儿童Kallmann综合征的影像表现  

Imaging features in children with Kallmann syndrome

作　　者：温洋[1] 彭芸[1] 尹光恒[1] 刘玥[1] 张明[1] 

机构地区：[1]100045首都医科大学附属北京儿童医院影像中心磁共振成像设备与技术北京市重点实验室

出　　处：《中华放射学杂志》2013年第7期603-606,共4页Chinese Journal of Radiology

基　　金：国家自然科学基金资助项目（31271161）;北京市卫生系统高层次卫生技术人才培养项目（2011-3-054）;北京市科技重大专项（2011zx09302-007-01）

摘　　要：目的总结儿童Kallmann综合征（Ks）的影像表现，提高对该病的认识。方法搜集经临床证实且有影像资料的Ks患儿13例，患儿均经颅脑MR、左腕骨x线检查，其中8例行腹盆腔B超检查，分析总结其影像表现。结果13例患儿中，9例双侧嗅球、嗅束缺如；4例一侧嗅球小、嗅束细小或显示不清，另一侧嗅球嗅束缺如。8例双侧嗅沟不同程度浅小，其中2例一侧嗅沟消失；3例一侧嗅沟浅、一侧正常；2例双侧嗅沟正常。3例伴垂体前叶小。6例骨龄落后。B超检查8例中，双侧睾丸均小。结论儿童KS均有嗅脑发育不良的MRI表现，部分伴垂体前叶发育不良；近半数伴骨龄落后；双侧睾丸均小。Objective To summarize imaging features of Kallmann syndrome （KS） in children and to improve diagnostic level of the disease. Methods The imaging manifestations of 13 patients with clinically proved KS were retrospectively studied. MRI of rhinencephalon and left wrist X-ray examinations were performed in all the 13 children. Ultrasound of abdomen and pelvis was implemented in the 8 of 13 patients. Results All the patients had abnormalities of olfactory system. Bilateral olfactory bulbs and tracts were absent in 9 patients. Unilateral olfactory bulbs and tracts were absent in 4 patients, in which contralateral olfactory bulbs and tracts were hypoplastic. These patients presented 2 aplastic, 17 hypoplastie and 7 normal olfactory sulci. Anterior pituitary was hypoplastie in 3 patients. Bone age showed retardation in 6 of 13 patients. Bilateral testicles were small in all the 8 cases by ultrasound. Conclusions Aplastic or hypoplastic olfactory bulbs and tracts and sulci are seen in children with KS. Anterior pituitary dysplasia is present in part of patients and bone age backward happens in approximate half of the children.

关 键 词：卡尔曼综合征 磁共振成像 嗅觉障碍 

分 类 号：R725.8[医药卫生—儿科]