Reed D, Robertson W D, Rootman J, Douglas G
AJNR Am J Neuroradiol. 1986 Mar-Apr;7(2):259-63.
Seven patients with orbital plexiform neurofibromas (PNFs) were evaluated by computed tomography (CT). These lesions are pathognomonic of von Recklinghausen disease and manifest a spectrum of radiologic abnormalities ranging from thickening of the eyelid and periorbital soft tissues to enlargement of the bony orbit and extensive infiltration of the orbital soft tissues. In analyzing the CT features of orbital PNF, three findings that have not been previously reported were identified. Increased density of the intraconal fat present in four of seven patients was believed to represent PNF involvement of small intraconal nerves. Three patients demonstrated enhancing, irregularly nodular thickening of the optic nerve sheath complex thought to be caused by PNF of the posterior ciliary nerves surrounding the optic nerve. Three patients with buphthalmos and congenital glaucoma demonstrated thickening and enhancement of the uveal/scleral layer believed to represent PNF of these structures. Involvement of the choroid and sclera, posterior ciliary nerves, and small intraconal nerves has been recognized pathologically but has not been previously identified by CT.
对7例眼眶丛状神经纤维瘤（PNF）患者进行了计算机断层扫描（CT）评估。这些病变是冯·雷克林豪森病的特征性表现，呈现出一系列放射学异常，从眼睑和眶周软组织增厚到眼眶骨质增大以及眶内软组织广泛浸润。在分析眼眶PNF的CT特征时，发现了3个此前未报告过的表现。7例患者中有4例眶内脂肪密度增加，据信这代表了眶内小神经受PNF侵犯。3例患者表现出视神经鞘复合体强化、不规则结节状增厚，认为是围绕视神经的睫状后神经的PNF所致。3例患有牛眼和先天性青光眼的患者表现出葡萄膜/巩膜层增厚和强化，据信这代表了这些结构的PNF。脉络膜和巩膜、睫状后神经以及眶内小神经受累在病理学上已得到确认，但此前CT尚未发现。
