通讯作者: 王烈宏, doctorwifi@126.com

DOI：10.12201/bmr.202501.00072

One case of congenital no cervix, no vaginal, bilateral chocolate cysts with MRKH syndrome (type II)

• 摘要：在妇产科临床工作中，苗勒管发育不全（Mayer-Rokitansky-KüsterHauser,MRKH）综合征II型合并先天性无宫颈、阴道闭锁、双侧巧克力囊肿是一种极其罕见的生殖系统先天性发育畸形性疾病，典型表现为子宫和阴道的发育不良或缺如。MRKH综合征II型患者除生殖道异常外，常伴随卵巢病变（如双侧巧克力囊肿）以及其他系统的异常（如泌尿系统或骨骼发育异常）。本文阐述了1 例MRKH综合征II型合并先天性无宫颈、阴道闭锁、双侧巧克力囊肿患者的治疗经过，总结该患者的诊疗经过并结合文献复习，以期提升临床医生对该病的认知。

  关键词： MRKH综合征；巧克力囊肿；女性生殖道先天性畸形；阴道成形术; ; ; 

   

  Abstract: In the clinical work of obstetrics and gynecology,congenital absence of cervix,absence of vagina,bilateral chocolate cyst with MRKH syndrome (type II) is an extremely rare congenital dysmorphic disease of the reproductive system,which is typically manifested by dysplasia or absence of uterus and vagina.Patients with MRKH syndrome type II often have ovarian lesions (eg,bilateral chocolate cysts) and other systemic abnormalities (eg,urinary or skeletal dysplasia) in addition to genital tract abnormalities.This article describes the treatment of a patient with congenital cervical,nonvaginal,bilateral chocolate cyst and MRKH syndrome (type II).The patient,a 17-year-old female,was admitted to the hospital complaining of menstrual cramps and lower abdominal pain for 2 days.Gynecologic examination reveals vulva:poorly developed pubic hair,poorly developed labia minora,small hymen orifice,visible separation in the middle,and cotton swabs unable to penetrate.Pelvic MRI was performed immediately afterwards,and the results showed endometrial thickening,cervical dysplasia,no visible in the lower one-third of the vagina,and no development of the caudal vertebrae.Combined with the results of the patients auxiliary examination, under the discussion of the department director and all doctors,and with the consent of the patients family,it is planned to decide to undergo laparoscopic vaginoplasty (acellular allodermis) + cervicopy + hysteroscopy + laparoscopic bilateral ovarian cystectomy + ultrasound-guided uterine balloon placement.After careful care after the operation,the results of the re-examination of B-ultrasound improved significantly,and the patient was successfully discharged from the hospital.

  Key words: MRKH syndrome; chocolate cyst; congenital malformation of the female genital tract; vaginoplasty.

  提交时间：2025-01-23

  版权声明：作者本人独立拥有该论文的版权，预印本系统仅拥有论文的永久保存权利。任何人未经允许不得重复使用。
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• 序号	提交日期	编号	操作
  1	2025-01-05	bmr.202501.00072V1	下载

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