解放军医学杂志2025，Vol.50Issue(12)：1533-1539,7.DOI:10.11855/j.issn.0577-7402.1660.2025.0718
MRKH综合征Ⅱ型合并腹股沟卵巢输卵管绞窄性疝1例并文献复习
Strangulated inguinal hernia of the ovary and fallopian tube in MRKH syndrome type Ⅱ:a case report and literature review
Chen Zhen-Guang 1Wu Song-Yang 1Luo Yao 1Yu Jin-Yuan 1Fang Xiang-Ming 1Ye Zai-Yuan1
作者信息
- 1. @@@1 Department of Gastrointestinal Surgery,2Department of Gynecology,Shulan(Hangzhou)Hospital Affiliated to Zhejiang Shuren University Shulan International Medical College,Hangzhou,Zhejiang 310022,China||3 Department of Gastrointestinal and Pancreatic Surgery,Zhejiang Provincial People's Hospital/Affiliated People's Hospital,Hangzhou Medical College,Hangzhou,Zhejiang 310014,China
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摘要
Abstract
Objective To report the clinical and imaging characteristics of a patient with Mayer-Rokitansky-Küster-Hauser(MRKH)syndrome type Ⅱ complicated with strangulated inguinal hernia involving the ovary and fallopian tube,and to review relevant literature to improve the understanding,diagnosis and treatment of this disease.Methods Clinical data of 1 patient with MRKH syndrome type Ⅱ presenting with strangulated inguinal hernia containing ovarian and fallopian tube tissues were retrospectively analyzed.Literature on MRKH syndrome complicated with inguinal hernia of reproductive organs was comprehensively reviewed by searching Chinese and English databases.Results A 14-year-old female patient presented with an 8-year history of reducible right inguinal mass and acute pain with irreducibility for 1 day.Abdominal CT suggested a right indirect inguinal hernia,with ovarian tissue suspected within the sac.The left kidney and uterus were not visualized on imaging.Emergency surgical exploration was performed,and the hernia content was found to be ischemic and necrotic right ovary and fallopian tube.Right salpingo-oophorectomy and high ligation of hernial sac were performed.Postoperative spinal X-ray revealed lumbar scoliosis.The postoperative diagnosis was MRKH syndrome type Ⅱ complicated with right strangulated inguinal hernia of ovary and fallopian tube,left kidney agenesis,and scoliosis.A total of 26 cases of MRKH syndrome complicated with inguinal hernia of reproductive organs were retrieved from databases such as PubMed,China National Knowledge Infrastructure,and Wanfang.Combined with this case,there were 27 patients:the average age was 20.4 years,with left-sided and bilateral inguinal hernia accounting for 51.9%(14/27),33.3%(9/27)and 14.8%(4/27),respectively.Among the hernia contents,7 cases were ovary,5 cases were ovary and fallopian tube,13 cases were ovary and rudimentary uterus(with or without fallopian tube),and 2 cases were rudimentary uterus.Among 25 patients who underwent surgery,the hernia contents were reduced into the abdominal cavity in 15 cases,resected in 9 cases,and not described in 1 case.There were 12 cases of type Ⅰ and 15 cases of type Ⅱ of MRKH syndrome.In type I,left-sided and bilateral hernias each accounted for 50.0%(6/12).In type Ⅱ,unilateral hernias accounted for 80.0%(12/15),of which left-sided hernias accounted for 66.7%(8/12),and 78.6%(11/14)of patients with renal malformation complications had renal malformation on the same side as the inguinal hernia.Conclusions The incidence of inguinal hernia in female patients with MRKH syndrome is higher than that in healthy women.The inguinal hernias are mainly left-sided and bilateral in location.Patients with MRKH syndrome type Ⅱ are often accompanied by ipsilateral renal malformation.The hernia content is mainly ovary.Early diagnosis and treatment are crucial to prevent incarceration or torsion and preserve ovarian organ function.关键词
MRKH综合征/苗勒管发育不全/腹股沟疝/卵巢/子宫Key words
MRKH syndrome/Müllerian agenesis/inguinal hernia/ovary/uterus分类
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Chen Zhen-Guang,Wu Song-Yang,Luo Yao,Yu Jin-Yuan,Fang Xiang-Ming,Ye Zai-Yuan..MRKH综合征Ⅱ型合并腹股沟卵巢输卵管绞窄性疝1例并文献复习[J].解放军医学杂志,2025,50(12):1533-1539,7.
