Abdolrahimzadeh Solmaz, Felli Lorenzo, Plateroti Rocco, Plateroti Andrea Maria, Giustini Sandra, Calvieri Stefano, Recupero Santi Maria
Ophthalmology Unit, DAI Testa/Collo, Azienda Policlinico Umberto I, University of Rome "Sapienza", Rome, Italy.
Ophthalmology Unit, Organi di Senso Department, University of Rome "Sapienza", Rome, Italy.
Br J Ophthalmol. 2015 Jun;99(6):789-93. doi: 10.1136/bjophthalmol-2014-306062. Epub 2014 Dec 8.
BACKGROUND/AIMS: A normal structural and functional choroid is essential in supplying blood flow to the retina. Neurofibromatosis type 1 (NF1) is a neurocristopathy where the choroid is altered due to the presence of nodules. The present transversal study was conducted to examine choroidal nodules and their effect on choroidal and retinal thickness in NF1 patients.
Near-infrared reflectance and optical coherence tomography with enhanced depth imaging were used to evaluate choroidal morphology and vasculature in 19 patients with NF1 and 19 healthy, age-matched control subjects. Choroidal thickness, neuroepithelium thickness, photoreceptors together with retinal pigment epithelium (RPE) thickness and outer nuclear layer (ONL) thickness were measured at the fovea and 1000 μm nasal, temporal, superior and inferior to the fovea in NF1 patients and control subjects. Choroidal and neuroepithelium thickness were assessed overlying and adjacent to nodules in NF1 patients.
Choroidal nodules were classified as 'dome-shaped' or 'placcoid' subtypes in 17 patients. Small and medium calibre choroidal vessels were observed above dome-shaped nodules where choroidal thickness was significantly reduced. There was a statistically significant reduction in mean choroidal thickness (p=0.013) in NF1 patients with respect to control subjects. The neuroepithelium, photoreceptors together with RPE and ONL had a statistically significant reduction in mean thickness in NF1 patients (p<0.001, p<0.001, p=0.012, respectively).
In NF1, there are dome-shaped and placcoid choroidal nodules which alter choroidal morphology and thickness. There is reduction in mean choroid thickness with generalised thinning of the neuroepithelium, photoreceptors together with RPE and ONL in NF1 patients.
背景/目的：正常的脉络膜结构和功能对于向视网膜供血至关重要。1型神经纤维瘤病（NF1）是一种神经嵴病，因存在结节而导致脉络膜发生改变。本横断面研究旨在检查NF1患者的脉络膜结节及其对脉络膜和视网膜厚度的影响。
采用近红外反射和增强深度成像光学相干断层扫描技术，对19例NF1患者和19名年龄匹配的健康对照者的脉络膜形态和血管系统进行评估。在NF1患者和对照者的黄斑中心凹以及黄斑中心凹鼻侧、颞侧、上方和下方1000μm处测量脉络膜厚度、神经上皮厚度、光感受器连同视网膜色素上皮（RPE）厚度以及外核层（ONL）厚度。对NF1患者结节上方及相邻部位的脉络膜和神经上皮厚度进行评估。
17例患者的脉络膜结节被分类为“圆顶状”或“扁平状”亚型。在圆顶状结节上方观察到中小口径的脉络膜血管，此处脉络膜厚度显著降低。与对照者相比，NF1患者的平均脉络膜厚度有统计学意义的降低（p = 0.013）。NF1患者的神经上皮、光感受器连同RPE和ONL的平均厚度有统计学意义的降低（分别为p < 0.001、p < 0.001、p = 0.012）。
在NF1中，存在圆顶状和扁平状脉络膜结节，它们会改变脉络膜形态和厚度。NF1患者的平均脉络膜厚度降低，同时神经上皮、光感受器连同RPE和ONL普遍变薄。
