[Management of multiple cerebral cavernomatosis].

INTRODUCTION

Cavernous angiomas are angiographically occult vascular malformations that are present in 0.4% of people, and represent 5 13% of all cerebrovascular malformations. They can be alone or multiple, and sporadic or familial. The presence of multiple lesions is more frequent in familial cavernomatosis.

OBJECTIVES

Improve our knowledge of the natural history of multiple cavernomatosis in order to improve our diagnostic and therapeutic management of this entity.

PATIENTS AND METHODS

We have retrospectively reviewed 18 cases of multiple cerebral cavernomatosis; 4 of them belonged to the same family and 2 belonged to another family. Number, size, characteristics and evolution of the lesions, symptoms, treatment and clinical outcome have been analysed during a follow up period longer than 5 years.

RESULTS

31.5% of the cavernous angiomas reviewed by our department were multiple (at least three lesions). During the 5 year follow up period only four (4/18) patients underwent surgical treatment. 50% of patients suffered at least one hemorrhagic event with clinical impairment, and the most frequent manifestations were headache, focal deficit and seizures. The hemorrhagic rate per lesion per year was under 1%, for the more than 200 lesions and the low frequency of hemorrhagic events with clinical impairment in the time.

CONCLUSIONS

Surgical treatment must be considered in patients with accessible lesions that have produced symptoms several or progressive symptoms. The non surgical patients should be followed with yearly MRI. When more than one first degree relative has a cavernous malformation or familial antecedent with cerebral hemorrhage or epilepsy, serial follow up monitoring consisting of physical examinations and MRI should be suggested to family members.