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Intracranial venous thrombosis and pulmonary embolism with antiphospholipid syndrome in systemic lupus erythematosus.

The presence of antiphospholipid antibodies is associated with arterial and venous thrombosis. A 14-year-old girl, with systemic lupus erythematosus (SLE), developed headache and cough and was found to have intracranial venous sinus thrombosis with secondary pulmonary embolism associated with antiphospholipid syndrome. Clinical and radiological improvement occurred with anticoagulation therapy. Because SLE is commonly associated with antiphospholipid antibodies, thromboembolic events should be considered in the differential diagnosis of both cough and headache in children with SLE.

Adolescent↗

Cerebral MR venography in children: comparison of 2D time-of-flight and gadolinium-enhanced 3D gradient-echo techniques.

PURPOSE: To prospectively compare two-dimensional (2D) time-of-flight cerebral magnetic resonance (MR) venography with gadolinium-enhanced three-dimensional (3D) gradient-echo cerebral MR venography in children. MATERIALS AND METHODS: This investigation had investigational review board approval and was Health Insurance Portability and Accountability Act compliant; parental informed consent was obtained. Thirty-seven patients (20 boys, 17 girls) who ranged in age from 4 days to 15 years underwent 2D and 3D MR venography. Two pediatric neuroradiologists compared the visibility of the superior sagittal, straight, transverse, and sigmoid sinuses and the internal jugular veins on images obtained with the two sequences. RESULTS: In 17 (46%) of the 37 patients, the sequences were equivalent in terms of their depiction of venous anatomy. In 19 (51%) of the 37 patients, 3D MR venography was superior to 2D MR venography. Suboptimal enhancement of veins occurred in one (3%) patient at 3D MR venography. Venous anomalies suggested at 2D MR venography but not present at 3D MR venography included flow gaps in the nondominant transverse sinuses of four patients, unilateral transverse sinus atresia in eight, and a narrowed superior sagittal sinus in two. Two-dimensional MR venography results failed to reveal a persistent falcine sinus associated with straight sinus atresia in one patient and suggested transverse sinus thrombosis in two patients in whom 3D MR venography results were normal. Additionally, the extent of dural thrombosis was overestimated at 2D MR venography in one patient. As compared with 3D MR venography, 2D MR venography failed to reveal sigmoid sinus stenosis in one patient and poorly depicted posterior fossa dural sinus anatomy in two patients with dural arteriovenous fistula. CONCLUSION: Three-dimensional MR venography is often superior to 2D MR venography in the delineation of major cerebral venous structures in children. Most of the artifactual loss of vascular signal seen with the use of 2D MR venography occurred in nondominant transverse sinuses.

Adolescent↗

Reflections upon the nature and management of intracranial and intraspinal vascular malformations and fistulae.

Evidence is presented that dural fistulae are preceded by sinus thrombosis and that their danger lies in arterialized venous pressure within the cranium or the orbit. Arterial side occlusion leads to recurrence, while venous side occlusion leads to permanent cure. Vein of Galen aneurysms embrace some features of cerebral arteriovenous malformations (AVM's), namely a reticulum, and some features of dural fistulae, namely evidence of previous sinus anomaly and direct drainage into a sinus. These aneurysms are also permanently cured by venous side thrombosis, although the dangers inherent in their reticulum demand that this be done in stages or preceded by arterial side embolization. A very limited experience with venous end occlusion of cerebral (and spinal) AVM's suggests that they, too, can be permanently cured by venous side occlusion without excision. Their reticulum demands maximum, multistage, preliminary arterial side embolization together with intraoperative hypotension during the venous occlusion stage in order to minimize intracerebral hemorrhage or swelling. Schematic models of both fistulae and malformations are presented, together with reasons why particulate embolization is safer than glue embolization. The theory is advanced that dural fistulae, vein of Galen aneurysms, and AVM's are venous- rather than arterial-based lesions, which is consistent with the experience that permanent cure has been effected by venous side occlusion without excision in all three anomalies. It is speculated that there may be a developmental link between AVM and the venous malformation, the AVM being essentially a fistulized venous malformation.

Adolescent↗

MRI of intracranial sinovenous thrombosis: the role of phase imaging.

Conventional spin-echo magnetic resonance (MR) imaging of venous thrombosis is complicated by the variable appearance produced by the stage of blood clot degradation and velocity of blood flow. Phase MR imaging is a simple method based primarily on whether protons are stationary or moving. A case of superior sagittal sinus thrombosis demonstrates the utility of phase imaging.

Adult↗

Acquired pial arteriovenous fistula following cerebral vein thrombosis.

BACKGROUND: We report a unique case of an acquired pial arteriovenous fistula occurring after an asymptomatic thrombosis of a superficial cerebral vein. CASE DESCRIPTION: A cerebral angiogram performed in a 51-year-old man with subarachnoid hemorrhage revealed a 10-mm ruptured anterior communicating artery aneurysm and a thrombosed left superficial middle cerebral vein. Coil embolization of the anterior communicating aneurysm was performed. Follow-up angiography 18 months later revealed a new, asymptomatic, pial arteriovenous fistula between the previously thrombosed left superficial middle cerebral vein and a small sylvian branch of the left middle cerebral artery. CONCLUSIONS: This case provides evidence that pial arteriovenous fistulas may develop as acquired lesions and furthermore may rarely follow cerebral vein thrombosis. Several cases of dural arteriovenous fistulas, as well as a single case of a mixed pial-dural arteriovenous fistula, occurring after dural sinus thrombosis have been reported previously. However, to our knowledge, this is the first report of an acquired pial arteriovenous fistula following a cerebral vein thrombosis.

Aneurysm, Ruptured↗

The intracranial complications of rhinosinusitis: can they be prevented?

OBJECTIVES/HYPOTHESIS: Reference textbooks on the intracranial complications of rhinosinusitis imply that many of the intracranial complications of rhinosinusitis can be prevented. We sought to examine whether or not this is true. STUDY DESIGN: A retrospective case series. METHODS: The study included 47 consecutive patients presenting with intracranial complications secondary to rhinosinusitis between 1992 to 1999 with a mean follow-up of 5 years and 1 month. RESULTS: The most common presenting symptoms of intracranial involvement were an altered mental state, headache, fever, seizure, vomiting, a unilateral weakness or hemiparesis, or a cranial nerve sign. These justify an urgent magnetic resonance imaging or computed tomography scan. The importance of imaging before a lumbar puncture cannot be overemphasized. Of particular note was the finding that 21 patients (45%) presented with a periorbital cellulitis or frontal swelling. Therefore, it does not follow that because a collection of pus presents anteriorly it precludes any intracranial involvement. More than half of our patients (55%) had visited their primary care physician with an upper respiratory tract infection and had been treated appropriately. Once any central symptoms or signs developed, there was little evidence of any significant delay in referral to our unit. Only six patients had a history of nasal disease, three having had recent sinus surgery and three having had nasal polyps. Nine patients had significant long-term morbidity, seven patients had epilepsy, one patient had dysphasia, and one patient had right arm weakness. The single death in our series was associated with a cavernous sinus thrombosis. CONCLUSIONS: The report emphasizes the need for surgeons to be alert to the diagnosis, particularly in patients with a periorbital abscess or frontal swelling. Sinus surgery has a role in obtaining pus for culture, as well as draining the sinus if it is in continuity with an intracranial collection. Intracranial infections secondary to rhinosinusitis occur sporadically and, although it appears that this cannot be prevented, early recognition and treatment are essential to reduce any subsequent morbidity or mortality.

Adolescent↗

Cerebral venous thrombosis in ulcerative colitis.

Cerebral venous thrombosis is a rare complication of ulcerative colitis. We report a case of 29 year old male who developed superior sagittal, left lateral and sigmoid sinus thrombosis secondary to ulcerative colitis. He was successfully treated with low molecular weight heparin and steroids.

Adrenal Cortex Hormones↗

Relation between intraocular pressure and size of transverse sinuses.

There are asymmetries in the sizes of transverse sinus and intraocular pressure. The purpose of this study was to investigate possible relationships between the asymmetry of transverse sinuses in TOF MR venography and intraocular pressures of right and left eyes. In this study, subjects were 63 male and 42 female medical school students, aged 18-21 years (mean+/-SD; 19.72+/-0.67 years). Subjects with neurological and ophthalmologic disease, particularly dural sinus thrombosis, myopia, trauma and glaucoma, were excluded the study. Subjects were divided into five groups according to the magnitudes of the right- and left-transverse sinuses in MR venography results. There is a functional relation between intraocular pressures of the right and left eyes and asymmetry of the transverse sinus. If the transverse sinus on one side is larger and its venous drainage is greater, the intraocular pressure of the eye on this side is lower. It can be speculated that the transverse sinus size may be associated with pathogenesis of diseases with increased intraocular pressure such as glaucoma. We aim to determine the relation between the size and drainage of transverse sinuses in TOF MR venography and intraocular pressure in patients with open-angle glaucoma in our next study.

Adolescent↗

Dural arteriovenous fistula associated with meningioma: spontaneous disappearance after tumor removal.

A 45-year-old woman presented with a non-dominant transverse-sigmoid sinus dural arteriovenous fistula (AVF) associated with convexity meningioma on the same side. The dural AVF disappeared spontaneously after surgical removal of the meningioma, even though there was no manipulation of the dural AVF. Dural AVFs are usually acquired lesions, and may develop after trauma, surgery, and dural sinus thrombosis. Dural AVFs of the acquired origins are rarely associated with brain tumor. Dural AVFs associated with a tumor may develop even in the absence of sinus occlusion.

Central Nervous System Vascular Malformations↗

Intracranial complications of rhinoplasty.

The practicing plastic surgeon acquires a wide experience of the complications of rhinoplasty. These range in severity from minor imperfections of contour to the more dramatic but re complications of haemorrhage and infection. While causing alarm to patient and surgeon alike, they rarely pose a threat to life. We wish to bring to the attention to our colleagues three potentially lethal intracranial complications of rhinoplasty which they may not have previously experienced: cerebrospinal fluid rhinorrhea, meningitis and cavernous sinus thrombosis. These three unusual cases have occurred in the author's practice over a period of 20 years.

Adult↗

Case report: cerebral venous thrombosis as a manifestation of acute ulcerative colitis.

Ulcerative colitis is associated with a number of extraintestinal complications, including the infrequent occurrence of thromboembolic disease. Cerebral venous thrombosis is extremely rare, with only 15 cases reported in the literature. The outcome in this group of patients is poor; the result in more than 80% of the cases is permanent neurologic sequelae or death. The precise mechanisms involved in thrombogenesis remain unclear. A hypercoagulable state may occur in ulcerative colitis because of well-documented associations of thrombocytosis, elevated factors V and VIII and fibrinogen, and decreased antithrombin III. Treatment regimens for cerebral venous thrombosis remain controversial and include anticoagulation with heparin, surgical thrombectomy, and systemic and local infusion of fibrinolytic regimens. A conservative approach with antiedemic agents, anticonvulsants, antiplatelet therapy, and acetazolamide also may be beneficial and offers a substantially reduced hemorrhagic potential. A case of primary superior sagittal sinus thrombosis associated with active ulcerative colitis treated by the latter method is reported. The patient's neurologic recovery was complete, with recanalization of the thrombosed venous sinus being demonstrated by magnetic resonance imaging studies.

Adult↗

Acute sinusitis.

Acute sinusitis is one of the most commonly observed entities in clinical practice. Despite the frequency of the disease, diagnosis and therapy often remain empiric. Most cases are secondary to sinus ostia obstruction associated with the common cold or allergies. Maxillary sinusitis is most common. Because of the proximity of vital anatomic structures and venous drainage systems, serious complications frequently arise from sphenoid, frontal, and ethmoid sinusitis. Clinical signs and symptoms most helpful in the diagnosis of maxillary sinusitis are the presence of a maxillary toothache, lack of improvement with decongestants, a purulent nasal discharge, cough, purulent secretions observed on nasal examination, abnormal transillumination, and sinus tenderness. Plain film radiographs are helpful, but do not adequately visualize the anterior ethmoid sinuses. Computed tomography provides superior visualization, but cost remains prohibitive for routine cases. Most maxillary sinusitis in adults is secondary to Streptococcus pneumoniae or Hemophilus influenzae. Moroxella catarrhalis is common in children. Staphylococcus aureus is observed more frequently in frontal or sphenoid disease. Most patients with acute sinusitis are treated without microbiological diagnosis and respond well to commonly used oral antimicrobials with activity against the usual pathogens. Complications of sinusitis include meningitis, periorbital infections, subdural empyema, epidural abscess, brains abscess, cavernous sinus thrombosis, and osteomyelitis.

Acute Disease↗

[Cerebral venous thrombosis in the Ile de la Réunion. A series of 20 patients].

Twenty cases of cerebral venous thrombosis (CVT) are reported. A significant majority of patients were young women receiving oral contraceptives. In the puerperium CVT were frequent and delayed; the number of septic lateral sinus thrombosis was the same as in the occidental countries. The clinical picture was not specific, and idiopathic intracranial hypertension or pseudotumor cerebri were not frequent. Focal signs were the most frequent clinical manifestations. The clinical course, of deep CVT was not as severe as previously reported. Due to the lack of MRI, only CT scan was performed for all patients. More than 50% showed direct signs of CVT (cord sign and delta sign). The diagnosis of CVT was confirmed by cerebral angiography. Early systematic treatment by heparin was used with neither appearance nor aggravation of hemorrhagic infarcts being observed. The outcome was favorable in 60% of the cases, except for venous hemorrhagic infarction.

Adolescent↗

Suppurative (bacterial) sinusitis.

Upper respiratory tract (viral) infection is the most common predisposing cause of suppurative sinusitis. Acute disease is manifested by pain, nasal discharge, systemic manifestations, and nasal obstruction. In chronic disease (greater than 3 months' duration) nasal airway obstruction and postnasal discharge may be the only symptoms. Diagnosis is made on the basis of the history and physical examination, supported by roentgenographic findings and, if necessary, cultures. Acute infection is treated with antibiotics, decongestants, and analgesics. If medical management fails, surgery is necessary. Complications of suppurative sinusitis include cellulitis, abscess, meningitis and cavernous sinus thrombosis, osteomyelitis, and oroantral fistula.

Acute Disease↗

Isolated thrombosis due to the cystathionine beta-synthase mutation c.833T>C (1278T).

Hereditary homocystinuria due to cystathionine beta-synthase (CBS) deficiency is a rare disease (about 1:20000 in Germany) often complicated by thromboembolism. Single mutations, which affect the C-terminal region of the CBS enzyme, lead to isolated thrombosis without further symptoms typical for homocystinuria such as atherosclerosis, psychomotor retardation, and dislocation of the ocular lenses. In this study, DNA samples of patients with stroke (n = 225) and sinus thrombosis (n = 46) were screened for the most common homocystinuria mutation, CBS 1278T. In each group one homozygous patient was identified. Thus, not only C-terminal mutations but also the most common mutation in classical homocystinuria, CBS 1278T, can lead to isolated thrombophilic events. These data support the hypothesis that homocystinuria is an underdiagnosed disease.

Cystathionine beta-Synthase↗

[Cerebral phlebitis and Crohn disease].

A 17 year-old man with a one-year history of Crohn's disease presented with superior sagittal sinus thrombosis in spite of an apparent remission of the inflammatory bowel disease. Under treatment, the neurological disorders rapidly improved. Hematological studies showed mild thrombocytosis, spontaneous platelet aggregation, and an increase of the fibrinogen level, plasmatic viscosity and red blood cells aggregability. Mild hypofibrinolysis and antiphospholipid antibodies were also noted. Nine fully documented cases of cerebral venous thrombosis associated with an inflammatory bowel disease are reviewed. The possible prothrombotic role of thrombocytosis and other hematological disorders is discussed.

Adolescent↗