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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 investigate the MRI features of Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome. Methods The clinical data and MRI findings of 23 patients with MRKH syndrome were retrospectively analyzed. Results All 23 cases had primary amenorrhea, 5 cases had periodic lower abdominal pain, 1 case had umbilical and shameless hair, and female hormone was low. In MRI, 10 cases showed bilateral primordial uterus, 4 cases showed unilateral primordial uterus, 9 cases showed complete uterine loss; 10 cases showed partial vaginal closure, 13 cases showed complete vaginal closure; 3 cases Combined urinary deformity, 6 cases of skeletal deformity. Conclusion MRI can clearly show the involvement of uterus, vagina and other accompanying deformities in MRKH syndrome. Uterine involvement can be manifested as bilateral primordial uterus, unilateral primordial uterus or uterus completely missing; vaginal involvement showed atresia or partial atresia, multiple system with deformity involving the kidney, skeletal system involvement more common. Different conditions involved, MRI performance is also different, but are easy to identify.