Fulminant intracranial hypertension as a result of otological surgery: case report and discussion of management.

BACKGROUND: Dural venous sinus injury is a rare complication of otological surgery that can lead to life-threatening sequelae, the management of which is complex and poorly described. CASE REPORT: This paper describes the case of a 40-year-old female who underwent routine right myringoplasty complicated by sigmoid sinus laceration. The patient subsequently developed right-sided lateral sinus thrombosis leading to fulminant intracranial hypertension. The patient underwent successful emergency management by surgical reconstruction of the sigmoid sinus, followed by endovascular thrombolysis, catheter balloon angioplasty and endovascular stenting. CONCLUSION: Torrential haemorrhage following otological procedures is uncommon and rarely requires packing of a bleeding venous sinus. This case highlights that injury to a highly dominant venous sinus can lead to venous outflow obstruction and life-threatening intracranial hypertension. To our knowledge, the development of this complication following otological surgery and its management has not been reported previously.

Intellectual abilities in tuberous sclerosis complex: risk factors and correlates from the Tuberous Sclerosis 2000 Study.

BACKGROUND: Tuberous sclerosis complex (TSC) is associated with intellectual disability, but the risk pathways are poorly understood. METHOD: The Tuberous Sclerosis 2000 Study is a prospective longitudinal study of the natural history of TSC. One hundred and twenty-five UK children age 0-16 years with TSC and born between January 2001 and December 2006 were studied. Intelligence was assessed using standardized measures at ≥2 years of age. The age of onset of epilepsy, the type of seizure disorder, the frequency and duration of seizures, as well as the response to treatment was assessed at interview and by review of medical records. The severity of epilepsy in the early years was estimated using the E-Chess score. Genetic studies identified the mutations and the number of cortical tubers was determined from brain scans. RESULTS: TSC2 mutations were associated with significantly higher cortical tuber count than TSC1 mutations. The extent of brain involvement, as indexed by cortical tuber count, was associated with an earlier age of onset and severity of epilepsy. In turn, the severity of epilepsy was strongly associated with the degree of intellectual impairment. Structural equation modelling supported a causal pathway from genetic abnormality to cortical tuber count to epilepsy severity to intellectual outcome. Infantile spasms and status epilepticus were important contributors to seizure severity. CONCLUSIONS: The findings support the proposition that severe, early onset epilepsy may impair intellectual development in TSC and highlight the potential importance of early, prompt and effective treatment or prevention of epilepsy in tuberous sclerosis.

Intractable headache after excision of an acoustic neuroma treated by stent revascularisation of the sigmoid sinus.

A 47 year old man developed severe headaches after resection of an acoustic neuroma ipsilateral to non dominant venous drainage. CSF pressures were normal but imaging studies showed acquired, severe narrowing of the sigmoid sinus where it traversed the surgical defect. Stenting the sinus gave a lasting clinical improvement.

Coupling of sagittal sinus pressure and cerebrospinal fluid pressure in idiopathic intracranial hypertension--a preliminary report.

BACKGROUND: Narrowing of the cranial dural venous sinuses has been implicated as contributing to elevated intracranial pressure in idiopathic intracranial hypertension [IIH]. Such narrowing may be either a fixed stenosis or secondary to raised ICP. We have investigated whether narrowing of the venous sinuses may reflect direct coupling between cerebrospinal fluid pressure and sagittal sinus pressure. METHODS: Nine patients with the clinical features of IIH [8F, 1M; mean age 41 (range 22-55)] were studied as part of their standard clinical investigations by simultaneous lumbar CSF infusion study and direct retrograde cerebral venography whereby a catheter is placed within the sagittal sinus under fluoroscopic guidance. FINDINGS: In all cases, both CSF pressure (Pcsf) and sagittal sinus pressure (Pss) were elevated with Pcsf slightly exceeding Pss (27.0 +/- 2.3 mm Hg. 25.2 +/- 7.5 mm Hg; difference P = 0.026; correlation R = 0.97, P = 0.0032). There was a gradient of pressure along the sagittal and transverse sinuses. CSF infusion provoked rises in both Pcsf and Pss (R = 0.97, P < 0.0007). During drainage of CSF after the test (8 cases), Pcsf decreased to values lower than Pss (-3.26 +/- 3.9 mm Hg; P = 0.0097). There was excellent correlation between slow waves of Pcsf and Pss (mean R = 0.9) and between baseline pulse amplitudes of both pressures (R = 0.91; P = 0.03). CONCLUSIONS: In the 9 patients studied with IIH, Pcsf and Pss were coupled both statically (mean values) and dynamically (vasogenic components). During drainage, both pressures decreased until probably central venous pressure was reached and then Pcsf decreased further while Pss remained constant. This suggests that, in many cases of IIH, there is functional obstruction of venous outflow through the dural sinuses. Raised Pcsf partly obstructs venous sinus outflow, thereby increasing Pss which, in turn, leads to a further rise in Pcsf, et sequor. This vicious cycle can be interrupted by draining CSF.

Diffusion tensor imaging of benign intracranial hypertension: absence of cerebral oedema.

Cerebral oedema, it has been suggested, may have a role in the pathophysiology of benign intracranial hypertension (BIH). We applied diffusion tensor MR imaging (DTI), a technique able to detect cerebral oedema, to the study of patients with BIH. A quantitative regional analysis of diffusion parameters (trace and relative anisotropy) was conducted by comparing five BIH patients and six healthy controls. A small but significant increase in anisotropy accompanied by a small but significant decrease in trace was found in the putamen and head of the caudate nucleus. No significant changes were demonstrated in the thalamus, cerebral white matter or cortical regions. Our findings support other recent work that suggests cerebral oedema is not a factor in the pathogenesis of BIH.

Treatment of a direct carotid-cavernous fistula in a patient with type IV Ehlers-Danlos syndrome: a novel approach.

We report a case of a 34-year-old female with type IV Ehlers-Danlos syndrome diagnosed with a carotid cavernous fistula presenting with progressive proptosis. Endovascular embolization using balloons or coils carries a high risk of complications in this group of patients, owing to the extreme fragility of the blood vessels. Initial treatment was conservative until an intracerebral haemorrhage occurred. To avoid transfemoral angiography, the ipsilateral carotid arteries and the internal jugular vein were surgically exposed for insertion of two endovascular sheaths. The patient was transferred from theatre to the angiography suite and the sheaths were used for embolization access. The fistula was closed, with preservation of the carotid artery, using Guglielmi detachable coils deployed in the cavernous sinus from the arterial and venous sides. Rapid resolution of symptoms and signs followed, which was sustained at 6-month follow-up. This technique offers alternative access for endovascular treatment, which may reduce the high incidence of mortality associated with catheter angiography in this condition.

Cranial venous outflow obstruction and pseudotumor Cerebri syndrome.

The pathophysiology of PTS including idiopathic intracranial hypertension or 'BIH', remains controversial. The older literature frequently referred to pathology in the cerebral venous drainage but more modern imaging techniques (CT and early MR) failed to reveal gross venous pathology. The role of impaired cranial venous outflow has recently been re-examined in the light of new methods of investigation (advanced MR venography and direct microcatheter venography with manometry) and of treatment (venous sinus stenting). Venous sinus obstruction in PTS is a more common factor in the pathogenesis of the condition than previously recognised. Venous obstruction may be primary, that is, it is the underlying aetiological factor in PTS. Venous sinus obstruction may also be secondary to raised CSF pressure which may exacerbate problems with intracranial compliance and raised CSF pressure. Early experience with venous stenting suggests that it may be a helpful treatment for patients with PTS but more experience and longer follow-up is required to define the subgroups of patients for whom it is most appropriate.

Transverse sinus stenoses in benign intracranial hypertension demonstrated on CT venography.

Catheter studies show that patients with benign intracranial hypertension (BIH) frequently have high pressures in the intracranial venous sinuses proximal to stenotic lesions in the transverse sinuses. These lesions have now been demonstrated on MR venography. This study investigated whether they would be visible on CT. CT venography was performed on 10 patients with BIH and compared with 10 controls, matched for age and sex, undergoing CT angiography for subarachnoid haemorrhage. All controls were confirmed to have had ruptured intracranial aneurysms at craniotomy. Using a semi-automated technique to develop a profile of the cross-sectional areas along the lateral sinuses and to minimize observer bias, the narrowest point on each side was identified and summated in every subject. All patients with BIH exhibited a region of marked narrowing in both transverse sinuses, usually near the junction with the sigmoid sinus, rarely seen in our control group. Measured cross-sectional areas in these venous outflow tracts were substantially different between patients with BIH and controls (p<0.001). CT venography frequently demonstrates transverse sinus narrowing in BIH.

Two stage management of petrositis with associated mycotic aneurysm of the intrapetrous carotid artery.

Mycotic aneurysm of the petrous temporal bone is extremely rare, with only 12 cases previously reported. We review the literature to date and present a case of petrositis complicated by a mycotic aneurysm of the internal carotid artery, which was managed by endovascular balloon occlusion and subsequent total petrosectomy. We can find no other case in the literature where balloon occlusion has been used to treat a mycotic aneurysm of the intrapetrous carotid artery prior to total petrosectomy to treat petrositis.

MR venography in idiopathic intracranial hypertension: unappreciated and misunderstood.

BACKGROUND: Venous sinus disease must be excluded before diagnosing idiopathic intracranial hypertension but is found only rarely in typical cases. Magnetic resonance venography (MRV) is the technique of choice for investigating this, and provides images that are diagnostic and easy to interpret. However, recent work using more invasive techniques has documented pressure gradients and stenoses in the lateral venous sinuses in many cases of idiopathic intracranial hypertension. OBJECTIVE: To examine the reason for this discrepancy and to establish whether there are characteristic appearances on MRV in idiopathic intracranial hypertension that are routinely overlooked in clinical practice. METHODS: MRVs from 20 patients with idiopathic intracranial hypertension were reviewed, unblinded, by two neuroradiologists, and their appearances rated for focal narrowings and signal gaps. A control group of 40 asymptomatic volunteers, matched for age and sex with the patient group, was recruited prospectively for MRV, and their scans rated in the same way. RESULTS: The lateral sinuses presented a range of appearances with quite different distributions in the two groups (p<0.001). Bilateral lateral sinus flow gaps were seen in 13 of 20 patients with idiopathic intracranial hypertension and in none of 40 controls. CONCLUSIONS: A historical failure to use normal healthy controls to establish the boundaries between imaging artefact, normal anatomical variant, and disease means that the pathological significance of the different appearances of the lateral sinuses on MRV has not so far been appreciated.

Idiopathic intracranial hypertension: 12 cases treated by venous sinus stenting.

BACKGROUND: The high pressures documented in the intracranial venous sinuses in idiopathic intracranial hypertension (IIH) could be the result of focal stenotic lesions in the lateral sinuses obstructing cranial venous outflow. OBJECTIVE: To explore the relation between venous sinus disease and IIH. METHODS: 12 patients with refractory IIH had dilatation and stenting of the venous sinuses after venography and manometry had shown intracranial venous hypertension proximal to stenoses in the lateral sinuses. Intrasinus pressures were recorded before and after the procedure and correlated with clinical outcome. RESULTS: Intrasinus pressures were variably reduced by stenting. Five patients were rendered asymptomatic, two were improved, and five were unchanged. CONCLUSIONS: The importance of venous sinus disease in the aetiology of IIH is probably underestimated. Lateral sinus stenting shows promise as an alternative treatment to neurosurgical intervention in intractable cases.

Tuberous sclerosis presenting in late adult life.

A 59 year old woman presented with a three year history of left sided weakness. Magnetic resonance imaging of the brain showed a large high signal lesion occupying most of the right temporal lobe with mass effect. A probable diagnosis of low grade glioma led to temporal lobectomy. Histology revealed dysplastic cortical morphology typical of tuberous sclerosis. There were no clinical signs or family history of the disease. Ultrasound showed multiple bilateral renal angiomyolipomas, confirming the diagnosis of tuberous sclerosis. Molecular genetic analysis of peripheral white blood cells identified a novel mis-sense mutation R1409W in exon 33 of the TSC2 gene.

Cavernous sinus syndrome as the only manifestation of sarcoidosis.

We describe a 63-year-old Afro-Caribbean female who presented with complete ophthalmoplegia of the right eye. Magnetic resonance imaging revealed a mass in the cavernous sinus, which was pathologically confirmed as due to sarcoidosis. No clinical or laboratory evidence of sarcoidosis was found in any other organs. We believe this is the first reported case of an isolated cavernous sinus syndrome due to sarcoidosis and suggest that sarcoidosis should be considered in the differential diagnosis of cavernous sinus syndromes even in the absence of systemic involvement of sarcoidosis.

Endarterectomy of an Occluded ICA: Short Segment Occlusion with Distal Patency Maintained by an Aberrant Ascending Pharyngeal Artery.

SUMMARY: The authors report a case in which an anomalous ascending pharyngeal artery maintained patency of the internal carotid artery beyond an occlusion. The patient presented with ipsilateral transient ischaemic attacks and was found to have an internal carotid artery occlusion on Doppler ultrasound. Subsequent angiography demonstrated retrograde flow in the ipsilateral APA, perfused by pharyngeal anastomoses from the contralateral APA with slow antegrade flow in the ipsilateral ICA distal to the occlusion. Carotid endarterectomy resulted in abolition of the TIA's.

Intraosseous vertebral haemangioblastoma: MRI.

A 30-year-old man with low back pain and gradual onset of cord compression was found to have a highly vascular extradural tumour in the lower thoracic region. Involvement of the right pedicle and a large part of the body of T9 suggested a vertebral haemangioma, but histology revealed haemangioblastoma. One case of an intraosseous haemangioblastoma has been described previously; we present the features of another case shown by MRI and discuss the differentiation between haemangioma and haemangioblastoma.

Organising haematoma mimicking tumour on MRI following resection of acoustic neuroma.

We describe a 26-year-old woman in whom an enhancing, intradural extramedullary mass was found at the craniocervical junction on MRI 3 years after resection of a large acoustic neuroma. The radiological appearances suggested a new tumour, raising the possibility of neurofibromatosis 2, provoking a review of family members in an attempt to confirm the diagnosis, as well as further surgery. Histology of the lesion revealed an organising haematoma, with no evidence of malignancy. The imaging features can be explained by the process by which blood clot in the subarachnoid space is resorbed. Caution is advised in interpreting CT OR MRI after neurosurgery.

CT-directed perineural infiltration in the localization of radicular pain in a patient with neurofibromatosis.

Computed tomography-guided infiltration of lumbar or sacral nerve roots with local anaesthetic can be a valuable aid in localizing the spinal level from which radicular pain originates especially when multiple abnormalities are present. This is illustrated by the case of a young woman with neurofibromatosis complaining of sciatica.