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年轻女性单侧腹股沟肿胀:MURCS的一种罕见表现

Unilateral Inguinal Swelling in a Young Female: An Unusual Presentation of MURCS.

作者信息

Kaur Navdeep, Satija Bhawna, Mahal Sayani, Arora Shubham

机构信息

Department of Radiodiagnosis, Lady Hardinge Medical College, New Delhi, India.

出版信息

J Hum Reprod Sci. 2025 Jan-Mar;18(1):54-56. doi: 10.4103/jhrs.jhrs_190_24. Epub 2025 Mar 29.

Abstract

Inguinal hernia in females is an uncommon entity. While most patients present in infancy or early in childhood, only a few cases are diagnosed in adulthood. Most cases of inguinal hernia have small bowel or omentum as its content. Herniation of the ovary or fallopian tube is rare. In our case, an 18-year-old female presented to the outpatient department with unilateral inguinal swelling, which on imaging was found to be ovarian inguinal herniation. This prompted further evaluation. There was an associated absence of the uterus and left kidney, and congenital block vertebrae involving the cervical spine. On probing it was found that she had primary amenorrhoea with normal secondary sexual characteristics. All the findings led to the diagnosis of Mayer Rokitansky Kuster Hauser type II or Mullerian duct aplasia renal agenesis cervicothoracic somite dysplasia (MURCS) with unilateral inguinal ovarian herniation. Mullerian duct aplasia renal agenesis cervicothoracic somite dysplasia (MURCS) present a challenge as they require a multidisciplinary team including gynaecologist, surgeon and psychologist to preserve the ovarian function and help the patient counsel regarding the reproductive outcome and wade through the associated emotional stress.

摘要

女性腹股沟疝是一种罕见的病症。虽然大多数患者在婴儿期或儿童早期发病,但只有少数病例在成年期被诊断出来。大多数腹股沟疝病例的疝内容物为小肠或网膜。卵巢或输卵管疝很少见。在我们的病例中,一名18岁女性因单侧腹股沟肿胀到门诊就诊,影像学检查发现是卵巢腹股沟疝。这促使进一步评估。患者伴有子宫和左肾缺如,以及涉及颈椎的先天性椎体融合。经检查发现她原发性闭经,但第二性征正常。所有这些发现导致诊断为梅耶-罗基坦斯基-库斯特-豪泽综合征II型或苗勒管发育不全-肾发育不全-颈胸节段发育异常(MURCS)伴单侧腹股沟卵巢疝。苗勒管发育不全-肾发育不全-颈胸节段发育异常(MURCS)是一个挑战,因为它们需要一个多学科团队,包括妇科医生、外科医生和心理学家,以保留卵巢功能,并帮助患者咨询生殖结果,以及应对相关的情绪压力。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/25ff/12057836/e6f4209a2cfa/JHRS-18-54-g001.jpg

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