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Objective: To evaluate the demographic, clinical, radiological features, and surgical outcomes of 31 patients who underwent surgery for cranial cavernous malformations (CCM). Methods: A retrospective analysis was conducted on 31 patients who underwent CCM between July 2020 and January 2024. Data included demographic and clinical, radiological, intraoperative, and histopathological findings, and postoperative complications. Detailed neurological examinations were performed before and after surgery. All patients underwent preoperative computed tomography and magnetic resonance imaging (MRI), and the lesions were evaluated using the Zabramski classification. Surgeries aimed at total resection using neuronavigation, intraoperative MRI, and ultrasonography when needed. The average follow-up duration was 25.3 months. Results: Thirty-three transcranial microsurgical excisions were performed in 31 patients (48% male, 52% female; mean age 30.8 years). Lesions were most commonly parietal (39%) and frontal (26%). Clinical findings included epilepsy (62%), headache (20%), and focal neurological deficits (6%). According to Zabramski, 42% were type I, 52% were type II, and 6% were type III. Total excision was achieved in 94% of the patients, with 6% requiring a second operation. Postoperative seizures were absent in 74% of patients with epilepsy. The average length of hospital stay was 5.7 days, with no permanent neurological deterioration or mortality. Conclusion: Surgical resection is effective for treating symptomatic CCM. Total resection cases should be closely monitored due to the risk of recurrence. A conservative approach is recommended for asymptomatic, deep-seated, or eloquent lesions, and radiosurgery is considered for high surgical risk cases. Multidisciplinary and personalized treatment protocols can improve outcomes in patients with CCM. [ABSTRACT FROM AUTHOR]

Cavernous angiomas (CA) affecting cranial nerves (CN) are exceptionally rare. Most reported cases involve the optic nerves, with even fewer occurrences in other CN. In this report, the authors describe a case of an extra-axial CA affecting the oculomotor nerve, which was managed conservatively. The authors also review the outcomes of other therapeutic options based on cases documented in the existing literature. Case report: 36-year-old woman presented to the Emergency Department with a headache in the left temporal region, predominantly at night, along with mild left palpebral ptosis and binocular diplopia of 2 months duration. Brain MRI revealed a lesion exhibiting typical characteristics of CA in the left lateral region of the interpeduncular cistern, in close contact with the left oculomotor nerve. After considering treatment options and in consultation with the patient, a conservative management plan with periodic MRI follow-up was chosen. After 2 years of follow-up, the patient showed favorable progress. Although exceptional, CA should be considered in the differential diagnosis of other more common extra-axial lesions involving CN. The therapeutic management of a CA of the oculomotor nerve remains controversial due to the limited number of cases described in the literature. The authors suggest that for individuals with asymptomatic or mildly symptomatic CA affecting the oculomotor nerve, a conservative treatment approach is the most suitable choice to preserve neurological function. In cases characterized by progressive symptoms, a history of recurrent bleeding, or evidence of lesion enlargement on sequential imaging assessments, total microsurgical resection should be considered. [ABSTRACT FROM AUTHOR]

Background Intracranial cavernous malformations (CMs), commonly known as cavernomas or cavernous angiomas, are low-flow, well-circumscribed vascular lesions composed of sinusoidal spaces lined by a single layer of endothelium and separated by a collagenous matrix without elastin, smooth muscle, or other vascular wall elements. A diameter greater than 3 cm for a CM is unlikely. These lesions may have atypical appearances on magnetic resonance imaging (MRI). MRI with advanced techniques such as a susceptibility-weighted image or T2-gradient echo, a diffusion-weighted image and corresponding apparent diffusion coefficient map, and diffusion tensor tractography have revolutionized the diagnostic approach to these lesions. Materials and Method The present study reviews the etiopathogenesis, clinical manifestations, MRI strategy, and MRI appearances of the CMs, with a few examples of the giant CMs from our archive. Results Intracranial giant CMs may have unexpected locations, sizes, numbers, and varied imaging appearances due to repeated hemorrhages, unusual enhancement patterns, intense perifocal edema, and unusual associations, making the differential diagnosis difficult. Conclusion Familiarity with the MRI appearances of the giant intracranial CMs and the differential diagnosis improves diagnostic accuracy and patient management. [ABSTRACT FROM AUTHOR]

Background: Cavernous malformations can occur throughout the cerebral nervous system, most commonly located in the supratentorial compartment. The intramedullary location is rare and accounts for approximately 2.4% to 5% of all spinal vascular tumors. The cervical cord location and its clinical manifestations are underreported. Methods: The authors report a case of spinal cord compression at the C5–C6 level by a cervical intramedullary cavernoma with a systematic review of the literature according to the Preferred Reporting Items for Systematic Reviews and meta-analysis guidelines. Relevant studies (1980 to 2023) that reported patients with cervical intramedullary cavernoma were identified from PubMed databases. Results: A total of 29 studies reporting 423 patients were included in this study with a mean age of 40.5 ± 6.3 years old. The overall male-to-female ratio was 1:2.5 with a median duration of follow-up of 46 months [18.5–63.9]. The type of resection as well as the patient's outcome was also reported. Conclusion: Intramedullary cavernous angiomas are rare. Early surgical total resection of the symptomatic lesions in adequate conditions is crucial for good outcomes. [ABSTRACT FROM AUTHOR]

Background: Patients with supratentorial cavernous malformations (SCMs) commonly present with seizures. First-line treatments for cavernoma-related epilepsy (CRE) include conservative management (antiepileptic drugs (AEDs)) and surgery. We compared seizure outcomes of CRE patients after early (≤6 months) vs. delayed (>6 months) surgery. Methods: We compared outcomes of CRE patients with SCMs surgically treated at our large-volume cerebrovascular center (1 January 2010–31 July 2020). Patients with 1 sporadic SCM and ≥1-year follow-up were included. Primary outcomes were International League Against Epilepsy (ILAE) class 1 seizure freedom and AED independence. Results: Of 63 CRE patients (26 women, 37 men; mean ± SD age, 36.1 ± 14.6 years), 48 (76%) vs. 15 (24%) underwent early (mean ± SD, 2.1 ± 1.7 months) vs. delayed (mean ± SD, 6.2 ± 7.1 years) surgery. Most (32 (67%)) with early surgery presented after 1 seizure; all with delayed surgery had ≥2 seizures. Seven (47%) with delayed surgery had drug-resistant epilepsy. At follow-up (mean ± SD, 5.4 ± 3.3 years), CRE patients with early surgery were more likely to have ILAE class 1 seizure freedom and AED independence than those with delayed surgery (92% (44/48) vs. 53% (8/15), p = 0.002; and 65% (31/48) vs. 33% (5/15), p = 0.03, respectively). Conclusions: Early CRE surgery demonstrated better seizure outcomes than delayed surgery. Multicenter prospective studies are needed to validate these findings. [ABSTRACT FROM AUTHOR]

Cavernous angiomas (CAs) are benign vascular malformations predominantly seen in the brain parenchyma and therefore referred to as intra-axial. Extra-axial dural-based cavernous angiomas, on the other hand, are rare vascular lesions found outside of the brain parenchyma. They occur in the middle fossa and may be easily misdiagnosed as meningiomas due to their extra-axial location. In addition, CAs that are located outside the middle fossa, such as in the convexity, have a better prognosis since they are more surgically accessible. Surgical resection is the main treatment of choice in CAs. However, other options, such as embolization and radiotherapy, may also be considered therapeutic choices or additive treatment options. The pathogenesis of CA and the involvement of other factors (genetics or environmental factors) are still unknown and require further investigation. We are presenting a young man who presented for evaluation of seizure-like events without any family history of neurologic conditions. The physical examination was unremarkable except for a slightly antalgic gait. Imaging studies showed an extra-axial left tentorial mass suggestive of a meningioma, hemangiopericytoma, or other extra-axial lesions. The lesion was resected where its vascular nature was mentioned initially, and the histology proved the diagnosis of cavernous angioma. Here we give an overview of the known pathogenesis, causes, clinical features, and diagnostic and therapeutic options in CA. Better knowledge about CA, its causes, clinical features, and treatment options would help clinicians in early diagnosis and patient management. [ABSTRACT FROM AUTHOR]

Cerebral cavernous malformations (CCM) are clusters of dilated sinusoidal channels, lined by a single layer of endothelium. Acquired form of these lesions was related to previous radiation-therapy, and might be related to its pathophysiology, because the vascular endothelial growth factor has higher expression in the immature brain of the child. Consequently, although it occurs mainly in the paediatric population, it can happen in adults. We report a case of radiation-induced CCM in an adult patient presenting with aggressive behaviour and cerebral haemorrhage. [ABSTRACT FROM AUTHOR]

Vascular malformations of the brain are aberrant vascular connections that are most likely congenital. Cerebral vascular malformations are the umbrella term for multiple conditions, each with different symptoms, signs, and imaging characteristics. These conditions are (1) Arteriovenous malformations, abnormal arteries, and veins; (2) Dural arteriovenous fistula; (3) Developmental venous anomalies; (4) Cavernous malformations, enlarged blood-filled spaces; (5) Cavernous angiomas, abnormal veins; (6) capillary telangiectasias, enlarged capillary-sized vessels; (7) vein of Galen malformations; and (8) mixed malformations. It is important to study the complications of each and their mimics to make an accurate diagnosis. Various imaging features of different vascular malformations seen on MRI are discussed that which would aid in diagnosis and planning management. [ABSTRACT FROM AUTHOR]

Background and purpose: We aimed to assess the course and predictors of functional outcome after single and multiple intracerebral hemorrhage (ICH) in pediatric patients with cerebral cavernous malformations (CCMs) and to conduct a risk assessment of a third bleed during the first follow‐up year after second ICH. Methods: We included patients aged ≤18 years with complete baseline characteristics, a magnetic resonance imaging dataset, ≥1 CCM‐related ICH and ≥1 follow‐up examination, who were treated between 2003 and 2021. Neurological functional status was obtained using modified Rankin Scale scores at diagnosis, before and after each ICH, and at last follow‐up. Kaplan–Meier analysis was performed to determine the cumulative 1‐year risk of third ICH. Results: A total of 55 pediatric patients (median [interquartile range] age 12 [11] years) were analyzed. Univariate analysis identified brainstem cavernous malformation (BSCM; p = 0.019) as a statistically significant predictor for unfavorable outcome after second ICH. Outcome after second ICH was significantly worse in 12 patients (42.9%; p = 0.030) than after first ICH and in five patients (55.6%; p = 0.038) after a third ICH compared to a second ICH. Cumulative 12‐month risk of rebleeding during the first year after a second ICH was 10.7% (95% confidence interval 2.8%–29.37%). Conclusions: Pediatric patients with a BSCM have a higher risk of worse outcome after second ICH. Functional outcome improves over time after an ICH but worsens following each ICH compared to baseline or previous ICH. Second bleed was associated with neurological deterioration compared to initial ICH, and this deteriorated further after a third ICH. [ABSTRACT FROM AUTHOR]

Purpose: Spinal cavernous malformations (SCM) present a risk for intramedullary hemorrhage (IMH), which can cause severe neurologic deficits. Patient selection and time of surgery have not been clearly defined. Methods: This observational study included SCM patients who underwent surgery in our department between 2003 and 2021. Inclusion required baseline clinical factors, magnetic resonance imaging studies, and follow-up examination. Functional outcome was assessed using the Modified McCormick scale score. Results: Thirty-five patients met the inclusion criteria. The mean age was 44.7 ± 14.5 years, and 60% of the patients were male. In univariate analysis, the unfavorable outcome was significantly associated with multiple bleeding events (p =.031), ventral location of the SCM (p =.046), and incomplete resection (p =.028). The time between IMH and surgery correlated with postoperative outcomes (p =.004), and early surgery within 3 months from IMH was associated with favorable outcomes (p =.033). This association remained significant in multivariate logistic regression analysis (p =.041). Conclusions: Removal of symptomatic SCM should be performed within 3 months after IMH when gross total resection is feasible. Patients with ventrally located lesions might be at increased risk for postoperative deficits. [ABSTRACT FROM AUTHOR]

Background and purpose: The purpose of this study was to investigate the 5‐year risk of a third bleeding event in cavernous malformations (CMs) of the central nervous system. Methods: Patients with cerebral or spinal CMs treated between 2003 and 2021 were screened using our institutional database. Patients with a complete magnetic resonance imaging dataset, clinical baseline characteristics, and history of two bleeding events were included. Patients who underwent surgical CM removal were excluded. Neurological functional status was obtained using the modified Rankin Scale score at the second and third bleeding. Kaplan–Meier and Cox regression analyses were performed to determine the cumulative 5‐year risk for a third haemorrhage. Results: Forty‐two patients were included. Cox regression analysis adjusted for age and sex did not identify risk factors for a third haemorrhage. 37% of patients experienced neurological deterioration after the third haemorrhage (p = 0.019). The cumulative 5‐year risk of a third bleeding was 66.7% (95% confidence interval [CI] 50.4%–80%) for the whole cohort, 65.9% (95% CI 49.3%–79.5%) for patients with bleeding at initial diagnosis, 72.7% (95% CI 39.3%–92.7%) for patients with a developmental venous anomaly, 76.9% (95% CI 55.9%–90.3%) for patients with CM localization to the brainstem and 75% (95% CI 50.6%–90.4%) for patients suffering from familial CM disease. Conclusions: During an untreated 5‐year follow‐up after a second haemorrhage, a significantly increased risk of a third haemorrhage compared to the known risk of a first and second bleeding event was identified. The third bleeding was significantly associated with neurological deterioration. These findings may justify a surgical treatment after a second bleeding event. [ABSTRACT FROM AUTHOR]

Objective: Cerebral cavernous malformations (CMs) presenting with focal neurological symptoms or mass effects require surgical removal. In recent years, cylindrical retractors have been widely utilized for the removal of deep-seated lesions during both microscopic and endoscopic surgery. In the present study, we evaluated the efficacy and safety of endoscopic transcylinder removal of CMs using a novel wet-field technique. Methods: We included 13 patients with supratentorial CMs who had undergone endoscopic transcylinder surgery between April 2013 and March 2022. One patient experienced recurrence of the CM and underwent a second endoscopic transcylinder surgery. Therefore, we retrospectively evaluated 14 procedures. The surgical field was continuously irrigated with artificial cerebrospinal fluid to maintain expansion and visualization of the tumor bed. We termed this method as the "wet-field technique." Patient characteristics, symptoms, and pre- and postoperative magnetic resonance imaging results were obtained from medical records. Results: The average maximum CM diameter was 35.3 mm (range: 10–65 mm). Cylinder diameters were 6 mm in eight procedures, 10 mm in four procedures, and 17 mm in one procedure. Wet-field technique was applied in all cases. The endoscope provided a bright field of view even under water. Continuous water irrigation made it easier to observe the entire tumor bed which naturally expanded by water pressure. Gross total resection was achieved in 13 procedures, while subtotal resection was achieved in one procedure. No surgical complications were observed. Conclusions: The endoscopic transcylinder removal using wet-field technique is safe and effective for the removal of symptomatic intracranial supratentorial CMs. [ABSTRACT FROM AUTHOR]

Background: Spinal intramedullary cavernous angioma is a rare form of spinal cord tumor that is associated with myelopathy and significant morbidity and surgical treatment is almost always required. Case: We report a case of spinal intramedullary cavernous angioma in a pain clinic with initial symptoms of unilateral interdigital space sensory change. Morton’s neuroma and piriformis syndrome were clinically suspected, however, symptoms acutely aggravated, and paraplegia developed following the patient’s COVID-19 vaccination. Vaccine-associated side effects were ruled out and spinal intramedullary cavernous angioma was confirmed through magnetic resonance image. The patient underwent surgery for complete mass excision. Conclusions: Recent reports of COVID-19 vaccine-associated side effects have raised sensitive concerns to both health care providers and the public, that in some cases when the symptoms coincide with vaccination history, it may delay time-sensitive diagnosis and treatment and spend unnecessary costs. [ABSTRACT FROM AUTHOR]

Objective: We performed a retrospective observational study to demonstrate the surgical risks and long-term prognoses of intramedullary tumors in Japan using a multicenter registry authorized by the Neurospinal Society of Japan. Methods: Data from 1,033 consecutive patients with intramedullary tumors, treated between 2009 and 2020, were collected from 58 centers. Patients with spinal lipomas or myxopapillary ependymomas were excluded. Patient characteristics, clinical presentations, imaging characteristics, treatments, and outcomes were analyzed. The modified McCormick scale was used to classify functional status. Survival was described using Kaplan-Meier curves, and multivariable logistic regression analyses were performed. Results: The mean age of the patients was 48.4 years. Data of 361 ependymomas, 196 hemangioblastomas, 168 astrocytic tumors, 160 cavernous malformations, and the remaining 126 cases including subependymomas, metastases, schwannomas, capillary hemangiomas, and intravascular B-cell lymphomas were analyzed. Twenty-two patients were undiagnosed. The mean follow-up duration was 46.1 ± 38.5 months. Gross total tumor removal was achieved in 672 tumors (65.1%). On the modified McCormick scale, 234 patients (22.7%) had worse postoperative grades at the time of discharge. However, neurological status gradually improved. At 6 months postoperatively, 251 (27.5%), 500 (54.9%), and 160 patients (17.6%) had improved, unchanged, and worsened grades, respectively. Preoperative functional status, gross total tumor removal, and histopathological type were significantly associated with mortality and functional outcomes. Conclusion: Our findings demonstrate better postoperative functional outcomes in patients with fewer preoperative neurological deficits. Degree of resection, postoperative treatments, and prognoses are closely related to the histology of intramedullary tumors. [ABSTRACT FROM AUTHOR]

Introduction: Pediatric cerebral cavernous malformations (CCMs) are commonly treated vascular anomalies with different clinical characteristics than their adult counterparts. Outcomes of Gamma Knife Radiosurgery (GKRS) for pediatric CCMs have not explicitly been reported. This paper reports our experience in managing pediatric CCMs with GKRS. Methods: We retrospectively reviewed the clinical features, GKRS parameters, and clinical and radiological outcomes of 46 children with 64 CCMs. Results: A total of 46 children, including 19 girls and 27 boys, with a median age of 16 years (3–17 years), were enrolled in the study. The median age at first CCM diagnosis was 13 years (range, 2–17 years). Twenty-two patients (47.8%) had more than one neurological symptom at the time of diagnosis, and the most common presenting symptom was seizure (28.3%). The most common location was supratentorial superficial (53.1%), and 17.4% of patients had multiple CCMs. A developmental venous anomaly was detected in 5 patients (10.9%). During a total of 52.4 retrospective patient-years in patients with > 1 hemorrhage episode, the calculated annual hemorrhage rate was 40.1%. The median post-GKRS follow-up was 79 months (range, 19–175 months), with an overall 306.2 prospective patient years. The annual hemorrhage rate (AHR) during the first 2 years after GKRS and after the initial 2 years was 1.11% and 0.46%, respectively. Regarding clinical factors and GKRS parameters, univariate analysis revealed a significant association with post-GKRS AHR and volume (p = 0.023) only. Patients with pre-GKRS seizures showed favorable seizure control (Engel class I and II) in 8 children (61.5%). There was no mortality in our series. Conclusion: Low AHR following GKRS with no radiation-induced toxicity makes GKRS a therapeutic alternative for pediatric CCMs. [ABSTRACT FROM AUTHOR]

Background and Purpose: Analyze and compare the natural course of confirmed familial cerebral cavernous malformation (FCCM), assumed FCCM and non‐familial multiple cerebral cavernous malformation (CCM) disease over a 5‐year period. Methods: Our institutional database was screened for patients with CCM admitted between 2003 and 2020. Patients with complete magnetic resonance imaging dataset, evidence of multiple CCM, clinical baseline characteristics, and follow‐up examination were included. Patients were separated into confirmed familial cases, assumed familial cases or non‐familial multiple cavernous malformations. Kaplan–Meier and Cox regression analyses were performed to determine the cumulative 5‐year risk for hemorrhage and recurrent hemorrhage. Results: A total of 238 patients with multiple CCM were analyzed; 90 individuals had a confirmed FCCM disease, 115 an assumed FCCM, and 33 were allocated to the non‐FCCM group. Univariate Cox regression analysis identified intracerebral hemorrhage (ICH) as mode of presentation (p = 0.001) as a predictor for occurrence of recurrent hemorrhage during the 5‐year follow‐up (FU). The cumulative 5‐year risk of (re)bleeding was 21.6% for the entire cohort, 30.7% for patients with ICH at diagnosis, 22.1% for those patients with a confirmed diagnosis of FCCM, 23.5% for those with an assumed FCCM, and 21% for the non‐FCCM cases. Conclusions: FCCM patients with ICH at diagnosis are prone to develop rebleeding. During untreated 5‐year FU, FCCM patients and patients with sporadic multiple CCM reveal an almost equal susceptibility for (re)hemorrhage. Moreover, confirmed, assumed and non‐FCCM patients showed an equal cumulative 5‐year risk of symptomatic ICH. The probability of hemorrhage tends to increase over time, particularly in cases with ICH at presentation. [ABSTRACT FROM AUTHOR]

Background: The present study hypothesized that some factors may distinguish between patients with a brain cavernous angioma (BCA), who were free from anti-seizure medication (ASM), and patients who still required ASMs postoperatively. The purpose of the study was thus to identify factors associated with ceasing ASMs for patients with drug-resistant epilepsy secondary to BCA, who underwent BCA removal surgery. Methods: We divided patients into those with drug-resistant epilepsy secondary to BCA who achieved complete seizure freedom without ASMs a year after surgery (No-ASM group) (International League Against Epilepsy (ILAE) classification class I with no epileptiform discharges), and others (ASM group) (ILAE classification ≤ II and/or epileptiform discharges). We statistically compared groups in terms of: (1) age at operation; (2) history of epilepsy; (3) size of BCA; and (4) location of BCA. Results: Overall, a year after the surgery, the No-ASM group comprised 12 patients (48%), and the ASM group comprised 13 patients (52%). In both multi- and univariate logistic regression analyses, age at BCA removal surgery correlated significantly with the No-ASM group (p = 0.043, p = 0.019), but history of epilepsy did not (p = 0.581, p = 0.585). Conclusions: Earlier age at surgery for patients with drug-resistant epilepsy is encouraged to achieve complete seizure freedom without the need for ASMs when the cause of epilepsy is BCA. [ABSTRACT FROM AUTHOR]

The insular cortex is considered one of the most complex regions of the brain, defined as the "hub" of somatosensory areas. Here, we examine the case of a surgically treated haemorrhagic cavernoma involving the middle and posterior insular cortex, presenting both sensory, gustative and speech symptoms. By reviewing the recent findings in humans' and primates' basic research, we illustrated clinical and radiological correlations of the reported case, confirming insular role in sensitive and gustatory functions. [ABSTRACT FROM AUTHOR]

Background  Cavernomas are usually found in the supratentorial area, and epileptic seizures are one of the presenting symptoms. Objective  This study aims to provide the seizure outcome in adult patients who underwent surgical excision of single supratentorial cavernomas. Materials and Methods  A total of 23 patients with single supratentorial cavernomas were operated between May 2011 and January 2019. Pre- and postoperative seizure semiology, clinical, and radiological findings were collected from medical records. At the last follow-up, each patient was seen during regular visits and clinical variables were noted. Results  The mean age was 37.08 ± 10.5 years, and 11 (57.8%) and 12 (52.2%) were females and males, respectively. Headache (43.5%) and seizure (43.5%) were the most common presenting symptoms. Cavernomas were located on the right side in 13 and on the left side in 10 patients. The most common locations were the frontal (43.5%) and temporal (43.5%) lobes. The mean follow-up in this series was 41.4 ± 30.8 months. Our results showed that surgery was effective in seizure outcome, as almost 70% of patients who had seizure before surgery was seizure free after surgery, and the difference between those who had seizure pre- and postoperative periods was statistically significant (p = 0.0001). Conclusion  Surgery is safe and effective for supratentorial cavernomas. The excision of cavernoma together with the surrounding hemosiderin should be performed to obtain a satisfactory seizure outcome. [ABSTRACT FROM AUTHOR]

There was a lack of natural history of incidental brainstem cavernous malformations (CMs), hemorrhage of which would lead to severe neuropathies. The study aimed to evaluate the prospective hemorrhage rate and neurological outcome of the disease. This prospective cohort included patients with incidental brainstem CMs referred to our institute from 2009 to 2015. The diagnosis was confirmed based on the patients' complain, physical examination, and radiographic evidence. Clinical data were collected, scheduled follow-up was performed, and the independent risk factors were identified by multivariate analysis. This cohort included 48 patients (22 female, 45.8%). The median follow-up duration was 60.7 months, and 13 prospective hemorrhages occurred within 244.0 patient-years yielding an annual hemorrhage rate of 5.3%. The hemorrhage-free survival at 1 and 5 years was 91.6% and 80.6%. Age ≥ 55 years (hazard ratio (HR) = 8.59, p = 0.003), lesion size (per 1-mm increase) (HR = 3.55, p = 0.041), developmental venous anomaly (HR = 10.28, p = 0.017), and perilesional edema (HR = 4.90, p = 0.043) were independent risk factors for hemorrhage. Seven patients (14.6%) received surgical resection, and the other 41 patients remained under observation. Neurological function was improved in 22 patients (45.8%), unchanged in 19 (39.6%), and worsened in 7 (14.6%). Prospective hemorrhage (odds ratio = 14.95, p = 0.037) was the only independent risk factor for worsened outcomes. The natural history of incidental brainstem CMs seemed to be acceptable with improved/unchanged outcomes in most patients (85.4%). These results improved our understanding of the disease, and the future study of a large cohort was required to verify our findings. [ABSTRACT FROM AUTHOR]

Aim: The aim of this case series is to report two cases of giant cavernomas treated at a tertiary level centre along with a literature review on the differences between giant cavernomas and normal sized cavernomas.Method: The first case was that of a 13-year-old male with habitual seizures since one year of age with normal examination findings. His imaging revealed a large parieto-occipital lesion which was excised microsurgically after a parieto-occipital craniotomy and a transcortical approach. The second case was a 26-year-old male who presented with generalised seizures and bilateral visual loss. Imaging was suggestive of a large left lateral ventricular lesion. A left fronto-temporo-parietal craniotomy was done followed by transventricular microsurgical excision. Histopathology of both cases were reported to be cavernomas. The first patient had transient hemiparesis post-operatively which improved within 24 hours. Eventually, both patients had an uneventful follow-up.Conclusion: Microsurgical excision remains the treatment of choice for giant cavernomas. In toto excision may be advocated depending upon the location of the cavernoma especially with respect to eloquence and venous anatomy. [ABSTRACT FROM AUTHOR]

Background: Patients with cerebral or spinal cavernous malformations (CM) and a primary headache disorder are often limited in medication options due to concern for bleeding risk. Methods: From a prospective cohort of CM patients (2015–2020), demographics, mode of clinical presentation, and radiographic data were collected. Follow up of patients was performed with electronic medical record review, in person visits and/or written surveys. Select medication use was ascertained from the time of the CM diagnosis to a censor date of first prospective symptomatic hemorrhage, complete surgical excision of sporadic form CM, or death. The influence of non-aspirin NSAID (NA-NSAID), triptan, or OnabotulinumtoxinA on prospective hemorrhage risk was assessed. Results: As of August 20, 2020, 329 patients with spinal or cerebral CM (58% female; 20.1% familial; 42.2% initial presentation due to hemorrhage; 27.4% brainstem) were included. During a follow-up of 1799.9 patient years, 92 prospective hemorrhages occurred. Use of NA-NSAIDs, triptans, and OnabotulinumtoxinA after the diagnosis of CM was unassociated with an increased risk of prospective hemorrhage. Conclusions: Use of triptans and NA-NSAIDs, does not precipitate CM hemorrhage. Similarly, we did not find that OnabotulinumtoxinA precipitated CM hemorrhage in a limited number of patients at doses <200 units per session. [ABSTRACT FROM AUTHOR]

Hemichorea may point to a structural lesion in the contralateral basal ganglia with a large list of possible causes. Cavernous angioma may be rarely a possible cause for acute appearance of this movement disorder. We present a rare case of a 32-year-old female patient with hemichorea caused by a cavernoma (or cavernous angioma) in the contralateral insula and putamen with complete improvement of symptoms with surgical resection of the lesion. We believe that surgical resection of basal ganglia cavernomas may be feasible with minor risks and resolution of clinical symptoms in the immediate postoperative period. [ABSTRACT FROM AUTHOR]

Cavernous angiomas (CAs) are vascular malformations that may result in stroke. Herein, we evaluate a CA patient with chronic migraine who experienced 2 documented symptomatic hemorrhages after receiving respective high doses of botulinum toxin (Btx). Recently, bacterial lipopolysaccharide has been reported to contribute to CA development through Toll-like receptor signaling, causing hemorrhagic angiogenic proliferation. Lipopolysaccharide and Btx share a common intracellular signaling pathway driving CA development and hemorrhage. Significance of these observations is demonstrated by previous works on plasma molecules showing prognostic associations with symptomatic hemorrhages in human CA, related to the same canonical pathways. Authors suggest careful tracking of the association of Btx and hemorrhage in CA patients. [ABSTRACT FROM AUTHOR]

Background: Brainstem cavernous malformations (CM) carry high risks of hemorrhage and neurologic morbidity. While much is published on physical effects of brainstem CM, very little is known about these patients' quality of life. This study aimed to assess the quality of life PROMIS-29 health domains of brainstem CM patients and identify quality of life predictors. Methods: This was a cross-sectional study of adult patients with at least one brainstem CM identified by advertising on the Angioma Alliance website and from our institutional CM registry. A web-based questionnaire was administered and included self-reported information about the patient, cavernous malformation, residual clinical symptoms, and treatment. In addition, patients filled out the PROMIS-29 (version 1.0). The PROMIS-29 has 7 health domains and is standardized against the general population. We defined impaired quality of life as at least one out of 7 abnormal domains and used a 1 standard deviation cutoff for abnormal. We verified clinical and radiographic data to self-reported data in 28.8% of patients. Results: A total of 104 patients (mean age of 46.5 ± 11.5 years; 77.9% females) were recruited. Most (82.7%) reported at least one symptomatic hemorrhagic event and 36.5% reported at least 1 surgical procedure. At least one abnormal PROMIS domain was present in 64.4% of patients with fatigue (34.6%), anxiety (35.6%), social (28.2%), and physical (27.9%) domains being the most common. Among patients with a Rankin Score of 0–2, 55% had at least one abnormal domain. Gait difficulty, but not age, sex, or surgery predicted impaired quality of life. Conclusion: More than half of patients with brainstem CM have impaired quality of life. Fatigue, anxiety, and social function, in addition to physical dysfunction, are common; practitioners should be aware of these concerns. PROMIS-29 provides additional information than modified Rankin Score and should be considered in clinical trials and when assessing treatment outcomes until a disease-specific outcome tool is available. [ABSTRACT FROM AUTHOR]

Intraventricular cavernoma (IVC) is a rare pathological entity constituting 2.5%–10.8% of cerebral cavernomas. The lateral ventricles are the most frequent site, followed by the third and fourth ventricles. IVCs usually attain a large size compared to parenchymal cavernomas and cause signs and symptoms mainly due to mass effect. IVCs lack specific clinical manifestations and radiological features. Microsurgical excision of IVCs is a safe and effective treatment option. We present a 71-year-old male patient with right lateral ventricle cavernous angioma. The patient underwent microsurgical resection of the vascular lesion with good neurological outcome. [ABSTRACT FROM AUTHOR]

Tumors that involve the orbit can be classified into two major groups: primary tumors of the orbit and tumors that extend into the orbit from other sites. The most frequent primary orbital lesions in adults include cavernous hemangiomas, lymphoid tumors, and meningiomas. The most common tumors that extend into the orbit are meningiomas, followed by sinonasal carcinomas. In this article, we report a case of intraconal orbital lesion operated at our center and a review of the surgical approaches to the orbit. [ABSTRACT FROM AUTHOR]

Klippel-Trenaunay-Weber syndrome (KTWS) is a rare syndrome characterized by the triad of cutaneous hemangiomas, venous varicosities, and osseous-soft tissue hypertrophy of the affected limb. Clinical manifestations, genetic testing, and radiologic imaging are the key steps in diagnosing this syndrome. An 18-month-old boy was brought for follow-up brain magnetic resonance imaging (MRI) with a history of right lower limb hypertrophy, cutaneous varicosities, and hemangiomas diagnosed at birth. A baseline MRI at 12 months revealed multiple hemorrhagic lesions within the cerebrum, the largest in the right temporal lobe, which was treated surgically at the age of 18 months because of its rapid growth. This is the youngest patient with KTWS treated surgically for intracranial hemangiomas. KTWS is a rare disease with a wide range of manifestations. Multisystemic evaluation of this group of patients should be performed to identify cavernous hemangiomas at the early stage of life and adequately treat them in the future. Treatment of KTWS patients with cavernous hemangiomas should not be different from the treatment of patients with any other hemangiomas, and surgical intervention should be considered on a case-to-case bases. [ABSTRACT FROM AUTHOR]

Aim: The authors reviewed their experience with 120 patients operated on for brain cavernomas. The clinical features, image findings and surgical results of the pediatric subgroup are compared with these of the whole series. Material and Methods: During the period of 19 years in our institution were operated on 120 patients with brain cavernomas. Nineteen of the patients were children. The patients were surgically treated by common operative techniques and in some cases frame-based stereotaxy, neuronavigation and neuroendoscopy were applied. The average age of the patients was 36.1 years and in the children‘s group 12.3 years. The ratio of men: women was 59:61 (8:11 in children). Long-term follow-up was performed for a period of 6 to 226 months, an average of 67.8 months for the general group and 60.5 months for children. Results: Only 3 of the children had transient neurological deterioration in the early postoperative period. The measured Karnofsky performance score in the long-term follow-up was 88.6. Eight out of 9 patients with epilepsy were improved (88.9%). The focal neurological deficit was improved in 8 of 8 cases. The intracranial hypertension was eliminated in all 6 cases. The localization and histology of the cavernomas in the pediatric subgroup were similar to these of the whole series, but there were significant differences in the frequency of the acute hemorrhages (26.3% versus 10%), the acute intracranial hypertension (36.8% vs. 8.3%) and the focal neurological deficit (47.4% vs. 26.7%). Conclusion: We concluded that brain cavernomas in childhood are related with higher frequency of hemorrhages, intracranial hypertension and focal neurological deficit. The cavernous malformations may be surgically removed with very good postoperative results, regardless of their localization. [ABSTRACT FROM AUTHOR]

Radiation was first demonstrated to be associated with cavernomagenesis in 1992. Since then, a growing body of literature has shown the unique course and presentation of radiation-induced cavernous malformations (RICMs). This study summarizes the literature on RICMs and presents a single-center experience. A prospectively maintained single institution vascular malformation database was searched for all cases of intracranial cavernous malformation (January 1, 1997–December 31, 2021). For patients with a diagnosis of RICM, information on demographic characteristics, surgical treatments, radiation, and surgical outcomes was obtained and analyzed. A comprehensive literature search was conducted using PubMed, Embase, Cochrane, and Web of Science databases for all reported cases of RICM. A retrospective review of 1662 patients treated at a single institution yielded 10 patients with prior radiation treatment in the neck or head region and a subsequent diagnosis of intracranial RICM. The median (interquartile range) latency between radiation and presentation was 144 (108–192) months. Nine of 10 patients underwent surgery; symptoms improved for 5 patients, worsened for 3, and were stable for 1. The systematic literature review yielded 64 publications describing 248 patients with RICMs. Of the 248 literature review cases, 71 (28.6%) involved surgical resection. Of 39 patients with reported surgical outcomes, 32 (82%) experienced improvement. RICMs have a unique course and epidemiology. RICMs should be considered when patients with a history of radiation present with neurologic impairment. When RICMs are identified, symptomatic patients can be treated effectively with surgical excision and close follow-up. [ABSTRACT FROM AUTHOR]

Background Arteriovenous malformations (AVMs) of the scalp are rare and infrequently encountered by the neurosurgeon. Case Description We report a unique case of a 42-year-old patient who presented with a progressive worsening of visual acuity in the right eye (lower quadrantanopia) and palpebral ptosis. Physical examination revealed a right exophthalmos and a right frontoparietal scalp soft swelling when the patient was in the supine position. Neurologic work-up showed a scalp AVM extending into the orbit and connected to an intraorbital cavernous angioma. The patient was treated with a frontotemporal craniotomy and decompression of the orbit. Conclusions In the rare case of intraorbital extension of a scalp AVM, neurologic symptoms may appear when the size of the vascular malformation increases with age. The aims of surgery should be decompression of the orbit and aesthetic preservation, rather than complete excision. A review of the literature is also provided. [ABSTRACT FROM AUTHOR]

Cavernous angiomas of the cranial nerves are extremely rare, and those of the oculomotor (third) cranial nerve are rarer still, with no previous cases having been reported in the literature. We report herein the first case of a presumed cavernous angioma involving the subarachnoid portion of the left third nerve presenting as an acute left third nerve palsy in an infant. The child was followed without intervention and the palsy completely resolved. Given the poor functional results with attempted surgical excision and the potential for spontaneous improvement in oculomotor nerve function, it is reasonable to follow patients with these lesions without intervention. [ABSTRACT FROM AUTHOR]

Background Hypertrophic olivary degeneration (HOD) is a rare phenomenon in the dento-rubro-olivary pathway caused by lesion or disruption of the fibers of the Guillain-Mollaret triangle. Hemorrhage of pontine and midbrain cavernous angiomas can rarely lead to HOD portending neurologic deterioration and possible concomitant life-threatening complications; for this reason, it may define a poignant consideration in planning intervention. Case Description The patient was a 57-year-old woman with known midbrain-pontine cavernous angioma. For several years, the lesion had been stable, as shown by imaging follow-up, until 10 months before the patient presented with falls, dysarthria, and headache. Imaging showed some decrease in size as well as blood product around the cavernous angioma, suggesting interim period hemorrhage and interval development of HOD. Conclusions The literature regarding imaging recommendations for stable cavernous angioma in the midbrain-pontine junction is reviewed. The implication of HOD for patient outcome is discussed and a comment is made on how the development of HOD may affect management of the cavernous angioma. [ABSTRACT FROM AUTHOR]

Microsurgery remains the gold standard treatment for intraventricular lesions, but it is not without limitations. The cerebral ventricles approach is considered a challenge for neurosurgeons because of their deep location and their intimate association with critical areas of the brain. It has been shown that direct endoscopic view is superior to magnetic resonance imaging (MRI) in identifying intraventricular structures and lesions. Also, during neuroendoscopy, there is no cerebrospinal fluid leak causing brain collapse as seen in microsurgery. Different surgical strategies should be shared and may improve and facilitate intraventricular lesions resection. Herein, a case of a successful microsurgical cavernoma resection with prior ventriculoscopy is described. A 28-year-old woman was admitted with intense holocranial headache. A mild stiff neck was observed. MRI showed a 3-cm lesion with heterogeneous signal intensity and no contrast enhancement suggestive of cavernoma. Initially, a ventriculoscopy was done to inspect the lesion and the surrounding ventricular structures. After that, a conversion technique from endoscopy to microsurgery was performed. The initial corticectomy was extended and the endoscope pathway was followed into the ventricular cavity. Then, the cavernoma was completely removed by microsurgical technique. No complications were observed. Control MRI showed total tumour removal. The patient remained asymptomatic with no recurrence after 1 year. This case illustrates a successful prior endoscopic approach followed by microsurgery as an alternative to a direct microsurgical approach for intraventricular cavernoma. A previous direct endoscopic view of the lesion and its relation with intraventricular structures may ensure a safe and complete resection by microsurgery. [ABSTRACT FROM AUTHOR]

Background: Cerebral cavernous malformations (CCM) are clusters of dilated sinusoidal channels lined by a single layer of endothelium. In contradistinction to arteriovenous malformations, these lesions do not have smooth muscle or elastin in their lining and they are angiographically occult, and the MRI is the most sensitive test for CCM detection. CCM are one of the most prevalent vascular malformations of the central nervous system, affecting about 0.4-0.6% of the general population. The main complication of this malformation is the risk of bleeding, which may cause neurological deficits that affect the quality of life (QoL) in patients. When symtomatic, they may be surgically treated for relieving the mass effect and seizures refractory to drug uses, hemorrhage and drug-refractory epilepsy. Patient-reported outcome (PRO) may be a strategy that can be used to evaluate QoL of CCM population and was used in a sample of non-operated patients. Methods: An observational, crosssectional analysis to evaluate the PRO using the SF-36 and EuroQol 5 dimensions (EQ-5D) questionnaires of QoL added to functional metrics using the Karnofsky Performance Status (KPS) in 49 patients not submitted to intervention and with long-term follow-up. Results: During the 364 personyears of follow-up, there was an average of individual follow- up of 7.42 years. The mean age was 46.8 years (18-84) - 57% of them were female, 71% had superficial lesions, and 65% had the familial form. Comparisons of SF-36 dimensions with KPS graded <100 had a worse score only in terms of the pain (p = 0.04), vitality (p = 0.001), and general state of health (p = 0.03) domains. The domain mental health was worse in patients without surgical indication (p = 0.032). The functional capacity domain had the highest overall grading in the group. The EQ-5D dimensions of mobility (p = 0.03) and pain/discomfort (p = 0.001) were the ones with lower score compared to KPS <100. Conclusion: The study is the first to evaluate, with validated tools, the PRO of non-operated CCM patients and has demonstrated in a selected group of patients that it was possible to achieve long-term clinical stability, thereby maintaining QoL and functional neurological outcome. [ABSTRACT FROM AUTHOR]

Background and importance: Giant cavernous malformations (GCM) are low flow, angiographically occult vascular lesions, with a diameter >4 cm. Cerebellar GCMs are extremely rare, with only seven cases reported based on English literature. These lesions are most commonly seen in the pediatric age group, which is known to have an increased risk of hemorrhage, being surgery clearly recommended. Clinical presentation: An 18-month-old girl presented with a 6-month history of cervical torticollis and upper extremities clumsiness. An MRI revealed a 57 × 46 × 42 mm multi-cystic, left cerebellar hemisphere mass, showing areas of hemorrhages and cysts with various stages of thrombus. There was no enhancement with contrast. Cerebral angiography ruled out an arteriovenous malformation. She underwent a left paramedian occipital craniotomy, and macroscopic gross total resection was accomplished. Histopathologic examination was consistent with a cavernous malformation. After surgery, the patient had no new neurological deficit and an uneventful postoperative recovery. Follow-up MRI confirmed total removal of the lesion. Conclusion: Cerebellar GCMs in children are symptomatic lesions, which prompt immediate surgical treatment. These are rare lesions, which can radiologically and clinically mimic a tumor with bleed, having to be considered in the differential diagnosis of neoplastic lesions. Cerebellar GCMs might be suspected in the presence of large hemorrhagic intra-axial mass with 'bubbles of blood,' multi-cystic appearance, surrounded by hemosiderin ring, fluid-fluid levels, and accompanying edema-mass effect. Careful radiological study provides a preoperative diagnosis, but its confirmation requires histopathological examination. Complete surgical removal should be attempted when possible. [ABSTRACT FROM AUTHOR]

Background: The clinical criteria for cluster headache (CH) are included in Chapter 3 of the International Classification of Headache Disorders, 3rd beta edition (ICHD-III). CH may sometimes be secondary to other pathologies. Case reports: We report two patients in whom the clinical features of CH initially fulfilled the ICHD-III criteria, but who later presented some radical modifications in headache natural history as a result of a secondary pathology. The first case of CH was secondary to a pontine cavernous angioma and the second to a cerebral venous thrombosis. Conclusion:We highlight the importance of clinical modifications of CH that could suggest clinical investigations should be performed or repeated to exclude a secondary pathology in a previously diagnosed cluster headache. Some of the pathological mechanisms of CH and brain lesions are discussed. [ABSTRACT FROM AUTHOR]

Cavernous malformations (CMs) of the central nervous system are benign, angiographically occult vascular lesions and are diagnosed by magnetic resonance imaging techniques. Giant CMs do not differ from smaller-sized CMs in their clinical, surgical or histopathological presentation but may be radiologically different. In this paper, we report a 10-year-old male with a giant CM of 8 ? 7.5 ? 7 cm in diameter that was completely removed by microsurgical treatment. This case addresses important points for practicing neurosurgeons toconsider when making a differential diagnosis of large intracranial mass lesions in pediatric patients. In this report, the radiological features of this vascular malformation are described based on a pertinent literature review. [ABSTRACT FROM AUTHOR]

Introduction: Intraventricular cavernous angiomas are rare pathological entities, and those located at the foramen of Monro are even rarer. We herein present a case of cavernous angioma at the foramen of Monro that was successfully treated by neuroendoscope-assisted surgical removal, and review the relevant literature. Case presentation: A 65-year-old woman had experienced headache and vomiting for 10 days before admission to another hospital. Magnetic resonance imaging (MRI) showed a mass at the foramen of Monro, and obstructive hydrocephalus of both lateral ventricles. The patient was then referred to our hospital. Neurological examination on admission to our hospital showed memory disturbance (Mini-Mental State Examination 20/30) and wide-based gait. A cavernous angioma at the foramen of Monro was diagnosed based on the typical popcorn-like appearance of the lesion on MRI. The lesion was completely removed by neuroendoscope-assisted transcortical surgery with the Viewsite Brain Access System (Vycor Medical Inc., Boca Raton, FL), leading to a reduction in the size of the ventricles. The resected mass was histologically confirmed to be cavernous angioma. The patient's symptoms resolved immediately and there were no postoperative complications. Conclusion: Minimally invasive neuroendoscope-assisted surgery was used to successfully treat a cavernous angioma at the foramen of Monro. [ABSTRACT FROM AUTHOR]

Cavernous angiomas originating in the internal auditory canal are very rare. In the available literature, only 65 cases of cavernomas in this location have been previously reported. We describe the case of a 22-year-old woman surgically treated for a cavernous hemangioma in the left internal auditory canal, mimicking on preoperative magnetic resonance imaging MRI an acoustic neuroma. Neurological symptoms were hypoacusia and dizziness. The cavernous angioma encased the seventh and, partially, the eighth cranial nerve complex. A 'nearly total' removal was performed, leaving a thin residual of malformation adherent to the facial nerve. Postoperative period was uneventful; hearing was unchanged, but the patient had a moderate inferior left facial palsy (House-Brackmann grade II) slightly improved during the following weeks. On the basis of the observation of this uncommon case, we propose a revision of the literature and discuss clinical features, differential diagnosis, and treatment. [ABSTRACT FROM AUTHOR]

Purpose: While cavernous angioma (CVA) after cranial irradiation has been documented, its development after high-dose chemotherapy with autologous peripheral blood stem cell transplantation (PBSCT) has not. We present a patient with desmoplastic/nodular medulloblastoma (DNMB) associated with anhidrotic ectodermal dysplasia (AED) who developed CVA 2 years after high-dose chemotherapy and PBSCT. Methods: A 1-year-old boy with ingravescent vomiting was admitted to our institute. He presented with a large head, a depressed nasal bridge, low-set ears, thick lips with peg-shaped teeth, hypohidrosis, sparse hair, thin atrophic skin, scaly dermatitis with frontal bossing, and a bulging anterior fontanel. Neuroradiological examination revealed multiple cerebellar masses with heterogeneous enhancement and speckled calcifications and severe obstructive hydrocephalus. The histological diagnosis of surgical specimens was DNMB, and he underwent postoperative multiple-drug chemotherapy with autologous PBSCT. The outcome was favorable and he did not undergo radiotherapy. Results: After 2 years, intracranial hemorrhage was detected at his regular radiological check-up and he again underwent surgery. The histological diagnosis was CVA. Conclusions: To our knowledge, this is the first report of AED-associated DNMB and CVA. [ABSTRACT FROM AUTHOR]

Cerebral cavernous angiomas are vascular malformations characterized by large adjacent vessels. Usually, these lesions are smaller than 3 cm, the mean age at presentation occurs between 20 and 40 years, and the neuroradiological findings are well described, especially for magnetic resonance imaging, where the “popcorn balls” appearance is due to the presence of locules containing blood. Among these, the giant cavernous angiomas are very rare, particularly in adults. We collected clinical and neuroradiological data from clinical file and hospital diagnostic archive. A comprehensive review of similar cases was performed. We describe the clinical, diagnostic, and surgical management of a giant cerebral cavernous angioma located in the left deep frontal lobe mimicking a high-grade glioma in an adult Chinese patient. Giant cerebral cavernous angioma may be misdiagnosed and should be considered as differential diagnosis. [ABSTRACT FROM AUTHOR]

Background: We report a case of progressive multiple cavernous angiomas. Patient and Method: A 16-year-old boy presented with a 2-month history of headache and dizziness. Six years earlier, he underwent surgery for cerebellar medulloblastoma and subsequent chemoradiotherapy according to the M-051 protocol of the Korean Society of Pediatric Neuro-Oncology. Follow-up brain magnetic resonance imaging (MRI) revealed a tiny hemorrhage on the cerebellum 3 months after 23.4-Gy craniospinal and 32.4-Gy boost radiotherapy. Result: The multiple hemorrhagic lesions had progressively developed on the whole brain without any symptoms for 6 years. On admission, MRI revealed a 1.5-cm enlarged mass with subacute hemorrhage on the right frontal area. The mass was totally removed and diagnosed as cavernous angioma. However, 5 months later, the patient complained of a headache. MRI revealed 1.4- and 0.7-cm enlarged masses on the left frontal and right temporal areas with internal hemorrhage, respectively. The left frontal mass was totally removed, and the histopathological finding was suggestive of cavernous angioma. Conclusion: This case showed early-developed multiple hemorrhagic lesions after radiotherapy, which had been progressive and were associated with some symptomatic cavernous angiomas. Pediatric patients with brain radiotherapy should undergo radiological check-up to identify vascular lesions, especially symptomatic patients. © 2015 S. Karger AG, Basel [ABSTRACT FROM AUTHOR]

Purpose: The purpose of this study is to investigate the incidence of cavernous angioma (CVA) in long-term survivors of childhood embryonal tumors treated by cranial irradiation. Materials and methods: Between 1990 and 2012, we treated 25 patients (13 males, 12 females) with embryonal tumors (17 medulloblastomas, 5 primitive neuroectodermal tumors (PNET), 3 pineoblastomas) with craniospinal irradiation. Follow-up ranged from 15.5 to 289.9 months, the irradiation dose to the whole neural axis from 18 to 36 Gy, and the total local dose from 49.6 to 60 Gy. All patients underwent follow-up magnetic resonance imaging (MRI) studies at least once a year, and the diagnosis of posttreatment CVA was based solely on MRI findings. Results: At the time of this writing, 18 were alive and free of the recurrence of the original disease or the development of secondary neoplasms other than CVA; another 2 were alive with medulloblastoma or diffuse astrocytoma. Posttreatment, 14 patients developed CVAs in the course of a median of 56.7 months; 13 of these presented with multiple CVAs. Patients who underwent radiation therapy (RT) at an age younger than 6 years developed multiple CVAs significantly earlier than those treated at a later age ( p = 0.0110). Patients with PNET or pineoblastoma developed Zabramski type 1 and 2 CVA significantly earlier than did medulloblastoma patients ( p = 0.0042). Conclusion: We attribute the high rate of post-RT CVA in our long-term follow-up study of pediatric patients to the delivery of cranial irradiation for embryonal tumors, especially PNET and pineoblastoma, and recommend the regular, long-term follow-up of patients whose embryonal tumors were treated by cranial irradiation. [ABSTRACT FROM AUTHOR]

The telovelar approach and its modifications are widely used to remove lesions of the fourth ventricle and brainstem. We offer a variant of a unilateral uvulotonsillar approach, which is performed using an open-door keyhole suboccipital craniotomy (Video 1). A 56-year-old woman presented with symptoms of 2 months' duration. Preoperative examination revealed paresis of the left sixth cranial nerve, left seventh nerve paralysis, right-sided hemihypoesthesia, right-sided severe hemiparesis owing to which the patient could not move independently, and dynamic ataxia in the left limbs. Magnetic resonance imaging showed a cavernoma with hemorrhage in the left side of the pons. The surgery was performed in the prone position. After a median skin and soft tissue incision about 6–7 cm long, an open-door keyhole craniotomy was performed. Dura over the left cerebellar hemisphere was opened in a semilunar fashion. The dissection of the uvulotonsillar fissure was performed from its cranial part, where the medial trunk of the cortical segment of the posterior inferior cerebellar artery was the reference point. Following the dissection, the tela choroidea was incised, and the left half of the rhomboid fossa was exposed. After the mapping, the floor of the fourth ventricle was incised in the suprafacial triangle. Cavernoma and hematoma were removed. Hemostasis was achieved. Dura was closed. The attached bone flap was then turned upward and reattached using nonresorbable sutures. Postoperatively, the patient had resolution of sensory and motor disturbances on the right half of the body, and coordination was improved in the left limbs. Postoperative imaging confirmed complete removal of the lesion. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

Objective: To analyze the outcome of gamma knife radiosurgery (GKS) using low marginal dose for the symptomatic brain stem cavernous angioma (BSCA). Methods: 39 patients (16 males, 23 females) were treated with GKS for BSCA from January 1997 to September 2012. Clinical data were analyzed retrospectively. The mean age was 41.5 years. All patients had a history of symptomatic bleeding once or more before performing GKS. Mean volume of BSCA was 1095.3 mm3 and median prescribed marginal dose was 13 Gy. Results: Mean follow-up period since diagnosis was 4.1 years. The number of hemorrhagic events between initial diagnosis and GKS was 5 over a total of 14.9 patients-years with annual hemorrhagic rate of 33.6%. Following GKS, there were five hemorrhagic events within the first 2 years (8.1%/year) and two after the first 2 years (2.4%/year). The difference was not statistically significant. Neurologic status improved in 24 patients (61.5%), and stationary in eleven (28.2%). 4 patients (10.3%) experienced the exacerbation of symptoms at the last follow-up and none of them were related to the radiation injury. Significant volume reduction after GKS was observed in 24 patients (61.5%). Surgical excision was performed in one patient due to swelling and rebleeding after GKS. Age at presentation, sex, mass size of BSCA, and location, GKS dose did not affect post-GKS hemorrhage. Conclusions: GKS for BSCA using relatively low marginal dose is safe and effective. Long-term prospective study is needed to confirm the optimal dose for BSCA. [ABSTRACT FROM AUTHOR]

Highlights: [•] A quantified study of SEEG seizures in drug resistant epilepsies with cavernoma is proposed. [•] The “epileptogenicity index” (EI) is a tool that allows mathematical quantification of rapid discharges at seizure onset. [•] Epileptogenicity extended beyond the limits of the lesion in 5/6 patients. [•] Secondary epileptogenesis process could be involved in the build-up of these complex networks. [ABSTRACT FROM AUTHOR]

Glioblastoma ( GBM) is the most common malignant CNS neoplasm, the prognosis of which remains poor even after multidisciplinary treatment. The 5-year overall survival rate of GBM is less than 10% and has remained unchanged for more than 50 years. Because GBM patients rarely survive over a decade, only very few cases of delayed complications caused by therapy have been reported. Here, we report the case of a 24-year-old man who is still alive 21 years after surgical resection and chemoradiotherapy for GBM. This patient developed a cavernous angioma 19 years after the initial surgery as a delayed complication of radiotherapy. The diagnosis of the initial tumor was confirmed by histopathological review, which indicated that the tumor had immunohistochemical and genetic profiles consistent with GBM. Long-term survival in the case of this GBM patient likely resulted from a combination of factors, including hypermethylation of the MGMT ( O6-methyl guanine methyl transferase) CpG island, young age at diagnosis, good performance status, and complete surgical resection of the tumor. To the best of our knowledge, this case report describes one of the longest-surviving GBM patients and is the first on radiation-induced cavernous angioma in a GBM patient. [ABSTRACT FROM AUTHOR]