Quaranta Luciano, Semeraro Francesco, Turano Raffaele, Gandolfo Enrico
Centro Glaucoma, Clinica Oculistica dell'Università di Brescia, Brescia, Italy.
J Glaucoma. 2004 Apr;13(2):90-5. doi: 10.1097/00061198-200404000-00002.
Based on the known neurocristopathic etiology of type 1 neurofibromatosis (NF1) and the neuroectodermal embryologic derivation of the iridocorneal angle, we examined a sample of young patients affected with NF1 to see if they have evidence of underdevelopment of the angular region.
We designed a case-controlled clinical study. Forty-two consecutive patients (42 eyes), 24 male and 18 female, affected with NF1 were recruited for the study. Forty-two eyes of 42 consecutive young patients (19 male and 23 female) served as a control group for the iridocorneal angle features studied. Indirect gonioscopy was performed by the means of a Goldmann lens. The intraocular pressure was measured with a Goldmann applanation tonometer. Photographs were taken of the anterior segment and of all the four quadrants of the iridocorneal angle to record the presence of abnormalities. The iridocorneal angle was graded according to the classification proposed by Spaeth. Evaluation of the angle also included the gonioscopic width of ciliary body band (CBB).
In this study we found that 29 of 42 eyes (69%) of the NF1 group had mild anteriorization, even if within normal limits, of the iris insertion and abundant basal iris processes. The CBB was either invisible (54.84%) or very narrow (21.4%). Three NF1 patients had bilateral juvenile congenital glaucoma.
It seems that patients affected with NF1 often have characteristic gonioscopic findings consistent with underdevelopment of the iridocorneal angle.
基于已知的1型神经纤维瘤病（NF1）的神经嵴病变病因以及虹膜角膜角的神经外胚层胚胎学起源，我们对一组患有NF1的年轻患者样本进行了检查，以确定他们是否有虹膜角膜角区域发育不全的证据。
我们设计了一项病例对照临床研究。连续招募了42例（42只眼）患有NF1的患者，其中男性24例，女性18例。连续42例年轻患者（19例男性和23例女性）的42只眼作为研究虹膜角膜角特征的对照组。使用戈德曼接触镜进行间接前房角镜检查。用戈德曼压平眼压计测量眼压。拍摄眼前段和虹膜角膜角所有四个象限的照片以记录异常情况。根据Spaeth提出的分类方法对虹膜角膜角进行分级。对房角的评估还包括前房角镜下睫状体带（CBB）的宽度。
在本研究中，我们发现NF1组42只眼中有29只眼（69%）即使在正常范围内也有虹膜附着轻度前移以及丰富的虹膜基质突起。睫状体带要么不可见（54.84%），要么非常狭窄（21.4%）。3例NF1患者患有双侧青少年先天性青光眼。
似乎患有NF1的患者常有与虹膜角膜角发育不全一致的特征性前房角镜检查结果。
