Resection of cavernous angioma located in eloquent areas using functional cortical and subcortical mapping under awake conditions. Outcomes in a 50-case multicentre series.

INTRODUCTION: Surgical resection of supratentorial cavernous angiomas located in eloquent areas poses a significant risk to the patient of postoperative neurological impairment and justifies intraoperative functional monitoring. METHODS: Multicentre retrospective series of adult patients with cavernous angiomas located within eloquent areas and treated with functional-based surgical resection according to functional boundaries under intraoperative functional cortico-subcortical monitoring under awake conditions. RESULTS: Fifty patients (18 males, mean 36.3±10.8 year-old) underwent surgical resection with intraoperative cortico-subcortical functional mapping using direct electrostimulation under awake conditions for a cavernous angioma located in eloquent areas with a mean postoperative follow-up of 21.0±21.2 months. At presentation, the cavernous angioma had previously resulted in severe impairment (neurological deficit in 34%, seizures in 70%, uncontrolled seizures in 34%, reduced Karnofsky Performance Status score of 70 or less in 24%, inability to work in 52%). Functional-based surgical resection allowed complete removal of the cavernous angioma in 98% and of the haemosiderin rim in 82%. Postoperative seizures and other complications were rare, and similarly so across all centres included in this series. Postoperatively, we found functional improvement in 84% of patients (reduced Karnofsky Performance Status score of 70 or less in 6%, uncontrolled seizures in 16%, and inability to work in 11%). CONCLUSION: Functional-based surgical resection aids the safe and complete resection of cavernous angiomas located in eloquent areas while minimizing the surgical risks. Functional mapping has to be considered in such challenging cases.

Development of minimally invasive surgery for sinonasal malignancy.

Sinonasal malignancies are rare and histologically heterogeneous. Treatment is complicated by tumor aggressiveness and location near critical anatomic structures (orbita, skull base, etc.). This low incidence and histologic diversity make prospective studies unfeasible, and thus therapeutic guidelines difficult to establish. The gold standard for surgery is a transfacial approach, with craniofacial resection in case of skull-base involvement. However, these techniques are associated with non-negligible perioperative morbidity. In the past two decades, endoscopic surgery has made major progress, extending its indications: initially developed for functional sinus surgery, it is now applied in benign skull-base pathologies (CSF leakage, meningocele, etc.) and, more recently, in sinonasal malignancy. Literature analysis shows a significant decrease in morbidity and improved quality of life associated with endoscopic endonasal surgery, with oncologic safety and efficacy in well-selected cases, although dependent on operator experience. Additional studies with longer follow-up and comparison between histologic subtypes will be needed.

Comparison of cardiac output measured by oesophageal Doppler ultrasonography or pulse pressure contour wave analysis.

BACKGROUND: Maintaining adequate organ perfusion during high-risk surgery requires continuous monitoring of cardiac output to optimise haemodynamics. Oesophageal Doppler Cardiac Output monitoring (DCO) is commonly used in this context, but has some limitations. Recently, the cardiac output estimated by pulse pressure analysis- (PPCO) was developed. This study evaluated the agreement of cardiac output variations estimated with 9 non-commercial algorithms of PPCO compared with those obtained with DCO. METHODS: High-risk patients undergoing neurosurgery were monitored with invasive blood pressure and DCO. For each patient, 9 PPCO algorithms and DCO were recorded before and at the peak effect for every haemodynamic challenge. RESULTS: Sixty-two subjects were enrolled; 284 events were recorded, including 134 volume expansions and 150 vasopressor boluses. Among the 9 algorithms tested, the Liljestrand-Zander model led to the smallest bias (0.03 litre min(-1) [-1.31, +1.38] (0.21 litre min(-1) [-1.13; 1.54] after volume expansion and -0.13 litre min(-1) [-1.41, 1.15] after vasopressor use). The corresponding percentage of the concordance was 91% (86% after volume expansion and 94% after vasopressor use). The other algorithms, especially those using the Winkessel concept and the area under the pressure wave, were profoundly affected by the vasopressor. CONCLUSIONS: Among the 9 PPCO algorithms examined, the Liljestrand-Zander model demonstrated the least bias and best limits of agreement, especially after vasopressor use. Using this particular algorithm in association with DCO calibration could represent a valuable option for continuous cardiac output monitoring of high risk patients. CLINICAL TRIAL REGISTRATION: Comité d'éthique de la Société de Réanimation de Langue Française No. 11-356.

Ossifying fibromas of the paranasal sinuses: diagnosis and management.

Fibro-osseous benign lesions rarely affect the sinonasal tract and are divided into 3 different entities, namely osteoma, fibrous dysplasia and ossifying fibroma. They share several clinical, radiological and histological similarities, but have different behaviours. Ossifying fibroma, and in particular the "juvenile" histological subtype, may have a locally aggressive evolution and a high risk for recurrence if removal is incomplete. The purpose of the present study is to compare the clinical behaviour of ossifying fibroma with the other benign fibro-osseous lesions; highlight different behaviour between the histological subtypes; compare the advantages, limitations and outcomes of an endoscopic endonasal approach with reports in the literature. We retrospectively reviewed 11 patients treated for sinonasal ossifying fibroma at a tertiary care centre. All patients underwent CT scan, and MRI was performed in cases of cranial base involvement or recurrence. Pre-operative biopsy was performed in cases where it was possible to use an endoscopic approach. One patient underwent pre-operative embolisation with ipsilateral visual loss after the procedure. Depending on its location, removal of the tumour was performed using an endoscopic (n = 7), or an external (n = 3) or combined (n = 1) approach. Histopathologically, 5 patients presented the conventional type, 5 the juvenile psammomatoid variant, which was associated in 1 case with an aneurismal bone cyst, and 1 case presented the trabecular juvenile variant. Three patients affected by the juvenile psammomatoid histological variant presented invasion of the skull base and underwent a subtotal removal that subsequently required, due to the regrowth of the remnant, a transbasal approach. Clinical, radiological and histological findings should all be considered to establish differential diagnosis among fibrous osseous lesions. More studies are necessary to conclude if the localisation and extension of the disease at the time of diagnosis is more important than the histological variant. An endoscopic approach is the first choice in most of cases even if an external open approach may be necessary in selected patients.

French collaborative group series on giant intracranial aneurysms: Current management.

OBJECTIVES: Giant intracranial aneurysms represent a major therapeutic challenge for each surgical team. The aim of our study was to extensively review the French contemporary experience in treating giant intracranial aneurysms in order to assess the current management. PATIENTS AND METHODS: This retrospective multicenter study concerned consecutive patients treated for giant intracranial aneurysms (2004-2008) in different French university hospitals (Bordeaux, Caen, Clermont-Ferrand, Lille, Lyon, Nice, Paris-Lariboisière, Rouen et Toulouse). Different variables were analyzed: the diagnostic circumstances, the initial clinical status based on the WFNS scale, aneurysmal features and exclusion procedure. At 6 months, the outcome was evaluated according to the modified Rankin Scale (mRS): favorable (mRS 0-2) and unfavorable (mRS 3-6). A multivariate logistic regression model included all the independent variables with P<0.25 in the univariate analysis (P<0.05). RESULTS: A total of 79 patients with a mean age of 51.5 ± 1.6 years (median: 52 years; range: 16-79) were divided into two groups, with the ruptured group (n=26, 32.9%) significantly younger (P<0.05, Student's-t-test) than the unruptured group (n=53, 67.1%). After SAH, the initial clinical status was good in 12 patients (46.2%), and in the unruptured group, the predominant diagnosis circumstance was a pseudo-tumor syndrome occurring in 22 (41.5%). The first procedure of aneurysm treatment in the global population was endovascular in 42 patients (53.1%), microsurgical in 29 (36.7%) and conservative in 8 (10.2). An immediate neurological deterioration was reported in 38 patients (48.1%) after endovascular treatment in 19 (45.2% of endovascular procedures), after miscrosurgical in 15 (51.7% of microsurgical procedures) and after conservative in 4 (the half). At 6 months, the outcome was favorable in 45 patients (57%) and after multivariate analysis, the predictive factors of favorable outcome after management of giant cerebral aneurysm were the initial good clinical status in cases of SAH (P<0.002), the endovascular treatment (P<0.005), and the absence of neurological deterioration (P<0.006). The endovascular procedure was obtained as a predictive factor because of the low risk efficacy of indirect procedures, in particular a parent vessel occlusion. CONCLUSION: The overall favorable outcome rate concerned 57% of patients at 6 months despite 53.8% of poor initial clinical status in cases of rupture. The predictive factors for favorable outcome were good clinical status, endovascular treatment and the absence of postoperative neurological deterioration. Endovascular treatment should be integrated into the therapeutic armenmatarium against giant cerebral aneurysms but the durability of exclusion should be taken into account during the multidisciplinary discussion by the neurovascular team.

Endoscopic endonasal approach for the treatment of schwannomas of the pterygopalatine fossa: case report and review of the literature.

INTRODUCTION: Pterygopalatine fossa (PPF) schwannomas are rare lesions most often arising from branches of the trigeminal nerve. Symptomatic lesions have been traditionally treated by conventional external approaches. However, the development of an expanded endonasal approach (EEA) enables skull base surgeons to reach these deeply seated lesions via a different route with its own advantages and drawbacks. METHODS: Case report and review of the literature. CASE DESCRIPTION: A 41-year-old woman presented with a 6-year history of right facial pain and numbness. Her symptoms had increased progressively over a year, and she recently had developed right-sided otalgia. MRI revealed a right PPF mass, hypointense on T1 and T2 sequences with homogeneous enhancement following the use of gadolinium. A biopsy, attempted at another institution, was considered non-diagnostic. We totally removed the lesion through an endoscopic endonasal transmaxillary approach. Final pathology confirmed the diagnosis of schwannoma. Post-operatively, the patient noted a significant improvement of her facial pain (V2 territory). CONCLUSION: The endonasal endoscopic transmaxillary approach provides adequate access to the PPF, thus enabling safe tumor removal with less morbidity than conventional routes.

[Chordoma]. / Les chordomes.

PURPOSES: To review in the literature, all the epidemiological, clinical, radiological, histological and therapeutic data regarding chordomas as well as various notochordal entities: ecchordosis physaliphora, intradural and intraparenchymatous chordomas, benign notochordal cell tumors, parachordomas and extra-axial chordomas. To identify different types of chordomas, including familial forms, associations with tuberous sclerosis, Ollier's disease and Maffucci's syndrome, forms with metastasis and seeding. To assess the recent data regarding molecular biology and progress in targeted therapy. To compare the different types of radiotherapy, especially protontherapy and their therapeutic effects. To review the largest series of chordomas in their different localizations (skull base, sacrum and mobile spine) from the literature. MATERIALS: The series of 136 chordomas treated and followed up over 20 years (1972-2012) in the department of neurosurgery at Lariboisière hospital is reviewed. It includes: 58 chordomas of the skull base, 47 of the craniocervical junction, 23 of the cervical spine and 8 from the lombosacral region. Similarly, 31 chordomas in children (less than 18 years of age), observed in the departments of neurosurgery of les Enfants-Malades and Lariboisière hospitals, are presented. They were observed between 1976 and 2010 and were located intracranially (n=22 including 13 with cervical extension), 4 at the craniocervical junction level and 5 in the cervical spine. METHODS: In the entire Lariboisière series and in the different groups of localization, different parameters were analyzed: the delay of diagnosis, of follow-up, of occurrence of metastasis, recurrence and death, the number of primary patients and patients referred to us after progression or recurrence and the number of deaths, recurrences and metastases. The influence of the quality of resection (total, subtotal and partial) on the prognosis is also presented. Kaplan-Meier actuarial curves of overall survival and disease free survival were performed in the entire series, including the different groups of localization based on the following 4 parameters: age, primary and secondary patients, quality of resection and protontherapy. In the pediatric series, a similar analysis was carried-out but was limited by the small number of patients in the subgroups. RESULTS: In the Lariboisière series, the mean delay of diagnosis is 10 months and the mean follow-up is 80 months in each group. The delay before recurrence, metastasis and death is always better for the skull base chordomas and worse for those of the craniocervical junction, which have similar results to those of the cervical spine. Similar figures were observed as regards the number of deaths, metastases and recurrences. Quality of resection is the major factor of prognosis with 20.5 % of deaths and 28 % of recurrences after total resection as compared to 52.5 % and 47.5 % after subtotal resection. This is still more obvious in the group of skull base chordomas. Adding protontherapy to a total resection can still improve the results but there is no change after subtotal resection. The actuarial curve of overall survival shows a clear cut in the slope with some chordomas having a fast evolution towards recurrence and death in less than 4 years and others having a long survival of sometimes more than 20 years. Also, age has no influence on the prognosis. In primary patients, disease free survival is better than in secondary patients but not in overall survival. Protontherapy only improves the overall survival in the entire series and in the skull base group. Total resection improves both the overall and disease free survival in each group. Finally, the adjunct of protontherapy after total resection is clearly demonstrated. In the pediatric series, the median follow-up is 5.7 years. Overall survival and disease free survival are respectively 63 % and 54.3 %. Factors of prognosis are the histological type (atypical forms), localization (worse for the cervical spine and better for the clivus) and again it will depend on the quality of resection. CONCLUSIONS: Many different pathologies derived from the notochord can be observed: some are remnants, some may be precursors of chordomas and some have similar features but are probably not genuine chordomas. To-day, immuno-histological studies should permit to differentiate them from real chordomas. Improving knowledge of molecular biology raises hopes for complementary treatments but to date the quality of surgical resection is still the main factor of prognosis. Complementary protontherapy seems useful, especially in skull base chordomas, which have better overall results than those of the craniocervical junction and of the cervical spine. However, we are still lacking an intrinsic marker of evolution to differentiate the slow growing chordomas with an indolent evolution from aggressive types leading rapidly to recurrence and death on which more aggressive treatments should be applied.

Radionecrosis of the frontal lobe as a consequence of malignant ethmoid tumor management: incidence, diagnosis, risk factors, prevention and management.

Malignant ethmoid tumors are treated by surgery followed by radiotherapy. This study aimed to evaluate the incidence, risk factors and outcome of radionecrosis of frontal lobe and determine preventive measures. Retrospective study of ethmoid malignancies treated from 2000 to 2011. All patients underwent surgery with/without anterior skull base resection using endoscopic or external approaches followed by irradiation (mean dose 64 Gy). Median follow-up was 50 months. Eight of 50 patients (16 %) presented with fronto-basal radionecrosis, connected to duraplasty, with a latent interval of 18.5 months. Although asymptomatic in six, radionecrosis triggered seizures and required surgery in two cases. Survival was not impacted. Risk factors included dyslipidemia, occurrence of epilepsy and dural resection. Radionecrosis may result from the combination of anterior skull base resection and radiotherapy for the treatment of ethmoid malignancies. Preventive measures rely on improving the duraplasty and optimization of the Gy-dose delivery.

Functionally tailored transcortical approach of deep-seated lesions: an alternative to the transulcal approach? a technical case report.

It is commonly believed that sulci offer a natural path to reach deep-seated lesions. However, it has also been argued that this approach carries a risk of damaging the vessels during the opening of the sulcus.We therefore were prompted to test the possibility of finding a transcortical path identified as non-functional by intraoperative brain mapping. A successful resection is presented of a left posterior is thmusclear cell ependymoma through a selected corridor based on functional mapping in an awake patient.MRI performed at 12 months showed no tumour recurrence. Pre- and postoperative extensive testing confirmed an improvement of the patient's cognitive functions. Therefore, we were able to demonstrate the feasibility of a functionally tailored transcortical approach as an alternative to the transulcal approach for deep-seated lesions. This concept should be validated in a larger patient series.

Endoscopic endonasal skull base surgery.

Skull base surgery has been transformed by the development of endoscopic techniques. Endoscopic procedures were first used for pituitary surgery and were then gradually extended to other regions. A wide range of diseases are now accessible to endoscopic skull base surgery. The major advantage of the endoscopic endonasal approach is that it provides direct anatomical access to a large number of intracranial and paranasal sinus lesions, avoiding the sequelae of a skin incision, facial bone flap or craniotomy, and brain retraction, which is inevitable with conventional neurosurgical incisions, resulting in decreased morbidity and mortality and, indirectly, decreased length of hospital stay and management costs. Moreover, the increasing number of publications in this field illustrates the growing interest in these techniques. This paper provides a review of endoscopic skull base surgery. The indications and general principles of endoscopic endonasal skull base surgery are described. Progress in exposure and especially reconstruction techniques is described. This progress now allows more extensive resections, while maintaining acceptable morbidity. The limits of this surgery are also discussed; in particular, although this surgery is often described as "minimally invasive", it is not completely devoid of morbidity.

The use of Onyx in different types of intracranial dural arteriovenous fistula.

BACKGROUND AND PURPOSE: Recently some series have been published about the use of Onyx for the treatment of DAFVs with satisfactory results. Our aim was to describe the treatment of different types of intracranial DAVFs with transcatheter injection of Onyx through an arterial approach. MATERIALS AND METHODS: At the Department of Interventional Neuroradiology, Hospital Lariboisière Paris, between January 2005 and January 2010, we treated 44 DAVFs in 42 patients. All patients were initially treated by arterial injection of Onyx. The average patient age was 56 years (range, 27-86 years), and there were 17 women and 25 men treated. RESULTS: A total of 58 arterial pedicles were catheterized, with the middle meningeal artery representing the most common site (n = 38). The average time of injection was 30 minutes (range, 15-60 minutes), and the average amount of Onyx was 2.5 mL (range, 0.6-6.5 mL). Of the 20 fistulas with direct venous drainage into a dural sinus (types I and II), we achieved the preservation of the sinus in 7 patients. Of the 44 fistulas embolized, 8 required a second embolization treatment and 1 fistula required a third treatment. In 9 cases, a complementary treatment was performed via transvenous embolization with coils and/or open surgery. Early complications were observed in 6 patients: Four had nerve injury (facial palsy, n = 2, and neuralgia, n = 2), and 2 had complications related to extension of venous thrombosis postembolization. All 6 patients had partial or complete resolution of these symptoms. CONCLUSIONS: The treatment of DAVFs by intracranial arterial injection of Onyx is safe, and, in most cases, results in the occlusion of the arterial venous shunt. In DAVFs with direct sinus drainage, sinus preservation was only possible in 7 of 20 patients (35%).

Vidian nerve neurofibroma removed via a transpterygoid approach.

BACKGROUND: Lesions originating in the vidian canal are extremely rare. Most frequently, they are extensions from contiguous carcinomas. We present a rare case of a vidian nerve neurofibroma and discuss its surgical management. CASE REPORT: A 62-year-old woman with a history of a basal cell skin cancer was evaluated for bilateral tinnitus. Imaging revealed a left-sided lesion at the medial aspect of the pterygoid process base, over the vidian canal. Under image-guidance, an endonasal endoscopic transpterygoid approach was performed. The histopathological examination supported the diagnosis of neurofibroma. CONCLUSION: Benign nerve sheath tumors of the vidian nerve should be considered in the differential diagnosis of a vidian canal lesion. Given the propensity of more aggressive tumors, a tissue diagnosis should be warranted in order to coordinate appropriate subsequent treatment. The expanded endonasal transpterygoid approach offers a safe, less invasive, and effective route to perform the excisional biopsy of such a lesion.

Skull bone flap fixation - reliability and efficacy of a new grip-like titanium device (Skull Grip) versus traditional sutures: a clinical randomized trial.

BACKGROUND: After completing a craniotomy, it is important to replace the removed bone flap in its natural position in order to guarantee brain protection as well as improve cosmesis. A skull defect can expose the brain to accidental damage, and in cases of larger defects it may also cause the patients psychosocial problems. The ideal fixation device should provide reliable attachment of the flap to the skull and promote fast bony healing to avoid possible pseudo-arthrosis and/or osteolytic changes. MATERIALS AND METHODS: This is a pilot randomized clinical trial on a series of 16 patients undergoing different craniotomies for benign brain lesions in which the bone flaps were replaced using traditional sutures (Prolene 0.0) in 8 cases and with a new skull fixation device (Skull Grip) in the other 8 (randomly allocated). All patients underwent CT scans of the head with 3D reconstruction at day 1 and day 90 postoperatively to evaluate bone flap position and fusion. These scans were independently reviewed by a neuroradiologist. Cosmesis was also evaluated clinically by the surgeon and radiologically by the neuroradiologist in the 2 patient groups. RESULTS: The new "Skull Grip" device has shown stronger fixation qualities with optimal bone flap fusion and increased cosmetic healing features vs. traditional sutures. CONCLUSION: The "Skull Grip" has shown to be a reliable, effective and stronger bone flap fixation device when compared to traditional sutures.

Neurosurgery and elderly: analysis through the years.

The aging of the population in westernized countries constitutes an important issue for the health systems struggling with limited resources and increasing costs. Morbidity and mortality rates reported for neurosurgical procedures in the elderly vary widely. The lack of data on risk benefit ratios may result in challenging clinical decisions in this expanding group of patients. The aim of this paper is to analyze the elderly patients cohort undergoing neurosurgical procedures and any trend variations over time. The medical records of elderly patients (defined as an individual of 70 years of age and over) admitted to the Neurosurgical and Neuro-ICU Departments of a major University Hospital in Paris over a 25-year period were retrospectively reviewed. The analysis included: (1) number of admissions, (2) percentage of surgically treated patients, (3) type of procedures performed, (4) length of hospital stay, and (5) mortality. The analysis showed a progressive and significant increase in the proportion of elderly presenting for neurosurgical elective and/or emergency procedures over the last 25 years. The number of procedures on patients over 70 years of age increased significantly whereas the mortality dropped. Though the length of hospital stay was reduced, it remained significantly higher than the average stay. The types of procedures also changed over time with more craniotomies and endovascular procedures being performed. Age should not be considered as a contraindication for complex procedures in neurosurgery. However, downstream structures for postoperative elderly patients must be further developed to reduce the mean hospital stay in neurosurgical departments because this trend is likely to continue to grow.

Primary malignant rhabdoid tumour of the brain in adults.

Malignant rhabdoid tumour (MRT) was described for the first time in the kidney, and is rarely reported in the brain. Most rhabdoid tumours affect infants and young children and there have been only isolated adult patients reported. The optimal treatment for this very aggressive tumour has not yet been established. We describe the clinical and pathological features of a rare primary malignant rhabdoid tumour of the brain in a 27-year-old pregnant female. The literature is reviewed briefly and the role of the INI1 gene in adult MRTs and the also possible interactions between MRTs and pregnancy are discussed.

Skull bone flap fixation--comparative experimental study to assess the reliability of a new grip-like titanium device (Skull Grip) versus traditional sutures: technical note.

INTRODUCTION: After completing a craniotomy, whenever possible, it is crucial to replace and fix the removed bone flap to the cranium; this in order to keep the brain's protection as well as for cosmetic purposes. Visible skull defects might cause patients psychosocial problems and, most importantly, expose the brain to accidental damage. A fixation device should not only provide optimal attachment of the flap to the skull but also allow fast bony healing to avoid possible pseudoarthrosis and/or osteolytic changes. METHODS: After performing 12 different craniotomies on 4 human cadaver heads the skull flaps were replaced using traditional sutures and a new skull fixation device; for each fixation technique a load-bearing test was performed and the results compared. RESULTS: Bone flaps fixed with the "Skull Grip" showed a strong fixation with optimal plastic deformation when compared to flaps held by sutures that showed less resistance to pressure and could be easily dislocated. CONCLUSION: The "Skull Grip" has shown to be a reliable, effective, and stronger bone flap fixation superior to suturing technique.

Dumbbell-shaped jugular foramen schwannomas: surgical management, outcome and complications on a series of 16 patients.

Schwannomas of the lower cranial nerves are very rare and the dumbbell-shaped ones are even rarer. The authors report their experience in managing such lesions, usually presenting either with intra- and/or extra-cranial extension through an enlarged jugular foramen. The juxtacondylar approach without sacrificing the labyrinth was used; clinical, radiological and complication features are discussed and analysed. This is a case series study on 16 consecutive patients with lower cranial nerves schwannomas surgically managed during a 14-year period using the juxtacondylar approach. In 13 cases, a complete resection has been achieved whereas in three, the excision was near total. The tumour nerve origin has been identified only in ten cases (62.5%). No death or additional post-operative cranial nerve deficits occurred. Aspiration pneumonia developed in one patient and cerebrospinal fluid leak in another. Pre-operative lower cranial nerve deficits improved in all patients. At a mean follow-up of 6.6 years (range 2-14 years), no radiological tumour recurrence was recorded amongst the patients having complete resection as well as no tumour progression in the group of near total removal. Jugular foramen schwannomas can be radically and safely resected with no additional neurological deficit if a careful pre-operative evaluation and the appropriate surgical approach is implemented. Finally, full cranial nerve functional recovery may be expected after complete resection.