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Dural arteriovenous fistula after craniotomy for pilocytic astrocytoma in a patient with protein S deficiency.

BACKGROUND: We report an unusual case, which may provide insight into the etiology and pathogenesis of dural arteriovenous malformation. CASE DESCRIPTION: A 24-year-old woman presented with hemorrhage into a pilocytic astrocytoma of the collicular plate. Angiography was normal and the tumor was surgically resected. She developed sigmoid sinus thrombosis and a transverse/sigmoid sinus dural arteriovenous fistula 11 months after this and was found to have protein S deficiency. The fistula was not treated. Angiography 4 years later was unchanged. CONCLUSION: This report illustrates an acquired etiology of a dural arteriovenous fistula. To our knowledge this is the first reported case of postoperative sigmoid sinus thrombosis along with arteriovenous fistula in a patient with previously undetected protein S deficiency.

Adult↗

Bacterial infections of the orbital and periorbital soft-tissues in children.

Periorbital soft-tissue bacterial infections are relatively common during childhood and with appropriate antimicrobial therapy usually resolve quickly. Orbital soft-tissue infections, in contrast, are rare and frequently cause serious morbidity. Two hundred forty-one children with periorbital or orbital soft-tissue infections have been hospitalized since 1962 and constitute the data base for this study. Two hundred twenty-six children, half under 1 year of age, had periorbital soft-tissue infections. Response to antimicrobials was prompt and only two children (1%) developed complications. True orbital infections occurred in the remaining 15 children, three of whom were under 6 years of age. Eleven children had orbital cellulitis, 3 had subperiosteal abscesses, and 1 had cavernous sinus thrombosis. Antecedent sinusitis was present in 12 cases and, in seven, effective treatment required surgical drainage in addition to intensive antimicrobial therapy. Three children (20%) developed significant complications.

Abscess↗

Primary pituitary abscess: case report.

Pituitary abscesses are potentially life-threatening lesions if not appropriately diagnosed and treated. The authors have operated on more than five hundred cases of pituitary tumors and only one represented a case of pituitary abscess. A 35-year-old woman was investigated for chronic frontal headache. CT scan showed a cystic sellar lesion with ring enhancement after contrast injection leading to an initial diagnosis of pituitary adenoma. She underwent a sublabial transsphenoidal approach to the pituitary gland. After dural opening, purulent material was obtained and no tumor or other associated lesion was detected. There was no evidence of current or previous septicemic illness, meningitis, cavernous sinus thrombosis or sinus infection. Cultures were negative. She was put on antibiotics and discharged after 4 weeks. Nowadays, 10 years after treatment, she is doing well, with no anterior pituitary hormone deficit. MRI shows a partially empty sella without residual lesion and the pituitary stalck is in the midline. The early diagnosis and adequate treatment of this life-threatening lesion may result in excellent prognosis.

Abscess↗

[Intracranial complications of acute mastoiditis].

We report a case of extradural abscess after acute mastoiditis in an 8-years-old boy. The clinic diagnostic of an intracranial complication is difficult. The most common present symptoms are fever, otalgia and otorrhea but are not specific. Neurologic symptoms are suggestive signs of an intracranial complication. We believe that, in acute mastoiditis performance of a CT scan of the brain and temporal bones with intravenous contrast, contribues to the diagnostic of intracranial complication. The diagnostic of thrombosis sinus sigmoid is evoked with slight contrast enhanced sinus sigmoid and failure to opacify. The characterisation of an epidural empyema is a hypodense epidural collection in a contrast-enhanced CT scan. The most common isolated organism are Streptococcus Pneumoniae, Staphylococcus aureus and Pseudomonas aeuginosa. The therapeutic management includes combination of intraveinous antibiotics, mastoidectomy and surgery of the intracranial complication.

Acute Disease↗

Cerebral sinus venous thrombosis in two patients with spontaneous intracranial hypotension.

We report 2 patients who had clinical and neuroimaging signs of spontaneous intracranial hypotension and who developed cerebral sinus venous thrombosis. This sequence of events -- known after dural puncture but not in spontaneous intracranial hypotension -- was suggested by the change in the pattern of headache, from a postural to a permanent and increasing one. The diagnostic and therapeutic difficulties that this complication raises are discussed.

Adult↗

Prolonged diplopia following sinus vein thrombosis mimicking Gradenigo's syndrome.

We report about a boy with the symptoms of Gradenigo's syndrome (abducens nerve palsy, acute otitis media, unilateral headache). The MR imaging showed a sinus vein thrombosis instead of the expected petroapicitis. After 2 weeks of conservative therapy with antibiotics and anticoagulation, without any clinical effort the boy underwent a mastoidectomy. Headache and otitis media recovered quickly but the diplopia still remained after 25 months.

Abducens Nerve↗

Sinus vein thrombosis following exposure to simulated high altitude.

A relation exists between high altitude exposure and a hypercoagulable state, the nature of which is not entirely clear. This has been mostly reported in mountain climbers. We report a 19-yr-old female, working as a high-altitude chamber instructor, who presented with severe frontal headaches which persisted for a month following routine high altitude chamber training. The patient was in generally good health and was using oral contraceptives for 3 yr prior to the event. Due to the unremitting nature of the symptoms, the patient was admitted to a neurology department, and computerized tomography (CT) and magnetic resonance imaging (MRI) were performed. Sagittal and transverse sinus vein thrombosis were diagnosed and anticoagulant therapy was initiated (low molecular weight heparin followed by warfarin). Following treatment, a slow symptomatic improvement was observed, and the patient was discharged. On discharge, it was recommended she continue oral anticoagulant therapy (warfarin). A complete coagulation screening panel was performed, which was negative. Although the relation between high altitude exposure and a hypercoagulable state is well known, this is the first time a case of sinus vein thrombosis has been reported after high altitude chamber training. Careful history and closely monitored medical follow-up should be performed on all designated staff exposed to simulated altitude. Even though there is no conclusive evidence regarding it, we suggest, as a matter of caution, that women using oral contraceptives should consider their risks before deciding to undertake exposure to simulated altitude in chambers. Literature review and detailed recommendations for prevention are provided.

Adult↗

Acute promyelocytic leukemia presenting as sudden blindness and sinus vein thrombosis.

An 11-year-old boy was hospitalized with sudden blindness and sagittal sinus vein thrombosis, which were the presenting signs of acute promyelocytic leukemia (APL). The association of disseminated intravascular clotting (DIC) with APL is well-known, and DIC usually affects the smaller blood vessels. The obliteration of a large blood vessel, as in our case, is an uncommon manifestation of DIC. This presentation of acute childhood leukemia is unusual and, to the best of our knowledge, has not been previously reported.

Blindness↗

[Behçet disease with primary involvement of the cerebral vessels].

HISTORY AND CLINICAL FINDINGS: Two months after the onset of mainly frontal headaches a 25-year-old man of Turkish descent additionally developed double vision. Fundoscopy revealed bilateral choked discs and right trochlear paralysis. Computed tomography and digital subtraction angiography demonstrated thrombosis of the superior sagittal sinus. On admission to hospital the patient was fully conscious but had marked meningism, bilaterally positive Lasègue's sign (painful straight leg raising) at a 50 degree angle, and multiple oral aphthous ulcers. The sinus thrombosis suggested a chronic inflammatory process, while the oral ulcers pointed to Behçet's syndrome. INVESTIGATIONS: Inflammatory parameters (erythrocyte sedimentation rate, C-reactive protein and white blood cell count) were increased and the HLA-B27 test was positive. Other laboratory tests, cerebrospinal fluid, chest radiogram and ECG were unremarkable. TREATMENT AND COURSE: Despite intravenous administration of heparin nad cefuroxim for one week the sagittal sinus thrombosis spread to the straight sinus. In the third week scrotal ulcerations were noted and taken to confirm Behçet's syndrome. Immunosuppressive treatment with methylprednisolone (initially 80 mg daily) and 2 weeks later together with chlorambucil (0.1 mg/kg daily) was started. Four weeks later the patient was free of symptoms and discharged. CONCLUSION: Neurological signs are not adequately stressed by the International Study Group for Behçet's Disease among its listed diagnostic criteria.

Adult↗

[Report on the ECR2003 (European Congress of Radiology): non-contrast intracranial MR venography with 3D-phase contrast imaging: optimization of presaturation pulse and velocity encoding].

BACKGROUND: Intracranial MR venography is useful for the diagnosis of dural sinus thrombosis and the preoperative assessment of sinus patency encased by tumors. Recently, contrast-enhanced MR venography has been applied for suspected dural sinus occlusion in a shorter time. However, it has some disadvantage for the evaluation of hypervascularized enhancing thrombus mimicking flow in chronic sinus thrombosis. So far, we have evaluated optimal imaging technique and slice orientation and have shown that sagittal three-dimensional (3D) -phase contrast (PC) imaging is the most suitable for the non-contrast intracranial MR venography. PURPOSE: To assess the optimal presaturation pulse (SAT) and velocity encoding (VENC) for the non-contrast intracranial 3D-PC MR venography. METHODS AND MATERIALS: Firstly, we performed phantom experiment to assess the best SAT thickness using arterial presaturation. Second, MR imaging was performed in 7 healthy volunteers to measure the dural sinus flow velocity using a 1.5 T MR. Third, 3D-PC MR venography was performed with a VENC settings at 10, 15, 20 and 30 cm/sec for healthy volunteers. All data were displayed as maximum intensity projection images and three neuroradiologists assessed the visibility of the dural sinuses and the cortical vein. RESULTS: The mean flow velocity of the dural sinuses was 6.3 cm/sec. The thickness of the best SAT was 100 mm. In the assessment of the visibility of the 3D-PC images, dural sinuses were adequately visualized at a VENC of 15 cm/sec. CONCLUSIONS: Non-contrast intracranial 3D-PC MR venography was optimized at 100mm thickness of SAT and a VENC of 15 cm/sec.

Blood Flow Velocity↗

Differential diagnosis of patients with intracranial sinus venous thrombosis related isolated intracranial hypertension from those with idiopathic intracranial hypertension.

In patients presenting with intracranial hypertension without hydrocephalus, mass lesions, and with normal cerebrospinal fluid (CSF) composition (pseudotumor cerebri syndrome), the diagnosis of intracranial sinus venous thrombosis (ISVT) has crucial etiological, therapeutic and prognostic implications. Utilizing two well-defined groups of pseudotumor cerebri patients, one with magnetic resonance imaging (MRI) or angiography confirmed ISVT (17 patients) and the other in whom ISVT has been excluded (idiopathic intracranial hypertension [IIH], 27 patients), we investigated the characteristics that might be helpful in distinguishing them. No clinical or auxiliary findings differed between the ISVT and IIH groups except for female gender and lower CSF protein level, which were significantly associated with the latter. While the syndrome pseudotumor cerebri could be due to multiple causes including ISVT, the term IIH should be restricted for patients with isolated intracranial hypertension attributable to no other neurological or systemic disease. Since CT frequently misses ISVT, patients with pseudotumor cerebri syndrome should undergo MRI and MR venography before being labeled as IIH. We conclude that Modified Dandy's Diagnostic Criteria of pseudotumor cerebri, formulated prior to MRI era, can no longer be applied for the diagnosis of IIH.

Adolescent↗

Local fibrinolysis in cerebral venous thrombosis.

Extensive cerebral venous sinus thrombosis may cause death or severe neurologic sequelae. A minimally responsive 10-year-old boy with thrombosis of the superior sagittal sinus, left transverse and left sigmoid sinus, torcular, vein of Galen, and straight sinus underwent fibrinolytic therapy with urokinase during transfemoral venous angiography. He improved dramatically during the procedure as antegrade venous flow was re-established. Local thrombolytic therapy may be beneficial for other patients with rapid neurologic deterioration caused by extensive thrombosis of superficial and deep venous structures.

Cerebral Angiography↗

Intracranial hemorrhage in a term newborn with severe von Willebrand disease type 3 associated with sinus venous thrombosis.

Intracranial hemorrhage of term neonates with severe von Willebrand disease type 3 and near normal delivery have not been published to date. We report on a term newborn with severe type 3 VWD who suffered a large intracranial bleeding presumably subsequent to sinus venous thrombosis. This case is remarkable in two aspects: i) with respect to the low risk of perinatal intracranial hemorrhage even in neonates with VWD type 3 and ii) since VWD type 3 with factor VIII:C levels < 5 IU/dl does not seem to protect from thromboembolism. Guidelines for the management of hemophilia advise atraumatic delivery of neonates with known or suspected bleeding disorders, however primary caesarean section in the absence of complications is not obligatory. To answer the question whether the presented case could have an impact on the recommended management we carried out a worldwide survey including 24 participating specialized centers. Except a single case from Sweden of a female neonate with VWD type 3 who also had an inhibitor against VWF no further cases were reported to us. We conclude that CNS bleeding in term neonates with severe VWD type 3 is a rare event that requires additional risk factors for manifestation. Therefore, the amendment of current guidelines does not seem to be mandatory.

Adult↗

Intracranial complications of acute mastoiditis.

OBJECTIVE: Oral antibiotic use may have changed the incidence and microbiology of otitic intracranial complications. We reviewed cases of acute mastoiditis to document: (1) incidence of intracranial complications; (2) risk factors; and (3) identify pathologic organisms. METHODS: A retrospective study of children at a tertiary care children's hospital with acute mastoiditis from July, 1986 through June, 1998. RESULTS: 118 children with acute mastoiditis were identified. Eight patients (6.8%), ages 20 months to 14 years, had intracranial complications related to acute mastoiditis. Three children had a sigmoid sinus thrombosis, two children had an epidural abscess, and two children had both complications of sigmoid sinus thrombosis and epidural abscess, and a sigmoid sinus thrombosis and meningitis was present in one child. Pre-admission oral antibiotics were administered for an average of 10 days in seven of the eight patients. Persistent otorrhea and/or otalgia were present in all patients. Intraoperative cultures were negative in four cases (50%). Organisms isolated included: Streptococcus pneumoniae (2); Proteus mirabilis (1); Pseudomonas aeruginosa (1); and coagulase negative Staphylococcus (1). Multi-drug resistant organisms were documented in only one case. All patients underwent a contrast enhanced CT of the temporal bones and brain. Surgical management included complete mastoidectomy in all patients and a pressure equalization tube in seven of the eight cases. CONCLUSIONS: Our review did not document an increase in the incidence of otitic intracranial complications. Persistent otalgia or otorrhea while on oral antibiotics with associated neurologic symptoms are ominous signs suggestive of a complication. Multi-drug resistant organisms are uncommon whereas negative intraoperative cultures are common.

Acute Disease↗

Cerebral venous thrombosis associated with inhalational drug abuse.

Cerebral venous thrombosis is a life-threatening disorder for which the management has been controversial. A 23-year-old critically-ill man with superior sagittal sinus thrombosis (SSST) and cavernous sinus thrombosis secondary to sinusitis following inhalational drug abuse was managed with full heparinization from the fourth day of presentation. He went on to make a full recovery. The association between this form of drug abuse, sinusitis and SSST is an important one, and warrants antibiotics and early aggressive anticoagulation therapy.

Adult↗