Literature DB >> 28893003

Spectrum of MRI Appearance of Mayer-Rokitansky-Kuster-Hauser (MRKH) Syndrome in Primary Amenorrhea Patients.

Deb Kumar Boruah1, Shantiranjan Sanyal2, Bidyut Bikash Gogoi3, Kangkana Mahanta4, Arjun Prakash5, Antony Augustine4, Sashidhar Achar4, Hiranya Baishya4.   

Abstract

INTRODUCTION: Mayer-Rokitansky-Kuster-Hauser (MRKH) synd-rome is a malformation in female genital tract due to interrupted embryonic development of para-mesonephric ducts leading to uterine and proximal vagina aplasia or hypoplasia. AIM: To analyse the MRI findings in females suspected of MRKH syndrome in a primary amenorrhea workup.
MATERIALS AND METHODS: A cross-sectional study comprising of 11 patients of MRKH syndrome presented in a tertiary care centre from March 2016 to February 2017 evaluated in 1.5 Tesla MRI scanner.
RESULTS: Normal positioned small vestigial uterus demonstrated in two patients (18.2%) and complete uterine and proximal vaginal agenesis were noted in nine patients (81.8%). The mean volume of rudimentary right uterine bud was 2.26 mL ±3.3 (SD) and left uterine bud was 1.27 mL±1.1 (SD). The mean volume of right ovary was 4.74 mL±2.58 (SD) and left ovary was 4.65 mL±2.2 (SD). The mean developed vaginal length was 26.2 mm±6.34 (SD).
CONCLUSION: The rudimentary uterine buds associated with MRKH syndrome always maintained caudal relationship with ovary. Ovaries or rudimentary uterine buds can be ectopic and should be recognized with MRI before undergoing fertility treatment. Following MRI diagnosis, surgery allows patients to have sexual function with possible attainment of reproduction after assisted reproduction technique or surrogacy.

Entities:  

Keywords:  Infertility; Mullerian anomalies; Rudimentary uterine buds; Utero-vaginal agenesis

Year:  2017        PMID: 28893003      PMCID: PMC5583891          DOI: 10.7860/JCDR/2017/29016.10317

Source DB:  PubMed          Journal:  J Clin Diagn Res        ISSN: 0973-709X


  29 in total

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Journal:  BJOG       Date:  2007-12       Impact factor: 6.531

3.  Mayer-Rokitansky-Kuster-Hauser syndrome diagnosed by magnetic resonance imaging in a 15-year-old girl.

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Journal:  J Pediatr Adolesc Gynecol       Date:  1997-05       Impact factor: 1.814

4.  Diagnosis of a variant of Mayer-Rokitansky-Kuster-Hauser syndrome: useful MRI findings.

Authors:  Sabina Giusti; Elena Fruzzetti; Daria Perini; Franca Fruzzetti; Patrizia Giusti; Carlo Bartolozzi
Journal:  Abdom Imaging       Date:  2011-12

5.  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.

Authors:  Valeria Fiaschetti; Amedeo Taglieri; Vito Gisone; Irene Coco; Giovanni Simonetti
Journal:  J Radiol Case Rep       Date:  2012-04-01

Review 6.  MR imaging diagnosis of uterovaginal anomalies: current state of the art.

Authors:  Sahar N Saleem
Journal:  Radiographics       Date:  2003-06-20       Impact factor: 5.333

7.  Mayer-Rokitansky-Küster-Hauser syndrome and ovarian cancer. Report of a case.

Authors:  G Ghirardini; A Magnani
Journal:  Clin Exp Obstet Gynecol       Date:  1995       Impact factor: 0.146

8.  Vaginal agenesis: classification by MR imaging.

Authors:  K Togashi; K Nishimura; K Itoh; I Fujisawa; Y Nakano; K Torizuka; H Ozasa; M Ohshima
Journal:  Radiology       Date:  1987-03       Impact factor: 11.105

9.  Magnetic resonance imaging in the preoperative assessment of Mayer-Rokitansky-Kuster-Hauser syndrome.

Authors:  G Pompili; A Munari; G Franceschelli; N Flor; R Meroni; G Frontino; L Fedele; G Cornalba
Journal:  Radiol Med       Date:  2009-05-29       Impact factor: 3.469

Review 10.  The Mayer-Rokitansky-Küster-Hauser syndrome (congenital absence of uterus and vagina)--phenotypic manifestations and genetic approaches.

Authors:  Daniel Guerrier; Thomas Mouchel; Laurent Pasquier; Isabelle Pellerin
Journal:  J Negat Results Biomed       Date:  2006-01-27
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  8 in total

1.  Development and characterization of human fetal female reproductive tract organoids to understand Müllerian duct anomalies.

Authors:  Varshini D Venkata; M Fairuz B Jamaluddin; Jyoti Goad; Hannah R Drury; Melissa A Tadros; Rebecca Lim; Ajay Karakoti; Rachel O'Sullivan; Yvette Ius; Kenneth Jaaback; Pravin Nahar; Pradeep S Tanwar
Journal:  Proc Natl Acad Sci U S A       Date:  2022-07-18       Impact factor: 12.779

2.  MRI evaluation of pelvis in Mayer-Rokitansky-Kuster-Hauser syndrome: interobserver agreement for surgically relevant structures.

Authors:  Aanchal Bhayana; Rohini Gupta Ghasi
Journal:  Br J Radiol       Date:  2019-03-15       Impact factor: 3.039

3.  A rare form of Mayer-Rokitansky-Küster-Hauser syndrome: Case report and review of literature.

Authors:  Omran Al Dandan; Ali Hassan; Amna Alsaihati; Lujain Aljawad; Fathiya Almejhim
Journal:  Case Rep Womens Health       Date:  2019-08-06

4.  Evaluation and Management of Unexpected Functional Rudimentary Uteri in Mayer-Rokitansky-Küster-Hauser Syndrome of Chinese Women.

Authors:  Shan Deng; Lan Zhu; Qinjie Tian
Journal:  Biomed Res Int       Date:  2020-11-24       Impact factor: 3.411

5.  A 19-year-old Female with Primary Amenorrhea: Mayer-Rokitansky-Küster-Hauser Syndrome.

Authors:  Tanvi Khanna; Sonal Saran
Journal:  J Med Ultrasound       Date:  2021-04-23

Review 6.  Age-dependent phenotypes of ovarian endometriomas.

Authors:  Giuseppe Benagiano; Sun-Wei Guo
Journal:  Reprod Med Biol       Date:  2022-01-21

7.  Caudal regression syndrome: Postnatal radiological diagnosis with literature review of 83 cases.

Authors:  Hana' Qudsieh; Emad Aborajooh; Abdallah Daradkeh
Journal:  Radiol Case Rep       Date:  2022-09-29

Review 8.  Diagnosis and Management of Ovarian Tumor in Mayer-Rokitansky-Küster-Hauser (MRKH) Syndrome.

Authors:  Yali Miao; Jirui Wen; Liwei Huang; Jiang Wu; Zhiwei Zhao
Journal:  Biomed Res Int       Date:  2018-03-12       Impact factor: 3.411

  8 in total

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