Intracranial cavernous malformations — natural history and management

Abstract

Thirteen papers on different aspects of cerebral cavernomas are reviewed; interest in this condition has increased since magnetic resonance imaging (MRI) became widely available. The prevalence of cavernomas is uncertain, but they are the most common of the angiographically occult vascular malformations. Some are isolated occurrences, and some are familial, with a dominant inheritance. Screening with MRI of first-degree relatives is justified. The reported annual risk of haemorrhage varies widely and is probably between 1 and 3%, with a possible increase in risk after a first haemorrhage; the risk may also be higher in deep or brain stem cavernomas. Opinions on when surgery should be offered vary, with prophylactic surgery not usually recommended. After symptomatic haemorrhages, surgery may be more justified for easily accessible lesions. For those that are more difficult to approach safely, especially in the brain stem, operation is not usually recommended unless there has been at least one clinically significant haemorrhage. Epilepsy owing to hemisphere cavernomas can often be successfully managed medically, with surgery reserved for intractable seizures. In children with epilepsy, there is a stronger argument for surgery. Radiosurgery has been used for symptomatic cavernomas that are surgically inaccessible. Strong arguments have been advanced both for and against this treatment, and the risks probably outweigh the benefits. Suggestions for a randomised trial have been made.

Cerebral cavernous malformations and epilepsy

“…Of patients who harbor a single CCM, undergo lesionectomy for treatment of recent-onset, localization-related seizures, and are seizure free postoperatively, up to half may successfully taper off all anticonvulsant medications. 21,35,64,69 This promising outcome, and its associated positive impact on quality of life, may play a role in the decision to excise a solitary accessible cortical CCM, even when seizures are not truly intractable to medical therapy.…”

Neurosurgical management of cerebellar cavernous malformations

“…11,39,51,52 In most series, however, an equal distribution between the sexes 13,24,26,31,40,43,48,53 has been reported, and this was also the case in ours. Some authors have suggested a higher propensity for bleeding episodes in female patients, 16,48 often during pregnancy. 45 There has also been a report of sudden death in a pregnant patient, in whom a cerebellar CM was diagnosed at autopsy.…”

“…For those who have suffered previous bleeding, the annual risk increases to 4.3% per patient. 16 In those harboring posterior fossa CMs and those who have experienced a previous hemorrhage it is 6.75 and 7.78 times more likely, respectively, that they will present with neurological sequelae. 39, 45 Cantu, et al, 10 reported that the risk of intracerebral hemorrhage was only 1.22% per patient per year in lobar CMs and 2.33, 2.39, and 2.82% per patient per year for brainstem, cerebellum, and deep hemispheric CMs, respectively.…”

“…Patients with symptomatic CMs, with sudden or progressive presentation (with or without radiological evidence of bleeding), and those with recurrent hemorrhage should be considered for surgical treatment. 16,29,31 Porter and coworkers 43 showed that after a hemorrhage from a CM treated conservatively, only 37% of patients experienced complete recovery and 27% did not improve at all. Consequently, some authors recommend surgical treatment even for asymptomatic CMs, especially if there is evidence of growth on neuroimages.…”

“…Consequently, some authors recommend surgical treatment even for asymptomatic CMs, especially if there is evidence of growth on neuroimages. 16 Surgical management of posterior fossa CMs depends on a wide number of factors such as the patient's clinical condition, age, lesion location, hemorrhage size, presence of mass effect and hydrocephalus, and the morbidity inherent to a surgical approach. 8 Lesions located in highly eloquent areas such the brainstem, the CPA, and the cranial nerves in the posterior fossa usually present with higher morbidity and mortality rates compared with cerebellar CMs, not only as the consequence of bleeding but also because these lesions frequently require a complex surgical approach.…”