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伴有盆腔融合肾的II型梅耶-罗基坦斯基-库斯特-豪泽综合征：一例报告

Mayer-Rokitansky-Kuster-Hauser Syndrome Type II with Fused Kidneys in Pelvic Cavity: A Case Report.

作者信息

Paudel Suman, Rokaha Prerana Singh, Kafle Pankaj

机构信息

Department of Radiology, Kathmandu Medical College And Teaching Hospital, Sinamangal, Kathmandu, Nepal.

Kathmandu Medical College And Teaching Hospital, Sinamangal, Kathmandu, Nepal.

出版信息

JNMA J Nepal Med Assoc. 2024 Mar 31;62(272):279-281. doi: 10.31729/jnma.8532.

DOI:10.31729/jnma.8532
PMID:39356850
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC11025487/
Abstract

Mayer-Rokitansky-Küster-Hauser syndrome (MRKH) also known as Müllerian agenesis, is caused by embryologic underdevelopment of the Mullerian duct, with resultant agenesis or atresia of the vagina, uterus, or both. Patients usually present with primary amenorrhea with normal growth and pubertal development. Here we present a case of a 29-year-old woman presented with primary amenorrhea. Secondary sexual characteristics and hormone evaluation were normal. Ultrasound and MRI were conducted and revealed complete absence of uterus, small vaginal canal. Bilateral renal fossa were empty and both the kidneys were located in the pelvic cavity fused to one-another with single renal pelvis giving pancake appearance.

摘要

迈耶-罗基坦斯基-库斯特-豪泽综合征（MRKH）也称为苗勒管发育不全，由苗勒管胚胎发育不全引起，导致阴道、子宫或两者发育不全或闭锁。患者通常表现为原发性闭经，但生长和青春期发育正常。在此，我们报告一例29岁原发性闭经女性病例。第二性征和激素评估均正常。进行了超声和磁共振成像检查，结果显示子宫完全缺如，阴道管短小。双侧肾窝空虚，双肾位于盆腔内，相互融合，形成单一肾盂，呈煎饼样外观。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/c578/11025487/53f647af88d6/JNMA-62-272-279-g5.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/c578/11025487/a7cc70d3e6a9/JNMA-62-272-279-g1.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/c578/11025487/8ad99d17fd6d/JNMA-62-272-279-g2.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/c578/11025487/3c3d6fcff7ec/JNMA-62-272-279-g3.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/c578/11025487/15768cdc9b8d/JNMA-62-272-279-g4.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/c578/11025487/53f647af88d6/JNMA-62-272-279-g5.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/c578/11025487/a7cc70d3e6a9/JNMA-62-272-279-g1.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/c578/11025487/8ad99d17fd6d/JNMA-62-272-279-g2.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/c578/11025487/3c3d6fcff7ec/JNMA-62-272-279-g3.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/c578/11025487/15768cdc9b8d/JNMA-62-272-279-g4.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/c578/11025487/53f647af88d6/JNMA-62-272-279-g5.jpg

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本文引用的文献

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Identification of Genetic Causes in Mayer-Rokitansky-Küster-Hauser (MRKH) Syndrome: A Systematic Review of the Literature.Mayer-Rokitansky-Küster-Hauser（MRKH）综合征遗传病因的鉴定：文献系统综述
Children (Basel). 2022 Jun 27;9(7):961. doi: 10.3390/children9070961.
2
Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome: a comprehensive update. Mayer-Rokitansky-Küster-Hauser（MRKH）综合征：全面更新。
Orphanet J Rare Dis. 2020 Aug 20;15(1):214. doi: 10.1186/s13023-020-01491-9.
3
Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome with unilateral pulmonary agenesis-a rarity indeed: radiologic review.
伴有单侧肺发育不全的迈耶-罗基坦斯基-库斯特-豪泽综合征（MRKH综合征）——实属罕见：影像学综述
BJR Case Rep. 2016 Feb 3;2(2):20150157. doi: 10.1259/bjrcr.20150157. eCollection 2016.
4
Spectrum of MRI Appearance of Mayer-Rokitansky-Kuster-Hauser (MRKH) Syndrome in Primary Amenorrhea Patients.原发性闭经患者中迈耶-罗基坦斯基-库斯特-豪泽综合征（MRKH）的MRI表现谱
J Clin Diagn Res. 2017 Jul;11(7):TC30-TC35. doi: 10.7860/JCDR/2017/29016.10317. Epub 2017 Jul 1.
5
Evaluation of Mayer-Rokitansky-Küster-Hauser syndrome with magnetic resonance imaging: Three patterns of uterine remnants and related anatomical features and clinical settings.磁共振成像评估梅耶尔-罗金坦斯基-库斯特-豪泽综合征：子宫残迹的三种模式及其相关解剖特征和临床背景。
Eur Radiol. 2017 Dec;27(12):5215-5224. doi: 10.1007/s00330-017-4919-4. Epub 2017 Jul 3.
6
Mayer-Rokitansky-Kuster-Hauser syndrome: a review.梅耶-罗基坦斯基-库斯特-豪泽综合征：综述
Int J Womens Health. 2015 Nov 2;7:865-70. doi: 10.2147/IJWH.S75637. eCollection 2015.
7
Mayer-Rokitansky-Kuster-Hauser syndrome diagnosed by magnetic resonance imaging. Role of imaging to identify and evaluate the uncommon variation in development of the female genital tract.通过磁共振成像诊断梅耶-罗基坦斯基-库斯特-豪泽综合征。影像学在识别和评估女性生殖道发育异常中的作用。
J Radiol Case Rep. 2012 Apr;6(4):17-24. doi: 10.3941/jrcr.v6i4.992. Epub 2012 Apr 1.
8
A population-based study of the incidence of Müllerian aplasia in Finland.芬兰基于人群的苗勒管发育不全发病率研究。
Fertil Steril. 2001 Sep;76(3):624-5. doi: 10.1016/s0015-0282(01)01963-x.