1,680 results match your criteria Mullerian Duct Anomalies


In vivo ablation of the conserved GATA binding motif in the Amh promoter impairs Amh expression in the male mouse.

Endocrinology 2019 Feb 13. Epub 2019 Feb 13.

Reproduction, Mother and Child Health, Centre de recherche du CHU de Québec-Université Laval and Centre de recherche en reproduction, développement et santé intergénérationnelle (CRDSI), Quebec, Canada.

GATA4 is an essential transcriptional regulator required for gonadal development, differentiation, and function. In the developing testis, proposed GATA4-regulated genes include steroidogenic factor 1 (Nr5a1), SRY-related HMG box 9 (Sox9), and anti-Müllerian hormone (Amh). While some of these genes have been validated as genuine GATA4 targets, it remains unclear whether GATA4 is a direct regulator of endogenous Amh transcription. Read More

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http://dx.doi.org/10.1210/en.2019-00047DOI Listing
February 2019

Sequence Variants in TBX6 Are Associated with Disorders of the Müllerian Ducts: An Update.

Sex Dev 2019 Feb 9. Epub 2019 Feb 9.

Müllerian anomalies comprise the Mayer-Rokitansky-Küster-Hauser syndrome as well as fusion defects of the müllerian ducts. Recurrent micro-aberrations like deletions in 16p11.2 encompassing TBX6 were found to be causative in these patients. Read More

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http://dx.doi.org/10.1159/000496819DOI Listing
February 2019
2 Reads

[Clinical characteristics and complications in patients with OHVIRA (obstructed hemivagina and ipsilateral renal anomaly) syndrome. Our experience].

Cir Pediatr 2019 Jan 21;32(1):11-16. Epub 2019 Jan 21.

UGC Cirugía Pediátrica, UGC Pediatría. Hospital Universitario Reina Sofía. Córdoba.

Introduction: Obstructed Hemivagina and Ipsilateral Renal Agenesis (OHVIRA) syndrome is a rare variant of Müllerian anomalies. Delay in diagnosis and treatment can produce serious complications in patient's life and fertility. The aim of this study is to present our experience in clinical presentation, diagnosis and complications of patients with OHVIRA syndrome in a 20-year period. Read More

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January 2019

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

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http://dx.doi.org/10.4103/njcp.njcp_404_17DOI Listing
January 2019
5 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

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

Two Korean girls with complete androgen insensitivity syndrome diagnosed in infancy.

Ann Pediatr Endocrinol Metab 2018 Dec 31;23(4):220-225. Epub 2018 Dec 31.

Department of Laboratory Medicine, Seoul National University Hospital, Seoul, Korea.

Androgen insensitivity syndrome (AIS) is a rare genetic disease caused by various abnormalities in the androgen receptor (AR). The AR is an essential steroid hormone receptor that plays a critical role in male sexual differentiation and development and preservation of the male phenotype. Mutations in the AR gene on the X chromosome cause malfunction of the AR so that a 46,XY karyotype male has some physical characteristics of a woman or a full female phenotype. Read More

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http://dx.doi.org/10.6065/apem.2018.23.4.220DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6312918PMC
December 2018
1 Read

Diagnosis and laparoscopic management of a 5-week ectopic pregnancy in a rudimentary uterine horn: A case report.

Case Rep Womens Health 2019 Jan 15;21:e00088. Epub 2018 Nov 15.

Department of Clinical and Experimental Medicine, Division of Gynecology and Obstetrics, University of Pisa, Italy.

Uterine anomalies result from the failure of complete fusion of the Müllerian ducts during embryogenesis. A unicornuate uterus with a rudimentary horn is the rarest anomaly and results from the failure of one of the Müllerian ducts to develop completely and an incomplete fusion with the contralateral side. Diagnosis and surgical management of a 5-week ectopic pregnancy in a non-communicating rudimentary horn in an 18-year-old nulliparous woman in whom this congenital uterine anomaly was previously unknown are described. Read More

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http://dx.doi.org/10.1016/j.crwh.2018.e00088DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6305893PMC
January 2019
2 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

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http://dx.doi.org/10.1186/s12884-018-2133-2DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6296152PMC
December 2018
3 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

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http://dx.doi.org/10.1097/MD.0000000000012822DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6250493PMC
November 2018
11 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

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

Obstetrical Outcomes in Adult Patients Born with Complex Anorectal Malformations and Cloacal Anomalies: A Literature Review.

J Pediatr Adolesc Gynecol 2019 Feb 24;32(1):7-14. Epub 2018 Oct 24.

Nationwide Children's Hospital, Columbus, Ohio.

Patients born with complex anorectal malformations often have associated Müllerian anomalies, which might affect fertility and obstetrical outcomes. Other vertebral-anorectal-tracheoesophageal-renal-limb associations, such as renal or cardiac anomalies, could also affect pregnancy intention, fertility rates, and recommendations about mode of delivery or obstetrical outcomes. Associated conditions present at birth, like hydrocolpos, could also potentially affect fertility. Read More

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http://dx.doi.org/10.1016/j.jpag.2018.10.002DOI Listing
February 2019
17 Reads

Ultrasound Features of Rudimentary Horn Ectopic Pregnancies.

J Ultrasound Med 2018 Oct 20. 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

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http://dx.doi.org/10.1002/jum.14847DOI Listing
October 2018
7 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

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https://linkinghub.elsevier.com/retrieve/pii/S0093691X183087
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http://dx.doi.org/10.1016/j.theriogenology.2018.09.035DOI Listing
January 2019
5 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

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http://Insights.ovid.com/crossref?an=00005792-201810120-0006
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http://dx.doi.org/10.1097/MD.0000000000012764DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6203555PMC
October 2018
4 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.

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http://doi.wiley.com/10.1111/1754-9485.58_12784
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http://dx.doi.org/10.1111/1754-9485.58_12784DOI Listing
October 2018
2 Reads

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

BMC Womens Health 2018 Oct 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

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

Case 29-2018: A 31-Year-Old Woman with Infertility.

N Engl J Med 2018 Sep;379(12):1162-1172

From the Department of Obstetrics and Gynecology, Hospital of the University of Pennsylvania, and the Department of Obstetrics and Gynecology, Perelman School of Medicine at the University of Pennsylvania, Philadelphia (C.C.); and the Departments of Radiology (A.K.), Urology (A.S.F.), Obstetrics and Gynecology (M.E.S.), and Pathology (E.O.), Massachusetts General Hospital, and the Departments of Radiology (A.K.), Surgery (A.S.F.), Obstetrics and Gynecology (M.E.S.), and Pathology (E.O.), Harvard Medical School - both in Boston.

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http://www.nejm.org/doi/10.1056/NEJMcpc1807497
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http://dx.doi.org/10.1056/NEJMcpc1807497DOI Listing
September 2018
11 Reads

Primary cervico-uterine anastomosis in a patient with agenesis of the uterine isthmus: A case report and review.

J Obstet Gynaecol Res 2018 Dec 9;44(12):2199-2203. Epub 2018 Sep 9.

Department of Obstetrics and Gynecology, Texas Tech University Health Sciences Center, Lubbock, Texas, USA.

Arrested caudal extension of the fused Müllerian ducts, either complete or segmental, causes obstruction of the outflow tract. We describe a case of agenesis of the uterine isthmus and the procedure to establish the continuity of the outflow tract. A 15-year-old girl with cyclic pelvic pain and amenorrhea was found to have a 3-cm gap between the uterine body and the cervix. Read More

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http://dx.doi.org/10.1111/jog.13779DOI Listing
December 2018
6 Reads

Advanced Imaging for the Diagnosis and Treatment of Coexistent Renal and Müllerian Abnormalities.

Curr Urol Rep 2018 Sep 6;19(11):89. Epub 2018 Sep 6.

Department of Obstetrics and Gynecology, University of Alabama at Birmingham, Birmingham, AL, USA.

Purpose Of Review: Abnormal development of the uterus, cervix, and proximal 2/3 of the vagina results in Müllerian duct anomalies. Because of the close embryologic relationship between the developing female genital and urinary tracts, abnormalities of the urinary tract often accompany Müllerian duct anomalies. Magnetic resonance imaging (MRI) is the current gold standard-imaging modality in the evaluation of the anomalies of the female reproductive tract. Read More

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http://dx.doi.org/10.1007/s11934-018-0840-xDOI Listing
September 2018
11 Reads

Bicornuate Bicollis Uterus with Obstruction of the Lower Uterine Segment and Cervical Prolapse Complicating Pregnancy.

Case Rep Obstet Gynecol 2018 13;2018:8910976. Epub 2018 Aug 13.

University of Illinois College of Medicine-Rockford, Department of Obstetrics and Gynecology, 1601 Parkview Ave, Rockford, IL 61101, USA.

Congenital Mullerian duct anomalies are conditions involving the female genital tract. Cases of complex Mullerian duct anomalies with involvement of the renal system are rare. Occasionally, these cases can be associated with obstetrical complications. Read More

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https://www.hindawi.com/journals/criog/2018/8910976/
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http://dx.doi.org/10.1155/2018/8910976DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6109999PMC
August 2018
11 Reads

[Value of MRI in the pre-operative diagnosis and classification of oblique vaginal septum syndrome].

Zhonghua Fu Chan Ke Za Zhi 2018 Aug;53(8):534-539

Department of Radiology, Peking Union Medical College Hospital, Peking Union Medical College, Chinese Academy of Medical Sciences, Beijing 100730, China.

To explore the role of MRI in the pre-operative diagnosis and classification of oblique vaginal septum syndrome (OVSS) . A retrospective analysis of the clinical records and pre-operative MRI images of 19 patients with surgery proved OVSS was carried out. Two experienced radiologists reviewed the pre-operative pelvic MRI of the 19 patients in consensus blind to the surgery results. Read More

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http://dx.doi.org/10.3760/cma.j.issn.0529-567x.2018.08.005DOI Listing
August 2018
12 Reads

OHVIRA syndrome in post-cesarean period: An exclusive clinical scenario managed by two-staged operative procedure.

Taiwan J Obstet Gynecol 2018 08;57(4):613-615

Department of Obstetrics & Gynecology, Teerthanker Mahaveer Medical College & Research Centre, Teerthanker Mahaveer University, Moradabad, India.

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http://dx.doi.org/10.1016/j.tjog.2018.06.026DOI Listing

Use of infragluteal folds as a full-thickness skin graft donor site for construction of the neovagina in cases of Mullerian agenesis.

J Obstet Gynaecol Res 2018 Oct 16;44(10):1985-1994. Epub 2018 Aug 16.

Department of Plastic, Reconstructive, and Aesthetic Surgery, Erciyes University, School of Medicine, Kayseri, Turkey.

Aim: The aims of this study were to present the results of using infragluteal folds as a skin graft donor site in cases of Mullerian agenesis undergoing full-thickness skin graft vaginoplasty and to evaluate the outcomes of the surgical technique.

Methods: Demographics and clinical parameters of 24 consecutive Mullerian agenesis patients refusing any dilatational procedure and undergoing neovaginal construction using infragluteal folds skin grafts from May 2004 to July 2015 were analyzed.

Results: The mean age of the patients was 21. Read More

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http://dx.doi.org/10.1111/jog.13752DOI Listing
October 2018
18 Reads

[A rare cause of cryptorchidism, the persistence of müllerian ducts syndrome].

Rev Med Liege 2018 Jul;73(7-8):376-379

Service d'Urologie, CHR Citadelle, Liège, Belgique.

The Persistent Müllerian Ducts Syndrome (PMDS) is a rare congenital syndrome. It is one of abnormalities of genito-sexual development that is found on the normally virilized boy (46XY). It is characterized by the development of both Wolf structures and Müller duct. Read More

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July 2018
3 Reads

Search for altered imprinting marks in Mayer-Rokitansky-Küster-Hauser patients.

Mol Genet Genomic Med 2018 11 11;6(6):1225-1228. Epub 2018 Aug 11.

Institute of Human Genetics, University of Münster, Münster, Germany.

Background: Mayer-Rokitansky-Küster-Hauser syndrome (MRKH) is the second most common cause of primary amenorrhea and characterized by absence of the uterus and the upper part of the vagina. The etiology of MRKH is mainly unknown but a contribution of genomic alterations is probable. A molecular disturbance so far neglected in MRKH research is aberrant methylation at imprinted loci. Read More

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http://doi.wiley.com/10.1002/mgg3.426
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http://dx.doi.org/10.1002/mgg3.426DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6305658PMC
November 2018
8 Reads

Using 3D MRI can potentially enhance the ability of trained surgeons to more precisely diagnose Mullerian duct anomalies compared to MR alone.

Eur J Obstet Gynecol Reprod Biol 2018 Sep 9;228:313-318. Epub 2018 Jul 9.

Department of Obstetrics and Gynecology, Nan Fang Hospital of Southern Medical University, Guangzhou, Guangdong 510515, PR China. Electronic address:

Objective: The aim of this study was to examine if the advanced tool of 3D MRI model provides more precise information on the anatomy of pelvic organs than MRI alone, and compare to clinical and operative finding of patients with vagina in Müllerian duct anomalies.

Study Design: One hundred and ten patients with clinically and operatively proven Müllerian duct anomalies were included. The consistency of diagnosis of Mullerian tract anomalies by MRI and 3D MRI compared to clinically and surgically proven anomalies defined according to ASRM classification. Read More

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http://dx.doi.org/10.1016/j.ejogrb.2018.07.007DOI Listing
September 2018
16 Reads

Multiorgan system structural malformations associated with Mayer-Rokitansky-Küster-Hauser Syndrome (MRKHS) type 2: avoiding pitfalls in diagnosis, counseling and treatment.

BMJ Case Rep 2018 Jul 30;2018. Epub 2018 Jul 30.

Department of Ob/Gyn, Texas Tech University Health Science Center School of Medicine, Amarillo, Texas, USA.

An 18-year-old virginal woman was referred to the reproductive endocrinology clinic with primary amenorrhoea and secondary sexual development in the absence of pelvic pain. Additionally, she had significant congenital sensorineural hearing loss, autism, bipolar disorder and class III obesity. On physical examination, secondary sexual development was confirmed (Tanner 5 breasts and Tanner 4 pubic hair). Read More

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http://dx.doi.org/10.1136/bcr-2018-225977DOI Listing
July 2018
7 Reads

Successful Pregnancy Outcome after Open Strassman Metroplasty for Bicornuate Uterus.

Case Rep Obstet Gynecol 2018 31;2018:4579736. Epub 2018 May 31.

Aga Khan University Hospital, Nairobi, Kenya.

Introduction: Müllerian duct anomalies represent a group of congenital malformations that result from failure to complete bilateral paramesonephric duct elongation, fusion, canalization, or septal resorption. These anomalies are rare in the general population with a bicornuate or didelphys uterus being among the common ones. Bicornuate uterine malformations are of clinical significance due to their adverse reproductive outcomes. Read More

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https://www.hindawi.com/journals/criog/2018/4579736/
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http://dx.doi.org/10.1155/2018/4579736DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6029456PMC
May 2018
4 Reads

Reproductive outcomes in women with unicornuate uterus undergoing in vitro fertilization: a nested case-control retrospective study.

Reprod Biol Endocrinol 2018 Jul 6;16(1):64. Epub 2018 Jul 6.

Center for Reproductive Medicine, Department of Obstetrics and Gynecology, Peking University Third Hospital, Beijing, 100191, China.

Background: Unicornuate uterus, a congenital uterine malformation resulting from unilateral maldevelopment of Mullerian duct, is more prevalent in women with infertility. Owing to relative rarity of the condition, the evidence on the associated reproductive outcomes is derived from small heterogeneous studies that report different clinical endpoints and often do not account for the anatomical variations of unicornuate uterus. The aim of this study was to evaluate the embryological and clinical outcomes following IVF-ICSI treatment in women with unicornuate uterus without rudimentary functional cavity (ESHRE-ESGE class IVb). Read More

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http://dx.doi.org/10.1186/s12958-018-0382-6DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6034283PMC
July 2018
6 Reads
2.410 Impact Factor

An unusual case of Herlyn-Werner-Wunderlich syndrome with a complete septate uterus and complicated urinary tract with rare ectopic ureteral opening.

Int J Gynaecol Obstet 2018 11 14;143(2):248-250. Epub 2018 Jul 14.

Department of Gynecology, Third Xiangya Hospital of Central South University, Changsha City, Hunan Province, China.

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http://dx.doi.org/10.1002/ijgo.12578DOI Listing
November 2018
3 Reads

Septate Uterus in a Girl with Rubinstein-Taybi Syndrome.

Case Rep Pediatr 2018 8;2018:7878156. Epub 2018 Apr 8.

Department of Gynecology and Obstetrics, Hospital Dr. Nélio Mendonça, Serviço de Saúde da Região Autónoma da Madeira, E.P.E. (SESARAM), Funchal, Portugal.

Rubinstein-Taybi syndrome is an extremely rare plurimalformative condition that can affect any organ. However, reports regarding gynecological problems are unusual. We report the first case of a septate uterus in an adolescent with this syndrome, in agreement with the American Society for Reproductive Medicine (ASRM) and the Congenital Uterine Malformations by Expert (CUME) criteria for uterine septum. Read More

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http://dx.doi.org/10.1155/2018/7878156DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5911335PMC
April 2018
1 Read

Ultrasound-guided follicle aspiration at time of laparotomy in a patient with Mayer-Rokitansky-Küster-Hauser syndrome.

Fertil Steril 2018 05;109(5):940

Department of Reproductive Endocrinology and Infertility, Washington University, St. Louis, Missouri.

Objective: To demonstrate a unique case of direct ultrasound-guided follicle aspiration at time of a laparotomy in a patient with Mayer-Rokitansky-Küster-Hauser Syndrome.

Design: Video presentation.

Setting: Academic fertility center PATIENT(S): A 32-year-old gravida 0 with Mayer-Rokitansky-Küster-Hauser Syndrome and bilateral ovarian masses presented as a fertility preservation consult from gynecologic oncology due to the possibility of bilateral oophorectomy. Read More

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http://dx.doi.org/10.1016/j.fertnstert.2018.02.007DOI Listing
May 2018
11 Reads

The Interest of Women with Mayer-Rokitansky-Küster-Hauser Syndrome and Laparoscopic Vecchietti Neovagina in Uterus Transplantation.

J Pediatr Adolesc Gynecol 2018 Oct 8;31(5):480-484. Epub 2018 May 8.

Transplant Surgery Department, Institute for Clinical and Experimental Medicine, Prague, Czech Republic.

Study Objective: The goal of this study was to assess a group of women with Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome with surgically created neovaginas in the interest of uterus transplantation (UTx) and to recruit the first group of applicants for a UTx trial.

Design And Setting: This was an original prospective study using semistructured interviews.

Participants: A study group of 50 women with MRKH syndrome with Vecchietti neovaginas was recruited via letter of invitation. Read More

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http://dx.doi.org/10.1016/j.jpag.2018.04.007DOI Listing
October 2018
11 Reads

Pelvic dystopia of right rudimentary multicystic dysplastic kidney as a rare cause of bedwetting in a patient with a single pelvic ectopic left kidney, and agenesis of the uterus and vagina (Mayer-Rokitansky-Küster-Hauser syndrome): a case report.

J Med Case Rep 2018 May 7;12(1):121. Epub 2018 May 7.

AkhmetYassawi International Kazakh-Turkish University, Clinical Diagnostic Center, Turkestan, Kazakhstan.

Background: Pelvic dystopia of rudimentary multicystic dysplastic kidney as a rare cause of bedwetting in children.

Case Presentation: We report the case of a 14-year-old Kazakh girl who presented with difficulty in starting the stream of urine and intermittent interruption of the urinary stream while voiding as well as bedwetting, caused by a rare congenital disease (pelvic dystopia of rudimentary multicystic dysplastic kidney). The diagnostic workup, differential diagnosis, and management, and a review of the literature are presented. Read More

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http://dx.doi.org/10.1186/s13256-018-1644-9DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5937041PMC
May 2018
3 Reads

Mayer-Rokitansky-Küster-Hauser Syndrome and 16p11.2 Recurrent Microdeletion: A Case Report and Review of the Literature.

J Pediatr Adolesc Gynecol 2018 Oct 3;31(5):533-535. Epub 2018 May 3.

Pediatric Highly Intensive Care Unit, Department of Pathophysiology and Transplantation, University of Milan, Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Milan, Italy. Electronic address:

Background: Mayer-Rokitansky-Küster-Hauser syndrome (MRKH; Online Mendelian Inheritance in Man #277000) is a rare disorder of the female reproductive tract. Its etiology is still unknown for most patients, although the genetic background of this condition has been intensively studied. Chromosome 16p11. Read More

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http://dx.doi.org/10.1016/j.jpag.2018.04.003DOI Listing
October 2018
2 Reads

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

Biomed Res Int 2018 12;2018:2369430. Epub 2018 Mar 12.

West China School of Preclinical and Forensic Medicine, Sichuan University, Chengdu, China.

In the most recent publications on Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome, the uterine remnants and ovaries in patients may develop uterine remnant leiomyoma, adenomyosis, or ovarian tumor, and this can lead to problems in differential diagnosis. Here we summarize the diagnosis methods and available interventions for ovarian tumor in MRKH syndrome, with emphasis on the relevant clinical findings and illustrative relevant case. According to the clinical findings and illustrative relevant case, with the help of imaging techniques, ovarian tumors can be detected in the pelvis in patients with MRKH syndrome and evaluated in terms of size. Read More

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http://dx.doi.org/10.1155/2018/2369430DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5867664PMC
September 2018
10 Reads

Uterine Paramesonephric Cysts in Sprague-Dawley Rats from National Toxicology Program Studies.

Toxicol Pathol 2018 06 29;46(4):421-430. Epub 2018 Apr 29.

1 Division of the National Toxicology Program, National Institute of Environmental Health Sciences, Research Triangle Park, North Carolina, USA.

Congenital uterine wall cysts arising from paramesonephric (Müllerian) and mesonephric (Wolffian) ducts are typically incidental findings in most species. We used immunohistochemistry to characterize and determine the origin of uterine cysts in Sprague-Dawley (SD) rats from multigeneration studies conducted by the National Toxicology Program. Subserosal uterine cysts were observed in 20 of the 2,400 SD rats evaluated in five studies, and 10 cysts were characterized for this study. Read More

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http://dx.doi.org/10.1177/0192623318772487DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5999558PMC
June 2018
12 Reads

Management of female congenital short patulous urethra with urethral tapering and pubovaginal sling: A report of two cases.

Indian J Urol 2018 Apr-Jun;34(2):149-151

Department of Urology, PGIMER and Dr. RML Hospital, New Delhi, India.

Congenital short patulous urethra is a rare entity and may be associated with developmental anomalies of mullerian ducts or urogenital sinus. We report the management of two cases of congenital short patulous urethra with stress urinary incontinence (SUI). Both the patients presented with SUI; one was diagnosed with Mayer-Rokitansky-Kuster-Hauser (MRKH) syndrome and the other had uterus didelphys with longitudinally septated vagina. Read More

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http://dx.doi.org/10.4103/iju.IJU_272_17DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5894289PMC
April 2018
6 Reads

What do you see in Fertility and Sterility video articles?

Authors:
J Preston Parry

Fertil Steril 2018 05;109(5):804

Parryscope Fertility, Madison, Mississippi.

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https://linkinghub.elsevier.com/retrieve/pii/S00150282183028
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http://dx.doi.org/10.1016/j.fertnstert.2018.03.025DOI Listing
May 2018
2 Reads

Transverse testicular ectopia with persistent mullerian duct syndrome.

BMJ Case Rep 2018 Apr 18;2018. Epub 2018 Apr 18.

Department of Surgery, Indira Gandhi Medical College, Shimla, India.

Persistent mullerian duct syndrome (PMDS) is a rare form of male pseudohermaphroditism caused by defects in synthesis or actions of mullerian inhibiting factor characterised by persistence of mullerian duct structures in a normal karyotype male. Transverse testicular ectopia (TTE) is a rare disease in which both testes are located in the same inguinal canal. We report a case of PMDS with TTE in a 1-year-old child who presented with non-palpable testis on right side with hernia on left side. Read More

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http://dx.doi.org/10.1136/bcr-2017-223994DOI Listing
April 2018
3 Reads

Increased incidence of abnormally located ovary in patients with Mayer-Rokitansky-Küster-Hauser syndrome: a retrospective analysis with magnetic resonance imaging.

Abdom Radiol (NY) 2018 Nov;43(11):3142-3146

Department of Radiology, Peking Union Medical College Hospital, Peking Union Medical College, Chinese Academy of Medical Sciences, Shuaifuyuan No.1, Wangfujing Street, Dongcheng District, Bejing, 100730, People's Republic of China.

Purpose: To explore the incidence of abnormally located ovary in patients with Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome as compared to a control group with magnetic resonance imaging.

Methods: Pelvic MR images of 83 MRKH patients and 60 age-matched normal females were retrospectively reviewed by two experienced gynecological radiologists in consensus. Characteristics including location of ovary, ovarian volume, and follicle counts were assessed. Read More

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http://link.springer.com/10.1007/s00261-018-1575-3
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http://dx.doi.org/10.1007/s00261-018-1575-3DOI Listing
November 2018
23 Reads

Female genital tract congenital malformations and the applicability of the ESHRE/ESGE classification: a systematic retrospective analysis of 920 patients.

Arch Gynecol Obstet 2018 Jun 16;297(6):1473-1481. Epub 2018 Mar 16.

Research Institute for Women's Health, Department of Women's Health and Department of Obstetrics and Gynecology, University of Tübingen, Calwerstr. 7, 72076, Tübingen, Germany.

Purpose: Several classification systems for female genital tract anomalies exist but are of limited use in clinical practice. We, therefore, assessed the applicability and ease of use of the new ESHRE/ESGE classification, using only patient records.

Methods: This retrospective, single-center, proof-of-principle study systematically analyzed the surgical reports and other hospital records of 920 inpatients and outpatients treated for confirmed female genital tract congenital malformations at a major German university hospital during 2003-2013. Read More

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http://dx.doi.org/10.1007/s00404-018-4749-xDOI Listing
June 2018
13 Reads

[Development and validation of the Chinese version of modified body imgae scale in Chinese population].

Zhonghua Fu Chan Ke Za Zhi 2018 Feb;53(2):88-91

Department of Obstetrics and Gynecology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences, Beijing 100730, China.

To develop the Chinese version of modified body image scale (MBIS) questionnaires, and to validate them in Chinese population. The original English MBIS questionnaire was translated into Chinese, following the WHO cross-cultural adaptation of health-related quality of life measures. The reliability and validity of the Chinese version of MBIS questionnaires were evaluated in Chinese population, MRKH syndrome patients. Read More

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http://dx.doi.org/10.3760/cma.j.issn.0529-567X.2018.02.004DOI Listing
February 2018
4 Reads

Uterus Septus Subtotalis Bicollis: A Rare Mullerian Duct Abnormality.

Ann Clin Lab Sci 2018 Jan;48(1):116-119

Department of Pathology and Genomic Medicine, Houston Methodist Hospital, Houston, TX

Mullerian duct anomalies (MDAs) are congenital defects of the female genital system that arise from abnormal embryological development of the Mullerian ducts. Septate uterus is the most common, resulting from incomplete resorption of the medial septum after fusion of the Mullerian ducts. Two main types of septate uterus exist, including septa that extend completely or partially from the uterine fundus to the cervical os. Read More

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January 2018
5 Reads

Vaginal reconstruction with the modified rectosigmoid colon: surgical technique, long-term results and sexual outcomes.

J Plast Surg Hand Surg 2018 Aug 8;52(4):210-216. Epub 2018 Mar 8.

c Department of Obstetrics and Gynecology , Akdeniz University Faculty of Medicine , Antalya , Turkey.

Although vaginal reconstructions with intestinal segments require particularly complex surgical procedures, this technique has become popular with respect to fairly good functional and esthetic outcomes. This study describes cases of vaginal reconstruction performed using a modified rectosigmoid colon held in an ischemic state in order to reduce secretion and denervated in order to prevent defecation problems. Vaginal reconstructions with rectosigmoid colon were performed on 43 patients. Read More

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http://dx.doi.org/10.1080/2000656X.2018.1444616DOI Listing
August 2018
37 Reads

Laparoscopic en bloc resection of a para-cervical cancer with OHVIRA syndrome.

Taiwan J Obstet Gynecol 2018 Feb;57(1):141-143

Department of Obstetrics and Gynecology, University of Perpetual Help Las Pinas and De La Salle University Medical Center, Cavite, Philippines. Electronic address:

Objective: There are some reports of cervical cancer with uterus didelphys but a case of clear cell carcinoma (CCCC) with Obstructed Hemivagina and Ipsilateral Renal Anomaly (OHVIRA) syndrome is extremely rare. The aim of this paper was to report a case of CCCC with OHVIRA syndrome and the difficulty in making a preoperative diagnosis.

Case Report: A 65 years old woman presented with postmenopausal bleeding and pelvic examination showed right paracervical mass. Read More

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http://dx.doi.org/10.1016/j.tjog.2017.12.024DOI Listing
February 2018
2 Reads

[Unicornuate uterus with cavitary non-communicating rudimentary horn: magnetic resonance characterization].

Rev Med Inst Mex Seguro Soc 2018 Jan-Feb;56(1):92-97

Curso de Alta Especialidad en Resonancia Magnética de Cuerpo Completo, Hospital Ángeles del Pedregal, Ciudad de México, México.

Mullerian duct or paramesonephric anomalies are a group of congenital malformations of the female genital tract that result from the alteration in one or more stages of embryonic development. The prevalence has increased, probably due to the progress of diagnostic imaging methods and the relevance that it has acquired in young women with infertility problems. Magnetic resonance imaging (MRI) is currently the method of choice for characterizing the different types of Mullerian anomalies, its complications and associated pathology. Read More

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August 2018
5 Reads

ACOG Committee Opinion No. 728: Müllerian Agenesis: Diagnosis, Management, And Treatment.

Authors:

Obstet Gynecol 2018 01;131(1):e35-e42

Müllerian agenesis, also referred to as müllerian aplasia, Mayer-Rokitansky-Küster-Hauser syndrome, or vaginal agenesis, has an incidence of 1 per 4,500-5,000 females. Müllerian agenesis is caused by embryologic underdevelopment of the müllerian duct, with resultant agenesis or atresia of the vagina, uterus, or both. Patients with müllerian agenesis usually are identified when they are evaluated for primary amenorrhea with otherwise typical growth and pubertal development. Read More

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http://Insights.ovid.com/crossref?an=00006250-201801000-0004
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http://dx.doi.org/10.1097/AOG.0000000000002458DOI Listing
January 2018
10 Reads

ACOG Committee Opinion No. 728 Summary: Müllerian Agenesis: Diagnosis, Management, And Treatment.

Authors:

Obstet Gynecol 2018 01;131(1):196-197

Müllerian agenesis, also referred to as müllerian aplasia, Mayer-Rokitansky-Küster-Hauser syndrome, or vaginal agenesis, has an incidence of 1 per 4,500-5,000 females. Müllerian agenesis is cau0073ed by embryologic underdevelopment of the müllerian duct, with resultant agenesis or atresia of the vagina, uterus, or both. Patients with müllerian agenesis usually are identified when they are evaluated for primary amenorrhea with otherwise typical growth and pubertal development. Read More

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http://Insights.ovid.com/crossref?an=00006250-201801000-0003
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http://dx.doi.org/10.1097/AOG.0000000000002452DOI Listing
January 2018
16 Reads

Etiology and management of primary amenorrhoea: A study of 102 cases at tertiary centre.

Taiwan J Obstet Gynecol 2017 Dec;56(6):761-764

Department of Obstetrics and Gynecology, All India Institute of Medical Sciences, Ansari Nagar, New Delhi 110029, India.

Objective: To determine the prevalence of etiologic causes of primary amenorrhea in Indian population.

Materials And Methods: A retrospective study was performed using 102 complete medical records of women with primary amenorrhea who attended the Gynaecologic Endocrinology Clinic, Department of Obstetrics and Gynaecology, AIIMS, New Delhi from September 2012 to September 2015. Cases were analysed according to clinical profile, development of secondary sexual characteristics, physical examination, pelvic and rectal examination, X-ray of chest and lumbo-sacral spine, hormone profile, pelvic USG, MRI, and cytogenetic study including karyotype. Read More

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http://dx.doi.org/10.1016/j.tjog.2017.10.010DOI Listing
December 2017
8 Reads