Rupprecht T, Wenzel D, Schmitzer E, Hofbeck M, Böwing B, Neubauer U
Pediatric Hospital, University Erlangen-Nürnberg, FRG.
Pediatr Radiol. 1992;22(7):527-8. doi: 10.1007/BF02013002.
Moyamoya disease is a rare vascular anomaly of the cerebral arteries. The etiology of the disease has not yet been clearly identified. We report the noninvasive diagnosis of Moyamoya disease in a patients with a very early onset of symptoms in infancy. The diagnosis was made by colour coded Doppler sonography and confirmed by angiography at the age of 6 months, following two episodes of cerebral infarction. A bilateral encephalodurosynangiosis was performed at the age of 7 months with subsequent slight improvement of the neurological deficits. Colour Doppler sonography revealed early vascularisation from the fascia temporalis graft into the arachnoid space. At the age of 10 months the patient developed arterial hypertension caused by left renal artery stenosis. Our case suggests, that in infancy Moyamoya disease can be suspected noninvasively by colour Doppler sonography of the cerebral arteries. Patients should be carefully screened for possible extracranial arterial stenoses which may develop in the course of time. Encephalodurosynangiosis seems to be a good therapeutic option for patients with severe neurological symptoms.
烟雾病是一种罕见的脑动脉血管异常疾病。该病的病因尚未明确。我们报告了1例在婴儿期极早期出现症状的烟雾病患者的无创诊断情况。该诊断通过彩色编码多普勒超声做出,并在6个月龄时,在发生两次脑梗塞后经血管造影证实。患者在7个月龄时接受了双侧脑硬膜-颞肌贴敷术,随后神经功能缺损略有改善。彩色多普勒超声显示颞肌筋膜移植物向蛛网膜下腔早期血管化。10个月龄时,患者因左肾动脉狭窄出现动脉高血压。我们的病例表明,婴儿期烟雾病可通过脑动脉彩色多普勒超声进行无创性怀疑。应仔细筛查患者是否可能随着时间推移出现颅外动脉狭窄。对于有严重神经症状的患者,脑硬膜-颞肌贴敷术似乎是一种很好的治疗选择。