1,798 results match your criteria Mullerian Duct Anomalies


Interpreting body MRI cases: classic findings in pelvic MRI.

Abdom Radiol (NY) 2020 Jun 30. Epub 2020 Jun 30.

Department of Radiology, Thomas Jefferson University Hospital, Philadelphia, PA, USA.

The high contrast resolution provided by magnetic resonance imaging (MRI) compared to all other modalities allows the interpreting radiologist to make a specific diagnosis for many common and uncommon findings. In some cases, the diagnosis can be so certain that there is no differential diagnosis. In this article, we review the most classically recognized findings when interpreting MRI of the pelvis including the following: Ovaries: Simple cyst, hemorrhagic cyst, corpus luteum, dermoid, fibroma/fibrothecoma. Read More

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http://dx.doi.org/10.1007/s00261-020-02615-2DOI Listing

Niche role of MRI in the evaluation of female infertility.

Indian J Radiol Imaging 2020 Jan-Mar;30(1):32-45. Epub 2020 Mar 30.

Department of Obstetrics and Gynecology, Maulana Azad Medical College, New Delhi, India.

Infertility is a major social and clinical problem affecting 13-15% of couples worldwide. The pelvic causes of female infertility are categorized as ovarian disorders, tubal, peritubal disorders, and uterine disorders. Appropriate selection of an imaging modality is essential to accurately diagnose the aetiology of infertlity, since the imaging diagnosis directs the appropriate treatment to be instituted. Read More

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http://dx.doi.org/10.4103/ijri.IJRI_377_19DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7240899PMC

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

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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

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

Mayer-Rokitansky-Küster-Hauser syndrome as an interdisciplinary problem.

Adv Clin Exp Med 2020 Apr;29(4):505-511

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

The Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome, also known as Müllerian agenesis or aplasia, is a congenital disease manifested by the aplasia of the uterus and the upper 2/3 of the vagina; its incidence is 1 in 4,000-5,000 female live births. We can distinguish 2 types of the MRKH syndrome: type I, which is characterized by an isolated absence of 2/3 of the vagina and uterus; and type II or MURCS (Müllerian duct aplasia, unilateral renal agenesis and cervicothoracic somite anomalies), which is also associated with other symptoms. The treatment of the MRKH syndrome patients aims at creating a neovagina and enabling sexual intercourse. Read More

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http://dx.doi.org/10.17219/acem/118850DOI Listing

Pregnancy and Childbirth in Uterus Didelphys: A Report of Three Cases.

Medicina (Kaunas) 2020 Apr 23;56(4). Epub 2020 Apr 23.

Department of Gynecologic Oncology, Medical University Pleven, 5800 Pleven, Bulgaria.

Uterus didelphys is a rare form of congenital anomaly of the Müllerian ducts. The clinical significance of this anomaly of the female reproductive tract is associated with various reproductive issues: increased risk of preterm birth before 37 weeks' gestation, abnormal fetal presentation, delivery by caesarean section, intrauterine fetal growth restriction, low birth weight less than 2500 g, and perinatal mortality. We present three cases of uterus didelphys and full-term pregnancy, which resulted in favorable birth outcomes of live-born, full-term infants. Read More

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http://dx.doi.org/10.3390/medicina56040198DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7231278PMC

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

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

Anorectal malformation with didelphys uterus: Extremely rare anomaly and successful neoanal sphincter reconstruction with gracilis muscle flap.

Arch Plast Surg 2020 May 10;47(3):272-276. Epub 2020 Apr 10.

Division of Colorectal Surgery, Department of Surgery, Phramongkutklao Hospital, Phramongkutklao College of Medicine, Bangkok, Thailand.

Anorectal malformation or imperforate anus is a congenital anomaly of rectum and anus. Mullerian duct anomalies are abnormal development of uterus, cervix, and vagina. Imperforate anus with double uterus is extremely rare and cannot explain by normal embryologic development. Read More

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http://dx.doi.org/10.5999/aps.2019.01032DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7264905PMC

Avoiding the fatal misdiagnosis of pregnancy in a noncommunicating rudimentary horn using 3D transvaginal ultrasound.

J Clin Ultrasound 2020 Apr 7. Epub 2020 Apr 7.

Department of Radiology, Wayne State University School of Medicine, Detroit, Michigan, USA.

Rudimentary horn pregnancies are an increasingly recognized complication of Mullerian duct anomalies. They can lead to uterine rupture with severe maternal morbidity and mortality. We present the case of a 28-year-old woman with a prior surgical diagnosis of bicornuate uterus who was diagnosed with a 7-week pregnancy in the left horn of a bicornuate uterus by 2D ultrasound. Read More

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http://dx.doi.org/10.1002/jcu.22836DOI Listing

MRI of female genital tract congenital anomalies: European Society of Urogenital Radiology (ESUR) guidelines.

Eur Radiol 2020 Aug 27;30(8):4272-4283. Epub 2020 Mar 27.

Department of Radiology, Universitätsklinikum Salzburg, PMU, Müllner-Hauptstr. 48, A-5020, Salzburg, Austria.

Objective: To develop imaging guidelines for the MR work-up of female genital tract congenital anomalies (FGTCA).

Methods: These guidelines were prepared based on a questionnaire sent to all members of the European Society of Urogenital Radiology (ESUR) Female Pelvic Imaging Working Group (FPI-WG), critical review of the literature and expert consensus decision.

Results: The returned questionnaires from 17 different institutions have shown reasonable homogeneity of practice. Read More

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http://dx.doi.org/10.1007/s00330-020-06750-8DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7338830PMC
August 2020
4.014 Impact Factor

Müllerian duct anomalies coincident with endometriosis: a review.

Abdom Radiol (NY) 2020 06;45(6):1723-1740

Mayo Clinic Rochester Department of Radiology, 200 1st St SW, Rochester, MN, 55905, USA.

The association between obstructed müllerian duct anomalies and endometriosis has been well established and the pathogenesis is attributed to the theory of retrograde menstruation. However, this relationship with endometriosis is less clear in women with unobstructed müllerian duct anomalies and in those with rudimentary uterine structures that lack functioning endometrial tissue. This article reviews the embryology, genetics, pathophysiology, and American Society for Reproductive Medicine (ASRM) classification for müllerian duct anomalies together with the genetics and pathophysiology of endometriosis to provide a framework for understanding the complex relationship between these two entities. Read More

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http://dx.doi.org/10.1007/s00261-020-02465-yDOI Listing

Persistent Müllerian duct syndrome: an update.

Reprod Fertil Dev 2019 Jul;31(7):1240-1245

INSERM UMRS 938, Centre de Recherche Saint Antoine, 27, rue Chaligny, 75571 Paris Cedex 12, France; and Faculté de Médecine Sorbonne Université, 27, rue Chaligny, 75571 Paris Cedex 12, France.

Male sex differentiation is driven by two hormones, testosterone and anti-Müllerian hormone (AMH), responsible for regression of Müllerian ducts in male fetuses. Mutations inactivating AMH or AMH receptor type 2 (AMHR2) are responsible for persistent Müllerian duct syndrome (PMDS) in otherwise normally virilised 46,XY males. This review is based on published cases, including 157 personal ones. Read More

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http://dx.doi.org/10.1071/RD17501DOI Listing

Typical and atypical pelvic MRI characteristics of Mayer-Rokitansky-Küster-Hauser syndrome: a comprehensive analysis of 201 patients.

Eur Radiol 2020 Jul 5;30(7):4014-4022. Epub 2020 Mar 5.

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

Objectives: To comprehensively evaluate the pelvic magnetic resonance imaging (MRI) findings of Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome and summarize the typical and atypical characteristics.

Methods: A retrospective analysis of 201 consecutive MRKH patients was carried out. Pelvic MRI was reviewed by two experienced gynecological radiologists in consensus. Read More

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http://dx.doi.org/10.1007/s00330-020-06681-4DOI Listing

Diagnosis and treatment of müllerian malformations.

Taiwan J Obstet Gynecol 2020 Mar;59(2):183-188

Center for Women's Health, University Hospital Professor Edgard Santos, Federal University of Bahia (UFBA), Salvador, Bahia, Brazil; Department of Gynecology and Obstetrics, Faculty of Medicine, Federal University of Bahia (UFBA), Salvador, Bahia, Brazil.

Anomalies in the müllerian ducts are congenital alterations with more prevalence than it is imagined, varying from 0.5 to 6.7% in the general population and up to 16. Read More

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http://dx.doi.org/10.1016/j.tjog.2020.01.003DOI Listing

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

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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

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

Sequence variant in the CDC42BPB gene is potentially associated with Mullerian duct anomalies.

J Obstet Gynaecol Res 2020 May 10;46(5):684-693. Epub 2020 Feb 10.

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

Aim: Mullerian duct anomalies (MDA) are common female genital tract malformations. Genetic and environmental factors are important causes of MDA in women. Although many genes and mutations have been found to be associated with the pathogenesis of MDA, in most cases, the genetic pathogenic factors of MDA are still unknown. Read More

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http://dx.doi.org/10.1111/jog.14211DOI Listing

Ruptured Ectopic Pregnancy in an Accessory Horn of Uterus: A Case Report.

Cureus 2019 Dec 21;11(12):e6436. Epub 2019 Dec 21.

Emergency Medicine, Civil Hospital Karachi, Karachi, PAK.

A unicornuate uterus with an accessory horn is one of the rarest congenital uterine anomalies; hence, the possibility of ectopic pregnancy in the accessory uterine horn is highly uncommon. It poses a significant risk to maternal life, as it is difficult to identify before surgery due to the severe hemoperitoneum in the event of rupture of the ectopic pregnancy. We report a case of a 20-year-old primigravida who presented to the emergency department of Civil Hospital Karachi, with sudden onset of generalized abdominal pain, vomiting, and dizziness at 17 weeks of gestation. Read More

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http://dx.doi.org/10.7759/cureus.6436DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6970437PMC
December 2019

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

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

An unusual appearance of the post-pubertal Herlyn-Werner-Wunderlich syndrome with acute abdominal pain: A case report.

Int J Reprod Biomed (Yazd) 2019 Dec 26;17(11):851-856. Epub 2019 Dec 26.

Department of Obstetrics and Gynecology, Trauma Research Center, Baqiyatallah University of Medical Sciences, Tehran, Iran.

Background: Herlyn-Werner-Wunderlich (HWW) syndrome is a rare congenital urogenital defect. It is detected by unilateral low vaginal obstruction, uterus didelphys, and ipsilateral kidney agenesis. It usually becomes apparent with pain, dysmenorrhea, and presence of a vaginal or pelvic mass. Read More

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http://dx.doi.org/10.18502/ijrm.v17i10.5498DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6906875PMC
December 2019

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

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December 2019

Transvers testicular ectopia: A case report and literature review.

Int J Surg Case Rep 2019 12;65:361-364. Epub 2019 Nov 12.

Department of Pediatric Surgery, EMSEY Hospital, Çamlık Mah, Selçuklu Cad. No: 22 Pendik, İstanbul, Turkey. Electronic address:

İNTRODUCTION: Transverse testicular ectopia (TTE) is a rare anomaly characterized by the presence of both testicles in the same hemiscrotum or inguinal region. The most common clinical findings of TTE are unilateral nonpalpable testis in the scrotum and inguinal hernia on the side of palpable testis in the scrotum. It should be kept in mind that TTE may coexist with Persistent Mullerian Duct Syndrome (PMDS). Read More

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http://dx.doi.org/10.1016/j.ijscr.2019.11.007DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6920318PMC
November 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

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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

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

Leiomyomas in a case of Mayer-Rokitansky-Kuster-Hauser syndrome: Case report.

Eur J Obstet Gynecol Reprod Biol 2020 Jan 31;244:199-200. Epub 2019 Oct 31.

Department of Surgery, Medical College, Jouf University, KSA, Al-Azhar University, Egypt. Electronic address:

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http://dx.doi.org/10.1016/j.ejogrb.2019.10.043DOI Listing
January 2020
1 Read

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

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

Pelvic pain in patients with complex mullerian anomalies including Mayer-Rokitansky-Kuster-Hauser syndrome (MRKH), obstructed hemi-vagina ipsilateral renal anomaly (OHVIRA), and complex cloaca.

Semin Pediatr Surg 2019 Oct 13;28(5):150842. Epub 2019 Sep 13.

Division of Pediatric Surgery, Phoenix Children's Hospital, 1919 E. Thomas Rd., Phoenix, AZ 85016, United States. Electronic address:

Caring for patients with congenital pelvic anomalies can be challenging in many ways but one crucial aspect is providing longitudinal into adulthood. Newborns with urinary, intestinal or vaginal obstruction require urgent operations to relieve obstruction followed by multiple reconstructive procedures involving the perineum. Openings are created in the pelvic floor musculature that did not exist in development. Read More

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http://dx.doi.org/10.1016/j.sempedsurg.2019.150842DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6936264PMC
October 2019

Comparison of neovaginoplasty using acellular porcine small intestinal submucosa graft or Interceed in patients with Mayer-Rokitansky-Küster-Hauser syndrome.

Arch Gynecol Obstet 2019 12 30;300(6):1633-1636. Epub 2019 Oct 30.

Department of Gynecology, Obstetrics and Gynecology Hospital, Fudan University, 128 Shen yang Road, Shanghai, 200090, China.

Objective: To compare using the acellular porcine small intestinal submucosa (SIS) graft or the Interceed in patients with MRKH syndrome undergoing creation of a neovagina.

Methods: In this retrospective study, patients with MRKH syndrome undergoing creation of a neovagina from 2016 to 2018 were retrospectively investigated. Wharton-Sheares-George neovaginoplasty was performed using the acellular porcine SIS graft or the Interceed. Read More

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http://dx.doi.org/10.1007/s00404-019-05352-0DOI Listing
December 2019

Long-term follow-up on MURCS (Müllerian duct, renal, cervical somite dysplasia) association and a review of the literature.

Ann Pediatr Endocrinol Metab 2019 Sep 30;24(3):207-211. Epub 2019 Sep 30.

Department of Pediatrics, Inha University Graduate school of Medicine, Inha University Hospital, Incheon, Korea.

Müllerian duct aplasia-renal aplasia-cervicothoracic somite dysplasia (MURCS) association is a unique development disorder with four common types of malformations that include uterine aplasia or hypoplasia, renal ectopy or agenesis, vertebral anomalies, and short stature. The majority of MURCS patients are diagnosed with primary amenorrhea from late-adolescence. However, a few cases with MURCS association are not well diagnosed during childhood and long-term outcomes are not well reported. Read More

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http://dx.doi.org/10.6065/apem.2019.24.3.207DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6790868PMC
September 2019
1 Read

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

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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

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

Müllerian Remnant Embedded in Labia Majora.

Urology 2019 Dec 24;134:209-212. Epub 2019 Sep 24.

Children's Hospital of Los Angeles.

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http://dx.doi.org/10.1016/j.urology.2019.09.013DOI Listing
December 2019

An Unusual Cause of Recurrent Urinary Retention in an Adolescent Female.

Cureus 2019 Jul 15;11(7):e5136. Epub 2019 Jul 15.

Pediatrics, The Brooklyn Hospital Center, New York, USA.

Herlyn-Werner-Wunderlich syndrome (HWWS) is a rare, combined Mullerian and Mesonephric duct anomaly characterized by the triad of uterus didelphys, obstructed hemivagina and ipsilateral renal agenesis. We present the case of an otherwise healthy 16-year-old female with acute urinary retention secondary to HWWS. The diagnosis was established with abdominal ultrasound and Magnetic Resonance Imaging (MRI). Read More

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http://dx.doi.org/10.7759/cureus.5136DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6741396PMC
July 2019
1 Read

Persistent Mullerian Duct Syndrome with Polysplenia and Short Pancreas: A Case Report.

JNMA J Nepal Med Assoc 2019 Mar-Apr;57(216):123-126

Department of Urology, B&C Medical College Teaching Hospital, Birtamod, Jhapa, Nepal.

Persistent Mullerian duct syndrome is a rare entity and usually presents with common symptoms of undescended testis and hernia. The syndrome is caused by an insufficient amount of Mullerian inhibiting substance or due to the insensitivity of the target organ to Mullerian inhibiting substance. Polysplenia is a rare congenital disease manifested by multiple small accessory spleens. Read More

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February 2020
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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

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February 2020
2 Reads

Goldenhar syndrome associated with genital tract abnormality.

BMJ Case Rep 2019 Aug 28;12(8). Epub 2019 Aug 28.

Baylor College of Medicine, Houston, Texas, USA.

Goldenhar syndrome, also known as , has been described since 1952. Traditionally, the syndrome has been described as having eye, ear, facial and vertebral anomalies. However, numerous case reports and reviews have highlighted multi-organ involvement, including cardiovascular, gastrointestinal, respiratory system and urinary abnormalities. Read More

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http://dx.doi.org/10.1136/bcr-2019-230686DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6720629PMC

Herlyn-Werner-Wunderlich syndrome: A rare cause of abdominal pain and dyspareunia.

Radiol Case Rep 2019 Oct 20;14(10):1297-1300. Epub 2019 Aug 20.

Department of Radiology, SUNY Upstate Medical University, 750 East Adam Street, Syracuse, NY, 13210, USA.

Herlyn-Werner-Wunderlich (HWW) syndrome is a rare congenital disease characterized by Mullerian duct anomalies. The characteristic triad of this syndrome includes didelphys uterus, obstructed hemivagina, and ipsilateral renal agenesis (hence, also known as Obstructed Hemivagina and Ipsilateral Renal Agenesis or OHVIRA syndrome). The clinical presentation of HWW is nonspecific and patients may present with abdominal/pelvic pain, dyspareunia, or dysmenorrhea. Read More

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http://dx.doi.org/10.1016/j.radcr.2019.08.003DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6710296PMC
October 2019
1 Read

Reproductive surgery for müllerian anomalies: a review of progress in the last decade.

Fertil Steril 2019 09;112(3):408-416

Ronald O. Perelman and Claudia Cohen Center for Reproductive Medicine, Weill Cornell Medicine, New York, New York. Electronic address:

Disorders of development, fusion, or resorption of paired müllerian ducts and urogenital sinus may cause various congenital malformations of the corpus uteri, cervix, vagina, and fallopian tubes. Classification systems have been developed to better characterize these anomalies, and each has advantages and disadvantages. Clinical correlation of classification of anomalies with pre- and postsurgical outcomes is needed to better direct treatment. Read More

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https://linkinghub.elsevier.com/retrieve/pii/S00150282193057
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http://dx.doi.org/10.1016/j.fertnstert.2019.07.005DOI Listing
September 2019
3 Reads

Congenital Uterine Malformation by Experts (CUME): diagnostic criteria for T-shaped uterus.

Ultrasound Obstet Gynecol 2020 Jun 15;55(6):815-829. Epub 2020 May 15.

SEMEAR Fertilidade, Reproductive Medicine, Ribeirão Preto, Brazil.

Objectives: To identify uterine measurements that are reliable and accurate to distinguish between T-shaped and normal/arcuate uterus, and define T-shaped uterus, using Congenital Uterine Malformation by Experts (CUME) methodology, which uses as reference standard the decision made most often by several independent experts.

Methods: This was a prospectively planned multirater reliability/agreement and diagnostic accuracy study, performed between November 2017 and December 2018, using a sample of 100 three-dimensional (3D) datasets of different uteri with lateral uterine cavity indentations, acquired from consecutive women between 2014 and 2016. Fifteen representative experts (five clinicians, five surgeons and five sonologists), blinded to each others' opinions, examined anonymized images of the coronal plane of each uterus and provided their independent opinion as to whether it was T-shaped or normal/arcuate; this formed the basis of the CUME reference standard, with the decision made most often (i. Read More

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http://dx.doi.org/10.1002/uog.20845DOI Listing
June 2020
3 Reads

Robotic-assisted Excision of Giant Prostatic Utricular Cysts: Technique, Outcomes and Follow-up.

Surg Technol Int 2019 11;35:43-47

Department of Urology, University Hospital Munich-Grosshadern, Ludwig-Maximilians-University Munich, Munich, Germany.

Background: Utricular cysts are usually the result of incomplete involution of the Müllerian ducts and may have no urethral connection. Treatment options for symptomatic utricular cysts include an open abdominal approach, trans-vesical excision, vasoligation without excision and laparoscopic/robotic-assisted excision.

Patients And Methods: Two patients (14 and 45 years old) with recurrent urinary tract infections associated with recurrent lower urinary tract irritative symptoms and recurrent perineal pains presented to our department. Read More

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November 2019

Evaluation of the abdominopelvic region using MRI in patients with primary amenorrhea.

J Pediatr Endocrinol Metab 2019 Sep;32(9):995-1003

Eskisehir Osmangazi University, Faculty of Medicine, Department of Radiology, Odunpazarı, 26480 Eskişehir, Turkey.

Background This study aimed to evaluate the imaging findings of patients who underwent an abdominal and pelvic magnetic resonance imaging (MRI) due to primary amenorrhea. Methods The pelvic and abdominal images of 34 female patients (mean age 15.61 years, range 14-19 years) were retrospectively analyzed by a single radiologist blinded to the clinical and laboratory data of the patients (other than primary amenorrhea) to evaluate the etiology of primary amenorrhea. Read More

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http://dx.doi.org/10.1515/jpem-2019-0223DOI Listing
September 2019
3 Reads

Asymptomatic microperforated transverse vaginal septum presenting with primary infertility: a rare form of mullerian anomaly.

Turk J Obstet Gynecol 2019 Jun 3;16(2):140-142. Epub 2019 Jul 3.

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

Transverse vaginal septum is a rare type of mullerian anomaly resulting from failure of the canalization of the vaginal plate at the point where the urogenital sinus meets the mullerian duct and usually presents at menarche with symptoms of outflow tract obstruction. Instead, patients with a perforated septum often have normal menses and usually present with difficulties with intercourse or infertility. A 24-year-old patient with 5 years of infertility is reported. Read More

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http://dx.doi.org/10.4274/tjod.galenos.2019.32956DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6637785PMC
June 2019
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

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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

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http://dx.doi.org/10.1016/j.tjog.2019.05.023DOI Listing
July 2019
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Carcinoma of the lower female genital tract in patients with genitourinary malformations: a clinicopathologic analysis of 36 cases.

J Cancer 2019 2;10(13):3054-3061. Epub 2019 Jun 2.

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

: Lower genital tract carcinomas that coexist with genitourinary malformations are rare. The aim of this study was to investigate the clinicopathologic characteristics and outcomes of patients with this rare joint condition. The aim of this study was to investigate the clinicopathologic characteristics and outcomes of these patients to analyze the associations among clinicopathologic features in this rare entity. Read More

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http://www.jcancer.org/v10p3054.htm
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http://dx.doi.org/10.7150/jca.30486DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6590040PMC
June 2019
5 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

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

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

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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

The Modified McIndoe Technique: A Scar-free Surgical Approach for Vaginoplasty With an Autologous Micromucosa Graft.

Urology 2019 Sep 28;131:240-244. Epub 2019 May 28.

Department of Plastic and Reconstructive Surgery, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, People's Republic of China.

Objective: To evaluate the effect of a scar-free surgical approach, the modified McIndoe technique, in cases of vaginal agenesis.

Materials And Methods: Seven patients with Mayer-Rokitansky-Kuster-Hauser syndrome underwent vaginoplasty with the modified McIndoe technique. Mucosa grafts harvested from the vulva were minced into small particles and transplanted to the neovagina. Read More

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http://dx.doi.org/10.1016/j.urology.2019.05.020DOI Listing
September 2019
8 Reads
2.188 Impact Factor