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Kulwin, Charles G., Troy D. Payner, Rick F. Nelson, Laurie L. Ackerman, and Daniel H. Fulkerson. "Pediatric Pontine Cavernous Malformations: The Presigmoid, Posterior Petrosal Approach." Operative Neurosurgery 15, no. 5 (March 3, 2018): 522–29. http://dx.doi.org/10.1093/ons/opy007.
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Abstract BACKGROUND Brainstem cavernous malformations (cavernomas) in children have a high risk of hemorrhage and neurological deterioration. This risk is magnified if the child has a genetic predisposition for cavernoma formation. The surgical management is challenging and carries a significant risk of morbidity. OBJECTIVE To describe the feasibility of a posterior petrosal approach to brainstem cavernomas in a pediatric population. METHODS A single institution operative experience with this technique was reviewed; 2 cases were identified and are technically described here with supportive figures and illustrations, as well as a focused literature review. RESULTS Two pediatric cases with multiple symptomatic hemorrhages from large expanding pontine cavernomas were identified. Both cavernomas were resected through a presigmoid posterior petrosal approach. While this approach is well described in the adult literature for ventral brainstem lesions, its description for the treatment of pontine cavernomas in the pediatric populations is scarce. CONCLUSION This study demonstrates the utility and feasibility of the posterior petrosal approach in two pediatric patients at different points in cranial base development.
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Gorgan, R. M., F. Brehar, M. Catana, V. Pruna, Ana Gheorghiu, G. Popescu, Catioara Cristescu, and A. Giovani. "Surgical management of symptomatic spinal cord and intracerebral cavernomas in a multiple cavernomas case." Romanian Neurosurgery 30, no. 1 (March 1, 2016): 28–34. http://dx.doi.org/10.1515/romneu-2016-0004.
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Abstract Multiple cavernous malformations are associated with familial cases and are present in 10-20% of all cavernoma cases. 5% of cavernomas are located intramedullary and of these only 10% present multiple cavernomas. With the availability of echo gradient MRI the cases of multiple cavernomas are diagnosed earlier and it is not rare that it uncovers multiple cavernomas in cases where only a single lesion can be identified on regular MRI sequences. We present the case of a 55 years old woman presented with a two years history of mild backache, followed by progressive lower legs motor deficit and urinary retention. The spine MRI showed an intramedullary T2/3 lesion and the cerebral MRI established the diagnosis of multiple cavernomas. One year after the intramedullary cavernoma was operated with success, she developed generalized seizures and a new cerebral MRI showed bleeding and volume growth of one right temporal pole cavernoma. The cerebral lesion was resected successfully and the patient was discharged free of seizures. This familial type multiple cavernomas cases should be screened and followed with repeated brain and spine MRI’s every year.
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Jagathesan, Tania, and Michael OBrien. "Aeromedical Implications of Cerebral Cavernomas." Aerospace Medicine and Human Performance 92, no. 2 (February 1, 2021): 120–23. http://dx.doi.org/10.3357/amhp.5747.2021.
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BACKGROUND: Cavernomas, cavernous angiomas, or cerebral cavernous malformations are clusters of endothelium-lined blood vessels usually found in the brain. With the increasing use of radiological imaging, these are being detected incidentally in asymptomatic aircrew. The UK Civil Aviation Authority (CAA) experience of cavernomas is described and the aeromedical concerns, that is, the risk of epilepsy, hemorrhage, and the development of a neurological deficit, are considered.METHODS: A search of the CAA database between 1990 and 2020 was performed for the term cavernoma. The gender, age at diagnosis, class of certification held, clinical presentation, location, and size of the lesion were noted. A PubMed literature review for papers with complications of cavernoma was performed.RESULTS: Six cases of cavernoma have been declared to the CAA: five professional pilots and one private pilot. Five were men and one was a woman. The age range was between 38 and 60 yr, with a mean of 48 yr. Two cases presented with clinical symptoms and four were asymptomatic. Complication rates for seizure and hemorrhage were extracted from the published literature together with the significance of other factors such as cavernoma size, family history, multiplicity, and the development of new lesions.DISCUSSION: A policy for the medical certification of aircrew with cavernomas that have presented with clinical symptoms and those that are detected incidentally is proposed.Jagathesan T, OBrien M. Aeromedical implications of cerebral cavernomas. Aerosp Med Hum Perform. 2021; 92(2):120123.
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Perna, Giuseppe Di, Fabio Cofano, Roberto Altieri, Bianca Maria Baldassarre, Luca Bertero, Francesco Zenga, and Diego Garbossa. "III cranial nerve cavernous malformation: A case report and review of the literature." Surgical Neurology International 11 (December 22, 2020): 452. http://dx.doi.org/10.25259/sni_650_2020.
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Background: Cavernous malformations generally occur in brain parenchyma but rarely these lesions arise from cranial nerves (CNs). Case Description: This paper described a case of a woman presented with III CN dysfunction due to the presence of a right III CN cavernoma. Surgical treatment with nerve sparing gross total resection was performed. A 3-month follow-up was documented. Conclusion: Only few cases of CNs cavernomas have been described in the literature. These lesions have been described to show a more aggressive behavior compared to intraparenchymal cavernomas, especially in symptomatic patients. Differential diagnosis and surgical treatment could be challenging, especially trying to preserve nerve integrity and function.
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Iacob, G., and Angela Olarescu. "Spinal intramedullary cavernomas. Personal experience reffering to six cases." Romanian Neurosurgery 21, no. 4 (December 1, 2014): 407–15. http://dx.doi.org/10.2478/romneu-2014-0056.
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Abstract Despite cavernous malformations of the CNS are pathologically similar, intramedullary cavernous malformations are very rare lesions, increasingly recognized after introduction of magnetic resonance image, generating gradual neurological decline, with severe deficits or acute loss of spinal function. We report our experience on six patients with intramedullary cavernomas defining the spectrum of presenting symptoms and signs analyzing the role of surgery as a treatment for these lesions. We present our experience with 2 cervical and 4 thoracal spinal intramedullary cavernoma from 2010 to 2014 searching history, onset of clinical manifestation, neurological status, radiological findings, operation, and clinical outcome. Among 6 patients male were 2 cases; female 4 cases; mean age was 42 years (range 25-72 years); mean duration of symptoms were 1,5 years (range 5 days and 2 years) with slowly progressive neurological decline. In two cases there was acute onset of neurological compromise. In all cases diagnosis was made on MRI and lesions were possible to be radically excised and gently extracted from the hemosiderin-stained bed inside of the spinal cord via a laminectomy and midline myelotomy with microsurgical techniques. The surgical outcome on a mean duration of follow up of 12 months were: for 4 cases - the patients neurological conditions remarkably improved 1 month later, for 2 cases no improvement were remarked. No recurrent hemorrhages were recorded. A follow-up MRI examination was made in all cases to confirm complete removal of the cavernous angioma. Spinal intramedullary cavernoma should be early recognized by MRI, can be positioned in a precarious position and generate significant neurologic deficits than cranial cavernomas. For symptomatic intramedullary cavernous malformations extended to the dorsal surface of the spinal cord, total resection with microsurgical techniques can offer good or excellent outcome, restoring neurological status and to stop chronic deterioration and acute rebleeding. To asymptomatic patients with deeper lesions which entail a higher operative risk, but also a surgically manageable cause of myelopathy a closed observation is mandatory.
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Kivelev, Juri, Mika Niemelä, Riku Kivisaari, Reza Dashti, Aki Laakso, and Juha Hernesniemi. "LONG-TERM OUTCOME OF PATIENTS WITH MULTIPLE CEREBRAL CAVERNOUS MALFORMATIONS." Neurosurgery 65, no. 3 (September 1, 2009): 450–55. http://dx.doi.org/10.1227/01.neu.0000346269.59554.db.
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Abstract OBJECTIVE Multiple cerebral cavernous malformations (MCCMs) typically occur in patients with a family history of these lesions. Literature on MCCMs is scarce, and little is known about their natural history. METHODS Of 264 consecutive patients with cerebral cavernomas treated at the Department of Neurosurgery, Helsinki University Central Hospital, in the past 27 years, 33 patients had MCCMs. Lesions were categorized according to the Zabramski classification scale. Follow-up questionnaires were sent to all patients. Outcome was assessed using the Glasgow Outcome Scale, and amelioration of epilepsy was assessed using the Engel scale. All clinical data were analyzed retrospectively. RESULTS The mean age of patients at diagnosis was 44 years. Sex presentation was almost equal. Nine percent of all patients had a family history of the disease. Patients presented with epilepsy, acute headache, and focal neurological deficits. MCCMs were incidental findings in 2 patients. Altogether, 416 cavernomas were found: 70% supratentorial and 30% infratentorial. Fifteen patients had symptomatic hemorrhage before admission to our department. Surgery was performed on 18 patients. In most cases, the largest cavernoma was removed. Postoperatively, 1 patient experienced temporary hemiparesis, and another developed permanent motor dysphasia. No mortalities occurred. The mean follow-up time was 7.7 years. Twenty-six patients (79%) were in good condition. Among patients with epilepsy who underwent lesionectomy, 70% had an Engel class I outcome. On follow-up magnetic resonance imaging, 52 de novo cavernomas were found. CONCLUSION Surgical treatment of patients with MCCMs is safe. An extirpation of the clinically active cavernoma prevents further bleedings and improves outcome of epilepsy.
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Fanous, Andrew A., Patrick K. Jowdy, Lindsay J. Lipinski, Lucia L. Balos, and Veetai Li. "Association between trauma and acute hemorrhage of cavernous malformations in children: report of 3 cases." Journal of Neurosurgery: Pediatrics 18, no. 3 (September 2016): 263–68. http://dx.doi.org/10.3171/2016.3.peds15517.
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OBJECTIVE Cavernous hemangiomas are benign congenital vascular abnormalities. Intracerebral cavernous hemangiomas have an appreciable risk of spontaneous hemorrhage. Little is known as to whether head trauma increases the risk of bleeding for these lesions. In this study, the authors present a case series of 3 patients with posttraumatic nonspontaneous hemorrhage of intracerebral cavernous malformations (CMs). For the first time, to the authors' knowledge, they propose that trauma might constitute a risk factor for acute hemorrhage in intracerebral cavernomas. METHODS The authors reviewed the charts of all patients with a new diagnosis of intracerebral cavernoma at their pediatric hospital between 2010 and 2014. Patients with a history of head trauma prior to presentation were subsequently studied to identify features common to these posttraumatic, hemorrhage-prone lesions. RESULTS A history of head trauma was identified in 3 of 19 cases. These 3 patients presented with seizures and/or headaches and were found to have acute hemorrhage within a cavernous hemangioma. None of these patients had any history of abnormal neurological symptoms. All 3 abnormal vascular lesions had associated developmental venous anomalies (DVAs). The 3 patients underwent resection of their respective vascular abnormalities, and the diagnosis of cavernous hemangioma was confirmed with postsurgical tissue pathology. All 3 patients had complete resolution of symptoms following complete excision of their lesions. CONCLUSIONS Trauma may represent a risk factor for acute hemorrhage in patients with CMs. The presence of associated DVAs may represent a risk factor for posttraumatic hemorrhage of cavernomas. Excision should be considered in such cases, if feasible.
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De Souza, Jorge Marcondes, Flavio S. Domingues, Leila Chimelli, and Judith Gault. "Spinal root arteriovenous malformations and same-segment cord cavernous malformation in familial cerebral cavernous malformation." Journal of Neurosurgery: Spine 9, no. 3 (September 2008): 249–52. http://dx.doi.org/10.3171/spi/2008/9/9/249.
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Spinal vascular malformations are uncommon lesions, and controversy persists regarding optimal investigation, classification, and treatment strategies. The authors report on a patient with a spinal root arteriovenous malformation (AVM) associated with a parenchymal cavernous malformation (CM) in the same spinal cord segment and describe a complete familial and molecular investigation. This 35-year-old woman presented with symptoms of progressive clinical spastic paraparesis. Magnetic resonance imaging results were suggestive of a spinal cord cavernoma associated with cerebral CMs. Her family history included 2 sisters treated for epilepsy. At surgery an intraspinal root AVM was found at the same level of the cord CM, and both lesions were completely removed. Cerebral gradient echo MR imaging disclosed multiple cavernomas in her relatives, which prompted the molecular diagnosis. On sequence analysis, a novel mutation on the cerebral CM1 (CCM1) gene (c796insA) was found. The authors report on a unique case of familial cerebral CM in which a spinal root AVM was situated next to a cord CM, and discuss the concomitant occurrence of altered nervous system angiogenesis and vasculogenesis.
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Erkmen, Kadir, and Ossama Al-Mefty. "Transcondylar Approach for Resection of Medullary Cavernous Malformation: 2-Dimensional Operative Video." Operative Neurosurgery 21, no. 3 (June 30, 2021): E233—E234. http://dx.doi.org/10.1093/ons/opab220.
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Abstract Cavernous malformations located within the brainstem present with a high rate of neurological symptoms and carry a more aggressive course in both pediatric and adult populations.1,2 Cavernomas within the medulla are the rarest form, representing only 5% of all brainstem lesions.3 Repeated hemorrhage of brainstem cavernomas is associated with significant and cumulative neurological deficits and thus requires treatment.4 Microsurgical resection has become the optimal mode of treatment with the aim of resecting the live malformation and not merely the multiaged, organized hematoma.4 This is best achieved by approaching the cavernoma at the location where it projects to the surface and entering the lesion through a safe brainstem anatomic zone. For ventrally located lesions in the medulla, a transcondylar skull base approach provides a direct trajectory to the entry zone through a short surgical distance without the need to manipulate or retract neurovascular structures.5-8 Neuronavigation and intraoperative neurophysiological monitoring of somatosensory evoked potential, motor, and lower cranial nerves are adjuncts to increase patient safety. Radiosurgery for the treatment of brainstem cavernous malformations has been proposed; however, it demonstrates high risk and variable and often poor response rates.9 We present a surgical video demonstrating the transcondylar approach and resection of a medullary cavernoma in a 54-yr-old woman who has had multiple known prior hemorrhages and presented with a new onset of facial numbness and weakness, ataxia, and left body sensory loss. The patient consented to surgery and to photograph publication. Images at 1:28, 1:43 (left), 2:02 from Al-Mefty O, Operative Atlas of Meningiomas, © LWW, 1997,5 with permission. Images at 1:43 (right) from Arnautovic et al,8 with permission from JNSPG.
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Schmidt, Tyler, Michelle Lawson, Emily Silberstein, Jonathan Jay Stone, and Howard J. Silberstein. "335 Radiation Induced Cerebral Cavernomas in Pediatric Neuro-Oncology: A 25 Year Single Institution Review." Neurosurgery 64, CN_suppl_1 (August 24, 2017): 274–75. http://dx.doi.org/10.1093/neuros/nyx417.335.
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Abstract INTRODUCTION Radiation has a significant role in the management of pediatric brain tumors. Secondary complications to irradiation can include vascular malformations such as cavernous malformations, brain atrophy, necrosis, and demyelination. Although recognized as a potential complication, the pathogenesis and natural history of radiation induced cavernous malformations continues to be defined. We present a retrospective, single institution study of 138 patients who underwent cerebral radiotherapy as part of their comprehensive pediatric brain tumor management. METHODS Institutional review board permission was obtained and a retrospective review of all available patients with a pediatric brain tumor at our institution was completed. Patients who underwent radiotherapy and developed cavernous malformations were selected and the initial tumor type, frequency, and the radiological as well as clinical history were recorded and analyzed. RESULTS >From 1980–2005, 134 patients received radiotherapy for a pediatric brain tumor. Of this cohort, 28 patients (21%) subsequently developed at least one cerebral cavernous malformation. 21 of the patients had multiple cavernomas while only 7 were solitary. 15 patients were male. The mean time until discovery was 13.5 years. The tumor biology included 14 gliomas, 9 medulloblastomas, and 1 ependymoma. 14 patients received chemotherapy and all received at least short term corticosteroids. One patient required surgical excision of a cavernous malformation for repeated symptomatic hemorrhages. CONCLUSION Cavernous malformations are a frequent long term sequalae of pediatric radiotherapy. Although common, they are rarely associated with neurological complications even decades later and are largely discovered asymptomatically during routine surveillance screening of their primary disease. Future research in our database will be focused on establishing the relationship of radiation dose, age at irradiation, localization of radiotherapy, chemotherapy, anti-epileptics, and other medications on radiation induced cavernous malformations with the hope of identifying modifiable risk factors for clinical decision making application in a prospective fashion.
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Karri, Sudhir Babu, Megha S. Uppin, A. Rajesh, K. Ashish, Suchanda Bhattacharjee, Y. Jyotsna Rani, B. P. Sahu, M. Vijaya Saradhi, A. K. Purohit, and Sundaram Challa. "Vascular malformations of central nervous system: A series from tertiary care hospital in South India." Journal of Neurosciences in Rural Practice 07, no. 02 (April 2016): 262–68. http://dx.doi.org/10.4103/0976-3147.176196.
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ABSTRACT Aims and Objectives: To describe clinicopathological features of surgically resected vascular malformations (VMs) of central nervous system (CNS). Materials and Methods: Histologically diagnosed cases of VMs of CNS during April 2010–April 2014 were included. Demographic data, clinical and radiological features were obtained. Hematoxylin and eosin slides were reviewed along with Verhoeff-Van Gieson (VVG), Masson’s trichrome, periodic acid-Schiff, and Perls’ stains. Morphologically, cavernomas and arteriovenous malformations (AVMs) were distinguished on the basis of vessel wall features on VVG and intervening glial parenchyma. Results: Fifty cases were diagnosed as VMs of CNS with an age range of 14–62 years. These included 36 cavernomas, 12 AVMs, 2 mixed capillary-cavernous angiomas. Most of the cavernoma patients (15/36) presented with seizures, whereas AVM patients (8/12) had a headache as the dominant symptom. Twenty-nine patients were reliably diagnosed on radiological features. Microscopic evidence of hemorrhage was seen in 24/36 cavernomas and 6/12 AVMs, as opposed to radiologic evidence of 10 and 4, respectively. Reactive gliosis was seen in 16 cavernomas. Conclusions: Histological features are important for classifying the VMs of CNS as there are no specific clinical and radiological features. Type of VM has a bearing on management, prognosis, and risk of hemorrhage.
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Chakravarthy, Hariprakash, Tzu-Kang Lin, Yao-Liang Chen, Yi-Ming Wu, Chin-Hua Yeh, and Ho-Fai Wong. "De novo formation of cerebral cavernous malformation adjacent to existing developmental venous anomaly – an effect of change in venous pressure associated with management of a complex dural arterio-venous fistula." Neuroradiology Journal 29, no. 6 (September 26, 2016): 458–64. http://dx.doi.org/10.1177/1971400916666558.
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This is a case report of de novo development of two cerebral cavernous malformations adjacent to existing developmental venous anomalies. The development of cavernomas was noted over a follow-up period of 10 years. These developments happened during the course of staged endovascular management of a complex dural arterio-venous fistula along the right sphenoid wing. The patient presented with a proptosis secondary to lympho-haemangiomatous lesion of the fronto-orbital region and a high-flow right sphenoid wing dural arterio-venous fistula. During the initial period of conservative management of the dural arterio-venous fistula, he developed de novo cavernous malformations in the left mesial temporal lobe adjacent to a developmental venous anomaly in the temporal lobe, and along with this there was engorgement of deep veins related to another existing developmental venous anomaly in the brainstem. Later during the course of endovascular treatment of the dural arterio-venous fistula, a large brainstem cavernoma developed adjacent to the brainstem developmental venous anomaly. This case report discusses the cause-effect relationship of venous pressure changes related to management of dural arterio-venous fistula and de novo formation of cerebral cavernous malformations adjacent to existing developmental venous anomalies.
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Al-Holou, Wajd N., Thomas M. O'Lynnger, Aditya S. Pandey, Joseph J. Gemmete, B. Gregory Thompson, Karin M. Muraszko, Hugh J. L. Garton, and Cormac O. Maher. "Natural history and imaging prevalence of cavernous malformations in children and young adults." Journal of Neurosurgery: Pediatrics 9, no. 2 (February 2012): 198–205. http://dx.doi.org/10.3171/2011.11.peds11390.
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Object This study was undertaken to define the age-related prevalence of cavernous malformations (CMs) in children and young adults undergoing intracranial imaging. In addition, the authors aim to clarify the natural history of CMs in young people, especially in those with incidentally discovered lesions. Methods To identify those patients with CMs, the authors retrospectively reviewed the electronic medical records of 14,936 consecutive patients 25 years of age or younger who had undergone brain MR imaging. In patients with a CM, clinical and imaging data were collected. Patients with untreated cavernomas who had more than 6 months of clinical and MR imaging follow-up were included in a natural history analysis. The natural history analysis included 110 CMs in 56 patients with a 3.5-year mean clinical follow-up interval (199 patient-years and 361 cavernoma-years). Results In 92 patients (0.6%), 164 CMs were identified. The imaging prevalence of cavernomas increased with advancing age (p = 0.002). Multiple CMs occurred in 28 patients (30%), and 8 patients (9%) had a family history of multiple CMs. Fifty patients (54%) presented with symptoms related to the cavernoma, of whom 30 presented with hemorrhage (33%). Of the 164 cavernomas identified, 103 (63%) were considered incidental, asymptomatic lesions. Larger size was associated with acute symptomatic presentation (p = 0.0001). During the follow-up interval, 6 patients with 8 cavernomas developed 11 symptomatic hemorrhages after initial identification. Five of the patients who had a hemorrhage during the follow-up interval had initially presented with hemorrhage, while only 1 had presented incidentally. The hemorrhage rate for all patients in the natural history group was 1.6% per patient-year and 0.9% per cavernoma-year. The hemorrhage rate was 8.0% per patient-year in the symptomatic group versus 0.2% in the incidental group. Symptomatic hemorrhage after long-term follow-up was associated with initial acute presentation (p = 0.02). Conclusions The imaging prevalence of CM increases with advancing age during childhood. Patients presenting without hemorrhage have a significantly lower risk of bleeding compared with those who present with acute neurological symptoms. Comparing this series of children to prior analyses of CM natural history in adults, the authors' data do not suggest a higher bleeding risk in younger patients.
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Zaidi, Hasan A., Shakeel A. Chowdhry, Peter Nakaji, Adib A. Abla, and Robert F. Spetzler. "Contralateral Interhemispheric Approach to Deep-Seated Cavernous Malformations." Neurosurgery 75, no. 1 (March 10, 2014): 80–86. http://dx.doi.org/10.1227/neu.0000000000000339.
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Abstract BACKGROUND: Deep-seated periventricular cavernous malformations of the basal ganglia or thalamus can be approached via an interhemispheric craniotomy. OBJECTIVE: To determine surgical efficacy and clinical outcomes of the contralateral interhemispheric approach. METHODS: Retrospective chart review was performed on patients undergoing an interhemispheric approach for the resection of deep-seated cavernous malformation by the senior author (R.F.S.) between 2005 and 2013. Demographic data and clinical outcomes were reviewed. Pre- and postoperative imaging were analyzed for lesion location, size, associated venous anomaly, proximity to ventricle, and presence of residual. RESULTS: Twenty-one patients underwent a contralateral interhemispheric-transventricular approach, 7 patients had a contralateral interhemispheric-transcingulate approach and 3 patients had a contralateral interhemispheric-transchoroidal approach. Mean age was 40.1 years, and the majority were female (58.1%). Mean maximum cavernoma diameter was 1.97 cm, and 43.8% reached the surface of the ventricle. Average follow-up was 8.9 months, with complete resection achieved in 96.8% of patients. At last follow-up, 61.3% of patients remained stable and 29.0% had improved. Of the patients, 6.5% experienced transient weakness that resolved at last follow-up, and 1 patient (3.2%) had short-term memory problems. There were no surgical mortalities. CONCLUSION: The contralateral interhemispheric approach is a safe, clinically well tolerated, and surgically efficacious approach to deep-seated cavernomas.
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Roser, Florian, Luigi Rigante, and Mohamed Samy Elhammady. "Endoscope-assisted resection of a brainstem cavernoma." Neurosurgical Focus: Video 1, no. 1 (July 2019): V6. http://dx.doi.org/10.3171/2019.7.focusvid.19158.
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Procedures on cavernous malformations of the brainstem are challenging due to their eloquent location. This accounts especially for recurrent cavernomas as surgical scars, adhesions, and functional shift might have occurred since primary surgery. We report on a 38-year-old female patient with a large recurrent brainstem cavernoma, who underwent previous successful surgery and experienced recurrent bleeding about 2 years later. She harbored a large associated developmental venous anomaly (DVA) traversing the cavernoma through the midline of the brainstem. In order to visualize complete resection and preservation of the DVA at the same time, endoscopic-assisted resection within the brainstem after decompression in the semisitting position was performed.The video can be found here: https://youtu.be/K1p-Sx7jUpA.
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SASARAN, A., A. MOHAN, H. A. MOISA, and A. V. CIUREA. "The microsurgical solution in supratentorial cavernous angiomas." Romanian Journal of Medical Practice 10, no. 3 (September 30, 2015): 288–94. http://dx.doi.org/10.37897/rjmp.2015.3.14.
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Cavernous angiomas of the central nervous system are rare, neurovascular lesions usually detected in patients aged 20-50 and represent up to 8-15% of all intracranial malformations. The association between cavernomas and arteriovenous malformations is met in 10-30% of cases. Treatment strategies for intracranial cavernomas include conservative management, open microsurgery and finally stereotactic radiosurgery (the use of which is still highly debatable as there are no available methods to determine its short term effects). The authors present their surgical experience in what regards a cohort of 149 consecutive patients, diagnosed with intracranial and supratentorial cavernomas, admitted, investigated and treated between January 2000 and January 2015. The clinical particularities of the patients together with the surgical approaches are debated. Last but not least the outcome and quality of life of the patients is discussed.
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Mouchtouris, Nikolaos, Nohra Chalouhi, Ameet Chitale, Robert M. Starke, Stavropoula I. Tjoumakaris, Robert H. Rosenwasser, and Pascal M. Jabbour. "Management of Cerebral Cavernous Malformations: From Diagnosis to Treatment." Scientific World Journal 2015 (2015): 1–8. http://dx.doi.org/10.1155/2015/808314.
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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.
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Mazza, C., R. Scienza, A. Beltramello, and R. Da Pian. "Cerebral cavernous malformations (cavernomas) in the pediatric age-group." Child's Nervous System 7, no. 3 (June 1991): 139–46. http://dx.doi.org/10.1007/bf00776709.
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Dalyai, Richard T., George Ghobrial, Issam Awad, Stavropoula Tjoumakaris, L. Fernando Gonzalez, Aaron S. Dumont, Nohra Chalouhi, Ciro Randazzo, Robert Rosenwasser, and Pascal Jabbour. "Management of incidental cavernous malformations: a review." Neurosurgical Focus 31, no. 6 (December 2011): E5. http://dx.doi.org/10.3171/2011.9.focus11211.
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Cavernous malformations (CMs) are angiographically occult vascular malformations that are frequently found incidentally on MR imaging. Despite this benign presentation, these lesions could cause symptomatic intracranial hemorrhage, seizures, and focal neurological deficits. Cavernomas can be managed conservatively with neuroimaging studies, surgically with lesion removal, or with radiosurgery. Considering recent studies examining the CM's natural history, imaging techniques, and possible therapeutic interventions, the authors provide a concise review of the literature and discuss the optimal management of incidental CMs.
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Silva Lima, Leonardo Conrado, Marcelo Paglioli Ferreira, Davidson Alba, Alisson Roberto Teles, and Frederico Kliemann. "Cavernoma of the lateral ventricule presenting as speech arrests." Arquivos Brasileiros de Neurocirurgia: Brazilian Neurosurgery 33, no. 01 (March 2014): 82–84. http://dx.doi.org/10.1055/s-0038-1626205.
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AbstractCavernous malformations (CM) are formed by dilated thin-walled vascular channels without intervening parenchyma and intraventricular lesions are rare. We report a case of an intraventricular cavernoma in a 54-year old female patient. She presented with speech arrests daily for 10 months. Neurological examination revealed no deficits. The brain MRI study revealed an intraventricular lesion at the level of the left atrium, heterogeneously hyperintense on both T1 and T2-weighted images. It was performed an interhemispheric transcallosal approach with gross-total resection of the lesion. The histological examination was compatible with the diagnosis of cavernous haemangioma. In the postoperative period the patient presented with transient right hemiparesis with recovery in two weeks. We briefly review the literature concerning intraventricular cavernomas.
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Fernando, Pasindu M., B. M. Munasinghe, M. D. C. J. P. Jayamanne, K. A. Jayasundara, W. S. N. W. B. M. A. G. Arambepola, Selliah Pranavan, and N. D. Ranathunge. "Cerebral cavernous malformation in a child leading to a fatal subarachnoid hemorrhage – “silent but sinister:” A case report and literature review." Surgical Neurology International 12 (June 7, 2021): 253. http://dx.doi.org/10.25259/sni_248_2021.
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Background: Cerebral cavernous malformations (CCMs), otherwise known as cavernous hemangiomas/ cavernomas, are a type of vascular malformation. It is the third most common cerebral vascular malformation, histologically characterized by ectatic, fibrous, blood filled “caverns” with thin-walled vasculature without intervening normal brain parenchyma. Case Description: Herein, we present a case of an original, spontaneous hemorrhage from a sporadic form of CCM without associated gross developmental venous anomaly in an 11-year-old child, which is an extremely rare occurrence, with the special emphasis on the demographic data of the affected population, risk factors associated with hemorrhage, and correlation of histopathological and radiological findings with an in-depth literature review. Conclusion: The significant majority of the CCM are clinically occult. Hence, the development of risk assessment tools and guidelines for timely neurosurgical intervention poses a greater clinical challenge for medical experts rendering the management of the affected individuals with CCM in an anecdotal situation. Presentation of life-threatening rebleeds and neurological deficits in the diagnosed population albeit uncommon is possibly preventable outcomes.
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Nimjee, Shahid M., Ciaran J. Powers, and Ketan R. Bulsara. "Review of the literature on de novo formation of cavernous malformations of the central nervous system after radiation therapy." Neurosurgical Focus 21, no. 1 (July 2006): 1–6. http://dx.doi.org/10.3171/foc.2006.21.1.5.
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✓In this paper the authors review the literature concerning de novo cavernoma formation after radiation treatment. PubMed and MEDLINE database searches were performed. Data were compiled on all patients in whom de novo cavernomas formed after radiation treatment and whose cases were reported in the literature. The authors found reports in the literature of 76 patients in whom cavernomas formed de novo after radiation treatment. The mean age of the patients was 11.7 years, and the majority of these lesions occurred in males. The patients received a mean radiation dose of 60.45 Gy. The mean latency period before detection of the cavernoma was 8.9 years, and most of these lesions were detected incidentally. In symptomatic patients, the most common presenting symptoms were seizures. Thirty-seven of the patients had evidence of hemorrhage, and 54% of these required surgical intervention. De novo formation of cavernomas after radiation treatment is a relatively rare phenomenon. Patients in whom these cavernomas develop need to be followed closely because there is a propensity for the lesions to hemorrhage. Surgical intervention to treat symptomatic lesions has a favorable outcome.
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Giovani, A., Aurelia Sandu, Angela Neacsu, and R. M. Gorgan. "Surgical treatment and outcome of cerebral cavernomas – a 10 years’ experience." Romanian Neurosurgery 21, no. 4 (December 1, 2014): 395–405. http://dx.doi.org/10.2478/romneu-2014-0055.
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Abstract Cavernous malformations (cavernomas) are congenital low flow angiographic occult vascular lesions with a high tencency to bleeding. The prevalence of cerebral cavernous vascular malformations is estimated to be 0.4% to 0.9%.2,7 CMs in deep locations, including the brainstem, thalamus, and basal ganglia, account for 9% to 35% of all malformations in the brain. We performed a retrospective 10 years study on 130 operated cerebral cavernomas and discussed the clinical status at presentation, the choice and timing of the surgical approach and the short and longterm follow up. The 130 operated cases were divided into a supratentorial 102 (78.46%) group and an infratantorial group 28 (21.53%). The average age at presentation was 43,62 years old (17-76) and there was no sex predominance, male/female = 1,44 (77/53). Only in 14 cases (10.76%) we could find multiple cavernomas which had relatives with multiple cavrnomas, but the familial inheritance was not studied, and only the symptomatic lesion was resected. We divided the outcome results reporting for agroup with superficial respectively profound lesions. The long term follow up for the patients in the profound lesions group showed that 31/37 (83,78%) of patients had a mRS between 0 and 2, and the rest had a poor long term outcome. After surgery there was no clinical deterioration in the superficial lesions group and 22 patients from those who presented in mRS 2 showed neurological improvement on long term follow up, meaning that 82,79% of patients had a 0 or 1 mRS. Microsurgery is the treatment of choice in symptomatic brain cavernomas, total resection being the only curative treatment, capable to prevent further bleeding and to offer an efficient control of seizures.
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Esposito, Vincenzo, Sergio Paolini, and Roberta Morace. "Resection of a left insular cavernoma aided by a simple navigational tool." Neurosurgical Focus 21, no. 1 (July 2006): 1–4. http://dx.doi.org/10.3171/foc.2006.21.1.17.
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✓The management of cavernous malformations of the brain is markedly influenced by the location of the lesions themselves. In the last decade, resection of cavernomas arising in the dominant insular lobe has been deemed safe only with the guidance of neuronavigation. Most navigation equipment, however, shares some minor drawbacks, including costs, longer operating time, and a variable loss of accuracy due to intraoperative brain shift. In this paper the authors present the case of a left dominant insular cavernoma that was successfully removed using a novel form of navigation that they call magnetic resonance imaging–based corticotopography. This technique, which is unaffected by the brain shift phenomenon, provided a simple and inexpensive alternative to standard neuronavigation. Selected cases of subcortical brain lesions could be conveniently approached using the same technique.
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Badhiwala, Jetan H., Forough Farrokhyar, Waleed Alhazzani, Blake Yarascavitch, Mohammed Aref, Almunder Algird, Naresh Murty, et al. "Surgical outcomes and natural history of intramedullary spinal cord cavernous malformations: a single-center series and meta-analysis of individual patient data." Journal of Neurosurgery: Spine 21, no. 4 (October 2014): 662–76. http://dx.doi.org/10.3171/2014.6.spine13949.
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Object Information pertaining to the natural history of intramedullary spinal cord cavernous malformations (ISCCMs) and patient outcomes after surgery is scarce. To evaluate factors associated with favorable outcomes for patients with surgically and conservatively managed ISCCMs, the authors performed a systematic review and metaanalysis of the literature. In addition, they included their single-center series of ISCCMs. Methods The authors searched MEDLINE, EMBASE, CINAHL, Google Scholar, and The Cochrane Library for studies published through June 2013 that reported cases of ISCCMs. Data from all eligible studies were used to examine the epidemiology, clinical features, and neurological outcomes of patients with surgically managed and conservatively treated ISCCMs. To evaluate several variables as predictors of favorable neurological outcomes, the authors conducted a meta-analysis of individual patient data and performed univariate and multivariate logistic regression analyses. Variables included patient age, patient sex, lesion spinal level, lesion size, cerebral cavernomas, family history of cavernous malformations, clinical course, presenting symptoms, treatment strategy (operative or conservative), symptom duration, surgical approach, spinal location, and extent of resection. In addition, they performed a meta-analysis to determine a pooled estimate of the annual hemorrhage rate of ISCCMs. Results Eligibility criteria were met by 40 studies, totaling 632 patients, including the authors' institutional series of 24 patients. Mean patient age was 39.1 years (range 2–80 years), and the male-to-female ratio was 1.1:1. Spinal levels of cavernomas were cervical (38%), cervicothoracic (2.4%), thoracic (55.2%), thoracolumbar (0.6%), lumbar (2.1%), and conus medullaris (1.7%). Average cavernoma size was 9.2 mm. Associated cerebral cavernomas occurred in 16.5% of patients, and a family history of cavernous malformation was found for 11.9% of evaluated patients. Clinical course was acute with stepwise progression for 45.4% of patients and slowly progressive for 54.6%. Symptoms were motor (60.5%), sensory (57.8%), pain (33.8%), bladder and/or bowel (23.6%), respiratory distress (0.5%), or absent (asymptomatic; 0.9%). The calculated pooled annual rate of hemorrhage was 2.1% (95% CI 1.3%–3.3%). Most (89.9%) patients underwent resection, and 10.1% underwent conservative management (observation). Outcomes were better for those who underwent resection than for those who underwent conservative management (OR 2.79, 95% CI 1.46–5.33, p = 0.002). A positive correlation with improved neurological outcomes was found for resection within 3 months of symptom onset (OR 2.11, 95% CI 1.31–3.41, p = 0.002), hemilaminectomy approach (OR 3.20, 95% CI 1.16–8.86, p = 0.03), and gross-total resection (OR 3.61, 95% CI 1.24–10.52, p = 0.02). Better outcomes were predicted by an acute clinical course (OR 1.72, 95% CI 1.10–2.68, p = 0.02) and motor symptoms (OR 1.76, 95% CI 1.08–2.86, p = 0.02); poor neurological recovery was predicted by sensory symptoms (OR 0.58, 95% CI 0.35–0.98, p = 0.04). Rates of neurological improvement after resection were no higher for patients with superficial ISCCMs than for those with deep-seated ISCCMs (OR 1.36, 95% CI 0.71–2.60, p = 0.36). Conclusions Intramedullary spinal cord cavernous malformations tend to be clinically progressive. The authors' findings support an operative management plan for patients with a symptomatic ISCCM. Surgical goals include gross-total resection through a more minimally invasive hemilaminectomy approach within 3 months of presentation.
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Brinjikji, Waleed, Kelly D. Flemming, and Giuseppe Lanzino. "De novo formation of a large cavernoma associated with a congenital torcular dural arteriovenous fistula: case report." Journal of Neurosurgery: Pediatrics 19, no. 5 (May 2017): 567–70. http://dx.doi.org/10.3171/2016.12.peds16600.
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The authors report a case of a developmentally normal child with a congenital complex torcular dural arteriovenous fistula (DAVF) who later, in his teenage years, developed several vermian cavernomas within a large cerebellar developmental venous anomaly (DVA). The patient had initially presented with an abnormally large head circumference but no neurological deficits. He underwent several partial embolization procedures in an attempt to decrease the blood supply of the fistula over the course of 8 years. Nine years following initial presentation, he presented with a fourth ventricular hemorrhage, due to development of a new vermian cavernoma adjacent to a previously known vermian DVA and suffered subsequent mild left-sided hemiataxia from which he later recovered. CT angiographic images demonstrated that the vermian DVA drained into the left transverse sinus, which also drained the torcular arteriovenous fistula. A routine follow-up MRI examination 10 years following initial presentation demonstrated interval development of several large cavernomas in the cerebellum, all within the DVA. The patient had no new symptoms at that time and was neurologically intact. This case report highlights the de novo development of multiple cavernous malformations potentially secondary to DAVF-induced venous congestion in a preexisting DVA.
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Kashefiolasl, Sepide, Markus Bruder, Nina Brawanski, Eva Herrmann, Volker Seifert, Stephanie Tritt, and Juergen Konczalla. "A benchmark approach to hemorrhage risk management of cavernous malformations." Neurology 90, no. 10 (February 2, 2018): e856-e863. http://dx.doi.org/10.1212/wnl.0000000000005066.
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ObjectiveDespite the low annual risk of hemorrhage associated with a cavernous malformation (CM) (0.6%–1.1% per year), the risk of rehemorrhage rate and severity of neurologic deficits is significantly higher; therefore, we aimed to evaluate the rupture risk of CMs depending on various factors.MethodsWe retrospectively analyzed medical records of all patients with CM admitted to our institution between 1999 and April 2016. Cavernoma volume, location of the lesion, existence of a developmental venous anomaly (DVA), number of cavernomas, and patient characteristics (sex, age, hypertension, and antithrombotic therapy) were assessed.ResultsOne hundred fifty-four patients with CM were included; 89 (58%) ruptured CMs were identified. In statistical univariable analysis, the existence of a DVA was significantly higher in the ruptured cavernoma group (p < 0.001; odds ratio [OR] 4.6). A multivariable analysis of all included independent risk factors designated young age (<45 years) (p < 0.05; OR 2.2), infratentorial location (p < 0.01; OR 2.9), and existence of a DVA (p < 0.0001; OR 4.7) with significantly higher risk of rupture in our patient cohort. A separate analysis of these anatomical locations, supratentorial vs infratentorial, indicated that the existence of a DVA (p < 0.01; OR 4.16) in ruptured supratentorial cases and CM volume (≥1 cm3) (p < 0.0001; OR 3.5) in ruptured infratentorial cases were significant independent predictors for hemorrhage.ConclusionsYoung age (<45 years), infratentorial location, and the presence of a DVA are associated with a higher hemorrhage risk. CM volume (≥1 cm3) and the existence of a DVA were independently in accordance with the anatomical location high risk factors for CM rupture.
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Nogueira, Ricardo Malcata, Luis Santos Cardoso, Lino Fonseca, Miguel Correia, Amets Iraneta, Pedro Roque, Mario Matos, and Manuela Mafra. "Hydrocephalus in children – A rare case of pineal cavernoma and literature review." Surgical Neurology International 11 (September 18, 2020): 294. http://dx.doi.org/10.25259/sni_231_2020.
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Background: Cavernous malformations prevalence ranges from 0.4 to 0.6% and accounts for 5–15% of all central nervous system vascular malformations. Pineal cavernomas constitute <1% of all locations published in the literature, with a total of 26 cases reported, only 5 regarding the pediatric population until 2020. Overall annual hemorrhage rate is 2.4%. Symptoms are often due to hydrocephalus and intracranial hypertension. Case Description: We report a case of a 5-year-old child with visual disturbances, headache, and progressive neurologic deterioration. MR showed a lesion in the pineal region and triventricular hydrocephalus. She was submitted to endoscopic third ventriculostomy and total excision of the lesion by the infratentorial supracerebellar approach a few days later. Histopathological examination confirmed a pineal cavernous malformation. The patient returned to her normal life without any neurologic deficit and a normal development. Conclusion: The ideal treatment is primary lesion removal; however, due to the infrequency and because it is a curable lesion, studies seeking to deepen the knowledge of this disease are considered relevant.
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Nwachuku, Enyinna, James Duehr, Scott Kulich, Daniel Marker, and John Moossy. "Cervical intramedullary spinal cavernoma in setting of unresolved myelopathy: A case report." Surgical Neurology International 11 (July 4, 2020): 176. http://dx.doi.org/10.25259/sni_87_2020.
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Background: Spinal cavernous malformations are rare, accounting for approximately 5–12% of all spinal cord vascular lesions. Fortunately, improvements in imaging technologies have made it easier to establish the diagnosis of intramedullary spinal cavernomas (ISCs). Case Description: Here, we report the case of a 63-year-old male with an >11-year history of left-sided radiculopathy, ataxia, and quadriparesis. Initially, radiographic findings were interpreted as consistent with spondylotic myelopathy with cord signal changes from the C3-C7 levels. The patient underwent a C3-C7 laminectomy/foraminotomy with instrumentation. It was only after several symptomatic recurrences and repeated magnetic resonance images (MRI) that the diagnosis of a ventrally-located intramedullary lesion, concerning for a cavernoma, at the level C6 was established. Conclusion: Early and repeated enhanced MR studies may be required to correctly establish the diagnosis and determine the optimal surgical management of ISCs.
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Munich, Stephan A., and Jacques J. Morcos. "Resection of Mesencephalic Cavernoma Using the “Half-and-Half” Approach: 2-Dimensional Operative Video." Operative Neurosurgery 17, no. 6 (March 11, 2019): E246. http://dx.doi.org/10.1093/ons/opz024.
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Abstract Brainstem cavernous malformations present a particular challenge to neurosurgeons. Their deep location and proximity to critical brainstem nuclei and fiber tracts make surgical access challenging. Despite this, microsurgical resection of brainstem cavernomas has been associated with good long-term neurological outcome, albeit with notable (but temporary) perioperative morbidity. Here, we demonstrate the microsurgical resection of a mesencephalic cavernoma via a “half-and-half” approach. Described as the temporopolar approach by Sano in 1980,1 this approach centers on the oculomotor nerve and combines the transsylvian and pre-/subtemporal corridors. A wide opening of the Sylvian fissure and posterior mobilization of the temporal lobe permits access to the interpenducular cistern, mesencephalon and rostral pons. In this way, the “half-and-half” ideally suited for lesions of the anterior and antero-lateral brainstem. While temporary postoperative oculomotor nerve palsy is not uncommon, rapid resolution is expected. Verbal consent was provided by the patient for reproduction and publication of her case.
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Capelle, Hans-Holger, Makoto Nakamura, Thomas Lenarz, Almuth Brandis, Bernd Haubitz, and Joachim K. Krauss. "Cavernous angioma of the geniculate ganglion." Journal of Neurosurgery 109, no. 5 (November 2008): 893–96. http://dx.doi.org/10.3171/jns/2008/109/11/0893.
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Intracranial extraaxial cavernous angiomas are rare vascular malformations. Their occurrence at the geniculate ganglion of the facial nerve within the temporal bone is exceptional. The authors describe a 35-year-old man who developed a slowly progressing facial palsy. Initial cranial MR imaging showed no pathological findings, but 2 years later another MR examination detected a small tumor located at the geniculate ganglion of the facial nerve. The tumor was removed via a subtemporal approach. Histological examination revealed a cavernous angioma. Even small cavernomas located at the geniculate ganglion of the facial nerve may result in facial palsy. Isolated facial palsy in a young person should be monitored closely using imaging studies even if the initial imaging study is negative. Early decompression of the facial nerve may help to preserve its function.
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Abi-Aad, Karl R., Devi P. Patra, Matthew E. Welz, Evelyn Turcotte, and Bernard R. Bendok. "Lateral supracerebellar infratentorial approach for pontomesencephalic cavernous malformations." Neurosurgical Focus: Video 1, no. 1 (July 2019): V29. http://dx.doi.org/10.3171/2019.7.focusvid.191227.
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Cavernomas at the posterolateral pontomesencephalic surface can be approached from a lateral infratentorial supracerebellar corridor. In this surgical video, we demonstrate two cases of brainstem cavernomas resected through a lateral supracerebellar infratentorial approach. A supine position with lateral turn of the head was used along with significant reverse Trendelenburg to allow the cerebellum to fall away with gravity from the tentorium. After exposure of the posterior surface of the brainstem between the tentorium and the superior cerebellar surface with aid of neuronavigation, the cavernomas were safely resected.The video can be found here: https://youtu.be/fUDdaprg26Y.
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Kharkar, Siddharth, John Shuck, James Conway, and Daniele Rigamonti. "The Natural History of Conservatively Managed Symptomatic Intramedullary Spinal Cord Cavernomas." Neurosurgery 60, no. 5 (May 1, 2007): 865–72. http://dx.doi.org/10.1227/01.neu.0000255437.36742.15.
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Abstract OBJECTIVE The presentation and natural history of untreated, symptomatic intramedullary spinal cavernomas at our institution were analyzed. The objective is to provide additional information regarding the natural history of conservatively managed, symptomatic, intramedullary spinal cord cavernous malformations. METHODS The medical records of patients treated in our institution between 1989 and 2002 were reviewed to identify those with intramedullary cavernomas. The medical, radiological, surgical, and pathological records from these patients were retrospectively reviewed and analyzed. RESULTS Fourteen patients were included in the study. The mean age at presentation was 42 years. Four lesions (29%) were located in the cervical region and 10 lesions (71%) were present in the thoracolumbar spinal cord. All patients were symptomatic at the time of presentation. In this cohort of 14 patients, 10 patients (71%) were conservatively managed. For these patients, the mean duration of symptoms before presentation was 10 months. The mean duration of follow-up from the time of presentation was 80 months. The median McCormick grade for conservatively treated patients at presentation was II. During this period, none of the conservatively managed patients had an acute intramedullary bleed. In nine patients, the McCormick grade at the last follow-up evaluation was the same as or better than their score at presentation. Four patients (29%) were treated surgically. The mean duration of symptoms before presentation was 33 months. The mean duration of follow-up from the time of presentation was 42 months. In two surgical patients, the McCormick grade at the last follow-up evaluation remained unchanged compared with their score at presentation, whereas the McCormick grade improved in one patient and deteriorated in another patient. CONCLUSION This cohort of conservatively managed patients with symptomatic, intramedullary spinal cord cavernomas was clinically stable throughout the follow-up period. In this series, patients harboring symptomatic spinal cord cavernous malformation did not have significant, permanent neurological decline during the follow-up period when treated with the conservative approach of observation. This data provides additional information for determining the appropriate treatment strategy for patients with intramedullary spinal cavernomas.
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Bravi, Luca, Noemi Rudini, Roberto Cuttano, Costanza Giampietro, Luigi Maddaluno, Luca Ferrarini, Ralf H. Adams, et al. "Sulindac metabolites decrease cerebrovascular malformations in CCM3-knockout mice." Proceedings of the National Academy of Sciences 112, no. 27 (June 24, 2015): 8421–26. http://dx.doi.org/10.1073/pnas.1501352112.
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Cerebral cavernous malformation (CCM) is a disease of the central nervous system causing hemorrhage-prone multiple lumen vascular malformations and very severe neurological consequences. At present, the only recommended treatment of CCM is surgical. Because surgery is often not applicable, pharmacological treatment would be highly desirable. We describe here a murine model of the disease that develops after endothelial-cell–selective ablation of the CCM3 gene. We report an early, cell-autonomous, Wnt-receptor–independent stimulation of β-catenin transcription activity in CCM3-deficient endothelial cells both in vitro and in vivo and a triggering of a β-catenin–driven transcription program that leads to endothelial-to-mesenchymal transition. TGF-β/BMP signaling is then required for the progression of the disease. We also found that the anti-inflammatory drugs sulindac sulfide and sulindac sulfone, which attenuate β-catenin transcription activity, reduce vascular malformations in endothelial CCM3-deficient mice. This study opens previously unidentified perspectives for an effective pharmacological therapy of intracranial vascular cavernomas.
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SASARAN, A., A. MOHAN, F. STOICA, H. MOISA, and A. V. CIUREA. "Multimodal management in intracranial cavernous angiomas (intracranial cavernomas). An experience of 99 cases." Romanian Journal of Medical Practice 10, no. 2 (June 30, 2015): 195–203. http://dx.doi.org/10.37897/rjmp.2015.2.18.
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Introduction. Intracranial cavernomas are rare neurovascular lesions, met frequently in patients with anomalies of the vasculature of the encephalon and medulla. Cavernomas account for 0.02-0.53% of all intracranial lesions and approximately 8-15% of all intracranial vascular malformations. A procentage of about 10-30% of all cases show an association between cavernomas and arteriovenous malformations. Clinically, the lesions become symptomatic when their size becomes larger than 1cm. The symptoms include headache, seizures, focal neurologic deficit and last but not least hemorrhage. Materials and methods. The authors present a study of 99 patients diagnosed and treated for intracranial cavernomas between January 2004 and January 2015 (11 years). The study encompasses 45 male patients and 44 female patients with ages ranging between 11 and 56, all treated at the Bagdasar-Arseni Emergency University Hospital in Bucharest, Romania. A large percentage of the cavernomas were supratentorial 72 cases (72.72%), while only 27 tumors were positioned in the infratentorial compartment of the skull. Regarding the position of the cavernomas, 29 of them (40.27%) were in the frontal lobe, 13 (18.05%) were in the parietal lobe, 20 (27.7%) were in the temporal lobe, while 3 were in the occipital lobe (4.16%). Infratentorial tumors affected the brainstem in 17 cases (62.9%) while 10 cases showed cerebellar implication (37.03%). There were 7 patients in which the authors described multiple cavernomas. The clinical onset was represented by seizures in 59 cases (59.59%), hemorrhage in 20 cases (20.20%) and focal neurologic deficit in 13 cases (13.13%). The symptoms consisted of seizures in 63 cases (63.63%), focal neurologic deficit in 16 cases (16.16%) and hemorrhage in 23 cases (23.23%) while 9 cases (9.9%) were completely asymptomatic. The authors chose to practice a conservative management for the 7 cases with multiple lesions, the 9 asymptomatic cases and 5 cases with deep positioning. In the 5 cases with deep cavernomas the patients were subjected to gamma knife stereotactic radiosurgery but only 2 patients showed response to treatment. Results. In the 99 patients presented by the authors, out of the 76 cases operated, a number of 57 interventions (75%) managed to completeley remove the lesion and perilesional gliosis. A number of 19 interventions only managed to remove the tumor as perilesional gliosis was impossible to remove without lesions to eloquent areas. Conclusions. Intracranial cavernomas are rare lesions, usually incriminated when seizures appear. When they are asymptomatic the best option for the surgeon is to wait and see how the tumor behaves. When seizures appear in the array of symptoms of a given tumor the best prognosis is offered by lesionectomy with the removal of perilesional gliosis. Neuronavigation guided surgery has managed in most cases to facilitate complete removal of such tumors and to avoid postoperative defficit with the improvement of clinical results. Furthermore, neuronavigation removes the necessity of an unpleasant stereotactic frame. When intracerebral hemorrhage occurs, surgery is mandatory and represents a neurosurgical emergency. In multiple tumors, the bleeding cavernoma must be removed. The effectiveness of Gamma Knife Surgery (GKS) is debatable.
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Abla, Adib A., Jay D. Turner, Alim P. Mitha, Gregory Lekovic, and Robert F. Spetzler. "Surgical approaches to brainstem cavernous malformations." Neurosurgical Focus 29, no. 3 (September 2010): E8. http://dx.doi.org/10.3171/2010.6.focus10128.
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Brainstem cavernous malformations (CMs) are low-flow vascular lesions in eloquent locations. Their presentation is often marked with symptomatic hemorrhages that appear to occur more frequently than hemorrhage from supratentorial cavernomas. Brainstem CMs can be removed using 1 of the 5 standard skull-base approaches: retrosigmoid, suboccipital (with or without telovelar approach), supracerebellar infratentorial, orbitozygomatic, and far lateral. Patients being referred to a tertiary institution often have lesions that are aggressive with respect to bleeding rates. Nonetheless, the indications for surgery, in the authors' opinion, are the same for all lesions: those that are symptomatic, those that cause mass effect, or those that abut a pial surface. Patients often have relapsing and remitting courses of symptoms, with each hemorrhage causing a progressive and stepwise decline. Many patients experience new postoperative deficits, most of which are transient and resolve fully. Despite the risks associated with operating in this highly eloquent tissue, most patients have had favorable outcomes in the authors' experience. Surgical treatment of brainstem CMs protects patients from the potentially devastating effects of rehemorrhage, and the authors believe that the benefits of intervention outweigh the risks in patients with the appropriate indications.
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Campero, Alvaro, Ignacio Casas-Parera, Juan F. Villalonga, and Matías Baldoncini. "Microsurgical Resection of a Chiasmatic Cavernoma: 3-Dimensional Operative Video." Operative Neurosurgery 21, no. 1 (March 17, 2021): E46—E47. http://dx.doi.org/10.1093/ons/opab063.
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Abstract According to reports from the literature,1,2 depending on the location where cavernomas appear, range from the very common locations to unusual. Cavernous malformations arising from the optic nerve and chiasm are rare, with only few cases reported to date.3-5 We present a case of a 28-yr-old man who suddenly started with sever visual loss in the right eye and homonymous lateral hemianopia in the left eye. Because of the acute symptomatology, a brain MRI was immediately performed in order to diagnose the etiology. The MRI showed a chiasmatic mass with right extension, heterogeneous on T1 and T2 sequences, without enhancement after gadolinium. The surgery was carried out a week after the diagnosis. A right pterional transsylvian approach was performed and the cavernoma was resected with microsurgical maneuvers, preserving the optic nerve fibers, chiasm, and optic tract. The patient evolved favorably, improving the visual deficit in the postoperative period as can be observed in the postoperative visual field study 7 mo after the surgery. The patient signed an informed consent for the procedure and agreed with the use of his images and surgical video for research and academic purposes. Our surgical case emphasizes the importance of a prompt diagnosis and surgery for chiasmatic cavernomas3 associated to visual loss, providing early decompression of the optic apparatus and improvement of the visual field defects after surgery.
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Gasparetto, Emerson Leandro, Soniza Alves-Leon, Flavio Sampaio Domingues, João Thiago Frossard, Selva Paraguassu Lopes, and Jorge Marcondes de Souza. "Neurocysticercosis, familial cerebral cavernomas and intracranial calcifications: differential diagnosis for adequate management." Arquivos de Neuro-Psiquiatria 74, no. 6 (June 2016): 495–500. http://dx.doi.org/10.1590/0004-282x20160054.
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ABSTRACT Neurocysticercosis (NCC) is an endemic disease and important public health problem in some areas of the World and epilepsy is the most common neurological manifestation. Multiple intracranial lesions, commonly calcified, are seen on cranial computed tomography (CT) in the chronic phase of the disease and considered one of the diagnostic criteria of the diagnosis. Magnetic resonance imaging (MRI) is the test that better depicts the different stages of the intracranial cysts but does not show clearly calcified lesions. Cerebral cavernous malformations (CCM), also known as cerebral cavernomas, are frequent vascular malformations of the brain, better demonstrated by MRI and have also epilepsy as the main form of clinical presentation. When occurring in the familial form, cerebral cavernomas typically present with multiple lesions throughout the brain and, very often, with foci of calcifications in the lesions when submitted to the CT imaging. In the countries, and geographic areas, where NCC is established as an endemic health problem and neuroimaging screening is done by CT scan, it will be important to consider the differential diagnosis between the two diseases due to the differences in adequate management.
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Kassardjian, Charles D., Ari Breiner, and Martin del Campo. "Fulminant Strokes Secondary to Radiation-induced Small-vessel Arteriopathy." Brain Impairment 15, no. 1 (May 2014): 58–60. http://dx.doi.org/10.1017/brimp.2014.5.
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Delayed vasculopathy associated with prior brain irradiation is a known cause of stroke. Radiation is implicated in large-vessel stenosis, cavernous malformations and, rarely, small-vessel disease. There have been no reported cases of fulminant ischaemic and haemorrhagic strokes secondary to radiation vasculopathy. We present the case of a 40-year-old woman with a history of childhood leukaemia and whole-body and cranio-cervical radiation. The patient presented with recurrent acute neurological deficits over a 4-month period resulting from haemorrhagic and ischaemic strokes. Imaging revealed numerous cavernomas and small-vessel acute infarctions. No traditional stroke aetiology was identified. Delayed radiation-induced vasculopathy should be considered in patients with a history of brain irradiation and ischaemic or haemorrhagic strokes, and can present in a fulminant manner with recurrent strokes over a short period of time.
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Manjila, Sunil, Karam Moon, Mark A. Weiner, Mark L. Cohen, R. John Leigh, Cliff A. Megerian, and Nicholas C. Bambakidis. "Cavernous Malformation of the Trochlear Nerve: Case Report and Review of the Literature on Cranial Nerve Cavernomas." Neurosurgery 69, no. 1 (April 5, 2011): E230—E238. http://dx.doi.org/10.1227/neu.0b013e31821cb28f.
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Abstract BACKGROUND AND IMPORTANCE: Cavernous malformations (CMs) arising intrinsically to the cisternal segment of the trochlear nerve are extremely rare. This case of a trochlear nerve cavernous angioma is the third to be reported in the neurosurgical literature and the first to be resected by a middle fossa approach. CLINICAL PRESENTATION: The authors present a case of a 31-year-old woman with progressive left-sided headache and left hemisensory symptoms, whose magnetic resonance imaging showed a solid enhancing tumor in the left ambient cistern at the level of the midbrain-pontine junction causing significant brainstem compression. Intraoperatively, a left trochlear nerve cavernous angioma circumferentially enveloping the nerve was visualized. The angioma was microsurgically resected by a middle fossa approach under frameless stereotactic guidance. Gross total resection of the intrinsic trochlear nerve lesion was achieved, although the trochlear nerve could not be preserved intact. CONCLUSION: CMs should be considered in a possible differential diagnosis of cisternal trochlear nerve tumors. Surgical resection remains the standard of care, and is indicated for relief of compressive symptoms and prevention of future bleeds. Postoperative diplopia often persists; however, resolution of diplopia reported in the literature can be attributed to either regeneration after direct surgical repair of the sacrificed nerve or a spontaneous adaptation over time.
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Steiger, Hans-Jakob, Bernd Turowski, and Daniel Hänggi. "Prognostic factors for the outcome of surgical and conservative treatment of symptomatic spinal cord cavernous malformations: a review of a series of 20 patients." Neurosurgical Focus 29, no. 3 (September 2010): E13. http://dx.doi.org/10.3171/2010.6.focus10123.
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Object In this study, the authors present a review of a series of 20 intramedullary spinal cord cavernous malformations (SCCMs) with particular focus on MR imaging and prognostic factors. Methods Between 1994 and 2009, 20 patients with SCCM were treated under the care of the senior author. The diagnosis was made in all patients after the onset of clinical symptoms. The age of the 9 men and 11 women ranged between 26 and 71 years (median 38.5 years). The duration of symptoms prior to referral ranged from 1 week to 9 years (median 6.5 months). At the time of referral, 4 patients had no significant neurological deficits, 10 patients suffered significant functional restrictions, and 6 patients presented with severe paraparesis and loss of functional strength. None of the patients had complete paraplegia. Seventeen patients underwent microsurgical removal, while 3 patients opted for conservative therapy. For the present analysis, the medical records and MR images and/or reports were reviewed. Classification of length of history, pretreatment status, MR imaging pattern, and treatment modality was done and correlated with outcome. Results The cavernoma was located at the cervical level in 8 patients and between T-1 and L-1 in 12 patients. The cavernoma appeared as mainly T2 hyperintense on MR images in 7 patients, mainly T2 hypointense in 2 patients, and mixed in the remaining 10 patients. The craniocaudal extension of the core varied between 5 and 45 mm. In 2 patients with cervical cavernomas, a distinct T2 signal of the spinal cord cranial and distal to the cavernoma was seen, and in a patient with a large thoracic cavernoma, T2 extinction cranial and caudal to the cavernoma was seen as a sign of hemosiderosis. Neurological deficits improved postoperatively in 12 of the surgically treated patients, remained stable in 2, and deteriorated in 3. The 3 patients who were conservatively treated remained stable over a follow-up of 3–9 years. Postoperative improvement was seen in 5 of 7 surgical patients with a history of symptoms of 2 months or less, 5 of 6 patients with a history of 2–24 months, and in 2 of 4 patients with a history of more than 2 years. Two of the 3 patients with postoperative deterioration had a history of more than 2 years and the third a short history of 1 month. Conclusions Although a satisfactory outcome can be achieved through surgical treatment of SCCMs, some patients worsen after surgery or during the postoperative course. Long-term stability is possible in oligosymptomatic conservatively treated patients. The prevalence and pathophysiological importance of segmental spinal cord edema and hemosiderosis is incompletely understood at the present time.
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Gewiss, Caroline, Christian Hagel, and Kara Krajewski. "Cerebral cavernomas in adults and children express relaxin." Journal of Neurosurgery: Pediatrics 25, no. 2 (February 2020): 144–50. http://dx.doi.org/10.3171/2019.9.peds19333.
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OBJECTIVETo shed light on the role of relaxin in cerebral cavernous malformations (CCMs) in adults and children, the authors investigated endothelial cell (EC) expression of relaxin 1, 2, and 3; vascular endothelial growth factor receptor–1 and –2 (VEGFR-1 and -2); Ki-67; vascular geometry; and hemorrhage, as well as the clinical presentation of 32 patients with surgically resected lesions.METHODSParaffin-embedded sections of 32 CCMs and 5 normal nonvascular lesion control (NVLC) brain tissue samples were immunohistochemically stained with antibodies to relaxin 1, 2, and 3; angiogenesis growth factor receptors Flt-1 (VEGFR-1) and Flk-1 (VEGFR-2); and proliferation marker Ki-67. For morphometric analysis, Elastica van Gieson stain was used, and for hemorrhage demonstration, Turnbull stain was used. Data from the pediatric and adult CCMs were compared with each other and with those obtained from the NVLCs. Statistical analyses were performed with Fisher’s exact test, the chi-square test, the phi correlation coefficient, and the Student t-test. A p value < 0.05 was considered significant.RESULTSPediatric and adult cavernoma vessels did not significantly differ in diameter. Hemorrhage was observed in CCMs but not in NVLC samples (p < 0.05). There was no difference in expression of Ki-67, VEGFR-1 and -2, and relaxin 1, 2, and 3 in the ECs of pediatric and adult CCMs. The ECs of CCMs were largely negative for relaxin 3 compared to NVLCs (p < 0.05), whereas CCMs, compared to control brain tissue samples, more frequently expressed Flt-1 and relaxin 2 (p < 0.05). Ki-67 was not expressed in the NVLCs, but the difference was not statistically significant. Relaxin 1 and 2 expression and increased expression of VEGFR-1 were associated with a supra- versus infratentorial location (p < 0.05).CONCLUSIONSRelaxin 1 and 2 and VEGFR-1 play a role in supratentorial cavernomas. Relaxin 3 may play a physiological role in normal brain vasculature. Relaxin 1 and 3 are also found in normal cerebral vasculature. Relaxin 1, 2, and 3 are associated with increased VEGFR-1 expression.
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Rennert, Robert C., Reid Hoshide, Mark Calayag, Joanna Kemp, David D. Gonda, Hal S. Meltzer, Takanori Fukushima, John D. Day, and Michael L. Levy. "Extended middle fossa approach to lateralized pontine cavernomas in children." Journal of Neurosurgery: Pediatrics 21, no. 4 (April 2018): 384–88. http://dx.doi.org/10.3171/2017.10.peds17381.
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OBJECTIVETreatment of hemorrhagic cavernous malformations within the lateral pontine region demands meticulous surgical planning and execution to maximize resection while minimizing morbidity. The authors report a single institution’s experience using the extended middle fossa rhomboid approach for the safe resection of hemorrhagic cavernomas involving the lateral pons.METHODSA retrospective chart review was performed to identify and review the surgical outcomes of patients who underwent an extended middle fossa rhomboid approach for the resection of hemorrhagic cavernomas involving the lateral pons during a 10-year period at Rady Children’s Hospital of San Diego. Surgical landmarks for this extradural approach were based on the Fukushima dual-fan model, which defines the rhomboid based on the following anatomical structures: 1) the junction of the greater superficial petrosal nerve (GSPN) and mandibular branch of the trigeminal nerve; 2) the lateral edge of the porus trigeminus; 3) the intersection of the petrous ridge and arcuate eminence; and 4) the intersection of the GSPN, geniculate ganglion, and arcuate eminence. The boundaries of maximal bony removal for this approach are the clivus inferiorly below the inferior petrosal sinus; unroofing of the internal auditory canal posteriorly; skeletonizing the geniculate ganglion, GSPN, and internal carotid artery laterally; and drilling under the Gasserian ganglion anteriorly. This extradural petrosectomy allowed for an approach to all lesions from an area posterolateral to the basilar artery near its junction with cranial nerve (CN) VI, superior to the anterior inferior cerebellar artery and lateral to the origin of CN V. Retraction of the mandibular branch of the trigeminal nerve during this approach allowed avoidance of the region involving CN IV and the superior cerebellar artery.RESULTSEight pediatric patients (4 girls and 4 boys, mean age of 13.2 ± 4.6 years) with hemorrhagic cavernomas involving the lateral pons and extension to the pial surface were treated using the surgical approach described above. Seven cavernomas were completely resected. In the eighth patient, a second peripheral lesion was not resected with the primary lesion. One patient had a transient CN VI palsy, and 2 patients had transient trigeminal hypesthesia/dysesthesia. One patient experienced a CSF leak that was successfully treated by oversewing the wound.CONCLUSIONSThe extended middle fossa approach can be used for resection of lateral pontine hemorrhagic cavernomas with minimal morbidity in the pediatric population.
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Mohamed, Z., LL Batista, M. Sachet, J. Mahadevan, H. Alvarez, and P. Lasjaunias. "Growing Dural Sinus Malformation with Associated Developmental Venous Anomaly, Multiple Cavernomas and Facial Venous Malformation in an Infant." Interventional Neuroradiology 8, no. 4 (December 2002): 421–30. http://dx.doi.org/10.1177/159101990200800412.
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This is an unusual case report of an infant, who initially presented with a facial haemangioma and was later diagnosed to have a dural sinus malformation (DSM) involving the torcula. The DSM increased in size lateralising to the right transverse sinus at three months of age. Postnatal enlargement of the dural sinus has not been described before suggesting a delay in the maturation of the dural sinus which normally would occur antenatally. There was a further association with a complex developmental venous anomaly (DVA) draining the right cerebral hemisphere into the deep cerebral vein and multiple cavernous malformations. The DVA was not clearly demonstrated at age one month but was more obvious at age three months. This would be the first reported case of DSM associated with a DVA. Increasing venous hypertension probably contributed to the poor opacification of the DVA on follow-up angiography at age six months and to the haemorrhagic changes within the cavernomas on magnetic resonance imaging (MRI). The therapeutic goal was to correct venous hypertension by partially embolising the dural shunts to remodel the cerebral vasculature and preserve the patent sinus. The treatment strategy and possible link between the complex disease entities presented in this infant are discussed. Despite these attemps, the lesion continued to grow compressing the posterior fossa structures. The infant died at nine months of age.
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Buzoianu, Dan-Sorin, Maria-Irina Munteanu, Armand Frasineanu, Radu-Dan Mihailescu, Georgiana Marin, Alina-Lucia Ciuciulete, Iuliana Cocianga, and Bogdan Ovidiu Popescu. "SEQUENTIAL HEMORRHAGE IN TWO LESIONS OF MULTIPLE CEREBRAL CAVERNOUS MALFORMATIONS: A CASE REPORT." Romanian Journal of Neurology 16, no. 4 (December 31, 2017): 149–53. http://dx.doi.org/10.37897/rjn.2017.4.3.
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We present the case of a 64-year old female, with a 5 year history of symptomatic multiple cerebral cavernomas, who developed paresthesia in the right leg and arm, followed by vertigo, slurred speech and forward gaze diplopia. Brain investigation revealed two lesions with supra- and infratentorial localization with signs of recent hemorrhage of different ages. The localization of the lesions in the midpons and the basal ganglia imposed a conservative approach.
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Mastronardi, Luciano, Luigi Ferrante, Marco Scarpinati, Franco Maria Gagliardi, Paolo Celli, and Aldo Fortuna. "Intradural Extramedullary Cavernous Angioma: Case Report." Neurosurgery 29, no. 6 (December 1, 1991): 924–26. http://dx.doi.org/10.1227/00006123-199112000-00023.
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Abstract Cavernous angiomas represent 5 to 12% of spinal vascular malformations and usually are located at the vertebral body level with possible extension into the extradural space. The intradural intramedullary cavernoma occurs in about 3% of cases, whereas extramedullary localization is extremely rare. A new case of an intradural extramedullary cavernous angioma is reported, and the clinical, diagnostic, and therapeutic aspects of this rare malformation are analyzed.
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Söderman, M., W. Y. Guo, B. Karlsson, D. M. Pelz, E. Ulfarsson, and T. Andersson. "Neurovascular Radiosurgery." Interventional Neuroradiology 12, no. 3 (September 2006): 189–202. http://dx.doi.org/10.1177/159101990601200301.
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This article focuses on the treatment of neurovascular diseases, in particular brain arteriovenous malformations (BAVMs), with radiosurgery. The target group for this review is physicians who manage patients with neurovascular diseases, but are not actively engaged in radiosurgery. Radiosurgery for BAVMs is an established treatment with clearly defined risks and benefits. The efficacy of radiosurgery for dural arteriovenous shunts (DAVSs) is probably similar but the treatment has not yet gained the same acceptance. Radiosurgical treatment of cavernomas (cavernous hemangiomas) remains controversial. Well founded predictive models for BAVM radiosurgery show: The probability of obliteration depends on the dose of radiation given to the periphery of the BAVM. The risk of adverse radiation effects depends on the total dose of radiation, i.e. the amount of energy imparted into the tissue. The risk is greater in centrally located lesions. The risk of damage to brainstem nucleii and cranial nerves must be added to the risk predicted from current outcome models. The risk of hemorrhage during the time span before obliteration depends on the BAVM volume, the dose of radiation to the periphery of the lesion and the age of the patient. Central location is a probably also a risk factor.
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Caza, Tabita Larisa, Ioana Andreea Dărămuș, B. C. Dumitrescu, V. Ciubotaru, and Ligia Gabriela Tătăranu. "AN UNUSUAL CASE OF CEREBELLAR VENOUS ANGIOMA ASSOCIATED WITH TEMPORAL CAVERNOMA – PATHOPHYSIOLOGICAL, DIAGNOSTIC, AND SURGICAL CONSIDERATIONS." Journal of Surgical Sciences 2, no. 4 (October 1, 2015): 198–203. http://dx.doi.org/10.33695/jss.v2i4.136.
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Cerebral vascular malformations are hamartomas, classified into four distinct groups:arteriovenous malformations, cavernous malformations, capillary telangiectasias, anddevelopmental venous anomalies. These abnormal vascular entities have distinct histopathological, radiological, and clinical features, which make them different from one another. We report a case of a 37-year-old man, who presented with headaches, generalized grand mal seizures, and an episode of loss of consciousness, due to a left temporal cavernoma. Gadolinium-enhanced T1-weighted MR images showed a left temporal “popcorn-like” lesion, with heterogeneousenhancement, measuring 15/17/18 mm, suggestive of a cavernoma (angiographically occultmalformation). The T2-weighted MRI showed a right cerebellar venous plexus, draining into alarger central vein and the angiogram revealed the pathognomonic caput medusae aspect of avenous angioma. Microsurgical resection of the left temporal cavernous malformation wasperformed using a left frontal temporal approach. The venous angioma was spared to avoid venousinfarction and cerebral edema with devastating vital consequences. The intra- and postoperativecourses were uneventful with total recovery. The seizures remitted under anticonvulsant therapy,and the postoperative computer tomography investigation were within normal limits. The venous angioma was situated in the right cerebellar hemisphere, rather than near the cavernoma, its location making this the particular aspect of this case.
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Janicijevic, Aleksandar, Nikola Repac, Igor Nikolic, Igor Djoric, Vuk Scepanovic, Vladimir Janicijevic, and Goran Tasic. "Cavernous malformations of the brain stem - the clinical features and surgical approaches." Acta chirurgica Iugoslavica 61, no. 4 (2014): 33–38. http://dx.doi.org/10.2298/aci1404033j.
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Introduction. Cavernous malformations localized in the brain stem are considered as a separate entity in relation to other intracranial cavernoma. Clinical presentation is specific in terms of focal neurologic deficit, they show aggressive biological behavior and unfavorable clinical course, whereas localization in the brain stem naturally represents the largest surgical problem and challenge and significantly higher operational risk. Results. We report a series of 10 patients with brainstem cavernoma, operated at the Department of Neurosurgery, Clinical Center of Serbia in the period of 2008-2012. In cavernous lesions of the dorsal pons and upper cerebellar peduncul we used the approach through the fourth ventricule, in the laterally localized pontine cavernoma we used the cerebellopontine angle approach, in the cavernoma localized in the central midbrain tegmental area was used supreacerebellar infratentorially approach. Conclusion. Surgical removal of the brainstem cavernoma is absolutely expedient both from the standpoint of preventing recurrent and debilitating hemorrhage and in terms of recovery of neurologic deficit. In patients with disturbed vital functions, the evacuation of the hematoma and removal of the malformation eliminate compression of effects on vital structures of the brain stem.
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Liu, James K. "Microsurgical resection of intramedullary spinal cord cavernous malformation. Operative video and technical nuances." Neurosurgical Focus 37, v2supplement (September 2014): Video1. http://dx.doi.org/10.3171/2014.v3.focus14155.
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Intramedullary spinal cord cavernous malformations account for approximately 5% of all intraspinal lesions. These lesions can present with either acute neurological compromise secondary to hemorrhage inside the spinal cord, or with chronic progressive myelopathy due to repeated microhemorrhages. Surgical resection of spinal cord cavernous malformations remains the definitive treatment strategy for symptomatic lesions. Because of the intimate relationship with surrounding eloquent neural tissue, these lesions can be technically challenging to remove with a significant risk for morbidity. In this operative video, the author demonstrates an illustrative step-by-step technique for microsurgical resection of a large intramedullary spinal cord cavernous malformation at C4–5 causing progressive myelopathy. Complete resection was achieved without neurologic compromise. The operative technique and surgical nuances, including the surgical approach, intradural cavernoma removal, and spinal stabilization are illustrated.The video can be found here: http://youtu.be/3FUjGSyrKO0.