Crouzon综合征颅面部的CT表现(附8例报告)

Craniofacial CT Findings of Crouzon Syndrome(Report of Eight Cases)

目的 总结Crouzon综合征颅面部的CT表现,提高对该病的认识。资料与方法回顾性总结分析8例Crouzon综合征颅面部CT征象。结果 CT均表现为眼眶、鼻腔鼻窦、颞骨、颌骨及颅底的异常。眼眶:眶腔变浅,眶外壁夹角增大,眶距增宽以及眼球突出16眼,视神经迂曲7眼,眼外肌增粗10眼;鼻腔鼻窦:鼻腔狭窄伴鼻中隔偏曲8例,鼻窦发育差6例,鼻咽腔变窄7例;颞骨:壶腹型内耳道8例,乳突气化差4例,乳突炎4例;颌骨:上颌骨发育不良,硬腭高拱,口腔顶壁呈倒"V"字形6例;颅底:颅骨内板弥漫性深大脑回压迹,蝶骨大翼狭长菲薄,颞窝变小,前颅窝底低位,颈静脉球窝增大8例,蝶鞍异常5例,乙状窦前位4例。结论 CT能够为Crouzon综合征颌面部的异常改变及其并发症的评估提供客观依据。

Objective To summarize craniofacial CT findings of Crouzon＇s syndrome.Materials and Meth-ods Craniofacial CT features in 8 patients with Crouzon＇s syndrome were analyzed retrospectively.Results The malformation involved orbit,rhinosinus,temporal bones,jaw bones and skull base.The CT abnormalities of orbit included shallow orbits with proptosis,hypertelorism in 16 eyes,circuitous optic nerve in7 eyes and thickening extraocular muscles in 10 eyes.The CT abnormalities of rhinosinus included rhinostenosis and septal deviation in 8 cases,narrowing cavum nasopharyngeum in 6 cases and hypoplastic accessory nasal cavityin 7 cases.Abnormalities of temporal bones included ampulla-type internal auditory canal in 8 cases,opacitified mastoid and mastoiditis in 4 cases.Maxillary bone hypoplasia,high-arched palate,roof of mouth with an inverted ＂V＂ appearance in 6 cases were found at jaw bones.The abnormalities of skull base included diffuse indentation of inner table of skull,the slender ala temporalis ossis sphenoidalis,shallow temporal fossa,low-set anterior cranial fossa,accrescent jugular fossa in 8 cases,anteposition of the sigmoid sinus in 4 cases and sella turcica abnormalities in 5 cases.Conclusion CT images can provide valuable information of craniofacial abnormalities of the Crouzon＇s syndrome and its complication.