Abdulrauf S I, Kaynar M Y, Awad I A
Department of Neurosurgery, Neurovascular Surgery Program, Yale University School of Medicine, New Haven, Connecticut 06520, USA.
Neurosurgery. 1999 Jan;44(1):41-6; discussion 46-7. doi: 10.1097/00006123-199901000-00020.
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.
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.
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.
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.
关于与脑静脉畸形（VMs）相关的海绵状血管畸形（CMs）的临床行为，人们了解甚少。本研究的目的是比较伴有和不伴有相关VMs的CMs患者的临床特征。
对4年间向单一神经血管团队就诊的55例连续性脑CMs患者进行回顾性分析。42例患者（76%）仅有CMs（CM组），13例患者（24%）的CMs与VMs相关（CM + VM组）。收集了每位患者的详细临床信息。使用Fisher精确检验对二元变量进行统计分析，使用Mann-Whitney U检验对连续变量进行统计分析。
CM组70个CMs中的19个（27%）病变位于幕下，CM + VM组21个CMs中的14个（67%）病变位于幕下（P = 0.001）。CM组42例患者中有7例（17%）有CMs家族史，CM + VM组13例患者中无一例有家族史。CM组女性与男性的性别比为1.6:1，CM + VM组为3.3:1。CM组42例患者中有16例（38%）出现症状性出血，CM + VM组13例患者中有8例（62%）出现症状性出血。CM组42例患者中有11例（26%）记录有癫痫发作，CM + VM组13例患者中有1例（8%）记录有癫痫发作。CM组42例患者中有4例（9.5%）被诊断为反复症状性出血，CM + VM组13例患者中有3例（23%）被诊断为反复症状性出血。两组在就诊时的平均年龄、病变大小或多发性方面无明显差异。
与仅有CMs的患者相比，伴有VMs的CMs患者更可能为女性，有相关症状性出血，后颅窝有病变（具有统计学意义），患有反复症状性出血，出现癫痫发作的可能性较小或无家族史。讨论了这些临床特征明显差异的可能机制。
