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Congenital Vascular Anomalies.

Congenital vascular anomalies are rare. The cardiovascular specialist should nevertheless be aware of the more common types of vascular anomalies and understand the implications for patient treatment and the likelihood of associated morbidity. The presentation of congenital arteriovenous malformations can range from asymptomatic or cosmetic lesions, to those causing ischemia, ulceration, hemorrhage, or high-output congestive heart failure. Treatment of large, symptomatic arteriovenous malformations often requires catheter-directed embolization prior to the attempt at complete surgical excision. Later recurrence, due to collateral recruitment, is frequent. Graded compression stockings and leg elevation are the mainstays of treatment for the predominantly venous congenital vascular anomalies. Most congenital central venous disorders are clinically silent. An exception is the retrocaval ureter. Retroaortic left renal vein, circumaortic venous ring, and absent, left-sided or duplicated inferior vena cava are relevant when aortic or inferior vena cava procedures are planned. The treatment of the venous disorders is directed at prevention or management of symptoms. Persistent sciatic artery, popliteal entrapment syndrome, and aberrant right subclavian artery origin are congenital anomalies that are typically symptomatic at presentation. Because they mimic more common diseases, diagnosis is frequently delayed. Delay can result in significant morbidity for the patient. Failure to make the diagnosis of persistent sciatic artery and popliteal entrapment can result in critical limb ischemia and subsequent amputation. Unrecognized aberrant right subclavian artery origin associated with aneurysmal degeneration can rupture and result in death. The treatment options for large-vessel arterial anomalies are surgical, sometimes in combination with endovascular techniques.

Journal Article↗

Treatment options for autonomic neuropathies.

Autonomic disorders may present in a varied fashion. Symptoms that may require treatment include orthostatic intolerance, gastrointestinal distress, sudomotor abnormalities, and urologic and sexual dysfunction. Realistic treatment goals should be outlined for patients, with the expectations that symptoms can be improved, but that the disease is unlikely to be cured and long-term therapy may be required. Orthostatic intolerance is usually treated by a combination of pharmacologic and nonpharmacologic therapies. Nonpharmacologic therapies include compression stockings, adequate fluid and salt intake, and lifestyle modifications. Polypharmacologic therapy is often the standard and can include a volume-expanding agent (such as fludrocortisone) and a sympathomimetic agent. This combination will improve symptoms dramatically in most patients. Treatment of gastrointestinal disorders should address the particular symptom involved: gastroparesis, constipation, or diarrhea. Treatment of sexual and urogenital dysfunction often requires a combination of pharmacologic intervention and lifestyle modifications for peak effectiveness. Patient education is the cornerstone of treatment in any of the autonomic disorders. Given enough education and motivation, many patients will be able to participate in their treatment, which will provide a feeling of empowerment and control over a disease that is often frustratingly resistant to treatment.

Journal Article↗

A controlled trial of acute and long-term medical therapy in tilt-induced neurally mediated syncope.

To study the efficacy of medical treatment for preventing syncopal recurrences in patients affected by tilt-induced neurally mediated syncope, a randomized placebo-treatment prospective study was performed in 30 patients (10 men and 20 women, mean age 42 +/- 21 years) who had syncope reproduced in 2 consecutive head-up tilt-table tests without pharmacologic intervention (n = 20) or during isoproterenol infusion (n = 10). Patients were randomly assigned to 2 groups: 15 to placebo, and 15 to drug therapy (determined on the basis of serial pharmacologic tilting tests). Therapy was either atenolol (n = 7), dihydroergotamine (n = 2), domperidone (n = 2), cafedrine (n = 1), or elastic compression stockings, alone or in association with drugs (n = 3). During a mean of 10 +/- 7 months of follow-up, syncope recurred in 3 patients (20%) in the treatment group and in 4 (27%) in the placebo group; actuarial rates of absence of syncopal recurrences after 20 months were 70 and 67%, respectively. Thus, the outcome of either treated or untreated patients was favorable (with a low recurrence rate of syncope), and the usefulness of tilting-guided medical therapy remains uncertain.

Adult↗

The use of thromboembolic prophylaxis in oral and maxillofacial surgery.

OBJECTIVES: To investigate the use of thromboembolic prophylaxis in oral and maxillofacial surgery. DESIGN: Postal questionnaire. SETTING: Oral and maxillofacial surgery departments in the UK. SUBJECTS: Consultant Oral and Maxillofacial surgeons as listed in the BAOMS Handbook of Hospitals, Oral and Maxillofacial Surgery. MAIN OUTCOME MEASURES: Factors used in the decision to use thromboembolic prophylaxis and the methods of prophylaxis used. RESULTS: The response rate was 73%. Previous thromboembolism (85% of respondents), prolonged immobilisation (79%) and length or magnitude of operation (78% and 74%) were the most commonly used risk factors, in the decision to use prophylaxis against DVT. Pre-operative trauma, cardiovascular disease and varicose veins were the risk factors most infrequently used. No forms of prophylaxis were used by 18% of respondents, with the remaining respondents using a variety of methods. The most widely used methods were graduated elastic compression stockings (74%) and low dose subcutaneous heparin (73%). CONCLUSION: The majority of known risk factors were taken into account when considering the use of prophylaxis against DVT. The methods of thromboembolic prophylaxis used in oral and maxillofacial surgery are considered to provide a satisfactory degree of protection, in moderate and low risk patient groups.

Bandages↗

Optical coherence tomography under general anesthesia in a child with nystagmus.

PURPOSE: To describe a method that permits optical coherence tomography scanning to be performed under general anesthesia. DESIGN: Observational case report. METHODS: A 7-year-old child underwent optical coherence tomography scanning under general anesthesia. He was positioned prone, with the neck extended to enable vertical facial alignment on the scanner. An anti-Trendelenburg trend on the operating table minimized the degree of neck extension required. Intravenous fluids, modest ventilator airway pressures, and compression stockings were used to minimize perioperative hemodynamic disturbance and the potential for orthostatic hypotension associated with the anti-Trendelenburg trend. RESULTS: High-quality, diagnostically important optical coherence tomography scans were obtained. CONCLUSION: With appropriate patient positioning and anesthetic management, optical coherence tomography scanning can be performed on uncooperative adults and children under general anesthesia.

Anesthesia, General↗

[Prophylaxis of thromboembolic events during urologic surgery].

Few scientific evidences are available in the literature, and the methodologic quality of the studies is often under average. Nevertheless, the conclusions are the following. Nephrectomy, renal transplantation, open surgery of the lower urinary tract and lumbar or pelvic lymph nodes dissection are at high risk for thromboembolic events. Other open or endoscopic urological procedures are at low risk. The laparoscopic approach doesn't change the risk associated with the procedure itself. Thromboprophylaxis is recommended in high-risk procedures. There was no evidence to recommend starting the prophylaxis before more than after the procedure. The use of low molecular weight heparin is recommended for prophylaxis. It can be associated with compressive stockings. It is recommended to treat for around seven days after the procedure. In case of cancer surgery, prophylaxis could be needed for four to six weeks.

Humans↗

[Thromboprohylaxis in orthopedic surgery and traumatology].

Orthopaedic and trauma surgery are classified according 3 groups of venous thromboembolic risk. Elective total hip replacement (THR) or total knee replacement (TKR), hip fracture surgery or trauma patients are at high risk. Isolated lower extremity injury with fracture is at moderate risk whereas this risk is low without fracture as well as with knee arthroscopy. In THR and TKR, low molecular weight heparin (LMWH), fondaparinux or melagatran-ximelagatran are strongly recommended. The routine use of other anticoagulants, in particular vitamin K antagonist are not recommended. In patients at high risk of venous thromboembolism as for example trauma patients, optimal use of intermittent pneumatic compression is an alternative option in case of contra-indication to anticoagulant prophylaxis. Graduated compression stockings enhance the efficacy of pharmacological methods. In schedule surgery, initiation of prophylaxis with LMWH may be started postoperatively. To reduce the haemorrhagic risk of anticoagulants, timing of first postoperative dose is essential and is proper to each drug. Duration of prophylaxis depends on the surgical and the individual patients' risk. Extended prophylaxis in THR for up to 42 days with LMWH and up to 35 days with fondaparinux in hip fracture surgery is recommended. However extended prophylaxis after 14 days in TKR has not demonstrated a higher efficacy and should only be considered for patients with additional risk factors. In patients with isolated lower extremity injury or undergoing knee arthroscopy, LMWH should not be routinely used according to a low or a moderate risk and/or the duration of prophylaxis required. But LMWH has to be considered for patients with additional risk factors. Prophylaxis in other orthopedic procedures has not been assessed and will be extrapolated from the above recommendations.

Animals↗

Painful fat syndrome in a male patient.

Painful fat syndrome or lipoedema is a distinct clinical condition, characterised by bilateral and symmetrical enlargement of the upper and lower leg with tenderness, but excluding the feet. Lipoedema occurs almost exclusively in females, and no male patient has been reported in the literature published in English. We report on an extremely rare presentation of lipoedema in a male patient. A thorough study based on the case history, physical manifestations, and magnetic resonance imaging (MRI) provided valuable clues for a differential diagnosis. Conservative treatment using weight reduction, compression-stocking application, and diuretic therapy was not effective. Tumescent liposuction with postoperative pressure garments provided a satisfactory treatment.

Adiposis Dolorosa↗

Quality of life in patients with vascular malformations of the lower extremity.

With the exception of capillary malformations (port-wine stains), the adverse psychosocial effects of vascular malformations have not received much attention in the medical literature. We, therefore, studied health related quality of life of patients with vascular malformations located primarily on the lower extremity, who presented to us over a 10-year period. Patient's self-assessment of quality of life was measured by the Multiple Outcomes Study (MOS) Short Form Health Survey Questionnaire (SF-36). Several possible predictors were also examined. Eighty-one patients (33 male, 48 female), aged 14-61 years, completed the SF-36. Seventy-one patients (88%) had a low flow lesion. Twenty-three patients (28%) had hypertrophy of the lower extremity, while 11 patients (14%) had hypotrophy. Sixty-nine patients (85%) had the vascular malformation located only on the lower extremity. Six (7%) also had their upper extremity (hand) involved, and 20 patients (24.7%) had >10% TBSA affected. Eight patients (10%) needed special shoes. Fifty-one patients (63%) had a previously performed MRI, of which 62.7% (32/51) had muscle involvement. Fifty-one patients (63%) had been operated on. Of the 34 patients wearing elastic compression stockings, 25 patients (74%) indicated that they were satisfied with the stockings. Compared to the general population sample, the SF-36 questionnaire indicated that our vascular malformation patients reported impaired vitality and higher levels of pain, while no differences were seen regarding the other dimensions of quality of life. Demographic, clinical and therapy characteristics could explain quality of life only to a limited extent. Explained variability of the SF-36 dimensions ranged from 0% for mental health to 34.5% for role functioning physical. To our surprise, this study suggests that patients with vascular malformations located primarily on the lower extremity do not have a greatly decreased quality of life when compared to the general Dutch population.

Adolescent↗

Travel and thrombosis.

The available evidence suggests an association between long distance travel and the development of venous thromboembolism. The main problem for travellers and physicians is the interpretation of the evidence and its translation into appropriate advice on the risk and the prevention of thrombosis. Most available data relate to air travel. Thrombosis risk is greater following journeys of more than 8 h and those at greatest risk are travellers with a history of venous thromboembolism or risk factors. Based on the best evidence available the risk of symptomatic venous thromboembolism after flights of more than 12 h is 0.5%. It is likely that stasis plays a major role in the aetiology of travel related thrombosis. The evidence for hypobaric hypoxia induced coagulation activation requires confirmation. There is evidence that compression stockings and low molecular weight heparin prevent asymptomatic deep vein thrombosis (DVT) but clinical DVT has been observed in long distance flyers in spite of prophylaxis with aspirin and stockings.

Humans↗

Venous thromboembolism in earthquake victims.

Sudden death from pulmonary thromboembolism following an earthquake has not been reported to date. This is a published case report of a 48-year-old woman who died suddenly of a pulmonary embolism on October 28, 2004. She lived in the Chuetu District in Japan, where a big epicentral earthquake occurred on October 23. She evacuated her house with her family and spent 4 of 5 nights in a private car. Until November 1, 8 persons who had been living out of their cars after evacuation from their houses in the District were treated for pulmonary thromboembolism. The immobility that comes with living in a car should be recognized as a risk factor for venous thromboembolism. In addition, potential preventive measures should be recommended, including provisions for emergency shelters, access to other temporary housing, public education, and the use of compression stockings.

Automobiles↗

Thromboprophylaxis in major abdominal surgery for cancer.

AIMS: To review the epidemiology and pathogenesis of venous thromboembolism (VTE) in surgical cancer patients, in addition to the use of thromboprophylaxis in major abdominal surgery, such as low-molecular-weight heparin (LMWH) and fondaparinux. METHODS: Systematic review of the literature, focussing on risk factors for VTE, parenteral methods of thromboprophylaxis, approaches to prolonged prophylaxis, and effects on patient survival. FINDINGS: Patients with cancer undergoing abdominal surgery are at substantially higher risk for VTE than patients without cancer. Furthermore, prolonged thromboprophylaxis for up to 4 weeks is more effective than short-term administration in these high-risk patients. The concurrent use of graduated compression stockings has a synergistic effect on the reduction in VTE risk. CONCLUSIONS: Thromboprophylaxis with LMWH has been shown to minimise the incidence of thromboembolic events, and is a well-established therapy worldwide. The American College of Chest Physicians recommends the routine use of thromboprophylaxis, with LMWH or unfractionated heparin, in patients with cancer who are undergoing surgical procedures, and the appropriate use of these thromboprophylactic agents has significant implications for the clinical care and quality of life of surgical patients with cancer.

Abdomen↗

Implementation of outpatient treatment of deep-vein thrombosis in private practices in Germany.

OBJECTIVE: Implementation of outpatient treatment (OT) of deep-vein thrombosis (DVT) is slow despite clear evidence that it is effective, safe and cost-efficient. DESIGN AND METHOD: An initiative was launched with the help of the Professional Association of Phlebologists of Germany and the industry to familiarize physicians in private practice who had no prior experience with OT of DVT. Data on quality of treatment with the low-molecular-weight heparin tinzaparin and phenprocoumon, compliance, clinical outcome, venous ultrasound, patients' satisfaction and quality of life were collected in a registry, which was open from July 1999 to December 2000. The results were published and their impact on further management of patients was assessed in second survey reported here. Patients of both series were followed-up clinically and with ultrasound over the 1st month of treatment. RESULTS: Of 67 physicians entering 827 patients into the registry 26 answered a questionnaire on how they treated further patients. Their case load had increased by 450% and data were provided on 540 consecutive patients managed between January and June 2002. OT increased overall from 76 to 92%, that of popliteo-femoral DVT from 71 to 92%, and that of pelvic DVT from 38 to 65%. Medical reasons to decide against OT decreased from 89 to 56% (p<.01). Immediate leg compression was changed from bandaging to medical compression stockings in 20 of the 26 centres (p<.05). In total, data were gathered from OT of 1124 patients. No secondary hospitalisations were required and only one patient had a documented progression of the DVT. CONCLUSIONS: OT was successfully implemented in private practices through the initiative of individual physicians with support of the professional association and sponsoring by the industry-to the benefit of the providers but as much of the patients and their cost bearers.

Ambulatory Care↗

Epidural analgesia improves early rehabilitation after total knee replacement.

STUDY OBJECTIVE: To compare epidural anesthesia and analgesia with spinal anesthesia with intravenous morphine analgesia for its effect on range of motion (ROM) and early rehabilitation after total knee replacement. DESIGN: Randomized prospective study. SETTING: Tertiary care, academic medical center. PATIENTS: Thirty-eight patients scheduled for total knee replacement. INTERVENTIONS: Patients were randomized into 2 groups. One group received spinal anesthesia with 0.5% bupivacaine and analgesia with intravenous patient-controlled analgesia morphine, demand mode only. The other group was given epidural anesthesia with 1.0% ropivacaine with 1:200,000 epinephrine and analgesia with 0.2% ropivacaine at 8 mL/h, maintained for 7 days. Both groups had compression stocking for deep venous thrombosis (DVT) prophylaxis, urinary catheter for the first 24 hours, and duplex scanning at days 3 and 10. The spinal group received low molecular-weight heparin for DVT prophylaxis. MEASUREMENTS: Data collected included pain scores at rest, and with ROM, frequency of DVT, and patient satisfaction. Data were evaluated with Wilcoxon rank sum test for continuous variables and Fisher exact test for categorical variables. Data were considered significant at P < .05. MAIN RESULTS: All 38 patients finished the study, 22 in the spinal group and 16 in the epidural group. There was no difference in demographics between groups. The pain sores at rest and with ROM were significantly less in the epidural group. ROM was better in the epidural group compared with the spinal group after day 1. No DVT was detected on day 3 or 10 in either group. No patient in either group required reinsertion of bladder catheter for urinary retention. CONCLUSION: By using epidural analgesia in the first 7 days postoperatively, we achieved improved early rehabilitation due to excellent pain relief effect and an antithrombotic effect with an efficacy comparable to low molecular-weight heparin.

Adult↗

Calf compression pressure required to achieve venous closure from supine to standing positions.

BACKGROUND: Compression therapy needs to narrow the veins of patients with venous disorders to achieve a hemodynamic effect. This study investigated the external pressure necessary to narrow and occlude leg veins in different body positions. METHODS: In nine healthy volunteers and five patients with incompetent small saphenous veins, the diameter of the small saphenous veins and of one posterior tibial vein was measured at the mid level of the calf by duplex ultrasound scans in sitting, standing, and supine positions. A modified blood pressure cuff with an acetate window that permitted ultrasound visualization of the veins was gradually inflated, and the pressures needed to narrow or occlude the veins were recorded. RESULTS: Initial narrowing occurs with a median pressure of between 30 and 40 mm Hg in the sitting and standing positions. Complete occlusion of superficial and deep leg veins occurs with 20 to 25 mm Hg in the supine position, between 50 and 60 mm Hg in the sitting position, and at about 70 mm Hg in the standing position. The difference between sitting and standing, and between standing and supine, was statistically significant (P < .01 and P < .001, respectively). CONCLUSION: The external pressure has to exceed the hydrostatic pressure in the vein to compress leg veins effectively. Higher external pressures than can be expected to be delivered by elastic compression stockings are required to achieve the collapse of lower-extremity veins in the upright position.

Adult↗

Clinical presentation and venous severity scoring of patients with extended deep axial venous reflux.

BACKGROUND: The objective of this study was to evaluate the prevalence and profile of patients presenting with chronic venous insufficiency (class C3-C6) and cascading deep venous reflux involving femoral, popliteal, and crural veins to the ankle. METHODS: From September 2001 to April 2004, 2,894 patients were referred to our center for possible venous disorders. The superficial, deep, and perforator veins of both legs were investigated with color duplex scanning. The criterion for inclusion in this study was the existence of cascading deep venous reflux involving the femoral, popliteal, and crural veins to the ankle whose duration had to be longer than 1 second for the femoropopliteal vein and longer than 0.5 seconds for the crural vein. The advanced CEAP classification, the Venous Clinical Severity Score (VCSS), the Venous Segmental Disease Score (reflux; VSDS), and the Venous Disability Score (VDS) were used. RESULTS: Seventy-one limbs in 60 patients were identified. Eleven limbs (15.5%) were classified as C3, 36 (50.7%) as C4, 21 (29.6%) as C5, and 3 (4.2%) as C6. A primary etiology was identified in 11 (15.5%) limbs, and a postthrombotic etiology was identified in 60 limbs (84.5%). In the latter group, all but four patients were aware that they had had a previous deep venous thrombosis. In addition to femoropopliteal and calf veins, reflux was present in the common femoral vein in 60 (84.5%), the deep femoral vein in 27 (38%), and the muscular calf veins in 62 (87.3%). Incompetent perforator veins were identified in 53 (74.6%) limbs. Fifty-one (71.8%) limbs had a combination of superficial venous insufficiency (AS(2), AS(2,3), AS(4), or their combination) previously treated or present. Of these, 11 had primary etiology alone, and 40 had a secondary etiology with or without primary disease. Means and 95% confidence intervals of the VCSS, VSDS, and VDS were 9.72 (8.91-10.53), 7.2 (6.97-7.42), and 1.08 (0.83-1.32), respectively. A significant increase in the VCSS and in the VSDS (P < .0001) paralleled the CEAP clinical class. The VDS was higher in the C3 and C6 classes but did not reach significance. There was a significant link between the pain magnitude in the VCSS and the VDS (P < .0001). Severity of pain and high VDS did not depend on the wearing of elastic compression stockings. VCSS increased significantly according to the presence of an incompetent perforator vein (P < .05) and/or reflux in the deep femoral vein (P < .05). CONCLUSIONS: This study confirmed the value of the Venous Severity Score as an instrument for evaluation of chronic venous insufficiency. A significant increase in the VCSS and VSDS paralleled CEAP clinical class; VDS was higher in classes C3 and C6 without reaching significance, probably because of the small size of the samples. Some clinical and anatomic features need to be clarified to facilitate scoring.

Adult↗

Vitrectomy for phacolytic glaucoma in a patient with homocystinuria.

PURPOSE: To determine the successful treatment of unilateral phacolytic glaucoma by vitrectomy and trabeculectomy in a patient with homocystinuria whose lens had dislocated into the vitreous at least 15 years earlier. METHODS: In a 32-year-old woman with homocystinuria, bilateral dislocation of the lens into the vitreous, and phacolytic glaucoma in her left eye a three-port pars plana vitrectomy was performed with the patient under general anesthesia. The lens was removed and a trabeculectomy fashioned. Special precautions for general anesthesia included preoperative aspirin and compression stockings for thromboembolic prophylaxis and intraoperative dextrose infusion, 5%, to maintain intravascular volume and prevent hypoglycemia. RESULTS: The intraocular pressure and uveitis resolved postoperatively with improvement in the visual acuity and intraocular pressure, which returned to normal without further treatment. CONCLUSIONS: Phacolytic glaucoma is best resolved by removal of the exciting lens material. Pars plana vitrectomy with the patient under general anesthesia can be carried out safely despite the risks traditionally associated with homocystinuria.

Adult↗

Iliocaval complications of retroperitoneal fibrosis.

BACKGROUND: Retroperitoneal fibrosis can compress ureters, nerves, and blood vessels in the abdomen. However, clinically significant large-vein obstruction secondary to this process is rare. METHODS: Three hundred forty patients with retroperitoneal fibrosis were treated at our institution between 1976 and 1993. The clinical data from seven of these patients, who were treated for iliocaval complications of retroperitoneal fibrosis, were reviewed. RESULTS: Six patients had signs and symptoms of chronic obstruction, and one patient presented with acute iliocaval thrombosis and underwent attempt at venous thrombectomy in the referring hospital. All patients exhibited extremity edema and three had venous claudication. Iliocaval occlusion was confirmed in all patients by venography, computed tomography, or magnetic resonance imaging. The obstructive process involved the iliocaval tree (four patients), the inferior vena cava alone (two patients), and the iliac vein alone (one patient). Five patients were managed conservatively with leg elevation, compression stockings, and anticoagulation. Two patients received prednisone. One patient underwent an iliocaval bypass from the external iliac vein to the juxtarenal cava using a ringed polytetrafluoroethylene graft with a femoral arteriovenous fistula. A second patient with an isolated left common iliac vein obstruction underwent a left-to-right femorofemoral saphenous vein bypass. Four patients treated conservatively continued to have extremity edema. The two patients managed surgically remain asymptomatic from venous insufficiency, with patent grafts at 25 and 12 months after surgery, respectively. CONCLUSION: Iliocaval obstruction is an unusual complication of retroperitoneal fibrosis. Although most cases can be managed conservatively, reconstruction is an option for patients who have failed medical treatment and are symptomatic secondary to chronic venous obstruction. Lifelong anticoagulation should be considered for all patients with progressive iliocaval obstruction secondary to retroperitoneal fibrosis.

Acute Disease↗