• Yifei Dai, Chenglu Qin, Linling Zhu, and Guangnan Luo.
• Department of Gynecology, Hangzhou Women's Hosptial, Hangzhou, Zhejiang, China.
• Medicine (Baltimore). 2023 Feb 3; 102 (5): e32802e32802.

RationaleMayer-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.Patient ConcernsCase 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.DiagnosesHernia uterine inguinale associated with MRKH syndrome.Interventions And OutcomesCase 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.LessonsLeft 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.Copyright © 2023 the Author(s). Published by Wolters Kluwer Health, Inc.

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