Fuchs F D, Francesconi C R, Caramori P R, da Silva Júnior R, Finger C, Costa L F
Divisions of Cardiology, Hospital de Clínicas de Porto Alegre, Porto Alegre, RS, Brazil.
J Hum Hypertens. 2001 Jul;15(7):499-501. doi: 10.1038/sj.jhh.1001207.
Moyamoya is a rare disease characterized by fibrous dysplasia of the internal carotid and proximal cerebral arteries, which has been described mainly in young Japanese. We present a case of Moyamoya disease with renal artery involvement in a young male patient with an African origin. A 15-year-old boy was referred to our hospital due to uncontrolled blood pressure, headache, somnolence, cognitive deficit and multiple lacunar infarcts in the computed tomography. Cerebral arteriography showed the absence of the normal vascular anatomy at the level of the circle of Willis. The intracranial vessels presented severe stenosis or were occluded and replaced by an extensive network of ectasic collateral vessels. Abdominal ultrasound examination identified asymmetric kidneys, and renal arteriography showed a tight stenosis of the ostium and proximal segment of right main artery, which was only partially relieved by balloon angioplasty. A saphenous bypass from aorta to the right renal artery re-established the renal blood flow. Blood pressure dropped after surgery and was controlled with low doses of diuretic and beta-blocker. After arteriography he presented right hemiplegia, with partial recovering in the following months. In conclusion, we described the first case of Moyamoya disease with concomitant renovascular disease in a young adult of African origin, an association that may be more frequent than usually suspected in clinical practice.
烟雾病是一种罕见疾病,其特征为颈内动脉和大脑近端动脉的纤维发育异常,主要在年轻日本人群中被描述。我们报告一例起源于非洲的年轻男性烟雾病患者合并肾动脉受累的病例。一名15岁男孩因血压控制不佳、头痛、嗜睡、认知缺陷以及计算机断层扫描显示多发腔隙性梗死而被转诊至我院。脑血管造影显示 Willis 环水平正常血管解剖结构缺失。颅内血管呈现严重狭窄或闭塞,并被广泛的扩张侧支血管网络所取代。腹部超声检查发现双侧肾脏不对称,肾动脉造影显示右主肾动脉开口及近端节段严重狭窄,球囊血管成形术仅部分缓解了狭窄。从主动脉到右肾动脉的大隐静脉搭桥术重建了肾血流。术后血压下降,通过低剂量利尿剂和β受体阻滞剂得以控制。血管造影术后他出现了右侧偏瘫,在随后几个月部分恢复。总之,我们描述了首例起源于非洲的年轻成年人烟雾病合并肾血管疾病的病例,这种关联在临床实践中可能比通常怀疑的更为常见。