scholarly journals Cerebral cavernous malformation in a patient with pontine hemorrhage: A case study

2020 ◽  
Vol 10 (3) ◽  
Author(s):  
Myrto Palkopoulou ◽  
Eleni Bakola ◽  
Marina Foliadi ◽  
Petros Stefanidis ◽  
P. Teresa Acquaviva

The cerebral cavernous malformations are benign vascular hamartomas, with thin and dilated vascular walls and therefore constantly susceptible to hemorrhage. Clinically, they present with recurrent headaches, acute intracranial hemorrhage and focal neurological deficits. They are considered as angiographically occult vascular malformations and the imaging technique of choice for their diagnosis is magnetic resonance tomography. We present the case of a female patient with acute-onset symptomatology, congruent with a lesion in the basal pons. Her medical history included an intracranial hemorrhage due to a cavernoma, which was surgically removed. The magnetic resonance imaging of the brain revealed two new cavernomas, which were not identified in the imaging conducted in the past. In literature, the cases of de novo appearance of cavernomas are considered highly rare, especially in patients with no consistent family history or medical history of radiation therapy. Resultantly, they should be considered as dynamic lesions, regarding their number, size and behavior.

Neurosurgery ◽  
1990 ◽  
Vol 27 (6) ◽  
pp. 892-900 ◽  
Author(s):  
Douglas Kondziolka ◽  
L. Dade Lunsford ◽  
Robert J. Coffey ◽  
David J. Bissonette ◽  
John C. Flickinger

Abstract Stereotactic radiosurgery has been shown to treat successfully angiographically demonstrated arteriovenous malformations of the brain. Angiographic obliteration has represented cure and eliminated the risk of future hemorrhage. The role of radiosurgery in the treatment of angiographically occult vascular malformations (AOVMs) has been less well defined. In the initial 32 months of operation of the 201-source cobalt-60 gamma knife at the University of Pittsburgh, 24 patients meeting strict criteria for high-risk AOVMs were treated. Radiosurgery was used conservatively; each patient had sustained two or more hemorrhages and had a magnetic resonance imaging-defined AOVM located in a region of the brain where microsurgical removal was judged to pose an excessive risk. Venous angiomas were excluded by performance of high-resolution subtraction angiography in each patient. Fifteen malformations were in the medulla, pons, and/or mesencephalon, and 5 were located in the thalamus or basal ganglia. Follow-up ranged from 4 to 24 months. Nineteen patients either improved or remained clinically stable and did not hemorrhage again during the follow-up interval. One patient suffered another hemorrhage 7 months after radiosurgery. Five patients experienced temporary worsening of pre-existing neurological deficits that suggested delayed radiation injury. Magnetic resonance imaging demonstrated signal changes and edema surrounding the radiosurgical target. Dose-volume guidelines for avoiding complications were constructed. Our initial experience indicates that stereotactic radiosurgery can be performed safely in patients with small, well-circumscribed AOVMs located in deep, critical, or relatively inaccessible cerebral locations. Because cerebral angiography is not useful in following patients with AOVMs, long-term magnetic resonance imaging and clinical studies will be necessary to determine whether the natural history of such lesions is changed by radiosurgery.


Neurosurgery ◽  
2008 ◽  
Vol 62 (4) ◽  
pp. E969-E970 ◽  
Author(s):  
Patrick J. Reid ◽  
Stephen S. Campbell ◽  
George E. Vates ◽  
Rafael Allende

Abstract OBJECTIVE Cerebral cavernous malformations (CCMs) are found in 0.1 to 0.5% of the population. With the advent of magnetic resonance imaging, they have been increasingly recognized, suggesting a higher prevalence than previously reported. The development of de novo lesions is well documented, with a reported rate of 0.2 to 0.4 lesions per patient per year. We present a patient who developed 70 new lesions over a 38-month period (approximately 22 per yr). CLINICAL PRESENTATION A 32-year-old woman was admitted to the obstetric service with a diagnosis of viral gastroenteritis. She became acutely unresponsive, and a computed tomographic scan of the head revealed an acute left frontal intraparenchymal hematoma with significant edema and midline shift. She rapidly deteriorated, developing a fixed, dilated left pupil and agonal respirations. INTERVENTION She was taken to the operating room for an emergent left frontal craniotomy and decompression with evacuation of the hematoma. A magnetic resonance imaging scan revealed multiple cavernous malformations, and histopathology was consistent with cavernous malformation. Genetic testing revealed a CCM1 mutation. CONCLUSION This patient demonstrates the truly dynamic nature of CCMs and the increased incidence of new lesions in the setting of CCM1 mutation. This case is remarkable not only for the unprecedented rate of lesion formation (approximately 22 per yr), but also because of the nearly unilateral distribution of the lesions.


Neurosurgery ◽  
2012 ◽  
Vol 71 (3) ◽  
pp. 626-631 ◽  
Author(s):  
Christopher D. Witiw ◽  
Amal Abou-Hamden ◽  
Abhaya V. Kulkarni ◽  
Joseph A. Silvaggio ◽  
Carol Schneider ◽  
...  

Abstract BACKGROUND: Cerebral cavernous malformations are brain vascular malformations associated with intracranial hemorrhage. It is unclear whether pregnancy is a risk factor for hemorrhage, yet there is speculation that it may be. OBJECTIVE: To compare the risk of clinically significant hemorrhage during pregnancy and nonpregnancy. METHODS: A total of 186 patients from the University of Toronto Vascular Malformations Study Group were enrolled. The obstetrical history of each patient was collected and matched to their neurological history from the records of the study group. All hemorrhagic events occurring during childbearing years were associated with either a defined pregnancy risk period or nonpregnancy period. Patients were also asked to recall advice that they received from health care professionals regarding risk of hemorrhage in pregnancy. RESULTS: Among our patient population there were 349 pregnancies (283 live births) and 49 hemorrhages during childbearing years, 3 of which were during pregnancy but none during delivery or within 6 weeks post partum. The hemorrhage rate for pregnant women was 1.15% (95% confidence interval: 0.23-3.35) per person-year and 1.01% (95% confidence interval: 0.75-1.36) per person-year for nonpregnant women. Relative risk of pregnancy was 1.13 (95% confidence interval: 0.34-3.75) (P = .84). Neurosurgeons and obstetricians were the source of most hemorrhage risk advice. The majority of neurosurgeons suggested that the risk was unchanged, but the obstetricians were divided. Four patients never conceived, and 2 others began contraception because of the advice that they received. CONCLUSION: The risk of intracranial hemorrhage from cerebral cavernous malformations is likely not changed during pregnancy, delivery, or post partum.


Neurosurgery ◽  
2009 ◽  
Vol 65 (3) ◽  
pp. 450-455 ◽  
Author(s):  
Juri Kivelev ◽  
Mika Niemelä ◽  
Riku Kivisaari ◽  
Reza Dashti ◽  
Aki Laakso ◽  
...  

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.


Neurosurgery ◽  
2009 ◽  
Vol 65 (1) ◽  
pp. 20-30 ◽  
Author(s):  
Stylianos K. Rammos ◽  
Raffella Maina ◽  
Giuseppe Lanzino

ABSTRACT DEVELOPMENTAL VENOUS ANOMALIES (DVAs), formerly known as venous angiomas, have become the most frequently diagnosed intracranial vascular malformation. DVAs are currently considered congenital cerebrovascular anomalies with mature venous walls that lack arterial or capillary elements. They are composed of radially arranged medullary veins, which converge in an enlarged transcortical or subependymal collector vein, and have characteristic appearances (caput medusae) on magnetic resonance imaging and angiography. DVAs were once thought to be rare lesions with substantial potential for intracerebral hemorrhage and considerable morbidity. The prevalence of incidental and asymptomatic DVAs has been more apparent since the advent of magnetic resonance imaging; recent cohort studies have challenged the once-held view of isolated DVAs as the cause of major neurological complications. The previously reported high incidence of intracerebral hemorrhage associated with DVAs is currently attributed to coexistent, angiographically occult cavernous malformations. Some patients may still have noteworthy neurological morbidity or die as a result of acute infarction or hemorrhage directly attributed to DVA thrombosis. DVAs can coexist with cavernous malformations and arteriovenous malformations. Such combination or transitional forms of malformations might suggest common pathways in pathogenesis. Recent data support a key role for DVAs in the pathogenesis of mixed vascular malformations.


2009 ◽  
Vol 67 (3b) ◽  
pp. 917-921 ◽  
Author(s):  
Adolfo Ramírez-Zamora ◽  
José Biller

Central nervous system (CNS) cavernous malformations (CMs) are developmental malformations of the vascular bed with a highly variable clinical course due to their dynamic nature. We present one case of "de novo" brainstem cavernous malformation after radiation therapy adding to the increasing number of reported cases in the medical literature, and the case of a pregnant patient with symptomatic intracranial hemorrhage related to brainstem CMs to illustrate the complex nature in management of these patients, followed by a review of clinical and radiographic characteristics. CMs account for 8-15% of all intracranial and intraspinal vascular malformations. Although traditionally thought to be congenital in origin, CMs may present as acquired lesions particularly after intracranial radiation therapy. Clinical manifestations are protean and surgical treatment should be considered for patients with progressive neurologic deficits.


Neurosurgery ◽  
2002 ◽  
Vol 51 (4) ◽  
pp. 1075-1078 ◽  
Author(s):  
Yoshihiro Muragaki ◽  
Hiroshi Ujiie ◽  
Masayuki Ohno ◽  
Osami Kubo ◽  
Tomokatsu Hori

Abstract OBJECTIVE AND IMPORTANCE Vascular malformations in the optic pathway are rare. Only one case of pathologically confirmed arteriovenous malformation (AVM) of the optic nerve has been reported previously. We document the case of a patient with an optic nerve AVM who presented with optic apoplexy that was diagnosed with the use of magnetic resonance imaging. CLINICAL PRESENTATION A 15-year-old girl developed left visual disturbance of sudden onset while playing badminton. A magnetic resonance imaging scan disclosed left optic nerve swelling and intraoptical hemorrhage, although an angiogram did not reveal abnormal vessels. INTERVENTION The patient underwent total removal of the hematoma and tangles of the abnormal vessels in the left optic nerve, which was diagnosed pathologically as an AVM. The patient recovered visual acuity, but the left visual field defect remained unchanged. CONCLUSION Along with cavernous malformations and optic gliomas, AVMs can be a rare cause of optic nerve apoplexy. T2-weighted magnetic resonance imaging is useful in rendering the diagnosis of an optic nerve AVM, observed as a mass lesion consisting of serpiginous, tangled, low-intensity bands. Early surgical treatment is recommended to obtain a rapid recovery.


2000 ◽  
Vol 2 (3) ◽  
pp. 321-330

Cerebral vascular malformations with potential surgical consequences mainly consist of arteriovenous malformations (AVM) and cavernous malformations. The standard preoperative workup of these lesions includes basic neuroradiological investigation, such as computed tomography, magnetic resonance imaging (MRI), and magnetic resonance angiography, and conventional angiography, to assess the exact neurotopographical relationships of the nidus, arterial feeding pedicles, and venous drainage. In cases where lesions are located near or within eloquent areas, precise documentation of the anatomy can be obtained using various functional tests including functional MRI, activated positron emission tomoqraphy, and magnetoencephalography (MEG), which may then be integrated into a neuronavigational system allowing for selective, image-guided surgery, thus potentially reducing surgical morbidity. Preoperative embolization may in certain cases improve the surqical excision by reducinq blood flow throuqh the AVM. Cavernous malformations may also be removed with minimally invasive and highly selective techniques.


2015 ◽  
Vol 2015 ◽  
pp. 1-8 ◽  
Author(s):  
Nikolaos Mouchtouris ◽  
Nohra Chalouhi ◽  
Ameet Chitale ◽  
Robert M. Starke ◽  
Stavropoula I. Tjoumakaris ◽  
...  

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.


2011 ◽  
Vol 31 (6) ◽  
pp. E5 ◽  
Author(s):  
Richard T. Dalyai ◽  
George Ghobrial ◽  
Issam Awad ◽  
Stavropoula Tjoumakaris ◽  
L. Fernando Gonzalez ◽  
...  

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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