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脑动脉扩张与结节性硬化症:病例报告

Cerebral arterial ectasia and tuberous sclerosis: case report.

作者信息

Spangler W J, Cosgrove G R, Moumdjian R A, Montes J L

机构信息

Neurosurgical Service, Massachusetts General Hospital, Harvard Medical School, Boston, USA.

出版信息

Neurosurgery. 1997 Jan;40(1):191-3; discussion 193-4. doi: 10.1097/00006123-199701000-00042.

DOI:10.1097/00006123-199701000-00042
PMID:8971842
Abstract

OBJECTIVE AND IMPORTANCE

Tuberous sclerosis is associated with a wide variety of central nervous system abnormalities. Cerebrovascular anomalies are extremely rare, but a case of cerebral arterial ectasia and giant fusiform aneurysm formation in a young child is reported.

CLINICAL PRESENTATION

A 5-month-old male patient with tuberous sclerosis presented with seizures, a subependymal tumor, and intraventricular hemorrhage. Cerebral angiography demonstrated a large fusiform aneurysm of the left cavernous internal carotid artery as well as arterial ectasia of the proximal left anterior cerebral and middle cerebral arteries. The patient developed hydrocephalus and died of infectious complications after repeated shunt procedures.

CONCLUSION

Tuberous sclerosis is commonly associated with central nervous system lesions. Although rare, cerebrovascular anomalies and aneurysms should be considered in the differential diagnosis of mass lesions to avoid an ill-advised biopsy of a vascular lesion, which could have disastrous consequences.

摘要

目的及重要性

结节性硬化症与多种中枢神经系统异常相关。脑血管异常极为罕见,但本文报告了一例幼儿发生脑动脉扩张和巨大梭形动脉瘤形成的病例。

临床表现

一名5个月大的结节性硬化症男性患者出现癫痫发作、室管膜下肿瘤和脑室内出血。脑血管造影显示左侧海绵窦段颈内动脉有一个巨大的梭形动脉瘤,以及左侧大脑前动脉近端和大脑中动脉的动脉扩张。该患者发生了脑积水,并在多次分流手术后死于感染性并发症。

结论

结节性硬化症通常与中枢神经系统病变相关。尽管罕见,但在对占位性病变进行鉴别诊断时应考虑到脑血管异常和动脉瘤,以避免对血管病变进行不当活检,这可能会带来灾难性后果。

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