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一名常染色体显性遗传性多囊肾病患者,此前记录的无症状性小颅内动脉瘤破裂。病例报告。

Rupture of a previously documented small asymptomatic intracranial aneurysm in a patient with autosomal dominant polycystic kidney disease. Case report.

作者信息

Schievink W I, Prendergast V, Zabramski J M

机构信息

Division of Neurological Surgery, Barrow Neurological Institute, St. Joseph's Hospital and Medical Center, Phoenix, Arizona, USA.

出版信息

J Neurosurg. 1998 Sep;89(3):479-82. doi: 10.3171/jns.1998.89.3.0479.

Abstract

Intracranial aneurysms are common extrarenal manifestations of autosomal dominant polycystic kidney disease (ADPKD). Although their natural history is not completely understood, small asymptomatic intracranial aneurysms in patients with ADPKD often are not treated but are followed with serial magnetic resonance (MR) angiography. The authors report the unique case of a patient with ADPKD who bled from a previously documented asymptomatic 3-mm intracranial aneurysm. This 42-year-old man with ADPKD suffered a subarachnoid hemorrhage (SAH) from a 7-mm left pericallosal artery aneurysm. This aneurysm was clipped and the patient made an excellent recovery. An irregular asymptomatic 3-mm right middle cerebral artery (MCA) aneurysm had also been demonstrated on angiography. While the patient was considering elective surgery for the MCA aneurysm, he suffered a hemorrhage from this lesion 10 weeks after the initial SAH. The aneurysm was clipped and the patient made a satisfactory recovery (he was moderately disabled). In this report the authors indicate that small asymptomatic intracranial aneurysms are not always innocuous in patients with ADPKD, and they suggest that treatment should be strongly considered for these lesions in this group of patients when there is a history of SAH or the aneurysm is irregular in appearance. Because MR angiography studies may not adequately define the configuration of small aneurysms and irregularity may easily be missed, conventional angiography is recommended for patients with ADPKD who are found to have an intracranial aneurysm on screening with MR angiography.

摘要

颅内动脉瘤是常染色体显性多囊肾病(ADPKD)常见的肾外表现。尽管其自然病程尚未完全明确,但ADPKD患者的无症状小颅内动脉瘤通常不予治疗,而是通过连续磁共振(MR)血管造影进行随访。作者报告了1例独特的ADPKD患者,其颅内一个之前记录为无症状的3mm动脉瘤发生了出血。这名42岁的ADPKD男性因左侧胼周动脉7mm动脉瘤发生蛛网膜下腔出血(SAH)。该动脉瘤进行了夹闭手术,患者恢复良好。血管造影还显示一个不规则的无症状3mm右侧大脑中动脉(MCA)动脉瘤。当患者考虑对MCA动脉瘤进行择期手术时,在首次SAH后10周,该病变发生了出血。该动脉瘤进行了夹闭手术,患者恢复情况令人满意(有中度残疾)。在本报告中,作者指出,ADPKD患者的无症状小颅内动脉瘤并非总是无害的,他们建议,对于有SAH病史或动脉瘤外观不规则的这组患者,应强烈考虑对这些病变进行治疗。由于MR血管造影研究可能无法充分明确小动脉瘤的形态,且容易漏诊不规则情况,因此对于经MR血管造影筛查发现有颅内动脉瘤的ADPKD患者,建议进行传统血管造影。

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