多脾综合征的CT诊断  

CT Diagnosis of Polysplenia Syndrome

作　　者：李文智 王卫斌[2] 罗林 杨昆良 王智平[3] 文利[3] 李虹玲 王光宪[3] 

机构地区：[1]云南曲靖市第一人民医院放射科,655000 [2]解放军第425医院放射科,海南省三亚市572000 [3]第三军医大学新桥医院放射科,重庆400037

出　　处：《临床放射学杂志》2015年第12期2009-2013,共5页Journal of Clinical Radiology

摘　　要：目的探讨多脾综合征的MSCT表现及其诊断价值。方法回顾性分析10冽多脾综合征患者的临床和CT表现。结果9例合并多器官及心血管系统畸形，1例仅合并房间隔缺损。CT表现主要有：（1）多个脾结节。脾脏数目4-14个；（2）内脏异常。全内脏反位1例，部分内脏反位3例，短胰6例，环状胰腺1例，水平肝2例，小肠旋转不良1例，马蹄肾1例，双肺左侧异构3例；（3）心血管畸形。下腔静脉肝段缺如，肝静脉直接汇入右心房，奇静脉或半奇静脉扩张与上腔静脉异常连接8例；双上腔静脉2例；脾动脉开口于肠系膜上动脉2例；胃左动脉开口于肠系膜上动脉1例；肝总动脉开口于腹主动脉1例；十二指肠前门静脉2例；合并先天性心脏病2例。结论CT不仅可清晰显示脾脏的数目及位置，而且可显示合并其他内脏异常及心血管畸形，对多脾综合征可做出准确、全面的诊断。Objective To investigate the diagnostic value of muhislice spiral CT polysplenia syndrome and to raise awareness of the disease. Methods To retrospective analysis 10 cases of CT data and clinical information of polysplenia syndrome. Results There were 9 cases with multiple organ and cardiovascular system abnormalities, 1 case of atrial sep- tal defect. CT manifestations ： （ 1 ） Multiple splenic nodules, the number of spleen from 4 to 14. （2） Visceral abnormalities ： Full situs inversus 1 case, partial situs inversus 3 cases, short pancreas 6 cases, 1 case of annular pancreas, level liver 2 cases, 1 case of intestinal malrotation, horseshoe kidney 1 case, two lungs left side heterogeneous 3 cases. （3） Cardiovas- cular malformations：IVC hepatic segment absent, hepatic vein directly into the right atrium, azygos vein or half azygos vein dilation connection with the superior vena cava 8 cases ; double superior vena cava 2 cases ; splenic artery opening in the su- perior mesenteric artery 2 cases;gastric left artery opening in the superior mesenteric artery 1 case; hepatic artery opening in the abdominal aorta 1 case ;2 cases with preduodenal portal vein, and 2 cases associated with congenital heart disease. Conclusion CT can clearly show not only the number and location of the spleen, but also combined with other visceral ab- normalities and cardiovascular malformations, it can accurate and comprehensive diagnosis.

关 键 词：多脾综合征 内脏 心血管 畸形 体层摄影术 X线计算机 

分 类 号：R551.1[医药卫生—血液循环系统疾病] R816[医药卫生—内科学]