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 【影像诊断】：Sphenoid wing dysplasia 蝶骨翼发育不良24I影像园XCTMR.com
【诊断要点】：Sphenoid wing dysplasia can occur as an isolated finding or in patients with neurofibromatosis type 1. 蝶骨翼发育不良可以孤立出现，也可以见于神经纤维瘤病I型。24I影像园XCTMR.com
Approximately 50% of cases are associated with neurofibromatosis type 1. 大约50％病例合并有神经纤维瘤病I型。24I影像园XCTMR.com
Sphenoid wing dysplasia is usually asymptomatic, but patients can present with pulsating exopthalmos or enophthalmos.通常无症状，但也可出现搏动性突眼或眼球内陷。24I影像园XCTMR.com
【讨论】：Sphenoid wing dysplasia can occur as an isolated finding or in patients who have Neurofibromatosis type 1. Approximately 50% of cases are associated with neurofibromatosis type 1. Sphenoid dysplasia has been reported to occur in 1 to 7% of patients with neurofibromatosis. Sphenoid wing lesions are usually asymptomatic, but patients can present with pulsating exophthalmos or enopthalmos. Others can present with vision impairment, extra-ocular muscle impairment or inflammation of the conjunctiva. 蝶骨翼发育不良可以是一个孤立性的发现，也可以见于神经纤维瘤病I型患者。大约有50％的病例合并有神经纤维瘤病I型。1-7％的神经纤维瘤病患者可见有蝶骨翼发育不良，其他的异常可以有视力障碍、眼外肌损伤或结膜炎症。24I影像园XCTMR.com
The radiological features of sphenoid wing dysplasia are a defect in a part or all of the greater wing, elevation of the lesser wing and distortion of the sella. There may be elevation of the superior orbital fissure, enlargement of the temporal fossa in all directions and lifting of the sphenoid ridge out of the orbit.蝶骨翼发育不良的放射学特征是蝶骨大翼的部分或全部缺如，蝶骨小翼的抬高和蝶鞍变形。眶上裂可能抬高、中颅窝的增大、蝶骨脊抬高超出眼眶24I影像园XCTMR.com
The most likely cause of sphenoid dysplasia is defective ossification of the sphenoid bone. In patients with NF-1, sphenoid wing dysplasia was initially thought to be congenital and non-progressive. It is now believed to be progressive in some patients with NF-1. Sphenoid wing dysplasia is one of six clinical criteria for the diagnosis of NF-1.引起蝶骨发育不良最可能的原因是蝶骨骨化不全。在NF-1病人中，蝶骨翼发育不良最初被认为是先天性的和非进行性的。现在我们相信在某些NF-1患者中，这是进行性的改变。蝶骨翼发育不良是NF-1诊断的六个临床规范之一。24I影像园XCTMR.com
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