hypothesis suggests an orderly progression from normal mucosa, to a small polyp, to a large polyp and finally to a CRC. In this progression transforming cells will be increasingly charged with molecular alterations. GENETIC FACTORS: 20% of CRCs have a genetic background although only a fourth of these are genetically based. The APC (or FAP) gene mutation is one of the earlier events that can be seen in tumour progression. When this mutation appears in the germ line it renders Familial Adenomatose Polyposis Syndrome (that evolves to CRC in a 100% of cases) or to the Gardner Syndrome (if it has extracolonic expressions). The p53 mutation is a late event in cellular transformation but it makes a rapid accumulation of mutations an easier process and it confers advantages for survival of the tumour cells. Mutations in the genes implicated in microsatellite instability are related to DNA error repair; their clinical correlates are the Lynch I and II syndromes. Other important genes related to CRC are DCC and ras. CLINICAL FACTORS: Inflammatory Bowel Disease (particularly Crohn's disease) is the most important clinical factor, it may enhance basal risk 30 times. Other risk factors are: previous malignant conditions, pelvic irradiation and previous surgery (cholecystectomy and ureterosigmoidostomy). The endocrinologic factors have a rising importance and so hormonal substitution therapy for menopause and multiparity can provide some protection. The use of NSAIDs can also be protective (specially COX-2 inhibitors). CONCLUSIONS: It is important that people who are identified as having a high risk for developing a CRC are subjected to a strict clinical and endoscopic follow-up in order to be able to identify an incipient tumour or to practice prophylactic surgery. The use of hormonal substitution therapy or anti COX-2 NSAIDs can be future useful chemoprophylactic agents.

Colorectal Neoplasms↗

PubMed1997

Importance of management of unruptured cerebral aneurysms.

BACKGROUND: Since excellent recovery is hardly expected in patients with severe subarachnoid hemorrhage, management of unruptured aneurysms is essential in reducing the overall mortality and morbidity rates. The widespread use of less invasive imaging tools such as magnetic resonance angiography (MRA) has made "Brain Check-up Systems" very popular in Japan. Therefore, unruptured aneurysms have been found much more often than before. METHODS: During the 1-year period of 1994, 80 patients underwent radical surgery for aneurysms in our institute. Thirty-two patients (40%) underwent surgery for unruptured aneurysms, while 48 patients (60%) underwent surgery for ruptured aneurysms. In patients with unruptured aneurysms, we have operated when the aneurysm is larger than approximately 5 mm in diameter, arising from the common sites in the circle of Willis, and when the patient's age is under 70 years. To prevent surgical complications in unruptured aneurysms, we often use a wrapping technique with Bemsheets (cotton) and adhesives. RESULTS: The outcomes were good in all 32 cases of unruptured aneurysms. CONCLUSIONS: We believe that early detection and prophylactic surgery for unruptured aneurysms will improve the overall outcome of aneurysm treatment in the future.

Aged↗