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Background: Cavernous malformation (CM) is a vascular malformation found in the encephalic parenchyma, spinal cord, nerve roots, and extraneural tissue. CM in the trigeminal distribution is exquisitely uncommon and its biological behavior not completely understood. The clinical picture might be diverse, depending on the affected sector of the trigeminal architecture, and literature debating its pathobiology is scarce., Case Description: We describe a case of 56-year-old woman who presented with left trigeminal neuralgia and a rapidly growing cavernous malformation of the entire distribution of the fifth nerve. The clinical picture evolved to a progressive gait ataxia and follow-up neuroimaging showed a large intracranial mass leading to a brainstem compression. After microsurgical resection, the mass proved to be a typical CM of the trigeminal root., Conclusion: We present an uncommonly aggressive progression of a CM of the trigeminal root, Gasserian ganglion, and cavernous sinus evolving to severe brainstem compression. The documentation of this unique case as well as its management is presented is discussed., (Copyright © 2016 Elsevier Inc. All rights reserved.)

The anterior interhemispheric approach is a workhorse for treatment of lesions in the third ventricle. In this case, we demonstrate the utility of this approach for resecting a complex third ventricular cavernous malformation. We discuss patient positioning, optimal location of the craniotomy, and surgical resection techniques for safe removal of these lesions. We also demonstrate the importance of gravity retraction using the falx to prevent injury to the dominant frontal lobe. The video can be found here: https://youtu.be/38woc28er7M .

Background: There is a relative paucity of long-term follow-up of treatment of brainstem cavernous angiomas. This has led to uncertainty and a lack of consensus regarding optimum therapy, especially radiosurgery., Methods: Report of a single case with a 24-year follow-up., Results: This patient was minimally symptomatic before her radiosurgery procedure. She developed an acute complication, from which she incompletely recovered. Two decades later she has become more symptomatic, with new deficits, and magnetic resonance imagings disclose progressive brainstem atrophy., Conclusions: Long-term follow-up of patients treated with radiosurgery is important., (Copyright © 2016 Elsevier Inc. All rights reserved.)

We report the case of a 66-year-old man with adult-onset seizures and multiple cerebral cavernous malformations who developed numerous eruptive cutaneous angiokeratomas on the legs, scrotum, abdomen, and back as well as lobular and cavernous hemangiomas on the arms. Genetic analysis demonstrated a mutation in the KRIT1, ankyrin repeat containing gene (also known as CCM1).

Object: Cerebellar cavernous malformations (CCMs) have not been specifically described in the pediatric age group. Authors of this study, after considering the published literature, describe the characteristic clinical, radiological, and surgical features of CCM in children. METHOSDS: Patients younger than 18 years of age who were known to have CCM and had undergone surgery between 1992 and 2014 at the authors' institution were reviewed. Pediatric CCM cases reported in the literature (case reports and cases included in series on CMs in the pediatric age group) were also analyzed for specific features of this entity., Results: Four male patients and 1 female patient (2.5-14 years of age) with CCM presented acutely with severe headache followed by cerebellar dysfunction. In all patients, neuroimaging (cranial CT and MRI) demonstrated hemorrhagic cerebellar lesions with heterogeneous T1 and T2 signal intensities and hyperintense blooming on susceptibility-weighted imaging. The lesions reached large sizes exhibiting spherical, cystic, and often "pseudotumoral" morphology. In 3 patients, developmental venous anomalies (DVAs) were found. In 4 of the 5 patients, the CCMs and hematomas were totally removed. All patients had a clinically excellent functional outcome without surgical complication and with complete resolution of their presenting symptoms., Conclusions: Cerebellar CMs occur in all pediatric age groups and display characteristic clinical and imaging features. In children, CCMs reach large sizes and can result in massive hemorrhage, often leading to a possible diagnosis of hemorrhage into a tumor. An associated DVA is quite common. Surgery is a safe and efficient treatment option with excellent outcomes in patients.

Cerebral cavernous malformations (CCMs) are vascular malformations of the brain and brainstem that arise via a number of different mechanisms and can result in non-specific presentations. Therefore, medical imaging is essential in the diagnosis of these lesions and important to guide their clinical or surgical management. MRI is the modality of choice with newer protocols, such as susceptibility weighted imaging, playing an increasingly important role in the detection of CCMs. In this review we will discuss lesion structure, its proposed origins, and common lesion presentations and complications, before covering their expected appearances with different imaging modalities. We conclude with a discussion of insights about lesion behaviour acquired from advanced imaging techniques and provide a general approach to characterizing and diagnosing CCM lesions with neuroimaging.

Cerebral cavernous malformations (CCMs) are vascular abnormalities that may cause seizures, headaches, intracerebral hemorrhages, and focal neurological deficits; they can also be clinically silent and occur as a sporadic or an autosomal dominant condition. Three genes have been identified as causing familial CCM: KRIT1/CCM1, MGC4607/CCM2, and PDCD10/CCM3, mapping, respectively, on chromosomes 7q, 7p, and 3q. Here, we report an Italian family affected by CCM due to a MGC4607 gene mutation, on exon 4. All the affected subjects suffered from seizures, and some of them underwent surgery for removal of a cavernous angioma. Brain MRI showed multiple lesions consistent with CCMs in all patients. Spinal and cutaneous cavernous angiomas were present too. This report underlines the need for a careful interdisciplinarity among neurologists, neuroradiologists, neurosurgeons, geneticists, ophthalmologists, and dermatologists for a total evaluation of the different manifestations of familial CCM. This points out that only referral centers are organized to offer a multidisciplinary management of this disease.

Objectives: In this study, we investigated whether visualization of the pyramidal tract and intraoperative MRI combined with functional navigation was helpful in the resection of paraventricular or centrum ovale cavernous hemangioma in children., Methods: Twelve patients with cavernous hemangioma located in the paraventricular area or in the centrum ovale adjacent to the pyramidal tract were prospectively enrolled in the study. The pyramidal tract of all patients was visualized preoperatively, and all patients underwent tailored craniotomy with white matter trajectory to resect the lesion, with the help of intraoperative MRI and microscope-based functional neuronavigation., Results: In our study, of the total of 12 patients (nine males and three females), five patients had lesions on the left side, and seven had lesions located in the right hemisphere. The lesion volume varied from 0.2 to 11.45 cm(3). In seven cases, the distance of the lesion from the pyramidal tract was 0-5 mm (the 0-5 mm group), and five cases were in the 5-10 mm group. The 3D visualization of the lesion and the pyramidal tract helped the surgeon design the optimal surgical approach and trajectory. Intraoperative functional neuronavigation allowed them to obtain access to the lesion accurately and precisely. All lesions had been removed totally at the end of the surgery. Compared with the preoperative level, muscle strength at 2 weeks had decreased in six cases, was unchanged in four cases, and improved in two cases; at 3 months, it was improved in five cases, unchanged in six cases, and decreased in one case., Conclusions: Pyramidal tract visualization and intraoperative MRI combined with functional neuronavigation can aid in safe removal of paraventricular or centrum ovale cavernous hemangioma involving the pyramidal tract.

The objective was to determine if there was seasonal variation in the month of symptom onset in patients with intracerebral cavernous malformations (ICM). Patients seen at our institution between 1989 and 1999 with ICM identified from a radiologic database were included. Demographic data and symptoms at onset were abstracted. Symptomatic patients in this ICM database were included if the month of symptom onset was known. Comparison of proportions of patients presenting in fall and winter and during flu season were performed with the chi squared test and Fisher's exact test with significance at p<0.05. Of 292 patients identified, 179 patients had symptomatic ICM. Of these, 135 patients with symptomatic ICM were identified for whom the exact month of symptom onset was known. Eighty-seven (64.4%) presented with intracerebral hemorrhage (ICH) or focal neurologic deficit (FND) and 48 (35.6%) due to seizures. When assessing all 135 patients, 75 (55.5%) presented in the fall and winter compared to the spring and summer (p=0.1962). Eighty-three (61.4%) patients presented during flu season (p=0.0074). When assessing only those initially presenting with ICH or FND, 53 (60.9%) patients presented in the fall and winter compared to spring and summer (p=0.041) and 54 (62.8%) presented during flu season (p=0.017). Patients with recurrent ICH more commonly presented in the fall and winter compared to summer and spring and during flu season. Patients with ICM who presented with FND or ICH or had recurrent ICH tended to present in the fall and winter or during flu season., (Copyright © 2015 Elsevier Ltd. All rights reserved.)

Object: The objective of this study was to characterize the clinical features of radiation-induced cavernous malformations (RICMs)., Methods: The authors retrospectively reviewed the clinical and radiological characteristics of patients with RICMs. The features of these RICMs were then compared with features of nonradiation cavernous malformations (CMs) in 270 patients., Results: Thirty-two patients with RICMs were identified (56.2% men), with a median age of 31.1 years at RICM diagnosis. The median latency from radiation treatment to RICM diagnosis was 12.0 years (interquartile range 5.0-19.6 years). RICMs were always within the previous radiation port. RICMs were symptomatic at diagnosis in 46.9%, and were associated with symptomatic intracranial hemorrhage at any time in 43.8%. Older age at the time of radiation treatment and higher radiation dose were associated with shorter latency. RICMs tended to be diagnosed at a younger age than nonradiation CMs (median 31.1 vs 42.4 years, respectively; p = 0.054) but were significantly less likely to be symptomatic at the time of diagnosis (46.9% vs 65.8%, respectively; p = 0.036). RICMs were more likely to be multiple CMs than nonradiation CMs (p = 0.0002). Prospectively, the risk of symptomatic hemorrhage was 4.2% for RICMs and 2.3% for nonradiation CMs per person-year (p = 0.556). In the absence of symptoms at presentation, the risk of hemorrhage for RICMs was higher than for nonradiation CMs (4.2% vs 0.35%, respectively; p = 0.118)., Conclusions: In this patient population, RICMs occurred within the radiation port approximately 12 years after radiation treatment. Compared with nonradiation CMs, RICMs were more likely to occur as multiple CMs, to present at a younger age, and were at least as likely to cause symptomatic hemorrhage.

Brainstem cavernous malformations can cause devastating neurologic disability when they hemorrhage, which occurs at a higher rate in the brainstem than in other locations. Traditional access to these lesions requires a large craniotomy with extensive exposure and manipulation of vital structures. We present a case series of patients who underwent surgical resection of brainstem cavernous malformations using minimally invasive approaches at our institution from January 2012 to August 2014, all of whom had experienced at least one hemorrhage prior to presentation. Approach choice was determined by location of the cavernous malformation in relation to the brainstem surface. Resection occurred through our described standardized method. Postoperatively, there were three instances of transient neurologic symptoms, all of which resolved at time of last follow-up. All eight patients experienced neurologic improvement after surgery, with four patients showing no deficits at last follow-up. Approach selection rationale and technical nuances are presented on a case-by-case basis. With carefully planned keyhole approaches to cavernous malformations presenting to the brainstem surface, excellent results may be achieved without the necessity of larger conventional craniotomies. We believe the nuances presented may be of use to others in the surgical treatment of these lesions., (Copyright © 2014 Elsevier Ltd. All rights reserved.)

Background: Neurofibromatosis 1 (NF1) belongs to the autosomal dominant neurocutaneous disorders' group, which mainly includes NF1 and NF2, tuberous sclerosis, von Hippel-Lindau disease and Cerebral Cavernous Malformations (CCMs). NF1 has a major impact on the nervous system, eye, skin, bone or cardiovascular system. Cerebrovascular lesions have been reported in NF1 including aneurysm, pseudoaneurysm, arteriovenous malformations, vascular stenosis or occlusion and Moya moya syndrome., Objective: To report a case of an NF1 patient with multiple CCMs., Observation: A 47-year-old man with café-au-lait skin lesions, countless cutaneous neurofibromas, short stature and scoliosis was admitted for progressive spinal cord compression due to histologically proven neurofibroma. Systematic cerebral MRI screening including gradient echo sequences showed multiple asymptomatic CCMs. Screening of CCM1, CCM2 and CCM3 genes was negative while a deleterious frameshift mutation was identified in NF1 gene., Conclusion: While single CCM can occur in NF1 patients following radiation exposure, they are only rarely reported in non-irradiated NF1 brain. Even if it could be a fortuitous association, plausible links and explanations exist. If cerebral MRI can be systematic in NF1 to detect asymptomatic gliomas, used protocols in neuroradiology do not usually include gradient echo sequences, the most sensitive test for CCM detection, leading possibly to failure to detect these vascular lesions. More reports having this combination and further investigations of NF1 families will certainly provide a better understanding of links between these 2 phakomatoses, as recently reported with "multiple meningiomas" phenotype associated with multiple CCMs in patients with CCM3 gene mutations or café-au-lait skin lesions in CCM1 mutation carriers., (Copyright © 2015. Published by Elsevier B.V.)

Object: Resection of brainstem cavernous malformations (BSCMs) may reduce the risk of stepwise neurological deterioration secondary to hemorrhage, but the morbidity of surgery remains high. Diffusion tensor imaging (DTI) and diffusion tensor tractography (DTT) are neuroimaging techniques that may assist in the complex surgical planning necessary for these lesions. The authors evaluate the utility of preoperative DTI and DTT in the surgical management of BSCMs and their correlation with functional outcome., Methods: A retrospective review was conducted to identify patients who underwent resection of a BSCM between 2007 and 2012. All patients had preoperative DTI/DTT studies and a minimum of 6 months of clinical and radiographic follow-up. Five major fiber tracts were evaluated preoperatively using the DTI/DTT protocol: 1) corticospinal tract, 2) medial lemniscus and medial longitudinal fasciculus, 3) inferior cerebellar peduncle, 4) middle cerebellar peduncle, and 5) superior cerebellar peduncle. Scores were applied according to the degree of distortion seen, and the sum of scores was used for analysis. Functional outcomes were measured at hospital admission, discharge, and last clinic visit using modified Rankin Scale (mRS) scores., Results: Eleven patients who underwent resection of a BSCM and preoperative DTI were identified. The mean age at presentation was 49 years, with a male-to-female ratio of 1.75:1. Cranial nerve deficit was the most common presenting symptom (81.8%), followed by cerebellar signs or gait/balance difficulties (54.5%) and hemibody anesthesia (27.2%). The majority of the lesions were located within the pons (54.5%). The mean diameter and estimated volume of lesions were 1.21 cm and 1.93 cm(3), respectively. Using DTI and DTT, 9 patients (82%) were found to have involvement of 2 or more major fiber tracts; the corticospinal tract and medial lemniscus/medial longitudinal fasciculus were the most commonly affected. In 2 patients with BSCMs without pial presentation, DTI/DTT findings were important in the selection of the surgical approach. In 2 other patients, the results from preoperative DTI/DTT were important for selection of brainstem entry zones. All 11 patients underwent gross-total resection of their BSCMs. After a mean postoperative follow-up duration of 32.04 months, all 11 patients had excellent or good outcome (mRS Score 0-3) at the time of last outpatient clinic evaluation. DTI score did not correlate with long-term outcome., Conclusions: Preoperative DTI and DTT should be considered in the resection of symptomatic BSCMs. These imaging studies may influence the selection of surgical approach or brainstem entry zones, especially in deep-seated lesions without pial or ependymal presentation. DTI/DTT findings may allow for more aggressive management of lesions previously considered surgically inaccessible. Preoperative DTI/DTT changes do not appear to correlate with functional postoperative outcome in long-term follow-up.

Medullary hemorrhage is rare, and the causative role of hypertension still remains controversial. Cavernous angioma and other vascular malformations have been reported to cause medullary hemorrhage. A 53-year-old man was admitted to our hospital for vertigo. Medullary hemorrhage and multiple small hypointense lesions were detected on T2-star weighted magnetic resonance imaging (T2*W MRI). One and four months later, the appearance of new lesions confirmed the diagnosis of cerebral cavernous angioma. Cavernous angioma is often characterized by de novo appearance/progression on MRI. A follow-up MRI is required to diagnose cavernous angioma in patients with medullary hemorrhage.

Spinal cord cavernous malformations (scCM) are rare vascular malformation representing approximately 5-12% of all intramedullary lesions. The authors reviewed over 20 publications available and have consolidated existing knowledge on the pathology, epidemiology, clinical and radiological characteristics scCM. scCM management and treatment methods have been outlined concisely and achieved outcomes have been presented. The average age of onset is 42 years without sexual predilections. scCM statistically most commonly occur in the thoracic region--57%, in 9% of patients familiar prevalence is present, in 27% of patients the co-existence of intracerebral vascular malformation is diagnosed. The most common clinical symptoms are the sensory and motor deficits (respectively 65% and 63%). The course of the disease in 54% of cases, is a progressive, slow neurologic deterioration, although it is also observed a rapid onset associated with cavernoma hemorrhage. The risk of hemorrhage is estimated at 2.5% annually. scCM that are located intramedullary, are asymptomatic should be the subject to observation. Cavernomas that are symptomatic, having contact with the surface of spinal cord or exophytic should be subject to surgical treatment that is intended to complete removal. Results of surgical treatment are satisfactory, in about 60% of patients clinical improvement has been observed.

Suprasellar and third ventricular region cavernous malformations originating from the floor of the third ventricle are extremely rare. We report a case of third ventricular cavernous malformation arising from the ventricle floor in a 24-year-old woman who presented with short-term memory loss and disorientation. Computed tomography revealed a suprasellar mass with calcification in the posterior chiasmatic region. T2-weighted magnetic resonance imaging revealed a mass with heterogeneous intensity and without hydrocephalus. The mass was slightly enhanced subsequent to gadolinium infusion. Using a basal interhemispheric translamina terminalis approach and a neuroendoscope, we confirmed that the tumor was located at the floor of the third ventricle and removed it. Histopathological examination confirmed the diagnosis of cavernous malformation. The postoperative course was uneventful, but the patient's short-term memory loss persisted. Despite its rarity, cavernous malformation should be suspected when a tumor is detected in the vicinity of the third ventricle floor. It is treatable through surgical resection., (Copyright © 2015 Polish Neurological Society. Published by Elsevier Urban & Partner Sp. z o.o. All rights reserved.)

Cerebral cavernous malformations are the most common vascular malformations and can be found in many locations in the brain. If left untreated, cavernomas may lead to intracerebral hemorrhage, seizures, focal neurological deficits, or headaches. As they are angiographically occult, their diagnosis relies on various MR imaging techniques, which detect different characteristics of the lesions as well as aiding in planning the surgical treatment. The clinical presentation and the location of the lesion are the most important factors involved in determining the optimal course of treatment of cavernomas. We concisely review the literature and discuss the advantages and limitations of each of the three available methods of treatment--microsurgical resection, stereotactic radiosurgery, and conservative management--depending on the lesion characteristics.

Cavernous malformations (CMs) are angiographically-occult clusters of dilated sinusoidal channels that may present clinically with seizures, focal neurological deficits and/or hemorrhage. Across natural history studies, the annual hemorrhage rate ranged from 1.6-3.1% per patient-year, decreasing to 0.08-0.2% per patient-year for incidental CMs and to 0.3-0.6% for the collective group of unruptured CMs. Prior hemorrhage is a significant risk factor for subsequent CM hemorrhage. Hemorrhage clustering, particularly within the first 2 years, is an established phenomenon that may confound results of natural history studies evaluating the rate of rehemorrhage. Indeed, rehemorrhage rates for hemorrhagic CMs range from 4.5-22.9% in the literature. Surgical resection is the gold standard treatment for surgically-accessible, symptomatic CMs. Incidental CMs or minimally symptomatic, surgically inaccessible eloquent lesions may be considered for observation. Stereotactic radiosurgery is a controversial treatment approach of consideration only for cases of highly aggressive, surgically inaccessible CMs.

Cavernous malformations are classified as a group of vascular malformations of the central nervous system. Conservative treatment of brainstem cavernomas is accompanied with poor outcome. Surgery ofbrainstem cavernomas still poses a challenge due to the high risk of neurological damage and respectable morbidity. We report a case of complete neurological recovery in a 24-year-old female patient with mesencephalic cavernoma treated surgically. This case highlights that careful microsurgical treatment with the goal of complete cavernoma excision remains the treatment of choice in cases with de novo or recurrent hemorrhage. Intraoperative neurophysiologic monitoring should be used as the gold standard during brainstem cavernoma operations in order to avoid nuclear and long tract damages.

Hyperphagia and obesity have been reported following damage to the hypothalamus in humans. Other brain sites are also postulated to be involved in the control of food intake and body weight regulation, such as the amygdala and brainstem. The brainstem, however, is thought to primarily integrate short-term meal-related signals but not affect long-term alterations in body weight, which is controlled by higher centers. The objective of this study was to identify structural pathways damaged in a patient with a brainstem cavernoma who experienced sudden onset of hyperphagia and >50 kg weight gain in <1 year following surgical drainage via a midline suboccipital craniotomy. Diffusion tensor imaging revealed loss of nerve fiber connections between her brainstem, hypothalamus and higher brain centers with preservation of motor tracks. Imaging and endocrine testing confirmed normal hypothalamic structure and function. Gastric bypass surgery restored normal appetite and body weight to baseline. This is the first report of 'brainstem obesity' and adds to the brain regions that can determine the long-term body weight set point in humans.

Background Context: Intradural extramedullary (IDEM) cavernomas are rare vascular malformations. They are well-circumscribed dark berry-like lesions with a histologic appearance of sinusoidal vascular channels. Neurofibromas are the most common IDEM tumors, originating from all nerve elements and leading to firm enlargement of the affected nerve root. These lesions are completely different; however, they may involve the spinal nerve roots or the major nerve trunks. Any similarities in clinical findings are based on different pathophysiology., Purpose: To present a rare resemblance of an IDEM cavernoma to a neurofibroma., Study Design: This is a case report with review of the literature focused on the differential diagnosis., Methods: A 79-year-old patient presented with acute sensorimotor disturbance from L2-S1 levels. The investigations showed an L2-L3 lesion occupying the canal. Findings resembled a neurofibroma and a surgical resection was decided., Results: The complete surgical resection revealed a vascular lesion originating from a nerve root. The histology confirmed an IDEM cavernoma. This is a unique case as such a clinical resemblance and a macroscopical appearance has not been reported for an IDEM cavernoma as yet. The patient showed full postoperative recovery from his initial symptoms., Conclusions: Intradural extramedullary cavernoma is a rare cause of compression to spinal cord or nerve roots. Its manifestation characteristics are well defined and should always be part of the differential diagnosis. Intraoperative findings aid the diagnosis in nontypical cases before the final histology. The nontraumatic and nerve tissue sparing surgical resection warrants optimal postoperative results and excellent prognosis., (Copyright © 2014 Elsevier Inc. All rights reserved.)

Cerebral cavernomatous malformations (CCM) are a subgroup of low-pressure cerebral vascular malformation. They have an estimated prevalence of 0,1-4% of the population and account for 8-15% of all cerebral vascular malformations. Depending on their size and localization (supratentorial or infratentorial, brain stem), CCM may present with focal bleedings with or without neurological deficits, as epileptic seizures, or are diagnosed incidentally by MRI performed for unspecific symptoms (e.g. headache, vertigo), especially in younger patients (mostly in 2nd or 3rd decade). The decision-making in patients with CCM can be classified into four categories: surgical or conservative treatment of symptomatic or incidental lesions. The decision regarding the optimal therapy in the individual patient must be made in the knowledge of the natural history (risk for bleeding or epilepsy) on the one hand, and the surgical possibilities (accessibility of the lesion) including their estimated outcome on the other hand. Therefore, careful patient selection and appropriate experience of the centre is warranted in the management of CCM.

Objectives: Cranial nerves (CN) cavernous malformations (CMs) are lesions that are isolated from the CNs. The authors present three cases of CN CMs, for which MR was demonstrated to be critical for management, and surgical resection produced good outcomes for the patients. Surgical removal is the recommended course of action to restore or preserve neurological function and to eliminate the risk of future haemorrhage. However, the anatomical location and the complexity of nearby neural structures can make these lesions difficult to access and remove. In this study, the authors review the literature of reported cases of CN CMs to analyse the clinical and radiographic presentations, surgical approaches and neurological outcomes., Patients and Methods: A MEDLINE/Pub Med search was performed and revealed 86 cases of CN CMs. The authors report three additional cases in this study for a total of 89 cases. CMs affecting the optic nerve (CN II), oculomotor nerve (CN III), facial/vestibule-cochlear nerves (CN VII, CN VIII) have been described. The records of three patients were reviewed with respect to the lesion locations, symptoms, surgical approaches and therapeutic considerations. Clinical and radiological follow-up results are reported. Three patients (2 females, 1 male; age range 21-37 year) presented with three CN lesions. One lesion involved CN III, one lesion involved CN VII-CN VIII, and one involved CN II. The patient with the CN III lesion had a one-month history of mild right ptosis and diplopia. The patient with the CN VII-CN VIII lesion exhibited acute hearing loss and on the left and left facial paresis. The patient with the opticchiasmatic lesion presented with acute visual deterioration on the right and a left temporal field deficit in the left eye. Pterional and orbitozygomatic craniotomies were performed for the CN III lesion and the CN II lesion, and retrosigmoid craniotomy was performed for the cerebello-pontine angle lesion., Results: All patients experienced symptom improvement after surgery. On MR follow-up, recurrence was excluded in all patients., Conclusions: CN CMs present with specific symptoms and require complex surgical techniques for resection. These lesions are frequently symptomatic, because of the complexity of the origin tissue. Symptomatic CN CMs should be resected microsurgically and completely when possible to prevent further losses of nerve function, improve function, avoid recurrence, and to eliminate the risk of future haemorrhages. The authors discuss the therapeutic options and the radiological features of these infrequent localisation of CMs. Specifically, the authors focus on the role of magnetic resonance imaging in the identification of these rare lesions., (Copyright © 2014 Elsevier B.V. All rights reserved.)

The incidence of neurologic disorders in the postoperative period of cardiac surgery is high and usually due to a combination of pre- and intraoperative factors. We present 2 patients with brain tumors diagnosed in the immediate postoperative period after sudden onset of neurologic dysfunction. Image studies yielded clinically useful information in these 2 cases., (Copyright © 2013 Sociedad Española de Anestesiología, Reanimación y Terapéutica del Dolor. Publicado por Elsevier España, S.L.U. All rights reserved.)

Background: The effectiveness of stereotactic radiosurgery (SRS) for cavernous malformation (CM) has not been fully assessed. Consequently, observation is usually recommended when a bleeding CM is initially discovered. Recurrent bleeding occurs with CMs, and these repeat hemorrhages can result in additional morbidity., Methods: From 1992 to 2011, 49 patients with brainstem CMs were treated with Gamma Knife radiosurgery (GKS). We classified patients into two groups: Group A (n = 31), patients who underwent GKS for a CM following a single symptomatic bleed, and group B (n = 18), patients who underwent GKS for a CM following two or more symptomatic bleeds. The mean marginal dose of radiation was 13.1 Gy (range 9.0-16.8 Gy): 12.8 Gy in group A and 13.7 Gy in group B. The mean follow-up period was 64.0 months (range 1-171 months)., Results: In group A, the annual hemorrhage rate (AHR) following GKS was 7.06 % within the first 2 years and 2.03 % after 2 years. In group B, four patients (22.2 %) developed new or worsening neurologic deterioration as a result of repeat hemorrhages. In group B, the AHR was 38.36 % prior to GKS, 9.84 % within the first two years, and 1.50 % after two years. There was no statistically significant difference in the AHRs at each follow-up period after GKS between the two groups. Adverse radiation effects (AREs) developed in a total of four patients (8.2 %); among them, one patient (2.0 %) developed a permanent case of diplopia. No mortality occurred in this series., Conclusion: In this study, GKS was demonstrated to be a safe and effective alternative treatment for brain stem CMs that resulted in a reduction in the AHR. Consequently, we suggest that even CM patients who have suffered only a single bleed should not be contraindicated for SRS.

Cerebral cavernous malformations, also known as cavernous angioma or cavernoma, are a type of vascular disorder. They consist of abnormally large vascular cavities or sinusoid channels of varying size. The majority of cavernous malformations in the brain are small and do not always present with symptoms. A minority of large cavernous malformations, known as giant cavernous malformations (GCM), can cause neurological symptoms (such as headaches, focal neurologic deficits and seizures), which are probably related to hemorrhage and mass effect. GCM grow steadily in size over time, due to repetitive episodes of bleeding. The purpose of this paper is to document two case reports of patients with GCM, illustrate the radiological appearance, discuss the neurosurgical consequences, and to provide a literature analysis.

Background: Melanoma lesions in the brainstem can be difficult to distinguish radiographically and clinically from cavernous malformations. However, the treatment modalities and clinical course of these two diseases differ considerably. We report two cases of melanoma presenting as brainstem hemorrhages., Case Description: A 69-year-old male was found to have a hemorrhagic lesion of the right dorsal midbrain. After a repeat hemorrhage, the lesion was resected and found to be hyperchromatic. Nonetheless, the patient suffered rebleeding and died 3 months later. A 62-year-old female was similarly found to have an acute pontine hemorrhage. After resection of the lesion, she underwent whole-brain radiation therapy but ultimately died 5.5 months later. The histopathology of both lesions was consistent with melanoma., Conclusions: Melanoma in the brainstem can mimic cavernous malformations. While management of these lesions includes stereotactic radiosurgery, whole-brain radiation, and surgical resection, metastatic brainstem melanoma follows an aggressive clinical course with a poor prognosis., (Copyright © 2014 Elsevier Masson SAS. All rights reserved.)

Developments in magnetic resonance imaging (MRI) techniques have dramatically increased the detection of cerebral cavernous malformations (CCMs). Conservative treatment is often recommended for asymptomatic cases. However, CCMs occasionally harbor malignant gliomas. Here, we describe a rare case of multiple probable CCMs and an anaplastic oligodendroglioma (AO) showing vasculogenic mimicry (VM) and discuss the potential pitfalls of conservative treatments. A 42-year-old otherwise healthy woman presented with generalized seizures. Magnetic resonance imaging (MRI) revealed multiple parenchymal hypointensities, particularly in the right frontal lobe, with hyperintensity on fluid-attenuated inversion recovery (FLAIR) images. The patient was diagnosed with multiple probable CCMs, and conservative treatment was administered. However, follow-up MRI showed a slightly enlarged hyperintense area in the right frontal lobe. The patient then underwent surgery; histological diagnosis was CCMs and AO with VM. The patient subsequently underwent radiotherapy and chemotherapy. No neurological deficits or tumor recurrence were evident 21 months after surgery. We present this rare case and emphasize the possibility of the coexistence of malignant gliomas with CCMs. Close observation with MRI is essential in cases of multiple probable CCMs, and a histological confirmation should be considered in cases showing any enlargement of hyperintensity on FLAIR images.

The authors report a rare case of a huge hypervascular tentorial cavernous angioma treated with preoperative endovascular embolization, followed by successful gross-total removal. A 15-year-old girl presented with scintillation, diplopia, and papilledema. Computed tomography and MRI studies revealed a huge irregularly shaped tumor located in the right occipital and suboccipital regions. The tumor, which had both intra- and extradural components, showed marked enhancement and invasion of the overlying occipital bone. Angiography revealed marked tumor stain, with blood supply mainly from a large branch of the left posterior meningeal artery. Therefore, this lesion was diagnosed as a tentorium-based extraaxial tumor. For differential diagnosis, meningioma, hemangiopericytoma, and malignant skull tumor were considered. Tumor feeders were endovascularly embolized with particles of polyvinyl alcohol. On the following day, the tumor was safely gross totally removed with minimum blood loss. Histopathological examination confirmed the diagnosis of cavernous angioma. To date, there have been no reports of tentorium-based cavernous angiomas endovascularly embolized preoperatively. A tentorial cavernous angioma is most likely to show massive intraoperative bleeding. Therefore, preoperative embolization appears to be quite useful for safe maximum resection. Hence, the authors assert that the differential diagnosis of tentorium-based tumors should include tentorial cavernous angioma, for which preoperative endovascular embolization should be considered.

Cavernomas are low-flow vascular lesions affecting approximately 0.5% of the population. Historically these have been considered congenital lesions, but numerous reports have demonstrated de novo formation. The phenomenon is well documented in patients with the familial disease form and after cranial radiotherapy, but outside of these circumstances there is scant evidence as to the potential etiology. The authors present a 5 year MRI series of a 56-year-old woman with no known risk factors demonstrating cavernoma formation and growth from previously normal brain. The patient was consistently asymptomatic during follow-up. Given the history and imaging findings, we propose that cavernomas can arise directly from angiogenic proliferation secondary to microhemorrhage from unrelated causes., (Copyright © 2013 Elsevier Ltd. All rights reserved.)

Object: The aim of this study was to determine the prospective hemorrhage rate in a group of retrospectively identified patients in whom symptoms had an unclear relationship to an intracerebral cavernous malformation (ICM) or the malformation itself was an incidental finding., Methods: Patients with incidentally discovered ICMs diagnosed between 1989 and 1999 were identified from a previously published cohort. Those with ICMs having an unclear relationship with existing symptoms were also eligible for analysis. Updated clinical and radiographic data pertaining to symptomatic intracerebral hemorrhage related to the ICM or new seizures were obtained through medical chart review and mail survey. In select patients, phone calls were made and death certificates were obtained when possible. The prospective hemorrhage rate was calculated as the number of prospective hemorrhages divided by the number of patient-years of follow-up., Results: There were 1311 patient-years of follow-up among the 107 patients (49.5% male; mean age at diagnosis 52 years) eligible for this study. Forty-four patients died in the follow-up period, and the cause of death could be determined in 34 (77%). Two patients had a prospective hemorrhage, which was definitively related to the ICM in only one. Thus, the definitive prospective bleed rate was 0.08% per patient-year. No new seizures developed in any of the patients during the follow-up period., Conclusions: The risk of prospective hemorrhage in patients presenting asymptomatically with ICM is very low. This information can be useful in managing such patients and may be most applicable to those with a single ICM.

Background: Hereditary syndromes frequently need the cooperation of different specialties to increase diagnostic competence. Multiple cutaneous and uterine leiomyomatosis syndrome is a rare autosomal dominant disorder caused by the mutations of the fumarate hydratase gene, demonstrated in 80 to 100 percent of affected individuals. This can be linked to an increased risk of renal cancer in both sexes. The skin involvement is described to highlight the diagnostic role of the cutaneous counterpart in identifying this rare syndrome., Case Presentation: A 37-year-old woman suffering from several uterine fibroids presented multiple, painful, papulo-nodules on her left subscapular side, both forearms and legs. The patient underwent surgery on six lesions: five were leiomyomas, whilst one was a dermatofibroma. Genetic sequencing did not evidence known fumarate hydratase gene mutations. Dermoscopy showed a brown delicate pigmented network and included leiomyomas among the non-melanocytic benign skin tumours featuring a dermatofibroma-like pattern. Abdominal computerized-tomography scan did not reveal renal cancer, but brain magnetic resonance imaging showed one asymptomatic cerebral cavernoma. The patient benefited from the surgical removal of the five larger cutaneous lesions and from gabapentin, which relieved her pain., Conclusions: This observation highlights the usefulness of dermoscopy in the diagnosis of cutaneous leiomyomas disclosing multiple cutaneous and uterine leiomyomatosis syndrome. Dermoscopy should be performed for non-melanocytic multiple lesions mimicking leiomyomas in a large number of patients, to establish a strict classification and identify false negative cases or evaluate them as dermatofibromas. In this case, the dermatologist recognized the risk of renal cancer and cerebral cavernomas.

Background: Some studies reported that cerebral developmental venous anomaly (DVA) is often concurrent with cavernous malformation (CM). But there is lack of statistical evidence and study of bulk cases. The factors associated with concurrency are still unknown. The purpose of this study was to determine the prevalence of concomitant DVA and CM using observational data on Chinese patients and analyze the factors associated with the concurrency., Methods: The records of all cranial magnetic resonance imaging (MRI) performed between January 2001 and December 2012 in Beijing Tiantan Hospital were reviewed retrospectively. The DVA and CM cases were selected according to imaging reports that met diagnostic criteria. Statistical analysis was performed using the Pearson chi-square statistic for binary variables and multivariable logistic regression analysis for predictors associated with the concurrent CM., Results: We reviewed a total of 165,230 cranial MR images performed during the previous 12 year period, and identified 1,839 cases that met DVA radiographic criteria. There were 205 patients who presented concomitant CM among the 1,839 DVAs. The CM prevalence in DVA cases (11.1%) was significantly higher than that in the non-DVA cases (2.3%) (P<0.01). In the multivariate analysis, we found that DVAs with three or more medullary veins in the same MRI section (adjusted OR = 2.37, 95% CI: 1.73-3.24), infratentorial DVAs (adjusted OR = 1.71, 95% CI: 1.26-2.33) and multiple DVAs (adjusted OR = 2.08, 95% CI: 1.04-4.16) have a higher likelihood of being concomitant with CM., Conclusions: CM are prone to coexisting with DVA. There is a higher chance of concurrent CM with DVA when the DVA has three or more medullary veins in the same MRI scanning section, when the DVA is infratentorial, and when there are multiple DVAs. When diagnosing DVA cases, physicians should be alerted to the possibility of concurrent CM.

We report the case of a 37-year-old male with Cowden disease that caused a gradual neurological deficit because of rupture of a brain stem cavernous hemangioma. Removal of the hemangioma and hematoma was performed with an infrafacial triangle approach. Nine months after the operation, magnetic resonance imaging showed abnormal vessels on the cerebellar surface. Digital subtraction angiography showed a dural arteriovenous fistula (dAVF) from part of the meningeal artery to the ectatic inferior vermian vein with cortical reflux. After embolization, surgical obliteration of the dAVF was performed. Surgical findings showed neovascularization in the thickened dura, in which dural vessels shunted to cerebellar vessels through adhesion between the dura mater and cerebellar surface. Therefore, the thickened dura was removed with the cerebellar surface. This case suggests that postoperative angiogenesis may cause arteriovenous fistula in patients with Cowden disease., (Published by Elsevier Inc.)

Familial cerebral cavernoma is an autosomal dominant phenotype with incomplete clinical and neuroimaging penetrance. The most common clinical manifestations include seizures and cerebral hemorrhage. We present the case of a 7-year-old boy who developed acute onset facial nerve paralysis secondary to previously unknown familial cerebral cavernoma. Genetic workup revealed a KRIT1 gene deletion which was later confirmed in the patient's asymptomatic father and younger brother., (© 2014.)

Objective: To present our experience of symptomatic cerebral cavernous malformations (CCMs) in pregnancy and to review the literature on the topic., Methods: We retrospectively collected a case series of symptomatic CCMs during pregnancy or the puerperium. A literature search was performed to identify all similar reports., Results: We collected 16 cases of symptomatic CCMs. Haemorrhage occurred in 10 patients. Two patients opted for termination of pregnancy. Delivery occurred preterm in four cases, in only one case due to neurological symptoms at 30 weeks' gestation. Caesarean section was performed in 9 cases; concern over CCM was the indication for delivery in eight of these cases. Four out of 16 patients underwent neurosurgery, three during pregnancy., Conclusion: Symptomatic CCMs seldom require neurosurgery either during or after pregnancy and are not associated with preterm delivery.

Background: By definition, a cavernous sinus is a vascular plexus. A hemangioma is a vascular tumor which is rich in vascular plexus and caused by vascular deformity or tumor-like proliferation. Radiosurgery can effectively control the growth of smaller cavernous sinus hemangiomas (CSHs). This paper aimed to quantify the MRI characteristics of CSHs which provided a basis for further treatment., Methods: A retrospective analysis was conducted of the MRI characteristics of 133 cavernous sinus lesion patients between January 2006 and December 2009, and none of them was diagnosed without histology. Two groups were set apart according to the pathological results: a CSH group (24 patients) and a non-CSH group (109 patients). The latter was further divided into 3 categories: a meningioma group (33 patients), a neurilemmoma group (37 patients) and a miscellaneous group (39 patients). A single-factor χ(2) analysis was performed to analyze the data on the 12 MRI characteristics., Results: T2-weighted ultrahigh signal, signal uniformity, dumbbell-like appearance and infiltration of the sellar region were considered statistically significant for the differentiation of CSHs from non-CSHs. If these 4 items served as the diagnostic criteria, the sensitivity, specificity and accuracy was 87.5, 96.3 and 94.7%, respectively., Conclusion: Smaller CSHs can be treated by radiosurgery directly, without performance of a pathological biopsy., (© 2014 S. Karger AG, Basel.)

The mutation detection rate for familial cerebral cavernous malformations (CCM) is extremely high, being about 90 % if direct sequencing of the three genes, CCM1, CCM2, and CCM3, is used in conjunction with quantitative analyses to detect larger CCM1-3 deletions/duplications. We here report on an individual who had presented with more than 30 cerebral and spinal cavernous malformations, two intracranial meningiomas, and disease manifestation only in the mid-forties. A CCM1 missense variant of unclear relevance was found during the first sequencing step. Thereafter, direct sequencing of all three CCM genes revealed the typical pathogenic loss-of-function mutation c.598C > T/p.Q200* in the CCM3 gene. Our results demonstrate that mutation analyses of all three CCM genes in the index patient regardless of previous identification of an unclassified CCM1 variant is crucial for reliable predictive testing of at-risk relatives.

In this video, we illustrate a right far-lateral craniotomy for resection of a 13-mm cavernous malformation of the pons in a healthy 53-year-old female patient presenting with diplopia and right 6th nerve palsy. The cavernous malformation was surrounded by normal pons, but was within 1 mm of the pontomedullary sulcus. The lesion was exposed from below through a far lateral craniotomy and accessed through the vasoaccessory triangle, superior to olivary nucleus and 12th cranial nerve. The alternative retrosigmoid craniotomy would have involved significant transgression of the middle cerebellar peduncle. The patient had gross-total resection and some temporary increase in her abducens nerve palsy without any complication. The video can be found here: http://youtu.be/8nOnrnTk3Tg .