1,902 results match your criteria Mullerian Duct Anomalies

Pregnancy in a Unicornuate Uterus with Non-Communicating Rudimentary Horn: Diagnostic and Therapeutic Challenges.

Ratko Delić

Acta Med Litu 2020 21;27(2):84-89. Epub 2020 Dec 21.

Department of Obstetrics and Gynecology, General and Teaching Hospital Celje, Slovenia.

Unicornuate uterus with non-communicating rudimentary horn is a type of congenital uterine abnormality that occurs as a consequence of the arrested development of one of the two Müllerian ducts. Patients with unicornuate uterus have increased incidence of obstetric and gynaecological complications. We present a report of a clinical case of a 28-years-old female, who was referred to the hospital for evaluation of her infertility. Read More

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December 2020

Congenital Obstructive Müllerian Anomaly: The Pitfalls of a Magnetic Resonance Imaging-Based Diagnosis and the Importance of Intraoperative Biopsy.

J Clin Med 2021 May 29;10(11). Epub 2021 May 29.

Department of Obstetrics and Gynecology, Asan Medical Center, University of Ulsan College of Medicine, 88, Olympic-ro 43-gil, Songpa-gu, Seoul 05505, Korea.

A retrospective cohort study of the concordance between the magnetic resonance imaging (MRI) diagnosis and final diagnosis in patients with Müllerian duct anomalies (MDAs) was conducted, and diagnostic clues were suggested. A total of 463 cases of young women who underwent pelvic MRIs from January 1995 to February 2019 at Seoul Asan Medical Center were reviewed. Interventions consisted of clinical examinations, abdominal or transvaginal/rectal ultrasound, MRI, and operative procedures, including hysteroscopy and laparoscopy. Read More

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Three degrees of separation: complete uterine and cervical septa.

Fertil Steril 2021 May 17. Epub 2021 May 17.

Division of Reproductive Endocrinology and Infertility, Mayo Clinic Rochester, Rochester, Minnesota.

Objective: To review the diagnosis and management of 3 variations of incomplete müllerian duct fusion and reabsorption.

Design: Narrated video delineating the surgical management of 3 müllerian anomalies; this video was deemed exempt from review by the institutional review board of the Mayo Clinic.

Setting: Tertiary care academic medical center. Read More

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Robot-assisted laparoscopic hysterectomy for endometrial cancer in a patient with Herlyn-Werner-Wunderlich syndrome.

BMJ Case Rep 2021 May 19;14(5). Epub 2021 May 19.

Seirei Hamamatsu General Hospital, Hamamatsu, Japan.

Herlyn-Werner-Wunderlich syndrome, a rare Mullerian duct anomaly, includes a triad of uterine didelphys, obstructed haemivagina and ipsilateral renal agenesis. A 58-year-old woman with Herlyn-Werner-Wunderlich syndrome, reported of recurrent genital bleeding for 9 years, was finally diagnosed with endometrial cancer. She had a history of vaginal septum resection and nephrectomy of atrophic right kidney. Read More

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A retrospective analysis of female Müllerian duct anomalies in association with congenital renal abnormalities.

J Pediatr Adolesc Gynecol 2021 May 12. Epub 2021 May 12.

Department of Pediatric Urology, Wilhelmina Children's Hospital UMC Utrecht, Utrecht, The Netherlands; Department of Pediatric Urology and Andrology, Sechenov Moscow State Medical University, Moscow, Russia.

Study Objective: Müllerian (paramesonephric) duct anomalies (MDAs) are associated with several coexisting congenital abnormalities, including renal abnormalities. Although congenital renal abnormalities may remain asymptomatic, the consequences should not be underestimated. In both the literature and clinical practice, it remains necessary to improve awareness of the co-occurrence of different congenital renal abnormalities in women with MDAs. Read More

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Incidental Finding of Leiomyoma in Mayer-Rokitansky-Kuster-Hauser Syndrome.

J Investig Med High Impact Case Rep 2021 Jan-Dec;9:23247096211014690

Howard University Hospital, Washington, DC, USA.

Mayer-Rokitansky-Kuster-Hauser (MRKH) syndrome is a sexual developmental disorder. In this disorder, there is a congenital absence of the uterus and vagina with normal external genitalia. The etiology is not well understood. Read More

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[Robotic-assisted resection of a symptomatic Müllerian duct cyst].

Aktuelle Urol 2021 May 5. Epub 2021 May 5.

Helios Universitätsklinikum Wuppertal, Universität Witten/Herdecke, Klinik für Urologie und Kinderurologie, Wuppertal.

Cysts in the lesser pelvis are a rare disease and most often an incidental finding from routine diagnostic investigation. Published information is controversial. These cysts are distinguished by localisation, content of the cyst and accompanying anatomical anomalies. Read More

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Congenital Uterine Anomaly and Pelvic Organ Prolapse: A Rare Case of Pelvic Organ Prolapse in a Complete Bicornuate Uterus with Successful Pregnancy Outcomes Undiagnosed until the Time of Sacrocolpopexy.

Gina Nam Sa Ra Lee

J Menopausal Med 2021 Apr;27(1):32-36

Department of Obstetrics and Gynecology, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea.

Müllerian development anomalies (MDAs) are most commonly diagnosed in the reproductive period. A bicornuate uterus is the result of a fusion defect of the Müllerian ducts, causing an abnormal fundal outline. Most of the cases are diagnosed early in life and present with obstetrical complications, such as recurrent pregnancy loss, preterm birth, intrauterine growth restriction, placental abruption, and cervical incompetence. Read More

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The accuracy of three-dimensional ultrasonography in the diagnosis of Müllerian duct anomalies and its concordance with magnetic resonance imaging.

J Obstet Gynaecol 2021 May 3:1-7. Epub 2021 May 3.

Department of Obstetrics and Gynecology, Dokuz Eylül University Faculty of Medicine, Izmir, Turkey.

This retrospective study was performed to comparatively evaluate the diagnostic accuracies of three-dimensional ultrasonography (3D-US) and magnetic resonance imaging (MRI) for identification of Müllerian duct anomalies (MDAs). A total of 27 women with suspected MDAs underwent gynaecological examination, 2D-US, 3D-US and MRI, respectively. The MDAs were classified with respect to the European Society of Human Reproduction and Embryology-European Society for Gynaecological Endoscopy (ESHRE/ESGE) and American Society of Reproductive Medicine (ASRM) systems. Read More

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Mayer-Rokitansky-Küster-Hauser syndrome with 22q11.21 microduplication: a case report.

J Med Case Rep 2021 Apr 21;15(1):208. Epub 2021 Apr 21.

Department of Medical Genetics, 'G. D'Annunzio' University, Chieti, Italy.

Background: Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome (Online Mendelian Inheritance in Man [OMIM] #277000) is a congenital condition characterized by the total or partial agenesis of vagina and uterus. Agenesis can be isolated (MRKH 1) or associated with other renal, vertebral or cardiac defects (MRKH 2).

Case Presentation: In this paper, we report a case of a Caucasian patient showing the clinical signs associated with MRKH. Read More

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Vaginal bleeding in a misdiagnosed Mayer-Rokitansky-Küster-Hauser syndrome.

BMJ Case Rep 2021 Apr 5;14(4). Epub 2021 Apr 5.

Department of Pediatric Endocrine, Parc Tauli Foundation-UAB University Institute, Sabadell, Catalunya, Spain

The Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome, a spectrum of anomalies arising from incomplete development of the Müllerian ducts, is characterised by congenital aplasia of the uterus and upper part of the vagina, often in the absence of other phenotypical abnormalities. We report the case of a 13-year-old girl referred to our endocrinology unit after an incidental finding of uterine agenesis during laparoscopy to correct suspected ovarian torsion. Initial transabdominal ultrasonography found no uterus. Read More

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Mechanistic Drivers of Müllerian Duct Development and Differentiation Into the Oviduct.

Front Cell Dev Biol 2021 8;9:605301. Epub 2021 Mar 8.

MRC Weatherall Institute of Molecular Medicine, University of Oxford, Oxford, United Kingdom.

The conduits of life; the animal oviducts and human fallopian tubes are of paramount importance for reproduction in amniotes. They connect the ovary with the uterus and are essential for fertility. They provide the appropriate environment for gamete maintenance, fertilization and preimplantation embryonic development. Read More

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Herlyn-Werner-Wunderlich (HWW) syndrome with kyphoscoliosis: a rare urogenital anomaly in a teenage girl.

BMJ Case Rep 2021 Mar 22;14(3). Epub 2021 Mar 22.

Radio diagnosis, AIIMS Jodphur, Jodhpur, Rajasthan, India.

The obstructed haemivagina with ipsilateral renal agenesis (OHVIRA) syndrome also known as Herlyn-Werner-Wunderlich syndrome, a rare congenital malformation in females, results due to developmental abnormality in the müllerian duct. We are reporting a case of a 14-year-old girl who presented with pain and a lump in abdomen for the last 5 months. The MRI abdomen and pelvis confirmed the diagnosis of OHVIRA syndrome. Read More

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Myomas in uterine rudiments in a patient with Mayer-Rokitansky-Küster-Hauser syndrome.

Ceska Gynekol 2021 ;86(1):36-39

Objective: To describe the case of a patient with Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome and a history of chronic pelvic pain due to myomas in the rudimentary uterine horns. The article highlights a rare origin of gynaecological pain.

Case Report: We present the case of 61-year-old woman with MRKH syndrome who has suffered from chronic abdominal pain for more than one year before surgery. Read More

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Adolescent gynaecology: anaesthetic and peri-operative care implications.

Anaesthesia 2021 04;76 Suppl 4:39-45

University Hospitals Bristol and Weston NHS Foundation Trust, Bristol, UK.

Adolescents represent a quarter of the world's population, yet their specific healthcare needs have often not been acknowledged. Whilst many operations in this population will be performed in specialist tertiary centres and children's hospitals, it is likely that care will be sought in a variety of healthcare settings, and so it is important to have an understanding of the particular approach to this age group. Paediatric and adolescent gynaecology emerged as a speciality in 2000 with the inauguration of the British Society for Paediatric and Adolescent Gynaecology, a specialist society of the Royal College of Obstetricians and Gynaecologists. Read More

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Herlyn-Werner-Wunderlich syndrome: clinical considerations and management.

BMJ Case Rep 2021 Mar 4;14(3). Epub 2021 Mar 4.

School of Medicine, PUCPR, Curitiba, PR, Brazil.

Herlyn-Werner-Wunderlich syndrome (HWWS), defined by the triad of uterus didelphys, obstructed hemivagina and ipsilateral renal agenesis, is a rare Mullerian duct malformation, usually diagnosed after menarche, when symptoms related to haematocolpos arise. We report a case of a 14-year-old patient who presented to the emergency department complaining of proctalgia and pelvic pain treated in our medical centre. Ultrasound and abdomino-pelvic MRI imaging studies confirmed the diagnosis. Read More

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Complete Uterine Septum with Cervical Duplication and Longitudinal Vaginal Septum: An Anomaly Supporting Alternative Embryological Development.

J Hum Reprod Sci 2020 Oct-Dec;13(4):352-355. Epub 2020 Dec 28.

Department of Obstetrics and Gynaecology, Hamdard Institute of Medical Science and Research, Jamia Hamdard, New Delhi, India.

The diagnosis and management of Mullerian abnormalities have revolutionized with the advent of magnetic resonance imaging, three-dimensional ultrasound, and endoscopic techniques. All the earlier unclassified abnormalities can now be classified as per the new European Society of Human Reproduction and Embryology 2013 nomenclature. The presence of complete uterine septum with cervical duplication and longitudinal vaginal septum reinforces the alternative theory of Mullerian development. Read More

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December 2020

Female counterpart of the Zinner syndrome in men: decoding the 'OHVIRA' syndrome.

BMJ Case Rep 2021 Feb 10;14(2). Epub 2021 Feb 10.

Urology, All India Institute of Medical Sciences, Rishikesh, India

The obstructed hemivagina and ipsilateral renal agenesis (OHVIRA) syndrome, a rare Mullerian duct anomaly, is a triad of uterine anomalies with ipsilateral renal agenesis and obstructed hemivagina. The aetiopathogenesis of this developmental anomaly is debatable, with several theories being postulated to explain its occurrence. We report two cases of this rare syndrome which were detected on imaging done for unrelated reasons. Read More

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February 2021

An unprecedented occult non-communicating rudimentary uterine horn treated with laparoscopic excision and preservation of both fallopian tubes: a case report and review of the literature.

J Med Case Rep 2021 Feb 4;15(1):51. Epub 2021 Feb 4.

Department of Obstetrics and Gynecology, University Hospitals Schleswig Holstein, Campus Kiel, Kiel, Germany.

Background: Müllerian duct anomalies are congenital malformations of the female genital tract and may be of various types. For decades they have been classified according to the American Society of Reproductive Medicine, which mentions unicornuate uterine malformations as the second subgroup. They result from the arrested development of one of the Müllerian ducts and appear in approximately 1/1000 women. Read More

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February 2021

Herlyn-Werner-Wunderlich syndrome with borderline serous cystadenoma of the ovary: case report and literature review.

Radiol Case Rep 2021 Mar 22;16(3):744-747. Epub 2021 Jan 22.

Department of Medical Imaging, Prince Sultan Military Medical City, Riyadh, Saudi Arabia.

Herlyn-Werner-Wunderlich syndrome, is a rare urogenital congenital anomaly. Coexisting Mullerian ducts anomalies and ovarian neoplasms are rarely reported. We present the first case of Herlyn-Werner-Wunderlich syndrome with borderline serous neoplasm of the ovary. Read More

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Effects of different vaginal mould use approaches after vaginoplasty with artificial dermis in patients with Mayer-Rokitansky-Küster-Hauser syndrome.

J Int Med Res 2021 Feb;49(2):300060521990519

Department of Gynecology Minimally Invasive Center, Beijing Obstetrics and Gynecology Hospital, Capital Medical University, Beijing, China.

Objective: To assess the therapeutic effects of different vaginal mould use approaches after vaginoplasty in patients with Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome.

Methods: Patients with MRKH syndrome who underwent surgery from 2010 to 2015 in our hospital were retrospectively evaluated. Vaginoplasty was performed with artificial dermis, and vaginal moulds were used for 6 months postoperatively. Read More

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February 2021

Magnetic Resonance Imaging in the Evaluation of Female Infertility.

Curr Probl Diagn Radiol 2021 Jan 9. Epub 2021 Jan 9.

Northwestern Memorial Hospital, Chicago, IL.

Pelvic MRI plays an important adjunctive role in the clinical workup of female infertility. Hysterosalpingography is the first line imaging modality in evaluation of female infertility, and hysterosalpingo-contrast sonography can also be used to evaluate both the uterine cavity and fallopian tubes. Pelvic MRI can be helpful in the workup of female infertility, particularly in cases of Mullerian duct anomalies, fibroids, adenomyosis, endometriosis, and tubal disease. Read More

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January 2021

Genetics of agenesis/hypoplasia of the uterus and vagina: narrowing down the number of candidate genes for Mayer-Rokitansky-Küster-Hauser Syndrome.

Hum Genet 2021 Apr 19;140(4):667-680. Epub 2021 Jan 19.

Section of Reproductive Endocrinology, Infertility, and Genetics, Department of Obstetrics and Gynecology, Neuroscience Program, Medical College of Georgia, Augusta University, Augusta, GA, 30912, USA.

Purpose: Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome consists of congenital absence of the uterus and vagina and is often associated with renal, skeletal, cardiac, and auditory defects. The genetic basis is largely unknown except for rare variants in several genes. Many candidate genes have been suggested by mouse models and human studies. Read More

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Estrogen Receptor 1 (ESR1) Agonist Induces Ovarian Differentiation and Aberrant Müllerian Duct Development in the Chinese Soft-shelled Turtle, .

Zool Stud 2020 11;59:e54. Epub 2020 Nov 11.

Department of Biological Science and Technology, Tokyo University of Science, Tokyo 125-8585, Japan. E-mail: (Miyagawa); (Toyota); (Masuda); (Sugita); (Miyaoku); (Yamagishi); (Akashi).

Estrogens play critical roles in ovarian and reproductive organ development, but the molecular signaling pathways in non-mammalian vertebrates are not well understood. Studies of reptiles have indicated that administration of exogenous estrogens during embryonic development causes ovarian differentiation and presumptive male to female sex-reversal. The Chinese soft-shelled turtle, , belongs to the family Trionychidae and exhibits genotypic sex determination system with ZZ/ZW sex chromosomes. Read More

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November 2020

Clinical Characteristics, Ultrasonographic Findings, and Treatment of Pediatric Transverse Testicular Ectopia: A 10-Year Retrospective Review.

Urology 2021 Jan 14. Epub 2021 Jan 14.

Department of Urology and Laboratory of Pelvic Floor Muscle Function, Shenzhen Children's Hospital, Shenzhen, Guangdong, PR China. Electronic address:

Objective: To evaluate the clinical data, ultrasonic features, treatment, and long-term outcome of transverse testicular ectopia (TTE) in children.

Methods: Children with TTE were enrolled in the study between November 2009 and August 2019 in Shenzhen Children's Hospital in China. Clinical information, including demographic characteristics, clinical manifestations, TTE-associated anomalies, ultrasonographic findings, and surgical procedures, were collected from all patients. Read More

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January 2021

Perturbations of genes essential for Müllerian duct and Wölffian duct development in Mayer-Rokitansky-Küster-Hauser syndrome.

Am J Hum Genet 2021 02;108(2):337-345

Department of Molecular and Human Genetics, Baylor College of Medicine, Houston, TX 77030, USA.

Mayer-Rokitansky-Küster-Hauser syndrome (MRKHS) is associated with congenital absence of the uterus, cervix, and the upper part of the vagina; it is a sex-limited trait. Disrupted development of the Müllerian ducts (MD)/Wölffian ducts (WD) through multifactorial mechanisms has been proposed to underlie MRKHS. In this study, exome sequencing (ES) was performed on a Chinese discovery cohort (442 affected subjects and 941 female control subjects) and a replication MRKHS cohort (150 affected subjects of mixed ethnicity from North America, South America, and Europe). Read More

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February 2021

Twelve Live Births After Uterus Transplantation in the Dallas UtErus Transplant Study.

Obstet Gynecol 2021 02;137(2):241-249

Annette C. and Harold C. Simmons Transplant Institute, the Fertility Center of Dallas, the Department of Obstetrics and Gynecology, and the Department of Neonatology, Baylor University Medical Center, and the Fetal Care Center, Dallas, Texas; and the Department of Obstetrics and Gynecology, PRISMA Health-University of South Carolina School of Medicine, Columbia, South Carolina.

Objective: To describe aggregated pregnancy outcomes after uterus transplantation from a single, experienced center.

Methods: This prospective study reports on live births among 20 women who received a uterus transplant from 2016 to 2019 at Baylor University Medical Center at Dallas. These live births occurred between November 2017 and September 2020. Read More

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February 2021

The development of the human uterus: morphogenesis to menarche.

Hum Reprod Update 2021 Jan;27(1):1-26

Department of Maternal and Child Health, Gynaecology and Urology, Sapienza University of Rome, Rome, Italy.

There is emerging evidence that early uterine development in humans is an important determinant of conditions such as ontogenetic progesterone resistance, menstrual preconditioning, defective deep placentation and pre-eclampsia in young adolescents. A key observation is the relative infrequency of neonatal uterine bleeding and hormone withdrawal at birth. The origin of the uterus from the fusion of the two paramesonephric, or Müllerian, ducts was described almost 200 years ago. Read More

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January 2021

Unraveling the Mysteries of PAX8 in Reproductive Tract Cancers.

Cancer Res 2021 02 23;81(4):806-810. Epub 2020 Dec 23.

Department of Obstetrics and Gynecology, Penn Ovarian Cancer Research Center, University of Pennsylvania, Philadelphia, Pennsylvania.

Paired Box 8 (PAX8) is a lineage-specific transcription factor that has essential roles during embryogenesis and tumorigenesis. The importance of PAX8 in the development of the reproductive system is highlighted by abnormalities observed upon the loss or mutation of this PAX family member. In cancer, PAX8 expression is deregulated in a key set of neoplasms, including those arising from the Müllerian ducts. Read More

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February 2021

Laparoscopic Approach Towards Non-Communicating Functional Rudimentary Uterine Horn: A Report of Two Cases.

Cureus 2020 Nov 10;12(11):e11419. Epub 2020 Nov 10.

Radiology, All India Institute of Medical Sciences, Bhubaneswar, IND.

A unicornuate uterus is a relatively rare Müllerian anomaly with an incidence of 2.5-13%. It may lead to various gynecological or obstetric complications, and diagnosis can often be confusing and delayed. Read More

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November 2020