Department of Gynecology, Hangzhou Women's Hosptial, Hangzhou, Zhejiang, China.
Department of Gynecology, The Third Affiliated Hospital of Shenzhen University, Luohu People Hospital, Shenzhen, Guangdong, China.
Medicine (Baltimore). 2023 Feb 3;102(5):e32802. doi: 10.1097/MD.0000000000032802.
Mayer-Rokitansky-Küster-Hauser syndrome (MRKH syndrome) present with genital inguinal hernia was rare and probably under reported, on account of lack in typical gynecological symptom. It should be regarded with care.Here 3 cases diagnosed at our institution with detailed clinical information were present, and the literature was reviewed to paint a comprehensive profile of hernia uterine inguinale associated with MRKH syndrome.
Case no. 1 was a 36-year-old female with recurrent dragalgia for 5 years. Left rudimentary uterus at the left groin area was revealed by sonography scan and confirmed by diagnostic laparoscopy.Case no. 2 was a 27-year-old woman diagnosed with MRKH syndrome and her MRI examination suggested a suspicious swelling measuring 2.0cm×2.0cm in left groin. The left nonfunctionally rudimentary uterus and adnexa were incarcerated in the left inguinal hernial sac, which was revealed by laparoscopy.Case no. 3 was a 29-year-old woman, admitted with right abdominal pain with a provisional diagnosis of appendicitis. After appendicectomy, pelvic exploration showed a part of left rudimentary uterus and elongated oviduct herniated through the left internal inguinal ring.
Hernia uterine inguinale associated with MRKH syndrome.
Case no.1: When the rudimentary uterus was pulled out from the hernia sac, it appearance dark ocher. Then the left rudimentary uterus was removed and the indirect defect of inguinal duct was closed.The patient was followed up for 18 months with no recurrence of abdominal pain.Case no.2 and 3:The left rudimentary uterus were replaced from the hernia sac, and the indirect defect was fixed with sutures.The patients recovered smoothly without complications for 12-month follow-up.
Left involvement of rudimentary uterus was frequently observed in patients with MRKH syndrome, along with ipsilateral ovary and/or fallopian tube horned in the hernia. Abdominal pain or inguinale mass could be the chief complaints while some individuals were asymptomatic. Either surgical removal or replacement of rudimentary uterus was an effectively optional treatment strategy for hernia uterine inguinale.When a patient with MRKH syndrome presented with abdominal pain of unknown cause or inguinal mass, rudimentary uterine inguinal hernia should be suspected.
梅克尔-罗基坦斯基-库斯特-豪泽综合征（MRKH 综合征）合并腹股沟疝的生殖器疝较为罕见，可能由于缺乏典型的妇科症状而被低估。应谨慎对待。本文报道了 3 例在本机构诊断的病例，详细的临床资料，并回顾文献，描绘了与 MRKH 综合征相关的疝子宫腹股沟的全面特征。
病例 1 为 36 岁女性，反复腹股沟疼痛 5 年。超声检查显示左侧腹股沟区有发育不全子宫，诊断性腹腔镜检查证实。病例 2 为 27 岁女性，诊断为 MRKH 综合征，MRI 检查提示左侧腹股沟可疑肿胀，大小为 2.0cm×2.0cm。腹腔镜检查显示左侧无功能发育不全子宫和附件被嵌顿在左侧腹股沟疝囊内。病例 3 为 29 岁女性，因右腹痛就诊，初步诊断为阑尾炎。阑尾切除术后，盆腔探查显示部分左侧发育不全子宫和细长输卵管通过左侧内环疝出。
疝子宫腹股沟与 MRKH 综合征相关。
病例 1：将发育不全子宫从疝囊内牵拉出来时，呈暗褐色。然后切除左侧发育不全子宫，关闭腹股沟管间接缺损。患者随访 18 个月，无腹痛复发。病例 2 和 3：将左侧发育不全子宫从疝囊内复位，用缝线固定间接缺损。患者在 12 个月的随访中恢复顺利，无并发症。
MRKH 综合征患者常伴有同侧卵巢和/或输卵管 horn 疝入发育不全子宫。腹痛或腹股沟肿块可能是主要症状，而有些患者无症状。无论是手术切除还是发育不全子宫复位，都是治疗疝子宫腹股沟的有效选择。当 MRKH 综合征患者出现原因不明的腹痛或腹股沟肿块时，应怀疑存在发育不全子宫腹股沟疝。
