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Vascular involvement in Behçet's disease: 8-year audit.

Behçet's disease is a multisystemic disorder characterized by recurrent ulcers of the mouth and genitalia and relapsing iritis. Four types of vascular lesion are recognized in Behçet's disease: arterial occlusions, aneurysms, venous occlusions, and variceal development. The incidence of vascular involvement reported in the literature ranges from 7% to 29%. The aim of this study was to determine the rate of vascular involvement in Behçet's disease at our hospital between 1983 and 1992. Of 1200 patients with Behçet's disease, 173 (14.4%) had venous manifestations and 19 (1.6%) had arterial manifestations (in some patients more than one organ was involved). In the group of patients with venous manifestations, there were 154 (12.8%) with venous thrombosis, 17 (1.4%) with vena cava superior syndrome, 5 (0.4%) with inferior vena cava syndrome, 5 (0.4%) with varices, 2 with upper extremity venous thrombosis, 1 with internal jugular vein thrombosis, 1 with cavernous sinus thrombosis, and 1 with hepatic vein thrombosis. In the arterial manifestation group there were 7 femoral, 3 abdominal, 3 popliteal, 2 iliac, 2 pulmonary, 1 axillary, and 1 carotid artery aneurysm as well as 3 arterial occlusions. We concluded that vascular surgeons dealing with young adults should bear Behçet's disease, an uncommon clinical entity, in mind.

Adult↗

Orbital venous congestion in childhood. Diagnostic and therapeutical implications.

A case study of 2 patients with venous congestion of the orbit due to different etiologies is presented. Both the children demonstrated orbital pain, proptosis, chemosis and conjunctival injection. In one case early diagnosis left to complete recovery by conservative treatment in a patient with a pyogenic cavernous sinus thrombosis. In the other case a traumatic carotid sinus cavernous fistula could be treated successfully using transvasculary navigated detachable balloons.

Arteriovenous Fistula↗

Antiphospholipid syndrome: patterns of life-threatening and severe recurrent vascular complications.

PURPOSE: To demonstrate the variety and recurrence patterns of severe arterial and venous thromboembolic events that occur in patients with the antiphospholipid syndrome. MATERIALS AND METHODS: Radiology records were reviewed in 800 of 1,633 patients with positive test results of antiphospholipid syndrome. Patients with radiologic evidence of antiphospholipid syndrome and no other hypercoagulable state were included if the observed thromboembolic event met one or more of four criteria for severity: extreme complications or mortality, three or more recurrent events, unusually young age, and/or unusual sites affected. RESULTS: In the 24 patients who met the selection criteria, 72 thromboembolic episodes and 56 (78%) recurrences were found. Arterial complications included aortic occlusions, visceral infarctions, upper- and lower-extremity arterial thrombosis, strokes, and repeated graft occlusions. Venous complications included portal vein thrombosis, transverse and sagittal sinus thrombosis, upper- and lower-extremity thrombosis, and recurrent pulmonary emboli. CONCLUSION: Vascular complications of antiphospholipid syndrome include serious and life-threatening events. Primary thromboembolic episodes often recur at the same site or within the same system (arterial vs venous system). These events can also trigger a rapid succession of critical thrombotic episodes at multiple sites.

Adult↗

Microbiology and management of deep facial infections and Lemierre syndrome.

This review describes the microbiology, diagnosis and management of deep facial infections and Lemierre syndrome. The origins of most of these infections are odontogenic infections that spread to fascial spaces of the lower head and upper neck. Other sources include pharyngotonsillar, nasal, otologic, salivary gland and dermatologic infections, hematogenic spread, cervical adenitis and trauma. These space infections can be divided into those around the face (masticatory, buccal, canine and parotid), the suprahyoid area (submandibular, sublingual and lateral pharyngeal) and the infrahyoid region or lateral neck (retropharyngeal and pretracheal spaces). The organisms accounting for these infections are aerobic and anaerobic that arise from the oropharyngeal flora. Complications of these infections can be life threatening and can result from hematogenic or direct spread. Complications that arise following local extension include suppurative jugular thrombophlebitis, cavernous sinus thrombosis, carotid erosion, maxillary sinusitis and osteomyelitis of the jaws. Management includes surgical drainage and antimicrobial therapy.

Face↗

Thrombophilia and stroke.

Thrombophilia is defined as an enhanced tendency to form intravascular thrombi, which may be arterial or venous. Of the inherited thrombophilias, factor V Leiden and the prothrombin 20210 mutation have been associated with stroke, but this association is statistically significant only in children and adults under age 40. The risk of stroke in persons with these mutations is substantially increased by concomitant exposure to oral contraceptives. Hyperhomocystinemia is a major risk factor for stroke as well as Alzheimer's disease; persons with deficiencies of vitamin B12 or folic acid are especially vulnerable to these complications. Of the acquired thrombophilias, the antiphospholipid antibody syndrome is strongly associated with transient ischemic attacks, cerebral infarction, Sneddon syndrome, and dementia. The diagnosis of thrombophilia should be considered in stroke patients who are young, have a family history of thrombosis, suffer venous dural sinus thrombosis, or have recurrent strokes.

Contraceptives, Oral↗

Pilot study of treatment with whole body hypothermia for neonatal encephalopathy.

BACKGROUND: There is extensive experimental evidence to support the investigation of treatment with mild hypothermia after birth asphyxia. However, clinical studies have been delayed by the difficulty in predicting long-term outcome very soon after birth and by concern about adverse effects of hypothermia. OBJECTIVES: The objectives of this study were to determine whether it is feasible to select infants with a bad neurological prognosis and to begin hypothermic therapy within 6 hours of birth, and to observe the effect of this therapy on relevant physiologic variables. METHODS: Sixteen newborn infants with clinical features of birth asphyxia (median cord blood pH: 6.74; range: 6.58-7.08) were assessed by amplitude integrated electroencephalography (aEEG), and mild whole body hypothermia was instituted within 6 hours of birth in the 10 infants with an aEEG prognostic of a bad outcome. Rectal temperature was maintained at 33.2 +/- (standard deviation).6 degrees C for 48 hours. Rectal and tympanic membrane temperature, blood pressure, heart rate, blood gases, blood lactate, full blood count, blood electrolytes, high and low shear rate viscosity, and coagulation studies were monitored during and after cooling. A preliminary assessment of neurological outcome was made by repeated magnetic resonance imaging (MRI) and neurological examination. RESULTS: All infants selected to receive hypothermia developed convulsions and a severe encephalopathy. During 48 hours of hypothermia infants had prolonged metabolic acidosis (median pH: 7.30; base excess: -6.3 mmol x L(-1), a high blood lactate (median lactate: 5.3 mmol x L(-1)) and low blood potassium levels (median value: 3.9 mmol x L(-1)) x Hypothermia was associated with lower heart rate and higher mean blood pressure. However, these changes did not seem to be clinically relevant and no significant complication of hypothermia was encountered. Blood viscosity and coagulation studies were similar during and after cooling. Unusual MRI findings were noted in 3 infants: transverse sinus thrombosis with subsequent small cerebellar infarct; probable thrombosis in the straight sinus; and hemorrhagic cerebral infarction. Six of the 10 cooled infants had minor abnormalities only or normal follow-up neurological examination; 3 infants died and 1 had major abnormalities. None of the 6 infants with a normal aEEG developed severe neonatal encephalopathy or neurological sequel. CONCLUSIONS: After birth asphyxia infants can be objectively selected by aEEG and hypothermia started within 6 hours of birth in infants at high risk of developing severe neonatal encephalopathy. Prolonged mild hypothermia to 33 degrees C to 34 degrees C is associated with minor physiologic abnormalities. Further studies of both the safety and efficacy of mild hypothermia, including further neuroimaging studies, are warranted.

Asphyxia Neonatorum↗

Safety and efficacy of endoscopic repair of CSF leaks and encephaloceles: a survey of the members of the American Rhinologic Society.

The purpose of this article is to review the endoscopic management of cerebrospinal fluid (CSF) leaks and encephaloceles, with particular emphasis on safety and efficacy, by retrospective assessment utilizing the results of a mailed questionnaire. Surveys were mailed to members of the American Rhinologic Society with practices in both academic centers and/or private settings. Survey results were then assessed and tabulated. There were 635 mailings, with 197 responses (31%). Seventy-two (36% of respondents) indicated that they performed endoscopic management of CSF leaks and encephaloceles, while 125 (64% of respondents) did not. Respondents reported approximately 522 cases of CSF leaks and approximately 128 cases of encephaloceles managed by endoscopy. Success rates after a single procedure were estimated at 90% for CSF leaks and 93% for encephaloceles. Success rates after a secondary procedure were estimated at 86% and 97%, respectively; 29% of respondents have, at some point, made a referral to neurosurgery. A total of 13 complications related to endoscopic repairs were reported (2.5%). For CSF leak repair, complications included seizures, 0.2%; meningitis, 1.1%; and one reported case each of cavernous sinus thrombosis, temporary visual problems, sinusitis, and intracranial hypertension/bleed. There was only one reported death in the approximately 522 cases. Eleven complications following encephalocele repairs (8.5%) included seizures, 3.1%; meningitis, 2.3%; and one reported case each of brain abscess, sinusitis, false aneurysm of middle cerebral artery, and mild dizziness. No deaths following encephalocele repair were reported. The endoscopic management of CSF leaks and encephaloceles has become increasingly popular and has proven to have low morbidity and mortality with high success. Overall, our results confirm that in the hands of the skilled endoscopist, endoscopic management of CSF leaks and encephaloceles is highly efficacious and has a very low incidence of significant complication.

Bandages↗

Frontal sinus ablation for frontal osteomyelitis.

Frontal sinusitis with frontal osteomyelitis is a potentially life threatening disease. Diagnostic and therapeutic errors occur frequently because of antibiotic masking of already silent frontal lobe complications or lack of suspicion on the part of the otolaryngologist or the neurosurgeon. Frontal sinus infection and/or trauma frequently require otoneuro cooperation for care. Four cases of complications of frontal sinus infection with osteomyelitis are discussed. Three had epidural empyemas and one had a subdural empyema with an anterior 1/3 superior sagittal sinus thrombosis and multiple brain abscesses. Each patient was approached through a frontal craniotomy and the frontal sinus posterior plate examined from behind. Each had posterior dehiscences. Follow-up of osteomyelitis requires multiple tests including computerized tomography, polytomography and possibly bone or gallium scans. Twenty year or more follow-up is essential.

Adolescent↗

[Intracranial MR angiography].

Magnetic resonance angiography (MRA) is gaining wide acceptance as a screening modality for the intracranial vasculature. Previous reports have demonstrated that MRA is capable of accurately depicting the normal vascular anatomy and anatomic variants. To date various techniques have been applied in the evaluation of vaso-occlusive disease, aneurysms, arteriovenous malformations and venous sinus thrombosis. Recent technical developments have significantly improved spatial resolution and vascular contrast and will further enhance its routine clinical use. The purpose of this essay is to summarize the technical approaches, clinical role and limitations of MRA as they apply to the intracranial circulation.

Blood Flow Velocity↗

Cerebrovascular accidents in children with cancer.

During a 4-year period, 26 children with systemic malignancies suffered cerebrovascular accidents. These occurred in 17 patients with lymphoreticular malignancy and nine patients with solid tumors. They were the presenting signs of malignancy in three patients and were the direct cause of death in six. Cerebrovascular accidents were directly related to disseminated intravascular coagulation in eight patients, to chemotherapy in eight patients, to metastatic tumor in three patients, to thrombocytopenia in three patients, and to fungal meningitis in one patient. All patients with disseminated intravascular coagulation had leukemia and at times, cerebrovascular thrombosis predated systemic or laboratory evidence of disseminated intravascular coagulation. This review indicates that four major syndromes are apparent in children with cancer: vascular thrombosis associated with disseminated intravascular coagulation, acute arterial or sagittal sinus thrombosis secondary to L-asparaginase in children with leukemia, acute neurologic dysfunction in patients with osteogenic sarcoma treated with high-dose methotrexate, and obtundation, seizures, and focal neurologic deficits in patients with neuroblastoma metastatic to the torcular region. Although elevated WBC counts and thrombocytopenia occur frequently in children with cancer, in themselves they uncommonly result in strokes. It is concluded that cerebrovascular accidents are a relatively frequent cause of acute neurologic compromise in children with cancer and that certain types of malignancies and their treatment predispose patients to this complication.

Acute Disease↗