Cerebral cavernous malformations: natural history and prognosis after clinical deterioration with or without hemorrhage

Abstract

Despite recent studies of the natural history of cavernous malformations, there remains significant uncertainty concerning hemorrhage rates and the importance of lesion location. Controversy arises over varying definitions of "hemorrhage." What is ultimately important to the patient is the occurrence of a neurological event, which may or may not be associated with radiologically documented hemorrhage, as well as the chance of recovery after such an event. The purpose of this study was to determine the rates of occurrence and sequelae of neurological events in 173 patients referred to our vascular malformation clinic with cavernous malformations. All patient data were entered into a database. The mean age at presentation for the 173 patients was 37.5 years. The lesion location was deep (brainstem, cerebellar nuclei, thalamus, or basal ganglia) in 64 patients (37%) and superficial in 109 (63%). Thirty-one patients (18%) had multiple lesions. Disease presentation was due to seizures in 62 patients (36%), hemorrhage in 44 (25%), focal neurological deficit without documented hemorrhage in 35 (20%), headache alone in 11 (6%), and incidental findings in 21 patients (12%). The results obtained in the 110 patients eligible for follow-up review were used to derive information on the rates of hemorrhage and neurological events. An interval event (neurological deterioration) required both symptoms and signs. The total mean follow-up period was 46 months, the majority (65%) of which was prospective. There were 18 interval events in 427 patient-years of follow-up review, for an overall annual event rate of 4.2%. Location was the most important factor for predicting interval event occurrence, with significantly higher rates for deeply located (10.6%/year) compared with superficially located lesions (0%/year) (p = 0.0001). Of patients suffering a neurological event, only 37% had complete resolution of their deficits. This largely prospective study indicates that deep cavernous malformations carry a worse prognosis than superficial lesions with respect to annual rates of neurological deterioration. The alarming rate of adverse clinical events occurring in patients with deep lesions is punctuated by the fact that less than one-half of them recover fully during long-term follow-up review.

The natural history of intracranial cavernous malformations

“…Robinson et al 26 reported an annual hemorrhage rate of 0.7% per lesion-year. The studies of Kondziolka et al, 15 Moriarity et al, 20 and Porter et al 23 reported annual hemorrhage rates ranging from 1.6% to 3.1% per patient-year. Across these 3 studies including a total of 363 patients followed up for 1121.5 patient-years, the overall hemorrhage rate was 2.4% per patient-year.…”

“…7,8,14,15,20,23,26 Developmental venous anomalies were seen in 54 (9%) of 593 patients in 6 studies reporting associated vascular malformations. 7,15,20,23,26,31 Table 2 summarizes overall epidemiological, clinical presentation, and hemorrhage rate data.…”

“…2,7,14,20,23,26,31 Across 7 general distribution studies with 656 patients, 125 patients (19%) harbored multiple intracranial CMs. 7,8,14,15,20,23,26 Developmental venous anomalies were seen in 54 (9%) of 593 patients in 6 studies reporting associated vascular malformations. 7,15,20,23,26,31 Table 2 summarizes overall epidemiological, clinical presentation, and hemorrhage rate data.…”

Ressonância magnética dos cavernomas intraventriculares: aspectos diagnósticos

“…Suas paredes são constituídas de células endoteliais simples e fina camada fibrosa de adventícia. Não possuem camada muscular, lâmina elástica e não existe tecido nervoso no seu interior 1,3,5 . Eles podem apresentar tromboses, calcificações e frequentemente são encontrados produtos de degradação sanguí-nea em vários estágios de evolução, sugerindo a ocorrência de repetidos sangramentos no interior da lesão 8,10 .…”

Direct Approach to the Ventrolateral Medulla for Cavernous Malformation. Case Report.

“…5,7,[13][14][15][16]21) Neurological deficits are likely to worsen due to repeated hemorrhages, so the current trend is toward aggressive management of lesions reaching the pial or ependymal surface of the brainstem, but extreme care is required perioperatively since vital structures in life-support functions are located at the brainstem. 2,5,9,[11][12][13][14]16,21) Direct resection through various approaches has been used for cavernous malformations located primarily in the midbrain and pons since there is only minor risk of inflicting further neurological damage. 4,6,8,[10][11][12][17][18][19] The indications for surgical treatment and selection of the most appropriate surgical routes may be indeterminable for cavernous malformations with neither bulging nor extension into the pial or ependymal surface.…”