目的：探讨Crouzon综合征CT影像表现。方法回顾性分析21例Crouzon综合征患者的头颅三维CT影像资料，总结其异常CT表现。结果 Crouzon综合征头颅三维影像表现包括颅骨、眼眶、面中部及颈椎的异常。颅骨：5例舟状头畸形，4例尖头畸形，3例短头畸形；15例颅骨内板“指压迹”，12例垂体窝增大；6例双侧内听道壶腹样扩大，2例双侧内听道狭窄；8例共10侧颈静脉球高位；8例蝶骨体发育不良；12例双侧外耳道向下倾斜，2例3侧骨性外耳道闭锁。眼眶：21例均表现为不同程度双侧眼眶外侧壁夹角增大，眶距增宽，眼眶变浅，眼球突出，视神经迂曲。面中部：21例病例均有不同程度面中部凹陷，下颌骨相对前突；16例表现为鼻中隔偏曲；14例表现为腭弓高耸；2例表现为鼻泪管缩短，管径增宽。颈椎：5例颈椎融合畸形；1例椎体蝴蝶椎。结论 Crouzon综合征颅CT表现纷繁多样，诊断时多平面重建及三维影像结合观察，可对这一少见疾病的正确诊断提供帮助。%Objective To discuss CT findings of Crouzon syndrome. Methods The imaging data of 3D skull CT in 21 cases with Crouzon syndrome were analyzed retrospectively. Results The malformation involved skull, orbit, midface and cervical spine. The CT abnormalities of skull included scaphocephaly in 5 cases, oxycephaly in 4 cases, brachycephaly in 3 cases, diffuse indentation of inner table of skull in 15 cases, widening of hypophysial fossa in 12 cases, ampulla-type internal auditory canal in 6 cases, internal auditory canal stenosis in 2 cases, 10 sides high riding jugular bulb in 8 cases, corpora ossis sphenoidalis dysplasia in 8 cases, downward sloping external acous-tic meatus in 12 cases, 3 sides atresia of external auditory canal in 2 cases. CT abnormalities of orbit included in-creased lateral orbital wall angle, hypertelorism, shallow orbit, proptosis and circuitous optic nerve. CT abnormalities of midface included concave midface and mandible relative to the protrusion in all 21 cases, septal deviation in 16 cas-es, hypoplasia, high-arched palate in 14 cases, shortening and widening naso-lacrymal duct in 2 cases. The CT abnor-malities of cervical spine included cervical fusions in 5 cases, butterfly vertebrae in 1 case. Conclusion CT findings of Crouzon syndrome vary. Multiplanar reconstruction and 3D images combined can provide valuable information in diagnosing this rare disease.
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