摘要
目的探讨Mayer-Rokitansky-Küster-Hauser(MRKH)综合征的MRI表现特点。方法回顾性分析23例MRKH综合征患者的临床资料和MRI影像表现。结果 23例均有原发性闭经,5例伴有周期性下腹痛,1例脐、耻无毛发,女性激素低。在MRI上,10例表现为双侧始基子宫,4例表现为单侧始基子宫,9例表现为子宫完全缺失;10例表现为阴道部分闭锁,13例表现为阴道完全闭锁;3例合并泌尿系畸形,6例合并骨骼畸形。结论 MRI可以清楚显示MRKH综合征的子宫、阴道受累及其他伴随畸形。子宫受累可表现为双侧始基子宫、单侧始基子宫或子宫完全缺失;阴道受累表现为闭锁或部分闭锁,多系统伴随畸形以肾脏、骨骼系统受累多见。受累情况不同,MRI表现亦不同,但均易于辨识。
Objective To explore the MRI features of Mayer-Rokitansky-Küster-Hauser（ MRKH） syndrome. Methods A retrospective analysis of clinical data and MRI findings in 23 patients with MRKH syndrome. Results All subjects suffered from primary amenorrhea,5 complained cyclic lower abdominal pain,1 with sparse pubic hair and low estrogen hormones. Bilateral rudimentary uteri were visible in 10 cases,4 cases showed unilateral rudimentary uterus,the uterus was totally absent for 9 cases. Ten cases were involved with partial vaginal atresia,the rest 13 cases manifested complete vaginal atresia. Three renal malformations and 6 skeletal abnormalities were detected. Conclusion Uterine and vaginal malformation as well as concomitant malformation can be clearly and thoroughly manifested upon MRI. Uterus involvement can appear as bilateral rudimentary uterus,unilateral rudimentary uterus or absence of the uterus. Vaginal involvement includes partial or complete agenesis. Renal and skeletal abnormalities are common accompanying malformations.MRI findings vary in consistent with the variable involvement,yet an accurate identification is not difficult in MRI.
出处
《临床放射学杂志》
CSCD
北大核心
2016年第4期552-555,共4页
Journal of Clinical Radiology
关键词
MRKH综合征
磁共振成像
原发闭经
MRKH syndrome
Magnetic resonance imaging
Primary amenorrhea
