Lateral skull base chondroblastoma resected with facial nerve posterior transposition.

INTRODUCTION: Chondroblastoma is a rare tumor that can involve the temporal bone. Because it is a benign tumor, functional surgery must be proposed. We report a case of a patient with a massive chondroblastoma operated on with preservation of the facial nerve, and description of the surgical technique. CASE PRESENTATION: A 37-year-old man presented with a 9-month history of a growing left pre-auricular mass and hearing loss. Neuroimaging showed an osteolytic mass invading the temporal bone and temporomandibular joint. Excision was performed via a transpetrosal and transcochlear approach with posterior transposition of the facial nerve. EMG monitoring was effective in preventing facial palsy. Four years later, no sign of recurrence was observed. DISCUSSION: Chondroblastoma is a locally aggressive tumor, especially when located in the petrous bone and temporomandibular joint. The suggested treatment is a complete excision.

Inter- and Intrarater Agreement on the Outcome of Endovascular Treatment of Aneurysms Using MRA.

BACKGROUND AND PURPOSE: Patients treated with coiling are often followed by MR angiography. Our objective was to assess the inter- and intraobserver agreement in diagnosing aneurysm remnants and recurrences by using multimodality imaging, including TOF MRA. MATERIALS AND METHODS: A portfolio composed of 120 selected images from 56 patients was sent to 15 neuroradiologists from 10 institutions. For each case, raters were asked to classify angiographic results (3 classes) of 2 studies (32 MRA-MRA and 24 DSA-MRA pairs) and to provide a final judgment regarding the presence of a recurrence (no, minor, major). Six raters were asked to independently review the portfolio twice. A second study, restricted to 4 raters having full access to all images, was designed to validate the results of the electronic survey. RESULTS: The proportion of cases judged to have a major recurrence varied between 16.1% and 71.4% (mean, 35.0% ± 12.7%). There was moderate agreement overall (κ = 0.474 ± 0.009), increasing to nearly substantial (κ = 0.581 ± 0.014) when the judgment was dichotomized (presence or absence of a major recurrence). Agreement on cases followed-up by MRA-MRA was similarly substantial (κ = 0.601 ± 0.018). The intrarater agreement varied between fair (κ = 0.257 ± 0.093) and substantial (κ= 0.699 ± 0.084), improving with a dichotomized judgment concerning MRA-MRA comparisons. Agreement was no better when raters had access to all images. CONCLUSIONS: There is an important variability in the assessment of angiographic outcomes of endovascular treatments. Agreement on the presence of a major recurrence when comparing 2 MRA studies or the MRA with the last catheter angiographic study can be substantial.

Imaging of giant cerebral aneurysms.

The aim of this study was to review the different imaging techniques for analysing giant intracranial aneurysms (digital subtraction angiography [DSA], magnetic resonance imaging [MRI], computed tomography [CT]) imaging and explain their respective contribution to the understanding of the characteristics of these complex aneurysms. Giant aneurysms have a complex pathology with multiple stages of evolution and consequences. Therefore, complex imaging is mandatory to enhance the understanding of these parameters and to plan an often complicated treatment strategy. DSA remains the gold standard for analysing aneurysms, but non-invasive sectional imaging (CT, MRI) also provides essential information in the specific case of giant aneurysms.

Coincidental vascular anomalies at the foramen magnum: dural arteriovenous fistula and high flow aneurysm on perimedullary fistula.

We report the case of a 59-year-old woman admitted for a sudden headache due to a subarachnoid haemorrhage. On CT scan, the clots predominated into the posterior fossa without high-density in the sylvian or interhemispheric fissures. The vertebral angiography revealed a dural arteriovenous fistula at the foramen magnum associated to an aneurysm of the cervical anterior spinal artery. Due to the high rebleeding risk of a dural shunt, we proposed curative treatment using microsurgical interruption of the intradural draining vein. On the postoperative angiography at 15-day follow-up, the 2 malformations were corrected and the outcome at 6 months was excellent. Based on the literature, we assess this exceptional association and suggest its possible management.

Elderly patients with aneurysmal subarachnoid hemorrhage: Coils but also clips.

The ageing of the population in good health or without severe morbidity expose them to the occurrence of a subarachnoid hemorrhage (SAH) and requires effective management. Currently, the pertinence of cerebral aneurysm treatment by clipping or coiling is accepted for patients in the 8th or 9th decade of life, and the risk of postoperative morbidity induced by our therapeutic alternative must be carefully assessed. In these decades, the female/male sex ratio for aneurysmal SAH was greater in female who had a 1.6 times higher ratio than in male. The initial clinical status did not appear worse with age despite the frequent severity of bleeding observed on CT scan probably due to the large subarachnoid space. The aneurysm distribution and size were similar to those classically reported in the global population. The endovascular (EV) coiling appears as the first option with a favorable outcome rate estimated at 48% to 63%. Nevertheless, the benefit of EV coiling compared to microsurgical clipping for treatment of ruptured aneurysm in the elderly has not been demonstrated in a large randomized study. This is the reason why the vascular section of the French Society of Neurosurgery developed a prospective and randomized study of the aneurysmal SAH (PHRC 2007-042/HP) on the elderly patients.

[Stroke and cornea verticillata revealing Fabry's disease in a female]. / Accident vasculaire cérébral ischémique et cornée verticillée révélant une maladie de Fabry chez une femme.

INTRODUCTION: Fabry's disease is a X-linked lysosomal storage disorder caused by an alpha-galactosidase A deficiency responsible for the accumulation of glycosphingolipids. Males are more severely and frequently affected than females. We report the case of a female who presented a stroke revealing Fabry's disease. CASE REPORT: An 53-year-old woman, with cardiovascular risk factors and two previous transient ischemic attacks, was admitted with a brutal right hemiparisia. Cerebral MRI showed multiple white matter lesions in the cerebral hemispheres with multiple lacunar infarcts and ectatic vessels, cardiac echography revealed a hypertrophic concentric cardiomyopathy, and slit-lamp examination demonstrated a cornea verticillata. The sequencing of the alpha-galactosidase gene (GLA) revealed the c.150_151del mutation responsible for a loss of function. DISCUSSION: As in the present case, ophthalmological examination is very useful to determine Fabry's disease as a cause of young onset stroke. Females may be affected by X-linked disease, as the Fabry's disease. Fabry's disease among females is mainly characterized by the involvement of the nervous and cardiovascular systems. The specific treatment is based on an enzyme replacement therapy by recombinant enzyme with cardiovascular benefit. Despite the presence of cardiovascular risk factors, this case demonstrates the importance of thorough standardized investigations including ophthalmological examination of young patients with stroke.

[Clinical and ossification outcome of custom-made hydroxyapatite prothese for large skull defect]. / Tolérance clinique et degré d'ossification des cranioplasties en hydroxyapatite de larges défects osseux.

OBJECTIVE: Cranioplasty is an everyday concern in neurosurgery, especially in decompressive craniectomy cases. Our surgical team uses custom-made hydroxyapatite implants for large and/or complex defects. PATIENTS AND METHOD: Eight patients had a custom-made prosthesis. Each of them has been reviewed by an independent observer. Each patient described his feeling of satisfaction, using a questionnaire, graduated from "A" (really satisfied) to "D" (unsatisfied). Each of them also underwent a CT-scan (helicoidal acquisition, 0.6mm thick for multiplanar reconstruction) to evaluate qualitatively the ossification graduated from "0" (no ossification) to "5" (continuous ossification). Maximal under-prosthetic bone thickness, intra-prosthetic calcic density were also reported. RESULTS: Supervision delay was 43.7 months [6-99 months], average defect surface was 85.5 cm(2) [27.6-137.6 cm(2)], the craniectomy etiologies were intracranial hypertension (seven patients) and calvarial invasion (one patient). Implant tolerance was reparted in "A" score (50%) and "B" score (50%). Concerning ossification, six patients (75%) had a score of "2" or less and two patients had a score of "3" or "4". DISCUSSION: Hydroxyapatite custom-made implants for cranioplasty appear to be ideal for good aesthetic and tolerance results, but their ossification is hardly analyzed due to the prosthesis density higher than the bone's density. This is why we recommend them for children and in cases of complex defects such as pterion location.

Outcomes of endovascular treatments of aneurysms: observer variability and implications for interpreting case series and planning randomized trials.

BACKGROUND AND PURPOSE: Angiographic results are commonly used as a surrogate marker of success of coiling of intracranial aneurysms. Inter- and intraobserver agreement in judging angiographic results remain poorly characterized. Our goal was to offer such an evaluation of a grading scale commonly used to evaluate results of endovascular treatment of aneurysms. MATERIALS AND METHODS: A portfolio of 90 angiographic images from 45 patients selected from the core lab data base of a randomized trial was sent to 12 observers on 2 occasions more than 3 months apart. The variability of a 3-value grading scale used to score angiographic results and of a final judgment regarding the presence of a recurrence was studied using κ statistics. RESULTS: Ten participants responded once and 6 responded twice. Agreement was poor to moderate (κ = 0.28-0.5) for senior and junior observers judging angiographic results immediately or 12-18 months after treatment. Agreement reached a reassuring "substantial" (κ = 0.62) level, with a dichotomous presence-absence of a major recurrence, and intraobserver agreement was better in experienced core lab assessors. CONCLUSIONS: There is an important variability in the assessment of angiographic outcomes of endovascular treatments, rendering comparisons between publications risky, if not invalid. A simple dichotomous judgment can be used as a surrogate outcome in randomized trials designed to assess the value of new endovascular devices.

Endovascular parent artery occlusion for the treatment of wide-neck A1 segment aneurysms: a single-center experience.

The goal of this study was to evaluate the feasibility and efficacy of A1 occlusion at the level of wide necked A1 aneurysms, where there are bilateral patent A1 segments and a patent AcomA. Between 2000 and 2010, 9 patients with wide necked A1 aneurysms were treated by coiling of the aneurysm along with parent vessel occlusion. All aneurysms had a wide neck (≥ 4 mm). None were treated in the acute phase of a subarachnoid hemorrhage. Three small infarcts were noted on routine post-treatment head CT, 1 of which was symptomatic (transient hemiparesthesia). On control angiogram at 6 months or more, 3 A1 recanalizations were found, 2 of which had a stable small neck recurrence. None of the aneurysms ruptured on follow-up. In this series, parent artery occlusion was effective in treating wide-necked aneurysms arising from the A1 segment in patients with adequate collateral supply.

[CT scan, MR imaging and anatomopathologic correlation in the glottic carcinoma T1-T2]. / Corrélation TDM, IRM et anatomopathologique dans les cancers glottiques T1-T2.

OBJECTIVES: Endoscopy and imaging are necessary to diagnose glottis carcinoma. Today, CT scan is the gold standard but MR imaging should be more sensitive for neoplastic invasion detection. The purpose of this study was to determine which exam to perform (CT scan or MRI) for neoplastic invasion. MATERIAL AND METHODS: This prospective study span a 18 months period. Seven patients with glottis carcinoma (TI-T2) underwent CT and MR imaging before surgery. Findings at imaging and pathologic examination were compared. RESULTS: Both CT and MR imaging were interesting, more specific (90%) than sensitive (74%). The anterior commissure, ventricles, subglottis, thyroid and arytenoid cartilages are the more difficult area analyzing. CONCLUSION: CT imaging stays the gold standard. MR imaging is more effective on second intention to refine the data. Indications are being improved, with complete cure and more preserved laryngealfunctions.

Usefulness of multislice computerized tomography angiography in preoperative diagnosis of ruptured cerebral aneurysms.

OBJECTIVE: Non-invasive imaging methods have become primordial in subarachnoid hemorrhage. The aim of our study was to evaluate the sensitivity and specificity of multislice computed tomographic angiography (MSCTA) for the diagnosis of cerebral aneurysm. METHODS: The 28 included consecutive patients with SAH underwent both MSCTA and digital subtraction angiography (DSA). The MSCTA studies were interpreted by two independent readers (A and B) for the presence, the location and size of the aneurysm comparatively to the DSA as reference examination. RESULTS: In 20 patients, 38 aneurysms were diagnosed and in eight no aneurysm was found. Per patient basis, the diagnostic sensitivity and specificity were excellent. Per aneurysm basis, the diagnostic sensitivity and specificity of MSCTA were, respectively, 97.4 and 100% for reader A, 100 and 100% for reader B. For aneurysms less than 3mm, sensitivity was 100% for both readers. Interobserver agreement was excellent for the detection of aneurysm (kappa=0.98, 95% CI [0.96-1]). Intertechnique and interobserver agreements were excellent for the measurement of aneurysms (slope=0.86, r=0.91 p=3.1x10(-7) and slope=1.04, r=0.99, p<10(-6), respectively). CONCLUSION: MSCTA was an accurate and reproducible non-invasive imaging technique for preoperative diagnosis of ruptured cerebral aneurysm. The MSCTA may be proposed in first intention after the diagnosis of SAH was established, with special care regarding injection procedure and a strict reading method using native images and thin MPR.

[Spontaneous supratentorial cerebral hemorrhage: role of surgical treatment]. / Hémorragie cérébrale supratentorielle spontanée: quelle place pour un traitement chirurgical?

Until very recently, no specific therapies have been demonstrated to improve outcome after spontaneous intracerebral haemorrhage (ICH). The STICH (surgical treatment for intracerebral haemorrhage) study showed no overall benefit from early surgery when compared with initial conservative treatment. In contrast, the stereotactic aspiration technique can be safely performed and in a uniform manner. Despite the reduction of ICH volume, no improvement in mortality and functional result was obtained. Endoscopy is a new therapeutic option for ICH with good results for hematoma removal. Based on these feasibility studies, a randomized control trial regarding this procedure would be required to assess the efficacy of this procedure. Due to the lack of benefit observed in the recent STICH trial, emergency surgical evacuation should be reserved for patients with large lobar haemorrhage, mass effect and rapidly deteriorating clinical condition.