1,033 results match your criteria Uterus Mullerian Duct Abnormalities


Genital abnormalities associated to lack of uterine adenogenesis or endometrial gland dysgenesis of female dromedary camels ().

Open Vet J 2020 Apr 29;10(1):44-52. Epub 2020 Feb 29.

Faculty of Veterinary Medicine, Department of Fundamental and Applied Research for Animal and Health. University of Liège Abstract, 4000 Liège, Belgium.

Background: The developmental disruption of the müllerian duct and the endometrial dynamic can generate genital lesions that could contribute to infertility.

Aim: This paper discusses two cases of genital conditions associated to endometrial gland pathologies in nulliparous female camels.

Methods: Macroscopic examinations and histopathological description were performed on congenital and acquired genital abnormalities with endometrial gland anomalies. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.4314/ovj.v10i1.8DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7193880PMC

GREB1L variants in familial and sporadic hereditary urogenital adysplasia and Mayer-Rokitansky-Kuster-Hauser syndrome.

Clin Genet 2020 May 7. Epub 2020 May 7.

Center for Human Genetics, Centre Hospitalier Universitaire, Liège, Belgium.

Congenital uterine anomalies (CUA) may have major impacts on the health and social well-being of affected individuals. Their expressivity is variable, with the most severe end of the spectrum being the absence of any fully or unilaterally developed uterus (aplastic uterus), which is a major feature in Mayer-Rokitansky-Kuster-Hauser syndrome (MRKH). So far, etiologies of CUA remain largely unknown. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1111/cge.13769DOI Listing

Aberrant DNA methylation in the PAX2 promoter is associated with Müllerian duct anomalies.

Arch Gynecol Obstet 2020 06 18;301(6):1455-1461. Epub 2020 Apr 18.

Department of Obstetrics and Gynecology, Reproductive Medicine Center, The First Affiliated Hospital of Anhui Medical University, No. 218 Jixi Road, Hefei, 230022, Anhui, China.

Purpose: Abnormalities during Müllerian duct and female reproductive tract formation during embryonic development result in Müllerian duct anomalies (MDA). Previous studies have identified a role for mutations in related genes and DNA copy number variation (CNV). However, the correlation between gene methylation and MDA remains to be understood. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1007/s00404-020-05539-wDOI Listing

A rare case report of bilateral recurrent inguinal hernia due to persistent Müllerian duct syndrome treated by transabdominal preperitoneal repair.

Medicine (Baltimore) 2020 Feb;99(7):e19079

Department of Digestive Surgery, Breast and Thyroid Surgery Graduate School of Metical Sciences Kagoshima University, Kagoshima, Japan.

Introduction: Persistent Müllerian duct syndrome (PMDS) is a rare disease occurring in men with an otherwise completely normal phenotype, in which female internal sex organs are present, including a uterus, fallopian tubes, cervix, and vagina. We report a case of bilateral recurrent inguinal hernia due to PMDS treated by transabdominal preperitoneal repair (TAPP).

Patient Concerns: A 72-year-old male presented with a complaint of swelling on both sides of the groin. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1097/MD.0000000000019079DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7035117PMC
February 2020

Imaging of Infertility, Part 2: Hysterosalpingograms to Magnetic Resonance Imaging.

Radiol Clin North Am 2020 Mar 31;58(2):227-238. Epub 2019 Dec 31.

Department of Radiology, Abdominal Imaging Section, The George Washington University Hospital, 900 23rd Street, Northwest, Washington, DC 20037, USA. Electronic address:

Infertility, or subfertility, is the inability to achieve a clinical pregnancy after a 1-year period of regular unprotected sexual intercourse in women younger than 35 and after 6 months in women older than 35. Although initial assessment involves a multitude of factors, including a detailed medical history, physical examination, semen analysis, and hormonal evaluation, diagnostic imaging of the female partner often plays an important role in establishing the etiology for infertility. This article provides an overview of the multimodality imaging assessment of female infertility and details the developmental and acquired pelvic abnormalities in which diagnostic imaging aids in evaluation. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1016/j.rcl.2019.10.011DOI Listing

Imaging of Infertility, Part 1: Hysterosalpingograms to Magnetic Resonance Imaging.

Radiol Clin North Am 2020 Mar 16;58(2):215-225. Epub 2019 Dec 16.

Department of Radiology, Abdominal Imaging Section, The George Washington University Hospital, 900 23rd Street, Northwest, Washington, DC 20037, USA. Electronic address:

Infertility, or subfertility, is the inability to achieve a clinical pregnancy after a 1-year period of regular unprotected sexual intercourse in women younger than 35 and after 6 months in women older than 35. Although initial assessment involves a multitude of factors, including a detailed medical history, physical examination, semen analysis, and hormonal evaluation, diagnostic imaging of the female partner often plays an important role in establishing the etiology for infertility. This article provides an overview of the multimodality imaging assessment of female infertility and details the developmental and acquired pelvic abnormalities in which diagnostic imaging aids in evaluation. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1016/j.rcl.2019.10.010DOI Listing

Evolving ethical issues with advances in uterus transplantation.

Am J Obstet Gynecol 2020 06 22;222(6):584.e1-584.e5. Epub 2020 Jan 22.

Women's Health Institute, Cleveland Clinic, Cleveland, OH.

While uterus transplantation was once considered only a theoretical possibility for patients with uterine factor infertility, researchers have now developed methods of transplantation that have led to successful pregnancies with multiple children born to date. Because of the unique and significant nature of this type of research, it has been undertaken with collaboration not only with scientists and physicians but also with bioethicists, who paved the initial path for research of uterus transplantation to take place. As the science of uterus transplantation continues to advance, so too must the public dialogue among obstetrician/gynecologists, transplant surgeons, bioethicists, and other key stakeholders in defining the continued direction of research in addition to planning for the clinical implementation of uterus transplantation as a therapeutic option. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1016/j.ajog.2020.01.032DOI Listing

Syndrome Mayer-Rokitansky-Küster-Hauser - uterine and vaginal agenesis: current knowledge and therapeutic options.

Ceska Gynekol 2019 ;84(5):386-392

Objective: Evaluation of existing knowledge of etiopathogenesis, clinical manifestations and treatment options to increase quality of life in women with Mayer-Rokitansky-Küster-Hauser syndrome (MRKH).

Design: Review article.

Setting: Department of Obstetrics and Gynaecology, Second Faculty of Medicine, Charles University and Motol University Hospital, Prague. Read More

View Article

Download full-text PDF

Source
December 2019

Reproductive Implications and Management of Congenital Uterine Anomalies: Scientific Impact Paper No. 62 November 2019.

BJOG 2020 Apr 20;127(5):e1-e13. Epub 2019 Nov 20.

Congenital uterine anomalies (CUAs) are malformations of the womb that develop during fetal life. When a baby girl is in her mother's womb, her womb develops as two separate halves from two tubular structures called 'müllerian ducts', which fuse together before she is born. Abnormalities that occur during the baby's development can be variable from complete absence of a womb through to more subtle anomalies, which are classified into specific categories. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1111/1471-0528.15968DOI Listing
April 2020
5 Reads

Successful external cephalic version in a patient with uterus didelphys and fetal malpresentation.

BMJ Case Rep 2019 Nov 19;12(11). Epub 2019 Nov 19.

The Virtual Nephrologist, Inc, Lynn Haven, Florida, USA.

Müllerian anomalies are congenital malformations of the female reproductive organs that occur when the müllerian ducts develop abnormally. Different types of müllerian anomalies have different pregnancy outcomes. Breech presentation is a common occurrence in pregnant women with uterus didelphys, and caesarean section is the traditional mode of delivery under such circumstances. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1136/bcr-2019-230965DOI Listing
November 2019

Ultrasound Evaluation in Female Infertility: Part 2, the Uterus and Implantation of the Embryo.

Authors:
Stuart Campbell

Obstet Gynecol Clin North Am 2019 Dec;46(4):697-713

Create Fertility, 150 Cheapside, London EC2V 6ET, UK. Electronic address:

This article discusses the role of ultrasound in assessing endometrial and uterine factors affecting the implantation of the embryo. Modern ultrasound equipment includes advanced Doppler and three-dimensional facilities that refine our understanding of factors that affect implantation such as endometrial blood flow. Three dimensional scanning is principally used to define the relationship of fibroids to the endometrial cavity, the position of polyps and other anatomic structures relative to the implantation site, to diagnose adenomyosis, to reveal and classify congenital uterine malformations and in the differential diagnosis od ovarian and extraovarian cystic structures in the pelvis. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1016/j.ogc.2019.08.002DOI Listing
December 2019

Herlyn-Werner-Wunderlich syndrome: Diagnosis and treatment of an atypical case and review of literature.

Int J Surg Case Rep 2019 13;63:129-134. Epub 2019 Sep 13.

Pediatric Surgery, Hospital de Clínicas, Federal University of Paraná, Curitiba, PR, Brazil.

Introduction: Herlyn-Werner-Wunderlich syndrome (HWWS), also known as OHVIRA syndrome, is a rare congenital abnormality of Müllerian duct development characterized by uterus didelphys, unilateral blind hemivagina, and ipsilateral renal agenesis.

Presentation Of Case: In this report, the authors present the case of a 12-year-old patient presenting with progressive and disabling abdominal pain after the onset of menarche diagnosed with HWWS, describe the various diagnostic modalities and treatment options available, along with a current review of the literature.

Discussion: With normal external genitalia, HWWS is usually asymptomatic until menarche when patients present with worsening abdominal pain during menses and a palpable pelvic or abdominal mass. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1016/j.ijscr.2019.08.035DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6796699PMC
September 2019
6 Reads

Repair of Iatrogenic Urethral and Bladder Neck Injury Due to Missed Diagnosis of Mayer-Rokitansky-Küster-Hauser Syndrome.

Urology 2019 Dec 24;134:213-216. Epub 2019 Sep 24.

Schulich School of Medicine and Dentistry, Western University, London, Ontario, Canada; Division of Urology, Department of Surgery, London Health Sciences Centre, London, Ontario, Canada; Division of Paediatric Surgery, Department of Surgery, London Health Sciences Centre, London, Ontario, Canada.

Mayer-Rokitansky-Küster-Hauser syndrome is a congenital malformation disorder resulting in agenesis of the proximal vagina, absence of cervix, and variable development of the uterus. This report describes the repair of a complex iatrogenic urethrovaginal injury due to a missed diagnosis of Mayer-Rokitansky-Küster-Hauser. Our treatment utilized a primary urethroplasty through a transvaginal approach with bladder neck reconstruction and a Martius flap for secondary coverage. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1016/j.urology.2019.09.017DOI Listing
December 2019

Coexistence of Gonadal Dysgenesis and Mullerian Agenesis in a Female with 46 XX Karyotype: A Case Report.

JNMA J Nepal Med Assoc 2019 Mar-Apr;57(216):119-122

Department of Obstetrics and Gynaecology, Kathmandu Medical College and Teaching Hospital, Sinamangal, Kathmandu, Nepal.

Gonadal dysgenesis is a rare genetically heterogeneous disorder characterized by underdeveloped ovaries with consequent, impuberism, primary amenorrhea, and hypergonadotropic hypogonadism. Mullerian agenesis or Mayer‑Rokitansky‑Kuster‑Hauser syndrome is characterized by congenital aplasia of the uterus and the upper part (2/3) of the vagina in a woman with normal development of secondary sexual characteristics and a normal 46 XX karyotype. The association of gonadal dysgenesis and Mayer-Rokitansky-Kuster-Hauser syndrome is very rare and appears to be coincidental. Read More

View Article

Download full-text PDF

Source
February 2020
2 Reads

Neovagina creation methods and their potential impact on subsequent uterus transplantation: a review.

BJOG 2019 Oct 13;126(11):1328-1335. Epub 2019 Aug 13.

Department of Obstetrics and Gynaecology, University of Tübingen, Tübingen, Germany.

Congenital uterovaginal aplasia commonly occurs in Mayer-Rokitansky-Küster-Hauser syndrome. Various methods of neovagina creation exist, including nonsurgical self-dilation, surgical dilation, and surgical procedures involving skin or intestinal transplants. Subsequent uterus transplantation is necessary to enable pregnancy. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1111/1471-0528.15888DOI Listing
October 2019
4 Reads

Diagnosis and gestational follow-up in a patient with Herlyn-Werner-Wünderlich syndrome, a case report.

Taiwan J Obstet Gynecol 2019 Jul;58(4):560-565

Department of Obstetrics and Gynecology, Hospital Quirón Málaga, Spain.

Objective: Herlyn-Werner-Wünderlich syndrome (HWW) is a rare congenital malformation of the urogenital tract due to a fusion failure in the Müllerian ducts. This anomaly consists of a didelphus uterus with obstructed hemivagina and sometimes associated with ipsilateral renal agenesis. The treatment of choice is surgical, it consists of a simple procedure of resection of the vaginal septum and drainage of the obstructed hemivagina and retained collections. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1016/j.tjog.2019.05.023DOI Listing
July 2019
2 Reads

AMH and AMHR2 mutations: A spectrum of reproductive phenotypes across vertebrate species.

Dev Biol 2019 11 10;455(1):1-9. Epub 2019 Jul 10.

Department of Genetics, University of Texas MD Anderson Cancer Center, Houston, TX, 77030, USA; Program in Developmental Biology, Baylor College of Medicine, Houston, Texas, 77030, USA; Program in Genetics and Epigenetics, MD Anderson Cancer Center UT Health Graduate School of Biomedical Sciences, Houston, TX, 77030, USA. Electronic address:

Anti-Müllerian hormone (AMH) is a member of the Transforming Growth Factor-β family of secreted signaling proteins. AMH is expressed in Sertoli cells of the fetal and adult testes and granulosa cells of the postnatal ovary. AMH is required for the regression of the Müllerian ducts in mammalian fetuses during male differentiation. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1016/j.ydbio.2019.07.006DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6754765PMC
November 2019
15 Reads

[Place of ultrasound in the management of Mayer-Rokitansky-Kuster-Hauser syndrome. Observational study from 2000 to 2017 within university hospital of Strasbourg].

Authors:
T Schwaab A Bryand

Gynecol Obstet Fertil Senol 2019 11 5;47(11):783-789. Epub 2019 Jul 5.

Service de chirurgie gynécologique, pôle de gynécologie-obstétrique, centre médico-chirurgical obstétrique (CMCO), CHRU de Strasbourg, 19, rue Louis-Pasteur, 67300 Schiltigheim, France.

Objectives: Mayer-Rokitansky-Küster-Hauser syndrome (MRKH) is the most common cause of congenital absence or severe hypoplasia of structures derived from Muller's canals including the upper vagina, uterus and fallopian tubes. The definition of this syndrome is the presence of normal female secondary sexual development criteria relating to the presence of functional ovaries associated with vaginal agenesis and uterine anomalies ranging from a rudimentary uterus to the total absence of uterus. The main clinical sign of MRKH is primary amenorrhea. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1016/j.gofs.2019.07.002DOI Listing
November 2019
4 Reads

Persistent Mullerian duct Syndrome in a Brazilian miniature schnauzer dog.

An Acad Bras Cienc 2019 Jun 19;91(2):e20180752. Epub 2019 Jun 19.

James A. Baker Institute for Animal Health, College of Veterinary Medicine, Cornell University, Ithaca, NY 14853, USA.

Here we describe an eight-year-old miniature schnauzer (MS) dog from Brazil with Persistent Mullerian Duct Syndrome (PMDS) and the single base pair substitution in AMHR2 exon 3, first detected in this breed in the USA. This finding is evidence of mutation dissemination to South America. In PMDS, a type of XY Disorder of Sex Development (DSD), dogs with a male karyotype and external phenotype also have a uterus, oviducts, and a cranial vagina internally. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1590/0001-3765201920180752DOI Listing
June 2019
9 Reads

A mutation inactivating the distal SF1 binding site on the human anti-Müllerian hormone promoter causes persistent Müllerian duct syndrome.

Hum Mol Genet 2019 10;28(19):3211-3218

Inserm UMR_S938, Centre de Recherche Saint Antoine, Sorbonne Université, IHU ICAN, Paris, France.

The persistent Müllerian duct syndrome (PMDS) is a 46,XY disorder of sexual development characterized by the persistence of Müllerian duct derivatives, uterus and tubes, in otherwise normally masculinized males. The condition, transmitted as a recessive autosomal trait, is usually due to mutations in either the anti-Müllerian hormone (AMH) gene or its main receptor. Many variants of these genes have been described, all targeting the coding sequences. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1093/hmg/ddz147DOI Listing
October 2019

Novel homozygous mutation in a colombian patient with persistent müllerian duct syndrome: expanded phenotype.

Int Braz J Urol 2019 Sep-Oct;45(5):1064-1070

Human Genetic Institute, Pontificia Universidad Javeriana, Bogotá, Colombia.

The anti-Müllerian hormone triggers the regression of uterus and fallopian tubes in male embryos; if there are problems in the synthesis or action of this protein, Müllerian structures persist in an otherwise phenotypic male. The most frequent clinical presentation of Persistent Mullerian Duct syndrome is cryptorchidism and inguinal hernia. The few cases reported in adults are incidental findings or inguinal hernias. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1590/S1677-5538.IBJU.2018.0808DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6844334PMC
October 2019
7 Reads

Unicornuate uterus with a rudimentary non-communicating cavitary horn in association with VACTERL association: case report.

BMC Womens Health 2019 05 30;19(1):71. Epub 2019 May 30.

Department of Obstetrics & Gynecology, Faculty of Medicine, Jordan University of Science & Technology, Irbid, Jordan.

Background: The unicornuate uterus is caused by abnormal or failed development of one Müllerian duct. Unicornuate uteri with functioning non-communicating rudimentary horns are susceptible to many gynaecologic and obstetric complications such as hematometra, endometriosis and ectopic pregnancy and thus surgical resection is usually recommended.. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1186/s12905-019-0768-4DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6543654PMC
May 2019
10 Reads

Tilapia fish skin as a new biologic graft for neovaginoplasty in Mayer-Rokitansky-Kuster-Hauser syndrome: a video case report.

Fertil Steril 2019 07 15;112(1):174-176. Epub 2019 May 15.

Maternidade Escola Assis Chateaubriand, Fortaleza, Ceará, Brazil. Electronic address:

Objective: To describe a McIndoe procedure technique for surgical management of Mayer-Rokitansky-Kuster-Hauser syndrome with the use of Nile tilapia skin as a scaffold for the proliferation of new vaginal epithelium.

Design: Surgical video article. Local Institutional Review Board approval and written permission from the patient were obtained. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1016/j.fertnstert.2019.04.003DOI Listing
July 2019
8 Reads

A unique case of aggressive uterine cancer in a 45-year-old man with persistent Müllerian duct syndrome.

Arch Esp Urol 2019 05;72(4):435-438

Department of Urology - Oncology. "Theagenio" Anticancer Hospital of Thessaloniki. Thessaloniki. Greece. Urology Department. The Pennine Acute Hospitals. NHS Trust. Manchester. U.K.

Objective: Persistent Müllerian Duct Syndrome (PMDS), one of the causes of male pseudohermaphroditism, is a rare syndrome characterized by the presence of internal female genitalia (uterus, fallopian, tubes, cervix and upper vagina) in otherwise phenotypically and normally virilized men. METHODS: We present the 4th documented case of uterine malignancy in a 45-year-old man with PMDS presenting with lower abdominal protuberance and hematuria.

Results: Although testicular malignancies are common in undescended testis associated with PMDS, very few cases of müllerian duct malignancies have been reported. Read More

View Article

Download full-text PDF

Source
May 2019
8 Reads

Clinical pregnancy after deceased donor uterus transplantation: Lessons learned and future perspectives.

J Obstet Gynaecol Res 2019 Aug 6;45(8):1458-1465. Epub 2019 May 6.

Department of Obstetrics and Gynecology, 2nd Faculty of Medicine, Charles University and Motol University Hospital, Prague, Czech Republic.

Aim: To describe our first clinical pregnancy following a uterus transplant from a brain-dead donor and to discuss current issues with deceased donor uterus transplantation as they relate to obstetrical success.

Methods: In August 2016, a 26-year-old woman with Mayer-Rokitansky-Küster-Hauser syndrome was the fourth person worldwide to receive a uterine transplant from a deceased donor and was the second in our trial. in vitro fertilization treatments using the long gonadotropin-releasing hormone agonist protocol preceded the transplantation procedure. Read More

View Article

Download full-text PDF

Source
https://onlinelibrary.wiley.com/doi/abs/10.1111/jog.13992
Publisher Site
http://dx.doi.org/10.1111/jog.13992DOI Listing
August 2019
11 Reads

Endometriosis in MRKH cases as a proof for the coelomic metaplasia hypothesis?

Reproduction 2019 08;158(2):R41-R47

Institute of Gynecology and Obstetrics, Giessen, Germany.

A diagnosis of endometriosis is based upon the histological identification of endometrial tissue at ectopic sites which are commonly located on the pelvic organs, the peritoneum and ovary. In rare cases, ectopic lesions can be found in other organs, such as kidney, bladder, lung or brain. Diagnosis is achieved by laparoscopic intervention followed by histological confirmation of endometriotic tissue. Read More

View Article

Download full-text PDF

Source
https://rep.bioscientifica.com/view/journals/rep/aop/rep-19-
Publisher Site
http://dx.doi.org/10.1530/REP-19-0106DOI Listing
August 2019
24 Reads

Septate uterus according to ESHRE/ESGE, ASRM and CUME definitions: association with infertility and miscarriage, cost and warnings for women and healthcare systems.

Ultrasound Obstet Gynecol 2019 Dec;54(6):800-814

Department of Obstetrics and Gynecology, Ribeirão Preto Medical School, University of São Paulo, Ribeirão Preto, Brazil.

Objectives: To estimate the differences in frequency of diagnosis of septate uterus using three different definitions and determine whether these differences are significant in clinical practice, and to examine the association between diagnosis of septate uterus, using each of the three definitions, and infertility and/or previous miscarriage as well as the cost of allocation to surgery.

Methods: This was a secondary analysis of data from a prospective study of 261 consecutive women of reproductive age attending a private clinic focused on the diagnosis and treatment of congenital uterine malformations. Reanalysis of the datasets was performed according to three different means of defining septate uterus: following the recommendations of the American Society for Reproductive Medicine (ASRM), a 2016 update of those of the American Fertility Society from 1988 (ASRM-2016: internal fundal indentation depth ≥ 1. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1002/uog.20291DOI Listing
December 2019
7 Reads

Retinoic acid signaling determines the fate of the uterus from the mouse Müllerian duct.

Reprod Toxicol 2019 06 30;86:56-61. Epub 2019 Mar 30.

Graduate School of Nanobioscience, Yokohama City University, Yokohama 236-0027, Japan. Electronic address:

In female mice, the Müllerian duct develops into the oviduct, uterus and vagina. The fate of epithelia is determined by factors secreted from the mesenchyme. Retinoic acid (RA) and its receptors are present in the mesenchyme of cranial Müllerian duct. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1016/j.reprotox.2019.03.006DOI Listing
June 2019
13 Reads

Urogynecological and Sexual Functions after Vecchietti Reconstructive Surgery.

Biomed Res Int 2019 25;2019:2360185. Epub 2019 Feb 25.

2nd Department of Gynecology, Medical University in Lublin, ul. Jaczewskiego 8, 20-954 Lublin, Poland.

Hypothesis/aims Of Study: Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome is the second most common cause of primary amenorrhea. The ESHRE/ESGE categorizes this disorder within the class 5 uterine malformation of the female genital tract anomalies. It is characterized by congenital absence of the uterus, cervix, and upper part of the vagina in otherwise phenotypically normal 46XX females. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1155/2019/2360185DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6413384PMC
July 2019
10 Reads

Simultaneous correction of breast hypertrophy and vaginal agenesis: Aesthetic surgery to the aid of reconstructive surgery.

J Obstet Gynaecol Res 2019 Jul 27;45(7):1398-1403. Epub 2019 Mar 27.

Department of Woman and Child Health and Public Health, Fondazione Policlinico Universitario A. Gemelli IRCCS - LargoAgostino Gemelli 1, Rome, Italy.

Mayer-Rokitansky-Küster-Hauser syndrome is characterized by aplasia of the uterus and the upper ⅔ of the vagina, with normal female genotype (46,XX) and the development of secondary sexual characters. One of the most popular surgical treatment is the McIndoe procedure, which major drawbacks are the scar of split thickness skin graft (STSG)harvesting, and risk of vaginal stenosis for graft retraction. We report the case of a patient, operated modifying the McIndoe technique, by harvesting a full thickness skin grafts (FTSG) from patient's breasts, undergone simultaneous reduction mammoplasty. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1111/jog.13967DOI Listing
July 2019
7 Reads

Mayer-Rokitansky-Küster-Hauser syndrome with a uterine cervix and normal vagina associated with gonadal dysgenesis in a 46,XX female.

J Obstet Gynaecol Res 2019 Jul 26;45(7):1386-1390. Epub 2019 Mar 26.

Department of Obstetrics and Gynecology, Federation of National Public Service Personnel Mutual Aid Associations, Tachikawa Hospital, Tokyo, Japan.

Coexistence of Mayer-Rokitansky-Küster-Hauser syndrome and gonadal dysgenesis is extremely rare, and a case of Mayer-Rokitansky-Küster-Hauser syndrome with a uterine cervix and normal vagina has not been reported. Here, we report such a case associated with gonadal dysgenesis. A 17-year-old female presented with primary amenorrhea and undeveloped secondary sexual characteristics. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1111/jog.13956DOI Listing
July 2019
10 Reads

Surgical management of congenital uterine anomalies (including indications and surgical techniques).

Best Pract Res Clin Obstet Gynaecol 2019 Aug 15;59:66-76. Epub 2019 Feb 15.

1st Department of Obstetrics and Gynaecology, Aristotle University of Thessaloniki, Papageorgiou Hospital, Thessaloniki, Greece.

The prevalence of congenital uterine anomalies (CUA) is reported to be 4.3-6.7% in the general population, 3. Read More

View Article

Download full-text PDF

Source
https://linkinghub.elsevier.com/retrieve/pii/S15216934193000
Publisher Site
http://dx.doi.org/10.1016/j.bpobgyn.2019.02.006DOI Listing
August 2019
24 Reads

Primary amenorrhoea secondary to two different syndromes: a case study.

BMJ Case Rep 2019 Mar 15;12(3). Epub 2019 Mar 15.

National Institute of Diabetes and Endocrinology, Department of Medicine, Dow University of Health Sciences, Karachi, Pakistan.

Turner syndrome is a relatively common chromosomal abnormality presenting as primary amenorrhoea in gynaecological and endocrine clinics, caused by complete or partial X monosomy in some or all cells. Mayer-Rokitansky-Kuster-Hauser syndrome is another common cause of primary amenorrhoea characterised by Mullerian agenesis of varying degrees. We report a case of an 18-year-old girl, who presented with primary amenorrhoea, absence of secondary sexual characteristics and short stature. Read More

View Article

Download full-text PDF

Source
http://casereports.bmj.com/lookup/doi/10.1136/bcr-2018-22814
Publisher Site
http://dx.doi.org/10.1136/bcr-2018-228148DOI Listing
March 2019
17 Reads

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

Br J Radiol 2019 May 15;92(1097):20190045. Epub 2019 Mar 15.

1 Department of Radiology and Imaging, Vardhman Mahavir Medical College and Safdarjung Hospital , New Delhi , India.

Objectives: Diagnostic role of MRI in Mayer-Rokitansky-Kuster-Hauser (MRKH) syndrome is well documented. Recent studies have shown the relevance of MRI in detailing the pelvic anatomy, particularly small Mullerian remnants. The results are, however, not consistent and reproducibility of MRI features has not been confirmed. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1259/bjr.20190045DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6580901PMC
May 2019
10 Reads

Abnormal hysteroscopy findings among a cross section of infertile Nigerian women.

Niger J Clin Pract 2019 Jan;22(1):9-15

Department of Obstetrics and Gynaecology, University of Portharcout Teaching Hospital, Port Harcourt, Nigeria.

Background: Intrauterine lesions are important causes of infertility. This study aims to evaluate the abnormal findings at hysteroscopy among infertile women seen in 2 new Fertility/Gynaecological Endoscopy units in Nigeria.

Methods: A prospective study of 159 infertile women who had diagnostic hysteroscopy in Nnamdi Azikiwe University Teaching hospital Nnewi and Holy Rosary Specialist Hospital, Onitsha to evaluate the intrauterine lesions seen. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.4103/njcp.njcp_404_17DOI Listing
January 2019
16 Reads

Multifaceted Hoxa13 function in urogenital development underlies the Hand-Foot-Genital Syndrome.

Hum Mol Genet 2019 05;28(10):1671-1681

Genetics and Development Research Unit, Institut de Recherches Cliniques de Montréal, Montreal, Quebec, Canada.

Hand-Foot-Genital syndrome is a rare condition caused by mutations in the HOXA13 gene and characterized by limb malformations and urogenital defects. While the role of Hoxa13 in limb development has been extensively studied, its function during the development of the urogenital system remains elusive mostly due to the embryonic lethality of Hoxa13 homozygous mutant mice. Using a conditional inactivation strategy, we show that mouse fetuses lacking Hoxa13 function develop megaureters, hydronephrosis and malformations of the uterus, reminiscent of the defects characterizing patients with Hand-Foot-Genital syndrome. Read More

View Article

Download full-text PDF

Source
https://academic.oup.com/hmg/advance-article/doi/10.1093/hmg
Publisher Site
http://dx.doi.org/10.1093/hmg/ddz013DOI Listing
May 2019
12 Reads

Double Cervix with Normal Uterus and Vagina - An Unclassified Müllerian Anomaly.

Int J Fertil Steril 2019 Apr 6;13(1):83-85. Epub 2019 Jan 6.

Gynaecology and Obstetrics Senior Consultant, Hospital Garcia de Orta, Almada, Portugal.

Müllerian anomalies are very common, and a frequent cause of infertility. The most used classification system until now, proposed by the American Society for Reproductive Medicine in 1988, categorizes comprehensively uterine anomalies but fails to classify defects of the cervix or vagina. This is based on a developmental theory that postulates that müllerian duct fusion is unidirectional, beginning caudally and extending cranially, which does not account for isolated cervical or vaginal defects. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.22074/ijfs.2019.5524DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6334021PMC
April 2019
15 Reads

Successful preterm pregnancy in a rare variation of Herlyn-Werner-Wunderlich syndrome: a case report.

BMC Pregnancy Childbirth 2018 Dec 17;18(1):498. Epub 2018 Dec 17.

Department of Woman's and Child's Health, Obstetrics and Gynecological Unit, San Camillo-Forlanini Hospital, Circonvallazione Gianicolense 87, 00152, Rome, Italy.

Background: Herlyn-Werner-Wunderlich syndrome (HWWS) is an uncommon congenital anomaly of the female urogenital tract, characterised by uterus didelphys, obstructed hemivagina, and ipsilateral renal agenesis. We reported the difficult pregnancy course complicated by an extremely rare and unique case of this syndrome associated with ectrodactyly, a clinical combination never described in literature.

Case Presentation: A 28- year-old nulliparous woman previously diagnosed for HWWS associated with ectrodactyly of the right foot and with a history of abdominal left hemi-hysterectomy, ipsilateral salpingectomy, vaginal reconstruction when she was an adolescent. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1186/s12884-018-2133-2DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6296152PMC
December 2018
28 Reads

Identification of Candidate Genes for Mayer-Rokitansky-Küster-Hauser Syndrome Using Genomic Approaches.

Sex Dev 2019 1;13(1):26-34. Epub 2018 Dec 1.

Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome is a disorder of sex development which affects 1 in 4,500 females and is characterized by agenesis of müllerian structures, including the uterus, cervix, and upper vagina. It can occur in isolation (type 1) or in conjunction with various anomalies (type 2), with a subset of these comprising müllerian, renal, and cervicothoracic abnormalities (MURCS) association. The genetic causes of MRKH have been investigated previously yielding limited results, with massive parallel sequencing becoming increasingly utilized. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1159/000494896DOI Listing
August 2019
3 Reads

Transverse testicular ectopia associated with persistent Müllerian duct syndrome treated by transseptal orchiopexy: A case report.

Medicine (Baltimore) 2018 Nov;97(48):e13305

Departments of Urology.

Rationale: Persistent Müllerian duct syndrome (PMDS) is rare form of male pseudohermaphroditism characterized by the presence of uterus and fallopian tubes with normal external genitalia and secondary sexual characteristics. Transverse testicular ectopia (TTE) is also a rare form of testicular ectopia that may be associated with PMDS.

Patient Concerns: We present a 2-year-old boy who presented with bilateral non-palpable testes with left inguinal mass. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1097/MD.0000000000013305DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6283222PMC
November 2018
10 Reads

Müllerian Aplasia with Severe Hematometra: A Case Report of Diagnosis and Management in a Low Resource Setting.

J Pediatr Adolesc Gynecol 2019 Apr 22;32(2):189-192. Epub 2018 Nov 22.

Department of Obstetrics and Gynecology, University of Iowa, Carver College of Medicine, Iowa City, Iowa.

Background: Müllerian aplasia or Mayer-Rokitansky-Küster-Hauser syndrome is described as congenital absence of the proximal vagina with or without absence of the cervix and uterus, most often recognized after the onset of primary amenorrhea.

Case: An 18-year-old woman presented to a free medical clinic in Arcahaie, Haiti with primary amenorrhea, abdominal distention, and cyclic monthly abdominal pain. Physical exam was significant for uterus palpable superior to the umbilicus, absence of vagina, and rectal exam without palpable vagina or cervix. Read More

View Article

Download full-text PDF

Source
https://linkinghub.elsevier.com/retrieve/pii/S10833188183035
Publisher Site
http://dx.doi.org/10.1016/j.jpag.2018.11.006DOI Listing
April 2019
19 Reads

Persistent Mullerian duct syndrome: Report of two cases with phenotypical immunohistochemical profiling.

Urologia 2018 Nov 28;85(4):177-181. Epub 2018 Mar 28.

1 Pathology Service, Addarii Institute of Oncology, S. Orsola-Malpighi Hospital, University of Bologna, Bologna, Italy.

Introduction:: Persistent Mullerian duct syndrome is a rare disorder of male organ development characterized by internal male pseudohermaphroditism. Persistent Mullerian duct syndrome is usually an incidental finding in patients presenting cryptorchidism, inguinal hernia, or a previous story of undescended testes.

Case Description:: We report on two cases of persistent Mullerian duct syndrome: an adult fertile male with uterus and ectopic prostate occurring as pelvic mass and a 75-year-old organ donor with uterus and two fallopian tubes, discovered in course of organ recruitment. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1177/0391560317749426DOI Listing
November 2018
34 Reads

Congenital anal atresia with rectovestibular fistula, scoliosis, unilateral renal agenesis, and finger defect (VACTERL association) in a patient with partial bicornuate uterus and distal vaginal atresia: A case report.

Medicine (Baltimore) 2018 Nov;97(45):e12822

Department of Obstetrics and Gynecology, Peking Union Medical College Hospital, Peking Union Medical College, Chinese Academy of Medical Sciences, Beijing, P. R. of China.

Rationale: Vertebral defect, anal atresia, cardiac defect, tracheoesophageal fistula/esophageal atresia, renal defect, and limb defect (VACTERL) association and Müllerian duct anomalies are rare conditions. We present a rare condition with the co-occurrence of the VACTERL association and Müllerian duct hypoplasia to characterize patients' clinical presentations, outcomes, and treatment.

Patient Concerns: An 11-year-old girl presented to our hospital with severe lower abdominal pain, lower vaginal atresia with enlargement of the upper vagina and a bicornuate uterus with a Y-shaped uterine cavity filled with hematometra on pelvic magnetic resonance imaging. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1097/MD.0000000000012822DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6250493PMC
November 2018
31 Reads

An Atypical Presentation of Ectopic Pregnancy With Unicornuate Uterus and Undescended Fallopian Tube.

J Obstet Gynaecol Can 2019 Feb 30;41(2):214-216. Epub 2018 Oct 30.

Department of Obstetrics and Gynecology, Faculty of Medicine and Dentistry, University of Alberta, Edmonton, AB.

Background: Müllerian duct abnormalities are common and may be related to pregnancy complications. Few cases of ectopic pregnancy within an undescended Fallopian tube have been reported, and they present a diagnostic challenge.

Case: A 32-year-old pregnant woman had three presentations to the emergency department for increasing left upper abdominal pain, chest pain, shortness of breath, and eventual hemodynamic instability in the setting of right unicornuate uterus and undiagnosed undescended left adnexa. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1016/j.jogc.2018.08.013DOI Listing
February 2019
14 Reads

Ultrasound Features of Rudimentary Horn Ectopic Pregnancies.

J Ultrasound Med 2019 Jun 20;38(6):1643-1647. Epub 2018 Oct 20.

Department of Radiology and Radiological Sciences, Vanderbilt University Medical Center, Nashville, Tennessee, USA.

Rudimentary horn ectopic pregnancies are uncommonly encountered in women with müllerian duct anomalies. The clinical presentation of this entity is nonspecific, giving ultrasound a critical role in making the diagnosis. Timely diagnosis and management of rudimentary horn ectopic pregnancies are pivotal in reducing the high rates of uterine rupture and maternal mortality historically associated with this condition. Read More

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1002/jum.14847DOI Listing
June 2019
18 Reads

Severe incomplete fusion of the Müllerian ducts influences reproduction in Holstein cattle.

Theriogenology 2019 Jan 28;123:209-215. Epub 2018 Sep 28.

Laboratory of Theriogenology, Department of Veterinary Medical Sciences, Graduate School of Agriculture and Life Sciences, The University of Tokyo, Tokyo 113-8657, Japan.

Fusion failure of the Müllerian ducts is thought to occur congenitally in cattle. We aimed to elucidate the contribution of incomplete fusion of the Müllerian ducts to reproductive difficulties in dairy cattle. We observed the vaginas of Holstein cattle to classify the anomalies into mild and severe types, based on severity of incomplete fusion, and recorded information about the cattle at the time of artificial insemination (AI) or embryo transfer. Read More

View Article

Download full-text PDF

Source
https://linkinghub.elsevier.com/retrieve/pii/S0093691X183087
Publisher Site
http://dx.doi.org/10.1016/j.theriogenology.2018.09.035DOI Listing
January 2019
18 Reads

Uterus transplantation: Which indications?

J Gynecol Obstet Hum Reprod 2019 Jan 11;48(1):7-8. Epub 2018 Oct 11.

Département de Gynécologie et Obstétrique, CHU Limoges, av Dominique Larrey, 87000 Limoges, France; INSERM, UMR-1248, CHU Limoges, 87000 Limoges, France. Electronic address:

View Article

Download full-text PDF

Source
https://linkinghub.elsevier.com/retrieve/pii/S24687847183035
Publisher Site
http://dx.doi.org/10.1016/j.jogoh.2018.10.003DOI Listing
January 2019
14 Reads

Three-dimensional scan of the uterine cavity of infertile women before assisted reproductive technology use.

Medicine (Baltimore) 2018 Oct;97(41):e12764

Department of Medical and Surgical Specialties, Faculty of Medicine, "Transilvania" University of Brasov, Brasov.

The primary objective was to assess the utility of routine 3-dimensional (3D) ultrasound in the evaluation of infertile women and to estimate the prevalence of uterine anomalies before the use of assisted reproductive technology (ART), using the European Society of Human Reproduction and Embryology and the European Society for Gynaecological Endoscopy classification system. A second objective was to assess the effect of uterine anomalies on the pregnancy rate in patients who underwent assisted reproductive techniques.We retrospectively studied 668 patients treated in the Department Obstetrics Gynecology and Neonatology "Sf Ioan" Clinical Emergency Hospital and in the Department of Medical and Surgical Specialties, Faculty of Medicine "'Transilvania" University of Brasov between July 2016 and February 2017 for subfertility. Read More

View Article

Download full-text PDF

Source
http://Insights.ovid.com/crossref?an=00005792-201810120-0006
Publisher Site
http://dx.doi.org/10.1097/MD.0000000000012764DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6203555PMC
October 2018
13 Reads

Uterus didelphys: Anatomic variant.

J Med Imaging Radiat Oncol 2018 Oct;62 Suppl 1:115

Department of Radiology, Concord Repatriation General Hospital, Concord, New South Wales, Australia.

View Article

Download full-text PDF

Source
http://doi.wiley.com/10.1111/1754-9485.58_12784
Publisher Site
http://dx.doi.org/10.1111/1754-9485.58_12784DOI Listing
October 2018
8 Reads

Late presentation, MR imaging features and surgical treatment of Herlyn-Werner-Wunderlich syndrome (classification 2.2); a case report.

BMC Womens Health 2018 10 3;18(1):161. Epub 2018 Oct 3.

Obstetrics and Gynecology Department, French Medical Institute for Mothers and Children (FMIC), Kabul, Afghanistan.

Background: Herlyn-Werner-Wunderlich syndrome is a very rare congenital genitourinary anomaly characterized by uterus didelphys, blind hemivagina and ipsilateral renal agenesis.

Case Presentation: Authors present a case of Herlyn-Werner-Wunderlich syndrome in a 19-year-old unmarried woman who presented with pelvic pain and pelvic mass. MR imaging revealed the typical features of didelphys uterus, obstructed right hemivagina and ipsilateral renal agenesis. Read More

View Article

Download full-text PDF

Source
https://bmcwomenshealth.biomedcentral.com/articles/10.1186/s
Publisher Site
http://dx.doi.org/10.1186/s12905-018-0655-4DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6171225PMC
October 2018
65 Reads
1.660 Impact Factor