Navigated intraoperative ultrasound in neuro-oncology: volumetric accuracy and correlation with high-field MRI.

OBJECTIVE: The use of intraoperative techniques to detect residual tumors has recently become increasingly important. Intraoperative MRI has long been considered the gold standard; however, it is not widely used because of high equipment costs and long acquisition times. Consequently, real-time intraoperative ultrasound (ioUS), which is much less expensive than MRI, has gained popularity. The aim of the present study was to evaluate the capacity of ioUS to accurately determine the primary tumor volume and detect residual tumors. METHODS: A prospective study of adult patients who underwent surgery for intra-axial brain tumors between November 2017 and October 2020 was performed. Navigated intraoperative ultrasound (nioUS) of the brain was used to guide tumor resection and to detect the presence of residual disease. Both convex (5-8 MHz) and linear array (6-13 MHz) probes were used. Tumor volume and residual disease were measured with nioUS and compared with MR images. A linear regression model based on a machine learning pipeline and a Bland-Altman analysis were used to assess the accuracy of nioUS versus MRI. RESULTS: Eighty patients (35 females and 45 males) were included. The mean age was 58 years (range 25-80 years). A total of 88 lesions were evaluated; there were 64 (73%) gliomas, 19 (21.6%) metastases, and 5 (5.7%) other tumors, mostly located in the frontal (41%) and temporal (27%) lobes. Most of the tumors (75%) were perfectly visible on ioUS (grade 3, Mair grading system), except for those located in the insular lobe (grade 2). The regression model showed a nearly perfect correlation (R2 = 0.97, p < 0.001) between preoperative tumor volumes from both MRI and nioUS. Ultrasonographic visibility significantly influenced this correlation, which was stronger for highly visible (grade 3) tumors (p = 0.01). For residual tumors, the correlation between postoperative MRI and nioUS was weaker (R2 = 0.78, p < 0.001) but statistically significant. The Bland-Altman analysis showed minimal bias between the two techniques for pre- and postoperative scenarios, with statistically significant results for the preoperative concordance. CONCLUSIONS: The authors' findings show that most brain tumors are well delineated by nioUS and almost perfectly correlated with MRI-based measurements both pre- and postoperatively. These data support the hypothesis that nioUS is a reliable intraoperative technique that can be used for real-time monitoring of brain tumor resections and to perform volumetric analysis of residual disease.

Microsurgical transcranial approaches to the posterior surface of petrosal portion of the temporal bone: quantitative analysis of surgical volumes and exposed areas.

Different microsurgical transcranial approaches (MTAs) have been described to expose the posterior surface of the petrous bone (PPB). A quantitative, anatomical comparison of the most used MTAs, for specific areas of the PPB, is not available. Anatomical dissections were performed on five formalin-fixed, latex-injected cadaver heads (10 sides). Six MTAs were analyzed: Kawase approach (KWA), retrosigmoid approach (RSA), retrosigmoid approach with suprameatal extension (RSAS), retrolabyrinthine approach (RLA), translabyrinthine approach (TLA), and transcochlear approach (TCA). Surgical volumes and exposed areas of each approach were quantified with a dedicated neuronavigation system (ApproachViewer, part of GTx-Eyes II, University Health Network, Toronto, Canada) and adjuvant software (ITK-SNAP and Autodesk Meshmixer 3.5). Areas and volumes were compared using linear mixed models. TCA provided the best exposure of Trautmann's triangle and the retromeatal, suprameatal, meatal, and premeatal regions. RSAs provided the best exposure of the inframeatal region, with RSAS gaining significant exposure of the suprameatal region. KWA had the highest surgical volume, and RLA the lowest. Transpetrosal approaches offer the widest exposure of PPB proportionally to their invasiveness. Retrosigmoid approaches, which get to the studied region through a postero-lateral path, are paramount for the exposure of the inframeatal and suprameatal region and, given the adequate exposure of the remaining PPB, represent an effective approach for the cerebellopontine angle (CPA). These anatomical findings must be considered with approach-related morbidity and the pathological features in order to choose the most appropriate approach in clinical practice.

Anatomical Step-by-Step Dissection of Complex Skull Base Approaches for Trainees: Endoscopic Endonasal Approach to the Orbit.

Introduction Although endonasal endoscopic approaches (EEA) to the orbit have been previously reported, a didactic resource for educating neurosurgery and otolaryngology trainees regarding the pertinent anatomy, techniques, and decision-making pearls is lacking. Methods Six sides of three formalin-fixed, color latex-injected cadaveric specimens were dissected using 4-mm 0- and 30-degree rigid endoscopes, as well as standard endoscopic equipment, and a high-speed surgical drill. The anatomical dissection was documented in stepwise three-dimensional (3D) endoscopic images. Following dissection, representative case applications were reviewed. Results EEA to the orbit provides excellent access to the medial and inferior orbital regions. Key steps include positioning and preoperative considerations, middle turbinate medialization, uncinate process and ethmoid bulla removal, complete ethmoidectomy, sphenoidotomy, maxillary antrostomy, lamina papyracea resection, orbital apex and optic canal decompression, orbital floor resection, periorbita opening, dissection of the extraconal fat, and final exposure of the orbit contents via the medial-inferior recti corridor. Conclusion EEA to the orbit is challenging, in particular for trainees unfamiliar with nasal and paranasal sinus anatomy. Operatively oriented neuroanatomy dissections are crucial didactic resources in preparation for practical endonasal applications in the operating room (OR). This approach provides optimal exposure to the inferior and medial orbit to treat a wide variety of pathologies. We describe a comprehensive step-by-step curriculum directed to any audience willing to master this endoscopic skull base approach.

Intraoperative use of heads-up display in skull base surgery.

In this video, the authors highlight the applications of virtual reality and heads-up display in skull base surgery by presenting the case of a 45-year-old woman with an incidental large clinoid meningioma extending into the posterior fossa. The patient underwent preoperative endovascular tumor embolization to facilitate tumor resection and reduce blood loss, followed by a right pterional craniotomy. The use of intraoperative Doppler, intraoperative neurophysiological monitoring, and endoscope-assisted microsurgery is also featured. A subtotal resection was planned given tumor encasement of the posterior communicating and anterior choroidal arteries. No new neurological deficits were noted after the surgical procedure. The video can be found here: https://stream.cadmore.media/r10.3171/2021.10.FOCVID21177.

Anatomical Step-by-Step Dissection of Complex Skull Base Approaches for Trainees: Surgical Anatomy of the Middle Fossa Approaches and Anterior Petrosectomy, Surgical Principles, and Illustrative Cases.

Introduction The middle fossa (MF) approaches encompass a group of versatile surgical accesses to pathologies in the MF, internal auditory canal (IAC), and superomedial aspect of the posterior fossa. Although many descriptions of the MF approaches have been published, a practical surgical guide that allows an easy understanding for Skull Base trainees is needed. Methods Three formalin-fixed, colored-injected specimens were dissected under microscopic magnification (six sides). A MF craniotomy followed by IAC drilling was performed on three sides, and anterior petrosectomy (AP) was performed in the remaining three sides. The anatomical dissection was documented in stepwise three-dimensional photographic images. Following dissection, representative case applications were reviewed. Results The MF approach provides direct access to the MF structures and IAC. The AP provides excellent access to the superomedial aspect of the posterior fossa. Key common steps include: positioning and skin incision; scalp and muscle flaps; burr holes; craniotomy flap elevation; dural dissection along the petrous ridge; division of the middle meningeal artery; and exposure of the greater superficial petrosal nerve, tegmen tympani, and V3. Then, to approach the IAC: superior IAC drilling, and longitudinal dura opening. The area drilled in the AP approach forms a pentagon limited by the petrous internal carotid artery, cochlea, IAC, petrous ridge, and lateral border of V3. Conclusion The MF approaches are challenging. Operatively oriented skull base dissections provide a crucial foundation for learning these techniques. We describe comprehensive step-by-step approaches intended to develop familiarity in the cadaver laboratory and facilitate understanding of their potential for skull base disorders. Basic surgical principles are described to help in the operating room as well as illustrative cases.

Endoscopic Endonasal Approaches to the Clivus with No Violation of the Nasopharynx: Surgical Anatomy and Clinical Illustration.

Background Despite the use of vascularized intranasal flaps, endoscopic endonasal posterior fossa defects remain surgically challenging with high rates of postoperative cerebrospinal fluid leak. Objective The aim of the study is to describe a novel surgical technique that allows complete drilling of the clivus and exposure of the craniovertebral junction with preservation of the nasopharynx. Methods Two formalin-fixed latex-injected anatomical specimens were used to confirm feasibility of the technique. Two surgical approaches were used: sole endoscopic endonasal approach and transnasion approach. The sole endonasal approach was used in a patient with a petroclival meningioma. Results In both anatomical dissections, the inferior clivectomy with exposure of the foramen magnum was achieved with a sole endoscopic endonasal approach. The addition of the transnasion approach helped to complete drilling of the inferior border of the foramen magnum and exposure of the arch of C1. Conclusion This study shows the anatomical feasibility of total clivectomy and exposure of the craniovertebral junction with preservation of the nasopharynx. A more favorable anatomical posterior fossa defect for the reconstruction is achieved with this technique. Further clinical studies are needed to assess if this change would impact the postoperative CSF leak rate.

Cerebral sinus thrombosis as an initial symptom of acute promyelocytic leukemia: Case report and literature review.

Background: Cerebral sinus thrombosis as presentation of acute promyelocytic leukemia (AMPL) is exceptional, with only three cases registered in the literature. Case Description: A 24-year-old female patient was transferred to our center after a car accident. The patient had a witnessed generalized seizure while driving. Computerized tomography (CT) demonstrated a temporal intraparenchymal hemorrhage and CT venogram diagnosed a cerebral sinus thrombosis on the left transverse and sigmoid sinus. The patient underwent surgical evacuation of the hematoma and was treated with anticoagulation 48 h after surgery. Pancytopenia alerted of a possible hematological disorder. The patient was subsequently diagnosed with AMPL and treated with arsenic trioxide. The patient had a complete neurological recovery with no postoperative complications. Conclusion: The management of cerebral sinus thrombosis in patients with AMPL remains controversial. The previous reported cases of cerebral sinus thrombosis preceding the diagnosis of AMPL are reviewed and treatment of cerebral sinus thrombosis with anticoagulation in the setting of intraparenchymal hemorrhage and bleeding disorders is also discussed.

Neuronavigated Ultrasound in Neuro-Oncology: A True Real-Time Intraoperative Image.

OBJECTIVE: Ultrasound is considered a real-time imaging method in neuro-oncology because of its highly rapid image acquisition time. However, to our knowledge, there are no studies that analyze the additional surgical time that it requires. METHODS: A prospective study of 100 patients who underwent intra-axial brain tumor resection with navigated intraoperative ultrasound. The primary outcomes were lesion visibility grade on ultrasound and concordance with preoperative magnetic resonance imaging (MRI) scan, intraoperative ultrasound usage time, and percentage of tumor resection on ultrasound and comparison with postoperative MRI scan. RESULTS: The breakdown of patients included the following: 53 high-grade gliomas, 26 metastases, 14 low-grade gliomas, and 7 others. Ninety-six percent of lesions were clearly visualized. The tumor border was clearly delimited in 71%. Concordance with preoperative MRI scan was 78% (P < 0.001). The mean time ± SD for sterile covering of the probe was 2.16 ± 0.5 minutes, and the mean image acquisition time was 2.49 ± 1.26 minutes. Insular tumor location, low-grade glioma, awake surgery, and recurrent tumor were statistically associated with an increased ultrasound usage time. Ultrasound had a sensitivity of 94.4% and a specificity of 100% for residual tumor detection. CONCLUSIONS: Neuronavigated ultrasound can be considered a truly real-time intraoperative imaging method because it does not increase surgical time significantly and provides optimal visualization of intra-axial brain lesions and residual tumor.

Orbital Anatomy: Anatomical Relationships of Surrounding Structures.

Advances in skull base and orbital surgery have led to an increased need to understand the anatomy of the orbit and surrounding structures to safely perform surgeries in this area. The purpose of this article is to review the surrounding anatomy of the orbit from a practical and operative point of view. We describe the orbit from an inferomedial endoscopic endonasal perspective (focusing on its inferior relationship with the maxillary sinus and related structures and its medial relationship with the ethmoid bone), from a posterior and superolateral intracranial perspective (describing the anatomy of the superior orbital fissure, optic canal, inferior orbital fissure, cavernous sinus, orbitofrontal cortex, and surrounding dura) and from an anterior perspective (focusing on the muscles, connective tissue, lateral and medial canthus, and relevant neurovascular anatomy). A deep knowledge of the critical neurovascular and osseous structures surrounding the orbit is necessary for adequately choosing and performing the most favorable orbital approach in every case.

Surgical Anatomy of the Labyrinthine and Subarcuate Arteries and Clinical Implications.

OBJECTIVE: To study the surgical anatomy of the labyrinthine artery (LA) and the subarcuate artery (SA), their anatomic relationships, and clinical implications, as injury of the LA can result in hearing loss. METHODS: Ten formalin-fixed, latex-colored specimens were studied (20 sides). After retrosigmoid craniotomy and neurovascular dissection under microscopic magnification, 4-mm 0° and 30° endoscopic lenses were used to improve visualization. Results were statistically analyzed. RESULTS: The LA was a constant artery that followed the vestibulocochlear nerve into the internal auditory canal. The SA was an inconstant artery that ended in the dura mater around the subarcuate fossa in 35% of cases. The LA originated from the anterior inferior cerebellar artery in 89.3% of specimens and from the basilar artery in 10.7% of specimens. The SA branched off from the anterior inferior cerebellar artery when present. The origin of the LA was inferomedial to the vestibulocochlear nerve in most cases (71.4%), whereas the SA was usually lateral (70%). The distal portion of the LA was inferomedial to the vestibulocochlear nerve in 71.4% of cases. The distal portion of the SA was superolateral to the nerve in all cases (P < 0.00001). CONCLUSIONS: Knowledge of the different trajectory and anatomic relationship of the LA and the SA with the vestibulocochlear nerve is of paramount importance to differentiate them during surgery. The LA is usually inferomedial to the vestibulocochlear nerve at its distal and proximal aspects, whereas the SA usually originates lateral and ends superolateral to the nerve.

Benign notochordal cell tumor of the clivus with chordoma component: report of 2 cases.

Benign notochordal cell tumors (BNCTs) are considered to be benign intraosseous lesions of notochord origin; however, recent spine studies have suggested the possibility that some chordomas arise from BNCTs. Here, the authors describe two cases demonstrating histological features of BNCT and concomitant chordoma involving the clivus, which, to the best of the authors' knowledge, have not been previously documented at this anatomical site.An 18-year-old female presented with an incidentally discovered clival mass. Magnetic resonance imaging revealed a 2.8-cm nonenhancing lesion in the upper clivus that was T2 hyperintense and T1 hypointense. She underwent an uneventful endoscopic transsphenoidal resection. Histologically, the tumor demonstrated areas of classic chordoma and a distinct intraosseous BNCT component. The patient completed adjuvant radiation therapy. Follow-up showed no recurrence at 18 months.A 39-year-old male presented with an incidentally discovered 2.8-cm clival lesion. The nonenhancing mass was T2 hyperintense and T1 hypointense. Surgical removal of the lesion was performed through an endoscopic transsphenoidal approach. Histological analysis revealed areas of BNCT with typical features of chordoma. Follow-up did not demonstrate recurrence at 4 years.These cases document histologically concomitant BNCT and chordoma involving the clivus, suggesting that the BNCT component may be a precursor of chordoma.

Long-Term Safety and Performance of a Polymeric Clamplike Cranial Fixation System.

OBJECTIVE: After a craniotomy procedure to access the brain, neurosurgeons have several options to fix the bone flap to the skull. The aim of this study was to assess if a polymeric clamplike fixation system (Cranial LOOP) is a safe and reliable system that maintains over time an appropriate alignment of the bone flap. METHODS: This is an observational, retrospective, case series study of 60 patients who underwent a craniotomy and were subject to cranial bone flap fixation with the Cranial LOOP fixation system. Baseline clinical parameters, surgical variables, medical records, and all postoperative medical images available were reviewed to assess the bone flap alignment and potential adverse events. RESULTS: A total of 182 Cranial LOOPs were implanted in the 60 patients (56.01 ± 20.21 years, 55% women) included in the study. The cranial fixation system maintained a good bone flap alignment in 95% of the patients studied immediately after surgery and in up to 96.7% of them at the end of follow-up. No intraoperative complications were reported. An ulcer potentially related to a device was detected, which was solved without the need for device removal. No artifacts were observed in any of the 219 medical images analyzed. CONCLUSIONS: Cranial LOOP is a safe and reliable postoperative long-term cranial bone flap fixation system. This device can fix the bone flap after a wide range of craniotomy procedures, performed in multiple locations, and provides good bone flap alignment. Cranial LOOP does not interfere in patient follow-up through medical imaging.

Efficacy of Sphenopalatine Ganglion Radiofrequency in Refractory Chronic Cluster Headache.

BACKGROUND: In the literature, there are only short series of radiofrequency of the sphenopalatine ganglion (SPG) to treat chronic refractory cluster headache (CCHr) with variable results. Furthermore, there is no consensus on which methodology to use: radiofrequency ablation (RFA) or pulsed radiofrequency (PRF). METHODS: We conducted a prospective analysis of 37 patients with CCHr who underwent RFA or PRF of the SPG in our center between 2004 and 2015. RESULTS: The mean age of the patients was 40 years (range, 26-59 years). PRF was performed in 24 patients, and RFA was performed in 13 patients. A total of 5 patients (13.5%) experienced complete clinical relief of both pain and parasympathetic symptoms, 21 patients (56.8%) had partial and transient relief, and 11 patients (29.7%) did not improve. There was no evidence of significant superiority of one radiofrequency modality over the other (P = 0.48). There were no complications associated with the technique. The passage of time tended to decrease the efficacy of both techniques (P < 0.001). The mean follow-up was 68.1 months (range, 15-148 months). To our knowledge, this is the series with the largest number of patients and the longest follow-up period published in the literature. CONCLUSIONS: Radiofrequency of the SPG is a safe, fast, and partially effective method for the treatment of CCHr. Given its low rate of complications and its low economic cost, we think it should be one of the first invasive treatment options, prior to techniques with greater morbidity and mortality, such as neuromodulation.

Clinoidectomía anterior extradural en el manejo de meningiomas paraselares: análisis de 13 años de experiencia y revisión de la literatura / Extradural anterior clinoidectomy in the management of parasellar meningiomas: Analysis of 13 years of experience and literature review

Antecedentes y objetivo: La clinoidectomía anterior extradural (CaE) es una técnica microquirúrgica clave que facilita la resección de tumores localizados en la región paraselar. Actualmente, no existe consenso de la ejecución vía extradural o intradural, o evidencia científica que apoye su uso de manera rutinaria. El propósito de este artículo es exponer nuestra experiencia en la realización de la CaE como parte del manejo de los meningiomas paraselares. Materiales y métodos: Se realizó un análisis retrospectivo de las CaE empleadas en las resecciones de meningiomas paraselares realizadas en nuestro centro entre los años 2003 y 2015. Se registraron un total de 53 pacientes. Realizamos un análisis de nuestra serie de casos, enfocándonos en los resultados visuales, las tasas de resección y las complicaciones postoperatorias. A través de una revisión bibliográfica, se discuten las ventajas e inconvenientes de la CaE, consideraciones técnicas, comparación con la clinoidectomía anterior intradural e impacto visual. Resultados: El tipo de tumor más frecuente fueron los meningiomas de clinoides anterior (33,9%). Los síntomas iniciales más frecuentes fueron la disminución de la agudeza visual (45,3%) y la cefalea (22,6%). La resección tumoral total se consiguió en el 67,9% de los casos, siendo subtotal en el restante 32,1%. Con respecto a los déficits visuales, el 67,9% de los pacientes presentaron estabilidad clínica, el 22,6% mejoraron y el 9,4% experimentaron empeoramiento. El grado de resección tumoral no influyó de manera significativa en los resultados visuales posquirúrgicos, ni en la agudeza visual (p=0,71) ni en los déficits campimétricos (p=0,53). El 24,5% de los pacientes experimentaron paresia del iiipar transitoria y el 1,9%, permanente. La tasa de fístula de líquido cefalorraquídeo postoperatoria fue del 3,8%. La mortalidad fue del 0%. El periodo medio de seguimiento fue de 82,3 meses. Conclusiones: En nuestra experiencia, la CaE es una técnica segura que facilita la resección de los meningiomas localizados en la región paraselar, favorece la desvascularización temprana del tumor, reduce la necesidad de retracción del parénquima cerebral y podría ejercer un papel positivo en la preservación de la función visual y en la aparición de recidivas tumorales en la apófisis clinoides anterior (ACa)

Background and aim: The extradural anterior clinoidectomy (EAC) is a key microsurgical technique that facilitates the resection of tumors located in the parasellar region. There is currently no consensus regarding the execution of the procedure via extradural or intradural nor scientific evidence that supports its routine use. The purpose of this article is to expose our experience in performing EAC as part of the management of the parasellar meningiomas. Materials and methods: A retrospective analysis of the EAC for parasellar meningioma resection performed in our center between 2003 and 2015 was done. A total of 53 patients were recorded. We analized our series focusing on visual outcomes, resection rates and complications. Through an extensive bibliographic research, we discussed the advantages and disadvantages of the EAC, technical considerations, comparison with the intradural clinoidectomy and its visual impact. Results: The most frequent tumors were anterior clinoidal meningiomas (33.9%). The most common initial symptoms were decreased visual acuity (45.3%) and headache (22.6%). A gross total resection was achieved in 67.9%, being subtotal in the remaining 32.1%. Regarding the visual deficits 67.9% of the patients presented clinical stability, 22.6% improvement and 9.4% worsening. The degree of tumor resection did not significantly influence post-surgical visual outcomes, either visual acuity (P=.71) or campimetric alterations (P=.53). 24.5% of the patients experienced iiinerve transient paresis and 1.9% permanent. The postoperative cerebrospinal fluid leak rate was 3.8%. Mortality rate was 0%. The mean follow-up was 82.3 months. Conclusions: In our experience, EAC is a safe technique that facilitates the resection of the meningiomas located in the parasellar area, helps to achieve early tumor devascularization, reduces the need for retraction of the cerebral parenchyma and could play a positive role in the preservation of visual function and the appearance of tumor recurrences in the anterior clinoid process (ACP)

Extradural anterior clinoidectomy in the management of parasellar meningiomas: Analysis of 13 years of experience and literature review. / Clinoidectomía anterior extradural en el manejo de meningiomas paraselares: análisis de 13 años de experiencia y revisión de la literatura.

BACKGROUND AND AIM: The extradural anterior clinoidectomy (EAC) is a key microsurgical technique that facilitates the resection of tumors located in the parasellar region. There is currently no consensus regarding the execution of the procedure via extradural or intradural nor scientific evidence that supports its routine use. The purpose of this article is to expose our experience in performing EAC as part of the management of the parasellar meningiomas. MATERIALS AND METHODS: A retrospective analysis of the EAC for parasellar meningioma resection performed in our center between 2003 and 2015 was done. A total of 53 patients were recorded. We analized our series focusing on visual outcomes, resection rates and complications. Through an extensive bibliographic research, we discussed the advantages and disadvantages of the EAC, technical considerations, comparison with the intradural clinoidectomy and its visual impact. RESULTS: The most frequent tumors were anterior clinoidal meningiomas (33.9%). The most common initial symptoms were decreased visual acuity (45.3%) and headache (22.6%). A gross total resection was achieved in 67.9%, being subtotal in the remaining 32.1%. Regarding the visual deficits 67.9% of the patients presented clinical stability, 22.6% improvement and 9.4% worsening. The degree of tumor resection did not significantly influence post-surgical visual outcomes, either visual acuity (P=.71) or campimetric alterations (P=.53). 24.5% of the patients experienced iiinerve transient paresis and 1.9% permanent. The postoperative cerebrospinal fluid leak rate was 3.8%. Mortality rate was 0%. The mean follow-up was 82.3 months. CONCLUSIONS: In our experience, EAC is a safe technique that facilitates the resection of the meningiomas located in the parasellar area, helps to achieve early tumor devascularization, reduces the need for retraction of the cerebral parenchyma and could play a positive role in the preservation of visual function and the appearance of tumor recurrences in the anterior clinoid process (ACP).

Hemorragia subaracnoidea no aneurismática secundaria a panarteritis nodosa en edad pediátrica: caso clínico / Non-aneurysmal subarachnoid haemorrhage secondary to panarteritis nodosa at paediatric age: a case report

Introducción. La panarteritis nodosa es una vasculitis necrotizante que afecta de manera predominante a arterias de pequeño y mediano calibre de la piel y los órganos internos. La afectación neurológica sucede en un 25% de casos: los ictus isquémicos son relativamente frecuentes, pero las lesiones hemorrágicas resultan extremadamente raras. La hemorragia subaracnoidea es una expresión poco común de la enfermedad, asociada la inmensa mayoría de las veces a patología aneurismática. En nuestro conocimiento, sólo existe otro caso descrito de panarteritis nodosa con hemorragia subaracnoidea en la edad pediátrica sin patología aneurismática subyacente. Caso clínico. Niña de 7 años que presentó una hemorragia subaracnoidea no aneurismática con extensión intraparenquimatosa a los ganglios basales izquierdos. Previamente se había diagnosticado panarteritis nodosa y enfermedad protrombótica, en tratamiento con fármacos inmunodepresores y antiagregantes en ese momento. Conclusiones. Las características clínicas de la panarteritis nodosa, junto con el tratamiento antiagregante para prevenir la enfermedad tromboembólica en nuestra paciente, podrían haber predispuesto al evento hemorrágico en el proceso de la vasculitis cerebral. El manejo estándar de la hemorragia subaracnoidea es necesario en estos casos debido a que los aneurismas cerebrales son la causa más común de hemorragia subaracnoidea en un contexto de panarteritis nodosa

Introduction. Polyarteritis nodosa is a necrotizing vasculitis that mainly affects small and medium-sized arteries in skin and internal organs. Neurological involvement is reported in around 25% of cases: ischemic stroke is relatively common, but haemorrhagic lesions are extremely rare. Subarachnoid haemorrhage in polyarteritis nodosa is an uncommon expression of this disease, mostly associated with aneurism rupture. To the best of the authors’ knowledge, there is just one published case in pediatric age with polyarteritis nodosa and subarachnoid haemorrhage with no underlying aneurismal disease. Case report. A 7-year-old girl, who presented a non-aneurismal subarachnoid haemorrhage with intraparenchymal extension in the left basal ganglia. She was previously diagnosed with polyarteritis nodosa and prothrombotic condition, being under immunosuppressive and anti-platelet treatment at that moment. Conclusions. The clinical features of polyarteritis nodosa together with continued anti-platelet therapy to prevent thromboembolic disease in this patient could have predisposed to the haemorrhagic event in the process of cerebral vasculitis. Standard subarachnoid haemorrhage management is initially required in such cases as cerebral aneurysms are the most common cause of haemorrhage in the context of polyarteritis nodosa disease

Importancia de la mutación de la isocitrato deshidrogenasa tipo I como factor pronóstico en pacientes con glioblastomas y revisión de la literatura / Isocitrate dehydrogenase type I mutation as a prognostic factor in glioblastoma and a literature review

Objetivo: La respuesta al tratamiento y el pronóstico de los glioblastomas multiformes (GBM) puede ser distinta entre diferentes individuos. Este hecho está haciendo que cada vez estén cobrando más importancia los biomarcadores genéticos para intentar diferenciar distintos subtipos de GBM. Uno de los que está adquiriendo más importancia es la isocitrato deshidrogenasa (IDH1). El objetivo del trabajo es analizar la diferencias tanto clínicas como pronósticas de los GBM con y sin mutación de la IDH1. Materiales y método: Estudio retrospectivo de pacientes con GBM intervenidos entre los años 2007 y 2012. Los criterios de inclusión fueron: pacientes entre 18-85 años, sometidos por primera vez a cirugía, resección macroscópicamente completa, tratamiento coadyuvante con quimioterapia y radioterapia y Karnofsky status > 70. Resultados: N = 61 pacientes (36 varones/25 mujeres). Edad media de 62,3 años. En 14 pacientes (23%) se encontró la IDH1 mutada. La supervivencia media de los pacientes con mutación de la IDH1 (IDH1-m) fue de 23,6 meses respecto a los 11,9 meses que presentaban los de la IDH1 en estado natural (IDH1-wt) (p = 0,028). Existió una tendencia en los pacientes con IDH1-m a presentar la enfermedad en edades más tempranas 58,7 vs. 63,4 años, pero estas diferencias no fueron significativas. Conclusiones: Se deberían considerar los GBM con IDH1-m como una entidad distinta a los IDH1-wt. Se trata de dos subtipos de GBM con una historia natural y un pronóstico totalmente distinto por lo que se deberían clasificar en un futuro en función de la presencia de la mutación de la IDH1

Objective: Treatment response and prognosis in glioblastoma (GBM) tumours can differ among patients, highlighting the growing relevance of genetic biomarkers to differentiate glioblastoma sub-types. The biomarker isocitrate dehydrogenase (IDH1) is currently receiving considerable attention. The objective of this work was to analyse the clinical and prognostic differences between glioblastomas with and without the IDH1 mutation. Methods: A retrospective study was performed on patients with GBM who underwent surgery between 2007 and 2012. The inclusion criteria were: patient age between 18-85 years who underwent surgery for the first time with complete macroscopic resection, complete adjuvant treatment with chemotherapy and radiotherapy, and a Karnofsky performance score > 70. Results: A total of 61 patients (36 males/25 famales) were included and with a mean age of 62.3 years. An IDH1mutation was found in 14 patients (23%). Median survival in patients with the IDH1 mutation (IDH1-m)was 23.6 months compared with 11.9 months in those with the wild type IDH1 (IDH1-wt) (P = .028). Disease onset in IDH1-m patients tended to be at a younger age, 58.7 vs. 63.4 years, but this difference was not statistically significant. Conclusion: Glioblastomas with IDH1-m should be considered a different entity from the IDH1-wt, as their natural history and prognosis differ. In the near future we should be classified glioblastomas based on the presence of the IDH1 mutation

[Isocitrate dehydrogenase type I mutation as a prognostic factor in glioblastoma and a literature review]. / Importancia de la mutación de la isocitrato deshidrogenasa tipo I como factor pronóstico en pacientes con glioblastomas y revisión de la literatura.

OBJECTIVE: Treatment response and prognosis in glioblastoma (GBM) tumours can differ among patients, highlighting the growing relevance of genetic biomarkers to differentiate glioblastoma sub-types. The biomarker isocitrate dehydrogenase (IDH1) is currently receiving considerable attention. The objective of this work was to analyse the clinical and prognostic differences between glioblastomas with and without the IDH1 mutation. METHODS: A retrospective study was performed on patients with GBM who underwent surgery between 2007 and 2012. The inclusion criteria were: patient age between 18-85 years who underwent surgery for the first time with complete macroscopic resection, complete adjuvant treatment with chemotherapy and radiotherapy, and a Karnofsky performance score>70. RESULTS: A total of 61 patients (36 males/25 famales) were included and with a mean age of 62.3 years. An IDH1mutation was found in 14 patients (23%). Median survival in patients with the IDH1 mutation (IDH1-m) was 23.6 months compared with 11.9 months in those with the wild type IDH1 (IDH1-wt) (P=.028). Disease onset in IDH1-m patients tended to be at a younger age, 58.7 vs. 63.4 years, but this difference was not statistically significant. CONCLUSION: Glioblastomas with IDH1-m should be considered a different entity from the IDH1-wt, as their natural history and prognosis differ. In the near future we should be classified glioblastomas based on the presence of the IDH1 mutation.