标题：21例Crouzon综合征头颅三维CT分析

      作者：谢毓芝 1，林 涛 2，朱 凌 1，朱文静 1
    (1.上海交通大学医学院附属第九人民医院放射科，上海 200011；
2.上海浦东新区周浦医院放射科，上海 201318)

      卷次： 2015年26卷2期

      【摘要】 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。

      【关键词】 Crouzon综合征；体层摄影术；X线计算机

      【中图分类号】 R442.8 【文献标识码】 A 【文章编号】 1003—6350（2015）02—0198—03


3D CT analysis of 21 cases of Crouzon syndrome.
XIE Yu-zhi 1, LIN Tao 2, ZHU Ling 1, ZHU Wen-jing 1. 1.
Department of Radiology, the Ninth People's Hospital of Shanghai Jiao Tong University School of Medicine, Shanghai
200011, CHINA; 2. Shanghai Pudong New Area Zhoupu Hospital, Shanghai 201318, CHINA

【Abstract】 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.

      【Key words】 Crouzon syndrome; Tomography; X-ray computed