Zhao Yao, Du Gu-Hong, Wang Yong-Fei, Wu Jing-Song, Xie Li-Qian, Mao Ying, Zhou Liang-Fu
Department of Neurosurgery, Shanghai Neurosurgical Center, Huashan Hospital, Shanghai Medical College, Fudan University, Shanghai 200040, China.
Surg Neurol. 2007 Nov;68(5):493-9; discussion 499. doi: 10.1016/j.surneu.2007.02.044. Epub 2007 Aug 17.
Although patients with MCMs have increasingly been found in clinics, little has been focused on them. Thus, we intended to investigate these patients' clinical presentations, family history, radiological characters, and treatment strategy.
A retrospective review of the files and family investigations were conducted for 30 patients with MCMs. All patients underwent MRI examination. Symptomatic patients underwent the surgical treatment with image-guided technique.
There were 19 male and 11 female patients with a total 79 lesions. The common presentations were seizures, hemorrhages, or focal neurological deficits. Nine patients had positive or doubtful family history. The FLAIR sequence of MRI showed the highest sensitivity in the detection of CM lesions. In 27 symptomatic patients with 69 lesions, total removal was achieved in 19 patients with 48 lesions. In the other 8 patients with 21 lesions, 13 lesions were removed. Preoperative symptoms were improved in 21 patients and unchanged in 5. Preoperative neurological deficits temporarily worsened in one, and a new onset of seizure occurred in other one; but both gradually improved during the follow-up period. Among 3 patients with asymptomatic MCMs, one patient had hemorrhage during the follow-up period and underwent surgical operation.
Because a high frequency of family CM occurs in MCMs, a detailed family investigation is mandatory for each patient with MCM. Selection of higher sensitive MRI sequence would contribute to detection of more CM lesions. Microsurgery assisted with the neuroimaging techniques is the treatment of choice for symptomatic MCMs.
尽管临床上越来越多地发现患有多发性海绵状血管瘤(MCMs)的患者,但对他们的关注却很少。因此,我们旨在调查这些患者的临床表现、家族史、影像学特征及治疗策略。
对30例MCMs患者的病历进行回顾性分析并开展家族调查。所有患者均接受了磁共振成像(MRI)检查。有症状的患者采用影像引导技术进行手术治疗。
30例患者中,男性19例,女性11例,共有79个病灶。常见表现为癫痫发作、出血或局灶性神经功能缺损。9例患者有阳性或可疑家族史。MRI的液体衰减反转恢复(FLAIR)序列在检测海绵状血管瘤(CM)病灶方面显示出最高的敏感性。在27例有症状的患者(共69个病灶)中,19例患者(48个病灶)实现了完全切除。另外8例患者(21个病灶)中,13个病灶被切除。21例患者术前症状改善,5例未变。1例患者术前神经功能缺损暂时恶化,另1例出现新发癫痫发作,但在随访期间均逐渐改善。3例无症状MCMs患者中,1例在随访期间出血并接受了手术。
由于MCMs中家族性CM的发生率较高,因此对每例MCMs患者进行详细的家族调查是必要的。选择更高敏感性的MRI序列有助于检测出更多的CM病灶。显微手术联合神经影像技术是有症状MCMs的首选治疗方法。