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丘脑海绵状畸形

Thalamic cavernous malformations.

作者信息

Pozzati E

机构信息

Division of Neurosurgery, Bellaria Hospital, Bologna, Italy.

出版信息

Surg Neurol. 2000 Jan;53(1):30-9; discussion 39-40. doi: 10.1016/s0090-3019(99)00164-0.

DOI:10.1016/s0090-3019(99)00164-0
PMID:10697231
Abstract

BACKGROUND

Only few anecdotal reports and small series of thalamic cavernous malformations have been reported. It follows that the clinical behavior and management are poorly understood; in particular, experiences with the surgical treatment of these lesions are scarce.

METHODS

The clinical course, treatment, and outcome of 12 patients (10 females and 2 males, mean age 36 years) with symptomatic cavernous malformations of the thalamus are reviewed. Eight patients (66%) presented with cerebral hemorrhage, one with progressive neurological deficit and three with hydrocephalus/increased intracranial pressure; associated venous anomalies were found in three cases. Treatment consisted of radical surgery in four cases with progressive neurological decline or recurrent disabling hemorrhage, radiosurgery (one case), evacuation of a chronic satellite hematoma (one case), ventriculoperitoneal shunt for hydrocephalus (one case) and observation (five cases). Operative treatment in four cases included transcallosal, trigonal, and occipital interhemispheric approaches.

RESULTS

In the surgical group, one patient died, two improved after operation, and one remained the same. Of the patients not operated on radically, one had recurrent hemorrhage 4 months after radiosurgery, one remains stable 8 years after ventriculoperitoneal shunt, and one 3 years after aspiration of a satellite hematoma. Five other patients presenting with thalamic hemorrhage were treated conservatively; recurrent hemorrhage occurred in two cases at 1 month and at 2 years, leaving a mild residual deficit in both cases. Overall, rehemorrhage occurred in four cases (50%) at a mean interval of 18 months after the first bleeding; the annual hemorrhage rate was 6.1%.

CONCLUSIONS

Thalamic malformations are more likely to be symptomatic from small hemorrhages compared with lesions in the cerebral hemispheres; progressive growth may also occur with third ventricle invasion or caudal extension to the midbrain. Their high-risk location deters heavy-handed management, but they should not be left long untreated. Both surgery and radiosurgery have been used in the management of thalamic cavernomas reported in the literature. Definite surgical indications include progressive neurological decline and recurrent hemorrhages of malformations abutting the ventricular surface or the posterior incisural space; the complex anatomy of the deep venous system and the association with unexpected venous anomalies complicates the removal of these lesions.

摘要

背景

仅有少数关于丘脑海绵状血管畸形的病例报告和小样本系列研究。因此,对其临床行为和治疗方法了解甚少;尤其是,关于这些病变手术治疗的经验稀缺。

方法

回顾了12例有症状的丘脑海绵状血管畸形患者(10例女性,2例男性,平均年龄36岁)的临床病程、治疗及结果。8例(66%)表现为脑出血,1例为进行性神经功能缺损,3例为脑积水/颅内压升高;3例发现有相关静脉异常。4例进行性神经功能衰退或复发性致残性出血患者接受了根治性手术,1例接受了放射外科治疗,1例清除了慢性卫星血肿,1例因脑积水行脑室腹腔分流术,5例进行观察。4例手术治疗采用经胼胝体、三角区和枕叶半球间入路。

结果

手术组中,1例患者死亡,2例术后改善,1例无变化。未接受根治性手术的患者中,1例放射外科治疗后4个月复发出血,1例脑室腹腔分流术后8年病情稳定,1例抽吸卫星血肿后3年病情稳定。另外5例丘脑出血患者接受保守治疗;2例分别在1个月和2年时复发出血,均遗留轻度残余功能缺损。总体而言,4例(50%)在首次出血后平均18个月复发出血;年出血率为6.1%。

结论

与大脑半球病变相比,丘脑畸形更易因小出血而出现症状;随着第三脑室受侵或尾端延伸至中脑,也可能发生渐进性生长。其高危位置阻碍了激进的治疗,但也不应长期不予治疗。文献报道中,手术和放射外科均用于丘脑海绵状血管瘤的治疗。明确的手术指征包括进行性神经功能衰退以及毗邻脑室表面或后切迹间隙的畸形复发性出血;深部静脉系统的复杂解剖结构以及与意外静脉异常的关联使这些病变的切除变得复杂。

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An analysis of the natural history of cavernous malformations.海绵状血管畸形的自然病史分析。
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