In a series of 145 patients with brain cavernous angiomas treated at our hospital in the last 16 years, the angiomas of 18 patients exhibited aggressive biological behavior characterized by recurrent overt bleeding, growth, or de novo appearance. The cavernomas were in the cerebellum in three patients, in the brain stem in one, in the thalamus in four, in the caudate nucleus in two, in the diencephalon in one, and in the white matter of the cerebral hemispheres in seven. Three of these patients suffered from the familial or multiple form of the disease, two were pregnant, three had previously been irradiated for other tumors, and one had been treated by radiosurgery in the past. Overall, new cavernous malformations not previously shown were discovered in six patients. In 10 patients (3 male and 7 female) presenting with recurrent hemorrhages, the mean period of time between bleedings was 11 months (range, 1 wk-3 yr). Eleven patients were treated by definitive surgery, and seven were conservatively treated. One patient with a diencephalic cavernoma died from progressive hypothalamic dysfunction; three patients in the nonsurgical group had repeated symptoms and were left with additional neurological deficits. The outcome of the surgical group was the same (seven patients) or improved (four patients). Risk factors favoring an aggressive behavior included pregnancy, familial or multiple form of the disease, previous whole brain or stereotactic radiotherapy, incomplete removal, brain location, and associated venous malformation. The female preponderance (female to male ratio, 13:5) may also suggest some role of hormonal factors in influencing the biological behavior of cavernous malformations.
Summary: Purpose:The optimal management of cerebral cavernous malformations (CCMs) with epileptic seizures is still a matter of debate. The aim of our study was to examine seizure outcome in the largest published series of surgically treated patients with epilepsy due to a supratentorial CCM, and to define predictors for good surgical outcome.Methods: We retrospectively studied 168 consecutive patients with a single supratentorial CCM and symptomatic epilepsy in a multicenter study. Pre-and postoperative clinical examinations, age at epilepsy onset, age at operation, type of symptoms due to the CCM (seizures, headache, hemorrhage, focal deficits), type and frequency of epileptic seizures, and the localization and size of the CCM were assessed. Seizure outcome was determined in the first, second, and third postoperative years. Results:The CCM was completely resected in all patients. More than two thirds of the patients were classified as seizure free in the first 3 postoperative years. Predictors for good seizure outcome were age older than 30 years at the time of surgery, mesiotemporal CCM localization, CCM size <1.5 cm, and the absence of secondarily generalized seizures. No mortality occurred in our series, but only mild postoperative neurologic deficits in 12 (7%) patients.Conclusions: Considering the natural history of CCMs, the favorable neurologic and seizure outcome, surgical resection of CCMs should be considered in all patients with supratentorial CCMs and concomitant epilepsy, irrespective of the presence or absence of predictors for a favorable seizure outcome.
Objective: The main clinico-diagnostic features, risk factors and associated diseases of cavernous malformations (CMs), also called cavernous angiomas or cavernomas, of the central nervous system (CNS) in children are described, and the most relevant differences compared to the affected adult population are pointed out, focusing on the management of pediatric patients harboring cranial and spinal CMs. Materials: This was a retrospective study of a series of 42 children symptomatic for CMs of the cranial and spinal compartments (35 supratentorial brain lesions, 5 infratentorial and 2 in the spinal region) operated on between 1975 and 2005, with a clinical follow-up ranging from 12 to 192 months. The results were compared with those found in the most recent literature dealing with pediatric CMs of the CNS. Results: Surgical treatment produced excellent or good results in 69% of our 42 children. Unchanged neurological deficits were observed in 23.8% of cases, while morbidity from surgical procedures was 7.14%. Mortality was absent in this series. These surgical results are comparable with those found in the literature, where morbidity and mortality rates from surgery are 8.8 and 1.13%, respectively, and are mostly associated with procedures for the excision of deep, critically located cavernomas. Conclusion: CMs represent the most common CNS vascular lesion in children, although their incidence is 4 times lower than that of the adult population. The natural history of pediatric CMs throughout the neuraxis seems to be more aggressive than in adult patients; these lesions have higher rates of growth and hemorrhage, larger dimensions and often atypical radiological pictures at diagnosis. Beside the familial form of the disease, which is more often associated with multiple lesions and an earlier age of clinical presentation, the major risk factor for CMs in children seems to be radiotherapy for CNS tumors. Furthermore, a greater number of CMs coexistent with mixed angiomatous lesions have been reported in children than in adults. Surgical results are related to the preoperative neurological status of the children; symptomatic patients who are operated on early, before they develop severe neurological deficits or long-standing seizures, may achieve the best clinical outcome. Radiosurgery does not seem to be advisable in children as an alternative treatment for deep CMs or those causing epilepsy.
Increased awareness of occult cerebrovascular malformations is necessary, because their occurrence is not infrequent and they have hemorrhagic potential. Children receiving cerebral irradiation are at greater risk of this complication.
In a series of 145 patients with brain cavernous angiomas treated at our hospital in the last 16 years, the angiomas of 18 patients exhibited aggressive biological behavior characterized by recurrent overt bleeding, growth, or de novo appearance. The cavernomas were in the cerebellum in three patients, in the brain stem in one, in the thalamus in four, in the caudate nucleus in two, in the diencephalon in one, and in the white matter of the cerebral hemispheres in seven. Three of these patients suffered from the familial or multiple form of the disease, two were pregnant, three had previously been irradiated for other tumors, and one had been treated by radiosurgery in the past. Overall, new cavernous malformations not previously shown were discovered in six patients. In 10 patients (3 male and 7 female) presenting with recurrent hemorrhages, the mean period of time between bleedings was 11 months (range, 1 wk-3 yr). Eleven patients were treated by definitive surgery, and seven were conservatively treated. One patient with a diencephalic cavernoma died from progressive hypothalamic dysfunction; three patients in the nonsurgical group had repeated symptoms and were left with additional neurological deficits. The outcome of the surgical group was the same (seven patients) or improved (four patients). Risk factors favoring an aggressive behavior included pregnancy, familial or multiple form of the disease, previous whole brain or stereotactic radiotherapy, incomplete removal, brain location, and associated venous malformation. The female preponderance (female to male ratio, 13:5) may also suggest some role of hormonal factors in influencing the biological behavior of cavernous malformations.
Cavernous angiomas, also called cavernous malformations or cavernomas, are vascular hamartomas accounting for 3-16% of all angiomatous lesions of the spinal district. Although histologically identical, these vascular anomalies may exhibit different clinical behavior and radiological features, depending on their location, hinting at different managements and therapeutic approaches. The authors report 11 cases of symptomatic spinal cavernous angiomas diagnosed and surgically treated over the past 18 years. Age of patients ranged from 15-75 years; males outnumbered females. Three patients had vertebral cavernous malformations, secondarily invading the epidural space; two had pure epidural lesions; two patients had intradural extramedullary lesions, and four intramedullary lesions. Surgical removal was completely achieved in four patients with intramedullary lesions, in two with subdural extramedullary lesions, and in one with a pure epidural lesion. Subtotal excision of another one epidural and three vertebral cavernous angiomas was followed by radiotherapy. There was no morbidity related to surgery; the mean follow-up was 2 years. The outcome was excellent in two cases, good in six, and unchanged in the other three. The authors discuss the different modalities of treatment of these vascular lesions variously placed along the spine.
We retrospectively studied 19 cases of occlusive cervical carotid dissection encountered at our hospital between 1974 and 1984 and followed for 5-13 (mean 8.2) years to assess the long-term prognosis of the disease. Five patients had transient ischemic attacks, seven had minor stroke, six had major stroke, and one had epileptic seizures. Angiography demonstrated the typical string sign in 17 cases, a double lumen with occlusion in one, and multiple scalloped narrowings with distal occlusion in the other. Three patients died within 1 month and three remain severely disabled (overall mortality and major morbidity 32%), five have permanent deficits, and seven are neurologically intact; the remaining patient was lost to follow-up. Five patients were treated surgically (two had extracranial-intracranial bypass and three had cervical carotid exploration), and the other 14 were treated medically. The overall rate of reopening was 47% with eight of 10 patients demonstrating recanalization on control angiography and another patient demonstrating recanalization at surgery. These nine patients remain clinically stable on follow-up evaluations. However, vascular abnormalities in the healed arteries were notable and include kinking, nbromuscular dysplasia, dissecting aneurysms, intracranial occlusion, and a residual mural defect (Stroke 1990;21:528-531)
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