Longitudinal comparison of dyssynchrony correction and strain improvement by conduction system pacing: LEVEL-AT trial secondary findings.

Longitudinal dyssynchrony correction and strain improvement by comparable cardiac resynchronization therapy techniques is unreported. AIMS: Our purpose was to compare echocardiographic dyssynchrony correction and strain improvement by conduction system pacing (CSP) vs. biventricular pacing (BiVP) as a marker of contractility improvement during one-year follow-up. METHODS AND RESULTS: A treatment-received analysis was performed in patients included in the LEVEL-AT trial (NCT04054895), randomized to CSP or BiVP, and evaluated at baseline (ON and OFF programming) and at 6 and 12 months (n = 69, 32% women). Analysis included intraventricular (septal flash), interventricular (difference between left and right ventricular outflow times), and atrioventricular (diastolic filling time) dyssynchrony and strain parameters (septal bounce, global longitudinal strain [GLS], left bundle branch block pattern and mechanical dispersion).Baseline left ventricular ejection fraction (LVEF) was 27.5 ± 7% and left ventricular end-systolic volume (LVESV) was 138 ± 77 ml, without differences between groups. Longitudinal analysis showed LVEF and LVESV improvement (p < 0.001), without between-group differences. At 12-month follow-up, adjusted mean LVEF was 46% with CSP (95%CI 42.2%, 49.3%) vs. 43% with BiVP (95%CI 39.6%, 45.8%) (p = 0.31) and LVESV was 80 ml (95%CI 55.3 ml, 104.5 ml) vs. 100 ml (95%CI 78.7 ml, 121.6 ml), respectively (p = 0.66).Longitudinal analysis showed a significative improvement of all dyssynchrony parameters and GLS over time (p < 0.001), without differences between groups. Baseline GLS significantly correlated with LVEF and LVESV at 12-month follow-up. CONCLUSION: CSP and BiVP provided similar dyssynchrony and strain correction over time. Baseline global longitudinal strain correction predicted ventricular remodeling at 12-month follow-up.

Stepwise application of ECG and electrogram-based criteria to ensure electrical resynchronization with left bundle branch pacing.

AIMS: To define a stepwise application of left bundle branch pacing (LBBP) criteria that will simplify implantation and guarantee electrical resynchronization. Left bundle branch pacing has emerged as an alternative to biventricular pacing. However, a systematic stepwise criterion to ensure electrical resynchronization is lacking. METHODS AND RESULTS: A cohort of 24 patients from the LEVEL-AT trial (NCT04054895) who received LBBP and had electrocardiographic imaging (ECGI) at 45 days post-implant were included. The usefulness of ECG- and electrogram-based criteria to predict accurate electrical resynchronization with LBBP were analyzed. A two-step approach was developed. The gold standard used to confirm resynchronization was the change in ventricular activation pattern and shortening in left ventricular activation time, assessed by ECGI. Twenty-two (91.6%) patients showed electrical resynchronization on ECGI. All patients fulfilled pre-screwing requisites: lead in septal position in left-oblique projection and W paced morphology in V1. In the first step, presence of either right bundle branch conduction delay pattern (qR or rSR in V1) or left bundle branch capture Plus (QRS ≤120 ms) resulted in 95% sensitivity and 100% specificity to predict LBBP resynchronization, with an accuracy of 95.8%. In the second step, the presence of selective capture (100% specificity, only 41% sensitivity) or a spike-R <80 ms in non-selective capture (100% specificity, sensitivity 46%) ensured 100% accuracy to predict resynchronization with LBBP. CONCLUSION: Stepwise application of ECG and electrogram criteria may provide an accurate assessment of electrical resynchronization with LBBP (Graphical abstract).

The Epidermal Growth Factor Domain of the Mutation Does Not Appear to Influence Disease Progression in CADASIL When Brain Volume and Sex Are Taken into Account.

BACKGROUND AND PURPOSE: By studying the evolution of brain volume across the life span in male and female patients, we aimed to understand how sex, brain volume, and the epidermal growth factor repeat domain of the mutation, the 3 major determinants of disability in CADASIL, interact in driving disease evolution. MATERIALS AND METHODS: We used validated methods to model the evolution of normalized brain volume with age in male and female patients using nonparametric regression in a large, monocentric cohort with prospectively collected clinical and high-resolution MR imaging data. We used k-means clustering to test for the presence of different clinical course profiles. RESULTS: We included 229 patients (mean age, 53 [SD, 12] years; 130 women). Brain volume was larger in women (mean size, 1024 [SD, 62] cm3 versus 979 [SD, 50] cm3; P < .001) and decreased regularly. In men, the relationship between brain volume and age unexpectedly suggested an increase in brain volume around midlife. Cluster analyses showed that this finding was related to the presence of a group of older male patients with milder symptoms and larger brain volumes, similar to findings of age-matched women. This group did not show specific epidermal growth factor repeat domain distribution. CONCLUSIONS: Our results demonstrate a detrimental effect of male sex on brain volume throughout life in CADASIL. We identified a subgroup of male patients whose brain volume and clinical outcomes were similar to those of age-matched women. They did not have a specific distribution of the epidermal growth factor repeat domain, suggesting that yet-unidentified predictors may interact with sex and brain volume in driving disease evolution.

Lactobacillus endocarditis: a case report in France and literature review.

Lactobacilli are commensal bacteria in the normal flora of the oral cavity, gastrointestinal and genital tract. However, few cases of lactobacilli-induced bacteremia or endocarditis have been reported, particularly in immunocompromised patients. We reported a rare case of a 57-year-old man with a Lactobacillus rhamnosus endocarditis without immunodeficiency in his medical history. He received a dental scaling one year before. Clinical presentation included weight loss, heart murmur, ankle arthritis and splinter hemorrhage. Echocardiography showed a mitral prolapse and a 16 mm vegetation associated with a valvular perforation. All blood cultures were positive for Lactobacillus rhamnosus. Antibacterial regimen with amoxicillin and gentamicin led to recovery without surgery. We present a literature review of the lactobacillary endocarditis cases published since 1992. Valvulopathy, dental or invasive procedures and probiotics use were the main underlying conditions, in contrary to immunodeficiency. Diagnosis of lactobacillary endocarditis should be more considered and a treatment with penicillin and aminoglycoside should be promptly introduced in life-threatening cases.

MRI evaluation of the endolymphatic space in otosclerosis and correlation with clinical findings.

PURPOSE: The purpose of this study was to investigate the clinical features of ears with otosclerosis and their correlation with endolymphatic hydrops and blood-labyrinth barrier (BLB) impairment on 3T magnetic resonance imaging (MRI). MATERIALS AND METHODS: This was a single-center retrospective imaging study. Thirty-nine ears from 29 patients (17 men, 12 women; mean age 52±12 [SD] years; range 27-74 years) with non-operated otosclerosis were included. All patients underwent three-dimensional fluid attenuated inversion recovery (FLAIR) MRI sequences performed 4hours after the intravenous administration of a single dose of gadolinium-based contrast material. MRI examinations were analyzed by two radiologists for the presence of saccular hydrops (SH) and BLB impairment. Results of MRI examinations were compared with clinical findings, hearing levels and extent of otosclerotic lesions based on high-resolution computed tomography findings. BLB impairment was evaluated using the signal intensity ratio, ratio of intensities between the basal turn of the cochlea and the medulla. RESULTS: SH was observed in 1/39 (3%) otosclerotic ears and BLB impairment in 8/39 (21%) while 8/29 patients with otosclerosis (28%) had vertigo. No significant associations were found between SH or BLB impairment on MRI, and the presence of vertigo or the degree of sensorineural hearing loss. CONCLUSION: Clinical manifestations of otosclerosis (sensorineural hearing loss and rotatory vertigo) were not significantly associated with MRI findings such as BLB impairment and endolymphatic hydrops. SH was only observed in one patient with obstruction of the vestibular aqueduct by an otosclerotic focus.

Comparison of Enhancement of the Vestibular Perilymph between Variable and Constant Flip Angle-Delayed 3D-FLAIR Sequences in Menière Disease.

BACKGROUND AND PURPOSE: Endolymphatic hydrops in patients with Menière disease relies on delayed postcontrast 3D-FLAIR sequences. The purpose of this study was to compare the degree of perilymphatic enhancement and the detection rate of endolymphatic hydrops using constant and variable flip angles sequences. MATERIALS AND METHODS: This was a retrospective study performed in 16 patients with Menière disease who underwent 3T MR imaging 4 hours after gadolinium injection using two 3D-FLAIR sequences with a constant flip angle at 140° for the first and a heavily-T2 variable flip angle for the second. The signal intensity ratio was measured using the ROI method. We graded endolymphatic hydrops and evaluated the cochlear blood-labyrinth barrier impairment. RESULTS: Both for symptomatic and asymptomatic ears, the median signal intensity ratio was significantly higher with the constant flip angle than with the heavily-T2 variable flip angle (7.16 versus 1.54 and 7.00 versus 1.45, P < .001). Cochlear blood-labyrinth barrier impairment was observed in 4/18 symptomatic ears with the heavily-T2 variable flip angle versus 8/19 with constant flip angle sequences. With heavily-T2 variable flip angle sequences, endolymphatic hydrops was observed in 7-10/19 symptomatic ears versus 12/19 ears with constant flip angle sequences. We found a significant association between the clinical symptomatology and the presence of endolymphatic hydrops with constant flip angle but not with heavily-T2 variable flip angle sequences. Interreader agreement was always perfect with constant flip angle sequences while it was fair-to-moderate with heavily-T2 variable flip angle sequences. CONCLUSIONS: 3D-FLAIR constant flip angle sequences provide a higher signal intensity ratio and are superior to heavily-T2 variable flip angle sequences in reliably evaluating the cochlear blood-labyrinth barrier impairment and the endolymphatic space.

Anatomo-radiological study supporting the use of ipsilateral nasoseptal flap for the transpterygoid management of temporo-sphenoidal meningoceles. A review of 21 cases.

OBJECTIVES: Surgical treatment of temporo-sphenoidal meningoceles involves the reduction of the meningocele, watertight closure and defect coverage with a nasoseptal flap (NSF). It can be performed contralaterally or ipsilaterally: in the latter situation, the pedicle of the flap must be dissected into the pterygopalatine fossa. The objective of this study was to evaluate the benefit of using an ipsilateral NSF in transpterygoid approaches for the management of temporo-sphenoidal meningoceles, compared to a contralateral NSF, based on a radiological study. MATERIAL AND METHODS: Retrospective monocentric study of 21 cases, between 2002 and 2018. Measurement of the NSF lengths, and lengths needed to cover the defect were evaluated on the preoperative scanner. Early and later failure and complication rates were evaluated. RESULTS: Seventeen cases of temporo-sphenoidal meningoceles with available CT scan were identified. The mean duration of follow up was 27.9 months [1-147]. Theoretical lengths of the ipsi and contralateral NSF were comparable: 71.4±7.8mm vs. 78.8±8mm, P=0.729. In 8 cases/18 (42%), the theoretical length of the contralateral NSF was not long enough to cover the defect beyond the V2 (mean lack of 8.87±6.6mm). In all cases, the theoretical length of the ipsilateral NSF was sufficient to cover the defect. In the case series, failure and complication rates were similar. CONCLUSION: The use of an ipsilateral NSF for the transpterygoid management of temporo-sphenoidal meningoceles, although more complex, allows a better coverage of the defect, compared to the contralateral NSF, which is not long enough in 42% of cases.

What's next after the clot? Residual pulmonary vascular obstruction after pulmonary embolism: From imaging finding to clinical consequences.

Surviving an embolism exposes patients to potential long-term complications, such as altered quality of life, persistent dyspnea, impaired exercise capacity or pulmonary hypertension. The common objective factor in most of these situations is the presence of residual pulmonary vascular obstruction (RPVO). Planar ventilation/perfusion scintigraphy (V/Q lung scan) is the gold standard for assessing RPVO, which occurs in 46 to 66% of patients at 3 months and persists in 25 to 29% of patients a year after acute PE. Assessed early (i.e. before discharge), RPVO could predict acute PE development with a high negative predictive value. Evaluated after anticoagulation therapy, RPVO could help to manage anticoagulation treatment and predict the risk of PE recurrence and patients identified at risk of developing chronic thromboembolic pulmonary hypertension. In this comprehensive review, we provide an overview of the current knowledge of RPVO after PE from imaging diagnosis to clinical consequences. In the first part, we mainly focus on the imaging modalities capable of detecting and quantifying RPVO. We then focus on the symptoms and syndromes linked with this residual obstruction after PE. Although the occurrence of RPVO and long-term complications varies greatly from one patient to another, we finally aim to identify the patients and diseases at risk of developing residual obstruction.

Sensorineural hearing loss in patients with vestibular schwannoma correlates with the presence of utricular hydrops as diagnosed on heavily T2-weighted MRI.

PURPOSE: The purpose of this study was to assess whether the volume of the vestibular endolymphatic space correlates with the degree of hearing loss using heavily T2-weighted fast imaging employing steady-state acquisition with cycle phase (FIESTA-C) MRI. MATERIALS AND METHODS: A total of 23 patients with vestibular schwannoma, as diagnosed on typical image findings, who underwent FIESTA-C MRI were included. There were 13 women and 10 men with a mean age of 63.5±9.3 (SD) years (range: 49-88years). Two radiologists independently evaluated the volume of the utricle and saccule. Correlation between tumor volume, vestibular endolymphatic space volume and degree of hearing loss - as evaluated with the levels of pure-tone average and speech recognition threshold - were searched for. RESULTS: The mean saccular, utricular and tumor volumes were 3.17±1.1 (SD) mm3 (range: 1.45-5.7mm3), 14.55±5 (SD) mm3; (range: 6.6-23.9mm3) and 17.4±5.5 (SD) mm3; (range: 8.3-25.4mm3), respectively. There was a moderate correlation between the volume of the utricle and the degree of hearing loss as evaluated with the levels of pure-tone average (rho=0.5; P=0.015) and speech recognition threshold (rho=0.58; P=0.004). There were no significant correlations between saccular and tumor volumes and the degree of hearing loss. CONCLUSION: The volume of the utricle in patients with obstructive vestibular schwannoma moderately correlates with the degree of hearing loss.

Postoperative MR imaging features after superior semicircular canal plugging in Minor syndrome.

PURPOSE: To report the postoperative magnetic resonance imaging (MRI) features after superior semicircular canal plugging in patients with Minor syndrome. MATERIALS AND METHODS: The MRI examinations with 3D T2-weighted SPACE sequence of 12 patients with superior semicircular canal dehiscence syndrome (SCDS) were retrospectively assessed. Two radiologists independently evaluated the presence of a filling defect of the superior semicircular canal above the superior ampulla and the common crus using an oblique plane parallel to the superior semicircular canal (Pöschl's plane). RESULTS: Postoperative MRI showed a filling defect above the ampulla of the superior semicircular canal and the common crus in 8/12 patients (67%). Three patients (3/12; 25%) had a filling defect involving also the superior ampulla that caused postoperative labyrinthitis with labyrinthine enhancement on MRI in 2 patients. One patient (1/12; 8%) had incomplete plugging of superior semicircular canal with abnormal functional tests and remaining symptoms. CONCLUSION: Postoperative MRI shows a normal plugging aspect of the superior semicircular canal in 67% of patients. MRI can reveal complications that may have therapeutic implications.

Infratemporal fossa tumors: When to suspect a malignant tumor? A retrospective cohort study of 62 cases.

OBJECTIVES: Infratemporal fossa (ITF) tumors are rare and little is known about their general epidemiology, making it sometimes difficult for clinicians, who seldom encounter them, to distinguish between benign and malignant forms on the basis of the initial clinical and radiological work-up alone. The objectives of this retrospective study were: (i) to determine the respective prevalences of the various histologic types of ITF tumor, and (ii) to assess associations between certain clinical and radiological features and malignancy. METHODS: A single-center observational study in a university hospital included all new consecutive cases of ITF tumor treated from January 2000 to December 2016. Histologic type, demographics, clinical presentation and imaging findings were analyzed. RESULTS: In total, 62 patients were included. 74% of tumors were benign (n=46) and 26% malignant. Juvenile nasopharyngeal angiofibroma, adenoid cystic carcinoma and schwannoma were the most frequent histologic types, accounting for 47%, 16% and 10% of cases, respectively. The only clinical or imaging signs significantly associated with malignancy were trismus, facial pain, facial hypoesthesia and neural invasion on magnetic resonance imaging (all P-values<0.05). CONCLUSION: This study provides general epidemiological data on ITF tumors, and identified several clinical and radiologic signs to help clinicians suspect malignancy.

[Large vessel vasculitis with myelodysplastic syndrome: A rare association]. / Vascularite des gros vaisseaux et syndrome myélodysplasique : une association rare.

INTRODUCTION: The vasculitis can be the consequence of malignancy: most often hematologic rather than solid tumors. The association between large vessels vasculitis and myelodysplastic syndrome is rare. CASE REPORT: A 55-year-old man experienced asthenia, fever, polyarthritis and inflammatory syndrome. Haematological investigations found a type 2 refractory anemia with excess blasts (RAEB-2) with discovery of severe anemia (Hb: 7,8g/dl) and thrombopenia (platelets: 40,000/mm3). Radiological examinations found thoracic aortitis and carotid vasculitis. Treatment in the form of steroids and azacitidine was instituted. The lack of control of both RAEB-2 and vasculitis was responsible for the death of the patient. CONCLUSION: Myelodysplastic syndrome and large vessels vasculitis is a rare but serious association disease. The lack of efficiency of corticosteroids seems to be common. Prognosis depends on the haematological treatment effectiveness.

Morphological and functional imaging of neck paragangliomas.

OBJECTIVE: To review the optimal techniques for localization and characterization of neck paragangliomas (PGL). MATERIAL AND METHODS: Systematic review of the literature from the PubMed/Medline database. RESULTS: Neck PGL are hypervascular tumours essentially arising from paraganglionic tissue situated at the carotid bifurcation (carotid body) and along the vagus nerve. Morphological and functional imaging are indicated to confirm the diagnosis, identify multifocal disease and for local and regional staging. MR angiography is the noninvasive technique of choice. CT scan and especially CT angiography are excellent alternatives for diagnosis and staging. Conventional arteriography remains useful preoperatively for embolization and occlusion tests. Functional imaging allows localization and characterization of PGLs. Somatostatin receptor scintigraphy (SRS) was the reference imaging technique for staging of sporadic PGLs. The indications for PET imaging have been extended over recent years in parallel with the development of new tracers such as [18F]-FDOPA PET or 68Gallium-labelled DOTA peptides. 68Gallium-labelled DOTA peptides has become the first-line imaging modality in the evaluation of cervical PGLs, regardless of the genetic background. CONCLUSION: Morphological and functional imaging is essential for the staging of neck PGL.

[A not so poorly-defined opacity]. / Un nodule pas si flou.

A 60-year-old patient was under follow-up for pulmonary hypertension consistent with pulmonary veno-occlusive disease. During his follow-up, a parenchymal opacity was discovered. We describe the management of the suspicion of lung cancer, highlighting the modification of the conventional diagnostic and therapeutic strategy on account of the pulmonary hypertension. Chest physicians should be able to adapt their diagnostic and therapeutic management in the case of neoplasia in patients with severe pulmonary hypertension.

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.

[Subcutaneous nodules of the head and neck heralding giant cell arteritis]. / Nodules sous-cutanés cervico-céphaliques précédant l'apparition d'une artérite à cellules géantes.

BACKGROUND: Giant cell arteritis is the most common form of systemic vasculitis affecting individuals aged over 50 years. While its clinical manifestations are numerous, cutaneous involvement is uncommon and rarely constitutes the initial sign. We discuss a case of atypical skin involvement as the initial symptom of giant cell arteritis. OBSERVATION: An 86-year-old woman presented purplish and painful subcutaneous nodules on the scalp and neck. Biological explorations showed systemic inflammation. The skin biopsy was evocative of polyarteritis nodosa. The nodules spontaneously disappeared completely, and asthenia and bitemporal headache gradually appeared. In view of the persistent inflammatory syndrome, a diagnosis of giant cell arteritis was proposed and was later confirmed by the temporal artery biopsy sample, with its typical histological appearance. Systemic corticosteroids resulted in complete regression of symptoms within a few days. DISCUSSION: To our knowledge, inflammatory cervical subcutaneous nodules have never been described in giant cell arteritis. The case we report herein thus raises the issue of differential diagnosis between various forms of vasculitis. While classification of the latter continues to progress thanks to improvements in physiopathological knowledge, the distinction between vasculitis of the large and small vessels remains tenuous on occasion. We discuss the differential diagnoses. CONCLUSION: The dermatological presentation of giant cell arteritis in the present case suggests the existence of a continuum between small-, medium- and large-vessel vasculitis.

[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.