A comparison of the clinical profile of cavernous malformations with and without associated venous malformations.

OBJECTIVE: Little is known about the clinical behavior of cavernous malformations (CMs) associated with venous malformations (VMs) of the brain. The aim of this study is to compare the clinical profile of patients harboring CMs with and without associated VMs.

METHODS: A retrospective analysis of 55 consecutive patients harboring CMs of the brain who presented to a single neurovascular team during a 4-year period was performed. Forty-two patients (76%) had CMs alone (CM group), and 13 patients (24%) had CMs associated with VMs (CM + VM group). Detailed clinical information regarding each patient was gathered. Statistical analysis was performed using Fisher's exact test for binary variables and Mann-Whitney U test for continuous variables.

RESULTS: The lesion location was infratentorial for 19 of the 70 CMs (27%) in the CM group and for 14 of the 21 CMs (67%) in the CM + VM group (P = 0.001). Familial histories of CMs were documented for 7 of the 42 patients (17%) in the CM group and none of the 13 patients in the CM + VM group. There was a female-to-male gender bias of 1.6:1 in the CM group and 3.3:1 in the CM + VM group. Sixteen of the 42 patients (38%) in the CM group and 8 of the 13 patients (62%) in the CM + VM group presented with symptomatic hemorrhage. Seizure presentation was documented in 11 of the 42 patients (26%) in the CM group and in 1 of the 13 patients (8%) in the CM + VM group. Repeated symptomatic hemorrhage was diagnosed in 4 of the 42 patients (9.5%) in the CM group and in 3 of the 13 patients (23%) in the CM + VM group. There were no apparent differences in the mean age at presentation, lesion size, or multiplicity between the two groups.

CONCLUSION: Patients with CMs associated with VMs are more likely to be female patients, have associated symptomatic hemorrhage, have lesions in the posterior fossa (statistically significant), suffer from repeated symptomatic hemorrhage, and are less likely to present with seizures or to have familial histories when compared with patients with CMs alone. The possible mechanisms for these apparent differences in clinical profile are discussed.