1,153 results match your criteria Uterus Mullerian Duct Abnormalities


Obstructed Hemivagina and Ipsilateral Renal Agenesis Syndrome: A Case Report.

JNMA J Nepal Med Assoc 2022 Jun 1;60(250):562-564. Epub 2022 Jun 1.

Department of Obstetrics and Gynaecology, Bharatpur Hospital, Chitwan, Nepal.

Obstructed hemivagina and ipsilateral renal anomaly syndrome also known as Herlyn-Werner-Wunderlich syndrome is a rare congenital urogenital anomaly characterised by Mullerian duct anomalies associated with mesonephric duct anomalies. A 10-year old female presented with acute lower abdominal pain, urinary retention and scanty menstrual flow during her first menstruation. Ultrasonography and contrast computed tomography showed uterine didelphys, hematocolpos, obstructed hemivagina and left renal agenesis. Read More

View Article and Full-Text PDF

Robert's uterus (asymmetric septate uterus): a rare congenital Müllerian duct anomaly.

BMJ Case Rep 2022 May 6;15(5). Epub 2022 May 6.

Department of Fetal Medicine, Liverpool Women's NHS Foundation Trust, Liverpool, UK.

Müllerian anomalies such as Robert's uterus, which was first described by the French gynaecologist Dr Helene Robert in 1969, are rare clinical entities and have been reported in <3% of the female population. Robert's uterus is a rare phenomenon with a relative dearth of reported cases. Affected individuals may present with pelvic pain and dysmenorrhoea that intensifies near menses or acutely, with severe abdominal pain to the emergency department. Read More

View Article and Full-Text PDF

An Association between EMX2 Variations and Mayer-Rokitansky-Küster-Hauser Syndrome: A Case-Control Study of Chinese Women.

J Healthc Eng 2022 13;2022:9975369. Epub 2022 Apr 13.

Department of Obstetrics and Gynecology, The First Affiliated Hospital of Jinan University, Guangzhou, Guangdong 511442, China.

Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome is characterized by congenital malformations of Müllerian structures, including the uterus and upper two-thirds of the vagina in women. Until now, the etiology of this disease has remained unknown. We hypothesized that EMX2 (the human homologue of empty spiracles gene (2) might be a candidate gene for MRKH syndrome because it plays an important role in the development of the urogenital system. Read More

View Article and Full-Text PDF

Endometrial organoids derived from Mayer-Rokitansky-Küster-Hauser syndrome patients provide insights into disease-causing pathways.

Dis Model Mech 2022 05 10;15(5). Epub 2022 May 10.

Research Institute for Women's Health, University of Tübingen, 72076 Tübingen, Germany.

The uterus is responsible for the nourishment and mechanical protection of the developing embryo and fetus and is an essential part in mammalian reproduction. Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome is characterized by agenesis of the uterus and upper part of the vagina in females with normal ovarian function. Although heavily studied, the cause of the disease is still enigmatic. Read More

View Article and Full-Text PDF

A "cluster" of ten uterine anomalies observed in a single center over a short period of 4 weeks: a case series.

J Med Case Rep 2022 Apr 2;16(1):130. Epub 2022 Apr 2.

Department of Obstetrics and Gynaecology, Karnataka Institute of Medical Sciences (KIMS), Hubli, Karnataka, India.

Background: Uterine anomalies occur because of Müllerian duct maldevelopment. Few of them are associated with adverse obstetric outcome (Reyes-Muñoz et al. in Diagnostics. Read More

View Article and Full-Text PDF

Diagnosis of Congenital Uterine Abnormalities: Practical Considerations.

J Clin Med 2022 Feb 25;11(5). Epub 2022 Feb 25.

St. George's Hospital, London SW17 0QT, UK.

As most congenital uterine abnormalities are asymptomatic, the majority of them are detected incidentally. While most women with uterine anomalies have a normal reproductive outcome, some may experience adverse reproductive outcomes. Accurate diagnosis and correct classification help in the appropriate counselling of women about their potential reproductive prognosis and risks and for planning any intervention. Read More

View Article and Full-Text PDF
February 2022

The Herlyn-Werner-Wunderlich (HWW) syndrome - A case report with radiological review.

Radiol Case Rep 2022 May 2;17(5):1435-1439. Epub 2022 Mar 2.

Department of Radiology, Sir Ganga Ram Hospital, Lahore, Pakistan.

Herlyn-Werner-Wunderlich (HWW) syndrome is a rare congenital anomaly of female urogenital tract involving combined mullerian duct anomalies and mesonephric duct malformation characterized by uterus didelphys, obstructed hemi-vagina and ipsilateral renal agenesis which is also known as OHVIRA syndrome. It can be classified based on a completely or incompletely obstructed hemivagina. It presents soon after menarche or shows delayed presentation depending on the type. Read More

View Article and Full-Text PDF

[Mayer-Rokitansky-Küster-Hauser syndrome: a cause of primary amenorrhea: about a case].

Pan Afr Med J 2021 23;40:260. Epub 2021 Dec 23.

Service de Radiologie, Hôpital d'Instruction Militaire Rabat, Rabat, Maroc.

Mayer-Rokitansky -Küster -Hauser syndrome (MRKH) is a rare cause of primary amenorrhea. It is defined as congenital aplasia of the uterus and of the upper two thirds of the vagina in women with normal development of secondary sexual characteristics. Diagnosis is essentially based on magnetic resonance imaging (MRI). Read More

View Article and Full-Text PDF

Duplex Collecting System with Ectopic Ureters Opening into Vagina: A Case Report.

JNMA J Nepal Med Assoc 2022 Feb 15;60(246):204-206. Epub 2022 Feb 15.

Department of Surgery, Kanti Children's Hospital, Maharajgunj, Kathmandu, Nepal.

Continuous urinary leakage, along with normal deliberate voiding, must suggest diagnosis of ectopic ureter, specifically in girls. Combination of a duplicated collecting system with distal, infra-sphincteric, vaginal insertion of ureter is an uncommon congenital anomaly and rare cause of urinary incontinence. We present a case report of a 7-year-old girl who presented to the urology department with urinary incontinence despite successful toilet training and history of recurrent urinary tract infections. Read More

View Article and Full-Text PDF
February 2022

Herlyn-Werner-Wunderlich syndromne: case review and report of the literature

Rev Colomb Obstet Ginecol 2021 12 30;72(4):407-422. Epub 2021 Dec 30.

Docente de la Universidad Militar Nueva Granada, Bogotá (Colombia)..

Objective: To report a case of Herlyn-Werner-Wunderlich syndrome (HWWS) and conduct a review of the literature to determine clinical and imaging findings in these patients.

Material And Methods: A 16-year-old female patient who presented to a military hospital in Bogotá complaining of recurrent pelvic pain, receiving a final diagnosis of HWWS. A systematic search was conducted in the different databases of systematic reviews, cohort studies, case series and case reports of patients of any age diagnosed with HWWS. Read More

View Article and Full-Text PDF
December 2021

Adenomyosis in a uterine horn of a patient with Mayer-Rokitansky-Kuster-Hauser syndrome.

BMJ Case Rep 2022 Feb 7;15(2). Epub 2022 Feb 7.

Obstetrics and Gynaecology, Centro Hospitalar do Baixo Vouga, EPE, Aveiro, Portugal.

A 37-year-old woman with a previous diagnosis of Mayer-Rokitansky-Kuster-Hauser syndrome at 18 years of age was referred from a primary healthcare physician to a gynaecology appointment in our centre. She presented with a 2-year worsening pelvic pain and dyspareunia, symptoms that were previously absent and, at the time, with inadequate relief with oral analgesia. Physical examination showed absent uterine cervix and hypoplastic superior vagina. Read More

View Article and Full-Text PDF
February 2022

Accessory cavitated uterine mass: a new müllerian anomaly?

Authors:
Beth W Rackow

Fertil Steril 2022 03 31;117(3):649-650. Epub 2022 Jan 31.

Columbia University Irving Medical Center, Department of Obstetrics & Gynecology, Columbia University Fertility Center, New York, New York.

View Article and Full-Text PDF

Mayer-Rokitansky-Küster-Hauser Syndrome: MR Manifestations Of Typical And Atypical Cases.

J Ayub Med Coll Abbottabad 2021 Oct-Dec;33(Suppl 1)(4):S711-S716

Holy Family Hospital, Rawalpindi, Pakistan.

Background: The Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome is a group of congenital malformations arising due to agenesis or arrest of the growth of paired Mullerian ducts leading to absence of uterus, fallopian tubes and upper part of vagina. MRI has an unmatched role in diagnosing MRKH syndrome. The aim of the present study is to elaborate MRI manifestations of typical and atypical MRKH syndrome and its common and rare associations. Read More

View Article and Full-Text PDF
January 2022

Donor robotic-assisted laparoscopy for uterus transplantation.

Fertil Steril 2022 03 17;117(3):651-652. Epub 2022 Jan 17.

Institute Clinic of Gynecology, Obstetrics and Neonatology, Hospital Clinic de Barcelona, Institut dÍnvestigacions Biomèdiques August Pi i Sunyer (IDIBAPS), Universitat de Barcelona, Barcelona, Spain.

Objective: Uterine transplantation has proven to be a viable solution in cases of absolute uterine factor infertility. Performing uterine explant surgery is one of the most difficult gynecologic surgical challenges owing to the complexity of the uterine vascular system. The goal of this video is to demonstrate uterine explant surgery and highlight the critical anatomy involved in this procedure. Read More

View Article and Full-Text PDF

A rare case of the new entity of müllerian anomalies mimicking the noncommunicating rudimentary cavity with hemi-uterus: accessory cavitated uterine mass.

Fertil Steril 2022 03 17;117(3):646-648. Epub 2022 Jan 17.

Department of Obstetrics and Gynecology, Eskisehir Osmangazi University Faculty of Medicine, Eskisehir, Turkey.

Objective: To demonstrate the laparoscopic excision of an accessory cavitated uterine mass (ACUM) and show that atypical anomalies can lead to the misdiagnosis of a noncommunicating rudimentary horn.

Design: Description and step-by-step demonstration of the surgical procedure using a video recording (Canadian Task Force Classification 3).

Setting: University hospital. Read More

View Article and Full-Text PDF

Uterine Transplantation: Recipient Patient Populations.

Clin Obstet Gynecol 2022 03;65(1):15-23

Division of Reproductive Endocrinology and Infertility, Department of Obstetrics and Gynecology, University Hospitals Cleveland Medical Center, Beachwood.

Uterine transplantation is an emerging treatment for patients with uterine factor infertility (UFI). In order to determine patient candidacy for transplant, it is imperative to understand how to identify, counsel and treat uterine transplant recipients. In this article, we focus on patient populations with UFI, whether congenital or acquired, including Mayer-Rokitansky-Kuster-Hauser, complete androgen insensitivity syndrome, hysterectomy, and other causes of nonabsolute UFI. Read More

View Article and Full-Text PDF

A genotype-first analysis in a cohort of Mullerian anomaly.

J Hum Genet 2022 Jun 13;67(6):347-352. Epub 2022 Jan 13.

Department of Obstetrics and Gynaecology, National Clinical Research Center for Obstetric & Gynecologic Diseases, State Key Laboratory of Complex Severe and Rare Diseases, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, Beijing, 100730, China.

Müllerian anomaly (M.A.) is a group of congenital anatomic abnormalities caused by aberrations of the development process of the Müllerian duct. Read More

View Article and Full-Text PDF

A rare variant of obstructed hemivagina and ipsilateral renal agenesis and its improvement of classification.

J Obstet Gynaecol Res 2022 Mar 10;48(3):869-874. Epub 2022 Jan 10.

Maternal and Child Health Hospital of Hubei Province, Wuhan, China.

Obstructed hemivagina and ipsilateral renal agenesis (OHVIRA) is a rare congenital malformation of the female reproductive organs, including uterine dysplasia, blind hemivagina, and ipsilateral renal dysplasia. It has a serious impact on patients' quality of life and fertility. We report a rare case of OHVIRA with a complete septate uterus, ectopic dysplasia of the left kidney, left ectopic ureter, and three oblique vaginal septa. Read More

View Article and Full-Text PDF

One patient with two uteri and two pregnancies - a rare case of twins in a patient with uterus didelphys.

Ginekol Pol 2021;92(12):905-906

1st Department of Obstetrics and Gynecology, Medical University of Warsaw, Poland.

We report an extraordinary case of double pregnancy in patient with uterus didelphys. This anatomic anomaly originates from the lack of fusion of the paired Mullerian ducts during embryological development with 0.3% prevalence in the population. Read More

View Article and Full-Text PDF

Giant hysteromyoma after vaginoplasty in a woman with Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome: case report and review of the literature.

J Int Med Res 2021 Dec;49(12):3000605211066394

Cheeloo College of Medicine, Shandong University, Jinan, China.

Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome is a congenital disorder characterized by congenital absence of both the uterus and vagina. Some patients require surgery to create a neovagina, however, the preservation of a nonfunctional rudimentary uterus after surgery may lead to long-term complications. Herein, a rare case of a giant hysteromyoma after vaginoplasty, in a 31-year-old Chinese female patient who was diagnosed with MRKH syndrome, is reported. Read More

View Article and Full-Text PDF
December 2021

[Laparoscopic management of persistent Müllerian duct syndrome: A case report and pedigree investigation].

Zhonghua Nan Ke Xue 2021 Mar;27(3):231-235

Department of Pediatric Surgery.

Objective: To investigate the clinical characteristics, diagnosis, treatment and etiology of persistent Müllerian duct syndrome (PMDS).

Methods: A 3-year-old boy was diagnosed with PMDS according to the clinical manifestations and the results of ultrasonography, laboratory examinations and earlier surgical examination. We performed genetic tests for the patient and his family members, removed the infantile uterus by laparoscopic wedge hysterectomy, biopsied and descended the bilateral testes, and ligated the bilateral internal rings, followed by a retrospective analysis and review of relevant literature. Read More

View Article and Full-Text PDF

Agenesis of female internal reproductive organs, the Mayer- Rokitansky-Küster-Hauser syndrome.

Bratisl Lek Listy 2021 ;122(12):839-845

Objectives: Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome is the most common cause of uterine and uterine tubes absence/underdevelopment and the second most common cause of primary amenorrhea. It is characterized by a congenital agenesis of the uterine tubes, uterus, cervix, and upper part of the vagina. This study presents our 10 years of experience with the diagnostics and therapeutic management of patients with MRKH syndrome. Read More

View Article and Full-Text PDF
December 2021

A Rare Association of Obstructed Hemivagina with Ipsilateral Renal Agenesis with Congenital Pouch Colon.

J Pediatr Adolesc Gynecol 2022 Jun 27;35(3):387-390. Epub 2021 Nov 27.

Department of Radiodiagnosis and Imaging, Post Graduate Institute of Medical Education and Research, Chandigarh, India.

Background: Obstructed hemivagina and ipsilateral renal agenesis (OHVIRA), or Herlyn-Werner-Wunderlich syndrome, is a rare Mullerian duct anomaly. Several associations of OHVIRA with other anomalies are being reported.

Case: A 13-year-old girl reported with acute onset abdominal pain. Read More

View Article and Full-Text PDF

Surgical and Pre-Operative Considerations for Managing Pelvic Organ Prolapse in a Patient with a Müllerian Duct Anomaly.

Int Urogynecol J 2022 Jan 26;33(1):159-161. Epub 2021 Nov 26.

Division of Urogynecology, Department of Obstetrics and Gynecology, Sunnybrook Health Sciences Centre, B732-2075 Bayview Avenue, Toronto, Ontario, M4N 3M5, Canada.

View Article and Full-Text PDF
January 2022

Surgical management and molecular diagnosis of persistent Müllerian duct syndrome in Chinese patients.

Asian J Androl 2022 Jan-Feb;24(1):78-84

Department of Urology, Children's Hospital, Zhejiang University School of Medicine, National Clinical Research Center for Child Health, Hangzhou 310052, China.

Persistent Müllerian duct syndrome (PMDS) is a rare clinically and genetically overlapping disorder caused by mutations in the anti-Müllerian hormone (AMH) gene or the anti-Müllerian hormone receptor type 2 (AMHR2) gene. Affected individuals present uterus and tubes in normally virilized males and are discovered unexpectedly during other surgeries. Since it is rare and complex, a definitive clinical diagnosis can be missed, and there are no guidelines regarding how to deal with the uterus. Read More

View Article and Full-Text PDF
January 2022

Case of successfully delivered dicavitary twins in uterus didelphus.

BMJ Case Rep 2021 Nov 15;14(11). Epub 2021 Nov 15.

Obstetrics and Gynecology, Nottingham University Hospitals NHS Trust, Nottingham, UK.

Uterus didelphus is a congenital abnormality arising from failure of fusion of Mullerian ducts, creating two separate uterine horns, two cervices and, in some cases, a vagina divided by a longitudinal septum. In this case, a 26-year-old woman with previously undiagnosed uterus didelphus spontaneously conceived dicavitary twins. Although initially wanting a vaginal birth, when both twins were in a breech presentation, a caesarean section was performed at 36 weeks, delivering two healthy babies. Read More

View Article and Full-Text PDF
November 2021

Primary Amenorrhea Due to Anatomical Abnormalities of the Reproductive Tract: Molecular Insight.

Int J Mol Sci 2021 Oct 25;22(21). Epub 2021 Oct 25.

Division of Gynecology, Department of Perinatology and Gynecology, Poznan University of Medical Sciences, 60-535 Poznan, Poland.

Congenital anomalies of the female reproductive tract that present with primary amenorrhea involve Müllerian aplasia, also known as Mayer-Rokitansky-Küster-Hauser syndrome (MRKHS), and cervical and vaginal anomalies that completely obstruct the reproductive tract. Karyotype abnormalities do not exclude the diagnosis of MRKHS. Familial cases of Müllerian anomalies and associated malformations of the urinary and skeletal systems strongly suggest a complex genetic etiology, but so far, the molecular mechanism in the vast majority of cases remains unknown. Read More

View Article and Full-Text PDF
October 2021

ASRM müllerian anomalies classification 2021.

Fertil Steril 2021 11;116(5):1238-1252

There are many proposed classification systems for müllerian anomalies. The American Fertility Society (AFS) Classification from 1988 has been the most recognized and utilized. The advantages of this iconic classification include its simplicity, recognizability, and correlation with clinical pregnancy outcomes. Read More

View Article and Full-Text PDF
November 2021

Ischial Spinous Fascia Fixation for Dilated Neovaginal Prolapse in Mayer-Rokitansky-Küster-Hauser Syndrome-2 Case Reports.

J Pediatr Adolesc Gynecol 2022 Jun 4;35(3):396-399. Epub 2021 Nov 4.

Department of Obstetrics and Gynecology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences, National Clinical Research Center for Obstetrical and Gynecological Diseases, State Key Laboratory of Complex Severe and Rare Diseases (Peking Union Medical College Hospital), Beijing, China. Electronic address:

Background: Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome, as congenital anomaly of the female reproductive tract, is characterized by the congenital absence of uterus and vagina. Dilation maneuver was recommended as the nonsurgical treatment for these patients. However, only several cases were reported depicting the prolapse of the dilated neovagina of patients with MRKH syndrome. Read More

View Article and Full-Text PDF

Clinical Features and Management of Endometriosis among Patients with MRKH and Functional Uterine Remnants.

Gynecol Obstet Invest 2021 4;86(6):518-524. Epub 2021 Nov 4.

Department of Obstetrics and Gynaecology, State Key Laboratory of Complex Severe and Rare Diseases, Peking Union Medical College Hospital, Peking Union Medical College and Chinese Academy of Medical Sciences, National Clinical Research Center for Obstetrical and Gynecological Diseases, Beijing, China.

Objective: This study aimed to determine the clinical features of Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome patients with functional uterine remnants and endometriosis in a large cohort of Chinese patients.

Design: This was a retrospective study.

Participants: This study had a cohort of 511 MRKH patients. Read More

View Article and Full-Text PDF
January 2022