MRKH综合征伴一侧子宫腺肌病1例并文献复习

A rare case of MRKH with adenomyosis and literature review.

目的探讨Mayer-Rokitansky-Kuster-Hauser综合征(MRKH综合征)合并子宫腺肌病发生的可能机制及诊治方法。方法回顾性分析北京协和医院2009年4月收治的1例47岁MRKH综合征合并子宫腺肌病患者的诊治经过,并复习相关文献。结果患者表现为周期性下腹痛10年,妇科检查发现阴道成盲端,长度6cm,无子宫。MRI检查提示盆腔实性包块。血CA125明显增高。腹腔镜探查见无正常发育子宫,右侧始基子宫,左侧子宫腺肌病孕10周左右大小。双侧卵巢输卵管正常。腹腔镜下切除左侧子宫,子宫标本切面为腺肌病改变,无内膜结构,病理提示子宫腺肌病,无内膜,雌激素受体(+)、孕激素受体(+)。结论MRKH综合征可以合并子宫腺肌病,提示子宫腺肌病的发生可能与苗勒管的残余化生有关。

Objective To investigate the possible pathogenesis, diagnosis and treatment of Mayer-Rokitansky-Kuster- Hauser syndrome （MRKH syndrome） with adenomyosis. Methods We reported a 47-year-old women of MRKH syndrome with adenomyosis treated in Peking Union Medical College Hospital in April 2009, and reviewed the related literature. Results The patient complained of cyclic low abdominal pain for 10 years. PeLvic examination revealed vagina of 6 cm in length, and no cervix and uterus. MRI showed a solid mass in the left pelvic cavity. Serum CA125 was 138.2U/ L. Laparoscopy showed adenomyosis of left rudimentary uterine horn with 10 weeks gestational age in size, and right rudi- mentary uterine horn of 2cm × 1.5cm ×1cm . Both ovaries and tubes were normal. The left uterine horn was laparoscopically excised. Macroscopic appearance showed adenomyotic change of myometrium without uterine cavity. The histopatho- logical examination confirmed the diagnosis of adenomyosis with positive ER and PR, and positive CD10. Conclusion The development of adenomyosis on uterine remnants is possible in patients with MRKH, may develop from the mullerian remnants.