1型神经纤维瘤病中与面部丛状神经纤维瘤相关的颅内异常。

Intracranial abnormalities associated with facial plexiform neurofibromas in neurofibromatosis type 1.

作者信息

Boltshauser E, Stocker H, Sailer H, Valavanis A

机构信息

Department of Neurology, Children's University Hospital, Zurich, Switzerland.

出版信息

Neurofibromatosis. 1989;2(5-6):274-7.

PMID:2518508

Abstract

From 1975 to 1988 seventeen patients with neurofibromatosis type 1 and a disfiguring facial plexiform neurofibroma (FPN) were investigated. The FPN was left-sided in 13 patients. It was orbital/periorbital in 4, lower facial in 7 and involved the whole face in 6 subjects. Neuroimaging (n = 13) revealed a tumor (of optic pathways or basal ganglia) in 8, an ipsilateral middle cranial fossa arachnoid cyst in 2, multiple areas of high signal intensity (in T2-weighted magnetic resonance imaging) in 1, and normal findings in 2 patients. In NF-1 patients with FPN there seems to be a high incidence of intracranial tumors and possibly of arachnoid cysts. Our observation has to be confirmed in a larger patient series.

摘要

1975年至1988年期间，对17例1型神经纤维瘤病患者及伴有毁容性面部丛状神经纤维瘤（FPN）的患者进行了研究。13例患者的FPN位于左侧。4例为眼眶/眶周型，7例为面下部型，6例累及整个面部。神经影像学检查（n = 13）显示，8例存在肿瘤（视神经通路或基底神经节肿瘤），2例存在同侧中颅窝蛛网膜囊肿，1例在T2加权磁共振成像中有多个高信号强度区域，2例检查结果正常。在患有FPN的NF-1患者中，颅内肿瘤以及可能的蛛网膜囊肿发病率似乎较高。我们的观察结果有待在更大规模的患者系列中得到证实。